Life-Threatening Infectious Complications in Sickle Cell Disease: A Concise Narrative Review
Identifieur interne : 000115 ( Pmc/Checkpoint ); précédent : 000114; suivant : 000116Life-Threatening Infectious Complications in Sickle Cell Disease: A Concise Narrative Review
Auteurs : Dominik Ochocinski [États-Unis] ; Mansi Dalal [États-Unis] ; L. Vandy Black [États-Unis] ; Silvana Carr [États-Unis] ; Judy Lew [États-Unis] ; Kevin Sullivan [États-Unis] ; Niranjan Kissoon [Canada]Source :
- Frontiers in Pediatrics [ 2296-2360 ] ; 2020.
Abstract
Sickle cell disease (SCD) results in chronic hemolytic anemia, recurrent vascular occlusion, insidious vital organ deterioration, early mortality, and diminished quality of life. Life-threatening acute physiologic crises may occur on a background of progressive diminishing vital organ function. Sickle hemoglobin polymerizes in the deoxygenated state, resulting in erythrocyte membrane deformation, vascular occlusion, and hemolysis. Vascular occlusion and increased blood viscosity results in functional asplenia and immune deficiency in early childhood, resulting in life-long increased susceptibility to serious bacterial infections. Infection remains a main cause of overall mortality in patients with SCD in low- and middle-income countries due to increased exposure to pathogens, increased co-morbidities such as malnutrition, lower vaccination rates, and diminished access to definitive care, including antibiotics and blood. Thus, the greatest gains in preventing infection-associated mortality can be achieved by addressing these factors for SCD patients in austere environments. In contrast, in high-income countries, perinatal diagnosis of SCD, antimicrobial prophylaxis, vaccination, aggressive use of antibiotics for febrile episodes, and the availability of contemporary critical care resources have resulted in a significant reduction in deaths from infection; however, chronic organ injury is problematic. All clinicians, regardless of their discipline, who assume the care of SCD patients must understand the importance of infectious disease as a contributor to death and disability. In this concise narrative review, we summarize the data that describes the importance of infectious diseases as a contributor to death and disability in SCD and discuss pathophysiology, prevalent organisms, prevention, management of acute episodes of critical illness, and ongoing care.
Url:
DOI: 10.3389/fped.2020.00038
PubMed: 32154192
PubMed Central: 7044152
Affiliations:
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<front><div type="abstract" xml:lang="en"><p>Sickle cell disease (SCD) results in chronic hemolytic anemia, recurrent vascular occlusion, insidious vital organ deterioration, early mortality, and diminished quality of life. Life-threatening acute physiologic crises may occur on a background of progressive diminishing vital organ function. Sickle hemoglobin polymerizes in the deoxygenated state, resulting in erythrocyte membrane deformation, vascular occlusion, and hemolysis. Vascular occlusion and increased blood viscosity results in functional asplenia and immune deficiency in early childhood, resulting in life-long increased susceptibility to serious bacterial infections. Infection remains a main cause of overall mortality in patients with SCD in low- and middle-income countries due to increased exposure to pathogens, increased co-morbidities such as malnutrition, lower vaccination rates, and diminished access to definitive care, including antibiotics and blood. Thus, the greatest gains in preventing infection-associated mortality can be achieved by addressing these factors for SCD patients in austere environments. In contrast, in high-income countries, perinatal diagnosis of SCD, antimicrobial prophylaxis, vaccination, aggressive use of antibiotics for febrile episodes, and the availability of contemporary critical care resources have resulted in a significant reduction in deaths from infection; however, chronic organ injury is problematic. All clinicians, regardless of their discipline, who assume the care of SCD patients must understand the importance of infectious disease as a contributor to death and disability. In this concise narrative review, we summarize the data that describes the importance of infectious diseases as a contributor to death and disability in SCD and discuss pathophysiology, prevalent organisms, prevention, management of acute episodes of critical illness, and ongoing care.</p>
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<author><name sortKey="Alvarez, Oa" uniqKey="Alvarez O">OA Alvarez</name>
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<author><name sortKey="Cancado, Rd" uniqKey="Cancado R">RD Cancado</name>
</author>
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<pmc article-type="review-article"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Front Pediatr</journal-id>
<journal-id journal-id-type="iso-abbrev">Front Pediatr</journal-id>
<journal-id journal-id-type="publisher-id">Front. Pediatr.</journal-id>
<journal-title-group><journal-title>Frontiers in Pediatrics</journal-title>
</journal-title-group>
<issn pub-type="epub">2296-2360</issn>
<publisher><publisher-name>Frontiers Media S.A.</publisher-name>
</publisher>
</journal-meta>
<article-meta><article-id pub-id-type="pmid">32154192</article-id>
<article-id pub-id-type="pmc">7044152</article-id>
<article-id pub-id-type="doi">10.3389/fped.2020.00038</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Pediatrics</subject>
<subj-group><subject>Review</subject>
</subj-group>
</subj-group>
</article-categories>
<title-group><article-title>Life-Threatening Infectious Complications in Sickle Cell Disease: A Concise Narrative Review</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Ochocinski</surname>
<given-names>Dominik</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
<uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/904577/overview"></uri>
</contrib>
<contrib contrib-type="author"><name><surname>Dalal</surname>
<given-names>Mansi</given-names>
</name>
<xref ref-type="aff" rid="aff2"><sup>2</sup>
</xref>
<uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/905346/overview"></uri>
</contrib>
<contrib contrib-type="author"><name><surname>Black</surname>
<given-names>L. Vandy</given-names>
</name>
<xref ref-type="aff" rid="aff2"><sup>2</sup>
</xref>
<uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/807492/overview"></uri>
</contrib>
<contrib contrib-type="author"><name><surname>Carr</surname>
<given-names>Silvana</given-names>
</name>
<xref ref-type="aff" rid="aff3"><sup>3</sup>
</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Lew</surname>
<given-names>Judy</given-names>
</name>
<xref ref-type="aff" rid="aff3"><sup>3</sup>
</xref>
<uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/896835/overview"></uri>
</contrib>
<contrib contrib-type="author"><name><surname>Sullivan</surname>
<given-names>Kevin</given-names>
</name>
<xref ref-type="aff" rid="aff1"><sup>1</sup>
</xref>
<xref ref-type="aff" rid="aff4"><sup>4</sup>
</xref>
<xref ref-type="corresp" rid="c001"><sup>*</sup>
</xref>
<uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/806459/overview"></uri>
</contrib>
<contrib contrib-type="author"><name><surname>Kissoon</surname>
<given-names>Niranjan</given-names>
</name>
<xref ref-type="aff" rid="aff5"><sup>5</sup>
</xref>
<uri xlink:type="simple" xlink:href="http://loop.frontiersin.org/people/703263/overview"></uri>
</contrib>
</contrib-group>
<aff id="aff1"><sup>1</sup>
<institution>Department of Anesthesiology, University of Florida</institution>
,<addr-line>Gainesville, FL</addr-line>
,<country>United States</country>
</aff>
<aff id="aff2"><sup>2</sup>
<institution>Division of Pediatric Hematology/Oncology, University of Florida</institution>
,<addr-line>Gainesville, FL</addr-line>
,<country>United States</country>
</aff>
<aff id="aff3"><sup>3</sup>
<institution>Division of Pediatric Infectious Disease, University of Florida</institution>
,<addr-line>Gainesville, FL</addr-line>
,<country>United States</country>
</aff>
<aff id="aff4"><sup>4</sup>
<institution>Congenital Heart Center, University of Florida</institution>
,<addr-line>Gainesville, FL</addr-line>
,<country>United States</country>
</aff>
<aff id="aff5"><sup>5</sup>
<institution>Department of Pediatrics, University of British Columbia and BC Children's Hospital</institution>
,<addr-line>Vancouver, BC</addr-line>
,<country>Canada</country>
</aff>
<author-notes><fn fn-type="edited-by"><p>Edited by: Luregn J. Schlapbach, University of Queensland, Australia</p>
</fn>
<fn fn-type="edited-by"><p>Reviewed by: Milind S. Tullu, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, India; Philipp Agyeman, University of Bern, Switzerland</p>
</fn>
<corresp id="c001">*Correspondence: Kevin Sullivan <email>ksullivan@anest.ufl.edu</email>
</corresp>
<fn fn-type="other" id="fn001"><p>This article was submitted to Pediatric Critical Care, a section of the journal Frontiers in Pediatrics</p>
</fn>
</author-notes>
<pub-date pub-type="epub"><day>20</day>
<month>2</month>
<year>2020</year>
</pub-date>
<pub-date pub-type="collection"><year>2020</year>
</pub-date>
<volume>8</volume>
<elocation-id>38</elocation-id>
<history><date date-type="received"><day>10</day>
<month>9</month>
<year>2019</year>
</date>
<date date-type="accepted"><day>27</day>
<month>1</month>
<year>2020</year>
</date>
</history>
<permissions><copyright-statement>Copyright © 2020 Ochocinski, Dalal, Black, Carr, Lew, Sullivan and Kissoon.</copyright-statement>
<copyright-year>2020</copyright-year>
<copyright-holder>Ochocinski, Dalal, Black, Carr, Lew, Sullivan and Kissoon</copyright-holder>
<license xlink:href="http://creativecommons.org/licenses/by/4.0/"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.</license-p>
</license>
</permissions>
<abstract><p>Sickle cell disease (SCD) results in chronic hemolytic anemia, recurrent vascular occlusion, insidious vital organ deterioration, early mortality, and diminished quality of life. Life-threatening acute physiologic crises may occur on a background of progressive diminishing vital organ function. Sickle hemoglobin polymerizes in the deoxygenated state, resulting in erythrocyte membrane deformation, vascular occlusion, and hemolysis. Vascular occlusion and increased blood viscosity results in functional asplenia and immune deficiency in early childhood, resulting in life-long increased susceptibility to serious bacterial infections. Infection remains a main cause of overall mortality in patients with SCD in low- and middle-income countries due to increased exposure to pathogens, increased co-morbidities such as malnutrition, lower vaccination rates, and diminished access to definitive care, including antibiotics and blood. Thus, the greatest gains in preventing infection-associated mortality can be achieved by addressing these factors for SCD patients in austere environments. In contrast, in high-income countries, perinatal diagnosis of SCD, antimicrobial prophylaxis, vaccination, aggressive use of antibiotics for febrile episodes, and the availability of contemporary critical care resources have resulted in a significant reduction in deaths from infection; however, chronic organ injury is problematic. All clinicians, regardless of their discipline, who assume the care of SCD patients must understand the importance of infectious disease as a contributor to death and disability. In this concise narrative review, we summarize the data that describes the importance of infectious diseases as a contributor to death and disability in SCD and discuss pathophysiology, prevalent organisms, prevention, management of acute episodes of critical illness, and ongoing care.</p>
</abstract>
<kwd-group><kwd>sickle cell disease</kwd>
<kwd>infection</kwd>
<kwd>children</kwd>
<kwd>sepsis</kwd>
<kwd>prophylaxis</kwd>
<kwd>vaccination</kwd>
<kwd>critical care</kwd>
</kwd-group>
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<table-count count="4"></table-count>
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</front>
</pmc>
<affiliations><list><country><li>Canada</li>
<li>États-Unis</li>
</country>
</list>
<tree><country name="États-Unis"><noRegion><name sortKey="Ochocinski, Dominik" sort="Ochocinski, Dominik" uniqKey="Ochocinski D" first="Dominik" last="Ochocinski">Dominik Ochocinski</name>
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<name sortKey="Dalal, Mansi" sort="Dalal, Mansi" uniqKey="Dalal M" first="Mansi" last="Dalal">Mansi Dalal</name>
<name sortKey="Lew, Judy" sort="Lew, Judy" uniqKey="Lew J" first="Judy" last="Lew">Judy Lew</name>
<name sortKey="Sullivan, Kevin" sort="Sullivan, Kevin" uniqKey="Sullivan K" first="Kevin" last="Sullivan">Kevin Sullivan</name>
<name sortKey="Sullivan, Kevin" sort="Sullivan, Kevin" uniqKey="Sullivan K" first="Kevin" last="Sullivan">Kevin Sullivan</name>
</country>
<country name="Canada"><noRegion><name sortKey="Kissoon, Niranjan" sort="Kissoon, Niranjan" uniqKey="Kissoon N" first="Niranjan" last="Kissoon">Niranjan Kissoon</name>
</noRegion>
</country>
</tree>
</affiliations>
</record>
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