FUNCTIONAL STATE OF THE HEPATOBILIARY SYSTEM IN CHILDREN WITH CYSTIC FIBROSIS

Abstract

Cystic fibrosis (CF), or cystofibrosis of the pancreas (pancreas), is a hereditary disease in which there is a secretion defect characteristic of all epithelial cells of the body, primarily for chloride ions with a secondary decrease in the total volume of secretion. The most common mutation of the CFTR gene (cysticfibrosistransmembraneconductanceregulator) is the deletion of trinucleotides by the 10th exon, resulting in the cpotereostat of kaphenylalanine in the 508th position of the protein molecule (F508). About 45% of all CF patients in the world are homozygotes for the F508del mutation. The genetic polymorphism of the disease causes a phenotypic diversity of CF from severe to erased forms [17]. Cystic fibrosis is one of the most common among the representatives of the Europosomal race of autosomal recessive hereditary diseases. The disease is characterized by the pathology of the exocrine glands of vital organs and systems and usually has a severe course and an unfavorable prognosis. Cystic fibrosis is an important medical and social problem associated with early disability, the need for constant treatment and active dispensary observation, as well as the need for early diagnosis. [21]. An important role in the pathogenesis of the disease is given to the defeat of the digestive system and, above all, the pancreas and liver. In recent decades, the life expectancy of patients with cystic fibrosis has increased, and therefore the frequency of involvement of the hepatobiliary system in the pathological process increases, ranging from 20 to 80%. [22].