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血色素沉着症中的骨质疏松症:铁过载、性腺功能减退还是其他因素?

Osteoporosis in hemochromatosis: iron excess, gonadal deficiency, or other factors?

作者信息

Diamond T, Stiel D, Posen S

机构信息

Royal North Shore Hospital, Sydney, New South Wales, Australia.

出版信息

Ann Intern Med. 1989 Mar 15;110(6):430-6. doi: 10.7326/0003-4819-110-6-430.

DOI:10.7326/0003-4819-110-6-430
PMID:2919850
Abstract

STUDY OBJECTIVE

To define the prevalence, severity, type and pathogenesis of osteopenia in idiopathic hemochromatosis.

DESIGN

Prospective study conducted over 18 months.

SETTING

Tertiary care center.

SUBJECTS

Twenty-two men with idiopathic hemochromatosis and 20 age-matched controls. There were 5 hypogonadal patients, 9 eugonadal nonvenesected patients, and 8 eugonadal venesected patients.

MEASUREMENTS AND MAIN RESULTS

All patients and controls were evaluated by spinal radiography, spinal and forearm bone mineral density estimations, dynamic skeletal histomorphometry, and serum biochemistry. Ten patients (45%; 95% CI, 24% to 68%) had osteoporosis as defined by spinal and forearm bone density measurements. Trabecular bone volumes were significantly reduced in the patients (the difference in means between patients and age-matched controls was 3.9%; CI, 1.3% to 6.7%). No patient had osteomalacia. Hypogonadal men had lower bone mass measurements than eugonadal men (radial bone density: beta coefficient = -20.5; CI, -29.2 to -11.8; trabecular bone volume: beta coefficient = -7.1; CI, -10.8 to -3.3). Osteoid and osteoblastic surfaces and bone formation rates were significantly greater in the eugonadal venesected compared with the eugonadal nonvenesected persons (P less than 0.05 for all measurements).

CONCLUSIONS

A significant decrease in bone density is seen in idiopathic hemochromatosis, particularly when hypogonadism is present. Low serum free-testosterone concentrations rather than the calciotrophic hormones determine bone mass in this condition.

摘要

研究目的

明确特发性血色素沉着症中骨质减少的患病率、严重程度、类型及发病机制。

设计

为期18个月的前瞻性研究。

地点

三级医疗中心。

研究对象

22名患有特发性血色素沉着症的男性以及20名年龄匹配的对照者。其中有5名性腺功能减退患者、9名性腺功能正常且未接受静脉切开术的患者以及8名性腺功能正常且接受静脉切开术的患者。

测量指标及主要结果

所有患者及对照者均接受了脊柱X线摄影、脊柱和前臂骨矿物质密度评估、动态骨组织形态计量学以及血清生化检查。根据脊柱和前臂骨密度测量结果,10名患者(45%;95%可信区间,24%至68%)患有骨质疏松症。患者的小梁骨体积显著减少(患者与年龄匹配的对照者之间的均值差异为3.9%;可信区间,1.3%至6.7%)。无患者患有骨软化症。性腺功能减退的男性骨量测量值低于性腺功能正常的男性(桡骨骨密度:β系数 = -20.5;可信区间,-29.2至-11.8;小梁骨体积:β系数 = -7.1;可信区间,-10.8至-3.3)。与性腺功能正常且未接受静脉切开术的人相比,性腺功能正常且接受静脉切开术的人的类骨质和成骨细胞表面以及骨形成率显著更高(所有测量指标P均小于0.05)。

结论

在特发性血色素沉着症中可见骨密度显著降低,尤其是存在性腺功能减退时。在此种情况下,低血清游离睾酮浓度而非钙调节激素决定骨量。

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