长期诊断为原发性肾上腺功能不全的维尔霍夫病。临床病例报告

I. Chernyavska, N. Skrypnyk, V.I. Pankiv
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引用次数: 0

摘要

慢性肾上腺功能不全是一种内分泌疾病,由于下丘脑-垂体-肾上腺轴的一个或多个环节功能失调,导致肾上腺激素分泌不足。斯堪的纳维亚国家的发病率最高:斯堪的纳维亚国家的发病率最高:每 10 万人中有 15-22 例,而其他欧洲国家报告的发病率为每 10 万人中有 10 例。自身免疫性肾上腺炎是导致成人原发性肾上腺功能不全的最常见原因,它既可以单独发病(40%),也可以是自身免疫性多腺综合征的一个组成部分(60%)。原发性肾上腺功能不全的病因包括结核病或艾滋病。这些肾上腺皮质功能不全的病因在乌克兰尤为常见。文章描述了一名同时患有原发性肾上腺功能不全和韦尔霍夫病的患者的临床病例。文章强调了肾上腺功能不全的发病率和原发性肾上腺功能不全的病因。详细介绍了原发性肾上腺功能不全与其他可能引起低血糖状态、严重消瘦、低血压、重症肌无力、低钠血症、高钾血症等临床症状的疾病的鉴别诊断。讨论了原发性肾上腺功能不全的诊断、治疗和预后的基本原则。尽管肾上腺功能不全具有典型、特殊的表现,但在实际临床实践中却很难诊断。医生对肾上腺功能不全症状的认识和警惕是必要的。慢性肾上腺功能不全发现过晚可能导致并发症。及时诊断和治疗慢性肾上腺功能不全可改善疾病预后和生活质量。
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Long-diagnosed primary adrenal insufficiency in the setting of Werlhof’s disease. A clinical case report
Chronic adrenal insufficiency is an endocrine disease caused by insufficient secretion of adrenal hormones due to dysfunction of one or more links of the hypothalamic-pituitary-adrenal axis. The highest prevalence has been documented in Scandinavian countries: 15–22 people per 100,000 population, while other European countries report 10 cases per 100,000 population. Autoimmune adrenalitis is the most common cause of primary adrenal insufficiency in adults, and it can be either alone (40 %) or a component of autoimmune polyglandular syndromes (60 %). The etiologic factors of primary adrenal insufficiency include tuberculosis or AIDS. These causes of adrenal cortical insufficiency are particularly relevant in Ukraine. The article describes a clinical case of a patient with both primary adrenal insufficiency and Werlhof’s disease. The prevalence of adrenal insufficiency and etiologic factors of primary adrenal insufficiency are highlighted. A detailed differential diagnosis of primary adrenal insufficiency with other diseases that could cause clinical symptoms such as hypoglycemic states, severe weight loss, hypotension, severe muscle weakness, hyponatremia, hyperkalemia is performed. The basic principles of diagnosis, treatment, and prognosis in primary adrenal insufficiency are discussed. Despite typical, specific manifestations of adrenal insufficiency, diagnosis in real clinical practice is difficult. Awareness and vigilance of doctors regarding the symptoms of adrenal insufficiency is necessary. Late detection of chronic adrenal insufficiency can lead to complications. Timely diagnosis and treatment of chronic adrenal insufficiency improves disease prognosis and quality of life.
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