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Maternal (Expert Forum)
17 month with neonatal hepatitis/intra-hepatic bile duct paucity
Patient medical question and answer from The Maternal and Child Health Forum. Health topic area and articles about newborn care
17 month with neonatal hepatitis/intra-hepatic bile duct paucity
by Susan__0, Nov 20, 1998 12:00AM
Our daughter was diagnosed with this at 1 month old. What I am looking for are answers to these questions: What are the signs of liver failure? Will her eating habits become disrupted with this disease? Sleeping patterns? Can this disease sometimes cure itself? And if not what are the life spans with no transplant? Any and all info would be greatly appreciated.Thank You.
Doctor's Answer
by HFHS.MD-HSW, Nov 20, 1998 12:00AM
Dear Susan:
Scarcity of bile ducts within the liver occurs in two primary forms, syndromic and nonsyndromic. Syndromic forms include Alagille syndrome and Byler syndrome. Byler syndrome, found most commonly among the Amish, is much more likely to progress to liver failure within the first two decades of life. Slightly more than 10% of patients with Alagille syndrome develop cirrhosis, but almost all patients with Byler are so afflicted. Both syndromes are associated with an increased risk for growth retardation and defects in cognitive abilities. Alagille syndrome is associated with abnormal vertebral development and congenital heart disease.
The nonsyndromic forms of intrahepatic bile duct paucity are not initially associated with anomalies in structure or function in other organ systems. Itching and elevated blood lipids are an early consequence of liver disease. However, nearly half of affected children ultimately develop liver failure.
Your note does not contain enough information to determine what particular form of the disorder your daughter suffers from. I presume your daughter is under the care of both a pediatrician and a pediatric gastroenterologist who are in possession of the relevant facts. They are primarily responsible for her care and should be able to provide you with a specific diagnosis as well as detailed answers to all of your questions. I hope my answer helps you organize your questions for them.
This information is provided for educational purposes only. Best wishes.
HFHS.MD-HSW
KEYWORDS: intrahepatic bile duct paucity, Alagille syndrome, Byler syndrome
