Hemoglobin Bart's fetalis syndrome (Q39516): Difference between revisions

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Latest 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
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.