Autosomal dominant hyper-IgE syndrome

Autosomal dominant hyper-IgE syndrome
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常染色体显性高 IgE 综合征

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2020
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常染色体显性高IgE综合征(AD-HIES),以前称为Job综合征,是一种影响多个身体系统,特别是免疫系统的疾病。复发性感染在患有这种疾病的人中很常见。受影响的人往往有频繁发作的肺炎,这是由某些类型的细菌感染肺部和引起炎症。炎症是免疫系统对损伤和外来入侵者(如细菌)的正常反应。然而,过度的炎症会损害身体组织。复发性肺炎通常会导致肺部形成充满空气的囊肿(肺囊肿)。频繁的皮肤感染和称为湿疹的炎症性皮肤病在AD-HIES中也很常见。这些皮肤问题引起皮疹、水泡、脓液积聚(脓液)、开放性溃疡和鳞屑。
Autosomal dominant hyper-IgE syndrome (AD-HIES), formerly known as Job syndrome, is a condition that affects several body systems, particularly the immune system. Recurrent infections are common in people with this condition. Affected individuals tend to have frequent bouts of pneumonia, which are caused by certain kinds of bacteria that infect the lungs and cause inflammation. Inflammation is a normal immune system response to injury and foreign invaders (such as bacteria). However, excessive inflammation can damage body tissues. Recurring pneumonia often results in the formation of air-filled cysts (pneumatoceles) in the lungs. Frequent skin infections and an inflammatory skin disorder called eczema are also very common in AD-HIES. These skin problems cause rashes, blisters, accumulations of pus (abscesses), open sores, and scaling.
DOI: 10.1016/j.jaci.2015.11.024
发表时间: 2016-07
期刊: The Journal of allergy and clinical immunology
影响因子: --
作者:
Hox V;O'Connell MP;Lyons JJ;Sackstein P;Dimaggio T;Jones N;Nelson C;Boehm M;Holland SM;Freeman AF;Tweardy DJ;Olivera A;Metcalfe DD;Milner JD
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