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Respiratory system abnormalities in Prader-Willi syndrome: a literature review Prader-Willi综合征呼吸系统异常:文献综述
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-102
G. Com, Haripriya Santhanam, Berrin Ergun-Longmire
Pediatric Pulmonology Medicine, University of Florida, Pensacola, FL, USA; Department of Pediatric and Adolescent Medicine, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, MI, USA Contributions: (I) Conception and design: All authors (II) Administrative support: none; (III) Provision of study materials or patients: None; (IV) Collection and assembly of data: All authors; (V) Data analysis and interpretation: All authors; (VI) Manuscript writing: All authors; (VII) Final Approval of manuscript: All authors. Correspondence to: Berrin Ergun-Longmire, MD. Department of Pediatric and Adolescent Medicine, Western Michigan University Homer Stryker M.D. School of Medicine, 1000 Oakland Drive, Kalamazoo, MI 49008, USA. Email: berrin.ergun-longmire@med.wmich.edu.
美国佛罗里达州彭萨科拉佛罗里达大学儿科肺病医学;美国密歇根州卡拉马祖市西密歇根大学霍默-史崔克医学院儿童和青少年医学系贡献:(I)构思和设计:所有作者(II)行政支持:无;(III) 提供研究材料或患者:无;(IV) 数据收集和汇编:所有作者;(V) 数据分析和解释:所有作者;(VI) 手稿写作:所有作者;(VII) 手稿的最终批准:所有作者。通信地址:Berrin Ergun Longmire,医学博士,西密歇根大学儿童和青少年医学系,Homer Stryker医学院,1000 Oakland Drive,Kalamazoo,MI 49008,USA电子邮件:berrin.ergun-longmire@med.wmich.edu.
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引用次数: 0
The impact of COVID-19 on the practice of pediatric cardiology: a narrative review COVID-19对儿科心脏病学实践的影响:叙述性回顾
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-64
C. Chen, R. Grignani, Y. Lim, S. Quek
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引用次数: 0
MIS-C is likely to be distinct from Kawasaki disease based on current studies: a narrative review 根据目前的研究,misc可能与川崎病不同:一篇叙述性综述
Pub Date : 2021-01-01 DOI: 10.21037/pm-20-113
Chaozhong Tan, Fang Liu
Background and Objective: Children with SARS-CoV-2 infection were paid little attention to during the early stages of the outbreak because of low morbidity as well as mild clinical symptoms. Since late April 2020, reports regarding Kawasaki-like syndrome and hyperinflammatory response in children associated with COVID-19 have rapidly emerged. Till now, no certain relationship between multisystem inflammatory syndrome in Children (MIS-C) and Kawasaki Disease (KD) has been determined, which should be explored through continuous study. Methods: In order to synthesize key findings for the objectives of this review, we searched English literature published up to November 16, 2020 using PubMed with the following keywords: Kawasaki disease 2020, Kawasaki-like disease, MIS-C, PIMS, PMIS and PIMS-TS. Key Content and Findings: Based on current researches, KD is regarded as an immune disorder induced by multiple unidentified pathogens, while MIS-C is confirmed to be associated with the infection of COVID-19. In addition, KD is popular in East Asian children under 3 years old, while MIS-C is reported more in older adolescents from Europe and North America. On the basis of multiple cohort studies, gastrointestinal symptoms, mechanical ventilation and inotropic support are more common in MIS-C. Instead, coronary arterial damage is more pronounced in KD. Moreover, the treatment regimen for MIS-C is more aggressive than KD because the cytokine storm is more violent and lasting. Conclusions: MIS-C is likely to be a distinct immunopathogenic illness associated with SARS-CoV-2 based on current studies, which could be used as a reference to help us better understand KD. In addition, MIS-C is an emerging syndrome for pediatricians, so the lack of relevant knowledge may result in under-diagnosis. Some individuals may fulfill full or partial criteria for KD but all should be reported if they meet the case definition for MIS-C. © 2022 AME Publishing Company. All right reserved.
背景与目的:SARS-CoV-2感染儿童在疫情爆发初期因发病率低、临床症状轻而未引起重视。自2020年4月下旬以来,迅速出现了与COVID-19相关的儿童川崎样综合征和高炎症反应的报告。儿童多系统炎症综合征(multi - system inflammatory syndrome in Children, MIS-C)与川崎病(Kawasaki Disease, KD)之间的关系尚不明确,有待进一步研究。方法:为了综合本综述目标的关键发现,我们在PubMed检索截至2020年11月16日发表的英文文献,关键词:Kawasaki disease 2020, Kawasaki-like disease, MIS-C, PIMS, PMIS和PIMS- ts。关键内容和发现:根据目前的研究,KD被认为是一种由多种不明病原体引起的免疫紊乱,而misc被证实与COVID-19感染有关。此外,KD在东亚3岁以下儿童中很流行,而据报道,misc在欧洲和北美的年龄较大的青少年中更多。在多队列研究的基础上,胃肠道症状、机械通气和肌力支持在MIS-C中更为常见。相反,冠状动脉损伤在KD中更为明显。此外,MIS-C的治疗方案比KD更具侵略性,因为细胞因子风暴更猛烈,更持久。结论:根据目前的研究,MIS-C可能是与SARS-CoV-2相关的一种独特的免疫致病性疾病,可以作为参考,帮助我们更好地了解KD。此外,对于儿科医生来说,MIS-C是一种新兴的综合征,因此缺乏相关知识可能导致诊断不足。有些人可能满足KD的全部或部分标准,但如果他们符合MIS-C的病例定义,则应全部报告。©2022 AME出版公司。版权所有。
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引用次数: 0
Laboratory testing for the diagnosis of neonatal and pediatric immune neutropenias: a narrative review 实验室检测诊断新生儿和儿童免疫性中性粒细胞减少症:叙述性回顾
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-43
A. Cattaneo, L. Porretti
Objectives: This review focuses on the role of anti-neutrophil antibodies in neonatal and pediatric neutropenias with particular regard to laboratory tests useful to detect them. Background: Neutropenia is a common medical problem for infants and children that can predispose them to the risk of bacterial infections. There are many causes for pediatric neutropenia, but at birth and for the first years of life, neutropenia is often attributable to immune mechanisms, either by the presence of auto or alloantibodies directed against human neutrophil antigens (HNA). The detection of these antibodies is a useful diagnostic tool to define immune-mediated neutropenia, which includes a variety of clinical conditions. Methods: PubMed was used to carry out a literature search for the last 10 years. In addition, pivotal papers on this topic, before 2010, were also considered. Conclusions: The detection and identification of anti-neutrophil antibodies with the techniques currently available are often laborious, difficult, and performed in specialized laboratories. Moreover, since neutrophils are fragile cells and difficult to preserve, several advances in laboratory procedures have been developed in the last years. The recent availability of new commercial assays, which do not require fresh cells, may represent an important step forward in neutrophil serology, as it could improve the standardization and availability of serological and genomic tests that can also be used in non-specialized laboratories. However, these assays still need to complete the necessary validations before replacing classical serological methods. 13
目的:这篇综述的重点是抗中性粒细胞抗体在新生儿和儿童中性粒细胞减少中的作用,特别是对检测它们有用的实验室测试。背景:中性粒细胞减少症是婴儿和儿童常见的医学问题,会使他们容易受到细菌感染。儿童中性粒细胞减少症有很多原因,但在出生时和生命的最初几年,中性粒细胞减症通常可归因于免疫机制,无论是针对人类中性粒细胞抗原(HNA)的自身抗体还是同种抗体的存在。这些抗体的检测是定义免疫介导的中性粒细胞减少症的有用诊断工具,其中包括各种临床条件。方法:采用PubMed进行近10年的文献检索。此外,还审议了2010年之前关于这一主题的关键文件。结论:用目前可用的技术检测和鉴定抗中性粒细胞抗体通常是费力、困难的,而且是在专业实验室进行的。此外,由于中性粒细胞是脆弱的细胞,很难保存,近年来在实验室程序方面取得了一些进展。最近新的商业检测不需要新鲜细胞,这可能是中性粒细胞血清学向前迈出的重要一步,因为它可以提高血清学和基因组检测的标准化和可用性,这些检测也可以在非专业实验室中使用。然而,在取代经典的血清学方法之前,这些检测仍然需要完成必要的验证。13
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引用次数: 0
Diagnosis and outcomes of pediatric central nervous system tumors in China: a single-center retrospective analysis from 2015 to 2020 中国儿童中枢神经系统肿瘤的诊断和预后:2015 - 2020年单中心回顾性分析
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-67
Lihua Yu, Jiale Liu, Minjie Luo, D. Lin, Li Wu, Lulu Huang, Yajie Zhang, J. Zi, Jingxin Zhang, Xuebin Liao, J. Guan, Xinde Zhao, Ming Zhou, Yong Yang, Junde Zhang, Wangming Zhang, Lihua Yang
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引用次数: 0
Clinical characteristics and prognosis of pediatric medulloblastoma: a case study of 40 patients at children’s hospital of Soochow university 苏州大学儿童医院40例小儿髓母细胞瘤临床特点及预后分析
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-59
Weiwei Du, Ye Lu, Fan Yang, Q. Lu, Zhiheng Li, Jun Lu, Shaoyan Hu
Background: To explore the clinical, histological, and molecular characteristics of childhood medulloblastoma (MB) and its prognosis. Methods: The authors analyzed clinical and pathological data from MB patients at Children’s Hospital of Soochow University who were diagnosed by pathology after surgical resection between November 2011 and October 2020. Results: A total of 40 patients were considered (20 males and 20 females), where the median age at MB onset was 81 months (5–163 months). Of these, complete resection was performed for 26 cases (26/40, 65%) and subtotal resection performed for 14 cases (14/40, 35%). Pathology after resection indicated that in 8 cases the MB had already metastasized. All 40 children were histologically classified, identifying: 32 cases of classic type (32/40, 80%), 6 cases of desmoplastic/nodular type (6/40, 15%), and 2 cases of anaplastic type (2/40, 5%). Molecular typing tests were performed on 19 children, identifying: 1 case in the wingless-activated (WNT-activated) group (1/19, 5.3%), 6 cases in the sonic hedgehog-activated (SHH-activated) group (6/19, 31.6%), 7 cases in Group 3 (7/19, 36.8%), and 5 cases in Group 4 (5/19, 26.3%). Of the 40 patients, 29 received a combined treatment of surgery, radiotherapy, and chemotherapy, and the median follow-up time was 25 months (1–102 months). The 3-year overall survival rate (OS) and event-free survival rate (EFS) were (64.3±10.4)% and (61.8±10.3)%, respectively. Univariate analyses showed that age, clinical stage, pathological subtype, and radiotherapy were potentially relevant variables in patient prognosis (P<0.05). Cox regression analysis showed that age and tumor metastasis were independent risk factors for poor prognosis of children with MB (P<0.05). Conclusions: Metastasis and age at initial diagnosis are unfavorable factors in the prognosis of childhood MB patients; however, radiotherapy could improve the prognosis of MB patients. 8
背景:探讨儿童髓母细胞瘤(MB)的临床、组织学和分子特征及其预后。方法:作者分析了2011年11月至2020年10月期间东吴大学儿童医院手术切除后经病理诊断为MB患者的临床和病理数据。结果:共考虑了40名患者(20名男性和20名女性),其中MB发病的中位年龄为81个月(5-163个月)。其中,完全切除26例(26/40,65%),次全切除14例(14/40,35%)。切除后病理学检查显示8例MB已经转移。所有40名儿童都进行了组织学分类,确定:经典型32例(32/40,80%),结缔组织增生性/结节型6例(6/40,15%),间变性型2例(2/40,5%)。对19名儿童进行了分子分型测试,确定:无翼激活(WNT激活)组1例(1/19,5.3%),声波刺猬激活(SHH激活)组6例(6/19,31.6%),第3组7例(7/19,36.8%),第4组5例(5/19,26.3%)。在40名患者中,29人接受了手术、放疗和化疗的联合治疗,中位随访时间为25个月(1-102个月)。3年总生存率(OS)和无事件生存率(EFS)分别为(64.3±10.4)%和(61.8±10.3)%。单因素分析显示年龄、临床分期、病理亚型、,Cox回归分析显示年龄和肿瘤转移是儿童MB预后不良的独立危险因素(P<0.05)。结论:转移和初诊年龄是影响儿童MB预后的不利因素;放疗可改善MB患者的预后。8.
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引用次数: 0
Current outcomes and future trends in paediatric and congenital cardiac surgery: a narrative review 儿科和先天性心脏手术的当前结果和未来趋势:叙述性回顾
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-47
L. Kiraly
Objective: To provide a narrative review of the current outcomes and future trends in paediatric and congenital cardiac surgery in a historical and multidisciplinary context. To present the paradigm shift in treating congenital heart disease that translate into improved outcomes. To identify current problems with directions of research. Background: Congenital heart disease (CHD) is the most common birth defect affecting approximately 1 neonate in every 120–166 births. More than half of CHD-patients need cardiac surgery in their lifetime; and half of the surgeries are required within the first six months of life. Methods: Narrative overview of the literature combining with current outcome data available from international databases is presented in comparison to the programme development of a newly-established tertiary-care centre. Conclusion: Congenital cardiac surgery is reconstructive surgery that aims for restoring biventricular circulation, when possible. Single-stage primary complete repair has become the central philosophy since the 1980s. In about thirty percent, physiologic and anatomical reasons do not permit repair by a single operation; these patients endure staged-repairs. Another 15% of CHD patients will require subsequent reoperations— mostly re-replacements of non-growing and/or deranged prostheses. Owing to advances of multidisciplinary treatment, CHD survival to adulthood now reaches 90–95% in high-income countries from less than 20% in the presurgical era. Treating CHD patients is a commitment for life. Research for viable and growing prostheses may solve the significant public health aspects currently associated with reoperations.
目的:在历史和多学科背景下,对儿科和先天性心脏手术的当前结果和未来趋势进行叙述性回顾。介绍治疗先天性心脏病的模式转变,转化为改善的结果。确定当前的问题和研究方向。背景:先天性心脏病(CHD)是最常见的出生缺陷,大约每120-166例新生儿中就有1例。超过一半的冠心病患者一生中需要心脏手术;一半的手术需要在出生后的头六个月内进行。方法:结合从国际数据库中获得的当前结果数据,对文献进行叙述性概述,并与新建立的三级护理中心的方案发展进行比较。结论:先天性心脏手术是一种以恢复双心室循环为目的的再造性手术。自20世纪80年代以来,单阶段初级完全修复已成为中心理念。在大约30%的病例中,生理和解剖原因不允许单次手术修复;这些病人接受分期修复。另外15%的冠心病患者将需要后续的再手术——主要是重新更换不能生长和/或紊乱的假体。由于多学科治疗的进步,在高收入国家,冠心病到成年期的存活率从手术前的不到20%提高到现在的90-95%。治疗冠心病患者是一项终身的承诺。研究可行和生长的假体可以解决目前与再手术相关的重大公共卫生问题。
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引用次数: 1
Clinical features and prognostic analysis of children with medulloblastoma in a single center in China 中国单中心儿童髓母细胞瘤的临床特点及预后分析
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-34
Yan Su, Shuai Zhu, Jing Qin, M. Ge, Yuan-qi Ji, Jian Gong, Tong Yu, Libing Fu, Zhikai Liu, Xiao-li Ma
Background: Medulloblastoma (MB) is the most common malignant pediatric brain tumor. This study systematically summarized the clinical characteristics and prognosis of children with MB respectively. Univariate survival analysis identified age <3 years and the absence of radiotherapy as poor prognostic factors (both P<0.05). Conclusions: Survival outcomes were good among patients with MB (MB) in our center. Young age and the omission of radiotherapy may be predictive of poor outcomes.
背景:髓母细胞瘤是儿童最常见的恶性脑肿瘤。本研究系统总结了小儿MB的临床特点和预后。单因素生存分析发现年龄<3岁和未接受放疗是不良预后因素(P<0.05)。结论:本中心MB患者的生存结局良好。年轻和未接受放射治疗可能预示预后不良。
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引用次数: 0
An attempt at building a database of children using donor human milk in Japan 在日本尝试使用捐赠母乳建立儿童数据库
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-31
Motoichiro Sakurai, Yuko Sakurai, Y. Wada, Yuuki Tani, K. Mizuno
A hospital-dedicated human milk bank was established in Japan at Koto Toyosu Hospital, Showa University in 2014. After a 3-year trial-and-error period, the Japanese Human Milk Bank Association was established in 2017. The supply of donor human milk (DHM) from the Japanese Human Milk Bank Association to various facilities nationwide has increased recently. However, as of 2021, there is only one human milk bank in Japan and the supply is limited. Therefore, there is an urgent need to understand the status of usage of DHM in the neonatal intensive care unit. Moreover, it is globally rare to build a database aimed at understanding the background and prognosis of all children supplied with DHM. In this paper, we have introduced the database and reported on aspects such as the salient points considered in building this database. The fundamental policy of this database included the following: (I) accessibility from the neonatal intensive care unit, (II) a simple input method, (III) reliability and continuity, (IV) safeguarding anonymity, (V) enriched search functionality, and (VI) enabling administration by the person in charge at each facility. In accordance with these six policies, the main items such as patient clinical information, DHM usage amount, and prognosis were set. In addition, the database was built to enable detailed search. The database was completed and became operational in November 2020. The input format was simplified as much as possible by adopting a selection-from-options approach. Enriched search functionality was implemented considering the function as a database in each facility. This database has enabled the proper operation of the human milk bank, and it would likely contribute further to perinatal care outcomes.
2014年,昭和大学东洋医院在日本成立了专门的母乳库。经过3年的试错,日本母乳库协会于2017年成立。最近,日本母乳库协会向全国各地提供的捐赠母乳有所增加。然而,截至2021年,日本只有一家母乳库,供应有限。因此,迫切需要了解DHM在新生儿重症监护室的使用情况。此外,建立一个旨在了解所有接受DHM的儿童的背景和预后的数据库在全球范围内是罕见的。在本文中,我们介绍了该数据库,并报告了在构建该数据库时考虑的要点等方面。该数据库的基本政策包括:(I)新生儿重症监护室的可访问性,(II)简单的输入方法,(III)可靠性和连续性,(IV)保护匿名性,(V)丰富的搜索功能,以及(VI)允许每个设施的负责人进行管理。根据这六项政策,设置了患者临床信息、DHM使用量和预后等主要项目。此外,建立数据库是为了进行详细搜索。该数据库已于2020年11月建成并投入使用。通过采用从选项中选择的方法,尽可能简化了输入格式。实现了丰富的搜索功能,将该功能视为每个设施中的数据库。这个数据库使母乳库能够正常运作,它可能会进一步促进围产期护理的结果。
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引用次数: 0
Bilirubin neurotoxicity: a narrative review on long lasting, insidious, and dangerous effects 胆红素神经毒性:对长期、潜伏和危险影响的叙述性回顾
Pub Date : 2021-01-01 DOI: 10.21037/pm-21-37
D. Brites, Rui F. M. Silva
Instituto de Investigação do Medicamento (iMed.ULisboa), Faculdade de Farmácia, Universidade de Lisboa, Lisbon, Portugal; Departamento de Ciências Farmacêuticas e do Medicamento, Faculdade de Farmácia, Universidade de Lisboa, Lisbon, Portugal Contributions: (I) Conception and design: Both authors; (II) Administrative support: None; (III) Provision of study materials or patients: None; (IV) Collection and assembly of data: Both authors; (V) Data analysis and interpretation: None; (VI) Manuscript writing: Both authors; (VII) Final Approval of manuscript: Both authors. Correspondence to: Dora Brites. Faculdade de Farmácia, Universidade de Lisboa, Lisbon, Portugal. Email: dbrites@ff.ulisboa.pt.
葡萄牙里斯本里斯本里斯本大学农场学院医学研究所;葡萄牙里斯本里斯本里斯本大学农场医学部贡献:(I)概念和设计:两位作者;(II) 行政支持:无;(III) 提供研究材料或患者:无;(IV) 数据收集和汇编:两位作者;(V) 数据分析和解释:无;(VI) 手稿写作:两位作者;(VII) 手稿的最终批准:两位作者。通信:多拉英国人。里斯本大学药学院,葡萄牙里斯本。电子邮件:dbrites@ff.ulisboa.pt.
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引用次数: 1
期刊
Pediatric medicine (Hong Kong, China)
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