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 …

While liver involvement is common in cystic fibrosis, the major liver disorder with impact on
the clinical outcome of individuals with CF is the development of multilobular cirrhosis with …

Objectives: Hepatobiliary complications are a leading cause of morbidity and mortality in
cystic fibrosis (CF) patients. Knowledge of the underlying pathological aspects and optimal …

Hepatobiliary disease is a common finding in patients with cystic fibrosis; the pathogenesis
is multifactorial in nature. As new therapeutic strategies emerge, life expectancy will …

Cystic fibrosis (CF) is the most common autosomal recessive genetic disorder in white
people, 1, 2 and is also one of the most lethal. 3, 4 It is caused by a mutation in the gene …

Background Liver disease is an important complication in CF. Aims To determine if CFLD is
a risk factor for mortality in CF, and which baseline characteristics predict all-cause mortality …

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 …

Incidence of liver disease (LD) associated with cystic fibrosis (CF) and its clinical
characterization still is unsettled. We have assessed prospectively the incidence and risk …

C herited disease that disrupts ion transport in epithelial-lined organs, is the most common
potentially lethal genetic disorder of the Caucasian population, affecting between 1 in 2000 …

Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described
in pediatric populations. Adult‐onset CFLD lacks sufficient characterization and diagnostic …