Answers are provided for your general information only.

What is cirrhosis?
Can my child have a healthy lifestyle after the Kasai?
What are the chances of my future children having PFIC?
What is NASH and what are some treatments for it?
What is the success rate of the biliary diversion operation?
Can cirrhosis cause low platelet counts like ITP?
Is it typical for liver function tests to fluctuate?

Q: What is cirrhosis?

A: Cirrhosis is often described as scar tissue, but cirrhosis is more than just scar tissue. The term cirrhosis is an anatomic description of the liver. It is defined as fibrosis (scarring) plus nodule formation (regeneration). Fibrosis alone does not qualify as cirrhosis. Fibrosis, like all scarring, basically never goes away. Thus cirrhosis is a pattern, not a disease or a specific phenomenon.

Cirrhosis can happen after any disease that causes liver cell death. Exactly why the regenerating nodules are not able to replace the function of the liver is not clear, although it may be the scarring itself that prevents recovery. We do know that if part of a normal liver is removed, the remaining liver will grow back to just the right size and function normally. This remarkable ability is not understood. Other organs like the kidneys, heart, and lungs are not able to regenerate. They can hypertrophy, (cells get larger) but they do not have cellular division that results in significant "regrowth" (more cells) like the liver does. This regrowth is what happens when a living donor gives part of their liver to be transplanted.

Two problems happen in patients with cirrhosis -- liver failure and portal hypertension. The liver failure causes fatigue, jaundice (yellow skin), and encephalopathy (a state of confusion that progresses to coma). The portal hypertension causes bleeding and ascites (fluid accumulation in the abdomen). These things happen in many forms of liver disease, not just in cirrhosis.

The scarring never goes away, but the liver has "reserve," meaning it can do all the body requires even though it is not working at 100 percent. The people that have long-standing (months to years) liver disease and develop the complications of cirrhosis that I mentioned will not, in general, recover. Some people can get a more sudden, "acute" form of liver failure (over days to weeks) and develop these complications, but these people do have the potential to recover on their own in some circumstances.
-Jeffery Punch, MD

Q: I have a 9-month-old daughter who has biliary atresia. My question is why do some children survive with a Kasai and some cannot? My daughter looks so healthy. Yes, she is jaundiced but otherwise she is healthy. Her bilirubin is high, but it doesn't affect her. Is there anything I can do so my daughter can live a healthy lifestyle without being sick?

A: Biliary atresia is a complex disease that often involves the bile ducts inside the liver as well as outside the liver. The earlier a Kasai procedure is done, the more likely there will be a good response. A successful Kasai will result in loss of jaundice. There may be complications related to severe liver damage such as fluid retention, bleeding and infections. The best lifestyle after the Kasai would be to have your child take the prescribed medications and follow the diet recommended for children with liver disease. It will be important to obtain laboratory tests or imaging to follow the liver function and to have your child seen regularly by your health provider.
-William Berquist, MD

Q: I have a 17 month old son who had a liver transplant seven months ago due to PFIC type II. What are the chances of my future kids having it? Is screening available?

A: PFIC (progressive familial intrahepatic cholestasis) type II disorder classically recurs in families and a gene mutation has been identified. Genetic consultation is strongly recommended. Your pediatrician, pediatric hepatologist or transplant center should be able to refer you to a competent genetic counselor.
-William Berquist, MD

Q: What is NASH and what are some treatments for it?

A:  NASH is Non-Alcoholic Steatohepatitis or fatty liver and is associated with obesity.  Other causes are nutritional imbalance.  Proper nutrition and adequate vitamin E are felt to be the proper means to improve the condition.
-William Berquist, MD

Q: My 18 month old daughter has Intrahepatic Cholestasis, and is very healthy other than her terrible itching. She is currently taking Actigall and Rifampin, but the itching persists. Her doctor has mentioned the possibility of a Biliary  Diversion, can you tell me anything about this procedure including the success rate? Also, has anyone with this disease ever died from having a high bile acid count?

A: The itching is due to retained bile salts or other products and biliary diversion permits a means to prevent accumulation of bile salts in tissue. Biliary Diversion has variable success depending on the type of liver disease, the surgery, age and other variables. Loss of bile will affect absorption of nutrients and attention towards nutritional issues is important. A high bile count is not a primary cause of death, but it is definitely not good for children who are developing and growing.
-William Berquist, MD

Q: My daughter was diagnosed with cirrhosis of the liver at age 18 months via liver biopsy. It was previously believed that she could have ITP.  She had bone marrow aspirations prior to the liver biopsy and ITP was ruled out. She continues to have low platelet counts. My main question: Does a person with ITP also have cirrhosis? How would it be decided that cirrhosis is the reason for all of her complications and not ITP?

A: Idiopathic Thrombocytopenic Purpura (ITP) refers to a clinical situation where an individual has low platelet counts but the cause is unknown. The marrow or "factory" of the platelets is not working well or there may be other causes.  In cirrhosis the liver becomes hardened due to scar tissue or fibrosis from some type of injury: infections, loss of oxygen, or toxic injury from chemicals or metabolic problems just to name a few causes.  Blood backs up in the portal veins that lead to the liver due to the resistance created by the fibrosis.  The spleen, as a part of the portal system, enlarges.  The enlarged spleen, which is like a blood filter, starts trapping platelets and  lowers the platelet count (which will resemble ITP) You may wish to discuss more of the details of your daughter's condition with your doctor to determine if she has an enlarged spleen.  The condition is called hypersplenism when there is a low platelet count due to a large spleen.
-William Berquist, MD

Q: My 2-year-old son has been diagnosed with liver disease of unknown origin. His liver function tests show an elevated AST & ALT that changes frequently. His doctor does not seem concerned. I am told that he may get better and we may never know what caused the problem. Today, I noticed that he is becoming jaundiced again. Is this typical of pediatric liver disease? Also, he has been referred to an endocrinologist because of the possibility of rickets. Is it typical for a child to keep getting normal/abnormal/normal/abnormal lab results? My concern is the high values are considered extremely high.

A: There are some liver disorders which may result in intermittently abnormal liver tests: BRIC or benign recurrent intrahepatic cholestasis or gallstones for example.  Rickets are a sign of vitamin D malabsorption and a sign of more prolonged liver dysfunction.  These issues may lead to serious consequences, but not in all cases. Recommendation: seek consultation with a pediatric gastroenterologist or hepatologist.
-William Berquist, MD