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Article: Haemoglobin level, proportion of haemoglobin Bart's and haemoglobin Portland in fetuses affected by homozygous α 0-thalassemia from 12 to 40 weeks' gestation

TitleHaemoglobin level, proportion of haemoglobin Bart's and haemoglobin Portland in fetuses affected by homozygous α 0-thalassemia from 12 to 40 weeks' gestation
Authors
KeywordsFetal blood
Hb Bart's
Homozygous α 0-thalassemia
Prenatal diagnosis
Issue Date2010
PublisherJohn Wiley & Sons Ltd. The Journal's web site is located at http://www3.interscience.wiley.com/cgi-bin/jhome/2252
Citation
Prenatal Diagnosis, 2010, v. 30 n. 12-13, p. 1126-1130 How to Cite?
AbstractObjectives: To determine haematological parameters in fetuses affected by homozygous α 0-thalassemia. Methods: This was a cross-sectional retrospective study reviewing 546 blood samples (268 fetal and 278 neonatal cord) being collected between 1993 and 2006, from 12 weeks' gestation onwards for any indication, including the prenatal diagnosis of homozygous α 0-thalassemia, other haematological disorders, hydrops or aneuploidy. The proportion of haemoglobin (Hb) fractions was determined by electrophoresis of haemolysate on cellulose acetate in all samples. Results: There were significant differences in the haematological parameters between homozygous α 0-thalassemia (n = 183) and control (n = 363) which were either heterozygous α 0-thalassemia (alpha thalassemia trait) or normal. In homozygous α 0-thalassemia, the median Hb level, proportion of Hb Bart's (γ 4) and Hb Portland 1(ζ 2γ 2) were 6.4 g/dL, 77.5% and 22.5%, respectively. While the Hb level and the proportion of Hb Bart's increased significantly with gestation, the proportion of Hb Portland 1 decreased. The Hb level contributed by Hb Portland 1 remained around 1.4 g/dL throughout gestation. The proportion of mild, moderate and severe anaemia in the affected fetuses was 27.5, 32.7 and 39.8%, respectively. There was no significant difference in these proportions across different gestation (P = 0.231). There were no differences in the haematological parameters between hydropic and non-hydropic fetuses. Conclusion: Although the degree of anaemia is mild in around one-quarter of the affected fetuses, the contribution by Hb Portland 1 (ζ 2γ 2) to the Hb level was very low throughout gestation, and the affected fetuses may therefore be at risk for hypoxia. Copyright © 2010 John Wiley & Sons, Ltd.
Persistent Identifierhttp://hdl.handle.net/10722/142387
ISSN
2015 Impact Factor: 3.043
2015 SCImago Journal Rankings: 1.450
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLi, TKTen_HK
dc.contributor.authorLeung, KYen_HK
dc.contributor.authorLam, YHen_HK
dc.contributor.authorTang, MHYen_HK
dc.contributor.authorChan, Ven_HK
dc.date.accessioned2011-10-28T02:44:53Z-
dc.date.available2011-10-28T02:44:53Z-
dc.date.issued2010en_HK
dc.identifier.citationPrenatal Diagnosis, 2010, v. 30 n. 12-13, p. 1126-1130en_HK
dc.identifier.issn0197-3851en_HK
dc.identifier.urihttp://hdl.handle.net/10722/142387-
dc.description.abstractObjectives: To determine haematological parameters in fetuses affected by homozygous α 0-thalassemia. Methods: This was a cross-sectional retrospective study reviewing 546 blood samples (268 fetal and 278 neonatal cord) being collected between 1993 and 2006, from 12 weeks' gestation onwards for any indication, including the prenatal diagnosis of homozygous α 0-thalassemia, other haematological disorders, hydrops or aneuploidy. The proportion of haemoglobin (Hb) fractions was determined by electrophoresis of haemolysate on cellulose acetate in all samples. Results: There were significant differences in the haematological parameters between homozygous α 0-thalassemia (n = 183) and control (n = 363) which were either heterozygous α 0-thalassemia (alpha thalassemia trait) or normal. In homozygous α 0-thalassemia, the median Hb level, proportion of Hb Bart's (γ 4) and Hb Portland 1(ζ 2γ 2) were 6.4 g/dL, 77.5% and 22.5%, respectively. While the Hb level and the proportion of Hb Bart's increased significantly with gestation, the proportion of Hb Portland 1 decreased. The Hb level contributed by Hb Portland 1 remained around 1.4 g/dL throughout gestation. The proportion of mild, moderate and severe anaemia in the affected fetuses was 27.5, 32.7 and 39.8%, respectively. There was no significant difference in these proportions across different gestation (P = 0.231). There were no differences in the haematological parameters between hydropic and non-hydropic fetuses. Conclusion: Although the degree of anaemia is mild in around one-quarter of the affected fetuses, the contribution by Hb Portland 1 (ζ 2γ 2) to the Hb level was very low throughout gestation, and the affected fetuses may therefore be at risk for hypoxia. Copyright © 2010 John Wiley & Sons, Ltd.en_HK
dc.languageengen_US
dc.publisherJohn Wiley & Sons Ltd. The Journal's web site is located at http://www3.interscience.wiley.com/cgi-bin/jhome/2252en_HK
dc.relation.ispartofPrenatal Diagnosisen_HK
dc.rightsPrenatal Diagnosis. Copyright © John Wiley & Sons Ltd.en_US
dc.subjectFetal blooden_HK
dc.subjectHb Bart'sen_HK
dc.subjectHomozygous α 0-thalassemiaen_HK
dc.subjectPrenatal diagnosisen_HK
dc.subject.meshFetal Blood - chemistry - metabolismen_US
dc.subject.meshHemoglobins - analysis - metabolismen_US
dc.subject.meshHemoglobins, Abnormal - analysis - metabolismen_US
dc.subject.meshPregnancy - blooden_US
dc.subject.meshalpha-Thalassemia - blood - epidemiology - genetics - metabolismen_US
dc.titleHaemoglobin level, proportion of haemoglobin Bart's and haemoglobin Portland in fetuses affected by homozygous α 0-thalassemia from 12 to 40 weeks' gestationen_HK
dc.typeArticleen_HK
dc.identifier.emailTang, MHY: mhytang@hkucc.hku.hken_HK
dc.identifier.emailChan, V: vnychana@hkucc.hku.hken_HK
dc.identifier.authorityTang, MHY=rp01701en_HK
dc.identifier.authorityChan, V=rp00320en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1002/pd.2619en_HK
dc.identifier.pmid20925047-
dc.identifier.scopuseid_2-s2.0-78649664762en_HK
dc.identifier.hkuros196649en_US
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-78649664762&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume30en_HK
dc.identifier.issue12-13en_HK
dc.identifier.spage1126en_HK
dc.identifier.epage1130en_HK
dc.identifier.isiWOS:000285659500003-
dc.publisher.placeUnited Kingdomen_HK
dc.identifier.scopusauthoridLi, TKT=36480268200en_HK
dc.identifier.scopusauthoridLeung, KY=8247106900en_HK
dc.identifier.scopusauthoridLam, YH=7202563903en_HK
dc.identifier.scopusauthoridTang, MHY=35362943900en_HK
dc.identifier.scopusauthoridChan, V=7202654865en_HK

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