Introduction Cardiomyopathy is a common cause of morbidity and death in patients with Duchenne muscular dystrophy (DMD). Methods A cross-sectional analysis of clinical data from a multi-institutional, international CINRG DMD Natural History Study of 340 DMD patients aged 2 to 28 years. Cardiomyopathy was defined as shortening fraction (SF) <28% or ejection fraction (EF) <55%. Results 231 participants reported a prior clinical echocardiogram study, and 174 had data for SF or EF. The prevalence of cardiomyopathy was 27% (47/174), and it was significantly associated with age and clinical stage. The association of cardiomyopathy with age and clinical stage was not changed by glucocorticoid use as a covariate (P>0.68). In patients with cardiomyopathy, 57 % (27/47) reported not taking any cardiac medications. Cardiac medications were used in 12% (15/127) of patients without cardiomyopathy. Discussion Echocardiograms were underutilized, and cardiomyopathy was undertreated in this DMD natural history cohort.
Rationale & Objective Home dialysis has been underused in the United States, especially among minority groups. We investigated whether adjustment for socioeconomic factors would attenuate racial/ethnic differences in the initiation of home dialysis. Study Design Retrospective observational cohort study. Setting & Population Adult patients in the US Renal Data System who initiated dialysis on day 1 with either in-center hemodialysis (HD), home HD (HHD), or peritoneal dialysis (PD) from 2005 to 2013. Predictor Race/ethnicity: non-Hispanic white, Hispanic, black, or Asian. Outcome Initiating dialysis with PD versus in-center HD and HHD versus in-center HD for each minority group compared with non-Hispanic whites. Analytical Approach Odds ratios and 95% CIs estimated by logistic regression. Results Of 523,526 patients, 55% were white, 28% were black, 13% were Hispanic, and 4% were Asian; 8% started dialysis on PD, and 0.1%, on HHD. In unadjusted analyses, blacks and Hispanics were 30% and 19% less likely and Asians were 31% more likely to start on PD than whites. The differences narrowed when fully adjusted for demographic, medical, and socioeconomic factors. Adjustment for socioeconomic factors reduced these differences between white and black, Hispanic, and Asian patients by 13%, 28%, and 1%, respectively. Blacks were just as likely and Hispanics and Asians were less likely to start on HHD than whites. This did not change appreciably when fully adjusted for demographic, medical, and socioeconomic factors. Limitations No data for physician and patient preferences or modality education. Conclusions Black and Hispanic patients are less likely to start on PD than white patients, attributable partly, though not completely, to socioeconomic factors. Hispanics and Asians are less likely to start on HHD than whites. This was materially unaffected by socioeconomic factors. More research is needed to determine whether urgent-start PD programs and transitional care units in socioeconomically disadvantaged areas might reduce these disparities and increase home dialysis use among all groups.
Objectives We sought to characterise the outcomes of patients with haematological malignancy and severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection in hospital in our regional network of 7 hospitals. Methods Consecutive hospitalised patients with haematological malignancy and SARS‐CoV‐2 infection were identified from 01/03/2020 to 06/05/2020. Outcomes were categorised as death, resolved or ongoing. The primary outcome was preliminary case fatality rate (pCFR), defined as the number of cases resulting in death as a proportion of all diagnosed cases. Analysis was primarily descriptive. Results 66 Patients were included, overall pCFR was 51.5%. Patients ≥ 70 years accounted for the majority of hospitalised cases (42, 63%) and fatalities (25, 74%). Mortality was similar between females (52%) and males (51%). Immunosuppressive or cytotoxic treatment within 3 months of the diagnosis of SARS‐CoV‐2 infection was associated with a significantly higher pCFR of 70%, compared with 28% in those not on active treatment (P = .0013, 2 proportions z test). Conclusions Mortality rates in patients with haematological malignancy and SARS‐CoV‐2 infection in hospital are high supporting measures to minimise the risk of infection in this population.
Introduction: Autoantibody to angiotensin II type 1 receptor (AT1R-Ab) has been recognized as a non-human leukocyte antigen (HLA) antibody relevant in transplantation. Endothelin type A receptor antibody (ETAR-Ab) has been strongly associated with AT1R-Ab, but the data in kidney transplantation are scarce. Methods: We examined the relationship of ETAR-Ab and AT1R-Ab with clinical outcomes, biopsy findings, inflammatory cytokines, and HLA donor-specific antibody (DSA) in a cohort of pediatric renal transplant recipients. Sixty-five patients were longitudinally monitored for ETAR-Ab, AT1R-Ab, HLA DSA, interleukin (IL)-8, tumor necrosis factor-a, IL-1b, interferon-g, IL-17, IL-6, renal dysfunction, hypertension, rejection, and allograft loss during the first 2 years post-transplant. Results: Fifteen patients (23%) had AT1R-Ab alone, 1 (2%) had ETAR-Ab alone, 23 (35%) had both ETAR-Ab and AT1R-Ab, and 26 (40%) were negative for both antibodies at all timepoints. Having both ETAR-Ab and AT1R-Ab was associated with >30% decline in estimated glomerular filtration rate (P ¼ 0.024), arteritis (P ¼ 0.016), and elevated IL-8 levels (P ¼ 0.010), but not rejection, HLA DSA, or allograft loss. Having both antibodies resulted in greater increases in IL-8 compared with AT1R-Ab alone, even when controlled for additional clinical factors, including HLA DSA (P ¼ 0.012). Conclusion: Our study demonstrates that, in pediatric kidney transplantation, ETAR-Ab is highly associated with AT1R-Ab, but there are a subset of patients with AT1R-Ab alone. Having both antibodies is significantly associated with arteritis, elevated IL-8, and decline in renal function, and our results suggest possible interaction effects. Better understanding of this interaction may be informative in developing protocols for testing, treatment, and prevention of allograft injury.
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