Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. This gene encodes a protein involved in epithelial anion channel. Cystic fibrosis is the most common life-limiting genetic disorder in Caucasians; it also affects other ethnic groups like the Blacks and the Native Americans. Cystic fibrosis is considered to...

This study evaluated adherence to current dietary recommendations of children with cystic fibrosis and mild lung disease and their siblings by comparing energy intake. Fifty children (25 with cystic fibrosis) aged between 7 and 12 years completed the study. Energy intake was assessed by weighed dietary intake, resting energy expenditure was used to calculate recommended daily intakes. The child...

BACKGROUND In term newborns meconium ileus is frequently associated with cystic fibrosis. Reports on meconium ileus in preterm infants being diagnosed with cystic fibrosis early after birth are very scarce. Associations between genotype and phenotype in cystic fibrosis and its particular comorbidities have been reported. CASE PRESENTATION Two extremely preterm twin infants (26 weeks of gestat...

BACKGROUND Pancreatic destruction affects the majority of patients with cystic fibrosis. We aimed to relate ultrasound findings to exocrine pancreatic function and cystic fibrosis genotype. METHODS Patients with cystic fibrosis and a matched group of healthy controls were included. We performed transabdominal ultrasound, and recorded echo intensities of the pancreas and parenchymal characteri...

Cystic fibrosis (CF) is an inherited disease that primarily affects the lungs and the digestive system, however, it also affects a number of other organs and systems. More than 90% of mortality of  CF patients is due to lung complications.  Healthy lungs are important for a long life for people with CF, We will discuss two important topics for maintaining respiratory health. Chronic use of drug...

The cystic fibrosis transmembrane regulator (CFTR) should no longer be viewed primarily as a ‘chloride Abstract channel’ but recognized as a channel that also controls the efflux of other physiologically important anions, such as glutathione (GSH) and bicarbonate. More effective approaches to cystic fibrosis treatment may result from this reconceptualization of the CFTR by researchers and clini...

Three recently sequenced strains isolated from patients during an outbreak of Mycobacterium abscessus subsp. massiliense infections at a cystic fibrosis center in the United States were compared with 6 strains from an outbreak at a cystic fibrosis center in the United Kingdom and worldwide strains. Strains from the 2 cystic fibrosis outbreaks showed high-level relatedness with each other and ma...

Chronic inflammation of the lungs is a major cause morbidity and mortality in patients with cystic fibrosis. One macrolides, azithromycin has antimicrobial, immunomodulatory, anti-inflammatory effects that are useful diseases chronic inflammatory processes such as This systematic review aimed to determine efficacy safety administration for fibrosis improving lung function. Pulmonary function wa...