一名内分泌科医生的 2 型自身免疫性多腺体综合征实践

S. Bulgakova, P. Y. Merzlova, O. A. Novikova, L. A. Sharonova, Y. Dolgikh, O. Kosareva
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引用次数: 0

摘要

自身免疫性多腺综合征是一组罕见的多器官病变,由自身免疫侵袭引起,以多形性内分泌和非内分泌器官病变为特征。根据自身免疫过程所涉及的器官组合,可将自身免疫性多器官综合征分为 4 种类型。1 型为常染色体隐性遗传,多见于儿童和青少年。2-4 型与 HLA 系统抗原的表达有关,通常表现为成年患者。本文简要介绍了所有类型的自身免疫性多腺综合征,更详细地介绍了第 2 型(施密特综合征),本文讨论的就是该型的临床病例。以下是一个临床病例:萨马拉市第五医院内分泌科对一名46岁男性患者的观察,该患者患有自身免疫性多腺综合征2型,并伴有肾上腺功能不全和甲状腺功能减退。患者提交的主诉、病史、实验室和仪器检查、抗体筛查结果证实了自身免疫性多腺综合征 2 型的诊断。进行调查以排除原发性肾上腺功能不全的其他病因。此外,还介绍了为确定自身免疫性多腺综合征 2 型的其他可能病因而进行的其他调查。介绍了根据国家临床建议进行的处方治疗,以及激素替代疗法处方的特点,进一步描述了门诊阶段的动态观察和实验室控制指标。对该病症的诊断和治疗中可能存在的困难做出了结论。
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Autoimmune polyglandular syndrome type 2 in the practice of an endocrinologist
Autoimmune polyglandular syndromes are a group of rare multi-organ pathologies resulting from autoimmune aggression and characterized by polymorphic endocrine and non-endocrine organ lesions. Depending on the combination of organs involved in the autoimmune process, there are 4 types of autoimmune polyglandular syndrome. Type 1 has an autosomal recessive type of inheritance, more common in children and adolescents. Types 2–4 are associated with the expression of antigens the HLA system and manifest typically in adult patients. The article provides a brief description of all types of autoimmune polyglandular syndromes, in more detail describes type 2 (Schmidt syndrome), the clinical case of which is addressed in this article. The following is a clinical case: observation of a 46-year-old male hospitalized in the endocrinological department of Samara City Hospital No. 5 with autoimmune polyglandular syndrome type 2 with decompensation of adrenal insufficiency and hypothyroidism. Submitted complaints, anamnesis, laboratory and instrumental examination of the patient, results of screening for the presence of antibodies that confirm the diagnosis of autoimmune polyglandular syndrome type 2. Surveys have been conducted to eliminate other causes of primary adrenal insufficiency. Additional surveys carried out to identify other possible components of autoimmune polyglandular syndrome type 2 are described. The prescribed treatment according to the nationalclinical recommendations, as well as the features of the prescription of hormone replacement therapy, described further dynamic observation at the outpatient stage and given laboratory control indicators. Conclusions are made about possible difficultiesin the diagnosis and treatment of this pathology.
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