Article: Hyperbilirubinemia and cholelithiasis in Chinese patients with hemoglobin H disease

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TitleHyperbilirubinemia and cholelithiasis in Chinese patients with hemoglobin H disease
AuthorsAu, WY1
Cheung, WC1
Hu, WH1
Chan, GCF1
Ha, SY1
Khong, PL1
Ma, SK1
Liang, R1
KeywordsGallstones
Gilbert syndrome
Hemoglobin H disease
Jaundice
Issue Date2005
PublisherSpringer Verlag. The Journal's web site is located at http://link.springer.de/link/service/journals/00277/index.htm
CitationAnnals Of Hematology, 2005, v. 84 n. 10, p. 671-674 [How to Cite?]
DOI: http://dx.doi.org/10.1007/s00277-005-1091-8
AbstractHemoglobin H disease (HbH) is a hemoglobinopathy peculiar to parts of the world with high incidence αthalassemia mutations. Among 90 HbH cases, 50 cases suffered from clinically significant jaundice (bilirubin >30 mmol/l), including 14 with severe jaundice (bilirubin >60 mmol/l). Cholelithiasis was found in 38 cases. The incidence is roughly eight times higher than that in background control population but 50% lower than that in β-thalassemia. The risk of gallstones was related to higher bilirubin levels but not αglobin genotype, sex, ferritin, and hemoglobin levels. Homozygotes or double heterozygotes for Gilbert alleles (17.2%), but not heterozgyotes (42.2%), were found to have a significantly increased risk of gallstones and jaundice. However, common Chinese Gilbert syndrome alleles do not completely explain the variable risks. © Springer-Verlag 2005.
ISSN0939-5555
2011 Impact Factor: 2.615
2011 SCImago Journal Rankings: 0.306
DOIhttp://dx.doi.org/10.1007/s00277-005-1091-8
ISI Accession Number IDWOS:000232340600008
ReferencesReferences in Scopus
DC Field
Value
dc.contributor.authorAu, WY
dc.contributor.authorCheung, WC
dc.contributor.authorHu, WH
dc.contributor.authorChan, GCF
dc.contributor.authorHa, SY
dc.contributor.authorKhong, PL
dc.contributor.authorMa, SK
dc.contributor.authorLiang, R
dc.date.accessioned2012-06-26T06:13:56Z
dc.date.available2012-06-26T06:13:56Z
dc.date.issued2005
dc.description.abstractHemoglobin H disease (HbH) is a hemoglobinopathy peculiar to parts of the world with high incidence αthalassemia mutations. Among 90 HbH cases, 50 cases suffered from clinically significant jaundice (bilirubin >30 mmol/l), including 14 with severe jaundice (bilirubin >60 mmol/l). Cholelithiasis was found in 38 cases. The incidence is roughly eight times higher than that in background control population but 50% lower than that in β-thalassemia. The risk of gallstones was related to higher bilirubin levels but not αglobin genotype, sex, ferritin, and hemoglobin levels. Homozygotes or double heterozygotes for Gilbert alleles (17.2%), but not heterozgyotes (42.2%), were found to have a significantly increased risk of gallstones and jaundice. However, common Chinese Gilbert syndrome alleles do not completely explain the variable risks. © Springer-Verlag 2005.
dc.description.natureLink_to_subscribed_fulltext
dc.identifier.citationAnnals Of Hematology, 2005, v. 84 n. 10, p. 671-674 [How to Cite?]
DOI: http://dx.doi.org/10.1007/s00277-005-1091-8
dc.identifier.doihttp://dx.doi.org/10.1007/s00277-005-1091-8
dc.identifier.epage674
dc.identifier.hkuros111085
dc.identifier.isiWOS:000232340600008
dc.identifier.issn0939-5555
2011 Impact Factor: 2.615
2011 SCImago Journal Rankings: 0.306
dc.identifier.issue10
dc.identifier.pmid16044312
dc.identifier.scopuseid_2-s2.0-27644579930
dc.identifier.spage671
dc.identifier.urihttp://hdl.handle.net/10722/150888
dc.identifier.volume84
dc.languageeng
dc.publisherSpringer Verlag. The Journal's web site is located at http://link.springer.de/link/service/journals/00277/index.htm
dc.publisher.placeGermany
dc.relation.ispartofAnnals of Hematology
dc.relation.referencesReferences in Scopus
dc.subject.meshAlleles
dc.subject.meshAsian Continental Ancestry Group
dc.subject.meshChina
dc.subject.meshCholelithiasis - Blood - Etiology - Genetics
dc.subject.meshFemale
dc.subject.meshGallstones - Etiology - Genetics
dc.subject.meshGenotype
dc.subject.meshGilbert Disease - Blood - Etiology - Genetics
dc.subject.meshHemoglobin H - Analysis - Genetics
dc.subject.meshHemoglobinuria - Blood - Classification - Genetics
dc.subject.meshHeterozygote
dc.subject.meshHumans
dc.subject.meshJaundice - Etiology - Genetics
dc.subject.meshMale
dc.subject.meshRisk Factors
dc.subject.meshSex Factors
dc.subject.meshAlpha-Thalassemia
dc.subjectGallstones
dc.subjectGilbert syndrome
dc.subjectHemoglobin H disease
dc.subjectJaundice
dc.titleHyperbilirubinemia and cholelithiasis in Chinese patients with hemoglobin H disease
dc.typeArticle
Author Affiliations
  1. Queen Mary Hospital Hong Kong