Background and aims The neurological complication and delay of development occur in ne0nates of high perinatal group. Improve the level of patient satisfaction. Methods The developmental assessment was performed in 60 VLBW infants and in 21 children with FAS. There were used Albert scale (gross motor) and KID - RSDI scale (cognitive, fine motor, speech and language, social and adaptive development). Results The comorbidity of VLBW were following: retinopathy of newbon (10%); neurosensory deafness (3,3%), bronchopulmonary dysplasia (5%), congenital heart diseases (12,5%). The medical problems of FAS were intrauterine growth retardation (42,8%, p < 0,05), premature VLBW (28%, p < 0,05), congenital cataract (9,5%), congenital heart diseases (19%). There was significant delay of motor development (-2 mo) in VLBI (45%) compare in infants FAS (19%, p = 0,03). The delay of cognitive development were in 45% of VLBW and in 52,3% infants with FAS on the first year. Mean cognitive delay in VLBW was 3,1 mo; in infants with FAS – 3,9 mo (p > 0,05) at the first year of life, at the second year - 3,3 mo and 5,6 mo respectively (p < 0,05). From the total neonates (112) of high risk group only 81 have been include to early interventional program due to absence of communication between perinatal centre, primary care and rehabilitative service. Conclusions The delay of motor development on the first year of life predominates in very low birth weight infants, the cognitive delay of development on the second years predominates in children with fetal alcohol syndrome. There was a significant risk of important information getting missed.

Atherosclerotic changes in the vascular walls begin early in childhood, especially in association with familial hypercholesterolemia (FH). The process may be subclinical, which nevertheless requires therapeutic and preventive measures. Purpose - to evaluate baseline lipid profiles, the thickness of carotid intima-media complexes, blood pressure indices and the association with concentration changes of dephosphorylated-uncarboxylated matrix Gla protein (dp-uc MGP) as a marker of subclinical arterial lesions in different age groups of pediatric patients with FH. Materials and methods. Children with heterozygous FH (n=15), stratified by age and sex, were included in the study. The control group consisted of healthy peers (n=21). Blood samples were analyzed to determine levels of total (TC), low-density (LDL-C), very-low-density (VLDL-C), high-density (HDL-C), remnant (rC) and non-high-density (non-HDL-C) cholesterol, triglycerides (TG), apolipoproteins A1 (apoA1) and B (apoB), lipoprotein (a), and dp-uc MGP. The intima-media complex thickness of the common carotid artery and blood pressure were measured in all study subjects. The obtained data were processed using the accepted methods of medical statistics and SAS® OnDemand for Academics. Results. Lipid profile changes in pediatric patients with FH were characterized by high levels of LDL-C, non-HDL-C and lipoprotein (a) in the 5-9 years age group; in the 10-14 years age group - high levels of LDL-C, TG, rC, non-HDL-C and lipoprotein (a); in the 15-18 years age group - high levels of LDL-C, TG, non-HDL-C and lipoprotein (a). At the same time, the most marked dyslipidemia changes were evident in children aged 10-14 years in the FH group. apoA1 levels were significantly decreased in all FH children. Elevated levels of lipoprotein (a) (>30 mg/dL) in FH children were found in all age groups, suggesting that elevated lipoprotein (a) levels can be used as a factor for cardiovascular risk stratification. Dp-uc MGP levels were significantly elevated in all age groups of FH children compared to healthy peers. Conclusions. A lipid profile examination is necessary to diagnose FH in children, along with family health history and cascade screening. As atherosclerotic changes at 5-18 years of age remain subclinical, and the instrumental tests available in routine medical practice are not sensitive enough to detect them, therefore, preventive or therapeutic measures cannot be initiated promptly. The evaluation of circulating matrix Gla protein in pediatric patients with FH can be used as a marker of vascular wall calcification, which may allow early preventive measures against microcalcification to be developed. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Introduction. Familial hypercholesterolemia (FH) is a genetically determined disease characterized by elevated low-density lipoprotein levels since birth and predisposes a person to develop atherosclerosis-induced cardiovascular disease. Healthcare providers should monitor the health status and physical activity level in pediatric patients with FH, as a high-risk group for cardiovascular disease. Purpose - to investigate the self-reported health-related quality of life (HRQoL) and physical activity energy expenditure based on the questionnaire data (PAEEq) in children with heterozygous FH compared with healthy peers to assess the health status. Materials and methods. The HRQoL scores were assessed using the KINDLR questionnaire in 15 patients with FH and 21 healthy peers. The physical activity level was evaluated using the C(Y)PAQ questionnaire. The KINDLR data together with other variables such as age, weight, height, sex, BMI and the PAEEq scores were analyzed in SAS® OnDemand for Academics. Results. HRQoL scores in FH children were similar to those of the control group (p>0.05). There was no association between PAEEq and the HRQoL scores in the FH (r=0.37, p=0.29) and the control group (r=0.43, p=0.20). The KINDLR Physical well-being score in the 5-9 years age group was significantly higher for FH children than for controls (p<0.01), while the total HRQoL score was not significantly different between FH children and controls. The group of FH children aged 5-9 with an intermediate level of PAEEq was the most physically active among the surveyed children. The controls aged 15-18 with low levels of PAEEq was the least active. All other age groups were characterized by a low intermediate level of PAEEq. Conclusions. Children with FH have HRQoL scores that are comparable to those of healthy peers. The FH and control groups were relatively satisfied with their quality of life. Thus, FH children consider themselves to be healthy, and from this underestimation of their cardiovascular risk they may have low adherence. Most FH children were found to have the low intermediate levels of PAEEq, which may indicate a lack of exercise and poor quality of life later on. Children with FH may have significant health problems in adulthood if they are not treated early and appropriately. In a similar manner, low PAEEq levels were demonstrated by the controls, so they should be also informed about the significance of regular physical activity and properly motivated. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patients was obtained for conducting the studies. No conflict of interests was declared by the authors.

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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.

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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.

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