Bakker B, Massa G G, Oostdijk W, Van Weel-Sipman M H, Vossen J M, Wit J M
Department of Paediatrics, Leiden University Medical Centre, The Netherlands.
Eur J Pediatr. 2000 Jan-Feb;159(1-2):31-7. doi: 10.1007/s004310050006.
Pubertal development after total-body irradiation (TBI) was investigated in 40 children (21 boys) treated with allogeneic bone marrow transplantation (BMT) for haematological malignancies at a mean age of 11.3 years. The mean age at the last visit was 19.0 years. Twenty-five patients (15 boys) were prepubertal at BMT. Data on secondary sexual characteristics, the pituitary-gonadal axis and longitudinal growth were retrospectively collected from the medical records. In boys not receiving additional testicular irradiation (n = 19), penile growth and pubic hair development was normal and all had serum testosterone levels within the adult range. The majority of them, however, had incidental elevations of LH, suggesting minor Leydig cell damage. Testicular volume at last measurement was small (mean: 10.5 ml) and serum FSH levels were elevated in all boys, with normalisation in only one, suggesting severe impairment of reproductive gonadal function. Of the ten girls who received BMT before puberty, six had a spontaneous onset of puberty and menarche; the four other girls needed hormonal substitution therapy. Recovery of gonadal function after cessation of substitution was seen in one girl, who became pregnant but had a spontaneous abortion. Decrease in height SDS was seen in the majority of patients and was positively correlated with male gender and lower age at the time of BMT.
Careful monitoring of both gonadal function and growth after bone marrow transplantation and total body irradiation is warranted in order to detect disturbances early and ensure normal pubertal development in children treated for haematological malignancies.
对40名儿童(21名男孩)进行了研究,这些儿童因血液系统恶性肿瘤接受异基因骨髓移植(BMT),平均年龄为11.3岁,研究全身照射(TBI)后的青春期发育情况。最后一次随访时的平均年龄为19.0岁。25名患者(15名男孩)在进行BMT时处于青春期前。从病历中回顾性收集了有关第二性征、垂体-性腺轴和纵向生长的数据。在未接受额外睾丸照射的男孩(n = 19)中,阴茎生长和阴毛发育正常,所有男孩的血清睾酮水平均在成人范围内。然而,他们中的大多数人促黄体生成素(LH)偶然升高,提示睾丸间质细胞有轻微损伤。最后一次测量时的睾丸体积较小(平均:10.5毫升),所有男孩的血清卵泡刺激素(FSH)水平均升高,只有一名男孩恢复正常,提示生殖性腺功能严重受损。在青春期前接受BMT的10名女孩中,6名自发进入青春期并月经初潮;另外4名女孩需要激素替代治疗。一名女孩在停止替代治疗后性腺功能恢复,怀孕但自然流产。大多数患者身高标准差评分(SDS)下降,且与男性性别和BMT时较低的年龄呈正相关。
对于接受骨髓移植和全身照射治疗血液系统恶性肿瘤的儿童,有必要仔细监测性腺功能和生长情况,以便早期发现异常并确保青春期正常发育。