Sequential use of deferiprone and desferrioxamine in primary school children with thalassaemia major in Turkey

Citation
Y. Aydinok et al., Sequential use of deferiprone and desferrioxamine in primary school children with thalassaemia major in Turkey, ACT HAEMAT, 102(1), 1999, pp. 17-21
Citations number
17
Categorie Soggetti
Hematology,"Cardiovascular & Hematology Research
Journal title
ACTA HAEMATOLOGICA
ISSN journal
00015792 → ACNP
Volume
102
Issue
1
Year of publication
1999
Pages
17 - 21
Database
ISI
SICI code
0001-5792(1999)102:1<17:SUODAD>2.0.ZU;2-O
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 main tain a negative iron balance were enrolled in the long-term trial. Deferipr one at a dose of 75 mg/kd/day in 3 divided doses was given for 4 school day s 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-m onth intervals and hepatic iron concentrations in liver tissues were determ ined 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 t he therapy but a transient increase in serum ALT levels was noted. A nonsig nificant decline in serum ferritin was observed (p = 0.08), a significant r eduction 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).