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Article: Glucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.
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TitleGlucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.
 
AuthorsAu, WY1
So, JC1
Ma, SK2
Lie, AK1
 
Issue Date2009
 
PublisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.html
 
CitationHong Kong Medical Journal = Xianggang Yi Xue Za Zhi / Hong Kong Academy Of Medicine, 2009, v. 15 n. 3 Suppl 3, p. 35-38 [How to Cite?]
 
AbstractDeficiency in glucose-6-phosphate dehydrogenase (G6PD), an X-linked recessive red cell enzymopathy, is endemic in Southern Chinese. Universal screening of newborn is done in Hong Kong, Taiwan and Singapore, among other places. In Hong Kong, 4.8% of males are affected and seven common G6PD alleles account for over 99% of all defects. Male hemizygotes suffer from severe deficiency, while female heterozygotes may also be affected. Deficiency of G6PD may affect haematopoietic stem cell transplantation (HSCT) recipients and donors, before and after HSCT. Female patients with clonal erythropoiesis (eg myelodysplasia/myeloproliferative diseases) will have the male population incidence of G6PD. Quantitative enzyme level screening is prudent for donors and recipients, and should be repeated after engraftment. Cotrimoxazole prophylaxis should be avoided in known male and female carriers, including those with low-normal G6PD enzyme levels. Our experience suggested that G6PD-deficient marrow, stem cell and cord blood donor units have no engraftment problems. Post-engraftment G6PD levels correlate with those in donors. An acquired change in G6PD status may serve as a surrogate marker for engraftment. For female heterozygote donors with normal G6PD levels, skewing of lyonized X-chromosome ratio during engraftment may result in over-expression of the deficient allele. This can result in unexpected significant G6PD deficiency. Hence, a repeat G6PD screening at stable engraftment is recommended, especially before commencement of oxidative medications.
 
ISSN1024-2708
2012 SCImago Journal Rankings: 0.255
 
DC FieldValue
dc.contributor.authorAu, WY
 
dc.contributor.authorSo, JC
 
dc.contributor.authorMa, SK
 
dc.contributor.authorLie, AK
 
dc.date.accessioned2012-05-29T06:14:05Z
 
dc.date.available2012-05-29T06:14:05Z
 
dc.date.issued2009
 
dc.description.abstractDeficiency in glucose-6-phosphate dehydrogenase (G6PD), an X-linked recessive red cell enzymopathy, is endemic in Southern Chinese. Universal screening of newborn is done in Hong Kong, Taiwan and Singapore, among other places. In Hong Kong, 4.8% of males are affected and seven common G6PD alleles account for over 99% of all defects. Male hemizygotes suffer from severe deficiency, while female heterozygotes may also be affected. Deficiency of G6PD may affect haematopoietic stem cell transplantation (HSCT) recipients and donors, before and after HSCT. Female patients with clonal erythropoiesis (eg myelodysplasia/myeloproliferative diseases) will have the male population incidence of G6PD. Quantitative enzyme level screening is prudent for donors and recipients, and should be repeated after engraftment. Cotrimoxazole prophylaxis should be avoided in known male and female carriers, including those with low-normal G6PD enzyme levels. Our experience suggested that G6PD-deficient marrow, stem cell and cord blood donor units have no engraftment problems. Post-engraftment G6PD levels correlate with those in donors. An acquired change in G6PD status may serve as a surrogate marker for engraftment. For female heterozygote donors with normal G6PD levels, skewing of lyonized X-chromosome ratio during engraftment may result in over-expression of the deficient allele. This can result in unexpected significant G6PD deficiency. Hence, a repeat G6PD screening at stable engraftment is recommended, especially before commencement of oxidative medications.
 
dc.description.natureLink_to_subscribed_fulltext
 
dc.identifier.citationHong Kong Medical Journal = Xianggang Yi Xue Za Zhi / Hong Kong Academy Of Medicine, 2009, v. 15 n. 3 Suppl 3, p. 35-38 [How to Cite?]
 
dc.identifier.epage38
 
dc.identifier.hkuros159744
 
dc.identifier.issn1024-2708
2012 SCImago Journal Rankings: 0.255
 
dc.identifier.issue3 Suppl 3
 
dc.identifier.pmid19494395
 
dc.identifier.scopuseid_2-s2.0-68549135107
 
dc.identifier.spage35
 
dc.identifier.urihttp://hdl.handle.net/10722/148609
 
dc.identifier.volume15
 
dc.languageeng
 
dc.publisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.html
 
dc.publisher.placeHong Kong
 
dc.relation.ispartofHong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine
 
dc.rightsHong Kong Medical Journal. Copyright © Hong Kong Medical Association.
 
dc.subject.meshChina - Epidemiology
 
dc.subject.meshDonor Selection
 
dc.subject.meshFemale
 
dc.subject.meshGlucosephosphate Dehydrogenase Deficiency - Ethnology - Genetics
 
dc.subject.meshHematopoietic Stem Cell Transplantation - Ethnology
 
dc.subject.meshHematopoietic Stem Cells - Enzymology
 
dc.subject.meshHumans
 
dc.subject.meshMale
 
dc.subject.meshPolymorphism, Single Nucleotide - Genetics
 
dc.subject.meshPrevalence
 
dc.titleGlucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.
 
dc.typeArticle
 
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<item><contributor.author>Au, WY</contributor.author>
<contributor.author>So, JC</contributor.author>
<contributor.author>Ma, SK</contributor.author>
<contributor.author>Lie, AK</contributor.author>
<date.accessioned>2012-05-29T06:14:05Z</date.accessioned>
<date.available>2012-05-29T06:14:05Z</date.available>
<date.issued>2009</date.issued>
<identifier.citation>Hong Kong Medical Journal = Xianggang Yi Xue Za Zhi / Hong Kong Academy Of Medicine, 2009, v. 15 n. 3 Suppl 3, p. 35-38</identifier.citation>
<identifier.issn>1024-2708</identifier.issn>
<identifier.uri>http://hdl.handle.net/10722/148609</identifier.uri>
<description.abstract>Deficiency in glucose-6-phosphate dehydrogenase (G6PD), an X-linked recessive red cell enzymopathy, is endemic in Southern Chinese. Universal screening of newborn is done in Hong Kong, Taiwan and Singapore, among other places. In Hong Kong, 4.8% of males are affected and seven common G6PD alleles account for over 99% of all defects. Male hemizygotes suffer from severe deficiency, while female heterozygotes may also be affected. Deficiency of G6PD may affect haematopoietic stem cell transplantation (HSCT) recipients and donors, before and after HSCT. Female patients with clonal erythropoiesis (eg myelodysplasia/myeloproliferative diseases) will have the male population incidence of G6PD. Quantitative enzyme level screening is prudent for donors and recipients, and should be repeated after engraftment. Cotrimoxazole prophylaxis should be avoided in known male and female carriers, including those with low-normal G6PD enzyme levels. Our experience suggested that G6PD-deficient marrow, stem cell and cord blood donor units have no engraftment problems. Post-engraftment G6PD levels correlate with those in donors. An acquired change in G6PD status may serve as a surrogate marker for engraftment. For female heterozygote donors with normal G6PD levels, skewing of lyonized X-chromosome ratio during engraftment may result in over-expression of the deficient allele. This can result in unexpected significant G6PD deficiency. Hence, a repeat G6PD screening at stable engraftment is recommended, especially before commencement of oxidative medications.</description.abstract>
<language>eng</language>
<publisher>Hong Kong Medical Association. The Journal&apos;s web site is located at http://www.hkmj.org/resources/supp.html</publisher>
<relation.ispartof>Hong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine</relation.ispartof>
<rights>Hong Kong Medical Journal. Copyright &#169; Hong Kong Medical Association.</rights>
<subject.mesh>China - Epidemiology</subject.mesh>
<subject.mesh>Donor Selection</subject.mesh>
<subject.mesh>Female</subject.mesh>
<subject.mesh>Glucosephosphate Dehydrogenase Deficiency - Ethnology - Genetics</subject.mesh>
<subject.mesh>Hematopoietic Stem Cell Transplantation - Ethnology</subject.mesh>
<subject.mesh>Hematopoietic Stem Cells - Enzymology</subject.mesh>
<subject.mesh>Humans</subject.mesh>
<subject.mesh>Male</subject.mesh>
<subject.mesh>Polymorphism, Single Nucleotide - Genetics</subject.mesh>
<subject.mesh>Prevalence</subject.mesh>
<title>Glucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.</title>
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<identifier.hkuros>159744</identifier.hkuros>
<identifier.volume>15</identifier.volume>
<identifier.issue>3 Suppl 3</identifier.issue>
<identifier.spage>35</identifier.spage>
<identifier.epage>38</identifier.epage>
<publisher.place>Hong Kong</publisher.place>
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Author Affiliations
  1. Queen Mary Hospital Hong Kong
  2. Hong Kong Sanatorium and Hospital