Abstract:

Cystic Fibrosis (CF) is one of the most commonly inherited genetic disorders which will show either asymptomatic features or it prone to severe multiorgan failure and sometimes it may turn into neoplasm and finally death if untreated. CF may hit more than 2000 genes in the human being, but of those ΔF508 mutation in Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein is the most common one. The leading cause of death in CF patients is due to the involvement of lung and pancreas (pancreatic insufficiency). Even though the primary organ involved in CF is lung, but pancreatic involvement also relates with some important clinical manifestations like diarrhea, steatorrhea, malabsorption, hypovitaminosis, Cystic Fibrosis Related Diabetes (CFRD), pancreatic adenocarcinoma etc., seen in these patients, which is helpful for the physicians to make proper diagnosis and treatment. So, this review article purely focuses on CF especially in relation with pancreatic abnormalities which will be discussed under genetics, pathophysiology (both exocrine and endocrine), cancer, diagnosis and finally treatment.

Keywords: Cystic Fibrosis (CF), Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), pancreatic insufficiency, pancreatic adenocarcinoma, Cystic Fibrosis Related Diabetes (CFRD).

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