对艾迪生病的描述,以及它的神经精神表现,将现在的疾病与1906年三一圣伊丽莎白所经历的疾病进行比较。

4区 医学 Q2 Medicine
Psychiatria Danubina Pub Date : 2025-09-01
Michaela Agius, Mark Agius
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引用次数: 0

摘要

我们描述Addison病,这是一种由肾上腺功能不全引起的疾病。艾迪生病是一种罕见的内分泌紊乱,其特征是肾上腺分泌的激素,主要是醛固酮和皮质醇分泌不足。这种情况的发生是由于负责激素合成的肾上腺皮质受损。Addison病的临床表现通常是隐匿的和非特异性的,包括肌无力、持续性疲劳、体重意外减轻、低血压和皮肤色素沉着,特别是在遭受摩擦的区域。许多神经精神表现,包括抑郁症和精神病也可能发生。病因主要是自身免疫性肾上腺炎,免疫系统错误地攻击和破坏肾上腺皮质细胞。其他病因包括传染性病原体,如结核分枝杆菌,它可以渗透并损害肾上腺。肾上腺皮质出血性损伤可继发于严重的应激、创伤或凝血功能障碍。此外,转移性肿瘤可能累及肾上腺,导致其破坏。少数情况下,遗传性疾病如肾上腺脑白质营养不良可通过影响脑白质和肾上腺皮质损害肾上腺功能。肾上腺皮质损伤也可由肾上腺切除术或使用抑制类固醇生成的药物引起。Addison病的病理生理学涉及下丘脑-垂体-肾上腺(HPA)轴的破坏,导致皮质醇缺乏,这对调节代谢、免疫功能和应激反应至关重要。醛固酮缺乏导致钠和钾体内平衡失调,引起低血压和脱水。Addison病的治疗需要终生使用糖皮质激素和矿皮质激素替代治疗,通常使用氢化可的松或氟化可的松,同时在压力、疾病或手术干预期间进行持续监测和剂量调整。在描述艾迪生氏病时,我们描述了三位一体的伊丽莎白所经历的疾病,她是一位天主教修女,在20世纪早期患有这种疾病,她的疾病是从她的信件和目击者的叙述中描述的,被认为是她的宣福礼和封圣的发现。我们描述了当时人们是如何看待这种疾病的,当它被认为是无法治愈的,在诊断和治疗方面都有很大的缺陷,以及这些年来病因学是如何变化的。因此,我们展示了这个人所忍受的痛苦是如何在精神上和身体上都有。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A DESCRIPTION OF ADDISON'S DISEASE, AND ITS NEUROPSYCHIATRIC MANIFESTATIONS COMPARING THE DISEASE AS IT IS NOW TO THE DISEASE AS EXPERIENCED BY SAINT ELIZABETH OF THE TRINITY IN 1906.

We describe Addison's disease, which is the disease caused by Adrenal Gland Insufficiency. Addison's disease is an uncommon endocrine disorder characterised by inadequate production of hormones, predominantly aldosterone and cortisol, by the adrenal glands. This condition occurs due to damage to the adrenal cortex, the region responsible for hormone synthesis. Clinical manifestations of Addison's disease are often insidious and nonspecific, including symptoms such as myasthenia, persistent fatigue, unintentional weight loss, hypotension, and hyperpigmentation of the skin, especially in areas subjected to friction. A number of Neuropsychiatric manifestation, including Depression, and Psychosis can also occur. The aetiology is primarily autoimmune adrenalitis, where the immune system erroneously attacks and destroys adrenal cortical cells. Other aetiologies include infectious agents like Mycobacterium tuberculosis, which can infiltrate and impair the adrenal glands. Hemorrhagic damage to the adrenal cortex can occur secondary to severe stress, trauma, or coagulopathies. Additionally, metastatic neoplasms may involve the adrenal glands, leading to their destruction. Less frequently, genetic disorders such as adrenoleukodystrophy can compromise adrenal function by affecting both the cerebral white matter and adrenal cortex. Adrenal cortex damage can also result from adrenalectomy or the administration of medications that inhibit steroidogenesis. The pathophysiology of Addison's disease involves the disruption of the hypothalamic-pituitary-adrenal (HPA) axis, resulting in cortisol deficiency, which is critical for regulating metabolism, immune function, and stress responses. Aldosterone deficiency leads to dysregulation of sodium and potassium homeostasis, causing hypotension and dehydration. Management of Addison's disease requires lifelong glucocorticoid and mineralocorticoid replacement therapy, typically with hydrocortisone or fludrocortisone, along with ongoing monitoring and dosage adjustments during periods of stress, illness, or surgical interventions. While describing Addison's Disease we describe the illness as experienced by Elizabeth of the Trinity, a Catholic Nun who suffered the illness in the early part of the Twentieth Century, whose illness is described from her letters and eyewitness accounts, taken for her beatification and canonisation findings. We describe how the illness it was viewed then, when it was seen as incurable, with great deficiencies in both diagnosis and treatment, and how the aetiology has changed over the years. Hence we show how the suffering endured by this person had both mental and physical aspects.

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来源期刊
Psychiatria Danubina
Psychiatria Danubina 医学-精神病学
CiteScore
3.00
自引率
0.00%
发文量
288
审稿时长
4-8 weeks
期刊介绍: Psychiatria Danubina is a peer-reviewed open access journal of the Psychiatric Danubian Association, aimed to publish original scientific contributions in psychiatry, psychological medicine and related science (neurosciences, biological, psychological, and social sciences as well as philosophy of science and medical ethics, history, organization and economics of mental health services).
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