87 participants of the German collaboratory study for children with phenylk
etonuria (PKU) were investigated in order to monitor effects of a low selen
ium (Se) supply. The low selenium intake results from the semisynthetic "PK
U-diet" which rs not supplemented with selenium. The influence of the low s
elenium state on different clinical and biochemical parameters was evaluate
d. Samples of 34 healthy children of matching age and sex (on a normal diet
) were analysed to achieve contemporary reference values.
All PKU children presented a low selenium state (low plasma, whole blood, a
nd hair Se values, reduced urinary selenium excretion, and decreased plasma
and erythrocyte glutathione peroxidase activity (GSH-Px A)) in comparison
with the healthy reference group (all figures p<0,001). The investigated he
matological and biochemical parameters were found to be within normal range
(except T-4). The aspartate amino transferase (ASAT) was found to be negat
ively correlated with plasma Se (ASAT / plasma Se: p=0,006). Plasma thyroxi
ne (T-4) was higher in PKU children compared with the reference group. This
hormone was inversely correlated with the selenium blood values of the PKU
children. (T-4/whole blood Se p=0,001). The somatic measurements showed a
negative standard deviation score (SDS) of the body height in the PKU child
ren compared with reference values. Despite the difference in Se supply the
infants did not present any specific Se deficiency symptoms. (C) 1999 Else
vier Science Inc.