Electronic Resource
Springer
European journal of pediatrics
155 (1996), S. S177
ISSN:
1432-1076
Keywords:
Key words Phenylketonuria
;
Pregnancy outcome
;
Maternal
;
phenylketonuria
;
Griffiths
;
Developmental Quotient
;
McCarthy
;
General Cognitive Index
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract By November 1994, 39 pregnancies had been completed in phenylketonuric mothers. Dietary control was post-conception in 6 and 2 of these offspring died of congenital heart disease and 1 other needed surgery for coarctation. There were no heart defects in the 34 offspring of the 33 pregnancies following pre-conception diet controlled by Guthrie assays of maternal phenylalanine three (Phe) weekly. These Phe results were analysed by trimester for the means, the number of days over 300 μmol/l or below 60 μmol/l. Generally good control was achieved suggesting the UK guidelines drawn up by the MRC Working Party are broadly achievable but excessively high and low values occur intermittently in many pregnancies both of which may adversely affect the fetus. Though developmental assessment scores at 1 year were over 100 in all but five, early outcome results suggest that intellectual development may still be impaired at 4 years. Until much more information is available caution is still needed in discussing outcome with phenylketonuria patients who wish to conceive.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/PL00014242
Permalink
| Library |
Location |
Call Number |
Volume/Issue/Year |
Availability |
