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Biomedical subjects

I Graustein

Publications and source records attributed to I Graustein.

9 recordsLinked to original sources
PubMed1990

[Effect of dietary non-compliance on development in phenylketonuria--studies of 14-year-old patients].

25 children with early treated PKU were studied at the age of 14 years. The IQ was higher at the age of 6-8, 10 and 14 years if the dietary control was good (75% of the control values up to 10 mg/dl) compared to children with poor control. The IQ however decreased up to the age of 14 years in both groups. Discontinuation of the diet in children with a good dietary control at the age of 6 years because of a normal EEG after phenylalanine loading causes a decrease of the IQ from 100 at 6 years to 90 at 10 years. The IQ remains stable thereafter up to 14 years. The IQ ist lower at the age of 7 or 8 years in those children in whom the discontinuation of diet is delayed because of abnormal EEG after phenylalanine loading at the age of 6 years, but remains stable up to the age of 14. According to these results a discontinuation of the diet at the age of 6 years can not be recommended even if the EEG is normal after phenylalanine load.

Adolescent↗
PubMed1989

[Results, problems and consequences of early treatment of phenylketonuria].

Screening of PKU was started in GDR by means of a napkin-test in 1967 and by Guthrie-test in 1971. A normal development is possible if treatment is started within the first 3 months of life and if the dietary control is good. Maintenance of a dietary control becomes difficult with growing age. The diet, however, should strictly be kept up to the age of 8 to 10 years. Thereafter there should be a low protein nutrition possible with supplementation of tyrosine. Prenatal diagnosis, which is possible in most of the families by RFLP technique, is justified despite the good results because of the social impact onto the family caused by the treatment. Treatment again must be started before pregnancy and must strictly be kept up to birth to avoid fetal damage by maternal PKU.

Child↗
PubMed1986

[Reduced optimal obstetric and postnatal conditions in children with phenylketonuria and their effect on the success of treatment].

We found a total of 433 reduced obstetric an postnatal conditions in 115 newborns with pku with a range of 0 (n = 4) to -11 (n = 1) per child. The 50th percentile of reduced optimal conditions was at -3, the 90th at -6. Early treated patients with excellent or good dietary control have significantly reduced developmental quotients (DQ) at the age of 3 years when the optimal conditions were reduced above the 90th percentile. Reduced optimal obstetric and postnatal conditions could be found in about 25 per cent of the patients with excellent or good dietary control and a DQ below 100, and in about 50 per cent with a DQ below 85. The effect of treatment is better in patients with excellent and good dietary control and a high value of reduced optimal conditions compared with patients with poor dietary control and an only low value of reduced optimal condition. The quality of dietary control therefore is the most important point to reach an optimal effect in early treated patients.

Child Development↗
PubMed1979

[Significance of early detection and early treatment of the development of children with phenylketonuria].

The amino acid l-phenylalanine (PA) accounts for 3-6 percent of all food proteins. Its breakdown is mainly through change into tyrosine. This irreversible metabolic step which makes PA an essential amino acid occurs in the liver and is catalyzed by PA-hydroxylase. In the autosome-recessive phenylketonuria syndrome (PKU, medium incidence rate 1:10,000) there is no PA-hydroxylase activity in the hepatic tissue. Infants suffering from PKU are born without any visible damage, but the untreated disease leads to irreversible brain injury. In the GDR legislation for early screening has been in force since 1971, so that the disease is recognized in all new-born babies. Brain damage can be prevented by dietetic treatment which starts during the first month but not later than the second month of life. The results of examinations performed on 107 infants suffering from PKU, with different onsets of dietetic treatment, underline the fundamental importance of early recognition and treatment for optimal development.

Child↗
PubMed1979

[Effect of the hypotrophy factor on the prognosis of the development of premature and newborn infants from the neurologic and psychologic viewpoint].

Neurological and psychological examinations have been conducted of prematurely born babies in good bodily condition and hypotrophic new-born infants (including control groups) as part of a not yet concluded longitudinal study. The hypoplastic premature infants showed a comparatively high proportion of statomotor retardation and neurological disturbances, which were also found in premature infants in good bodily condition with a pregnancy duration of 26-30 weeks. Cerebral motor disturbances were found primarily in hypoplastic new-born babies and especially in premature infants. The EQ (development quotient) for the premature infants in good bodily condition with a pregnancy duration between 26 and 30 weeks, and for the hypoplastic premature infants was lower than for other high-risk groups. These two groups were also responsible for the larger proportion of children showing light and medium psychic retardation. Behavioral peculiarities were, however, distributed also over other high-risk groups, and some were even more frequent there. It was also found that speech development was delayed. The study shows the necessity of following the development of high-risk babies and using individual therapies.

Brain Damage, Chronic↗