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Cystic fibrosis (also known as CF or mucoviscidosis) is an autosomal recessive genetic disorder affecting most critically the lungs, and also the pancreas, liver, and intestine.
Cystic fibrosis is an inherited (genetic) disease that can present with symptoms within the first two years of life. The genetic defect results in the production of thick mucus in the lungs, pancreas, liver, small intestine, and reproductive organs.
CFRD occurs in some 20% of adolescents and 40–50% of adults affected by CF. [3] Though rare in children, it has been described in CF patients of all ages, including infants. Beginning in the teenage years, CFRD has an annual incidence of ~3%, and may be more common in females. It is associated with more severe CF gene mutation types. [4]
Cystic Fibrosis Research Institute has implemented strategies to increase awareness in underrepresented populations. [38] Though there is ongoing research about cystic fibrosis in underrepresented populations, many of the studies leave much to be desired and are not performed to the standards of studies conducted in white patients.
Idiopathic pulmonary fibrosis (IPF) synonymous with cryptogenic fibrosing alveolitis [5] is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.
Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid malformation (CCAM), is a congenital disorder of the lung similar to bronchopulmonary sequestration. In CPAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue.
Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include cholangitis, hepatomegaly and signs of portal hypertension. [citation needed]
[12] [3] [13] Cystic fibrosis eventually results in severe bronchiectasis in nearly all cases. [14] The cause in 10–50% of those without cystic fibrosis is unknown. [3] The mechanism of disease is breakdown of the airways due to an excessive inflammatory response. [3] Involved airways become enlarged and thus less able to clear secretions. [3]