Репозиторий Университета

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. Background. To assign genetically engineered biologic drugs, we need data on the predictors for response to therapy. Prognostic factors for the response to tocilizumab in patients with juvenile idiopathic arthritis (JIA) without systemic symptoms are poorly studied. Objective. Our aim was to reveal early predictors for the response to tocilizumab therapy in patients with JIA without systemic symptoms. Methods. A retrospective cohort study enrolled patients with JIA without systemic symptoms who received tocilizumab therapy between July 2009 and August 2017. We assessed the association between the initial demographic, clinical, and laboratory parameters in patients and the best response (according to the ACR90 criteria) to treatment after a year. Results. The study included 95 (girls 85%) patients; the mean age was 10.3 (6.0; 13.6). During the first year of therapy, 71 (75%), 55 (58%), 38 (40%), and 22 (23%) patients achieved the improvement according to ACR30/50/70/90 criteria, respectively; 22 (23%) patients reached disease inactive stage according to the Wallace criteria. When performing multivariate analysis, the following improvement predictors were revealed based on the ACR90 criteria after a year of treatment: decrease in serum C-reactive protein level during the first month of therapy [odds ratio (OR) 1.024; 95% confidence interval (CI) 1.007-1.051], decrease in disease activity score on the visual analogue scale according to the parent/patient assessment (OR 1.048; 95% CI 1.005-1.105), early onset of the disease (OR 0.38; 95% CI 0.16-0.72), persistent oligoarthritis according to the ILAR (OR 9.9; 95% CI 1.5-109.3). During the first year of tocilizumab administration, neutropenia was registered in one patient, leukopenia - in three cases, and urticaria - in one case. Conclusion. The variant of JIA, the age at the disease onset, and the disease course pattern in the first month of tocilizumab therapy are the predictors of treatment efficacy throughout the year.

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. Background. There are limited data on the efficacy of long-term enzyme replacement therapy (ERT) in children with mucopolysaccharidosis (MPS). Objective. Our aim was to study the efficacy and safety of long-term ERT in children with MPS type I, II and VI. Methods. We analyzed the results of ERT with laronidase, idursulfase and galsulfase in children with MPS type I, II and VI admitted to the federal research center from January 2007 to November 2016. The response rate was assessed by the level of normalized urinary excretion of glycosaminoglycans (GAGs) (the ratio of GAGs concentration to urine creatinine) recalculated in percent (%) exceedance of the upper limit of normal for the corresponding age. Data on the administered therapy and its results, including adverse events, is extracted from the medical records of in-patients. Results. The results of treatment (intravenous infusions, intervals between administrations from 4 to 10 days) were studied in 33 children (5 of them were girls) with MPS type I (n =4; laronidase at a dose of 0.58 mg/kg), II (n =26; idursulfase at a dose of 0.5 mg/kg), and VI (n =3; galsulfase at a dose of 1 mg/kg). A decrease in the normalized urinary excretion of GAGs from 376% (172; 791) to 54% (0; 146) exceedance of the upper limit of normal for the age (p <0.001) was noted in the course of ERT lasting (median) 27 (14; 41) months. A decrease in the normalized GAGs excretion below the upper limit of normal for the age was established in 12/33 (36%) patients. ERT-associated adverse events were identified in 12 patients; one case required a two-fold therapy interruption. The development of nephrotic syndrome in the course of ERT in patients with severe MPS II was first described. Conclusion. Long-term ERT in children with MPS type I, II and VI is characterized by acceptable efficacy and safety.

© 2018, Dynasty Publishing House. All rights reserved. The article reviews scientific literature, current recommendations dealing with the topical problems of the incidence and methods of diagnosing H. pylori infection, including among the pediatric population. The authors of the article pay attention to different figures of the global prevalence of H. pylori, to the dependence of infection on many factors, among them low socio-economic status and educational levels, overpopulation, pollution of sources of drinking water. Based on the principles of evidence-based medicine, the article analyses the level of sensitivity and specificity of the main methods of identification of H. pylori, emphasizes that the sensitivity of serological diagnostic method does not depend on factors decreasing bacterial load, such as intake of proton-pump inhibitors (PPIs), antibiotics, bismuth preparations, and also on gastric haemorrhage and gastric metaplasia. The serological diagnostic method in pediatrics remains attractive due to its non-invasiveness and low cost. But the problem of looking for highly informative and non-invasive methods of diagnosing H. pylori infection remains topical and needs further research.

© 2018 Russian Transplant Society. All rights reserved. Aim. To evaluate the functioning of an implantable pediatric axial pump «DON-3» for bypassing the left ventricle of the heart in experiments on sheep. Materials and methods. Five sheeps at the age of 12–18 months performed paracorporeal installation of the children’s axial pump «DON-3» according to the scheme «left ventricle – aorta». Results. A technique was developed and 5 chronic experiments were conducted on sheep to assess the model of the children’s axial pump «DON-3». The duration of the experiments in this series averaged 9 ± 5 days. The main indicators of hemodynamics, acid-base balance were within the norm. Conducted morphological and histological studies of the kidneys, liver and lungs did not reveal the presence of zones of ischemia and thromboembolism. Conclusion. The results of this series of experiments showed satisfactory results, suggesting further research on the development of prototypes of a pediatric pump for clinical practice.

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. Background. Cystic fibrosis is a hereditary disease that occurs as a result of mutations in the regulator gene of chloride ion transmembrane transport (CFTR). Finding mutations in the CFTR gene is necessary for identification of the clinical features of cystic fibrosis. Objective. Our aim was to identify genotype-phenotype correlations between mutations of the first class of pathogenicity and clinical manifestations of cystic fibrosis based on studying the prevalence and structure of CFTR gene mutations. Methods. The study included children under 18 years with cystic fibrosis admitted to hospital between 2013 and 2017. Biallelic mutations in the CFTR gene were the non-inclusion criterion. The CFTR gene variants were analyzed by next-generation sequencing method. Results. In 125 patients with cystic fibrosis, 59 different variants of the CFTR gene were detected, 11 of them not previously described. The most common was the deletion c.1521-1523del found in 98 (39.2%) of the 250 analyzed CFTR gene alleles and the deletion c.1545-1546del found in 22/250 (8.8%) alleles. It has been shown that the mutation c.1545-1546del, p.Y515∗ was more often found in children of the Chechen nation-odds ratio (OR) 139 (95% confidence interval 15-1,257). It has been established that meconium ileus, pancreatic deficiency and cirrhosis are more common in patients with mutations of the first category of pathogenicity: OR 3.9 (95% CI 1.0-15.0), 4.4 (95% CI 1.8-11.1), and 351 (95% CI 17.5-7,046), respectively. The association of CFTR gene mutations with the development of bronchiectases and polypous pancinusitis has not been found. Conclusion. Correlations between the genotype and clinical manifestations of cystic fibrosis in Russian children with CFTR gene mutations of the first class of pathogenicity have been established.

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. The article presents current data on the etiology, pathogenesis, and epidemiology of systemic lupus erythematosus (SLE). The SLE diagnosis details are considered with a description of each examination technique. Moreover, an assessment of reliability level of both evidence and recommendations for each thesis-recommendation are discussed thoroughly. The aspects of differential diagnosis and criteria for the diagnostic quality of SLE are revealed.

© 2018 Izdatel'stvo Meditsina. All rights reserved. Background: Inherited epidermolysis bullosa (EB) refers to a group of rare inherited disorders characterized by severe damage of skin and in most patients - the gastrointestinal mucosa, what leads to a violation of skin and mucosal barrier properties in relation to allergens. However, the issues of food sensitization and food allergy in this category of patients have not been studied, and the study of this problem is important. Aim: To evaluate the clinical manifestations of food allergy (FA) and IgE-response to food proteins in children with EB. Methods: 82 patients with EB aged from 2 months to 16 years were entered this open non-randomized observational prospective study, including 20 patients with simple form of EB and 62 patients with dystrophic form of EB. We analyzed allergic history and clinical manifestations of the FA in all the patients. Every patient in this study underwent of determination of the concentration of total serum IgE and specific serum IgE to the most important food allergens, as well as to mixtures of household allergens in some cases (UniCAP System, Phadia AB). Results: Skin lesion in patients with EB masks allergic skin manifestations, causing a hypodiagnosis of the FA in this category of patients, which in turn leads to erroneous organization of nutritional support. FA (clinical manifestations) was identified in 20.7% of children with EB (in 10% of cases with simple form of EB and in 24.2% - in patients with dystrophic form of EB). Products containing cow's milk protein, cereals, and eggs were identified as etiologic factors of FA in most cases. In the group of children with comorbidity FA and EB high and very high levels of total IgE (>1000 kUA / l) were detected most frequently. The main cause-significant allergens are cow's milk proteins, cereals, eggs. Conclusions: Comorbidity with FA is high in patients with dystrophic form of EB. The main cause-significant allergens are cow's milk proteins, cereals, eggs.

© 2017 New Terra Publishing House. All rights reserved. The article presents a comprehensive description of tuberculous infection nidi, where children were exposed to this infection and developed multiple drug resistant tuberculosis. It was found out that the risk to develop MDR TB in children was also related to exposure to a TB case with undetected resistance to rifampicin, which was to be taken into account when prescribing the chemotherapy regimen. When prescribing preventive treatment to children exposed to MDR tuberculosis one should consider the potential risks and benefits.

© Team of authors, 2018. The article describes the clinical case of subacute myocarditis in a child with an outcome in dilated cardiomyopathy, complicated with congestive heart failure. Difficulties in diagnosing the disease that manifested with cardiac arrhythmia are discussed. The main signs suggesting postmiocardic cardiomyopathy included a reduced deflection voltage and frequent ventricular extrasystoles with episodes of the ventricular tachycardia in the onset of the disease, left ventricular dilatation, a significant decrease in ejection fraction, increased activity of natriuretic peptide, findings of perfusion scintigraphy and magnetic resonance imaging. Medical therapy and implantation of a cardioverter defibrillator were ineffective. The child repeatedly suffered from syncopal conditions with circulatory arrest, which required resuscitation. Due to the ineffectiveness of drug therapy conducted in the leading federal centres, considering cardiac transplantation abroad was recommended.

© 2018 Publishing House of the Union of Pediatricians. All rights reserved. The article presents modern ideas about the treatment of systemic lupus erythematosus (SLE). The details of the management of patients with SLE during immunosuppressive and genetically engineered therapy is given. The article also reflects the aspects of rehabilitation, prevention of exacerbations, and follow-up care of children with SLE. The criteria for assessing the quality of medical care for children with SLE are presented. The detailed information on systemic lupus erythematosus for patients with SLE and their parents is outlined specifically.

© 2018, Media Sphera Publishing Group. All rights reserved. Objective. A pharmacoeconomic analysis of direct costs on treatment with high dose intravenous immunoglobulins (IVIG) and plasmapheresis (PP) in children. Material and methods. Literature data on the pathogenesis of Guillain—Barre syndrome (GBS) were analyzed. The results of pharmacoeconomic analysis of direct costs on treatment of GBS using IVIG and PP are presented. Risks for complications during treatment with IVIG and PP are calculated. Results and conclusion. The pharmacoeconomic analysis demonstrates comparable costs of treatment with IVIG or PP in the Russian Federation. Nevertheless, a less number of complications, convenience in use and the good safety and tolerability profile make it more preferable to this group of patients. In a clinical case of a 7-year child described in the article, treatment with 10% IVIG — privigen in dose 2 g/kg during 5 days started in the 3rd week of disease showed a marked positive effect.

© 2018, Media Sphera Publishing Group. All rights reserved. Studies aimed at raising awareness of various diseases among the population are becoming more relevant now. Different educational programs for the rapid recognition of stroke symptoms can be aimed at educating children and adolescents. Objective - to investigate the results of STOP-STROKE lessons at Moscow schools. Subject and methods. The Youth Council, Moscow Healthcare Department (MHD), in collaboration with the ORBI foundation gave open STOP-STROKE lessons among the pupils of Moscow schools. The investigation included the results of educating 29,653 4-10-form pupils who completed a specially designed questionnaire before and after the lecture that explained the main symptoms of stroke, as well as the actions to be taken when the latter was suspected. The lecture included the mnemonic test UDAR (blow) (U for smile, D for movement, A for aphasia, D for decision). Results. It was found that after education, there was better knowledge about stroke symptoms (aphasia, facial muscle weakness, and limb weakness) and actions to be taken when stroke was suspected. There was a significant increase in the number of children who correctly identified the three symptoms of the disease. Conclusion. The investigation demonstrated the effectiveness of the program. After being educated, the children can more quickly call for an ambulance if stroke occurs in a family member. Further investigations are needed to evaluate the effectiveness of these lectures, including in the long-term.

Цель. Определить у детей в возрасте от 2 до 7 лет ультразвуковых параметров лимфатических узлов, подверженных наибольшей антигенной стимуляции (шейные, мезентериальные), и установить взаимосвязь этих параметров с относительными размерами селезенки. Пациенты и методы. Проведено открытое скрининговое поперечное одноцентровое исследование на базе ФНЦ медико-профилактических технологий управления рисками здоровью населения г. Перми. В исследование включено 133 ребенка (62 мальчика, 71 девочка) из дошкольных учреждений Пермского края в возрасте от 2 до 7 лет. В течение 2011-2013 гг. проведено анкетирование и интервьюирование родителей респондентов с последующим анализом историй их развития, а всем детям, включенным в исследование, выполнены стандартная клиническая гемограмма, а также ультразвуковое исследование (УЗИ) селезенки с вычислением коэффициента массы селезенки (КМС) по оригинальной формуле и шейных и мезентериальных лимфатических узлов (ЛУ). В зависимости от значения КМС дети были поделены на 2 группы: в основной (n = 46) КМС превышал 4 ед. В группе сравнения (n = 87) этот показатель варьировал от 2,0 до 4,0 ед. Результаты. Дети из основной группы в 2,5 раза чаще (p < 0,05) болели острыми респираторными вирусными инфекциями, которые протекали с осложнениями у 27 (58,7%) детей со спленомегалией; в группе сравнения их было 13 (15,3%, p < 0,01). У детей основной группы в 1,8 раз чаще диагностировали гипертрофию небных миндалин и/или аденоидов 2-3-й степени, они достоверно чаще болели пневмонией. Сравнительный анализ ультразвуковых характеристик глубоких латеральных яремных ЛУ показал статистически значимые различия (p < 0,05) по всем измеряемым параметрам (длина, индекс округлости, толщина коркового слоя), за исключением максимальной систолической скорости кровотока в артерии узлов. Характеристики мезентериальных ЛУ (кроме их длины) в обеих группах пациентов также имели статистически значимые различия. Обнаружено, что у 43 (93,5%) детей из основной группы преобладали цепочки или конгломераты (более 2 в ультразвуковом срезе) шейных ЛУ; в группе сравнения - у 71 (81,6%). Множественные мезентериальные ЛУ обнаружены в 39 (84,8%) и 64 (73,6%) случаях соответственно в группах. Корреляционный анализ выявил достоверную (p < 0,05) прямую связь между значениями КМС и толщиной коркового слоя у глубоких латеральных яремных ЛУ шеи (r = 0,44). Выводы. У практически здоровых детей дошкольного возраста с установленной при УЗИ относительной спленомегалией изменения параметров ЛУ 2-3-го порядка могут отражать процессы онтогенеза иммунной системы и свидетельствуют о повышенной антигенной нагрузке. За детьми, значение КМС у которых превышает 4 ед, рекомендуется динамическое наблюдение с обязательным исследованием параметров мезентериальных и средних глубоких латеральных яремных ЛУ шеи.

Авторами рассмотрены современные аспекты лечения неспецифического аортоартериита (НАА) у детей. Освещены вопросы патогенеза, клинической картины, основных принципов лечения болезни Такаясу в детском возрасте, подходы к оценке эффективности и безопасности проводимой базисной терапии. Представлены алгоритм базисной терапии детей с НАА и проблемы хирургического лечения. Наиболее эффективным является комплексный подход к лечению, подразумевающий назначение в активной фазе заболевания противоспалительной иммуносупрессивной терапии глюкокортикостероидами и цитостатиками, а при неэффективности - использование генно-инженерной биологической терапии.

Бронхиальная астма (БА) является хроническим воспалительным заболеванием дыхательных путей. Основной целью терапии БА является достижение и поддержание полного контроля болезни. Контроль заболевания включает в себя снижение частоты обострений и частоты госпитализаций или полное их отсутствие, нормализацию показателей функции внешнего дыхания на фоне правильно подобранной базисной (противовоспалительной) терапии. Ингаляционные глюкокортикостероиды (ИГКС) обладают противовоспалительной активностью, воздействуя на основу заболевания, и высокой клинической эффективностью у детей всех возрастных групп. Если течение астмы не контролируется ИГКС, необходимо повысить их дозу или использовать их в комбинации с другими препаратами, чаще всего с бета-2-агонистами длительного действия (ДДБА). Существует вариант проведения длительной терапии при лечении БА - это интермиттирующая схема дозирования препаратов базисной терапии (ИГКС или комбинации ИГКС/ДДБА). В этом случае доза выбирается в зависимости от выраженности симптомов и течения БА с подбором минимальной поддерживающей дозы и возможностью временного увеличения ингаляций во время периодов обострения.

Бронхиальная астма - мультифакторное заболевание, в основе которого лежат генетическая предрасположенность и агрессивное воздействие различных аллергенов. Заболевание сопровождается такими симптомами, как кашель, дистанционные свистящие хрипы, одышка. Однако данные симптомы сопровождают и бронхообструктивный синдром (БОС), который наблюдается у детей в возрасте 5 лет и младше в связи с инфекциями верхних дыхательных путей, которые часто встречаются в этой возрастной группе. При оказании помощи ребенку с бронхиальной обструкцией необходимо оценить тяжесть БОС до лечения. В педиатрии для купирования остро возникающих нарушений бронхиальной проходимости используют ß2-агонисты, антихолинергические препараты и их комбинацию.

BACKGROUND:

The 2013 International Classification of Headache Disorders-3 (ICHD-3) was published in a beta version to allow the clinicians to confirm the validity of the criteria or to suggest improvements based on field studies. The aim of this work was to review the Primary Headache Disorders Section of ICHD-3 beta data on children and adolescents (age 0-18 years), and to suggest changes, additions, and amendments.
METHODS:

Several experts in childhood headache across the world applied different aspects of ICHD-3 beta in their normal clinical practice. Based on their personal experience and the literature available on pediatric headache, they made observations and proposed suggestions for the primary headache disorders section of ICHD-3 beta data on children and adolescents.
RESULTS:

Some headache disorders in children have specific features which are different from those seen in adults and which should be acknowledged and considered. Some features in children were found to be age-dependent: clinical characteristics, risks factors and etiologies have a strong bio psycho-social basis in children and adolescents making primary headache disorders in children distinct from those in adults.
CONCLUSIONS:

Several recommendations are presented in order to make ICHD-3 more appropriate for use with children.

BACKGROUND:

The 2013 International Classification of Headache Disorders-3 (ICHD-3) was published in a beta version to allow the clinicians to confirm the validity of the criteria or to suggest improvements based on field studies. The aim of this work was to review the Primary Headache Disorders Section of ICHD-3 beta data on children and adolescents (age 0-18 years), and to suggest changes, additions, and amendments.

METHODS:

Several experts in childhood headache across the world applied different aspects of ICHD-3 beta in their normal clinical practice. Based on their personal experience and the literature available on pediatric headache, they made observations and proposed suggestions for the primary headache disorders section of ICHD-3 beta data on children and adolescents.

RESULTS:

Some headache disorders in children have specific features which are different from those seen in adults and which should be acknowledged and considered. Some features in children were found to be age-dependent: clinical characteristics, risks factors and etiologies have a strong bio psycho-social basis in children and adolescents making primary headache disorders in children distinct from those in adults.

CONCLUSIONS:

Several recommendations are presented in order to make ICHD-3 more appropriate for use with children.

BACKGROUND:

The 2013 International Classification of Headache Disorders-3 (ICHD-3) was published in a beta version to allow the clinicians to confirm the validity of the criteria or to suggest improvements based on field studies. The aim of this work was to review the Primary Headache Disorders Section of ICHD-3 beta data on children and adolescents (age 0-18 years), and to suggest changes, additions, and amendments.
METHODS:

Several experts in childhood headache across the world applied different aspects of ICHD-3 beta in their normal clinical practice. Based on their personal experience and the literature available on pediatric headache, they made observations and proposed suggestions for the primary headache disorders section of ICHD-3 beta data on children and adolescents.
RESULTS:

Some headache disorders in children have specific features which are different from those seen in adults and which should be acknowledged and considered. Some features in children were found to be age-dependent: clinical characteristics, risks factors and etiologies have a strong bio psycho-social basis in children and adolescents making primary headache disorders in children distinct from those in adults.
CONCLUSIONS:

Several recommendations are presented in order to make ICHD-3 more appropriate for use with children.

BACKGROUND:

The 2013 International Classification of Headache Disorders-3 (ICHD-3) was published in a beta version to allow the clinicians to confirm the validity of the criteria or to suggest improvements based on field studies. The aim of this work was to review the Primary Headache Disorders Section of ICHD-3 beta data on children and adolescents (age 0-18 years), and to suggest changes, additions, and amendments.

METHODS:

Several experts in childhood headache across the world applied different aspects of ICHD-3 beta in their normal clinical practice. Based on their personal experience and the literature available on pediatric headache, they made observations and proposed suggestions for the primary headache disorders section of ICHD-3 beta data on children and adolescents.

RESULTS:

Some headache disorders in children have specific features which are different from those seen in adults and which should be acknowledged and considered. Some features in children were found to be age-dependent: clinical characteristics, risks factors and etiologies have a strong bio psycho-social basis in children and adolescents making primary headache disorders in children distinct from those in adults.

CONCLUSIONS:

Several recommendations are presented in order to make ICHD-3 more appropriate for use with children.