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      Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature

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          Abstract

          Sickle cell disease is an inherited hemoglobinopathy leading to the synthesis of hemoglobin S. Hemoglobin S results in the formation of abnormal sickle-shaped erythrocytes that lead to hematologic abnormalities such as hemolytic anemia and increased risks of thrombosis. Another particular problem encountered with the disease is pulmonary hypertension. The objective of this narrative review is to discuss the prevalence, pathophysiology mechanisms, diagnostic techniques, treatment options, and prognostic indicators in the setting of sickle cell disease with pulmonary hypertension. Additionally, the review also highlights other advancements that are being investigated. Considering the significant morbidity, mortality, and prevalence of pulmonary hypertension in patients with sickle cell disease, it is important to account for the aforementioned domains in the future guidelines to provide optimal and individualized care to the high-risk individuals as well as reduce the progression of disease, morbidity, and mortality rates.

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          Most cited references32

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          Sickle cell disease

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            Sickle-cell disease

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              Intravascular hemolysis and the pathophysiology of sickle cell disease

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                Author and article information

                Journal
                PLS
                PLS
                10.1159/issn.2235-8668
                Pulse
                S. Karger AG
                2235-8676
                2235-8668
                2021
                December 2021
                23 September 2021
                : 9
                : 3-4
                : 57-63
                Affiliations
                [_a] aDepartment of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
                [_b] bDepartment of Internal Medicine, Allegheny Health Network, Pittsburgh, Pennsylvania, USA
                [_c] cDivision of Cardiology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
                [_d] dShifa College of Medicine, Shifa Tameer-e-Millat University, Islamabad, Pakistan
                [_e] eDivision of Hospital Medicine, Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA
                Article
                519101 Pulse 2021;9:57–63
                10.1159/000519101
                35083171
                a4c4b548-74db-44fb-b591-168e1af3495e
                © 2021 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 20 June 2021
                : 27 July 2021
                Page count
                Figures: 2, Tables: 1, Pages: 7
                Categories
                Review Article

                General medicine,Neurology,Cardiovascular Medicine,Internal medicine,Nephrology
                Pulmonary hypertension,Diagnosis,Management,Sickle cell disease,Pathophysiology

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