Summary
Definition
History and exam
Key diagnostic factors
- right upper quadrant abdominal pain
- hepatomegaly
- ascites
- jaundice
Other diagnostic factors
- splenomegaly
- gastrointestinal bleeding
- leg edema
- truncal venous collaterals
- hepatic encephalopathy
Risk factors
- female sex
- myeloproliferative disorders (MPD)
- thrombophilic disorders
- oral contraceptive pill use
- pregnancy and immediate postpartum period
Diagnostic investigations
1st investigations to order
- thrombophilia screening
- color and pulsed Doppler ultrasound
- LFTs
- BUN and creatinine
- coagulation profile
- CBC
- Janus kinase 2 (JAK2) mutation
- flow cytometry for CD55- and CD59-deficient blood cells
Investigations to consider
- serum-ascites albumin gradient (SAAG)
- total ascitic protein
- CT abdomen
- MRI abdomen
- hepatic venography
- contrast-enhanced 3-dimensional magnetic resonance angiography
- transjugular liver biopsy
- bone marrow biopsy
Treatment algorithm
primary: nonfulminant
primary: fulminant
secondary BCS
Contributors
Authors
Soha Saoud Abdel Moneim, MD, PhD

Lecturer of Gastroenterology and Hepatology
Assiut University
Assiut
Egypt
Disclosures
SSAM declares that she has no competing interests.
Vijay H. Shah, MD

Professor of Medicine
Physiology and Cancer Cell Biology
Mayo Clinic
Rochester
MN
Disclosures
VHS is the author of studies referenced in this topic. VHS is on the advisory boards of the following companies: Novartis Pharmaceuticals, Merck and Company, Enterome Corporation, Durect Corporation, Afimmune Ltd., Vital Therapies.
Peer reviewers
Howard J. Worman, MD
Professor of Medicine and Cell Biology
Columbia University College of Physicians and Surgeons
New York
NY
Disclosures
HJW declares that he has no competing interests.
Philip Newsome, PhD, FRCPE
Senior Lecturer in Hepatology & Honorary Consultant Physician
Liver Research Group
Institute of Biomedical Research
The Medical School
University of Birmingham
Birmingham
UK
Disclosures
PN declares that he has no competing interests.
Muhammad Dawwas, MRCP
Specialist Registrar
Liver Unit
Addenbrooke's Hospital
Cambridge
UK
Disclosures
Not disclosed.
Differentials
- Sinusoidal obstruction syndrome (SOS) (veno-occlusive disease)
- Fulminant hepatic failure due to other etiologies
- Congestive hepatopathy
More DifferentialsGuidelines
- Vascular diseases of the liver
- AASLD Practice Guidelines: vascular disorders of the liver
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