LCK Low

The cause of growth failure in patients with, β-thalassaemia major has not been fully elucidated. GH reserve is usually normal but IGF-1 and IGF-BP3 have been found to be low in our patients The lack of difference in serum IGF-1 levels between children with or without growth failure would suggest that the growth stunting may not be related to the GII-IGF-1 axis. In a recent survey of 71 patients attending Queen Mary Hospital, 27% of the boys and 32% of the girls were found to have growth retardation. 60% of the boys and 63% of the girls had an U/L ratio below the 10th percentile for age. Only 32% of children over the age of 14 years had spontaneous onset of preberty. The of a prebertal growth spurt is detrimental to final adult height in these children. Other endocrinepathies include diabetes mellitus (6.5%), hypothyroidism (4%), GH deficiency (7%) whereas adrenal and parathyroid insufficiency are rare. Excessive chelation with desferrioxamine can result in bone dysplasia and growth retardation. Regular chelation should only be started after the serum ferritin has exceeded 1000ng/ml. Chelation should be guided by the toxicity index (DFO in mg/kg/day÷serum ferritin in, μg/L should not exceed 0.025). GH therapy in children with, β-thalassaemia results in an increase in short term growth and a rise in IGF-1 and IGF-BP3 concentrations. After 4 years of GH treatment, 66% of the patients have achieved satisfactory height gain with 6 patients still on treatment. In patients with delayed preberty, induction of preberty with sex steroids should be started at an appropriate age.