Microtia-anotia: a global review of prevalence rates.

Microtia-anotia: a global review of prevalence rates.
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DOI:
10.1002/bdra.20836
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发表时间:
2011-09
影响因子:
--
通讯作者:
Mastroiacovo, Pierpaolo
Mastroiacovo, Pierpaolo
中科院分区:
医学4区
文献类型:
--
作者:
Luquetti, Daniela Varela;Leoncini, Emanuele;Mastroiacovo, Pierpaolo

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很少有关于小耳-无耳症频率的已发表研究。利用来自世界各地出生缺陷监测项目的数据,我们对小耳畸形-无耳畸形的发生率进行了系统评价,以进一步探讨各国患病率的差异。对92个出生缺陷监测项目进行了评估,共8,917例小耳-无耳症。我们计算了每10,000例新生儿中每10,000例新生儿中小耳畸形-无耳畸形(I型至IV型)、小耳畸形(I型至III型)和无耳畸形(IV型)的患病率。患病率是按大的地理区域、种族/民族和监测方法计算的。小耳畸形-无耳畸形的总体患病率为2.06(CI:2.02-2.10),小耳畸形为1.55(CI:1.50-1.60),无耳畸形为0.36(CI:0.34-0.38)。在美洲、北方和亚洲、西班牙裔和亚洲人中以及在主动查明和以医院为基础的监测项目中观察到较高的患病率。我们观察到不同监测项目和国家内部小耳畸形-无耳畸形的患病率存在显着差异。这些结果必须谨慎解释,因为这种变异性可能主要由监测方法的差异来解释。然而,鉴于一些差异的幅度,也可能涉及其他因素。本研究通过对现有数据进行批判性分析,有助于了解小耳畸形-无耳畸形的患病率。此外,它支持需要一个编码系统,允许完整的小耳畸形-无耳畸形的表型表征,包括严重性和偏侧性;以及进一步研究其频率的变化与种族和民族。
There are few published studies on microtia-anotia frequency. Using data from birth defects surveillance programs around the world, we conducted a systematic review on the frequency of microtia-anotia to further explore the differences in prevalence across countries. Ninety-two birth defects surveillance programs were evaluated with a total of 8,917 cases of microtia-anotia. We computed the prevalence per 10,000 births for each surveillance program for total cases of microtia-anotia (microtia types I to IV), microtia (types I to III), and anotia (type IV). Prevalence ratios were calculated by large geographical areas, race/ethnicity, and by surveillance methodologies. The overall prevalence were for microtia-anotia 2.06 (CI: 2.02–2.10), for microtia 1.55 (CI: 1.50–1.60), and for anotia 0.36 (CI: 0.34–0.38). Higher prevalence was observed in the Americas, Northern Europe and Asia, among Hispanics and Asians, and among active ascertainment and hospital-based surveillance programs. We observed marked variation in the prevalence of microtia-anotia across surveillance programs and within countries. These results must be interpreted cautiously as this variability may be explained mainly by differences in surveillance methods. However, given the magnitude of some of the differences, other factors may also be involved. This study contributes to the knowledge on the prevalence of microtia-anotia by providing a critical analysis of the existing data. In addition, it supports the need for a coding system that allows complete phenotype characterization of microtia-anotia, including severity and laterality; as well as further studies on the variation of its frequency related to race and ethnicity.
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