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      Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection

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          Key Points

          • SCD patients with COVID-19 display a broad range of severity, with a higher case fatality than the non-SCD population (10.9% vs 3.3%).

          • Older patients not treated with hydroxyurea with end organ damage who present with acute kidney injury, and elevated LDH and D-dimer level are at higher risk of death.

          Abstract

          We aimed to identify predictors of outcomes and survival in patients living in 4 major metropolitan areas who had sickle cell disease (SCD) and COVID-19 to inform best approaches to prevention and care. Data were collected at baseline and during the clinical course in SCD patients diagnosed with COVID-19 in four COVID-19 epicenters. Patients were followed up posthospital discharge for up to 3 months. Of sixty-six SCD patients with COVID-19, fifty patients (75%) required hospitalization, and seven died (10.6%). Patients with preexisting kidney disease (chronic kidney disease) were more likely to be hospitalized. The most common presenting symptom was vaso-occlusive pain. Acute chest syndrome occurred in 30 (60%) of the 50 hospitalized patients and in all who died. Older age and histories of pulmonary hypertension, congestive heart failure, chronic kidney disease, and stroke were more prevalent in patients who died, as were higher creatinine, lactate dehydrogenase, and D-dimer levels. Anticoagulation use while inpatient was twice less common in patients who died. All deaths occurred in individuals not taking hydroxyurea or any other SCD-modifying therapy. Patients with SCD and COVID-19 exhibited a broad range of disease severity. We cannot definitively state that the overall mortality is higher in patients with SCD, although our case fatality rate was ∼10% compared with ∼3% in the general population, despite a median age of 34 years. Individuals with SCD aged >50 years, with preexisting cardiopulmonary, renal disease, and/or stroke not receiving hydroxyurea, who present with high serum creatinine, lactate dehydrogenase, and D-dimer levels, are at higher risk of death, irrespective of genotype or sex.

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          Most cited references32

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          A Novel Coronavirus from Patients with Pneumonia in China, 2019

          Summary In December 2019, a cluster of patients with pneumonia of unknown cause was linked to a seafood wholesale market in Wuhan, China. A previously unknown betacoronavirus was discovered through the use of unbiased sequencing in samples from patients with pneumonia. Human airway epithelial cells were used to isolate a novel coronavirus, named 2019-nCoV, which formed a clade within the subgenus sarbecovirus, Orthocoronavirinae subfamily. Different from both MERS-CoV and SARS-CoV, 2019-nCoV is the seventh member of the family of coronaviruses that infect humans. Enhanced surveillance and further investigation are ongoing. (Funded by the National Key Research and Development Program of China and the National Major Project for Control and Prevention of Infectious Disease in China.)
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            First Case of 2019 Novel Coronavirus in the United States

            Summary An outbreak of novel coronavirus (2019-nCoV) that began in Wuhan, China, has spread rapidly, with cases now confirmed in multiple countries. We report the first case of 2019-nCoV infection confirmed in the United States and describe the identification, diagnosis, clinical course, and management of the case, including the patient’s initial mild symptoms at presentation with progression to pneumonia on day 9 of illness. This case highlights the importance of close coordination between clinicians and public health authorities at the local, state, and federal levels, as well as the need for rapid dissemination of clinical information related to the care of patients with this emerging infection.
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              Covid-19 in Critically Ill Patients in the Seattle Region — Case Series

              Abstract Background Community transmission of coronavirus 2019 (Covid-19) was detected in the state of Washington in February 2020. Methods We identified patients from nine Seattle-area hospitals who were admitted to the intensive care unit (ICU) with confirmed infection with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). Clinical data were obtained through review of medical records. The data reported here are those available through March 23, 2020. Each patient had at least 14 days of follow-up. Results We identified 24 patients with confirmed Covid-19. The mean (±SD) age of the patients was 64±18 years, 63% were men, and symptoms began 7±4 days before admission. The most common symptoms were cough and shortness of breath; 50% of patients had fever on admission, and 58% had diabetes mellitus. All the patients were admitted for hypoxemic respiratory failure; 75% (18 patients) needed mechanical ventilation. Most of the patients (17) also had hypotension and needed vasopressors. No patient tested positive for influenza A, influenza B, or other respiratory viruses. Half the patients (12) died between ICU day 1 and day 18, including 4 patients who had a do-not-resuscitate order on admission. Of the 12 surviving patients, 5 were discharged home, 4 were discharged from the ICU but remained in the hospital, and 3 continued to receive mechanical ventilation in the ICU. Conclusions During the first 3 weeks of the Covid-19 outbreak in the Seattle area, the most common reasons for admission to the ICU were hypoxemic respiratory failure leading to mechanical ventilation, hypotension requiring vasopressor treatment, or both. Mortality among these critically ill patients was high. (Funded by the National Institutes of Health.)
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                Author and article information

                Journal
                Blood Adv
                Blood Adv
                bloodoa
                Blood Adv
                Blood Advances
                Blood Advances
                American Society of Hematology (Washington, DC )
                2473-9529
                2473-9537
                12 January 2021
                07 January 2021
                07 January 2021
                : 5
                : 1
                : 207-215
                Affiliations
                [1 ]Division of Hematology, Montefiore Health Systems, Albert Einstein College of Medicine, Bronx, NY;
                [2 ]Division of Hematology/Oncology, Children’s Hospital of Michigan/Central Michigan University, Detroit, MI;
                [3 ]Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA;
                [4 ]Division of Pediatric Hematology, Montefiore Health Systems, Albert Einstein College of Medicine, Bronx, NY;
                [5 ]Division of Pediatric Hematology/Oncology, Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY;
                [6 ]Department of Emergency Medicine, Boston University School of Medicine, Boston, MA;
                [7 ]Department of Emergency Medicine, Icahn School of Medicine at Mount Sinai, New York, NY;
                [8 ]Center for Pharmacoepidemiology and Pharmacoeconomic Research, College of Pharmacy, and
                [9 ]Division of Hematology and Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, IL; and
                [10 ]The Pulmonary Center, Boston University School of Medicine, Boston, MA
                Author notes
                [*]

                C.P.M and A.U.Z. contributed equally to this study.

                Author information
                https://orcid.org/0000-0002-7059-2959
                https://orcid.org/0000-0003-4551-661X
                https://orcid.org/0000-0001-7465-0581
                https://orcid.org/0000-0002-7307-3927
                https://orcid.org/0000-0003-4725-7295
                Article
                2020/ADV2020003456
                10.1182/bloodadvances.2020003456
                7802524
                33570644
                7e81a0ed-b849-41b4-ad10-8e6b3f6a49e7
                © 2020 by The American Society of Hematology

                This article is made available via the PMC Open Access Subset for unrestricted reuse and analyses in any form or by any means with acknowledgment of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic or until permissions are revoked in writing. Upon expiration of these permissions, PMC is granted a perpetual license to make this article available via PMC and Europe PMC, consistent with existing copyright protections.

                History
                : 21 September 2020
                : 25 November 2020
                Page count
                Pages: 9
                Categories
                20
                28
                Clinical Trials and Observations
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