PEDIATRIA Journal named after G N SPERANSKY
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The authors describe clinical observations of children with acute mesenteric adenitis with PCRconfirmed COVID-19 infection. Attention during ultrasound diagnostics is drawn to the condition of the lymph nodes – mesenteric adenitis in COVID-19 infection was manifested by a significant increase in the size of the nodes – more than 15 mm, a large number of nodes in the cut with a tendency to form a conglomerate, a more rounded shape, significantly increased blood flow, a reaction of the peritoneum in the form its thickening and the presence of free fluid in the abdominal cavity. Unusual for a «ordinary» respiratory viral or intestinal infection type of lymph nodes was the only sign that distinguishes the manifestations of mesenteric infection in COVID-19 infection.

The purpose of the study was to assess the efficacy, safety and pharmacokinetics of the moroctocog alfa (Octofactor) in children aged 2-6 with haemophilia A. Materials and methods of research : six patients between 2 and 6 years of age (average age 4.3±0.8 years) were included in the open multicenter prospective clinical trial. The efficacy of the drug was assessed against the background of the introduction of 30±10 IU/kg every 2–3 days, the safety was assessed by the frequency and causality of adverse reactions. Results: 7 post-traumatic bleeding was registered. The average prophylactic dose of the drug is 37.84±7.13 IU/kg. The dose of the drug for stopping bleeding was 1000 IU. 2 adverse events have been reported that are not related to moroсtocog alfa. Conclusion: the obtained data indicate the efficacy and safety of moroсtocog alfa in the study group of patients.

Chronic constipation (CC) is an important medical and social problem, as it can lead to the development of complications, fecal intoxication, social disadaptation and self-isolation of a child, as well as significantly reduce the quality of life. The article presents the results of a clinical study of CC in children using a new diagnostic method - transabdominal and transperianal ultrasound examination (US) of the colon and anorectal zone. The objective of the study was to analyze the course of CC in children depending on the mechanism of its formation according to US of the colon and anorectal zone. Materials and methods of research: the prospective cohort study included 470 children with an established diagnosis of CC at the age of 1 month to 17 years old. The patients were divided into 3 age groups: 1 month. – 3 years (n=166), 4–10 years (n=102), 11–17 years (n=202). The median follow-up was 48 months. The age distribution of children with CC was carried out on the basis of the frequency of visits, against the background of the beginning of attending kindergarten and/or elementary school groups, and the emergence of psychological problems due to the difficulty of going to a public toilet. An evaluation of analysis of the dynamics of US changes in the colon and anorectal zone was carried out by the method of ultrasound diagnosis of Hirschsprung's disease in children (patent № 2547614, authors M.I. Pykov, M.M. Kolisnichenko, I.V. Poddubny). Results: in children aged 1 month to 3 years, 66.9% of cases are statistically significantly dominated by proctogenic constipation type of constipation with US signs of anus spasm and anal sphincter insufficiency. In children aged 4 to 10 years, the mixed mechanism of CC formation prevails (41.1%), and in patients aged 11 to 17 years – cologenic (77.7%), for which the most typical ultrasound signs are dolichocolon (22.8%), anus spasm (8.7%), anal sphincter insufficiency (4.7%). It has been established that despite adequate therapy, the number of patients with the above-described mechanisms of formation of CC increases, which indicates the recurrence and progression of pathological process in children of different age groups. Conclusion: the proposed diagnostic method – transabdominal and transperianal US of the colon and anorectal zone is a highly informative, pathogenetically substantiated method for examining the state of the colon and anorectal zone in children with CC, built on precise objective criteria that are available at any age.

Crohn's disease (CD) is chronic recurrent bowel disease of unknown etiology, characterized by segmental transmural granulomatous inflammation, mainly with the development of local and systemic complications. Despite the active development of conservative therapy methods, the number of drug-resistant forms of CD and complications of the disease requiring surgical treatment continues to increase. The article reflects modern scientific ideas about the methods of diagnosis, conservative and surgical treatment of CD in children.

The aim of the study was to evaluate the technical feasibility of performing a Santulli-type side-toend inter-intestinal anastomosis in infants with cystic fibrosis (CF) using laparoscopy. A clinical case of a 3-month-old child with CF who was operated on in the neonatal period for meconium ileus is presented. The baby's gestational age was 32 weeks. The primary operation consisted of the formation of a terminal enterostomy. In the postoperative period, a malabsorption syndrome was noted, accompanied by a lack of growth. As a temporary measure to restore nutritional status, a Santulli-style side-to-end laparoscopic intestinal anastomosis was performed. The observed child was found to have the F508del mutation in both alleles of the CFTR gene. The patient's weight at the time of the Santulli anastomosis construction was 2900 g, the age – 3 months. During the operation, there were no difficulties associated with the mobilization of the separated segments of the ileum. The duration of the surgical intervention was 70 min. Enteral nutrition was started on the 3rd day after the operation. The recovery period for intestinal transit through the rectum was 15 days. The postoperative period was uneventful. The duration of hospitalization was 18 days. No electrolyte imbalance or excessive fluid loss or underweight associated with enterostomy was observed. Over the next 6 months, the normalization of age-related weight and height parameters was achieved, after which the continuity of the digestive tract was restored by closing the terminal enterostomy. Currently, the patient has minimal respiratory symptoms and is receiving adequate CF therapy with pancreatic enzyme replacement therapy included with each meal. The initial experience of performing laparoscopic Santulli inter-intestinal anastomosis in an infant with CF presented in the study showed the possibility and reproducibility of this technique, expanding the boundaries of laparoscopy in pediatric practice. The decision to close the enterostomy in these patients should be deferred until a full diet is introduced and should be made in conjunction with a pediatrician specializing in the treatment of CF.

The objectives of this study were to determine the frequency and search for additional criteria for the diagnosis of visceroabdominal disproportion syndrome (VADS) in newborns with gastroschisis. Materials and methods of research: prospective controlled observational cross-sectional analytical study was carried out in 61 newborns with gastroschisis, admitted from June 2009 to July 2021. To search for the most significant factors indicating the presence of VADS, the following parameters were recorded in the studied patients: the size of the defect in the anterior abdominal wall, the composition of eventrated organs, the presence of a conglomerate of intestinal loops, the diameter of the intestinal tube, the thickness of the intestinal wall, the degree of visceroabdominal disproportion (VAD). Results: VAD was detected in 50 (82%) newborns with gastroschisis. In 10 (17%) cases, VAD was mild, in 27 (44%) – moderate, and in 13 (21%) patients – severe. A moderate direct relationship was found between the size of the anterior abdominal wall defect (r=0.29, p=0.022), intestinal tube diameter (r=0.56; p=0.001) and the severity of VAD. There was a strong direct correlation between the thickness of the intestinal wall, and the frequency and severity of VAD (r=0.93, p=0.001). A direct association was found between the presence of a conglomerate of intestinal loops in the eventrated organs and the frequency of VAD (p=0.002). There was no statistically significant relationship between the number of eventrated anatomical structures with the frequency and degree of VAD (p=0.36). Conclusion: to determine VADS, it is advisable to diagnose the following pathological conditions in patients with gastroschisis: thickening of the intestinal wall, dilatation of the intestinal tube, the presence of a conglomerate of tightly welded eventrated organs, the severity of which is directly proportional to the degree of disproportion.

A rare clinical case demonstrates the difficulty of diagnosing intussusception in preterm infants. As it is often misdiagnosed as NEC, and surgery gets delayed. Distinguishing between NEC and intussusception in preterm infants when they coexist can be difficult as these conditions share common symptoms.

The aim of the study was to determine the clinical features of anti-NMDAR encephalitis in children. Materials and methods of research: 11 patients were selected from the group of children with autoimmune encephalitis, who met the diagnostic criteria for reliable anti-NMDAR encephalitis. A retro-prospective analysis of clinical symptoms, laboratory, neurophysiological and neuroimaging data, treatment, duration of primary hospitalization and long-term results of treatment was carried out, neuropsychological testing of patients was performed in the follow-up. Results: the age of the patients was 8.5±4.4 years, the gender composition of boys/girls was 1/10 (9.1%/90.9%). The average follow-up period was 17±12 months. In 73% of cases, there was an acute onset of the disease without a prodromal phase and with rapidly growing signs of neurological dysfunction: behavior change (11/100%), epileptic seizures (11/100%), speech impairment (10/90.9%), movement disorders (10/90.9%), disturbed sleep/wakefulness rhythm (9/81.8%), hallucinations (5/45.5%), autonomic disorders (6/54.5%), sensory disturbance (1/9,1%). In 82% of cases, therapy was required in the intensive care unit. When analyzing EEG monitoring in children, the extreme delta brush pattern was revealed in only one patient. Neuroimaging revealed no specific changes in the substance of the brain. The paraneoplastic nature of the disease could not be established in any case. 4 patients (36.36%) had relapses of the disease after 1.5–27 months. from the onset of the disease. All children showed a favorable outcome without a gross neurological deficit, but complaints of increased fatigue, headaches, poor memory, a decrease in vocabulary, and impulsive behavior persisted.

During the COVID-19 pandemic, it is necessary to be wary of the development of pediatric multisystem inflammatory syndrome in children (PMIS) who have had a COVID-19 and had antibodies to the SARS-CoV-2 virus. The aim of this work is to describe two clinical cases in children with antibodies to SARS-CoV-2 against the background of yersiniosis in a 12-year-old child and salmonellosis in a 3-year-old child, which proceeded with a pronounced inflammatory reaction and required a differential diagnosis with multisystem inflammatory syndrome. These bacterial infections proceeded with severe intoxication and fever, had a polymorphic clinical picture with exanthema syndrome, conjunctivitis/scleritis, swelling of the palms/feet, diarrhea and toxic kidney damage, with a pronounced systemic inflammatory reaction – high leukocytosis with neutrophilia and lymphopenia, a significant increase in C-reactive protein, procalcitonin, hypercoagulability (increased fibrinogen, D-dimer). Etiotropic antibiotic therapy led to recovery in both cases. Conclusions: During the COVID-19 pandemic, if antibodies to the SARS-CoV-2 virus are detected in children in the presence of signs of systemic inflammation and corresponding symptoms, the alertness of doctors and a timely comprehensive examination are necessary to exclude bacterial infections, also characterized by signs of systemic inflammation, for the purpose of differential diagnosis of PMIS.

The combination of duodenal atresia (DA) with esophageal atresia with tracheoesophageal fistula (EA-TEF) is a rare pathology, the frequency of which ranges from 1% to 6% of all cases of EA. Surgical treatment of the DA+EA-TEF combination always causes significant difficulties, primarily in determining the timing and stages of surgical correction. Objective of the study: to evaluate the results of treatment with a combination of DA+EA-TEF to determine the effective tactics of surgical treatment. Materials and methods of research: a retrospective, nonrandomized, uncontrolled, multicenter study was carried out. The work is based on the results of treatment of 15 newborns – 6 (40%) boys, 9 (60%) girls with a combination of DA+EA-TEF, who were treated in clinics of 6 university centers for pediatric surgery in the Russian Federation in 2015–2021. Simultaneous operations (SIMOPs) were performed in 10 (60%) patients, two-stage operations (TO) – in 5 (40%) newborns. The following criteria were taken into account: the period of antenatal (weeks) and postnatal (days) of establishing the diagnosis of obstruction of the gastrointestinal tract (GIT), gestational age (weeks), birth weight (g), weight at the time of surgery (g), type of concomitant pathology , sequence and methods of surgical treatment, terms of complete enteral feeding (days), outcomes of operations and reasons for unsatisfactory outcomes. The average gestational age of children who underwent SIMOPs was 35.1 weeks. (Q1 – 31.5, Q3 – 39; Me – 37; SD – 5.1; min/max – 25–40; 95% CI: 31.1–39.0) versus 29.8 weeks. at DO (Q1 – 29, Q3 – 30.5; Me – 30; SD – 1.0; min/max – 28–31; 95% CI: 28.4–31.6) did not differ statistically significantly (р=0.083). The mean body mass values did not have statistically significant differences (p=0.081) and amounted to 2224 g (Q1 – 1410, Q3 – 2930; Me – 2665; SD – 890.8; min/max – 760–3260; 95% CI: 1556–2926) for SIMOPs, versus 1322 g (Q1 – 1165, Q3 – 1450; Me – 1380; SD – 196; min/max – 980–1450; 95% CI: 1078.4–16565) in the TO group. Results: the average duration of SIMOPs was on average 144.4 min (Q1 – 125, Q3 – 155; Me – 147.5; SD – 22; min/max – 120–190; 95% CI: 1321–159.3), TO – 147.0 (Q1 – 125, Q3 – 172; Me – 140; SD – 33.4; min/max – 120–205; 95% CI: 126–178.6). The sequence of surgical correction of defects in SIMOPs in 8 (53%) patients consisted of thoracotomy, ligation of the TEF, direct anastomosis of the esophagus and the imposition of duodeno-duodenoanstomosis (DDA). In one case, DDA was selected as the first operation, which was supplemented with Kader gastrostomy followed by thoracotomy, ligation of the TEF and anastomosis of the esophagus after elongation according to I. Livaditis. In one patient, after thoracotomy and ligation of the TEF in connection with an insurmountable diastasis of the esophagus, a cervical esophagostomy (CE), duodenojejunoanastomosis (DEA) and a gastrostomy according to Kader were applied. In a two-stage correction (TO), the first operation in 3 patients was DDA (20%), supplemented in one case (7%) with Kader gastrostomy, and the second stage after 2 days performed thoracotomy with the elimination of TEF and EA. In 2 (13%) newborns, the first stage was thoracotomy, elimination of TEF and EA, followed by imposition of DDA 2 days later. In one case, due to an insurmountable diastasis of the esophagus after thoracotomy and ligation of the TEF, intrathoracic elongation of the esophagus according to Foker with delayed anastomosis of the esophagus (on the 7th day) and laparoscopic fundoplication according to Nissen (at 5 months) were performed. The duration of hospitalization did not statistically significantly depend on the chosen method for correcting the combination of DA+EA-TEF (p=0.79) and averaged 28.4 days for SIMOPs (Q1 – 16, Q3 –34.5; Me – 26; SD – 21.4; min/max – 5.0–79; 95% CI: 17.6–42.4), and for TOs – 27,2 days (Q1 – 21, Q3 – 33; Me – 28; SD – 7.1; min/max – 19–38; 95% CI: 27.2–33). In the group of patients with SIMOPs, 2 deaths (13%) were recorded on the 5th and 7th days after surgery due to progressive multiple organ failure and intractable pulmonary hypertension. In one case (7%), a lethal outcome was recorded 8 months after primary surgery due to progressive cardiovascular failure. Early postoperative mortality after SIMOPs was 20%, overall mortality was 30%. In the TO group, 3 (20%) deaths were recorded: 2 in the early postoperative period (on day 3 and day 19) and one at the age of 3 months of life. Early postoperative mortality after TO was 40%, overall mortality – 60%. Conclusion: it is preferable to choose the ligation of the TEF as the first operation and, if the child's condition allows, to impose an esophageal anastomosis and restore duodenal patency, followed by a nasogastric tube through the esophageal anastomosis into the stomach. If, after ligation of the TEF, the patient's cardiorespiratory status does not stabilize, it is possible to restore the patency of the esophagus and pass the probe into the stomach without imposing a gastrostomy, which will allow the patient to be further treated as an isolated DA, and the operation to restore the patency of the duodenum is delayed. In the presence of insurmountable diastasis, the use of esophageal elongation technology with subsequent delayed EA is justified.