Epidemiology and clinical aspects of Adamantiades-Behcet disease in Gemany. Current data
Epidemiology and clinical aspects of Adamantiades-Behcet disease in Gemany. Current data
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DOI：
10.1007/s00347-012-2601-4
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发表时间：
2012-06-01
期刊：
影响因子：
--
通讯作者：
Zouboulis, C. C.
中科院分区：
文献类型：
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作者：
Altenburg, A.;Mahr, A.;Zouboulis, C. C.
在德国Adamantiades-Beh双匕首病登记处（登记的慈善机构）记录的721名患者中，258名为德国人，308名为土耳其人，沿着还有30个其他国家的原籍国。德国的患病率为0.9：100，000。该疾病的表现主要是在30岁（中位年龄：26.5岁），10.7%的患者发病年龄在16岁以下。平均2.9年（中位数3个月）形成完整的临床症状。土耳其血统的患者表现出男性化，而德国血统的患者（女性：男性1.9：1），这也是在整个集体中检测到的比例为1.4：1。12.4%的患者有家族史，德国和土耳其患者之间存在差异（3.8% vs 14.6%），年轻和成年患者之间也存在差异（25.0% vs 7.3%）。最常见的特征包括口腔口疮（98.5%）、皮肤病变（81%）、生殖器溃疡（64.7%）、眼部表现（51.6%）、关节炎（52.4%）和病理学试验阳性（30.8%）。与德国人相比，土耳其患者的眼部表现明显更常见，而在德国患者中，前列腺炎/附睾炎和胃肠道受累更常见。严重并发症包括失明6.8%，脑膜脑炎4.0%，严重关节炎2.6%，死亡1.2%，咯血1.1%，胃肠道穿孔0.5%。HLA-B5抗原阳性率为58.1%，并与眼部表现有关。德国人（6.57）和土耳其人（5.81）中HLA-B5阳性个体的相对风险较高。
Of the 721 documented patients in the German Registry for Adamantiades-Beh double dagger et disease (registered charity), 258 were of German and 308 of Turkish descent, along with 30 other countries of origin. The prevalence in Germany is 0.9:100,000. Manifestation of the disease was predominantly in the third decade of life (median age: 26.5 years) and in 10.7% the disease onset was under 16 years of age. The full clinical picture developed on average in 2.9 years (median 3 months). Patients of Turkish descent showed androtopism in contrast to those of German descent (female:male 1.9:1), which was also detected in the whole collective in a ratio of 1.4:1. In 12.4% there was a family history with differences between German and Turkish patients (3.8% versus 14.6%) as well as in patients with disease onset in young and adult age (25.0% versus 7.3%). Most frequent features included oral aphthae (98.5%), cutaneous lesions (81%), genital ulcers (64.7%), ocular manifestations (51.6%), arthritis (52.4%) and positive pathergy test (30.8%). Turkish patients suffered significantly more often from eye manifestations compared with Germans, while in German patients prostatitis/epididymitis and gastrointestinal involvement were more frequently documented. As serious complications arose blindness 6.8%, meningoencephalitis 4.0%, severe arthritis 2.6%, fatal outcome 1.2%, hemoptysis 1.1% and gastrointestinal perforation 0.5%. The HLA-B5 antigen was positive in 58.1% and showed an association with eye manifestations. The relative risk of HLA-B5 positive individuals is high in both Germans (6.57) and Turks (5.81).