Diarrhea and Hepatosplenomegaly..
Ravindra Kumar Sharma - TNMC, Nair, Mumbai
Pediatric Gastroenterologist, D.Mangeshikar Hospital, Pune
Pediatric Gastroenterologist, Kanchi K C Trust Hospital, Chennai
Girish Gupte -Pediatric Hepatologist,
Birmingham Children's Hospital, UK
Rajesh Sainani - Gastroenterologist,
Jaslok Hospital, Mumbai
Diarrhoea and hepato-splenomegaly
Dr. Ravindra Kumar Sharma
Post Graduate, Dept. of Pediatrics , T.N.M.C
& Nair Hospital, Mumbai.
Under the guidance of:
Dr. Aabha Nagral
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
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
• 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
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
Child conscious ,oriented
Anthropometry: Weight- 21 kg - 50th centile.
No pallor, icterus, lymphadenopathy , clubbing.
No dysmorphic features.
Abdominal examination: liver- 2 cm soft, non tender, spleen
• Other systems: NAD
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
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
(Duodenal biopsies in infancy ?suggestive of autoimmune enteropathy and had been
given 6 month course of prednisolone)
• Started on oral prednisolone and azathioprine and
• Now child’s weight is appropriate for age and LFT normalised
over a period of 6 months of treatment
Autoimmune enteropathy with