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Addison disease

内分泌

关键词内分泌 疾病 肾上腺功能不全

词汇介绍

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解析

Addison   英 /'ædisn/

释    义   n. 艾迪生(姓氏)

例    句   Increased pigmentation of the skin is common in Addison's disease. 皮肤色素沉着的增加是常见的艾迪生病的体征。

 

disease   英 /dɪ'ziːz/   美 /dɪ'ziz/

释    义   n. 病,[医] 疾病;弊病

               vt. 传染;使…有病

同根词   diseased adj. 不健全的;患病的;病态的

               diseased v.使生病;传染(disease的过去分词)

例    句   They researched into the causes of the disease. 他们研究了这种疾病的起因。

概述

概述


艾迪生氏病是一种罕见病,发病率为十万分之一,无论男女老少都可能发生,但是只要服用药物,艾迪生氏病患者就能过正常的生活。称为原发性肾上腺功能不全的艾迪生氏病中,肾上腺不能摄取足够多的称为皮质醇的激素,或者较少地摄取一种称为醛固酮的相关激素。


病理机制


艾迪生氏病是由肾上腺受损引起的,导致皮质醇激素不足,而且醛固酮通常也不足。肾上腺是内分泌系统的一部分,它产生的激素几乎可以影响体内的每个器官和组织。肾上腺由两部分组成,内部(髓质)产生肾上腺素样激素,外层(皮质)产生一组称为皮质类固醇的激素。皮质类固醇包括:糖皮质激素,这些激素(包括皮质醇)会影响人体将食物转化为能量的能力,在免疫系统的炎症反应中发挥作用,并帮助人体应对压力。盐皮质激素,这些激素(包括醛固酮)可以维持体内钠和钾的平衡,从而使血压正常。雄激素,男性和女性的肾上腺都会少量产生男性性激素。它们引起男性的性发育,并影响男性和女性的肌肉质量,性欲和幸福感。


临床表现


①慢性疲劳和肌肉无力;②食欲不振和体重减轻;③低血压;④皮肤斑点,暗黑和雀斑;⑤血糖异常,包括危险的低血糖;⑥恶心,呕吐和腹泻;⑦无法应付压力;⑧情绪烦躁和沮丧;⑨不耐高温或低温;⑩喜咸味食物。


治疗方法


艾迪生氏病的所有治疗方法均涉及药物治疗:荷尔蒙替代疗法,以纠正体内不产生的类固醇激素水平,治疗选择包括口服皮质类固醇。

Early Clinical Indicators of Addison Disease in Adults With Type 1 Diabetes: A Nationwide, Observational, Cohort Study复制标题

成人1型糖尿病患者Addison病的早期临床指标: 一项全国性、观察性、队列研究

发表时间:2019-04-01

影响指数:5.6

作者: Dimitrios Chantzichristos

期刊:J Clin Endocrinol Metab

Despite advances in diabetes care, type 1 diabetes mellitus (T1DM) is still associated with premature death and shortened life span. A nationwide Swedish study has shown a more than twofold higher risk of death (mainly from cardiovascular diseases) in patients with on-target glycemic control and an eightfold higher risk of death in patients with poor glycemic control compared with the general population. Life span is reduced by 16 years among patients with T1DM who developed the disease in childhood. Addison disease (AD) is a rare disease that also results in premature death with a twofold increased risk despite diagnosis and treatment. When undiagnosed or untreated, AD leads to certain death. In contrast to T1DM, data are rather scarce on how both morbidity and mortality can be ameliorated in patients with AD. Patients with an autoimmune disorder, such as T1DM, are at increased risk of developing another autoimmune disease, such as AD, autoimmune thyroid disease, celiac disease, pernicious anemia, or rheumatoid disease. Moreover, autoimmune AD can be one component of autoimmune polyendocrine syndrome type 2 when it occurs in combination with T1DM, autoimmune thyroid disease, or both. The combination of concomitant T1DM and AD is rare and is associated with a more than fourfold higher risk of premature death compared with patients with T1DM alone. As the risk of developing AD among patients with T1DM is 10 times higher than in the general population, awareness of this risk is of utmost importance among physicians who manage patients with T1DM. Autoimmune AD is often characterized by an insidious onset leading to overt disease after a varying period of time. In the natural history of AD, there is an individual underlying genetic susceptibility followed by unknown triggering factors (immune and/or environmental) that lead to autoimmune destruction of the adrenal cortex with a gradual deterioration of adrenal function. AD diagnosis is often delayed because of its nonspecific signs and symptoms. Early detection and treatment is life saving in AD, and an early indication of the disease would therefore be of great value. Increased prescription of gastrointestinal, antianemic, thyroid, and lipid-modifying drugs as well as corticosteroids and systemic antibiotics have been reported prior to AD diagnosis in one study and low serum sodium and elevated serum thyroidstimulating hormone in another study.

译文

尽管在糖尿病护理方面取得了进步,但1型糖尿病(T1DM)仍与过早死亡和寿命缩短有关。瑞典的一项全国性研究表明,与目标人群相比,具有目标血糖控制的患者的死亡风险(主要是心血管疾病)高出两倍以上,而血糖控制不良的患者的死亡风险则高出八倍。在儿童期发展为T1DM的T1DM患者的寿命缩短了16年。 Addison病(AD)是一种罕见的疾病,尽管进行了诊断和治疗,但也会导致过早死亡,风险增加了两倍。未经诊断或未经治疗,AD会导致某些死亡。与T1DM相比,关于AD患者的发病率和死亡率如何改善的数据非常缺乏。患有自身免疫性疾病(例如T1DM)的患者罹患另一种自身免疫性疾病(例如AD),自身免疫性甲状腺疾病,腹腔疾病,恶性贫血或类风湿性疾病的风险增加。此外,当自身免疫性AD与T1DM,自身免疫性甲状腺疾病或两者同时出现时,它可以是自身免疫性多内分泌综合征2型的一种成分。与单独的T1DM患者相比,合并的T1DM和AD很少见,与过早死亡的风险高出四倍以上。由于T1DM患者罹患AD的风险是普通人群的10倍,因此在管理T1DM患者的医生中,这一风险的认识至关重要。自身免疫性AD通常以隐匿性发作为特征,在一段时间后会导致明显的疾病。在AD的自然史中,存在个体潜在的遗传易感性,其后是未知的触发因素(免疫和/或环境),这些触发因素导致肾上腺皮质自身免疫破坏,肾上腺功能逐渐恶化。 AD诊断通常由于其非特异性的体征和症状而延迟。尽早发现和治疗可以挽救AD的生命,因此,对该病的早期发现将具有巨大的价值。在一项研究中,AD诊断之前已报告增加了胃肠道,抗贫血,甲状腺和脂质调节药物以及皮质类固醇和全身性抗生素的处方,而在另一项研究中,血清钠水平较低且甲状腺刺激激素水平升高。

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