<unk>  (VODI) is characterized by Primary<unk>  and terminal hepatic lobular vascular occlusion and hepatic fibrosis manifest as hepatomegaly and/or hepatic failure.
Onset is before age 12 months. The<unk>  comprises severe hypogammaglobulinemia, clinical evidence of T-cell<unk>  with normal numbers of circulating T cells, absent lymph node germinal centers, and absent tissue plasma cells. Bacterial and opportunistic infections including Pneumocystis jerovici infection, mucocutaneous candidiasis, and enteroviral or cytomegalovirus infections occur.<unk>  (also called VODI) is a hereditary disorder of the liver and immune system. Its signs and symptoms appear after the first few months of life.
Hepatic<unk><unk><unk>  is a condition that blocks (occludes) small veins in the liver, disrupting blood flow in this organ. This condition can lead to enlargement of the liver (hepatomegaly), a buildup of scar tissue (hepatic fibrosis), and liver failure.
Children with VODI are prone to recurrent infections caused by certain bacteria, viruses, and fungi. The organisms that cause infection in people with this disorder are described as opportunistic because they ordinarily do not cause illness in healthy people. These infections are usually serious and may be life-threatening.  In most people with VODI, infections occur before hepatic<unk><unk><unk>  becomes evident.
Hepatic veno-occlusive disease with immunodeficiency
Congenital hepatic fibrosis
Autoimmune hepatitis
Primary biliary cirrhosis
Indian familial childhood cirrhosis
ABCD syndrome
Secondary Biliary Cirrhosis
Pediatric end-stage liver disease