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dc.contributor.authorFelice, Alex E.-
dc.contributor.authorWebber, Brooke B.-
dc.contributor.authorMiller, Augustus-
dc.contributor.authorMayson, Sylvia M.-
dc.contributor.authorHarris, Herman F.-
dc.contributor.authorHenson, Jean B.-
dc.contributor.authorGravely, Marsha E.-
dc.contributor.authorHuisman, Titus Hendrik Jan-
dc.date.accessioned2018-03-16T10:46:30Z-
dc.date.available2018-03-16T10:46:30Z-
dc.date.issued1979-
dc.identifier.citationFelice, A. E., Webber, B., Miller, A., Mayson, S. M., Harris, H. F., Henson, J. B.,...Huisman, T. H. J. (1979). The association of sickle cell anemia with heterozygous and homozygous α‐thalassemia‐2: In vitro HB chain synthesis. American Journal of Hematology, 6(2), 91-106.en_GB
dc.identifier.urihttps://www.um.edu.mt/library/oar//handle/123456789/28011-
dc.descriptionThe authors are greatly indebted to the parents and the patients who consented to participate in this study.en_GB
dc.description.abstractThe in vitro synthesis of hemoglobin chains was investigated in 34 scikle cell anemia (SS) patients and five patients with Hb S‐βo‐thalassemia. Incubations were made for 30 minutes and for 120 minutes. Hematological and family data were also obtained. Although the 30‐minute α/non‐α total activity ratios were more widely distributed than the ratios at 120 minutes, a distinct classification of SS patients into groups without an α‐thalassemia, with a heterozygous α‐thal‐2, or with a homozygous α‐thal‐2 could not be made. Family studies indicated that four patients who had 30‐minute α/non‐α ratios below 0.82 and mean corpuscular volume (MCV) values below 70 fl had a homozygosity for both α‐thal‐2 and Hb S. They had mild hematological features of SS disease. Many SS patients with 30‐minute α/non‐α ratios between 0.8 and 1.0 and MCV values above 70 fl had an associated α‐thal‐2 heterozygosity. Their hematological features were similar to those of SS patients with four active α chain genes. It appears that an α‐thal‐2 heterozygosity (−α/αα; βS/βS) does not alter the hematological expression of SS disease. An α‐thal‐2 homozygosity −α/−α;βS/βS results in a microcytosis similar to that seen in Hb S‐βo‐thalassemia patients. The diagnosis of these α chain deficiencies in association with SS disease (by in vitro chain synthesis analyses) leaves several uncertainties.en_GB
dc.description.sponsorshipThis research was supported in part by United States Public Health Service grants HLB-05168 and HLB-15158.en_GB
dc.language.isoenen_GB
dc.publisherWiley Online Libraryen_GB
dc.rightsinfo:eu-repo/semantics/restrictedAccessen_GB
dc.subjectSickle cell anemiaen_GB
dc.subjectHemoglobin -- Synthesisen_GB
dc.subjectFetal hemoglobinen_GB
dc.titleThe association of sickle cell anemia with heterozygous and homozygous α-thalassemia-2 : in vitro HB chain synthesisen_GB
dc.typearticleen_GB
dc.rights.holderThe copyright of this work belongs to the author(s)/publisher. The rights of this work are as defined by the appropriate Copyright Legislation or as modified by any successive legislation. Users may access this work and can make use of the information contained in accordance with the Copyright Legislation provided that the author must be properly acknowledged. Further distribution or reproduction in any format is prohibited without the prior permission of the copyright holder.en_GB
dc.description.reviewedpeer-revieweden_GB
dc.identifier.doi10.1002/ajh.2830060202-
dc.publication.titleAmerican Journal of Hematologyen_GB
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