The effect of platyspondyly and pubertal growth spurt on the stature of patients with β-thalassaemia major

Abstract

Objective To study the effect of the body proportion and pubertal growth spurt on the stature of children with β-thalassaemia major. Methods The height, sitting height, upper to lower segment (U : L) ratio and pubertal development were determined in 71 Chinese children (38 girls and 33 boys) with β-thalassaemia. The growth patterns of 20 patients with complete growth data between 3 years and final height, were analyzed 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 the 10th percentile for age. Abnormal body proportion was found in patients with or without growth retardation. 34% of the 41 children over the age of 14 years underwent spontaneous puberty. In 28 patients over the age of 16 years, a growth spurt was observed in 46% of the children during spontaneous or induced puberty. The retrospective analysis showed that the height deviation from the mean in adulthood was significantly higher in patients without pubertal growth acceleration than in those with a growth spurt (x̄= -11.8 cm, s = 7.6 cm vs x̄= -4.4cm, s= 4.4cm; P= 0.02). Conclusions An abnormal U:L ratio was commonly observed in patients with β-thalassaemia major and may be one factor contributing to the short stature of these patients. Abnormal puberty was present in a significant proportion of children and the lack of a pubertal growth spurt was found to be detrimental to adult height.