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von Hippel Lindau syndrome presenting as obstructive jaundice with involvement of pancreas in two siblings
S K Issar1, N Kumar, A K Sachdeva
1Department of Gastroenterology, Radiology and G.I. Surgery, G.B. Pant Hospital, New Delhi, India.
Abstract:
Von Hippel Lindau disease is rare autosomal dominant disorder. In our patient's family, 4 out of 7 siblings had manifestations of the disease; 3 of those affected had pancreatic lesions with obstructive jaundice in 2 siblings caused by pancreatic cysts is reported for the first time.