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41例库欣综合征:库欣综合征(肾上腺腺瘤)与库欣病(肾上腺增生)的比较

[Forty-one cases of Cushing's syndrome: a comparison between Cushing's syndrome (adrenal adenoma) and Cushing's disease (adrenal hyperplasia)].

作者信息

Komiya I, Takasu N, Ohara N, Nagasawa Y, Ohtsuka H, Asawa T, Yamada T

机构信息

Department of Geriatrics, Endocrinology and Metabolism, Shinshu University School of Medicine, Nagano.

出版信息

Nihon Naibunpi Gakkai Zasshi. 1992 Jun 20;68(6):607-22. doi: 10.1507/endocrine1927.68.6_607.

Abstract

We experienced 41 cases of Cushing's syndrome (12 males and 29 females, 15 years old - 65 years old) during the last 20 years. These included 20 patients with unilateral adrenal adenoma (Cushing's syndrome), 19 patients with bilateral adrenal hyperplasia (Cushing's disease), one patient with adrenal carcinoma and one patient with primary adrenocortical nodular dysplasia (PAND). Moreover, these cases included some special ones, i.e. 5 cases with destructive thyroiditis after treatment, 2 cases with aggravation of arthritis after treatment, a case of Carney's complex with PAND, one case with paradoxical response to dexamethasone, and one case combined with empty sella syndrome. The most specific clinical signs were moon face (95% occurrence), hypertension (95%) and subcutaneous bruising (80%). Other significant signs were eye edema (66%), buffalo hump (68%), subcutaneous purpura (63%) and osteoporosis (49%). Skin striae was not a common sign in our cases (41%). Renal stone was observed in only 20% of our patients but was a significant sign in this syndrome. There was no difference in the occurrence of each clinical sign between Cushing's syndrome and Cushing's disease. The elevation of white blood cell count (WBC) and serum sodium, a decrease of serum potassium, and a decrease of reabsorption of phosphate (%TRP) were observed. Thyroid-stimulating hormone (TSH) and human growth hormone (HGH) were suppressed in patients with Cushing's syndrome and patients with Cushing's disease. These results were consistent with those of previous reports. However, luteinizing hormone (LH), follicle-stimulating hormone (FSH) and prolactin (PRL) were high in those patients with Cushing's syndrome and those with Cushing's disease. Oral glucose tolerance test was carried out in 34 patients before and after treatment. Thirty-one percent of those had diabetes mellitus and 26% had impaired glucose tolerance (IGT). The response of IRI in this test was high in patients with Cushing's syndrome and patients with Cushing's disease, and decreased 4 weeks after treatment in those with Cushing's syndrome but remained high in those with Cushing's disease. Plasma ACTH level and urinary 17-OHCS excretion were significantly higher in Cushing's disease than in Cushing's syndrome. During an 8mg-high-dose dexamethasone suppression test, urinary 17-OHCS excretion in 13 of 14 patients with Cushing's disease (93%) was suppressed by more than 50% of baseline on the second day of testing. However, all of 18 patients with Cushing's syndrome, who had an 8mg-dexamethasone suppression test, failed to suppress urinary 17-OHCS by 50% of baseline.(ABSTRACT TRUNCATED AT 400 WORDS)

摘要

在过去20年中,我们共收治了41例库欣综合征患者(男性12例,女性29例,年龄15岁至65岁)。其中包括20例单侧肾上腺腺瘤(库欣综合征)、19例双侧肾上腺增生(库欣病)、1例肾上腺癌和1例原发性肾上腺皮质结节性发育异常(PAND)。此外,这些病例还包括一些特殊情况,即5例治疗后出现破坏性甲状腺炎、2例治疗后关节炎加重、1例伴有PAND的卡尼综合征、1例对地塞米松出现矛盾反应以及1例合并空蝶鞍综合征。最具特征性的临床体征为满月脸(发生率95%)、高血压(95%)和皮下瘀斑(80%)。其他显著体征包括眼睑水肿(66%)、水牛背(68%)、皮下紫癜(63%)和骨质疏松(49%)。皮肤紫纹在我们的病例中并不常见(41%)。仅20%的患者观察到肾结石,但这是该综合征的一个显著体征。库欣综合征和库欣病患者各临床体征的发生率无差异。观察到白细胞计数(WBC)和血清钠升高、血清钾降低以及磷酸盐重吸收(%TRP)降低。库欣综合征和库欣病患者的促甲状腺激素(TSH)和人生长激素(HGH)均受到抑制。这些结果与既往报道一致。然而,库欣综合征和库欣病患者的黄体生成素(LH)、卵泡刺激素(FSH)和催乳素(PRL)均升高。对34例患者在治疗前后进行了口服葡萄糖耐量试验。其中31%患有糖尿病,26%有糖耐量受损(IGT)。在该试验中,库欣综合征和库欣病患者的胰岛素释放指数(IRI)反应较高,库欣综合征患者治疗4周后降低,但库欣病患者仍保持较高水平。库欣病患者的血浆促肾上腺皮质激素(ACTH)水平和尿17-羟皮质类固醇(17-OHCS)排泄量显著高于库欣综合征患者。在8mg高剂量地塞米松抑制试验中,14例库欣病患者中有13例(93%)在试验第二天尿17-OHCS排泄量被抑制超过基线的50%。然而,18例接受8mg地塞米松抑制试验的库欣综合征患者均未能将尿17-OHCS抑制至基线的50%。(摘要截断于400字)

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