Orphanet: Hb Bart's hydrops fetalis

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Hb Bart's hydrops fetalis

Disease definition

A rare, severe form of alpha-thalassemia that is almost always lethal. It is characterized by fetal onset of generalized edema, pleural and pericardial effusions, and severe hypochromic anemia.


Classification level: Subtype of disorder
  • Synonym(s):
    • Alpha-thalassemia hydrops fetalis
    • Alpha-thalassemia major
    • Hemoglobin Bart's hydrops fetalis
    • Homozygous alpha0-thalassemia
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: D56.0
  • OMIM: 236750
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

Article for general public


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