Clinical, endocrine and metabolic aspects of the Werner syndrome compared with those of normal aging.
Clinical, endocrine and metabolic aspects of the Werner syndrome compared with those of normal aging.
复制标题
维尔纳综合征的临床、内分泌和代谢方面与正常衰老情况的比较。
DOI：
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发表时间：
1985
影响因子：
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通讯作者：
Kazunori Yamada
中科院分区：
文献类型：
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作者：
H. Imura;Y. Nakao;H. Kuzuya;M. Okamoto;Mikiko Okamoto;Kazunori Yamada
通过对日本各大医院进行问卷调查，收集了102例维尔纳综合征患者的临床特征资料并进行了分析。男女比例为3:2，直系和家族发病率分别为51%和39.4%。患者分为3个亚组；1组、2组和3组分别缺乏身材矮小、白内障和性腺功能减退。各组的临床特征有所不同。将Werner综合征患者的内分泌和代谢异常与正常老年人进行比较。血浆生长激素对胰岛素和精氨酸的反应受损更常见，血浆促甲状腺激素对TRH的反应受损在Werner综合征患者中比在老年受试者中更少见。大多数患者的血浆LH和FSH水平高于年龄和性别匹配的对照组；此外，他们的血清睾酮浓度低于年龄匹配的对照组，睾丸活检显示比老年受试者更明显的萎缩。血清三碘甲状腺原氨酸水平倾向于低于年龄匹配的对照组。口服葡萄糖耐量试验显示55%的患者患有糖尿病，22%的患者葡萄糖耐量受损，而空腹血糖水平升高的仅占20%。与正常老年人相比，沃纳综合征患者血浆胰岛素对葡萄糖的反应更为夸张。正糖钳法显示，Werner综合征患者的葡萄糖处置率和胰岛素敏感性指标均低于同龄正常人。Werner综合征患者红细胞胰岛素结合位点数量正常。这些结果提示Werner综合征中胰岛素抵抗存在受体后缺陷。
Data on the clinical features of the Werner syndrome in 102 patients in Japan were collected by sending questionnaires to major hospitals and analyzed. The male-to-female ratio was 3 to 2 and the incidences of consanguinity and familial occurrence were 51% and 39.4%, respectively. These patients were divided into 3 subgroups; group 1, 2, and 3 lacked short stature, cataract, and hypogonadism, respectively. Each group had somewhat different clinical features. Endocrine and metabolic abnormalities in the Werner syndrome patients were compared with those in normal aged subjects. Impaired plasma growth-hormone responses to insulin and arginine were more common and impaired plasma thyrotropin responses to TRH were less common in the Werner syndrome patients than in aged subjects. Plasma LH and FSH levels were higher in most patients than those in age- and sex-matched controls; also, their serum testosterone concentrations were lower than those in age-matched controls and testicular biopsy revealed more marked atrophy than in aged subjects. Serum triiodothyronine levels tended to be lower than in age-matched controls. Oral glucose tolerance test revealed diabetic glucose tolerance in 55% and impaired glucose tolerance in 22%, although fasting blood glucose levels were elevated only in 20%. Plasma insulin response to glucose was more exaggerated in those with the Werner syndrome than in normal aged subjects. The euglycemic glucose clamp method revealed lower glucose disposal rates and insulin sensitivity indices in the Werner syndrome than in normal subjects of similar age. The number of erythrocyte insulin-binding sites was normal in the Werner syndrome patients. These results suggest a postreceptor defect in insulin resistance in the Werner syndrome.