LINEAR GROWTH IN THALASSEMIC CHILDREN TREATED WITH INTENSIVE CHELATION-THERAPY - A LONGITUDINAL-STUDY

The aim of our study was to investigate the effect of a definite sched ule of treatment on the growth of children with beta-thalassemia major . Ten children (6 girls, 4 boys) participated in this longitudinal stu dy. They were treated with transfusions to maintain the levels of hemo globin above 10.5 g/dl from the time of diagnosis and desferrioxamine mesylate 40 mg/kg/day by subcutaneous pump, 5 days a week, during 7 +/ - 1.4 years. The patients were followed up from the age at diagnosis(1 8.9 +/- 9.8 months) until they reached their adult bone age(19.1 +/- 1 .6 and 20.8 +/- 0.8 years in girls and boys, respectively). We observe d growth retardation before the age of 10 in 3 out of 4 boys and in 1 out of 6 girls. Between the ages of 10 and 15, the growth failure was evident in other children (1 boy and 2 girls), and became more marked in the above mentioned children. After the age of 15, amelioration of the growth failure was observed in relation to sexual maturity. Eight out of 10 patients attain normal stature in accordance with their targ et height. We had evidence of poor compliance with the chelation thera py in the 2 children of short adult stature. These patients had higher serum ferritin levels than the children with normal height within the study period: 4,461 +/- 1,469 and 1,429 +/- 479 mu g/l respectively ( p = 0.049). Our study reveals a positive effect of intensive chelation therapy on the linear growth in these patients.