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Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F61989592%3A15110%2F21%3A73608846" target="_blank" >RIV/61989592:15110/21:73608846 - isvavai.cz</a>

  • Result on the web

    <a href="https://aasldpubs.onlinelibrary.wiley.com/doi/10.1002/hep.31911" target="_blank" >https://aasldpubs.onlinelibrary.wiley.com/doi/10.1002/hep.31911</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1002/hep.31911" target="_blank" >10.1002/hep.31911</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study

  • Original language description

    Background and Aims Recurrent primary sclerosing cholangitis (rPSC) following liver transplant (LT) has a negative impact on graft and patient survival; little is known about risk factors for rPSC or disease course in children. Approach and Results We retrospectively evaluated risk factors for rPSC in 140 children from the Pediatric PSC Consortium, a multicenter international registry. Recipients underwent LT for PSC and had &gt;90 days of follow-up. The primary outcome, rPSC, was defined using Graziadei criteria. Median follow-up after LT was 3 years (interquartile range 1.1-6.1). rPSC occurred in 36 children, representing 10% and 27% of the subjects at 2 years and 5 years following LT, respectively. Subjects with rPSC were younger at LT (12.9 vs. 16.2 years), had faster progression from PSC diagnosis to LT (2.5 vs. 4.1 years), and had higher alanine aminotransferase (112 vs. 66 IU/L) at LT (all P &lt; 0.01). Inflammatory bowel disease was more prevalent in the rPSC group (86% vs. 66%; P = 0.025). After LT, rPSC subjects had more episodes of biopsy-proved acute rejection (mean 3 vs. 1; P &lt; 0.001), and higher prevalence of steroid-refractory rejection (41% vs. 20%; P = 0.04). In those with rPSC, 43% developed complications of portal hypertension, were relisted for LT, or died within 2 years of the diagnosis. Mortality was higher in the rPSC group (11.1% vs. 2.9%; P = 0.05). Conclusions The incidence of rPSC in this cohort was higher than previously reported, and was associated with increased morbidity and mortality. Patients with rPSC appeared to have a more aggressive, immune-reactive phenotype. These findings underscore the need to understand the immune mechanisms of rPSC, to lay the foundation for developing new therapies and improve outcomes in this challenging population.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30219 - Gastroenterology and hepatology

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2021

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Hepatology

  • ISSN

    0270-9139

  • e-ISSN

    1527-3350

  • Volume of the periodical

    74

  • Issue of the periodical within the volume

    4

  • Country of publishing house

    US - UNITED STATES

  • Number of pages

    11

  • Pages from-to

    2047-2057

  • UT code for WoS article

    000694652000001

  • EID of the result in the Scopus database

    2-s2.0-85114458548