朗格汉斯细胞组织细胞增生症患儿的肺部受累情况

Çağrı Coşkun, A. Varan, N. Kurucu, B. Aydın, U. Özçelik, İnci Yaman Bajin, Eren Müngen, Ebru Yalçın, Diclehan Orhan, B. Oğuz, B. Yalçın, T. Kutluk, M. Haliloğlu
{"title":"朗格汉斯细胞组织细胞增生症患儿的肺部受累情况","authors":"Çağrı Coşkun, A. Varan, N. Kurucu, B. Aydın, U. Özçelik, İnci Yaman Bajin, Eren Müngen, Ebru Yalçın, Diclehan Orhan, B. Oğuz, B. Yalçın, T. Kutluk, M. Haliloğlu","doi":"10.24953/turkjpediatr.2024.4515","DOIUrl":null,"url":null,"abstract":"Background. Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH.\nMethods. We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022.\nResults. 10% (n=37) of 367 patients with LCH had lung involvement. The median age was 1.8 years (range: 0.4 & 17.7) with a male-to-female ratio of 2.3. At admission 29.7% (n=11) presented with respiratory symptoms. Imaging showed a spectrum from nodular opacities to multiple cysts. All but one patient had multisystem disease. Twenty-nine received vinblastine-containing therapy. Ten-year event-free (EFS) and overall survival (OS) rates were 47.8% and 63.3%, respectively. In children younger and older than two years of age, the 10-year EFS was 53.3% vs. 40.2% and the 10-year OS was 58.7% vs. 68.8%, respectively. In children with and without risk organ involvement, 10-year EFS was 51.9% vs. 46.3% and 10-year OS was 51.9% vs. 73.7%.\nConclusions. Lung and multisystem involvement are significant concerns in LCH, highlighting the need for careful management to reduce morbidity and mortality.","PeriodicalId":101314,"journal":{"name":"The Turkish journal of pediatrics","volume":"7 20","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pulmonary involvement in children with Langerhans cell histiocytosis\",\"authors\":\"Çağrı Coşkun, A. Varan, N. Kurucu, B. Aydın, U. Özçelik, İnci Yaman Bajin, Eren Müngen, Ebru Yalçın, Diclehan Orhan, B. Oğuz, B. Yalçın, T. Kutluk, M. Haliloğlu\",\"doi\":\"10.24953/turkjpediatr.2024.4515\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Background. Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH.\\nMethods. We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022.\\nResults. 10% (n=37) of 367 patients with LCH had lung involvement. The median age was 1.8 years (range: 0.4 & 17.7) with a male-to-female ratio of 2.3. At admission 29.7% (n=11) presented with respiratory symptoms. Imaging showed a spectrum from nodular opacities to multiple cysts. All but one patient had multisystem disease. Twenty-nine received vinblastine-containing therapy. Ten-year event-free (EFS) and overall survival (OS) rates were 47.8% and 63.3%, respectively. In children younger and older than two years of age, the 10-year EFS was 53.3% vs. 40.2% and the 10-year OS was 58.7% vs. 68.8%, respectively. In children with and without risk organ involvement, 10-year EFS was 51.9% vs. 46.3% and 10-year OS was 51.9% vs. 73.7%.\\nConclusions. Lung and multisystem involvement are significant concerns in LCH, highlighting the need for careful management to reduce morbidity and mortality.\",\"PeriodicalId\":101314,\"journal\":{\"name\":\"The Turkish journal of pediatrics\",\"volume\":\"7 20\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"The Turkish journal of pediatrics\",\"FirstCategoryId\":\"0\",\"ListUrlMain\":\"https://doi.org/10.24953/turkjpediatr.2024.4515\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Turkish journal of pediatrics","FirstCategoryId":"0","ListUrlMain":"https://doi.org/10.24953/turkjpediatr.2024.4515","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

背景:肺朗格汉斯细胞组织细胞增生症(pLCH肺朗格汉斯细胞组织细胞增生症(pLCH)是一种罕见疾病,大多为多系统性 LCH 的组成部分。我们旨在研究pLCH患儿的临床特征和治疗效果。我们回顾性研究了1974年至2022年期间确诊的37例pLCH患者的临床、放射学和治疗数据。在367名LCH患者中,10%(n=37)的患者肺部受累。中位年龄为1.8岁(范围:0.4至17.7岁),男女比例为2.3。入院时,29.7%(n=11)的患者出现呼吸道症状。影像学检查显示从结节性不透明到多发性囊肿。除一名患者外,其他患者均患有多系统疾病。29名患者接受了含长春新碱的治疗。十年无事件生存率(EFS)和总生存率(OS)分别为47.8%和63.3%。在年龄小于两岁和大于两岁的儿童中,10年无事件生存率分别为53.3%和40.2%,10年总生存率分别为58.7%和68.8%。在有和没有危险器官受累的儿童中,10年生存率分别为51.9%对46.3%,10年生存率分别为51.9%对73.7%。肺部和多系统受累是LCH的一个重要问题,强调了谨慎管理以降低发病率和死亡率的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Pulmonary involvement in children with Langerhans cell histiocytosis
Background. Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH. Methods. We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022. Results. 10% (n=37) of 367 patients with LCH had lung involvement. The median age was 1.8 years (range: 0.4 & 17.7) with a male-to-female ratio of 2.3. At admission 29.7% (n=11) presented with respiratory symptoms. Imaging showed a spectrum from nodular opacities to multiple cysts. All but one patient had multisystem disease. Twenty-nine received vinblastine-containing therapy. Ten-year event-free (EFS) and overall survival (OS) rates were 47.8% and 63.3%, respectively. In children younger and older than two years of age, the 10-year EFS was 53.3% vs. 40.2% and the 10-year OS was 58.7% vs. 68.8%, respectively. In children with and without risk organ involvement, 10-year EFS was 51.9% vs. 46.3% and 10-year OS was 51.9% vs. 73.7%. Conclusions. Lung and multisystem involvement are significant concerns in LCH, highlighting the need for careful management to reduce morbidity and mortality.
求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
期刊最新文献
Single-center experience of four cases with iron-refractory iron deficiency anemia (IRIDA). Sleep in hospitalized children with cancer: relationship with psychiatric disorders and hospital conditions. Comprehensive analysis of genotypic and phenotypic characteristics of biotinidase deficiency patients in the eastern region of Türkiye. Dent's disease: case series from a single center. Differences in physical activity, sedentary time, and anthropometric variables among children and adolescents: The TUBON project.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
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
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
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
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