Background: Limited data exists regarding impact of graft type on outcomes following liver transplantation (LT) in Primary Sclerosing Cholangitis (PSC). Our goal was to evaluate the impact of graft type on outcomes following LT in PSC and determine predictors of outcomes.
Methods: Using the Scientific registry of transplant recipients (SRTR), retrospective cohorts were constructed of recipients with PSC over the time period 2010-2020, divided into 2 eras: 2010-2014, 2015-2020, stratified by graft type: living donor (LDLT), donation after circulatory death (DCD) and donation after brain death (DBD). Outcome measures evaluated were graft and patient survival. Survival comparison was performed using Kaplan-Meier method and multivariable analysis using Cox proportional hazard models.
Results: 2966 recipients underwent LT for PSC over the study period: LDLT-PSC 153 (5.2%), DCD-PSC 131 (4.4%) and DBD-PSC 2682 (90.4%). While LDLT utilization was higher in PSC (5.2% vs. 1.3%; p < 0.001), DCD use was lower (4.4% vs. 7.2%; p < 0.001) but increased over time (era 1 vs. era 2: 3.3% vs. 5.2%; p = 0.02). Outcomes following DCD-PSC were comparable to DBD and improved over time. Compared to DBD-PSC, there was a trend toward lower short-term graft survival following LDLT-PSC (1 Yr. 85.3 vs. 91.9; p = 0.07) with higher retransplant rate (LDLT-PSC vs. DCD-PSC vs. DBD-PSC: 15% vs 11% vs 7%; p < 0.001). Compared to recipients without PSC, long-term patient survival was superior in LDLT-PSC (5 Yr. 90.1 vs. 83.7%; p = 0.05) and DCD-PSC (93.3 vs. 79.7%, p = 0.01). On multivariable analysis, LDLT but not DCD graft type, was associated with inferior graft survival in PSC (adjusted hazard Ratio = 1.65 (1.16-2.34); p = 0.005).
Conclusions: In PSC, utilization of LDLT is higher, while DCD use is lower but increased over time. Outcomes following DCD LT in PSC are comparable to DBD and superior to recipients without PSC. Reduced graft survival and higher re-transplant rate following LDLT in PSC warrants further study. Consideration of DCD could help expand the donor pool in PSC.
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http://dx.doi.org/10.1007/s12072-024-10733-y | DOI Listing |
Lipids Health Dis
December 2024
Department of Endocrinology, Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, Zhejiang, 310052, China.
Background: Familial hypercholesterolemia (FH) is an inherited disorder mainly marked by increased low-density lipoprotein cholesterol (LDL-C) concentrations and a heightened risk of early-onset arteriosclerotic cardiovascular disease (ASCVD). This study seeks to characterize the genetic spectrum and genotype‒phenotype correlations of FH in Chinese pediatric individuals.
Methods: Data were gathered from individuals diagnosed with FH either clinically or genetically at multiple hospitals across mainland China from January 2016 to June 2024.
Sci Rep
December 2024
Laboratory of Biology, Engineering, and Imaging for Ophthalmology, BiiO, Faculty of Medicine, University of Jean Monnet, 10 rue de la Marandière, 42270, Saint-Priest en Jarez, France.
The cornea, the anterior meniscus-shaped transparent and refractive structure of the eyeball, is the first mechanical barrier of the eye. Its functionality heavily relies on the health of its endothelium, its most posterior layer. The treatment of corneal endothelial cells (CECs) deficiency is allogeneic corneal graft using stored donor corneas.
View Article and Find Full Text PDFNPJ Regen Med
December 2024
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, 100730, Beijing, China.
As an emerging type of pluripotent stem cells, chemically induced pluripotent stem cells (CiPSCs) avoid the risks of genomic disintegration by exogenous DNAs from viruses or plasmids, providing a safer stem cell source. To verify CiPSCs' capacity to differentiate into retinal organoids (ROs), we induced CiPSCs from mouse embryonic fibroblasts by defined small-molecule compounds and successfully differentiated the CiPSCs into three-dimensional ROs, in which all major retinal cell types and retinal genes were in concordance with those in vivo. We transplanted retinal photoreceptors from ROs into the subretinal space of retinal degeneration mouse models and the cells could integrate into the host retina, establish synaptic connections, and significantly improve the visual functions of the murine models.
View Article and Find Full Text PDFJ Pediatr Surg
December 2024
Pediatric Surgery, Dipartimento di Medicina di Precisione e Rigenerativa a Area Jonica, Azienda Ospedaliera-Universitaria Consorziale Ospedale Pediatrico Giovanni XXIII, Bari, Italy.
Background And Aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability.
View Article and Find Full Text PDFTransfus Apher Sci
December 2024
Alexion, AstraZeneca Rare Disease, 121 Seaport Blvd, Boston, MA 02210, USA. Electronic address:
Plasma exchange (PE) outcomes in patients with trigger-associated thrombotic microangiopathy (TMA) have not been comprehensively reviewed. Embase and MEDLINE® were searched on 03/14/2022 for English language articles published after 2007, alongside a congress materials search (2019-2022; PROSPERO: CRD42022325170). Studies with patients with trigger-associated TMA (excluding thrombotic thrombocytopenic purpura, 'typical' hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli, post-partum TMA, and TMAs with known genetic cause) who received PE or plasma infusion (PI) and reported treatment response (including measures), safety, patient-/caregiver-reported outcomes, or economic burden data were examined.
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