首页 > 最新文献

The Lancet. Child & adolescent health最新文献

英文 中文
Prevalence, management, health-care burden, and 90-day outcomes of prolonged mechanical ventilation in the paediatric intensive care unit (LongVentKids): an international, prospective, cross-sectional cohort study. 儿科重症监护室(LongVentKids)延长机械通气的患病率、管理、卫生保健负担和90天结局:一项国际前瞻性横断面队列研究。
Pub Date : 2025-01-01 DOI: 10.1016/S2352-4642(24)00296-7
Atsushi Kawaguchi, Analia Fernandez, Florent Baudin, Fabrizio Chiusolo, Jan H Lee, Joe Brierley, José Colleti, Karl Reiter, Kyung Won Kim, Yolanda Lopez Fernandez, Martin Kneyber, Marti Pons-Òdena, Natalie Napolitano, Robert J Graham, Tatsuya Kawasaki, Daniel Garros, Gonzalo Garcia Guerra, Philippe Jouvet
<p><strong>Background: </strong>The number of children requiring prolonged mechanical ventilation (PMV) has increased with the advancement of medical care. We aimed to estimate the prevalence of PMV worldwide, document demographic and clinical characteristics of children requiring PMV in paediatric intensive care units (PICUs), and to understand variation in clinical practice and health-care burden.</p><p><strong>Methods: </strong>This international, multicentre, cross-sectional cohort study screened participating PICUs in 28 countries for children aged >37 postgestational weeks to 17 years who had been receiving mechanical ventilation (MV; invasive or non-invasive) for at least 14 consecutive days. Screening days took place every 90 days for 3 years. Patients were eligible for inclusion in the analysis if they had been receiving MV (invasive or non-invasive) for at least 14 consecutive days by their first day of screening. Eligible patients were followed up on the subsequent screening day 90 days later or at time of hospital discharge, whichever came first. Outcome data were recorded in a validated web-based case report file. The primary outcome was the prevalence of PMV. Secondary outcomes were mortality, duration of MV, tracheostomy, and number of complications. All outcomes were assessed at 90 days post-screening. The study was registered with ClinicalTrials.gov, NCT04112459.</p><p><strong>Findings: </strong>Between Sept 4, 2019 and Dec 7, 2022, 14 595 children were screened on four separate screening days in 158 PICUs, and 2773 patients had been receiving MV for at least 14 days and were included in the analysis. The point prevalence of PMV was 25·8% (IQR 24·1-28·5). Median age was 0·4 years (IQR 0·2-5·3) and median weight was 8·1 kg (IQR 4·7-19·1). 625 (24·0%) of 2610 patients had a history of prematurity (<37 weeks gestational age at birth). 90-day outcome data were collected for 2430 patients. 441 (18·2%) of 2430 patients had died within 90 days. 649 (29·8%) of 2176 patients who initiated ventilation support upon hospital admission had a tracheostomy placed after the first 14 days of MV. The median time to tracheostomy placement after MV initiation was 26 days (IQR 18-52). 462 (21·2%) of 2176 patients had at least one failed extubation between MV initiation and their first screening date. 556 (25·6%) of 2174 patients who started MV upon hospital admission required MV for 21 days or less, whereas 1618 (74·4%) patients required MV for 22 days or more; 90-day mortality did not differ between these groups (18·2% vs 20·30%, p=0·288). Complications were recorded for 810 (38·4%) 2109 patients who initiated MV upon hospital admission; of these 539 (67%) had ventilator-associated pneumonia, and 212 (39%) of 539 patients had multiple episodes of ventilator-associated pneumonia.</p><p><strong>Interpretation: </strong>Timing of tracheostomy was variable, and duration of MV was longer than previously reported. The large variability in patients requiri
背景:随着医疗水平的提高,需要长时间机械通气(PMV)的儿童数量有所增加。我们的目的是估计全球PMV的患病率,记录儿科重症监护病房(picu)需要PMV的儿童的人口统计学和临床特征,并了解临床实践和卫生保健负担的变化。方法:这项国际、多中心、横断面队列研究筛选了28个国家中接受机械通气(MV;侵入性或非侵入性)至少连续14天。筛查日每90天进行一次,持续3年。如果患者在筛查的第一天接受MV(侵入性或非侵入性)至少连续14天,则有资格纳入分析。在90天后或出院时对符合条件的患者进行随访,以先到者为准。结果数据记录在经过验证的基于网络的病例报告文件中。主要结果是PMV的患病率。次要结局是死亡率、MV持续时间、气管切开术和并发症数量。所有结果在筛查后90天进行评估。该研究已在ClinicalTrials.gov注册,编号NCT04112459。在2019年9月4日至2022年12月7日期间,14595名儿童在158个picu中进行了4个单独的筛查,其中2773名患者接受了至少14天的MV,并被纳入分析。PMV点患病率为25.8% (IQR为24.1 ~ 28.5)。年龄中位数为0.4岁(IQR为0.2 ~ 3.5),体重中位数为8.1 kg (IQR为4.7 ~ 19.1)。2610例患者中有625例(24.0%)有早产史(解释:气管切开术的时间是可变的,MV持续时间比先前报道的要长。需要MV的患者以及相关的picu医疗负担和结果的巨大差异表明,有必要进一步研究影响MV患儿护理的因素。资助:魁北克呼吸研究网络、魁北克呼吸研究网络、妇女和儿童健康研究所-临床/社区研究整合和支助方案、法语国家的妇女和儿童健康研究所。翻译:关于摘要的法语和西班牙语翻译,请参见补充材料部分。
{"title":"Prevalence, management, health-care burden, and 90-day outcomes of prolonged mechanical ventilation in the paediatric intensive care unit (LongVentKids): an international, prospective, cross-sectional cohort study.","authors":"Atsushi Kawaguchi, Analia Fernandez, Florent Baudin, Fabrizio Chiusolo, Jan H Lee, Joe Brierley, José Colleti, Karl Reiter, Kyung Won Kim, Yolanda Lopez Fernandez, Martin Kneyber, Marti Pons-Òdena, Natalie Napolitano, Robert J Graham, Tatsuya Kawasaki, Daniel Garros, Gonzalo Garcia Guerra, Philippe Jouvet","doi":"10.1016/S2352-4642(24)00296-7","DOIUrl":"10.1016/S2352-4642(24)00296-7","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;The number of children requiring prolonged mechanical ventilation (PMV) has increased with the advancement of medical care. We aimed to estimate the prevalence of PMV worldwide, document demographic and clinical characteristics of children requiring PMV in paediatric intensive care units (PICUs), and to understand variation in clinical practice and health-care burden.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This international, multicentre, cross-sectional cohort study screened participating PICUs in 28 countries for children aged &gt;37 postgestational weeks to 17 years who had been receiving mechanical ventilation (MV; invasive or non-invasive) for at least 14 consecutive days. Screening days took place every 90 days for 3 years. Patients were eligible for inclusion in the analysis if they had been receiving MV (invasive or non-invasive) for at least 14 consecutive days by their first day of screening. Eligible patients were followed up on the subsequent screening day 90 days later or at time of hospital discharge, whichever came first. Outcome data were recorded in a validated web-based case report file. The primary outcome was the prevalence of PMV. Secondary outcomes were mortality, duration of MV, tracheostomy, and number of complications. All outcomes were assessed at 90 days post-screening. The study was registered with ClinicalTrials.gov, NCT04112459.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Findings: &lt;/strong&gt;Between Sept 4, 2019 and Dec 7, 2022, 14 595 children were screened on four separate screening days in 158 PICUs, and 2773 patients had been receiving MV for at least 14 days and were included in the analysis. The point prevalence of PMV was 25·8% (IQR 24·1-28·5). Median age was 0·4 years (IQR 0·2-5·3) and median weight was 8·1 kg (IQR 4·7-19·1). 625 (24·0%) of 2610 patients had a history of prematurity (&lt;37 weeks gestational age at birth). 90-day outcome data were collected for 2430 patients. 441 (18·2%) of 2430 patients had died within 90 days. 649 (29·8%) of 2176 patients who initiated ventilation support upon hospital admission had a tracheostomy placed after the first 14 days of MV. The median time to tracheostomy placement after MV initiation was 26 days (IQR 18-52). 462 (21·2%) of 2176 patients had at least one failed extubation between MV initiation and their first screening date. 556 (25·6%) of 2174 patients who started MV upon hospital admission required MV for 21 days or less, whereas 1618 (74·4%) patients required MV for 22 days or more; 90-day mortality did not differ between these groups (18·2% vs 20·30%, p=0·288). Complications were recorded for 810 (38·4%) 2109 patients who initiated MV upon hospital admission; of these 539 (67%) had ventilator-associated pneumonia, and 212 (39%) of 539 patients had multiple episodes of ventilator-associated pneumonia.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Interpretation: &lt;/strong&gt;Timing of tracheostomy was variable, and duration of MV was longer than previously reported. The large variability in patients requiri","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":"9 1","pages":"37-46"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142866943","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: 0
A new Youth Advisory Panel for 2024-27. 新成立的2024-27年度青年顾问小组。
Pub Date : 2025-01-01 DOI: 10.1016/S2352-4642(24)00336-5
Jules Morgan
{"title":"A new Youth Advisory Panel for 2024-27.","authors":"Jules Morgan","doi":"10.1016/S2352-4642(24)00336-5","DOIUrl":"10.1016/S2352-4642(24)00336-5","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":"9 1","pages":"10-14"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142866918","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: 0
Developmental considerations in the quest for paediatric mTBI biomarkers. 寻求儿科mTBI生物标志物的发育考虑。
Pub Date : 2025-01-01 Epub Date: 2024-12-02 DOI: 10.1016/S2352-4642(24)00326-2
Miriam Beauchamp
{"title":"Developmental considerations in the quest for paediatric mTBI biomarkers.","authors":"Miriam Beauchamp","doi":"10.1016/S2352-4642(24)00326-2","DOIUrl":"10.1016/S2352-4642(24)00326-2","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":" ","pages":"3-5"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142788340","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: 0
End violence against children and adolescents: integrate climate policy into the 2030 SDGs. 消除针对儿童和青少年的暴力:将气候政策纳入 2030 年可持续发展目标。
Pub Date : 2025-01-01 Epub Date: 2024-10-30 DOI: 10.1016/S2352-4642(24)00301-8
Ilan Cerna-Turoff, Karen M Devries
{"title":"End violence against children and adolescents: integrate climate policy into the 2030 SDGs.","authors":"Ilan Cerna-Turoff, Karen M Devries","doi":"10.1016/S2352-4642(24)00301-8","DOIUrl":"10.1016/S2352-4642(24)00301-8","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":" ","pages":"7-8"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142565279","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: 0
Children are not future producers and customers: a plea for the moral imperative of acting now. 儿童不是未来的生产者和顾客:呼吁现在就采取行动的道德必要性。
Pub Date : 2024-11-19 DOI: 10.1016/S2352-4642(24)00312-2
Mark Tomlinson, James Radner
{"title":"Children are not future producers and customers: a plea for the moral imperative of acting now.","authors":"Mark Tomlinson, James Radner","doi":"10.1016/S2352-4642(24)00312-2","DOIUrl":"https://doi.org/10.1016/S2352-4642(24)00312-2","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-11-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142694084","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: 0
Monitoring change from residential housing care to family-based care for children. 监测儿童从寄宿照料向家庭照料的转变。
Pub Date : 2024-08-01 Epub Date: 2024-05-14 DOI: 10.1016/S2352-4642(24)00102-0
Marian J Bakermans-Kranenburg, Marinus H van IJzendoorn
{"title":"Monitoring change from residential housing care to family-based care for children.","authors":"Marian J Bakermans-Kranenburg, Marinus H van IJzendoorn","doi":"10.1016/S2352-4642(24)00102-0","DOIUrl":"10.1016/S2352-4642(24)00102-0","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":" ","pages":"549-550"},"PeriodicalIF":0.0,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140961265","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: 0
Lipoprotein apheresis and long-term cardiovascular health: a real answer for children with HoFH? 脂蛋白分离与长期心血管健康:HoFH 儿童的真正答案?
Pub Date : 2024-07-01 Epub Date: 2024-05-14 DOI: 10.1016/S2352-4642(24)00105-6
Ari Horton
{"title":"Lipoprotein apheresis and long-term cardiovascular health: a real answer for children with HoFH?","authors":"Ari Horton","doi":"10.1016/S2352-4642(24)00105-6","DOIUrl":"10.1016/S2352-4642(24)00105-6","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":" ","pages":"468-469"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140961262","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: 0
Cardiovascular outcomes in patients with homozygous familial hypercholesterolaemia on lipoprotein apheresis initiated during childhood: long-term follow-up of an international cohort from two registries. 童年时期开始接受脂蛋白分离治疗的同型家族性高胆固醇血症患者的心血管预后:两个登记处国际队列的长期随访。
Pub Date : 2024-07-01 Epub Date: 2024-05-14 DOI: 10.1016/S2352-4642(24)00073-7
M Doortje Reijman, Tycho R Tromp, Barbara A Hutten, G Kees Hovingh, Dirk J Blom, Alberico L Catapano, Marina Cuchel, Eldad J Dann, Antonio Gallo, Lisa C Hudgins, Frederick J Raal, Kausik K Ray, Fouzia Sadiq, Handrean Soran, Jaap W Groothoff, Albert Wiegman, D Meeike Kusters

Background: Homozygous familial hypercholesterolaemia (HoFH) is a rare genetic disease characterised by extremely high plasma LDL cholesterol from birth, causing atherosclerotic cardiovascular disease at a young age. Lipoprotein apheresis in combination with lipid-lowering drugs effectively reduce LDL cholesterol, but long-term health outcomes of such treatment are unknown. We aimed to investigate the long-term cardiovascular outcomes associated with lipoprotein apheresis initiated in childhood or adolescence.

Methods: In this cohort study, data were drawn from the HoFH International Clinical Collaboration (HICC) and the international registry for Children with Homozygous Hypercholesterolemia on Lipoprotein Apheresis (CHAIN). An overall cohort included patients diagnosed with HoFH aged 0-18 years who were alive and in follow-up between Jan 1, 2010, and Nov 8, 2021, and whose high plasma LDL cholesterol concentrations made them eligible for lipoprotein apheresis. To compare cardiovascular outcomes, patients who initiated lipoprotein apheresis in childhood (lipoprotein apheresis group) and patients who only received lipid-lowering drugs (pharmacotherapy-only group) were matched by sex and untreated plasma LDL cholesterol concentrations. The primary outcome was a composite of cardiovascular death, myocardial infarction, ischaemic stroke, percutaneous coronary intervention, coronary artery bypass grafting, aortic valve replacement, peripheral artery disease, carotid endarterectomy, angina pectoris, and supra-aortic or aortic stenosis (collectively referred to as atherosclerotic cardiovascular disease), for which survival analyses were performed in the matched cohort. Cox regression analyses were used to compare disease-free survival between cohorts and to calculate hazard ratio (HR) and 95% CI adjusted for sex, age at diagnosis, untreated plasma LDL cholesterol concentration, and number of lipid-lowering therapies other than lipoprotein apheresis.

Findings: The overall cohort included 404 patients with a median age at diagnosis of 6·0 years (IQR 3·0-9·5) and median untreated plasma LDL cholesterol of 17·8 mmol/L (14·7-20·8). The matched cohorts included 250 patients (125 patients per group), with a median untreated LDL cholesterol of 17·2 mmol/L (14·8-19·7). Mean reduction in plasma LDL cholesterol concentrations between baseline and final follow-up was greater in the lipoprotein apheresis group (-55% [95% CI -60 to -51] vs -31% [-36 to -25]; p<0·0001). Patients in the lipoprotein apheresis group had longer atherosclerotic cardiovascular disease-free survival (adjusted HR 0·52 [95% CI 0·32-0·85]) and longer cardiovascular death-free survival (0·0301 [0·0021-0·4295]). Cardiovascular death was more common in the pharmacotherapy-only group than in the lipoprotein apheresis group (ten [8%] vs one [1%]; p=0·010), whereas median age at coronary artery bypass grafting was lower in the lipopro

背景:同卵家族性高胆固醇血症(HoFH)是一种罕见的遗传疾病,其特点是出生时血浆中的低密度脂蛋白胆固醇就极高,导致患者在年轻时就患上动脉粥样硬化性心血管疾病。脂蛋白分离术与降脂药物联合使用可有效降低低密度脂蛋白胆固醇,但这种治疗的长期健康效果尚不清楚。我们旨在研究儿童或青少年时期开始的脂蛋白清除术对心血管的长期影响:在这项队列研究中,数据来自于HoFH国际临床合作组织(HICC)和 "接受脂蛋白分离治疗的高胆固醇血症患儿国际登记处"(CHAIN)。总体队列包括2010年1月1日至2021年11月8日期间存活并接受随访的0-18岁确诊为HoFH的患者,这些患者的血浆低密度脂蛋白胆固醇浓度较高,符合脂蛋白分离治疗的条件。为了比较心血管预后,在儿童期开始接受脂蛋白清除术的患者(脂蛋白清除术组)和只接受降脂药物治疗的患者(单纯药物治疗组)按性别和未经治疗的血浆低密度脂蛋白胆固醇浓度进行了配对。主要结果是心血管死亡、心肌梗死、缺血性中风、经皮冠状动脉介入治疗、冠状动脉旁路移植术、主动脉瓣置换术、外周动脉疾病、颈动脉内膜切除术、心绞痛、主动脉上或主动脉狭窄(统称为动脉粥样硬化性心血管疾病)的综合结果,并对匹配队列进行了生存分析。采用 Cox 回归分析比较不同队列之间的无病生存率,并计算危险比(HR)和 95% CI(根据性别、诊断时的年龄、未经治疗的血浆低密度脂蛋白胆固醇浓度以及除脂蛋白清除术以外的降脂疗法次数进行调整):总体队列包括 404 名患者,诊断时的中位年龄为 6-0 岁(IQR 3-0-9-5),未经治疗的血浆低密度脂蛋白胆固醇中位数为 17-8 mmol/L (14-7-20-8)。配对队列包括 250 名患者(每组 125 名患者),未经治疗的低密度脂蛋白胆固醇中位数为 17-2 mmol/L (14-8-19-7)。从基线到最终随访期间,脂蛋白分离组血浆低密度脂蛋白胆固醇浓度的平均降幅更大(-55% [95% CI -60 to -51] vs -31% [-36 to -25];p解释:在HoFH患者中,儿童和青少年时期开始进行脂蛋白清除术与降低动脉粥样硬化性心血管疾病和死亡的长期风险有关,并且发现早期开始高频治疗对降低血浆胆固醇有明显益处。现在需要达成共识建议,以指导更广泛、更及时地使用脂蛋白清除术治疗HoFH患儿,还需要开展研究以进一步优化治疗,确保早期积极治疗的益处与对生活质量的影响相平衡:阿姆斯特丹大学医学中心、位置学术医学中心、宾夕法尼亚大学佩雷尔曼医学院、欧洲动脉粥样硬化协会、美国国立卫生研究院国家心肺血液研究所。
{"title":"Cardiovascular outcomes in patients with homozygous familial hypercholesterolaemia on lipoprotein apheresis initiated during childhood: long-term follow-up of an international cohort from two registries.","authors":"M Doortje Reijman, Tycho R Tromp, Barbara A Hutten, G Kees Hovingh, Dirk J Blom, Alberico L Catapano, Marina Cuchel, Eldad J Dann, Antonio Gallo, Lisa C Hudgins, Frederick J Raal, Kausik K Ray, Fouzia Sadiq, Handrean Soran, Jaap W Groothoff, Albert Wiegman, D Meeike Kusters","doi":"10.1016/S2352-4642(24)00073-7","DOIUrl":"10.1016/S2352-4642(24)00073-7","url":null,"abstract":"<p><strong>Background: </strong>Homozygous familial hypercholesterolaemia (HoFH) is a rare genetic disease characterised by extremely high plasma LDL cholesterol from birth, causing atherosclerotic cardiovascular disease at a young age. Lipoprotein apheresis in combination with lipid-lowering drugs effectively reduce LDL cholesterol, but long-term health outcomes of such treatment are unknown. We aimed to investigate the long-term cardiovascular outcomes associated with lipoprotein apheresis initiated in childhood or adolescence.</p><p><strong>Methods: </strong>In this cohort study, data were drawn from the HoFH International Clinical Collaboration (HICC) and the international registry for Children with Homozygous Hypercholesterolemia on Lipoprotein Apheresis (CHAIN). An overall cohort included patients diagnosed with HoFH aged 0-18 years who were alive and in follow-up between Jan 1, 2010, and Nov 8, 2021, and whose high plasma LDL cholesterol concentrations made them eligible for lipoprotein apheresis. To compare cardiovascular outcomes, patients who initiated lipoprotein apheresis in childhood (lipoprotein apheresis group) and patients who only received lipid-lowering drugs (pharmacotherapy-only group) were matched by sex and untreated plasma LDL cholesterol concentrations. The primary outcome was a composite of cardiovascular death, myocardial infarction, ischaemic stroke, percutaneous coronary intervention, coronary artery bypass grafting, aortic valve replacement, peripheral artery disease, carotid endarterectomy, angina pectoris, and supra-aortic or aortic stenosis (collectively referred to as atherosclerotic cardiovascular disease), for which survival analyses were performed in the matched cohort. Cox regression analyses were used to compare disease-free survival between cohorts and to calculate hazard ratio (HR) and 95% CI adjusted for sex, age at diagnosis, untreated plasma LDL cholesterol concentration, and number of lipid-lowering therapies other than lipoprotein apheresis.</p><p><strong>Findings: </strong>The overall cohort included 404 patients with a median age at diagnosis of 6·0 years (IQR 3·0-9·5) and median untreated plasma LDL cholesterol of 17·8 mmol/L (14·7-20·8). The matched cohorts included 250 patients (125 patients per group), with a median untreated LDL cholesterol of 17·2 mmol/L (14·8-19·7). Mean reduction in plasma LDL cholesterol concentrations between baseline and final follow-up was greater in the lipoprotein apheresis group (-55% [95% CI -60 to -51] vs -31% [-36 to -25]; p<0·0001). Patients in the lipoprotein apheresis group had longer atherosclerotic cardiovascular disease-free survival (adjusted HR 0·52 [95% CI 0·32-0·85]) and longer cardiovascular death-free survival (0·0301 [0·0021-0·4295]). Cardiovascular death was more common in the pharmacotherapy-only group than in the lipoprotein apheresis group (ten [8%] vs one [1%]; p=0·010), whereas median age at coronary artery bypass grafting was lower in the lipopro","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":" ","pages":"491-499"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140961259","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: 0
Amy Rule: transforming paediatric education and training. 艾米·鲁尔:改变儿科教育和培训。
Pub Date : 2022-09-01 DOI: 10.1016/s2352-4642(22)00270-x
Udani Samarasekera
{"title":"Amy Rule: transforming paediatric education and training.","authors":"Udani Samarasekera","doi":"10.1016/s2352-4642(22)00270-x","DOIUrl":"https://doi.org/10.1016/s2352-4642(22)00270-x","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":"47 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82089027","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: 0
Greater Horn of Africa's crown of thorns. 大非洲之角的荆棘之冠。
Pub Date : 2022-09-01 DOI: 10.1016/s2352-4642(22)00253-x
V. Balakrishnan
{"title":"Greater Horn of Africa's crown of thorns.","authors":"V. Balakrishnan","doi":"10.1016/s2352-4642(22)00253-x","DOIUrl":"https://doi.org/10.1016/s2352-4642(22)00253-x","url":null,"abstract":"","PeriodicalId":94246,"journal":{"name":"The Lancet. Child & adolescent health","volume":"10 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74325996","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: 3
期刊
The Lancet. Child & adolescent health
全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1