Iron overload in transfusion-dependent survivors of hemoglobin Bart’s hydrops fetalis
نویسندگان
چکیده
منابع مشابه
Optimizing chronic transfusion therapy for survivors of hemoglobin Barts hydrops fetalis.
Hemoglobin Barts hydrops fetalis (homozygous a-thalassemia) results from deletion of all 4 a-globin genes. It was previously considered a universally fatal condition; however,with recent advances in prenatal care and the availability of intrauterine blood transfusions, an increasing number of patients are now surviving into adulthood. Similar to patients with transfusion-dependent thalassemia d...
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Advances in the management of transfusion dependent thalassemic patients have improved the survival of these patients. The most important consequence of repeated and frequent transfusions is iron accumulation in vital organs. The magnetic resonance imaging (MRI) is a non-invasive and valid technique for the estimation of iron stores. Despite multiple studies about cardiac and liver MRI T2*, the...
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tion, which removes both the 1 and 2 genes from 1 allele, leaving only 2 functional genes. This finding explains the comparatively high expression level (40%) of the variant chain. In an alignment of 449 globin sequences from different species, 91 is absolutely conserved as leucine, and this extraordinary level of conservation suggests an important functional role. X-ray structural analyses sho...
متن کاملAetiology of hydrops fetalis.
These 2 cases provide additional examples of HUS occurring in sibs after a long interval. The family resided in New York City, which is not considered as an endemic area for HUS, and the clinical course of the disease in both infants was almost identical, culminating in early death. The aetiology of HUS may indeed be multiple, and the genetic basis for a significant proportion of cases does not...
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ژورنال
عنوان ژورنال: Haematologica
سال: 2018
ISSN: 0390-6078,1592-8721
DOI: 10.3324/haematol.2017.178368