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dc.contributor.authorvan Beers, Eduard J.
dc.contributor.authorNewburger, Peter E.
dc.contributor.authorGrace, Rachael F.
dc.date2022-08-11T08:09:51.000
dc.date.accessioned2022-08-23T16:45:58Z
dc.date.available2022-08-23T16:45:58Z
dc.date.issued2018-09-13
dc.date.submitted2018-11-14
dc.identifier.citation<p>Haematologica. 2018 Sep 13. pii: haematol.2018.196295. doi: 10.3324/haematol.2018.196295. [Epub ahead of print] <a href="https://doi.org/10.3324/haematol.2018.196295">Link to article on publisher's site</a></p>
dc.identifier.issn0390-6078 (Linking)
dc.identifier.doi10.3324/haematol.2018.196295
dc.identifier.pmid30213831
dc.identifier.urihttp://hdl.handle.net/20.500.14038/40788
dc.description<p>Full author list omitted for brevity. For the full list of authors, see article.</p>
dc.description.abstractPyruvate kinase (PK) deficiency is the most common red cell glycolytic enzyme defect causing hereditary non-spherocytic hemolytic anemia. Current treatments are mainly supportive and include red cell transfusions and splenectomy.11 Regular red cell transfusions are known to result in iron overload; however, the prevalence and spectrum of transfusion-independent iron overload in the overall PK deficient population has not been well defined. This analysis describes the prevalence and clinical characteristics of iron overload in patients enrolled in the PK Deficiency Natural History Study (NHS) with a focus on those patients who are not regularly transfused.2
dc.language.isoen_US
dc.relation<p><a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&list_uids=30213831&dopt=Abstract">Link to Article in PubMed</a></p>
dc.rightsCopyright © 2018, Ferrata Storti Foundation. Authors' accepted manuscript posted as allowed by the publisher's author rights policy at http://www.haematologica.org/information-for-authors.
dc.subjectChelation
dc.subjectIron Overload
dc.subjectPyruvate Kinase Deficiency
dc.subjectRed Cell Enzyme Abnormalities
dc.subjectSplenectomy
dc.subjectEnzymes and Coenzymes
dc.subjectHematology
dc.subjectHemic and Lymphatic Diseases
dc.subjectInorganic Chemicals
dc.subjectNutritional and Metabolic Diseases
dc.subjectTherapeutics
dc.titlePrevalence and management of iron overload in pyruvate kinase deficiency: report from the Pyruvate Kinase Deficiency Natural History Study
dc.typeAccepted Manuscript
dc.source.journaltitleHaematologica
dc.identifier.legacyfulltexthttps://escholarship.umassmed.edu/cgi/viewcontent.cgi?article=4609&amp;context=oapubs&amp;unstamped=1
dc.identifier.legacycoverpagehttps://escholarship.umassmed.edu/oapubs/3597
dc.identifier.contextkey13312397
refterms.dateFOA2022-08-23T16:45:58Z
html.description.abstract<p>Pyruvate kinase (PK) deficiency is the most common red cell glycolytic enzyme defect causing hereditary non-spherocytic hemolytic anemia. Current treatments are mainly supportive and include red cell transfusions and splenectomy.11 Regular red cell transfusions are known to result in iron overload; however, the prevalence and spectrum of transfusion-independent iron overload in the overall PK deficient population has not been well defined. This analysis describes the prevalence and clinical characteristics of iron overload in patients enrolled in the PK Deficiency Natural History Study (NHS) with a focus on those patients who are not regularly transfused.2</p>
dc.identifier.submissionpathoapubs/3597
dc.contributor.departmentDepartment of Pediatrics, Division of Hematology Oncology


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