Background. One of the effective ways to maintain the epidemic well-being of vaccine preventable diseases is to estimate immunization rates and coverage.

Objective. The aim of the study is to conduct comparative analysis of immunization levels among children in various regions of Russian Federation. Methods. Children aged from 6 months to 15 years conducted comparative analysis of indicators of documented immunization and immunization coverage according to forms 112/y and 036/y in four federal districts of Russian Federation. The immunization rate was estimated in individuals who had completed vaccination scheme according to the national immunization schedule (NIS) (of 2014). Immunization coverage was estimated by number of persons with at least one vaccination in past medical history.

Results. The analysis was performed on 2687 children medical records from the Central, Ural, Siberian and Volga federal districts (FD). BCG immunization rates in all FDs tend to be 100%. There are high levels of immunization against hepatitis B, as well as against measles, rubella and parotitis (the lowest values are noted in the Central Federal District: against hepatitis B — 74%, against measles, rubella, mumps — 69%, high — in the Volga Federal District: against hepatitis B — 95%, against measles, rubella, mumps — 97%). There is significant mismatch in immunization rates and immunization coverage for pertussis, diphtheria, tetanus and poliomyelitis in all FDs (from 20% to 40%) indicating that there are disturbances in vaccination schemes. Low immunization rates are noted against pneumococcal infection (from 12% in the Siberian Federal District to 36% in the Volga Federal District) and influenza (from 0.5% in the Volga Federal District to 9% in the Ural Federal District).

Conclusion. The highest immunization rates for all infections included in the NIS (apart from influenza) were revealed in the Volga Federal District. Influenza immunization rate is critically low in all FDs.

Background. Nasal cavity and nasopharyngeal pathology are crucial and common issue in pediatric otorhinolaryngology. One of the major reasons for the nasal breathing difficulties are adenoids, allergic rhinitis, acute rhinitis, rhinosinusitis, nasal septum deviation. All these diseases can occur individually and in combination with each other, while clinical signs can be similar.

Objective. The aim of the study is study and estimate the condition of nasal breathing and sense of smell in children with different nasal and nasopharyngeal pathology.

Methods. The study included 128 children, all children were divided into 3 groups comparable by gender and age. Children with confirmed acute ENT organ disease were included in the 1st group, children with allergic disease exacerbation were included in the 2nd group, and clinically healthy children (control group) were included in the 3rd group. Survey methodology: rhinomanometry (RMM), rhinoresistometry (RRM), olfactometry.

Results. It was revealed that nasal resistance in children with allergic diseases exacerbation is significantly higher both before and after anemisation of nasal mucosa with a solution of nasal decongestants than in children of the control group (according to the RRM). The air flow rate increases by average of 25% in children with various ENT organs diseases, and in children with allergic diseases — by average of 43% after anemisation of the nasal mucosa. The obtained data shows the role of nasal mucosal edema in patients with allergic diseases during exacerbation. Analysis of our RMM and RRM data suggests the importance of further use of these methods in children.

Conclusion. The obtained results will allow us to optimize the diagnosis approach and personalize the management of patients with various nasal cavity and nasopharynx pathologies. Nowadays, the study is ongoing.

Background. Gluten-free diet (GFD) is a nutritional pattern that excludes such component of grain crops as gluten. This diet administration should be strictly regulated due to its influence on the growing child nutritional status and on the course of skin diseases. Thus, studying the effects of GFD on the course of dermatologic diseases in various patients’ groups is crucial.

Objective. The aim of the study is to study the effects of GFD on nutritional status and skin condition of patients with dermatologic pathology and associated celiac disease, as well as gluten hypersensitivity.

Methods. The study included children hospitalized in the dermatology department. We have used obtained anthropometric data (weight, height, body mass index (BMI)) to estimate nutritional status, all calculations were performed via programs WHO Anthro (for children under 5 years old) and WHO AntroPlus (for children over 5 years old). Weight-for-age (WAZ), height-for-age (HAZ), BMI-for-age (BAZ) z-scores were evaluated. Estimation of skin pathological process was performed via SCORAD index for patients with atopic dermatitis, PASI — for psoriasis, SALT — for alopecia, VASI — for vitiligo, LoSCAT — for scleroderma. Assessment of anthropometric measures and skin disease severity was performed at study initiation and 6 months after nutrition correction.

Results. 1000 children with various dermatologic pathologies admitted to the dermatology department during the period from August 2020 to August 2021 were examined. Three patients’ groups were formed: with celiac disease, gluten hypersensitivity, and without any gluten-associated conditions. Assessment of anthropometric measures has shown positive dynamics in patients with celiac disease and gluten hypersensitivity on GFD, as well as in patients without any gluten-associated conditions on normal caloric diet according to age norms. All three groups have shown positive dynamics for skin pathologies.

Conclusion. Estimation of nutritional status and skin conditions on various stages of the study has shown the importance of differentiated approach in GFD administration in dermatologic patients.

Experts of the Union of Pediatricians of Russia have developed modern guidelines on the management of children with acute obstructive laryngitis and epiglottitis. Croup is the most common cause of acute upper respiratory obstruction in children aged from 6 months to 6 years. Usually respiratory viruses are the pathogenic agents of the disease. The etiological factor of epiglottitis is Haemophilus influenzae type b in the vast majority of cases (> 90%). However, nowadays, epiglottitis caused by this pathogen agent is more common in adults in countries with mass immunization against hemophilic infection. This article considers in detail etiology, pathogenesis, and classification issues. Special attention is given to diagnosis, differential diagnosis, and therapy (via evidence-based approach) of acute obstructive laryngitis in children.

The article describes issues of legislative environment for medical activities in educational institutions implementing supplementary general education programs. The area of children recovery and rehabilitation (in terms of ensuring medical activities) is in regulatory deficit and requires special attention from the state system. The analysis of the procedure for providing medical care to minor children during the recovery period and organized leisure in terms of any activities in children’s centers at international and all-Russian levels (approved by the order of the Ministry of Health of Russian Federation dated June 13, 2018, № 327н) was carried out. Features of work in organizations of recreation and rehabilitation of children under conditions of new coronavirus infection are described. The assessment of previously existing and modern health legislation for atypical educational institutions is presented. Several ways on improvement of legislative environment for medical activities on recovery and rehabilitation for the full implementation of children rights on recovery, rehabilitation and supplementary education have been proposed.

This literature review provides information on the deficit of clinically proven data on the drugs use in pediatric practice. Issues on why children are widely administrated with drugs that are normally intended for use in adults without any prior qualitative estimation of their safety and efficacy in appropriate clinical studies involving children have been analyzed. The authors considered the issues of carrying out clinical trials with the participation of children as study subjects: ethical, regulatory, and psychological aspects of this complex task. There are decisions for every of the listed aspect. The authors of analyzed sources and the authors of this article believe that these decisions may help to address all the challenges and evade the incorrect assessment and interpretation of initial data by children’s parents and adoptive parents (legal representatives). Issues of doctors’ alertness were considered separately. Information and excerpts from regulatory acts were given, as well as information from publications on awareness raising and in support of doctors-researches. This literature review has been carried out to solve problems of information gap in the field of drugs safety and efficacy in pediatric practice. The disclosure of discussion points for clinical trials with children will ultimately help to improve the quality of healthcare provided to children in the future and take advantage of recent technologies used in patients’ cohorts of other age groups.

Background. Primary intestinal lymphangiectasia (PIL) is severe and rare disease with poor prognosis. Its management is complex due to the lack of clinical guidelines (both Russian and international). Therefore, consideration of modern principles of this pathology treatment is still crucial, it also can improve the quality and life expectancy in these patients.

Objective. The aim of the study is to schematize and unify all relevant data on PIL cases over the past 10 years, to identify modern treatment principles for provision of timely medical car. Methods. Systematic review of publications from 2011 to 2021 in PubMed database was performed. Clinical cases, observational studies, and literature reviews were included.

Results. Nutrition therapy in the treatment of PIL has many advantages and it is the firstline therapy. However, modern medicine allows to help children with this pathology via somatostatin group drugs, immunosuppressants, and surgery in case of insufficient nutrition correction.

Conclusion. Primary intestinal lymphangiectasia has certain scientific interest in pediatricians and gastroenterologists’ practice. Understanding of the disease, its early diagnosis and pathological process localization allow us to choose the correct diet, to determine the need for surgical intervention, and to prescribe targeted drug therapy.

Background. The morbidity of new coronavirus infection remains high (in children as well). Children with bronchial asthma are not at risk this disease development and severe course of COVID-19 according to the previous studies results.

Objective. The aim of the study is to compare the morbidity rate of COVID-19 among children with and without bronchial asthma between May 2020 and October 2021 with the results from the beginning of pandemics. Methods. Online questionnaires in Google form have been developed and sent to parents of 83 patients aged from 7 to 17 years who have been interviewed at the beginning of the pandemics. The main group includes 49 patients diagnosed with bronchial asthma, and the control group includes 25 children without bronchial asthma.

Results. 45% (22) of patients had COVID-19 in the main group and 32% (8) — in the control group (p = 0.636). 80% of patients had mild course of disease and 20% — moderate in the main group, and 62% and 37% in the control group respectively. Febrile fever was observed in 40% of respondents in the main group and in 33% — in the control group; anosmia — in 35% in the main and in 14% — in the control group; pulse oximetry was used by 44% of patients in the main group (SpO₂ 94–98%) and 42% in the control group (SpO₂ 98–99%); 66% of patients had baseline therapy and 6% had rescue medications in the main group. No one from both groups has been hospitalized.

Conclusion. Pediatric patients both with and without bronchial asthma have COVID-19 more often compared to the pandemics beginning. The disease in most cases had mild course and did not require hospitalization, aggravations of bronchial asthma were rare, and saturation values did not decrease below 94%.

Children with chicken egg protein allergy are denied performing vaccination against measles, mumps, influenza in Russian Federation thus far, thus it is unreasonable. This article presents a clinical case of 2 years 6 months old boy with cow’s milk protein allergy symptoms and high levels of specific IgE antibodies (sIgE) to chicken egg protein. He was unreasonably denied performing vaccination against measles, rubella, and mumps at the place of residence according to false interpretation of the clinical situation as the absolute contraindication for vaccination. Lately, the child was successfully vaccinated against these infections at Research Institute of Pediatrics and Children’s Health in “Central Clinical Hospital of the Russian Academy of Sciences”. The examination dynamics is presented, as well as the data on the extended allergy diagnostics of separate components via the ISAC allergochip. Finally, the prognosis on food allergy resolution and recommendations on nutrition and further vaccination were given for this patient.

Background. This clinical case is of practical interest due to the lack of epidemiological and clinical data in Russian Federation and worldwide, difficulties in diagnosis at the disease onset, as well as little experience in enzyme replacement therapy for mucopolysaccharidosis type IVA (MPS IVA).

Clinical case description. MPS IV (Morquio syndrome) diagnosis at the age of 11 was based on typical progressive external signs such as thoracolumbar scoliosis, coxarthrosis with restriction of both active and passive movements. Enzymodiagnostics in dried blood spots has shown dramatic decrease of N-acetylgalactosamine-6-sulfatase activity — 0.01 μM/l/h specific to MPS IVA. Direct automatic sequencing of the GALNS gene has revealed pathogenic nucleotide variants in the compound heterozygous state: c.143T>G; p(Val48Gly) and c.697G>A; p.(Asp233Asn). That allowed us to establish MPS IVA diagnosis. Enzyme replacement therapy with elosulfase alpha was started according to the protocol. According to significant changes in the musculoskeletal system, the expected therapeutic effect (stabilization and no disease progression) can be achieved not earlier than after 8–12 months.

Conclusion. Timely MPS diagnosis allows us to implement enzyme replacement therapy at the earliest possible time and thereby to avoid non-reversible complications of the disease itself.