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Article: Glucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.

TitleGlucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.
Authors
Issue Date2009
PublisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.html
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 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.
Persistent Identifierhttp://hdl.handle.net/10722/148609
ISSN
2014 Impact Factor: 0.872
2014 SCImago Journal Rankings: 0.237

 

DC FieldValueLanguage
dc.contributor.authorAu, WYen_US
dc.contributor.authorSo, JCen_US
dc.contributor.authorMa, SKen_US
dc.contributor.authorLie, AKen_US
dc.date.accessioned2012-05-29T06:14:05Z-
dc.date.available2012-05-29T06:14:05Z-
dc.date.issued2009en_US
dc.identifier.citationHong Kong Medical Journal = Xianggang Yi Xue Za Zhi / Hong Kong Academy Of Medicine, 2009, v. 15 n. 3 Suppl 3, p. 35-38en_US
dc.identifier.issn1024-2708en_US
dc.identifier.urihttp://hdl.handle.net/10722/148609-
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.en_US
dc.languageengen_US
dc.publisherHong Kong Medical Association. The Journal's web site is located at http://www.hkmj.org/resources/supp.htmlen_US
dc.relation.ispartofHong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicineen_US
dc.rightsHong Kong Medical Journal. Copyright © Hong Kong Medical Association.-
dc.subject.meshChina - Epidemiologyen_US
dc.subject.meshDonor Selectionen_US
dc.subject.meshFemaleen_US
dc.subject.meshGlucosephosphate Dehydrogenase Deficiency - Ethnology - Geneticsen_US
dc.subject.meshHematopoietic Stem Cell Transplantation - Ethnologyen_US
dc.subject.meshHematopoietic Stem Cells - Enzymologyen_US
dc.subject.meshHumansen_US
dc.subject.meshMaleen_US
dc.subject.meshPolymorphism, Single Nucleotide - Geneticsen_US
dc.subject.meshPrevalenceen_US
dc.titleGlucose-6-phosphate-dehydrogenase deficiency and haematopoietic stem cell transplantation in Chinese patients.en_US
dc.typeArticleen_US
dc.identifier.emailSo, JC:scc@pathology.hku.hken_US
dc.identifier.authoritySo, JC=rp00391en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid19494395en_US
dc.identifier.scopuseid_2-s2.0-68549135107en_US
dc.identifier.hkuros159744-
dc.identifier.volume15en_US
dc.identifier.issue3 Suppl 3en_US
dc.identifier.spage35en_US
dc.identifier.epage38en_US
dc.publisher.placeHong Kongen_US

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