Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome

•We identified high prevalence of Behçet's syndrome (BS) among Egyptian patients with primary BCS.•Patients with BCS should be screened for BS, especially with the presence of suggestive symptoms, e.g. recurrent oral ulcers, genital ulcers, lower extremity venous thrombosis, prominent abdominal...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Clinics and research in hepatology and gastroenterology 2020-09, Vol.44 (4), p.503-512
Hauptverfasser: Sakr, Mohammad A., Reda, Mohga A., Ebada, Hend E., Abdelmoaty, Ahmed S., Hefny, Zeinab M., Ibrahim, Zakaria H., Aboelmaaty, Mohamed E.
Format: Artikel
Sprache:eng
Schlagworte:
Online-Zugang:Volltext
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
container_end_page 512
container_issue 4
container_start_page 503
container_title Clinics and research in hepatology and gastroenterology
container_volume 44
creator Sakr, Mohammad A.
Reda, Mohga A.
Ebada, Hend E.
Abdelmoaty, Ahmed S.
Hefny, Zeinab M.
Ibrahim, Zakaria H.
Aboelmaaty, Mohamed E.
description •We identified high prevalence of Behçet's syndrome (BS) among Egyptian patients with primary BCS.•Patients with BCS should be screened for BS, especially with the presence of suggestive symptoms, e.g. recurrent oral ulcers, genital ulcers, lower extremity venous thrombosis, prominent abdominal veins, and IVC thrombosis in young adult male patients.•Treatment of BS and BCS should be initiated soon after diagnosis with early medical treatment in the form of immunosuppression and anticoagulation±endovascular interventions whenever indicated.•TIPS is rarely indicated in BS patients.•Despite worse prognosis and higher mortality in BCS due to BS than those without BS, survival and mortality were not significantly different between both groups. Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients. We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed. In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013). The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups.
doi_str_mv 10.1016/j.clinre.2019.10.006
format Article
fullrecord <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2315526145</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S2210740119302499</els_id><sourcerecordid>2315526145</sourcerecordid><originalsourceid>FETCH-LOGICAL-c339t-f39c5b128fb2edc21af24c323101695dbe783c69ebd2afad8bf1fbad3fca28be3</originalsourceid><addsrcrecordid>eNp9UMtqwzAQFKWFhjZ_0INu7cWuHrZjXwqN6QsCvTTQm5ClFVFwrFSSC_mifkh_rDYOOXYvu-zOzDKD0A0lKSW0uN-mqrWdh5QRWg2rlJDiDM0YoyRZZPTz_DQTeonmIWzJUFlOygWdoXW9kV6qCN6GaFXAstPY9VG5HWBn8N7bnfQHvOy1TuqNld7icOi0H--6BxwdXsLm9wfibThdrtGFkW2A-bFfofXz00f9mqzeX97qx1WiOK9iYnil8oay0jQMtGJUGpYpzvhorMp1A4uSq6KCRjNppC4bQ00jNTdKsrIBfoXuJt29d189hCh2NihoW9mB64MYlPKcFTTLB2g2QZV3IXgw4mhNUCLGf2IrpiDFGOS4HYIcaA8TDQYb3xa8CMpCp0BbDyoK7ez_An9J0YA8</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2315526145</pqid></control><display><type>article</type><title>Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome</title><source>Elsevier ScienceDirect Journals Complete</source><creator>Sakr, Mohammad A. ; Reda, Mohga A. ; Ebada, Hend E. ; Abdelmoaty, Ahmed S. ; Hefny, Zeinab M. ; Ibrahim, Zakaria H. ; Aboelmaaty, Mohamed E.</creator><creatorcontrib>Sakr, Mohammad A. ; Reda, Mohga A. ; Ebada, Hend E. ; Abdelmoaty, Ahmed S. ; Hefny, Zeinab M. ; Ibrahim, Zakaria H. ; Aboelmaaty, Mohamed E.</creatorcontrib><description>•We identified high prevalence of Behçet's syndrome (BS) among Egyptian patients with primary BCS.•Patients with BCS should be screened for BS, especially with the presence of suggestive symptoms, e.g. recurrent oral ulcers, genital ulcers, lower extremity venous thrombosis, prominent abdominal veins, and IVC thrombosis in young adult male patients.•Treatment of BS and BCS should be initiated soon after diagnosis with early medical treatment in the form of immunosuppression and anticoagulation±endovascular interventions whenever indicated.•TIPS is rarely indicated in BS patients.•Despite worse prognosis and higher mortality in BCS due to BS than those without BS, survival and mortality were not significantly different between both groups. Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients. We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed. In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013). The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups.</description><identifier>ISSN: 2210-7401</identifier><identifier>EISSN: 2210-741X</identifier><identifier>DOI: 10.1016/j.clinre.2019.10.006</identifier><language>eng</language><publisher>Elsevier Masson SAS</publisher><subject>Behçet's disease ; Budd-Chiari syndrome ; Hepatocellular carcinoma ; Immunosuppression ; Vasculitis ; Vena cava occlusion</subject><ispartof>Clinics and research in hepatology and gastroenterology, 2020-09, Vol.44 (4), p.503-512</ispartof><rights>2019 Elsevier Masson SAS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c339t-f39c5b128fb2edc21af24c323101695dbe783c69ebd2afad8bf1fbad3fca28be3</citedby><cites>FETCH-LOGICAL-c339t-f39c5b128fb2edc21af24c323101695dbe783c69ebd2afad8bf1fbad3fca28be3</cites><orcidid>0000-0001-6982-1710</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.clinre.2019.10.006$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids></links><search><creatorcontrib>Sakr, Mohammad A.</creatorcontrib><creatorcontrib>Reda, Mohga A.</creatorcontrib><creatorcontrib>Ebada, Hend E.</creatorcontrib><creatorcontrib>Abdelmoaty, Ahmed S.</creatorcontrib><creatorcontrib>Hefny, Zeinab M.</creatorcontrib><creatorcontrib>Ibrahim, Zakaria H.</creatorcontrib><creatorcontrib>Aboelmaaty, Mohamed E.</creatorcontrib><title>Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome</title><title>Clinics and research in hepatology and gastroenterology</title><description>•We identified high prevalence of Behçet's syndrome (BS) among Egyptian patients with primary BCS.•Patients with BCS should be screened for BS, especially with the presence of suggestive symptoms, e.g. recurrent oral ulcers, genital ulcers, lower extremity venous thrombosis, prominent abdominal veins, and IVC thrombosis in young adult male patients.•Treatment of BS and BCS should be initiated soon after diagnosis with early medical treatment in the form of immunosuppression and anticoagulation±endovascular interventions whenever indicated.•TIPS is rarely indicated in BS patients.•Despite worse prognosis and higher mortality in BCS due to BS than those without BS, survival and mortality were not significantly different between both groups. Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients. We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed. In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013). The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups.</description><subject>Behçet's disease</subject><subject>Budd-Chiari syndrome</subject><subject>Hepatocellular carcinoma</subject><subject>Immunosuppression</subject><subject>Vasculitis</subject><subject>Vena cava occlusion</subject><issn>2210-7401</issn><issn>2210-741X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><recordid>eNp9UMtqwzAQFKWFhjZ_0INu7cWuHrZjXwqN6QsCvTTQm5ClFVFwrFSSC_mifkh_rDYOOXYvu-zOzDKD0A0lKSW0uN-mqrWdh5QRWg2rlJDiDM0YoyRZZPTz_DQTeonmIWzJUFlOygWdoXW9kV6qCN6GaFXAstPY9VG5HWBn8N7bnfQHvOy1TuqNld7icOi0H--6BxwdXsLm9wfibThdrtGFkW2A-bFfofXz00f9mqzeX97qx1WiOK9iYnil8oay0jQMtGJUGpYpzvhorMp1A4uSq6KCRjNppC4bQ00jNTdKsrIBfoXuJt29d189hCh2NihoW9mB64MYlPKcFTTLB2g2QZV3IXgw4mhNUCLGf2IrpiDFGOS4HYIcaA8TDQYb3xa8CMpCp0BbDyoK7ez_An9J0YA8</recordid><startdate>202009</startdate><enddate>202009</enddate><creator>Sakr, Mohammad A.</creator><creator>Reda, Mohga A.</creator><creator>Ebada, Hend E.</creator><creator>Abdelmoaty, Ahmed S.</creator><creator>Hefny, Zeinab M.</creator><creator>Ibrahim, Zakaria H.</creator><creator>Aboelmaaty, Mohamed E.</creator><general>Elsevier Masson SAS</general><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0001-6982-1710</orcidid></search><sort><creationdate>202009</creationdate><title>Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome</title><author>Sakr, Mohammad A. ; Reda, Mohga A. ; Ebada, Hend E. ; Abdelmoaty, Ahmed S. ; Hefny, Zeinab M. ; Ibrahim, Zakaria H. ; Aboelmaaty, Mohamed E.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c339t-f39c5b128fb2edc21af24c323101695dbe783c69ebd2afad8bf1fbad3fca28be3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Behçet's disease</topic><topic>Budd-Chiari syndrome</topic><topic>Hepatocellular carcinoma</topic><topic>Immunosuppression</topic><topic>Vasculitis</topic><topic>Vena cava occlusion</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sakr, Mohammad A.</creatorcontrib><creatorcontrib>Reda, Mohga A.</creatorcontrib><creatorcontrib>Ebada, Hend E.</creatorcontrib><creatorcontrib>Abdelmoaty, Ahmed S.</creatorcontrib><creatorcontrib>Hefny, Zeinab M.</creatorcontrib><creatorcontrib>Ibrahim, Zakaria H.</creatorcontrib><creatorcontrib>Aboelmaaty, Mohamed E.</creatorcontrib><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinics and research in hepatology and gastroenterology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sakr, Mohammad A.</au><au>Reda, Mohga A.</au><au>Ebada, Hend E.</au><au>Abdelmoaty, Ahmed S.</au><au>Hefny, Zeinab M.</au><au>Ibrahim, Zakaria H.</au><au>Aboelmaaty, Mohamed E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome</atitle><jtitle>Clinics and research in hepatology and gastroenterology</jtitle><date>2020-09</date><risdate>2020</risdate><volume>44</volume><issue>4</issue><spage>503</spage><epage>512</epage><pages>503-512</pages><issn>2210-7401</issn><eissn>2210-741X</eissn><abstract>•We identified high prevalence of Behçet's syndrome (BS) among Egyptian patients with primary BCS.•Patients with BCS should be screened for BS, especially with the presence of suggestive symptoms, e.g. recurrent oral ulcers, genital ulcers, lower extremity venous thrombosis, prominent abdominal veins, and IVC thrombosis in young adult male patients.•Treatment of BS and BCS should be initiated soon after diagnosis with early medical treatment in the form of immunosuppression and anticoagulation±endovascular interventions whenever indicated.•TIPS is rarely indicated in BS patients.•Despite worse prognosis and higher mortality in BCS due to BS than those without BS, survival and mortality were not significantly different between both groups. Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients. We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed. In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013). The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups.</abstract><pub>Elsevier Masson SAS</pub><doi>10.1016/j.clinre.2019.10.006</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0001-6982-1710</orcidid></addata></record>
fulltext fulltext
identifier ISSN: 2210-7401
ispartof Clinics and research in hepatology and gastroenterology, 2020-09, Vol.44 (4), p.503-512
issn 2210-7401
2210-741X
language eng
recordid cdi_proquest_miscellaneous_2315526145
source Elsevier ScienceDirect Journals Complete
subjects Behçet's disease
Budd-Chiari syndrome
Hepatocellular carcinoma
Immunosuppression
Vasculitis
Vena cava occlusion
title Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome
url https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-24T12%3A01%3A13IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Characteristics%20and%20outcome%20of%20primary%20Budd-Chiari%20syndrome%20due%20to%20Beh%C3%A7et's%20syndrome&rft.jtitle=Clinics%20and%20research%20in%20hepatology%20and%20gastroenterology&rft.au=Sakr,%20Mohammad%20A.&rft.date=2020-09&rft.volume=44&rft.issue=4&rft.spage=503&rft.epage=512&rft.pages=503-512&rft.issn=2210-7401&rft.eissn=2210-741X&rft_id=info:doi/10.1016/j.clinre.2019.10.006&rft_dat=%3Cproquest_cross%3E2315526145%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2315526145&rft_id=info:pmid/&rft_els_id=S2210740119302499&rfr_iscdi=true