Chai, P.F. and Lee, Way Seah and Brown, R.M. and McPartland, J.L. and Foster, K. and McKiernan, P.J. and Kelly, D.A. (2010) Childhood autoimmune liver disease: indications and outcome of liver transplantation. Journal of Pediatric Gastroenterology and Nutrition, 50 (3). pp. 295-302. ISSN 0277-2116, DOI https://doi.org/10.1097/MPG.0b013e3181bf0ef7.
Full text not available from this repository.Abstract
Background: Graft rejection and disease recurrence are well-recognized complications of liver transplantation (LT) for autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC). We describe indications and outcome of LT for childhood AIH and AISC. Patients and Methods: Twenty-year retrospective review of a cohort of children (n = 101) with AIH, AISC, or AIH/sclerosing cholangitis overlap syndrome from a single center. Results: AIH type 1 (AIH1, n 67) was more common than AIH type 2 (AIH2, n 18), AISC (n = 8), or overlap syndrome (n = 8). Overall, 18 patients (18) required LT, the indications being failure of medical therapy (n = 16) and fulminant liver failure (n = 2). Patients with AIH who required LT had a more prolonged prothrombin time at presentation compared with those who did not undergo transplantation (P = 0.01). Patients with AIH1 who received LT had a lower aspartate transaminase (P - 0.009) and alanine transaminase (P = 0.02) levels at initial diagnosis compared with those with AIH1 who did not undergo transplantation. Post-LT, 11 patients (61) had 18 episodes of rejection, most were steroid sensitive. Disease recurrence was observed in 7 patients (39, median duration post-LT 33 months), more common in AIH2 (80 recurrence rate), and those taking cyclosporine (71, 5/7 patients) compared with those taking tacrolimus (18, 2/11 patients; P< 0.05) and in 3 of 3 children who did not have maintenance steroids post-LT. The overall 5- and 7-year post-LT survival rate was 94 and 88, respectively. Conclusions: LT is a good therapeutic option for progressive AIH and AISC, although recurrence of the primary autoimmune process limits the outcome.
| Item Type: | Article |
|---|---|
| Funders: | UNSPECIFIED |
| Uncontrolled Keywords: | Autoimmune liver disease; childhood; liver transplant; primary sclerosing cholangitis; ursodeoxycholic acid; allograft-rejection; overlap syndrome; hepatitis |
| Subjects: | R Medicine R Medicine > RJ Pediatrics |
| Divisions: | Faculty of Medicine |
| Depositing User: | Ms Haslinda Lahuddin |
| Date Deposited: | 13 Jul 2014 06:29 |
| Last Modified: | 21 Mar 2019 06:47 |
| URI: | http://eprints.um.edu.my/id/eprint/10888 |
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