Lin K D, Lin J D, Hsu H H, Juang J H, Huang M J, Huang H S
Department of Internal Medicine, Chang Gung Medical, Linkou, Taiwan, R.O.C.
J Endocrinol Invest. 1998 Jul-Aug;21(7):428-33. doi: 10.1007/BF03347321.
Langerhans cell histiocytosis (LCH) is a rare disorder and may be complicated with hypopituitarism and diabetes insipidus (DI) due to invasion of the hypothalamic-pituitary area. In this study, 10 patients with complete (4) and partial (6) type central DI were found among 125 LCH patients in our hospital records. The water deprivation test, followed by the pitressin test, was performed to confirm DI. Hypothalamic-pituitary endocrine function tests were carried out on these 10 patients at the initial diagnosis and during follow-up. All patients revealed growth hormone insufficiency in the insulin hypoglycemic tolerance test. Four patients had impairment of cortisol secretion, demonstrated by insulin hypoglycemic stimulating test results. Two patients had poor response in the thyrotropin releasing hormone stimulating test. Two patients had only partial responses in the luteinizing hormone releasing hormone test. Four patients had hyperprolactinemia. All patients underwent surgical treatment followed by chemotherapy and/or radiotherapy. One patient completely recovered from the endocrine disorder, 3 patients required smaller doses of desmopressin, and one patient had normal adrenal, thyroid, and gonadal function. Hypothalamic-pituitary disorders in LCH should not be neglected. Treatment of LCH can partially or completely reverse associated endocrine disorders. Therefore, endocrine studies and hormone replacement should be mandatory for patients with LCH.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见疾病，可能因下丘脑 - 垂体区域受侵而并发垂体功能减退和尿崩症（DI）。在本研究中，在我院记录的125例LCH患者中发现10例完全性（4例）和部分性（6例）中枢性DI患者。进行禁水试验，随后进行垂体后叶素试验以确诊DI。对这10例患者在初诊时及随访期间进行下丘脑 - 垂体内分泌功能检查。所有患者在胰岛素低血糖耐量试验中均显示生长激素缺乏。胰岛素低血糖刺激试验结果显示4例患者皮质醇分泌受损。2例患者促甲状腺激素释放激素刺激试验反应不佳。2例患者促黄体生成素释放激素试验仅部分反应。4例患者有高泌乳素血症。所有患者均接受手术治疗，随后进行化疗和/或放疗。1例患者内分泌紊乱完全恢复，3例患者所需去氨加压素剂量减小，1例患者肾上腺、甲状腺和性腺功能正常。LCH中的下丘脑 - 垂体疾病不容忽视。LCH的治疗可部分或完全逆转相关内分泌紊乱。因此，对于LCH患者，内分泌检查和激素替代治疗应成为常规。
