Abstract：
BACKGROUND: Turner syndrome (TS) is characterized by short stature and premature ovarian insufficiency (POI). The main long-term complication of POI is osteoporosis, which can be prevented by hormone replacement therapy (HRT).
OBJECTIVE: The objective of our study was to compare initial bone mineral density (BMD) and progression between TS and idiopathic POI patients under HRT.
METHODS: A single-center retrospective study was conducted between 1998 and 2018. All women had undergone at least two bone densitometry assessments at least 2 years apart.
RESULTS: Sixty-eight TS patients and 67 idiopathic POI patients were included. Mean age at initial assessment was 27 years (IQR, 21-35.5 years) in TS patients and 31.5 years (IQR, 23-37 years) in idiopathic POI patients (P=0.1). Lumbar and femoral neck BMD were lower in the TS group than in the idiopathic POI group (respectively 0.89g/cm2 versus 0.95g/cm2, P=0.03; 0.70g/cm2 versus 0.77g/cm2, P<0.0001). Mosaic karyotype was associated with better BMD in TS patients while history of growth hormone treatment had no impact on BMD. Over time, a significant gain in vertebral BMD was observed in TS patients versus a loss of BMD in idiopathic POI patients (P=0.0009).
CONCLUSIONS: TS patients had a lower BMD at baseline than idiopathic POI patients, at both spinal and femoral levels. Over time, on HRT, a significant gain in vertebral BMD was observed in patients with TS, compared with a loss of BMD in patients with idiopathic POI. We hypothesized that earlier initiation and longer duration of HRT played an important role in this finding. Long-term prospective follow-up to assess the incidence of fractures in TS would be useful.
OBJECTIVE: The objective of our study was to compare initial bone mineral density (BMD) and progression between TS and idiopathic POI patients under HRT.
METHODS: A single-center retrospective study was conducted between 1998 and 2018. All women had undergone at least two bone densitometry assessments at least 2 years apart.
RESULTS: Sixty-eight TS patients and 67 idiopathic POI patients were included. Mean age at initial assessment was 27 years (IQR, 21-35.5 years) in TS patients and 31.5 years (IQR, 23-37 years) in idiopathic POI patients (P=0.1). Lumbar and femoral neck BMD were lower in the TS group than in the idiopathic POI group (respectively 0.89g/cm2 versus 0.95g/cm2, P=0.03; 0.70g/cm2 versus 0.77g/cm2, P<0.0001). Mosaic karyotype was associated with better BMD in TS patients while history of growth hormone treatment had no impact on BMD. Over time, a significant gain in vertebral BMD was observed in TS patients versus a loss of BMD in idiopathic POI patients (P=0.0009).
CONCLUSIONS: TS patients had a lower BMD at baseline than idiopathic POI patients, at both spinal and femoral levels. Over time, on HRT, a significant gain in vertebral BMD was observed in patients with TS, compared with a loss of BMD in patients with idiopathic POI. We hypothesized that earlier initiation and longer duration of HRT played an important role in this finding. Long-term prospective follow-up to assess the incidence of fractures in TS would be useful.
摘要：
背景：特纳综合征（TS）的特征是身材矮小和过早的卵巢功能不全（POI）。POI的主要长期并发症是骨质疏松,可以通过激素替代疗法（HRT）来预防。
目的：我们的研究目的是比较HRT下TS和特发性POI患者的初始骨矿物质密度（BMD）和进展。
方法：1998年至2018年进行了一项单中心回顾性研究。所有女性至少相隔2年接受了至少两次骨密度测定评估。
结果：纳入68例TS患者和67例特发性POI患者。初始评估的平均年龄为27岁(IQR，TS患者21-35.5年)和31.5年(IQR，特发性POI患者23-37岁）（p=0.1）。TS组的腰椎和股骨颈BMD低于特发性POI组（分别为0.89g/cm²和0.95g/cm²，p=0.03；0.70g/cm²对0.77g/cm²，p<0.0001)。在TS患者中，镶嵌核型与更好的BMD相关，而生长激素治疗史对BMD没有影响。随着时间的推移,与特发性POI患者的BMD丢失相比，TS患者的椎体BMD显着增加（p=0.0009）。
结论：TS患者在基线时的BMD低于特发性POI患者，在脊髓和股骨水平。随着时间的推移,在HRT上,在TS患者中观察到椎骨BMD的显着增加，与特发性POI患者的BMD丢失相比。我们假设HRT的更早开始和更长的持续时间在这一发现中起着重要作用。长期前瞻性随访以评估TS中骨折的发生率将是有用的。
目的：我们的研究目的是比较HRT下TS和特发性POI患者的初始骨矿物质密度（BMD）和进展。
方法：1998年至2018年进行了一项单中心回顾性研究。所有女性至少相隔2年接受了至少两次骨密度测定评估。
结果：纳入68例TS患者和67例特发性POI患者。初始评估的平均年龄为27岁(IQR，TS患者21-35.5年)和31.5年(IQR，特发性POI患者23-37岁）（p=0.1）。TS组的腰椎和股骨颈BMD低于特发性POI组（分别为0.89g/cm²和0.95g/cm²，p=0.03；0.70g/cm²对0.77g/cm²，p<0.0001)。在TS患者中，镶嵌核型与更好的BMD相关，而生长激素治疗史对BMD没有影响。随着时间的推移,与特发性POI患者的BMD丢失相比，TS患者的椎体BMD显着增加（p=0.0009）。
结论：TS患者在基线时的BMD低于特发性POI患者，在脊髓和股骨水平。随着时间的推移,在HRT上,在TS患者中观察到椎骨BMD的显着增加，与特发性POI患者的BMD丢失相比。我们假设HRT的更早开始和更长的持续时间在这一发现中起着重要作用。长期前瞻性随访以评估TS中骨折的发生率将是有用的。
