PubMed · 7148113
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B Schott, L Safrany, B Neuhaus. 1982-09-30. [The large gallbladder].. https://pubmed.ncbi.nlm.nih.gov/7148113/
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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 cholangitis. All have undergone operative treatment for persistent abdominal pain. These cases confirm the occurrence of common bile duct pathology and liver disease in patients with CF and pancreatic sufficiency. They demonstrate that liver and biliary tract disease can occur independently of the underlying disease severity and the presence of steatorrhea. Further, they suggest that obstruction of the biliary tract may be an additional factor in the evolution of liver disease in CF.
The procedures used for imaging of the gastrointestinal tract, the gall bladder and the bile ducts were registered in six Norwegian hospitals during the period 1975-90. Data obtained from all hospitals showed a significant decrease in radiological examinations of the stomach and the large bowel, and a dramatic decrease of such examinations in the case of the gall bladder and bile ducts. The changes are mainly due to the increasing use of newer methods such as ultrasonography, gastroscopy and colonoscopy. The shift in the pattern of examinations, as shown in the present study, may well have many implications for patients and for the health service as a whole.
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