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以内分泌急症形式呈现的Ⅱ型自身免疫性多腺体综合征:一例报告

Autoimmune Polyglandular Syndrome Type II Presenting as an Endocrine Emergency: A Case Report.

作者信息

Sonthalia Nikhil, Ray Sayantan, Maiti Animesh, Maitra Subhasis

机构信息

Department of General Medicine, Medical College and Hospital, Kolkata, India.

Calcutta National Medical College and Hospital, Kolkata, West Bengal. India.

出版信息

Oman Med J. 2013 May;28(3):e048. doi: 10.5001/omj.2013.64.

Abstract

We describe the case of a 30-year-old male, a known patient of type 1 diabetes mellitus (DM) on insulin therapy, seeking medical attention for recent onset repeated attacks of hypoglycemia associated with generalized weakness and darkening of skin. Further evaluation and screening revealed autoimmune adrenal failure together with presence of Hashimoto's thyroiditis. The patient was diagnosed as a case of autoimmune polyglandular syndrome (APS) type II with complete triad of Addison's disease, type 1 DM and autoimmune thyroid disease. Anti-thyroid peroxidase, anti-glutamic acid decarboxylase and anti-endomysial antibodies were present in our patient. He was started on replacement therapy with physiological dose of prednisolone and thyroxine resulting in marked improvement in his symptoms. Recurrent hypoglycemia in a type 1 DM patient should raise a suspicion of underlying autoimmune adrenal insufficiency. Absence of obvious signs of thyroid dysfunction also poses a diagnostic challenge for the clinicians. This article aims at highlighting the importance of detailed evaluation together with long term followup of these patients and their relatives as overt clinical disease may only be the tip of the iceberg of other underlying organ-specific autoimmune diseases that may develop later in the course.

摘要

我们描述了一名30岁男性的病例,该患者为1型糖尿病(DM)已知患者,正在接受胰岛素治疗,因近期反复出现低血糖发作并伴有全身无力和皮肤变黑而就医。进一步评估和筛查发现自身免疫性肾上腺衰竭以及桥本甲状腺炎。该患者被诊断为II型自身免疫性多腺体综合征(APS),伴有艾迪生病、1型DM和自身免疫性甲状腺疾病的完整三联征。我们的患者存在抗甲状腺过氧化物酶、抗谷氨酸脱羧酶和抗肌内膜抗体。他开始接受生理剂量的泼尼松龙和甲状腺素替代治疗,症状明显改善。1型DM患者反复出现低血糖应怀疑存在潜在的自身免疫性肾上腺功能不全。甲状腺功能障碍缺乏明显体征也给临床医生带来诊断挑战。本文旨在强调对这些患者及其亲属进行详细评估以及长期随访的重要性,因为显性临床疾病可能只是后续可能发生的其他潜在器官特异性自身免疫性疾病的冰山一角。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c697/6669299/d483feff7804/OMJ-D-12-00441-f1.jpg

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