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

High risk babies include those born prematurely with chronic lung disease, those with cystic fibrosis, those who are severely immunocompromised and those of American Indian or Alaskan Native heritage. —Omer Awan, Forbes, 3 Nov. 2024

The researchers evaluated this strategy in cells carrying one of the mutations that leads to cystic fibrosis. —Bymitch Leslie, science.org, 24 Oct. 2024

Prenatal tests for many diseases, like cystic fibrosis and sickle cell disease, already exist, and the fears of autism advocates are not unfounded. —Celia Ford, Vox, 7 Dec. 2018

Pneumothorax can also develop in people with certain lung diseases, such as cystic fibrosis, asthma, pneumonia, and COPD.18 Lung Cancer Lung cancer develops when abnormal cells grow uncontrollably, forming a tumor. —Lindsay Curtis, Health, 16 Oct. 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