Hepatic veno-occlusive disease with immunodeficiency
Autosomal recessive
A combined immune deficiency with low antibody levels and liver vein blockage, leading to severe infections and liver failure if untreated.
What a diagnosis changes
Early antibody replacement therapy and preventive treatment against Pneumocystis infection can be life-saving.
Genes analysed
Also known as
familial veno-occlusive disease with immunodeficiency; hepatic venoocclusive disease with immunodeficiency; veno-occlusive disease and immunodeficiency syndrome; VODI; VODI syndrome
