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Iron Overload and Desferrioxamine Chelation Therapy in Beta-thalassemia Intermedia

Overview
Journal Eur J Pediatr
Specialty Pediatrics
Date 1981 Nov 1
PMID 7318837
Citations 15
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Abstract

This study on serum ferritin levels in urinary iron excretion after 12h subcutaneous infusion of desferrioxamine in 10 thalassemia intermedia patients shows that even nontransfusion-dependent patients may have positive iron balance resulting in iron overload from 5 years of age. However, the iron overload found in these patients appears to be much lower than in age matched patients with transfusion-dependent thalassemia major. Iron overload increases with advancing age, as shown by increasing serum ferritin levels and desferrioxamine-induced urinary iron elimination. After a six month trial of 12h continuous subcutaneous desferrioxamine administration there was a significant decline in serum ferritin levels. From this study it seems that iron chelation is indicated in thalassemia intermedia patients over 5 years of age in order to prevent iron accumulation. However, the appropriate treatment schedule should be tailored to the individual needs of each patients, established by close monitoring of serum ferritin levels and desferrioxamine-induced urinary iron elimination.

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References
1.
Pembrey M, McWade P, Weatherall D . Reliable routine estimation of small amounts of foetal haemoglobin by alkali denaturation. J Clin Pathol. 1972; 25(8):738-40. PMC: 477492. DOI: 10.1136/jcp.25.8.738. View

2.
Pippard M, CALLENDER S, Warner G, Weatherall D . Iron absorption and loading in beta-thalassaemia intermedia. Lancet. 1979; 2(8147):819-21. DOI: 10.1016/s0140-6736(79)92175-5. View

3.
Weiner M, Karpatkin M, Hart D, Seaman C, Vora S, Henry W . Cooley anemia: high transfusion regimen and chelation therapy, results, and perspective. J Pediatr. 1978; 92(4):653-8. DOI: 10.1016/s0022-3476(78)80316-3. View

4.
Cumming R, Millar J, Smith J, GOLDBERG A . Clinical and laboratory studies on the action of desferrioxamine. Br J Haematol. 1969; 17(3):257-63. DOI: 10.1111/j.1365-2141.1969.tb01371.x. View

5.
Hussain M, Green N, Flynn D, Hussein S, Hoffbrand A . Subcutaneous infusion and intramuscular injection of desferrioxamine in patients with transfusional iron overload. Lancet. 1976; 2(7998):1278-80. DOI: 10.1016/s0140-6736(76)92035-3. View