$Unique_ID{BRK03814}
$Pretitle{}
$Title{Hepatitis, Neonatal}
$Subject{Hepatitis Neonatal Giant Cell Hepatitis Congenital Liver Cirrhosis
Giant Cell Cirrhosis of Newborn Giant Cell Disease }
$Volume{}
$Log{}

Copyright (C) 1986, 1990 National Organization for Rare Disorders, Inc.

199:
Hepatitis, Neonatal

** IMPORTANT **
It is possible the main title of the article (Neonatal Hepatitis) is not
the name you expected.  Please check the SYNONYMS listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Giant Cell Hepatitis
     Congenital Liver Cirrhosis
     Giant Cell Cirrhosis of Newborn
     Giant Cell Disease

General Discussion

** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only.  It should not be used for diagnostic or treatment
purposes.  If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.


Neonatal Hepatitis is a disorder in which the bile ducts inside the liver
are closed and liver cells are of varied size; some are giant cells with
multiple nuclei.

Symptoms

In Neonatal Hepatitis, the absence of an opening of the bile ducts inside the
liver causes jaundice (yellow color of the skin) because bile does not flow
freely from the liver to the small intestine.  Dark urine, pale stools and an
enlarged liver are early signs of the disorder.  Neonatal Hepatitis does not
usually become apparent until two weeks after birth.

By the age of two to three months, slow growth, irritability from
pruritus (itchiness), and signs of portal hypertension (elevated pressure in
the liver blood vessel system) may be present.

Causes

The cause of Neonatal Hepatitis is unknown in the majority of cases.  It may
be inherited by an autosomal recessive mechanism.  (Human traits including
the classic genetic diseases, are the product of the interaction of two genes
for that condition, one received from the father and one from the mother.  In
recessive disorders, the condition does not appear unless a person inherits
the same defective gene from each parent.  If one receives one normal gene
and one gene for the disease, the person will be a carrier for the disease,
but usually will show no symptoms.  The risk of transmitting the disease to
the children of a couple, both of whom are carriers for a recessive disorder,
is twenty-five percent.  Fifty percent of their children will be carriers,
but healthy as described above.  Twenty-five percent of their children will
receive both normal genes, one from each parent and will be genetically
normal.)

Affected Population

Infants of both sexes may be affected by Neonatal Hepatitis.

Related Disorders

The bile ducts inside the liver are underdeveloped in Neonatal Hepatitis or
Giant Cell Hepatitis, while the bile duct outside the liver is closed in
Biliary Atresia.  The symptoms of these two diseases are similar.

Therapies:  Standard

Diagnosis of Neonatal Hepatitis is done by surgically opening the abdomen and
obtaining an X-ray of the bile ducts using an opaque dye.  An open liver
biopsy also can be performed.  Both should be done by age three months or
younger.  Treatment consists of surgically repairing closed bile ducts, which
is successful only in 5 to 10% of cases.  In the remainder, the Kasai
procedure (portoenterostomy) can be done to surgically make a connection
between the main bile duct and the duodenum (first part of the small
intestine).  The majority of patients with Neonatal Hepatitis will
reestablish bile flow with these procedures.

Cholestyramine, which binds bile salts in the intestine, can be
administered to relieve itchiness.

Therapies:  Investigational

This disease entry is based upon medical information available through
January 1990.  Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.

Resources

For more information on Neonatal Hepatitis, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     (203) 746-6518

     The National Adrenal Diseases Foundation
     505 Northern Blvd., Suite 200
     Great Neck, NY  11021
     (516) 487-4992

     American Liver Foundation
     998 Pompton Avenue
     Cedar Grove, NJ  07009
     (201) 857-2626
     (800) 223-0179

     The United Liver Foundation
     11646 West Pico Blvd.
     Los Angeles, CA  90064
     (213) 445-4204 or 445-4200

     Children's Liver Foundation
     14245 Ventura Blvd.
     Sherman Oaks, CA  91423
     (818) 906-3021

     National Digestive Diseases Information Clearinghouse
     Box NdDIC
     Bethesda, MD  20892
     (301) 468-6344

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  Pp. 1913, 1943.