Publications by authors named "Michelle O'Shaughnessy"

Article Synopsis
  • The term atypical hemolytic uremic syndrome (aHUS) originated in the 1970s to differentiate between familial/sporadic cases and typical epidemic cases associated with Shiga toxin.
  • Over time, aHUS has become a broad term for various diseases that don't relate to Shiga toxin, complicating the definition and treatment strategies due to its diverse causes.
  • A group of experts used a consensus-building method called the Delphi approach to discuss and clarify the terminology and issues surrounding aHUS in light of advancements in medical science and targeted therapies.
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Article Synopsis
  • Immunosuppressive treatment for rheumatic diseases helps maintain remission but raises infection risk and diminishes response to SARS-CoV-2 vaccination.
  • The study evaluated immune responses to SARS-CoV-2 in two patient groups: one during acute infection and one post-vaccination, comparing results among those exposed to rituximab, other immunosuppressive treatments, and non-immunosuppressed individuals.
  • Results showed that while non-immunosuppressed patients had stronger T cell responses during infection, those on immunosuppression had reduced activation, with rituximab-treated patients showing preserved T cell responses but poor antibody production post-vaccination.
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Background And Hypothesis: Advances in organ procurement, surgical techniques, immunosuppression regimens, and prophylactic antibiotic therapies have dramatically improved kidney transplant graft failure. It is unclear how these interventions have affected longer-term graft failure. It is hypothesized that graft failure has improved over the last 20 years.

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A case report of hemolysis in a female patient with Evans syndrome is described. The patient was admitted with anemia and jaundice and, during her 26-day hospital admission, had 83 samples taken for biochemistry analyses. The laboratory hemolytic index (HI) was frequently elevated due to persistent complement-mediated hemolysis despite multiple lines of therapy.

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Introduction: Edema is a common manifestation of proteinuric kidney diseases, but there is no consensus approach for reliably evaluating edema. The objective of this study was to develop an edema clinician-reported outcome measure for use in patients with nephrotic syndrome.

Methods: A literature review was conducted to assess existing clinician-rated measures of edema.

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Rationale & Objective: Membranoproliferative glomerulonephritis (MPGN), encompassing several distinct diseases, is a rare but significant cause of kidney failure in the United States. The potential etiologies of MPGN are unclear, but prior studies have suggested dysregulation of the alternative complement pathway and, recently, autoimmunity as potential mechanisms driving MPGN pathogenesis. In this study, we examined HLA associations with end-stage kidney disease (ESKD) due to MPGN and dense deposit disease (DDD) in a large racially and ethnically diverse US-based cohort.

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Immunoglobulin A nephropathy (IgAN) is the most common primary form of glomerular disease worldwide and carries a high lifetime risk of kidney failure. The underlying pathogenesis of IgAN has been characterized to a sub-molecular level; immune complexes containing specific O-glycoforms of IgA1 are central. Kidney biopsy remains the gold-standard diagnostic test for IgAN and histological features (i.

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Introduction: Preeclampsia increases the risk for future chronic kidney disease (CKD). Among those diagnosed with CKD, it is unclear whether a prior history of preeclampsia, or other complications in pregnancy, negatively impact kidney disease progression. In this longitudinal analysis, we assessed kidney disease progression among women with glomerular disease with and without a history of a complicated pregnancy.

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Background: Tobacco exposure has been recognized as a risk factor for cardiovascular disease (CVD) and progression of kidney disease. Patients with proteinuric glomerulopathies are at increased risk for cardiovascular morbidity and mortality. Multiple studies have linked tobacco exposure to CVD and chronic kidney disease, but the relationships between smoking and proteinuric glomerulopathies in adults and children have not been previously explored.

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The diagnosis of nephrotic syndrome relies on clinical presentation and descriptive patterns of injury on kidney biopsies, but not specific to underlying pathobiology. Consequently, there are variable rates of progression and response to therapy within diagnoses. Here, an unbiased transcriptomic-driven approach was used to identify molecular pathways which are shared by subgroups of patients with either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS).

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Background: Whether fracture rates, overall and by fracture site, vary by cause of kidney failure in patients receiving dialysis is unknown.

Methods: Using the US Renal Data System, we compared fracture rates across seven causes of kidney failure in patients who started dialysis between 1997 and 2014. We computed unadjusted and multivariable adjusted proportional sub-distribution hazard models, with fracture events (overall, and by site) as the outcome and immunoglobulin A nephropathy as the reference group.

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Rationale & Objective: Infections cause morbidity and mortality in patients with glomerular disease. The relative contributions from immunosuppression exposure and glomerular disease activity to infection risk are not well characterized. To address this unmet need, we characterized the relationship between time-varying combinations of immunosuppressant exposure and infection-related acute care events while controlling for disease activity, among individuals with glomerular disease.

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Purpose Of Review: Rituximab is increasingly prescribed for glomerular diseases. However, the recently published Kidney Disease Improving Global Outcomes (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases lacks details on recommended dosing regimens for most individual glomerular diseases. We performed this scoping review summarizing the evidence for rituximab dosing in glomerular disease.

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Article Synopsis
  • The study investigates the impact of race, ethnicity, and socioeconomic status on acute care usage among patients with glomerular disease, noting gaps in existing knowledge.
  • Conducted as a prospective cohort study, it analyzed data from 1,456 adults and 768 children with confirmed glomerular disease in the CureGN cohort.
  • Results showed that Black and Hispanic individuals experienced higher acute care utilization rates compared to White and Asian participants, while socioeconomic factors were also linked to disease severity, though some findings varied after adjusting for multiple factors.
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This article contains the largest analysis of pRBC transfusion and renal angiogram in inpatient pediatric patients.We provide accurate estimates of rates of pRBC transfusion and renal angiography after kidney biopsy in inpatient pediatric patients.

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The field of rheumatology has advanced significantly in recent years to provide rheumatologists with an extensive array of medications to combat rheumatic joint conditions. In contrast to an older era, when NSAIDs and other nephrotoxic agents were the mainstay of treatment, modern DMARDs vary considerably in their nephrotoxic potential and their use is not always precluded in populations with pre-existing chronic kidney disease (CKD). This review will explore in detail the safety and efficacy profiles of medications used to treat rheumatologic disease, specifically in the setting of CKD.

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Registries are essential for health infrastructure planning, benchmarking, continuous quality improvement, hypothesis generation, and real-world trials. To date, data from these registries have predominantly been analyzed in isolated "silos," hampering efforts to analyze "big data" at the international level, an approach that provides wide-ranging benefits, including enhanced statistical power, an ability to conduct international comparisons, and greater capacity to study rare diseases. This review serves as a valuable resource to clinicians, researchers, and policymakers, by comprehensively describing kidney failure registries active in 2021, before proposing approaches for inter-registry research under current conditions, as well as solutions to enhance global capacity for data collaboration.

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Article Synopsis
  • The study aimed to investigate how the cause and severity of chronic kidney disease (CKD) affect pregnancy outcomes, particularly in women with CKD compared to those without.
  • A systematic search across databases like PubMed and Embase identified 31 relevant studies out of 4,076 citations, focusing on outcomes such as preeclampsia, cesarean delivery, and preterm birth.
  • Results showed that CKD was linked to significantly increased odds of preeclampsia, cesarean delivery, preterm birth, and having babies small for their gestational age, while the association with stillbirth was not significant.
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Article Synopsis
  • The study investigates the influence of race/ethnicity, socioeconomic status, and disease severity on health-related quality of life (HRQOL) in patients with glomerular disease, focusing on an ethnically diverse cohort.
  • Results showed that Black and Hispanic participants experienced worse socioeconomic conditions and more severe disease compared to White or Asian participants, impacting their HRQOL.
  • After adjusting for socioeconomic factors and disease severity, the differences in HRQOL among racial groups in adults diminished, while no significant racial/ethnic differences were found in children.
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We describe antiglomerular basement membrane (anti-GBM) disease with rapidly progressive glomerulonephritis and concurrent parainfluenza pneumonia. Circulating anti-GBM antibodies were barely detectable and disappeared rapidly following corticosteroids, cyclophosphamide and plasma exchange. Kidney biopsy demonstrated strong linear GBM staining for IgG and IgG4 and unusually prominent endocapillary hypercellularity, suggesting 'atypical anti-GBM disease', although glomerular necrosis and crescents were also seen.

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Introduction: Patients with chronic health conditions, particularly chronic kidney disease, are at heightened risk for psychiatric disorders; yet, there are limited data on those with primary glomerular disease.

Methods: This study included patients with glomerular disease enrolled in the kidney research network multisite patient registry. Registry data include encounter, diagnoses, medication, laboratory, and vital signs data extracted from participants' electronic health records.

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