Lowry R B, Morgan K, Holmes T M, Gilroy S W
Am J Med Genet. 1985 Nov;22(3):545-52. doi: 10.1002/ajmg.1320220312.
The Alberta Provincial Congenital Anomaly (CA) Surveillance reporting forms were scanned for infants whose surnames and addresses identified them as belonging to the Hutterite Brethren. Death registrations (of infants up to 1 year) and stillbirth registrations were similarly scanned. While the overall percentage of total malformations (5%) and major malformations (2%) was no different from that of the total population of Alberta, closer examination of the actual entities showed a large number of monogenic disorders among the Hutterites. The frequency of multifactorial congenital anomalies was approximately 1%. The inbreeding coefficients, using a genealogic data base, were computed for each "case baby" and two Hutterite "control babies," the latter being births preceding and succeeding the case baby. There was no statistical difference in the distribution of inbreeding coefficients between the case and control groups. While the data are based on small numbers and therefore are preliminary, they suggest that the Hutterite lifestyle, of good nutrition (using largely home prepared foods), absence of tobacco and minimal alcohol consumption, may be one factor responsible for fewer multifactorial CAs whose occurrence is environmentally susceptible to such influences. Studies of populations with a low incidence of disorders are just as important as those with a high incidence.
对艾伯塔省先天性异常(CA)监测报告表进行了扫描,以查找姓氏和地址显示其属于哈特派兄弟会的婴儿。同样扫描了(1岁以下婴儿的)死亡登记和死产登记。虽然畸形总数(5%)和主要畸形(2%)的总体百分比与艾伯塔省总人口的百分比没有差异,但对实际病种的仔细检查显示,哈特派人群中存在大量单基因疾病。多因素先天性异常的发生率约为1%。利用一个家谱数据库,计算了每个“病例婴儿”以及两名哈特派“对照婴儿”(后者为病例婴儿出生前后的出生情况)的近亲系数。病例组和对照组的近亲系数分布没有统计学差异。虽然数据基于少量样本,因此只是初步的,但它们表明,哈特派良好的营养生活方式(主要食用自制食品)、不吸烟和极少饮酒,可能是多因素先天性异常较少的一个原因,这类异常的发生在环境上易受此类影响。对疾病发病率低的人群进行研究与对发病率高的人群进行研究同样重要。