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Published by : PROFESSIONAL MEDICAL PUBLICATIONS |
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ISSN 1681-715X |
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REVIEW ARTICLE |
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Volume 25 |
July - September 2009 |
Number 4 |
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Complications in transfusion–dependent
patients of ß-thalassemia major
Sara Malik1, Serajuddaula Syed2, Nisar Ahmed3
SUMMARY
ß- thalassemia is an inherited disorder of hemoglobin synthesis characterized by deficient synthesis of the ß-globin chain that causes severe anemia. Over the years, the combination of hypertransfusion and chelation therapy has significantly increased the survival of patients of ß-thalassemia. At the same time, there has been an increase in the frequency of complications, mainly caused by iron overload. These include cardiac disorders which are the main determinants of survival. Endocrine complications include hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism and hypoadrenalism. Low bone mineral density and trace element deficiencies are among the metabolic complications seen in chronically transfused patients of ß- thalassemia whereas hepatic problems and neuropsychologic disorders are also common.
KEY WORDS:
ß- thalassemia, chelation, Cardiac complications, multiendocrine dysfunctionPak J Med Sci July - September 2009 Vol. 25 No. 4 678-682
How to cite this article:
Malik S, Syed S, Ahmed N. Complications in transfusion–dependent patients of ß-thalassemia major. Pak J Med Sci 2009;25(4):678-682.
1. Dr. Sara Malik
Department of Pathology,
Ziauddin University,
Shahrah-e-Ghalib, Clifton,
Karachi - Pakistan.
2. Dr. Serajuddaula Syed, FRCPath.
Professor of Pathology Ziauddin University,
Shahrah-e-Ghalib, Clifton, Karachi.
3. Dr. Nisar Ahmed, FCPS
Head Consultant Hematologist,
Assistant Professor Hematology & Transfusion
Medicine, The Children’s Hospital &
Institute of Child Health,
Lahore - Pakistan
Correspondence
Dr. Sara Malik
House No. 9-W,
Defence Housing Authority,
Lahore-Cantt, Pakistan.
E-mail: saramalik89@hotmail.com
* Received for Publication: May 13, 2009
* Accepted: June 24, 2009
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