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Disease OverAny cause liver, bile duct disease, leading to cholestasis, biliary cirrhosis can be developed. Points of primary biliary cirrhosis and secondary biliary cirrhosis."Etiology"Biliary cirrhosis and primary biliary cirrhosis points (Primary Biliary Cirrhosis, PBC) and secondary biliary cirrhosis (Secondary Biliary Cirrhosis). The latter by the extrahepatic bile duct obstruction caused by long-term. Generally believed that the PBC is an autoimmune disease, lymphocytes were activated, the attack, the small bile ducts, causing inflammation. Histologically, just like the host of graft rejection. And liver allograft rejection have many similarities. Clinically, alternating remission and progression, often accompanied by other autoimmune diseases such as Sjogren's syndrome, systemic lupus erythematosus, rheumatoid arthritis and chronic lymphocytic thyroiditis and so on. Significant abnormality of humoral immunity, anti-mitochondrial antibody-positive rate of 90% to 100%, 80% of patients whose titer greater than 1:80, when someone of PB C, even as anti-mitochondrial antibody-positive cases were included. Some patients are still anti-nuclear antibody, rheumatoid factor, thyroid antibodies, these antibodies with the corresponding antigen can form large immune complexes, complement system caused by the immune injury.Enlargement of the liver was pale green, smooth, or showing a finely granular texture. Tissue damage, roughly the following process: there are lymphocytes, pla


sma cells, IgM, and immune complex deposition, is the cause of granuloma. Bile reflux, bile duct injury and inflammation around the bile duct, causing bile duct damage and bile duct proliferation, portal area around the joint inflammation and scar formation separated by the formation of cholestasis and the surrounding area of copper, iron deposition, and further damage the liver cells, fibers become stretched, and ultimately lead to cirrhosis. PBC based on the occurrence and development of the pathological changes of the PBC is divided into four.The first phase, inflammation of the bile duct, characterized by lobular septum between the bile duct or bile duct with chronic non-purulent inflammation, timid tube cavity, the wall and the surrounding inflammatory cell infiltration. Mainly lymphocytes, plasma cells. Portal area expanded by inflammatory cell infiltration, and granulomatous changes, but the limiting plate of liver cells and normal.The second phase, proliferation of bile duct, bile duct due to chronic inflammation of the destruction and replace them with fibrous tissue, the majority of portal area is difficult to find interlobular bile duct, but there does not typical of small bile duct hyperplasia, this period is still visible granuloma. Lobular bile ducts around the great expansion of capillaries, containing concentrated bile plugs capillary bile duct rupture, the formation of bile paste, and its surrounding liver cell swelling, cytoplasm was translucent loose network, the feather-like degeneration.The third phase, scar formation, increased collagen content in portal area and bile duct cells and reduce inflammation, occasionally associated lymphoid follicle development center, medium-sized portal area fibrosis in the book, granulomas are not common, fiber separated from the portal area to another a portal area stretching, or extending to the hepatic lobules, necrosis of the coexistence of the debris and cholestasis, iron, copper deposition, causing liver cell injury, resulting in limiting plate blurred.IV, liver cirrhosis stage, portal area expanded and connected to each other fibrous septa, dividing the formation of false lobules hepatic lobules, showing regenerative nodules, usually small nodular cirrhosis, can also be separated by sex were not entirely false centrilobular necrosis .90% occurred in women, particularly more common in women 40 to 60 years, male: female was 1:8. Have reported the incidence of pregnancy by about 10% when the disease is insidious onset, slow onset.Only mild symptoms of fatigue and intermittent pruritus, 1 / 2 with hepatomegaly and 1 / 4 with splenomegaly, serum alkaline phosphatase, and -GT elevated often is the only positive findings, at light nights as the first symptom itching weight of 47% .1 / 4 of patients first, feeling tired, and can cause depression, followed the itching. As the first manifestation of jaundice accounted for 13% of such patients often have hepatosplenomegaly, warts can be yellow, color film corneal ring, liver palms, spider angioma, scratch parts of a wing skin pigmentation, skin thicker, change thick, scratch and vitamin A may lack.A small number of patients with serum cholesterol of up to 8g / L, palms, soles, backs and chests nodular yellow skin warts, but also along the knee, elbow, hip tendon, nerve sheath distribution of persons, clubbing, periostitis of long bones may be associated with pain and tenderness.Marked the emergence of clinical jaundice jaundice of the beginning of deepening jaundice progression to the late indicates that life is shorter than 2 years, then often accompanied by osteoporosis, osteomalacia, vertebral compression, or even a rib and long bone fractures, those with related to vitamin D metabolism.Straight up serum bilirubin, liver and spleen was swollen, itching, fatigue, feeling worse. Increasing signs of chronic liver disease increased, with esophageal variceal bleeding and ascites of patients increased. Due to copper deposition, a small number of visible corneal pigment rings. As the intestine lack of bile salt and fat emulsification and malabsorption, steatorrhea can occur at this time of vitamin A, D, K absorption, can produce night blindness, skin keratinocytes, skeletal changes and the clotting mechanism. Bold tube cholangiography showed normal small bile ducts distorted. Finally, liver failure, varices, hepatic encephalopathy, ascites, edema associated with the depth of jaundice, often end-stage performance.Associated with the disease and its manifestations, 2 / 3 of a connective tissue disease, autoimmune thyroiditis is also common, but also with scleroderma, calcinosis, Raynaud's phenomenon, 75% had dry keratoconjunctivitis, 35% with asymptomatic bacteriuria, hypertrophic osteoarthropathy, 1 / 3 pigment gallstones, the other can be a membrane glomerulonephritis and renal tubular acidosis.Diagnosis is based on: middle-aged women, the skin was itching, liver, yellow tumor; significantly higher serum total cholesterol, serum bilirubin light, moderately elevated, elevated alkaline phosphatase, bile acid concentration; IgM l high titer of anti-mitochondrial antibody-positive and high. If the puncture to obtain histological evidence of even more help to confirm the diagnosis.1. Laboratory(1) blood bilirubin mainly direct bilirubin, urine bilirubin positive, due to reduced bilirubin is excreted from the bile, fecal bile and urobilinogen were originally reduced.(2) Serum alkaline phosphatase is the of increased alkaline phosphatase bile duct epithelium, PBC may be that is before the appearance of clinical symptoms was significantly higher alkaline phosphatase.(3) elevated blood lipids, especially phospholipids and cholesterol increased the most significant may be normal or moderately elevated triglycerides.(4), liver function tests of serum bile acid concentration, prothrombin time prolonged, the early return to normal after the injection of vitamin K and late liver failure can not be corrected. Serum albumin in the disease early, mid normal, late decrease; globulin increased, mainly and -globulin increased.(5), immunological tests of anti-mitochondrial antibody positive rate of 90% to 100%, can be used as important reference for diagnosis. The antibodies in chronic active hepatitis positive rate of 10% to 25%, a small number of occult cirrhosis, systemic lupus erythematosus and rheumatoid arthritis can also be positive. 1 / 3 of patients with anti-bile duct cell antibodies, a small number of anti-smooth muscle antibodies and antinuclear antibodies, 1 / 2 rheumatoid factor positive. Serum IgM can be increased.Anti-mitochondrial antibodies, alkaline phosphatase, IgM has confirmed the three co-exist on the significance of PBC.Available intravenous method, percutaneous transhepatic or endoscopic retrograde cholangiography imaging method to exclude extrahepatic biliary obstruction.Biopsy, a diagnosis value, but if severe cholestasis, or bleeding tendency should be carefully considered, if necessary, laparotomy and biopsy.Including obstructive jaundice, such as bile duct stones, common bile duct tumors, pancreatic and bile duct stenosis, the main identification methods cholangiography. Identification of the following diseases should be:Where anti-mitochondrial antibodies, accompanied by deposition and histological bile duct abnormalities are, first, except CAH, chronic active liver and gallbladder sludge type of PBC more common than short-term effect of corticosteroid therapy can help distinguish these two observations kinds of disease.Disease is rare, mainly involving daring tube, the immune markers negative, and the fever more than a bacterial infection, bile duct imaging can help identify.Such as chlorpromazine, methyl testosterone, sulfonamides, arsenic preparations and so on. More from individual allergy, there is medication history, after taking the drug within a few weeks the disease, jaundice for several years, often accompanied by increased blood eosinophils, liver biopsy is not the typical histological features of PBC.1. UDCAHeathcote, etc. with ursodeoxycholic acid (daily 14mg/kg) treatment of 222 patients with PBC patients with ursodeoxycholic acid can find cholestasis improved serum markers, 3 months can significantly serum bilirubin decreased and serum alkaline phosphatase, transaminase, cholesterol and IgM decreased significantly, some patients improved liver lesions.To reduce liver copper levels, inhibit the inflammatory response, reducing fibrosis and prolong survival of patients began daily amount of 0.125g, every 2 weeks to increase 0.125g, until a daily maintenance dose of 0.5g, should be long-term treatment. Serious adverse reactions are rash, proteinuria, thrombocytopenia or neutropenia, should check urine protein every 4 weeks, 1 month after the investigation, observed changes in white blood cell count, if necessary, need to stop treatment. cyclosporin A, plays a significant role on the Ts, but not a long liver and kidney toxicity with daily 10mg/kg, after 8 weeks treatment significantly decreased serum alkaline phosphatase. methotrexate, early low-dose (daily 15mg, 3 times orally, 1 time per week) applications can improve the histological changes. azathioprine, can improve the excretion of bile ducts, like the skin itching effective, but side effects, and can not prevent liver cell failure. The dosage of 2mg/kg, requires long-term medication.(1) itching available antihistamines, such as phenergan, chlorpheniramine, diphenhydramine and so on. Cholestyramine daily 5 ~ 10g, start small doses in order to be able to control for the degree of itching. Aluminum hydroxide, daily 4 ~ 20g, with the bile acid binding on the hepatic itching effect.(2) osteoporosis, and ossification of vitamin D per day can be 1 to 2 million U intramuscularly. Calcium gluconate daily 15mg/kg, diluted to glucose infusion, day 1, 10, a course of treatment takes time to repeat every 1, 2 ~ 3 months of treatment.(3) night blindness and vitamin A, 2.5 ~ 50 000 U. daily serum zinc is lower than normal zinc sulfate can be taken orally, daily 220mg, even for 4 weeks, to return to normal dark adaptation.(4) impaired clotting mechanism of vitamin K1, daily 10mg.(5) liver transplant patients with end-stage PBC liver transplantation object.(6) diet low in fat (50g per day or less), high-sugar, high protein diet, eat more vegetables.(7), esophageal variceal bleeding, hepatic coma treated with cirrhosis.And serum total bilirubin, albumin, age, prothrombin time and the degree of edema, 1989, Dickson and other indexes based on these 5 developed a Mayo score.X1 = In serum total bilirubin (mg / dL), X2 = In serum albumin (mg / dL), X3 = age (years), X4 = In prothrombin time (seconds), X5 = edema (0 : no edema, 0.5: diuretics to eliminate the edema, 1: diuretics can not eliminate the edema).Mayo constant: 1 = 0.872 =- 2.533 = 0.034 = 2.35 = 0.859R0 for the S0 (t) a constant risk scoring, R0 = 5.07Corresponding period survival S0 (t) is:Time (years) 1 2 3 4 5 6 7 S0 (t) 0.970 0.941 0.883 0.883 0.774 0.721 0.651The above formula can calculate the corresponding period prognosis of survival probability of the object.The same laboratory in 1994, the mathematical model has been modified in the two years that a new model of good prognosis, 3 to 7 years is the old model is superior.


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