The overall long-term objectives of this application are to understand thalassemia is several aspects to provide guidelines for better management and prevention. DNA mapping, gene cloning and sequencing, oligonucleotide hybridization and polymerase chain reaction are used to exhaustively characterize alpha- and beta-thalassemias in Thailand. Pathophysiologic studies make use of multidisciplinary approach to different problems. Determination of different degree of anemia in beta o-thalassemia/Hb E patients with apparently identical genotypes is systematically investigated. Hypoxemia which is a unique phenomenon in splenectomized thalassemics is being studied for its pathogenesis and prevention through platelet function assessment. Increased susceptibility to infections which have been shown by prospective studies will be assessed by studying of the immune system. The Thalassemic Red Blood Cell is studied will be assessed by studying of the immune system. The Thalassemic Red Blood Cell is studied by several methods. Abnormal cytoskeleton proteins are evaluated in correlations with shape and deformability changes. What occur inside the thalassemic RBC appear to affect the outer surface of the RBC, since immune complex is accumulated. This immune complex phenomenon of the thalassemic RBC which causes superimposing auto-immune hemolytic anemia and perhaps other immune aberration in the body is studied in details. Dynamics of the complex relationship between the erythron and iron kinetics is studied counter. Evaluation of new oral iron chelating agents in thalassemia will be carried out before recommendation for their use in general.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Project (R01)
Project #
5R01HL034408-20
Application #
3347283
Study Section
Hematology Subcommittee 2 (HEM)
Project Start
1985-12-01
Project End
1992-06-30
Budget Start
1990-09-30
Budget End
1991-09-29
Support Year
20
Fiscal Year
1990
Total Cost
Indirect Cost
Name
Mahidol University
Department
Type
DUNS #
City
Nakhon Pathom
State
Country
Thailand
Zip Code
73170
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Winichagoon, P; Fucharoen, S; Wilairat, P et al. (1994) Nondeletional type of hereditary persistence of fetal haemoglobin: molecular characterization of three unrelated Thai HPFH. Br J Haematol 87:797-804
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