Final height and body disproportion in thalassaemic boys and girls with spontaneous or induced puberty

The aim of the study was to evaluate whether sex hormone replacement therap y adversely affected final height and body disproportion in thalassaemic bo ys and girls. Thirty-six patients with spontaneous (SP) or induced puberty (IP) were studied in order to define the pattern of height growth through t hree observations: the first (A) at the age of 7-9; the second (B) at onset of spontaneous or induced puberty; and the third (C) when final height was reached. We examined 14 females with SP (f-SP) and 8 with IP (f-IP); 7 mal es with SP (m-SP) and 7 with IP (m-IP). Girls with IP reached the same fina l height of girls with SP (f-IP 153.8 (4.3) versus f-SP 154.4 (5.5) cm); p > 0.05) close to target height (f-IP 155.9 (5.2) cm versus f-SP 155.5 (3.6) cm). Girls with IP reached the final height at older chronological age (CA ) (17.0 (0.6) y) than girls with SP (CA of 15.3 (0.7) y), but at the same b one age (BA) (f-IP 15.1 (0.9)y versus f-SP 14.8 (0.6) y). There was no diff erence between the two groups for pubertal growth (f-SP 16.2 (7.7) cm versu s f-IP 12.2 (7.4) cm (p > 0.05)) that was negatively correlated with both p repubertal growth and HA at onset of puberty in both groups. Values of sitt ing height (sds) with respect to BA (SHsdsBA) were not significantly differ ent between the two groups, and showed a worsening from the first observati on to final height, reaching values around -2 SD, in both groups. Values of subischial leg length (sds) with respect to HA (SLLsdsBA) were in the norm al range at both observations in all girls. High serum ferritin levels were observed in both groups (f-SP 3189 (2296) ng/ml and f-IP 3998 (2545) ng/ml ; p > 0.05). Also boys with induced puberty reached the same final height o f those with spontaneous one (m-IP 160.9 (5.5) cm versus m-SP 161.8 (2.4) c m; p > 0.05), but it was lower than target height in both groups (m-IP 168. 1 (4.1)cm versus m-SP 169.6 (3.2)cm). Boys with IP reached final height at CA of 18.6 (1.1)y slightly older than boys with SP (CA 17.2 (0.9) y), but a t the same HA (m-IP 15.9 (1.5) y versus m-SP 16.3 (0.8) y). Pubertal growth values were significantly different between boys with SP 18.9 (5.3) cm and those with IP 13.8 (4.9) cm (p < 0.05), but they were negatively correlate d with prepubertal growth values in both groups (m-SP r = -0.91; p < 0.002 and m-IP r = -0.51; p < 0.05). SHsdsBA showed a worsening from the first ob servation to final height, reaching values around -3 SD in both groups, whi le SLLsdsBA were always in the normal range in all patients. Serum ferritin levels were higher in boys with IP (3400 (1179) ng/ml) than in those with SP (2020 (496) ng/ml). Conclusions: Our date showed that: (a) patients of both sexes with induced puberty reached the same final height of patients with spontaneous puberty; (b) all patients showed a body disproportion with truncal shortening and n ormal leg length that was more severe in boys of both groups at final heigh t; (c) body disproportion was independent of pubertal or prepubertal period of greater height gain, suggesting that sexual steroids replacement therap y did not adversely affect either final height or body disproportion. Furth er studies, focused on the pathogenesis of the truncal shortening, are nece ssary in order to acquire more insight into the cause of this impairment.