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[遗传性血色素沉着症的内分泌并发症]

[Endocrine complications of genetic hemochromatosis].

作者信息

Paris I, Hermans M, Buysschaert M

机构信息

Service d'Endocrinologie et Nutrition, Cliniques Universitaires St Luc, Bruxelles, Belgique.

出版信息

Acta Clin Belg. 1999 Dec;54(6):334-45. doi: 10.1080/17843286.1999.11754257.

Abstract

The authors report the prevalence and severity of endocrine complications in a cohort of 115 patients suffering form genetic hemochromatosis and followed since two decades. Already 40% of them had developed diabetes at the time of diagnosis of hemochromatosis, which was made at the age of 50 +/- 12 years (m +/- SD). Hypogonadism was evidenced in 42% of the patients. In most of them, it was considered as secondary to pituitary lesions as assessed by GnRH tests. No other endocrine complications, in particular thyroid disease, were evidenced during follow up. On the other hand, it was also of interest to note that liver cirrhosis was observed in 52% of the patients at the time of hemochromatosis diagnosis. A relationship between cirrhosis, diabetes and hypogonadism was also assessed in these patients. We conclude to the still high prevalence of endocrine complications in genetic hemochromatosis.

摘要

作者报告了115例遗传性血色素沉着症患者队列中内分泌并发症的患病率和严重程度,这些患者已被随访了二十年。在血色素沉着症诊断时(诊断年龄为50±12岁,均值±标准差),其中已有40%的患者患上了糖尿病。42%的患者存在性腺功能减退。在大多数患者中,根据促性腺激素释放激素(GnRH)测试评估,性腺功能减退被认为是继发于垂体病变。在随访期间未发现其他内分泌并发症,尤其是甲状腺疾病。另一方面,值得注意的是,在血色素沉着症诊断时,52%的患者出现了肝硬化。还评估了这些患者中肝硬化、糖尿病和性腺功能减退之间的关系。我们得出结论,遗传性血色素沉着症中内分泌并发症的患病率仍然很高。

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