Abstract Approximately 5–10% of cystic fibrosis (CF) patients develop multilobular cirrhosis
during the first decade of life. Most CF patients later develop signs of portal hypertension …

Meconium ileus (MI) is often the first manifestation of cystic fibrosis (CF) and occurs in
approximately 20% of patients diagnosed with CF. This article reviews the pathophysiology …

Peribiliary glands (PBG) are a source of stem/progenitor cells organized in a cellular
network encircling large bile ducts. Severe cholangiopathy with loss of luminal biliary …

Context A subset (≈ 3%-5%) of patients with cystic fibrosis (CF) develops severe liver
disease with portal hypertension. Objective To assess whether any of 9 polymorphisms in 5 …

Cystic fibrosis-associated liver disease (CFLD) affects ca. 30% of patients. The CFLD is now
considered the third cause of death, after lung disease and transplantation complications, in …

Cystic fibrosis (CF) is a common lethal genetic disease in the North American population,
affecting 1: 2500 births. There are about 30,000 individuals in the United States with CF, and …

Liver disease is a relatively frequent and early complication of cystic fibrosis. The
pathogenesis is apparently multifactorial, with contributions from environmental and genetic …

Meconium ileus at birth, distal intestinal obstruction syndrome (DIOS), and constipation are
an interrelated group of intestinal obstruction syndromes with a variable severity of …

Malnutrition is one of the main burdens of disease in cystic fibrosis (CF) along with lung
disease. Data from the most recent Cystic Fibrosis Foundation registry report show the …

Objective Cystic fibrosis associated liver disease (CFLD) is the third largest cause of
mortality in CF. Our aim was to define the burden of CFLD in the UK using national registry …