Cystic fibrosis (CF) be a genetic disorder wey be inherited insyd an autosomal recessive manner wey dey impair de normal clearance of mucus from de lungs, wich dey facilitate de colonization den infection of de lungs by bacteria, notably Pseudomonas aeruginosa den Staphylococcus aureus. CF be a rare genetic disorder wey dey affect mostly de lungs, buh sanso de pancreas, liver, kidneys, den intestine. De hallmark feature of CF be de accumulation of thick mucus insyd different organs. Long-term issues dey include difficulty breathing den coughing up mucus as a result of frequent lung infections. Oda signs den symptoms fi include sinus infections, poor growth, fatty stool, clubbing of de fingers den toes, den infertility insyd most males. Different people fi get different degrees of symptoms.
Cystic fibrosis be inherited insyd an autosomal recessive manner. E be caused by de presence of mutations insyd both copies (alleles) of de gene wey dey encode de cystic fibrosis transmembrane conductance regulator (CFTR) protein. Those plus a single working copy be carriers den otherwise mostly healthy. CFTR be involved insyd de production of sweat, digestive fluids, den mucus. Wen de CFTR no be functional, secretions wey be usually thin instead cam be thick. Na de condition be diagnosed by a sweat test den genetic testing. De sweat test dey measure sodium concentration, as people plus cystic fibrosis abnormally get salty sweat, wich fi often be tasted by parents wey dey kiss dema kiddies. Screening of infants at birth dey take place insyd sam areas of de world.
Der be no known cure for cystic fibrosis. Lung infections be treated plus antibiotics wich fi be given intravenously, inhaled, anaa by mouth. Sam times, dem dey use de antibiotic azithromycin long-term. Inhaled hypertonic saline den salbutamol sanso be useful. Lung transplantation fi be an option if lung function dey continue to worsen. Pancreatic enzyme replacement den fat-soluble vitamin supplementation be important, especially insyd de young. Airway clearance techniques such as chest physiotherapy fi get sam short-term benefit, buh long-term effects be unclear. De average life expectancy be between 42 den 50 years insyd de developed world, plus a median of 40.7 years, although na improving treatments contribute to a more optimistic recent assessment of de median insyd de United States as 59 years. Lung problems be responsible for death insyd 70% of people plus cystic fibrosis.
CF be most common among people of Northern European ancestry, for whom e dey affect about 1 out of 3,000 newborns, den among wich around 1 out of 25 people be a carrier. E be least common insyd Africa den Asia. Na dem first recognize am as a specific disease by Dorothy Andersen insyd 1938, plus descriptions wey dey fit de condition wey dey occur at least as far back as 1595. De name "cystic fibrosis" dey refer to de characteristic fibrosis den cysts wey dey form within de pancreas.
