Cystic fibrosis (CF) is the most common autosomal recessive disease in the Caucasian
population. Cystic fibrosis-related liver disease (CFLD) is defined as the pathogenesis …

Highly effective CFTR modulator drug therapy is increasingly available to those with cystic
fibrosis. Multiple observational research studies are now being conducted to better …

Cystic fibrosis liver disease (CFLD) is characterised by a wide heterogenity of manifestations
and severity. It represents a major cause of morbidity in people with cystic fibrosis (PwCF) …

Cystic fibrosis (CF) may cause a spectrum of hepatobiliary complications, including portal
hypertension, multilobular cirrhosis, and liver failure. Current guidelines on the detection …

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 …

Objective To assess if a heterogeneous pattern on research liver ultrasound examination
can identify children at risk for advanced cystic fibrosis (CF) liver disease. Study design …

Objectives: Cirrhosis occurs in 5% to 10% of cystic fibrosis (CF) patients, often accompanied
by portal hypertension. We analyzed 3 adverse liver outcomes, variceal bleeding (VB), liver …

Abstract Background and Objective Reliable markers are needed for early diagnosis and
follow-up of liver disease in Cystic Fibrosis (CF). The objective was to evaluate the …

Background Ursodeoxycholic acid (UDCA) might prevent progression of cystic fibrosis liver
disease, but objective parameters for its effect are lacking. Methods We used liver stiffness …

Background Pediatric radiologists can identify a liver ultrasound (US) pattern predictive of
progression to advanced liver disease. However, reliably discriminating these US patterns …