Clinical meeting Paediatrics

1. Dr. KANTA HALDER
Resident (MD;Phase A)
BICH

2. Particulars of the patient
 Name: Meem
 Age: 2years 1month
 Sex: Female
 Informant: Mother
 Address: Savar,Dhaka
 Date of Admission: 20.04.2015
 Date of Examination: 22.04.2015

4. Chief Complaints
 Gradual pallor since 1 year of age
 Gradual abdominal distension for
last 1 year

5. History of present illness:
According to the statement of mother,Meem
was reasonably well 1 year back. Then she
became pale gradually since her 1 year of age.
She also complainded of gradual abdominal
distension for last 1 year.
With these complaints, she was treated in a
local hospital and referred to Dhaka Shishu
Hospital for further evaluation and better
management.

6. History of Past illness:
She had no significant past illness.
Birth History :
She was delivered by NVD at term at home
without any perinatal complication.

7. Feeding History:
She was on exclusive breast feeding upto 6
months of her age.Then proper complementary
feeding was started.Now she is on family diet.
Immunization History:
She is immunized as per EPI schedule.

8. Familly History :
She is the 2nd issue of her non-consanguinous
parents.Her elder brother and other family
members are healthy.
Socio-economic History :
She came from a low socio-economic
background. She lived in a tin-shed house,drinks
boil water and use sanitary latrine.

9. Treatment History
She got blood transfusion for 2 times after
admission in this hospital .
Developmental History
She is developmentaly age appropriate.

10. General Examination :
 Appearance: Dysmorphic face (frontal bossing
,depressed nasal bridge)
Anaemia: Moderate
Jaundice: Mild
Cyanosis:
Clubbing: Absent
Oedema:
Dehydration:

11. Cont..
Skin: BCG mark present
Bony tenderness: Absent
Lymphnode: Not palpable
Signs of meningeal irritation: Absent
Neck Vein: Not engorged
Ear:
Nose: Normal
Throat:

12. Cont..
Vital Signs:
Heart Rate: 100 beat/min
Respiratory Rate: 26 breath /min
Temperature: 99°F
Blood Pressure: 90/50 mmHg

13. Anthropometry:
Cont..
Weight: 9.5 kg
Height: 79cm
WAZ: -2.7 (moderately underweight)
HAZ: -2.2 (moderately stunted)
WHZ: -1.5 (mildly wasted)

14. Systemic Examination
Hemopoietic System:
Mouth and Oral Cavity: Normal
Anaemia: Moderate
Jaundice: Mild
No lymphadenopathy
No bony Tenderness

15. Inspection: Abdomen is distended,
umbillicus is centraly placed
Palpation:
• Liver: Palpable , 1 cm from right costal
margin along right mid clavicular line
which is non tender ,surface is smooth,
regular border .Upper border of liver
dullnes lies at right 5th intercoastal
space.
Abdomen:

16. Cont..
• Spleen: Palpable , 5cm from left costal
margin along it’s long axis which is non
tender,surface is smooth.Splenic notch
present.
• Fluid thill: absent
Percussion:Shifting dullness absent
Auscultation: Bowel sound present
Other Systemic examination: No abnormality

17. Salient feature
Meem ,a 2 years 1 month old girl,2nd issue of
her non-consanguinous parents was admitted
in this hospital with the complaints of gradual
pallor since 1 year of age ,gradual abdominal
distension for last 1 year. Meem was
moderately pale, mildly icteric, having
dysmorphic face. She had hepato-
splenomegaly without ascitis. Other systemic
examination reveals no abnormality.

18. Provisional Diagnosis:
Congenital hemolytic anaemia most probably
Beta thalassemia major
Differential Diagnosis:
 Hemoglobin E-beta thalassemia
 Hereditary spherocytosis

19. Investigations:
Complete Blood Count :
• Hb: 6gm/dl
• WBC: Total count: 9,500/cumm
Differential count:
o Neutrophil: 66%
o Lymphocyte: 28%
o Monocyte: 03%
o Eosinophil: 03%
o Basophil : 00%

20. Cont..
o RBC:Gross aniso-poikilocytosis with
predominant microcytic
hypochromic cells,many
schistocytes,polychromatic
cells,few target cells and NRBCs
o WBC:Mature with above
distribution
o Platelet: Adequate
• Platelet : 280,000/cumm
• PBF:

21. Cont..
• RBC Indices:
o RBC Count: 2.72m/µL
o HCT: 16%
o MCV : 57 fl
o MCH : 18 pg
o MCHC : 32 g/dl
o RDW: 33%
S.bilirubin: 1.2 mg/dl
Blood grouping and Rh typing: B (+ve)

22. Cont..
Hemoglobin electrophoresis:
• HbA : 07.04%
• HbF : 27.30%
• HbE : 65.66%
Comments: Hemoglobin E-Beta thalassemia

23. FINAL DIAGNOSIS
Hemoglobin E-Beta thalassemia

24. Management :
A.Counseling: -About disease,treatment option
and complication
-Genetic counseling
B.Supportive:
1.Packed Cell Transfusion: 10ml/kg
2.Tab.Folic Acid
3.Dietary Advice
4.Advice for -Regular blood Transfusion
-Adequate chelation

25. Cont..
C.Specific treatement:
-Haemopoietic stem cell tranplantation
-Gene therapy
-Modulation of Fetal hemoglobin
D.Follow up

26. Thank You