Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI are recognized in the majority of the studies: (1) multisystem disease, (2) the occurrence of reactivations or active disease for a prolonged period, and (3) the presence of craniofacial bone lesions. Since CDI may occur as the first manifestation of LCH, differential diagnosis of malignant diseases like germ cell tumours must be made. APD is almost always associated with CDI and can appear several years after the diagnosis of CDI. Growth hormone is the most commonly affected anterior pituitary hormone. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.
Horm Res Paediatr

中文翻译：

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小儿朗格汉斯细胞组织细胞增生症：下丘脑内分泌系统的长期后遗症

朗格汉斯细胞组织细胞增生症 (LCH) 是一种单核吞噬细胞系统疾病，几乎可以影响任何器官和系统。LCH 中最常见的中枢神经系统 (CNS) 表现是下丘脑 - 垂体区域的浸润，导致中枢神经系统组织的破坏和神经变性。后者导致最常见的内分泌表现，即中枢性尿崩症 (CDI)，较少出现垂体前叶激素缺乏症 (APD)。据报道，CDI 的发生率估计在 11.5% 到 24% 之间，被认为是神经退行性疾病和 APD 的危险因素。大多数研究都认识到 CDI 发生的三个危险因素：(1) 多系统疾病，(2) 长时间发生再激活或活动性疾病，(3)颅面骨病变的存在。由于CDI可能是LCH的首发表现，因此必须对生殖细胞肿瘤等恶性疾病进行鉴别诊断。APD 几乎总是与 CDI 相关，并可在 CDI 诊断数年后出现。生长激素是最常受影响的垂体前叶激素。尽管近年来 LCH 的知识取得了重大进展，但在预防长期后遗症（例如影响下丘脑 - 垂体系统的后遗症）方面进展甚微。生长激素是最常受影响的垂体前叶激素。尽管近年来 LCH 的知识取得了重大进展，但在预防长期后遗症（例如影响下丘脑 - 垂体系统的后遗症）方面进展甚微。生长激素是最常受影响的垂体前叶激素。尽管近年来 LCH 的知识取得了重大进展，但在预防长期后遗症（例如影响下丘脑 - 垂体系统的后遗症）方面进展甚微。
儿科研究