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 …

The effects of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators
on lung function, pulmonary exacerbations, and quality of life have been well documented …

The interplay between airway inflammation and infection is now recognized as a major
factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen …

Highly effective modulator therapies (HEMTs) have revolutionised the management
approach of most patients living with cystic fibrosis (CF) who have access to these therapies …

Children with elevated liver enzymes are occasionally discovered through laboratory work-
up from different clinical scenarios. Although the majority will have transient and/or benign …

With the improved treatment of the pulmonary complications of cystic fibrosis (CF),
gastrointestinal problems have become more important in the morbidity in CF. A hallmark of …

Biliary atresia (BA) and choledochal cysts are diseases of the intrahepatic and extrahepatic
biliary tree. While their exact etiopathogeneses are not known, they should be treated …

In this series of articles with comments and illustrations on the World Federation for Medicine
and Biology (WFUMB) guidelines on contrast-enhanced ultrasound (CEUS) the topics of …

Objective To develop a scientific consensus on nutrition in cystic fibrosis. Methods Sixteen
coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which …

Hepatobiliary involvement is a hallmark in cystic fibrosis (CF), as the causative CF
Transmembrane Conductance Regulator (CFTR) defect is expressed in the biliary tree …