The objectives of this study were to describe the birth prevalence of limb reduction defects (LRD) in Argentina, their clinical features, and to review the literature on this topic. The data source was the National Network of Congenital Anomalies of Argentina, a surveillance system that has been operative since 2009. Data were collected from November 1, 2009 to December 31, 2016. 1,663,610 births and 702 affected patients were registered during this period. The prevalence of LRD was 4.22/10,000 births (CI 95%: 3.93-4.54). In 15,094 stillbirths, prevalence was 30.80/10,000 (CI 95%: 22.31-40.65). Perinatal mortality (stillbirths plus early neonatal deaths) was 24.6%, mostly explained by postnatal deaths. LRD were classified according to different variables, including Gold's anatomic classification. Then, 41.0% of patients had transverse terminal defects and 50.2% had longitudinal defects. We found multiple and syndromic clinical presentation to be associated with both preaxial LRD and lethality. The prevalence of LRD was lower and perinatal mortality was higher in our study compared to that of previously published studies. Because there is heterogeneity in the inclusion and exclusion criteria among publications, a greater effort should be made in order to include similar populations and to use a unified anatomic classification and nomenclature.

中文翻译：

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阿根廷有160万人出生的先天性肢体减少缺陷。

这项研究的目的是描述阿根廷肢体减少缺陷（LRD）的出生率，其临床特征，并复习有关该主题的文献。数据来源是阿根廷国家先天性异常国家网络，该系统自2009年开始运行。从2009年11月1日至2016年12月31日收集数据。在此期间，登记了1,663,610例新生儿和702例患者。LRD的患病率为4.22 / 10,000出生（CI 95％：3.93-4.54）。在15,094个死胎中，患病率为30.80 / 10,000（CI 95％：22.31-40.65）。围产期死亡率（死产加新生儿早期死亡）为24.6％，主要由产后死亡引起。LRD根据不同的变量进行分类，包括Gold的解剖学分类。然后，41。0％的患者有横向末梢缺损，50.2％的患者有纵向缺损。我们发现多种和综合症的临床表现均与前轴LRD和致死率有关。与先前发表的研究相比，本研究中LRD的患病率较低，围产期死亡率较高。由于出版物之间的纳入和排除标准存在异质性，因此应做出更大的努力，以包括相似的人群并使用统一的解剖学分类和术语。