Abstract
Background There may be a marked reduction in essential amino acids in the serum of children with thalassemia major and this is related to decreased growth in affected children. Methods One hundred patients with β-thalassemia and 50 control children selected from among those who had presented with minor disorders unrelated to hematological disease were recruited. Urine and heparinized blood were collected from fasting thalassemic patients. After deproteinization and dilution, amino acid concentrations were measured using ion-exchange chromatography. Results Isoleucine ( p<0.0001), phenylalanine ( p<0.05), tyrosine ( p<0.0001), taurine ( p<0.0001) and glutamine ( p<0.01) were significantly decreased in the plasma of thalassemic patients compared to the control group. Whereas glutamate ( p<0.0001), serine ( p<0.05) and proline ( p<0.05) were significantly higher in thalassemic patients, threonine, glycine, alanine, valine, methionine, leucine, ornithine, lysine, histidine and arginine values were not different. The essential amino acids taurine ( p<0.0001), methionine ( p<0.01), valine ( p<0.01), phenylalanine ( p<0.01) and leucine ( p<0.05) were significantly decreased in urine of thalassemic patients vs. controls, but threonine and ornithine were not different. The mean urinary excretion rate of β-aminoisobutyric acid was not different (69±96 in thalassemics vs. 41±52 in controls). However, most plasma and urinary essential amino acids were found to be lower in thalassemics. Thalassemic patients were also found to be significantly growth impaired for age, both in height and weight compared to controls. Conclusion Lower plasma values of essential amino acids and a decrease in urinary amino acids occur in thalassemic patients. Growth impairment both in height and weight also occurs in thalassemic patients compared to a control population.
Published Version
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
