Biliary cirrhosis

　　Biliary cirrhosis is divided into primary biliary cirrhosis (Primary Biliary Cirrhosis, PBC) and secondary biliary cirrhosis. The latter is caused by long-term obstruction of extrahepatic bile ducts. It is generally believed that PBC is an autoimmune disease. After lymphocytes are activated, they attack medium and small bile ducts, causing an inflammatory reaction. Histologically, it is somewhat similar to the host's rejection of a graft, and has many similarities with the rejection reaction of liver allografts.

　　1. Hepatic encephalopathy (hepatic coma):It is the most common cause of death.

　　2. Upper gastrointestinal hemorrhage:Among them, bleeding due to variceal rupture of the gastric fundus or esophagus caused by portal hypertension is common. Other causes of bleeding include erosive gastritis, gastric and duodenal ulcers, and Mallory-Weiss syndrome.

　　3. Infection:Liver cirrhosis patients are prone to bronchitis, pneumonia, intestinal infections, spontaneous peritonitis, and gram-negative bacillary sepsis, etc.

　　4. Primary liver cancer:The relationship between liver cirrhosis and liver cancer is noteworthy. It is speculated that the mechanism may be related to the liver cell damage caused by hepatitis B virus leading to hyperplasia or atypical hyperplasia, as well as the integration of hepatitis B virus and liver cells, and the changes in immune function of liver cirrhosis patients.

　　5. Hepatorenal syndrome:The duration of refractory ascites in liver cirrhosis is long, or it is complicated with infection, the original liver disease is exacerbated, and other factors such as oliguria, anuria, azotemia, hyponatremia, and low urinary sodium. Initially, the kidneys often have no organic lesions, so it is called functional renal failure, and this complication has a very poor prognosis.

　　1. Early stage

　　Symptoms include mild fatigue and intermittent itching, 1/2 have liver enlargement, 1/4 have spleen enlargement. Elevated serum alkaline phosphatase and γ-GT are often the only positive findings. Itching that is worse at night is the first symptom in 47% of patients, and 1/4 patients have fatigue first, which can cause depression, followed by itching. Jaundice as the first manifestation accounts for 13% of patients, and these patients often have liver and spleen enlargement, may have xanthomas, corneal pigmented rings, liver palms, spider nevi, butterfly-shaped skin pigmentation spots on the site of scratching, and skin thickening and roughening, which may be related to scratching and vitamin A deficiency.

　　2. Non-jaundice stage

　　In a few patients, serum cholesterol levels can be as high as 8g/L, with nodular xanthomas on the palms, soles, chest, and back skin, and also along the tendons of the knees, elbows, and gluteal muscles, as well as the sheaths of nerves. Clubbing fingers may be accompanied by pain and tenderness, and periostitis of long bones may also be accompanied by pain and tenderness.

　　3. Jaundice stage

　　The appearance of clinical jaundice marks the beginning of the jaundice period, and the deepening of jaundice indicates that the disease has progressed to the late stage. The lifespan is less than 2 years. At this time, osteoporosis, osteomalacia, vertebral compression, and even rib and long bone fractures may occur, which are related to vitamin D metabolism disorders.

　　4. Terminal stage

　　Serum bilirubin levels rise sharply, the liver and spleen become significantly enlarged, itching and fatigue worsen, chronic liver disease symptoms become increasingly severe, and there is an increase in patients with esophageal varices bleeding and ascites. Due to copper deposition, a few patients may have corneal pigmented rings. Due to the lack of bile salts in the intestinal lumen, malabsorption of fat can occur, leading to steatorrhea. At this time, poor absorption of vitamins A, D, and K can cause night blindness, skin keratosis, skeletal changes, and coagulation disorders. Biliary tract imaging shows normal large bile ducts, twisted small bile ducts, and finally liver failure, variceal rupture, hepatic encephalopathy, ascites, edema with profound jaundice, which are often terminal manifestations.

　　Mainly for the prevention and active treatment of the etiology of intrahepatic and extrahepatic bile duct diseases, and for the intake of low-fat and low-oil foods.

　　1. Absolutely abstain from alcohol (including beer and rice wine), drink less of various beverages, and hot tea is permissible.

　　2. Maintain a constant diet, preferably with low salt, low fat, low sugar, and high protein. Avoid spicy, greasy, fried, and sticky foods. Do not overeat and pay attention to food hygiene to prevent diarrhea.

　　3. Try not to eat foods that are harmful to the liver.

　　4. Maintain the habit of exercise and a good mental state.

　　1. Laboratory examination

　　(1) Elevated blood bilirubin: The direct bilirubin is predominant, and urinary bilirubin is positive. Due to the decreased excretion of bilirubin from bile, both fecal urobilinogen and urinary urobilinogen are reduced.

　　(2) Elevated serum alkaline phosphatase: The source of alkaline phosphatase is the biliary duct epithelium, and PBC can have a significant increase in alkaline phosphatase before clinical symptoms appear.

　　(3) Elevated blood lipids: Especially the increase in phospholipids and cholesterol is most obvious, and triglycerides can be normal or moderately increased.

　　(4) Liver function tests: The concentration of serum bile acids increases, prothrombin time prolongs, and it can return to normal after early injection of vitamin K. In the late stage of liver failure, it cannot be corrected. Serum albumin is normal in the early and middle stages of the disease, and it decreases in the late stage; globulin increases, mainly α2, β, and γ globulins increase.

　　(5) Immunological examination: The positive rate of anti-mitochondrial antibodies can reach 90% to 100%, which can be an important reference for diagnosis. This antibody has a positive rate of 10% to 25% in chronic active hepatitis, and a few occult cirrhosis, systemic lupus erythematosus, and rheumatoid arthritis can also be positive. One-third of the patients have anti-biliary duct cell antibodies, a few have anti-smooth muscle antibodies and anti-nuclear antibodies, one-half have positive rheumatoid factors, and serum IgM can increase.

　　The coexistence of anti-mitochondrial antibodies, alkaline phosphatase, and IgM has diagnostic significance for PBC.

　　2. Biliary ductography

　　Venojugular methods can be used, such as percutaneous transhepatic biliary ductography or endoscopic retrograde cholangiography, to exclude extrahepatic biliary tract obstruction.

　　3. Liver biopsy

　　Living pathological examination has diagnostic value, but if cholestasis is severe or there is a tendency to hemorrhage, it should be considered carefully, and laparotomy with biopsy may be necessary if necessary.

　　Dietary therapy for biliary cirrhosis

　　1. Black sesame Poria porridge:Black sesame 10 grams, Poria 15 grams, ginger 3 slices, rice 100 grams. Cut the ginger into slices, crush the Poria, soak for half an hour, then decoct the medicine juice, and decoct twice. Mix the two decoctions and then cook them with rice and sesame to make thin porridge. It can be taken as breakfast or dinner, and it is effective for patients with ascites.

　　2. Lily porridge:Lily bulb 60 grams, rice 100 grams, ginger 3 slices. Clean and chop the lily bulb, cook it with rice to make porridge, which can be taken as breakfast or dinner, and it can be used when there is ascites.

　　1. Ursodeoxycholic acid

　　Heathcote et al. treated 222 patients with primary biliary cholangitis (PBC) with ursodeoxycholic acid (14mg/kg per day) and found that ursodeoxycholic acid can improve the serum markers of cholestasis, and it can significantly reduce serum bilirubin within three months, as well as significantly decrease serum alkaline phosphatase, transaminases, cholesterol, and IgM. In some patients, the liver tissue lesions also improved.

　　2. D-penicillamine

　　Can reduce copper levels in the liver, inhibit inflammatory reactions, alleviate fibrosis, extend the survival time of patients, the initial dose is 0.125g per day, increase by 0.125g every 2 weeks until the maintenance dose of 0.5g per day is reached, and should be treated for a long time. Severe adverse reactions include rash, proteinuria, thrombocytopenia, or granulocytopenia. Urine protein should be checked weekly, and then once a month after 4 weeks, observe the changes in white blood cell count, and discontinue treatment if necessary.

　　3. Immunosuppressants

　　(1) Cyclosporin A has a significant effect on Ts but should not be used for a long time due to renal and liver toxicity, 10mg/kg per day, and after 8 weeks of treatment, serum alkaline phosphatase decreases significantly. (2) Methotrexate, early use of low dose (15mg per day, divided into 3 times, once a week) can improve histological changes. (3) Azathioprine can improve bile duct excretion, seems to be effective for pruritus, but has significant side effects and cannot prevent the failure of liver cell function. The dosage is 2mg/kg and needs to be taken for a long time.

　　4. Symptomatic Treatment

　　(1) Pruritus can be treated with antihistamines such as promethazine, chlorpheniramine, and diphenhydramine, etc. Cholestyramine, 5-10g per day, start with a small dose and increase to control pruritus. Aluminum hydroxide, 4-20g per day, can combine with bile acids and is effective for pruritus associated with liver disease. (2) Osteoporosis and ossification can be treated with vitamin D injections of 10,000-20,000 U per day. Calcium gluconate, 15mg/kg per day, diluted into glucose solution for intravenous infusion, once a day, 10 days per course, and repeat the course every 2-3 months. (3) Vitamin A for night blindness, 25,000-50,000 U per day. If serum zinc is below normal, oral zinc sulfate can be taken, 220mg per day, for 4 weeks until dark adaptation returns to normal. (4) Vitamin K1, 10mg per day, for those with coagulation disorder. (5) End-stage PBC patients who are candidates for liver transplantation. (6) Low-fat diet (less than 50g per day), high sugar, high protein diet, eat more vegetables. (7) Treatment for esophageal variceal bleeding and hepatic coma is the same as that for liver cirrhosis.