Paul di Sant'Agnese (1914–2005) was the founder of the

Cystic Fibrosis Foundation The Cystic Fibrosis Foundation (CFF) is a 501(c)(3) non-profit organization in the United States established to provide the means to cure cystic fibrosis (CF) and ensure that those living with CF live long and productive lives. The Foundation prov ...

and of Cystic Fibrosis Care in the United States. He was a physician, researcher and one of the first clinicians who devoted his life to understanding and conquering

cystic fibrosis Cystic fibrosis (CF) is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. O ...

through his work at the

Columbia University Medical Center NewYork-Presbyterian/Columbia University Irving Medical Center (NYP/CUIMC), also known as the Columbia University Irving Medical Center (CUIMC), is an academic medical center and the largest campus of NewYork-Presbyterian Hospital. It includes C ...

, New York City. Undoubtedly the major advance during the Fifties was the recognition of the increased salt content of the sweat in people with CF by Paul di Sant’Agnese in 1953. During the heat wave of 1949, he was the first to recognize that infants suffering from cystic fibrosis were at special risk for heat

prostration Prostration is the gesture of placing one's body in a reverentially or submissively prone position. Typically prostration is distinguished from the lesser acts of bowing or kneeling by involving a part of the body above the knee, especially t ...

. He thus discovered that sweat was abnormal in CF. His discovery of abnormalities in sweat electrolytes lead to the

sweat test The sweat test measures the concentration of chloride that is excreted in sweat. It is used to screen for cystic fibrosis (CF). Due to defective chloride channels (CFTR), the concentration of chloride in sweat is elevated in individuals with CF. ...

, which is a primary method for diagnosing cystic fibrosis and was developed and improved over the next decade. This remains the cornerstone of the diagnosis of the disease— but also opened up areas of research that ultimately helped to identify the basic defect in cystic fibrosis.

Resources

External links

* https://www.cff.org/Research/Researcher-Resources/North-American-CF-Conference/Paul-di-Sant-Agnese-Distinguished-Scientific-Achievement-Award/ * http://www.cfmedicine.com/history/forties.htm * https://mylegacywithcff.org/paul-di-santagnese-society/ * https://cysticfibrosis.online/mega-papers * https://www.amjmed.com/article/0002-9343(80)90482-9/fulltext {{DEFAULTSORT:Di Sant'Agnese, Paul 1914 births 2005 deaths Physicians from New York (state) Cystic fibrosis