Junctional Epidermolysis Bullosa Incidence and Survival: 5-Year Experience of the Dystrophic Epidermolysis Bullosa Research Association of America ( DebRA) Nurse Educator, 2007 to 2011
Junctional Epidermolysis Bullosa Incidence and Survival: 5-Year Experience of the Dystrophic Epidermolysis Bullosa Research Association of America ( DebRA) Nurse Educator, 2007 to 2011
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DOI：
10.1111/pde.12157
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发表时间：
2014-03-01
影响因子：
1.5
通讯作者：
Lucky, Anne W.
中科院分区：
文献类型：
--
作者：
Kelly-Mancuso, Geraldine;Kopelan, Brett;Lucky, Anne W.
接合性大疱性表皮松解症（JEB）是一种破坏性特别强的大疱性表皮松解症，尤其发生在新生儿时期。有关美国乙脑新发病例数的数据来自美国大疱性营养不良表皮松解症研究协会（DebRA）护士教育工作者的记录。据报道，在2007年至2011年的五年间，有71名患有JEB的儿童出生，反映了每年至少3.59 /百万人的发病率，显著高于先前估计的2.04% /百万人。发病率和婴儿死亡率很高，至少达到73%，截至2012年6月，71例病例中有52例死亡。这些数据强调了未来研究开发治疗方法并最终治愈这种疾病的必要性。
Junctional epidermolysis bullosa (JEB) is a particularly devastating type of epidermolysis bullosa, especially in the newborn period. Data about the number of new cases of JEB in the United States were collected from the records of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) nurse educator. Seventy-one children with JEB were reported to have been born in the 5years between 2007 and 2011, reflecting an incidence of at least 3.59 per million per year, significantly higher than previously estimated (2.04 per million). There was a high prevalence of morbidity and infant mortality of at least 73%, as 52 of the 71 cases proved fatal by June 2012. These data emphasize the need for future research to develop treatment and ultimately a cure for this disorder.