Bedard Tanya, Lowry R Brian, Sibbald Barbara, Crawford Susan, Kiefer Gerhard N
Alberta Congenital Anomalies Surveillance System, Alberta Health, Alberta Children's Hospital, Calgary, Alberta, Canada.
Departments of Pediatrics and Medical Genetics, University of Calgary, Alberta Children's Hospital, Calgary, Alberta, Canada.
Am J Med Genet A. 2018 Jan;176(1):19-28. doi: 10.1002/ajmg.a.38513. Epub 2017 Nov 23.
There is a wide range of the proportion of congenital anomalies associated with limb deficiencies reported in the literature. This variation is primarily attributed to methodology and classification differences. The distribution of associated anomalies among cases with congenital limb deficiencies in Alberta born between January 1, 1980 and December 31, 2012 is described. Of the 170 cases identified, most were live born (75.3%), male (61.8%), had longitudinal limb deficiencies (78.8%), and had associated anomalies outside the musculoskeletal system (77.6%). Significant associations between the preaxial longitudinal group and the central nervous, gastrointestinal, and cardiovascular systems are reported as well as between the postaxial longitudinal group and congenital hip and foot anomalies. Probable and possible syndrome diagnoses are described for cases with recognized malformation patterns.
文献中报道的与肢体缺损相关的先天性异常比例范围很广。这种差异主要归因于方法学和分类的不同。本文描述了1980年1月1日至2012年12月31日在艾伯塔省出生的先天性肢体缺损病例中相关异常的分布情况。在确诊的170例病例中,大多数为活产儿(75.3%),男性(61.8%),患有肢体纵向缺损(78.8%),且伴有肌肉骨骼系统以外的相关异常(77.6%)。报告了轴前纵向组与中枢神经、胃肠和心血管系统之间以及轴后纵向组与先天性髋关节和足部异常之间存在显著关联。对具有公认畸形模式的病例描述了可能的和疑似的综合征诊断。