Seminar In Medicine, 19.06.2018, Prof. Dr. Zeynep Seda Uyan

Author: KUSOM
Time: 15:00
Location: MED 176



Tuesday, June 19th, 2018

Speaker         : Prof. Dr. Zeynep Seda Uyan, Koç University Hospital, Department of 
Pediatric Pulmonology

Title               : Cystic Fibrosis – From Childhood To Adulthood

Time              : 15.00 (Refreshments will be served at 14:45)

Place              : MED 176

TelePresence   : AH 5th floor Chief Medical Officer / KUH 9th floor Meeting Room


Cystic Fibrosis – From Childhood To Adulthood

Cystic fibrosis (CF) is the most common life-limiting autosomal recessive disease among Caucasian population, with a frequency of 1 in 2000 - 3000 live births. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein which is a cAMP-regulated chloride channel expressed primarily at the apical plasma membrane of secretory epithelia in the airways, pancreas, intestine and other organs. Although it is a multi-systemic disease, pulmonary disease is the major cause of morbidity and mortality in CF. The diagnosis of CF is based upon compatible clinical findings with biochemical or genetic confirmation. The principles of treatment are enhancement of mucociliary clearance, prevention and aggressive treatment of pulmonary infection, treatment of airway inflammation, maintenance of good nutrition and early identification and treatment of complications. CF has benefited from the development of new and effective treatments such as CFTR modulators. It was once considered a childhood disease, but with improvements in diagnosis and treatment, many people with CF are living well into adulthood. The early diagnosis and treatment of CF have resulted in remarkable improvements in quality of life and clinical outcomes in these patients, with median life expectancy now older than 40 years.