McNamara Lynette, Panz Vanessa R, Raal Frederick J, Paiker Janice, Joffe Barry I, Gordeuk Victor R, MacPhail A Patrick
Division of Haematology/Oncology, Department of Medicine, University of the Witwatersrand Medical School, Johannesburg, South Africa.
Endocrine. 2003 Aug;21(3):241-4. doi: 10.1385/ENDO:21:3:241.
Endocrine disturbances, notably diabetes, have been well described as a complication of iron overload due to hereditary hemochromatosis and beta-thalassemia. Dysfunction of the hypothalamic-pituitary-adrenal (HPA) axis has also been well documented. The pattern of iron loading in African iron overload with saturated transferrin is similar to that seen in hereditary hemochromatosis. In addition, many symptoms ascribed to pituitary dysfunction are common to both conditions. The present study was undertaken to assess whether a similar pattern of endocrine dysfunction occurs in African iron overload. Thirty subjects with African iron overload and transferrin saturation >50%, plus 30 age and sex matched normal controls were studied. An iron profile, fasting plasma glucose, cortisol, DHEA-S, LH, FSH, growth hormone, prolactin, TSH, and FT4 levels were measured in all 60 subjects as well as testosterone in the males and estradiol in the females. Iron loading in the subjects with increased transferrin saturation ranged from moderate to severe. No significant differences were found in the mean testosterone, estradiol, LH, DHEA-S, growth hormone, prolactin, or TSH levels between the subjects and normal controls. In female subjects, although within the normal range, the mean FSH level was significantly higher, probably due to their being somewhat older and in a more advanced stage of menopause than the control females. Mean cortisol concentrations were increased in both genders in the patient group, significantly so in the females; however, values were within the reference range. We conclude therefore that there appears to be no major impairment of endocrine function in the basal state in African iron overload subjects with moderate to severe degrees of iron loading.
内分泌紊乱,尤其是糖尿病,已被充分描述为遗传性血色素沉着症和β地中海贫血所致铁过载的一种并发症。下丘脑-垂体-肾上腺(HPA)轴功能障碍也有充分的文献记载。非洲人铁过载伴转铁蛋白饱和时的铁负荷模式与遗传性血色素沉着症所见相似。此外,两种情况都有许多归因于垂体功能障碍的症状。本研究旨在评估非洲人铁过载是否会出现类似的内分泌功能障碍模式。研究了30名转铁蛋白饱和度>50%的非洲人铁过载患者,以及30名年龄和性别匹配的正常对照者。测定了所有60名受试者的铁参数、空腹血糖、皮质醇、硫酸脱氢表雄酮(DHEA-S)、促黄体生成素(LH)、促卵泡生成素(FSH)、生长激素、催乳素、促甲状腺激素(TSH)和游离甲状腺素(FT4)水平,男性还测定了睾酮水平,女性测定了雌二醇水平。转铁蛋白饱和度增加的受试者的铁负荷从中度到重度不等。受试者与正常对照者之间的平均睾酮、雌二醇、LH、DHEA-S、生长激素、催乳素或TSH水平未发现显著差异。在女性受试者中,尽管平均FSH水平在正常范围内,但显著较高,这可能是因为她们比对照女性年龄稍大且处于更晚期的绝经阶段。患者组中男女的平均皮质醇浓度均升高,女性升高显著;然而,数值在参考范围内。因此,我们得出结论,在中度至重度铁负荷的非洲人铁过载受试者的基础状态下,内分泌功能似乎没有重大损害。