원발성 간질환성 간硬化

    원발성 간질환성 간硬化의 원인은 명확하지 않으며 자가면역과 관련이 있을 가능성이 있습니다.

　　원발성 간질환성 간硬化(PBC)이 자가면역과 관련이 있을 수 있기 때문에, 병리학적 합병증은 다음과 같습니다:

　　1가려움

　　현재 피부 가려움에 대한 표준적인 효과적인 치료법이 없습니다.1months before it becomes significant. Rifampin may achieve an antipruritic effect by changing the internal environment of bile acids in liver cells and improving the biochemical indicators of PBC patients. Nalmephene, Naltrexone, and other opiate drugs can be used for patients who are ineffective with cholestyramine and rifampin. There are many other methods (such as ultraviolet light, phototherapy, and plasma apheresis therapy, etc.) used for the control of pruritus in PBC, but none have been confirmed by regular clinical trials. For intractable pruritus that cannot be controlled, liver transplantation surgery can be performed.

　　2Osteoporosis

　　After the diagnosis of PBC is confirmed, bone density should be measured regularly, and then every2yearly follow-up. Educate patients to develop good living habits (such as regular sleep and smoking cessation) and supplement vitamin D and calcium. Hormone replacement therapy is recommended for postmenopausal female patients, and it is best administered through the skin. If osteoporosis is very obvious, biophosphonate treatment can be used.

　　About11PBC patients have osteoporosis. Supplementing calcium and vitamin D can prevent demineralization, the recommended doses are1000～1200mg/d and25000～50000IU, weekly2～3times. Recent studies have found that raloxifene can prevent bone loss and reduce serum cholesterol.60mg/d, continuous use1year, can increase lumbar bone mineral density (LS2BMD), without liver toxicity. In addition, alendronate can effectively increase bone mass.

　　3SICCA syndrome

　　All PBC patients should be asked about symptoms such as dry eyes, dry mouth, and difficulty swallowing, and female patients should also be asked about sexual difficulties, and appropriate treatment measures should be taken if present.

　　4Raynaud's syndrome

　　For patients in cold areas, the treatment of Raynaud's syndrome is a difficult problem, patients should avoid exposing their hands and feet to cold environments, and smokers should quit smoking. Calcium channel blockers may be used if necessary, but they may worsen lower esophageal sphincter dysfunction.

　　5Portal hypertension

　　PBC patients can develop pre-sinusoidal portal hypertension before liver cirrhosis, the treatment of portal hypertension in liver cirrhosis patients is the same as other types of liver cirrhosis. However, the efficacy of beta-blockers for non-cirrhotic pre-sinusoidal portal hypertension needs to be confirmed, and shunt surgery can be considered if necessary. It is recommended that PBC be screened for the presence of esophageal varices at the time of the first definite diagnosis, and thereafter2yearly. If varices are found, measures should be taken to prevent bleeding.

　　6Lack of fat-soluble vitamins

　　Hyperbilirubinemia can be accompanied by lack of fat-soluble vitamins and poor calcium absorption, in patients without jaundice, little is known about the level of fat-soluble vitamins and the value of oral supplementation. Fat-soluble vitamins should be supplemented in a water-soluble form. Monthly subcutaneous injection of vitamin K can correct coagulopathy secondary to vitamin K deficiency.

　　7thyroid disease

　　thyroid disease can affect about15%~25PBC의 환자 중 %는, 일반적으로 PBC 환자가 병변을 시작하기 전에 존재할 수 있습니다. PBC가 진단된 환자의 경우, 혈청 중 효소 내 인자의 농도를 측정하고 定期的에 검사할 것을 권장합니다.

　　Primary biliary cirrhosis often coexists with other autoimmune diseases such as rheumatoid arthritis, Sjögren's syndrome, systemic sclerosis, chronic lymphocytic thyroiditis, etc., and is more common in middle-aged women, with a stealthy onset, a slow course, mild early symptoms, and the general condition of the patient is generally good, appetite and weight loss is not significant, about10％의 환자는 어떤 증상도 없을 수 있습니다. 원인 불명의 만성 진행성 방해성黄疸 환자, 특히 지방변비가 동반된 경우, 발병의 유발 원인 및 질병 진행 상황을 상세히 파악해야 하며, 다른 자가면역 질환이 존재하는지 주의하고, 상대성黄疸성 간硬化 및 다른 원인 간硬化와 구별해야 합니다.

　　Since the etiology of primary biliary cirrhosis is unknown, there is no effective prevention method for this disease. Try not to eat foods that harm the liver. Maintain the habit of exercise and a good attitude.

　　Primary biliary cirrhosis is mainly judged clinically according to the following criteria: blood lipids, serum bile acid, conjugated bilirubin, alkaline phosphatase and glutamyl transpeptidase, etc., significantly increased, transaminase normal or slightly increased, blood antimitochondrial antibody positive, IgM increased, prothrombin time prolonged, urine bilirubin positive, urine bilirubin normal or decreased.

　　영상학: 초음파, 내시경 역행성 간챨관造影술, CT, 피부 내 간 챨관造影 등 간 내외 챨관 확장 및 간 외 방해성黄疸 질환의 존재 여부를 파악합니다.

　　Primary biliary cirrhosis treatment mainly includes adequate rest, high-protein, high-carbohydrate, high-vitamin, low-fat diet, daily fat