Publication

Disease progression in paediatric- and adult-onset sclerosing cholangitis: Results from two independent Dutch registries

Joosse, M. E., Haisma, S. M., Sterk, M. F. M., van Munster, K. N., Ponsioen, C. I. J., Houwen, R. H. J., Koot, B. G. P., de Meij, T., van Rheenen, P. F. & de Koning, B. A. E., Sep-2019, In : Liver International. 39, 9, p. 1768-1775 8 p.

Research output: Contribution to journalArticleAcademicpeer-review

APA

Joosse, M. E., Haisma, S. M., Sterk, M. F. M., van Munster, K. N., Ponsioen, C. I. J., Houwen, R. H. J., Koot, B. G. P., de Meij, T., van Rheenen, P. F., & de Koning, B. A. E. (2019). Disease progression in paediatric- and adult-onset sclerosing cholangitis: Results from two independent Dutch registries. Liver International, 39(9), 1768-1775. https://doi.org/10.1111/liv.14159

Author

Joosse, Maria E. ; Haisma, Sjoukje M. ; Sterk, Marlou F. M. ; van Munster, Kim N. ; Ponsioen, Cyriel I. J. ; Houwen, Roderick H. J. ; Koot, Bart G. P. ; de Meij, Tim ; van Rheenen, Patrick F. ; de Koning, Barbara A. E. / Disease progression in paediatric- and adult-onset sclerosing cholangitis : Results from two independent Dutch registries. In: Liver International. 2019 ; Vol. 39, No. 9. pp. 1768-1775.

Harvard

Joosse, ME, Haisma, SM, Sterk, MFM, van Munster, KN, Ponsioen, CIJ, Houwen, RHJ, Koot, BGP, de Meij, T, van Rheenen, PF & de Koning, BAE 2019, 'Disease progression in paediatric- and adult-onset sclerosing cholangitis: Results from two independent Dutch registries', Liver International, vol. 39, no. 9, pp. 1768-1775. https://doi.org/10.1111/liv.14159

Standard

Disease progression in paediatric- and adult-onset sclerosing cholangitis : Results from two independent Dutch registries. / Joosse, Maria E.; Haisma, Sjoukje M.; Sterk, Marlou F. M.; van Munster, Kim N.; Ponsioen, Cyriel I. J.; Houwen, Roderick H. J.; Koot, Bart G. P.; de Meij, Tim; van Rheenen, Patrick F.; de Koning, Barbara A. E.

In: Liver International, Vol. 39, No. 9, 09.2019, p. 1768-1775.

Research output: Contribution to journalArticleAcademicpeer-review

Vancouver

Joosse ME, Haisma SM, Sterk MFM, van Munster KN, Ponsioen CIJ, Houwen RHJ et al. Disease progression in paediatric- and adult-onset sclerosing cholangitis: Results from two independent Dutch registries. Liver International. 2019 Sep;39(9):1768-1775. https://doi.org/10.1111/liv.14159


BibTeX

@article{f407b66d53484b83934227551642ea11,
title = "Disease progression in paediatric- and adult-onset sclerosing cholangitis: Results from two independent Dutch registries",
abstract = "Background & Aims Sclerosing cholangitis (SC) is a severe liver disease leading to destruction of bile ducts. It is believed to run a milder course in children than in adults. To test this assumption, we evaluated time-to-complication curves in two independent paediatric-onset cohorts from the same geographical area. Methods Short-term disease outcomes were evaluated with an online clinical registry that was filled with data on children with SC diagnosed between 2000 and 2017 and who were followed bi-annually thereafter. Long-term disease outcomes were evaluated in a paediatric-onset subcohort derived from a previously published population-based study from the Netherlands. Time-to-complication in the first cohort was defined as the time from diagnosis until portal hypertension, biliary obstructions and infections, development of malignancy, or liver transplantation, whichever came first. In the second cohort time-to-complication was defined as the time until liver transplantation or PSC-related death. Results Median age at diagnosis in the first cohort (n = 86) was 12.3 years. In the first 5 years post-diagnosis 23% of patients developed complications. The patients in the population-based study (n = 683) were stratified into those diagnosed before the age of 18 years ('paediatric-onset' subcohort, n = 43) and those diagnosed after the age of 18 years ('adult-onset' subcohort, n = 640). Median age at diagnosis was 14.6 and 40.2 years, respectively. Median time-to-complication in the paediatric-onset and adult-onset subcohorts was not statistically different. Conclusion Paediatric and adult-onset SC run a similar long-term disease course. Paediatricians who treat children with SC should monitor them closely to recognize early complications and control long-term sequelae.",
keywords = "disease progression, inflammatory bowel disease, paediatric, prognosis, sclerosing cholangitis, AUTOIMMUNE HEPATITIS, ALKALINE-PHOSPHATASE, NATURAL-HISTORY, CHILDREN, EPIDEMIOLOGY, DIAGNOSIS, RISK",
author = "Joosse, {Maria E.} and Haisma, {Sjoukje M.} and Sterk, {Marlou F. M.} and {van Munster}, {Kim N.} and Ponsioen, {Cyriel I. J.} and Houwen, {Roderick H. J.} and Koot, {Bart G. P.} and {de Meij}, Tim and {van Rheenen}, {Patrick F.} and {de Koning}, {Barbara A. E.}",
year = "2019",
month = sep,
doi = "10.1111/liv.14159",
language = "English",
volume = "39",
pages = "1768--1775",
journal = "Liver International",
issn = "1478-3223",
publisher = "Wiley",
number = "9",

}

RIS

TY - JOUR

T1 - Disease progression in paediatric- and adult-onset sclerosing cholangitis

T2 - Results from two independent Dutch registries

AU - Joosse, Maria E.

AU - Haisma, Sjoukje M.

AU - Sterk, Marlou F. M.

AU - van Munster, Kim N.

AU - Ponsioen, Cyriel I. J.

AU - Houwen, Roderick H. J.

AU - Koot, Bart G. P.

AU - de Meij, Tim

AU - van Rheenen, Patrick F.

AU - de Koning, Barbara A. E.

PY - 2019/9

Y1 - 2019/9

N2 - Background & Aims Sclerosing cholangitis (SC) is a severe liver disease leading to destruction of bile ducts. It is believed to run a milder course in children than in adults. To test this assumption, we evaluated time-to-complication curves in two independent paediatric-onset cohorts from the same geographical area. Methods Short-term disease outcomes were evaluated with an online clinical registry that was filled with data on children with SC diagnosed between 2000 and 2017 and who were followed bi-annually thereafter. Long-term disease outcomes were evaluated in a paediatric-onset subcohort derived from a previously published population-based study from the Netherlands. Time-to-complication in the first cohort was defined as the time from diagnosis until portal hypertension, biliary obstructions and infections, development of malignancy, or liver transplantation, whichever came first. In the second cohort time-to-complication was defined as the time until liver transplantation or PSC-related death. Results Median age at diagnosis in the first cohort (n = 86) was 12.3 years. In the first 5 years post-diagnosis 23% of patients developed complications. The patients in the population-based study (n = 683) were stratified into those diagnosed before the age of 18 years ('paediatric-onset' subcohort, n = 43) and those diagnosed after the age of 18 years ('adult-onset' subcohort, n = 640). Median age at diagnosis was 14.6 and 40.2 years, respectively. Median time-to-complication in the paediatric-onset and adult-onset subcohorts was not statistically different. Conclusion Paediatric and adult-onset SC run a similar long-term disease course. Paediatricians who treat children with SC should monitor them closely to recognize early complications and control long-term sequelae.

AB - Background & Aims Sclerosing cholangitis (SC) is a severe liver disease leading to destruction of bile ducts. It is believed to run a milder course in children than in adults. To test this assumption, we evaluated time-to-complication curves in two independent paediatric-onset cohorts from the same geographical area. Methods Short-term disease outcomes were evaluated with an online clinical registry that was filled with data on children with SC diagnosed between 2000 and 2017 and who were followed bi-annually thereafter. Long-term disease outcomes were evaluated in a paediatric-onset subcohort derived from a previously published population-based study from the Netherlands. Time-to-complication in the first cohort was defined as the time from diagnosis until portal hypertension, biliary obstructions and infections, development of malignancy, or liver transplantation, whichever came first. In the second cohort time-to-complication was defined as the time until liver transplantation or PSC-related death. Results Median age at diagnosis in the first cohort (n = 86) was 12.3 years. In the first 5 years post-diagnosis 23% of patients developed complications. The patients in the population-based study (n = 683) were stratified into those diagnosed before the age of 18 years ('paediatric-onset' subcohort, n = 43) and those diagnosed after the age of 18 years ('adult-onset' subcohort, n = 640). Median age at diagnosis was 14.6 and 40.2 years, respectively. Median time-to-complication in the paediatric-onset and adult-onset subcohorts was not statistically different. Conclusion Paediatric and adult-onset SC run a similar long-term disease course. Paediatricians who treat children with SC should monitor them closely to recognize early complications and control long-term sequelae.

KW - disease progression

KW - inflammatory bowel disease

KW - paediatric

KW - prognosis

KW - sclerosing cholangitis

KW - AUTOIMMUNE HEPATITIS

KW - ALKALINE-PHOSPHATASE

KW - NATURAL-HISTORY

KW - CHILDREN

KW - EPIDEMIOLOGY

KW - DIAGNOSIS

KW - RISK

U2 - 10.1111/liv.14159

DO - 10.1111/liv.14159

M3 - Article

VL - 39

SP - 1768

EP - 1775

JO - Liver International

JF - Liver International

SN - 1478-3223

IS - 9

ER -

ID: 98080478