Renal transport of amino acids in children with oculocerebrorenal syndrome.

Inulin and amino acid clearance studies were made in 6 boys with oculocerebrorenal syndrome (Lowe) aged 11 months to 5 years. Glomerular filtration rate ranged between 50 and 112 ml/min/1.73 m2. Clearance values of all amino acids were increased. Tubular reabsorption of cystine, ornithine, lysine and arginine was relatively more reduced than that of the other amino acids. In Lowe's syndrome the transport system of the basic amino acids and cystine appears to be more impaired than that of other amino acids.