Swine Models of Cystic Fibrosis Reveal Male Reproductive Tract Phenotype at Birth

Cystic fibrosis from genotype to phenotype: review article

Cystic fibrosis (CF) is the most common autosomal recessive genetic disease, which is caused by defection in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR gene codes chloride channels to modulate the homeostasis of epithelial environments. Defective CFTR affects various organs such as the lungs, pancreas, intestine, liver and skin; however, lung impairment is the mai...

Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets.

Diabetes is a common comorbidity in cystic fibrosis (CF) that worsens prognosis. The lack of an animal model for CF-related diabetes (CFRD) has made it difficult to dissect how the onset of pancreatic pathology influences the emergence of CFRD. We evaluated the structure and function of the neonatal CF endocrine pancreas using a new CFTR-knockout ferret model. Although CF kits are born with onl...

Male fertility in cystic fibrosis.

Infertility rates among males with cystic fibrosis (CF) approximate 97%. No information is currently available within Ireland determining an understanding of fertility issues and the best methods of information provision to this specialized group. This study aimed to determine understanding and preferred approaches to information provision on fertility issues to Irish CF males. A Descriptive St...

Cystic fibrosis and the month of birth.

Viral Respiratory Tract Infections in Cystic Fibrosis

Cystic Fibrosis (CF) is the most commonly inherited potentially lethal disease amongst Caucasian children and young adults. In Europe, approximately 35,000 children and adults are affected by CF. The prevalence in the US and in Canada is approximately 30,000 and 3,000, respectively. CF is an autosomal recessive disorder and is caused by mutations in the Cystic Fibrosis Transmembrane Conductance...