Download A young girl with chronic sufferings

A young girl with chronic
sufferings ( cystic fibrosis )
Presented by
Dr. Jalal Mohsin Uddin
DTCD , FCPS (Pulmonology)
Chief complains :
Shortness of breath has increased for 7
days
 Fever for 7days
 Productive cough for same duration

History of present illness:


According to the statement of the patient’s mother, her
child has been suffering from recurrent cough and
fever since her 9 months of her age .
The cough is preceded by fever. It is always
productive. Its volume has increased and become
purulent in last three years . Occasionally it is foul
smelling and blood stained. Cough is more in the
morning. In last seven days it has become so severe
that it interrupt her sleep and associated with copious
amount of purulent sputum.
H/O present illness (continue)

The young girl has been suffering from
recurrent fever since her child hood. It is
usually high grade and is subsided by taking
antipyretics and antibiotics. In last seven days
the fever was continuous in nature and always
high grade. Highest recorded temperature is
103F.
 She has been suffering from shortness of
breath for last three years. It is progressive in
nature, initially it occurred during running but
now she become breathless even in mild
exertion.
History of present illness (continue)
She also complain about recurrent
headache which aggravate during attack
of fever.
 She often suffer from passage of loose
stool, mucoid in nature, not blood stained
.
 Though she is 14yrs old, she reads in
class IV. Her mother complain about her
delayed physical growth .

Treatment history :
Patient has received various type of
antibiotics , antipyretic frequently.
 Previously patient was admitted in
different hospitals in three occasions . In
April 1995 she was admitted in Dhaka
Shishu Hospital and was diagnosed as
severe pneumonia . Later on she was
admitted in NIDCH in two occasions on
August 2006 and November 2007.

Treatment history (continue)

During this period of her admission she
received cat – l anti tubercular therapy
two times . She was never smear
positive patient .
H/O past illness :

Patient is non diabetic , she has not
suffered from measles or whooping
cough . She has never come into
contact with any patient suffering from
tuberculosis.
Family history :

The girl is from a low socio economic
status . She has two sisters and one
brother, all are in good health . There is
no history of consanguineous marriage
among her parents.
Immunization history :

She was immunized against tuberculosis
whooping cough , measles and other
infectious diseases in due time .
General examination :






Appearance: ill
looking
Behavior:
Cooperative
Conscious
Decubitus:
According to her
choice.
Clubbing: Present
Koilonychia,
leuchonychia:
Absent
General examination (continue)
Anemia , cyanosis , jaundice , oedema :
absent .
 Lymphadenopathy : absent .
 Neck vein : not engorged .

Examination of respiratory system:

Inspection : shape and size of the chest is
normal , there is no chest deformity ,
respiratory rate – 24 breaths/min , intercostal
recession present , chest movement bilaterally
symmetrical .
 Palpation : Trachea is centrally placed , apex
beat is in left 5th intercostal space just medial
to the left mid clavicular line . Chest movement
bilaterally symmetrical . Total chest expansion
2 cm. Vocal fremitus normal in both side .
Examination of respiratory system
(continue)
Percussion : it is normal in both side .
Upper border of liver dullness is present
in 6th intercostal space along the right
midclavicular line .
 Auscultation : Breath sound vesicular ,
bilateral coarse crepitation present ,
which alter after coughing . Vocal
resonance is normal in both side .

Examination of CVS
Apex beat is in 5th intercostal space just
medial to the left mid clavicular line . It is
normal in character . There is no left
parasternal heave . Pulmonary
component of 2nd heart sound is not
palpable .
 Both 1st and 2nd heart sound is audible
with normal character . Murmur is absent
.

Examination of other systems
There is no hepato-spleno megaly,
ascites absent .
 Other systemic examination revealed
normal finding .

Salient features :

Kulsum a 14yrs young girl admitted with the
complain of increased shortness of breath,
productive cough and fever for 7 days.
According to her mother the girl has been
suffering from repeated bout of productive
cough , fever and shortness of breath since
her 9 months age . For this she was admitted
to various hospitals and received antibiotics,
even Cat-1 two times though she was smear
negative. Patient having clubbing and bilateral
coarse crepitations .
Provisional diagnosis:

Post tubercular
bronchiectasis
Differential diagnosis :

Bilateral
bronchiectasis due
to recurrent
pneumonia or
 Cystic fibrosis or
 Primary ciliary
dyskinesias etc.
Investigations
Total count of WBC : 13000/cu mm
 Differential count : Neutrophil : 78%,
lymphocyte : 18%
 ESR : 40 mm in 1st hr
 Sputum for AFB (3 sample) : negative
 Sputum for C/S : shows growth of
pseudomonus sensitive to Gentamycin
and Ceftriaxone .

X-ray chest P/A view showing

There are multiple ring
shadows involving all
the zones of both lung
fields specially
prominent in upper and
mid zones in right side ,
mid and lower zones in
left side. There is
intervening fibrous band
within the ring shadows.
X-ray chest (continue)

Trachea is slightly
shifted to the right.
Cardiac shadow is
normal in transverse
diameter. Costo-phrenic
angles and cardiophrenic angles of both
sides are clear. Radio
logically it is bilateral
bronchiectasis with
fibrosis .
Sweat test

Sweat electrolytes of
the patient :
 Sodium- 197.90 m
mol/l
 Chloride- 190.60 m
mol/l

REMARKS :
 both ions >70m mol/l
is indicative of CF.
 both ions <50m mol/l
is normal
 If in between 50-70m
mol/l is equivocal
need further
evaluation.
Investigations (continue)
FBS - 110 mg/dl ( indicate IFG)
 Blood sugar 2hrs after 75 gm glucose :
130 mg/dl.
 Serum billirubin – 0.7mg/dl
 SGPT- 17u/l
 X- ray PNS- Maxillary sinusitis
 USG of whole abdomen : Normal study.
 Saccharin test : within normal limit .

CT scan of chest

Bilateral
bronchiectasis with
fibrosis.
Confirmatory diagnosis

Bilateral
bronchiectasis with
fibrosis due to cystic
fibrosis.
Treatment given
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Complete bed rest.
Oxygen inhalation
Physiotherapy include breathing exercise
huffing , coughing , chest percussion , postural
drainage etc.
Inj Cefuroxime was started empirically then inj
gentamycin was added according to culture
sensitivity report .
Nebulization with salbutamol and ipratropium
Theophylline etc .
Comments :

Bronchiectasis is not a disease rather it is
ultimate presentation of various chest
diseases . So diagnosis of underlying causes
will be beneficial for the patients.
 Our institute is very much able to diagnose the
underlying causes of bronchiectasis .
 Like asthma and COPD , bronchiectasis
having a chronic course . Regarding its
management this may deserve a definite
guideline . At the same time health education
may play a great role .
…………………………………………
THANK
YOU ALL