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A narrative review on the role of genetics in children with acute recurrent pancreatitis and chronic pancreatitis 关于遗传学在儿童急性复发性胰腺炎和慢性胰腺炎中的作用的叙述综述
4区 医学 Q3 Medicine Pub Date : 2023-10-21 DOI: 10.1002/ped4.12404
Bo Yu, Yi Yu, Xinqiong Wang, Chundi Xu, Yuan Xiao
ABSTRACT The incidence of pancreatitis in children has increased over the past two decades. With advances in molecular biological techniques and clinical research, genetic variations have emerged as a pivotal etiological factor in pediatric pancreatitis. This review aims to summarize recent clinical research advancements in understanding pediatric pancreatitis caused by various gene mutations. As of the year 2020, researchers had identified 12 genes implicated in the pathogenesis of pancreatitis. These genes primarily contributed to the development of pancreatitis through three mechanisms. Pancreatitis resulting from these gene mutations exhibits several distinct characteristics, including early onset, a heightened risk of developing pancreatic duct stones, rapid disease progression, and a significantly increased risk of pancreatic endocrine and exocrine dysfunction, as well as pancreatic cancer in the future. Genetic sequencing is recommended for children with pancreatitis based on six indications. The sequencing not only assists in the clinical diagnosis but also enhances our understanding of the pathophysiology of pancreatitis.
在过去的二十年中,儿童胰腺炎的发病率有所增加。随着分子生物学技术和临床研究的进步,遗传变异已成为儿童胰腺炎的关键病因。本文综述了近年来各种基因突变引起的小儿胰腺炎的临床研究进展。截至2020年,研究人员已经确定了12个与胰腺炎发病机制有关的基因。这些基因主要通过三种机制促进胰腺炎的发展。由这些基因突变引起的胰腺炎表现出几个明显的特征,包括发病早、发生胰管结石的风险增加、疾病进展迅速、胰腺内分泌和外分泌功能障碍的风险显著增加,以及未来发生胰腺癌的风险。根据六个适应症,建议对患有胰腺炎的儿童进行基因测序。测序不仅有助于临床诊断,而且提高了我们对胰腺炎病理生理学的认识。
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引用次数: 0
Nurses' perceptions about neonatal intensive care units providing family‐centered care are associated with skin‐to‐skin contact implementation 护士对新生儿重症监护室提供以家庭为中心的护理的看法与皮肤对皮肤接触的实施有关
4区 医学 Q3 Medicine Pub Date : 2023-10-20 DOI: 10.1002/ped4.12402
Marilyn Aita, Gwenaëlle De Clifford‐Faugère, Geneviève Laporte, Sébastien Colson, Nancy Feeley
Skin-to-skin contact (SSC) consists of positioning the diaper-clad infant against the parent's chest and is considered a nursing practice rooted in family-centered care (FCC).1 SSC implies simultaneous parental presence and helps to facilitate their involvement from the earliest hours of their preterm infants’ lives as this intervention is delivered by parents.1 FCC has gained worldwide popularity in recent years to promote parental presence and active participation of parents in care during neonatal intensive care units (NICUs) hospitalization.2 Both practices relate to developmental care (DC), which regroups specific interventions aimed at reducing stress and improving infant neurological development.3 It is well recognized that promoting SSC and FCC are recommended in the NICU, given its many health benefits for preterm infants and parents. SSC has been found to favor maternal attachment, enhance paternal role achievement and interactive behavior, reduce maternal and paternal anxiety, and promote infants’ long-term cognitive development.4, 5 On the other hand, FCC can improve the clinical outcomes of preterm infants such as greater weight gain, as well as the psychological well-being of the parents.6 More specifically, parental involvement in NICU care is associated with reduced infants’ length of hospital stay and collaboration with professionals increased parental satisfaction.7 Daily maternal presence during NICU hospitalization is associated with a decrease in emotional and behavioral problems in school-age children.8 Noteworthy, parental presence during NICU hospitalization with more frequent holding of their infant in their arms, is associated with better infants’ neurobehavioral development, such as better quality of movements and reduced arousal, at term equivalent age.9 Given their benefits and driven by the DC philosophy, nurses should encourage SSC as well as FCC to provide parents with opportunities for collaboration and care involvement in the NICU. Yet, significant barriers may impede nurses from implementing SSC and FCC in the NICU, namely the lack of adequate training for nurses about SSC.10 As optimal implementation of FCC and SSC may depend on the nurses’ perceptions of these DC practices, along with the training and education about these practices provided in the NICU, the aim of this manuscript is to explore the association between NICU nurses’ perceptions about SSC and FCC and their unit's ability to provide FCC and SSC. Secondary analysis was performed from a larger comparative international study that was conducted between October 2017 and July 2018, where 202 NICU nurses completed paper or online questionnaires on their perceptions about their unit provision of FCC and SSC.11 Prior to the beginning of the study, ethical approval was obtained in both Canada (MP-21-2018-1854) and France (20181306005 and CNIL 2211490 v0). Nurses were recruited from four level III university-affiliated NICUs (intensive and intermediate care
皮肤对皮肤接触(SSC)包括将穿着尿布的婴儿靠在父母的胸前,被认为是一种以家庭为中心的护理实践(FCC)SSC意味着父母同时在场,并有助于促进他们从早产儿生命的最初几个小时开始参与,因为这种干预是由父母提供的近年来,FCC在新生儿重症监护病房(NICUs)住院期间促进父母在场和父母积极参与护理方面得到了全球的普及这两种做法都与发育护理(DC)有关,它重新组合了旨在减轻压力和改善婴儿神经发育的特定干预措施鉴于SSC和FCC对早产儿和父母的诸多健康益处,推荐在新生儿重症监护室推广SSC和FCC是公认的。SSC有利于母亲依恋,提高父亲角色成就和互动行为,减少父母焦虑,促进婴儿的长期认知发展。另一方面,FCC可以改善早产儿的临床结果,如体重增加,以及父母的心理健康更具体地说,父母参与新生儿重症监护室护理与缩短婴儿住院时间和与专业人员合作增加父母满意度有关在新生儿重症监护病房住院期间,母亲每天出现与学龄儿童情绪和行为问题的减少有关值得注意的是,在新生儿重症监护病房住院期间,父母更频繁地将婴儿抱在怀里,与婴儿在足月等效年龄时更好的神经行为发育有关,例如更好的运动质量和更低的觉醒考虑到他们的好处,并在DC理念的推动下,护士应该鼓励SSC和FCC为父母提供合作和参与新生儿重症监护室护理的机会。然而,重大障碍可能妨碍健康护士实施SSC和FCC,即缺乏足够的培训护士SSC.10作为最优的实现的FCC和SSC可能取决于护士”对这些直流实践的看法,以及培训和教育对NICU中提供的这些做法,这手稿的目的是探索之间的联系NICU护士对SSC和FCC及其单位提供FCC和SSC的能力。在2017年10月至2018年7月期间进行的一项更大规模的国际比较研究中进行了二次分析,其中202名新生儿重症监护病房护士完成了关于其单位提供FCC和ssc的看法的书面或在线问卷调查。11在研究开始之前,加拿大(MP-21-2018-1854)和法国(20181306005和CNIL 2211490 v0)获得了伦理批准。护士从加拿大和法国的四所三级大学附属新生儿重症监护病房(重症监护和中级监护)招募,这些新生儿的出生年龄在23至40周之间。加拿大的床位总数为30至65张,法国为26至54张。在法国新生儿重症监护病房工作的护士人数从60人到100人不等,加拿大从113人到190人不等。护士的平均年龄为33.9±9.1岁,平均具有8.1±7.0年的新生儿护理经验,在抽样单位平均为7.1±6.9年。大多数是女性(97.5%,197/202),几乎一半的样本在白天工作(49.7%,94/189),其次是晚上工作(27.0%,51/189),晚上工作(7.9%,15/189),然后是三班轮流工作(15.30%,29/189)。85名护士(42.1%,85/202)完成了学士学位。SSC问卷评估了新生儿重症监护室护士对SSC的态度和知识,以及他们对所在单位SSC培训和教育以及实施的看法它包含20个项目,李克特量表分为5分,从1“从不”到5“总是”。它分为四个子量表:知识(五个项目)、个人态度和信念(四个项目)、员工培训和教育(五个项目)以及单位层面的SSC实施(六个项目)。培训、教育和实施是指SSC实践的几个方面,包括其实践指南的可用性、对SSC卫生保健专业人员的适当培训、支持SSC的跨学科合作以及在单位的充分实施越高的分数反映了越好的看法。FCC调查问卷询问护士对病房工作人员是否尊重家属、与家属合作并提供支持的看法它包括20个项目,李克特量表从1“从不”到4“总是”,分为三个子量表:尊重(6个项目),合作(9个项目)和支持(5个项目)。 各子量表的得分范围为6至24分(尊重)、9至36分(协作)和5至20分(支持),总分范围为20至80分。除总分外,每个子量表得分越高,表明他们对该单位提供FCC这些方面的看法越有利。两份问卷的法文和英文版本都有足够的效度和信度。12-14对每个问卷的子量表以及FCC问卷总分进行描述性分析(均值、标准差)。采用Pearson相关法探讨FCC问卷和SSC问卷的分量量表得分与总量表得分之间的关系。描述性分析也被计算来描述样本的人口统计数据。统计学分析采用SPSS v.26, alpha值为0.05。护士SSC问卷和FCC问卷各分量表的平均得分和总分见表1,各分量表的相关关系见表2。护士的FCC总分(64.79/80)、SSC态度量表得分(18.24/20)和知识量表得分(21.53/25)均较高(表1)。结果显示,护士的FCC总分与SSC各分量表得分显著相关。相关性从弱(0.17)到中等(0.30)不等(表2)。我们的研究结果表明,护士对所在单位提供FCC的良好看法与更高的SSC个人知识(0.17)和态度(0.19)以及他们在培训和教育方面对所在单位表现的更好看法(0.24)以及SSC实施(0.30)之间的关系最小。在两种实践的所有子量表中,护士对其新生儿重症监护室为家庭提供支持的看法(FCC支持子量表得分)与SSC可用培训和教育(0.29)以及FCC支持子量表得分与SSC在其单位的实施(0.31)之间的相关性最高。此外,护士对所在单位实施SSC的看法与NICU以家庭为中心的护理总分之间也存在类似的关联(0.30)。这一次要分析提供了一个探索如何护士的看法,他们的新生儿重症监护室的能力提供FCC与SSC相关,并带来新的知识,以指导新生儿实践。总体而言,护士认为本单位在护理护理方面表现良好,对护理护理的认识和态度良好。有趣的是,护士对新生儿重症监护室为家长提供支持的看法与员工培训和教育以及在新生儿重症监护室实施SSC之间存在关联。这些发现可以解释为,当SSC培训和教育提供给护士,并在新生儿病房得到很好的实施时,护士对NICU支持FCC有更有利的看法,反之亦然。加强护理实践不仅需要护士的积极认知,还需要高水平的专业能力,以及良好的组织结构,这可以通过护理教育培训和适当的管理支持来维持最近的一项研究表明,虚拟教育计划可以提高新生儿重症监护室护士对DC的认知和知识因此,通过与FCC或SSC相关的实施科学研究,可以促进这些实践在新生儿重症监护病房的实施。护士对其FCC和SSC的单位表现持积极态度,可能会转化为早产儿及其父母的积极健康结果。NICU住院期间的干预措施,如SSC,应在其他DC实践之外实施,因为这些干预措施鼓励父母在场并参与婴儿的护理由于我们的研究护士大多在白班工作,我们的研究结果可能可以解释为父母在白天更多的在场和护理参与,父母可能会要求这些实践,这反过来可能促进FCC并支持护士对SSC的有利态度。护士关于SSC的知识和良好态度也可能与他们的教育水平有关,因为较高的教育程度与更多的SSC知识和良好态度有关18,我们样本中接近45%的护士完成了护理学士学位。作为FFC的一个组成部分,SSC被认为是父母实施的最有力的干预措施之一,对父母和早产儿都有好处虽然SSC和FCC可以被视为独立的DC实践,但这一次要分析表明,nicu促进其中一种实践似乎也支持另一种实践。 因此,认为所在单位在向家长提供FCC方面表现良好的护士也认为所在单位实施了SSC,并提供了关于SSC的培训和教育。从理论和实践的角度来看,强调这种关系是必要的,以
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引用次数: 0
Dwell time and bloodstream infection incidence of umbilical venous catheterization in China 中国脐静脉置管停留时间与血流感染发生率
4区 医学 Q3 Medicine Pub Date : 2023-10-15 DOI: 10.1002/ped4.12403
Xu Zheng, Dan He, Zixin Yang, Lu Chen, Min Jiang, Yujie Qi, Fei Qin, Jie Yu, Yaguang Peng, Ling Liu, Mingyan Hei
ABSTRACT Importance Central line‐associated bloodstream infection (CLABSI) is one of the most serious complications of central venous access devices. Reducing the risk of CLABSI is of utmost significance in efforts to improve neonatal mortality rates and enhance long‐term prognosis. Objective To determine the dwell time and incidence of CLABSI of umbilical venous catheterization (UVC) for preterm infants in China. Methods Preterm infants with UVC admitted to 44 tertiary neonatal intensive care units in 24 provinces in China were enrolled. Study period was from November 2019 to August 2021. The end point of observations was 48 h after umbilical venous (UV) catheter removal. The primary outcomes were dwell time of UV catheter and UVC‐associated CLABSI. Data between infants with UV catheter dwell time ≤7 days and >7 days, and with birth weight (BW) ≤1000 g and >1000 g were compared. Results In total, 2172 neonates were enrolled (gestational age 30.0 ± 2.4 weeks, BW 1258.5 ± 392.8 g). The median UV catheter dwell time was 7 (6–10) days. The incidence of UVC‐associated CLABSI was 3.03/1000 UV catheter days. For infants with UV catheter dwell time ≤7 days and >7 days, the UVC‐associated CLABSI incidence was 3.71 and 2.65 per 1000 UV catheter days, respectively, P = 0.23. For infants with UVC dwell times of 3–6, 7–12, and 13–15 days, the UVC‐associated CLABSI rates were 0.14%, 0.68%, and 2.48% ( P < 0.01). The Kaplan–Meier plot of UV catheter dwell time to CLABSI showed no difference between infants with BW ≤1000 g and >1000 g ( P = 0.60). Interpretation The median dwell time of UV catheter was 7 days, and the incidence of UVC‐associated CLABSI was 3.03/1000 catheter days in China. The daily risk of UVC‐associated CLABSI and other complications increased with the dwell time.
中心静脉相关血流感染(CLABSI)是中心静脉通路装置最严重的并发症之一。降低CLABSI的风险对于提高新生儿死亡率和提高长期预后具有重要意义。目的了解中国早产儿脐静脉置管(UVC)中CLABSI的停留时间和发生率。方法选取全国24个省44个新生儿三级重症监护病房收治的UVC早产儿。研究时间为2019年11月至2021年8月。观察终点为脐静脉(UV)导管拔除后48小时。主要结果为UV导管的停留时间和UVC相关的CLABSI。比较UV导管停留时间≤7天和≤7天、出生体重(BW)≤1000天和≤1000天婴儿的数据。结果共纳入新生儿2172例(胎龄30.0±2.4周,体重1258.5±392.8 g),中位UV导管停留时间为7 (6-10)d。UVC相关CLABSI的发生率为3.03/1000 UV导管日。对于UV导管停留时间≤7天和≤7天的婴儿,UVC相关的CLABSI发生率分别为3.71和2.65 / 1000 UV导管天,P = 0.23。对于UVC滞留时间为3 - 6,7 - 12,13 - 15天的婴儿,UVC相关的CLABSI率分别为0.14%,0.68%和2.48% (P <0.01)。在Kaplan-Meier图中,BW≤1000 g和>1000 g婴儿的UV导管停留时间对CLABSI没有差异(P = 0.60)。在中国,UV导管的中位停留时间为7天,UVC相关CLABSI的发生率为3.03/1000导管天。UVC相关CLABSI和其他并发症的每日风险随着滞留时间的增加而增加。
{"title":"Dwell time and bloodstream infection incidence of umbilical venous catheterization in China","authors":"Xu Zheng, Dan He, Zixin Yang, Lu Chen, Min Jiang, Yujie Qi, Fei Qin, Jie Yu, Yaguang Peng, Ling Liu, Mingyan Hei","doi":"10.1002/ped4.12403","DOIUrl":"https://doi.org/10.1002/ped4.12403","url":null,"abstract":"ABSTRACT Importance Central line‐associated bloodstream infection (CLABSI) is one of the most serious complications of central venous access devices. Reducing the risk of CLABSI is of utmost significance in efforts to improve neonatal mortality rates and enhance long‐term prognosis. Objective To determine the dwell time and incidence of CLABSI of umbilical venous catheterization (UVC) for preterm infants in China. Methods Preterm infants with UVC admitted to 44 tertiary neonatal intensive care units in 24 provinces in China were enrolled. Study period was from November 2019 to August 2021. The end point of observations was 48 h after umbilical venous (UV) catheter removal. The primary outcomes were dwell time of UV catheter and UVC‐associated CLABSI. Data between infants with UV catheter dwell time ≤7 days and >7 days, and with birth weight (BW) ≤1000 g and >1000 g were compared. Results In total, 2172 neonates were enrolled (gestational age 30.0 ± 2.4 weeks, BW 1258.5 ± 392.8 g). The median UV catheter dwell time was 7 (6–10) days. The incidence of UVC‐associated CLABSI was 3.03/1000 UV catheter days. For infants with UV catheter dwell time ≤7 days and >7 days, the UVC‐associated CLABSI incidence was 3.71 and 2.65 per 1000 UV catheter days, respectively, P = 0.23. For infants with UVC dwell times of 3–6, 7–12, and 13–15 days, the UVC‐associated CLABSI rates were 0.14%, 0.68%, and 2.48% ( P < 0.01). The Kaplan–Meier plot of UV catheter dwell time to CLABSI showed no difference between infants with BW ≤1000 g and >1000 g ( P = 0.60). Interpretation The median dwell time of UV catheter was 7 days, and the incidence of UVC‐associated CLABSI was 3.03/1000 catheter days in China. The daily risk of UVC‐associated CLABSI and other complications increased with the dwell time.","PeriodicalId":19992,"journal":{"name":"Pediatric Investigation","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136185286","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Safety of removal of ProSeal laryngeal mask airway in children in the supine versus lateral position in a deep plane of anesthesia: A randomized controlled trial 深平面麻醉下儿童仰卧位与侧卧位去除ProSeal喉罩气道的安全性:一项随机对照试验
4区 医学 Q3 Medicine Pub Date : 2023-09-26 DOI: 10.1002/ped4.12401
Shweta Dhiman, Anju R. Bhalotra, Kavita R. Sharma
ABSTRACT Importance When a ProSeal laryngeal mask airway (PLMA) is removed with the child in a deep plane of anesthesia, the upper airway muscle tone and protective upper airway reflexes may be obtunded. Objective To determine whether the supine or lateral position is safer for the removal of a PLMA in deeply anesthetized children by comparing the incidence of upper airway complications. Methods This randomized single‐blind comparative trial was conducted at a tertiary care hospital between January 2020 and September 2020. Forty children of the American Society of Anesthesiologists class I/II of ages 1–12 years age undergoing surgery under general anesthesia with PLMA used as the definitive airway device were recruited. Patients were randomly allocated to lateral group or supine group for PLMA removal in a deep plane of anesthesia in the lateral or supine position. The primary outcome was the number of patients experiencing one or more upper airway complications and the secondary outcomes were incidence of individual respiratory adverse effects and of severe airway complications. Results The incidence of airway complications was 30% in the supine group and 20% in the lateral group ( P = 0.6641). Incidence of laryngospasm, immediate stridor, and excessive secretions were similar. Early stridor and oxygen desaturation were higher in the supine group ( P = 0.0374, P = 0.0183 respectively). Interpretation The overall incidence of upper airway complications was similar with the removal of a PLMA in the supine or lateral position in deeply anesthetized children. The incidence of oxygen desaturation and stridor were higher with PLMA removal in the supine as compared to the lateral position.
当儿童在深度麻醉平面下移除ProSeal喉罩气道(PLMA)时,上气道肌张力和保护性上气道反射可能会被阻断。目的通过比较深度麻醉患儿上呼吸道并发症的发生率,探讨仰卧位和侧卧位是否更安全。方法:该随机单盲比较试验于2020年1月至2020年9月在一家三级保健医院进行。招募了40名年龄在1-12岁的美国麻醉师学会I/II级儿童,他们在全身麻醉下接受手术,使用PLMA作为最终气道装置。将患者随机分为侧卧位组和仰卧位组,分别在侧卧位和仰卧位深度麻醉平面下取出PLMA。主要结局是出现一种或多种上呼吸道并发症的患者数量,次要结局是个体呼吸不良反应和严重气道并发症的发生率。结果仰卧位组气道并发症发生率为30%,侧卧位组为20% (P = 0.6641)。喉痉挛、立即喘鸣和分泌物过多的发生率相似。仰卧位组早期喘鸣和氧饱和度较高(P = 0.0374, P = 0.0183)。结论:深度麻醉患儿在仰卧位或侧卧位取下PLMA时,上呼吸道并发症的总体发生率相似。与侧卧位相比,在仰卧位移除PLMA时,氧不饱和和喘鸣的发生率更高。
{"title":"Safety of removal of ProSeal laryngeal mask airway in children in the supine versus lateral position in a deep plane of anesthesia: A randomized controlled trial","authors":"Shweta Dhiman, Anju R. Bhalotra, Kavita R. Sharma","doi":"10.1002/ped4.12401","DOIUrl":"https://doi.org/10.1002/ped4.12401","url":null,"abstract":"ABSTRACT Importance When a ProSeal laryngeal mask airway (PLMA) is removed with the child in a deep plane of anesthesia, the upper airway muscle tone and protective upper airway reflexes may be obtunded. Objective To determine whether the supine or lateral position is safer for the removal of a PLMA in deeply anesthetized children by comparing the incidence of upper airway complications. Methods This randomized single‐blind comparative trial was conducted at a tertiary care hospital between January 2020 and September 2020. Forty children of the American Society of Anesthesiologists class I/II of ages 1–12 years age undergoing surgery under general anesthesia with PLMA used as the definitive airway device were recruited. Patients were randomly allocated to lateral group or supine group for PLMA removal in a deep plane of anesthesia in the lateral or supine position. The primary outcome was the number of patients experiencing one or more upper airway complications and the secondary outcomes were incidence of individual respiratory adverse effects and of severe airway complications. Results The incidence of airway complications was 30% in the supine group and 20% in the lateral group ( P = 0.6641). Incidence of laryngospasm, immediate stridor, and excessive secretions were similar. Early stridor and oxygen desaturation were higher in the supine group ( P = 0.0374, P = 0.0183 respectively). Interpretation The overall incidence of upper airway complications was similar with the removal of a PLMA in the supine or lateral position in deeply anesthetized children. The incidence of oxygen desaturation and stridor were higher with PLMA removal in the supine as compared to the lateral position.","PeriodicalId":19992,"journal":{"name":"Pediatric Investigation","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134957900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Biologics and oral small-molecule inhibitors for treatment of pediatric atopic dermatitis: Opportunities and challenges. 生物制品和口服小分子抑制剂治疗儿童特应性皮炎:机遇和挑战。
IF 2.2 4区 医学 Q3 Medicine Pub Date : 2023-09-14 eCollection Date: 2023-09-01 DOI: 10.1002/ped4.12400
Anqi Zhao, Chaolan Pan, Ming Li

Atopic dermatitis (AD) is a complex disease characterized by recurrent eczematous lesions and refractory pruritus that drastically impairs quality of life. Due to the chronic and relapsing course, patients are easily trapped in the debilitating condition. Classical therapies show limitations, especially for patients with moderate-to-severe phenotypes. Advanced new insights in targeted therapies exhibit great application prospects which were reinforced by the more profound understanding of the disease pathogenesis. However, the sustained efficiency, biosafety, and long-term benefits still remain in further exploration. This review summarizes recent clinical studies on oral small-molecule inhibitors and biological agents for pediatric AD patients, which provides the latest frontiers to clinicians.

特应性皮炎(AD)是一种复杂的疾病,其特征是复发性湿疹样病变和顽固性瘙痒,严重损害生活质量。由于慢性和复发过程,患者很容易陷入衰弱状态。传统疗法显示出局限性,尤其是对于具有中度至重度表型的患者。靶向治疗的先进新见解显示出巨大的应用前景,对疾病发病机制的更深入理解加强了这一前景。然而,持续的效率、生物安全和长期效益仍有待进一步探索。本文综述了近年来儿童AD患者口服小分子抑制剂和生物制剂的临床研究,为临床医生提供了最新的前沿。
{"title":"Biologics and oral small-molecule inhibitors for treatment of pediatric atopic dermatitis: Opportunities and challenges.","authors":"Anqi Zhao,&nbsp;Chaolan Pan,&nbsp;Ming Li","doi":"10.1002/ped4.12400","DOIUrl":"https://doi.org/10.1002/ped4.12400","url":null,"abstract":"<p><p>Atopic dermatitis (AD) is a complex disease characterized by recurrent eczematous lesions and refractory pruritus that drastically impairs quality of life. Due to the chronic and relapsing course, patients are easily trapped in the debilitating condition. Classical therapies show limitations, especially for patients with moderate-to-severe phenotypes. Advanced new insights in targeted therapies exhibit great application prospects which were reinforced by the more profound understanding of the disease pathogenesis. However, the sustained efficiency, biosafety, and long-term benefits still remain in further exploration. This review summarizes recent clinical studies on oral small-molecule inhibitors and biological agents for pediatric AD patients, which provides the latest frontiers to clinicians.</p>","PeriodicalId":19992,"journal":{"name":"Pediatric Investigation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2023-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/34/51/PED4-7-177.PMC10509388.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41144126","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A case of familial incontinentia pigmenti in infancy without hyperpigmented stage. 一例婴儿期无色素沉着期的家族性色素失禁。
IF 2.2 4区 医学 Q3 Medicine Pub Date : 2023-09-04 eCollection Date: 2023-09-01 DOI: 10.1002/ped4.12399
Yumeng Wang, Xinyi Wang, Ting Chen, Chaolan Pan, Ming Li
FIGURE 1 Clinical presentations of the patient with incontinentia pigmenti and her mother, and the sequencing chromatogram of the family. (A–E) Clinical presentations of the proband. (A) Erythema on the scalp and (B) Linear erythemas on the groin at birth; (C) Alopecia and (D) Hypopigmentation along Blaschko’s lines on the trunk at 6 months old; (E) Facial hypopigmentation. (F, G) Clinical presentations of the mother. (F) Milder hypopigmentation on the trunk and (G) alopecia. (H) A heterozygous variant c.388C>T (p. Arg130Ter) in exon 2 of IKBKG in the patient. (I) The suspected mosaic variant in her mother. (J) Part of the normal sequence from exon 2 of the IKBKG gene of the patient’s maternal grandmother and (K) grandfather. Red arrows indicate the pathogenic variants and black arrows indicate the normal site.
{"title":"A case of familial incontinentia pigmenti in infancy without hyperpigmented stage.","authors":"Yumeng Wang,&nbsp;Xinyi Wang,&nbsp;Ting Chen,&nbsp;Chaolan Pan,&nbsp;Ming Li","doi":"10.1002/ped4.12399","DOIUrl":"https://doi.org/10.1002/ped4.12399","url":null,"abstract":"FIGURE 1 Clinical presentations of the patient with incontinentia pigmenti and her mother, and the sequencing chromatogram of the family. (A–E) Clinical presentations of the proband. (A) Erythema on the scalp and (B) Linear erythemas on the groin at birth; (C) Alopecia and (D) Hypopigmentation along Blaschko’s lines on the trunk at 6 months old; (E) Facial hypopigmentation. (F, G) Clinical presentations of the mother. (F) Milder hypopigmentation on the trunk and (G) alopecia. (H) A heterozygous variant c.388C>T (p. Arg130Ter) in exon 2 of IKBKG in the patient. (I) The suspected mosaic variant in her mother. (J) Part of the normal sequence from exon 2 of the IKBKG gene of the patient’s maternal grandmother and (K) grandfather. Red arrows indicate the pathogenic variants and black arrows indicate the normal site.","PeriodicalId":19992,"journal":{"name":"Pediatric Investigation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2023-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/50/d2/PED4-7-220.PMC10509384.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41105889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The update of treatment strategies in pediatrics with generalized pustular psoriasis in China. 中国儿科泛发性脓疱性银屑病治疗策略的更新。
IF 2.2 4区 医学 Q3 Medicine Pub Date : 2023-08-31 eCollection Date: 2023-09-01 DOI: 10.1002/ped4.12395
Yunliu Chen, Xin Xiang, Zhaoyang Wang, Chaoyang Miao, Zigang Xu

Generalized pustular psoriasis (GPP) is a severe subtype of psoriasis, commonly combined with systemic inflammation. Gene mutations have been found to be associated with GPP and vary by ethnicity. Systemic treatments are usually required for the severity and potential complications of GPP. However, there is no common consensus in China, especially among pediatric patients, whose data are scarce. Acitretin, methotrexate, and cyclosporine are widely used in pediatrics with GPP, while the adverse effects should be highlighted. The emergence of different biological agents brings us into a new era. This article discusses the genetic background of Chinese patients and demonstrates the evidence of treatment in pediatrics with GPP.

泛发性脓疱型银屑病(GPP)是银屑病的一种严重亚型,通常伴有全身炎症。基因突变已被发现与GPP有关,并因种族而异。GPP的严重程度和潜在并发症通常需要系统治疗。然而,在中国并没有达成共识,尤其是在儿科患者中,他们的数据很少。阿曲汀、甲氨蝶呤和环孢菌素在患有GPP的儿科中广泛使用,同时应强调其不良反应。不同生物制剂的出现将我们带入了一个新时代。本文讨论了中国患者的遗传背景,并证明了GPP在儿科治疗的证据。
{"title":"The update of treatment strategies in pediatrics with generalized pustular psoriasis in China.","authors":"Yunliu Chen,&nbsp;Xin Xiang,&nbsp;Zhaoyang Wang,&nbsp;Chaoyang Miao,&nbsp;Zigang Xu","doi":"10.1002/ped4.12395","DOIUrl":"https://doi.org/10.1002/ped4.12395","url":null,"abstract":"<p><p>Generalized pustular psoriasis (GPP) is a severe subtype of psoriasis, commonly combined with systemic inflammation. Gene mutations have been found to be associated with GPP and vary by ethnicity. Systemic treatments are usually required for the severity and potential complications of GPP. However, there is no common consensus in China, especially among pediatric patients, whose data are scarce. Acitretin, methotrexate, and cyclosporine are widely used in pediatrics with GPP, while the adverse effects should be highlighted. The emergence of different biological agents brings us into a new era. This article discusses the genetic background of Chinese patients and demonstrates the evidence of treatment in pediatrics with GPP.</p>","PeriodicalId":19992,"journal":{"name":"Pediatric Investigation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/fd/44/PED4-7-191.PMC10509407.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41146726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Rapid response with good toleration of sirolimus for life-threatening neonatal lymphatic malformations. 西罗莫司对危及生命的新生儿淋巴管畸形反应迅速,耐受性良好。
IF 2.2 4区 医学 Q3 Medicine Pub Date : 2023-08-30 eCollection Date: 2023-09-01 DOI: 10.1002/ped4.12397
Shixiao Dong, Li Li, Jiaosheng Xu, Zhonglong Han, Xia Zheng

Introduction: Lymphatic malformations (LMs) are rare vascular anomalies predominantly affecting infants, which can be debilitating or life-threatening when complicated with intralesional bleeding or infection. Effective and safe management strategies are essential in such cases.

Case presentation: We report a case series involving four Chinese neonates with life-threatening LMs, initially treated with oral sirolimus. All patients achieved rapid relief and sustained remission, using a lower sirolimus dosage than previously recommended. Furthermore, adverse events were rarely recorded during follow-up.

Conclusion: Sirolimus can be considered a promising choice for neonates with intricate and life-threatening LMs. Initiation with a reduced sirolimus dose is advisable.

引言:淋巴畸形(LMs)是一种罕见的血管异常,主要影响婴儿,当合并病变内出血或感染时,可能会使人衰弱或危及生命。在这种情况下,有效和安全的管理战略至关重要。病例介绍:我们报告了一系列病例,涉及四名中国新生儿,他们患有危及生命的LMs,最初接受口服西罗莫司治疗。所有患者使用的西罗莫司剂量均低于之前建议的剂量,从而实现了快速缓解和持续缓解。此外,在随访期间很少记录不良事件。结论:西罗莫司可被认为是患有复杂且危及生命的LMs的新生儿的一种有前途的选择。建议减少西罗莫司的剂量。
{"title":"Rapid response with good toleration of sirolimus for life-threatening neonatal lymphatic malformations.","authors":"Shixiao Dong,&nbsp;Li Li,&nbsp;Jiaosheng Xu,&nbsp;Zhonglong Han,&nbsp;Xia Zheng","doi":"10.1002/ped4.12397","DOIUrl":"https://doi.org/10.1002/ped4.12397","url":null,"abstract":"<p><strong>Introduction: </strong>Lymphatic malformations (LMs) are rare vascular anomalies predominantly affecting infants, which can be debilitating or life-threatening when complicated with intralesional bleeding or infection. Effective and safe management strategies are essential in such cases.</p><p><strong>Case presentation: </strong>We report a case series involving four Chinese neonates with life-threatening LMs, initially treated with oral sirolimus. All patients achieved rapid relief and sustained remission, using a lower sirolimus dosage than previously recommended. Furthermore, adverse events were rarely recorded during follow-up.</p><p><strong>Conclusion: </strong>Sirolimus can be considered a promising choice for neonates with intricate and life-threatening LMs. Initiation with a reduced sirolimus dose is advisable.</p>","PeriodicalId":19992,"journal":{"name":"Pediatric Investigation","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2023-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/65/f0/PED4-7-206.PMC10509387.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41137562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Frequent detection of genetic aberrations reveals novel pathogenesis and treatment modalities in systemic juvenile xanthogranuloma. 对遗传畸变的频繁检测揭示了系统性青少年黄色肉芽肿的新发病机制和治疗模式。
IF 2.2 4区 医学 Q3 Medicine Pub Date : 2023-08-28 eCollection Date: 2023-09-01 DOI: 10.1002/ped4.12398
Jiaosheng Xu, Hongyan Ma, Xingfeng Yao, Xiaofeng Han, Yang Wen, Siwei Wang, Zigang Xu, Lin Ma
Juvenile xanthogranuloma (JXG), the most common form of non-Langerhans cell histiocytosis (non-LCH), is generally confined to the skin during infancy and early childhood.1 JXG rarely involves extracutaneous tissues or systemic organs, resulting in high morbidity and mortality rates. Clonality in JXG has been verified by histopathological and genetic analyses, which have shown it to be tumorous rather than a reactive disorder of JXG.2 However, the exact etiopathogenesis of JXG remains unclear.
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引用次数: 0
Clinical epidemiology and disease burden of adenoviral encephalitis in hospitalized children in China: A nationwide cross-sectional study. 中国住院儿童腺病毒性脑炎的临床流行病学和疾病负担:一项全国性的横断面研究。
IF 2.2 4区 医学 Q3 Medicine Pub Date : 2023-08-25 eCollection Date: 2023-12-01 DOI: 10.1002/ped4.12396
Jiao Tian, Xinyu Wang, Linlin Zhang, Qi Li, Guoshuang Feng, Yueping Zeng, Ran Wang, Zhengde Xie

Importance: Adenovirus encephalitis is a significant infectious disease of the central nervous system that commonly affects children under the age of 5 and has a profound impact on the health of infants and young children throughout China. National multicenter epidemiological studies have significant public health implications.

Objective: This study aims to report the epidemiology of adenovirus encephalitis in hospitalized children in China, providing valuable guidance for clinicians.

Methods: The data utilized in this study were extracted from the comprehensive Futang Update Medical Records database, which comprises discharge medical records collected by 27 tertiary children's hospitals between January 2016 and December 2018 in China. Specifically, the face sheet of discharge medical records encompassed critical sociodemographic variables and basic medical care details.

Results: In this database, a total of 544 children were hospitalized due to adenoviral encephalitis. The male-to-female ratio was 1.62:1, with more boys being affected across different age groups and places of residence. Of the children hospitalized, the highest number of hospitalizations occurred in the 1-3-year age group and the number of hospitalizations decreased each year from 2016 to 2018. The disease exhibits seasonal characteristics with a pronounced peak in the summer months of June and July. While most children (58%) did not have any significant complications, one-third of them developed respiratory complications, including pneumonia and acute bronchitis. The median length of stay for adenoviral encephalitis was 7 days, and the median cost of hospitalization was 2145.56 US dollars.

Interpretation: This study highlights the prevalence of adenovirus encephalitis in hospitalized children in China. Children aged 1-3 years were found to be the main demographic hospitalized due to this condition, with boys being significantly more affected than girls. The seasonal variations of adenovirus encephalitis were also found to be significant. Fortunately, the fatality rate associated with this condition was low, and the prognosis was generally favorable.

重要性:腺病毒脑炎是一种重要的中枢神经系统感染性疾病,常见于5岁以下儿童,对中国婴幼儿健康有着深远的影响。国家多中心流行病学研究具有重要的公共卫生意义。目的:报道中国住院儿童腺病毒脑炎的流行病学,为临床医生提供有价值的指导。方法:本研究使用的数据提取自福堂更新病历综合数据库,该数据库包含2016年1月至2018年12月全国27家三级儿童医院的出院病历。具体来说,出院病历的人脸表包含了关键的社会人口变量和基本的医疗细节。结果:在本数据库中,共有544名儿童因腺病毒性脑炎住院。男女比例为1.62:1,不同年龄组和居住地的男孩受影响较多。在住院儿童中,住院人数最多的是1-3岁年龄组,2016 - 2018年住院人数逐年下降。该病具有季节性特征,夏季6、7月发病高峰明显。虽然大多数儿童(58%)没有任何明显的并发症,但其中三分之一出现呼吸道并发症,包括肺炎和急性支气管炎。腺病毒性脑炎的住院时间中位数为7天,住院费用中位数为2145.56美元。解释:本研究强调了中国住院儿童中腺病毒脑炎的患病率。发现1-3岁儿童是因这种情况住院的主要人口,男孩比女孩受影响明显更大。腺病毒脑炎的季节变化也很显著。幸运的是,与这种情况相关的死亡率很低,预后一般良好。
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引用次数: 0
期刊
Pediatric Investigation
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