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

Maria E. Joosse, Sjoukje M. Haisma, Marlou F. M. Sterk, Kim N. van Munster, Cyriel I. J. Ponsioen, Roderick H. J. Houwen, Bart G. P. Koot, Tim de Meij, Patrick F. van Rheenen, Barbara A. E. de Koning

Research output: Contribution to journalArticleAcademicpeer-review

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.
Original languageEnglish
JournalLiver International
DOIs
Publication statusPublished - 2019

Cite this

Joosse, M. E., Haisma, S. M., Sterk, M. F. M., van Munster, K. N., Ponsioen, C. I. J., Houwen, R. H. J., ... de Koning, B. A. E. (2019). Disease progression in paediatric- and adult-onset sclerosing cholangitis: Results from two independent Dutch registries. Liver International. https://doi.org/10.1111/liv.14159
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.
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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.",
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",
doi = "10.1111/liv.14159",
language = "English",
journal = "Liver International",
issn = "1478-3223",
publisher = "Wiley-Blackwell",

}

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, 2019.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

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

Y1 - 2019

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.

UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85068175111&origin=inward

UR - https://www.ncbi.nlm.nih.gov/pubmed/31152478

U2 - 10.1111/liv.14159

DO - 10.1111/liv.14159

M3 - Article

JO - Liver International

JF - Liver International

SN - 1478-3223

ER -