Infantile diarrhoea and splenomegaly

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Infantile diarrhoea and splenomegaly

  1. 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. 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. 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. 4. Past History • • • • 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. 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. 6. On examination • • • • • • 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. 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. 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. 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. 10. • What next ?
  11. 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. 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. 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. 14. Final diagnosis Autoimmune enteropathy with autoimmune hepatitis

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