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Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi最新文献

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Kawasaki disease. 川崎病。
Pub Date : 2012-06-08 DOI: 10.1542/9781581107357-part03-kawasaki
Teh-Yang Huang
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引用次数: 0
[Pediatric asthma and obesity]. [儿童哮喘和肥胖]。
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引用次数: 0
[Determination of brain death in children]. [儿童脑死亡的测定]。
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引用次数: 0
Current classification and status of primary immunodeficiency diseases in Taiwan. 台湾原发性免疫缺陷疾病之分类现况。
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0003
F. Liang, Yiqiu Wei, T. Jiang, Meng-Ying Hsiehi, Yu‐Chuan Wen, Y. Chiou, Shu-Hua Wu, Li-Chen Chen, Jing-Long Huang, Wen-I. Lee
The incidence of primary immunodeficiency diseases (PIDD) in Taiwan is estimated at 2.17 per 100,000 live births. This is much lower than in Sweden, with 8.4 per 100,000 live births. Patients with critical combined T-cell and B-cell immunodeficiency (CID) seem to be under-diagnosed because of delayed referrals to a tertiary care center which is able to organize a cooperative transplantation team encompassing, at least, a pediatric hematologist and a immunologist for severe combined immunodeficiency (SCID) classified as "pediatric emergency". Moreover, there are rare reported cases of adult-onset (over 18-years-old) common variable immunodeficiency (CVID). These cases are possibly treated as autoimmune diseases, but not PIDD. To date around the world, 206 kinds of PIDD have been found and 110 causal genetic effects were identified. Although epidemiological studies show wide geographical and racial variations in the prevalence and distribution of PIDD, we believe in Taiwan that those patients with Mendelian susceptibility to mycobacteria disease (MSMD), belonging to "congenital phagocyte defect", are often treated as isolated refractory mycobacterial infections or chronic granulomatous disease. Also, "diseases of innate immunity" and "autoimflammatory disorders" are not yet identified. To manage patients with hemophagocytic lymphohisticytosis syndromes, one of "disease of immune dysregulation, stem cell transplantation will be considered if there is poor response to chemotherapy. Patients with PIDD need better access to specialized clinical, laboratory and therapeutic resources.
台湾原发性免疫缺陷疾病(PIDD)的发病率估计为每10万活产2.17例。这比瑞典低得多,瑞典每10万活产婴儿中有8.4人死亡。重症联合t细胞和b细胞免疫缺陷(CID)患者似乎被诊断不足,因为延迟转诊到三级保健中心,三级保健中心能够组织一个合作移植小组,至少包括一名儿科血液学家和一名被归类为“儿科急诊”的严重联合免疫缺陷(SCID)的免疫学家。此外,也有罕见的成人发病(18岁以上)共同变异性免疫缺陷(CVID)的病例报道。这些病例可能被视为自身免疫性疾病,而不是PIDD。迄今为止,全球已发现了206种PIDD,并确定了110种因果遗传效应。虽然流行病学研究显示PIDD的患病率和分布存在广泛的地理和种族差异,但我们认为在台湾,那些属于“先天性吞噬细胞缺陷”的分枝杆菌病孟德尔易感性(MSMD)患者通常被视为孤立的难愈分枝杆菌感染或慢性肉芽肿病。此外,“先天免疫疾病”和“自身炎症性疾病”尚未确定。作为“免疫失调疾病”之一的噬血细胞性淋巴细胞病,如果化疗效果不佳,可以考虑干细胞移植。PIDD患者需要更好地获得专门的临床、实验室和治疗资源。
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引用次数: 11
Fetal alcohol spectrum disorder: report of one case. 胎儿酒精谱系障碍1例报告
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0028
Yi-Chen Huang, Hsin-Yu Lo, W. Chaou
One seven-year-old aboriginal boy visited our outpatient department for survey of study difficulty. The physical examination revealed microcephaly, broad depressed nasal bridge, thin upper lip, smooth philtrum, epicanthal folds and clinodactyly. He also had mild mental retardation and abnormal findings on brain MRI. His mother had confirmed daily alcoholic consumption (72 to 144 gm) during pregnancy. Besides the short stature and microcephaly, the patient had developmental delay in language. According to the history, clinical presentation, and the finding of brain imaging, this case matches the diagnosis of fetal alcohol spectrum disorder. It seems reasonable to consider that some cases with the idiopathic developmental delay may fit in this disorder, thus suggesting the importance of total abstinence from alcohol during pregnancy.
一名七岁原住民男童到我门诊部进行学习困难调查。体格检查显示:小头畸形,鼻梁宽凹陷,上唇薄,中膈平滑,上膈皱褶,趾突。他也有轻微的智力迟钝和脑部核磁共振异常。他的母亲证实在怀孕期间每天饮酒(72至144克)。除了身材矮小和小头畸形外,患者还有语言发育迟缓。根据病史、临床表现和脑成像结果,本病例符合胎儿酒精谱系障碍的诊断。考虑到一些特发性发育迟缓的病例可能符合这种疾病,这似乎是合理的,因此建议在怀孕期间完全戒酒的重要性。
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引用次数: 0
The role of Mycoplasma pneumoniae in acute exacerbation of asthma in children. 肺炎支原体在儿童哮喘急性加重中的作用。
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0014
C. Ou, Yu-Fang Tseng, Y. Chiou, BaoRen Nong, Y. Huang, K. Hsieh
BACKGROUNDThe aim of this retrospective study was to test the connection between acute M. pneumoniae infection and the exacerbation of asthma. The clinical characteristics of Mycoplasma infection seen during emergent visits in asthmatic children were reviewed.METHODSWe examined the cases of 316 asthma exacerbation patients aged from two to fourteen-years-old. They were divided into two groups according to their asthma history. One hundred and eighty-eight cases had evidence of a history of asthma (group 1) and 128 had only had their first asthma attack (group 2). The control group (group 3) was made up of 151 asthmatic children who had no acute exacerbation during the previous six months. In all three groups, we looked whether those children had acute M. pneumoniae infection or not. Acute M. pneumoniae infection was defined by positive results in serologic testing, with specific immunoglobulin M (IgM) antibody or with a > or = fourfold increase in IgG titer by the third week in the same children.RESULTSIn group 1, acute M. pneumionae infection was found in 42 (23%) of the 188 children. In group 2, acute M. pneumoniae infection was proven in 57 (45%) of the 128 children. In the control group, 10 (7%) of the 151 children had M. pneumoniae infection. Twenty-three (54%) asthmatic children that presented with fever as the chief complaint were infected with M. pneumoniae, compared with 18 (12%) children without infection (p = 0.014). Twenty-nine (50%) children having their first asthma attack with fever were infected with M. pneumoniae, compared with 22 (32%) without infection (p = 0.009). In group 1, 17 (41%) children with M. pneumoniae infections and 28 (19%) children without M. pneumoniae infections presented with rale breathing sounds of the physical examination (p = 0.027). In group 2, 26 (46%) children with M. pneumoniae infections and 17 (24%) children without M. pneumoniae infections presented with rale breathing sounds (p = 0.019).CONCLUSIONSWe found that M. pneumoniae may play a role in asthmatic exacerbation among children, especially in those experiencing their first asthma attack. Moreover, among children with acute M. pneumoniae infection, the number was significantly increased of children having fever as the chief complaint and rales in auscultations compared with those without M. pneumoniae infection.
背景:本回顾性研究的目的是检验急性肺炎支原体感染与哮喘恶化之间的联系。本文综述了哮喘患儿急诊时支原体感染的临床特点。方法对316例2 ~ 14岁哮喘加重患者进行分析。根据哮喘病史将患者分为两组。188例有哮喘病史(第一组),128例只有第一次哮喘发作(第二组)。对照组(第三组)由151名哮喘儿童组成,他们在过去6个月内没有急性发作。在这三组中,我们观察了这些儿童是否患有急性肺炎支原体感染。急性肺炎支原体感染的定义是血清学检测结果阳性,具有特异性免疫球蛋白M (IgM)抗体,或者在同一儿童中,IgG滴度在第三周增加>或= 4倍。结果1组188例患儿中有42例(23%)出现急性肺炎支原体感染。2组128例患儿中有57例(45%)确诊急性肺炎支原体感染。对照组151例患儿中有10例(7%)发生肺炎支原体感染。以发热为主诉的哮喘患儿感染肺炎支原体23例(54%),未感染的患儿18例(12%)(p = 0.014)。29例(50%)首次哮喘发作伴有发热的儿童感染肺炎支原体,22例(32%)未感染(p = 0.009)。第1组肺炎支原体感染患儿17例(41%),非肺炎支原体感染患儿28例(19%)体检时出现呼吸音重(p = 0.027)。第2组肺炎支原体感染患儿26例(46%),非肺炎支原体感染患儿17例(24%)出现呼吸音重(p = 0.019)。结论:我们发现肺炎支原体可能在儿童哮喘加重中起作用,特别是在首次哮喘发作的儿童中。在急性肺炎支原体感染患儿中,以发热为主诉和听诊啰音的患儿数量明显高于非肺炎支原体感染患儿。
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引用次数: 19
Relationship between umbilical cord blood insulin-like growth factors and anthropometry in term newborns. 足月新生儿脐带血胰岛素样生长因子与人体测量的关系。
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0019
T. Hung, Chin-Chuan Lin, Yea-Shwu Hwang, Shio‐Jean Lin, Yen-Yin Chou, Wen-Hui Tsai
BACKGROUNDBirth size is associated with long-term morbidity. Insulin-like growth factor (IGF) system is the most important endocrine factor influencing fetal growth. During rapid somatic growth, free-to-total IGF-I ratio is increased, resulting in higher IGF-I bioavailability. The purpose of this study was to investigate the association of free-to-total IGF-I ratios, IGF-II, and IGF-binding protein (IGFBP)-3 umbilical cord levels with anthropometric data of term neonates.METHODSUmbilical venous plasma samples were obtained from 95 term neonates and analyzed by enzyme-linked immunosorbent assay.RESULTSThe large-for-gestational age (LGA) neonates had higher free IGF-I, total IGF-I, and IGFBP-3 levels than small-for-gestational age (SGA) neonates (P < 0.01, 0.001, 0.01, respectively) and higher total IGF-I and IGFBP-3 levels than appropriate-for-gestational age (AGA) neonates (P < 0.05, 0.01, respectively). The free-to-total IGF-I ratios and IGF-II levels were not different among SGA, AGA, and LGA neonates. Free IGF-I, total IGF-I, and IGFBP-3 levels were positively correlated with birth weight (r = 0.34, P < 0.001; r = 0.41, P < 0.001; r = 0.25, P < 0.05, respectively). Multiple linear regression analyses revealed that only total IGF-I levels was the independent predictive variable for birth weight.CONCLUSIONSOur data suggest total IGF-I is the most important factor in the IGF system for determining fetal growth, at least near term gestation. Free-to-total IGF-I ratios may mostly be determined by total IGF-I. If birth size is associated with adult chronic metabolic diseases, total IGF-I may be involved in the pathogenesis.
出生大小与长期发病率相关。胰岛素样生长因子(IGF)系统是影响胎儿生长的最重要的内分泌因子。在体细胞快速生长过程中,游离-总IGF-I比例增加,导致IGF-I的生物利用度提高。本研究的目的是研究足月新生儿脐带游离总IGF-I比率、IGF-II和igf结合蛋白(IGFBP)-3水平与人体测量数据的关系。方法采用酶联免疫吸附法对95例足月新生儿脐静脉血浆进行分析。结果大胎龄(LGA)新生儿游离IGF-I、总IGF-I和IGFBP-3水平高于小胎龄(SGA)新生儿(P分别< 0.01、0.001和0.01),总IGF-I和IGFBP-3水平高于适胎龄(AGA)新生儿(P分别< 0.05、0.01)。SGA、AGA和LGA新生儿的游离总IGF-I比率和IGF-II水平没有差异。游离IGF-I、总IGF-I和IGFBP-3水平与出生体重呈正相关(r = 0.34, P < 0.001;r = 0.41, P < 0.001;r = 0.25, P < 0.05)。多元线性回归分析显示,只有总IGF-I水平是出生体重的独立预测变量。结论总的IGF- 1是IGF系统中决定胎儿生长的最重要因素,至少在妊娠早期是如此。游离-总IGF-I比率可能主要由总IGF-I决定。如果出生尺寸与成人慢性代谢性疾病相关,igf - 1总量可能参与其发病机制。
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引用次数: 9
Non-ketotic hyperglycinemia with a novel GLDC mutation in a Taiwanese child. 台湾儿童非酮症性高糖血症伴GLDC突变。
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0035
Chia-Ying Chang, Shuan-Pei Lin, Hsiang-Yu Lin, C. Chuang, Che-Sheng Ho, Chyong-hsin Hsu
We report a newborn boy with the classic neonatal form of non-ketotic hyperglycinemia (NKH). He had a typical presentation of frequent hiccups and myoclonic movements since birth. Genetic analysis demonstrated a mutant allele with a single substitution at nucleotide 1111 of exon 8 (c. 1111 C > G) in the GLDC gene inherited from his mother, resulting in a histidine-to-aspartic acid change at amino acid position 371 (p. His371Asp mutation) in the gene product. The other allele of the GLDC gene was deleted, a mutation inherited from the father.
我们报告一个新生儿与典型的新生儿形式的非酮症高血糖症(NKH)。他自出生以来就有频繁打嗝和肌阵挛性运动的典型表现。遗传分析表明,遗传自其母亲的GLDC基因的第8外显子(c. 1111 c > G)的核苷酸1111处突变等位基因导致基因产物中371个氨基酸位置的组氨酸变为天氨酸(p. His371Asp突变)。GLDC基因的另一个等位基因被删除,这是遗传自父亲的突变。
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引用次数: 3
Facial palsy in Kawasaki disease: report of two cases. 川崎病并发面瘫2例报告。
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0024
Sung-Tse Li, N. Chiu, Che-Sheng Ho, Ming-Ren Chen
Facial palsy is an unusual complication associated with Kawasaki disease, with only a few published case reports. We report two patients with typical Kawasaki disease and facial palsy. Both had coronary artery aneurysms and were treated with intravenous immunoglobulin. The facial palsy resolved completely over the next several months in both patients. Coronary artery aneurysms resolved completely in one patient, and the other has not regressed to normal till now. The incidence of coronary artery aneurysm appears to be higher in the handful of reported cases of Kawasaki disease with facial palsy.
面瘫是一种罕见的并发症与川崎病,只有少数发表的病例报告。我们报告2例典型川崎病伴面瘫患者。两人都有冠状动脉瘤,并接受静脉注射免疫球蛋白治疗。在接下来的几个月里,两名患者的面瘫完全消失了。1例患者冠状动脉瘤完全消退,另1例至今未恢复正常。在少数报道的川崎病伴面瘫病例中,冠状动脉动脉瘤的发生率似乎更高。
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引用次数: 6
Microbial contamination of the Tzu-Chi Cord Blood Bank from 2005 to 2006. 子池脐带血库2005年至2006年微生物污染情况。
Pub Date : 2008-02-01 DOI: 10.7097/APT.200802.0009
Shu‐Huey Chen, Ya-Jun Zheng, Shang-hsien Yang, Kuo‐Liang Yang, M. Shyr, Y. Ho
BACKGROUNDIn total, 4502 units of cord blood (CB) were collected during a 2-year period from 2005 to 2006 by the Buddhist Tzu-Chi Stem Cells Center. The aim of this study was to analyze the incidence of microbial contamination and type of organism present in the cord blood. The clinical impact of microbial contamination on hematopoietic progenitor cell (HPC) grafts used for HPC transplantation is also discussed.METHODSFirst and second specimens were obtained for microbial assessment. These were collected in laboratory after cord blood collection and after cord blood unit manipulation, respectively. The samples were cultured and the results reviewed.RESULTSThe overall incidence of microbiological contamination was 1.8% (82/4502). Three CB units were contaminated with two different organisms. Infectious organisms comprised 9.4% (8/85) of total isolated microbes. These infectious microorganisms were beta-Streptococci group B, Candida tropicalis and Staphylococcus aureus which were isolated in 6, 1 and 1 of CB units respectively. Escherichia coli, Bacteroides fragilis, Lactobacillus spp., Enterococcus, beta-Streptococcus Group B, Bacteroides valgatus, Corynebacterium spp., Klebsiella pneumonia and Peptococcus spp. were the most frequently encountered microorganisms. A higher contamination rate of the CB units was noted after vaginal delivery (2.16%) compared to caesarian section (0.85%) (p < 0.01).CONCLUSIONSExtensive training in CB collection, good procedures and good protocols can decrease the rate of microbial contamination. The use of a closed collecting system and an ex utero method have the advantage of a lower contamination rate. In our cord blood bank, we use a closed system but an in utero method. Similar to other studies, most of microorganisms reported here as contaminants are non-pathogenic.
在2005年至2006年的2年间,佛教慈济干细胞中心共收集了4502单位脐带血(CB)。本研究的目的是分析脐带血中微生物污染的发生率和存在的生物体类型。本文还讨论了微生物污染对用于造血祖细胞移植的移植物的临床影响。方法采集第1、2份标本进行微生物鉴定。这些分别在脐带血采集和脐带血单位操作后在实验室收集。对样品进行了培养,并对结果进行了审查。结果总微生物污染发生率为1.8%(82/4502)。三个CB单位被两种不同的生物污染。感染菌占分离微生物总数的9.4%(8/85)。感染微生物分别为β - B群链球菌、热带念珠菌和金黄色葡萄球菌,分别在6、1和1个CB单位中分离得到。大肠杆菌、脆弱拟杆菌、乳杆菌、肠球菌、乙型链球菌、变形拟杆菌、棒状杆菌、肺炎克雷伯菌和胃球菌是最常见的微生物。阴道分娩后的CB单位污染率(2.16%)高于剖宫产(0.85%)(p < 0.01)。结论加强CB收集培训,完善流程和方案,可降低微生物污染发生率。使用封闭收集系统和体外方法具有污染率较低的优点。在我们的脐带血银行,我们使用一个封闭的系统,但在子宫内的方法。与其他研究类似,这里报道的大多数作为污染物的微生物是非致病性的。
{"title":"Microbial contamination of the Tzu-Chi Cord Blood Bank from 2005 to 2006.","authors":"Shu‐Huey Chen, Ya-Jun Zheng, Shang-hsien Yang, Kuo‐Liang Yang, M. Shyr, Y. Ho","doi":"10.7097/APT.200802.0009","DOIUrl":"https://doi.org/10.7097/APT.200802.0009","url":null,"abstract":"BACKGROUND\u0000In total, 4502 units of cord blood (CB) were collected during a 2-year period from 2005 to 2006 by the Buddhist Tzu-Chi Stem Cells Center. The aim of this study was to analyze the incidence of microbial contamination and type of organism present in the cord blood. The clinical impact of microbial contamination on hematopoietic progenitor cell (HPC) grafts used for HPC transplantation is also discussed.\u0000\u0000\u0000METHODS\u0000First and second specimens were obtained for microbial assessment. These were collected in laboratory after cord blood collection and after cord blood unit manipulation, respectively. The samples were cultured and the results reviewed.\u0000\u0000\u0000RESULTS\u0000The overall incidence of microbiological contamination was 1.8% (82/4502). Three CB units were contaminated with two different organisms. Infectious organisms comprised 9.4% (8/85) of total isolated microbes. These infectious microorganisms were beta-Streptococci group B, Candida tropicalis and Staphylococcus aureus which were isolated in 6, 1 and 1 of CB units respectively. Escherichia coli, Bacteroides fragilis, Lactobacillus spp., Enterococcus, beta-Streptococcus Group B, Bacteroides valgatus, Corynebacterium spp., Klebsiella pneumonia and Peptococcus spp. were the most frequently encountered microorganisms. A higher contamination rate of the CB units was noted after vaginal delivery (2.16%) compared to caesarian section (0.85%) (p < 0.01).\u0000\u0000\u0000CONCLUSIONS\u0000Extensive training in CB collection, good procedures and good protocols can decrease the rate of microbial contamination. The use of a closed collecting system and an ex utero method have the advantage of a lower contamination rate. In our cord blood bank, we use a closed system but an in utero method. Similar to other studies, most of microorganisms reported here as contaminants are non-pathogenic.","PeriodicalId":7156,"journal":{"name":"Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2008-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73586243","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: 10
期刊
Acta paediatrica Taiwanica = Taiwan er ke yi xue hui za zhi
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