Current Pediatric Research

Sure, there is great association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD). AS about 60% of PSC patients have or will develop ulcerative colitis. Despite immunological dysregulation mostly have role in both entities, there is no clear definite aetiology for each separate disease or explaining the relation of both. Case presentation We describe male adolescent named Mustafa Mahmoud aged 15 years old attended at hepatology clinic at Mansoura university hospital complaining 1 year ago of easy fatigability, recurrent nonspecific abdominal pain and citric sclera. Laboratory markers (mild elevated aminases with high total IGG and immune marker) were suggestive initially of auto immune hepatitis or PSC waiting for pathological diagnosis to be done but 2 month later, the patient developed excess weight loss with mucoid diarrhea and moderate recurrent abdominal pain when he was admitted to be scooped (upper and lower) to exclude IBD. Gross endoscopic finding with pathological analysis confirm moderate diffuse ulcerative colitis. Starting steroid and amino salicylic acid (azathioprine was spared due to thrombocytopenia) gave response with regard to general condition, resolved abdominal pain, also diarrhea improved and platelet count was elevated Unfortunately, 4 month later during steroid tapering, Mustafa showed severe relapse with severe bloody diarrhea and HG drop. After stabilizing general condition, biological therapy was decided to be started during grand round, but after only 2 doses, Mustafa was attacked by severe fulminant sepsis and died during ICU admission. Conclusion ? Cases of overlap syndrome (PSC and ulcerative colitis) are not rare in pediatric population. Close and meticulus observation should be given to patients presented with one system affection to recognize any developed hepatological or gastroenterological symptoms or signs for fear of overlap. ? Other point is biological therapy in IBD patient as much as it would be useful, it carry lethal complication.

Author(s): Mansour Sakr

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