Literature DB >> 7318837

Iron overload and desferrioxamine chelation therapy in beta-thalassemia intermedia.

P Cossu, C Toccafondi, F Vardeu, G Sanna, F Frau, R Lobrano, G Cornacchia, A Nucaro, F Bertolino, A Loi, S De Virgiliis, A Cao.   

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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Year:  1981        PMID: 7318837     DOI: 10.1007/bf00443255

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  13 in total

1.  Microchromatography of hemoglobins. II. A simplified procedure for the determination of hemoglobin A2.

Authors:  T H Huisman; W A Schroeder; A N Brodie; S M Mayson; J Jakway
Journal:  J Lab Clin Med       Date:  1975-10

2.  The identification of 2, 3-dihydroxybenzoic acid as a potentially useful iron-chelating drug.

Authors:  J H Graziano; R W Grady; A Cerami
Journal:  J Pharmacol Exp Ther       Date:  1974-09       Impact factor: 4.030

Review 3.  Advances in the use of iron-chelating agents for the treatment of iron overload.

Authors:  B Modell
Journal:  Prog Hematol       Date:  1979

4.  Reliable routine estimation of small amounts of foetal haemoglobin by alkali denaturation.

Authors:  M E Pembrey; P McWade; D J Weatherall
Journal:  J Clin Pathol       Date:  1972-08       Impact factor: 3.411

5.  Clinical and laboratory studies on the action of desferrioxamine.

Authors:  R L Cumming; J A Millar; J A Smith; A Goldberg
Journal:  Br J Haematol       Date:  1969-09       Impact factor: 6.998

6.  Subcutaneous infusion and intramuscular injection of desferrioxamine in patients with transfusional iron overload.

Authors:  M A Hussain; N Green; D M Flynn; S Hussein; A V Hoffbrand
Journal:  Lancet       Date:  1976-12-11       Impact factor: 79.321

7.  Cooley anemia: high transfusion regimen and chelation therapy, results, and perspective.

Authors:  M Weiner; M Karpatkin; D Hart; C Seaman; S K Vora; W L Henry; S Piomelli
Journal:  J Pediatr       Date:  1978-04       Impact factor: 4.406

8.  Chelation therapy in beta-thalassemia major. I. Intravenous and subcutaneous deferoxamine.

Authors:  J H Graziano; A Markenson; D R Miller; H Chang; M Bestak; P Meyers; P Pisciotto; A Rifkind
Journal:  J Pediatr       Date:  1978-04       Impact factor: 4.406

9.  Iron absorption and loading in beta-thalassaemia intermedia.

Authors:  M J Pippard; S T Callender; G T Warner; D J Weatherall
Journal:  Lancet       Date:  1979-10-20       Impact factor: 79.321

10.  Iron chelation therapy with deferoxamine in Cooley anemia.

Authors:  A Cohen; E Schwartz
Journal:  J Pediatr       Date:  1978-04       Impact factor: 4.406

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  14 in total

Review 1.  β-thalassemia intermedia: a clinical perspective.

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Review 2.  Survival of the fittest: in vivo selection and stem cell gene therapy.

Authors:  Tobias Neff; Brian C Beard; Hans-Peter Kiem
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3.  Decreased differentiation of erythroid cells exacerbates ineffective erythropoiesis in beta-thalassemia.

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Journal:  Blood       Date:  2008-05-14       Impact factor: 22.113

Review 4.  Management of non-transfusion-dependent thalassemia: a practical guide.

Authors:  Ali T Taher; Maria Domenica Cappellini
Journal:  Drugs       Date:  2014-10       Impact factor: 9.546

5.  Ineffective erythropoiesis in beta-thalassemia is characterized by increased iron absorption mediated by down-regulation of hepcidin and up-regulation of ferroportin.

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Review 6.  Regulation of iron absorption in hemoglobinopathies.

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Review 7.  Ineffective erythropoiesis and thalassemias.

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Journal:  Curr Opin Hematol       Date:  2009-05       Impact factor: 3.284

Review 8.  Non-transfusion-dependent thalassemias.

Authors:  Khaled M Musallam; Stefano Rivella; Elliott Vichinsky; Eliezer A Rachmilewitz
Journal:  Haematologica       Date:  2013-06       Impact factor: 9.941

9.  Thalassaemia intermedia: an update.

Authors:  Ali T Taher; Khaled M Musallam; Maria D Cappellini
Journal:  Mediterr J Hematol Infect Dis       Date:  2009-08-29       Impact factor: 2.576

Review 10.  Treating iron overload in patients with non-transfusion-dependent thalassemia.

Authors:  Ali T Taher; Vip Viprakasit; Khaled M Musallam; M Domenica Cappellini
Journal:  Am J Hematol       Date:  2013-03-08       Impact factor: 10.047

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