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未经治疗的成年拉伦综合征(原发性生长激素不敏感)患者的身体成分

Body composition in untreated adult patients with Laron syndrome (primary GH insensitivity).

作者信息

Laron Zvi, Ginsberg Shira, Lilos Pearl, Arbiv Mira, Vaisman Nahum

机构信息

Endocrinology and Diabetes Research Unit, Schneider Children's Medical Centre, Petah Tikva, Israel.

出版信息

Clin Endocrinol (Oxf). 2006 Jul;65(1):114-7. doi: 10.1111/j.1365-2265.2006.02558.x.

Abstract

OBJECTIVE

To quantify body adiposity and its distribution in untreated adult patients with Laron syndrome (LS; primary GH insensitivity) caused by molecular defects of the GH receptor gene or postreceptor pathways and characterized by dwarfism, obesity, insulin resistance and hyperlipidaemia.

PATIENTS

Eleven LS patients (seven females and four males) aged 28-53 years were studied. Seven healthy males and six healthy females served as controls.

MEASUREMENTS

Body composition of the total body trunk, upper and lower extremities was determined using dual-energy X-ray absorptiometry (DEXA). Statistical analysis using an analysis of variance (anova) and Mann-Whitney nonparametric methods was performed separately in males and females.

RESULTS

Percentage body fat in the LS patients was much higher (P < 0.01) than that in the control population and the female LS patients were significantly more obese (59% total body fat) than the male patients (39% total body fat) (P < 0.002). It was also evident that in these types of patients with markedly increased body fat and decreased muscle and bone mass, body mass index (BMI) does not accurately reflect the body composition.

CONCLUSIONS

Lifelong congenital IGF-I deficiency leads to extreme adiposity.

摘要

目的

对因生长激素(GH)受体基因或受体后途径分子缺陷导致的、以侏儒症、肥胖症、胰岛素抵抗和高脂血症为特征的未经治疗的成年拉伦综合征(LS;原发性GH不敏感)患者的身体肥胖程度及其分布进行量化。

患者

研究了11例年龄在28 - 53岁之间的LS患者(7例女性和4例男性)。7例健康男性和6例健康女性作为对照。

测量

使用双能X线吸收法(DEXA)测定全身、躯干、上肢和下肢的身体成分。分别对男性和女性进行方差分析(anova)和曼-惠特尼非参数方法的统计分析。

结果

LS患者的体脂百分比比对照组高得多(P < 0.01),且女性LS患者(全身脂肪59%)比男性患者(全身脂肪39%)明显更肥胖(P < 0.002)。同样明显的是,在这些身体脂肪明显增加、肌肉和骨量减少的患者中,体重指数(BMI)不能准确反映身体成分。

结论

终身先天性胰岛素样生长因子-I(IGF-I)缺乏导致极度肥胖。

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