Budd-Chiari syndrome
MONDO:0010947Mondo
Findings
No curated finding names Budd-Chiari syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Budd-Chiari syndrome (BCS) is caused by obstruction of hepatic venous outflow involving either the hepatic veins or the terminal segment of the inferior vena cava.
Definition from the Mondo Disease Ontology (MONDO:0010947), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal recessive inheritance
- Onset and course
- Adult onset
HPO, annotations 2026-09-02
Features
24 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- AscitesHPOHP:0001541
- Very frequent (80% to 99% of cases)
- Portal hypertensionHPOHP:0001409
- Very frequent (80% to 99% of cases)
- SplenomegalyHPOHP:0001744
- Very frequent (80% to 99% of cases)
- Abdominal painHPOHP:0002027
- Frequent (30% to 79% of cases)
- Bipedal edemaHPOHP:0033045
- Frequent (30% to 79% of cases)
- CirrhosisHPOHP:0001394
- 6 of 43 reported patients
- Frequent (30% to 79% of cases)
Show the remaining 12
- Acute hepatic failureHPOHP:0006554
- Occasional (5% to 29% of cases)
- CholecystitisHPOHP:0001082
- Occasional (5% to 29% of cases)
- Gastrointestinal hemorrhageHPOHP:0002239
- Occasional (5% to 29% of cases)
- Gastrointestinal infarctionsHPOHP:0005244
- Occasional (5% to 29% of cases)
- Hepatic encephalopathyHPOHP:0002480
- Occasional (5% to 29% of cases)
- Intestinal obstructionHPOHP:0005214
- Occasional (5% to 29% of cases)
Where it sits
- A kind of
Other names
1 name
Resolves to: Budd-Chiari syndrome
- Also called
- Budd-Chiari syndrome, somatic