| CASE REPORT |
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Year : 2010 | Volume : 30 | Issue : 2 | Page : 153-155 |
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Homozygosity for the Mediterranean a-thalassemic deletion (hemoglobin Barts hydrops fetalis)
Nasir A.S Al-Allawi1, Maida Y Shamdeen2, Najeeb S Rasheed3
1 Department of Pathology, College of Medicine, University of Dohuk, Dohuk, Iraq
2 Department of Gynecology and Obstetrics, College of Medicine, University of Dohuk, Dohuk, Iraq
3 Department of Hematology, Azadi Teaching Hospital, Dohuk, Iraq
Correspondence Address:
Nasir A.S Al-Allawi
Department of Pathology, College of Medicine, University of Dohuk, Dohuk
Iraq
DOI: 10.4103/0.
PMID:
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| Hemoglobin Barts hydrops fetalis syndrome is the most severe and generally fatal clinical phenotype of α-thalassemia. We diagnosed a fetus at 23-weeks gestation with having hydrops fetalis, by ultrasound. At 32 weeks, intrauterine death was detected. Molecular studies revealed that the fetus had the hemoglobin Barts hydrops fetalis syndrome due to homozygosity for the Mediterranean α-thalassemia deletion. This clinical phenotype is generally rare in the Eastern Mediterranean, and this is the first report of this syndrome from Iraq. Techniques for molecular characterization became available only very recently in this country, in a diagnostic setting. Thus, the detection of further cases might be expected in future. |
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