Current Paediatrics最新文献

{"title":"100 ЛЕТ СОВЕТСКОЙ СИСТЕМЕ ОХРАНЫ ЗДОРОВЬЯ МАТЕРИ И РЕБЕНКА: УСПЕХИ, ПРОБЛЕМЫ, УРОКИ","authors":"Александр Александрович Баранов, В. Ю. Альбицкий","doi":"10.15690/VSP.V17I1.1851","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1851","url":null,"abstract":"The article presents the text of the authors' report at the plenary session of the XX Congress of Pediatricians of Russia (dated February 16, 2018) dedicated to the centenary of the Soviet state mother and child welfare system. The features of its formation and development were described. The most important achievements in the field of child health care were outlined. Attention is focused on the personalities of the first facilitators of pediatric healthcare in Soviet Russia. Authors summarise the findings resulting from the history of the Soviet pediatric service.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"26 1","pages":"11-15"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81307651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Клинико-фармакологические подходы к оптимизации режима дозирования антибактериальных препаратов в педиатрии","authors":"Natal’ya B. Lazareva, Evgeniya V. Chikh, V. Drozdov, E. Rebrova","doi":"10.15690/VSP.V17I1.1855","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1855","url":null,"abstract":"The rational use of antibacterial drugs in children implies an adequate choice of the necessary medication, its dosing regimen, and the duration of treatment in order to achieve maximum efficacy and minimize toxic effects. The knowledge of pharmacokinetic and pharmacodynamic profiles of the antibacterial drug plays a crucial role for optimizing the dosing regimen. The strategy of individual choice of the dosing regimen, taking into account the principles of pharmacokinetics and pharmacodynamics, can be especially effective in patients with the expectedly changed parameters of pharmacokinetics and in infections caused by bacteria strains with low sensitivity to antibiotics. The review presents a contemporary view of pharmacokinetic and pharmacodynamic profiles of antibacterial drugs most commonly used in pediatrics and their relationship to the clinical efficacy of the administered therapy.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"192 1","pages":"54-60"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89471705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ЭФФЕКТИВНОСТЬ И БЕЗОПАСНОСТЬ ФЕРМЕНТОЗАМЕСТИТЕЛЬНОЙ ТЕРАПИИ У ДЕТЕЙ С МУКОПОЛИСАХАРИДОЗАМИ I, II И VI ТИПОВ: ОДНОЦЕНТРОВОЕ КОГОРТНОЕ ИССЛЕДОВАНИЕ","authors":"Л. А. Осипова, Людмила Михайловна Кузенкова, Лейла Сеймуровна Намазова-баранова, Анаит Казаровна Геворкян, Т. В. Подклетнова, Николай Андреевич Маянский, Григорий Валерьевич Ревуненков, Н. Д. Вашакмадзе","doi":"10.15690/VSP.V17I1.1858","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1858","url":null,"abstract":"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. Key words: children, mucopolysaccharidosis, enzyme replacement therapy, laronidase, idursulfase, galsulfase, glycosaminoglycans.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"2 1","pages":"76-84"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89530378","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ГЕНОТИП-ФЕНОТИПИЧЕСКИЕ КОРРЕЛЯЦИИ ТЕЧЕНИЯ КИСТОЗНОГО ФИБРОЗА У РОССИЙСКИХ ДЕТЕЙ. ПЕРВОЕ ОПИСАНИЕ ОДИННАДЦАТИ НОВЫХ МУТАЦИЙ","authors":"Ю. В. Горинова, К. В. Савостьянов, А. А. Пушков, А. Г. Никитин, Е. Л. Пеньков, С. А. Красовский, Ольга Игоревна Симонова, Лейла Сеймуровна Намазова-баранова","doi":"10.15690/VSP.V17I1.1856","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1856","url":null,"abstract":"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 noninclusion 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.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"28 1","pages":"61-69"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91277444","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ПЕДАГОГИКА В ПОДГОТОВКЕ ВРАЧЕЙ-ПЕДИАТРОВ НА УРОВНЕ ОРДИНАТУРЫ","authors":"Е. Н. Гринько","doi":"10.15690/VSP.V17I1.1852","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1852","url":null,"abstract":"The training program for a modern pediatrician involves the study of Pedagogy in medical residency in specialty 31.08.19 Pediatrics. The urgency of such training for the future doctor is uncontroversial today. Currently the experience of studying this discipline has been gained in Russian medical universities. At the same time this work requires further improvement and exchange of experience with colleagues.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"13 1","pages":"16-18"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73084134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Системная красная волчанка: клинические рекомендации. Часть 1","authors":"Екатерина Иосифовна Алексеева, Т. М. Дворяковская, Ирина Петровна Никишина, Рина Валериановна Денисова, Н. С. Подчерняева, О. А. Сухоруких, Л. С. Шубина","doi":"10.15690/VSP.V17I1.1853","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1853","url":null,"abstract":"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.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"1 1","pages":"19-37"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72597638","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ЭКСКРЕЦИЯ ВОДОРАСТВОРИМЫХ ВИТАМИНОВ (С, В1, В2 И В6) С МОЧОЙ У ЗДОРОВЫХ ДЕТЕЙ ДОШКОЛЬНОГО И ШКОЛЬНОГО ВОЗРАСТА: ОДНОМОМЕНТНОЕ ИССЛЕДОВАНИЕ","authors":"Светлана Геннадьевна Макарова, О. А. Вржесинская, В. М. Коденцова, О Г Переверзева, С. Н. Леоненко, Татьяна Владимировна Турти, Д. С. Ясаков","doi":"10.15690/VSP.V17I1.1857","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1857","url":null,"abstract":"Background . Children of preschool and school age are at risk of developing vitamin deficiency. Screening of the vitamin provision of children remains an urgent problem of pediatrics. Objective. Our aim was to determine the prevalence of low excretion of watersoluble vitamins among healthy preschool and school-age children. Methods. The study was conducted in March-April 2017. We determined the urinary excretion (fasting morning portion collected during 30–120 min after night-time urination) of metabolites of vitamins C, B 1 , B 2 , and B 6 in healthy children. Riboflavin (vitamin B 2 metabolite) was determined spectrophotometrically by titration with a riboflavin-binding apoprotein; 4-pyridoxyl acid (vitamin B 6 metabolite) and thiamine (vitamin B 1 metabolite) — by fluorescent method, ascorbic acid (vitamin C metabolite) — by visual titration with Tillman’s reagent. The excretion considered to be low (equivalent to vitamin deficiency) when thiamine excretion was < 7, 10, 11, and 12 μg/h and riboflavin < 6, 9, 10, and 13 μg/h in children aged 3–5, 6–8, 9–11, and above 12 years, respectively; 4-pyridoxylic acid — < 40, 60, and 70 μg/h in children aged 3–5, 6–8, and ≥ 9 years, ascorbic acid — < 0.2 and 0.4 mg/h in children aged 3–11 and ≥ 12 years, respectively. Results. Metabolites were excreted in 39 children (20 girls), 14 of them aged 4–6 years and 25 children aged 7–14 years. A low level of ascorbic acid excretion was found in 13 (33%) children, of thiamine — in 24 (62%), of riboflavin — in 16 (41%), of 4-pyridoxyl acid — in 26 (67%). Low excretion of at least one vitamin metabolite was detected in 30 (77%) children, of 3 or more metabolites simultaneously — in 15 (39%). Conclusion. A low level of urinary excretion of metabolites of at least one water-soluble vitamin (C, B 1 , B 2 , and B ≥ ) occurs in most preschool and schoolage children.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"46 1","pages":"70-75"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84720259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Типовые формы патологии печени у детей","authors":"Пётр Францевич Литвицкий, Лариса Дмитриевна Мальцева, Ольга Леонидовна Морозова","doi":"10.15690/VSP.V17I1.1854","DOIUrl":"https://doi.org/10.15690/VSP.V17I1.1854","url":null,"abstract":"This lecture for the system of postgraduate medical education analyzes causes, types, key links of pathogenesis, and manifestations of the main typical forms of liver pathology — liver failure, hepatic coma, jaundice, cholemia, acholia, cholelithiasis, and their complications in children. To control the retention of the lecture material, case problems and multiple-choice tests are given.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"64 1","pages":"38-53"},"PeriodicalIF":0.0,"publicationDate":"2018-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86209784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Случай течения первичного туберкулезного комплекса у ребенка при позднем выявлении // The Case of a Primary Tuberculosis Complex in a Child With Late Detection","authors":"V. B. Yukhimenko, Irina Petrakova Yu., A. M. Bagirov, F. M. Gubkina, S. Y. Berezovsky","doi":"10.15690/vsp.v16i6.1827","DOIUrl":"https://doi.org/10.15690/vsp.v16i6.1827","url":null,"abstract":"We present a case of late detection and course of the primary tuberculosis complex in a child who was previously in an unknown contact with a tuberculosis patient at the first year of life. The  disease onset simulating pneumonia took place at the age of 1 year  8 months. Hereafter, the child did not have any clinical signs of the  disease. At the age of 5 years, the child was examined in connection  with the hyperergic reaction to tuberculin: the results of computed  tomography revealed calcinated foci in the lung tissue and  calcifications in the intrathoracic lymph nodes; bronchoscopy  revealed infiltrative bronchial tuberculosis. Surgery was performed:  video-assisted thoracoscopy, resection of C9,10 part of the left lung  with precision removal of the focus in its C6 segment, extirpation of  bifurcation, and left bronchopulmonary lymph nodes. The results of  the histological examination indicated the chronic course of the  tuberculosis process with formation of bronchiectasis and presence  of bronchonodular fistulas. After surgery and the main course of  antituberculous therapy, a clinical cure with small residual changes was achieved. Представлен случай позднего выявления и течения первичного туберкулезного комплекса у ребенка, находившегося в ранее неизвестном контакте с больным туберкулезом на 1-м году  жизни. Дебют заболевания под маской пневмонии имел место в возрасте 1 года 8 мес. В  дальнейшем у ребенка не отмечалось никаких клинических признаков заболевания. В  пятилетнем возрасте ребенок обследован в связи с гиперергической реакцией на  туберкулин: по результатам компьютерной томографии выявлены кальцинированные  фокусы в ткани легкого и кальцинаты во внутригрудных лимфатических узлах; при  бронхоскопии — инфильтративный туберкулез бронхов. Проведено оперативное вмешательство: видеоассистированная торакоскопия, резекция части С9,10 левого легкого с прецизионным удалением очага в его С6 сегменте, экстирпацией бифуркационных и левых  бронхопульмональных лимфатических узлов. Результаты гистологического исследования  свидетельствовали о хроническом течении туберкулезного процесса с формированием  бронхоэктазов, наличием бронхонодулярных свищей. После операции и основного курса  противотуберкулезной терапии достигнуто клиническое излечение с малыми остаточными  изменениями.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"90 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76564550","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Двенадцать мифов о пищевой аллергии у детей // Twelve Myths About Food Allergies in Children","authors":"Гюзель Ильясовна Макарова, S. G. Makarova","doi":"10.15690/vsp.v16i6.1826","DOIUrl":"https://doi.org/10.15690/vsp.v16i6.1826","url":null,"abstract":"Food allergy is often the first manifestation of allergy in a child, and this problem is most relevant at an early age. With an allergic reaction to food, a seemingly not very significant pathology,  a so-called «atopic march» may begin with the development of  severe forms of allergic diseases in a child. At the same time, a favourable course of food allergy is possible with the disappearance of disorder signs with age. The disease prognosis depends on many factors, both genetic and environmental, but early diagnosis and adequate treatment of children with food allergies in most cases allow to  improve the prognosis of tolerance formation or, at least, to achieve  a mild course of food allergy manifestations. It is pediatricians who  first deal with early manifestations of food allergies, and adherence  to clinical guidelines for managing patients avoids the most common  mistakes. This article is addressed to practicing doctors. It considers  the most common mistakes of pediatricians in the management of  children with food allergies and discusses the surviving «myths»  about this pathology. Пищевая аллергия часто является дебютным признаком аллергии у ребенка, и эта проблема наиболее актуальна в раннем возрасте. С аллергической реакции на пищу — казалось бы,  не очень значимой патологии — может начаться так называемый «атопический марш» с  развитием у ребенка тяжелых форм аллергических заболеваний. Вместе с тем возможно и  благоприятное течение пищевой аллергии с исчезновением признаков нарушения с  возрастом. Прогноз заболевания зависит от многих факторов, как генетических, так и  средовых, однако ранняя диагностика и адекватное лечение детей с пищевой аллергией в  большинстве случаев позволяют улучшить прогноз формирования толерантности или, как  минимум, добиться легкого течения проявлений пищевой аллергии. С ранними  проявлениями пищевой аллергии в первую очередь сталкиваются педиатры, и четкое  следование клиническим рекомендациям по ведению пациентов позволяет избежать  большинства типичных ошибок. Данная статья адресована практическим врачам: в ней  рассматриваются наиболее частые ошибки педиатров в ведении детей с пищевой аллергией  и обсуждаются прижившиеся «мифы» об этой патологии.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"147 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77323254","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}