caa a22 4500 475785762 CHVBK 20180406123657.0 cr unu---uuuuu 170329e20000901xx s 000 0 eng 10.1007/PL00014383 doi (NATIONALLICENCE)springer-10.1007/PL00014383 The international collaborative study of maternal phenylketonuria: status report 1998 [Elektronische Daten] [Richard Koch, Eva Friedman, Colleen Azen, William Hanley, Harvey Levy, Reuben Matalon, Bobbye Rouse, Fritz Trefz, Susan Waisbren, Kimberley Michals-Matalon, Phyllis Acosta, Fleming Güttler, Kurt Ullrich, Lawrence Platt, Felix de la Cruz] The Maternal Phenylketonuria Study began in 1984 and during the intervening years, 572 pregnancies in hyperphenylalaninemic women and 99 controls and their outcomes have been evaluated. Among hyperphenylalaninemic women who delivered a live infant, only 15.9% were treated and in metabolic control preconceptually, however, another 18.4% were in control by 10 weeks. Compared to the results reported by Lenke and Levy in 1980, there is a marked improvement in outcome with treatment. Microcephaly was unusual in preconceptually treated pregnancies with well controlled phenylalanine restricted diets. Even in pregnancies that established control after conception but before the 8th week, congenital heart disease did not occur in the offspring, however, it did occur in 12% of pregnancies not achieving control until after 10 weeks of pregnancy. Conclusion The recommended level of blood phenylalanine during pregnancy is 120-360 μmol/l. Best results were obtained by close cooperation between the attending obstetrician and a metabolic team experienced in the care of persons with phenylketonuria. Springer-Verlag Berlin Heidelberg, 2000 Key words Maternal phenylketonuria nationallicence Phenylalanine nationallicence Collaborative study nationallicence AbbreviationsCHD congenital heart disease nationallicence MPKUCS collaborative study on maternal phenylketonuria nationallicence Phe phenylalanine nationallicence PKU phenylketonuria nationallicence Koch Richard PKU #73, Childrens Hospital Los Angeles, 4650 Sunset Blvd., Los Angeles, CA 90027, USA, US aut Friedman Eva Childrens Hospital Los Angeles, Division of Medical Genetics and University of Southern California, Department of Pediatrics, Los Angeles, CA 90027, USA, US aut Azen Colleen Childrens Hospital Los Angeles, Division of Medical Genetics and University of Southern California, Department of Pediatrics, Los Angeles, CA 90027, USA, US aut Hanley William PKU Program, The Hospital for Sick Children, Toronto, Ontario M5G1X8, Canada, CA aut Levy Harvey Childrens Hospital and Medical Center, Boston, MA 02115, USA, US aut Matalon Reuben Child Development Division, University of Texas, Medical Branch, Galveston, TX 77550, USA, US aut Rouse Bobbye Child Development Division, University of Texas, Medical Branch, Galveston, TX 77550, USA, US aut Trefz Fritz Children's Hospital, Reutlingen, Germany, DE aut Waisbren Susan Childrens Hospital and Medical Center, Boston, MA 02115, USA, US aut Michals-Matalon Kimberley Child Development Division, University of Texas, Medical Branch, Galveston, TX 77550, USA, US aut Acosta Phyllis Ross Laboratories, Columbus, OH 43215, USA, US aut Güttler Fleming The John F. Kennedy Institute, Glostrup, Denmark, DK aut Ullrich Kurt Department of Paediatrics, University Hospital, Hamburg, Germany, DE aut Platt Lawrence Cedars-Sinai Medical Center, Department of Obstetrics and Gynecology, UCLA Medical Center, Los Angeles, CA 90048, USA, US aut de la Cruz Felix National Institute of Child Health and Human Development, Bethesda, MD 20892, USA, US aut https://doi.org/10.1007/PL00014383 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/PL00014383 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Koch Richard PKU #73, Childrens Hospital Los Angeles, 4650 Sunset Blvd., Los Angeles, CA 90027, USA, US aut NATIONALLICENCE 700 1- Friedman Eva Childrens Hospital Los Angeles, Division of Medical Genetics and University of Southern California, Department of Pediatrics, Los Angeles, CA 90027, USA, US aut NATIONALLICENCE 700 1- Azen Colleen Childrens Hospital Los Angeles, Division of Medical Genetics and University of Southern California, Department of Pediatrics, Los Angeles, CA 90027, USA, US aut NATIONALLICENCE 700 1- Hanley William PKU Program, The Hospital for Sick Children, Toronto, Ontario M5G1X8, Canada, CA aut NATIONALLICENCE 700 1- Levy Harvey Childrens Hospital and Medical Center, Boston, MA 02115, USA, US aut NATIONALLICENCE 700 1- Matalon Reuben Child Development Division, University of Texas, Medical Branch, Galveston, TX 77550, USA, US aut NATIONALLICENCE 700 1- Rouse Bobbye Child Development Division, University of Texas, Medical Branch, Galveston, TX 77550, USA, US aut NATIONALLICENCE 700 1- Trefz Fritz Children's Hospital, Reutlingen, Germany, DE aut NATIONALLICENCE 700 1- Waisbren Susan Childrens Hospital and Medical Center, Boston, MA 02115, USA, US aut NATIONALLICENCE 700 1- Michals-Matalon Kimberley Child Development Division, University of Texas, Medical Branch, Galveston, TX 77550, USA, US aut NATIONALLICENCE 700 1- Acosta Phyllis Ross Laboratories, Columbus, OH 43215, USA, US aut NATIONALLICENCE 700 1- Güttler Fleming The John F. Kennedy Institute, Glostrup, Denmark, DK aut NATIONALLICENCE 700 1- Ullrich Kurt Department of Paediatrics, University Hospital, Hamburg, Germany, DE aut NATIONALLICENCE 700 1- Platt Lawrence Cedars-Sinai Medical Center, Department of Obstetrics and Gynecology, UCLA Medical Center, Los Angeles, CA 90048, USA, US aut NATIONALLICENCE 700 1- de la Cruz Felix National Institute of Child Health and Human Development, Bethesda, MD 20892, USA, US aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer