朗格汉斯细胞组织细胞增多症
流行病学
医学
三级转诊医院
组织细胞增多症
回顾性队列研究
淋巴结
疾病
儿科
介绍
外科
病理
家庭医学
作者
Soon‐Hyo Kwon,Jae Woo Choi,Hyo Jin Kim,Sang Woong Youn
标识
DOI:10.1111/1346-8138.12232
摘要
Abstract Epidemiological study of Langerhans cell histiocytosis ( LCH ) has been limited due to its rarity and multisystemic involvement. The aim of this study was to investigate the epidemiological features of LCH via the clinical data warehouse ( CDW ). Clinical data of 30 LCH patients from the all departments of a tertiary referral hospital between 2003 and 2012 were analyzed retrospectively by searching the CDW . The male‐to‐female ratio was 2.8:1. The age of onset ranged 7 days to 57 years with a median of 13 years. Of the patients, 36.7% presented initial symptoms before the age of 10 years. The involved organs at diagnosis were: bone (66.7%), skin (16.7%), lungs (13.3%) and lymph node (3.3%). For all of the 30 cases, there were 31 disease sites because of a single case of multisystemic disease involving both skin and bone. Of the 96.7% of patients with single‐system disease, 69.0% had bony involvement. This study elucidated the clinical features of LCH from all the departments of a tertiary hospital via the CDW , which suggests a potential role of the CDW as a new epidemiological approach for rare diseases.
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