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Гемоглобінова водянка плода Барта

ORPHA:163596· ICD-10 D56.0· Hemoglobin Bart's fetalis syndrome

Визначення(English summary)

A severe form of alpha-thalassemia that is mostly lethal, and associated with severe long-term outcome and lifelong transfusions in survivors. It is characterized by fetal onset of generalized edema, pleural and pericardial effusions, and severe hypochromic anemia.

Поширеність
Unknown
Успадкування
Autosomal recessive
Вік початку
Antenatal, Neonatal