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

Autor: Bernadette Vitola, Christine K. Lee, Nanda Kerkar, Trevor J. Laborda, Kathleen M. Loomes, Kaija-Leena Kolho, Saeed Mohammed, Madeleine Aumar, Kathleen B. Schwarz, Alexandre Rodrigues Ferreira, Binita M. Kamath, Bart G. P. Koot, Cara L. Mack, Annemarie Broderick, Venna L. Venkat, Katryn N. Furuya, Mary Elizabeth M. Tessier, Girish S. Rao, Mercedes Martinez, Marek Woynarowski, Eleonora Druve Tavares Fagundes, Atsushi Tanaka, Tamir Miloh, Pamela L. Valentino, Laura G. Draijer, Douglas Mogul, Mark Deneau, Vratislav Smolka, Nitika A. Gupta, Amanda Ricciuto, Emily R. Perito, Melissa Zerofsky, Nadia Ovchinsky, Simon Horslen, Maureen M. Jonas, Kyung Mo Kim
Přispěvatelé: Graduate School, Amsterdam Gastroenterology Endocrinology Metabolism, Amsterdam Reproduction & Development (AR&D), Paediatric Gastroenterology, Children's Hospital, HUS Children and Adolescents, Clinicum
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Graft Rejection
Male
BILIARY RECONSTRUCTION
Internationality
Time Factors
Drug Resistance
Autoimmune hepatitis
030230 surgery
Inflammatory bowel disease
Gastroenterology
DISEASE
0302 clinical medicine
Recurrence
Risk Factors
Interquartile range
Registries
Child
education.field_of_study
Incidence (epidemiology)
Graft Survival
Age Factors
Alanine Transaminase
gamma-Glutamyltransferase
3. Good health
Cohort
Disease Progression
Portal hypertension
Female
030211 gastroenterology & hepatology
medicine.medical_specialty
Adolescent
Cholangitis
Sclerosing
Population
Article
Primary sclerosing cholangitis
03 medical and health sciences
Internal medicine
Hypertension
Portal
medicine
Humans
Aspartate Aminotransferases
education
Glucocorticoids
Hepatology
business.industry
Inflammatory Bowel Diseases
medicine.disease
Liver Transplantation
3121 General medicine
internal medicine and other clinical medicine
AUTOIMMUNE HEPATITIS
RISK-FACTORS
T-CELLS
business
Zdroj: Hepatology, 74(4), 2047-2057. John Wiley and Sons Ltd
Hepatology
ISSN: 0270-9139
Popis: 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.
Databáze: OpenAIRE
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