青春期发育及其决定因素在依赖输血的地中海贫血青少年中。

Pubertal Development and its Determinants in Adolescents With Transfusion-Dependent Thalassemia.

机构信息

Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi.

Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi. Correspondence to: Dr Anju Seth, Director Professor, Department of Pediatrics, Lady Hardinge Medical College and Kalawati Saran Children's Hospital, New Delhi 110 001, India.

出版信息

Indian Pediatr. 2021 Jul 15;58(7):635-638. Epub 2021 Mar 26.

PMID:33772533

Abstract

OBJECTIVES

To assess pubertal development and its determinants in adolescents with transfusion-dependent thalassemia (TDT).

METHODS

In this cross-sectional study from a tertiary teaching hospital in Delhi, records of adolescents aged 17-19 years with TDT on regular transfusion at thalassemia day-care centre were reviewed. Pubertal development and its determinants were assessed.

RESULTS

Records of 58 (33 male) adolescents with TDT were reviewed. Among them, 42 (72.4%) had normal/delayed onset with spontaneous progression of puberty, while 16 (27.6%) had pubertal arrest/failure and received hormonal replacement therapy (HRT). Short stature was observed in all adolescents on HRT. Amongst other endocrinopathies, only hypoparathyroidism was found to be significantly higher in the HRT group. On multivariate analysis, serum ferritin (OR-1.005, 95% CI 1.002, 1.009) was observed to be the only significant determinant of pubertal arrest/failure.

CONCLUSIONS

A significant proportion of adolescents with TDT continue to have pubertal arrest/failure. High systemic iron load is the key determinant for this.

摘要

目的

评估依赖输血的地中海贫血（TDT）青少年的青春期发育及其决定因素。

方法

本研究为德里一家三级教学医院的横断面研究，对地中海贫血日间护理中心接受定期输血的 17-19 岁 TDT 青少年的记录进行了回顾。评估了青春期发育及其决定因素。

结果

共回顾了 58 名（33 名男性）TDT 青少年的记录。其中，42 名（72.4%）有正常/延迟开始的青春期，自发进展，而 16 名（27.6%）有青春期停滞/失败，并接受了激素替代治疗（HRT）。所有接受 HRT 的青少年都存在身材矮小。在其他内分泌疾病中，仅发现甲状旁腺功能减退症在 HRT 组中的发生率明显更高。多变量分析显示，血清铁蛋白（OR-1.005，95%CI 1.002，1.009）是青春期停滞/失败的唯一显著决定因素。