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伴有常见变异型免疫缺陷患者的部分性中枢性尿崩症

Partial central diabetes insipidus in patient with common variable immunodeficiency.

作者信息

Megías Marta Cano, Matei Ana Maria, Gonzalez Albarran Olga, Perez Lopez Gilberto

机构信息

Endocrinology Department, University Hospital Ramon y Cajal, Madrid, Spain.

出版信息

BMJ Case Rep. 2012 Jul 3;2012:bcr1120115067. doi: 10.1136/bcr.11.2011.5067.

Abstract

Approximately 20% of patients with common variable immunodeficiency (CVID) have any autoimmune disease, as concurrent as prior to diagnosis, even during follow-up. In recent years, cases of CVID associated to endocrine autoimmune diseases have been reported. To our knowledge, no cases of CVID with diabetes insipidus has been reported previously. The authors present the case of a 37-year-old male, diagnosed of CVID, who had thirst, polyuria and nocturia for several years. After a water deprivation test and a complete resolution of patient's symptoms with vasopressin (DDAVP) treatment, diagnosis of partial central diabetes insipidus was finally made. Patients diagnosed of CVID could develop water misbalance due to posterior hypophysis autoimmune disorder. A high index of clinical suspicion, an early diagnosis and treatment of these disease could avoid future complications and improve the quality of life of these patients.

摘要

约20%的常见可变免疫缺陷(CVID)患者患有某种自身免疫性疾病,无论是在诊断时、诊断前,甚至是随访期间。近年来,已有CVID与内分泌自身免疫性疾病相关的病例报道。据我们所知,此前尚无CVID合并尿崩症的病例报道。作者介绍了一名37岁男性病例,该患者被诊断为CVID,数年来一直有口渴、多尿和夜尿症状。经过禁水试验以及使用加压素(去氨加压素)治疗后患者症状完全缓解,最终确诊为部分性中枢性尿崩症。诊断为CVID的患者可能因垂体后叶自身免疫性疾病而出现水代谢失衡。对这些疾病保持高度的临床怀疑、早期诊断和治疗可避免未来的并发症并改善这些患者的生活质量。

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