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儿童库欣病诊断时及成功治疗后的骨矿物质密度

Bone mineral density at diagnosis and following successful treatment of pediatric Cushing's disease.

作者信息

Scommegna S, Greening J P, Storr H L, Davies K M, Shaw N J, Monson J P, Grossman A B, Savage M O

机构信息

Department of Endocrinology, St Bartholomew's Hospital, Birmingham, UK.

出版信息

J Endocrinol Invest. 2005 Mar;28(3):231-5. doi: 10.1007/BF03345378.

Abstract

Bone mineral density (BMD) is frequently reduced in children and adolescents with Cushing's disease (CD), but there is little follow-up data after cure. BMD was determined by dual energy X-ray absorptiometry (DEXA) in two groups of patients with CD. Group 1 comprised 8 patients, 5 males and 3 females, aged 12.4 yr (8.2-16.8), assessed at diagnosis. Group 2 comprised 11 subjects, 6 males and 5 females, diagnosed at age 13.3 yr (6.4-17.4), cured by transsphenoidal surgery (TSS) (no.=7) or TSS + pituitary irradiation (no.=4). They had measurement of BMD, at mean age of 18.3 yr (11.1-28.5), i.e. 4.5 yr (0.8-11.4) after cure. Four patients, mean age 20.2 yr (17.6-22.4), had repeated DEXA'scans, 1-4 times, for up to 5.8 yr. After cure, GH deficiency was present in 9 patients and treated with hGH in 8. In Group 1, patients' L2-L4 volumetric (v)BMD Z-score was variable with a mean of -1.04 (-3.21-0.11). L2-L4 vBMD Z-score values correlated negatively with midnight cortisol (p < 0.05). In Group 2, mean L2-L4 vBMD was -0.38 (-1.0-0.13); and in 7/11, mean femoral neck (FN) areal (a)BMD Z-score was 0.14 (-1.62-2.46). FN aBMD Z-score was higher than L2-L4 aBMD Z-score (p < 0.05). In patients with repeated scans, mean change in L2-L4 vBMD Z-score was 0.20 (-0.15-0.45), and mean change in FN aBMD Z-score 0.03 (-0.53-0.38). These findings show variability of BMD at diagnosis and near normal BMD after cure of pediatric CD, suggesting that with appropriate replacement of pituitary hormone deficiency normal peak bone mass is achievable.

摘要

库欣病(CD)患儿及青少年的骨矿物质密度(BMD)常降低,但治愈后的随访数据很少。通过双能X线吸收法(DEXA)测定了两组CD患者的BMD。第1组包括8例患者,5例男性和3例女性,诊断时年龄为12.4岁(8.2 - 16.8岁)。第2组包括11例受试者,6例男性和5例女性,诊断时年龄为13.3岁(6.4 - 17.4岁),通过经蝶窦手术(TSS)(n = 7)或TSS +垂体照射(n = 4)治愈。他们在平均年龄18.3岁(11.1 - 28.5岁)时进行了BMD测量,即治愈后4.5年(0.8 - 11.4年)。4例患者,平均年龄20.2岁(17.6 - 22.4岁),进行了1 - 4次DEXA扫描,最长达5.8年。治愈后,9例患者存在生长激素缺乏,其中8例接受了生长激素治疗。在第1组中,患者L2 - L4椎体(v)BMD Z值变化不定,平均为 - 1.04(- 3.21 - 0.11)。L2 - L4 vBMD Z值与午夜皮质醇呈负相关(p < 0.05)。在第2组中,L2 - L4平均vBMD为 - 0.38(- 1.0 - 0.13);在11例中的7例中,股骨颈(FN)面积(a)BMD Z值平均为0.14(- 1.62 - 2.46)。FN aBMD Z值高于L2 - L4 aBMD Z值(p < 0.05)。在进行重复扫描的患者中,L2 - L4 vBMD Z值的平均变化为0.20(- 0.15 - 0.45),FN aBMD Z值的平均变化为0.03(- 0.53 - 0.38)。这些发现表明,小儿CD诊断时BMD存在变异性,治愈后BMD接近正常,提示通过适当补充垂体激素缺乏可实现正常的骨峰值。

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