Recommendations for management of liver and biliary tract disease in cystic fibrosis

RJ Sokol, PR Durie… - Journal of pediatric …, 1999 - journals.lww.com
Although involvement of the liver and biliary tract has been recognized as a complication of
cystic fibrosis (CF) since Anderson's initial description of CF in 1938 (1), the clinical …

Gastrointestinal manifestations of cystic fibrosis: a review

RW Park, RJ Grand - Gastroenterology, 1981 - Elsevier
Cystic fibrosis is the most common, lethal, genetic defect of white populations (1). Once
limited largely to infants and children, the immediate prognosis has changed drastically over …

[PDF][PDF] Natural history of liver disease in cystic fibrosis

A Lindblad, H Glaumann, B Strandvik - Hepatology, 1999 - Wiley Online Library
The median age of the population with cystic fibrosis (CF) has increased worldwide, which
has led to the suggestion that the prevalence of liver disease would increase. The aim of this …

Splenomegaly, hypersplenism and coagulation abnormalities in liver disease

PA McCormick, KM Murphy - Best Practice & Research Clinical …, 2000 - Elsevier
Splenomegaly is a frequent finding in patients with liver disease. It is usually asymptomatic
but may cause hypersplenism. Thrombocytopenia is the most frequent manifestation of …

Prevalence of liver disease in cystic fibrosis.

R Scott-Jupp, M Lama, MS Tanner - Archives of disease in childhood, 1991 - adc.bmj.com
To assess the prevalence, demography, and clinical features of liver disease among
patients with cystic fibrosis the case notes of 524 patients of all ages who were attending the …

Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature

PA di Sant'Agnese, PB Davis - The American Journal of Medicine, 1979 - Elsevier
Cystic fibrosis is now the most common cause of chronic obstructive pulmonary disease
(COPD) and of pancreatic insufficiency in the first three decades of life in the United States …

Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension

D Debray, P Lykavieris, F Gauthier, B Dousset… - Journal of …, 1999 - Elsevier
Background/Aim: Variceal bleeding is the most severe complication in patients with cystic
fibrosis-associated liver cirrhosis, who often do not have severe respiratory failure. The …

[PDF][PDF] Bile‐duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis

A Lindblad, R Hultcrantz, B Strandvik - Hepatology, 1992 - Wiley Online Library
To study the liver disease of patients with cystic fibrosis, percutaneous liver biopsies were
performed in 10 patients with cystic fibrosis aged 6 to 22 yr. Nine of 10 patients had high …

Liver cirrhosis and portal hypertension in cystic fibrosis

O Efrati, A Barak, D Modan-Moses… - European journal of …, 2003 - journals.lww.com
Objectives Liver disease is the second cause of death in cystic fibrosis. The most deleterious
complication of liver disease is portal hypertension, which has an estimated prevalence of …

Ursodiol for hepatobiliary disorders

RA Rubin, TE Kowalski, M Khandelwal… - Annals of internal …, 1994 - acpjournals.org
Purpose: To explain the rationale supporting the use of ursodiol (ursodeoxycholic acid) for
the treatment of patients with cholesterol gallstones and chronic liver diseases and to …