Role of Liver Stiffness in Hematological Disorders: Assessment of Sinusoidal Obstruction Syndrome, Budd–Chiari Syndrome, and Treatment Complications

2020 ◽  
pp. 169-176
Author(s):  
Thomas Karlas
Keyword(s):  
Liver Stiffness ◽  
Sinusoidal Obstruction Syndrome ◽  
Budd Chiari Syndrome ◽  
Hematological Disorders ◽  
Treatment Complications ◽  
Chiari Syndrome

scholarly journals The potential role of Transient Elastography in assessing patients with Primary Budd Chiari Syndrome

2020 ◽  
Author(s):  
Muriel Webb ◽  
Oren Shibolet ◽  
Yoav Lurie ◽  
Yakov Maor ◽  
Helena Katchman ◽  
...  
Keyword(s):  
Liver Cirrhosis ◽  
Potential Role ◽  
Transient Elastography ◽  
Liver Stiffness ◽  
Abdominal Ultrasound ◽  
P Value ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome ◽  
Cirrhotic Patients

Abstract Background: Budd-Chiari syndrome (BCS) is a rare disease defined as hepatic venous outflow obstruction at any level from the hepatic venules up to the cavo-atrial junction. Transjugular Intrahepatic Portosystemic Shunt (TIPS) is performed as a decompressive treatment in some patients.Aim: To evaluate the potential role of Transient Elastography (TE) in assessing liver stiffness in patients with primary BCS.Methods: Twenty one BCS patients and 10 patients with liver cirrhosis with different underlying etiologies underwent abdominal ultrasound and TE.Results: Ninety-five percent of BCS patients had liver stiffness compatible with F4 with a median of 21 kPa, values which are usually obtained in patients with liver cirrhosis. Ten BCS and 10 cirrhotic patients underwent repeated TE with a median of 320 days between exams for BCS and 4.5 years for cirrhotic patients. The change of liver stiffness in BCS patients was 5.75 kPa (range − 0.4 to 26.6), compared with − 4.85 kPa (range − 15.6 to 15.0) in cirrhotic patients (p-value = 0.0029). Change in liver stiffness from baseline to follow-up in BCS patients who underwent TIPS (n = 4) was 0.2 kPa (range − 0.4 to 15.3), whereas in patients without intervention (n = 6) it was 6.75 kPa (range 1.3 to 26.6). The difference was not statistically significant.Conclusion: Liver stiffness in BCS patients is a dynamic progressive process with parameters of TE resembling liver cirrhosis. Even if TIPS seem to slow down the increment of liver stiffness, because of decreased liver congestion, it kept most patients with high score. The TE in BCS patients may be considered for monitoring for stable or upfront disease deterioration.

Reliability of Transient Elastography as a non-Invasive Technique for Detection of Fibrosis in Budd Chiari Syndrome Patients after Endovascular Intervention

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Mohammed F Montasser ◽  
Eman M Barakat ◽  
Mohamed S Ghazy ◽  
Sara M Abdelhakam ◽  
Hend E Ebada ◽  
...  
Keyword(s):  
Liver Fibrosis ◽  
Liver Biopsy ◽  
Transient Elastography ◽  
Liver Stiffness ◽  
Endovascular Intervention ◽  
Invasive Technique ◽  
Budd Chiari Syndrome ◽  
Metavir Score ◽  
Before And After ◽  
Chiari Syndrome

Abstract Aim of the work To test the reliability of fibroscan in detection of fibrosis in patients with Budd Chiari syndrome before and after endovascular intervention (after elimination of hepatic congestion). Background transient elastography (TE) is a noninvasive methodology that has been used to monitor liver stiffness in patients with chronic viral hepatitis. One of the limitations for accurate assessment of liver fibrosis by TE is the liver congestion. Liver congestion can result from Budd Chiari syndrome (BCS).The treatment of BCS is through restoring the flow of the blood between the portal vein to the inferior vena cava, which will lead to decongestion of the liver.TE, will be tested after liver decongestion for proper detection of liver fibrosis. Patients and methods This was a prospective cohort study conducted on 25 Egyptian patients with confirmed diagnosis of primary Budd-Chiari Syndrome (BCS) in the period from June 2017 to September 2019. TE was performed three days before endovascular intervention and three months after it. Liver biopsy was taken during the intervention for assessment of METAVIR score. Comparison was done between TE assessments before and after intervention in detection of the degree of liver fibrosis in comparison to METAVIR score measured in liver biopsy. Results FVLM was the most common hypercoagulable cause in the involved patients. There was significant drop in Liver Stiffness Measurements (LSM) measured three months post-intervention indicating improvement of liver fibrosis after relieving liver congestion but still not correlated to the METAVIR scores measured in the liver biopsy. Conclusion Liver congestion has high impact on Liver stiffness measurement giving overestimation which improves significantly after decongestion of the liver by the endovascular intervention.

scholarly journals Liver stiffness is a useful marker for assessing the therapeutic effect of angioplasty in Budd-Chiari syndrome: A case report

Kanzo ◽  
2018 ◽  
Vol 59 (10) ◽  
pp. 563-570
Author(s):  
Ran Nagahara ◽  
Tomomitsu Matono ◽  
Masahiko Koda ◽  
Yukako Matsuki ◽  
Masashi Yamane ◽  
...  
Keyword(s):  
Case Report ◽  
Therapeutic Effect ◽  
Liver Stiffness ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome

Role of radiological imaging and interventions in management of Budd–Chiari syndrome

2018 ◽  
Vol 73 (7) ◽  
pp. 610-624 ◽  
Author(s):  
C.J. Das ◽  
M. Soneja ◽  
S. Tayal ◽  
A. Chahal ◽  
S. Srivastava ◽  
...  
Keyword(s):  
Radiological Imaging ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome

scholarly journals Imaging findings and differential diagnosis of hepatic sinusoidal occlusion syndrome and Budd-Chiari syndrome

2021 ◽  
Author(s):  
Cui yu Jia ◽  
Da wei Zhao ◽  
Ji liang Feng ◽  
Rui li Li ◽  
Xin xin Wang ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Reduction Rate ◽  
Gallbladder Wall ◽  
Wall Thickening ◽  
Sinusoidal Obstruction Syndrome ◽  
Imaging Findings ◽  
Laboratory Examination ◽  
Budd Chiari Syndrome ◽  
Hepatic Sinusoidal Obstruction Syndrome ◽  
Chiari Syndrome

Abstract Objective To retrospectively analyze the clinical and imaging manifestations differential points between hepatic sinusoidal obstruction syndrome (SOS) and Budd-Chiari syndrome (BCS). Material The clinical symptoms, laboratory examination and imaging findings of 15 cases of hepatic sinusoidal obstruction syndrome and 33 cases of Budd-Chiari syndrome were statistically analyzed, find the identification points. The study used the Fisher test, P values of 0.05 or less were considered to indicate significant differences. The retrospective study was approved by the hospital's ethics committee. Results Laboratory tests: There were significant differences in total protein reduction rate (66.7% vs 9.1%, p༜0.01), albumin reduction rate (66.7% vs 9.1%, p༜0.01), Gamma-glutamyltransferase elevation rate (100% vs 6.1%, P༜0.01), alkaline phosphatase elevation rate (60% vs 6.1%, p༜0.01), and abnormal rate of prothrombin time (100% vs 21.2%, p༜0.01) between the two groups (BCS vs SOS). It also indicates that the liver function of SOS patients is more seriously impaired. Image findings: The following image findings were observed significant more frequently in SOS than in BCS and were statistically significant: gallbladder wall thickening (66.7% vs 78.2%, p༜0.01), ascites (80% vs 27.3%, p༜0.01), cloverleaf or claw-like shapes (80% vs 0%, p༜0.01). The following images appeared more frequently in BCS than in BCS and were statistically significant: caudate lobe enlargement (33.3% vs 75.8%, p༜0.01), collateral circulation (46.7% vs 93.9%, p༜0.01), diffuse patchy enhancement (20% vs 93.9%, p༜0.01), homogeneous in delayed phase (13.3% vs 90.1%, p༜0.01). Conclusion To Identification of SOS and BCS should be based on the patient's laboratory examination and imaging findings to improve diagnostic accuracy.

scholarly journals Association between JAK2 rs4495487 Polymorphism and Risk of Budd-Chiari Syndrome in China

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Peijin Zhang ◽  
Yanyan Zhang ◽  
Jing Zhang ◽  
Hui Wang ◽  
He Ma ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Myeloproliferative Neoplasms ◽  
Jak2 V617f ◽  
Recessive Model ◽  
Elevated Risk ◽  
Budd Chiari Syndrome ◽  
Candidate Locus ◽  
Susceptibility Factor ◽  
Chiari Syndrome

Myeloproliferative neoplasms (MPNs) are the leading cause of Budd-Chiari syndrome (BCS), and the C allele of JAK2 rs4495487 was reported to be an additional candidate locus that contributed to MPNs. In the present study, we examined the role of JAK2 rs4495487 in the etiology and clinical presentation of Chinese BCS patients. 300 primary BCS patients and 311 healthy controls were enrolled to evaluate the association between JAK2 rs4495487 polymorphism and risk of BCS. All subjects were detected for JAK2 rs4495487 by real-time PCR.Results. The JAK2 rs4495487 polymorphism was associated with JAK2 V617F-positive BCS patients compared with controls (P<0.01). The CC genotype increased the risk of BCS in patients with JAK2 V617F mutation compared with individuals presenting TT genotype (OR = 13.60, 95% CI = 2.04–90.79) and non-CC genotype (OR = 12.00, 95% CI = 2.07–69.52). We also observed a significantly elevated risk of combined-type BCS associated with CC genotype in the recessive model (OR = 4.44, 95% CI = 1.31–15.12). This study provides statistical evidence that the JAK2 rs4495487 polymorphism is susceptibility factor JAK2 V617F positive BCS and combined BCS in China. Further larger studies are required to confirm these findings.

The Role of Intravascular Intervention in the Management of Budd-Chiari Syndrome

2009 ◽  
Vol 55 (9) ◽  
pp. 2659-2663 ◽  
Author(s):  
Hui Xue ◽  
Ying-Chao Li ◽  
Pramod Shakya ◽  
Muna Palikhe ◽  
Rajiv Kumar Jha
Keyword(s):  
Budd Chiari Syndrome ◽  
Chiari Syndrome

A Case Report on Budd Chiari Syndrome – Which Mimics Multiple Disorders

2021 ◽  
Vol 2 (3) ◽  
pp. 4-7
Author(s):  
Niveditha Dileep ◽  
John Thomas ◽  
Jisha James ◽  
Abhijith V
Keyword(s):  
Liver Parenchyma ◽  
Sinusoidal Obstruction Syndrome ◽  
Biliary Disease ◽  
Budd Chiari Syndrome ◽  
Clinical Presentations ◽  
Chiari Syndrome ◽  
History Of ◽  
Clinical Variants ◽  
Diagnostic Approaches

Background: Budd Chiari Syndrome (BCS) is the king of disorders which will mimic other disorders like chronic biliary disease, constrictive pericarditis, sinusoidal obstruction syndrome and so on, as it be like that, it is less diagnosed and treated in many countries. The prevalence of BCS is one in one million population, so it is a very rare case therefore it should be treated properly because many disorders like hematologic or malignant disease are the complications of BCS [1], [2]. Objective: To access the clinical variants of BCS along with the similarities and differences in clinical presentation, diagnostic approaches, and general treatment pattern which mimic, BCS thus gives the physician a clear outline about those disorders. Method: A man of 42 years old having BCS was taken for the study to carry out the differences in clinical features of BCS which distinguish the mimicking disorders. Clinical presentations were noted. Laboratory tests and diagnostic tests showed that the patient is having comorbidities including fatty liver with cholelithiasis, mild splenomegaly, liver parenchyma diseases, large esophageal varies with signs of recent hemorrhage, port hypertensive gastropathy and minimal ascites [3]. Patient get discharged after feeling better. Result: This patient is having chronic BCS with DIPS dysfunction. The patient had no history of liver disease before diagnosing BCS. BCS is almost curable when it is diagnosed correctly as early as possible. If not diagnosed early and treated well, comorbidities will occur, and it will affect the patient quality of life. Discussion: Early detection and proper treatment will help to control the disease up to an extent. This is depending on the physician’s knowledge. Hence, this case study clearly explains the disorders which mimic BCS for better understanding.

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