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Multiomics dissection of human RAG deficiency reveals distinctive patterns of immune dysregulation but a common inflammatory signature.
Sci Immunol
January 2025
Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA.
Human recombination-activating gene (RAG) deficiency can manifest with distinct clinical and immunological phenotypes. By applying a multiomics approach to a large group of -mutated patients, we aimed at characterizing the immunopathology associated with each phenotype. Although defective T and B cell development is common to all phenotypes, patients with hypomorphic variants can generate T and B cells with signatures of immune dysregulation and produce autoantibodies to a broad range of self-antigens, including type I interferons.
View Article and Find Full Text PDFEnhanced Costimulation Blockade With αCD154, αCD2, and αCD28 to Promote Heart Allograft Tolerance in Nonhuman Primates.
Transplantation
January 2025
Department of Surgery, Center for Transplantation Sciences, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Background: Long-term renal allograft acceptance has been achieved in macaques using a transient mixed hematopoetic chimerism protocol, but similar regimens have proven unsuccessful in heart allograft recipients unless a kidney transplant was performed simultaneously. Here, we test whether a modified protocol based on targeting CD154, CD2, and CD28 is sufficient to prolong heart allograft acceptance or promote the expansion of regulatory T cells.
Methods: Eight macaques underwent heterotopic allo-heart transplantation from major histocompatibility complex-mismatched donors.
The most important prognostic factors for predicting major adverse cardiovascular, cerebrovascular, and renal events during 5-year follow-up of patients with chronic kidney disease with or without haemodialysis.
Nucl Med Commun
January 2025
Division of Cardiology, Onishi Hospital, Fujioka, Japan.
Objective: Patients with chronic kidney disease (CKD) have an increased risk of adverse cardio-cerebrovascular events. The purpose of this study is to evaluate the prognostic predictors over 5 years in patients with CKD including haemodialysis.
Methods: In this multicenter, prospective cohort study performed with the Gunma-CKD SPECT Study protocol, 311 patients with CKD [estimated glomerular filtration rate (eGFR) < 60 min/ml/1.
Resolution of hypertension after kidney transplantation is associated with improved kidney transplant outcomes: a nationwide cohort study.
J Hypertens
December 2024
Division of Nephrology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine.
Objectives: Patients with advanced chronic kidney disease suffer from hypertension, and kidney transplantation (KT) has potential to induce hypertension resolution. We hypothesized that hypertension resolution after KT is associated with better KT outcomes.
Methods: We identified KT recipients (2006-2015) who had pretransplant hypertension.
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