Merck

[Maternal phenylketonuria].

Orvosi hetilap (2013-05-01)
János Bókay, Erika Kiss, Erika Simon, László Szőnyi
ABSTRACT

Elevated maternal phenylalanine levels during pregnancy are teratogenic, and may result in embryo-foetopathy, which could lead to stillbirth, significant psychomotor handicaps and birth defects. This foetal damage is known as maternal phenylketonuria. Women of childbearing age with all forms of phenylketonuria, including mild variants such as hyperphenylalaninaemia, should receive detailed counselling regarding their risks for adverse foetal effects, optimally before contemplating pregnancy. The most assured way to prevent maternal phenylketonuria is to maintain the maternal phenylalanine levels within the optimal range already before conception and throughout the whole pregnancy. Authors review the comprehensive programme for prevention of maternal phenylketonuria at the Metabolic Center of Budapest, they survey the practical approach of the continuous maternal metabolic control and delineate the outcome of pregnancies of mothers with phenylketonuria from the introduction of newborn screening until most recently.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
L-苯基丙氨酸, from non-animal source, meets EP, JP, USP testing specifications, suitable for cell culture, 98.5-101.0%
Sigma-Aldrich
L-苯基丙氨酸, reagent grade, ≥98%
Supelco
L-苯基丙氨酸, Pharmaceutical Secondary Standard; Certified Reference Material
Sigma-Aldrich
L-苯基丙氨酸, BioUltra, ≥99.0% (NT)
Sigma-Aldrich
L-苯基丙氨酸, 99%
Supelco
L-苯基丙氨酸, certified reference material, TraceCERT®
Supelco
L-苯基丙氨酸, analytical standard, for Nitrogen Determination According to Kjeldahl Method
Sigma-Aldrich
L-苯基丙氨酸, 99%, FCC
SAFC
L-苯基丙氨酸