The problem of premature infants is well recognized in Russia and all over the world. The article discusses the mortality level and structure of extremely premature infant during 2015–2019. The aim of the study was to identify significant cause-effect relations for high mortality of premature infants and infants with extremely low body weight (ELBW) according to reports from obstetric departments, pediatric outpatient clinics, children's hospitals, statistical agencies, queries results from Territorial Compulsory Medical Insurance Fund (TCMIF) in Saint Petersburg and Russian Association of Human Reproduction (RAHR). The main demographic indices are presented by the Petrostat association. Russian Federal State Statistics Service (Rosstat) forms provided by the Center for Analysis and Prediction of Maternal and Child Health of the Saint Petersburg Health care Committee (No. 32 “Information on medical care for pregnant women, women in labour and new mothers” (approved by order of Rosstat No. 591 of 27.11.2015); No. 19 “Information on children with disabilities” registered in children's clinics (approved by order of Rosstat No. 866 of 27.12.2016); No. 14 “Information on the activities of medical facilities units providing inpatient medical care” (approved by order of Rosstat No. 723 of 05.12.2014); No. 30 “Information on the medical facility” (approved by order of Rosstat No. 483 of 03.08.2018 “On the approval of statistical tools for the organization of federal statistical observation in the field of healthcare by the Ministry of Health of the Russian Federation”) presented by Saint Petersburg Medical Informational and Analytical Center (MIAC)), and answers from TCMIF in Saint Petersburg and RARCH have been investigated. The authors have analyzed the fertility and mortality rates of premature infants, the group of children with ELBW has been established. High mortality levels have been revealed in the first weeks of life (22–23), they were mainly associated with infectious processes. The authors associate the premature delivery increase with the widespread implementation of assisted reproductive technology (ART). ART can be performed either via compulsory medical insurance funds, or other non-government sources. The assumption was proposed that there are possible violations of ART indications, contraindications, and the number of procedures. The ART efficacy is difficult to estimate as well due to insufficient information provided by Rosstat report form No. 32. The need of implementation of the new statistical form that will cover the data on the of ART administration, the possibility of efficacy estimation of modern invasive methods for infertility treatment and mandatory submission of reports about the use of these methods by medical facilities is discussed.

Background. Long-term immunosuppressive therapy in children (including genetically engineered biologic drugs, GEBD) is associated with a high risk of local tuberculosis (TB) development. Objective. The aim of the study was to examine efficacy of tuberculosis services in children with high risk of developing tuberculosis associated with immunosuppressive therapy.

Methods. The study included children at the age from 0 to 17 years on immunosuppressive therapy due to autoimmune disease and who were referred to phthisiatrician consultation. The incidence of TB was estimated one year after in groups receiving preventive TB services (isoniazid and pyrazinamide for 3–6 months) due to the high risk of TB development (contact with TB patients and/or controversial or positive test results with tubercular recombinant allergen) or not receiving such therapy (no indications for preventive treatment, parents’ refusal). The source of any data was medical documentation.

Results. Preventive tuberculosis service was performed in 167 (60%) out of 279 children included in the study, 112 children did not receive such treatment (5 cases — parents’ refusal, 107 cases — lack of indications for preventive treatment). TB was detected in 1 (0.6%) child after one year in the preventive treatment group, and in 14 (12.5%) children (p < 0.001) in the group without preventive treatment. Thoracic lymph nodes tuberculosis was diagnosed in 4 (27%) patients among all who has developed TB, tuberculous primary complex — in 3 (20%) patients, focal tuberculosis in 7 (46%) patients, disseminated tuberculosis in 1 (7%) patient.

Conclusion. Preventive tuberculosis service reduces the risk of tuberculosis in children on administration of immunosuppressive drugs, including GEBD.

Background. Premature infants have high risk of developing of neutropenia and infections in the early neonatal period. The correlation of these events requires further studies.

Objective. The aim of the study was to investigate the frequency of absolute neutropenia and infectious complications cases in premature infants in the early neonatal period with estimation of phenotypical and functional features of cord blood neutrophils.

Methods. The study included premature infants (gestational age 25–36 weeks) with APGAR score < 8 on the 1st and 5th minutes of life. The frequency of absolute neutropenia (at least once < 1.510⁹/l) and infectious complications (localized infections of bacterial etiology, early neonatal sepsis) cases in the first 14 day of life was analysed. Additionally, we have determined the expression of CD64, CD16, CD32 by cord blood neutrophils in premature (n = 102) and mature infants (n = 30) via method of flow cytofluorometry. We have used FITC labeled Escherichia coli to estimate their phagocytic activity, and stimulation of E. coli neutrophils in the presence of 5 mM of dihydrorhodamine 123 to estimate their stimulation index (ratio of mean fluorescent intensity (MFI) of activated neutrophils in stimulated samples and in negative controls, E. coli free samples).

Results. The episodes of absolute neutropenia in the first 14 days of life were recorded in 17 cases, infectious complications — in 87 children (in 24 cases — sepsis) in the group of premature infants. The frequency of infectious complications in premature children did not correlate with the frequency of absolute neutropenia episodes. Cord blood neutrophils in premature infants had higher CD64 expression and, on the contrary, lower CD16 expression, as well as low phagocytic activity and stimulation index value (in all cases p < 0.001).

Conclusion. Absolute neutropenia in premature infants in early neonatal period does not correlate with high risk of bacterial infections. However, cord blood neutrophils in premature infants had lower functional activity.

Background. Degos acanthoma is rare benign tumor. Typical signs of this disease are localization on the lower limbs, abdomen and anterior chest in middle-aged and elderly patients (50–70 years old). The only one clinical case of pale cell acanthoma with the atopic dermatitis, and eczema in the area of areoles in the 26 years old patient was described.

Clinical Case Description. The boy had redness and swelling in the area of the right mammary gland at the age of 11 years. This mass lesion was regarded as the eczema of the areola region due to burdened allergic history (atopic dermatitis), thus, antihistamine therapy was prescribed. Similar lesion has appeared in the area of the left areola 3 months later, the same therapy was used. Saniopurulent discharge was occasionally noted from the lesions. The child was consulted by dermatologist, endocrinologist and surgeon at the place of residence, and diagnosed with chronic purulent mastitis. Conservative therapy had no positive dynamics. Bilateral changes in areoles were revealed during hospitalization at the age of 13 years. There were red lesions with non-homogenous surface, yellow discharge, hyperemia around the right areola. The lesion sizes were 2.03.0 cm on the left and 3.03.5 cm on the right side. Biopsy has revealed pale cell acanthoma. Conservative treatment with oral and systemic glucocorticosteroids was prescribed, positive dynamics was obtained for 3 days after the therapy initiation.

Conclusion. The presence of eczema in the area of the nipple associated with sanioserous discharge requires the differential diagnosis with pale cell acanthoma. The conservative therapy (with glucocorticosteroids) efficacy for pale cell acanthoma is shown.

Background. Mucopolysaccharidosis type II (MPS II, Hunter syndrome) is a rare hereditary lysosomal storage disease associated with iduronate-2-sulfatase deficiency. Patients with MPS II require life-long enzyme replacement therapy (ERT) to replace the deficiency of endogenous enzyme. There are two medications — idursulfase and idursulfase beta — that are licensed and recommended for these patients in Russian Federation. However, it is well known that ERT can cause hypersensitivity reactions development.

Clinical Case Description. The ERT (idursulfase in the dose of 0.5 mg/kg once per week) onset in the male patient with severe MPS II was at the age of 2.5 years. Hypersensitivity reactions (urticaria, fever) were noted incidentally, thus, the premedication with antihistamines and antipyretics was performed. The ERT side effects has aggravated at the age of 8 years despite the glucocorticosteroids admission and infusion rate reduction up to 8–16 ml/h. That is why we have changed the medication on idursulfase beta with major clinical response: we have achieved control on both disease itself and hypersensitivity reactions.

Conclusion. The availability of two ERT medications for patients with MPS II expands treatment opportunities. In case of any allergic reactions due to idursulfase, the change on idursulfase beta reduces the risk of any ERT complications with sufficient control of MPS II course.

Celiac disease is an immune-mediated systemic disorder caused by gluten in people with genetic predisposition. Celiac disease is characterized by wide range of clinical manifestations (both gastroenterological and extraintestinal), that can complicate the diagnosis. Thus, celiac disease often remains undiagnosed. ESPGHAN has published updated clinical guidelines with adjusted coeliac disease diagnosis algorithms in 2020. It is proposed to determine antibodies to tissue transglutaminase (TGA-IgA) and total IgA within normal content of gluten-containing products in the diet on the first stage of children screening. The diagnosis of celiac disease can be established without small intestine biopsy in case of increased levels of TGA-IgA ≥ 10 of upper limit of normal and presence of antibodies to endomysium (EMA-IgA) in secondary serum. In such cases, ESPGHAN does not recommend any additional genetic testing to confirm celiac disease as it does not increase the reliability of the diagnosis. Antigen tests on class G or A antibodies against native gliadin are not specific and are not recommended for use in the diagnosis of celiac disease.

The aspects of the refusal to perform preventive vaccination and its outcomes in the frame of admission to educational organizations, as well as aspects of separation of vaccinated children from non-vaccinated ones are considered in this article according to the current legal regulation for this topic and to the litigation practice materials. Recommendations on Russian legislation improvement in the area of interest have been developed. The possibility of children (without performed tuberculin sensitivity test) admission to educational organizations is analyzed. The admissibility of the requirements for medical data provision on the child's health state at his admission to school is being considered. The current change initiatives on the legislation in the field of preventive immunization are discussed.