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Urinary amino acid excretion by patients with beta-thalassemia
Abstract:
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.
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