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Adrenal gland disorders manifest when the production of adrenal hormones deviates from the norm, resulting in either excessive or insufficient concentrations.
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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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Adrenal hormones play a pivotal role in maintaining the body's electrolyte balance and orchestrating responses to stress, showcasing the intricate functions of the adrenal cortex and medulla.
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The response to stress—be it physical or psychological, acute or chronic—involves activation of the Hypothalamic-Pituitary-Adrenal (HPA) axis. The HPA axis is part of the neuroendocrine system because it involves both neuronal and hormonal communication. Its function is to regulate homeostatic systems—metabolic, cardiovascular, and immune—providing the necessary means to respond to a stressor.
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Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
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The adrenal or supra-renal glands, situated above the kidneys and aligned with the twelfth rib, are paired pyramid-shaped structures crucial for the body's stress response. During stress, these glands secrete hormones vital for adaptive physiological reactions.
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主要双边上腺增生 (PBMAH):从罕见到常见的库辛综合征在临床实践中的原因

Sergii Cherenko1, Liuchiia Shchekaturova2

  • 1Department of Endocrine Surgery, International Medical Center "CITI DOCTOR", Stepana Bandery Ave, 17/1, Kiev, 04073, Ukraine. Sergmakar5@gmail.com.

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概括
此摘要是机器生成的。

主要双边上腺增生症 (PBMAH) 是日益公认的库辛综合征 (CS) 的原因. 通过手术切除较大的上腺,可以长期缓解上腺功能不全.

关键词:
库希综合征主要的阿尔多斯特主义初级双边上腺增生外科手术

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科学领域:

  • 内分泌学
  • 外科瘤学
  • 上腺疾病

背景情况:

  • 主要双边上腺增生症 (PBMAH) 经常被误诊为库辛综合征 (CS) 的罕见原因.
  • 了解PBMAH的发病,临床表现和治疗对于有效的患者管理至关重要.
  • 最近的数据表明PBMAH比以前认为的更频繁地导致上腺癌.

研究的目的:

  • 评估PBMAH患者的临床特征和治疗结果.
  • 评估PBMAH患者同时存在的原发性阿尔多斯特隆症的患病率.
  • 为了确定腹腔镜上腺切除术对PBMAH的疗效和安全性.

主要方法:

  • 两个不同的时期 (2009-2015年和2016-2023年) 的机构/医院数据库的回顾性分析.
  • 包括接受上腺切除术的患者,重点是PBMAH病例.
  • 对79名患者的临床状况,激素水平和治疗结果的后续评估.

主要成果:

  • 在这两组研究中,PBMAH分别占上腺癌病例的21%和32%.
  • 患者 (平均年龄为53. 4岁,女性为77. 6%) 主要有轻度的脑脊髓炎,高血压,肥胖,高血糖和骨质疏松症.
  • 显著的百分比 (44. 1% 和 37. 3%) 具有同时存在的原发性阿尔多斯特隆症;4. 7% 经历了手术后的CS复发.

结论:

  • PBMAH是上腺库辛综合征的一个重要原因,并不是罕见的疾病.
  • 大上腺的单侧腹腔镜上腺切除术对长期缓解有效,并避免永久性上腺功能不全.
  • 主要的阿尔多斯特隆症经常与PBMAH有关,因此应进行查.