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Morning Report
August 24, 2009
Visual Diagnosis
You are seeing a 10 year old AA
male with fever, increased WOB,
chest pain and cough. His RA
saturation is 92%
What else do you want to know???
• Duration of symptoms has been only 1 day.
There has been no improvement with tylenol
or tylenol with codeine (foster mom had some
left over from her hospitalization and tried
one)
• The temp at home has been as high as 102.
• No sick contacts.
• PMH: this is foster mom and she has no
information about this child as she has had
him for only 3 weeks. His mom died from a
drug overdose and his dad is not involved and
unknown to the child.
He looks unwell……what do you
do??
Labs??
Images??
Fluids??
The CXR is done
The CBC comes back:
Hgb is 7 Hct is 21
WBC is 21 (no differential yet) PLTs “nl”
Lytes are normal
LFT’s are mildly elevated (200’s)
Differential Diagnosis
Time ???
HINT (your turn to talk)
After 20 cc per kg of NS, his
dyspnea worsens and you are
called to the bedside by a
frightened ER RN. Your patient
has diminished BS on the right.
He is crying because he feels as
if he cannot breathe. He has
been placed on a nonrebreather mask and his
saturation is 96%
Now what?
The repeat CXR
Time to circle your number one
thought…….10 year old AA male
dyspnea, fever, chest pain, mild
cardiomegaly, rapidly progressive
change in CXR, significant anemia,
mild elevations in LFT’s
Sickle cell disease with
Acute Chest Syndrome
Sickle Cell Statistics
•1 in every 375 AA in USA
• 1 in every 1200 Latino
•9% AA in US carry trait
•Up to 25% in parts of Africa
carry trait
•NBS in 44 states & DC
•Life Expectancy 45; (Tripled
over Last 30 years)
normal
Not normal
Molecular Mechanisms
Single Nucleotide substitution (GTG
for GAG) @ codon 6 Beta globin
gene on chromosome 11
Valine substitution for glutamine
causes hypoxia induced
polymerization leading to the classic
crescent shape
•Increased adhesiveness
•Increased reticulocytes
•Vasoconstriction (+ET1 and –NO)
•Abnormal NO metabolism
•Elevated Plts and Thrombin Activity
Acute Chest Syndrome
• 50% of patient with SCD will experience
at least one episode
• Typically present with fever, cough, chest
pain, SOB, new infiltrate, hypoxia
• Atelectasis
hypoxemia
sickling
• 50% admitted with another diagnosis
Acute Chest Syndrome
• Cause identified in 55% (30% Infection,
16% presumed pulmonary infarction, 9%
Fat Embolism)
• In Children, Infection More Likely
Infectious Agents: Chlamydia, Mycoplasma
and RSV
• 20% have bronchospasm responsive to
bronchodilators (Corollary: Asthmatics
have a two fold higher risk of ACS)
Management
•Oxygen
•IVF
•Pain Control
•Antibiotics (CTX & Azithro)
•Bronchodilators
•Incentive Spirometer
•Ambulation
•Simple vs. Exchange Transfusion
Goal HbS <30% & Final Hb no more than 10
Best Practices for SS Disease
• Penicillin prophylaxis prevents pneumococcal
sepsis in children [evidence from Prophylactic
Penicillin Studies I and II(PROPS I & II)].
• Pneumococcal vaccine prevents
pneumococcal infection in children.
• In surgical settings, simple transfusions to
increase hemoglobin (Hb) levels to 10g/dL are
as good as or safer than aggressive
transfusions to reduce sickle hemoglobin (Hb
S) levels to below 30 percent.
More Best Practice for Children
• Transfusions to reduce Hb S levels to
below 30 percent prevent strokes in
children with high velocity central
nervous system blood flow by TCD
[evidence from the Stroke Prevention
Trial in Sickle Cell Anemia(STOP I)].
• Hydroxyurea decreases crises in
patients with severe sickle cell disease
[evidencefrom the Multicenter Study of
Hydroxyurea in Sickle Cell Anemia (MSH)
trial].
Take Home Messages
• SCD is common
• SCD impacts every organ system
• ACS is treated with diligent supportive
care
Two more Cases from PREP
A 6month old girl, who was born in Nigeria, presents
for an urgent visit as soon as the family arrives in the
United States because of fever and irritability.
Physical examination reveals a fussy infant who has
anorexia, a temperature of 100°F (37.8°C), and
swelling of all of the fingers of the right
hand . The remainder of the examination
findings are negative.
Of the following, the MOST likely cause of this pattern
of swelling in this child is
A. cellulitis
B. juvenile idiopathic arthritis
C. malaria
D. sickle cell disease
E. trauma
You are evaluating a 7year old boy who has sickle cell disease for a 5 day
history of fever, rhinorrhea, headache, and worsening fatigue.
Physical examination reveals a tired appearing, pale boy
VS: 101.5°F (38.6°C); HR: 150
very pale conjunctivae and mucous membranes
He has a faint diffuse erythematous lacy rash that is most prominent on
his cheeks and trunk.
Laboratory tests show a white blood cell count of 12 with 50%
neutrophils, 45% lymphocytes, and 5% monocytes; hemoglobin of 4 ,
hematocrit of 16 and a reticulocyte count of less than 1% (0.01).
Of the following, the MOST likely cause for this patient's present illness
is infection with
A. coxsackievirus
B. cytomegalovirus
C. EpsteinBarr virus
D. parainfluenza virus
E. parvovirus B19
Parvo B19 Fifth’s Disease