In general, liver transplantation is required in kids and adults who is suffering from constant liver dysfunction because of various reasons and where the benefits of transplantation out weight the risk of alternative modalities.
The most usual indications for liver transplantation are the hepatitis b and c infection and alcoholic liver disease. Liver transplantation is a life-saving system for patients with chronic end-stage liver disease and selected patients with acute liver failure.
Specific indications can include:
- Fulminant hepatic failure
- Extrahepatic biliary atresia
- Inherent errors of metabolism including
- Primary Sclerosing cholangitis
- Hepatocellular Carcinoma (HCC), Stage I or II, or: single lesion
- Cirrhosis including:
- Alpha-I antitrypsin
- Crigler-Najjar disease, Type I
- Byler’s disease
- Glycogen storage disease, Type I
- Wilson’s disease
- Hemochromatosis
- Tyrosinemia
- Wolman’s disease
- Familial amyloidotic polyneuropathy
- Primary hyperoxaluria type 1
- Alcohol related cirrhosis
- Biliary cirrhosis (primary or secondary)
- Chronic active hepatitis (A, B, C, non A, non B, autoimmune)
- Congenital biliary cirrhosis
- Cryptogenic cirrhosis
- Hemochromatosis
- Alpha I Antitrypsin Deficiency
- NASH
- Viral cirrhosis
- Other
what is acute hepatic fulminant failure ?
Fulminant Hepatic Failure or Acute Liver failure is described by severe liver insult which causes hepatocellular dysfunction, hepatic encephalopathy (any grade) and coagulopathy (INR>1.5), all occurring within 26 weeks, in a patient with earlier normal liver function.
Regardless of the cause, typical clinical features include nonspecific signs such as malaise, fatigue, nausea, vomiting occurring in an earlier healthy person, which is later followed by jaundice, rapid onset of altered mental status, and eventually coma.
In general, patients with fulminant hepatic failure can worsen very rapidly; therefore, admission to the ICU is advised at the time of diagnosis, preferably in a liver transplant center, especially when a patient has an advanced grade of encephalopathy.
What are sings & symptoms of extrahepatic biliary atresia?
The signs of biliary atresia usually manifest by the age of two to six weeks and include a yellowish coloration of the skin and eyes (jaundice), abnormally pale stools, and dark colored urine. Children may also have a swollen stomach or irregular expansion of the liver (hepatomegaly). By the age of six to 10 weeks, further signs may also occur including poor weight gain, irritability or an increase in blood pressure within the veins that carry blood from the intestine to the liver (portal hypertension). Bile ducts inside the liver (intrahepatic bile ducts) are also involved. If left untreated, biliary atresia may result in permanent scarring of the liver (cirrhosis) and, eventually, liver (hepatic) failure.
Some children with biliary atresia may have some other abnormalities including malformations of the heart (e.g., situs inversus, levocardia, and ventricular septal defects) and/or kidneys. Situs Inversus is a state in which the inner organs are on the opposite side of the body from normal. Levocardia is a state in which the heart is malpositioned.
Biliary atresia may also be associated with absence of the spleen (asplenia), appearance of more than one spleen (polysplenia), and/or other anatomical abnormalities.
What is hepatic vein thrombosis?
Hepatic vein thrombosis (HVT) is an obstruction in the hepatic veins of the liver caused by a blood clot.
This condition blocks the outflow system of patient’s liver. Because of back pressure in liver patient can develop fluid in belly and jaundice. This can severely damage the liver and may lead to liver failure.
The symptoms caused by HVT vary from person to person. Some people may have no symptoms until the blockage causes liver damage. HVT is also called Budd-Chiari syndrome. Certain medications, diseases, and inherited disorders can cause HVT. Anything that can cause blood clotting in the veins of your liver may lead to HVT.