En ärftlig sjukdom i de exokrina körtlarna som drabbar främst bukspottkörteln, andningsvägarna och svettkörtlarna, och som brukar börja i barnaåren. Sjukdomen kännetecknas av kroniska luftvägsinfektioner, nedsatt kapacitet hos bukspottkörteln och benägenhet för värmeutmattning. Skrumplever under barndomen förekommer ofta och kan leda till portahypertension, mjältförstoring och hypersplenism.
An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
Inga svenska synonymer finns.
Fibrosis, Cystic — Mucoviscidosis — Pulmonary Cystic Fibrosis — Cystic Fibrosis, Pulmonary — Pancreatic Cystic Fibrosis — Cystic Fibrosis, Pancreatic — Fibrocystic Disease of Pancreas — Pancreas Fibrocystic Disease — Pancreas Fibrocystic Diseases — Cystic Fibrosis of Pancreas