Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11130%2F14%3A10292967" target="_blank" >RIV/00216208:11130/14:10292967 - isvavai.cz</a>
Alternative codes found
RIV/61989592:15110/14:33149554 RIV/00216224:14740/14:00078458 RIV/00098892:_____/14:#0000683
Result on the web
<a href="http://dx.doi.org/10.3324/haematol.2014.104034" target="_blank" >http://dx.doi.org/10.3324/haematol.2014.104034</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.3324/haematol.2014.104034" target="_blank" >10.3324/haematol.2014.104034</a>

Result language
angličtina
Original language name
Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency
Original language description
Diamond-Blackfan anemia (DBA) is a rare congenital red cell aplasia associated with mutations in ribosomal proteins (RP) in 49-71% of cases. DBA is a clinically heterogeneous disorder with one-third of patients developing transfusion-acquired iron overload. The severity of anemia and transfusion dependency in DBA is comparable to transfusion-dependent beta-thalassemia major; however, moderate to severe suppression of erythropoiesis in DBA is in contrast to accelerated ineffective erythropoiesis in beta-thalassemia. The knowledge of systemic iron regulation in DBA is limited.
Czech name
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Czech description
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Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FD - Oncology and haematology
OECD FORD branch
—

Project
Result was created during the realization of more than one project. More information in the Projects tab.
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year
2014
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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