The International Collaborative Study on maternal phenylketonuria: Organization, study design and description of the sample

被引:16
作者
Friedman, EG
Koch, R
Azen, C
Levy, H
Hanley, W
Matalon, R
Rouse, B
Trefz, F
delaCruz, F
机构
[1] CHILDRENS HOSP,DIV MED GENET,LOS ANGELES,CA 90027
[2] UNIV SO CALIF,DEPT PEDIAT,LOS ANGELES,CA 90089
[3] BOSTON CHILDRENS HOSP,BOSTON,MA
[4] HOSP SICK CHILDREN,TORONTO,ON M5G 1X8,CANADA
[5] MIAMI CHILDRENS HOSP,RES INST,MIAMI,FL
[6] UNIV TEXAS,MED BRANCH,GALVESTON,TX 77550
[7] UNIV TUBINGEN,TUBINGEN,GERMANY
[8] NICHHD,BETHESDA,MD 20892
关键词
maternal phenylketonuria; indices of dietary control; phenylalanine; pregnancy; microcephaly;
D O I
10.1007/PL00014236
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
The International Maternal Phenylketonuria (PKU) Collaborative Study commenced in 1984 to evaluate the efficacy of the low-phenylalanine diet in reducing the morbidity associated with maternal PKU syndrome. Four hundred and sixty eight (468) pregnancies resulted in 331 live births, 3 stillbirths, 61 spontaneous abortions, 2 ectopic pregnancies and 71 elective terminations. Since its inception, the study has steadily progressed toward its goal of diet initiation preconception or early in pregnancy. By 1994, 51% of the sample began the diet preconceptionally, with an additional 41% doing so by 8 weeks gestation. The number of adolescent pregnancies has decreased from 31% to 9%, college attendance has increased from 5% to 16%, number of women in the lowest socioeconomic classes has decreased from 95% to 59% and average IQ has increased from 78 to 88, The organization of the network of 130 referral centers and clinics within the U.S.A., Canada and Germany and the objectives of the scientific research investigation have served to provide a derived benefit of outreach, education, reproductive counseling and early diet intervention in a large cohort of PKU women.
引用
收藏
页码:S158 / S161
页数:4
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