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主要的 硬化性 胆管炎

Overview

主要的 sclerosing cholangitis (PSC) causes inflammation and scarring of the bile ducts located inside and outside the liver. The ducts carry bile — the liquid that helps break down fat in food — out of the liver. 随着疤痕的积累, the ducts become blocked causing bile to build up in the liver, 会损害肝细胞. 最终, 如果不及时治疗, PSC can cause liver failure or hardening of the liver, 被称为肝硬化.

PSC typically begins between the ages of 30 and 50, occurring most often in men. 尽管这种情况的原因尚不清楚, research has shown that it may be an autoimmune disorder, meaning that the immune system mistakes certain body parts and organs as foreign invaders and thus wrongly attacks them. PSC also may be genetically linked to another condition, 叫做溃疡性结肠炎, 是什么引起了结肠炎症. It is estimated that 70 percent of PSC patients also suffer from ulcerative colitis. 除了, people with PSC have an increased risk of developing cholangiocarcinoma and cancer of the bile ducts.

原发性硬化性胆管炎的治疗方法

UCSF's dedicated team of hepatologists delivers cutting-edge, compassionate care for all kinds of liver disorders, 包括原发性硬化性胆管炎. 作为一个强大的研究项目的一部分, we are at the forefront of an exciting new era of better treatment options for liver disease.

对于需要移植的病人, UCSF offers a liver transplant program known for outstanding outcomes and survival statistics that are among the best in the country. Our program helped pioneer techniques that have made transplantation safer 和更多的 successful. Transplant provides a cure for some patients with primary sclerosing cholangitis.

奖 & 识别

迹象 & 症状

In the early stages of primary sclerosing cholangitis (PSC), 你可能根本没有任何症状. 随着病情的发展, 症状 may come and go and are caused by the bile not being drained properly. This can affect liver function and cause the bile to seep into your bloodstream. 症状可能包括:

  • 慢性疲劳
  • 黄疸,皮肤和眼睛变黄
  • 食欲不振
  • 减肥
  • 慢性疲劳
  • 发冷
  • 发热
  • 上腹部压痛

诊断

Your doctor will first ask you about your medical history, which can suggest a likelihood of primary sclerosing cholangitis (PSC), especially if there is a history of inflammatory bowel disease and abnormal blood test results.

PSC is diagnosed through a procedure called cholangiography, which involves injecting dye into the bile ducts and taking an X-ray. Cholangiography can be performed as an endoscopic procedure, called endoscopic retrograde cholangiopancreatography (ERCP), 通过放射学或外科手术, 或者用核磁共振成像(MRI)扫描. 胆管造影在镇静状态下进行. 一根点燃的, 可弯曲的内窥镜通过口腔插入, 胃然后进入小肠. A thin tube is place through the scope into the bile ducts and dye is injected to highlight the bile ducts on the X-ray. If there is a narrowing of the bile ducts, the diagnosis of PSC is confirmed.

随着病情的发展, 通常需要肝活检来确诊PSC, 确定已经造成了多大的破坏. 局部麻醉下, a slender needle is inserted through the right lower chest to extract a small piece of liver for microscopic analysis.

治疗

Treatment for primary sclerosing cholangitis (PSC) includes medication to reduce itching and jaundice, antibiotics to treat infections and vitamin supplements since people with PSC are often deficient in vitamins A, D和K. In some cases, surgery to open major blockages in the common bile duct also is necessary. 除了, liver transplantation may be required and in some cases, can cure PSC.

加州大学旧金山分校健康 medical specialists have reviewed this information. 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.

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