Biliary cirrhosis
Introduction
Introduction to biliary cirrhosis Biliary cirrhosis is caused by long-term intrahepatic biliary retention or more obstruction of the extrahepatic biliary tract. The former is called primary biliary cirrhosis, and the latter is called secondary biliary sclerosis. 90% of the disease occurs in women, especially in women aged 40 to 60 years old, male: female is 1:8. Early symptoms were only mild fatigue and intermittent itching, 1/2 with hepatomegaly, 1/4 with splenomegaly, and elevated serum alkaline phosphatase and -GT were often the only positive findings. End-stage manifestations of liver failure, variceal rupture, hepatic encephalopathy, ascites, edema with deep jaundice. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: hepatic encephalopathy, primary liver cancer, hepatorenal syndrome
Cause
Causes of biliary cirrhosis
Autoimmune (60%):
The disease is unknown, chronic progressive cholestasis liver disease, may be related to autoimmunity. PBC is generally considered to be an autoimmune disease. When lymphocytes are activated, they attack the middle and small bile ducts, causing an inflammatory reaction. Histologically, it appears that the host's rejection of the graft has many similarities with the rejection of liver allografts.
Clinically, the condition is relieved and worsened, often accompanied by other autoimmune diseases such as Sjogren's syndrome, systemic lupus erythematosus, rheumatoid arthritis and chronic lymphocytic thyroiditis. Humoral immunity was significantly abnormal, the anti-mitochondrial antibody positive rate was 90% to 100%, and 80% of patients had a titer greater than 1:80. Some people even included anti-mitochondrial antibody positive as a case when the PB C was studied.
Extrahepatic bile duct obstruction (30%):
Biliary cirrhosis is divided into primary biliary cirrhosis (PBC) and secondary biliary cirrhosis (Secondary Biliary Cirrhosis). The latter is caused by long-term obstruction of the extrahepatic bile duct.
Prevention
Biliary cirrhosis prevention
More intake of some high-fiber and fresh vegetables and fruits, balanced nutrition, including essential nutrients such as protein, sugar, fat, vitamins, trace elements and dietary fiber, with a combination of vegetarian and vegetarian foods, diversified foods, and full use of food nutrition The complementary role of matter.
Complication
Complications of biliary cirrhosis Complications hepatic encephalopathy primary liver cancer hepatorenal syndrome
1 Hepatic encephalopathy (hepatic coma): is the most common cause of death.
2 Upper gastrointestinal bleeding: Among them, the rupture of the fundus or esophageal varices caused by portal hypertension is common. Others such as hemorrhagic erosive gastritis, stomach, duodenal ulcer, and cardia mucosal tear syndrome are also causes of bleeding. one.
3 infection: patients with cirrhosis are prone to bronchitis, pneumonia, intestinal infection, spontaneous peritonitis and Gram-negative bacilli sepsis.
4 primary liver cancer: the relationship between liver cirrhosis and liver cancer is remarkable, it is speculated that the mechanism may be hepatitis B virus caused by hepatocyte damage secondary to hyperplasia or dysplasia, and the integration of hepatitis B virus and hepatocytes and immune function of liver cirrhosis patients Change and other factors are related.
5 hepatorenal syndrome: cirrhosis with persistent refractory ascites for a long time, or combined infection, the original liver disease aggravation and other factors, oliguria, no urine, azotemia, hyponatremia, low urinary sodium, the initial kidney often There is no organic disease, so it is called functional renal failure, and the prognosis of this complication is very poor.
Symptom
Symptoms of biliary cirrhosis Common symptoms Biliary retention liver lobe atrophy Liver palm ascites hepatomegaly Alkaline phosphatase increased portal vein blood stasis stagnation gallstone corneal ring jaundice
Early
Symptoms only mild fatigue and intermittent itching, 1/2 with hepatomegaly, 1/4 with splenomegaly, elevated serum alkaline phosphatase and -GT are often the only positive findings, light day and night weight Itching is as a first symptom of 47%, a quarter of patients have a feeling of fatigue, and can cause depression, followed by itching, jaundice as the first performance of 13%, such patients often have hepatosplenomegaly, can have yellow, corneal color film ring, liver palm, spider mites, scratch-like parts of the butterfly skin pigmentation spots, skin thickening, thickening, may be related to scratches and vitamin A deficiency.
2. No jaundice
A small number of patients with serum cholesterol can be as high as 8g / L, palm, sputum, chest and back skin with nodular jaundice, also along the knee, elbow, gluteal muscle spasm, nerve sheath distribution, clubbing, long bone periostitis can be associated with pain and tenderness.
3. Huang Qi period
The appearance of clinical jaundice marks the beginning of the jaundice period. The deepening of jaundice indicates that the disease progresses to the advanced stage, and the life span is shorter than 2 years. At this time, osteoporosis, osteomalacia, vertebral compression, and even rib and long bone fractures are often accompanied. Related to vitamin D metabolic disorders.
4. End period
Serum bilirubin rises linearly, liver and spleen are swollen, itching, fatigue is aggravated, signs of chronic liver disease are getting worse, patients with esophageal varices bleeding and ascites increase, due to copper deposition, a few visible corneal pigment rings, Due to the lack of bile salts in the intestine, fat emulsification and malabsorption, fat diarrhea can occur, vitamin A, D, K malabsorption at this time, can produce night blindness, skin keratosis, bone changes and coagulation mechanism disorders, biliary angiography shows bold The tube is normal, the small bile duct is distorted, and finally the liver failure, varicose vein rupture, hepatic encephalopathy, ascites, edema with deep jaundice, often end-stage performance.
Examine
Examination of biliary cirrhosis
Laboratory examination
(1) Increased blood bilirubin: mainly bilirubin, urinary bilirubin positive, due to reduced bilirubin excretion from bile, fecal biliary and urinary biliary.
(2) Increased serum alkaline phosphatase: The source of alkaline phosphatase is the bile duct epithelium, and PBC can have a significant increase in alkaline phosphatase before the onset of clinical symptoms.
(3) Increased blood lipids: especially phospholipids and cholesterol are most obvious, triglycerides can be normal or moderately increased.
(4) Liver function test: serum cholic acid concentration increased, prothrombin time prolonged, early return to normal after vitamin K injection, can not be corrected in advanced liver failure, serum albumin in the early, intermediate, normal, late reduction The increase in globulin is mainly due to the increase in 2, and globulin.
(5) Immunological examination: the positive rate of anti-mitochondrial antibodies can reach 90%-100%, which can be used as an important reference for diagnosis. The positive rate of this antibody in chronic active hepatitis is 10% to 25%, and a small number of occult cirrhosis Systemic lupus erythematosus and rheumatoid arthritis can also be positive, 1/3 of patients have anti-cholangiocyte antibodies, a few have anti-smooth muscle antibodies and anti-nuclear antibodies, 1/2 rheumatoid factor positive, serum IgM can be increased.
Anti-mitochondrial antibodies, alkaline phosphatase, IgM coexisting have a diagnostic significance for PBC.
2. Cholangiography
Excessive extrahepatic biliary obstruction can be excluded by intravenous, percutaneous transhepatic cholangiography or endoscopic retrograde cholangiography.
3. Liver biopsy
Live pathological examination, there is a diagnostic value, but if the cholestasis is serious, or bleeding tendency should be carefully considered, if necessary, laparotomy and biopsy.
Diagnosis
Diagnosis and diagnosis of biliary cirrhosis
Diagnose based on
1 middle-aged women, the skin is obviously itchy, liver, yellow tumor. Xanthoma is a lipid deposition disease characterized by skin damage. Cells that phagocytose lipids are localized in the dermis and tendons and appear clinically as yellow, orange or brownish red papules, nodules or plaques. Often accompanied by systemic lipid metabolism disorders and cardiovascular system damage, can be secondary or secondary to other systemic diseases.
2 serum total cholesterol was significantly increased, serum bilirubin was mild, moderately elevated, alkaline phosphatase increased, and bile acid concentration increased.
3IgM is elevated, anti-mitochondrial antibody is positive and the titer is high. If the histological evidence can be obtained by puncture, it is more helpful for diagnosis.
