Liver transplantation in a patient with severe cystic fibrosis

Abstract

Cystic fibrosis (CF) is a severe progressive hereditary disease associated with mutations in the CFTR gene, characterized by multiple organ pathology, including the upper and lower respiratory tract, gastrointestinal and reproductive tracts, and the endocrine system. Despite new therapy, which significantly increases survival among patients with cystic fibrosis, the development of complications of this disease is still a factor affecting the duration and quality of life. The aim of the study is to demonstrate a clinical case of severe course of cystic fibrosis in a patient with meconium ileus, cystic fibrosis-associated diabetes mellitus, pulmonary and arterial hypertension, cirrhosis, who was under the observation of specialists of the Department of Cystic fibrosis of the Research Clinical Institute of Childhood of the Ministry of Health of the Moscow Region and National Medical Research Center of Transplantology and Artificial Organs named after V. I. Shumakov and underwent death donor liver transplantation. New methods of diagnosis and therapy of complications allow timely detection and control of their course, while maintaining a satisfactory quality of life for the patient