继发性肾上腺皮质功能减退症临床分析

慢性肾上腺皮质功能减退症是各种原因引起肾上腺皮质功能低下的疾病,从病因上可分为原发性和继发性肾上腺皮质功能减退症.近年来继发性肾上腺皮质功能减退症的发病率较前有升高趋势[1].但其发病较隐匿,早期症状缺乏特异性,临床早期诊断率不高.本研究对21例继发性肾上腺皮质功能减退症患者的临床资料进行回顾分析,旨在探讨该病的病因构成和早期诊断的意义,以加深临床医生对于本病的认识.     

肾上腺皮质功能减退症的诊断和鉴别诊断

肾上腺皮质功能减退症按病因可分为原发性和继发性，按病程可分为慢性和急性，现分述如下：１原发性肾上腺皮质功能减退症１．１病因和发病机制１．１．１慢性起病原发性慢性肾上腺皮质功能减退症，即阿狄森（Ａｄｄｉｓｏｎ）病，发病率不高，我国尚无确切的流行病学资料。其病因主要有：肾上腺结核：在结核病发病率高的国家和地区，是原发性肾上腺皮质功能减退症的首要原因，双侧肾上腺组织被破坏的程度常常超过９０％，约５０％的患者有肾上腺钙化，肾上腺体积明显增大。自身免疫性肾上腺炎：过去称为特发性肾上腺皮质萎缩，肾上腺皮质呈…     

药源性肾上腺皮质功能减退症因恶心呕吐误诊胃炎

目的探讨继发性肾上腺皮质功能减退症的临床特点及误诊原因,以提高临床确诊率。方法回顾性分析误诊为慢性胃炎的继发性肾上腺皮质功能减退症1例的临床资料。结果本例因食欲缺乏伴恶心、呕吐1个月,发热20 d入院。曾先后2次在当地医院就诊,按慢性胃炎予抑酸、止吐等治疗无效。入我院后经详细询问病史(患慢性阻塞性肺疾病20余年并间断不规则应用糖皮质激素史),结合低血压、低钠血症临床表现,查血皮质醇、血管紧张素Ⅰ、17酮类固醇、17羟类固醇及肾上腺CT、垂体MRI平扫及增强扫描确诊为继发性肾上腺皮质功能减退症。予糖皮质激素替代治疗,病情明显好转出院。后以泼尼松5 mg/d维持,病情稳定。结论继发性肾上腺皮质功能减退症临床表现较为隐匿,易误诊,临床应高度关注。     

成人慢性继发性肾上腺皮质功能减退症患者的血脂谱特征

目的探讨成人慢性继发性肾上腺皮质功能减退症患者与皮质醇功能正常志愿者血脂差异情况。方法比较我院住院87例成人继发性肾上腺皮质功能减退患者(SAI组)及172例皮质醇功能正常志愿者(对照组)血脂情况。结果 SAI组血脂异常患病率高于对照组,总胆固醇[(5.18±1.46)mmol/L]、甘油三酯[(2.57±2.56)mmol/L]高于对照组[分别为(4.81±1.08)mmol/L和(1.25±0.92)mmol/L],高密度脂蛋白胆固醇[(1.11±0.4)mmol/L]低于对照组[(1.31±0.37)mmol/L],亚组分析中甲状腺功能正常组及甲状腺功能减退组甘油三酯均高于对照组,甲状腺功能减退组高密度脂蛋白胆固醇低于对照组。结论本组继发性肾上腺皮质功能减退症患者较皮质醇功能正常志愿者血脂异常患病率升高,总胆固醇、甘油三酯升高,高低密度脂蛋白胆固醇降低,此种血脂异常并不能以任一单因素所解释,可能为糖皮质激素不足,过量的糖皮质激素替代,生长激素和甲状腺激素不足综合影响所致,提示在继发性肾上腺皮质功能减退症患者管理中需关注血脂情况及其潜在的心血管风险。     

原发性肾上腺皮质功能减退症20例临床分析

目的 探讨原发性肾上腺皮质功能减退症的临床特点及诊疗措施。方法 对20例原发性肾上腺皮质功能减退症患者的临床资料进行回顾性分析。结果 20例患者中13例为结核性，5例为特发性，2例为肾上腺切除术后；临床表现中以皮肤色素沉着较特异；非特异性症状出现早，且较常见。确诊有赖于皮质醇测定及快速促肾上腺皮质激素兴奋试验，其他激素及生化指标也有改变。结核性患者肾上腺CT以增生、钙化为主要表现。结论 确诊后给予泼尼松替代治疗，应激状态下应加大剂量，避免发生危象。     

急性肾上腺皮质功能减退症63例临床分析

回顾分析2000年1月～2011年6月我院收治的63例急性肾上腺皮质功能减退症患者的年龄、人群分布、病因、诱因及临床特点。63例肾上腺皮质功能减退症病例中原发性12例(19.0%);继发性51例(81.0%),继发性包括药源性49例(77.8%),席汉综合征1例(1.6%),垂体肿瘤1例(1.6%)。诱因包括骤停激素27例(42.9%)、感染23例(36.5%)、手术9例(14.3%)及其他4例(6.3%)。临床表现以乏力63例(100%)、纳差62例(98.4%)、发热51例(80.9%)及低血压45例(71.4%)多见,其他表现还有腹痛11例(17.5%)、腹泻9例(14.3%)、嗜睡6例(9.5%)等,本地区急性肾上腺皮质功能减退症以药源性多见,多发于农村中老年关节炎患者,易合并肺部感染,预后较好。     

1例艾迪森氏病病人的危象护理

艾迪森氏病又称原发性慢性肾上腺皮质功能减退症,系由于双侧肾上腺绝大部分被破坏所致,全身皮肤、黏膜色素加深为其临床症状.肾上腺危象为本病急骤加重的表现,应予以积极抢救[1].     

COPD合并继发性慢性肾上腺皮质功能减退患者的肾上腺皮质激素治疗方案探讨——附60例报告

目的：探讨COPD合并继发性慢性肾上腺皮质功能减退患者的肾上腺皮质激素治疗方法。方法：60例COPD急性加重期合并继发性慢性肾上腺皮质功能减退患者随机分为激素逐渐减量组（A组）和非激素逐渐减量组（B组）各30例,同期选取16名健康志愿者作为对照组。分别检测3组入院时、出院后第3个月、第6个月、第9个月、第12个月、第15个月、第18个月的血浆皮质醇水平,记录18个月观察期内A组、B组急性加重发作的次数及激素的总用量,比较上述指标的差异。结果：A组血浆皮质醇水平于出院后第12个月恢复正常,B组在18个月观察期内血浆皮质醇水平一直低于对照组。A组18个月内急性加重发作（0．7±0．3）次,明显少于B组的（4．7±1．3）次（P〈0．01）;A组18个月内泼尼松总用量（2055±310）mg,明显少于B组的（2675±518）mg（P〈0．05）。结论：对于COPD合并继发性慢性肾上腺皮质功能减退患者,宜在控制COPD急性加重期症状后,逐渐减少激素的用量并长期维持,以减少急性加重的发作次数和激素的总用量。动态测定血浆皮质醇水平有利于及时了解COPD合并继发性慢性肾上腺皮质功能减退患者的肾上腺皮质功能。     

关于阿狄森氏病问答

问 :肾上腺功能不全的原因可以分为原发性和继发性吗 ?答 :是的。问 :阿狄森氏病多由感染和外伤引起吗 ?答 :该病约 70 %是因自身免疫失调引起。原发性阿狄森氏病是因肾上腺皮质逐渐破坏 ,继发性是因垂体促肾上腺皮质激素( ACTH)分泌不足所致。问 :大多数病例是突然出现症状吗 ?答 :是逐渐出现临床症状。常见症状和体征为慢性渐进性疲劳、肌无力、食欲不佳、体重减轻、腹痛、恶心、呕吐、腹泻、抗感染及抗精神压力的能力降低、体位性低血压、闭经或月经不规则、性欲下降、阴部和腋毛稀少、易怒、沮丧、色素沉着、喜咸食 ,可表现为低钠和高…     

内科

内分泌及代谢疾病(二) 六、原发性慢性肾上腺皮质功能减退症【诊断标准】 1.软弱无力,易疲劳。胃纳不振,常表现恶心、呕吐、腹泻。 2.体重下降。皮肤色素沉着,以乳头部及压迫部位明显,腋毛易脱落。     

Endocrine disorders: causes of hyponatremia not to neglect

Hyponatremia is a common electrolyte abnormality with the potential for significant morbidity and mortality. Endocrine disorders, including adrenal deficiency and hypothyroidism, are uncommon causes of hyponatremia. Primary adrenal insufficiency (i.e. Addison's disease) may well be recognized by clear hall-marks of the disease, such as pigmentation, salt craving, hypotension, and concomitant hyperkalemia. Addison's disease is an important diagnosis not to be missed since the consequences can be grave. On the other hand, hypothyroidism and secondary adrenocortical insufficiency originating from diseases of the hypothalamus and/or pituitary (hypopituitarism) require a high index of suspicion, because the clinical signs can be quite subtle. This review focuses on clinical and pathophysiological aspects of hyponatremia due to endocrine disorders.     

Potassium disturbances as a cause of metabolic neuromyopathy

We report two cases of ascending muscular weakness progressing to areflexic quadriplegia caused by severe derangement of potassium homeostasis. The first patient presented with a 17-alpha-hydroxylase deficiency and severe hypokalemia. The second case had primary adrenocortical deficiency (Addison's disease) and extreme hyperkalemia. Complete recovery ensued after correction of the metabolic disorder in both cases. The role of potassium in the pathophysiology of neuromuscular excitation is discussed. We conclude that when neuromyopathy is present, metabolic causes should be considered and the serum potassium determined.     

Technical notes and clinical use of radioimmunologic determination of plasma ACTH]

Particular aspects of plasma ACTH radioimmunoassay are examinated. The results obtained with this method in adrenocortical diseases (Addison's disease, Cushing's syndrome, congenital adrenal hyperplasia) are reported and clinical value in etiological diagnosis of Cushing's syndrome is outlined. Moreover the results obtained in other syndromes of endocrinologic interest (massive obesity, Turner's and Klinefelter's syndromes, Laurence-Moon-Biedl syndrome, true precocious puberty, primary hypothyroidism, hypopituitary dwarphism) are reported and discussed.     

The clinical significance of the radioimmunological determination of plasma ACTH (author's transl)]

The clinical significance of the direct determination of plasma ACTH was investigated in healthy persons and in patients with primary or secondary adrenocortical insufficiency, with Cushing's syndrome or with acromegaly. The sensitivity of the radioimmunological method facilitated the detection of diurnal changes in plasma ACTH in healthy subjects and of variations in plasma ACTH after the administration of dexamethasone and glucagon. The determination of plasma ACTH appears to be a useful procedure of diagnostic value in patients suffering from primary adrenal insufficiency accompanied by high concentrations of plasma ACTH. However, in patients suffering from Cushing's syndrome or secondary adrenocortical insufficiency it is still essential to carry out the dexamethasone suppression test or the metopiron test, respectively.     

Diseases of the adrenal cortex

The adrenal cortex is functionally a three-dimensional gland that secretes glucocorticoids, mineralocorticoids, and sex steroids. Of these three classes of steroids only the gluco- and mineralocorticoid hormones are necessary to sustain life. The availability of sensitive and specific radioimmunoassays has permitted accurate measurement of practically every steroid hormone secreted by the adrenal cortex. As in other endocrinopathies, suppression studies are employed when hyperfunction is suspected, while provocative tests are used to detect hypofunction. These dynamic studies enable the clinician to evaluate the functional status of the adrenal cortex. The anatomic configuration of the adrenal cortices is delineated by high-resolution computed tomography (and magnetic resonance imaging), obviating the need for invasive procedures such as venography or arteriography. The disorders of the adrenal cortex can be viewed from the dual perspectives of hyperfunction and hypofunction. Clinical expressions of hyperfunctional adrenocortical syndromes include Cushing's syndrome, primary hyperaldosteronism, and the adrenogenital syndrome. The expressions of hypofunctional syndromes include Addison's disease and selective hypoaldosteronism. The diagnosis and treatment of these disorders are outlined in this issue.     

Polyglandular autoimmune syndrome, type 2

We have described two patients with Addison's disease and associated endocrinopathies, a condition termed polyglandular autoimmune (PGA) syndrome, type 2. One of our patients also had autoimmune hypothyroid disease, and the other had premature gonadal failure and Hashimoto's thyroiditis. This syndrome shows that glandular disorders tend to occur together. It has been suggested that an HLA-associated genetic predisposition coupled with environmental factors triggers an autoimmune process resulting in glandular hypofunction or hyperfunction. We stress the necessity for evaluation of every individual with idiopathic Addison's disease for associated endocrinopathies.     

Effects of repetitive administration of recombinant human interleukin-1 beta, an analog or corticotropin-releasing hormone combined with lysine vasopressin on rats with glucocorticoid-induced secondary adrenocortical insufficiency.

We investigated effects of corticotropin-releasing hormone (CRH), lysine vasopressin and interleukin (IL)-1 beta[1-148], a less pyrogenic analog of human IL-1 beta, on the hypothalamo-pituitary-adrenal axis in a rat model of secondary adrenocortical insufficiency. After 2 weeks of corticosterone 21-sodium succinate treatment, hypothalamic CRH, anterior pituitary adrenocorticotropic hormone (ACTH) and the adrenal weight of the rats decreased significantly and their plasma ACTH showed a significantly smaller response to ether stress, as did plasma corticosterone level. A mixed solution of CRH (10 micrograms) and lysine vasopressin (2 micrograms) or recombinant human IL-1 beta[1-148] (1 micrograms), administered to these rats for 7 days, apparently accelerated the recovery of the pituitary and adrenocortical responsiveness to ether stress and significantly increased the recovery rate of anterior pituitary ACTH contents and adrenal weight. The IL-1 beta analog also increased hypothalamic CRH. These data indicated that, in a rat model with glucocorticoid-induced adrenocortical insufficiency, synthesis and release of hypothalamic CRH, pituitary ACTH and adrenal glucocorticoid were all considerably affected. CRH combined with lysine vasopressin or a less pyrogenic IL-1 beta analog, when administered to these rats, accelerated the recovery of the pituitary and the adrenocortical functions significantly, suggesting the potential clinical usefulness of these peptides.     

Normal and abnormal regulation of β-MSH in man

The regulation of plasma beta-melanocyte-stimulating hormone (beta-MSH) in man has been studied utilizing a radioimmunoassay previously described (1). In normal subjects plasma beta-MSH values ranged from 20 to 110 pg/ml. Metyrapone increased and dexamethasone decreased plasma beta-MSH levels. Surgical stress stimulated beta-MSH secretion. Plasma beta-MSH levels were elevated in patients with untreated Addison's disease and untreated congenital adrenal hyperplasia, and these levels fell to normal during glucocorticoid therapy. In patients with Cushing's syndrome due to pituitary adrenocorticotropic hormone (ACTH) excess, plasma beta-MSH was slightly elevated before treatment. In those patients who developed pituitary tumors and hyperpigmentation after bilateral adrenalectomy, plasma beta-MSH was greatly elevated. In patients with Cushing's syndrome due to adrenal tumor, plasma beta-MSH was subnormal. In patients with the ectopic ACTH syndrome, the levels of plasma beta-MSH were high. Plasma beta-MSH had a diurnal variation in normal subjects, patients with Addison's disease, and patients with congenital adrenal hyperplasia; but the normal diurnal variation was lost in patients with Cushing's disease. In patients with high plasma beta-MSH, simultaneous determinations of plasma ACTH showed close correlation between the degree of elevation of ACTH and that of beta-MSH. In extracts of tumors from patients with the ectopic ACTH-MSH syndrome the quantities of the two hormones were roughly equivalent. In patients with hyperpigmentation due to a variety of disorders other than pituitary-adrenal abnormalities, plasma beta-MSH was normal. It is concluded that the secretion of beta-MSH is regulated by the same factors that regulate ACTH.     

Autoimmune polyglandular syndrome]

Among many etiologies for hypoparathyroidism, one of the inheritable forms of hypoparathyroidism, called as autoimmune polyglandular syndrome (APS), appears as a complex of hypofunction of several endocrine glands, candidiasis, pernicious anemia and vitiligo. Idiopathic hypoparathyroidism in APS typically presents by 20 years of age. Among the three most components of APS I, candidiasis is usually the first manifestation. Hypoparathyroidism almost invariably precedes the onset of Addison's disease. One should remember that Addison's disease can mask the presence of hypoparathyroidism and that glucocorticoid replacement therapy alone can cause hypocalcemic crisis.     

Altered interaction between triiodothyronine and its nuclear receptors in absence of cortisol: a proposed mechanism for increased thyrotropin secretion in corticosteroid deficiency states

Thyroid hormones occasionally appear less effective when administered alone to patients with panhypopituitarism, and manifestations suggestive of hypothyroidism have been reported in patients suffering from untreated Addison's disease. In the latter condition, thyrotropin secretion is increased: this occurs already after as little as 2 days of temporary withdrawal of therapy with substitution doses of corticosteroids while circulating levels of thyroid hormones remain within normal limits. Therefore, a possible role of cortisol in interaction between triiodothyronine and its nuclear receptors was examined at the level of circulating lymphocytes obtained from patients with primary or secondary adrenocortical failure. The affinity of these receptors was found to be decreased, by more than 50% on average, in the absence of cortisol treatments. This change was promptly corrected upon resumption of therapy. The number of binding sites was not significantly modified. The influence of cortisol on thyroid hormone receptors discussed here might account for the clinical observations mentioned above.