Biliary complications of cystic fibrosis.

  title={Biliary complications of cystic fibrosis.},
  author={S. O’Brien and Mary Keogan and M. Casey and G. J. Duffy and Dympna McErlean and Muiris X. FitzGerald and John E. Hegarty},
  pages={387 - 391}
One hundred and four adult patients with cystic fibrosis were evaluated for the presence of liver disease as defined by abnormal liver function tests of six months' duration, histological evidence of fibrosis or cirrhosis, or the presence of portal hypertension, or both. Twenty patients fulfilled these criteria and were evaluated further for the presence of biliary tract abnormalities with biliary scintigraphy using 99Tc diisopropylphenyl-carboxymethyl iminodiacetic acid (DISIDA) and endoscopic… 

Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group.

  • R. SokolP. Durie
  • Medicine
    Journal of pediatric gastroenterology and nutrition
  • 1999
The Cystic Fibrosis Foundation has recognized the need to revise the previous Recommendations for Management of Liver and Biliary Tract Disease in Cystic fibrosi, published in 1989, to reflect the new scientific advances and clinical information in this area.

[Diagnostic screening of liver disease in cystic fibrosis].

The results of this study enhance the need for longitudinal assessment in order to define cases of liver disease in CF and suggest several clinical and laboratory variables should be considered.

Liver Disease in Cystic Fibrosis

  • D. Debray
  • Medicine
    Pediatric Hepatology and Liver Transplantation
  • 2019
Several noninvasive tools to measure liver stiffness as an indirect measure of fibrosis are being evaluated among the pediatric CF population in order to identify patients at risk of cirrhosis and to document disease progression.

The gallbladder and biliary tract in cystic fibrosis

The administration of ursodeoxycholic acid appears to be beneficial in patients with end-stage liver disease and future treatments involve targeted gene therapy and activation of mutant forms of the cystic fibrosis transmembrane conductance regulator.

The role of hepatobiliary scintigraphy in cystic fibrosis

It is proposed that scintigraphy with DISIDA has a role in the detection of early liver involvement in cystic fibrosis.

Natural history of liver disease in cystic fibrosis

Liver disease was no more frequent at the end of the study period although the median age of the patient population had increased, and modern treatment might positively influence liver disease because it seemed less common, less progressive, and less serious than previously reported.

Review of the abdominal manifestations of cystic fibrosis in the adult patient.

Manifestations in the pancreas and abdomen are common and affect multiple organ systems, and awareness of these manifestations is important to successfully guide management of cystic fibrosis in adult patients.

Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients.

The relation between abnormal liver architecture at US and results of three liver function tests in children with CF was significant and the most specific US abnormalities related to abnormal function are signs suggestive of portal hypertension and cirrhosis.

Ursodeoxycholic Acid in Cystic Fibrosis-Related Liver Disease: A Systematic Review

The pathogenesis of liver involvement in CF remains unclear, partly because there are still insufficient long-term data on large numbers of patients, and the risk factors for the development of liver disease associated with CF showed no difference in allele frequencies between those with and those without liver involvement.



Liver disease and common-bile-duct stenosis in cystic fibrosis.

It is concluded that strictures of the distal common bile duct are common in patients with cystic fibrosis and liver disease, and fasting levels of serum bile acids were elevated in nearly half of these patients, irrespective of the severity of their liver disease.

A distinctive type of biliary cirrhosis of the liver associated with cystic fibrosis of the pancreas; recognition through signs of portal hypertension.

It is shown that in patients with cystic fibrosis of the pancreas the initial hepatic lesions may progress to severe and at times fatal clinical manifestations due to a distinctive type of multilobular biliary Cirrhosis, and that this disease must be included among the important causes of portal hypertension secondary to cirrhosis of the liver in the pediatric age group.

Sclerosing cholangitis in cystic fibrosis.

Four of 102 patients with cystic fibrosis with symptoms or signs suggesting sclerosing cholangitis had typical findings at endoscopic retrograde cholangiography (ERC), indicating this syndrome. All


The pathology and pathologic physiology of celiac disease remain obscure in spite of the many attempts that have been made to understand them and few cases of either disease in which careful clinical observations have been followed by adequate postmortem examination are revealed.


The aim has been to recognize the disease as early in life as possible and to provide a comprehensive medical program which includes all aspects of health care.