cystic fibrosis-related diabetes

alshukrey, Ali (2020)

Cystic fibrosis is a lethal autosomal recessive disorder that arises from mutations in chromosome 7 that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) gene.{1} This mutation causes the CFTR protein to become dysfunctional, as a result patient experience thick, sticky mucus secretions in multiple mucin-producing organs leading to many complications, including cystic fibrosis related diabetes.{3} There are two known types of diabetes in people without cystic fibrosis: Type 1 diabetes, an autoimmune disease that occurs when the body’s immune system attacks and destroy the beta cells in the pancreas that make insulin. Which causes an increase in the blood sugar levels.{4} This type of diabetes is commonly diagnosed in childhood and requires insulin injections as a treatment.{3} The other known type of diabetes is Type 2 diabetes, which occurs more often in adults who are overweight or obese. Type 2 diabetes is caused by the lack of the body’s normal response to insulin in addition to the pancreas not making enough insulin.{1}


Cystic fibrosis-related diabetes (CFRD) is the principle extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adults cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. CFRD has many complications as it significantly impacts the pulmonary function which is the most common cause of death in this condition. (CFRD) diagnosis and management have considerably changed since diabetes was first shown to be associated with a poor prognosis in individuals with CF. current trends in CFRD prevalence incidence, and mortality were determined from a comprehensive clinicaldatabase.

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