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The effect of HLA genotype on disease onset and severity in CTLA-4 insufficiency.
Front Immunol
January 2025
Institute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Introduction: Human Cytotoxic-T-lymphocyte-antigen-4 (CTLA-4) insufficiency caused by heterozygous germline mutations in is a complex immune dysregulation and immunodeficiency syndrome presenting with reduced penetrance and variable disease expressivity, suggesting the presence of disease modifiers that trigger the disease onset and severity. Various genetic and non-genetic potential triggers have been analyzed in CTLA-4 insufficiency cohorts, however, none of them have revealed a clear association to the disease. Multiple HLA haplotypes have been positively or negatively associated with various autoimmune diseases and inborn errors of immunity (IEI) due to the relevance of MHC in the strength of the T cell responses.
View Article and Find Full Text PDFDamage control resuscitation: how it's done and where we can improve. A view of the Brazilian reality according to trauma professionals.
Rev Col Bras Cir
January 2025
- School of Medical Sciences Orebro university, Department of Surgery - Orebro - OR - Suécia.
Introduction: Hemorrhage is the leading cause of preventable deaths in trauma patients, resulting in 1.5 million deaths annually worldwide. Traditional trauma assessment follows the ABC (airway, breathing, circulation) sequence; evidence suggests the CAB (circulation, airway, breathing) approach to maintain perfusion and prevent hypotension.
View Article and Find Full Text PDFImpact of Massive Transfusion Activation on Time to Delivery of the First Cooler and Patient Survival: A Study of 4,313 Consecutive Activations.
J Am Coll Surg
January 2025
Department of Surgery, McGovern Medical School, Houston, Texas.
Background: In 2012, TQIP guidelines for massive transfusion protocols (MTP) recommended delivery of blood product coolers within 15 minutes. Subsequent work found that every minute delay in cooler arrival was associated with a 5% increased risk of mortality. We sought to assess the impact and sustainability of quality improvement (QI) interventions on time to MTP cooler delivery and their association with trauma patient survival.
View Article and Find Full Text PDFIs pre-hospital haemoglobin an efficient marker of the need for transfusion and haemorrhagic shock in severe trauma patients? A retrospective observational study.
Eur J Trauma Emerg Surg
January 2025
Intensive Care Department, Sainte Anne Military Teaching Hospital, Toulon, France.
Background: Haemorrhagic shock is the leading cause of preventable death among trauma patients. Early detection of severe haemorrhage is essential for initiating timely resuscitation and mobilizing resources for massive transfusion (MT) protocols and damage control procedures. This study aimed to assess the predictive value of prehospital haemoglobin (Hb) levels for the need for transfusion at admission, the presence of haemorrhagic shock (HS), and the necessity for MT or haemostatic surgery.
View Article and Find Full Text PDFHLA class I supertypes and HLA class I alleles influence the outcome after allogeneic hematopoietic stem cell transplant from unrelated matched donor.
Transpl Immunol
December 2024
Tissue Typing Centre, Clinical Department for Transfusion Medicine and Transplantation Biology, University Hospital Centre Zagreb, Croatia.
This retrospective study analyses the impact HLA heterozygosity, supertypes, and alleles have on incidence of graft versus host disease (GvHD), relapse, overall survival (OS), disease-free survival (DFS) and transplant-related mortality (TRM) after HSCT. The study included patients who underwent HSCT, typed at allele resolution level for HLA-A, -B, -C, -DRB1, -DQB1, and -DPB1 loci. The analysis performed on the entire patient cohort (N = 232) showed that HLA-B07 supertype positive patients demonstrated decreased incidence of relapse, better OS and DFS in comparison to those negative for HLA-B07 supertype.
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