Enter your Email address:
Wolters Kluwer Health may email you for journal alerts and information, but is committed
to maintaining your privacy and will not share your personal information without
You currently have no recent searches
Piccoli, D A; Goldman, A P; Loomes, K M; McBride, K E
Children's Hospital of Philadelphia, Univ. of PA School of Medicine, Phila, PA
Alagille syndrome (AGS) and biliary atresia (BA) are the most common bile duct disorders leading to transplantation in children. The degree of cholestasis in severe AGS is more profound than in BA, but the implications for transplantation are different. A toddler with jaundice due to BA will require eventual transplantation, but the same may not be true for AGS, AIMS: To document the extent and the temporal progression of cholestasis in AGS. METHODS: Records of 11 older AGS pts (7M:4F, ages 4.0 to 21.9 yrs) with severe disease were analyzed for biochemical parameters of cholestasis and synthesis. The mean, median and range of peak and final values and age (in yrs) at peak were recorded for bilirubin (bili), bile salts (BS), ALT, AST, GGT, alkaline phosphatase (AP), albumin and PT. (Table)
Albumin and PT demonstrate stable synthetic function in all pts.
CONCLUSIONS: Measures of cholestasis may continue to worsen until the 3rd or 4th year of life in AGS pts whose cholestasis will subsequently improve. Mean peak cholestasis occurs at 2.5 to 3.8 yrs of age, according to the biochemical parameter assessed. Serum bile salts (167×) and GGT (40×) are most elevated, followed by alkaline phosphatase (17×), bilirubin (9×) and cholesterol (5×) in pts with normal synthetic capability. SPECULATIONS: Cholestasis can dramatically improve in pts with severe AGS. Commitment to transplantation should not be based solely on the level of cholestasis, particularly in young children, as long term survival without transplantation is common. Further investigations into the mechanisms whereby cholestasis is emeliorated may provide information that will lead to effective therapy.
© 1998 Lippincott Williams & Wilkins, Inc.
Colleague's E-mail is Invalid
Your Name: (optional)
Separate multiple e-mails with a (;).
Thought you might appreciate this item(s) I saw at Journal of Pediatric Gastroenterology and Nutrition.
Send a copy to your email
Your message has been successfully sent to your colleague.
Some error has occurred while processing your request. Please try after some time.
An Existing Folder
A New Folder
The item(s) has been successfully added to "".
Login with your LWW Journals username and password.
Username or Email:
Enter and submit the email address you registered with. An email with instructions to reset your password will be sent to that address.
Link to reset your password has been sent to specified email address.
What does "Remember me" mean?
By checking this box, you'll stay logged in until you logout. You'll get easier access to your articles, collections,
media, and all your other content, even if you close your browser or shut down your
To protect your most sensitive data and activities (like changing your password),
we'll ask you to re-enter your password when you access these services.
What if I'm on a computer that I share with others?
If you're using a public computer or you share this computer with others, we recommend
that you uncheck the "Remember me" box.
Save my selection
Article Level Metrics