Walsh C H, Murphy A L, Cunningham S, McKenna T J
South Infirmary, Victoria Hospital, Cork, Ireland.
Clin Endocrinol (Oxf). 1994 Oct;41(4):439-43. doi: 10.1111/j.1365-2265.1994.tb02574.x.
A number of studies have suggested that both primary (Addison's disease) and secondary adrenal failure may be caused by idiopathic haemochromatosis. There is little information on mineralocorticoid secretion. We have assessed the mineralocorticoid and glucocorticoid status of patients with idiopathic haemochromatosis.
Cross-sectional study.
Eighteen males and one female with confirmed idiopathic haemochromatosis were investigated. Seven of the subjects had hepatic cirrhosis and three had hypogonadotrophic hypogonadism.
A short Synacthen (tetracosactrin) test was undertaken in addition to an overnight metyrapone test and an insulin stress test. In addition, plasma aldosterone (PA) and plasma renin activity (PRA) were measured in a group of patients in the basal state and again in the upright position.
The short Synacthen test, overnight metyrapone test and insulin stress test were normal in all subjects. The PA, PRA, and PA/PRA ratios in the upright position were not significantly different in the patients with idiopathic haemochromatosis compared to control subjects.
In this patient population, no abnormality of either the pituitary adrenal axis or mineralocorticoid status was detected. Adrenocortical dysfunction is likely to be exceptionally rare in idiopathic haemochromatosis.
多项研究表明,原发性(艾迪生病)和继发性肾上腺功能不全可能由特发性血色素沉着症引起。关于盐皮质激素分泌的信息较少。我们评估了特发性血色素沉着症患者的盐皮质激素和糖皮质激素状态。
横断面研究。
对18名男性和1名确诊为特发性血色素沉着症的女性进行了调查。其中7名受试者患有肝硬化,3名患有低促性腺激素性腺功能减退症。
除了进行过夜甲吡酮试验和胰岛素应激试验外,还进行了短程促肾上腺皮质激素(二十四肽促皮质素)试验。此外,对一组患者在基础状态下以及站立位时测量血浆醛固酮(PA)和血浆肾素活性(PRA)。
所有受试者的短程促肾上腺皮质激素试验、过夜甲吡酮试验和胰岛素应激试验均正常。与对照受试者相比,特发性血色素沉着症患者站立位时的PA、PRA以及PA/PRA比值无显著差异。
在该患者群体中,未检测到垂体-肾上腺轴或盐皮质激素状态的异常。肾上腺皮质功能障碍在特发性血色素沉着症中可能极为罕见。