PubMed 26281081

Referenced in Channelpedia wiki pages of: none

Automatically associated channels: Kir2.3


Authors: Keren Nathan, Michal Shteinberg, Joseph Rivlin

Journal, date & volume: Harefuah, 2015 Jun , 154, 373-6, 404

PubMed link:

Cystic fibrosis (CF) is an autosomal recessive disorder. The disorder is caused by a mutation in the gene that encodes a protein which functions as a chloride channel. The chloride channel, cystic fibrosis transmembrane conductance regulator (CFTR) exists in the apical membrane of exocrine epithelial cells in the body. In the last 75 years the survival of CF patients has risen dramatically from a few months to the average age of 37 years. The rise in life expectancy is due to several reasons: improved medical treatment, treating patients in specialized CF centers, early diagnosis, respiratory physiotherapy and liver or lung transplantation. The purpose of this study was to review characteristics of our oldest living patients, transplantations and mortality of CF patients in our center.Retrospective data have been collected regarding survival and other features in CF patients who were admitted to Carmel Medical Center in the years 2000 to 2013.One hundred and four CF patients were registered at the CF center between the years 2000 and 2013. Over this period 6 patients have passed away, all of whom were females. The average age of death was 21.4 years (not including one 10 months old baby who died from metabolic syndrome, not CF) with SD of 7 years, median of 20 and range of 17 years. The average age at the clinic is 22.5 years. The death incidence was less than 1% per year; the leading cause of death was respiratory failure. Of the living patients, ten patients are above the average survival age of 37 years. Four percent of the patients have undergone lung transplantation.CF is a multisystem disorder. In our center the mean age of death is in the third decade with an incidence of less than 1% per year, which is comparable to CF registries worldwide. Four percent of the patients have undergone lung transplantation. A gender gap with more female than male deaths was observed, a finding which was previously described in the literature. Life expectancy continues to rise as a result of early diagnosis, improved medical treatment and lung transplantation. As the age of survival rises, physicians with knowledge of adult internal medicine are needed to treat CF patients.