cystic fibrosis

noun

: a common, progressive hereditary disease of exocrine gland function that typically appears in infancy or early childhood and is marked by the accumulation of thick, sticky mucus in the ducts and passages of various organs and especially those of the lungs and pancreas resulting in shortness of breath, persistent cough, chronic respiratory infection, pancreatic enzyme insufficiency, faulty digestion, malnutrition, and poor growth

Note: The genetic mutation linked to cystic fibrosis causes disruption in the movement of chloride ions and water across cell membranes resulting in mucus that is thick and sticky instead of thin and slippery and in elevated concentrations of sodium and chloride in sweat and saliva. Cystic fibrosis occurs especially in people of northern European ancestry and is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.

… children with cystic fibrosis are born with healthy lungs; only later do their lungs become plugged with mucus and incur infections.

—The Journal of the American Medical Association

Cystic fibrosis is the most common lethal inherited disorder in the United States. Approximately 30,000 people in this country have inherited two defective copies of the CF gene and thus the disease; approximately 1 in 20 Americans has just one copy of the gene and is a carrier of the disease.

—Joe Palca

—abbreviation CF

Examples of cystic fibrosis in a Sentence

Recent Examples on the Web

Yet cystic fibrosis has received considerably more attention, funding and research. —Ambroise Wonkam, Scientific American, 17 Sep. 2024

For example, pancreatic enzyme replacement therapy (PERT) is a medical treatment used to treat pancreatic insufficiency caused by medical conditions like cystic fibrosis and pancreatic cancer. —Jillian Kubala, Health, 8 Aug. 2024

So, the researchers obtained lung cells from a volunteer who has cystic fibrosis and cultured them in a way to form a three-dimensional tissue. —Jon Brodkin, Ars Technica, 13 June 2024

Flying can be risky for people with untreated cystic fibrosis. —Joe Snell, Washington Post, 30 July 2024

Word History

Etymology

Note: The name cystic fibrosis refers to the cysts and fibrous scar tissue that form in the pancreas of people with the disease. The term was introduced by the American pediatrician and pathologist Dorothy Hansine Andersen (1901-63) in "Cystic Fibrosis of the Pancreas and Its Relation to Celiac Disease: a Clinical and Pathological study," American Journal of Diseases of Children, vol. 56, no. 2 (August, 1938), pp. 344–99. A similar term was used two years earlier by the Swiss pediatrician Guido Fanconi, in "Das Coeliakiesyndrom bei angeborener Zystischer Pancreasfibromatose und Bronchiektasien" ("Celiac syndrome with congenital cystic fibromatosis [development of multiple fibromas] of the pancreas and bronchiectases"), (Wiener medizinische Wochenschrift, vol. 86 [1936], pp. 753-56).

First Known Use

1938, in the meaning defined above

Kids Definition

cystic fibrosis

noun

: an inherited disease marked by the buildup of thick sticky mucus chiefly in the lungs and pancreas leading to recurrent lung infections and digestive problems

Medical Definition

cystic fibrosis

noun

: a common, progressive, hereditary disease of exocrine gland function that typically appears in infancy or early childhood and is marked by the accumulation of thick, sticky mucus in the ducts and passages of various organs and especially those of the lungs and pancreas resulting in shortness of breath, persistent cough, chronic respiratory infection, pancreatic enzyme insufficiency, faulty digestion, malnutrition, and poor growth

Note: The genetic mutation linked to cystic fibrosis causes disruption in the movement of chloride ions and water across cell membranes resulting in mucus that is thick and sticky instead of thin and slippery and in elevated concentrations of sodium and chloride in sweat and saliva. Cystic fibrosis occurs especially in people of northern European ancestry and is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.

called also fibrocystic disease of the pancreas, mucoviscidosis

—abbreviation CF