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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
2013 Impact Factor: 2.396
 
DOIhttp://dx.doi.org/10.1007/s00277-005-1091-8
 
ISI Accession Number IDWOS:000232340600008
 
ReferencesReferences in Scopus
 
DC FieldValue
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
2013 Impact Factor: 2.396
 
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
 
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Author Affiliations
  1. Queen Mary Hospital Hong Kong