Download NOPHO / NOBOS Progr amme and Abstr act Book

NOPHO / NOBOS
ANNUAL MEETING
9 – 1 3 M a y 2 0 1 4 – B e r g e n – N o r w a y
Progr amme an d A bs t r act Book
O
r ganis i n g c o m m itte e s
NOPHO
Maria Winther Gunnes head of organising committee
Martha Dirdal
Dorota Wojcik
NOBOS
Sunniva Helland head of organising committee
Marianne Bentzen
Marianne Bøe
Ida Kari Ivarhus
Kjerstin Mongstad
Jorid Skjenken
Britt-Ingunn Wee Sævig
PCO
Inger Lise Ravnanger
Trine Haatuft Mjellem
Bergen Kongress & Kultur AS
kongress.no
NOPHO / NOBOS
ANNUAL MEETING
9 – 1 3 M a y 2 0 1 4 – B e r g e n – N o r w a y
Contents
Organising committees .............................................. 2
Practical information ............................................... 9
Welcome ....................................................................... 11
Sponsors and exhibitors .......................................... 12
Map of Bergen . . .......................................................... 18
Social programme .. .................................................... 20
NOPHO programme ................................................... 22
Invited speakers ................................................... 28
Oral presentations . . ............................................ 42
Poster presentations .. ......................................... 62
NOBOS programme .................................................... 92
Keynote speakers . . ............................................... 96
Oral presentations . . .......................................... 102
Poster presentations .. ........................................ 114
Cover illustration: Malerduoen Bringager & Malmstrøm
Design and layout: IMP kommunikasjon / Sviggum
2
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
3
jernkontroll.
hver dag.
Rare?
It depends
how you look at it
jernkontroll.
hver dag.
To us, there is no difference between
a disease affecting 300 million people
and a disease affecting 300 people
Dedicated to providing new therapies and new hope
to people suffering from rare and neglected diseases
www.legemiddelverket.no
NO1203029754
NO1203029754
sigma-tau.co.uk
Sigma-Tau Pharma Ltd. 2014-ST-006 April 2014
An expertly written and
practical handbook
Management of children with
inherited and acquired bleeding
and clotting disorders
Pediatric and Adolescent Medicine
Editors: D. Branski, W. Kiess
Vol. 17
Controversies in
Pediatric and
Adolescent Hematology
Editors
A.E. Thomas
C. Halsey
SickKids Handbook
of Pediatric Thrombosis and
Hemostasis
Pediatric and
Adolescent Hematology
Editors:
Victor S. Blanchette, Toronto, Ont.
Vicky R. Breakey, Hamilton, Ont.
Shoshana Revel-Vilk, Jerusalem
Editors:
Angela E. Thomas,, Edinburgh
Christina Halsey,, Glasgow
XVIII + 254 p., 23 fig., 9 in color, 9 algorithms, 59 tab., soft cover, 2013
CHF 98.– / EUR 82.– / USD 115.00
ISBN 978–3–318–02197–4
Written and reviewed by international experts in the field, this
handbook is intended for health care professionals involved in
the assessment and care of children with inherited and acquired
bleeding and clotting disorders, including general and specialist
pediatricians (in particular intensivists, neonatologists, cardiologists/cardiac surgeons, rheumatologists and nephrologists), hematologists/oncologists (pediatric and adult), as well as medical
trainees, nurses, nurse practitioners and pharmacists.
www.karger.com/sickkids
KI14659
Prices subject to change
EUR price for Germany, USD price for
USA and Latin America only
Karger – Medical and Scientific Publishers
CH–4009 Basel, Switzerland
[email protected], f: +41 61 306 12 34
www.karger.com
VIII + 178 p., 13 fig., 19 tab., hard cover, 2014
List price: CHF 148.– / EUR 123.– / USD 174.00
Special price: CHF 104.– / EUR 86.– / USD 122.00
ISBN 978–3–318–02422–7
In this book an internationally acclaimed panel of authors, each
chosen for expertise in their field, have produced a state-ofthe-art collection of review articles focusing on the very latest
advances and controversies in the management of pediatric and
adolescent hematological problems.
Providing an up-to-date look at both specific hematologic disorders and also hematologic problems that arise in association
with systemic disease, this book is essential reading not only for
pediatric and adult haematologists but also for pediatricians,
pediatric or hematologic specialist nurse practitioners and pediatric pharmacologists.
Go to www.karger.com/pamed17 and use
the promotional code PedHem14 to profit from this
special offer.
Hjælp
danske børn med kræft
Søg midler til forskning, uddannelse,
møde- og konferencedeltagelse
Læs vejledningen på
www.boernecancerfonden.dk
Ansøgningsfrist den 1. oktober hvert år
Meze Design Photo: David Bicho
NOPHO / NOBOS
ANNUAL MEETING
Vi tilbyr verdifulle
legemidler til
pasienter med
sjeldne sykdommer
Sobi fører og utvikler innovative legemidler for å hjelpe
pasienter med sjeldne sykdommer og store medisinske
behov.
Viktige behandlingsområder er blodsykdommer,
inflammasjonssykdommer, genetiske sykdommer og
kreftterapi.
Innen disse områdene ønsker vi å øke kunnskapen om
sjeldne sykdommer gjennom våre nettverk med pasienter
og deres familier, helsevesenet og myndigheter, og på den
måten bidra til et bedre liv for pasientene.
Sobi har flere samarbeidsprosjekter med OCC innen kreft
og blodkreft behandling. Våre forskningsprosjekter i sen
klinisk fase er primært rettet mot nye legemidler innenfor
hemofili A og B, samt forebygging av veksthemming hos
for tidlig fødte barn.
Fakta om Sobi
Swedish Orphan Biovitrum, Sobi, er et ledende europeisk
spesiallegemiddelfirma der hele verdikjeden fra forskning
og utvikling, produksjon, distribusjon, markedsføring og
kundestøtte er representert.
Vår produktportefølje består av mer enn 40 markedsførte
produkter og flere prosjekter i sen klinisk fase.
Vår organisasjon dekker ett 20-talls land i Europa samt våre
nyetablerte datterselskaper i USA og Midt-Østen. Vi er også
representert gjennom partnere i Israel, Sør-Korea, Australia
og New Zealand.
9 – 1 3 M a y 2 0 1 4 – B e r g e n – N o r w a y
Pr act ical informat ion
Meeting venue
Radisson Blu Royal Hotel, Bergen
Bryggen 5, 5003 Bergen
radissonblu.com/royalhotel-bergen
The airport shuttle bus stops directly outside the
conference venue. The driver will announce the stop
upon request.
Registration and Conference Secretariat
Saturday May 10 ........................... 16.00–19.00
Sunday May 11 ............................. 07.30–10.00
Name badge
Your name badge gives you admission to the scientific
sessions, lunch and coffee breaks. Your badge must be
worn at all times at the conference venue.
Lost/misplaced badges can be replaced at the
Registration desk at a cost of NOK 50.
Official language
The official working language of the meeting is English.
Mer informasjon finnes på www.sobi.com
Coffee breaks
Coffee is served in the exhibition area.
Lunch
A two course lunch will be served Sunday and Monday,
and a light lunch will be served before departure on
Tuesday.
N O P H O / N O B O S
www.sobi.com
M E E T I N G
Internet access
There is free wi-fi at the congress venue.
Log on information is:
Network name: Meeting Delegate
Password: JvzU | VspJ
( four letters in two separate boxes )
Smoking policy
The congress venue is entirely non smoking.
Presentations ( invited speakers )
Presentations should be handed to the technician at
the latest 2 hours before your session starts.
Tourist information in Bergen
The Tourist information at Vågsalmenningen 1 (near
the Fish Market) is open daily 08.30–22.00.
Trip up Mount Fløien
While in Bergen we recommend taking the funicular
up Mount Fløien, and would therefore like to offer
you tickets for free. These tickets will be available at
the registration desk from Sunday after 12.00.
Shopping
The shops at Bergen Storsenter and Galleriet are open
Mon–Fri 09.00–21.00 and Sat 09.00–18.00.
Transport to Bergen Airport Flesland
Taxi: The taxi fare to Bergen Airport Flesland (20 km)
is about NOK 450.
The airport bus: (Flybussen) departs every 15 min
from Radisson Blu Royal Hotel and costs NOK 90
for a one way ticket.
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
9
Welcome
Dear NOPHO/NOBOS friends, colleagues, sponsors and invited guests
and speakers
On behalf of the organising committee and the Pediatric
Oncology Department at Haukeland University
Hospital we would like to welcome you all to the
32nd Annual NOPHO meeting and the 10th Biannual
NOBOS meeting! We are honored to host this event
here in beautiful Bergen, Norway’s second largest city.
Bergen was founded by King Olav Kyrre in 1070, and
was part of the Hanseatic League from the 14th century
until around 1750, and one of four Hanseatic League
centres were found in Bergen. Today, Bergens old
quayside, Bryggen, is part the UNESCO list of World
Heritage Sites, and we are proud to host the conference in the SAS Radisson Hotel situated at Bryggen.
The Pediatric Oncology Department at Haukeland
University Hospital is Norway’s second largest pediatric
oncology department, and is responsible for pediatric
cancer and hematology patients from the wide geographical area of western Norway, covering the counties
of Hordaland, Rogaland and Sogn og Fjordane.
We are pleased to present a varied and interesting scientific program, with well recognized invited speakers
in their respective fields, from across the globe.
We would like to thank all of our sponsors and contributors, especially our Gold Sponsors; this meeting would
not have been possible without the generous support
by all of our collaborating partners.
We hope you will all have a wonderful scientific conference as well as fruitful social gatherings where you
will continue to develop relations with your fellow
members of the NOPHO/NOBOS family!
On behalf of the Organising Committee
Maria Winther Gunnes
NOPHO
10
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
Photo: Bergen Tourist Board/Robin Strand - visitBergen.com
N O P H O / N O B O S
M E E T I N G
Sunniva Helland
NOBOS
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
11
Hjælp
Viktige behandlingsområder er blodsykdommer,
inflammasjonssykdommer, genetiske sykdommer og
kreftterapi.
Innen disse områdene ønsker vi å øke kunnskapen om
sjeldne sykdommer gjennom våre nettverk med pasienter
og deres familier, helsevesenet og myndigheter, og på den
måten bidra til et bedre liv for pasientene.
Spo n s o r s a n d e x hibitor s
danske børn med kræ
Sobi har flere samarbeidsprosjekter med OCC innen kreft
og blodkreft behandling. Våre forskningsprosjekter i sen
klinisk fase er primært rettet mot nye legemidler innenfor
hemofili A og B, samt forebygging av veksthemming hos
for tidlig fødte barn.
Gold Sponsors
Exhibitors
Fakta om Sobi
GlaxoSmithKline
Swedish Orphan Biovitrum, Sobi, er et ledende europeisk
spesiallegemiddelfirma der hele verdikjeden fra forskning
og utvikling, produksjon, distribusjon, markedsføring og
kundestøtte er representert.
MSD Norge
Light Integra Technology
Vår produktportefølje består av mer enn 40 markedsførte
produkter og flere prosjekter i sen klinisk fase.
Vår organisasjon dekker ett 20-talls land i Europa samt våre
nyetablerte datterselskaper i USA og Midt-Østen. Vi er også
representert gjennom partnere i Israel, Sør-Korea, Australia
og New Zealand.
Søg midler til forskning, uddannelse,
mødeog konferencedeltagelse
Pierre Fabre
Pharma Norden
Læs vejledningen
på
Therakos
www.boernecancerfonden.dk
Medac
Ansøgningsfrist den 1. oktober hvert år
Mer informasjon finnes på www.sobi.com
Other Sponsors
Grimsgaards stiftelse
Gilead
Hordaland fylkeskommune
Helse vest
Mary Béves stiftelse
Strømberggruppen as
Bronze Sponsors
GALEN
07.08.13 15.39
24112_170x240_BCF_annonce.indd 1
Advancing Human Health
KIWANIS CLUB SOTRA
12
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
13
Welcome t o Bergen
– G at eway t o t he Fjords
Bergen is located on the south-western coast of Norway, with its
centre situated between a group of mountains known collectively as
De syv fjell ( ”The seven mountains” ).
Fløibanen Photo: Pål Hoff - visitBergen.com
Bergen is host to part of the country’s large oil industry,
as well as deep sea operations. Its harbours are used by
everything from small pleasure vessels to cruise ships
and cargo vessels, and are the base of many of the
country's fishing vessels.
Bergen has been a focal point of cultural and commercial
activity from medieval to modern times. Its population
of 272 000 proudly invites you to share the bustling
14
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
Photo: Bergen Tourist Board / Willy Haraldsen - visitBergen.com
N O P H O / N O B O S
M E E T I N G
Zachariasbryggen Photo: Bergen Tourist Board / Robin Strand
maritime atmosphere, the rich architectural heritage
and the cultural and academic institutions of their
home city. Located in the heart of the famous fjords,
Bergen is also known as the Gateway to the Fjords.
We are certain that the City of Bergen would give the
participants a long lasting memory as and extended
value; this includes the scenery, the old Hanseatic quay
and housing structure.
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
15
Photo: Bergen Tourist Board / Per Nybø
Photo: Bergen Tourist Board / Jan M. Lillebø
Tips from the locals
Restaurants:
• Matbørsen, Småstrandgaten; close to Radisson
Bryggen and the fish market, an Old Stock market
with unique fresco paintings from 1918–1923, now
“Food Market” with several restaurants
• Bølgen & Moi, Vågsalmenningen 16;
close to Radisson Bryggen and the fish market
• Lysverket, Rasmus Meyers Allè 9.
Close to the museums and “lille Lungårdsvann”.
A quite new restaurant and probably the best in town
at the moment. Pricy.
• Nama Japanese Fusion, Lodin Lepps gt 2B.
Close to Bryggen, best sushi restaurant in town
• Enhjørningen, Bryggen.
One of the best seafood restaurants in Bergen
• Bryggeloftet, Bryggen.
Traditional Bergen restaurant, located at Bryggen
• Potetkjelleren, Kong Oscars gt. 1B.
Traditionally one of the best restaurants in town. Pricy.
16
N O P H O / N O B O S
M E E T I N G
A N D
• Restaurant 1877, Kjøttbasaren, Vetrlidsalmenning 2.
Close to Bryggen and the fish marked
• Maharaja, Rosenkranzgt 5.
Close to Bryggen. Good Indian food.
• Red Sun, Kong Oscars gt 4.
Close to Bryggen. Asian Fusion.
• Escalon, Vetrlidsalmenningen 21.
Close to Bryggen. Very good Tapas food.
Bars:
• Don Pippo, Christies gt 11;
small, cosy wine bar in city centre
• Altona, C. Sundts gt 22;
old cellar, now wine bar, with lots of atmosphere
• Henrik, Engen 10. Close to the theatre, laid-back
atmosphere, the bar in town with the largest number
on microbrewery beers on tap
• Pingvinen, Vaskerelven 14.
Cool retro bar in the city centre
C O N G R E S S
–
B E R G E N
2 0 1 4
Cafès:
• Kaffemisjonen, Øvre Korskirkealmenning 5;
probably the best coffee in town
• Smaksverket, Rasmus Meyers allè 3; close to the
main museums and “lille Lungårdsvann”
Shopping:
• Galleriet, Torgalmenningen; large shopping centre
in town, by the large square “Torgalmenningen”
• Kløverhuset, Strandgaten 13–15.
• Illums Bolighus
• Skomakerstredet, small, cosy pedestrian street with
speciality shops and second-hand shops
• Bergen Storsenter, large shopping mall by the
railway station
N O P H O / N O B O S
M E E T I N G
Attractions:
• Fløybanen Funicular (tickets free of charge available
from the registration desk), close to Bryggen
• Akvariet, Bergen Aquarium, Nordnesbakken 4
Museums:
• KODE
• Rasmus Meyer
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
17
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Sunday May 11
Soc i al p ro g r a m m e
Saturday May 10
Troldhaugen N O P H O / N O B O S
Photo: Bergen Tourist Board / Per Nybø
M E E T I N G
Cornelius NOBOS Dinner
USF is located on the pier accommodating Bergen's
largest outdoor restaurant in the summertime, where
500 guests can enjoy the most beautiful fjord views.
USF V erftet at 6 pm
NOPHO dinner
Cornelius has an idyllic location, right on the waterfront
just outside Bergen. Here, both fishingboats and divers
deliver their best catch of the day.
boat departs at 7 pm
Photo: Truls J. Løtvedt
Monday May 12
Guided tour of the composer Edvard Griegs home,
including concert.
Aproximate duration 2 hrs. Small extra cost ( 100
NOK ) for participation, NB! Limited spaces available!
B us departs at 2 pm from R adisson B lu R oyal
20
USF Verftet A N D
Bryggen Meet-and-greet and poster session
Come and enjoy a bit of socialising with colleagues
and friends before exploring the city on your own for
the rest of the evening!
7–9 pm at R adisson B lu R oyal H otell
C O N G R E S S
–
B E R G E N
Grieghallen Photo: Bergen Tourist Board / Robin Strand
2 0 1 4
NOBOS / NOPHO Fun and Run
Rise and shine early to join the fun. Meeting place
will be outside Radisson Blu Royal Hotell Bryggen.
Shades are optional.
R adisson B lu R oyal H otell at 6.30 am
N O P H O / N O B O S
M E E T I N G
Photo: Bergen Tourist Board / Willy Haraldsen
The annual NOBOS / NOPHO dinner
The annual dinner will be held at Grieghallen in the
city centre. Dinner, entertainment and dancing until
the late hours!
G rieghallen at 7.30 pm
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
21
S u n d ay 11 M ay
08:30–09:00 Opening ceremony and info from the organizers
09:00–10:30:
Joint session NOPHO/NOBOS
Chair: Britt Ingunn Sævig
How does cancer treatments affect the developing child?
Marianne Straume, Bergen
10:30–11:00: Coffee break
11:00–11:45: Ethical dilemmas when treating seriously ill children
Trond Markestad, Bergen
11:45–12:30
NO P HO p ro g r a m me
Ethics
Chair: Trond Markestad
Free papers
O1 A Nordic platform for clinical ethics in pediatric oncology
Pernille Wendtland Edslev, Aarhus
O2 Treatment decisions in pediatric health care- Who’s the one to decide?
Lisa Törnudd, Linköping
O15 Conceptual clarity of values in end-of-life deliberations in pediatric oncology
Anders Castor, Lund
12:30–13:45 Lunch
13:00–17:00
Infections
Chair: Maria Winther Gunnes
F r i d ay 9 M ay
13:45–14:30: Fever and neutropenia in pediatric cancer patients; is a choice between outpatient v/s
inpatient management a routine one? Critical review and recommendations.
Lillian Sung, Toronto
08:30–12:00 NOPHO Working groups (see separate program)
13:00–17:00 NOPHO Working groups
14:35–15:10 Implementing a diagnostic strategy for management of invasive fungal disease in
hemato-oncology
Samir Agrawal, London
15:10–15:30 Coffee break
S at u r d ay 10 M ay
15:30–16:15 Challenges in the diagnosis and management of invasive fungal infections in 2014
Paul Verweij, Nijmegen
08:30–12:00 NOPHO Working groups
15:45–16:00 Discussion with speakers
13:00–17:00 NOPHO Working groups
13:00–15:00
16:15–16:45
Young NOPHO educational session
Chair: Ellen Ruud
Chair: Marta Dirdal
Free papers
Statistics in clinical trials, challenges and pitfalls
Eva Skovlund, Oslo
Evening
Late-effects
O3 Hospital admissions for respiratory disorders in adult life after childhood cancer
in Scandinavia (ALiCCS) – A population-based cohort study
Thorgerdur Gudmundsdottir, Danish Cancer Society Research Center
Poster session / “Meet-and-greet” 19–21
O4 Diseases of renal function and bone metabolism following treatment of earlyonset cancer. A registry-based study
Marika Grönroos, Turku
Info from organizers
Evening
22
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
NOPHO dinner Cornelius, boat departs at 19:00
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
23
M o n d ay 12 M ay
Tu e s d ay 13 M ay
06:30–08:00 Fun and Run
08:30–10:15 Free papers
08:30–09:15
Sarcomas
Chair: Finn Wesenberg
Leukemia
Chair: Bem Zeller
O5 Hepatic Veno-Occlusive Disease in Children with Acute Lymphoblastic Leukemia
During Maintenance Therapy with Continuous Asparaginase
Silvia De Pietri, Rigshospitalet, Copenhagen
O7 Intra-tumoral heterogeneity of T-ALL leukemic blasts at diagnosis and follow-up;
implications for minimal residual disease detection
Nina Friesgaard Øbro, Rigshospitalet, Copenhagen
O8 Genomic characterization of dic(9;20)(p13.2;q11.2)-positive B-cell precursor acute
lymphoblastic leukemia
Vasilios Zachariadis, Karolinska, Stockholm
Miscellaneous
O9 RESPECT – A feasibility study of a school re-entry, physical- and social activity
intervention for school children with cancer
Anne Sofie Helms, Rigshospitalet, Copenhagen
O10 Flow cytometric measurement of platelet associated immunoglobulin in children
with newly diagnosed immune thrombocytopenia
Ole Haubjerg Nielsen, Aalborg
O11 PPM1D/Wip is a novel neuroblastoma oncogene and potential therapeutic target
in high-risk neuroblastoma
Jelena Milosevic, Karolinska, Stockholm
O12 Pediatric germ cell tumors in Denmark, 1985–2012
Madeline Evers, Rigshospitalet, Copenhagen
10:15–10:45 Coffee break
10:45–11:45 NOPHO Lecture:
Chair: Secretary General NOPHO, Marit Hellebostad
Medulloblastoma today
Birgitta Lannering, Gothenburg
11:45–13:00 Lunch
13:00–17:00 Bone marrow failure / Myelodysplastic syndrome /
Recent developments in osteosarcoma
Stefan Bielack, Stuttgart
09:15–10:00 Free papers
O13 Late mortality among Finnish 5-year survivors of early onset cancer
Päivi Lähtenmäki, Turku
O14 The SALUB registry: survivorship passport and nationwide registration of lateeffects after childhood cancer in Sweden
Cecilia Petersen, Karolinska, Stockholm
O16 Incidence of thrombosis in children treated according to non-HR NOPHO ALL
2008- a prospective single center study
Ellen Ruud, Rikshospitalet, Oslo
10:00–10:45 The SSG central register. 6000 patients after 25 years of monitoring referral and treatment in orthopedic soft tissue sarcoma
Clement Trovik, Bergen
10:45–11:15 Coffee break
11:15–12:00 Free papers
O17 Hyperferritinemia in severely ill patients: associations with clinical and
laboratory findings
Tatiana Greenwood, Karolinska, Stockholm
O18 Thalassemia and Diamond Blackfan Anemia in Sweden. Data from the Nordic
Transfusion Registry
Ulf Tedgård, Skåne University Hospital
O19 Iron deficiency and iron deficiency anemia in toddlers in Oslo. Is there a difference between children of different ethnic backgrounds?
Einar Stensvold, Rikshospitalet, Oslo
12:00
Presentation of next years meeting
12:10
Closing remarks from the organizers
12:15
Lunch bag / small buffet and departure
Stem cell transplantation – Mary Béve Symposium
Chair: Dorota Wojcik
13:00–13:45 Clinical approach to inherited bone marrow failure syndromes
Inderjeet Dokal, London
13:50–14:35 Novel cellular treatment approaches after allo-SCT
Peter Bader, Frankfurt
14:35–15:00 Coffee break
15:00–15:45 Diagnosis and management of childhood MDS and JMML
Henrik Hasle, Århus
15:45–16:00 Panel discussion and info from organizers
16:00–18:00 NOPHO General Assembly
19:30
Annual dinner, Grieghallen
24
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
25
NOP HO W o rk i ng grou ps
Prog r amme
F r i d ay 9 M ay
Activity
Room
#
09:00–12:00 NHL
Dræggen 1
10
12:00–18:00 Young Nopho
Dræggen 3
30
12:00–18:00 LLC
Dræggen 4b
30
16:00–18:00 ALL 2008
Dræggen 4a
20
18:00–19:00 ALL relapse
Dræggen 4a
10
Room
#
07:30–08:50 Novel Therapy Working Group
Dræggen 4a
10
07:30–09:00 ALL 2016
Dræggen 3
10
08:00–10:00 Late effect
Dræggen 4b
15
09:00–13:00 Brain Tumors
Dræggen 4a
14
09:00–12:00 LLC
Dræggen 3
30
13:00–18:00 Nopho board
Dræggen 4b
20
13:00–15:00 Red cell disorder
Dræggen 4a
10
20:00–22:00 Histiocytose
Dræggen 4b
20
Room
#
Dræggen 4a
8
S at u r d ay 10 M ay
Activity
S u n d ay 11 M ay
Activity
07:30–14:00 Thrombosis and hemostasis
26
N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
Photo: Bergen Tourist Board / Per Eide
Inderjeet Dokal
Professor, Centre for Paediatrics, Blizard Institute,
Barts and The London School of Medicine and
Dentistry, Queen Mary University of London
Barts Health NHS Trust, London
Inderjeet Dokal graduated in Medicine from
the University of Leicester in 1983. He moved
to Hammersmith Hospital (London) in 1984
where he received his post graduate clinical
and research training. He was appointed Consultant in Paediatric Haematology in 1995
and was conferred the title of Professor of
Haematology at Imperial College in 2003. In
INVI TE D S P E A K E RS
2006 he was recruited to the Chair of Child
Health at Barts and The London/Queen Mary
University of London.
His principal research interest is the pathophysiology of aplastic anaemia (AA)/bone
marrow failure. Over the past 20 years his
group has determined the genetic basis and
pathophysiology of several sub types of bone
marrow failure. This has shown the importance of telomerase and telomeres in humans
and the consequences of their dysfunction.
Current research is focussed on elucidating
the genetic basis and pathophysiology of the
many uncharacterized cases of dyskeratosis
congenita, aplastic anaemia, myelodysplasia
and related disorders.
Overview
Inderjeet Dokal
Clinical Approach to Inherited Bone Marrow Failure
Syndromes
Peter Bader
Novel cellular treatment approaches after
allogeneic stem cell transplantation: from specific
immunotherapy to tolerance induction
Henrik Hasle
Diagnosis and management of childhood
myelodysplastic syndrome and juvenile
myelomonocytic leukemia
28
N O P H O / N O B O S
M E E T I N G
Samir Agrawal
Implementing a Diagnostic Strategy for Management
of Invasive Fungal Disease in Haemato-Oncology
Paul Verweij
Challenges in the diagnosis and management of
invasive fungal infections in 2014
Lillian Sung
Fever and neutropenia in pediatric cancer patients:
is a choice between outpatient v/s inpatient
management a routine one? Critical review and
recommendations
Stefan Bielack
Recent developments in osteosarcoma
Clement Trovik
The Scandinavian Sarcoma Group Central Register.
6000 patients after 25 years of monitoring referral
and treatment in Orthopaedic Soft Tissue Sarcoma
Marianne Straume
How does cancer treatment affect the developing
child, and what can we do to help children integrate
the hardship they experience
Trond Markestad
Ethical dilemmas when treating seriously ill children
Eva Skovlund
Statistics in clinical trials – challenges and pitfalls
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
Inherited bone marrow failure syndromes
The inherited bone marrow (BM) failure syndromes
are a diverse group of disorders characterized by BM
failure usually in association with one or more extrahaematopoietic abnormality. The BM failure, which
can involve all or a single lineage, often presents in
childhood but this may not be until adulthood in
some cases. Furthermore, some patients initially labeled
as “idiopathic aplastic anaemia” are cryptic presentations of these genetic syndromes. Significant advances
in the genetics of these syndromes have been made
with more than fifty disease genes identified to date.
These advances have provided a better understanding
of normal haematopoiesis and how this is disrupted
in patients with BM failure. Additionally, as these
disorders are usually associated with developmental
N O P H O / N O B O S
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abnormalities and an increased risk of cancer they are
providing insights into human development and the
genesis of cancer. In the clinic, genetic tests stemming
from the recent advances are facilitating diagnosis and
personalized management. Haematopoietic stem cell
transplantation using fludarabine based protocols has
improved outcomes significantly; management of some
of the non-haematopoietic complications remains a
challenge.
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29
Peter Bader
Henrik Hasle
Professor, Goethe University and
University Childrens Hospital, Frankfurt, Germany
Professor, Department of Pediatrics
Aarhus University Hospital Skejby, Denmark
After graduating at the University Tübingen,
Prof. Bader started his education to become
a pediatrician at the University Children’s
Hospital in Tübingen under the supervision
of Prof. Dr. Dietrich Niethammer.
In 2000, he was a pointed as attending physician in the Children’s Hospital and continued
with his research program (see below). After
successful publication of several manuscripts
as first author he received the “Habilitation”
and received a lecturer title. He was awarded a
Full Professorship at the Goethe University in
Frankfurt at the University Children’s Hospital
and appointed as the Head of the Division for
Stem Cell Transplantation and Immunology
in 2004. Since then he is responsible for the
Stem Cell Transplantation Program and he
become Vice Director of the Department for
Children and Adolescents in 2010.
Research Interest and Program:
He has started his research in the field of
pediatric stem cell transplantation. The prevention of relapse post transplant has become
one of the major challenges. Prof. Bader’s
Group has developed PCR based techniques
for the quantitative analysis of chimerism and
minimal residual disease (MRD). Based on
several large grants the Group has performed
prospective trials were it could be shown that
using molecular techniques relapses could be
anticipated in a great cohort of children’s after
allogeneic stem cell transplantations.
Since his move to Frankfurt in 2004 he built up
a large transplant program focusing on T-cell
depleted transplants using different donors,
especially HLA non-identical relatives after reduced intensity conditioning. Based on these
activities he successfully widened the research
profile of his group towards cellular therapies
post transplant. This includes tumor specific
T-cell transfer and cytokine induced killer cell
(CIK) including chimeric antigen receptor
(CAR) modified therapies for the treatment
of children with malignancies. These projects
have been recently awarded with two large
research grants. An additional focus of this
group is on the generation and clinical use
of mesenchymal stromal cells.
Novel cellular treatment approaches after
allogeneic stem cell transplantation: from specific
immunotherapy to tolerance induction
Immunotherapy has progressively acquired an important part in the treatment of children with refractory/
resistant hemato-oncological diseases. Its ultimate goal is
that of increasing the immunological driven anti cancer
effect without causing further immunological complications. Through recent experience some basic principles
in the use of immunotherapy have become clear: it has
better success rate when applied in the pre-emptive
setting and it should be preceded by lymphodepletion.
It is therefore straightforward to think that the stem
cell transplantation setting could be a perfect match
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for this kind of therapy. The presentation summarizes
some of the many different strategies that are nowadays
under pre-clinical and clinical evaluation, mostly in the
pediatric field, and hints at their possible application
in the allogeneic stem cell transplantation field.
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Henrik Hasle is professor in pediatric hematology/oncology at the department of Pediatrics,
Aarhus University Hospital Skejby in Aarhus
Denmark.
Henrik Hasle trained in epidemiology, hematology and pediatrics at Odense University
Hospital, where he also completed a research
fellowship in pediatric oncology with a thesis
on myelodysplastic syndrome. Since 2000
consultant and associate professor in pediatric
hematology/oncology at Aarhus University
Hospital Skejby in Aarhus and was awarded
full professor in 2009.
Henrik Hasle is a founding member of the
European Working group on Myelodysplastic
Syndromes in Childhood (EWOG-MDS) and
served as chairman of the group 1998-2002.
Henrik Hasle was the chairman of the AML
group of the Nordic Society for Pediatric Hematology and Oncology (NOPHO) 2002 to
2010.
Henrik Hasle is the author or coauthor of
more than 170 peer reviewed journal articles
mainly dealing with myeloid leukemia in children and genetic predisposition to cancer.
Diagnosis and management of childhood
myelodysplastic syndrome and juvenile
myelomonocytic leukemia
Myelodysplastic and myeloproliferative disorders are
rare in children. Contemporary classification includes
three main groups; myelodysplastic syndrome (MDS),
juvenile myelomonocytic leukemia (JMML), and the
myeloid leukemias of Down syndrome (ML-DS).
MDS is subdivided into refractory cytopenia of childhood (RCC) and advanced MDS (RAEB). Some
patients have underlying constitutional bone marrow
failures. Hypoplastic RCC has a very favorable outcome
with immunosuppressive therapy and hematopoietic
stem cell transplantation (HSCT) as salvage option.
The only curative therapy for advanced MDS is HSCT.
The conditioning regiment and GvHD prophylaxis
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have major impact on the outcome. Genetic aberration
in the RAS signal transduction pathway is found in
most patients with JMML and has contributed major
insight in the pathogenesis of JMML and facilitates the
diagnosis. JMML with CBL mutation represent a special
group that may regress spontaneously like in infants with
JMML and Noonan syndrome. Azacitidine is being
tested as a possible bridge to HSCT for advanced
MDS and JMML Reduced AML therapy is very successful in ML-DS.
[email protected]
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31
Stefan Bielack
Lillian Sung
Associate Professor and Scientist,
The Division of Haematology / Oncology,
Hospital for Sick Children, Toronto, Canada
Dr. Lillian Sung is an Associate Professor and
Scientist at The Hospital for Sick Children,
Toronto, Ontario, Canada. She is certified by
the Royal College of Physicians and Surgeons
of Canada in the specialties of pediatrics, infectious diseases, hematology and clinical
investigation. During her training, she also
completed a PhD in Clinical Epidemiology
from the Department of Health Policy, Management and Evaluation at the University of
Toronto in 2004. Since her faculty appointment, she has developed a clinical research
program focused on supportive care for
children with cancer and more specifically,
understanding predictors of infectious complications in pediatric leukemia and development of a new measure of oral mucositis in
pediatric cancer. Her methodological focus is
on randomized and observational trials, metaanalysis, and patient-reported outcomes. She
is supported by a New Investigator award from
the Canadian Institutes of Health Research.
She was awarded an Early Research Award by
the Ontario Ministry of Health and Innova-
tion and she is the principal investigator on
multiple operating grants from the National
Cancer Institute of Canada and the Canadian
Institutes of Health Research. She currently is
also the principal investigator of an R21. She
was the recipient of the Excellence in Cancer
Research - William E. Rawls Award in Cancer
Control in 2009, awarded from the Canadian
Cancer Society. Dr. Sung is also the Chair of
Cancer Control in the Children’s Oncology
Group (COG), which oversees all studies of
supportive care.
Fever and neutropenia in pediatric cancer patients:
is a choice between outpatient v/s inpatient
management a routine one?
Critical review and recommendations
Fever and neutropenia (FN) is a common complication of cancer therapy. Traditional approaches have
included admission to hospital with administration of
intravenous antibiotics. This presentation will review
the evidence supporting outpatient management with
oral or intravenous antibiotics and discuss parental
preferences and considerations.
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C O N G R E S S
Professor and Head of Department of Pediatric
Oncology and Hematology, Olgahospital,
Stuttgart, Germany. Cooperative Osteosarcoma
Stucy Group COSS
Prof. Stefan Bielack is a pediatric oncologist
and head of the Department of Pediatric
Oncology, Hematology, Immunology; Gastroenterology, Rheumatology and General
Pediatrics at Klinikum Stuttgart - Olgahospital, Stuttgart, Germany. His main scientific
and clinical interest lies in the field of bone
sarcoma, particularly osteosarcoma. Stefan Bielack is chairman of the Cooperative
German-Austrian-Swiss Osteosarcoma Study
Group COSS, ECT project leader of the European and American Osteosarcoma Study
EURAMOS1, and leader of the bone tumor
work package of the European Network for
Cancer Research in Children and Adolescents
ENCCA. He has served as President of the
European Musculo-Skeletal Oncology Society
EMSOS and board member of the German
Recent developments in osteosarcoma
The past decades have witnessed little improvement in
survival from osteosarcoma. Nevertheless, there have
been many important developments: Modern techniques, particularly MRI, offer very precise imaging of
the tumor, a prerequisite for the ever increasing use of
limb-salvage surgery. Expandable endoprostheses allow
limb-salvage even in growing children. European and
American groups collaborate in the EURAMOS1-trial
to better define standard medical therapy. Results of
the Good Responder randomization (standard chemotherapy +/- pegylated interferon alpha) were presented
at ASCO 2013, those of the Poor Responders are
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Society for Pediatric Oncology and Hematology GPOH and is currently a member of the
board of the Southern German Society for
Pediatric and Adolescent Medicine SGKJ. He
is a current member of the editorial boards of
Cancer Treatment Reviews and the Journal of
Adolescent and Young Adult Oncology.
N O P H O / N O B O S
M E E T I N G
expected for 2015. Drugs currently under discussion
include mifamurtide, sorafenib, and various other targeted therapies. Novel radiotherapy techniques, such as
carbon ion radiation, may offer hope for patients with
unresectable primaries. At recurrence, surgery remains
the mainstay of treatment, while the debate about the
role of (adjuvant) second line treatments continues.
Work-Package 7 (bone sarcoma) of the ENCCA-FFP7
network currently works to integrate clinical trials and
tumor biology research.
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33
Samir Agrawal
Clement S. Trovik
Professor dr.med
Departments of Musculoskeletal Tumour Service,
Haukeland University Hospital, Bergen
Clement S. Trovik, Professor dr.med is a specialist in general surgery and orthopedics and
leader of the Bergen Sarcoma Centre, one of
two sarcoma centers in Norway. He achieved
his PhD in 2000 and became Norways first
professor in orthopedic oncology in 2012.
Professor Trovik has for many years been the
leader of the surgical group and of the central
registry in the Scandinavian Sarcoma Group
(SSG). Associate Editor in Sarcoma and referee for several journals. He has around 30
publications.
BSc (Hons), MB ChB, FRCP, FRCPath, PhD
Senior Lecturer in Haematology,
Queen Mary University of London and
Consultant Haemato-Oncologist at
St Bartholomew’s Hospital and
The Barts Health NHS Trust
Dr Agrawal qualified initially at the University
of Bristol subsequently trained at The Royal
Marsden Cancer Hospital and being awarded
his PhD (in Immunology) at the University of
Paris. He is a fluent French speaker.
He is Director of The Stem Cell Laboratory
and Head of Diagnostic Immunophenotyping. He was a winner of the NHS Innovator
Awards in 2006 for the introduction of new
diagnostic tests for the diagnosis of human
leukaemias.
He has designed, funded, and implemented
studies on myelodysplastic syndromes, invasive aspergillosis, and chronic lymphocytic
leukemia. He is a member of the UK CLL
trials committee and trustee for CLLSA (the
patient-led support organisation for patients
in the UK with CLL), as well as a NICE reviewer and the Haemato-Oncology representative
on the UK IVIg initiative.
His current activities in the field of invasive
fungal disease are:
- an ongoing diagnostic study of high-risk
haematology patients looking at early diagnosis with a novel Aspergillus PCR and
investigating exhaled breath condensate for
IFD diagnosis
- developing clinical guidelines and integrated
care pathways for managing IFD in the highrisk haemato-oncology setting
- promoting best practice and highlighting
new developments through educational
meetings and a new website for all interested
in fungal disease (www.fungalcentral.com,
currently in development)
Dr Agrawal has published over 50 papers
in journals such as the Journal of Clinical
Investigation, Journal of Immunology, Blood,
the British Medical Journal, and the Journal
of Clinical Oncology.
The Scandinavian Sarcoma Group Central Register.
6000 patients after 25 years of monitoring referral
and treatment in Orthopaedic Soft Tissue Sarcoma
Implementing a Diagnostic Strategy for Management
of Invasive Fungal Disease in Haemato-Oncology
Based on the mean population of Norway, Finland,
Iceland and Sweden 1987-2011 (18.6 mil) and an
annual incidence of STS in extremities and trunk wall
of 2-3/100.000, there should have been approximately
11.000 (9000-13.000) sarcomas registered in a 25 year
period. 6127 are in fact in the register. 5858 have complete
registration of key variables.
11 institutions have been reporting to the register. From
Norway and Sweden, five institutions, representing
approximately 70% of the population, have been reporting
all their cases consistently during the entire period. From
Finland, two institutions representing approximately 50%
of the population, have been reporting most years. Iceland
has just started reporting.
The five consistently reporting institutions are treating
approximately 90% of the cases in their region. The
The development of care pathways for IFD management involves not just the clinical team looking after
the patient, but everyone required in the pathway
must be involved in order to have “ownership” of the
pathway: radiology, microbiology, infectious disease
and respiratory physicians…This care pathway will be
different in each institution and dependent on local
factors. By ensuring each step of the pathway is clear,
each individual knows their responsibility in the process
(from the nurse on the ward to the radiologist), the
chances of a successful outcome for the patient will
be maximized.
My presentation will focus on the current debate
on management strategies for invasive fungal disease
(FD) in Haemato-Oncology. I will discuss empirical
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remaining institutions have mostly reported all their treated
patients during certain time periods, and not at all during
other periods. The register may therefore be considered
representative for the population of Scandinavia, treated
at the reporting institutions.
59% of patients are referred to a sarcoma centre untouched,
before any attempt at biopsy. There was an improvement
from 51% during the first 5 years to 68% during the last.
50% had wide or better margins at surgery. There seems
to be a change of attitude among Scandinavian surgeons
concerning the importance of a wide margin, parallel
to an increase in use of radiotherapy. Wide margins are
now achieved significantly less often than 20 years ago.
For the consistently reporting institutions, the rate of
follow-up is 97%.
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M E E T I N G
therapy versus a pre-emptive or a diagnostic-driven
approach.
I will describe the changes in our management algorithms over the last 12 years at St Bartholomew’s
Hospital, London, and discuss the various factors that
impact at a local level in terms of which strategy to
use. This process has brought us nearer and nearer to
what can be called a care pathway. A diagnostic-driven
approach requires, of course, that tests (blood tests as
well as imaging) are rapidly available and I will describe
our experience with routine galactomannan testing
and CT scanning and their limitations. Finally, future
diagnostics – the lateral flow device, proximity ligation
assay – will be presented.
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35
Paul E. Verweij
MD, PhD, professor of medical microbiology and
consultant microbiologist,
Department of Medical Microbiology, Radboud
University Medical Center, The Netherlands
Paul Verweij is professor of medical microbiology and consultant microbiologist at the Department of Medical Microbiology at Radboud
University Medical Center in The Netherlands.
Dr Verweij received his medical degree
from University of Leiden in The Netherlands.
He served as registrar in medical microbiology
at University Hospital Nijmegen, where he
completed his PhD thesis (“Microbiological
diagnosis of invasive aspergillosis”). Dr Ver-
weij then received research training at Victoria
University Manchester in England.
Dr Verweij’s research interests include
diagnosis of invasive aspergillosis, resistance in moulds, and clinical studies of new
antifungal agents. He has authored or coauthored numerous articles for journals such
as Antimicrobial Agents and Chemotherapy,
PLoS Medicine, and the New England Journal
of Medicine.
Dr Verweij is a member of several professional
organizations, including the International Society for Human and Animal Mycology, the
European Society for Clinical Microbiology
and Infectious Diseases. He is member of
the executive committee of the EORTC-IDG
and chairman of the Dutch Society for Medical Mycology.
Eva Skovlund
MSc pharm, PhD statistics
Eva Skovlund, MSc pharm, PhD statistics
1990. Since 2011 she holds her main position
at the Norwegian Institute of Public Health,
presently as acting director of the Division of
Epidemiology. In addition holds a position
as professor II at the School of Pharmacy,
University of Oslo. From 2001 to 2011 she was
scientific director at the Norwegian Medicines
Agency. Her main role was then to be the Norwegian member of CHMP at the European
Medicines Agency, the body that assesses
safety and efficacy of new active substances
as well as all medicinal products on the market in EU/EEA. Previous experience includes:
statistician Glaxo Norway, associate professor
Department of Mathematics, University of
Oslo, supervisor in clinical cancer research at
the Norwegian Radium Hospital (Norwegian
Cancer Society), and 12 years as professor II
at the Section of Medical Statistics, University
of Oslo. Long experience in teaching medical
statistics and supervising clinical research
projects on all levels.
Challenges in the diagnosis and management of
invasive fungal infections in 2014
Statistics in clinical trials – challenges and pitfalls
The timely diagnosis of invasive fungal infection
(IFI) is important to allow early antifungal therapy
and improve outcome. The use of biomarkers and
imaging have clearly improved our ability to diagnose
certain IFIs, but there are several developments that
complicate early diagnosis. The taxonomy of many
fungi has changed, and new species are increasingly
recognized as cause of IFI. These new sibling species
often differ in susceptibility to the conventional species
and presents problems in treatment choice. In addi-
Basic facts on elementary statistical analyses in clinical
trials are presented, and common misunderstandings
are discussed. The weaknesses of p-values and misunderstandings in how to interpret them are illustrated
with practical examples. The effect of sample size
is presented and the difference between statistical
significance and clinical relevance is highlighted, and
recommendations on use of confidence intervals for
effect estimates are made.
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tion to intrinsic resistance, also in some fungi acquired
resistance is increasingly found. In Candida glabrata
resistance rates for fluconazole and echinocandins are
increasing in some hospitals. In Europe azole resistance
is an emerging problem Aspergillus fumigatus, primarily
due to environmental use of azole fungicides. These
developments increasingly challenge are diagnostic
approach and requires new tests and approaches to
patient management to be developed.
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M E E T I N G
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NO P HO / NOB OS j oin t se ssion
Trond Markestad
professor of Pediatrics, University of Bergen,
research coordinator at the Department of
Pediatrics, Haukeland University Hospital, Bergen,
research advisor at Hospital Innlandet
Trust, Eastern Norway
Trond Markestad has worked within different
fields of pediatrics since 1975, but mostly as
a neonatologist. He is currently professor of
Pediatrics at the University of Bergen, research
coordinator at the Department of Pediatrics,
Haukeland University Hospital, Bergen, and
With the advances in medicine and rising and often
unrealistic expectations of what medical care can
provide, pressure to continue or offer futile treatment
and to continue life prolonging treatment beyond what
may be considered in the child’s best interest may be
challenging for health care providers. Based on such
experiences, The Norwegian Medical Association took
the initiative to make a national guideline on how to
handle such dilemmas. The guideline was published
N O P H O / N O B O S
Cand. Psychol. Psychologist
Specialists in clinical psychology,
speaker, crisis management
research advisor at Hospital Innlandet Trust,
Eastern Norway. The last 16 years he has been
member, the last 8 years the chairman, of the
Ethics Committee at The Norwegian Medical
Association.
Ethical dilemmas when treating seriously ill children
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Marianne Straume
M E E T I N G
A N D
by the Directorate of Health in 2009 and revised in
2013. The subject will be discussed with reference to
this guideline.
C O N G R E S S
How does cancer treatment affect the developing
child, and what can we do to help children integrate
the hardship they experience
Children are malleable and very adaptable. This also
makes them vulnerable. Children develop in relation to
their genetic potential and the experiences they make.
Cancer treatment in children is a very specific experience
in critical and sensitive periods of their development.
Cancer and cancer treatment represents great stress
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M E E T I N G
for children. Children have lower integration capacity
than adults. This makes children totally dependent on
adults to integrate the strains they are exposed to into
their psychobiographic memory. In this presentation
I will discuss how adults can help children integrate
the hardship they experience.
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39
NO P HO L e c t u re 2014
Birgitta Lannering
Professor, University of Gothenburg, Sweden.
MD, Pediatric Oncology Department, Drottning
Silvias Hospital, Gothenburg
Birgitta Lannering, professor of Pediatric Oncology, was born in Uppsala, Sweden and received her medical degree from the University
of Gothenburg in 1978, specialist in Pediatrics
in 1983. She spent a year in Nashville in 1980
working with neonatology before entering the
field of pediatric oncology.
In 2000 she worked one year at Karolinska
University Hospital with pediatric oncology.
Birgitta has been deeply involved in brain
tumors since the beginning of her career. She
was one of the founders of the Swedish group
for brain tumors; VCTB as well as of the Nordic
group for brain tumors. She has for long time
been involved in the European group for brain
tumors and has coordinated the international
multicenter PNET-study. A few years ago she
started the Swedish network for Neuroblastomas and CNS-tumors, NBCNS which has
now grown to a strong research platform for
neuronal tumors.
She has been the Head of the Pediatric
Oncology Department in Gothenburg from
2006-2012.
Birgitta is a talented singer and has demonstrated her excellent skills for impersonating
a certain member of the Swedish Royal family
at staff get-togethers. In Göteborg she is famous for her German accent and also known
as Queen Silvia. She likes spending time at her
summer house by the coast and is a much valued colleague both at the department in Gothenburg and within the whole NOPHO family.
Medulloblastoma today
Which are the new insights into medulloblastoma biology during the last years?
Do these have implications for treatment? How to treat relapse?
Can treatment related late effects be mitigated?
We are honored to present Birgitta Lannering as the 2014 NOPHO Lecturer.
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Photo: Bergen Tourist Board / Per Eide
NOPHO / NOBOS
Annual Meeting
9 – 13 May 2014 – Bergen – Norway
FREE PA P E R S
O R A L P R E S E NTAT I ONS
Overview
O–01A Nordic platform for clinical ethics in
pediatric oncology
O–02Treatment decisions in pediatric healthcare
– Who’s the one to decide?
O–03Hospital Admissions for Respiratory Disorders
in Adult Life after Childhood Cancer in
Scandinavia (ALiCCS) – A population-based
cohort study www.aliccs.org
O–04Diseases of renal function and bone
metabolism following treatment of early-onset
cancer. A registry-based study.
O–05Hepatic Veno-Occlusive Disease in Children
with Acute Lymphoblastic Leukemia During
Maintenance Therapy with Continuous
Asparaginase.
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O–07Intra-tumoral heterogeneity of T-ALL leukemic
blasts at diagnosis and follow-up; implications
for minimal residual disease detection
O–11
PPM1D/Wip1 is a novel neuroblastoma
oncogene and potential therapeutic target in
high-risk neuroblastoma
O–16Incidence Of Thrombosis In Children Treated
According To Non-Hr Nopho All 2008 – A
Prospective Single Center Study
O–08Genomic characterization of dic(9;20)
(p13.2;q11.2)-positive B-cell precursor acute
lymphoblastic leukemia
O–12Paediatric germ cell tumors in Denmark,
1985–2012
O–17
Hyperferritinemia in severely ill patients:
associations with clinical and laboratory
findings
O–09RESPECT – A feasibility study of a school ­
re-entry, physical- and social activity
intervention for school children with cancer
O–10Flow Cytometric Measurement Of Platelet
Associated Immunoglobulin In Children With
Newly Diagnosed Immune Thrombocytopenia
C O N G R E S S
O–13
Late mortality among Finnish 5-year survivors
of early onset cancer
O–14The SALUB registry: survivorship passport
and nationwide registration of late-effects after
childhood cancer in Sweden
O–15
Conceptual clarity of values in end-of-life
deliberations in pediatric oncology
O–18Thalassemia and Diamond Blackfan Anemia
in Sweden. Data from the Nordic Transfusion
Registry
O–19Iron deficiency and iron deficiency anaemia in
toddlers in Oslo. Is there a difference between
children of different ethnic backgrounds?
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O–01
NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–02
A Nordic platform for clinical ethics in
pediatric oncology
Treatment decisions in pediatric healthcare
– Who’s the one to decide?
Pernille Wendtland Edslev, Aarhus University Hospital, pediatric
Oncology, Denmark
Lisa Törnudd, University Hospital of Linköping, Sweden, Pediatric
Oncology, Sweden
Introduction
Ethical problems in pediatric oncology have traditionally been handled at an individual level, and are often
considered challenging. A joint working group on ethics, consisting of pediatric oncology nurses and physicians, was constituted during the NOPHO/NOBOS
Annual Meeting in Linköping 2008. The intention of
the working group is to be a Nordic competence group
addressing ethical questions within pediatric oncology.
By improving and sharing ethical knowledge, patient
care and staff decisions may improve. Aim We present the activities and achievements of the NOPHO/
NOBOS Working Group on Clinical Ethics (WGE)
2008-2013. Method The WGE has 14 members (7
nurses and 7 physicians) with at least two representatives from each of the Nordic countries. The group
has met yearly at two meetings and one workshop.
Meetings are organizational and educational with
invited speakers. All members are educated through
international courses and conferences in clinical ethics
and are trained facilitators in moral case deliberation.
Results All WGE members participate in, or have
initiated, formalized clinical ethics projects at their
pediatric departments, hospitals, regions or countries.
Most clinical projects provide deliberation on ethically
difficult cases on a regular basis as an integrated part
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of daily work in pediatric oncology. Ten members are
active in local clinical ethics committees. Two members
have initiated or supervise research projects on ethical
matters. Two members teach ethics to nurse or medical students. One is a board member in a national
society for clinical ethics. Conclusion It has proved
beneficial to combine pediatric oncology nurses and
physicians from different countries in a joint working
group. Through collaboration and education we have
created a common Nordic platform for developing
clinically applied ethics. Importantly, the WGE has
inspired and enabled all members to initiate or engage
actively in projects locally, regionally and nationally,
thus increasing the focus on clinical ethics.
Keywords
clinical ethics, moral case deliberation
Authors Trine Brøner1, Anders Castor2, Sigrún Þóroddsdóttir3,
Pernille Wendtland Edslev1, Britt-Marie Frost4, Heidi
Glosli5, Solveig Hafsteinsdóttir3, Hilde Frøland
Hauge5, Kristian Juusola6, Satu Lehtinen6, Pernilla
Pergert7, Gitte Petersen8, Astrid Sehested8 and Lisa
Törnudd9, on behalf of the NOPHO/NOBOS
Working Group on Clinical Ethics.
C O N G R E S S
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Introduction
In the field of pediatric healthcare the area of autonomy
and self-determination vs. a joint decision, raises many
questions. What say does a pediatric patient have in
decisions regarding treatment that sometimes might
be of life and death character? What say do the parents have? And who decides when a child is old and/
or mature enough to make his/her own decisions?
These dilemmas can be lifted in ethical consultations
but they are also to some extent legally regulated.
Aim The aim was to get a better understanding of the
legal aspects of decision-making for children in our
healthcare system and also to compare the legal status
of children in the healthcare system in the different
Nordic countries. Method A workshop was held by
the WGE where legal expertise from Sweden, Norway,
Finland and Denmark where invited to give lectures on
the legal layout in the different countries and to give
legal guidance in case discussions. Results An article
on the basic legal rules applying to decision-making
regarding minors and the differences between the
countries was drafted. Conclusion Autonomy has a
N O P H O / N O B O S
M E E T I N G
central role in all of the four countries. When it comes
to minors and their right to self-determination another
important concept is maturity. Before a child is mature
enough to be deemed capable of full self-determination
the right and duty of decision-making is divided
between the child, the parents and the physician. All
countries have signed the UN Convention of Rights
of the Child (CRC) stating that the best interest of
the child should take first place. How much guidance
the law gives the physician in assessing the maturity
and ability to self-determination differs between the
countries where the legislation in Sweden is vague, as
it is in Finland while Denmark and especially Norway
have more detailed laws.
Keywords Law, ethics, decision-making, pediatric
Authors Author: Lisa Törnudd and Britt-marie Frost on behalf of
NOPHO/NOBOS Working Group on Ethics (WGE).
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NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–04
Hospital Admissions for Respiratory Disorders
in Adult Life after Childhood Cancer in
Scandinavia (ALiCCS) – A population-based
cohort study www.aliccs.org
Diseases of renal function and bone metabolism
following treatment of early-onset cancer. A
registry-based study.
Thorgerdur Gudmundsdottir, Danish Cancer Society Research
Center, Survivorship Unit, Denmark
Marika Grönroos, Turku University Hospital, Pediatrics, Finland
Background
Pulmonary diseases are the second leading nonmalignant cause of death among childhood cancer
survivors. We therefore assessed the risk of hospitalization for respiratory disorders in Nordic childhood
cancer survivors in a large population-based study.
Material and methods First time hospitalizations for
respiratory disorders were evaluated in a cohort of
29 247 one-year childhood cancer survivors and 196
222 population comparisons from the five Nordic
countries. The nationwide cancer registries, central
population registries and hospital registries were used
to identify cohort members and their hospitalizations.
Survivors were diagnosed with cancer below age 20 and
recruited from the beginning of cancer registration in
the 1940s and 1950s through 2008. Cohort members
were followed individually for diagnoses for respiratory
disorders through register linkages. Absolute excess
risk (AER) per 100 000 person-years and standardized hospitalization rate ratios (RR) were calculated
with corresponding 95% CIs. Results Survivors had
a two-fold increased risk of a first time hospitalization
for any respiratory disorder (n=5271; RR=2.2; 95%
CI 2.1–2.2; AER=700). The risk persisted throughout
life with a cumulative risk >60% >60 years of age.
The highest risk was seen for radiation pneumonitis
(n=24; RR=189; 26–1266), respiratory failure (n=184;
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RR=6.4; 5.3–7.8), pleural effusion (n=160; RR=6.5;
5.3–8.0), pneumonia (n=1901; RR=5.2; 4.9–5.5),
and acute upper respiratory tract infections (n=1945;
RR=2.7; 2.6–2.8). However, pulmonary fibrosis (n=3;
RR=4.2; 1.0–16.8) was registered less frequently than in
previous reports. Survivors of leukaemia (RR=3.7) and
Hodgkin lymphoma (RR=2.6) had the highest risk of
respiratory disorders while survivors of retinoblastoma
had the lowest risk (RR=1.0). Conclusions Survivors
of childhood cancer are at an increased risk for respiratory disorders compared with the general population.
Awareness of this excess risk is important for clinicians
and the growing survivor population; and essential for
optimizing patient counselling and follow-up.
Keywords Childhood cancer survivorship, late effects, respiratory disorders, hospitalizations, clinical epidemiology
Authors Thorgerdur Gudmundsdottir, MD, Jeanette Falck
Winther, MD, DMSc, Sofie de Fine Licht MSc, Trine
Gade Bonnesen, MD, Peter Haubjerg Asdahl, MD,
Laufey Tryggvadottir, MSc, Harald Anderson, PhD,
Finn Wesenberg, PhD, Nea Malila, PhD, Henrik Hasle,
Professor, MD, PhD, Jørgen H Olsen, MD, DMSc,
on behalf of the ALiCCS study group.
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Background
Constant progress in cancer therapy has led to a
growing number of early-onset cancer survivors who
are prone to increased morbidity owing to the lateeffects of their anticancer therapy. The aim of this
study was to investigate pediatric and young adult
cancer survivors’ morbidity on renal diseases and on
diseases of bone metabolism in a registry setting in a
population-based level. The patient cohort was identified from the Finnish Cancer Registry, and consisted
of 13,860 5-year-survivors of cancer diagnosed below
the age of 35. Their siblings without early-onset cancer
were identified from the central population register
and were used as the control cohort. Information
on their morbidity on renal diseases and on diseases
of bone metabolism was collected from the national
hospital discharge registry and was used to assess hazard
ratios for various outcomes. The patient cohort was
separated into two age groups, pediatric (age at cancer
diagnosis 0-19 years) and young adults (age at cancer
diagnosis 20-34 years). Significantly elevated hazard
ratios compared to the controls were observed in the
following outcomes: scoliosis HR 1,6 (95% CI 1,3-2,0),
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osteoporosis HR 5,2 (95% CI 2,4-11,4), osteonecrosis
HR 12,7 (95% CI 5,4-29,7), nephritis HR 1,9 (95%
CI 1,5-2,2) and kidney failure HR 3,6 (95% CI 2,45,3), p<0,0001 for all. All of the mentioned hazard
ratios were significantly elevated in both diagnostic
age groups. The hazard ratio for obesity was elevated
in the pediatric age group for females HR 3,4 (95%
CI 1,6-7,2) and for all survivors of CNS tumors HR
2,8 (95% CI 1,4-5,7). Survivors of pediatric and
young-adult cancers are at increased risk for several
long term adverse outcomes, and this must be taken
into account in their follow-up. Our study provides
new population-based information on the early-onset
cancer survivors’ morbidity on renal diseases and on
diseases of bone metabolism.
Keywords cancer survivors, late-effects, obesity, renal failure,
osteoporosis
Authors Marika Grönroos Niilo Liuhto Nea Malila Laura
Madanat-Harjuoja Jaakko Matomäki Päivi Lähteenmäki
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47
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NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
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Hepatic Veno-Occlusive Disease in Children
with Acute Lymphoblastic Leukemia During
Maintenance Therapy with Continuous
Asparaginase.
Intra-tumoral heterogeneity of T-ALL leukemic
blasts at diagnosis and follow-up; implications
for minimal residual disease detection
Silvia De Pietri, University Hospital Rigshospitalet, Department of
Pediatrics and Adolescent Medicine, Denmark
Nina Friesgaard Øbro, Copenhagen University Hospital,
Rigshospitalet, Department of Clinical Immunology, Denmark
Introduction:
Hepatic veno-occlusive disease (VOD) is a severe toxic
liver-syndrome occurring in relation to chemotherapy
exposure. Outside the transplantation setting, VOD
in children with acute lymphoblastic leukemia (ALL)
has been primarily associated with oral 6-thioguanine
(6TG) administration. Nevertheless we observe an
increased incidence of VOD during maintenance
therapy with 6-mercaptopurine (6MP). In this retrospective case-control study we describe VOD cases
occurring during non-high risk treatment. Method:
among children treated in Rigshospitalet according
to NOPHO-ALL 2008 under standard/intermediate
risk regimen, only the ones assigned to continuous
asparaginase therapy were included (n=42). VOD was
defined by presence of at least 2 of three core features
and no other explanation for liver impairment; 1)
hyperbilirubinemia, 2) hepatomegaly and/or rightupper quadrant pain, 3) ascites and/or unexplained
weight gain (>2,5% from baseline). Results: 9 patients
(21,4 %) met the criteria for VOD (3 IR and 6 SR).
4 (2 SR and 2 IR) experienced a second (or third)
VOD episode. Total number of VOD episodes: 16.
All events occurred in Maintenance-I during continuous asparaginase. Median time-points for first VOD:
week 4 (2-7) and week 7 (3-7) of Maintenance-I for
SR and IR, respectively. VOD incidence was 30%
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(9/30) after excluding patients who did not receive
any PEG-asparaginase during Maintenance-I due to
prior side effects. Clinical presentation at diagnose:
hyperbilirubinemia (n=16) with mean value 104
µmol/l (=6x UNL, SD 52), rapid weight gain >2,5
% (n=9), diffuse abdominal pain (n=9), right-upper
quadrant pain (n=3), ultrasound-verified ascites (n=4),
ultrasound-verified hepatomegaly (n=1), reduced
portal vein flow (n=1). Discussion: Diagnosis of
VOD is challenged by the lack of objective and specific parameters developed for non-transplanted ALL
patients. The high incidence underlines the need for
further investigations on pathogenetic factors. We
will prospectively investigate 6MP metabolism under
continuous PEG-asparaginase therapy as a risk factor
for VOD (results will be presented).
Keywords Childhood Acute Lymphoblastic Leukemia, VenoOcclusive Disease, Maintenance therapy, Asparaginase,
6-Mercaptopurine.
Authors Silvia De Pietri (presenting author), Stine Nygaard
Nielsen, Jacob Nersting, Thomas Leth Frandsen and
Kjeld Schmiegelow.
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Background
Heterogeneity of T-cell acute lymphoblastic leukemia
(T-ALL) blasts may compromise MRD monitoring,
the most important prognostic tool in T-ALL. PCR is
currently used for MRD monitoring, but potentially
both PCR-MRD and flow-MRD methods might miss
blast subpopulations that differ from the dominating
clone at diagnosis, which is important if subpopulations have divergent chemo-sensitivity. Methods: We
investigated intra-tumoral heterogeneity of leukemiaassociated immunophenotype (LAIP) by 8-color FC in
diagnostic bone marrow (BM) samples from 49 T-ALL
patients (NOPHO ALL-2008). 22 of these were also
analyzed for diversity of TCR gene-rearrangements in
flow-sorted blast subpopulations. MRD LAIP markers
were evaluated by PCR TCR-marker detection in flowsorted cells (61 early follow-up BM samples from 30
pts). Preliminary results: >80% of the T-ALL patients
had at diagnosis heterogeneous LAIPs with bimodal
marker expression, most often of CD1a, CD4, and
TdT. Dominant TCR clonal gene rearrangements were
generally conserved across the phenotypically diverse
blasts, except in one patient. We did not detect any
association between a high number blast subpopulations
at diagnosis (>two markers bimodally expressed) and
high MRD level (PCR-MRD d29 >0.1%). We observed
antigen-specific LAIP changes during treatment (e.g.
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M E E T I N G
loss of CD1a and TdT). The percentage of predicted
LAIP-defined sorted MRD cells being PCR-positive
was: 94% in pt. with fully informative LAIP and 76%
in pt. with partly informative LAIPs (not all blasts
having aberrant marker expression)). The percentage
of cells classified as normal cells being PCR-negative
when sorted was: 93-95% (in ptt. with informative
LAIP) or 62-75% (in ptt. with partly informative
LAIP). Summary: Intra-tumoral heterogeneity of
immaturity and T-linage markers was common in
T-ALL. The phenotypically diverse blasts generally
had invariable TCR gene-rearrangements; accordingly
PCR-MRD would detect all subpopulations. When
all blast of heterogeneous LAIPs were informative,
MRD identified by flow was highly concordant with
cells positive for TCR MRD markers.
Keywords T-cell acute lymphoblastic leukemia (T-ALL); Intratumoral heterogeneity; Leukemic cell sub-populations;
Minimal residual disease (MRD).
Authors Nina Friesgaard Øbro, Lars P. Ryder, Hans O. Madsen,
Birgitte K. Albertsen, Peder S. Wehner, Steen Rosthøj,
Nina Toft, Kjeld Schmiegelow, and Hanne V. Marquart.
Presenting author: Nina Friesgaard Øbro
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NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–09
Genomic characterization of dic(9;20)
(p13.2;q11.2)-positive B-cell precursor acute
lymphoblastic leukemia
RESPECT – A feasibility study of a school
re-entry, physical- and social activity
intervention for school children with cancer
Vasilios Zachariadis, Karolinska Institutet, Molecular Medicine and
Surgery, Sweden
Anne Sofie Helms, University Hospital Rigshospitalet , Pediatrics and
Adolescent Medicine , Denmark
Background
The chromosomal aberration dic(9;20)(p13.2;q11.2)
occurs in up to 5 percent of pediatric B-cell precursor
acute lymphoblastic leukemia (BCP ALL). It is associated with a poorer prognosis than the most common
cytogenetic aberrations, such as t(12;21)(p13;q22)
and high hyperdiploidy, and therefore patients with
dic(9;20) are stratified to the intermediate risk treatment arm in the NOPHO ALL-2008 protocol. We have
previously shown that in addition to the common, gross
chromosomal rearrangement, dic(9;20)+ cases also share
submicroscopic genomic losses and gains - including
small, bi-allelic deletions of the tumor suppressor gene
CDKN2A. Still, our understanding of the genetic basis
of dic(9;20)-positive BCP ALL is limited. Some cases
are thought to express fusion transcripts as a result
of the dicentric rearrangement, which may produce
chimeric proteins influencing leukemic development.
Previous studies are, however, all limited in resolution
and number of cases included. Here we present the
genomic characterization of 25 dic(9;20)(p13.2;q11.2)
BCP ALL cases. To understand the full spectrum of
genomic lesions involved in developing and maintaining
dic(9;20)+ leukemia, we examined diagnostic samples
using high resolution single nucleotide polymorphism
microarrays (Illumina Omni 2.5M), DNA methylation
arrays (Illumina Infinium 450k), and deep transcrip-
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tome sequencing (total RNA-seq). Homozygous deletions of CDKN2A, encoding the tumor suppressors
P16INK4 and P14ARF, were detected in all cases. In
addition, deletions of IKZF1, encoding the lymphoid
transcription factor Ikaros, were present in 40% of
cases - a clear enrichment compared to other subtypes
of BCP ALL. Transcriptome sequencing also revealed
recurrent gene fusions involving PAX5, including the
in-frame fusion PAX5-C20orf112. With this study
we expect to gain important new insights into the
pathogenetic basis of dic(9;20)+ BCP ALL and, by
integrating different modalities of genetic information,
reveal common signaling pathways available for future
targeted therapies.
Keywords dic(9;20), BCP ALL, transcriptome sequencing,
fusion genes
Authors Vasilios Zachariadis (presenting author) Jessica
Nordlund Ingegerd Öfverholm Johan Dahlberg
NOPHO representatives* Magnus Nordenskjöld
Gudmar Lönnerholm Erik Forestier Gisela Barbany
Ann-Christine Syvänen Ann Nordgren (*NOPHO
representatives of centers with cases enrolled in final
study cohort)
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Background
During cancer treatment children have reduced contact with their social network of friends, including
participation in education, sports and leisure activities. During and following cancer treatment children
describe school related problems, reduced physical
fitness and social problems. Purpose: Ensure children’s
level of education, physical fitness and age appropriate
everyday-life and to enhance quality of life. Primary
endpoints: One year after first-line cancer therapy; a)
level of educational achievement, b) level of VO2-max.
Secondary endpoints: a) quality of life, b) physical
performance. Patients/methods: RESPECT study
is a nationwide prospective controlled intervention
study addressing children newly diagnosed with cancer (6-18 years) in eastern Denmark (N = 120) and
a matched control group in western Denmark (N =
120). Intervention program: • An educational program
on cancer aimed at the classmates. • Assignment of
two ambassadors (classmates), who biweekly visit the
child and participate in the intervention program. •
Academic education program developed by the teachers
followed by children and ambassadors at the hospital.
• Individual and biweekly group based physical activity
training. Measure: Diagnosis, three and six months
after diagnosis, and one year after cessation of treat-
N O P H O / N O B O S
M E E T I N G
ment. Methods: Validated questionnaires, physical
performance test, DNA profile, Dexa-scan, qualitative interviews and observational study. Preliminary
RESULTS: 33 children with cancer are included in
the intervention group. All schools have accepted the
cancer education program (100%), all children have
ambassadors (100%). It is possible to ensure safe transportation (1-350km), and achieve a biweekly visit rate
during treatment. Conclusion: The study is powered
to quantify the impact of a combined educational,
physical and social intervention program. It is the
first population-based study to examine the effect of
early rehabilitation and to use healthy classmates as
ambassadors to facilitate normalization of social life
at the hospital and contributes with knowledge on
rehabilitation that can facilitate rehabilitation of other
long-term hospitalized children.
Keywords childhood cancer, school re-entry, intervention, peers,
physical activity
Authors Helms, Anne Sofie (presenting author) Schmiegelow,
Kjeld Larsen, Hanne Bækgaard Thorsteinsson, Troels
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NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–11
Flow Cytometric Measurement Of Platelet
Associated Immunoglobulin In Children With
Newly Diagnosed Immune Thrombocytopenia
PPM1D/Wip1 is a novel neuroblastoma
oncogene and potential therapeutic target in
high-risk neuroblastoma
Ole Haubjerg Nielsen, Aalborg University
Hospital, Pediatrics, Denmark
Jelena Milosevic, Karolinska Institutet, Women’s and children’s
health, Kvinnors och barns hälsa, Sweden
Background
Immune thrombocytopenia (ITP) is an immune mediated disorder, but attempts to develop a useful direct
antiglobulin test have been unsuccessful. We have used
flow cytometry (FCM) to measure platelet associated
immunoglobulin (PAIG) at the time of diagnosis and
here review the clinical utility. Methods: Since 1993,
PAIG has been measured within 15 days of diagnosis
and before any treatment in 68 of 88 children with
newly diagnosed ITP. Using fluorescent murine antiIgM and IgG, the amount of platelet bound antibody
was graded on a semiquantitative scale from 0 to 3.
The results have been related to clinical manifestations
and clinical course. Results: PAIG was elevated (grade
1-3) in 74%. IgM elevation was found in 63%, IgG
elevation in 46%. Raised PAIG was most frequent in
children with insidious symptom onset (89%), less
frequent in those with postinfectious (73%) or acute
(59%) onset. An isotype shift to IgG from IgM was
seen in the three groups. PAIG-positive cases had very
low platelet counts (< 5/nL) and mucosal bleeding
more frequently than PAIG-negative cases at the time
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of diagnosis. Clinically significant bleeding episodes
during subsequent follow-up also were more frequent in
positive cases (18% vs. 6%), but there was no relation
to duration of thrombocytopenia. The isotype profile,
on the other hand, appeared to be predictive: elevation
of both IgM and IgG was associated with duration <3
months (19 of 23). Conclusion: FCM measurement of
PAIG can be performed easily and rapidly. The result
may provide some prognostic information. One quarter of children are PAIG-negative and have mild and
“dry” ITP with an uneventful course. PAIG-positive
children with elevation of both isotypes usually have
early spontaneous platelet recovery. The impact of raised
PAIG on platelet function needs to be investigated.
Keywords Platelet associated immunoglobulin, immune thrombocytopenia, ITP, flow cytometry
Authors Ole Haubjerg Nielsen, Ruta Tuckuviene, Kaspar Rene
Nielsen, Kim Varming & Steen Rosthøj
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Background
In neuroblastoma gain of 17q is the most powerful
genetic predictor of adverse clinical outcome. 17q+
correlates with poor survival in our population-based
material where we found aberrations of chromosome
17 in 85% of primary neuroblastomas, specifically,
gain of PPM1D/Wip1 at 17q23. Wip1 is a serine/
threonine phosphatase encoded by the gene PPM1D,
described as a gatekeeper in the Mdm2-p53 regulatory
loop involved in genetic stability, inflammation and
a potential oncogene contributing to carcinogenesis.
Methods: Comparative genomic hybridization (CGH),
immunostaining, mRNA arrays, qPCR, exome- and
RNA-sequencing was used to examine PPM1D/Wip1
in neuroblastoma. Genetical and pharmacological
inhibition was used to analyse the function of Wip1 in
preclinical neuroblastoma models. Results: CGH-array
analysis detected PPM1D/Wip1 extra copies in all
tumors and cell lines containing 17q-gain. Expression
arrays and immunostaining showed high expression of
Wip1 in neuroblastoma corresponding to poor survival.
RNA-sequencing confirmed PPM1D-gain and revealed
truncated isoforms with oncogenic potential. Exomesequencing detected a mutation leading to constitutive
PPM1D/Wip1 activation in an aggressive metastatic
infant neuroblastoma. Wip1 knockdown experiments
showed significant decrease of cell viability, proliferation
and colony formation as well as substantial increase
N O P H O / N O B O S
M E E T I N G
of DNA-damage response in neuroblastoma cells.
Tumor xenograft development was significantly delayed
showing median tumor development (0.10 mL) to
be more than doubled (median 15 days, vs. 33 days,
p<0.001) after Wip1 downregulation compared to
scrambled controls. A novel Wip1 inhibitor was highly
potent in cytotoxic/cytostatic effect in neuroblastoma
cell lines (median IC50 0.8 μM). Furthermore, this
Wip1 inhibitor significantly inhibited growth of
established human neuroblastomas in nude mice after
12 days of treatment (P<0.01). Tumor volumes were
reduced 56% compared with controls after treatment.
Conclusions: Our results show that PPM1D/Wip1 is
oncogenic in neuroblastoma development activated
due to chromosomal gain, alternative RNA-isoforms
and/or DNA-mutation. PPM1D/Wip1 provides a
novel therapeutic target in high-risk neuroblastoma.
Keywords Neuroblastoma, PPM1D/Wip1, oncogene, DNArepair, Carcinogenesis
Authors Jelena Milosevic, Diana Treis, Malin Wickström,
Susanne Fransson, Hjalmar Ståhlberg Nordegren,
Baldur Sveinbjörnsson, Ninib Baryawno, Santhilal
Subhash, Chandrasekhar Kanduri, Tommy Martinsson,
Kazuyasu Sakaguchi, John Inge Johnsen, Per Kogner
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NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–13
Paediatric germ cell tumors in Denmark,
1985–2012
Late mortality among Finnish 5-year survivors
of early onset cancer
Madeline Evers, Rigshospitalet, Cph University Hospital, Hematology
and Oncology Pediatric department, Denmark
Päivi Lähteenmäki, Turku University Hopital, Pediatrics, Finland
Background/Purpose
Germ cell tumors (GCTs) are a heterogeneous group of
tumors derived from primordial germ cells. GCTs are
benigne or malignant tumors localized either gonadal or
extragonadal. We mapped the Danish paediatric cohort
of GCTs and extracted data on localization, gender,
histology, tumor markers and treatment. Methods All
Danish paediatric GCTs in 1985-2012 were collected
from the Danish Children Cancer Registry and case
records were reviewed. Our preliminary results concern patients from one of the four centers who were
treated at Rigshospitalet, Copenhagen University
Rigshospitalet (area population approx. 2,5 mill).
Results for the whole country will be presented at
the meeting. Results We identified 120 GCTs (45%
gonadal) in patients aged 0-15 years (crude incidence:
1,5/100.000, 67% girls). Overall mature teratomas were
the most observed histological classification (57%) and
usually localized in the ovaries. Extragonadal GCTs
were mainly observed in early childhood (76%) with
sacrococcygeal localization as the most common. In
contrast, gonadal GCTs were observed in late child-
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hood (59%) with ovaries as the most frequent site
of origin. Serum AFP was elevated in 15% and only
4% showed elevated HCG. Approximately one third
(37 children) of the GCTs were malignant. Of these,
50% were treated with chemotherapy. Almost all cases
had surgery. Only 1 patient died and a few had severe
morbidity. Conclusions In Denmark paediatric GCTs
are rare and are mainly benign with mature teratoma
being the most frequent. We identified a peak of
extragonadal GCTs in early childhood and a peak of
gonadal GCTs in late childhood. In malignant GCTs
mortality/morbidity is very low but chemotherapy is
administered for half the patients.
Keywords Germ Cell Tumors, benigne, malignant, Denmark,
paediatric
Authors Madeline Evers, Jesper Brok, Catherine Rechnitzer
Presenting author: Madeline Evers
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Background
Increasing survival rates have been reported especially
for childhood cancer patients after 1970. Up to date,
only few studies are available concerning the late
mortality among early onset cancer survivors, especially regarding young adulthood (YA) malignancies.
Methods: Our nation-wide population-based registry
study provides information concerning cause-specific
long-term mortality up to year 2012 among 16,769
5-year survivors of early onset cancer (aged 0-34 years
at diagnosis). A sibling cohort in addition to population
data was used as reference. Results: Compared with
population data, the overall standardized mortality
ratio (SMR) of cancer patients was 4.6-fold, (95%
CI 4.4-4.8). The SMRs were highest for malignancies
(12.8, 95% CI 12.3-13.3), followed by infectious (4.8,
95%CI 2.9-6.7) and cardio-vascular diseases (1.9,
95% CI 1.7-2.1). Malignancies and cardiovascular
diseases accounted for largest death numbers. Same
primary cancer diagnosis for childhood and YA cancer
survivors displayed elevated overall SMRs at the same
range, with the exception of markedly higher values
after childhood Hodgkin lymphoma (HL). Moreover,
childhood central nervous system (CNS)-tumor, HL
and non-Hodgkin lymphoma (NHL) survivors were
predisposed to higher SMRs due to cardiovascular
N O P H O / N O B O S
M E E T I N G
causes than survivors of corresponding YA malignancies . The highest cumulative non-malignancy-related
mortality was due to cardiovascular disease with a
steady rise throughout the follow-up, but strongly
dependent on the primary cancer diagnosis and age at
diagnosis. Different from survivors of YA malignancies,
no reduction of cumulative cardiovascular mortality
was observed in childhood cancer survivors towards
the recent treatment periods.. However, overall and
malignancy-related mortality showed declining proportions towards the most recent periods after both,
childhood and YA cancer. Conclusion: Our findings
on non-malignancy-related mortality stress the need
to set up long-term individual follow-up with a focus
on cardiovascular late effects for early onset cancer
survivors, especially for YA cancer survivors still lacking those.
Keywords cancer, child, late mortality, young adult
Authors Andreina Kero, Liisa Järvelä, Mikko Arola, Nea Malila,
Laura-Maria Madanat-Harjuoja, Jaakko Matomäki,
Päivi M. Lähteenmäki The presenting author: Päivi
Lähteenmäki.
A N D
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55
O–14
NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
The SALUB registry: survivorship passport
and nationwide registration of late-effects after
childhood cancer in Sweden
Conceptual clarity of values in end-of-life
deliberations in pediatric oncology
Cecilia Petersen, Karolinska Institutet, Women’s and Children’s
Health, Sweden
Anders Castor, pediatric oncology, pediatrics, sweden
Background
The Swedish working group for long-term follow-up
after childhood cancer (SALUB) has developed an
extension of the on-line based Swedish Childhood
Cancer Registry. Beside the purpose of a nationwide
registration of late-effects, the aim was also to create
a patient-friendly summary with recommendations
for future follow-up. The SALUB-registry contains
detailed treatment information on cumulative doses
of traditional chemotherapy, other anti cancer drugs,
radiotherapy doses towards targets and risk organs,
surgery, central catheters and severe complications
during treatment. The treatment data is separated
into primary and relapse treatment with calculation
of cumulative doses from all treatments. The health
status at transition to adult care is reported in detail
and relevant parts are transferred to the summary for
the patient, the so-called survivorship passport. The
passport contains recommendations on future follow-up
and contact information to the physician responsible
for the transition. The passport function is primarily
based on free text, in order to personalize the information for the patient. The registry also contains an event
56
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reporting form, aimed to be used in late-effect clinics.
The SALUB-registry was launched in October 2012
and currently more than 400 patients are registered.
The treatment data may be entered by a research nurse,
but the health status and follow-up plan is signed by
the physician responsible for transition. The SALUBregistry forms a basis for future research on long-term
side effects of cancer treatment during childhood. It
provides detailed health status information at the age
of 18 and a survivorship passport for the patient. With
the further development of late-effect clinics the registry
can be used for prospective registration of late-effects
during adult life, as well as retrospective studies of the
approximately 7,000 former childhood cancer patients
in Sweden now 18 years of age or older.
Keywords Late-effects, survivorship passport
Authors Cecilia Petersen, Omid Mavadati, Mats Heyman, Lars
Hjorth on behalf of all SALUB members
C O N G R E S S
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B E R G E N
2 0 1 4
Background
Approximately 20% of children with cancer die of
their disease or the treatment. These deaths are often
preceded by difficult decisions about continuation or
abandonment of curative treatment when the prognosis
is dubious. The challenges are partly medical, due to
difficulties in determining the precise prognosis for
each individual, available options might be supported
by insufficient data, and/or the total situation might
be highly complex. But in the discourse on what to
do, these medical difficulties (as important as they
are) must be clearly separated from the moral question “what should we do?” A major component of
the moral question is the balancing of burdens and
benefits for the patient. Morally important factors are
values attached to the patient’s life, and can broadly
be separated into questions of three different concepts
of value: 1) sanctity, or inviolability, of the patient’s
life (regardless of his or her own perception of it), 2)
quality of life, and 3) dignity. These concepts represent
distinct values, since a life with any two, but lacking
the third, is conceivable. We would argue that these
N O P H O / N O B O S
M E E T I N G
O–15
notions sometimes, in thinking or discussing the benefit/burden balance in the individual case, might be
confused by the decision maker. One interesting reason
for this confusion is, that in the Nordic languages all
three concepts (sanctity, quality, and dignity of life)
can be, and often are, expressed by words containing
the word value (värde, verdi, værdi, gildi), where the
precise intended meaning is poorly defined. Because
of this risk, we would recommend treatment teams
to place special emphasis on clarification of these
concepts, and question which value is intended, when
deliberating treatment decisions. If they are not clear,
the justification of the decision will not be either.
Keywords Decision-making, ethics, value, quality-of-life
Authors Anders Castor, Hilde Fröland Hauge, Heidi Glosli,
Sigrún Þóroddsdóttir, Gitte Pedersen, on behalf of
the NOPHO/NOBOS Working Group on Ethics
A N D
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57
O–16
NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–17
Incidence Of Thrombosis In Children Treated
According To Non-Hr Nopho All 2008
– A Prospective Single Center Study
Hyperferritinemia in severely ill patients:
associations with clinical and laboratory
findings
Ellen Ruud, Oslo University Hospital, Department of Pediatric
medicine, Norway
Tatiana Greenwood, Karolinska Institiute, Childhood Cancer
Research Unit, Dept of Women’s and Children’s Health, Sweden
Background
Thrombosis is a complication to treatment of acute
lymphoblastic leukemia (ALL), associated with corticosteroid and asparaginase (ASP) administration.
Objective: The aim was to study the incidence of
symptomatic and asymptomatic thrombosis in children
treated according to non-HR NOPHO ALL 2008
prospectively, and the association with asparaginase
intensity (interval between injections), antithrombin
(AT) activity and minimal residual disease (MRD).
Method: We included 47 children (31 boys/16 girls)
with median age 4 years (range 1.9–15.6 y). They were
enrolled at protocol day 29 and observed for clinical
relevant events until the last scheduled injection of
ASP, 7 months later. Following the first 5 fortnightly
scheduled doses of PEG-ASP after day 29, 18 children
were further stratified to PEG-ASP every other week, 25
children every 6th week and 4 children had Erwinase®.
We analyzed AT activity prior to ASP injections and
performed ultrasonography of neck veins and lineogram
(fluoroscopy of central line function) at protocol day
79 and around protocol week 35. Results: Five children
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had symptomatic thrombosis (11%). Ultrasonography
revealed neck vein thrombosis in 30/47 children
(64%) and in 27/41 children (66%) at day 79 and at
the last examination respectively. Study-lineograms
showed catheter tip occlusion in 7/45 children (15%).
Reduced AT activity (< 80%) was found in 35/45
children (78%) with median lowest activity 57% (range
18–111%). We observed MRD > 0.1% at day 29 in 6
children (13%). Persistent neck vein thrombosis was
associated with MRD > 0.1% at day 29 (p = 0.02),
but not associated with ASP intensity (p = 0.54) or
lowest AT activity (p = 0.15). Symptomatic thrombosis
was not significantly associated with any of the tested
variables. Conclusion: In non-HR ALL-patients, the
incidence of thrombosis is high and MRD > 0,1% at
day 29 may be a prothrombotic risk factor.
Keywords ALL, thrombosis, antithrombin, asparaginase, MRD
Authors Ellen Ruud (presenting author), Målfrid Tveiterås,
Kirsti Try, Charlotte de Lange
C O N G R E S S
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B E R G E N
2 0 1 4
Background
Hyperferritinemia has been identified as an important
biomarker of the genetic (primary) form of hemophagocytic lymphohistiocytosis (HLH), a disease associated
with excessive inflammation and characterized by
defect lymphocyte cytotoxicity function. HLH may
also develop in a secondary (acquired) form (sHLH),
a potentially treatable hyperinflammatory condition
that may be present in severely ill patients. Here we
analyzed ferritin levels in patients at an intensive care
unit (ICU) in order to correlate hyperferritinemia
to clinical and other laboratory parameters in this
group of severely ill patients. METHODS: Patients
with ferritin levels >500 microg/L were prospectively
studied at an ICU with regard to clinical and laboratory features, including soluble CD25 (sCD25) and
detailed lymphocyte cytotoxicity analyses in some, and
subsequent genetic studies as appropriate. RESULTS:
Hyperferritinemia and elevated sCD25, both markers
of inflammation, were positively associated to each
other in ICU patients with septicemia, a state of
known excessive inflammation. At ICU admission,
ferritin and sCD25 levels were inversely correlated
to platelet counts in the total cohort and in patients
with septicemia. Notably, thrombocytopenia is a sign
N O P H O / N O B O S
M E E T I N G
of severe illness that affects outcome and mortality of
ICU patients. Hyperferritinemia was also associated
with elevated CRP in septicemia patients. Elevated
sCD25 was inversely correlated to hypoalbuminemia in both groups. Interestingly, hyperferritinemia
(>5,000 microg/L) was also associated with decreased
cytotoxic function (10 LU). Notably, of four patients
with abnormally low cytotoxic function, three (75%)
had <5% circulating NK cells and one had a heterozygous variant in an HLH-causing gene (STXBP2).
CONCLUSION: Our study suggests a correlation
between hyperferritinemia and other laboratory values
indicative of poor outcome in severely ill patients at
intensive care units.
Keywords ferritin, hemophagocytic lymphohistiocytosis, hyperferritinemia, intensive care, septicemia
Authors Tatiana von Bahr Greenwood, Kajsa Palmkvist-Kaijser,
Samuel C. C. Chiang, Terry Huang, Bianca Tesi, Eva
Rudd, Magnus Nordenskjöld, Hans Hjelmqvist, Yenan
T. Bryceson, and Jan-Inge Henter
A N D
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59
O–18
NOPH O O r a l P r e s e n tati o n
NO PH O O r al Pr esen tati on
O–19
Thalassemia and Diamond Blackfan Anemia
in Sweden. Data from the Nordic Transfusion
Registry
Iron deficiency and iron deficiency anaemia in
toddlers in Oslo. Is there a difference between
children of different ethnic backgrounds?
Ulf Tedgård, Dept of Pediatrics, Sweden
Einar Stensvold, Oslo University Hospital, Department of
pediatrics, Norway
Background
Children with transfusion dependent anemia’s (TDA)
are at risk for severe complications due to iron overload
affecting many organs, particularly the heart, liver and
endocrine function. Good knowledge of iron chelation
therapy, how it is followed and high patient compliance
are important for event free survival. The number of
patients in the Nordic countries with TDA is increasing and the Nordic blood transfusion registry (NTR)
was initiated with the aim to improve awareness and
knowledge of TDA treatment and its side effects. The
NTR is a new NOPHO registry that is approved as a
quality registry by Sveriges Kommuner och Landsting
(SKL). In the NTR demographic, disease specific,
treatment related data are registered as well as quality
of life (QoL) and other patient reported outcome
measurers. This study is a pilot testing of the registry
for all patients up to 18 years of age in Sweden with
transfusion dependent Thalassemia and Diamond
Blackfan anemia. The above mentioned variables will
60
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be presented. One aim of the study is also two compare
two QoL questionnaires (Disabkids and PedsQL) and
to find out which is preferred by the patients and which
gives most relevant information. The results of these
data will be important in order to improve patient
care for these rare diseases.
Keywords Thalassemia, Diamond Blackfan anemia, children,
registry, quality of life
Authors Anne Sjögren and Ulf Tedgård on behalf of the
NOPHO Red Cell Disorders Working Group (Birgitte
Lausen, Niels Clausen, Pernille Wendtland Edslev,
Kirsi Jahnukainen, Kirsti Sirkiä, Island Olafur G.
Jónsson, Marit Hellebostad, Anne Grete Bechensteen,
Einar Stensvold, Göran Elinder, Jan-Inge Henter, Rolf
Ljung, Magnus Göransson, Mimi Kjærsgaard, Nadine
Gretenkort, Tina Lund Leunbach, Niina Valtanen)
C O N G R E S S
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B E R G E N
2 0 1 4
Background
The aim of the study was to compare the prevalence
of iron deficiency (ID) and iron deficiency anaemia
(IDA) in children of non-European origin with children of European ethnicity. In addition we wanted
to demonstrate the benefit of including MCV and
soluble transferrin receptor (sTfR) in the test panel
when screening for ID and IDA, in addition to haemoglobin and ferritin. The patients were included from
the routine health-screening program for one-year-old
children. In the study 172 children were examined, 73
girls (42.4 %) and 99 boys (57.6 %). 76 children were
European (44 %), 96 were non-European (56 %). We
found that 11 children (6.4 %) had ID. Four of these
were European (2.3 %), seven were non-European
(4.1 %). Mean Hb values did not differ, (11.7 (0.8)
vs. 11.9 (0.9)) between the children of different ethnic
background. There were however significant (p < 0.05)
lower values for several iron parameters (like sTfR,
MCV, reticulocytes, Ret-Hb and TIBC) in the nonEuropean group, but ferritin did not differ between
the groups. We identified a total of 7 haemoglobi-
N O P H O / N O B O S
M E E T I N G
nopathies (7.6 %); all of these had microcytosis, and
were of non-European origin. When screening for ID
using MCV, an overt microcytosis might indicate ID
and a child at risk for developing anaemia, even with
normal ferritin levels. Children with non-European
background are more at risk of developing ID and
IDA. With increasing non-European immigration
to Norway, however, it is important to have in mind
that children with microcytosis, not necessarily have
ID, but might suffer from haemoglobinopathies Thus,
when screening for ID and IDA in young children
in Norway at health centres, we will recommend
using a combination of Hb, MCV, and sTfR. With
microcytosis and normal levels of sTfR, haemoglobin
sub-typing analyses should be performed.
Keywords Anaemia, ethnic groups, iron deficiency, iron status
Authors Einar Stensvold, Anne Grete Bechensteen, Knut Liestøl,
Petter Urdal and Marit Hellebostad.
A N D
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61
NOPHO / NOBOS
Annual Meeting
9 – 13 May 2014 – Bergen – Norway
FREE PA P E R S
PO S TE R P R E S E NTAT I ONS
Overview
P–01
Hospitalizations for Gastrointestinal Disease in
Adult Life After Childhood Cancer in Scandinavia: a Population-based Cohort Study
P–02
Late morbidity in Finnish long-term survivors
of childhood brain tumors
P–03
Military Service in Male Survivors of Childhood
Brain and Solid Tumors
P–04Is there a need for clinical pharmacist in the
pediatric oncology ward?
P–05
Premature Arterial Aging in Long Term Survivors of High Risk Neuroblastoma Treated with
TBI
P–06
The influence of TPMT polymorphisms on minimal residual disease after 6-mercaptopurine
consolidation therapy of childhood acute lymphoblastic leukemia.
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A N D
P–07
Increased frequency of cancer in female relatives of patients with primary defects in lymphocyte cytotoxicity
P–08
Acute and late effects after radiotherapy – data
from RADTOX registry
P–09Extramedullary Leukaemia in Children With
Acute Myeloid Leukaemia
P–10
Clinical characteristics and outcome in children
with hereditary spherocytosis: A single center
observational study in 35 consecutive
P–11
Immunosuppressed children and respiratory
viral infections – A prospective follow-up study
P–12
Case report of four patients with diffuse pontine glioma.
P–13
Interleukin-7 levels in plasma predict the rate
of T cell immune reconstitution in allogeneic
stem cell transplantation
C O N G R E S S
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B E R G E N
2 0 1 4
P–14
Exosomes from MYCN-amplified neuroblastoma cells contain oncogenic microRNAs
P–15
Trisomy 8 in pediatric acute myeloid leukemia;
a NOPHO-AML study
P–16
Thromboembolism in children with acute
lymphoblastic leukemia in the Nordic countries
P–17
Medullary Thyroid Carcinoma in children with
MEN 2B in Norway. Clinical course of an orphan disease.
P–18
Role of minor ALL-subclones carrying IKZF1
deletions in the development of relapse.
P–19
6-Thioguanine and 6-Mercaptopurine Combination Maintenance Therapy of a Girl with
pre-B ALL
P–20
Early human germ cell development.
P–21
Organ culture of rodent testicular tissue – Optimization of fertility preservation strategies.
N O P H O / N O B O S
M E E T I N G
P–22
Effect of basic culture media. – Do the storage
and media per se affect testicular tissue?
P–23
Antibiotics Prior to Insertion of Central Venous
Catheters does not prevent Bacteremia in Children with ALL
P–24
Self-perception and self-esteem consequences
of CNS malignancy and brain tumour treatment in childhood/adolescence: Populationbased survivor and general population outcomes compared
P–25
Assessment Of Transfusion Burden With Erythrocyte Concentrates In Children Treated For Acute
Lymphoblastic Leukemia According To Nopho
All-2008 Protocol
P–26
JMML with germlineCBL mutation in 1 year old
girl, succesful outcome without treatment.
P–27
Intercranial Hemorrhage in Children with Immune ThrombocytoPenia (ITP)
A N D
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63
P–01
NOPH O P o s te r P r e s e n tati o n
NO PH O Poster Pr esen tati on
P–02
Hospitalizations for Gastrointestinal Disease
in Adult Life After Childhood Cancer in
Scandinavia: a Population-based Cohort Study
Late morbidity in Finnish long-term survivors of
childhood brain tumors
Peter H. Asdahl, Aarhus University Hospital, Department of
Pediatrics, Denmark
Päivi Lähteenmäki, Turku University Hospital, Pediatrics, Finland
Background
In the growing population of childhood cancer
survivors, many late-onset treatment-related health
consequences have been documented but little is
known about gastrointestinal diseases. The objective
of this study was to give a comprehensive and detailed
overview of all gastrointestinal late effects in childhood
cancer survivors. Methods Adult Life After Childhood
Cancer in Scandinavia (aliccs.org) is an inter-Nordic
collaboration investigating late effects of childhood
cancer therapy. From the cancer registries in the Nordic
countries, we identified all patients diagnosed with
cancer under the age of 20 in the last six decades. We
used population-based registries to obtain information
on hospital admissions and deaths. For each one-year
survivor we randomly selected five population comparison subjects. Result The 31,132 one-year survivors
had a total of 3,824 first time hospitalizations for
gastrointestinal disease. The standardized hospitalization rate ratio (RR) for any gastrointestinal disease was
1.6 (95% Confidence Interval (CI): 1.6–1.7) and the
absolute excess risk (AER) was 360 (95% CI: 330–390)
extra hospitalizations per 100,000 person-years. Of six
diagnostic groups, liver diseases had the highest RR
(2.8 (95% CI: 2.5–3.1)) and intestinal diseases had
the highest AER (220 (95% CI: 190–240)). Survivors
of hepatic tumors, neuroblastoma, and leukemia had
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A N D
the highest risk of hospitalization for gastrointestinal
disease. Notably, we found increased risk for a number
of serious specific diseases, e.g. esophageal strictures
(RR: 13 (95% CI: 9.2–20), paralytic ileus (RR: 6.6
(95% CI: 5.7–7.5), acute and chronic pancreatitis
combined (SHRR: 1.9 (95% CI: 1.6–2.3), and liver
cirrhosis (SHRR: 2.9 (95% CI: 2.0–4.1). Among
these, paralytic ileus had the highest AER (75 (95%
CI: 66–84)). Conclusion Survivors of childhood cancer
are at an increased risk of gastrointestinal diseases. The
risk of certain serious specific diseases was increased
but incidence remained low.
Keywords survivorship, late effects, gastrointestinal diseases, liver
diseases, clinical epidemiology
Authors
Peter H. Asdahl, MD, Jeanette F. Winther, MD,
PhD, DMSc, Trine G. Bonnesen, MD, Sofie De Fine
Licht, MSc, Thorgerdur Gudmundsdottir, MD, Jens
F. Dahlerup, MD, DMSc, Laufey Tryggvadottir, MSc,
Harald Anderson, MD, PhD, Finn Wesenberg, MD,
PhD, Nea Malila, MD, PhD, Jørgen H. Olsen, MD,
DMSc, Henrik Hasle, MD, PhD, on behalf of the
ALiCCS study group.
C O N G R E S S
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B E R G E N
2 0 1 4
Background
The population of long-term survivors of childhood
brain tumors is growing. Survivors¡¯ follow-up should
be organized in a structured way. Most of the earlier
research on the morbidity of this patient group has
suffered from small sample sizes. On the other hand,
studies with large sample sizes are based on selfreporting with a possibility for recall and selection
bias. Methods: All brain tumor patients diagnosed
with a neuroepithelial brain tumor at age 0-15 years in
Finland between 1970 and 2004 were identified from
the Finnish Cancer Registry and their late morbidity
(¡Ý5 years after cancer diagnosis) was assessed using
the Hospital Discharge Registry containing data on
hospitalizations and outpatient visits in specialist health
care. Results: The 5-year survivors of childhood brain
tumors had a significantly increased hazard ratio for
endocrine diseases (HR 14.7), mental and behavioral
disorders (HR 1.8), mental retardation/disorders of
psychological development (HR 16,6), diseases of
the nervous system (HR 9.8), disorders of vision
N O P H O / N O B O S
M E E T I N G
and hearing (HR 10.5), and diseases of the circulatory system (2.7) compared with the sibling control
group. Most of the outcomes also had an increasing
prevalence up to 10 to 30 years after primary diagnosis.
The irradiated survivors had significantly increased
HR compared with the survivors with no history of
irradiation for endocrine diseases (HR 6.2), diseases
of nervous system (HR 1.8) and disorders of vision or
hearing (HR 2.1), but not for other main outcomes
studied. Conclusions: Systematic long term follow-up
and supportive measures are essential due to numerous
late effects among childhood brain tumor survivors.
Keywords
brain tumor, childhood, survivor, outcome, morbidity
Authors Erika Gunn, Tuire Lähdesmäki, Nea Malila, Mikko
Arola, Marika Grönroos, Jaakko Matomäki, Päivi M.
Lähteenmäki The presenting author: Päivi Lähteenmäki
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65
P–03
NOPH O P o s te r P r e s e n tati o n
NO PH O Poster Pr esen tati on
P–04
Military Service in Male Survivors of Childhood
Brain and Solid Tumors
Is there a need for clinical pharmacist in the
pediatric oncology ward?
Päivi Lähteenmäki, Turku University Hospital, Pediatrics, Finland
Ranaa El Edelbi, Kvalitet och ut, Sweden
Background
Childhood cancer and its treatment may cause
multiple limitations on physical, neurocognitive and
social functions of survivors. During military service,
physical performance is tested into extreme, and good
cognitive and social capacity are needed in order to
manage the training. The aim of this study was to
examine the acceptance of childhood solid and brain
tumor survivors to the still mandatory military service
in Finland, how the conscripts perform in the physical and cognitive tests during the service, and what
is the level of military education in childhood cancer
survivors compared to controls without a cancer history. Male cancer (below age 16 yrs) survivors that
were born from 1960 to 1992, and alive at the age of
18 years (call-up age) (N=1143) were identified from
Finnish Cancer Registry. From the Population Registry
Centre, five age, sex and place of residence matched
controls were identified (N=5714). Information on
call-up decisions and military service of the study
subjects was collected from the databases of Finnish
Defence Forces. Enlistment frequency was 55% in
Hodgkin lymphoma, 35 % in brain tumors, 55 % in
neuroblastoma, 13 % in malignant bone tumors, 56
% in soft tissue sarcomas, and 68 %in kidney tumors.
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A N D
Treatment with irradiation (p<0.001) and older age
at cancer diagnosis (p=0.04) affected the military
fitness category. Interruption of service occurred to
same extent in survivors and controls, and the level
of military education did not differ between groups
(p=0.83). On average, enlisted solid tumor survivors
managed physical tests and training similarly as controls. Only performance in standing long jump test
was worse (p=0.005). Solid tumor survivors managed
well in cognitive tests compared with controls. Enlisted
survivors of brain tumors had slightly poorer physical
performance than controls (p= 0.05), both in Cooper
running test (p= 0.011) and in general muscle strenght
(p=0.023). Brain tumor survivors had a decline in all
tested cognitive skills, and irradiation treatment did
not explain the findings.
Keywords cancer, childhood, military service, performance,
survivor
Authors Ritva Ahomäki, Tiina Remes, Kai Parkkola, Jaakko
Matomäki, Arja Harila-Saari, Päivi Lähteenmäki The
presenting author: Päivi Lähteenmäki
C O N G R E S S
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B E R G E N
2 0 1 4
Background
Drug treatment in pediatric patients is challenging
mainly because the majority of available drugs have
been developed and tested to be use in adults. Agespecific changes in pharmacokinetics and pharmacodynamics during growth make pediatric patients drug
therapy challenging. Specific information concerning
proper use including dosing in children are lacking
resulting in that about 50 % of all drugs prescribed to
children in Swedish hospitals are off-label (unlicensed)
(1). A pharmacist on the ward contributing with their
knowledge with the aim to improve patient drug
therapy and safety is appreciated (2). The pharmacist
on the ward can ensure safe, effective, and economic
use of medicines by monitoring drug therapy, routines
and processes as well as evaluation of pediatric drug
information, drug interactions, effectiveness, recommendations, storage and costs. The pharmacist on the
ward can futhermore contribute with education of
patients/parents on medication use to improve compliance and ensure correct use (3,4). There is a need
for a Clinical Pharmacist Group within NOPHO! I
therefore suggest that such an expert group should
N O P H O / N O B O S
M E E T I N G
be started for all pharmacists working with pediatric
oncology or have interests in the subject. References 1.
Kimland E, Nydert P, Odlind V, Bottiger Y, Lindemalm
S. Paediatric drug use with focus on off-label prescriptions at Swedish hospitals - a nationwide study. Acta
Paediatrica. 2012; 101:772-8 2. Sanghera N, Chan
PY, Khaki ZF, Planner C, Lee KK, Cranswick NE, et
al. Interventions of hospital pharmacists in improving drug therapy in children: a systematic literature
review. Drug Safety. 2006; 29:1031-47 3. Alsultan
MS, Mayet AY, Khurshid F, Al-Jedai AH. Hospital
pharmacy practice in Saudi Arabia: Drug monitoring
and patient education in the Riyadh region. Saudi
Pharm Journal. 2013; 21:361-70 4. Kaboli PJ, Hoth
AB, McClimon BJ, Schnipper JL. Clinical pharmacists
and inpatient medical care: a systematic review. Arch
Intern Med. 2006; 166:955-64
Keywords Clinical pharmacist, oncology ward, patient safety
Authors Ranaa El Edelbi M sc Pharm, Staffan Eksborg Professor
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67
P–05
NOPH O P o s te r P r e s e n tati o n
NO PH O Poster Pr esen tati on
P–06
Premature Arterial Aging in Long Term
Survivors of High Risk Neuroblastoma Treated
with TBI
The influence of TPMT polymorphisms on
minimal residual disease after 6-mercaptopurine
consolidation therapy of childhood acute
lymphoblastic leukemia.
Anu Vatanen, Children’s Hospital, Helsinki University
Hospital, Division of Hematology- Onkology and Stem Cell
Transplantation, 02100
Emilie Damgaard Brünner, University Hospital
Rigshospitalet, Department of Pediatrics and Adolescent
Medicine, Denmark
Abstract Objective:
The aim of the study was to evaluate arterial morphology and function among long term survivors of
high risk neuroblastoma treated with hematopoietic
stem cell transplantation (HSCT) with and without
total body irradiation (TBI). Subjects and methods:
Common carotid, femoral, brachial and radial artery
morphology, carotid artery stiffness and brachial artery
endothelial function were evaluated by ultrasound,
fasting serum triglycerides and cholesterol measured,
and 24h ambulatory blood pressure (BP) monitored
in 19 adult or pubertal (age 22.7±4.9 years, range
16-30) survivors transplanted at Helsinki University
Hospital during 1984-1999 at the mean age of 2.5±1
years, and compared with 20 age- and sex- matched
healthy controls. Results: Survivors had consistently
smaller arterial lumens, increased carotid intima-media
thickness (IMT), plaque formation (N=3) and stiffness
compared with controls. Survivors displayed higher
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serum triglyceride and cholesterol levels, increased HR,
and increased systolic and diastolic BPs. Multivariate
analysis identified TBI and a low body surface area as
independent predictors for decreased arterial lumen size,
increased IMT and occurrence of plaque. Conclusions:
High risk neuroblastoma survivors treated with TBI
display significant signs of premature arterial aging
during adolescence and young adulthood.
Keywords Cardiovascular Risk, Endothelial Function, High Risk
Neuroblastoma, Late Effect, HSCT
Authors Vatanen Anu, Sarkola Taisto, Ojala Tiina H, Turanlahti
Maila, Jahnukainen Timo, Pihkala-Saarinen Ulla M,
Jahnukainen Kirsi Presenting author: Anu Vatanen,
[email protected]
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Background
Minimal residual disease (MRD) measured after
initial therapy is a potent prognostic factor for longterm outcome for children with acute lymphoblastic
leukemia (ALL). During NOPHO ALL-2008 consolidation therapy, patients receive 6-mercaptopurine
(6-MP) in combination with HD-MTX. Thiopurine
methyltransferase (TPMT) is a critical modifier of
6-MP pharmacokinetics/-dynamics. Low activity
TPMT alleles are frequent and may enhance 6-MP
treatment efficacy. Currently TPMT wild-type and
TPMT heterozygous patients receive the same dose
of 6-MP during this treatment phase. We explored
the relationship between TPMT genotype and
minimal residual disease (MRD) before and after
6-mercaptopurine based consolidation therapy according to the NOPHO ALL-2008 protocol. Eligibility:
Patients treated according to the NOPHO ALL-2008
protocol and receiving fixed doses of 6-MP (25 mg/
m2/day) during consolidation therapy. In total, 483
childhood B-precursor ALL patients were included.
Method: Minimal residual disease was measured by
flow cytometry on treatment day 29 and 79. TPMT
genotype was analyzed by polymerase chain reaction
(PCR). Results: We found no significant difference
between the TPMT wild-type patients (N=433) and
the TPMT heterozygous patients (N=48) with regard
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to MRD levels on day 29 (P = 0.85) and day 79 (P =
0.34). The two genotype groups did not differ with
regard to other characteristics known to be associated
with treatment outcome such as age, sex, and white
blood cell count at diagnosis. 39 patients had positive
MRD (>10-4) on day 79. These patients did not differ
significantly from the MRD negative patients with
regard to the known prognostic variables, including
TPMT genotype (P= 0.40). The actual median interval
from protocol treatment day 29 to 79 was 54 (95%
range: 47 - 80) and did not differ between TPMT wild
type and TPMT heterozygous patients. Conclusions:
TPMT genotype does not influence the MRD level
after exposure to low doses of 6-MP during consolidation treatment according to NOPHO ALL-2008.
Keywords Childhood acute lymphoblastic leukemia, 6-mercaptopurine, minimal residual disease, thiopurine
methyltransferase.
Authors Emilie Damgaard Brünner (presenting author), Louise
Rold Helt, Jacob Nersting, Thomas Leth Frandsen,
Jonas Abrahamsson, Finn Wesenberg, Mats Heyman,
Olafur Gisli Jonsson, Goda Vaitkeviciene, Kaie
Pruunsild, Kim Vettenranta and Kjeld Schmiegelow.
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Increased frequency of cancer in female
relatives of patients with primary defects in
lymphocyte cytotoxicity
Acute and late effects after radiotherapy
– data from RADTOX registry
Alexandra Löfstedt, Karolinska Institutet, Department of women’s and
children’s health, Sweden
Kristina Nilsson, Uppsala University Hospital, Oncology, Sweden
Background
Mutations in genes required for the perforin-dependent
lymphocyte cytotoxicity are associated with early-onset
hemophagocytic lymphohistiocytosis (HLH). Previous
studies have demonstrated that individuals with hypomorphic biallelic mutations in HLH-associated genes
have an increased risk of developing hematological
malignancies. In this study, we hypothesized that even
relatives of primary HLH patients, i.e. heterozygous
carriers of HLH-associated mutations, may display
an increased risk of developing cancer. In an unbiased
approach, we identified relatives of 79 Swedish primary
HLH patients (diagnosed 1971-2011) using a multigeneration-registry. Cases as well as matched controls
were cross-linked with the Swedish cancer registry. The
overall cancer frequency and relative risk of cancer were
established for the entire cohort, as well as separately
for first- and second-degree relatives, and for women
and men. Additionally, NK-cell-mediated cytotoxicity
was assessed in a subgroup of first-degree relatives.
A significantly increased frequency of malignancies
was observed in first-degree relatives (parents; 12.8%
compared to 7.2%, p=0.038). Remarkably, mothers
accounted for the increased cancer frequency among
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first-degree relatives (19.4% compared to 7.3%,
RR=2.7; p=0.004) with cervical carcinomas as the most
frequent cancer. Despite this finding, functional analysis
of heterozygous carriers of HLH-associated mutations
did not display significantly reduced lymphocyte cytotoxicity as measured by current functional assays. In
conclusion, the higher frequency of cancers in relatives
of primary HLH patients indicates haploinsufficiency
in female carriers for cytotoxic lymphocyte-mediated
immunosurveillance of cancer. Our results signify the
impact of mutations in genes required for lymphocyte
cytotoxicity to human health and may speak in favor
of intensified screening for malignancies in female
relatives of primary HLH patients.
Keywords Hemophagocytic lympohistiocytosis, cancer susceptibility, malignancy, immunodeficiency, lymphocyte
cytotoxicity
Authors Alexandra Löfstedt, Marie Meeths, Erik Onelöv, Sam
C.C. Chiang, Yenan T. Bryceson, Jan-Inge Henter
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Introduction:
All children who have received radiotherapy (RT)
after 2008 in Sweden are registered in a web based
registry, RADTOX. The registry is based on the same
information used by the German/Austrian/Swiss RiSK
group registry, a group SvBRG have collaborated with
since 2008. The aim of this study is to monitor the
occurrence of grade 3 or grade 4 effects. Material and
methods: The registry includes information about
given RT and radiation dose data for most organs. In
addition, we collect the complete DICOM data set
for the treatment for future research. Acute and late
side effects are documented on a RTOG/EORTC
scale at end of RT, after 2 months and after 1, 3, 5
and 10 years after completing the radiotherapy. From
Jan 2008 until Jan 2014 we have collected data for
484 patients from six Swedish radiotherapy centres.
Follow-up data were available for approximately half
of these children. The longest follow-up this far is five
years. Results: At end of RT we scored 8% (11 of 132
reports) of the children with at least one grade 3 or 4
adverse effect. Two months after the end of the RT,
13 % (21 of 156) of the children suffer from one or
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more adverse effects. After one to five years after RT,
11% (27 patients) are still affected by a grade 3 and/or
4 late effect. Most severe late problems arise from the
upper GI (9 patients), followed by peripheral nerves (5),
skin (4) eyes, ears, bone marrow (3 respectively) and
CNS (brain) and joints (1 respectively). Conclusion:
The adverse effects of the radiotherapy diminish over
time, but many patients have serious side effect in long
term follow-up. Dose relationships and contribution
of side effects from surgery and chemotherapy will be
further studied.
Keywords acute/late effects, pediatric radiotherapy
Authors Kristina Nilsson (Uppsala), Ingrid Kristensen (Lund),
Anna-Maja Svärd (Umeå), Gun Wickart Johansson
(Stockholm), Måns Agrup (Linköping), Hedda
Haugen (Göteborg), Karin Belfrage (Lund), Jack
Lindh (Umeå) For the Swedish Pediatric Radiotherapy
Group (SvBRG)
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Extramedullary Leukaemia in Children With
Acute Myeloid Leukaemia
Clinical characteristics and outcome in children
with hereditary spherocytosis: A single center
observational study in 35 consecutive
Heidi Kristine Støve, Aarhus University Hospital, Skejby, Department
of Paediatrics, Denmark
Tina Lund Leunbach, Aalborg University
Hospital, Børneafdelingen, ­Denmark
Background
Extramedullary leukaemia (EML) is a common finding in paediatric acute myeloid leukaemia (AML).
The prognostic significance of EML is not clarified
and consequently, the optimal treatment of AML
patients with EML is unknown. The aim of this study
was to characterize the clinical and prognostic aspects
of EML. Methods The cohort consisted of children
diagnosed with AML and treated according to the
NOPHO-AML 2004 protocol from January 2004 to
September 2013 in the Nordic countries and Hong
Kong. Patients were classified by the presence of EML
(CNS disease, myeloid sarcoma, or non-EML). Data
on characteristics were collected from the NOPHOAML database. Survival estimates were calculated using
the Kaplan-Meier method. The prognostic factors
will be examined in a multivariate analysis. Results
By December 2013, 322 children treated on the
NOPHO-AML 2004 protocol were registered in the
NOPHO-AML database. Due to missing data, nine
children were excluded from the preliminary analysis.
At diagnosis, 72 (22.4%) children had EML (CNS
disease and/or myeloid sarcoma). EML was associated
with young age (median: 2.0 years; p = .009), FAB
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M5 (p < .001), and 11q23 aberrations (p = .053).
The 5-year estimated event-free survival was 55% for
EML patients and 44% for non-EML patients (p =
.48). The 5-year estimated overall survival was 63%
for EML patients and 72% for non-EML patients (p
= .032). Death during induction occurred in 8.3% of
EML patients versus 0.8% of non-EML patients (p =
.002). The cumulative incidence of relapse 5 years after
diagnosis was 34% for EML patients versus 49% for
non-EML patients; however, this was not statistically
significant (p = .166). Conclusion EML was associated
with young age, FAB M5, MLL aberrations, and inferior survival. EML is a risk factor for induction death.
Keywords Extramedullary leukaemia; AML; paediatrics
Authors Heidi Kristine Støve, Julie Damgaard Sandahl, Jonas
Abrahamsson, Shau-Yin Ha, Jesper Heldrup, Kirsi
Jahnukainen, Ólafur G. Jónsson, Birgitte Lausen,
Josefine Palle, Bernward Zeller, Erik Forestier, and
Henrik Hasle
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Introduction:
Hereditary spherocytosis (HS) is the most common
inherited hemolytic anemia in Scandinavia. Hemolysis
occurs in different grades, and can be cured by
splenectomy. Splenectomy is associated with a risk
of sepsis, and may increase the risks of long-term
thromboembolic events. Aim: to examine the clinical characteristics, and splenectomy rate in children
with HS. Methods: We reviewed the clinical history
of 35 children at the Pediatric Department Aalborg
University Hospital from 1995-2012. We recorded
clinical events (neonatal jaundice, transfusion dependent anemia, splenomegaly, symptomatic cholelithiasis,
aplastic crisis, and splenectomy) and classified patients
according to grade of hemolysis depending on blood
samples (hemoglobin, reticulocytes, and bilirubin).
The risk of splenectomy was calculated using the
Kaplan-Meier method. Results: In 32 cases hemolysis
was graded. 47% had mild (n=17), 34% moderate
(n=12), and 9% severe (n=3) hemolysis. In the severe
group all had neonatal icterus and needed erythrocyte transfusion before 1 year of age. In the severe
group 100% were splenectomized at a median age of
5.1 years, in the moderate group 58% at a median
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age of 13.4 years, and in the mild group 12% at a
median age of 15.4 years. At 17.5 years 40% were
splenectomized. The probability of splenic survival at
6 years was 90 % (95%CI 71-96%), and at 18 years
23 % (95%CI 4-52%). Discussion: The occurrence
of splenectomy increases during childhood and into
adulthood. Splenectomy occurred more frequent and
at an earlier age concomitantly with hemolytic activity.
The probability of having the spleen at 18 years was
23 % consistent with the increased proportion of splenectomized parents in childbearing age. Conclusion:
The occurrence of splenectomy increases with age and
is reaching high levels in adult life. Prospective studies
are needed among patients with HS to illuminate the
risk of long-term thromboembolic events.
Keywords Hereditary spherocytosis, hemolysis, clinical events,
splenectomy, thromboembolic events
Authors Tina Lund Leunbach (presenting author) Ruta
Tuckuviene
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Immunosuppressed children and respiratory
viral infections – A prospective follow-up study
Diffuse Intrinsic Pontine Glioma
– A Case Report of Four Children
Martina Söderman, Karolinska institutet, Institution of medicine,
Solna, Sweden
Kirsten Brunsvig Jarvis, John Asle Bjørlykke, Maria Gunnes, Dorota
Wojcik, Haukeland Universitetssykehus, Barneklinikken, Norway
Introduction:
Neutropenic fever is a common complication in children undergoing treatment for cancer. The etiology
is only known in 15-30% of the neutropenic febrile
episodes. Previous studies detected a respiratory virus
in about half of the neutropenic febrile cases, but these
have not been able to correlate the respiratory virus to
the febrile episode. Aims: The aim of the study was to
examine whether findings of a respiratory virus could
be correlated to neutropenic febrile episodes by following the patients longitudinally. We also aimed to
investigate whether the shedding-time of respiratory
viruses is prolonged in immunosuppressed children.
Material and Methods: The study was conducted at
Astrid Lindgren Children’s Hospital and children
between 0-18 years that were treated for a malignancy
were included. A nasopharyngeal aspirate was taken in
children with neutropenic fever and sent to the microbiology unit for PCR detection of 16 different subtypes
of respiratory viruses. Re-sampling of virus-positive
patients was repeated after 4-6 weeks, using the same
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methods. Results: Fiftynine episodes of neutropenic
fever have been collected, and in 24 episodes (41%)
a respiratory virus was detected. Follow-up samples
have been collected in 18 of the episodes and 13
patients had been able to clear their respiratory virus.
The viruses found in the patients that had not cleared
their respiratory virus were rhinovirus and coronavirus,
however some of these patients had a new episode of
neutropenic fever. Conclusions: The results indicate
that the finding of a respiratory virus can be correlated
to a neutropenic febrile episode, especially among
certain type of viruses and that prolonged viral shedding is not a major problem in this group of patients.
Keywords Keywords: Neutropenic fever, respiratory virus, children, cancer, polymerase chain reaction
Authors Authors: Martina Söderman, Thomas Tolfvenstam,
Kristina Broliden, Anna Lindblom
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Background
The treatment and outcome for pediatric malignancies
has improved greatly in the last few decades, but for
diffuse brainstem tumors the prognosis is still dismal.
We report our experience at the University Hospital
of Bergen, Norway, with four patients diagnosed
with diffuse pontine glioma in the period from April
2009 to September 2011 and treated according to the
Angiocomb protocoll. All four patients were girls, ages
five (patient A), four (patient B), nine (patient C), and
six (patient D) years at diagnosis. Their symptoms at
diagnosis varied from abducens nerve palsy (patients A
and C), to ataxia (patient D), to acute hydrocephalus
(patient B). Diagnosis was based in MRI findings, all
cases were inoperable and no biopsies were performed.
All four patients were treated with initial radiotherapy,
1,8 Gy x 30 fractions, totally 54 Gy, with adjuvant
Topotecan as a radiosensitizer. Only one patient
(patient D) had progression of the tumor during
radiotherapy. Four weeks after completed radiotherapy,
they started on investigational chemotherapy in the
form of Thalidomide, Celecoxib, and Etoposide accord-
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ing to the Angiocomb protocol. The duration of the
triple therapy ranged from three to nine months, and
in each case the treatment was stopped due to tumor
progression; radiological, clinical, or both. The major
side effect of the treatment was neutropenia. Patients
B and D died shortly after the end of triple therapy;
the former after three months of triple therapy, the
latter after eight months and followed by alternative
medicine from Tibet the last weeks of life. Patients A
and C received six and seven months of triple therapy
respectively, followed by alternative chemotherapy
with Topotecan/Dicloracetate for six and two months
respectively. Patient A also received Nimotuzomab/
Vinorelbine for two months. All four patients died
within two years of diagnosis.
Keywords Brainstem tumor, pontine glioma, Angiocomb
Authors Kirsten Brunsvig Jarvis, John Asle Bjørlykke, Maria
Gunnes, Dorota Wojcik
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Interleukin-7 levels in plasma predict the rate of
T cell immune reconstitution in allogeneic stem
cell transplantation
Exosomes from MYCN-amplified neuroblastoma
cells contain oncogenic microRNAs
Klaus G Müller, Copenhagen University Hospital
Rigshospitalet, Department of Pediatrics and Adolescent
Medicine, Denmark
Trond Flægstad, Univ of Tromsø and Univ Hosp North
Norway, Pediaytrics, Norway
Introduction:
Allogeneic hematopoietic stem cell transplantation
(HSCT) is challenged by delayed T cell reconstitution and acute graft-versus-host disease (aGVHD),
both contributing to treatment-related mortality.
Interleukin-7 (IL-7) is a cytokine essential for T-cell
generation in thymus and peripheral homeostasis of T
cells. We prospectively investigated associations between
IL-7 levels and lymphocyte reconstitution, aGVHD
and mortality in HSCT. Materials and methods: 81
paediatric and adult patients transplanted with sibling
or unrelated donors at Rigshospitalet, Denmark, from
2010-2013 were included. Plasma IL-7 was measured
by ELISA (R&D Systems) before transplantation, at the
day of transplantation and at day +7, +14, +21, +28,
+60 and +90 post-transplant. T, B and NK cells were
counted using flow cytometry. Results: Plasma IL-7
rose from 2.4 gl/ml following conditioning to a peak
of 22.1 pg/ml at day +7 (P<0.0001) and then gradually
declined to pre-transplant levels. High IL-7 levels at
day +7 were associated with the use of anti-thymocyte
globulin (P=0.0079), but not with C-reactive protein
levels. IL-7 level day +7 was significantly negatively
associated with T cell subset counts day +30 to +60
in multivariate analysis. IL-7 levels above median
(18.2 pg/ml) at day +14 tended to be associated with
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acute GVHD grade 2-4 (OR=4.2, P=0.071). This was
confirmed in adults when stratifying for age (OR=5.4,
P=0.036). Overall survival was significantly increased
in adults with low IL-7 levels at day +7 (P=0.046).
Discussion: This study confirms previous findings of
elevated IL-7 levels during lymphopenia, most pronounced in patients treated with ATG. Importantly,
the present data indicate that IL-7 is determined by
feed-back regulation based on the size of the total T
cell population. Furthermore, high plasma IL-7 levels
in the early post-transplant period can predict inferior
T-cell reconstitution, possibly reflecting lower total T
cell numbers early after HSCT or aGVHD mediated
suppression of haematopoiesis.
Keywords Interleukin-7, allogeneic stem cell transplantation,
immune reconstitution, graft-versus-host disease
Background
The MYCN oncogene is frequently amplified in neuroblastoma and is associated with aggressive disease
and treatment failure. MYCN induces expression
of different miRNAs, which are posttranscriptional
regulators of gene expression with established roles in
neuroblastoma development. In this study, we show that
MYCN -amplified cell lines not only express miRNAs
with functions inside the cell, but also secrete populations of miRNAs inside small vesicular structures called
exosomes, with the ability to shuttle to other cells. We
have isolated and characterized exosomes from MYCN
amplified cell lines, and here demonstrate their ability
to be taken up by other cells. By profiling the miRNA
expression, we demonstrate high expression of a group
of established oncomirs in exosomes from two MYCNamplified cell lines. Despite the fact that other studies
have demonstrated the ability of exosomal miRNAs
to regulate their targets in recipient cells, we did not
observe this with exosomes from MYCN -amplified
cells. However, exosomes were able to induce in vitro
angiogenic tube formation, suggesting that they can
have tumorigenic properties. These new findings reveal
a potential new way for MYCN -amplified neuroblastoma cells to interact with their environment.
Keywords Neuroblastoma, MYCN, mcroRNA, exosomes
Authors Bjørn Helge Haug1 , Øyvind H. Hald2 , Peter
Utnes1, Cecilie Løkke2 , Trond Flægstad1,2 , Christer
Einvik1* 1 Dpt. Of pediatrics, University-hospital of
Northern-Norway (UNN) 2 Pediatric research group,
Department of clinical medicine, University of Tromsø
Authors Katrine Kielsen, Karina Jordan, Hilde H. Uhlving, Peter
L. Pontoppidan, Zaiba Shamim, Marianne Ifversen,
Carsten Heilmann, Claus H. Nielsen, Henrik Sengeløv,
Lars P. Ryder, Klaus G. Müller
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Trisomy 8 in pediatric acute myeloid leukemia; a
NOPHO-AML study
Thromboembolism in children with acute
lymphoblastic leukemia in the Nordic countries
Anne Cathrine Lund Laursen, Aarhus University Hospital, Skejby,
Denmark, Pediatric Oncology, Denmark
Nadine Gretenkort Andersson, Skåne Univiersity Hospital, Paediatric
Haematology and Oncology, Sweden
Background
Trisomy 8 is one of the most common cytogenetic aberrations in acute myeloid leukemia (AML), however, the
isolated effects of trisomy 8 in pediatric AML are largely
unknown. Our purpose was to determine the clinical
and cytogenetic consequences of trisomy 8 in pediatric
AML and investigate the individual prognostic impact
of this aberration. We retrospectively investigated 609
patients from the NOPHO-AML database. Complete
cytogenetic data were available in 596 patients (98%),
aged 0-18 years, and diagnosed from 1993 to 2012
and treated according to the NOPHO-AML 1993
and 2004 protocols in the Nordic countries and Hong
Kong. We identified 87 patients (15%) with trisomy
8, in 69 patients (12%) trisomy 8 was combined
with other aberrations (+8 other) and in 18 (3%) it
was the sole cytogenetic abnormality. Trisomy 8 was
associated with FAB M5 (36%) but otherwise clinically
comparable with the non trisomy 8 group. The most
common additional aberrations in the +8 other group
were the MLL rearrangements t(9;11)(p22;q23) (28%)
and 11q23 other than t(9;11) (15%), +19 (25%), +6
(23%), +21 (20%), +22 (13%), inv(16) (10%), and
t(8;21) (9%). Pediatric AML with isolated trisomy
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8 was associated with older age at onset (median
age 10.2 years), 33% had FAB M2 and FLT3-ITD
mutations were present in 58%. The 5-year EFS for
patients with isolated trisomy 8 was 50% and 5-year
OS was 75%. In conclusion, trisomy 8 was present in
15% of the cohort and was one of the most common
cytogenetic aberrations in pediatric AML. Trisomy 8
is a heterogeneous group and the majority of patients
have additional aberrations. Patients with isolated
trisomy 8 differed from patients with +8 other and
were associated with older age at onset (median age
10.2 years), FAB M2 (33%), and FLT3-ITD aberrations (58%). There were no differences in survival.
Keywords acute myeloid leukemia, trisomy 8, cytogenetics,
pediatric, NOPHO study
Authors Anne Cathrine Lund Laursen, Julie Damgaard Sandahl,
Eigil Kjeldsen, Jonas Abrahamsson, Shau-Yin Ha,
Jesper Heldrup, Kirsi Jahnukainen, Ólafur G. Jónsson,
Birgitte Lausen, Josefine Palle, Bernward Zeller, Erik
Forestier, and Henrik Hasle
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Background
Children with acute lymphoblastic leukemia (ALL)
are at high risk for thromboembolism (TE) due to
several factors like central venous catheters, immobilization, infections and treatment with asparaginase
and steroids, leading to increased morbidity and
mortality. Identifying the clinical risk factors and
high risk treatment phases for TE is important and
can lead to a better outcome and quality of life for
these children. The Nordic countries have a common
treatment protocol and registry for childhood ALL
offering an excellent opportunity to study a large
cohort of children. We conducted this prospective
study on symptomatic thromboembolism in children
with ALL to characterize the prevalence, the clinical
characteristics, and potential clinical predictive factors for symptomatic TE in children with ALL and
the impact of thrombosis on treatment delays. From
2008 to 2013 the study group could include 1038
children (aged 1- 18 y) diagnosed with ALL from
seven countries. The overall prevalence of thrombosis
was 6.1 % (n = 64). All cases were venous TE and no
arterial TE was found. Of all thrombotic events 47
% (n=30) were catheter-related, and 31 % (n=20)
were cerebral venous sinus thrombosis. In univariate
analyses age, mediastinal mass, stratification to high
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risk protocol, T-ALL/bilineage ALL, and induction with
dexamethasone were significant risk factors for TE. Of
those, only age ¡Ý 15 year remained a significant risk
factor in multivariate analysis. Contrary to previous
international findings, most thrombotic events occurred
after the induction phase. The observed mortality
directly or indirectly related to thrombosis was 12%
(n=7). Prophylactic anticoagulation in children with
ALL and high risk for TE could lead to a lower TE
prevalence with lower morbidity and mortality. It
should be discussed whether low-molecular heparines
or other prophylactic strategies for risk groups e.g.
teenagers should be implemented in prothrombotic
phases of the new NOPHO-ALL protocol.
Keywords thrombosis, acute lymphoblastic leukemia, children,
asparaginase, steroids
Authors Nadine Gretenkort Andersson, on behalf of the
NOPHO Thrombosis Working Group (Ruta
Tuckuviene, Susanna Ranta, Jon Helgestad, Ellen
Ruud, Anne Mäkipernaa, Tony Frisk, Olafur Gisli
Jonsson, Kaie Pruunsild, Sonate Saulyte Trakymiene,
Ulf Tedgård)
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Medullary Thyroid Carcinoma in children with
MEN 2B in Norway. Clinical course of an
orphan disease.
Role of minor ALL-subclones carrying IKZF1
deletions in the development of relapse.
Else Marie Opsahl, Oslo University Hospital, The Norwegian Radium
Hospital, Department of Surgery, Norway
Peter Hoogerbrugge, RadboudUMC, Pediatric Oncology, The Netherlands
Background
Multiple Endocrine Neoplasia Type 2B (MEN2B) is
an orphan disease with an incidence below 1:500,000.
MEN 2B is a combination of early onset medullary
thyroid carcinoma (MTC) before 1 year of age, bilateral pheochromocytoma and clinical abnormalities as
orofacial and conjunctival neuromas, thickened corneal
nerves, marfanoid habitus and constipation (PseudoHirschsprung disease) due to intestinal ganglioneuromatosis. Early recognition and management of MEN
2B is crucial but may be challenging in Norway with
5 million inhabitants and 60,000 live births annually. Material: All Norwegian patients with germ line
mutations in the RET proto oncogene (n=69) were
screened for MEN 2B causing mutations. Clinical and
outcome data for all identified patients were collected.
Results: Five patients with MEN 2B were identified
giving an incidence of 1:520,000 live births and not
more than 1-2 new cases per decade in Norway. The
diagnosis was based on clinical recognition, confirmed
by RET analysis, all with M918T de novo mutation.
Median age at diagnosis was 13 (range 8-34) years. All
patients had node positive medullary thyroid carcinoma
and none could be biochemically cured. Median time
interval between first recognition of “classical” clinical
MEN 2B symptoms and syndrome diagnosis was 9
(range 4-22) years. However, neuroma and marfanoid
habitus were not present before 4 to 6 years of age.
Whereas the most recently diagnosed patient was
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identified due to neuroma, the other patients were
diagnosis based on endocrine syndrome components.
After median follow up of 132 (range 13-301) months,
all patients were alive. Conclusion: Even in countries
with high developmental indices, management of
orphan diseases such as MEN 2B remains a challenge
concerning recognition and management at an early
age. Recognition of the “classic” MEN 2B phenotype
is not sufficient for timely detection and treatment of
MEN 2B patients with de novo M918T mutations.
Keywords Key Words: Multiple endocrine neoplasia. M918T
RET proto oncogene mutation. Medullary thyroid
carcinoma. Intestinal ganglioneuromatosis. Orphan
disease.
Authors Else Marie Opsahl1, Ann Elisabeth Åsberg2, Trine
Bjøro1,3, Lovise Olaug Mæhle1, Lars Fredrik
Engebretsen4, Krystina K Grøholt1, Lars Andreas
Akslen4, Lars Hilmar Jørgensen1, Jan Erik Varhaug4,5
and Michael Brauckhoff 4,5 Presenting author: Else
Marie Opsahl 1Oslo University Hospital, Oslo, 2St.
Olavs University Hospital, Trondheim, 3Institute of
Clinical Medicine, University of Oslo 4Haukeland
University Hospital, Bergen, 5Department of Clinical
Science, University of Bergen
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Background
Relapse is the major cause of treatment failure in
childhood ALL, and improvement of risk stratification is essential for obtaining better cure rates. We and
others demonstrated that the presence at diagnosis of
deletions in IKZF1, the gene encoding the lymphoid
transcription factor IKAROS, is a strong risk factor
for relapse. Furthermore, preliminary results show
that these deletions can be present at subclonal levels,
often undetectable by routine methodologies. These
findings are in line with recent studies, which have
shown a complex, dynamic architecture of clonal
diversity in ALL, both at diagnosis and at relapse.
This multiclonal diversity likely contributes to the
selective outgrowth of therapy-resistant leukemic cells
during or after chemotherapy treatment, resulting in
relapse. Whether subclonal deletions in IKZF1 are
also associated with relapse has not been thoroughly
investigated. Several recurrent intragenic deletions in
IKZF1 show clustering of their genomic breakpoints,
which allows efficient detection of subclonal deletions
by breakpoint-spanning PCR. Using this method, we
screened a cohort of 331 B-cell precursor ALL patients,
treated according to the DCOG-ALL9 and ALL10
protocols, for the presence of subclonal exon 4-7 IKZF1
deletions (IKZF1Δ4-7) at diagnosis. The cohort was
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slightly enriched for relapsed cases (36.6%). A total of
34 IKZF1Δ4-7 deletions were found, of which 17 were
not detected by routine MLPA. All deletions carried
unique breakpoints and TdT-mediated non-templated
sequences. The 17 MLPA-negative cases had an allelic
burden of the IKZF1Δ4-7 deletion varying from 28%
to <1%. Whereas 12 cases with full clonal IKZF1Δ4-7
deletions eventually relapsed, only 6 cases with subclonal IKZF1Δ4-7 deletions developed a relapse. In
none of the cases for which material was available for
testing (n=4), subclonal lesions were detected in the
relapsed clone. Our data suggest that, in contrast to
clonal IKZF1Δ4-7 deletions, subclonal deletions of
IKZF1Δ4-7 rarely contribute to relapse in ALL.
Keywords ALL, IKZF, genomics
Authors Peter Hoogerbrugge, Esme Waanders, Anke den
Engelsman, Simon van Reijmersdal, Jiangjan Yu, Edwin
Sonneveld, Rob Pieters, Ad GeurtsvanKessel, Frank
van Leeuwen, Roland Kuiper; Radboud University
Nijmegen Medical Center, Nijmegen, Netherlands;
DCOG, The Hague, Netherlands
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6-Thioguanine and 6-Mercaptopurine
Combination Maintenance Therapy of a Girl
with pre-B ALL
Early human germ cell development.
Stine Nygaard Nielsen, Rigshospitalet, BørneUngeKlinikken, Denmark
Jan-Bernd Stukenborg, Karolinska Institutet, Department of Women’s
and CHildren’s Health, Sweden
Background
Maintenance therapy (MT) with oral methotrexate
(MTX) and 6-mercaptopurine (6MP) has proven
essential to eliminate residual disease and attain cure in
childhood acute lymphoblastic lymphoma (ALL). Still,
contemporary MT is challenged by treatment related
hepatotoxicity, indirect parameters for monitoring
treatment efficacy and currently lacking intensification
regimens. The interindividual variability in response
to 6MP is strongly associated with the genetically
determined activity of the 6MP metabolizing enzyme
TPMT. The vast majority of patients (90%) has high
TPMT activity (homozygous for functional TPMT
alleles). Yet, patients with low TPMT activity (carrying a non-functional TPMT allele) have higher levels
of thioguanine nucleotides (TGNs), lower levels of
hepatotoxic methylated 6MP metabolites (MeMPs)
and lower relapse rates. Addition of 6-thioguanine
(6TG) to MT of patients with high TPMT activity
will mimic the more favorable thiopurine metabolite
profile seen in patients with low TPMT activity, as
6TG is a poor substrate for TPMT and 6TG is more
directly metabolized to TGNs. Case Report: An 8
year-old girl with pre-B ALL and high TPMT activity was treated with MTX/6MP/6TG therapy during
the maintenance phase. After 52 days with 6TG
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therapy (6TG max. dose of 10 mg/m2/day) the thiopurine metabolite profile (Ery-TGN/Ery-MeMP) had
increased 5.5-fold due to both a lowered MeMP-level
and an increased TGN-level. No hepatotoxicity was
observed during 6TG therapy. Discussion/Conclusion:
MTX/6MP/6TG MT proved feasible and altered the
thiopurine metabolite profile in a patient with high
TPMT activity. Alternative approaches of lowering
the level of MeMPs have been proposed. Both splitdose administration of 6MP and allopurinol-mediated
inhibition of 6MP-methylation have been successful in
reducing MeMP-levels and hepatotoxicity. However,
none of these approaches seem sufficient in addressing the lack of intensification regimens. A prospective
clinical phase 1-2 trial testing MTX/6MP/6TG MT
in patients with non-B cell NHL and high TPMT
activity is to be conducted.
Keywords Maintenance therapy, ALL, TPMT, 6-thioguanine.
Authors
Stine Nygaard Nielsen, Thomas Leth Frandsen,
Jacob Nersting, Lisa Hjalgrim, Kjeld Schmiegelow
BørneUngeKlinikken, Rigshospitalet, Blegdamsvej 9,
2100 Copenhagen, Denmark.
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Objectives:
When primordial germ cells (PGCs) colonize the
developing gonads at the genital ridge they start
expressing genes such as KIT, PLAP, MAGE-4A, and
DDX4. This expression panel is also displayed by germ
cell tumors and thereby supports the hypothesis that
GCTs originate from the precursor carcinoma in situ,
that are differentially arrested PGC/gonocytes. The
differentiation of PGC/gonocytes in humans is not
synchronous and thereby leads to the simultaneous
presence of different subtypes of gonocytes during
fetal development. Methods: Gonadal tissue pieces of
5.5 to 10.5 weeks old human fetuses were cultured as
organ cultures up to 3 weeks with or without hCG
and rFSH stimulation. Samples for immunohistochemical evaluation were fixed in 4% paraformaldehyde overnight at 4ºC and embedded in paraffin.
Tissue samples for transmission electron microscopy
were fixed in glutaraldehyde and embedded in Epon.
Immunohistochemistry and immunofluorescence were
used to detect and characterize protein expression in
different cells/tissue compartments and follow the
temporal distribution of various proteins e.g. KIT,
POU5F1, DDX4 and VIMENTIN. TUNEL assay
was used to define the overall survival rate before and
after culture periods. Results: The amount of apoptotic
cells after 14 days of culture was between 0.11 and
1.23%. The expression of KIT, POU5F1, DDX4 and
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VIMENTIN was observed after 14 days in vitro. In
vitro testicular cord formation of 6.5 weeks old male
fetal gonads cultured for 21 days exhibited similar
cord formation morphology observed in 9.5 weeks old
control tissue. Conclusions: Our study demonstrates
that organ culture of human fetal gonadal tissue gives
rise to viable tissue expressing proteins present in germ
and somatic cells and a similar testicular cord formation
found in age-matched control tissue. Therefore, the
study provides an ideal platform for ongoing experiments to investigate specific cellular mechanisms in
early human gonadal development and its failures.
Keywords testis, germ cell tumors, in vitro spermatogenesis,
fertility, germ cells
Authors Kristín Rós Kjartansdóttír1*, Rika Lindh1*, Ahmed
Reda1, Valentina Pampanini1,2, Outi Hovatta3,
Olle Söder1, Jan-Bernd Stukenborg 1 1Department
of Women’s and Children’s Health, Pediatric
Endocrinology Unit; Q2:08; Karolinska Institutet
and University Hospital, SE-17176 Stockholm,
Sweden; 2Bambino Gesu` Children´s Hospital, Piazza
di Sant’Onofrio, 4, 00165 Roma, Italia, 3CLINTEC,
Division of Obstetrics and Gynecology, Karolinska
Institutet, Huddinge, Sweden
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Organ culture of rodent testicular tissue –
Optimization of fertility preservation strategies.
Effect of basic culture media. – Do the storage
and media per se affect testicular tissue?
Jan-Bernd Stukenborg, Karolinska Institutet, Department of Women’s
and Children’s Health, Sweden
Jan-Bernd Stukenborg, Karolinska Institutet, Department of Women’s
and Children’s Health, Sweden
Objectives:
Late side effects affecting future fertility in children
undergoing gonadotoxic treatments do still exist. So
far, no treatment can be offered to rescue fertility in
those patients. In 2011, Japanese researchers demonstrated for the first time viable offspring from in vitro
generated murine sperm using an organ culture. This
experiment is big step towards the establishment of
clinical tools to use in vitro differentiated gametes
as potential fertility preservation for young cancer
patients. More studies are required to meet efficiency/
safety concerns and translate results to the situation in
humans. To address this, we established the testis organ
culture in our group and started to adapt the conditions
described for mouse to rat. Methods: Organ cultures
of pre-pubertal 5dpp rat and 3dpp mouse testes were
setup by cutting the testes into pieces. The tissue fragments were positioned with or without adipose tissue
pieces on the agarose gel strands placed in 6 well culture
plates and cultured in MEMα medium±Glutamax at
35°C in 5% CO2 for up to 52 days. Cell proliferation
and expression of proteins specific for testicular cells
were evaluated after up to 52 days in rats and up to
35 days in mouse organ cultures. Results: A positive
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effect of adipose tissue on peritubular cell function was
found. Morphological evaluation revealed a similar
spermatogenic development in vitro when compared to
the situation in vivo. Proliferation of germ cells could
be observed up to 35 days in murine and 52 days in
rat tissue. Conclusions: Our study demonstrates, an
activation of peritubular cells and the expression of
proliferation markers in cultured murine and rat tissue
for 35 and 52 day, respectively. Therefore, we conclude
that the organ culture, established for murine testicular
tissue, can be applied to other species, for male germ
cell differentiation in vitro.
Keywords testis, late effects, in vitro spermatogenesis, fertility,
germ cells
Authors Ahmed Reda, Mi Hou, Halima Al-Balushi, Rika
Lindh, Olle Söder, Jan-Bernd Stukenborg Department
of Women’s and Children’s Health, Pediatric
Endocrinology Unit; Q2:08; Karolinska Institutet
and University Hospital, SE-17176 Stockholm, Sweden
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Objectives:
Experiments focusing on the establishment of a system
for in vitro differentiation of human male germ cells in
the frame of the recently launched NORDFERTIL, will
include those boys undergoing an oncological treatment
who have a very high risk of infertility. By combination
of testicular biopsies of those cases a sufficient number for high quality based research can be obtained.
Therefore, this study aims to evaluate the effects of
different media on pre-pubertal rat testicular tissue
storage for 24 hours at 6-8°C to mimic the situation
given by a transport on ice within one day. Methods:
Testicular tissue of pre-pubertal 5dpp rats were stored
in seven different culture media for up to 24 hours at
6-8°C. Cell proliferation was evaluated by immunohistochemistry. Testicular tissues stored for up to 24
hours in the refrigerator, were stimulated for 24 hours
with 5IU/L hCG and rFSH at 35°C and 5% CO2
in order to measure testosterone production. Analysis
of genes related to spermatogenesis, proliferation,
apoptosis, steroidogenesis, angiogenesis and energetics
was done by using TLDA assays. Results: Among the
investigated 90 genes, Tk1 and Sdha gene expression
was significantly higher in DMEM-Glutamine stored
tissue after 12 hours. However, after 24 hours, there
was no difference at all between the different media in
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gene expression. In addition the storage for 12 hours
at this conditions revealed in a higher production of
testosterone when cell were stimulated for 24 hours
with hCG and rFSH, but there was no difference
between the different media used. Conclusions: In
this study, we show that the media per se has no or
only a minor effect on the expression of genes related
to spermatogenesis, steroidogenesis, proliferation and
apoptosis, angiogenesis, and energetic profiles of testicular cells when stored at 6-8°C for up to 24 hours.
Keywords testis, fertility preservation, late effects, germ cells
Authors Ahmed Reda1, Karin Reuter2, Valentina Pampanini1,3,
Kristin Rós Kjartansdottír1, Mi Hou1, Olle Söder1,
Jan-Bernd Stukenborg1 1Department of Women’s
and Children’s Health, Pediatric Endocrinology
Unit; Q2:08; Karolinska Institutet and University
Hospital, SE-17176 Stockholm, Sweden, 2Centre of
Reproductive Medicine and Andrology, University
of Muenster, 48149 Muenster, Germany, 3Bambino
Gesu` Children´s Hospital, Piazza di Sant’Onofrio 4,
00165 Roma, Italia
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Antibiotics Prior to Insertion of Central Venous
Catheters does not prevent Bacteremia in
Children with ALL
Self-perception and self-esteem consequences of
CNS malignancy and brain tumour treatment in
childhood/adolescence: Population-based survivor
and general population outcomes compared
Kristin Thrana Bergmann, Aarhus University Hospital, Pediatric
Department of Hematology and Oncology, Denmark
Krister K. Boman, Karolinska Institutet, Department of Women’s and
Children’s Health, Childhood Cancer Research Unit, Sweden
Background
The efficacy of prophylactic antibiotics at the time
of central venous catheter (CVC) insertion has been
debated. The aim of this study was to evaluate if the
administration of broad-spectrum antibiotics at the
time of initial CVC placement reduces the subsequent
risk of bacteremia and CVC removal due to infection.
Methods All patients diagnosed with newly diagnosed
ALL from January 1997 to January 2014 and treated at
the Pediatric Department of Hematology and Oncology
at Aarhus University Hospital, Denmark were included.
Data on infectious episodes were included until removal
of the nt-CVC. Results During the study period 182
patients were included. A nt-CVC was placed in 145
patients representing 3,000 CVC-days and 37 received
a tunneled CVC (t-CVC) at diagnosis. Antibiotics
were administrated to 69 (48%) of the patients with
nt-CVC. Of the 37 patients with t-CVCs seventeen
(46%) received antibiotics at time of CVC insertion.
Nt-CVCs were removed after a median of 21 (range
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1-56) days and 8 (5.5%) were removed due to infection.
No significant difference in median CVC survival or
removal due to infection was noted between patients
receiving antibiotics prior to CVC insertion compared
with patients not receiving antibiotics. A high rate of
bacteremia was registered in patients with nt-CVC
(12/1.000 CVC-days) independent of the use of antibiotics at insertion. Conclusion Our data suggest that
administration of antibiotics at time of CVC insertion
does not reduce the risk of subsequent bacteremia and
catheter removal in children with newly diagnosed ALL.
Keywords ALL, antibiotics, CVC, bloodstream infection,
prophylaxis.
Authors Kristin Thrana Bergmann, Mette Møller Handrup,
Henrik Hasle and Henrik Schrøder.
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Background
Survivors of paediatric central nervous system (CNS)
tumours are at risk for persistent tumor/treatmentrelated morbidity, physical disability and social consequences which may intrude into self-perception,
vital for mental health and quality of life. Within
the longitudinal Swedish CNS tumour LIFE-study,
we studied the long-term impact of the childhood
CNS tumour and its’ treatment on self-perception
in significant domains in adult survivors, by comparing outcomes with those of the general population.
METHODS: The target cohort included 700 Swedish
survivors diagnosed between 1982 and 2001 with a
primary CNS tumor. Comparison data were collected
from a stratified general population random sample.
Survivors and general population individuals were
compared as regards self-perception in five domains:
body image, sports/physical activities, peers, work,
and family, and as regards a global self-esteem index.
Within the survivor group, determinants of impact
on self-perception were identified. RESULTS: The
study group from which complete data was received
included 528 survivors, 75.6% of the entire national
study cohort. The control sample consisted of 995
individuals, 41% of the 2,500 addressed. Survivors
had significantly poorer self-perception outcomes in
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domains of peers, work, body image, sports/physical
activities, and in the global self-perception index,
compared with those from the general population (all
P<0.001). Within the survivor group, female gender
and persistent visible physical appearance sequelae predicted poorer outcomes in several of studied domains.
CONCLUSION: Intrusion into self-perception
appears as a potential long-term psychological late
effect in adult survivors after paediatric CNS tumours
and the brain tumour treatment. Because of this risk,
patient care and psychosocial follow-up should include
psychological identity-addressing screening measures
similar to what was used in this study. Paying attention
to self-perception in follow-up care enables identifying,
preventing, and managing of here identified adverse
psychological impact of the illness on self-identity,
crucially related to mental health and quality of survival.
Keywords CNS tumours, adult survivors, long-term late-effects,
psychological impact, identity
Authors Lina Hörnquist, Jenny Rickardsson, Birgitta Lannering,
Göran Gustafsson, Krister K Boman*
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Assessment Of Transfusion Burden With
Erythrocyte Concentrates In Children Treated
For Acute Lymphoblastic Leukemia According
To Nopho All-2008 Protocol
JMML with germlineCBL mutation in 1 year old
girl, succesful outcome without treatment.
Jelena Rascon, Children‘s Hospital, Affiliate of Vilnius University
Hospital Santariskiu Klinikos, Center for Pediatric Oncology and
Hematology, Lithuania
Anita Andrejeva, Haukeland University Hospital, Pediatric
oncology, Norway
Background
The current long-term survival rate of children treated
for acute lymphoblastic leukemia (ALL) exceeds 80%.
Iron overload related to the numerous transfusions of
erythrocyte concentrate (EC) during intensive chemotherapy is an emerging problem among long-term
survivors. However in the clinical routine the amount
of the transfused EC is rarely being traced. The aim
of our study was to assess the number of EC transfusions during the chemotherapy according to NOPHO
ALL-2008 protocol. METHODS. Retrospective
analysis of patient records’ treated at our institution
for ALL according to NOPHO ALL-2008 protocol
was performed. Patients who completed the treatment
or entered the Maintenance-2 phase of the protocol
and were alive at the time of evaluation were included.
RESULTS. Totally 78 patients were treated according
to NOPHO ALL-2008 from 2009 till 2012. Data
of 64 (82.1%) met the inclusion criteria. Risk group
distribution was as follows: SR=35 (54.7%), IR=26
(40.6%) and HR=3 (4.7%). All three HR-patients
received HSCT in CR1. Median age at diagnosis in
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the SR vs IR vs HR was 3.6 years (75% range: 3.0-5.2)
vs 8.6 years (75% range: 4.5-11.4) vs 14.6 years (75%
range: 10.7-15.6) respectively. Mean volume of transfused EM adjusted to recipient body weight did not
differ between SR vs IR groups (115.9±58.6 ml/kg vs
123.0±75.5 ml/kg, p=0.95). However it appeared to be
significantly higher in HR group (300.7±326.7 ml/kg)
as compared to both SR and IR groups (p=0.003 and
p=0.004 respectively). CONCLUSIONS. Transfusion
burden is significantly higher in children treated in
the HR arm of the NOPHO ALL-2008 protocol.
Transfusion history should be carefully monitored in
this group of patients. After the completion treatment
iron overload should be regularly checked for resolution.
Keywords Acute lymphoblastic leukemia, NOPHO ALL-2008
protocol, survivors, iron overload
Authors Rugile Cesaite Jelena Rascon
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Background
JMML with germline CBL mutation in 1 year old
girl, succesful outcome without treatment. Anita
Andrejeva, Haukeland University Hospital, Norway
Henrik Hasle, Aarhus University, Aarhus, Denmark
1year old girl was admitted to the hospital due to
prominent splenomegaly(spleen 15 cm under costal
margin). She was in good clinical condition, weight
9,83 kg, height 79 cm, no fever. Slighly delayed
motor development. Stenosis of the pulmonal artery.
Granulomatous rash: juvenil xantogranulomas from
age 3 moths. Broad forehead, low nasale bridge.
Blood tests showed anemia Hb 6,7 g/dl, WBC 89
×10**9/l, PLT65 ×10**9/l, monocytes 24%, blasts
5%, reticulocytes 1,140 × 10**12(↑). HbF 8,2%(↑).
Flowcytometry of bone marrow, bone marrow biopsy
shows hypercellular bone marrow with 7% blasts , 11%
monocytoid cells. She was dependent of blood and
platelet transfusions. Bone marrow with monocytosis
and hypercellular bone marrow. Diagnosed as JMML
with homozygot mutation of CBL exon 8, germline
origin. No mutation KRAS, NRAS or PTPN11.
Patient was clinically stable without chemotherapy
treatment. After four months patient developed SVK
releated Staph. aureus sepsis requiring intensive care
unit for one week. No trasfusion dependent 6 months
after JMML diagnosis. 1 and ½ year after diagnosis
she is still in good clinical condition, still has massiv
splenomegaly, liver 3 cm under costal margine. Hb
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M E E T I N G
9,7 g/dl, WBC 19,1 × 10**9/l, PLT 48 × 10**9/l,
reticulocytes 0,157 × 10**12/l, HbF 6,5%. Patient`s
older brother has slight motor development, multiple
juvenile xantogranulomas in the skin with tendency to
dissapear, broad forehead, spleen palpable 2 cm under
costal margin monocytosis in blood test, normal WBC
and PLT, no anemia, heterozygot CBL exon 8 mutation.
Patient`s farther has heterozygot CBL exon 8mutation,
no dismorphic futures, normal blood test. Grandparents
has no CBL mutation. Conclusion. Germline CBL
mutation predispose to juvenil myelomonocytic leukemia, cause developmental and functional adnormalities,
cause Noonan syndrome- like phenotype. Patients
with JMML and CBL mutation should be closely
observed without HSCT because of high chanse for
spontaneuos remission of JMML. Patients have high
risk of vascular pathology developing(Retrospectiv
analisys of 21 children with JMML and CBL mutation. Ch. Niemeyer, M. W. Kang, D. Shin, H.Hasle
et al, NatGen sep.2010).
Keywords JMML, CBL mutation
Authors Anita Andrejeva, Haukeland University Hospital,
Bergen, Norway Henrik Hasle, Aarhus University,
Aarhus, Denmark
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Intercranial Hemorrhage in Children with
Immune ThrombocytoPenia (ITP)
Mimi Kjærsgaard, Aarhus University Hospital, on behalf of the
Platelet Working Group
The risk of intercranial hemorrhage (ICH) is still an
issue in ITP. Earlier published frequency is 0.1-0.6%.
We report six cases of ICH in the Nordic countries
from 1998 to 2014.
At diagnosis the children were median 10.5 years old,
and the platelet count was <5x109/L. Within 48 hours
after ITP diagnosis two children received intravenous
immunoglobulin (IVIG), one steroids, two have
unknown treatment status, and one was untreated.
Four children were generally unwell with increasing
bleeding symptoms the days before ICH. Three children decreased 17g/L, 63g/L, and 50g/L in hemoglobin,
10 hours, two, and 14 days before ICH, respectively.
One case reported a head trauma. Case 2 had a coupcontrecoup injury on CT scan, but trauma was not
confirmed.
Three of the six children received IVIG three, two,
and seven days before ICH. Case 6 was treated during
her disease course with steroids (prednisolone, betamethasone, methylprednisolone), IVIG, azathioprine,
and Nplate with no significant response to any of the
medications. Case 2 received IVIG only at diagnosis
of ITP, 20 days before ICH.
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The true ICH frequency in ITP has not yet been
documented. Likely, the only ITP related ICH cases
not captured here, are children dying from ICH with
undiagnosed ITP. Hence, the six cases represent at
least 75% of all the ICH cases in the five Nordic
countries during the 16 years period. There are 4.7
million children in the Nordic countries, and the
incidence of newly diagnosed ITP is 4.8/100,000.
With these assumptions, the yearly incidence of ICH
in ITP is 0.22%.
Initial ITP care has moved towards a watch-and-wait
approach, and the important question is, whether more
children are put at risk for ICH. At least five of the six
children received one or more medical treatments before
ICH occurred. However, unexplained Hgb-decrease
should raise suspicion of occult bleeding.
Keywords ITP, ICH, bleeding symptoms, hemoglobin decrease
Authors Mimi Kjærsgaard
C O N G R E S S
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Photo: Bergen Tourist Board / Oddleiv Apneseth
S at u r d ay 10 M ay
19:00–21:00 Ice breaker – Radisson Blu Royal, Bryggen
S u n d ay 11 M ay
8:30
Opening ceremony
Chair: Britt Ingunn Sævig
09:00–10:30
Joint session with NOPHO
How does cancer treatment affect the developing child, and what can we do to help
children integrate the hardship they experience
Marianne Straume, Cand. Psychol. Psychologist, Specialist in clinical psychology, Bergen
NO B O S P R O G R A M ME
10:30–11:00 Coffee break
11:00–11:45 Ethical dilemmas when treating seriously ill children
Trond Markestad, professor of Pediatrics, University of Bergen,
Research coordinator at the Department of Pediatrics, Haukeland University Hospital,
Bergen, Research advisor at Hospital Innlandet Trust, Eastern Norway
11:45–12:30 3x15 min oral presentations from Ethics group – NOPHO/NOBOS
A Nordic platform for clinical ethics in pediatric oncology
Pernille Wendtland Edslev, Aarhus
Treatment decisions in pediatric health care- Who’s the one to decide
Lisa Törnudd, Linköping
Conceptual clarity of values in end-of-life deliberations in pediatric oncology
Anders Castor, Lund
12:30–13:30 Lunch
13:30–13:45 Opening NOBOS
Chairs: Sigrún Þóroddsdóttir and Sunni Helland
13:45–14:30
Keynote lecture
Why, when, where and how to inform parents within paediatric oncology?
Anders Ringnér, PhD, Senior lecturer, Umeå University
14:30–15:00 Break
15:00–15:45
Free papers
15:00–15:20 Infringing on autonomy – an ethical concern experienced by nursing staff.
Cecilia Bartholdson, Sweden
15:25–15:45 Children and adolescents’ experience of donating bone marrow /
stem cells to surviving siblings.
Carina Rinaldo, Katarina Wallin, Sweden
18:00
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NOBOS dinner at USF Verftet
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M o n d ay 12 M ay 2014
Tu e s d ay 13 M ay
06:30–07:30 Fun and Run. Meeting point Radisson Blu Royal
09:00–09:15 News from NOBOS
Chairs: Marianne Bentsen and Eila Huotari
09:00
Opening
Chairs: Anders Rignér and Kjerstin Mongstad
09:15–10:00
Keynote lecture
09:15–10:00
Youth and rehabilitation
May Aasebø Hauken, PhD student Hemil, UIB / Røde Kors Haugland Rehabilitation Centre,
Center for Crisis Psychology, Bergen
10:00–10:45 Poster presentations
10:45–12:30
Keynote lecture
To loose a child to cancer
Ulrika Kreicbergs, Registered Nurse and Researcher at the Department of Women’s and Children’s
Health, Karolinska Institute, Stockholm.
10:30–12:15
Free papers
10:30–10:50 Visiting nursing experiences working bedside in the only pediatric
oncology ward in Ethiopia.
Hilde Frøland Hauge, May Morken, Norway
Free papers
10:45–11:05 Appetite, senses and joy of life- a nutrition project.
Anne Kaspersen, Denmark
11:05–11:25 The process of striving for an ordinary, everyday life, in preschool aged children living
with cancer, during the first year post diagnosis.
Laura Darcy, Sweden
11:30–11:45 Break
10:55–11:15 Study of childrens’ experiences with venous acces (vap). Need for a Nordic study?
Steinunn E Egeland, Norway
11:15–11:30 Break
11:30–11:50 Complications related to gastrostomy in children and adolescents with cancer.
Tina Ekängen, Johanna Kjellberg, Sweden
11:55–12:15 Kit for symptomatic relief for children in the terminal stage.
Marianne Bentsen, Norway
11:45–12:05 Homecare nursing.
Houtari Eila, Finland
12:15–12:20 Presentation of next NOBOS conference 2016 in Iceland
12:05–12:25 Local anesthetic of intramuscular Asparginase.
Margaretha af Sandberg, Sweden
12:20–12:30 Closing remarks
Gitte Petersen, NOBOS Board
12:30–13:30 Lunch
12:30
13:30–14:15 Chairs: Mariann Hvarnes and Marianne Bøe
Lunch to go
Keynote lecture
Psychosocial health and quality of life and social support in children and adolescents
who survive cancer
Mary-Elizabeth Bradley Eilertsen, Dr. Philos, Centre for Health Promotion Research HisT /
NTNU, Trondheim.
14:30–15:30 Working groups / NOBOS Board meeting
Nutrition .................
Pain ..........................
Palliative care ..........
Late effects ...............
Rehabilitation ..........
Maria Molin and Carina Fondin
Margaretha af Sandeberg
Marianne S.Bentsen
Elna Hamilton
Mary E.Bradley Eilertsen
15:30–16:00 Coffee break
16:00–17:00 National meetings
19:30
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Festive dinner at Grieghallen
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NO B O S KEYNO TE SPEAKERS
Mary-Elizabeth Bradley Eilertsen
Lecturer/Senior Lecturer, Dr. Philos, Centre
for Health Promotion Research HisT / NTNU,
Trondheim
KE YNO T E S P E A KE RS
Children and Adolescents Surviving Cancer:
Psychosocial Health, Quality of Life (QoL) and
Social Support
Background
Childhood cancer involves a crisis for the child and their
family where they face many challenges to achieve normality after diagnosis, even after successful treatment.
Aim
This study focuses on social support, psychosocial
health and QoL for children and adolescents surviving
cancer. The specific aim was to explore and describe
social support in view of professionals’ perception of
collaboration. Moreover, this study sought to explore
and describe psychosocial health and QoL of children and adolescents surviving cancer at least 3 years
after their cancer diagnosis, compared with a healthy
control group.
study including 50 children and adolescents diagnosed
with cancer between January 1, 1993 – January 1, 2003.
Results and conclusion
To improve the child’s psychosocial health and QoL
our results indicate that a collaborative approach is an
important resource for social support and is essential
in planning and implementing the necessary interventions for long-term follow-up care and rehabilitation
for children and adolescents with cancer, especially
for survivors with brain tumours, and those with late
effects. To gain a comprehensive understanding our
results also show the need to particularly take into
account subjectively perceived and proxy reported QoL.
Material and Methods
Health and non-health professionals from the families’
home communities caring for children treated in MidNorway, between 1990 and 1996 were invited to participate in part 1 of this study. Part 2 was a case-control
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NO BO S K E YNOT E S P E A KE R S
NO B O S KEYNO TE SPEAKERS
Anders Ringnér
May Aasebø Hauken
RN, PhD, Senior lecturer, Department of Nursing,
Umeå University, Umeå University Hospital, Sweden
RN, researcher; Center for Crisis Psychology,
Bergen, Norway
Why, when, where and how to inform parents
within paediatric oncology?
Rehabilitation of young adult cancer survivors
Information about the illness and its implications
is a key factor for parents of children with cancer.
Nevertheless, many parents experience low satisfaction
with the information provided. As a starting point
for this research project, we interviewed health care
professionals and parents about their experiences of
information. Health care professionals described difficulties in matching the amount of information to
the parents’ needs and shortcomings in responsibilities, setting, timing, and language when parents were
informed. The parents felt acknowledged as persons
of significance when the information worked well.
However, some parents felt abandoned at important
milestones and were forced to nag for information.
We also studied the interaction between parents and
health care professionals and identified different patterns
depending on how active parents were the interaction
combined with the health care professionals primary
focus of the interaction.
Background
Cancer in young adults (18–35 years) is rare, but the
vulnerable phase of life and the intense cancer treatment
increase their risk of lifelong threats to health. There
is limited knowledge about rehabilitation interventions
for this population.
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Grounded on these findings and previous research, we
designed a person-centred information intervention
aiming at parents of children with cancer. So far, it has
been pilot tested on eight parents. They were highly
satisfied with the person-centred information intervention and perceived benefits from having their own
time to discuss the child’s disease and pose questions
that they otherwise would not have an opportunity to
ask. However, no effects from the intervention were
demonstrated on perceived stress, physical symptoms
of stress, anxiety, and depressive mood.
To sum up, central tasks are to assess the amount of and
what information the parents need, to pay attention
to possible differences in information needs between
parents and children, and, to organise the care so that
the responsibilities are clear and that parents are met in
a calm setting. Improvements at important milestones
during the illness period should be prioritised.
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Purpose
To investigate if a tailored, complex and goal-oriented
rehabilitation program for young adult cancer survivors
(YACS) improved participation, quality of life and
physical capacity.
Method
Twenty YACS with different cancer diagnoses were
allocated to a six-month rehabilitation program focusing
on goal setting, exercise, psychoeducation, individual
follow-up and peer support.
N O P H O / N O B O S
Results
The participants were unprepared for re-entering
everyday life, and experienced a range of late effects,
lack of understanding, and felt neither sick nor healthy.
After attending the rehabilitation program, the participants reported increased participation, quality of life
and physical capacity. These results were explained by
building capacity and finding the balance, gaining new
insight and follow-up.
Conclusions
A complex cancer rehabilitation programme especially
tailored for YACS seems to build positive health outcomes such as participation, QOL and physical capacity
in a long-term perspective. The results underline the
importance of targeting rehabilitation interventions to
YACS in need after cancer treatment, acknowledging
rehabilitation as a process that requires adequate time
and follow-up.
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Ulrika Kreicbergs
Registered Nurse and Researcher at the
Department of Women's and Children's Health,
Karolinska Institute, Stockholm
Information about a child´s imminent death in
cancer – impact on family members long-term
psychological health
Objective
To assess the impact of communication in pediatric
oncology on bereaved family members’ long-term
psychological health.
Methods
Swedish parents and siblings who lost a child, brother
or sister to cancer 2-9 years earlier were invited to participate in postal questionnaire studies. 449 bereaved
parents out of 561 (80%) participated, 251 mothers
(56%) and 191 fathers. 174 out of 240 bereaved siblings (73%) participated, 73 (42%) brothers and 101
sisters. The analysis focused on the risk of psychological
morbidity.
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Results
Most parents (86%) want immediate information
about the incurability of their child’s malignancy. Short
awareness time (<24 hrs) about their child’s imminent
death commonly due to lack of relevant formation and
ongoing curative treatment was found to be associated
with a two fold increased risk of depression among
parents and an 8-fold increase risk of sick-leave among
fathers. Most siblings (55%) avoid talking to their parents about their deceased brother or sister in respect of
their parents feelings. This entails a two-fold increased
risk of anxiety.
Conclusion
Health care staff in pediatric oncology should provide
information about imminent death to family members
in attempts to mitigate long-term psychological morbidity after the loss of a child.
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Photo: Bergen Tourist Board / Philippe Brechet
NOPHO / NOBOS
Annual Meeting
9 – 13 May 2014 – Bergen – Norway
FREE PA P E R S
O R A L P R E S E N TAT I ONS
Overview
O–01Infringing on autonomy – an ethical concern
experienced by nursing staff
O–07Visiting nursing experiences working bedside
in the only pediatric oncology ward in Ethiopia
O–02Children and adolescents experience of
donating bone marrow / stem cells to
surviving siblings
O–08Study of children’s experiences with venous
port access (vap).
Need for a nordic study?
O–03Appetite, senses and joy of life – a nutrition
project.
O–04The process of striving for an ordinary,
everyday life, in preschool aged children living
with cancer, during the first year post diagnos.
O–05Home care nursing – since 2002 in Helsinki
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O–06Local anesthetic of intramuscular asparaginase
O–09Complications related to gastrostomy in
children and adolescents with cancer
O–10Kit for symptomatic relief for children in the
terminal stage
A N N U A L
M E E T I N G
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O–01
NOBOS O r a l P r e s e n tati o n
NO B O S O r al Pr esen tati on
O–02
Infringing on autonomy – an ethical concern
experienced by nursing staff
Children and adolescents experience of donating
bone marrow / stem cells to surviving siblings
Bartholdson, C., Lützén, K., Blomgren, K., Pergert, P. Women and
Children’s Health, Karolinska Institutet, Children’s Cancer Research
Unit Q6:05, Astrid Lindgrens´Hospital, Stockholm, Sweden
Carina Rinaldo, Katarina Vallin, Department of Pediatric Oncology
and Hematology, Uppsala University Children’s Hospital, Sweden
Background
A study regarding ethical issues and ways to deal with
them has been conducted in pediatric cancer care.
Ethical issues are common in pediatric care and arise
in connection with value conflicts within an individual
and/or between individuals, concerning which of the
possible options should be chosen. Each child’s specific
situation might lead to disagreements about treatment
and care. Furthermore, in pediatric care, children’s
growing autonomy has to be considered.
Objective
The purpose of this presentation is to describe one
of the ethical concerns which were identified in our
study and experienced by nursing staff when caring
for children with cancer.
Method
Physicians, registered nurses and nurse aides working
at a children’s hospital in Sweden answered a questionnaire. Qualitative content analysis was applied to the
open-ended answers.
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Results/ Conclusion
To infringe on a child’s autonomy is not to give the
child a chance to decide upon care related concerns
by her/himself or to oppose the child’s wishes and
perform actions and caring procedures that the child
does not want. Nurse and nurse aide participants
described children’s autonomy as something that can
be violated and they experienced powerlessness in these
situations. Inflicting suffering and limiting truth-telling
are subcategories to infringing on autonomy.
Health care professionals’ experiences of ethical concerns when, caring for children with cancer, seem to
produce strong feelings and moral confusion among
nursing staff. Not wanting to inflict suffering on the
child and feeling prevented from telling the truth
about the circumstances of the child’s illness are some
examples of nursing care responsibilities that often are
connected to medical treatment decisions.
[email protected]
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Background
Family and sibling relationships are affected in both
positive and negative ways when a child in the family
is afflicted with a severe illness during a long period
of time. There are few national studies conducted in
Sweden on how siblings who were bone marrow /
stem cell donors think and feel about their experience.
Objective
The aim of this study was to describe children’s and
adolescents’ experiences of donating stem cells to a
sick sibling in Sweden.
Method
A descriptive interview study with an inductive
approach was performed using qualitative content
analysis. The six participants were of both sexes and
between 11-21 years. They were recruited from three
different children’s transplant centers, had donated stem
cells before the age of 17 and all had surviving siblings.
N O P H O / N O B O S
Result
The theme proud heroes without a choice summarize
the results. The category proud but anxious to be a
donor describes a desire and a joy to help, but also
concerns how they would endure the procedures and
a concern of not being good enough as a donor. They
were very anxious for their sick sibling. The category
heroes without real choices in need of support highlights the strong family ties make them not having a
choice situation for the donation, but a need of support
from their environment, healthcare and from receiving
information but also all of its weaknesses.
Conclusion: These donors were happy to contribute to
the sibling’s recovery. They were proud and gained a
positive view of life from this experience. However the
questions remain who will consider the psychological
risks of these children and adolescents and if it is right
to expose young siblings to this risk.
[email protected]
[email protected]
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O–03
NOBOS O r a l P r e s e n tati o n
NO B O S O r al Pr esen tati on
O–04
Appetite, senses and joy of life
– a nutrition project
The process of striving for an ordinary, everyday
life, in preschool aged children living with
cancer, during the first year post diagnos.
Anne Kaspersen, Naja Panduro, Dorte Ellegaard Jensen, Children’s
Oncology Ward, 303B Aalborg University Hospital, Denmark
Laura Darcy, Paediatric Nurse, PhD student, Maria Björk, Paediatric
Nurse. Med Dr., Susanne Knutsson Paediatric Nurse, Med.Dr., & Karin
Enskär Paediatric Nurse, Professor of Nursing. Institution of Health
Science, University College Borås, Sweden
The project is towards children admitted to the paediatrics oncology ward at Aalborg University Hospital.
The purpose of the project is to ad focus on nutrition
to reduce the weight loss induced by chemotherapy.
And also minimize the need for tube feeding formula
and parenteral nutrition.
Our intend is to change the hospitalised child’s
perception of food, to generate new knowledge and
create the settings for, how the meal that favours the
children’s needs and wishes, can be implemented into
the paediatrics ward.
The project is an interdisciplinary project in co- operation with the paediatric oncology ward, the hospital
kitchen and the company Unisans. Both parents
and children have been included in the project with
interviews regarding wishes and needs concerning the
children’s diet. It has been studied, when the children’s
nutritional value is most threatened.
A new kitchen has been build, where the families can
cook and a new food concept has been developed
with better content and more exciting food serving.
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In addition, a new pamphlet with inspiration has
been developed.
Food shops where parents and children have been
cooking with a sense coach and chefs from Unisans
were a part of the project. The “food shop” turned
up every 14 days.
All of the results have not yet been calculated, but we
can conclude that a number of success criteria’s have
been fulfilled.
Greater fellowship, joy in the eating situation, children
who wanted to cook and participate in social gatherings including food. The children and teenagers have
become more outgoing and eat more of the food
served. We expect that the project will help improve
the children’s psycho-social development.
In the future, the experiences from this project can
be used when designing new eating environments in
paediatric wards.
[email protected]
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Background
The majority of children who receive a cancer diagnosis
are in the 1-to-6 year age group. Survival rates are
high, roughly 75%, but treatment is aggressive and
requires long and frequent hospital admissions and
causes adverse side effects. Health care focus is shifting
from surviving childhood cancer to living with it on a
daily basis. The young child’s experiences are crucial
to providing evidence based care.
Objectives/Aim
The aim of this study was to explore the everyday life
of preschool aged children as expressed by the child
and their parents during the first year post diagnosis
Methods
Interviews were conducted with children and their
parents connected to a paediatric oncology unit in
Southern Sweden. A qualitative content analysis of
interview data from three time points, shortly after
diagnosis, six months and one year post diagnosis
were made.
N O P H O / N O B O S
Results
A dramatic change in the young child’s everyday life
was described, with experiences of feeling like a stranger, under attack and lonely. Experiences over time
of gaining control, making a normality of the illness
and treatment and feeling lonely were described. This
process may be seen as a striving for an everyday life.
Conclusion
Nurses have a major role to play in the process of
striving the child goes through by giving and updating
information, making them participary in their care and
assuring access to both parents and peers. Addressing
these issues and updating them regularly can assist the
young child in their transition to living with cancer.
Longitudinal studies with young children are vital
in capturing their variety of experiences through the
cancer trajectory and necessary to ensure quality care.
[email protected]
A N N U A L
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O–05
NOBOS O r a l P r e s e n tati o n
NO B O S O r al Pr esen tati on
O–06
Home care nursing – since 2002 in Helsinki
Local anesthetic of intramuscular asparaginase
Eila Huotari. K10 Division of Hematology-Oncology and Stem Cell
Transplantation, Helsinki, Finland
Margareta af Sandeberg. Pediatric Hematology and Oncology, Astrid
Lindgrens Children’s Hospital, Karolinska University Hospital,
Stockholm, Sweden
K10 Division of Haematology-Oncology and Stem Cell
Transplantation includes three workstations: Stem Cell
Transplantation Ward, Chemotherapy Ward with Day
Hospital and Outpatient Clinic. All these cooperate
with our Home Care Unit.
The first paediatric cancer unit in Finland was founded
in 1982 in Helsinki University Central Hospital.
It took 20 years before we were able to begin to manage some of the nursing activities at the child’s home
instead of the hospital. One of our senior nurses started
to do home visits: taking blood samples and giving
some intravenous medications. Very important group
from the beginning were the children in palliative care.
Parents who had chosen to take care of their dying
child at home needed a lot of support and guidance.
We started our Home Care Unit with one nurse who
was able to make home visits five days a week; from
Monday to Friday. Now we have 1,5 nurses and they
work 7 days a week, however only during the daytime.
The aim of our home care nursing is to increase
humanity - to the child and the whole family. At home
parents often are more open-minded to take guidance
and tuition concerning their child’s treatment. Nurse
therefore has dedicated time only for the child and the
family during the visit without interruptions.
We have been trying to develop our home care nursing
to respond the family’s needs. One perspective is also
the economy: home care is less expensive than hospital
care. Our home care nurses consider their work manysided: they are able to work independently and still be
a member of the team.
This year we are facing a new exciting challenge: our
own small Home Care Unit is planned to unite to the
Pediatric Home Care Hospital.
[email protected]
Background
Asparaginase is an important part in the treatment of
acute lymphoblastic leukemia (ALL). In the protocol
NOPHO ALL 2000, used between 2002 and 2008,
ordinary E.Coli asparaginase was given by the intramuscular route due to lower frequency of severe allergic
reactions than the intra venous. This preparation was
hypotonic and painful. As a result of a Danish study
from 2005 the routine to inject lidocaine together with
asparaginase, was implemented in most of the Nordic
countries. In the protocol NOPHO ALL 2008 however, the pegylated form of E coli asparaginase (PEGasparaginase), dissolved in isotonic sodium chloride
was used and the pain due to a hypotonic solution was
eliminated. Initially data of PEG-asparaginase activity
mixed with lidocaine was missing and the routine with
lidocaine was kept at most hospitals although the risk
of severe pain had diminished. Lidocaine itself also
result in pain when it is injected.
Objectives/Aim
To compare children’s/parents’ experiences when intramuscular PEG asparaginase injection was given with
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and without lidocaine and to describe their perceptions
of pain and discomfort during the injections.
Method
During six month 2012/2013, all children (5-19 years)
in a children’s cancer center, with ALL, previously
treated with PEG asparaginase with lidocaine and
planned for at least two further treatments with PEG
asparaginase, was offered to try without lidocaine and
subsequently to choose whether the next treatment
injection should include lidocaine or not.
Results/Conclusion
All participants (n = 14) chosed to try without lidocaine. Nine reported less discomfort with no lidocaine
and three experienced no difference and the majority
considered the time factor as most positive. All participants preferred to continue without the injected
lidocaine. Based on the results of this study, lidocaine
is no longer injected together with PEG asparaginase
at this specific children’s cancer center.
[email protected]
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O–07
NOBOS O r a l P r e s e n tati o n
NO B O S O r al Pr esen tati on
O–08
Visiting nursing experiences working bedside in
the only pediatric oncology ward in Ethiopia
Study of children’s experiences with venous
port access (vap). Need for a nordic study?
R.N. ped.nurse specialist May Morken, Children’s Department,
Outpatient Clinic, Akershus University Hospital,
R.N. oncology nurse spacialist and coordinator Hilde Frøland Hauge,
Department of Pediatric hematology and Oncology, Children’s
Department, Rikshospitalet, University Hospital of Oslo, Norway
Steinunn E. Egeland. Department of Hæmatology and Oncology for
children, Department of Peadiatric Medicine, Woman and Children’s
Division, Oslo University Hospital, Rikshospitalet, Norway
Background:
Ethiopia is Africa’s second most populous country
with 92 million people; the majority live in rural areas.
Globally, cancer kills more people than malaria, HIV/
AIDS, and TB combined. Governments in low-income
countries are scaling up cancer care. In partnership
with International Network for Cancer Treatment
and Research USA (NGO) since 2011, Tikur Anbessa
Hospital in Addis Abeba opened a pediatric oncology
department (D7) in April 2013; 27 beds for children
up to 12. Currently, 257 children receive treatment,
but >3000/year probably have cancer, but are not
diagnosed or cannot make it to hospital. The vast
majority of families live in extreme poverty and receive
free treatment including chemotherapy. Most have
had little contact with formal medical care, so many
children have advanced disease. Ethiopians speak 90
languages; communication with families can be difficult. Orthodox Christianity is the dominant religion
(62%), however, one-third of Ethiopians are Muslim.
Aim:
Exploring the value of support, guidance, sharing
knowledge and experience with the nurses at D7.
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Method:
In May 2013, two nurses from Oslo, Norway (both
with >30 years experience in pediatric oncology), visited
D7 with Julia Challinor from INCTR to explore how
and if we could help.
Results/conclusion:
The conclusion was that we would return and work
bedside for the month of March 2014. Experiences
from both our visits will be shared including working
in a pediatric oncology unit
with frequent shortages of chemotherapy, antibiotics, supportive care supplies and equipment. How
the nurses’ prioritize their nursing care, including
chemotherapy preparation, will be described. Can
and should Nordic nurses join forces in supporting
colleagues in low-income countries with deficient
resources? What are our possibilities based on feedback
from the Ethiopian nurses?
[email protected]
[email protected] [email protected]
M E E T I N G
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Background
Most children with malignant diseases needcentral
venous catheters (CVC´s). There are two different
types of CVC´s; Hickman/Broviac (external access) and
VAP (internal access)Hickman is visible outside the
body, but easy to get access to. Vap is invisible under
the skin but has to be punctured with a needle to get
access to. We know little about children’s personal
experience’s with this procedure. We know, however,
that most children fear needles and that some children
with cancer find painful procedures as the worst part
of their illness.
Aim
Our aim is to study children’s experiences related to
needle access to their VAP. What is the level of anxiety
before the port access and pain during the insertion
of the needle?
Method
Population/inclusion criteria’s:
• Children and adolescents (0-18 years) with cancer
or haematological diseases who have had VAP for
3-6 months are included.
• Each child participates only once.
• Signed consent
N O P H O / N O B O S
Questionnaire
The child (if old enough), at least one of the parents
and a nurse are asked to fill out a questionnaire before
and immediately after the insertion of the needle into
VAP. The questionnaire includes 3 different pain score
scales validated for children of different ages:
FLACC (Face Legs Arms Cry Consolability) for
children < 3 years.
FPS-R (Faces Pain Scale – Revised) for children >
4-5 years.
NRS (Numeric Rating Scales) for children > 8 years.
Results / Conclusion
A pilot study of 6 children has been conducted successfully at Oslo University Hospital.The participants
report various levels of anxiety and/or pain and in most
cases nurses rate the discomfort higher than children/
parents. To gain power, we search for collaborative
Nobos/Nopho centres to increase the value of the study.
[email protected]
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O–09
NOBOS O r a l P r e s e n tati o n
NO B O S O r al Pr esen tati on
O–10
Complications related to gastrostomy in
children and adolescents with cancer
Kit for symptomatic relief for children in the
terminal stage
Tina Ekängen & Johanna Kjellberg, Pediatric Hematology and
Oncology, Astrid Lindgrens Children’s Hospital, Karolinska
University Hospital, Stockholm, Sweden
1
Background
Children and adolescents with cancer are at high risk
of suffering from malnutrition, which may affect their
tolerance of treatment and even influence the overall
survival. At long-term nutrition problems enteral nutrition via a gastrostomy is the best choice, even though a
gastrostomy causes complications. Fear of gastrostomy
complications may be one reason that they are not used
to the desired extent in pediatric oncology.
Aim
The aim of this study was to identify the presence of
gastrostomies and complications related to gastrostomy
in children and adolescents with cancer. The study was
carried out at the department of pediatric oncology
in Stockholm.
Method
Medical records of children and adolescents, 0-20 years,
diagnosed with cancer during approximately two and
a half year and having a gastrostomy inserted during
this period were retrospectively reviewed.
Result
Twenty-two medical records of seven girls and 15 boys
were reviewed. Eleven children had their gastrostomy
inserted before treatment started and 11 during ongoing treatment. Ninety-five percent of the children had
gastrostomy-related complications. The most common
complications were episodes of granuloma, pain and
local infection of the gastrostomy site. The children
who received their gastrostomy before treatment started
had less major complications than the children who had
their gastrostomy inserted during ongoing treatment.
Conclusion
Children and adolescents with cancer who had a gastrostomy often suffered from complications related to
their gastrostomy, however most of the complications
were minor. Hopefully this study can support nurses
and other caregivers in the nursing care of children
with cancer who suffer from malnutrition and those
who have a gastrostomy.
[email protected]
Marianne Bentsen1 Tone Høivik1, Mikael Donner2, Margrete Einen3,4
Centre for Pain Management and Palliative Care, Department of Anaesthesia and
Surgical Services, Haukeland University Hospital, Bergen, Norway
2
The Children’s Clinic, Haukeland University Hospital, Bergen, Norway
3
Regional Centre of Excellence for Palliative Care, Western Norway, Haukeland
University Hospital, Bergen, Norway
4
The Hospital Pharmacy in Bergen, Norway
Background
In the terminal stage unpleasant symptoms such
as pain, dyspnea, nausea, respiratory secretion and
anxiety / agitation frequently occurs. When children
die at home, good pain and symptom management
is important to create a safe environment, for both
the family and health professionals. Experience with
treating children in terminal stage varies. Since 2006,
a kit with drugs, equipment for administration and
treatment guidelines for adults in the terminal stage
has been in use. The experiences are good and a similar
kit for children has been asked for.
Materials and methods
A multidisciplinary group consisting of an anesthesiologist, consultant paediatric oncologist, oncology
nurse and hospital pharmacist has adjusted the kit for
adult to children. The drugs in the kit and treatment
guidelines are updated and adjusted for use in children;
the dosing is based on body weight.
The kit contains:
1. Instructions for use
2. Treatment guidelines
3. A summary of drug details
4. Instructions for use of subcutaneous needle
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5. E
quipment for subcutaneous administration
of drugs
6. Guideline and monitoring form for pain management in children
7. Form for evaluating the use and usefulness
of the kit
The kit has to be prescribed by a physician, the drugs
has to be specified in the prescription. The kit is filled
at the local hospital pharmacy. The drugs must be
administered by a doctor or nurse.
Conclusion
Good pain and symptom relief can be challenging in
children in the terminal stage. This kit is hopefully as
useful for planned home death in children as the similar
kit has been for adults. Treatment guidelines help to
give security for the family and for health professionals
that rarely treats dying children, and where the drugs
chosen and the dosages may be scarcely known.
Guidelines and other documents are available in
Norwegian from: www.helse-bergen.no/lindrendebehandling
[email protected]
A N N U A L
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NOPHO / NOBOS
Annual Meeting
9 – 13 May 2014 – Bergen – Norway
FREE PA P E R S
PO S TE R P R E S E NTAT I ONS
Overview
P–01
Children and adolescents experience of
donating bone marrow / stem cells to
surviving siblings
P–05
Severe oral mucositis in children diagnosed
with cancer in Iceland; prevalence, frequency
and risk factors
P–02
Establishing common information for
adolesents and young adults with cancer and a
joint forum for health care personnel
P–06 Complications of severe oral mucositis in
children diagnosed with cancer in Iceland
P–03 Improvement project for nutrition support of
paediatric hematopoietic stem cell transplant
patients
P–04Oral care and nutrition in children with cancer
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N O P H O / N O B O S
P–07
Timing the breaking point: a difficult ethical
problem in childhood cancer care
P–08
Kit for symptomatic relief for children in the
terminal stage
P–09A Swedish perspective on caring science
research in pediatric oncology: a litterateur
review
A N N U A L
M E E T I N G
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NOBOS P o s te r P r e s e n tati o n
NO B O S Poster Pr esen tati on
P–02
Children and adolescents experience
of donating bone marrow / stem cells to
surviving siblings
Establishing common information for
adolesents and young adults with cancer and a
joint forum for health care personnel
Carina Rinaldo, Katarina Vallin, Department of Pediatric Oncology
and Hematology, Uppsala University Children’s Hospital, Sweden
Sunniva Helland1, Hilde Beate Birkeland2, Therese Jørgensen3, Britt
Ingunn Wee Sævig4, Rune Hafslund2
Haukeland Universitety Hospital, Department of Pediatrics, Bergen, Norway
Department of Oncology and Medical Physics, Bergen, Norway
3
Department of Hematology, Bergen, Norway
4
The Norwegian Cancer Society, Bergen, Norway
1
Background:
Family and sibling relationships are affected in both
positive and negative ways when a child in the family
is afflicted with a severe illness during a long period
of time. There are few national studies conducted in
Sweden on how siblings who were bone marrow /
stem cell donors think and feel about their experience
Aim
The aim of this study was to describe children’s and
adolescents’ experiences of donating stem cells to a
sick sibling in Sweden.
Method: A descriptive interview study with an inductive approach was performed using qualitative content
analysis. The six participants were of both sexes and
between 11-21 years. They were recruited from three
different children’s transplant centers, had donated stem
cells before the age of 17 and all had surviving siblings.
Result
The theme proud heroes without a choice summarize
the results. The category proud but anxious to be a
donor describes a desire and a joy to help, but also
concerns how they would endure the procedures and
a concern of not being good enough as a donor. They
were very anxious for their sick sibling. The category
heroes without real choices in need of support highlights
the strong family ties make them not having a choice
situation for the donation, but a need of support from
their environment, healthcare and from receiving
information but also all of its weaknesses.
Conclusion
These donors were happy to contribute to the sibling’s
recovery. They were proud and gained a positive view
of life from this experience. However the questions
remain who will consider the psychological risks of
these children and adolescents and if it is right to
expose young siblings to this risk.
[email protected]
[email protected]
2
Background
Adolescents and young adults face major psychosocial
challenges diagnosed with cancer. Early recognition of
unmet practical and emotional needs at a critical developmental stage is essential. To facilitate developmentally
appropriate and coordinated care, continues support
and a good relationship with health care professionals
is essential. Moreover, providing necessary information
related to the disease and management across different
units is fundamental.
Objectives/Aim
The main objectives of the project were to 1) develop
and evaluate common information leaflet for adolescents and young adults with cancer (15-25 years) and
2) to establish a joint forum for health care personnel
to facilitate high quality cancer care and successful
transfer from pediatric to adult care services.
Methods
Nurses from two adult units and from pediatric care
were invited to participate in the project. Literature
and guidelines were reviewed to identify important
information necessary for decisions to be made on the
format and content of the leaflet guide. Outcomes to be
measured during the evaluation phase were identified.
Results /Conclusion
Follow-up routines differ between different adult
units as well as between the pediatric and adult units.
An information leaflet was designed, developed and
distributed within the hospital. The leaflet has been
revised twice, and efforts to develop individualized care
plans are in progress. A formalized forum for further
collaboration will be establish to continuously evaluate
and promote the outcome of the process. More work
is needed to develop additional recommendations to
promote successful transition in services.
[email protected]
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P–03
NOBOS P o s te r P r e s e n tati o n
Improvement project for nutrition support of paediatric hematopoietic stem cell transplant patients
Peltola Terttu, authorized nutritionist; Heino Terhi, authorized
nutritionist; Bamberg Sirpa, ward manager; Myllymaa Satu, nurse;
Leino Jaana, nurse; Arola Mikko, MD; Parto Katriina, MD; Virkki Marjo,
project coordinator
Paediatric hematology and oncology ward, Tampere University
Hospital, Finland
The aim of this improvement project was to develop
nutrition support given to malnutrition risk paediatric
hematopoietic stem cell transplant (HSCT) patients.
Baseline measurements were carried out in spring 2013,
literature review included, while in autumn, improvement measures, pilot study, final measurements and
report were planned and implemented.
Surveyed paediatric hematology and oncology ward
managers of Finnish University Hospitals saw incoherence in both nutrition support and patient brochures.
Five patients’ medical records were used to collect
information on nutrition status and the implementation
of nutrition support during nine 2012 HSCT ward
periods. Weight and length were regularly recorded
in electronic medical charts, but poorly in electronic
growth software. Malnutrition screening was nonexisting, and guidelines for nutrition support were only
found in the documentation related to small children.
All the children received parenteral nutrition two weeks
post-transplant, after which they ate mostly favourite
foods and snacks, and family foods.
Baseline interviews of nurses and doctors indicated
varying practices. Use of different growth software
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NO B O S Poster Pr esen tati on
was found confusing. Malnutrition screening was
regarded as a workable method, and its more active
use recommended. Increase in enteral nutrition was
emphasized. The interviewees suggested compiling a
patient brochure in line with the Helsinki University
Central Hospital (HUCH) guidelines.
As a result of the project, malnutrition screening form
was updated and converted into electrical format.
Documentation praxes were concluded and diet to
promote food safety was planned. A HSCT patient
nutrition brochure was compiled in co-operation with
experts of HUCH. Nutrition counseling given by
nutritionist was decided, and a form for monitoring
Children’s food intake during enteral or parenteral
nutrition developed. Ward’s food service underwent
several changes.
Due to lack of in-ward HSCT patients during pilot
study, the suggested improvements were tested on other
paediatric cancer patients. Follow-up will continue till
late September 2014.
Oral care and nutrition in children with cancer
Irina Käyrä and Anna-Maija Mertaniemi. Department of pediatrics
University hospital of Oulu, Children’s Hematology and Oncology
Ward, Finland
Background
One of the most important issues for the children’s
cancer treatment is good nutritional status and oral
health.
Objektives/Aim
The aim of our study was to evaluate an oral health
and nutrition promotion program for children with
cancer. The main purpose of our study was to improve
children’s oral health and nutrition during cancer treatments. By doing this Children’s feel better, children’s
treatment can progress as planned and hospitalization
can be reduced.
Method
We started in 2008 systematic co-operation between
Children’s hematologic and oncologic ward and
Institute of Dentistry. Co-operation occurs so that
special child´s dentist visit once a week on the children’s cancer ward and checks up Children’s health
of mouth. If necessary dentist will meet children more.
We pay more attention to malnutrition risk.
Evaluation form was taken in use at 2013. We pay at
least once a week attention to child´s health and nutri-
tional status. In situation of malnutrition, acquiring
nutrition balance is primaly recommended by using
of gastro-intestional.
Results/Conclusion
Now the mucositis treatment is more effective and we
have low lever laser in use. Child has better quality of
life, because his mouth is painless faster. The child is
able to eat and speak.
Malnutrition risks early identifying occurs in
increased use of Percutaneous Endoscopic Gastrostomy
(PEG) since 2008:
2008
2009
2010
2011
2012
2013
1/2014
1
0
2
3
1
5
2
[email protected]
[email protected]
M E E T I N G
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A N N U A L
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P–05
NOBOS P o s te r P r e s e n tati o n
NO B O S Poster Pr esen tati on
P–06
Severe oral mucositis in children diagnosed
with cancer in Iceland; prevalence, frequency
and risk factors
Complications of severe oral mucositis in
children diagnosed with cancer in Iceland
Oddný Kristinsdóttir, Barnaspítali Hringsins, Reykjavík, Iceland
Oddný Kristinsdóttir, Barnaspítali Hringsins, Reykjavík, Iceland
Background
Severe oral mucositis (3°-4°) is a frequent and severe
complication of chemotherapy in children undergoing chemotherapy. Limited data is available on the
prevalence and how to assess the extent of severe oral
mucositis.
Aim
To estimate the prevalence of severe oral mucositis in
children treated with chemotherapy in Iceland. Also
to examine the frequency and risk factors of severe
oral mucositis.
Method
A retrospective population-based study was performed.
A data recording form was designed to gather information from patients´ medical records on complications,
risk factors, assessment and interventions of severe oral
mucositis. The study sample was children from 1 to
18 years of age who received chemotherapy in Iceland
from 2002 to 2011. According to the Icelandic Cancer
Registry, 120 children were diagnosed with cancer
during this period, of which 64 were included in the
current study. Oral mucosal assessment scale WHO
and NIC were used to identify children with severe
oral mucositis.
Results
Twenty five (39%) children developed severe oral
mucositis, each up to 6 times (a total of 60 episodes).
Nine children (36%) had severe oral mucositis
once, but one six times. Children with severe oral
mucositis were significantly older (p = 0.008) than
those with mild or no oral mucositis. Sixty percent
of those developing severe oral mucositis were boys.
The highest frequency of severe oral mucositis was
among patients with acute leukemia, malignant bone
tumors and lymphoma. A significant correlation was
found between the number of days with neutropenia
and the number of days with symptoms of severe oral
mucositis (r = 0.736, p <0.0001).
A large proportion of children who are treated with
chemotherapy in Iceland develop severe oral mucositis.
This is in agreement with studies from other countries.
Incidence figures are inconsistent, most likely due to
different measuring methods.
Keyword
Severe oral mucositis, chemotherapy, prevalence, risk
factors, children.
[email protected]
Background
Severe oral mucositis (3°-4°) is a frequent and severe
complication of chemotherapy in children undergoing
chemotherapy. Limited data is available on the complications and consequences of severe oral mucositis
on children.
Aim
To examine the symptoms and complications of severe
oral mucositis in children treated with chemotherapy
in Iceland.
Method
A retrospective population-based study was performed.
A data recording form was designed to gather information from patients´ medical records on complications,
risk factors, assessment and interventions of severe oral
mucositis. The study sample was children from 1 to
18 years of age who received chemotherapy in Iceland
from 2002 to 2011. According to the Icelandic Cancer
Registry, 120 children were diagnosed with cancer
during this period, of which 64 were included in the
current study. Oral mucosal assessment scale WHO
and NIC were used to identify children with severe
oral mucositis.
Twenty five (39%) children developed severe oral
mucositis (a total of 60 episodes). In all episodes
the children had pain in the mouth and in 60% of
the episodes in the throat. In 95% of the episodes
the children could not eat solid food. The mean
number of symptoms was 3.5. Most children (76%)
received parenteral nutrition or enteral (NG-tube)
nutrition because of pain in the mouth or throat.
Blood cultures were drawn in 80% of the episodes
and antibiotics administered intravenously in 77%.
Bacteria were cultured in 21%, of which 50% were
considered contamination. The peak CRP value and
peak temperature were significantly higher for severe
oral mucositis compared to milder cases.
Children with severe oral mucositis are severely affected.
Pain is common and serious, impaired nutritional
intake is common, and the majority of children are
nourished intravenously or via NG-tube. Children are
at a increased risk of infections and a majority of cases
requires antibiotics intravenously.
Keyword
Severe oral mucositis, chemotherapy, complications,
symptoms, children.
[email protected]
Results
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NOBOS P o s te r P r e s e n tati o n
NO B O S Poster Pr esen tati on
P–08
Timing the breaking point: a difficult ethical
problem in childhood cancer care
Kit for symptomatic relief for children in the
terminal stage
Bartholdson, C., Lützén, K., Blomgren, K., Pergert, P.
Women and Children’s Health, Karolinska Institutet, Children’s
Cancer Research Unit, Astrid Lindgren’s Hospital, Stockholm, Sweden
Marianne Bentsen1Tone Høivik1, Mikael Donner2, Margrete Einen3,4
Background
The treatment for paediatric cancer is physically, socially
and psychologically demanding, and often gives rise to
ethical problems about treatment and care.
Objective
The purpose of this presentation is to describe a
difficult ethical problem frequently experienced in
childhood cancer care.
Method
Health care professionals from three paediatric units,
which all treat children with cancer, at a major hospital
in Sweden answered a study-specific questionnaire.
Qualitative content analysis was used to analyse answers
to open-ended questions.
Results / Conclusion
Different perspectives affected which treatment was
considered to be ethically right, especially when the
treatment was of uncertain benefit for the patient.
Writing about the breaking point, they described an
invisible point in time when the transition from curative to palliative treatment occurred. They expressed
that opinions diverged between physicians, nurses
and parents about when the breaking point occurred
and the breaking point was a major concern for all
professional groups and often led to conflicts in the
team. Physicians had to make the decisions regarding limitations of life-sustaining treatment and the
changed aim of the treatment. Uncertainty aroused
about when to make this decision to achieve the best
possible care for the patient. Nurses wished for the
breaking point to appear earlier in the care process
in relation to the perceived suffering of the children.
Parents were sometimes perceived to be blinded by the
fear of losing their child and by grief, resulting in them
wanting to continue with life-sustaining treatment as
long as possible.
Even though all health care professionals were focusing on what they thought was the best for the child,
they ended up having different perspectives of when
the breaking point occurred. There could be various
reasons for the differences including professional and
cultural as well as personal values or preferences.
[email protected]
Centre for Pain Management and Palliative Care, Department of Anaesthesia and
Surgical Services, Haukeland University Hospital, Bergen, Norway
2
The Children’s Clinic, Haukeland University Hospital, Bergen, Norway
3
Regional Centre of Excellence for Palliative Care, Western Norway, Haukeland
University Hospital, Bergen, Norway
4
The Hospital Pharmacy in Bergen, Norway
1
Background
In the terminal stage unpleasant symptoms such
as pain, dyspnea, nausea, respiratory secretion and
anxiety / agitation frequently occurs. When children
die at home, good pain and symptom management
is important to create a safe environment, for both
the family and health professionals. Experience with
treating children in terminal stage varies. Since 2006,
a kit with drugs, equipment for administration and
treatment guidelines for adults in the terminal stage
has been in use. The experiences are good and a similar
kit for children has been asked for.
Materials and methods
A multidisciplinary group consisting of an anesthesiologist, consultant paediatric oncologist, oncology
nurse and hospital pharmacist has adjusted the kit for
adult to children. The drugs in the kit and treatment
guidelines are updated and adjusted for use in children;
the dosing is based on body weight.
The kit contains:
1. Instructions for use
2. Treatment guidelines
3. A summary of drug details
4. Instructions for use of subcutaneous needle
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5. E
quipment for subcutaneous administration
of drugs
6. Guideline and monitoring form for pain management in children
7. Form for evaluating the use and usefulness
of the kit
The kit has to be prescribed by a physician, the drugs
has to be specified in the prescription. The kit is filled
at the local hospital pharmacy. The drugs must be
administered by a doctor or nurse.
Conclusion
Good pain and symptom relief can be challenging in
children in the terminal stage. This kit is hopefully as
useful for planned home death in children as the similar
kit has been for adults. Treatment guidelines help to
give security for the family and for health professionals
that rarely treats dying children, and where the drugs
chosen and the dosages may be scarcely known.
Guidelines and other documents are available in
Norwegian from:
www.helse-bergen.no/lindrendebehandling
[email protected]
A N N U A L
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P–09
NOBOS P o s te r P r e s e n tati o n
A Swedish perspective on caring science research
in pediatric oncology: a litterateur review
Karin Enskär, RN, PhD, Professor of Nursing, Maria Bjork, RN, PhD, Laura
Darcy, RN, MPH, School of Health Sciences, Jonkoping University
Sweden and CHILD research Group, Jonkoping University, Sweden
Background
The body of research-based knowledge in pediatric
caring sciences has been increasing thanks to a dramatic improvement in outcomes related to research
and advances in treatment.
Objectives/Aim
The aim of this review was investigate the content
of published studies in pediatric oncology related to
caring sciences.
Method
A systematic litterateur review of 137 published articles on pediatric oncology related to caring science in
Sweden was performed.
Results / Conclusion
The result shows that most of the studies were descriptive or comparative studies with a quantitative design.
Most of them had parents in focus, and only 22% had
focus on the child. Most of the studies investigated wellbeing, using questionnaires or interviews. The result,
as stated in the articles, demonstrated that the child´s
disease has affected the wellbeing of all people coming
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in contact with the child, in both positives and negative
ways. Also the child´s disease causes distress related to
physical, psychological, existential and social aspects.
Several mediating factors for the experience of distress
and wellbeing were found, as; disease and treatment
severity, gender, time since diagnose, and the use of
internal and external support. Frequent reported health
promoting aspects were: family togetherness, coping
strategies and engaging in activities and normal life, as
well as quality of care; as emotional support, information and family participation in care. Suggestions for
clinical implications, stated in the articles, were often
described in a diffuse manner making translation
into clinical practice difficult. However, some areas of
clinical implications could be identified and described.
To reflect the child’s perspective in pediatric oncology
requires that future researchers take on the challenge
of including children. The biggest challenge for the
future would be to make a shift from explorative studies to intervention studies. There is an urgent need to
transform research results into clinical practice.
Åpning:
fredag 9. mai kl. 1800
Møt kunstnerne til
samtale og visning:
lørdag 10. mai kl. 1400
søndag 11. mai kl 1800
[email protected]
M E E T I N G
Kreftforeningens lokaler
Strandgaten 62 i Bergen
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B E R G E N
2 0 1 4
Gjennom maleriene søker duoen å
inspirere og motivere betrakteren
gjennom visuelle fortellinger. Som for
eksempel om øyeblikkene der et medmenneske eller en hendelse gir livet
ny retning, der gleden ved å virke, det
å bety noe for noen eller noe kjennes
altomfattende. Ofte formidles det
glade, energiske og humoristiske, av og
til det såre, sorgfulle og vanskelige.
Malerduoen
Bringager & Malmstrøm
HEL
Velkommen til maleriutstilling i Kreftforeningens
lokaler i forbindelse med NOBOS og NOPHO´s
nordiske konferanse i Bergen i mai 2014.
Eva Malmstrøm
[email protected]
tlf: 932 51 572
Hanne Bringager
[email protected]
tlf: 930 95 922
9th - 13th May
NOPHO/NOBOS 2014 Bergen
NOPHO / NOBOS
ANNUAL MEETING
9 – 1 3 M a y 2 0 1 4 – B e r g e n – N o r w a y
Meeting venue
Radisson Blu Royal Hotel, Bergen
Bryggen 5, 5003 B ergen | radissonblu.com/royalhotel-bergen
Formål
- å bedre barnas helhetlige behandlings- og
rehabiliteringstilbud
- å støtte og tilrettelegge forholdene for familiene
- å samarbeide med leger, pleiepersonell og andre
som har omsorg for barna
- å drive informasjonsvirksomhet omkring
sykdommen og Barnekreftforeningen
- å søke positivt samarbeid med andre foreninger
der dette er naturlig
TELEFON: 02099
[email protected]
Photos: Radisson Blu Royal Hotel, Bergen
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N O P H O / N O B O S
M E E T I N G
A N D
C O N G R E S S
–
B E R G E N
2 0 1 4
BESØKSADRESSE: Øvre Vollgate 11, 0158 Oslo
POSTADRESSE: Postboks 4 Sentrum, 0101 Oslo
ORG. NUMMER: 985 550 999
www.barnekreftforeningen.no
NOPHO
Nordic Society of Paediatric Haematology and Oncology
Annual Meeting
Main topics
• NHL: genetics, allotransplant for ALCL, new drugs in NHL
• Immunodeficiencies: diagnostic challenges,
SCT and viral infections after SCT
• Fertility and pregnancy after childhood cancer
• Educational session: Genetics of MDS, Immunodeficiencies,
treatment of osteosarcoma
WELCOME
Please see more info http://www.nopho.org/
2015
Organizing committee:
Merja Möttönen
Satu Lehtinen
Hanna Juntti
Riitta Niinimäki
Anne Hekkala
Minna Honkila
Conference secretariat
May 22nd - May 26th
Oulu, Finland
Aira Raudasoja (Ms)
Congress Manager, CongCreator CC Oy
[email protected]
tel +358 (0)500 604 686
Venue
Radisson Blu Hotel
Hallituskatu 1, 90100 Oulu, Finland
NOPHO / NOBOS
ANNUAL MEETING
9 – 1 3 M a y 2 0 1 4 – B e r g e n – N o r w a y
T han k y o u
f o r att en d i n g!