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镰状细胞贫血患者的内分泌功能

Endocrine functions in sickle cell anaemia patients.

作者信息

el-Hazmi M A, Bahakim H M, al-Fawaz I

机构信息

Department of Medical Biochemistry, College of Medicine & King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.

出版信息

J Trop Pediatr. 1992 Dec;38(6):307-13. doi: 10.1093/tropej/38.6.307.

Abstract

In this study, 80 male and female sickle cell patients, aged 4-50 years, with mild (severity index, SI < 6) and severe (SI > or = 6) forms of the disease were investigated). The levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone, cortisol, growth hormone (GH), free thyroxine (T4), and free triiodothyronine (T3) were determined. The results were evaluated and the mean +/- 2 SD values were compared with those obtained in age- and sex-matched normal controls. The findings indicated gonadal hypofunction in the sickle cell patients, but with varied deviations from the mean results. Patients with the severe form of the sickle cell disease showed more frequent abnormalities of LH, FSH, cortisol and testosterone in comparison with the patients with a mild disease. The LH, FSH, cortisol and testosterone levels were lower, while T3 and T4 did not show significant differences between patients and the controls. The results suggest that the sickle cell gene abnormality has an adverse effect on endocrine functions. Follow-up and appropriate management of endocrine dysfunctions are advocated in such patients.

摘要

在本研究中,对80名年龄在4至50岁之间、患有轻度(严重程度指数,SI<6)和重度(SI≥6)镰状细胞病的男性和女性患者进行了调查。测定了促黄体生成素(LH)、促卵泡生成素(FSH)、睾酮、皮质醇、生长激素(GH)、游离甲状腺素(T4)和游离三碘甲状腺原氨酸(T3)的水平。对结果进行了评估,并将平均值±2标准差与年龄和性别匹配的正常对照组的结果进行了比较。研究结果表明镰状细胞病患者存在性腺功能减退,但与平均结果的偏差各不相同。与轻度镰状细胞病患者相比,重度镰状细胞病患者的LH、FSH、皮质醇和睾酮异常更为频繁。患者的LH、FSH、皮质醇和睾酮水平较低,而T3和T4在患者和对照组之间没有显著差异。结果表明,镰状细胞基因异常对内分泌功能有不良影响。提倡对这类患者进行内分泌功能障碍的随访和适当管理。

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