van der Pal-de Bruin K M, Buitendijk S E, Hirasing R A, den Ouden A L
TNO Preventie en Gezondheid, divisie Jeugd, CE Leiden.
Ned Tijdschr Geneeskd. 2000 Sep 2;144(36):1732-6.
To describe the incidence and the expected clinical picture of neural tube defects (NTD) in years when periconceptional folic acid use increased.
Descriptive.
Data on the prevalence of NTD in the Netherlands in 1994-1998 and on the prognosis at the time of the report obtained from the Nederlands Signalerings Centrum Kindergeneeskunde (Netherlands Paediatric Spotting Centre), where all practising physicians in the Netherlands monthly report children in whom a rare disease has been diagnosed. Prevalence figures from before and after 1996 were compared, because periconceptional use of folic acid increased since late 1996.
In 1994-1998, 414 children with NTD were reported, 164 boys, 191 girls and 59 unreported. The prevalence of NTD was 4.6 per 10,000 live births (95% confidence interval (CI): 3.7-5.6) compared with 3.8 per 10,000 live births (95% CI: 2.9-4.6) in the period 1997-1998. Of the 414 children, 257 had a meningomyelocele; the early mortality in this group amounted to 37%. Hydrocephalus was found in 84.8% of the infants, 40.9% of the infants were believed never be able to sit, stand, and walk.
The prevalence of NTD was lower in the period 1997-1998 than in the period 1994-1996 although the difference was not statistically significant.
描述围孕期叶酸使用增加年份中神经管缺陷（NTD）的发病率及预期临床症状。
描述性研究。
从荷兰儿科监测中心获取1994 - 1998年荷兰神经管缺陷患病率数据以及报告时的预后情况，荷兰所有执业医师每月向该中心报告诊断出罕见疾病的儿童。比较了1996年前后的患病率数据，因为自1996年末起围孕期叶酸使用有所增加。
1994 - 1998年，共报告414例神经管缺陷患儿，其中164例为男孩，191例为女孩，59例未报告性别。神经管缺陷的患病率为每10000例活产儿中有4.6例（95%置信区间（CI）：3.7 - 5.6），而1997 - 1998年期间为每10000例活产儿中有3.8例（95% CI：2.9 - 4.6）。在这414例患儿中，257例患有脊髓脊膜膨出；该组早期死亡率为37%。84.8%的婴儿有脑积水，40.9%的婴儿被认为永远无法坐、站和行走。
1997 - 1998年期间神经管缺陷的患病率低于1994 - 1996年期间，尽管差异无统计学意义。
