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"Family Clinic Merck Manual"> Chapter XIII Secti17 of hepatobiliary disease fatty liver, cirrhosis and related diseases
Fatty liver, alcoholic liver disease, cirrhosis, primary biliary cirrhosis, primary sclerosing cholangitis, and -antitrypsin deficiency, is due to liver damage caused the disease. There are many factors that can damage the liver, but some of the causes of these diseases is not clear.Fatty liver is excessive fat in liver cells of the accumulation.
Causes of fatty liver is sometimes unclear, especially newborns. In general, several factors are known to cause liver damage in some way.
Fatty liver usually has no symptoms, very few cases, fatty liver can cause jaundice, nausea, vomiting, abdominal pain and swelling tight.
Physical examination found that the liver is big but not with any symptoms suggestive of fatty liver, liver biopsy confirmed to be dependent. Liver biopsy is hollow with a thin needle to obtain a small amount of liver tissue samples for microscopic examination methods.
Mere presence of excessive fat liver is not a serious problem. Treatment aims to remove the cause or treatment of primary disease, liver damage, such as repeated alcohol will eventually lead to fatty liver into cirrhosis.* Chemicals and drugs (such as alcohol, leather acid, tetracycline, valproic acid, methotrexate, carbon tetrachloride, yellow phosphorus)
* Malnutrition and a lack of protein* Cystic fibrosis (often accompanied by malnutrition)
* Glycogen


, lactose, tyrosine, cystine, inherited metabolic defects* Phytane acid storage disease (Trevor prime Farm disease)Alcoholic liver disease is liver damage due to excessive drinking.
Alcoholic liver disease is common, but is preventable health problems. In general, alcohol consumption (number and frequency) determine the risk and extent of liver damage. Women are more vulnerable to injury than men. Few years, women drinking, alcohol consumption, the amount of pure alcohol per day up to 19g (182g wine, 364g beer, 56g whiskey), can cause liver damage; drinking men for several years, alcohol consumption, the amount of pure alcohol per day up to 56g (560g wine, 1120g or 168g beer whiskey) can cause liver damage. However, liver damage caused by alcohol consumption, or individual.
Three types of alcohol can cause liver damage: liver fat accumulation (fatty liver), inflammation (alcoholic hepatitis), fibrosis (cirrhosis).
Alcohol only heat and lack of basic nutrients, reduce appetite, because of the toxic effects of the small intestine and pancreas cause malabsorption, which alcoholics and with no proper diet, people will develop malnutrition.
Symptoms and diagnosis
In general, the appearance of symptoms depends on how much alcohol, the length of time. Drinking alcohol are usually the first symptom after 30 years, 40 years after serious problems. Men, the symptoms caused by alcohol similar to estrogen and androgen excess produced too few results - testicle shrinking, breast enlargement.
Fat accumulation (fatty liver) is often asymptomatic, of which 1 / 3 of the liver of patients increases, the occasional liver tenderness.
Alcohol induced liver inflammation (alcoholic hepatitis) may have fever, jaundice, elevated white blood cell, liver increases, tenderness and a perceived pain, skin spider.
Liver fibrosis (cirrhosis) of the patients may have some symptoms of alcoholic hepatitis or characteristics, but also can occur in alcoholic liver cirrhosis complications of portal hypertension with splenomegaly, ascites, liver failure caused by renal failure (liver renal syndrome), disorders of consciousness (the main symptoms of hepatic encephalopathy) or liver cancer. To confirm the diagnosis, the doctor for some patients, liver biopsy, abdominal wall with a hollow needle puncture into the liver, to obtain a small piece of liver tissue for histological examination by microscopy.
Patients with alcoholic liver disease, liver function test results can be normal or abnormal, but the blood of alcoholics in the -glutamyl GGT levels can be particularly high. In addition, the patients blood larger than normal red blood cells, platelets meaningful level can be lowered.
Prognosis and treatment
If the patient continues to drink, liver damage will continue to progress, and may be fatal. Such as stopping drinking, some liver damage (except fibrosis) may be recovered, the change is getting better, the patient will prolong survival.
The only treatment of alcohol induced liver injury is to stop drinking, this is very difficult. Most people required to attend formal alcohol organizations, such as the club to stop drinking alcohol.Cirrhosis is the destruction of normal liver structure is characterized by the formation of radial scar tissue around the non-functional functional liver tissue.
The most common outcome of liver injury is cirrhosis, in the United States, the most common cause of cirrhosis was alcohol in 45 to 65-year-old crowd, cirrhosis of the liver routed to the third cause of death, behind heart disease and cancer. In most Asian and African countries, chronic hepatitis, cirrhosis of the liver is the main reason.* Exposure to certain chemical substances
* Inflammation (including hepatitis B, hepatitis C)No symptoms in many patients with mild liver cirrhosis, the performance of health over the years. With symptoms showed weakness, loss of appetite, nausea, weight loss. In the case of chronic biliary obstruction, the patient has jaundice, itching, yellow skin nodules (particularly distributed in the eyelid.) Loss of appetite and lack of bile salts, lipids, fat-soluble vitamin malabsorption can lead to malnutrition.
Occasionally, patients with esophageal varices bleeding due to cause vomiting or massive hemoptysis (esophageal varices). Esophageal vein swelling and varicose blood vessels to the liver by the drainage of the blood vessels within the intestinal pressure increase caused. This increase in blood pressure called portal hypertension. Have liver damage, but also can cause ascites. Renal failure and hepatic encephalopathy will gradually develop and form.
Other symptoms of chronic liver disease may also occur, such as muscle atrophy, liver palms (palmar erythema) curled fingers, skin, spider angioma, male breast enlargement (gynecomastia), increased submental salivary gland, hair loss, testicle shrinkage (testicular atrophy) and abnormal neurological function (peripheral neuropathy).
Diagnosis
Ultrasound scan suggest liver, liver radionuclide scanning can show the function of the liver area and the scar tissue area, as needed to maintain basic physiological functions of only a small amount of liver tissue to complete. Therefore, patients with liver cirrhosis of liver function tests are often normal. Microscopic examination of liver tissue to determine the diagnosis of liver cirrhosis.
Prognosis and treatment
Cirrhosis is usually progressive, such as early stop drinking alcoholic cirrhosis, liver scar to stop, but the formation of scar tissue has not changed. In general, serious complications such as vomiting, ascites, brain dysfunction (hepatic encephalopathy) has occurred, the prognosis is poor.
Liver cancer (hepatocellular carcinoma) by the hepatitis B, hepatitis C, hemochromatosis, glycogen storage disease caused by long-term more common in cirrhosis of the liver can also occur in patients with alcoholic cirrhosis.
There is no cure for liver cirrhosis, treatment including the elimination of causes such as alcohol, to ensure proper nutrition, including vitamin supplement, the occurrence of complications treatment complications.
Some progress in liver transplantation for cirrhosis is helpful, but as a transplant patient to continue drinking, or did not eliminate the causes cirrhosis, liver transplantation may eventually develop into cirrhosis.Primary biliary cirrhosis is inflammation of intrahepatic bile duct, and ultimately the formation of scar formation and intrahepatic bile ducts atresia.
Primary biliary cirrhosis is most common in women aged 35 to 60, but also can occur at any age, men and women, for unknown reasons, but usually occurs in patients with immune diseases such as rheumatoid arthritis, scleroderma, autoimmune thyroiditis.
Early primary biliary cirrhosis is inflammation of intrahepatic bile duct, inflammation, blocking the bile excretion, therefore, stay in the bile or liver cells into the blood circulation, with the inflammation spto the whole liver, lattice-like scar tissue has also spto the whole liver.
Symptoms and diagnosis
Usually gradual onset of primary biliary cirrhosis, 56% of patients the first symptom is itching, sometimes accompanied by fatigue, these symptoms tend to precede other symptoms for months or years. Physical examination, 50% of patients can reach increases, hardened liver, spleen increased 25% of patients, 15% of the patients had yellow plaque deposition in the skin (xanthoma) or eyelids (yellow spots), about 10 % of the patients increased skin pigmentation, less than 10% of patients only jaundice. Other symptoms include increased fingertips (clubbing), bone, nerve and kidney abnormalities, pale stool, oily odor associated with the late, all the symptoms and complications of cirrhosis occur.
30% of patients in the general antecedents of the onset of symptoms and are diagnosed with blood tests. Anti-mitochondrial antibody (Mitochondria are tiny structures within cells) in the blood of more than 90% of patients can be found.
When the performance of jaundice or abnormal liver function significantly, the value of the diagnosis is endoscopic retrograde cholangiopancreatography. Examination, endoscopic injection of contrast medium radiography, showed no biliary obstruction, so that doctors can better identify the site of liver disease. To obtain liver tissue using a hollow needle aspiration (biopsy) can be diagnosed by microscopic examination.
Prognosis and treatment
Progression of primary biliary cirrhosis are very different, since the beginning of the disease may affect quality of life, a good prognosis. The chronic progress of the patients seem to live longer. Some patients, the disease progressed very quickly, within a few years to develop into severe cirrhosis. Elevated serum bilirubin (jaundice) of the patients with poor prognosis, most patients had metabolic bone disease (osteoporosis).
No effective treatment, services cholestyramine to control itching. Due to lack of bile, inadequate absorption of nutrients should be added calcium, vitamin A, D and K. Ursodeoxycholic acid drugs can delay the progress of the disease appears, and generally well accepted. Patients with advanced cirrhosis of liver transplantation is the best treatment, the prognosis of liver transplantation is very good, but recurrence after liver transplantation primary biliary cirrhosis is unknown.Primary sclerosing cholangitis is a liver bile duct inflammation, scar formation and the eventual development of biliary obstruction.
Primary sclerosing cholangitis, bile duct stenosis hypertrophic scars that eventually blocked bile duct, causing cirrhosis, unexplained, but may be related to immune system abnormalities. The disease is most common in young males, occur in the existing inflammatory bowel disease, particularly ulcerative colitis patients.
Symptoms and diagnosis
Slow onset of the disease, associated with more severe fatigue, itching and jaundice. Bile duct inflammation in abdominal attacks of abdominal pain and fever have occurred, but not common. Patients with liver, spleen or liver cirrhosis increased symptoms also can occur portal hypertension, ascites, and fatal liver failure.
Endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous transhepatic cholangiography in diagnosis available. Endoscopic retrograde cholangiopancreatography examination, endoscopic photography after contrast agent injection, percutaneous transhepatic cholangiography and direct injection of contrast medium into the biliary tract after the photography. Hollow needle when necessary, to obtain liver tissue specimens for microscopic examination to confirm the diagnosis.
Prognosis and treatment
Some patients asymptomatic for 10 years (by the disease found in routine liver function tests), typically, primary sclerosing cholangitis gradually increased, drugs such as corticosteroids, azathioprine, and methotrexate is not green enzyme amine very effective and can cause serious side effects, effectiveness of ursodeoxycholic acid is not yet clear. Primary sclerosing cholangitis may require liver transplantation, which is after all the ineffective treatment of patients with advanced development to the only treatment.
Repeated inflammation of the bile duct (bacterial cholangitis) is the disease complications, required antibiotic treatment. Endoscopic biliary stricture and surgery can be expanded, and 10% to 15% of patients may be secondary to bile duct cancer (cholangiocarcinoma), slow-growing tumor, the treatment required to distraction disease endoscopic biliary stent. Occasionally, the required surgery. 1-antitrypsin deficiency is inherited antitrypsin deficiency caused by disease, can cause lung and liver lesions.
1-antitrypsin produced by the liver, found in saliva, duodenal fluid, lung secretions, tears, nasal fluid and cerebrospinal fluid. Blocking the protease enzyme is the role of the other decomposition, 1-antitrypsin deficiency resulting in damage to lung tissue of other enzymes. Lack of blood means the liver can not secrete the enzyme the enzyme, the enzyme retained in the liver cells can cause damage, that is, liver fibrosis and cirrhosis.
Symptoms and Outcomes
25% of children contracting this disease, the development of cirrhosis, portal hypertension, and 12 died before the age of about 25% of the patients died before the age of 28 and another 25% only in patients with mild hepatic dysfunction can enter adulthood, and the remaining 25 % of patients developed progressive disease-free performance.
1-antitrypsin deficiency in adults is uncommon, even exists, may lead to cirrhosis of the liver, the more common is the development of emphysema, a shortness of breath caused by the progressive lung disease. Adult 1-antitrypsin deficiency ultimately lead to liver cancer.
Treatment
Synthetic 1-antitrypsin replacement therapy has some effect, but liver transplantation is still the only effective treatment. Liver transplantation for 1-antitrypsin produced without recurrence of liver damage.
Adults suffering from this disease mainly for the treatment of lung diseases, including prevention of infections and smoking cessation.



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