Management of Langerhans Cell Histiocytosis (LCH)-Induced Central Diabetes Insipidus and Its Associated Endocrinological/Neurological Sequelae
Management of Langerhans Cell Histiocytosis (LCH)-Induced Central Diabetes Insipidus and Its Associated Endocrinological/Neurological Sequelae
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朗格汉斯细胞组织细胞增多症 (LCH) 诱发的中枢性尿崩症及其相关内分泌/神经后遗症的治疗
DOI：
10.5772/23520
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发表时间：
2011
期刊：
影响因子：
--
通讯作者：
A. Morimoto
中科院分区：
文献类型：
--
作者：
S. Imashuku;A. Morimoto
中枢性尿崩症 (CDI) 是由精氨酸加压素 (AVP)（也称为抗利尿激素）缺乏引起的。尽管 CDI 在儿童和年轻人中很少见，但应记住，它与中枢神经系统 (CNS) 中罕见的组织细胞疾病相关，即朗格汉斯细胞组织细胞增多症 (LCH)、黄色肉芽肿病和埃德海姆-切斯特病，所有这些疾病都会特别影响下丘脑和垂体柄，从而诱发 CDI (1,2)。特别是，CDI 是多灶性 LCH 患者中最常发生的 CNS 事件，这些患者通常具有多系统病变，包括颅面骨病变 (3)。 LCH 是一种罕见疾病，其特征是具有激活的朗格汉斯细胞表型的细胞增殖 (4)。早期研究报告称，25-50% 的 LCH 患者发生 CDI，但自从引入全身化疗以来，这一发生率似乎已下降至 7-20% (5)。根据各种颅外病变的存在，CDI 可以在诊断 LCH 之前、同时或之后发生。它也可能在全身性 LCH 化疗期间或治疗后发生。 LCH 诱发的 CDI 患者表现出典型的临床症状，例如多尿/多尿，并伴有异常影像学表现，例如垂体柄增厚或下丘脑肿块以及脑磁共振成像 (MRI) 上垂体后叶热信号（T1 加权）丢失 (5-8)。一旦发生 CDI，大多数患者的病情就变得不可逆转，需要终生使用 1-去氨-8-D-精氨酸加压素 (DDAVP) 进行去氨加压素替代治疗。此外，30-58% 的 CDI 患者在随访期间表现出垂体前叶激素缺乏 (APHD) (1,7)。 LCH 相关的 APHD 似乎与垂体柄增厚有关 (6,7)。此外，LCH 诱发的 CDI 患者最终可能会发展为神经退行性 (ND) 疾病 (9,10)。 LCH 引起的 CDI 的适当治疗包括 (a) 及时正确的诊断； (b) 如果可能的话，早期进行化疗/放疗干预以逆转 CDI； (c) 适当治疗 CDI，以预防以后发展为 APHD 或 ND 疾病； (d) 一旦 CDI 成为永久性，用 DDAVP 对其进行良好控制；理想情况下 (e) 探索未来可以预防 LCH 患者发生 CDI 的创新措施。
Central diabetes insipidus (CDI) is caused by a deficiency of arginine vasopressin (AVP), also known as antidiuretic hormone. Although CDI is rare in children and young adults, it should be kept in mind that it is associated with rare histiocytic disorders in the central nervous system (CNS), namely Langerhans cell histiocytosis (LCH), xanthogranulomatosis and Erdheim-Chester disease, all of which specifically affect the hypothalamus and pituitary stalk, thereby inducing CDI (1,2). In particular, CDI is the most frequently occurring CNS event in patients with multi-focal LCH, who often have multisystem lesions, including craniofacial bone lesions (3). LCH is a rare disorder that is characterized by the proliferation of cells that bear the activated Langerhans cell phenotype (4). Early studies reported that CDI occurs in 25–50% of LCH patients but this incidence appears to have dropped to 7–20% since the introduction of systemic chemotherapy (5). CDI can develop either before, simultaneously with, or subsequent to a diagnosis of LCH based on the presence of various extracranial lesions. It can also develop during chemotherapy for systemic LCH or after therapy. Patients with LCH-induced CDI show typical clinical symptoms, such as polyuria/polydispsia, in association with abnormal radiographic findings, such as a thickened pituitary stalk or a hypothalamic mass and the loss of a hot signal (T1 weighted) for the pituitary posterior lobe on brain magnetic resonance imaging (MRI) (5-8). Once CDI develops, it becomes irreversible in most patients, who will require life-long desmopressin replacement therapy with 1-desamino-8-D-arginine vasopressin(DDAVP). In addition, 30– 58% of patients with CDI exhibit anterior pituitary hormone deficiencies (APHD) during follow-up (1,7). LCH-associated APHD appears to be linked to a thickening of the pituitary stalk (6,7). In addition, patients with LCH-induced CDI can eventually develop neurodegenerative (ND) disease (9,10). Appropriate management of LCH-induced CDI involves (a) a prompt correct diagnosis; (b) early intervention with chemo/radiotherapy to reverse the CDI, if possible; (c) appropriately treating CDI to prevent the later development of APHD or ND disease; (d) good control of CDI, once it has become permanent, with DDAVP; and ideally (e) exploring future innovative measures that could prevent the occurrence of CDI in patients with LCH.
影响因子：
3.2
作者：
Imashuku, Shinsaku;Okazaki, Nagisa (Amamoto);Morimoto, Akira
通讯作者：
Morimoto, Akira
DOI：
--
发表时间：
2009
期刊：
Int J Hematol 90(4)
影响因子：
--
作者：
Imashuku S;Kinugawa N;Matsuzaki A;Kitoh T;Ohki K;Shioda Y;Tsunematsu Y;Imamura T;Morimoto A
通讯作者：
Morimoto A