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Fingerprinting Cardiolipin in Leukocytes by Mass Spectrometry for a Rapid Diagnosis of Barth Syndrome
Published on: March 23, 2022
Diagnosis of Budd-Chiari syndrome
Morgane Van Wettere1, Onorina Bruno1, Pierre-Emmanuel Rautou2
1Department of Radiology, Hôpital Beaujon, HUPNVS, 100, Bd du Général Leclerc, Clichy, France.
Insights
Budd-Chiari syndrome (BCS) impairs hepatic venous drainage, often due to myeloproliferative neoplasms. Imaging, including ultrasound and MRI, is crucial for diagnosing BCS by identifying direct and indirect signs of venous obstruction and liver changes.
Area of Science:
- Radiology
- Hepatology
- Vascular Medicine
Background:
- Budd-Chiari syndrome (BCS) is characterized by impaired hepatic venous outflow.
- Primary BCS often results from hypercoagulable states like myeloproliferative neoplasms.
- Secondary BCS arises from extrinsic compression or tumor invasion.
Purpose of the Study:
- To review the diagnostic role of imaging in Budd-Chiari syndrome.
- To highlight key imaging features of BCS.
Main Methods:
- Review of imaging modalities for BCS diagnosis.
- Focus on ultrasound and magnetic resonance imaging (MRI) as gold standards.
- Analysis of direct and indirect imaging signs.
Main Results:
- Direct signs include hepatic vein/inferior vena cava occlusion and collateral formation.
- Indirect signs involve liver morphology changes like caudate lobe hypertrophy.
- Ultrasound and MRI are essential for accurate BCS diagnosis.
Conclusions:
- Imaging is pivotal in diagnosing Budd-Chiari syndrome.
- Recognizing specific imaging findings aids in early detection and management.
- This review emphasizes the importance of advanced imaging techniques for BCS.
Abstract:
Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition. Acute BCS is rare, and patients present with hepatomegaly, ascites, and hepatic insufficiency. The diagnosis is based on imaging. Imaging features include (1) direct signs, in particular occlusion or compression of the hepatic veins and/or the inferior vena cava and venous collaterals and (2) indirect signs, in particular morphological changes in the liver with hypertrophy of the caudate lobe and delayed nodule formation. Ultrasound and magnetic resonance imaging are the gold standard for diagnosis. The aim of this review is to provide an overview of the role of imaging in the diagnosis of BCS.
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