title: Sequential use of deferiprone and desferrioxamine in primary school children with thalassaemia major in Turkey.
abstract: The effectiveness of the sequential use of deferiprone and desferrioxamine (DFO) in children with thalassaemia major was examined. Seven thalassaemic children in whom urinary iron induced by deferiprone was sufficient to maintain a negative iron balance were enrolled in the long-term trial. Deferiprone at a dose of 75 mg/kd/day in 3 divided doses was given for 4 school days a week. The group was given DFO at a dose of 40-50 mg/kg/day s.c. over 8-12 h with a battery-operated pump for 2 days at the weekend. In addition to the safety variables, they were monitored for serum ferritin levels at 2-month intervals and hepatic iron concentrations in liver tissues were determined at the beginning and the 6th month of therapy. The severity of hepatic damage was graded according to the Knodell hepatic activity index and the fibrosis was quantified. None of the patients suffered adverse effects of the therapy but a transient increase in serum ALT levels was noted. A nonsignificant decline in serum ferritin was observed (p = 0.08), a significant reduction in hepatic iron concentration was also determined (p = 0. 03). The hepatic activity index in liver tissues of the patients at the 6th month of the sequential therapy significantly decreased (p = 0.03) whereas fibrosis scores did not differ significantly (p = 0. 25).
authors.affiliation: Department of Pediatric Hematology, Medical Faculty of Ege University, Bornova, Izmir, Turkey. aydinok@hotmail.com
authors.forename: Y
authors.initials: Y
authors.lastname: Aydinok
authors.forename: G
authors.initials: G
authors.lastname: Nisli
authors.forename: K
authors.initials: K
authors.lastname: Kavakli
authors.forename: C
authors.initials: C
authors.lastname: Coker
authors.forename: M
authors.initials: M
authors.lastname: Kantar
authors.forename: N
authors.initials: N
authors.lastname: Cetingül
mesh_terms: D000293:Adolescent; D002614:Chelating Agents / Q000627:therapeutic use*; D002648:Child; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D008297:Male; D011728:Pyridones / Q000627:therapeutic use*; D013334:Students; D014421:Turkey; D017086:beta-Thalassemia / Q000188:drug therapy*
chemical_list: D002614:Chelating Agents; D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine