Abstract
Three (3.0%) of the 102 patients with cystic fibrosis (CF) attending our CF-center were shown to have ERCP-findings indicating sclerosing cholangitis. All had pulmonary symptoms. Two of them, both females (aged 17 and 20 years), presented with abdominal pain while a 26 years old male was asymptomatic. One of the females also had an unspecific colitis. Two of the patients had persistently altered liver function tests with increase of serum concentration of transaminases and glutamyltransferase while one normalized her laboratory findings. This patient, who was the only one with a microgall bladder, showed no progress during a three years follow up. Liverbiopsies revealed portal fibrosis, bile duct proliferation and an inflammatory reaction and in one case heavy steatosis. Congenital anomalies, biliary calculi, previous operative trauma and malignancy was not found.
Primary sclerosing cholangitis is usually related to inflammatory bowel diseases which may be associated with a wide variety of extraintestinal lesions including pulmonary disease. Gastrointestinal manifestations such as liver and biliary diseases are well known complications to CF. To the best of our knowledge this is the first report of sclerosing cholangitis in this disease.
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Hjelte, L., Gabrielsson, N. & Strandvik, B. 2. SCLEROSING CHOLANGITIS IN CYSTIC FIBROSIS. Pediatr Res 22, 96 (1987). https://doi.org/10.1203/00006450-198707000-00023
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DOI: https://doi.org/10.1203/00006450-198707000-00023