THE EFFECT OF PLATYSPONDYLY AND PUBERTAL GROWTH SPURT ON THE STATURE OF PATIENTS WITH BETA-THALASSEMIA MAJOR

Objective To study the effect of the body proportion and pubertal grow th spurt on the stature of children with P-thalassaemia major. Methods The height, sitting height, upper to lower segment (U:L) ratio and pu bertal development were determined in 71 Chinese children (38 girls an d 33 boys) with beta-thalassaemia. The growth patterns of 20 patients with complete growth data between 3 years and final height, were analy zed according to whether they underwent a pubertal growth spurt or not . Results 27% of the boys and 32% of the girls had a height below the 3rd percentile. About 60% of all the children had a U:L ratio below th e 10th percentile for age. Abnormal body proportion was found in patie nts with or without growth retardation. 34% of the 41 children over th e age of 14 years underwent spontaneous puberty. In 28 patients over t he age of 16 years, a growth spurt was observed in 46% of the children during spontaneous or induced puberty. The retrospective analysis sho wed that the height deviation from the mean in adulthood was significa ntly higher in patients without pubertal growth acceleration than in t hose with a growth spurt ((x) over bar = - 11.8 cm, s = 7.6 cm vs (x) over bar = -4.4 cm, s = 4.4 cm; P = 0.02). Conclusions An abnormal U:L ratio was commonly observed in patients with beta-thalassaemia major and may be one factor contributing to the short stature of these patie nts. Abnormal puberty was present in a significant proportion of child ren and the lack of a pubertal growth spurt was found to be detrimenta l to adult height.