Гепаторенальний синдром у дитини з муковісцидозом: аналіз клінічного випадку

Abstract

Hepato-renal syndrome (HRS) is a rare complication that may accompany end-stage cystic fibrosis-associated cirrhosis in patients with cystic fibrosis. There is currently no unified protocol for the diagnosis and treatment of HRS in cystic fibrosis, particularly in pediatric patients. The main approaches to diagnosing the condition are to detect signs of acute renal failure in a patient with severe liver damage in the absence of shock and in the absence of signs of organic kidney damage or the use of nephrotoxic drugs. Treatment is based on the appointment of vasoconstrictors in combination with albumin infusion and the exclusion of factors that promote the development of HRS, but the method of final therapy remains liver transplantation. The article presents an analysis of the clinical case of HRS and its treatment in a child with cystic fibrosis. Immediate detection of liver cirrhosis in patients with cystic fibrosis, exclusion of the risk factors (massive and protracted surgical interventions, significant volume of extracted ascitic rudin, limiting the use of nephrotoxic drugs and high-dose loop diuretics) is a way to prevent the development of severe liver cirrhosis complications. Immediate recognition and proper management of HRS is a way to protect life before liver transplantation, as well as to preserve the quality of life of patients with cystic fibrosis. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Key words: cystic fibrosis, hepatorenal syndrome, liver cirrhosis, ascites, terlipressin.