Biliary atresia is a rare but serious disease of the liver that affects newborn infants. It occurs in about one in 10,000 children and is more common in girls than in boys and newborns Asian and African Americans than in Caucasian infants. Biliary atresia is the most common reason for liver transplantation in children in the United States and most of the western world.
Liver damage incurred by biliary atresia is caused by damage and loss (atresia) of the bile ducts, which is responsible for draining bile from the liver. Bile is made in the liver through the bile ducts and intestines where it helps digest food, fat and cholesterol. The loss of bile ducts causes bile to remain in the liver. When bile accumulates, it can damage the liver, causing scarring and loss of liver tissue. When the liver fails, a liver transplant becomes necessary. Biliary atresia can lead to liver failure and the need for a liver transplant in 1-2 years of life.
The pathology of the extrahepatic biliary system widely varies in these patients, and the following classification is based on the predominant site of atresia:
Type I involves obliteration of the common duct; the proximal ducts are patent
Type II is characterized by atresia of the hepatic duct, with cystic structures found in the porta hepatis
Type III (>90% of patients) involves atresia of the right and left hepatic ducts to the level of the porta hepatis. These variants should not be confused with intrahepatic biliary hypoplasia, which comprises a group of distinct and surgically noncorrectable disorders.
Currently, more than 17,000 people in the United States are waiting for liver transplants. According to the United Network for Organ Sharing (UNOS), about5,300 liver transplantations were performed in the United States in 2002. The liver is the second most commonly transplanted major organ, after the kidney, so it is clear that liver disease is a common and serious problem in this country. It is important for liver transplant candidates and their families to understand the basic process involved with liver transplants, to appreciate some of the challenges and complications that face liver transplant recipients (people who receive livers), and to recognize symptoms that should alert recipients to seek medical help.
Determining whose need is most critical: The United Network for Organ Sharing uses measurements of clinical and laboratory problems to divide patients into groups that determine who is in most critical need of a liver transplant. In early 2002, UNOS enacted a major modification to the way in which people were assigned the need for a liver transplant. Previously, patients awaiting livers were ranked as status 1, 2A, 2B, and 3, according to the severity of their current disease. Although the status 1 listing has remained, all other patients are now classified using the Model for End-Stage Liver Disease (MELD) scoring system if they are aged 18 years or older, or the Pediatric End-Stage Liver Disease (PELD) scoring system if they are younger than 18 years. These scoring methods were set up so that donor livers could be distributed to those who need them most urgently.
Who may not be given a liver: A person who needs a liver transplant may not qualify for one because of the following reasons:
Active alcohol or substance abuse: Persons with active alcohol or substance abuse problems may continue living the unhealthy lifestyle that contributed to their liver damage. Transplantation would only result in failure of the newly transplanted liver.
Cancer: Cancers in locations other than just the liver weigh against a transplant.
Advanced heart and lung disease: These conditions prevent a transplanted liver from surviving.
Severe infection: Such infections are a threat to a successful procedure.
Massive liver failure: This type of liver failure accompanied by associated brain injury from increased fluid in brain tissue rules against a liver transplant.
HIV infection
Liver Transplant Causes
Liver disease severe enough to require a liver transplant can come from many causes. Doctors have developed various systems to determine the need for the surgery. Two commonly used methods are by specific disease process or a combination of laboratory abnormalities and clinical conditions that arise from the liver disease. Ultimately, the transplantation team takes into account the type of liver disease, the person's blood test results, and the person's health problems in order to determine who is a suitable candidate for transplantation.
In adults, chronic active hepatitis and cirrhosis (from alcoholism, unknown cause, or biliary) are the most common diseases requiring transplantation. In children, and in adolescents younger than 18 years, the most common reason for liver transplantation is biliary atresia, which is an incomplete development of the bile duct.
Liver Transplant Symptoms
People who have liver disease may have many of the following problems:
Jaundice - Yellowing of the skin or eyes
Itching
Dark, tea-colored urine
Gray- or clay-colored bowel movements
Ascites - An abnormal buildup of fluid in the abdomen
Drink alcohol in moderation, if at all. Limit the amount of alcohol you drink to no more than one drink a day for women and no more than two drinks a day for men.
Avoid risky behavior. Get help if you use illicit
intravenous drugs. Don't share needles used to inject drugs. If you
choose to have sex, use condoms. If you choose to have tattoos or body
piercings, be picky about cleanliness and safety when it comes to
selecting a shop.
Get vaccinated. If you're at increased risk of
contracting hepatitis or if you've already been infected with any form
of the hepatitis virus, talk to your doctor about getting the hepatitis B
vaccine. A vaccine is also available for hepatitis A.
Use medications wisely. Only use prescription and
nonprescription drugs when you need them and take only the recommended
doses. Don't mix medications and alcohol. Talk to your doctor before
mixing herbal supplements or prescription or nonprescription drugs.
Avoid contact with other people's blood and body fluids.
Hepatitis viruses can be spread by accidental needle sticks or improper
cleanup of blood or body fluids. It's also possible to become infected
by sharing razor blades or toothbrushes.
Take care with aerosol sprays. When you use an
aerosol cleaner, make sure the room is ventilated, or wear a mask. Take
similar protective measures when spraying insecticides, fungicides,
paint and other toxic chemicals. Always follow the manufacturer's
instructions.
Watch what gets on your skin. When using insecticides and other toxic chemicals, cover your skin with gloves, long sleeves, a hat and a mask.
Choose a healthy diet. Choose a plant-based diet with a variety of fruits and vegetables. Limit high-fat foods.
Maintain a healthy weight. Obesity can cause a condition called nonalcoholic fatty liver disease, which may include fatty liver, hepatitis and cirrhosis.
Liver Disease Symptoms - Learning to recognize and understand Liver Disease Symptoms
Your liver is the largest organ inside your body. It is also one of the most important. The liver has many jobs, including changing food into energy and cleaning alcohol and poisons from the blood. Your liver also makes bile, a yellowish-green liquid that helps with digestion.
Definition Liver problems include a wide range of diseases and conditions that can affect your liver. Your liver is an organ about the size of a football that sits just under your rib cage on the right side of your abdomen. Without your liver, you couldn't digest food and absorb nutrients, get rid of toxic substances from your body or stay alive.
Liver problems can be inherited, or liver problems can occur in response to viruses and chemicals. Some liver problems are temporary and go away on their own, while other liver problems can last for a long time and lead to serious complications.
Symptoms Signs and symptoms of liver problems include:
Lipids that accumulate in alcoholic fatty liver may come from many sources: diet, adipose tissue, hepatic synthesis of novo from glucide. Predominant source depends on the chronic or acute consumption of alcohol, lipid content in diet. In alcoholics, the majority of the lipids in the liver are derived from diet and from oxidation of fatty acids. Accumulation of lipids during the chronic consumption of alcohol does not continue forever, modification of the redox state of the whole liver will slow down during the chronic consume of the alcohol.
Morphopathologicallys teatosis represents the presence of the lipid in more than 5% of hepatocyte. Macroscopically, the liver is enlarged, firm with a yellowish color. Microscopically it can be observed an increase in fat deposits, later intrahepatic cholestasis, migh appear.
Clinically an asymptomatic hepatomegaly is observed and sometimes stigmas of chronic liver disease like contraction of Dupuytren, testicular atrophy, palmar erithrosis, sidernavy, and gynecomastia. Later, fatigue, cachexia, fever, anorexia, nausea, vomiting, jaundice, painful hepatomegaly, splenomegaly and ascites might appear. Majority of this is associated with alcoholic hepatitis.
Positive diagnosis is done by histological examination and imagistic evidence by doing an ultrasound examination. A particular form is the focal steatosis, which makes it difficult to differentiate with a tumoral formation.
Complications that might appear in patients with alcoholic steatosis are sudden death through fatty embolida, alcoholic cessation syndrome or hypoglicemia.
Treatment consits in stopping of alcohol consumption, correction of malnutrition and drugs so called hepatoprotectors (slimarine and mainly vitamin B complex) with disputable efficiency.
Metabolism of alcohol in the liver takes place in three ways, result being the same, and in the liver takes place in three ways, result being the same, and acetaldehyde is metabolized with high hepato toxicity. Those three ways are: 1. alcoholdehydrogenasis path is the major way of metabolizing alcohol. 2. microsomal oxidation system - P450, interfere in oxidation of alcohol when its concentration is more than 50 mg/dl. 3. catalyze way, which has a seconday role.
Acetaldehyde is later oxidated up to acetate, but in alcoholics, the capacity of mitochondrion to oxidate acetaldehyde is reduced. Their accumulation leads to the promotion of lipid peroxidates and the formation of complex proteins. Besides the toxic effect of the acetaldehyde, alcohol has its role in developing cirrhosis. It has been proven that adipocytes involved in fibrogenesis are active after chronic consumption of alcohol.
Morphologic aspects in alcoholic liver: there are three typer of hepatic histologic lesions in consumers of alcohol. 1. Alcoholic fatty liver is a genign reversible form produced by accumulation of some drops of lipids in hepatocyte. 2. Alcoholic hepatic comprise of degeneration and necrosis of the hepatocyte, acute infiltration of neutrophyles sometimes pericellular, sinusoidal line and venular line fibrosis, as well characteristic Mallory corpuscles (alcoholic hialin). Alcoholic hepatitis may be reversible, but the lesions are much more severe being the most important precursors of cirrhosis. 3. Alcoholic cirrhosis compriseof fibrosis in all hepatic tissue, from the portal space up to centrolobular vein, and nodule of regeneration.
Alcoholic livercauses or alcoholic liver disease is represented by a nonspecific total morphological lesion associated with clinical / paraclinical manifestation, among which some are induced by the abuse of alcohol.
Prevalence: alcoholic liver varies from country to country depending on the tradition, religion and especially on the cost of the alcoholic drinks in comparison with the income.
Alcoholic liver causes: excessive alcohol consumption has varied effect on organisms. Due to some unknown cause, 1/3 of chronic consumers of alcohol do not have hepatic consequences. 1. Duration and consumption of alcohol, it is considered that 60-80 ml of absolute alcohol/day for men and 40-50 ml of absolute alcohol women as a toxic dose. The duration of consumption is also important, a consumption of more than 5 years is considered as a risk. Continous consumption is more dangerous than intermittent consumtion. As well, hepatic injury does not depend on the type of drink but in this alcohol content. 2. Gender: females are more susceptible than men are, because at the same quantity of alcohol ingested reach a higher blood concentration. In women, gastric metabolism is reduced comparing to men and cytochrome P450 is less efficient. 3. Genetic factors: although no genetic markers has proved to have a clear association with susceptibility to alcoholism, seems to inherit certain behavior related to the alcohol consumpion. 4. Coinfection with hepatic viruses (B or C) accelerates the severity of the alcoholic liver disease. 5. Nutritional factors: protein caloric malnutrition precedes alcoholism in patients with socio-economic level leading to the appearance of alcoholic liver. It has been proved that healthy diet may protect those who consume alcohol, at least for a period.
Biliary atresia is a rare but serious disease of the liver that affects newborn infants. It occurs in about one in 10,000 children and is more common in girls than in boys and newborns Asian and African Americans than in Caucasian infants. Biliary atresia is the most common reason for liver transplantation in children in the United States and most of the western world.
The pathology of the extrahepatic biliary system widely varies in these patients, and the following classification is based on the predominant site of atresia:
