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接受酶替代疗法的Ⅰ型戈谢病患者体内的胃饥饿素、瘦素和脂联素水平。

Ghrelin, leptin and adiponectin levels in Gaucher disease type I patients on enzyme replacement therapy.

作者信息

Doneda Divair, Lopes André L, Teixeira Bruno C, Mittelstadt Suzana D, Moulin Cileide C, Schwartz Ida V D

机构信息

LTD Laboratory, Nutrition Program, Medical School, Universidade Federal do Rio Grande do Sul, Brazil.

School of Physical Education, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil; Physical Education Program, University Center UNIVATES, Lageado, RS, Brazil.

出版信息

Clin Nutr. 2015 Aug;34(4):727-31. doi: 10.1016/j.clnu.2014.08.010. Epub 2014 Aug 30.

Abstract

BACKGROUND

Gaucher disease type I (GD type I) is characterized by clinical heterogeneity and is associated with metabolic abnormalities such as increased basal metabolic rate.

OBJECTIVE

To evaluate ghrelin, leptin and adiponectin levels in patients with GD type I on enzyme replacement therapy (ERT).

SUBJECTS AND METHODS

A cross-sectional study of patients with GD type I (n = 15), matched for sex, age and BMI with healthy controls. The levels of glucose, insulin, ghrelin, leptin and adiponectin were assessed in both groups. Insulin resistance was defined by the index HOMA-IR.

RESULTS

Eight patients had adequate weight, seven were overweight (4 preobese, 3 obese class I). Eight patients presented metabolic syndrome, five of whom with insulin resistance. The median levels of ghrelin, leptin and adiponectin of the patients did not differ from those of the controls. Ghrelin and adiponectin levels were correlated with each other; inversely correlated with BMI, waist circumference and triglyceride levels; and directly correlated with HDL-cholesterol. Leptin levels were inversely correlated with LDL-cholesterol and directly correlated with BMI, waist circumference, enzyme dosage, triglycerides, insulin, and HOMA-IR.

CONCLUSIONS

Metabolic syndrome and overweight appear to be common in patients with GD type I on ERT. As leptin was strongly associated with insulin and HOMA index, it could become a biomarker to assess early evidence of insulin resistance in patients with GD. Further studies are needed to investigate the associations found.

摘要

背景

I型戈谢病(GD I型)具有临床异质性,且与基础代谢率升高等代谢异常有关。

目的

评估接受酶替代疗法(ERT)的GD I型患者的胃饥饿素、瘦素和脂联素水平。

对象与方法

一项对15例GD I型患者的横断面研究,这些患者在性别、年龄和体重指数方面与健康对照相匹配。对两组患者的血糖、胰岛素、胃饥饿素、瘦素和脂联素水平进行评估。胰岛素抵抗通过HOMA-IR指数定义。

结果

8例患者体重正常,7例超重(4例为肥胖前期,3例为I级肥胖)。8例患者出现代谢综合征,其中5例有胰岛素抵抗。患者的胃饥饿素、瘦素和脂联素的中位数水平与对照组无差异。胃饥饿素和脂联素水平相互相关;与体重指数、腰围和甘油三酯水平呈负相关;与高密度脂蛋白胆固醇呈正相关。瘦素水平与低密度脂蛋白胆固醇呈负相关,与体重指数、腰围、酶剂量、甘油三酯、胰岛素和HOMA-IR呈正相关。

结论

接受ERT的GD I型患者中代谢综合征和超重似乎很常见。由于瘦素与胰岛素和HOMA指数密切相关,它可能成为评估GD患者胰岛素抵抗早期证据的生物标志物。需要进一步研究来调查所发现的关联。

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