Hemoglobin Bart's fetalis syndrome (Q39516): Difference between revisions
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A hidropsia fetal da Hb Bart (HBFS) é a forma mais grave da alfa talassemia e é quase sempre letal. É caracterizada por inicio fetal de edema generalizado, derrames pleural e pericárdico, e anemia hipocrômica grave._x000D_ A HBFS pode ser causada por talassemia alfa0 (alfa zero) homozigótica ou heterozigótica composta, resultando em ausência de genes funcionais da hemoglobina alfa. | |||
| description / en | description / en | ||
Hb Bart's hydrops fetalis (HBFS) is the most severe form of alpha-thalassemia and is almost always lethal. It is characterised by fetal onset of generalised oedema, pleural and pericardial effusions, and severe hypochromic anaemia. HBFS can be caused by either homozygous or compound heterozygous alpha0 (alpha zero) thalassaemia, resulting in zero functional alpha haemoglobin genes. | |||
Revision as of 04:57, 13 August 2026
Hb Bart's hydrops fetalis (HBFS) is the most severe form of alpha-thalassemia and is almost always lethal. It is characterised by fetal onset of generalised oedema, pleural and pericardial effusions, and severe hypochromic anaemia. HBFS can be caused by either homozygous or compound heterozygous alpha0 (alpha zero) thalassaemia, resulting in zero functional alpha haemoglobin genes.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 3A50.03 |
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| English | Hemoglobin Bart's fetalis syndrome |
Hb Bart's hydrops fetalis (HBFS) is the most severe form of alpha-thalassemia and is almost always lethal. It is characterised by fetal onset of generalised oedema, pleural and pericardial effusions, and severe hypochromic anaemia. HBFS can be caused by either homozygous or compound heterozygous alpha0 (alpha zero) thalassaemia, resulting in zero functional alpha haemoglobin genes. |
