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Как не пропустить мягкие формы мукополисахаридоза I типа у пациентов с суставными проявлениями заболевания 如何避免在关节型疾病患者中错过一种软性的多糖剂量I ?
Pub Date : 2019-01-31 DOI: 10.15690/vsp.v17i6.1978
Н. В. Бучинская, Михаил Михайлович Костик, О. Л. Колобова, Л. Н. Мельникова
Mucopolysaccharidosis type I (MPS I) is a hereditary metabolic disease that manifests itself in childhood by systemic damage to tissues and organs, a constantly progressive course leading to disability. Diagnosis of mild forms of the disease is particularly difficult due to the absence of specific symptoms. A specific symptom of the mild forms of MPS I (as for other types of MPS) is joint stiffness in children combined with hernia, frequent infections, or valvular defects. Stiffness in MPS I is often interpreted as a manifestation of rheumatological diseases (arthrogriposis, juvenile idiopathic arthritis). The article offers a simple algorithm for diagnosing MPS I, which helps to eliminate the disease using a simple test for determining the activity of an enzyme called alpha-L-iduronidase in a dried blood spot.
粘多糖病I型(MPS I)是一种遗传性代谢性疾病,表现为儿童期组织和器官的全身性损害,其病程不断进展,最终导致残疾。由于没有特定的症状,诊断这种疾病的轻度形式特别困难。轻度型MPS I(与其他类型的MPS一样)的一个特定症状是儿童关节僵硬并伴有疝、频繁感染或瓣膜缺损。MPS I的僵硬常被解释为风湿病的表现(关节关节炎、幼年特发性关节炎)。这篇文章提供了一种简单的诊断MPS I的算法,通过一种简单的测试来确定一种叫做α - l -伊杜醛酸酶的酶在干燥血斑中的活性,这种方法有助于消除这种疾病。
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引用次数: 3
Голимумаб в лечении хронических воспалительных заболеваний суставов во взрослой популяции: клиническая эффективность и переносимость golimomab治疗成人关节慢性炎症疾病:临床效能和耐受性
Pub Date : 2019-01-30 DOI: 10.15690/vsp.v17i6.1975
Н. В. Чичасова
Due to the recent introduction of golimumab into paediatric rheumatology practice, an overview of the clinical studies of this tumour necrosis factor alpha inhibitor, most of which were conducted with adult patients with rheumatic diseases, has been presented. Clinical laboratory effects and tolerability of golimumab in the form of subcutaneous injections have been analysed for rheumatoid arthritis, psoriatic arthritis, and spondylitis. Evaluation of the efficacy and tolerability of golimumab in long-term observational studies (up to 5 years) has been discussed.
由于最近将golimumab引入儿科风湿病实践,对这种肿瘤坏死因子α抑制剂的临床研究进行了概述,其中大多数是针对患有风湿病的成人患者进行的。临床实验室效果和皮下注射形式的golimumab的耐受性已分析类风湿关节炎,银屑病关节炎和脊柱炎。在长期观察性研究(长达5年)中评估golimumab的疗效和耐受性已经进行了讨论。
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引用次数: 0
Младенческая смертность вне лечебных учреждений и пути ее снижения 在治疗机构之外的新生儿死亡率及其下降方式
Pub Date : 2019-01-30 DOI: 10.15690/VSP.V17I6.1973
Д. С. Крючко, Ирина Ивановна Рюмина, В В Челышева, Е. В. Соколова, Е. Н. Байбарина
Infant mortality is one of the key indicators of demography, characterizing not only the state of health and a social standard of living in a country, region, city, but also an indicator of the level of state development. In recent years there has been a steady downward trend in infant mortality. To the greatest extent, this trend is driven by improved quality of medical care. However, death of children outside healthcare facilities is the least controlled in the structure of infant mortality. The article considers the main causes of deaths of children under the age of one year outside healthcare facilities in the Russian Federation in 2017, presents data of the world and national statistics, analyses possible solutions to the problem.
婴儿死亡率是人口统计的关键指标之一,不仅反映了一个国家、地区、城市的健康状况和社会生活水平,而且也是国家发展水平的指标。近年来,婴儿死亡率呈稳步下降趋势。在很大程度上,这一趋势是由医疗保健质量的提高所推动的。然而,在婴儿死亡率的结构中,医疗机构以外的儿童死亡是控制最少的。本文考虑了2017年俄罗斯联邦一岁以下儿童在医疗机构外死亡的主要原因,介绍了世界和国家统计数据,分析了该问题的可能解决方案。
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引用次数: 5
О перспективах научных исследований в области профилактики детской инвалидности
Pub Date : 2019-01-30 DOI: 10.15690/VSP.V17I6.1972
Александр Александрович Баранов, Р. Н. Терлецкая
The presented review of the special literature data (on MEDLINE and eLIBRARY.RU databases) makes it possible to determine a number of topical issues for preventing child disability as well as trends of scientific research in this area, taking into account the causes of disability and its structure by the underlying disease. The World Health Organization has established a relentless focus on the problem of disability, including that of children, which is reflected in the continuous improvement of methods for assessing and classifying persistent disabilities. It has been shown that the prevention of disability in children and the support of families raising handicapped children and children with disabilities are among the main priorities of the state social policy of the Russian Federation. The data on modern technologies for reducing the genetic burden in the population from the perspective of prevention of hereditary and congenital diseases, including orphan ones, has been presented. The results of studies indicating the increasing role of the intestinal microbiota in the development and prevention of a number of diseases affecting the formation of disability in children have been presented. The problem of combating antibiotic resistance is considered from a preventive perspective. A number of scientific studies is devoted to non-infectious pathology, which is becoming increasingly important in the formation of disability in children of different age groups, starting from the neonatal period. The actual data on the role of obesity in the formation of serious health disorders has been given. The opinion is expressed on the need to create a system of hygienic and medico-psychological safety of children's life in conditions of hyperinformatisation and the modern environmental situation. It is assumed that the implementation of the results of the proposed research into health care practice will make it possible to influence the processes of disability in a child on a deeper pathogenetic level as well as to improve the arrangement of preventive activities in this direction.
对MEDLINE和图书馆的特殊文献资料进行综述。RU数据库)使得能够确定预防儿童残疾的一些专题问题以及这一领域的科学研究趋势,同时考虑到残疾的原因及其由潜在疾病构成的结构。世界卫生组织坚持不懈地关注残疾问题,包括儿童残疾问题,这反映在持续改进评估和分类持续性残疾的方法上。事实表明,预防儿童残疾和支助抚养残疾儿童和残疾儿童的家庭是俄罗斯联邦国家社会政策的主要优先事项之一。从预防遗传病和先天性疾病,包括孤儿病的角度,介绍了用于减轻人口遗传负担的现代技术的数据。研究结果表明,肠道微生物群在影响儿童残疾形成的一些疾病的发展和预防中发挥着越来越大的作用。从预防的角度来考虑对抗抗生素耐药性的问题。一些科学研究致力于非传染性病理学,这在从新生儿期开始的不同年龄组儿童残疾的形成中变得越来越重要。关于肥胖在形成严重健康失调方面的作用的实际数据已经给出。有人认为,在高度信息化和现代环境条件下,有必要建立儿童生活卫生和医疗心理安全体系。人们认为,将拟议的研究结果落实到保健实践中,将有可能在更深的病理层面上影响儿童的残疾过程,并改善这方面预防活动的安排。
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引用次数: 2
Пациент в педиатрии: ребенок, взрослый или оба? 儿科病人:婴儿、成人还是两者兼而有之?
Pub Date : 2018-12-02 DOI: 10.15690/vsp.v17i5.1958
В. П. Чемеков, А. В. Шашелева
The paediatric patient is considered to be a child. However, when it comes to communication with a patient, his consent, implementation of appointments,  then the party of interaction is supposed to be an adult. In this regard, the paediatric patient is represented  by a child-adult alliance, which allows us to speak of a ‘complex patient’. At the same time, his personal agency (the ability to independently formulate a complaint, accept appointments  and achieve compliance) changes with the age of a child, coming to him from an adult. This determines the efficacy of screening as well as the main tactics of interaction between the doctor and the patient, explanation, and compliance. Based on the theory of periodization of the personality development, answers are given to the questions: who, a child or an adult, formulates a complaint, who accepts the explanation and who is the subject to compliance? It has been shown that in early childhood (up to 3 years) the patient's subjectivity in the formulation of a complaint and a picture of the disease, the perception of prescriptions  and  adherence  to compliance  is provided  by the parent.  During  the pre-school  (3–7  years)  and  primary  school (7–11 years) periods, the picture is mosaic: the doctor compares the opinion of the parent and his child to get a picture of the disease, he gives prescriptions  to the parent and/or to the child, so both of them can be a subject to compliance. And only in adolescence (12–17 years) a child can be almost a completely independent subject in all aspects of interaction in the doctor-patient  system.
该儿科患者被视为儿童。然而,当涉及到与病人的沟通,他的同意,约定的执行,那么互动的一方应该是一个成年人。在这方面,儿科患者由儿童-成人联盟代表,这使我们能够谈论“复杂患者”。与此同时,他的个人代理(独立制定投诉、接受预约和实现合规的能力)随着孩子的年龄而变化,从成年人变成了他。这决定了筛查的有效性,以及医患互动、解释和依从性的主要策略。以人格发展分期理论为基础,对儿童和成人谁提出抱怨、谁接受解释、谁是服从主体等问题进行了回答。研究表明,在幼儿期(3岁以下),病人主观性地提出申诉和疾病情况,对处方的看法和遵守规定是由父母提供的。在学前班(3-7岁)和小学(7-11岁)期间,情况是马赛克的:医生比较父母和孩子的意见,以了解疾病的情况,他给父母和/或孩子开处方,使他们都能遵守。只有在青春期(12-17岁),一个孩子才能在医患互动系统的各个方面成为几乎完全独立的主体。
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引用次数: 3
Распространенность и клиниколабораторные особенности криптоспоридиоза у детей в возрасте до 5 лет: одномоментное исследование госпитальных случаев острой кишечной инфекции 5岁以下儿童的隐孢子菌病的常见和临床特征:对急性肠感染的医院病例立即进行研究
Pub Date : 2018-10-04 DOI: 10.15690/VSP.V17I4.1925
Е. Г. Старикова, Н. И. Шубина, Ольга Владимировна Воронкова, Ю. В. Ковширина, Н Д Яровой
Background. Cryptosporidium protozoa are the leading causative agent of diarrhea and cause of death in children under 5 years of age. The role of cryptosporidia in the development and course of acute intestinal infections (AII) in children in Russia remains unstudied. Objective. Our aim was to study the prevalence and clinical laboratory features of cryptosporidium-associated aII in children under 5 years of age. Methods. A cross-sectional study (conducted in March-June 2017) included children admitted to hospital with symptoms of AII (fever, loose watery stools, weakness, decreased appetite and/or vomiting) by the ambulance service. On admission, stool samples were collected from all patients. Cryptosporidium oocysts were determined by microscopic examination of faecal smears stained according to Tsil-Nielsen after preliminary concentration by a modified formalin-ether technique. The presence of intestinal pathogens was determined by a bacteriological technique and using a polymerase chain reaction. Results. The study included 107 children with AII (girls — 51%). Cryptosporidia were detected in 28 (26%) patients, in 93% of cases — together with bacterial and/or viral pathogens. The etiological structure of cryptosporidium-associated AII and AII in cryptosporidiosis negative children (n = 79) did not differ. On admission, children with cryptosporidium-associated AII had a higher blood leukocyte count — 13.0_109/L (9.2; 16.0) versus 8.3_109/L (6.1; 11.2) in children without cryptosporidiosis (p < 0.001). It has been also found that antibiotics were more often used in the treatment of children with cryptosporidium-associated AII — in 21 (75%) versus 39 (49%) in the comparison group (p = 0.026). Conclusion. Cryptosporidia are detected in every fourth child with AII under 5 years of age. Patients with cryptosporidia are distinguished by a higher level of blood leukocytes upon admission and a more frequent prescription of antibiotics than in the group of cryptosporidiosis negative patients.
背景。隐孢子虫是5岁以下儿童腹泻和死亡的主要病原体。隐孢子虫在俄罗斯儿童急性肠道感染(AII)的发展和过程中的作用仍未研究。目标。我们的目的是研究5岁以下儿童隐孢子虫相关aII的患病率和临床实验室特征。方法。一项横断面研究(于2017年3月至6月进行)纳入了救护车服务因AII症状(发烧、稀便、虚弱、食欲下降和/或呕吐)入院的儿童。入院时,收集所有患者的粪便样本。采用改良的福尔马林乙醚技术进行初步浓度测定后,用Tsil-Nielsen染色的粪便涂片显微镜检查隐孢子虫卵囊。肠道病原体的存在是通过细菌学技术和使用聚合酶链反应确定的。结果。该研究包括107名患有AII的儿童(女孩占51%)。在28例(26%)患者中检测到隐孢子虫,在93%的病例中-连同细菌和/或病毒病原体。隐孢子虫病阴性儿童(n = 79)隐孢子虫相关AII和AII的病因结构无差异。入院时,隐孢子虫相关AII患儿的血白细胞计数较高,为13.0 - 109/L (9.2;16.0) vs . 8.3 - 109/L (6.1;11.2),无隐孢子虫病患儿(p < 0.001)。研究还发现,抗生素更常用于治疗隐孢子虫相关AII患儿,对照组中有21例(75%)使用抗生素治疗,对照组中有39例(49%)使用抗生素治疗(p = 0.026)。结论。在5岁以下全呼吸道感染儿童中,每四名儿童中检出隐孢子虫。与隐孢子虫病阴性患者相比,隐孢子虫患者入院时血液白细胞水平较高,抗生素处方更频繁。
{"title":"Распространенность и клиниколабораторные особенности криптоспоридиоза у детей в возрасте до 5 лет: одномоментное исследование госпитальных случаев острой кишечной инфекции","authors":"Е. Г. Старикова, Н. И. Шубина, Ольга Владимировна Воронкова, Ю. В. Ковширина, Н Д Яровой","doi":"10.15690/VSP.V17I4.1925","DOIUrl":"https://doi.org/10.15690/VSP.V17I4.1925","url":null,"abstract":"Background. Cryptosporidium protozoa are the leading causative agent of diarrhea and cause of death in children under 5 years of age. The role of cryptosporidia in the development and course of acute intestinal infections (AII) in children in Russia remains unstudied. Objective. Our aim was to study the prevalence and clinical laboratory features of cryptosporidium-associated aII in children under 5 years of age. Methods. A cross-sectional study (conducted in March-June 2017) included children admitted to hospital with symptoms of AII (fever, loose watery stools, weakness, decreased appetite and/or vomiting) by the ambulance service. On admission, stool samples were collected from all patients. Cryptosporidium oocysts were determined by microscopic examination of faecal smears stained according to Tsil-Nielsen after preliminary concentration by a modified formalin-ether technique. The presence of intestinal pathogens was determined by a bacteriological technique and using a polymerase chain reaction. Results. The study included 107 children with AII (girls — 51%). Cryptosporidia were detected in 28 (26%) patients, in 93% of cases — together with bacterial and/or viral pathogens. The etiological structure of cryptosporidium-associated AII and AII in cryptosporidiosis negative children (n = 79) did not differ. On admission, children with cryptosporidium-associated AII had a higher blood leukocyte count — 13.0_109/L (9.2; 16.0) versus 8.3_109/L (6.1; 11.2) in children without cryptosporidiosis (p < 0.001). It has been also found that antibiotics were more often used in the treatment of children with cryptosporidium-associated AII — in 21 (75%) versus 39 (49%) in the comparison group (p = 0.026). Conclusion. Cryptosporidia are detected in every fourth child with AII under 5 years of age. Patients with cryptosporidia are distinguished by a higher level of blood leukocytes upon admission and a more frequent prescription of antibiotics than in the group of cryptosporidiosis negative patients.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"31 1","pages":"316-321"},"PeriodicalIF":0.0,"publicationDate":"2018-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82802833","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}
引用次数: 1
Роль железа в патогенезе расстройств аутистического спектра у детей
Pub Date : 2018-10-04 DOI: 10.15690/VSP.V17I4.1920
Ольга Васильевна Костина
The review presents an analysis of the mechanisms of iron effect on the brain development. The importance of iron deficiency in the perinatal period is considered as a risk factor for the development of neuropsychiatric disorders in children with autism spectrum disorders (ASDs). Possible causes of sideropenia are discussed; data on haematological and biochemical parameters characterizing iron metabolism in children with ASDs are presented. The demand for studying the role of iron metabolism imbalance in the development of neuropsychiatric disorders in order to clarify pathogenetic mechanisms of ASDs and to determine methods for their correction is emphasized.
本文就铁对大脑发育的影响机制作一综述。围生期缺铁的重要性被认为是自闭症谱系障碍(asd)儿童神经精神障碍发展的一个危险因素。讨论了铁缺乏症的可能原因;介绍了表征asd儿童铁代谢的血液学和生化参数数据。强调需要研究铁代谢失衡在神经精神疾病发展中的作用,以阐明asd的发病机制和确定其纠正方法。
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引用次数: 1
Результаты лечения гиардиаза у детей с применением противолямблиозных препаратов и их комбинации с пробиотиками: ретроспективное когортное исследование 使用抗组胺药及其与益生菌结合的儿童患有黄疸病的结果:回溯步兵研究
Pub Date : 2018-10-04 DOI: 10.15690/VSP.V17I4.1923
О. г. Кимирилова, Г. А. Харченко
Background. Giardiasis in children remains an urgent problem, the significance of which is determined by the endemicity for many countries and regions, outbreaks of sporadic epidemics, polymorphism of clinical symptoms, and insufficient treatment efficacy. Objective. Our aim was to study the results of giardiasis treatment using giardicidal drugs or their combination with probiotics in children. Methods. We analyzed the frequency of Giardia lamblia elimination (the main outcome) as a result of giardiasis treatment (laboratory confirmed) in children aged from 3 months to 18 years who received giardicidal drugs (nifuratel, albendazole) or their combination with probiotics based on Saccharomyces boulardii (probiotic 1) and live freeze-dried lactic acid bacteria Lactobacillus acidophilus (L. gasseri), Bifidobacterium infantis, and Enterococcus faecium (probiotic 2) in outpatient or inpatient settings. Additionally, we registered the duration of the main symptoms of giardiasis (abdominal pain, diarrhea), the prevalence of intestinal dysbiosis and lactase deficiency. The considering period is from January 2015 to September 2017. Results. The results of giardiasis treatment were studied in 4 groups: monotherapy with nifuratel (n = 65) or albendazole (n = 64), a combination of nifuratel + probiotic 1 (n = 67) or albendazole + probiotic 2 (n = 64). The groups were comparable by sex, age, and clinical manifestations of the disease. The elimination of lamblia on the 14–16th day of monotherapy with giardicidal drugs was achieved in 56–60%, when combined with probiotics — in 84% of patients for each combination (df = 3, p < 0.001). Against the background of combination therapy, the disease symptoms (abdominal pain, diarrhea, vomiting) were reversed 1.5 times faster; the number of patients with lactase deficiency and intestinal dysbiosis decreased two and more times, under monotherapy with giardicidal drugs — 1.2 times (df = 3, p < 0.001). Conclusion. Giardiasis treatment in children using combination therapy, including giardicidal drugs and probiotics, is more effective than monotherapy with giardicidal drugs.
背景。儿童贾第虫病仍然是一个紧迫的问题,其重要性取决于许多国家和地区的地方性流行、零星流行的暴发、临床症状的多态性以及治疗效果不足。目标。我们的目的是研究贾第虫药物或其与益生菌联合治疗儿童贾第虫病的结果。方法。我们分析了3个月至18岁的儿童在接受贾第虫治疗(实验室确诊)后,贾第虫消除(主要结果)的频率,这些儿童接受贾第虫药物(尼夫拉特、阿苯达唑)或它们与基于博拉氏酵母菌(益生菌1)和活的冻干乳酸菌嗜酸乳杆菌(L. gasseri)、婴儿双歧杆菌、和肠球菌(益生菌2)在门诊或住院设置。此外,我们记录了贾第虫病主要症状(腹痛、腹泻)的持续时间、肠道生态失调和乳糖酶缺乏症的患病率。考虑期为2015年1月至2017年9月。结果。研究贾第虫病的治疗结果分为4组:尼夫拉特(n = 65)或阿苯达唑(n = 64),尼夫拉特+益生菌1 (n = 67)或阿苯达唑+益生菌2 (n = 64)。两组在性别、年龄和疾病的临床表现方面具有可比性。在单药治疗的14 - 16天,56-60%的患者可消除兰螺旋体,而在联合益生菌治疗的情况下,每种联合治疗的84%的患者可消除兰螺旋体(df = 3, p < 0.001)。在联合治疗的背景下,疾病症状(腹痛、腹泻、呕吐)逆转速度快1.5倍;乳糖酶缺乏和肠道生态失调的患者数量减少了2倍以上,单药治疗组减少了1.2倍(df = 3, p < 0.001)。结论。使用包括鞭毛虫药物和益生菌在内的联合疗法治疗儿童贾第虫病比使用鞭毛虫药物的单一疗法更有效。
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引用次数: 0
ЗНАЧЕНИЕ ДЕТСКО-РОДИТЕЛЬСКОГО ВЗАИМОДЕЙСТВИЯ КАК ФАКТОРА РИСКА АТИПИЧНОГО РАЗВИТИЯ ДЕТЕЙ С РАССТРОЙСТВАМИ АУТИСТИЧЕСКОГО СПЕКТРА 儿童父母互动作为自闭症频谱障碍儿童非典型发育风险因素的重要性
Pub Date : 2018-07-17 DOI: 10.15690/VSP.V17I3.1887
М. А. Лаврова, Ольга Александровна Львова, Людмила Валерьевна Токарская, З. С. Лазаускене
The findings of investigations published between 2000 and 2016 on the study of parental interaction with children with a risk of autism spectrum disorders (ASD) were analyzed. The article demonstrates that early parent-child interaction in such families has distinctive features and can be one of the significant factors of both the risk of ASD development in children at an early age and the compensation of their manifestations. At the same time, such characteristics of the parent-child interaction as maternal sensitivity (the ability to perceive and accurately interpret the child's attachment signals and be able to respond promptly and adequately to these signals) and synchronism (the coordination of movements, actions and other time-dependent indicators; joint attention) are not early markers of ASD development in a child. The authors noted that the issues of parent-child interaction are rarely studied, and the survey samples are limited.
对2000年至2016年间发表的关于父母与自闭症谱系障碍(ASD)风险儿童互动的研究结果进行了分析。本文表明,此类家庭的早期亲子互动具有鲜明的特点,可能是儿童早期ASD发展风险和表现补偿的重要因素之一。与此同时,亲子互动的特征包括母亲的敏感性(能够感知和准确解释孩子的依恋信号,并能够迅速和充分地对这些信号作出反应)和同步性(动作、动作和其他时间依赖性指标的协调);共同注意)并不是儿童ASD发展的早期标志。作者指出,亲子互动问题的研究很少,调查样本有限。
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引用次数: 0
МЕД ОТ КАШЛЯ
Pub Date : 2018-05-23 DOI: 10.15690/vsp.v17i2.1885
В. К. Таточенко
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引用次数: 0
期刊
Current Paediatrics
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