Prevalence of anemia in patients with epidermolysis bullosa registered in Australia.
Prevalence of anemia in patients with epidermolysis bullosa registered in Australia.
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DOI：
10.1016/j.ijwd.2014.12.001
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发表时间：
2015-02
影响因子：
--
通讯作者：
Murrell DF
中科院分区：
文献类型：
--
作者：
Hwang SJE;Daniel BS;Fergie B;Davey J;Murrell DF
贫血是大疱性表皮病（EB）的常见并发症。迄今为止，没有广泛的数据，在EB患者的贫血患病率已在世界范围内得到很好的表征。通过进行回顾性横断面研究，确定并描述澳大利亚EB人群中贫血的患病率。对2006年至2012年在澳大利亚大疱性表皮松解登记处（AEBR）登记的所有（n = 368）EB患者的贫血病理学证据进行了审查。没有贫血的EB患者和没有血液学参数的患者被排除在研究之外。患者的详细资料分为儿童（< 18岁）和成人（≥ 18岁）男性和女性亚组。368例EB患者中有169例有合格的血液结果进行分析，因为轻度EB没有常规的实验室检测; 27.8%（n = 47/169）的EB患者在其一生中的任何时间点都有贫血。所有泛发性重度交界型EB（JEB-GS）病例（100%，n = 4/4）、68.0%（n = 17/25）的隐性营养不良型EB（RDEB）和37.5%（n = 6/16）的泛发性中间型JEB（JEB-I）患者均贫血。由于EB是一种孤儿病，有限的样本量可能影响了研究结果的显著性。在我们的队列中，RDEB和JEB全身性重度（JEB-GS）患者中贫血的高患病率与病例系列中报告的相似。
Anemia is a common complication of epidermolysis bullosa (EB). To date, no extensive data on the prevalence of anemia in EB patients have been well characterized worldwide. To determine and to characterize the prevalence of anemia in the Australian EB population by conducting a retrospective cross-sectional study. All (n = 368) EB patients registered in the Australasian Epidermolysis Bullosa Registry (AEBR) from 2006 to 2012 were reviewed for pathological evidence of anemia. Patients with EB without anemia and those without hematological parameters were excluded from the study. Patients’ particulars were separated into pediatric (< 18 years old) and adult (≥ 18 years old) male and female subgroups. One-hundred sixty-nine out of 368 EB patients had eligible blood results to be analyzed, as milder forms of EB did not routinely have laboratory testing; 27.8% (n = 47/169) of EB patients were anemic at any time point in their lifetime. All generalized severe junctional EB (JEB-GS) cases (100%, n = 4/4); 68.0% (n = 17/25) of recessive dystrophic EB (RDEB); and 37.5% (n = 6/16) of generalized intermediate JEB (JEB-I) patients were anemic. As EB is an orphan disease, the limited sample size may have affected the significance of the study result. The high prevalence of anemia seen in RDEB and JEB generalized severe (JEB-GS) patients in our cohort is similar to those reported in case series.