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Urinary amino acid excretion by patients with β-thalassemia

Authors
Journal
Clinica Chimica Acta
0009-8981
Publisher
Elsevier
Publication Date
Volume
80
Issue
3
Identifiers
DOI: 10.1016/0009-8981(77)90144-9

Abstract

Abstract Twenty-four hour urinary amino acid excretion has been studied in patients with β-thalassemia (four adult major, two adult intermedia, and three children major). β-Aminoisobutyric acid was found to be increased (14-fold on the average) in patients with β-thalassemia without evidence for increase in the excretion of most other amino acids. No correlation of β-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 β-thalassemia. Greatly increased excretion of β-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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