Meta-analysis: the significance of screening for JAK2V617F mutation in Budd-Chiari syndrome and portal venous system thrombosis

Abstract Myeloproliferative neoplasms (MPNs) are the most common cause of Budd-Chiari syndrome (BCS) and nonmalignant, noncirrhotic portal vein thrombosis (PVT). In this meta-analysis, we determined the prevalence of MPNs and their subtypes as well as JAK2V617F and its diagnostic role in these uncommon disorders. MEDLINE and EMBASE databases were searched. Prevalence of MPNs, JAK2V617F, and MPN subtypes were calculated using a random-effects model. A total of 1062 BCS and 855 PVT patients were included. In BCS, mean prevalence of MPNs and JAK2V617F was 40.9% (95% CI, 32.9%-49.5%) and 41.1% (95% CI, 32.3%-50.6%), respectively. In PVT, mean prevalence of MPNs and JAK2V617F was 31.5% (95% CI, 25.1%-38.8%) and 27.7% (95% CI, 20.8%-35.8%), respectively. JAK2V617F and MPNs were more frequent in BCS compared with PVT (P = .03 and P = .09, respectively). Polycythemia vera was more prevalent in BCS than in PVT (P = .001). JAK2V617F screening in splanchnic vein thrombosis (SVT) patients without typical hematologic MPN features identified MPN in 17.1% and 15.4% of screened BCS and PVT patients, respectively. These results demonstrate a high prevalence of MPNs and JAK2V617F in SVT patients and show differences in underlying etiology between these disorders. Furthermore, these results validate routine inclusion of JAK2V617F in the diagnostic workup of SVT patients.

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Prevalence of Budd-Chiari Syndrome during Pregnancy or Puerperium: A Systematic Review and Meta-Analysis

Gastroenterology Research and Practice ◽

10.1155/2015/839875 ◽

2015 ◽

Vol 2015 ◽

pp. 1-13 ◽

Cited By ~ 16

Author(s):

Weirong Ren ◽

Xiang Li ◽

Jia Jia ◽

Yan Xia ◽

Fengrong Hu ◽

...

Keyword(s):

Systematic Review ◽

Meta Analysis ◽

Asian Countries ◽

Budd Chiari Syndrome ◽

China National Knowledge Infrastructure ◽

Common Risk Factor ◽

Knowledge Infrastructure ◽

Pooled Prevalence ◽

Chiari Syndrome ◽

Huge Variation

Women during pregnancy or puerperium are likely to develop Budd-Chiari syndrome (BCS). However, the reported prevalence of pregnancy-related BCS varied considerably among studies. Our study aims to systematically review this issue. Overall, 817 papers were initially identified via the PubMed, EMBASE, China National Knowledge Infrastructure, and Chinese Scientific and Technological Journal databases. Twenty of them were eligible. The prevalence of pregnancy-related BCS varied from 0% to 21.5%. The pooled prevalence was 6.8% (95% CI: 3.9–10.5%) in all BCS patients, 6.3% (95% CI: 3.8–9.4%) in primary BCS patients, and 13.1% (95% CI: 7.1–20.7%) in female BCS patients. Among them, one study was carried out in Africa with a prevalence of 10.6%; 14 studies in Asian countries with a pooled prevalence of 7.1% (95% CI: 3.1–12.6%); and 5 studies in European countries with a pooled prevalence of 5.0% (95% CI: 3.1–7.3%). The pooled prevalence was 6.7% (95% CI: 2.6–12.3%) in studies published before 2005 and 7.3% (95% CI: 4.2–12.5%) in those published after 2005. In conclusion, pregnancy is a relatively common risk factor for BCS, but there is a huge variation in the prevalence among studies. Physicians should be aware of pregnancy-related BCS.

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Diagnostic accuracy of Doppler ultrasound, CT and MRI in Budd Chiari syndrome: systematic review and meta-analysis

British Journal of Radiology ◽

10.1259/bjr.20190847 ◽

2020 ◽

Vol 93 (1109) ◽

pp. 20190847 ◽

Cited By ~ 4

Author(s):

Pankaj Gupta ◽

Varun Bansal ◽

Praveen Kumar-M ◽

Saroj K Sinha ◽

Jayanta Samanta ◽

...

Keyword(s):

Diagnostic Accuracy ◽

Doppler Ultrasound ◽

Meta Analysis ◽

Area Under The Curve ◽

Diagnostic Odds Ratio ◽

Budd Chiari Syndrome ◽

Chiari Syndrome ◽

Sensitivity Specificity ◽

Ct And Mri

Objective: To evaluate the sensitivity, specificity, and diagnostic odds ratio (DOR) of Doppler ultrasound, CT, and MRI in the diagnosis of Budd Chiari syndrome (BCS). Methods: We performed a literature search in PubMed, Embase, and Scopus to identify articles reporting the diagnostic accuracy of Doppler ultrasound, CT, and MRI (either alone or in combination) for BCS using catheter venography or surgery as the reference standard. The quality of the included articles was assessed by using the Quality Assessment of Diagnostic Accuracy Studies-2 tool. Results: 11 studies were found eligible for inclusion. Pooled sensitivities and specificities of Doppler ultrasound were 89% [95% confidence interval (CI), 81–94%, I2 = 24.7%] and 68% (95% CI, 3–99%, I2 = 95.2%), respectively. Regarding CT, the pooled sensitivities and specificities were 89% (95% CI, 77–95%, I2 = 78.6%) and 72% (95% CI, 21–96%, I2 = 91.4%), respectively. The pooled sensitivities and specificities of MRI were 93% (95% CI, 89–96%, I2 = 10.6%) and 55% (95% CI, 5–96%, I2 = 87.6%), respectively. The pooled DOR for Doppler ultrasound, CT, and MRI were 10.19 (95% CI: 1.5, 69.2), 14.57 (95% CI: 1.13, 187.37), and 20.42 (95% CI: 1.78, 234.65), respectively. The higher DOR of MRI than that of Doppler ultrasound and CT shows the better discriminatory power. The area under the curve for MRI was 90.8% compared with 88.4% for CT and 86.6% for Doppler ultrasound. Conclusion: Doppler ultrasound, CT and MRI had high overall diagnostic accuracy for diagnosis of BCS, but substantial heterogeneity was found. Prospective studies are needed to investigate diagnostic performance of these imaging modalities. Advances in knowledge: MRI and CT have the highest meta-analytic sensitivity and specificity, respectively for the diagnosis of BCS. Also, MRI has the highest area under curve for the diagnosis of BCS.

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