Cognitive Functioning in Two Sisters with Carbamyl Phosphate Synthetase I Deficiency

Abstract
The case histories of two teen-age girls with carbamyl phosphate synthetase I deficiency are presented. Appropriate psychometric studies show that these women are functioning in the borderline range of cognitive skills. These results emphasize that individuals with Krebs-Henseleit urea cycle enzyme deficiencies who survive the neonatal period are not necessarily severely mentally retarded as has been stressed previously.

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