Neely E K, Marcus R, Rosenfeld R G, Bachrach L K
Department of Pediatrics, Stanford University Medical Center, California 94305.
J Clin Endocrinol Metab. 1993 Apr;76(4):861-6. doi: 10.1210/jcem.76.4.8473397.
Deficits in bone mineral have been widely reported in Turner syndrome. The bone mineral status of 19 adolescents with Turner syndrome (16 receiving GH therapy) was evaluated by dual photon absorptiometry of the lumbar spine and whole body and compared with a normal female control group (n = 45) with the same mean age (14.3 yr). The conventional measurements of bone mass, bone mineral content (BMC = g), and bone mineral density (BMD = g/cm2), as well as bone mineral apparent density (BMAD = g/cm3), an expression of bone mineral adjusted for bone volume, were determined for both sites. Although mean BMC was decreased in Turner females, mean BMD and BMAD in the two groups were not significantly different. Analyzed in relation to chronologic age, bone age, height, and pubertal status, mean BMD and BMAD values in Turner subjects were equal to or greater than that of controls. BMD and BMAD were elevated in the Turner group vs. controls matched for height. In subjects with bone age less than or equal to 12.5 yr, mean spinal BMAD was unexpectedly greater in Turner patients compared with controls (0.148 +/- 0.011 vs. 0.134 +/- 0.013, P = 0.009). When data were analyzed by pubertal status, mean spinal BMD and BMAD in subjects with Tanner breast stages 1-2 were higher in the Turner group than in the controls (BMAD 0.146 +/- 0.011 vs. 0.132 +/- 0.015, P = 0.015). No differences were seen in mid- to late pubertal females. Bone mineral properties were additionally reassessed after a mean interval of 1.3 yr in 10 of the subjects with Turner syndrome. Percentage increases in mean follow-up spinal BMD and BMAD were greater in 5 subjects begun on estrogen replacement than in 5 untreated patients. We conclude that: 1) bone mineral values in adolescents with Turner syndrome on GH therapy are not abnormal, 2) lumbar bone mineral is greater in younger Turner adolescents matched with controls for bone age or pubertal status, a difference which could relate to GH therapy, and 3) estrogen therapy may augment bone mineral accretion in Turner syndrome, but early estrogen replacement cannot be justified on the basis of bone mineral status.
特纳综合征患者骨矿物质缺乏的情况已被广泛报道。通过腰椎和全身双光子吸收法评估了19例特纳综合征青少年患者（其中16例接受生长激素治疗）的骨矿物质状况，并与平均年龄相同（14.3岁）的正常女性对照组（n = 45）进行比较。测定了两个部位的传统骨量测量指标，即骨矿物质含量（BMC = g）、骨矿物质密度（BMD = g/cm²）以及骨矿物质表观密度（BMAD = g/cm³，一种根据骨体积调整的骨矿物质表达方式）。虽然特纳综合征女性的平均BMC降低，但两组的平均BMD和BMAD并无显著差异。根据实际年龄、骨龄、身高和青春期状态进行分析，特纳综合征患者的平均BMD和BMAD值等于或高于对照组。与身高匹配的对照组相比，特纳综合征组的BMD和BMAD升高。在骨龄小于或等于12.5岁的患者中，与对照组相比，特纳综合征患者的平均脊柱BMAD出人意料地更高（0.148±0.011 vs. 0.134±0.013，P = 0.009）。根据青春期状态分析数据时，坦纳乳房分期为1 - 2期的特纳综合征组患者的平均脊柱BMD和BMAD高于对照组（BMAD 0.146±0.011 vs. 0.132±0.015，P = 0.015）。青春期中后期女性未见差异。对10例特纳综合征患者平均间隔1.3年后再次评估骨矿物质特性。开始接受雌激素替代治疗的5例患者的平均随访脊柱BMD和BMAD的百分比增加幅度大于5例未治疗患者。我们得出以下结论：1）接受生长激素治疗的特纳综合征青少年的骨矿物质值并无异常；2）骨龄或青春期状态与对照组匹配的较年轻特纳综合征青少年的腰椎骨矿物质含量更高，这种差异可能与生长激素治疗有关；3）雌激素治疗可能会增加特纳综合征患者的骨矿物质积聚，但基于骨矿物质状况，早期雌激素替代治疗并无依据。
