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Backgrоund. HIV infected patients are at risk for vaccine-preventable infections such as tetanus and diphtheria. It is important to know about the protection of HIV-infected children from these infections, as there is a risk even after complete immunization. The protective immunity after vaccination against diphtheria and tetanus is measured by the antibodies level against these pathogens. The aim of this study was to evaluate the persistence of immunity to tetanus and diphtheria toxoid in HIV-infected children. Materials and methods. The protective immunity against diphtheria and tetanus was studied in children with HIV infection in the Kyiv City AIDS Center. Immunization rate was evaluated in 142 HIV-infected children. Serum samples were obtained in 59 HIV-infected children (study group) vaccinated against diphtheria and tetanus toxoid during routine check-ups. The results of anti-tetanus and anti-diphtheria antibodies were compared with the data of 17 children without HIV (the control group). The mean age of HIV-infected patients was 9.2 years old (range from 1.6 to 15.5 years old). Most children (38/59, 64.4 %) had mild clinical manifestations of HIV infection (I and II clinical stage of the disease). 89.8 % (53/59) of children of the group received antiretroviral therapy. The level of CD4+ T-lymphocytes within age norms was in 88.2 % of children receiving ART. Results. The research revealed lower vaccination coverage of HIV-infected children against diphtheria and tetanus (63.3 %) than in the general population. Only 19.7 % (28/142) of HIV-infected children were fully vaccinated according to the National schedule against these bacterial infections. A large proportion of children with HIV infection (79.3 %) vaccinated against these pathogens does not have protective levels of antibodies against diphtheria. The protective level of antibodies against tetanus was absent in 28.8 % of vaccinated HIV-positive children. Mean level of tetanus antibodies in serum was significantly lower in HIV-infected children (0.28 IU/ml) compared to the children not infected with HIV (0.53 IU/ml), p < 0.001. The average serum level of antibodies against diphtheria was also significantly lower in HIV-infected children (0.22 IU/ml) compared to the children not infected with HIV (0.43 IU/ml), p < 0001. Only 18.6 % of children in the study group were protected against both pathogens compared with 41.2 % of children in the control group. HIV-infected children are not immune to diphtheria and tetanus and are at risk of these infections. Conclusions. The main predictor of immune protection against diphtheria and tetanus is an early treatment of HIV infection (in the first 2 years of life), higher levels of CD4 + T-cells at the beginning of ART and ART vaccination after being started on ART. We recommend controlling the level of specific antibodies HIV-infected children who have received vaccination against diphtheria and tetanus before the ART beginning. In case of the absence of protective antibodies an extra booster against these bacterial infections is recommended to HIV-infected children.

Primary immunodeficiencies (PID) are a group of rare immune congenital disorders. Detection of PID, counting their number and nosology identification are important problems of pediatrics as it is important for the development of measures for diagnosis and treatment. The objective of this work was to study the incidence of PID in Ukraine and its nosologic forms by creating a registry of patients. Information about all patients with PID diagnosed during the 1995–2014 was collected in 27 administrative centers of Ukraine. At the beginning of 2015, the register included 814 patients, 684 of them — alive, among them 561 children and 123 patients older than 18 years. The minimal incidence of PID is 1.23 alive patients per 100,000, minimal incidence of giving birth to a child with PID — 9.94 per 100,000 live births. In Ukraine, the structure of PID in terms of immune system impairment is close to that of worldwide, the main groups of PID are presented with 39 nosological forms. The findings suggest a lack of PID detection in Ukraine, so the issue of increased alertness in doctors of different specialties concerning PID and timely referral of patients to child immunologist remains topical.

Over the last decades immunization significantly reduced morbidity and mortality from vaccinatedinfections. However, the children at risk are not always adequately vaccinated, despite a greater frequency of severe infections and complications. This may be due to the lack of recommendations for vaccination of risk groups or of attachment to their implementation. The overall goal is to raise vigilance concerning immunization of children at risk. Specific objectives: to be able to differentiate specific groups of risk in children, know the features vaccination of children at risk.

Hereditary angioedema — a rare disease caused by a congenital deficiency of C1-inhibitor. Clinical manifestations of the disease — recurrent episodes of angioedema, which manifest in childhood or adolescence, more often localized in the subcutaneous tissue (limbs, face, trunk, genitals) or submucosa (intestine, larynx). Swellings of the larynx are potentially harmful to the patient’s life. Early detection enables to carry out timely appropriate treatment and prevention of angioedema. The paper presents a case of hereditary angioedema with manifestation in early childhood. The case study demonstrates the complexity of diagnosis and treatment of hereditary angioedema.

Лекція «Імунологічні основи вакцинації» рекомендована для післядипломної освіти педіатрів та лікарів загальної практики.

Chronic granulomatous disease — primary immunodeficiency with X-linked and autosomal recessive inheritance, characterized by impaired bactericidal function of phagocytic immune system. Chronic granulomatous disease is accompanied by the development of severe bacterial and fungal infections of the skin, lymph nodes, lungs, liver, and excessive inflammation with granuloma formation. For the first time in Ukraine the paper describes cases of chronic granulomatous disease in children with a characteristic clinical phenotype in the form of severe bacterial infections with the lesion of the different organs and systems, the manifestation in the first months of life. We should note patients’ predisposition to mycobacterial infection, caused both by typical Mycobacterium tuberculosis, and atypical mycobacteria, including Mycobacterium bovis, BCG strain. Now the question of early diagnosis and optimization of treatment of chronic granulomatous disease in Ukraine remaines extremely topical.

Chronic mucocutaneous candidiasis is a genetically heterogeneous group of disorders associated with mutations in genes STAT1, IL17RA, IL17F, AIRE, STAT3, TYK2, IL12RB1, and IL12B. Chronic mucocutaneous candidiasis can present as a clinical syndrome in primary and secondary immunodeficiency, or distinct nosological entity of primary immunodeficiency. Today the major components of immunopathology of chronic mucocutaneous candidiasis are defects of immune response, mediated by IL-17 and IL-22. The article describes two cases of chronic mucocutaneous candidiasis, associated with mutations in gene STAT1, in children.