淀粉样变性|病情| 加州大学旧金山分校健康-十大赌博平台排行榜

Overview

淀粉样变性是一类蛋白质异常的疾病, 叫做淀粉样蛋白, 积聚在器官或器官系统中，如心脏, 肾脏, 神经系统或胃肠道. 淀粉样变有不同的类型, 取决于组织中沉积的蛋白质类型.

The most common form of amyloidosis in the United States is called 原发性淀粉样. 原发性淀粉样蛋白, the protein being deposited is a part of the antibody protein called the light chain.

原发性淀粉样蛋白现在是一种可治疗的疾病. The mainstays of therapy are the same agents used to treat a related 骨髓 cancer, called 多发性骨髓瘤. 治疗方案包括化疗, 皮质类固醇药物, 生物制剂, 在某些情况下, 自体干细胞移植. The goal of therapy is to halt the buildup of amyloid protein in organs and to prolong life.

继续阅读

为tunately, amyloidosis is rare, affecting about eight out of every 1 million people annually. The majority of those affected are more than 40 years of age and 60 percent are male, 尽管这种情况可能影响任何人. 原因尚不清楚.

淀粉样变的类型

淀粉样变主要有三种类型:

原发性淀粉样 这是一种起源于骨髓的浆细胞疾病. 异常细胞产生抗体蛋白，以淀粉样蛋白的形式沉积. It is the most common type of amyloidosis in the United States, affecting 2,000 people each year.

This condition, which is usually treated with chemotherapy, is not considered to be a type of cancer. 然而，它可能与多发性骨髓瘤这是一种骨癌. The severity of the illness often depends on the number and extent of organ involvement.
继发性淀粉样 这种情况是由慢性感染或炎症性疾病引起的类风湿性关节炎, 家族性地中海热(一种肠道疾病), 骨髓炎(骨感染)或肠道炎症性疾病. The amyloid deposits in this type of the disease are made up of a protein called the AA protein. Medical or surgical treatment of the underlying chronic infection or inflammatory disease can slow or stop the progression of this type of amyloid.
家族性(或遗传性)淀粉样蛋白 这种罕见的淀粉样蛋白是唯一可以遗传的. 它影响到几乎所有种族背景的家庭. The deposits in this type are most commonly made up of the transthyretin protein produced in the liver. 这种情况是由这种蛋白质的突变引起的. 肝脏移植手术用来治疗这种淀粉样蛋白吗.

我们治疗淀粉样变的方法

UCSF is dedicated to delivering the most advanced treatment options for amyloidosis with care and compassion. 淀粉样变有几种类型, and the best therapy depends on the type and severity of the patient's condition. 治疗 range from medications such as antibiotics to stem cell or liver transplants.

We are also dedicated to discovering even better treatments for amyloidosis through research. 感兴趣的患者可以选择参加临床试验, 评估潜在的新疗法.

迹象 & 症状

淀粉样变的症状取决于它所影响的器官. 淀粉样变性的广泛症状往往使其难以诊断. 有些人可能没有任何症状，而其他人可能有许多症状. 常见的症状包括:

弱点
减肥
呼吸急促（气促）
手脚麻木或刺痛
腹泻
严重的疲劳
舌头肿大
吃了比平时少的食物后感到饱
站立眩晕
脚踝或腿部肿胀
晕厥
心悸

根据涉及的器官和由此产生的症状, 淀粉样变可引起特定组的症状, 被十大赌博平台排行榜称为综合症. 这些包括:

充血性心力衰竭或者心脏不能泵出足够的血液来满足身体的需要.
肾病综合征，蛋白质通过肾脏过量流失
自主神经病变, or nerve damage that affects involuntary body functions such as heart rate and perspiration.
Peripheral neuropathy, or nerve damage that affects the peripheral nervous system (PNS). The PNS relays information between the brain and the spinal cord and the rest of the body.
吸收不良，或从食物中吸收营养有问题.

诊断

A complete medical history and physical examination is essential for diagnosing amyloidosis. 还必须进行血液、尿液和骨髓检查. 血液或尿液测试可以检测出淀粉样蛋白, but only 骨髓 tests or other small samples of tissue — called biopsies — can positively establish the diagnosis of amyloidosis.

一个小的组织样本，或活组织检查，通常会从腹部脂肪, 直肠和/或骨髓确认淀粉样变的诊断. These biopsies are relatively minor procedures done in an outpatient clinic with a local anesthetic (numbing medication). 偶尔, 样本需要取自受损的器官，比如肝脏, 肠, 心脏或肾脏. 所需的活检程序类型因患者而异.

治疗

淀粉样变的治疗是为了改善症状和延长寿命. 治疗可以限制淀粉样蛋白的进一步产生, 在某些情况下, 促进受累器官中淀粉样蛋白的分解. The type of treatment required varies depending on the type of amyloidosis and the patient's 症状.

与 继发性淀粉样, 治疗的主要目标是治疗潜在的疾病，例如, taking an anti-inflammatory medication for 类风湿性关节炎 or antibiotics for an infection.

In 遗传性淀粉样, 肝移植是最有效的治疗方法吗. The new liver does not produce the abnormal amyloid proteins and consequently the disease improves. Investigational drugs are also being evaluated to try and prevent this type of amyloid protein from depositing in organs.

为 原发性淀粉样, 治疗方法包括与治疗多发性骨髓瘤相同的药物, 比如化疗, 皮质类固醇药物 (lenalidomide or thalidomide) and/or bortezomib (Velcade). These treatments slow organ deterioration and some have been shown to prolong life, 但没有一个能治愈.

Because 原发性淀粉样 is such a difficult disease to treat and survival is limited, 研究人员已经开始研究使用大剂量化疗与自体干细胞移植作为延长生存的一种手段. 自体干细胞移植的初步结果令人鼓舞.

自体干细胞移植

病人 with 原发性淀粉样 undergo an extensive work-up to evaluate organ function and the effects that amyloidosis has had on the body. 那些心脏，肝脏和肺功能正常的人被鼓励继续自体干细胞移植.

大剂量美法兰化疗在一天内进行. Then the patient's own stem cells (骨髓) are re-administered two to three days later. An additional three to four weeks are spent in the hospital awaiting recovery and growth of the 骨髓.

希望这种疗法能延缓疾病的发展, 在某些情况下, 通过从器官中去除异常蛋白质来改善症状. 然而，这种疗法并不能治愈，淀粉样变性会在每个人身上复发. 也就是说, we have had patients who have been successfully treated with stem cell transplantation and when their disease progressed, 能再接受一次干细胞移植吗.

Several new investigational agents are being evaluated in the treatment of 多发性骨髓瘤, 另一种浆细胞紊乱. The hope is that some of these agents also may be effective in treating amyloidosis. 对于不适合干细胞移植的患者, 这些药物可能是目前最好的治疗方法.

遗传性淀粉样蛋白患者应转诊肝移植诊所进行评估.

加州大学旧金山分校健康医学专家已经审查了这些信息. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.