Answers are provided for your general information only.

What is autoimmune hepatitis?
What are some ideas to treat itching?
What are liver cysts?
Can a tick bite cause an enlarged liver?
What is neonatal hepatitis?
What is pediatric primary sclerosing cholangitis?
What is "rickets"?

Q: I just found out that my niece was diagnosed with auto-immune hepatitis and cirrhosis of the liver. Please provide any info you have regarding auto-immune hepatitis in children.

A: Autoimmune hepatitis is a form of chronic hepatitis that may often present without symptoms. It responds generally well to prednisone and azothiaprine with a favorable prognosis if detected early. Sometimes it resolves and the patients may not require further immunosuppression. Autoimmune markers, absence of other causes, and a confirmatory liver biopsy are necessary. One must be careful of checking for Wilson's Disease which could be present in the over 3 year old group. 
-William Berquist, MD

Q: I have a 21 month old daughter who had a Kasai procedure at 11 weeks of age. Her bilirubin is normal. Over the last few months, however, she has become increasingly "itchy" and scratches herself to the point of bleeding. Any suggestions would be appreciated.

A: Itching is associated with retention of bile salts made by the liver. This is called "pruritus." Some of the other contributing factors or causes include eczema, dermatitis, dry skin, fatty acid deficiency and assorted allergies. Ursodeoxycholate (Ursodiol, Actigall) is used to help alleviate the symptoms. Certainly a trial off and on again with any medication (with physician guidance), soap or type of clothing fabric (cotton is best) may be tried. Other common treatments include rifampin (an antibiotic), antihistamines (Benadryl, Atarax), and cholestyramine which binds the bile salts. Proper skin care and consultation with pediatrics or dermatology is important, too. Just because the jaundice is gone does not mean that the bile flow or other complications related to liver injury and biliary atresia might not remain problems. If the itching is a problem, I trust you would review the situation with your daughter's physician. 
-William Berquist, MD

Q:  My son has a cyst on his liver. He had surgery to remove it. They said it was a simple cyst and should not come back, but unfortunately it has reappeared. If they took the cyst off his liver how can it continue to reappear?

A: There are many types of liver cysts. Some may be vascular, some contain bile, some have tissue fluid and some have parasites in them. Simple cysts are benign and may be followed by ultrasound at regular intervals (every 6-12months).  If they do not cause symptoms such as abdominal pain they may be left alone. Vascular cysts may bleed and enlarge causing symptoms and may require resection after appropriate studies (possibly an angiogram or MR or CT may help). Care on biopsies must be emphasized as needles could precipitate a bleed.
-William Berquist, MD

Q: My 10-year-old daughter went to the doctor for fever and headache. She had been bitten by a tick the week before. The doctor found her liver to be enlarged and has ordered blood tests. What could this be? Rocky Mountain Spotted fever has been ruled out.

A: The liver may become enlarged in infectious conditions. The liver contains many cells which trap foreign organisms and this may set up an enlarged, sometimes tender liver. The possibilities should be investigated by your primary physician. The liver enzymes and functional status should be checked. An ultrasound of the liver may be helpful.
-William Berquist, MD

Q:  What is neonatal hepatitis?

A: Neonatal hepatitis is an inflammatory reaction within the liver in newborn infants. It presents with jaundice and a large liver. Infectious causes (particularly viruses) might cause it but often no agent is found.  Most cases resolve, but some cases progress and may even require liver transplantation.
-William Berquist, MD

Question:  My 9-year-old son has been diagnosed with possible primary sclerosing cholangitis. I am interested in any information you have about this disease in children. Are there any specialists in this field?

A: Primary sclerosing cholangitis refers to a pattern of inflammatory changes associated with the bile ducts inside and outside the liver. It is often associated with inflammatory bowel disease but may be seen in a number of other conditions. It may be diagnosed by liver biopsy and cholangiograms (radiographs of the biliary tree). Sometimes it must be treated by liver transplantation. Therapy includes ursodeoxycholate (Actigall, Ursodiol).  Most centers with pediatric hepatologists and liver transplant programs are familiar with this disease in children.
-William Berquist, MD

Question:  What is "rickets"?

A: Rickets is a general term that refers to vitamin D deficiency in children. The more common and classic form in normal children was due to the lack of vitamin D and sunlight resulting in a bending of weight bearing bones and the appearance of nodular ends of the bones. Rickets is rare in healthy children in the United States.

Vitamin D deficiency is common in children with liver and kidney disorders. In cholestatic liver disorders where there is significant jaundice, bile salts necessary for the absorption of fat and fat soluble vitamins (including vitamin D) do not get into the intestine. Vitamin D is then lost or not absorbed. In addition, the liver processes vitamin D into an active form, so as the liver disease advances, even if vitamin D is absorbed it is not activated.

Since Vitamin D helps in calcium absorption and bone mineralization, the bones become weak and bend. The growth plates of bones enlarge and become more nodular. Diuretics, used to manage ascites (abnormal fluid retention), may lead to calcium loss in the urine. Kidney disease may affect calcium and phosphorus balance as well as Vitamin D activation.

With so many variables, including calcium intake, degree of cholestasis, presence of kidney disease, degree of liver disease, and use of diuretics, it may be expected that the degree of rickets and bone disease may vary from patient to patient.

Generally, it is advisable to follow vitamin D, calcium, and phosphorus levels in children with chronic liver disease and to add some vitamin D to the diet or by supplements.

Rickets is a complex issue to solve in some patients with advanced liver disease. Successful liver transplant, combined with good nutrition, will lead to a cure assuming that there is not persistence of other factors which contribute to rickets.
-William Berquist, MD