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.

Cystic fibrosis (CF) is an autosomal recessive, life-shortening disease in which generalized exocrine gland involvement occurs with predominantly pancreatic insufficiency, airway obstruction, and progressive lung involvement. Due to pancreatic insufficiency, malabsorption and malnutrition are observed in patients. The close attributive association of CF with nutritional status and adverse clinical outcomes determines a thorough assessment of the nutritional status, individual management and monitoring of all children with CF. Attainment of optimal physical development and adequate nutrition in patients with CF is currently possible through early diagnosis and aggressive nutritional medical therapy. Medical dietary interventions are aimed at ensuring normal growth and preventing of malnutrition, thus will upgrade the prognosis and quality of life of pediatric patients with CF. The reasons for the development of malnutrition in CF, specific screening tools for monitoring the growth of ill children, criteria for stratifying children into nutritional risk groups are reviewed in the lection. The procedure for calculating energy needs, taking into account activity, the severity of the disease and pancreatic insufficiency is presented. Attention is drawn to the ratio of macronutrients and the need to consume energy-dense and nutrient-rich foods. The prescription of medical nutritional therapy with the inclusion of special foods for medical purposes (clinical nutrition) to meet increased energy requirements and avoid the consumption of energy-dense but nutrient-poor foods also is discussed. It is meaningful to involve into a multidisciplinary team of a psychologist who can on a regular basis correct eating behavior and promote compliance with nutritional recommendations in order to achieve optimal physical development of patients. 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, children, malnutrition, medical nutrition therapy.