Background. Studies have shown that vaccination in the first hours/days after birth shifts the immune response from intrauterine Th2 towards Th1-type activation and reduces the risk of atopic conditions. However, we did not find published data from prospective studies on this topic.

Objective. The aim of the study is to define the presence of negative correlation between vaccination against tuberculosis and hepatitis B in the first hours/days of life and atopic dermatitis development in infants.

Methods. Continuous prospective study of children cohort born from April to June 2021 and observed in one outpatient’s clinic was carried out. Data from 307 infant’s records (F. 112/y), vaccination record cards (F. 063/y), prenatal and delivery records (F. 113/y-20, section № 3), and neonatal discharge summaries were analyzed for the decreed period. The child vaccination status (by the time of vaccination against tuberculosis and hepatitis B), presence of risk factors for allergic disease development, and presence of atopic dermatitis were evaluated.

Results. Atopic dermatitis (AD) was significantly less likely to be diagnosed by the age of 1 year in infants from the group of BCG-M vaccinated at maternity hospital than in those vaccinated later or not vaccinated at all (15.2% versus 66% and 35.7%, respectively; p < 0,01). AD was less likely to develop in children with risk factors for allergic disease who were vaccinated against tuberculosis in the maternity hospital than in those vaccinated later or unvaccinated at all (18, 75 and 62.5%, respectively; p < 0.01). The ratio of children with diagnosed AD by the age of 12 months was significantly less in the group of children vaccinated against hepatitis B in the maternity hospital than in those vaccinated later or unvaccinated at all (17.6, 44.9 and 31.8%, respectively; p < 0.01). These ratios for children with risk of allergic disease development were 24%, 50% and 44.4%, respectively (p = 0.043). It has also been shown that timely vaccination with both vaccines in the early neonatal period significantly reduces the risk of AD in general infant population compared to non-vaccinated individuals or those who had only one vaccine (odds ratio [OR] 0.374; 95% confidence interval [CI] 0.253-0.552; p < 0.01). Whereas the disease development in children with allergic risk is less likely with timely vaccination (20.8% versus 53.3%; OR = 0.252; 95% CI 0.145–0.440; OR = 0.374; 95% CI 0,253–0,552; p < 0,01).

Conclusion. The obtained results may indicate possible risk reduction for AD development due to timely preventive vaccination against tuberculosis and hepatitis B, especially in children with allergic risk. The study is currently ongoing. 

Experts of The Union of Pediatricians of Russia have developed current clinical guidelines for management of children with community-acquired pneumonia, which were approved by the Scientific and Practice Council of Ministry of Public Health of the Russian Federation in January 2022. Particular attention is paid to the etiological structure, modern classification, diagnostic tests and flagship approaches to antibacterial therapy of community-acquired pneumonia in children based on the principles of evidentiary medicine. 

Background. Balanced feeding with timely supplemental feeding implementation ensures optimal rates of physical and psychomotor development. It is the most effective way for preventing chronic non-communicable diseases and programming health during human life.

Objective. The aim of the study is to analyze the timing of the supplemental feeding administration in infants regarding their physical development.

Methods. Online questionnaire was developed via Google Forms. Responses from mothers of 108 children aged from 5 months to 3 years were analyzed. Physical development was evaluated via the WHO Anthro program.

Results. The timing of first supplemental feeding implementation corresponded to the Optimizing program on infants feeding in Russian Federation (2019) in 89.8% (n = 97) of cases, after 6 months — 6.5% (n = 7) of cases, and before 4 months — 3.7% (n = 4) of cases. The first supplemental feeding most commonly was vegetable puree (65.7%; n = 71), less often — porridge (18.5%; n = 20), or fruit puree (13.9%; n = 15). The meat puree was implemented after 8 months in 57.4% (n = 62) of cases. The mean of z-score for the body mass index to age ratio in children with late supplemental feeding implementation was − 1.1 ± 0.58. It has turned out to be significantly lower than in case of timely administration: 0.08 ± 0.11; p ≤ 0.05.

Conclusion. Late supplemental feeding administration (especially foods with high biological value) resulted in nutritional status violation through body weight deficiency in children both on breastfeeding and formula feeding. 

Background. Atopic dermatitis (AD) treatment is based on clinical recommendations (CR, 2021). It is reasonable to use other effective treatment methods due to continuous and often relapsing course of dermatosis, and thus physiotherapy treatment is important.

Objective. The aim of the study is to evaluate the efficacy of chromotherapy with a spectrum of 470 nm (blue spectrum) in children with AD complicated by secondary infection.

Methods. The study included 40 children aged from 2 to 12 years. All patients received standard treatment regimen according to the current CR and were divided into 2 groups, equal in number (20 patients each): the control group (CG — only traditional therapy), and the experimental group (EG — traditional therapy + blue spectrum chromotherapy (470 nm)).

Results. The SCORAD index (the sum of objective and subjective features) before treatment in the whole patient cohort was 32.5 ± 6.99 points, which corresponds to the moderate-severe form of the disease. The positive effect of therapy was registered in all patients (n = 40). Persistent process stabilization on the 7th day 7 of treatment in EG was observed in 8 patients, and in CG — in 2 (40% and 10%, respectively); p = 0.065. After the end of the treatment (10th day) persistent remission prevailed in EG and was registered in 90% (n = 18) of patients, and in CG — only in half of all patients, 55% (n = 11); p = 0.031. The evaluation of the AD severity due to the SCORAD index in patients with residual clinical manifestations clearly indicates that in EG its value corresponded to a mild disease course (p < 0.001), and in CG — the lower border of the moderate-severe and the upper border of the mild disease course (p < 0.05).

Conclusion. The use of blue spectrum chromotherapy in treatment of children with AD is the effective and accessible method that can be used as an addition to traditional treatment in cases with secondary infectious complications. Its use has a potential to exclude polypragmasy in this dermatosis treatment. 

Background. Necrotizing enterocolitis (NEC) is severe gastrointestinal disease in newborns, its early clinical symptoms are nonspecific. Fecal сalprotectin (Cp) is considered as one of the early biomarkers of NEC, thus, its use in newborns is poorly known.

Objective. The aim of the study is to evaluate clinical significance of fecal Cp measuring as an early NEC marker in newborns. Methods. The prospective cohort observational study included high-risk newborns (gestational age (GA) ˂ 33 weeks and/or body weight ˂ 1500 g) and moderate risk newborns (GV≥ 33 weeks at suspicion on NEC) of NEC admitted to the neonatal intensive care unit on the 1st day of life. The Cp level was studied via immune colorimetric method on the 3rd and 7th days of life in high-risk children and in all children at NEC manifestation. All patients were divided into 2 main subgroups after the end of the follow-up period: newborns with developed NEC (a) and children without NEC (b). Obtained Cp values were compared between these subgroups.

Results. There were no statistically significant differences in Cp levels at the 3rd and 7th days of life in the high-risk group of children with NEC and without NEC. However, it was noted that children without NEC showed a decrease in Cp levels on the 7th day, while children with NEC manifestation, indeed, had increased levels. Cp levels did not differ in the moderate risk group.

Conclusion. Single measurement of Cp level in newborns of any GA is uninformative towards the NEC prognosis and diagnosis. However, evaluation of the Cp level dynamics at the 7th day of life compared to the 3rd day of life may be considered as a promising non-invasive method for prognosis of NEC manifestation in premature infants (GA<33 weeks). 

Background. Methemoglobinemia is a group of diseases caused by various factors where methemoglobin (MetHb) content in the blood increases above the physiological norm.

Clinical case description. Clinical case of methemoglobinemia that was an incidental finding in two-year-old girl who was in the clinic due to the head injury is presented. Our examinations have revealed zero activity of cytochrome-b5-reductase enzyme. This fact indicates homozygous mutation. The cyanosis and hypoxia were relieved by ascorbic acid courses (250 mg/day).

Conclusion. Increased alertness regarding long-term isolated cyanosis should be presented when excluding its most common causes. Timely management could prevent severe complications development. 

Background. Neonatal thromboses, both venous and arterial, are a rare pathology, and experience in systemic thrombolysis is very low. Thus, the methods of adult management are currently being adapted to pediatric practice.

Clinical case description. This report describes clinical case of abdominal aortic thrombosis in a premature infant with later diagnosed Wolff-ParkinsonWhite syndrome and acquired hypothyroidism developed due to antiarrhythmic therapy.

Conclusion. The premature boy had high comorbidity that required many specialists’ involvement. Timely systemic thrombolytic therapy and antiarrhythmic drugs selection made it possible to save the child's life and most likely to avoid disability in the future. 

Background. X-linked lymphoproliferative (XLP) syndrome is hereditary disease with the incidence of 1-3 per 1 million born boys. This clinical case demonstrates a rare picture of XLP type 2 manifestation without prior Epstein-Barr virus.

Clinical case description. Boy D., 15 years old, was admitted to Morozovskaya Children's City Hospital with complaints on fever, abdominal pain, loose stools, weight loss. The past medical history included hemophagocytic syndrome (remission) and acute erythema nodosum. We have performed several studies: abdominal ultrasound (hepatomegaly, dynamic changes in the intestine: parts of the small intestine were enlarged and walls were thickened, mass peristalsis, walls of transverse colon and descending colon are thickened up to 5 mm, mesenteric lymphadenopathy), rectosigmoidoscopy (high-activity ulcerative proctosigmoiditis corresponds to Crohn's disease), biochemical and clinical blood tests (active hemophagocytic syndrome), coagulogram (secondary hypocoagulation), myelogram (no data on hemoblastosis or aplastic condition). Virological blood tests (CMV, EBV, HHV-VI): negative. Laboratory and instrumental tests have revealed recurrence of hemophagocytic syndrome and Crohn's disease. The child was consulted by rheumatologist, hematologist, gastroenterologist, geneticist, neurologist, and clinical pharmacologist. The primary immune deficiency disease was suspected in this patient due to his medical history. Molecular genetic study was performed (deletion including the XIAP gene was revealed) and the diagnosis of primary immune deficiency was verified: X-linked lymphoproliferative syndrome type 2. Thus, allogeneic haematopoietic stem cell transplantation (HSCT) was performed.

Conclusion. XLP diagnosis and management require multidisciplinary approach. The early diagnosis is crucial due to the high risk of secondary complications development that can significantly worsen the disease's prognosis. Allogeneic HSCT is the only effective treatment for the disease. 

Background. Purpura fulminans (PF) – is an acute rapidly progressive thrombosis of small-diameter blood vessels located mainly on the skin of the extremities. PF is characterized by high mortality rates. Patients can have serious consequences, including amputations and loss of fingers, foots or even extremities in general.

Clinical case description. A clinical case of developing transient deficiency of protein S complicated by idiopathic PF on the 7th day after acute otitis in a 3-year-old boy due to past infection is presented. The progression of the disease developed within a few hours. The patient became hemorrhagic elements on the skin of extremities, and later — tissue necrosis. The conducted therapy facilitated to stop the pathological process in the form of PF, and also prevented the development of severe disabling complications in the child.

Conclusion. Differential diagnosis and treatment should be fast and accurate, as the development of PF occurs in mere hours. Diagnostics should include expanded panel of coagulological tests and instrumental examinations, which determines the justifiability of a multidisciplinary approach in the patient management with disorders in the hemostatic system. 

Background. Glucose-6-phosphate dehydrogenase deficiency is one of the most frequent enzyme defects leading to hemolysis. About 2% of Russian Federation population have this pathology. This clinical case demonstrates such complication of this disease as cholelithiasis.

Clinical case description. Patient B., 17 years old, is followed up by hematologist for anemia due to glucose6-phosphate dehydrogenase deficiency. Biliary calculi were revealed in 2019. Skin and scleral icterus was noted during examination, as well as soreness in the right hypochondrium during palpation. Magnetic resonance cholangiopancreatography has shown two calculi in the distal parts of the ductus choledochus, gallstones, dilatation of the common hepatic and cystic ducts. Complete blood count: hyperchromic megalocytic anemia. Biochemical blood test: hyperbilirubinemia (mostly direct one) after 2 days of hospitalization. Endoscopic lithoextraction from ductus choledochus was performed. Drug therapy was prescribed. Positive dynamics were mentioned at control tests. The patient was discharged in a satisfactory condition on the 10th day.

Conclusion. Adequate diagnosis and adequate management promote favorable disease outcome. 

Background. Angelman syndrome (AS) is rare genetic disease characterized by severe mental retardation, gross developmental speech delay, facial dysmorphia, disorders of motor activity, behavior and sleep, epileptic seizures manifestation. Pathology refers to diseases of genomic imprinting. There are four possible mechanisms of pathology development in Angelman syndrome: 15q11.2-q13 deletion on the chromosome 15 of maternal origin, mutation of the ubiquitin ligase (UBE3A) gene at the 15q11.2 locus on the chromosome 15 of maternal origin, uniparental disomy of the 15q11.2-q13 region of paternal origin, imprinting center defect.

Clinical case description. Clinical picture of Angelman syndrome in two twin sibs (boy E., girl V.) observed in Children’s City Clinical Hospital № 1 in Nizhny Novgorod is presented. Children from consecutive pregnancy with dichorionic-diamniotic twins; preterm birth at 30 weeks. There was burdened obstetric-biological and perinatal history due to the threatened miscarriage during this pregnancy and due to asphyxia of both children during delivery. The disease has classical course in both patients: static functions development delay, gross developmental speech and intellectual delay, behavioral and motor disorders (stereotypy, tremor, ataxia, episodes of unmotivated laughter, sleep disorders), presence of typical facial dysmorphia. This diagnosis was confirmed by the molecular genetic study (boy — mutation in exon 7 of the UBE3A gene, girl — mutations in exons 6 and 7 of the UBE3A gene). Features of epileptic seizure therapy in both patients are presented.

Conclusion. The presented clinical case demonstrates typical clinical picture of AS in two sibs confirmed by molecular genetic study. The therapy of this syndrome is very complicated. The described patients require constant anticonvulsant therapy, observation of neurologist, psychotherapist, working with speech pathologist. Complete correction of this syndrome is impossible.