Backround-Physicians reverse patients' warfarin anticoagulation with frozen plasma or prothrombin complex concentrate.Our objective was to determine adverse event frequency after urgent reversal with frozen plasma versus the prothrombin complex concentrate Octaplex. Methods and Results-This natural before-after retrospective cohort study in 2 tertiary care emergency departments compared anticoagulation reversal with frozen plasma (September 2006-August 2008 and with Octaplex (September 2008-August 2010, without other system changes. We included adult patients on warfarin with an international normalized ratio ≥1.5 who received frozen plasma or Octaplex. Our primary outcome was serious adverse events (death, ischemic stroke, myocardial infarction, heart failure, venous thromboembolism, or peripheral arterial thromboembolism) within 7 days. Secondary outcomes included time to international normalized ratio reversal, hospital length of stay, and red blood cells transfused within 48 hours. We included 149 patients receiving frozen plasma and 165 receiving Octaplex. The incidence of serious adverse events for the frozen plasma group was 19.5% compared with 9.7% for the Octaplex group (P=0.014; relative risk, 2.0; 95% confidence interval, 1.1-3.5). This remained significant after adjustment for baseline history and reason for treatment (P=0.038; adjusted relative risk, 1.85; 95% confidence interval, 1.03-3.3) in multivariable regression analysis. Median international normalized ratio reversal was 11.8 hours with frozen plasma and 5.7 hours with Octaplex (P<0.0001). Mean red cell transfusion was 3.2 with frozen plasma and 1.4 with Octaplex (P<0.0001). Conclusions-Octaplex Hickey et al Complications From Octaplex Versus FFP 361The objective of this study is to compare the efficacy and safety of frozen plasma with that of Octaplex, the available 4-factor prothrombin complex concentrate, in our emergency departments (EDs) during the study period. Specifically, we examine adverse effects, time to INR reversal, hospital length of stay, and red cell transfusion requirements. Methods Design and SettingThis retrospective cohort study was conducted using health records of patients who were treated with frozen plasma or Octaplex for emergency reversal of warfarin anticoagulation therapy in 2 tertiary EDs at our institution, which see a total of ≈120 000 ED visits per year. We compared patients who received frozen plasma over a 2-year period before the introduction of Octaplex in September 2008 with those who received Octaplex over an equivalent time period after September 2008. The treatments for both groups were given or started in the ED. The dose of Octaplex administered during the study period was governed by the department of Transfusion Medicine. A standard dose of 1500 IU was administered to patients with intracranial hemorrhage, and a dose of 1000 IU was used for all other patients. This study was approved by our institution's research ethics board. Study PopulationWe included patients who were ≥18 years of age, were ...

The incidence of inflammatory bowel disease (IBD) is rising worldwide, with a particularly sharp increase in children. Rates are highest in North America and Europe, with rapid increases noted in developing nations adopting the Westernized environment. While many genetic risk loci have been identified that predispose people to IBD, incomplete penetrance and overlapping genotypes among patients with different phenotypes inadequately explain the etiology of these chronic diseases. Therefore, environmental risk factors have been the subject of much recent research. This article reviews the role of the environment in IBD, with particular focus on early-life exposures and pediatric-onset disease. The literature surrounding environmental risk factors is reviewed, including prenatal and perinatal exposures, the hygiene hypothesis, the urban environment, infection and antibiotic use, and secondhand tobacco smoke exposure. In addition, the possible role of the environment in altering the intestinal microbiome is addressed.

Background: Chronic mucocutaneous candidiasis (CMCC) has traditionally encompassed endocrinopathy, autoimmunity, and infection of the skin, nails, oral and genital mucosa. It is typically caused by Candida albicans, an organism that is found to be commensal in healthy individuals. To date, most patients with CMCC have mutations in AIRE or STAT1. While chronic Candida spp. infection is a feature of multiple profound T cell deficiencies, it has also been identified in rare cases involving selective immune defects, including interleukin-17 receptor A (IL-17RA) deficiency. An association between Staphylococcus aureus infections and candidiasis due to IL-17RA deficiency has recently been proposed.Aim: We sought to identify the genetic defect in a patient presenting with recurrent oral thrush and S. aureus infections, but otherwise unremarkable immune workup.Methods: Whole exome sequencing and Sanger confirmation was performed, and protein expression analysis utilized to assess the impact of the genetic aberration. A comprehensive immune workup was completed to characterize any possible deficits in his immune system.Results: Next generation sequencing techniques identified a homozygous mutation in IL17RA, c.1696insAG, resulting in the frameshift mutation p.Q566fs. Western blot analysis confirmed the loss of IL-17RA expression. Conclusion:We describe here a novel frameshift mutation in IL17RA. Clinically, the patient was a diagnostic challenge as he did not present with a classic CMCC phenotype. This case emphasizes the importance of genetic analysis in patients presenting with recurrent infections. Statement of novelty:We identify a novel frameshift mutation in IL17RA in a patient presenting with recurrent bacterial and fungal mucocutaneous infections.