Urinary amino acid excretion by patients with beta-thalassemia.
Twenty-four hour urinary amino acid excretion has been studied in patients with beta-thalassemia (four adult major, two adult intermedia, and three children major). beta-Aminoisobutyric acid was found to be increased (14-fold on the average) in patients with beta-thalassemia without evidence for increase in the excretion of most other amino acids. No correlation of beta-aminoisobutyric acid excretion with the hematological status of the patient was found. Elevated taurine excretion (2-fold) was also noted in the adult patients with beta-thalassemia. Greatly increased excretion of beta-aminoisobutyric acid appears to correlate with a poor prognosis and may reflect generalized tissue catabolism rather than being a specific indicator of ineffective erythropoiesis.
D000293:Adolescent; D000596:Amino Acids / Q000652:urine*; D000621:Aminoisobutyric Acids / Q000652:urine; D006720:Homozygote; D006801:Humans; D013156:Splenectomy; D013789:Thalassemia / Q000652:urine*
D000596:Amino Acids; D000621:Aminoisobutyric Acids