Learn About Hepatic Venoocclusive Disease with Immunodeficiency

What is the definition of Hepatic Venoocclusive Disease with Immunodeficiency?
Hepatic veno-occlusive disease with immunodeficiency (VODI) is a genetic disorder of the liver and immune system that appears before the age of 12 months in which the small veins of the liver become blocked.
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What are the symptoms of Hepatic Venoocclusive Disease with Immunodeficiency?
Symptoms of veno-occlusive disease with immunodeficiency may include failure to thrive, abnormally small head, liver enlargement, fibrosis of the liver, recurrent bacterial and opportunistic infections, diarrhea, anemia, fluid in the abdomen (ascites), seizures, weakness on one side of the body (hemiparesis), leg paralysis (paraplegia), lung fibrosis, lung hemorrhage, recurrent pneumonia, recurrent ear infections, yellowing of the skin, mucous membranes, and whites of eyes (jaundice), low platelets, urinary retention, portal hypertension (high blood pressure), liver abnormalities, fibrosis of the heart, and liver failure. Most individuals with veno-occlusive disease with immunodeficiency do not survive childhood, while a few may survive into early adulthood.
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What are the current treatments for Hepatic Venoocclusive Disease with Immunodeficiency?
Treatment for veno-occlusive disease with immunodeficiency include intravenous immunoglobulin (IVIG), pneumocystis jeroveci (fungal infection) prophylaxis, treatment for infections, and liver transplantation in selected patients.
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What are the latest Hepatic Venoocclusive Disease with Immunodeficiency Clinical Trials?
Using Ultrasound Elastography to Predict Development of Sinusoidal Obstruction Syndrome

Summary: The long-term goal of our research is to accurately identify SOS patients who would benefit from defibrotide treatment using US SWE. The overall objective of this study is to validate SWE as an early diagnostic marker for SOS. Our central hypothesis is that SWE changes will precede clinical and conventional US diagnostic criteria for SOS. Our hypothesis has been formulated on the basis of our own ...

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Antithrombin-III for Patients With Hepatic Veno-occlusive Diseases Following Hematopoietic Stem Cell Transplantation

Summary: To evaluate efficacy and safety of AT-III treatment in patients with hepatic veno-occlusive diseases following hematopoietic stem cell transplantation.

What are the Latest Advances for Hepatic Venoocclusive Disease with Immunodeficiency?
A Retrospective Cohort Study of the Efficacy, Safety, and Clinical Value of 6-TG versus 6-MP Maintenance Therapy in Children with Acute Lymphoblastic Leukemia.
Therapeutic Plasma Exchange in Pediatric Patients With Sinusoidal Obstruction Syndrome/Veno-Occlusive Disease After Hematopoietic Stem Cell Transplantation: A Single-Center Experience.
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