Bertelloni S, Baroncelli G I, Federico G, Cappa M, Lala R, Saggese G
Pediatric Endocrine Unit, Department of Reproductive Medicine and Pediatrics, University of Pisa, 'Santa Chiara' Hospital, Pisa, Italy.
Horm Res. 1998;50(6):309-14. doi: 10.1159/000023296.
Androgens have major influences on the regulation of bone mineralization. Because of their unique peripheral metabolism androgens may act on bone via activation of the androgen and/or estrogen receptor. Patients with complete androgen insensitivity syndrome (cAIS) are natural models to assess androgen actions on bone. We studied bone mineral density (BMD) in 10 patients with cAIS (mean age 13.70, range 4.7-19.8 years); 3 patients were studied before gonadectomy; the others were castrated and 6 were on hormonal replacement therapy. The BMD area (aBMD) was measured by dual energy X-ray; lumbar 'apparent' volumetric density (vBMD) was calculated using the formula vBMD = aBMD x [4/(pi x width)]. In the patients, aBMD (0.72 +/- 0.16 g/cm2) and vBMD (0.23 +/- 0.04 g/cm3) were significantly (p < 0.001) reduced in comparison with those of a control group (n = 15, age 5.0-20.5 years: aBMD 1.028 +/- 0.20 g/cm2; vBMD 0.35 +/- 0.04 g/cm3). Both aBMD and vBMD were also reduced in comparison with normal values for males (aBMD -2.66 +/- 0. 99 SDS, p < 0.001; vBMD -3.08 +/- 1.53 SDS, p < 0.0005) and females (aBMD -2.88 +/- 1.05 SDS, p < 0.001; vBMD -2.84 +/- 1.18 SDS, p < 0. 0007). Real lumbar bone density, assessed by computed tomography in 1 patient, was also reduced (-6.2 SDS and -3.5 SDS for male and female normal values, respectively). Biochemical markers of bone metabolism were normal and not significantly different in patients and controls. Girls with cAIS did not have more fractures than controls. In conclusion, both aBMD and vBMD are reduced in cAIS patients, while bone turnover and the fracture risk seem not to be increased. Our data indicate that both androgens and estrogens may be required for acquisition of bone density during childhood.
雄激素对骨矿化的调节有重要影响。由于其独特的外周代谢，雄激素可能通过激活雄激素和/或雌激素受体作用于骨骼。完全性雄激素不敏感综合征（cAIS）患者是评估雄激素对骨骼作用的天然模型。我们研究了10例cAIS患者（平均年龄13.70岁，范围4.7 - 19.8岁）的骨密度（BMD）；3例患者在性腺切除术前进行了研究；其他患者接受了去势手术，6例接受了激素替代治疗。通过双能X射线测量骨密度面积（aBMD）；使用公式vBMD = aBMD x [4/(π x宽度)]计算腰椎“表观”体积密度（vBMD）。与对照组（n = 15，年龄5.0 - 20.5岁：aBMD 1.028 +/- 0.20 g/cm²；vBMD 0.35 +/- 0.04 g/cm³）相比，患者的aBMD（0.72 +/- 0.16 g/cm²）和vBMD（0.23 +/- 0.04 g/cm³）显著降低（p < 0.001）。与男性正常值（aBMD -2.66 +/- 0.99 SDS，p < 0.001；vBMD -3.08 +/- 1.53 SDS，p < 0.0005）和女性正常值（aBMD -2.88 +/- 1.05 SDS，p < 0.001；vBMD -2.84 +/- 1.18 SDS，p < 0.0007）相比，aBMD和vBMD也降低。通过计算机断层扫描评估的1例患者的实际腰椎骨密度也降低（男性和女性正常值分别为-6.2 SDS和-3.5 SDS）。骨代谢的生化标志物正常，患者与对照组之间无显著差异。患有cAIS的女孩骨折情况并不比对照组多。总之，cAIS患者的aBMD和vBMD均降低，而骨转换和骨折风险似乎并未增加。我们的数据表明，儿童期获取骨密度可能需要雄激素和雌激素共同作用。
