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所在平台: Udemy |
课程主页: https://www.udemy.com/course/amyloidosis-introduction-pathogenesis-clinical-features/
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**课程名称：** 淀粉样变性：介绍、发病机制、临床表现 **课程概述：** 本课程将深入介绍淀粉样变性，这是一种由异常蛋白质（淀粉样纤维）在组织中积聚引起的疾病。课程将详细阐述淀粉样变性的非特异性体征和症状，包括疲劳、外周水肿、体重减轻、呼吸困难，心悸以及站立时头晕等。 淀粉样变性是一种系统性疾病，根据其前体蛋白的不同可分为多种类型，并且可以表现为全身性或局灶性。 * **AL型淀粉样变性（又称原发性淀粉样变性）：** 这是最常见的系统性淀粉样变性类型。它源于骨髓内浆细胞的异常（称为浆细胞病），与多发性骨髓瘤密切相关。 * **AA型淀粉样变性（又称继发性淀粉样变性）：** 该类型淀粉样变性由炎症蛋白血清淀粉样蛋白A（SAA）引起。它常与慢性炎症性疾病相关，如风湿性疾病、家族性地中海热、慢性炎症性肠病、肺结核或脓胸。 * **遗传性淀粉样变性：** 这是一种罕见的淀粉样变性，由基因异常引起。虽然有多种致病基因，但最常见的类型是ATTR型，由转甲状腺素蛋白（TTR）基因的病理性变异引起。 * **年龄相关性淀粉样变性（ATTRwt）：** 由野生型（正常）TTR引起，是一种缓慢进展的疾病，主要影响老年人的心脏，多见于男性，女性较少。 * **孤立性淀粉样变性：** 淀粉样物质可能孤立地沉积在身体特定部位，而不伴有系统性疾病的证据，例如孤立性膀胱或气管淀粉样变性是最常见的表现。 * **透析相关β2-微球蛋白淀粉样变性（AB2MG）：** 这种系统性淀粉样变性可能发生在长期接受肾脏透析的个体身上。它与β2-微球蛋白的异常聚集有关，而β2-微球蛋白是一种通常由功能正常的肾脏清除的淀粉样蛋白。该类型淀粉样变性仅影响接近终末期肾脏疾病的患者，不会影响肾功能正常或轻度减退者，也不会影响肾移植患者。 本课程将全面解读淀粉样变性的不同类型、发病机制以及临床表现，为学习者提供系统性的知识框架。
Amyloidosis is a group of diseases in which abnormal proteins, known as amyloid fibrils, build up in tissue. There are several non-specific and vague signs and symptoms associated with amyloidosis. These include fatigue, peripheral edema, weight loss, shortness of breath, palpitations, and feeling faint with standing.Amyloidosis is a systemic disorder that is classified into several types based on the precursor protein. The different types of amyloidosis are classified as systemic or localized. AL (immunoglobulin light chain, historically known as primary) amyloidosis is the most common type of systemic amyloidosis. AL amyloidosis results from an abnormality (dyscrasia) of a type of white blood cell called plasma cells in the bone marrow and is closely related to multiple myeloma. AA (historically known at secondary) amyloidosis is derived from the inflammatory protein serum amyloid A. AA amyloidosis occurs in association with chronic inflammatory diseases such as the rheumatic diseases, familial Mediterranean fever, chronic inflammatory bowel disease, tuberculosis or empyema. Hereditary amyloidosis is a rare type of amyloidosis that is caused by an abnormal gene. There are several abnormal genes that can cause hereditary amyloidosis, but the most common type of hereditary amyloidosis is called ATTR and caused by changes (pathogenic variants or mutations) in the transthyretin (TTR) gene. Age related amyloidosis, in which the amyloid is derived from wild-type (normal) transthyretin, is a slowly progressive disease that affects the hearts of elderly individuals, usually men and less commonly women, and is called ATTRwt amyloidosis. Amyloid deposits may occasionally occur in isolation without evidence of a systemic disease; isolated bladder or tracheal amyloidosis are the most common such presentations. Dialysis-related beta2-microglobulin amyloidosis is a type of systemic amyloidosis that can occur in individuals who have experienced long-term kidney dialysis to remove accumulated impurities or wastes in the blood by mechanical filtration. This form of amyloidosis, also known as AB2MG (amyloid associated with the beta-2m protein), is associated with the aggregation of beta2-microglobulin, a type of amyloid protein that is cleared by the normally functioning kidney. Dialysis-related beta2-microglobulin amyloidosis occurs in patients with near end-stage renal disease. It does not affect individuals with normal or mildly reduced renal function or patients with a functioning renal transplant.
