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Nephrotic Syndrome I : Introduction01:24

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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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Tumor Necrosis Factor (TNF), a proinflammatory cytokine, contributes significantly to the inflammation seen in Crohn's disease. It exists as soluble TNF and membrane-bound TNF, with actions mediated through TNF receptors (TNFR). TNFR activation leads to the release of proinflammatory cytokines, T-cell activation, collagen production, and leukocyte migration, all contributing to inflammation in Crohn's disease. Anti-TNF monoclonal antibodies, namely infliximab (Remicade), adalimumab...
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Crohn's disease is an inflammatory bowel disorder marked by chronic inflammation of the GI tract. Various treatment strategies for Crohn's disease are employed, such as immunomodulatory agents, glucocorticoids, and biologics or anti-TNF therapy. Azathioprine (Imuran), a commonly used immunomodulatory drug for Crohn's disease, is converted in the body to mercaptopurine, which inhibits purine biosynthesis and cell proliferation. Both are utilized in severe cases of Inflammatory Bowel...
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Atherosclerosis is a progressive disorder that leads to the thickening and narrowing of arterial walls due to plaque buildup. This condition can cause various symptoms depending on the arteries affected:Coronary Artery Disease (CAD): This condition affects the coronary arteries and may lead to chest pain (angina), shortness of breath (dyspnea), heart attacks, and other heart disease symptoms.Cerebrovascular Disease: This affects blood flow to the brain, causing transient ischemic attacks (TIAs)...
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与阿纳金拉相关的全身性氨基粉症.

Sara Alehashemi1, Surendra Dasari2, Anvitha Metpally1

  • 1National Institute of Allergy and Infectious Diseases, NIH, Bethesda, Maryland.

Arthritis & rheumatology (Hoboken, N.J.)
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概括

这一案例研究报告了因复合性互乐金-1受体对抗剂 (IL-1Ra) 蛋白质疗法引起的亚原性系统性粉症. 这些粉样蛋白沉积物被确定为来自anakinra药物本身.

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科学领域:

  • 免疫学 免疫学 免疫学
  • 蛋白质治疗药物 蛋白质治疗药物
  • 腎臟病學 (nephrology) 是一種醫學專業.

背景情况:

  • 新生儿发作的多系统炎症性疾病需要长期治疗.
  • 复合型互白素-1受体对抗剂 (IL-1Ra) 蛋白,anakinra,用于慢性炎症疾病.
  • 从长期生物治疗的并发症可以包括新的病理.

研究的目的:

  • 描述一种罕见的阳性全身性氨基粉症病例.
  • 在接受anakinra治疗的患者中调查粉样蛋白沉积的原因.
  • 为了描述粉样沉积物的性质.

主要方法:

  • 皮肤,胃粘膜和脏活检的组织学检查.
  • 刚果红色染色用于识别粉样沉积物.
  • 基于质谱的蛋白质组学来分析粉样蛋白的组成.

主要成果:

  • 在皮肤,胃和脏组织中发现了粉样蛋白沉积物.
  • 蛋白质学证实的沉积物是由重组IL-1Ra (anakinra) 蛋白组成的.
  • 粉样蛋白前体被确定为治疗药物,而不是内源蛋白质.

结论:

  • 这是首次报告一种可注射蛋白质药物引起的阳性性系统性粉症.
  • 再组合IL-1Ra (anakinra) 可以导致全身性粉样性粉症.
  • 了解药物诱导的氨基粉症对于长期的生物治疗管理至关重要.