BackgroundAutoimmune neutropenia (AIN) is divided into primary and secondary forms. The former is more prevalent in children and is usually a self-limiting disease. Secondary AIN is more common in adults and often occurs in the setting of another autoimmune disorder or secondary to infections, malignancies or medications. Several viral and bacterial pathogens were described to trigger AIN. Here we report a case of AIN in an adult woman associated with human herpesvirus-6 (HHV-6) infection.Case PresentationWe report a case of AIN in an adult woman associated with HHV-6 infection. The patient presented to the emergency department with fever and painful genital ulcers. Upon arrival, her laboratory workup demonstrated severe neutropenia and elevated inflammatory markers. She was hospitalized and underwent a thorough infectious, hematological, autoimmune and inflammatory workup. Malignancy was also excluded using an advanced whole body radiological scan. Serological tests confirmed the presence of both acute and chronic types of HHV-6 antibodies, at very high titers. Polymerase chain reaction demonstrated a numerous copies of the virus in the patient’s blood. Specific immunofluorescence test confirmed the diagnosis of autoimmune neutropenia.ConclusionSecondary AIN is a rare disease that may affect all range of ages. The adult type is a challenging disorder that has different etiologies and may be triggered by a variable infectious pathogen. The finding of HHV-6 as a possible culprit pathogen may warrant physicians into widening the evaluation and include HHV-6 in the analysis.
BackgroundLeft ventricular assist devices (LVADs) may reverse elevated pulmonary vascular resistance (PVR) which is associated with worse prognosis in heart failure (HF) patients. We aim to describe the temporal changes in hemodynamic parameters before and after LVAD implantation among patients with or without elevated PVR.MethodsHF patients who received continuous-flow LVAD (HeartMate 2&3) at a tertiary medical center and underwent right heart catheterization with PVR reversibility study before and after LVAD surgery. Patients were divided into 3 groups: normal PVR (<4WU); reversible PVR (initial PVR ≥4WU with positive reversibility); and non-reversible (persistent PVR ≥4WU).ResultsOverall, 85 LVAD patients with a mean age of 58 years (IQR 49–64), 65 patients (76%) were male; 60 patients had normal PVR, 20 patients with reversible and 5 patients with non-reversible PVR pre-LVAD. All patients with elevated PVR (≥4WU) had higher pulmonary pressures (PP) and increased trans-pulmonary gradient (TPG) compared to patients with normal PVR (p < 0.05). Patients with non-reversible PVR were more likely to have a significantly lower baseline cardiac output (CO) compared to all other groups (p ≤ 0.02). Hemodynamic parameters and PVR post LVAD were similar in all study groups. Patients with baseline elevated PVR (reversible and non-reversible) demonstrated a significant improvement in PP and TPG compared to patients with normal baseline PVR (p ≤ 0.05). The improvement in CO and PVR post-LVAD in the non-reversible PVR group was significantly greater compared to all other groups (p < 0.01). There were no significant differences between study groups in post LVAD and post heart transplantation course.ConclusionHemodynamic parameters improved after LVAD implantation, regardless of baseline PVR and reversibility, and enabled heart transplantation in patients who were ineligible due to non-reversible elevated PVR. Our findings suggest that mitigation of elevated non-reversible PVR is related to reduction in PP and increase in CO.
Introduction Recent series have demonstrated the benefit of trans-catheter edge-to-edge mitral valve repair (TEER) in acutely ill hospitalised patients with severe mitral regurgitation (MR) and intractable heart failure despite intensive intravenous therapy. We describe our cumulative experience with such patients at the Sheba Medical Centre over a 10 year period. Purpose The purpose of this study was to evaluate the safety and efficacy of TEER in hospitalized patients with acute decompensated heart failure and severe MR that was deemed to play a major role in the patients' deterioration. Methods We included 30 hospitalised patients with intractable heart failure and MR ≥3+ (20 males; mean age 74.2±10.6 years; 10 had cardiogenic shock). MR was primary in 4, secondary in 24 and mixed in 2 patients. Acute results were assessed by echocardiography prior to discharge and safety was evaluated clinically, according to the occurrence of procedure-related adverse events. Mortality data were drawn from the national death registry. Results TEER devices were successfully implanted in 28 patients. Early (POD 1) procedure-failure was noted in one patient due to recurrence of flail. There was no peri-procedural mortality. Two patients were hemodynamically unstable during the procedure. One patient had peri-procedural access site bleeding necessitating blood transfusion. Following intervention, 16 patients required ICU care (mean stay 4.8±2.5 days), shock was recorded in 7 patients, 16 required hemodynamic support, and 8 required invasive ventilation. At 30 days 7 patients had died and an additional 4 died 1 to 6 months following intervention. However, mortality in the remaining patients was low with only 3 additional deaths up to 4 years after the procedure (80% of patients alive at 6 months). MR reduction was achieved in 24 patients (to ≤mild in 10 and to moderate in 11). At 12 months follow up MR severity was mild in 3 (37.5%) and moderate in 5 (62.5%) patients. Only 1 patient reported HF rehospitalisation during the year following the procedure. Conclusion TEER for hospitalised patients with severe MR and intractable heart failure is safe, associated with high early mortality, and good long-term outcome for patients alive 6 months after the procedure. More research is needed to better characterise patients likely to benefit from TEER in this clinical scenario. Funding Acknowledgement Type of funding sources: None.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.