David T J, McCrae F C, Bound J P
J Med Genet. 1983 Oct;20(5):338-41. doi: 10.1136/jmg.20.5.338.
The necropsy reports of 174 cases of anencephaly, born in the Fylde peninsula of Lancashire between 1957 and 1980, have been analysed for the presence of other malformations. The results were compared with a similar previous series from Bristol, though the Bristol study differed both in time (1948 to 1975) and in the fact that it was hospital based and, unlike the present study, did not achieve near complete ascertainment. Of the Lancashire anencephalics, 24% had other malformations, a significantly lower rate than in the Bristol series. There was a much higher rate of renal and urinary tract defects in the Bristol series, and a higher rate of cardiovascular defects in the Lancashire series. The distribution of associated malformations differed in the two areas, possibly representing different patterns of aetiological heterogeneity. The iniencephaly rate was so much lower in Lancashire as to suggest an artefact, perhaps owing to the lack of a precise definition of the condition.
对1957年至1980年间出生在兰开夏郡菲尔德半岛的174例无脑儿尸检报告进行分析，以确定是否存在其他畸形。将结果与之前布里斯托尔的类似系列研究进行比较，尽管布里斯托尔的研究在时间上（1948年至1975年）有所不同，而且是基于医院的研究，与本研究不同的是，该研究没有实现近乎完全的病例确定。在兰开夏郡的无脑儿中，24%有其他畸形，这一比例明显低于布里斯托尔系列研究中的比例。布里斯托尔系列研究中肾脏和泌尿系统缺陷的发生率要高得多，而兰开夏郡系列研究中心血管缺陷的发生率更高。两个地区相关畸形的分布不同，这可能代表了病因异质性的不同模式。兰开夏郡的枕颈裂畸形率低得多，这表明可能存在人为因素，也许是由于对该病症缺乏精确的定义。
