PMID: 7535738Apr 1, 1995Paper

Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency

Hepatology : Official Journal of the American Association for the Study of Liver Diseases
D L WatersK J Gaskin

Abstract

Focal and multilobular biliary cirrhosis are considered pathognomonic of cystic fibrosis (CF) and almost invariably have been reported in patients with steatorrhea. In contrast, patients with pancreatic sufficiency and normal absorption are considered less likely to develop liver or biliary tract problems. The authors report three patients with CF and pancreatic sufficiency, presenting with recurrent abdominal pain (unrelated to pancreatitis). All had common bile duct disease, one with multilobular cirrhosis and portal hypertension. Pancreatic sufficiency was proven by quantitative pancreatic stimulation tests, 3-day fecal fat analyses, and serum pancreatic isoamylases. All three patients had mild lung disease. Two were homozygous for the common delta F508 mutation, and the other, a delta F508 compound heterozygote. Hepatobiliary structure and function were determined by serial hepatobiliary scintigraphy, percutaneous transhepatic cholecystography, and biochemical liver function tests. Patients 1 and 3 had mild hepatomegaly, normal liver biochemistry, and distal common bile duct strictures. Patient 2 had a firm nodular liver with splenomegaly, abnormal liver biochemistry, and a cholangiographic appearance of sclerosing cholangi...Continue Reading

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Citations

Dec 24, 1998·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·T ChinetC Housset
Dec 19, 2007·Surgery Today·Toru IkegamiGoran B Klintmalm
Nov 4, 2006·Current Treatment Options in Gastroenterology·Catherine Brigman, Andrew Feranchak
Jun 26, 2001·Pathology International·S IwasaT Watanabe
Oct 14, 2009·Journal of Gastroenterology and Hepatology·Don Cameron
May 29, 1999·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·N Dray-CharierC Housset
Apr 4, 2006·The Surgical Clinics of North America·Robert D Winfield, Elizabeth A Beierle
Dec 8, 2004·Journal of Hepatology·Thierry LamireauFernando Alvarez
Jun 1, 2006·Journal of the American College of Surgeons·Maximilian BockhornChristoph E Broelsch

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