[Cholestasis caused by chronic pancreatitis in childhood. Surgical treatment and genetic analysis].
Authors report two cases of childhood chronic pancreatitis, causing severe symptoms and common bile duct stenosis with cholestasis. Both patients had to be operated on. Chronic pancreatitis with calcification led to significant common bile duct stenosis in a 13 years old girl. After ERCP a double bypass procedure was performed (Wirsungo-jejunostomy and hepatico-jejunostomy). During 42 months follow-up the patient remained pain- and symptom-free gaining 16 kilograms. In a 9 years old girl severe stenosis of the intrapancreatic common bile duct and a small duct type chronic pancreatitis with extensive fibrosis was found. Treatment was Roux-en-Y hepatico-jejunostomy. Thirty-four months after the operation she is symptom-free with normal enzyme parameters. Authors report results of genetic investigations performed on registered chronic pancreatitis children and their families in Hungary, including the two operated cases. Two of the 5 patients were hereditary type, despite negative family history. Cationic trypsinogen gene R122H (R117H) mutation were detected in both patients. Chronic non-hereditary pancreatitis is a very rare disease in childhood but may cause severe secondary conditions requiring surgery.
Oláh
A
A
Petz Aladár Megyei Oktató Kórház, Sebészeti Osztály.
Ruszinkó
V
V
Garab
E
E
Horváth
V
V
Vasi
I
I
D000293:Adolescent; D001662:Biliary Tract Surgical Procedures / Q000379:methods; D002648:Child; D002779:Cholestasis / Q000209:etiology* / Q000235:genetics; D002908:Chronic Disease; D003135:Common Bile Duct / Q000473:pathology*; D003251:Constriction, Pathologic; D005260:Female; D005355:Fibrosis; D006801:Humans; D006814:Hungary; D008297:Male; D009154:Mutation; D010195:Pancreatitis / Q000150:complications / Q000201:enzymology / Q000235:genetics* / Q000601:surgery*; D014362:Trypsinogen / Q000235:genetics*
D014362:Trypsinogen