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结节病继发尿崩症伴干酪样肉芽肿

Diabetes insipidus secondary to sarcoidosis presenting with caseating granuloma.

作者信息

Alam Taimour, Thomas Steven

机构信息

Department of Respiratory Medicine, Calderdale Royal Hospital, Salterhebble, Halifax, West Yorkshire, UK.

出版信息

BMJ Case Rep. 2011 Mar 3;2011:bcr0120113702. doi: 10.1136/bcr.01.2011.3702.

Abstract

Diabetes insipidus is a rare complication of sarcoid infiltration of the hypothalamic-pituitary region. Non-caseating granuloma formation is typical of sarcoidosis. Anterior and posterior pituitary function may be affected. MRI coupled with endocrinology assessment is the usual method of investigation. A 25-year-old Caucasian male with no significant medical history presented with polyuria and polydipsia. Water deprivation test confirmed diabetes insipidus. CT scanning of the chest confirmed lymphadenopathy. Lymph node biopsy revealed caseating granuloma. Extensive investigation for tuberculosis was negative. The patient was started on intranasal desmopressin and steroids with marked improvement in symptoms. This is the first reported case of neurosarcoidosis with diabetes insipidus and caseation on histology that we are aware of. Differentiating between caseation due to sarcoidosis and tuberculosis on histology is possible by the use of special stains. Return of normal endocrine function is unusual and the patient is likely to require desmopressin therapy for life.

摘要

尿崩症是结节病浸润下丘脑 - 垂体区域的一种罕见并发症。非干酪样肉芽肿形成是结节病的典型表现。垂体前叶和后叶功能可能会受到影响。磁共振成像(MRI)结合内分泌评估是常用的检查方法。一名25岁无明显病史的白种男性出现多尿和烦渴症状。禁水试验确诊为尿崩症。胸部CT扫描证实有淋巴结病。淋巴结活检显示干酪样肉芽肿。广泛的结核病检查结果为阴性。患者开始使用鼻内去氨加压素和类固醇治疗,症状有明显改善。这是我们所知的首例有组织学上干酪样变的神经结节病合并尿崩症的报道病例。通过使用特殊染色,在组织学上区分结节病引起的干酪样变和结核病是可行的。内分泌功能恢复正常并不常见,该患者可能需要终身接受去氨加压素治疗。

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本文引用的文献

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