Objective. To assess the efficacy of nocturnal hyperalimentation via a low-profile gastrostomy tube for in the improvement of nutritional status and lung function in children with severe cystic fibrosis (CF). Patients and methods. We used nocturnal hyperalimentation with a nutrient solution via a low-profile gastrostomy tube installed under endoscopic control to improve nutritional status of 16 CF children with severe protein-calorie malnutrition aged between 5.5 and 17.5 years. The mean duration of nocturnal hyperalimentation was 1.3 years (range: 7 months–54 months). The nutritional status was evaluated using the WHOAntho Plus software; pulmonary function was evaluated by assessing forced expiratory volume in 1 minute (FEV1) and the number of bronchopulmonary exacerbations per year. Results. All children demonstrated an improvement in their weight and height. After the first 12 months, the mean body weight gain was 5 kg; during the second and third years, children gained 4 kg and nearly 2 kg, respectively. The mean increase in height was 7 cm in the first year, 10 cm in the second year, and 4 cm in the third year. All patients except one had an increase in FEV1 and fewer exacerbations. Complications after gastrostomy were rare and easily addressed. Conclusion. Nocturnal hyperalimentation via a percutaneous endoscopic gastrostomy tube is an effective and safe method of improving nutritional status in children with CF, which delays the progression of bronchopulmonary lesions and lung function reduction, improves the prognosis and quality of life. Key words: cystic fibrosis, children, nutritional status, lung function, enteral nutrition, low-profile gastrostomy tube, nocturnal hyperalimentation

Introduction. To improve the quality of surgical treatment and prevent postoperative complications, there is needed an integrated multidisciplinary professional approach including a combination of nutritional support with therapeutic methods and psychological assistance that reduces stress throughout the perioperative period. Clinical guidelines and standards to manage the patients on this issue in pediatric surgical hospitals have not been developed. Objectives: to assess the nutritional risk of malnutrition and nutritional status in children with surgical diseases, to elaborate algorithms for nutritional support over the pre- and postoperative periods. Materials and methods. The single center non-comparative study included sixty children aged from 1 month to 17 years 5 months, with diseases of the esophagus, intestine, and genitourinary system, were admitted for surgical treatment at the Research Institute of Pediatric Surgery of the Federal State Autonomous Institution «National Medical Research Center for Children’s Health» of the Ministry of Health of Russia. All patients underwent a nutritional risk screening according to a validated Russian version of the STRONGkids. Clinical and anamnestic data were analyzed. Anthropometric indices (Z-scores: weight/age, height/age, BMI/age) using the WHO AnthroPlus program (2009), and biochemical parameters (concentrations of C-reactive protein, total protein, albumin, prealbumin, transferrin) were evaluated. Results. A high nutritional risk at admission was established in more than half (57%) of patients, moderate — in 36% of patients, low — only in 7% of cases. Malnutrition (Z-score BMI/age from –1 to –3) was diagnosed in 26 (43%) patients, 58% of them suffered from diseases of the esophagus, 37% — the intestine pathology, and 36% of the genitourinary disorders. Conclusion. Nutritional risk screening and nutrition status assessment are necessary in all patients admitted to pediatric surgical hospitals for the timely appointment of adequate nutritional support, which will reduce the incidence of postoperative complications, and allow diminishing the length of the hospital stay.

Background. Numerous studies aimed at assessing the nutritional status and organizing nutritional support for surgical patients cover the adult patients, only a handful assess underaged patients with surgical pathology in the pre- and postoperative period and their needs for assessment of the nutritional status and approaches to their dietary correction. Aim. To assess the possible risks of developing malnutrition in children with various pathologies of the chest organs during the preoperative period, and to conduct a comprehensive assessment of their nutritional status. Materials and methods. The single-center non-comparative cross-sectional study included 50 children between 5 months and 17 years 7 months, hospitalized in the thoracic department of the National Medical Research Center for Children's Health of the Ministry of Health of the Russian Federation. All patients underwent an assessment of clinical and anamnestic data, somatometric and clinical laboratory parameters, and Z-scores analyzed: body weight/age, height/age, BMI/age, shoulder circumference, skin-fat folds above the triceps and under the scapula, as well as a number of biochemical parameters (concentrations of total protein, albumin, prealbumin, transferrin and C-reactive protein). Results. When assessing the nutritional status of children upon admission to the thoracic department, acute malnutrition was detected in 21 (42%) patients (of which 10% had mild, 14% moderate, and 18% severe malnutrition), chronic malnutrition was found in 18% of children. For the first time in Russia, using a validated Russian-language version of the STRONGkids screening questionnaire, nutritional risks of malnutrition have been assessed. The vast majority of patients were at moderate (28%) or high (70%) risk of developing malnutrition or its aggravation. Z-scores of skin-fat folds above the triceps and under the scapula, as well as the circumference of the shoulder were within the reference values. 16 (36%) patients had a decrease in the concentration of total protein, 10 (22%) had a drop of prealbumin accompanied by an increase (40%) of the C-reactive protein level. The levels of other proteins were within the reference values. Conclusion. The identified risks of malnutrition and its moderate/severe manifestations in children with surgical pathology are able to contribute to the development of infectious and non-infectious postoperative complications, increase the length of stay in the clinic, and require nutritional support from the first day of hospitalization in a surgical hospital.

Introduction. Children with cystic fibrosis often have gastrointestinal symptoms despite properly selected primary therapy and pancreatic substitution therapy. A possible reason is lactase deficiency. Aim is to determine the frequency of lactase deficiency in children with cystic fibrosis and the need for appropriate dietary correction of their diets. Materials and methods. In 213 children with cystic fibrosis, during esophagogastroduodenoscopy in biopsies of the small intestinal mucosa 359 rapid tests, «Lactose intolerance quick test” were performed. According to the results of an express test, some patients diagnosed with hypolactasia to determine their tolerance to lactose underwent a glycemic load test with lactose and measured the level of fecal calprotectin to assess the level of faecal calprotectin the degree of chronic intestinal inflammation. Results. Normal lactase activity was observed in 129 (36%) biopsies studied, moderate hypolactasia in 91 (25%) biopsies, severe hypolactasia in 139 (39%) biopsies. In patients with moderate hypolactasia, according to the express test results, 20% confirmed intolerance to lactose as a result of the load test. In patients with severe hypolactasia, 72% were confirmed to be lactose intolerant, according to the results of an express test. There was no correlation between the level of calprotectin and the results of the glycemic load test with lactose. Conclusion. Lactase deficiency is observed in a significant proportion of patients with cystic fibrosis, which may be a consequence of primary adult hypolactasia and chronic intestinal inflammation. In children with cystic fibrosis with dyspeptic symptoms persisted despite correctly prescribed basic therapy and pancreatic enzymes, it is necessary to exclude lactose intolerance and prescribe a low-lactose diet.

Breastfeeding (BF) in the first 6 months of a child’s life with the addition of other foods until the age of 12–24 months is globally recognized as the “gold standard” for infant feeding. For children with cystic fibrosis (CF), it is particularly relevant that breast milk contains a wide range of protective factors against pathogenic bacteria colonization, thermolabile lipase, which facilitates fat digestion, and polysaccharides, which are essential for the trophism of colonocytes and the formation of normal gut microbiota. Objective. To study the effect of BF and its duration on the course of the disease, nutritional status of children with cystic fibrosis. Patients and methods. Questionnaire results on the duration of breastfeeding in 60 mothers of children with cystic fibrosis were analyzed, with further retrospective assessment of the severity of CF in these children. Results. The nutritional status of children who did not receive breast milk at both 1 and 3 years of age was lower than the mean age standards in contrast to patients who were breastfed; the number of bronchopulmonary exacerbations was significantly greater compared with children who received breast milk. The longer the duration of BF was, the later Pseudomonas aeruginosa was isolated from the sputum of CF patients (r = 0.68, p < 0.005). Conclusion. Maintenance of BF leads to a milder course of the disease in later years, which becomes especially important for children with CF. Key words: cystic fibrosis, children, breastfeeding