[Electroencephalogram features in children with congenital hyperinsulinism treated according to the international protocol in Russian Federation].

BACKGROUND: Congenital hyperinsulinism (CHI) is a rare life-threatening disease characterised by persistent hypoglycaemia as a result of inappropriate insulin secretion, which can lead to irreversible neurological defects in infants. AIM: To evaluate neurophysiological characteristics of central nervous system in children with congenital hyperinsulinism treated according to the international protocol in Russian Federation. MATERIALS AND METHODS: Our retrospective, prospective cohort study included 73 patients who received treatment for CHI according to the current international protocol at different departments of the Almazov National Medical Research Centre from 2017 to 2022. All patients underwent a comprehensive examination, including electroencephalography (EEG). RESULTS: Among 73 patients with CHI, 35% (23) had focal form of the disease, 65% had non-focal form (49% (39) - diffuse form, 16% (11) - atypical form). All patients with focal form of CHI had a recovery as an outcome.Analysing the EEG data we found that paroxysmal activity was recorded in 23 patients (32%), 50 patients did not have paroxysmal activity (68%). Diffuse changes were observed in 47 patients (64%), whereas 26 patients (36%) were absent of it. By constructing Kaplan-Meier curves we found that the alpha rhythm is formed significantly (p=0.026) earlier in patients with a focal form of CHI. CONCLUSION: CHI patients treated according to the international guidelines in Russian Federation show rather positive neurological outcome. We established that alpha rhythm earliest formation is associated with focal form of CHI.

[Comparative analysis of enteral feeding practices for prevention of gastrointestinal complications in infants with ductus-dependent congenital heart diseases].

Mesenterial hypoperfusion is one of the main pathogenetic factors of necrotizing enterocolitis (NEC) in infants with ductus-dependent congenital heart diseases. NEC in infants undergoing congenital heart surgery increases mortality and length of hospital stay. NEC is also associated with adverse neurodevelopmental outcome. Optimization of enteral feeding can reduce the risk of gastrointestinal complications. NEC risk factors in infants with congenital heart are of special interest in the literature. This article discusses criteria for enteral feeding initiation and increasing preoperatively and after heart surgery. Enteral feeding protocols of leading cardiac surgical centers are reviewed. Practices to provide high energy and nutrient consumption in infants with congenital heart disease are described.

[Clinical, genetic, and radionuclide characteristics of the focal form of congenital hyperinsulinism].

BACKGROUND: Congenital hyperinsulinism (CHI) is a severe disease with a high risk of complications including neurological deficit. Persistent hypoglycemia in patients with focal form of CHI can not be managed with medical treatment in 96.4% of cases, what subsequently leads to surgical treatment. Currently, there is a lack of information regarding patients with focal form of CHI. This study is aimed at finding better approaches for diagnosis and treatment of patients with focal form of CHI. AIMS: To study clinical, genetic and PET/CT findings of the focal form of CHI in Russian group of patients. MATERIALS AND METHODS: The observational research included all patients with a histologically confirmed focal form of CHI, who were admitted to Endocrinology Research Centre during the period from January 2008 to January 2019. A statistical analysis of clinical data, genotype, and positron emission tomography (PET) with 18F-dihydroxyphenylalanine (18F-DOPA) was performed. The median follow-up was 18 months. RESULTS: The study included 31 patients with focal CHI (14 boys, 45.2%). All patients had a neonatal presentation of the disease and demanded high levels of continuous glucose infusion to maintain euglycemia. The difference between the age of hypoglycemia presentation and the age of diagnosis ranged from 1 day to 3.9 months. In all cases, diazoxide was found to be ineffective. However, in 9 patients, it was possible to withdraw continuous glucose infusion and maintain euglycemia using octreotide in the preoperative period. A molecular genetic study allowed us to detect diverse pathogenic variants in ABCC8 and KCNJ11 genes in 30 patients. According to PET data with 18F-DOPA, the pancreatic index (PI) varied widely from 1.16 to 3.59. After partial resection of the pancreatic region with insulin hypersecretion, all patients showed complete recovery. CONCLUSIONS: The focal form of CHI is a severe condition with high prevalence of neurological complications. For preoperative diagnosis of the morphological form of the disease, it is necessary to conduct genetic analysis and radionuclide studies. Solely evaluation of mathematical parameters in 18F-DOPA PET without taking into account the visual data and the results of genetic analysis does not allow establishing the robust diagnosis. Timely diagnosis, identification of risk factors, and prevention of complications of persistent hypoglycemia are important tasks for clinicians.

[Diagnosis and treatment of gastroesophageal reflux in children]. / Diagnostika i lechenie gastroézofageal'nogo refliuksa u detei.

The work is based on an analysis of results of treatment of 643 children with gastroesophageal reflux (operations were performed on 69 of them). Methods of anesthesia are described. Conservative treatment was used in correlation with the degree of reflux-esophagitis. Indications to operations were considered to be as follows: hiatal hernias, reflux-esophagitis in children with cerebral paralysis, failure of conservative treatment in the group of children having no anatomical causes of the reflux. A method of surgical treatment is proposed including a combination of fundoplication with the posterior gastropexy and fixation of the esophagus to the diaphragm at a distance of 3 cm from the gastric cuff (43 cases, no recurrences). In patients with a combination of gastroesophageal reflux with the esophagus stenosis resection of the altered portion was made followed by plasty with a colonic transplant or local tissues (10 patients with the diagnosed Barrett esophagus).

[Heterodelphus--a rare form of twin developmental defect]. / Geterodel'fus--redkaia forma dvoinikovogo poroka razvitiia.

The authors saw a rare developmental twin anomaly--heterodelphus. The parasite was an organism devoid of the head, fixed with its thoracic wall an epigastric area to the front wall and the epigastric area of the autosite. Respiration and palpitation of the parasite was not auscultated. The abnormal twins had a single umbilical cord. The surgical division of the conjoined twins was performed in 4 months. The operation and operative findings are described. The postoperative period was hard. At the present time the boy's state is good, he feels well and develops in accordance with his age.

[The history of the surgery of esophageal atresia (based on data from the Saint-Petersburg Center for Neonatal Surgery)]. / Istoriia khirurgii atrezii pishchevoda (po materialam Sankt-Peterburgskogo tsentra khirurgii novorozhdennykh).

The article presents 42-year experiences with surgical treatment of atresia of the esophagus in 814 neonatals in the St. Petersburg Center of surgery in neonatals. Under analysis are the consecutive periods of work in this field, development and introduction into practice of the methods of surgical interventions, the original Bairov's operation--double esophagostomy--included. Factors responsible for results of the treatment of esophageal atresia are described. Improvement of the methods of treatment has resulted in a possibility mainly to make anastomoses of the esophagus and reduce lethality of neonatals with esophageal atresia from 81 to 7.7%.

[Methods of surgical treatment of Hirschsprung's disease in newborns]. / Khirurgicheskaia taktika pri bolezni Girshprunga u novorozhdennykh.

Fifty-nine newborn infants with Hirschsprung's disease were treated at the Saint-Petersburg Center of Surgery of Developmental Anomalies from 1978 to 1991. The typical rectosigmoid form was found in 47.5% and a long zone of agangliosis in 35.6% of children. Mortality rate--16.7%. According to the development of enterocolitis, three variants of the course of the disease in the newborns were distinguished. It is shown that the informativeness of the diagnostic methods increases with the growth of the infants, and the final diagnosis of Hirschsprung's disease is best established at the age of over two weeks on the basis of repeated or initially postponed contrast study of the large intestine. Inefficacy of nonoperative treatment conducted for 2-3 days and recurrence of symptoms of intestinal obstruction are indications for operative decompression of the intestine. The performance of one-stage radical operation at the age of about one month is advisable in an uncomplicated course of the disease.

[Relaparotomies following the creation of intestinal anastomoses in newborn infants]. / Relaparotomii posle nalozheniia kishechnykh anastomozov u novorozhdennykh.

An analysis of the immediate postoperative period in 295 newborns after putting intestinal anastomoses was made. Postoperative surgical complications were found in 38 children who required 47 relaparotomies. Surgical tactics was determined for the complications of the postoperative period as well as the indications for relaparotomy.

[Failure of intestinal anastomosis in newborns]. / Nesostoiatel'nost' shvov kishechnogo anastomoza u novorozhdennykh.

The analysis of clinical course of the immediate postoperative period in 295 newborns who had the intestinal anastomosis placed was carried out. In 31 (10.5%) child, a failure of the anastomotic sutures was revealed. A dependence of the clinical course of this complication on manifestations of peritonitis, concomitant diseases and degree of an organism maturity was determined. A difficulty to diagnose the anastomotic suture leakage is shown. The indications for operative and conservative treatment were established. Causes of the complication development were revealed.

[Immediate results of the surgical treatment of umbilical hernia in children]. / Neposredstvennye rezul'taty khirurgicheskogo lecheniia gryzh pupochnogo kanatika u detei.

Based upon an experience with treatment of 126 newborns the authors consider the main cause of unfavourable outcomes resulting from the operation proper to be inadequately elevated intraperitoneal pressure. Measures for prevention of this complication are recommended.

[Results of treatment of newborn infants with diaphragmatic hernias]. / Rezultaty lecheniia novorozhdennykh s diafragmal'nymi gryzhami.

The article generalizes an experience with the treatment of 91 newborns with diaphragmatic hernias for the period from 1955 to 1985. The diagnostics has improved and greater amount of newborns were admitted to the clinic within the first day after birth. The frequency and character of postoperative complications were analyzed as well as causes of their appearance and effective measures for their prevention and structure of lethality. The tactics of resuscitation both before and after operation has been changed: the artificial ventilation of the lungs was performed without a positive pressure at the end of the expiration with the use of Tubarin and infusion of Dopamine. Lethality was 2 times reduced.

[Immediate results of radical surgical treatment of newborn infants with anorectal defects of development]. / Neposredstvennye rezul'taty radikal'nogo khirurgicheskogo lecheniia novorozhdennykh s anorektal'nymi porokami razvitiia.

Results of the treatment of 334 newborns with anorectal defects are analyzed. Radical operations were performed on 181 children, praeternatural anus was made in 64 children, 37 children were operated upon for other associated defects. Conservative treatment was used in 52 children.

[Postoperative eventration in newborn infants]. / Posleoperatsionnaia éventratsiia u novorozhdennykh.

A method of prophylactics and treatment of postoperative eventrations in newborns (25 observations), is described. The incidence of this complication was decreased from 2,19% (1960-1971) to 0,95% (1972-1983).

[Treatment of injuries of the cervical spine in newborn infants]. / Lechenie povrezhdenii sheinogo otdela pozvonochnika u novorozhdennykh.

The method of treatment of fractures of the cervical part of the vertebral column in newborns with injuries of the spinal cord by cleol extension by the base of the skull is described. The method was successfully used in 17 patients.