Medicina: Ematologia: Talassemie e trapianto del midollo osseo
| English |
| hb bart´s (en.) |
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| Attestation |
3
|
| Part of speech |
Noun syntagm
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| Grammatical label |
Countable
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| Variant |
Bart´s Hemoglobin
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| Definition |
Hb Bart´s hydrops fetalis is caused by deletion of all four a-globin genes, and it is associated with a dismal prognosis for the affected fetus, who will usually die in utero during the third trimester or shortly after birth. Moreover, there is an increased incidence of serious maternal complications in these pregnancies. In order to prevent Hb Bart´s hydrops fetalis, at risk couples in whom both partners carry the (--SEA) a-thalassemia mutation should be identified, so that genetic counseling and prenatal diagnosis may be offered.
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| Definition source |
Ma ES., et al. (2000). ‘Screening for (--SEA) alpha-globin gene deletion in beta-thalassemia carriers and prevention of hydrops fetalis’. Haematologica 85(9):991-3. (RISCEN33)
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| Context |
Homozygous a-thal 1 causes Bart´s hemoglobin hydrops fetalis, and various obstetrical complications, while compound heterozygous a-thal 1 and a -thal 2 causes HbH disease.
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| Context source |
Ko, TM., et al. (1999). ‘Prevalence study and molecular characterization of alpha-thalassemia in Filipinos’. Annals of Hematology 78(8):355-7. (RISCEN90)
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| Subject field |
Haemopoiesis
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| Sub-field (level 1) |
Thalassemias
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| Specific concept |
Heterozygote Hb Bart´s, homozygote Hb Bart´s
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| Related concept |
Hydrops Fetalis, hemoglobin, globin chain, globin gene, Hb Lepore
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| it |
Hb Bart´s (It.)
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| Reliability code |
3
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