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Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy.
- Source :
-
The Journal of pediatrics [J Pediatr] 1995 Aug; Vol. 127 (2), pp. 220-4. - Publication Year :
- 1995
-
Abstract
- Objective: To determine the outcome, in index patients followed at an American Center, of syndromic paucity of interlobular bile ducts (sPILBD; Alagille syndrome), with onset of cholestasis in infancy.<br />Design: Cohort.<br />Setting: Regional referral center for infants and children with liver disease.<br />Results: During the past 10 years, 26 unrelated children with sPILBD were identified. Fifteen (58%) are alive without liver transplantation at a median age of 12.1 years. Three (11%) died, all before 2 years of age. Eight patients (31%) underwent liver transplantation at a median age of 6.5 years; all eight are alive a median 5.4 years after transplantation. The most common factors contributing to the decision for transplantation were bone fractures, pruritus, and severe xanthoma. The predicted probability of reaching 19 years of age without transplantation is about 50%; however, with transplantation, the predicted probability of long-term survival is 87%. Of 26 patients 4 (15%) have had significant central nervous system disease, and two of them have died of intracranial hemorrhage. Of the four patients who underwent cholecystoportostomy or portoenterostomy, three required liver transplantation.<br />Conclusions: Children with sPILBD identified in infancy because of cholestasis have a 50% probability of long-term survival without liver transplantation, a worse prognosis than other follow-up studies have reported. In selected patients, liver transplantation provides the opportunity for long-term survival with improved quality of life. Patients with sPILBD are at risk of having intracranial hemorrhage.
- Subjects :
- Alagille Syndrome complications
Alagille Syndrome surgery
Child
Child, Preschool
Cohort Studies
Colorado epidemiology
Female
Humans
Infant
Infant, Newborn
Male
Portoenterostomy, Hepatic
Prognosis
Survival Analysis
Survival Rate
Time Factors
Alagille Syndrome mortality
Cholestasis etiology
Liver Transplantation
Subjects
Details
- Language :
- English
- ISSN :
- 0022-3476
- Volume :
- 127
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- The Journal of pediatrics
- Publication Type :
- Academic Journal
- Accession number :
- 7636645
- Full Text :
- https://doi.org/10.1016/s0022-3476(95)70298-9