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Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease

Sickle cell disease is one of the most common inherited hemoglobinopathies diagnosed in the United States. Patients often present with severe anemia, pain crises, infections, and vaso-occlusive phenomena. Complications of these disorders can lead to significant debilitating morbidity and mortality....

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Autores principales: Oyedeji, Oluwayomi, Anusim, Nwabundo, Alkhoujah, Mohammad, Dabak, Vrushali, Otrock, Zaher K
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9559515/
https://www.ncbi.nlm.nih.gov/pubmed/36258991
http://dx.doi.org/10.7759/cureus.29111
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author Oyedeji, Oluwayomi
Anusim, Nwabundo
Alkhoujah, Mohammad
Dabak, Vrushali
Otrock, Zaher K
author_facet Oyedeji, Oluwayomi
Anusim, Nwabundo
Alkhoujah, Mohammad
Dabak, Vrushali
Otrock, Zaher K
author_sort Oyedeji, Oluwayomi
collection PubMed
description Sickle cell disease is one of the most common inherited hemoglobinopathies diagnosed in the United States. Patients often present with severe anemia, pain crises, infections, and vaso-occlusive phenomena. Complications of these disorders can lead to significant debilitating morbidity and mortality. Fat embolism syndrome (FES) is a rare and devastating complication of sickle cell disease. It usually presents with a rapidly deteriorating clinical course, and the prognosis is dismal. We report a case of FES in a 19-year-old African American male with a history of sickle cell disease who presented with tonic-clonic seizures and was found to have multi-organ failure. FES was diagnosed 20 days from a presentation based on blood cytopenias and magnetic resonance imaging findings that were obscured at the initial presentation. We describe in this report, the patient’s course from presentation until diagnosis and resolution. Our case is peculiar as the patient had a very good outcome without the need for red blood cell (RBC) exchange; instead, supportive treatment and simple RBC transfusions were enough to change the clinical course of this almost fatal syndrome.
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spelling pubmed-95595152022-10-17 Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease Oyedeji, Oluwayomi Anusim, Nwabundo Alkhoujah, Mohammad Dabak, Vrushali Otrock, Zaher K Cureus Internal Medicine Sickle cell disease is one of the most common inherited hemoglobinopathies diagnosed in the United States. Patients often present with severe anemia, pain crises, infections, and vaso-occlusive phenomena. Complications of these disorders can lead to significant debilitating morbidity and mortality. Fat embolism syndrome (FES) is a rare and devastating complication of sickle cell disease. It usually presents with a rapidly deteriorating clinical course, and the prognosis is dismal. We report a case of FES in a 19-year-old African American male with a history of sickle cell disease who presented with tonic-clonic seizures and was found to have multi-organ failure. FES was diagnosed 20 days from a presentation based on blood cytopenias and magnetic resonance imaging findings that were obscured at the initial presentation. We describe in this report, the patient’s course from presentation until diagnosis and resolution. Our case is peculiar as the patient had a very good outcome without the need for red blood cell (RBC) exchange; instead, supportive treatment and simple RBC transfusions were enough to change the clinical course of this almost fatal syndrome. Cureus 2022-09-13 /pmc/articles/PMC9559515/ /pubmed/36258991 http://dx.doi.org/10.7759/cureus.29111 Text en Copyright © 2022, Oyedeji et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Oyedeji, Oluwayomi
Anusim, Nwabundo
Alkhoujah, Mohammad
Dabak, Vrushali
Otrock, Zaher K
Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease
title Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease
title_full Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease
title_fullStr Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease
title_full_unstemmed Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease
title_short Complete Neurologic Recovery of Cerebral Fat Embolism Syndrome in Sickle Cell Disease
title_sort complete neurologic recovery of cerebral fat embolism syndrome in sickle cell disease
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9559515/
https://www.ncbi.nlm.nih.gov/pubmed/36258991
http://dx.doi.org/10.7759/cureus.29111
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