Suppr超能文献

一名15岁患有IIIc型抗磷脂综合征的女孩,胸腺切除术后重症肌无力缓解12个月。

15-Year old girl with APS type IIIc, 12 months post-thymectomy remission of myasthenia.

作者信息

Jamiołkowska Milena, Bossowski Artur

机构信息

Department of Pediatrics, Endocrinology and Diabetes with a Cardiology Unit, Medical University in Bialystok, Poland.

出版信息

Pediatr Endocrinol Diabetes Metab. 2017;23(1):49-55. doi: 10.18544/PEDM-23.01.0074.

Abstract

Polyglandular autoimmune syndromes (PAS) is a group of heterogenous conditions characterized by the association of at least two organ-specific autoimmune disorders, concerning both endocrine and non-endocrine organs. Type III is defined as the combination of autoimmune thyroid disease and other autoimmune condition (other than Addison's disease) and is divided into four subtypes. We describe a teenage female patient - with the family history of autoimmune diseases, who has simultaneously developed the symptoms of autoimmune thyroid disease with the clinical picture of hyperthyroidism and myasthenia gravis at the age of fifteen. Graves' disease was diagnosed approximately 2 months before myasthenia. Co-occurance of those two diseases allowed us to diagnose PAS type IIIC, however it caused specific diagnostic and treatment difficulties. Furthermore, several months after the diagnosis the patient was found to be GAD-Ab positive, whilst the glycaemic control remained normal. No evidence of other autoimmune conditions was observed. This patient received the standard GD and MG treatment. When the CT scan revealed thymus enlargement, thymectomy was performed. After the surgery we have observed not only remission of MG, but also a significant decrease of TRAb as well, that lasted for a year after the thymectomy. Our patient's case suggests that in patients diagnosed with PAS, the organ-specific Ab screening can help identify other latent and subclinical autoimmune diseases before clinical symptoms develop. The achievement of post-thymectomy remission of both MG and GD may indicate a close immunological relationship between PAS components.

摘要

多腺体自身免疫综合征(PAS)是一组异质性疾病,其特征是至少两种器官特异性自身免疫性疾病相关联,涉及内分泌和非内分泌器官。III型定义为自身免疫性甲状腺疾病与其他自身免疫性疾病(Addison病除外)的组合,并分为四个亚型。我们描述了一名有自身免疫性疾病家族史的青少年女性患者,她在15岁时同时出现了自身免疫性甲状腺疾病的症状,伴有甲状腺功能亢进和重症肌无力的临床表现。Graves病在重症肌无力诊断前约2个月被诊断出来。这两种疾病的同时出现使我们能够诊断出IIIC型PAS,但这也带来了特定的诊断和治疗困难。此外,诊断后几个月发现患者谷氨酸脱羧酶抗体(GAD-Ab)呈阳性,而血糖控制仍正常。未观察到其他自身免疫性疾病的证据。该患者接受了标准的Graves病和重症肌无力治疗。当CT扫描显示胸腺肿大时,进行了胸腺切除术。手术后,我们不仅观察到重症肌无力缓解,促甲状腺素受体抗体(TRAb)也显著下降,胸腺切除术后持续了一年。我们患者的病例表明,在诊断为PAS的患者中,器官特异性抗体筛查有助于在临床症状出现之前识别其他潜在和亚临床自身免疫性疾病。胸腺切除术后重症肌无力和Graves病均缓解,这可能表明PAS各组成部分之间存在密切的免疫关系。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验