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青年男性淋巴细胞性垂体炎伴尿崩症。

Lymphocytic hypophysitis with diabetes insipidus in a young man.

机构信息

Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.

出版信息

Nat Rev Endocrinol. 2010 Aug;6(8):464-70. doi: 10.1038/nrendo.2010.104. Epub 2010 Jun 29.

DOI:10.1038/nrendo.2010.104
PMID:20585348
Abstract

BACKGROUND

A 29-year-old man was referred to a multidisciplinary pituitary clinic with a 3.5-year history of central diabetes insipidus, initially presumed to be idiopathic based on a normal MRI scan of the pituitary gland. Subsequent scanning revealed a suprasellar mass, which demonstrated progressive enlargement on serial imaging. He also developed hypogonadotropic hypogonadism.

INVESTIGATIONS

Measurement of levels of serum morning fasting cortisol, adrenocorticotropic hormone, total testosterone, luteinizing hormone, follicle-stimulating hormone, prolactin, insulin-like growth factor 1, TSH and free T(4), MRI of the pituitary gland and a transsphenoidal biopsy of a pituitary mass were performed.

DIAGNOSIS

Lymphocytic hypophysitis presenting with diabetes insipidus, with development of hypogonadotropic hypogonadism and a suprasellar mass.

MANAGEMENT

The patient was treated with intranasal desmopressin and transdermal testosterone. The underlying lymphocytic hypophysitis was initially managed conservatively with serial MRI and visual field testing. No immunosuppressant medication was given and, aside from the diagnostic transsphenoidal biopsy, no surgical intervention was required. He subsequently developed secondary hypothyroidism, secondary adrenal insufficiency and growth hormone deficiency. These disorders were managed with levothyroxine and prednisone.

摘要

背景

一名 29 岁男性因 3.5 年中枢性尿崩症病史就诊于多学科垂体疾病门诊,最初根据垂体 MRI 正常而推测为特发性。随后的扫描显示鞍上肿块,连续影像学检查显示其进行性增大。他还出现了促性腺激素低下性性腺功能减退症。

检查

进行了血清晨空腹皮质醇、促肾上腺皮质激素、总睾酮、黄体生成素、卵泡刺激素、催乳素、胰岛素样生长因子 1、促甲状腺激素和游离 T(4)的测定,以及垂体 MRI 和垂体肿块经蝶窦活检。

诊断

淋巴细胞性垂体炎伴尿崩症,伴有促性腺激素低下性性腺功能减退症和鞍上肿块。

治疗

患者接受了鼻腔内去氨加压素和经皮睾酮治疗。最初采用连续 MRI 和视野检查对潜在的淋巴细胞性垂体炎进行保守治疗。未给予免疫抑制剂,除了诊断性经蝶窦活检外,无需手术干预。随后他出现了继发性甲状腺功能减退症、继发性肾上腺皮质功能不全和生长激素缺乏症。这些疾病通过左甲状腺素和泼尼松进行治疗。

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J Neurosurg. 2010 Jan;112(1):118-21. doi: 10.3171/2009.6.JNS081176.
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Empty sella may be the final outcome in lymphocytic hypophysitis.空蝶鞍可能是淋巴细胞性垂体炎的最终结局。
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Diagnosis and treatment of autoimmune hypophysitis: a short review.自身免疫性垂体炎的诊断与治疗:简短综述。
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