DIABETES-INSIPIDUS IN LANGERHANS CELL HISTIOCYTOSIS - RESULTS FROM THE DAL-HX-83 STUDY
DIABETES-INSIPIDUS IN LANGERHANS CELL HISTIOCYTOSIS - RESULTS FROM THE DAL-HX-83 STUDY
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DOI：
10.1002/mpo.2950240407
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发表时间：
1995-04-01
期刊：
影响因子：
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通讯作者：
GADNER, H
中科院分区：
文献类型：
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作者：
GROIS, N;FLUCHERWOLFRAM, B;GADNER, H
尿崩症（D1）是朗格汉斯细胞组织细胞增生症（LCH）的常见并发症，病因不明。不同出版物的发生率从15%到50%不等。在前瞻性DAL-HX 83研究中，199例新诊断的LCH患者中有19例（9.5%）被诊断为D1。根据统一标准对所有患者进行分层。106例播散性疾病患者在确诊后立即接受规范化的综合化疗。在诊断LCH时，199例患者中有8例（4%）已经确定为D1。诊断后，D1仅发生在剩下的91例局限性疾病患者中的1例（1%）和剩下的100例播散性疾病患者中的10例（10%）。在8例患者中，D1的发作与活动性LCH的其他体征相关。中位观察时间为5年3个月后，发生D1的累积风险为11%。临床特征的回顾性分析显示多系统受累、颅骨和眼眶病变，特别是骨性病变的颅内延伸构成D1的危险因素。12例患者进行了磁共振成像（MRI）检查，10例儿童显示垂体区异常。在已确定D1的患者中，没有一个通过任何治疗逆转或改善。然而，对播散性疾病进行快速全身化疗似乎可以预防D1的发生，这可能是DAL-HX 83研究中D1频率较低的原因。(C)1995 Wiley-Liss，Inc.
Diabetes insipidus (D1) in Langerhans cell histiocytosis (LCH) is a common complication of unclear etiology. The incidence varies among different publications from 15% to 50%. In the prospective DAL-HX 83 study, 19 out of 199 patients (9.5%) registered with newly diagnosed LCH were diagnosed to have D1. All patients were stratified according to uniform criteria. One hundred and six patients with disseminated disease were treated with standardized polychemotherapy promptly after diagnosis. At the time of diagnosis of LCH, D1 was already established in 8 out of 199 patients (4%). After diagnosis, D1 occurred in only one out of the remaining 91 patients with localized disease (1%) and in 10 out of 100 remaining patients with disseminated disease (10%). In 8 patients, the onset of D1 was associated with other signs of active LCH. The cumulative risk to develop D1 after a median observation time of 5 years 3 months was 11%. Retrospective analysis of clinical features revealed multisystem involvement, skull and orbital lesions, and in particular intracranial extension from osseous lesions to constitute risk factors for D1. Magnetic resonance imaging studies (MRI) were available in 12 patients and showed abnormalities of the pituitary region in 10 children. in none of the patients with established D1 was it reversed or ameliorated by any treatment. However, the rapid institution of systemic chemotherapy for disseminated disease seems to prevent the occurrence of D1 and may be responsible for the low frequency of D1 in the DAL-HX 83 study. (C) 1995 Wiley-Liss, Inc.