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迟发性皮肤卟啉症中的碳水化合物代谢

Carbohydrate metabolism in porphyria cutanea tarda.

作者信息

Franks A G, Pulini M, Bickers D R, Rayfield E J, Harber L C

出版信息

Am J Med Sci. 1979 Mar-Apr;277(2):163-71. doi: 10.1097/00000441-197903000-00004.

Abstract

Porphyria cutanea tarda (PCT) has a known increased incidence of diabetes mellitus and hepatic involvement. We investigated glucose tolerance and glucoregulatory hormone alterations in seven patients with PCT and correlated these results with hepatic histology by percutaneous liver biopsy. Abnormal glucose tolerance was observed in six of the seven patients (87%). Fasting serum insulin levels were normal range, and normal glucose and growth hormone responses to standard, exogenous intravenous insulin were observed. Fasting serum glucagon and urine free cortisol levels were normal in those patients in whom they were measured. While varying degrees of abnormalities were found on histopathologic exam of the liver biopsies, no patient met the criteria for cirrhosis, and none of the patients demonstrated abnormal levels of insulin counterregulatory hormones commonly seen in cirrhosis. Thus, liver disease may not be the sole cause of the observed glucose intolerance and hyperinsulinemia in PCT patients.

摘要

迟发性皮肤卟啉病(PCT)患者患糖尿病和肝脏受累的发生率已知会增加。我们调查了7例PCT患者的糖耐量和糖调节激素变化,并通过经皮肝活检将这些结果与肝脏组织学进行关联。7例患者中有6例(87%)糖耐量异常。空腹血清胰岛素水平在正常范围内,对标准外源性静脉注射胰岛素的血糖和生长激素反应正常。所测患者的空腹血清胰高血糖素和尿游离皮质醇水平正常。虽然肝活检的组织病理学检查发现了不同程度的异常,但没有患者符合肝硬化标准,且没有患者出现肝硬化中常见的胰岛素对抗调节激素水平异常。因此,肝脏疾病可能不是PCT患者中观察到的糖耐量异常和高胰岛素血症的唯一原因。

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