In:
Hepatology Research, Wiley, Vol. 49, No. 12 ( 2019-12), p. 1386-1397
Abstract:
Primary sclerosing cholangitis (PSC) is very rare in Japan. Although a large‐scale cohort study of 781 pediatric‐onset PSC patients in Europe and North America showed that the 5‐year survival with native liver was 88%, the long‐term outcomes of pediatric‐onset PSC in Japan are unknown. Here, we evaluated the clinical outcomes of pediatric‐onset PSC in Japan. Methods We carried out a retrospective cohort study with a medical records review of pediatric PSC patients diagnosed between 1986 and 2017 at a single center. The PSC diagnoses were based on cholangiography, liver histology, and biochemical findings. The patients' survival was analyzed using the Kaplan–Meier method. Prognostic factors were determined by univariate and multivariate analyses using the Cox proportional hazards regression model. Results We identified 39 pediatric‐onset PSC patients (22 boys, 17 girls). The median age at diagnosis was 9 years (interquartile range 6.0–13.5 years). The median follow‐up period was 5.5 years (interquartile range 3.4–8.7 years). The phenotypes of PSC‐autoimmune hepatitis, PSC‐inflammatory bowel disease, and small‐duct PSC were diagnosed in 13 (33.3%), 36 out of 38 (94.8%), and three (7.7%) patients, respectively. The 5‐year liver transplantation‐free survival of the whole cohort was 93.5%. Nine patients underwent liver transplantation, and four of these nine cases resulted in death. Both the univariate and multivariate analyses showed that the phenotype of “PSC‐autoimmune hepatitis overlap” was an independent poor prognostic factor. Conclusions The overall survival of pediatric‐onset PSC in Japan was comparable to those in Western countries. The phenotype of PSC‐autoimmune hepatitis was identified as a prognostic factor associated with a poorer long‐term outcome.
Type of Medium:
Online Resource
ISSN:
1386-6346
,
1872-034X
Language:
English
Publisher:
Wiley
Publication Date:
2019
detail.hit.zdb_id:
2006439-1
Permalink