国际内分泌代谢杂志
國際內分泌代謝雜誌
국제내분비대사잡지
INTERNATIONAL JOURNAL OF ENDOCRINOLOGY AND METABOLISM
2011年
1期
62-65
,共4页
自身免疫%多内分泌腺病%人白细胞抗原%自身免疫调节基因
自身免疫%多內分泌腺病%人白細胞抗原%自身免疫調節基因
자신면역%다내분비선병%인백세포항원%자신면역조절기인
Autoimmunity%Polyendocrinopathy%Human lymphocyte antigen%Autoimmune regulatory gene
自身免疫性多内分泌腺病综合征(APS)是指由自身免疫引起的多内分泌腺功能受损为主要表现的系列综合征,可分为APS Ⅰ型和APSⅡ型.二者的疾病组成成分和发病机制有显著区别.APS Ⅰ型是由位于21号染色体上的自身免疫调节基因(AIRE)的突变导致,所以用基因检测的.方法 即可确诊,而APSⅡ型为多基因遗传病,遗传性状表现与人白细胞抗原(HLA)的表型有关,但HLA只决定其易患性,并非致病的直接原因,APSⅡ型的发病还与诸多后天因素有关.现对两型APS的流行病学、病因、发病机制、临床表现和治疗进行综述,以加强临床对APS的筛查和随访.
自身免疫性多內分泌腺病綜閤徵(APS)是指由自身免疫引起的多內分泌腺功能受損為主要錶現的繫列綜閤徵,可分為APS Ⅰ型和APSⅡ型.二者的疾病組成成分和髮病機製有顯著區彆.APS Ⅰ型是由位于21號染色體上的自身免疫調節基因(AIRE)的突變導緻,所以用基因檢測的.方法 即可確診,而APSⅡ型為多基因遺傳病,遺傳性狀錶現與人白細胞抗原(HLA)的錶型有關,但HLA隻決定其易患性,併非緻病的直接原因,APSⅡ型的髮病還與諸多後天因素有關.現對兩型APS的流行病學、病因、髮病機製、臨床錶現和治療進行綜述,以加彊臨床對APS的篩查和隨訪.
자신면역성다내분비선병종합정(APS)시지유자신면역인기적다내분비선공능수손위주요표현적계렬종합정,가분위APS Ⅰ형화APSⅡ형.이자적질병조성성분화발병궤제유현저구별.APS Ⅰ형시유위우21호염색체상적자신면역조절기인(AIRE)적돌변도치,소이용기인검측적.방법 즉가학진,이APSⅡ형위다기인유전병,유전성상표현여인백세포항원(HLA)적표형유관,단HLA지결정기역환성,병비치병적직접원인,APSⅡ형적발병환여제다후천인소유관.현대량형APS적류행병학、병인、발병궤제、림상표현화치료진행종술,이가강림상대APS적사사화수방.
Autoimmune polyendocrinopathy syndrome (APS)is a multiple endocrine dysfunction caused by autoimmunity disorders. It can commonly be divided into two types,including APS type Ⅰ and APS type Ⅱ. There are significant differences in composition and pathogenesis of these two types. APS Ⅰ is caused by mutations of the autoimmune regulatory (AIRE) gene on chromosome 21 and genetic screening may identify patients with APS Ⅰ. For APS Ⅱ , the susceptibility are often related with human lymphocyte antigen (HLA) genes which only indicated the increased risk for developing autoimmune disorders. The occurence of APS Ⅱ is also influenced by many other factors. Here discusses the epidemiology, etiological factors,pathogenesis, clinical signs and symptoms and treatment of APS, so as to enhance the screening and follow-up of APS in clinical investigations.
