Teijema et al. (1974) described a female child with a defect in renal and probably intestinal transport of 2 acidic amino acids, glutamic and aspartic acids. The child was mentally retarded and also showed moderate hyperprolinemia and hypoglycemia. ... Teijema et al. (1974) described a female child with a defect in renal and probably intestinal transport of 2 acidic amino acids, glutamic and aspartic acids. The child was mentally retarded and also showed moderate hyperprolinemia and hypoglycemia. Melancon et al. (1977) reported a 38-month-old male infant with large amounts of the dicarboxylic amino acids, aspartic and glutamic, in the urine without generalized aminoaciduria and without neurologic and developmental abnormalities. The patient was found soon after birth by routine screening. Intestinal transport and in vitro oxidation of dicarboxylic amino acids suggested that the basic defect was selective, one of renal conservation. Transport defects have been observed in 4 other groups of amino acids: beta-amino acids, dibasic amino acids, amino acids and glycine, and neutral amino acids. The authors found a report of dicarboxylic aminoaciduria and associated fasting hypoglycemia and mental retardation. In a later study in which 500 mentally retarded children were screened for amino acid disorders, Swarna et al. (1989) found 1 case of dicarboxylic aminoaciduria in a girl.