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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Novel insights in the management of sickle cell disease in childhood</title><author><name sortKey="Iughetti, Lorenzo" sort="Iughetti, Lorenzo" uniqKey="Iughetti L" first="Lorenzo" last="Iughetti">Lorenzo Iughetti</name></author><author><name sortKey="Bigi, Elena" sort="Bigi, Elena" uniqKey="Bigi E" first="Elena" last="Bigi">Elena Bigi</name></author><author><name sortKey="Venturelli, Donatella" sort="Venturelli, Donatella" uniqKey="Venturelli D" first="Donatella" last="Venturelli">Donatella Venturelli</name></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">26862499</idno><idno type="pmc">4737690</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4737690</idno><idno type="RBID">PMC:4737690</idno><idno type="doi">10.5409/wjcp.v5.i1.25</idno><date when="2016">2016</date><idno type="wicri:Area/Pmc/Corpus">000108</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Novel insights in the management of sickle cell disease in childhood</title><author><name sortKey="Iughetti, Lorenzo" sort="Iughetti, Lorenzo" uniqKey="Iughetti L" first="Lorenzo" last="Iughetti">Lorenzo Iughetti</name></author><author><name sortKey="Bigi, Elena" sort="Bigi, Elena" uniqKey="Bigi E" first="Elena" last="Bigi">Elena Bigi</name></author><author><name sortKey="Venturelli, Donatella" sort="Venturelli, Donatella" uniqKey="Venturelli D" first="Donatella" last="Venturelli">Donatella Venturelli</name></author></analytic><series><title level="j">World Journal of Clinical Pediatrics</title><idno type="eISSN">2219-2808</idno><imprint><date when="2016">2016</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Sickle cell disease (SCD) is a life-threatening genetic disorder characterized by chronic hemolytic anemia, vascular injury and multiorgan dysfunctions. Over the last few decades, there have been significant improvements in SCD management in Western countries, especially in pediatric population. An early onset of prophylaxis with Penicillin and a proper treatment of the infections have increased the overall survival in childhood. Nevertheless, management of painful episodes and prevention of organ damage are still challenging and more efforts are needed to better understand the mechanisms behind the development of chronic organ damages. Hydroxyurea (Hydroxycarbamide, HU), the only medication approved as a disease-modifying agent by the United States Food and Drug Administration and the European Medicines Agency, is usually under-used, especially in developing countries. Currently, hematopoietic stem-cell transplantation is considered the only curative option, although its use is limited by lack of donors and transplant-related toxicity. SCD symptoms are similar in children and adults, but complications and systemic organ damages increase with age, leading to early mortality worldwide. Experts in comprehensive care of young patients with SCD, especially those approaching the transition age to adulthood, are missing, leading people to rely on urgent care, increasing health care utilization costs and inappropriate treatments. It would be important to establish programs of comprehensive healthcare for patients with SCD from birth to adulthood, to improve their quality and expectancy of life.</p></div></front></TEI><pmc article-type="review-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">World J Clin Pediatr</journal-id><journal-id journal-id-type="publisher-id">WJCP</journal-id><journal-title-group><journal-title>World Journal of Clinical Pediatrics</journal-title></journal-title-group><issn pub-type="epub">2219-2808</issn><publisher><publisher-name>Baishideng Publishing Group Inc</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">26862499</article-id><article-id pub-id-type="pmc">4737690</article-id><article-id pub-id-type="other">jWJCP.v5.i1.pg25</article-id><article-id pub-id-type="doi">10.5409/wjcp.v5.i1.25</article-id><article-categories><subj-group subj-group-type="heading"><subject>Review</subject></subj-group></article-categories><title-group><article-title>Novel insights in the management of sickle cell disease in childhood</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Iughetti</surname><given-names>Lorenzo</given-names></name></contrib><contrib contrib-type="author"><name><surname>Bigi</surname><given-names>Elena</given-names></name></contrib><contrib contrib-type="author"><name><surname>Venturelli</surname><given-names>Donatella</given-names></name></contrib><aff>Lorenzo Iughetti, Elena Bigi, Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41124 Modena, Italy</aff><aff>Donatella Venturelli, Transfusion Medicine Department, University Hospital of Modena, 41124 Modena, Italy</aff></contrib-group><author-notes><fn><p>Author contributions: Iughetti L and Bigi E wrote the paper; Iughetti L and Venturelli D supervised the manuscript drafting.</p><p>Correspondence to: Lorenzo Iughetti, Associate Professor of Pediatrics, Pediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, via del Pozzo 71, 41124 Modena, Italy. <email>iughetti.lorenzo@unimore.it</email></p><p>Telephone: +39-059-4225382</p></fn></author-notes><pub-date pub-type="collection"><day>8</day><month>2</month><year>2016</year></pub-date><pub-date pub-type="epub"><day>8</day><month>2</month><year>2016</year></pub-date><volume>5</volume><issue>1</issue><fpage>25</fpage><lpage>34</lpage><history><date date-type="received"><day>31</day><month>7</month><year>2015</year></date><date date-type="rev-recd"><day>13</day><month>10</month><year>2015</year></date><date date-type="accepted"><day>18</day><month>12</month><year>2015</year></date></history><permissions><copyright-statement>©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.</copyright-statement><copyright-year>2016</copyright-year></permissions><abstract><p>Sickle cell disease (SCD) is a life-threatening genetic disorder characterized by chronic hemolytic anemia, vascular injury and multiorgan dysfunctions. Over the last few decades, there have been significant improvements in SCD management in Western countries, especially in pediatric population. An early onset of prophylaxis with Penicillin and a proper treatment of the infections have increased the overall survival in childhood. Nevertheless, management of painful episodes and prevention of organ damage are still challenging and more efforts are needed to better understand the mechanisms behind the development of chronic organ damages. Hydroxyurea (Hydroxycarbamide, HU), the only medication approved as a disease-modifying agent by the United States Food and Drug Administration and the European Medicines Agency, is usually under-used, especially in developing countries. Currently, hematopoietic stem-cell transplantation is considered the only curative option, although its use is limited by lack of donors and transplant-related toxicity. SCD symptoms are similar in children and adults, but complications and systemic organ damages increase with age, leading to early mortality worldwide. Experts in comprehensive care of young patients with SCD, especially those approaching the transition age to adulthood, are missing, leading people to rely on urgent care, increasing health care utilization costs and inappropriate treatments. It would be important to establish programs of comprehensive healthcare for patients with SCD from birth to adulthood, to improve their quality and expectancy of life.</p></abstract><kwd-group><kwd>Clinical management</kwd><kwd>Hydroxyurea</kwd><kwd>Sickle cell disease</kwd><kwd>Children</kwd><kwd>Hematopoietic stem-cell transplantation</kwd></kwd-group></article-meta></front></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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