Decouflé P, Boyle C A, Paulozzi L J, Lary J M
National Center on Birth Defects and Developmental Disabilities Centers for Disease Control and Prevention, Atlanta, Georgia 30341, USA.
Pediatrics. 2001 Sep;108(3):728-34. doi: 10.1542/peds.108.3.728.
We sought to quantify the strength of associations between each of four specific developmental disabilities (DDs) and specific types of major birth defects.
We linked data from 2 independent surveillance systems, the Metropolitan Atlanta Congenital Defects Program and the Metropolitan Atlanta Developmental Disabilities Surveillance Program. Children with major birth defects (n = 9142; born 1981-1991 in metro Atlanta) and 3- to 10-year-old children who were born between 1981 and 1991 in metro Atlanta and identified between 1991 and 1994 as having mental retardation, cerebral palsy, hearing impairment, or vision impairment (n = 3685) were studied. Prevalence ratio (PR), which is the prevalence of a DD in children with 1 or more major birth defects divided by the prevalence of the same DD in children without major birth defects, was measured.
Among the 9142 children who were born with a major birth defect, 657 (7.2%) had a serious DD compared with 0.9% in children with no major birth defect, yielding a PR of 8.3 (95% confidence interval: 7.6-9.0). In general, the more severe the DD, the higher was the PR. Birth defects that originated in the nervous system and chromosomal defects resulted in the highest PRs for a subsequent DD. For all other categories of birth defects, PRs were lowest when all major birth defects present were confined to a single category (ie, isolated defects). PRs for any DD increased monotonically with the number of coded birth defects per child or the number of different birth defect categories per child, regardless of the severity of the defect or whether defects of the nervous system, chromosomal defects, or "other syndromes" were counted.
These data highlight the possible early prenatal origins of some DDs and suggest that both the number of coded birth defects present and the number of anatomic systems involved are strongly related to functional outcomes.
我们试图量化四种特定发育障碍（DDs）与特定类型的主要出生缺陷之间关联的强度。
我们将来自两个独立监测系统的数据进行了关联，即大亚特兰大先天性缺陷项目和大亚特兰大发育障碍监测项目。研究对象包括患有主要出生缺陷的儿童（n = 9142；1981 - 1991年出生于大亚特兰大）以及1981年至1991年出生于大亚特兰大、在1991年至1994年期间被确定为患有智力障碍、脑瘫、听力障碍或视力障碍的3至10岁儿童（n = 3685）。测量了患病率比（PR），即患有1种或更多主要出生缺陷的儿童中发育障碍的患病率除以无主要出生缺陷儿童中同一发育障碍的患病率。
在9142名患有主要出生缺陷的儿童中，657名（7.2%）患有严重发育障碍，而无主要出生缺陷儿童中的患病率为0.9%，患病率比为8.3（95%置信区间：7.6 - 9.0）。一般来说，发育障碍越严重，患病率比越高。起源于神经系统的出生缺陷和染色体缺陷导致后续发育障碍的患病率比最高。对于所有其他类别出生缺陷，当所有存在的主要出生缺陷局限于单一类别（即孤立缺陷）时，患病率比最低。无论缺陷的严重程度如何，也无论是否将神经系统缺陷、染色体缺陷或“其他综合征”计算在内，任何发育障碍的患病率比都随着每个儿童编码的出生缺陷数量或每个儿童不同出生缺陷类别的数量单调增加。
这些数据突出了某些发育障碍可能的早期产前起源，并表明存在的编码出生缺陷数量和涉及的解剖系统数量都与功能结局密切相关。
