The progression from obesity to type 2 diabetes in Alström syndrome.
The progression from obesity to type 2 diabetes in Alström syndrome.
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DOI：
10.1111/j.1399-5448.2011.00789.x
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发表时间：
2012-02
影响因子：
3.4
通讯作者：
Vettor R
中科院分区：
文献类型：
--
作者：
Bettini V;Maffei P;Pagano C;Romano S;Milan G;Favaretto F;Marshall JD;Paisey R;Scolari F;Greggio NA;Tosetto I;Naggert JK;Sicolo N;Vettor R
Alström综合征（ALMS）是一种罕见的常染色体隐性单基因疾病，与肥胖、高胰岛素血症和葡萄糖代谢改变相关，常导致年轻时发生2型糖尿病。在一组ALMS患者和匹配的对照组中研究了体重和代谢之间的关系。在一项横断面研究中，将15例ALMS患者（8例男性，7例女性，年龄3-51岁）与年龄和体重匹配的对照人群进行了比较。人体测量参数，脂肪量，葡萄糖和胰岛素分泌的基础和动态（OGTT）条件进行了测量。进一步的人体测量和身体成分数据来自一个国际组的27名ALMS患者（13名男性，14名女性，年龄范围：4-29岁）。在ALMS中，我们观察到年龄与身高、体重和BMI的SDS之间呈负相关。ALMS受试者的OGTT血糖曲线与年龄匹配的对照组相似，而胰岛素反应明显更大。在ALMS个体中，胰岛素反应随年龄的增长而降低。我们记录了ALMS中衍生指数HOMA-IR、ISI、HOMA%β细胞和胰岛素原指数的病理值，但与胰岛素抵抗指数不同，β细胞功能指数显示出随年龄的显著降低。在ALMS中，从早发性肥胖向空腹血糖受损或IGT和显性糖尿病的进展主要是由于β细胞胰岛素分泌的进行性失败，即使在体重减轻的情况下，胰岛素抵抗也不会随着年龄的增长而进一步恶化。
Alström syndrome (ALMS) is a rare autosomal recessive monogenic disease associated with obesity, hyperinsulinemia and alterations of glucose metabolism that often lead to the development of type 2 diabetes in a young age. Relationship between weight and metabolism has been studied in a group of ALMS patients and matched controls. Fifteen ALMS patients (8 M, 7 F, aged 3-51 yrs) were compared in a cross-sectional study with an age- and weight-matched control population. Anthropometric parameters, fat mass, glucose and insulin secretion in basal and dynamic (OGTT) conditions were measured. Further anthropometric and body composition data were obtained from an International group of 27 ALMS patients (13 M, 14 F, age range: 4-29 yrs). In ALMS we observed an inverse correlation between age and SDS for height, weight and BMI. The OGTT glycemic curves of ALMS subjects were similar to those of age-matched controls, while insulin response was clearly greater. In ALMS individuals the insulin response showed a reduction with age. We documented pathologic values of the derived indices HOMA-IR, ISI, HOMA%β cell and Insulinogenic Index in ALMS, but unlike the insulin-resistance indices, the beta-cell function indices showed a significant reduction with age. In ALMS the progression from the early onset obesity towards the impaired fasting glucose or IGT and overt diabetes is mostly due to a progressive failure of β-cell insulin secretion without any further worsening of insulin resistance with age, even in the presence of weight reduction.
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影响因子：
5.1
作者：
Satman, I;Yilmaz, MT;Devrim, S
通讯作者：
Devrim, S
影响因子：
5.8
作者：
Minton, J. A. L.;Owen, K. R.;Barrett, T. G.
通讯作者：
Barrett, T. G.
影响因子：
4.5
作者：
Li, Guochun;Vega, Raquel;Nelms, Keats;Gekakis, Nicholas;Goodnow, Christopher;McNamara, Peter;Wu, Hua;Hong, Nancy A.;Glynne, Richard
通讯作者：
Glynne, Richard
影响因子：
8.2
作者：
MATTHEWS, DR;HOSKER, JP;TURNER, RC
通讯作者：
TURNER, RC
影响因子：
--
作者：
Marshall, JD;Bronson, RT;Nishina, PM
通讯作者：
Nishina, PM