During adolescence, patients with BCS present less commonly with ascites and may present with hepatomegaly alone. JAK-2 V617F mutation is the most common thrombophilic disorder during adolescence; though thrombophilic disorders are less common in adolescents than adults. Response to therapy is similar to adults, but better than children.

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Intra-hepatic portal-venous collaterals are characteristic of Budd-Chiari syndrome (BCS) and are usually of small caliber and seen on Doppler. Creation of large portal-systemic shunt, either radiologically (Transjugular intrahepatic porto-systemic shunt) or surgically results in excellent long term outcomes in BCS. Here, we report a series of three rare cases of asymptomatic BCS, who had spontaneous large intra-hepatic portal-systemic shunts.

Fully covered SEMS is safe and effective for refractory benign non-corrosive esophageal strictures.

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Renal tubular acidosis (RTA) is not uncommon in patient with chronic autoimmune hepatitis (AIH), but usually remains latent. Here, we report a case of renal tubular acidosis RTA who presented with AIH. She was also diagnosed to have partial bowel malrotation. A 9-year-old girl, a case of distal RTA, presented with jaundice, abdominal distension and altered sensorium. She was diagnosed to be AIH, which was successfully treated with steroids and azathioprine. Coexistent midgut partial malrotation with volvulus was diagnosed during the treatment. She was treated successfully with anti-tuberculous treatment for cervical lymphadenitis. Autoimmune hepatitis should not be ruled out in each case of RTA presenting with jaundice.How to cite this articleModi TK, Parikh H, Sadalge A, Gupte A, Bhatt P, Shukla A. Autoimmune Hepatitis with Distal Renal Tubular Acidosis and Small Bowel Partial Malrotation. Euroasian J Hepato-Gastroenterol 2015;5(2):107-109.

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