Predictor Variables of Developing Anterior Pituitary Deficiencies in a Group of Paediatric Patients with Central Diabetes Insipidus and Langerhans Cell Histiocytosis
Predictor Variables of Developing Anterior Pituitary Deficiencies in a Group of Paediatric Patients with Central Diabetes Insipidus and Langerhans Cell Histiocytosis
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一组患有中枢性尿崩症和朗格汉斯细胞组织细胞增多症的儿科患者出现垂体前叶缺陷的预测变量
DOI：
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发表时间：
2016
影响因子：
3.2
通讯作者：
A. Belgorosky
中科院分区：
文献类型：
--
作者：
E. Vaiani;Carmen Malossetti;Lina Margarita Vega;P. Zubizarreta;J. Braier;A. Belgorosky
背景：朗格汉斯细胞组织细胞增生症（LCH）是一种罕见的组织细胞疾病，其发病机制尚不清楚。中枢性尿崩症（CDI）是最常见的内分泌表现，是进一步发展为垂体前叶激素缺乏症（APD）的已知危险因素。然而，并非所有CDI患者都发生APD，如在长期随访期间所观察到的。目的：寻找在我们机构随访的患有CDI的LCH儿童中发生APD的预测因素。方法：我们回顾性分析了44例患者，中位时间（四分位数）为12.3年（8.79-14.24）。根据APD的存在或不存在，分别将患者分为第1组和第2组。研究的主要变量是：（1）LCH诊断时的实足年龄（CA），（2）诊断时LCH的原发部位：低风险（LR）和多系统风险器官，（3）再激活的存在。结果如下：多变量考克斯回归分析显示，APD与LCH诊断时的CA [相对危险度（RR）1.14，p < 0.01]、LR临床形式（RR 8.6，p < 0.03）呈正相关，与再激活的存在呈负相关（RR 0.3，p < 0.01）。结论：在LCH诊断、LR临床形式和较少再激活发作时具有较老CA的患者可能代表了具有较高APD发生风险的儿科LCH CDI患者亚组。
Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder of unknown etiopathogenesis. Central diabetes insipidus (CDI) is the most frequent endocrine manifestation and is a known risk factor for the development of further anterior pituitary hormone deficiencies (APD). However, not all CDI patients develop APD, as observed during prolonged periods of follow-up. Aim: To find predictors of developing APD in LCH children with CDI followed in our institution. Methods: We retrospectively analysed 44 patients over a median period (quartiles) of 12.3 years (8.79-14.24). Patients were subdivided into group 1 and group 2, according to absence or presence of APD, respectively. The main variables studied were: (1) chronological age (CA) at LCH diagnosis, (2) the primary site of LCH at diagnosis: low risk (LR) and multisystemic risk organs, and (3) the presence of reactivation. Results: Multivariate Cox regression analysis showed that APD was positively associated with CA at LCH diagnosis [relative risk (RR) 1.14, p < 0.01], the LR clinical form (RR 8.6, p < 0.03), and negatively associated with the presence of reactivations (RR 0.3, p < 0.01). Conclusions: Patients with older CA at LCH diagnosis, LR clinical forms, and fewer reactivation episodes might represent a subgroup of paediatric LCH CDI patients with a higher risk of developing APD.
影响因子：
20.3
作者：
Badalian-Very, Gayane;Vergilio, Jo-Anne;Rollins, Barrett J.
通讯作者：
Rollins, Barrett J.
影响因子：
6.5
作者：
Berres ML;Merad M;Allen CE
通讯作者：
Allen CE
组织细胞增多症-X。
DOI：
10.1056/nejm198101153040304
发表时间：
1981
期刊：
The New England journal of medicine
影响因子：
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作者：
Osband,ME;Lipton,JM;Lavin,P;Levey,R;Vawter,G;Greenberger,JS;McCaffrey,RP;Parkman,R
通讯作者：
Parkman,R