EFFECT OF DIFFERENT TREATMENT REGIMES ON LINEAR GROWTH AND FINAL HEIGHT IN BETA-THALASSEMIA MAJOR

OBJECTIVE The clinical picture of thalassaemia major has changed progr essively over the years. Our study is a retrospective analysis of data on growth in a group of patients who have completed puberty spontaneo usly and have attained their adult height. Our objective was to evalua te the effect of different transfusion regimes and desferrioxamine adm inistration on the growth pattern in beta-thalassaemia major. DESIGN A ND PATIENTS We studied 64 patients (28 males and 36 females). The pati ents were divided into three groups (A, B and C) according to the diff erent transfusion regimes and the schedules of chelating therapy. Grou p A consisted of 16 patients who were transfused regularly at low haem oglobin levels (on average 8.5 g/dl) from an early age and started sub cutaneous chelation therapy during adolescence. Group B consisted of 1 9 patients who were transfused regularly at high haemoglobin levels (o n average 10 g/dl) from an early age and started subcutaneous therapy during childhood. Group C consisted of 29 patients who were transfused regularly at high haemoglobin levels (on average 10.5 g/dl) from an e arly age and started subcutaneous chelation therapy very early, at a m ean age of 2 years. Standard auxological measurements were made at 3-m onthly intervals throughout childhood and puberty until adult height w as achieved. For group C patients the data on linear growth are provid ed only until the age of 12 years. RESULTS Our study indicates that gr oup A male and female patients did not grow significantly better than those in group B. Group C male and female patients, surpristherapy lat e in childhood (group A). The most striking feature in the majority of both group A and B patients was reduced spurt in height at puberty. I n addition, in both groups, a reduced sitting height due to spinal gro wth abnormality was found. An inverse correlation between sitting heig ht and serum ferritin levels was observed in group A patients (r = -0. 55, P < 0.05), whereas there was a direct correlation in group B patie nts (r = 0.42, P < 0.05). CONCLUSIONS These data suggest that an ideal therapeutic regime has yet to be found which avoids the toxic effect of iron overload and on the other hand avoids interference with growth , secondary to desferrioxamine. Therefore we recommend that the growth of thalassaemia patients be monitored routinely at every follow-up vi sit and documented on growth velocity charts in order to detect early changes in their growth pattern and to establish an appropriate protoc ol for investigation and treatment.