Health status of cystic fibrosis patients with liver cirrhosis and after liver transplantation
Authors:   Research Centre for Medical Genetics, Moscow, Russian Federation, Kondratyeva E.I. ,   Research Clinical Institute of Childhood of the Moscow Region, Moscow, Russian Federation, Ilenkova N.A. ,   V.F.Voyno-Yasenetskiy Krasnoyarsk State Medical University, Krasnoyarsk, Russian Federation, Chikunov V.V. ,   V.F.Voyno-Yasenetskiy Krasnoyarsk State Medical University, Krasnoyarsk, Russian Federation, Voronkova A.Yu. ,   Research Centre for Medical Genetics, Moscow, Russian Federation,   Research Clinical Institute of Childhood of the Moscow Region, Moscow, Russian Federation, Zhekaite E.K. ,   Research Centre for Medical Genetics, Moscow, Russian Federation,   Research Clinical Institute of Childhood of the Moscow Region, Moscow, Russian Federation, Sherman V.D. ,   Research Centre for Medical Genetics, Moscow, Russian Federation,   Research Clinical Institute of Childhood of the Moscow Region, Moscow, Russian Federation, Nikolaeva E.D. ,   Research Centre for Medical Genetics, Moscow, Russian Federation,   Research Clinical Institute of Childhood of the Moscow Region, Moscow, Russian Federation, Monakhov A.R. ,   V.I.Shumakov National Research Center for Transplantology and Artificial Organs, Moscow, Russian Federation, Silina O.V. ,   V.I.Shumakov National Research Center for Transplantology and Artificial Organs, Moscow, Russian Federation
Published: November 2022

Objective. To study the features of liver cirrhosis (LC) in patients with cystic fibrosis (CF) and the course of the disease after liver transplantation according to the 2019 registry. Materials and methods. We analyzed the data of 3111 patients with recorded data on the state of the liver in the register of 2019 in age groups: children under 18, children from 9 to 18, adults. LC was diagnosed in 190 patients, including 121 (63.7%) with LC with portal hypertension. LC was more often recorded in the male group – 62.4% (p = 0.015). The age at diagnosis in the group of children with LC was higher – 0.7 (2.5) years, than in the group without liver damage – 0.2 (0.8) years (p = 0.01). "Severe" mutations were characteristic of patients with LC and hypertension in all age groups. The body mass index of children (aged 2–18 years) in the group with LC with hypertension was Me = 21.5 (42.3), and in the group without liver damage Me = 29.8 (49.3), p = 0.008. Chronic colonization of the respiratory tract by Pseudomonas aeruginosa in the group of patients with LC and hypertension was more common than in children without liver damage (46.2% versus 24.4%, p < 0.001). By 2019, 12 patients have received liver transplantation. After liver transplantation, in the first three years, there is an increase in function of external respiration and nutritional status of patients. Conclusion. LP with portal hypertension is more common in children aged 9–18 years, patients are characterized by a predominance of males, "severe genotypes", low indices of physical development, a high frequency of pathogenic microbial agents of the respiratory tract and complications, which leads to a significant reduction in life. Key words: genotype, cystic fibrosis, nutritional status, portal hypertension, liver transplantation, lung function, cirrhosis