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Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study
M. Martinez, ER. Perito, P. Valentino, CL. Mack, M. Aumar, A. Broderick, LG. Draijer, EDT. Fagundes, KN. Furuya, N. Gupta, S. Horslen, MM. Jonas, BM. Kamath, N. Kerkar, KM. Kim, KL. Kolho, BGP. Koot, TJ. Laborda, CK. Lee, KM. Loomes, T. Miloh, D....
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články, pozorovací studie, Research Support, N.I.H., Extramural, práce podpořená grantem
Grantová podpora
KL2 TR001065
NCATS NIH HHS - United States
UL1 TR001863
NCATS NIH HHS - United States
TL1 TR001066
NCATS NIH HHS - United States
UL1 TR000105
NCATS NIH HHS - United States
UL1 TR001067
NCATS NIH HHS - United States
PubMed
34008252
DOI
10.1002/hep.31911
Knihovny.cz E-zdroje
- MeSH
- alanintransaminasa krev MeSH
- aspartátaminotransferasy krev MeSH
- časové faktory MeSH
- dítě MeSH
- gama-glutamyltransferasa krev MeSH
- glukokortikoidy terapeutické užití MeSH
- idiopatické střevní záněty epidemiologie MeSH
- internacionalita MeSH
- léková rezistence MeSH
- lidé MeSH
- mladiství MeSH
- portální hypertenze epidemiologie patofyziologie MeSH
- přežívání štěpu MeSH
- progrese nemoci MeSH
- recidiva MeSH
- registrace MeSH
- rejekce štěpu farmakoterapie epidemiologie patologie MeSH
- rizikové faktory MeSH
- sklerozující cholangitida krev epidemiologie chirurgie MeSH
- transplantace jater * MeSH
- věkové faktory MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- pozorovací studie MeSH
- práce podpořená grantem MeSH
- Research Support, N.I.H., Extramural MeSH
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 >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 < 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 < 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.
Department of Medicine Teikyo University School of Medicine Tokyo Japan
Department of Pediatrics Amsterdam University Medical Center Amsterdam Amsterdam the Netherlands
Department of Pediatrics Amsterdam University Medical Center Amsterdam the Netherlands
Department of Pediatrics Boston Children's Hospital and Harvard Medical School Boston MA
Department of Pediatrics Boston Children's Hospital Boston MA
Department of Pediatrics Children's Hospital of Philadelphia Philadelphia PA
Department of Pediatrics Children's Hospital of Pittsburgh at UPMC Pittsburgh PA
Department of Pediatrics Columbia University Irving Medical Center New York NY
Department of Pediatrics Emory University School of Medicine Atlanta GA
Department of Pediatrics Faculdade de Medicina da UFMG Belo Horizonte Brazil
Department of Pediatrics Federal University of Minas Gerais Belo Horizonte Brazil
Department of Pediatrics Indiana University Indianapolis IN
Department of Pediatrics Johns Hopkins University Baltimore MD
Department of Pediatrics Lurie Children's Hospital Chicago IL
Department of Pediatrics Mayo Clinic Rochester MN
Department of Pediatrics Medical College of Wisconsin Milwaukee WI
Department of Pediatrics Palacky University Olomouc Czech Republic
Department of Pediatrics Texas Children Hospital Houston TX
Department of Pediatrics University of California San Diego San Diego CA
Department of Pediatrics University of California San Francisco San Francisco CA
Department of Pediatrics University of Colorado School of Medicine Aurora CO
Department of Pediatrics University of Miami Miami FL
Department of Pediatrics University of Rochester Medical Center Rochester NY
Department of Pediatrics University of Toronto Toronto ON Canada
Department of Pediatrics University of Ulsan Seoul South Korea
Department of Pediatrics University of Washington Seattle WA
Department of Pediatrics Yale University School of Medicine New Haven CT
Institute for Translational Research in Inflammation University of Lille CHU Lille Lille France
Citace poskytuje Crossref.org
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