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Autoimmune polyglandular diseases.
Best Practice & Research Clinical Endocrinology & Metabolism ( IF 7.4 ) Pub Date : 2019-10-04 , DOI: 10.1016/j.beem.2019.101344
George J Kahaly 1 , Lara Frommer 1
Affiliation  

Autoimmune polyglandular diseases (APD) are defined as the presence of two autoimmune -induced endocrine failures. With respect to the significant morbidity and potential mortality of APD, the diagnostic objective is to detect APD at an early stage, with the advantage of less frequent complications, effective therapy and better prognosis. This requires that patients at risk be regularly screened for subclinical endocrinopathies prior to clinical manifestation. Regarding the time interval between manifestation of first and further endocrinopathies, regular and long-term follow-up is warranted. Quality of life and psychosocial status are poor in APD patients and involved relatives. Familial clustering is high in patients with APD. Considering the high incidence of one or more endocrinopathies in first-degree relatives of patients with APD, family members should be regularly screened since they may also develop autoimmune endocrinopathies. Multidisciplinary management of these multiplex families in specialized centers is warranted.

中文翻译:

自身免疫性多腺疾病。

自身免疫性多腺疾病(APD)定义为存在两种自身免疫性内分泌衰竭。对于APD的高发病率和潜在死亡率,诊断目标是尽早发现APD,其并发症少,治疗有效,预后更好。这就要求在临床表现之前定期对有风险的患者进行亚临床内分泌病变筛查。关于首次内分泌病和进一步内分泌病之间的时间间隔,有规律和长期的随访是必要的。APD患者和相关亲属的生活质量和社会心理状况较差。APD患者的家族聚集度很高。考虑到APD患者一级亲属中一种或多种内分泌病变的发生率很高,家庭成员应定期进行筛查,因为他们也可能会发展自身免疫性内分泌病。必须在专门中心对这些多元化家庭进行多学科管理。
更新日期:2019-10-04
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