Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study
Section snippets
Patients
Twenty-seven adult CF patients (16 males and 11 females; mean age 27 (19–42)) referred to the Adult Cystic Fibrosis Center were prospectively included from May 1996 to May 1998, whatever their hepatobiliary status. Diagnosis of CF had been made by the association of typical symptoms of pulmonary or pancreatic disease together with at least two abnormal chloride concentrations on sweat testing. Body mass index was 20.1 (13.6–25); 23 patients had pancreatic insufficiency and four diabetes
Biological data and ultrasound findings
Nine of the 27 patients had clinical, morphological or biological evidence of liver involvement, defining the group II. One had clinical and ultrasound criteria of cirrhosis with a nodular appearance (16) and portal hypertension (esophageal varices, portal dilatation and splenomegaly). Three patients had clinical hepatomegaly with a heterogeneous appearance on ultrasonography, and repeated abnormal liver function tests (LFT). One patient had clinical hepatomegaly, normal LFT, and a homogeneous
Discussion
The 33% prevalence of clinical or biological involvement of the liver in our 27 patients is similar to published data in adult patients with CF 1., 2.. Intrahepatic biliary anomalies were shown in 18 out of 26 MRC studies (69%), very close to the prevalence of biopsy and autopsy studies in adult patients 4., 5.. All the patients with known liver disease had an abnormal appearance of their intrahepatic biliary tract, suggesting good sensitivity of the MRC, although the number of patients in this
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