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Article: Hydrops fetalis due to an unusual form of Hb H disease

TitleHydrops fetalis due to an unusual form of Hb H disease
Authors
Issue Date1985
PublisherAmerican Society of Hematology. The Journal's web site is located at http://bloodjournal.hematologylibrary.org/
Citation
Blood, 1985, v. 66 n. 1, p. 224-228 How to Cite?
AbstractThe occurrence of Hb H hydrops fetalis is reported for the first time. The mother has ζ-α thalassemia 1 (ζζαα/--) and the father has non-deletion α thalassemia [ζζαα/ζζ(αα)(T)]. The complete deletion of the ζ-α cluster on one chromosome was confirmed by quantitation of α and ζ gene numbers, the normal α and ζ gene patterns arising from the remaining normal chromosome, and the decreased α/β globin chain ratio of 0.57. The non-deletion α thalassemia defect could only be identified by the imbalanced α/β globin chain ratio of 0.65 in the presence of normal gene numbers and patterns. The newborn was markedly anemic, unlike those with classical Hb H disease, because the non-deletion α thalassemia defect is more severe than α thalassemia 2. The decreased ζ genes during fetal life might have additional deleterious effects. In this family, the distinct BamHI restriction fragment length polymorphism in the hypervariable region of the ζ genes may be used for future prenatal diagnosis.
Persistent Identifierhttp://hdl.handle.net/10722/161683
ISSN
2015 Impact Factor: 11.841
2015 SCImago Journal Rankings: 6.395
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorChan, Ven_US
dc.contributor.authorChan, TKen_US
dc.contributor.authorLiang, STen_US
dc.date.accessioned2012-09-05T05:13:52Z-
dc.date.available2012-09-05T05:13:52Z-
dc.date.issued1985en_US
dc.identifier.citationBlood, 1985, v. 66 n. 1, p. 224-228en_US
dc.identifier.issn0006-4971en_US
dc.identifier.urihttp://hdl.handle.net/10722/161683-
dc.description.abstractThe occurrence of Hb H hydrops fetalis is reported for the first time. The mother has ζ-α thalassemia 1 (ζζαα/--) and the father has non-deletion α thalassemia [ζζαα/ζζ(αα)(T)]. The complete deletion of the ζ-α cluster on one chromosome was confirmed by quantitation of α and ζ gene numbers, the normal α and ζ gene patterns arising from the remaining normal chromosome, and the decreased α/β globin chain ratio of 0.57. The non-deletion α thalassemia defect could only be identified by the imbalanced α/β globin chain ratio of 0.65 in the presence of normal gene numbers and patterns. The newborn was markedly anemic, unlike those with classical Hb H disease, because the non-deletion α thalassemia defect is more severe than α thalassemia 2. The decreased ζ genes during fetal life might have additional deleterious effects. In this family, the distinct BamHI restriction fragment length polymorphism in the hypervariable region of the ζ genes may be used for future prenatal diagnosis.en_US
dc.languageengen_US
dc.publisherAmerican Society of Hematology. The Journal's web site is located at http://bloodjournal.hematologylibrary.org/en_US
dc.relation.ispartofBlooden_US
dc.subject.meshDna Restriction Enzymes - Diagnostic Useen_US
dc.subject.meshErythroblastosis, Fetal - Diagnosis - Etiology - Geneticsen_US
dc.subject.meshFemaleen_US
dc.subject.meshGlobins - Geneticsen_US
dc.subject.meshHumansen_US
dc.subject.meshPregnancyen_US
dc.subject.meshPrenatal Diagnosisen_US
dc.subject.meshThalassemia - Complications - Diagnosis - Geneticsen_US
dc.titleHydrops fetalis due to an unusual form of Hb H diseaseen_US
dc.typeArticleen_US
dc.identifier.emailChan, V:vnychana@hkucc.hku.hken_US
dc.identifier.authorityChan, V=rp00320en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid2988669-
dc.identifier.scopuseid_2-s2.0-0021793905en_US
dc.identifier.volume66en_US
dc.identifier.issue1en_US
dc.identifier.spage224en_US
dc.identifier.epage228en_US
dc.identifier.isiWOS:A1985AMF5400033-
dc.publisher.placeUnited Statesen_US
dc.identifier.scopusauthoridChan, V=7202654865en_US
dc.identifier.scopusauthoridChan, TK=7402687762en_US
dc.identifier.scopusauthoridLiang, ST=7402146735en_US

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