1. Diarrhea and Hepatosplenomegaly..
Presenter:
Ravindra Kumar Sharma - TNMC, Nair, Mumbai
Moderator:
Vishnu Biradar
Pediatric Gastroenterologist, D.Mangeshikar Hospital, Pune
Pediatric Gastroenterologist, Kanchi K C Trust Hospital, Chennai
Panelists:
Girish Gupte -Pediatric Hepatologist,
Birmingham Children's Hospital, UK
Rajesh Sainani - Gastroenterologist,
Jaslok Hospital, Mumbai

2. Diarrhoea and hepato-splenomegaly
Presented by:
Dr. Ravindra Kumar Sharma
Post Graduate, Dept. of Pediatrics , T.N.M.C
& Nair Hospital, Mumbai.
Under the guidance of:
Dr. Aabha Nagral

3. Case summary
A 5 year old boy, 2nd by birth order, born of a non
consanguineous marriage was found to have an incidental
finding of a moderate splenomegaly by the pediatrician
during routine examination

4. Past History
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The child was a full term normal delivery , no H/O NICU stay
The child was gaining weight adequately till age of 5months
History of multiple admission in view of diarrhea.
1st admission at 6wks of age treated with IV fluids and
antibiotics.
• 2nd admission - e/o acute gastroenteritis at 5 months of age with
UTI ( Klebsiella ). USG, MCU and DMSA scan normal
• From 5 months of age onwards had chronic diarrhea with
failure to thrive (weight < 3rd centile) with features suggestive
vitamin and mineral deficiencies

5. Past History continued:
• Diarrhea was large volume, not associated with blood or mucus.
There was no history of fever
• Was treated with IV fluids, intermittent parental nutrition,
antibiotics, probiotics, supplements and elemental diet.
Investigated for the same
• stool showed fat globules with trace reducing substance
• serum liver and renal biochemistry-normal
• thyroid function tests -normal
• Antigliadian antibody negative
• Serum Immunoglobulins – low normal
• Cystic fibrosis genetic analysis- negative
• Underwent an upper GI scopy and jejunal biopsy following which
given a 6 month course of medications and improved

6. On examination
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Child conscious ,oriented
Vitals stable
Anthropometry: Weight- 21 kg - 50th centile.
No pallor, icterus, lymphadenopathy , clubbing.
No dysmorphic features.
Abdominal examination: liver- 2 cm soft, non tender, spleen
3cm, firm
• Other systems: NAD

7. Investigations
Investigation
Value
T.PROTEIN/
S.ALBUMIN
S.Globulin
8.4
3.6/
4.8
S.G.P.T
415
S.G.O.T
315
ALK.PHOS
212
GGT
73
Hb
10.6
MCV
71
Retic
1.5
Platelets
132
PT/INR
15/14/secs
INR 1.08

8. Summarising..
• 5 year male child with chronic diarrhea in infancy -treated
• Now with splenomegaly and liver dysfunction under
evaluation
What are the differential diagnosis?

9. Investigstions
Investigations
Results
HBsAg
Negative
Anti HCV
Negative
ANA
1:320
ASMA
Weak positive
Anti LKM Ab
Negative
Sr. Ceruloplasmin
Normal
24 hr U Copper
35 mcg -normal

10. •
What next ?

11. Investigation cont…
Serum protien electrophoresis done –
Increased Gamma globulins(30.6; normal- 7.9-18.9%)
Liver biopsy done
Gastroscopy done - No evidence of varices, Duodenal biopsies
taken

12. Investigation continued…
Liver biopsy - Interface hepatitis – lymphocytes, plasma cell and neutrophils with
rossette formation and pan lobular inflamation. HAI score 11/18 and fibrosis score 1/6
Duodenal biopsy – Mild villous blunting
Lamina propria shows increased
lymphoplasma cell infiltrate with
lymphoid aggregates
Intraepithelial lymphocytes
(Duodenal biopsies in infancy ?suggestive of autoimmune enteropathy and had been
given 6 month course of prednisolone)

13. Treatment
• Started on oral prednisolone and azathioprine and
supplements
• Now child’s weight is appropriate for age and LFT normalised
over a period of 6 months of treatment

14. Final diagnosis
Autoimmune enteropathy with
autoimmune hepatitis