Dysfibrinogenemia and thrombosis. A case report
Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia. To report a Tunisian case of the association dysfibrinogenemia and thrombosis. A woman with inherited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep...
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| Veröffentlicht in: | Tunisie Medicale 2010-10, Vol.88 (10), p.757-760 |
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| creator | Kraiem, Imen Guermazi, Sami Ben Abid, Héla Meddeb, Balkis |
| description | Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia.
To report a Tunisian case of the association dysfibrinogenemia and thrombosis.
A woman with inherited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significantly prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerisation. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded.
Although it is rare, this cause of thrombophilia must not be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplastin time and functional fibrinogen might be helpful. |
| format | Article |
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To report a Tunisian case of the association dysfibrinogenemia and thrombosis.
A woman with inherited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significantly prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerisation. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded.
Although it is rare, this cause of thrombophilia must not be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplastin time and functional fibrinogen might be helpful.</description><identifier>ISSN: 0041-4131</identifier><identifier>PMID: 20890827</identifier><language>fre</language><publisher>Tunisia: Maghreb-Editions; 1999</publisher><subject>Adult ; Afibrinogenemia ; Afibrinogenemia - complications ; Biochemistry, Molecular Biology ; Fatal Outcome ; Female ; Fibrinogens, Abnormal ; Fibrinogens, Abnormal - genetics ; Humans ; Life Sciences ; Pulmonary Embolism ; Pulmonary Embolism - etiology ; Thrombophilia ; Thrombophilia - complications ; Venous Thrombosis ; Venous Thrombosis - blood</subject><ispartof>Tunisie Medicale, 2010-10, Vol.88 (10), p.757-760</ispartof><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,780,784,885</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/20890827$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://riip.hal.science/pasteur-00620986$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Kraiem, Imen</creatorcontrib><creatorcontrib>Guermazi, Sami</creatorcontrib><creatorcontrib>Ben Abid, Héla</creatorcontrib><creatorcontrib>Meddeb, Balkis</creatorcontrib><title>Dysfibrinogenemia and thrombosis. A case report</title><title>Tunisie Medicale</title><addtitle>Tunis Med</addtitle><description>Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia.
To report a Tunisian case of the association dysfibrinogenemia and thrombosis.
A woman with inherited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significantly prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerisation. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded.
Although it is rare, this cause of thrombophilia must not be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplastin time and functional fibrinogen might be helpful.</description><subject>Adult</subject><subject>Afibrinogenemia</subject><subject>Afibrinogenemia - complications</subject><subject>Biochemistry, Molecular Biology</subject><subject>Fatal Outcome</subject><subject>Female</subject><subject>Fibrinogens, Abnormal</subject><subject>Fibrinogens, Abnormal - genetics</subject><subject>Humans</subject><subject>Life Sciences</subject><subject>Pulmonary Embolism</subject><subject>Pulmonary Embolism - etiology</subject><subject>Thrombophilia</subject><subject>Thrombophilia - complications</subject><subject>Venous Thrombosis</subject><subject>Venous Thrombosis - blood</subject><issn>0041-4131</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo9kD1rwzAURTW0NCHNXyjeOrmVLEu2xpB-pBDokl08Sc-Nim25kl3Iv68hae5yl8OBe2_IktKS5SXjbEHWKX3TOZIyJdgdWRS0VrQuqiV5fjmlxpvo-_CFPXYeMuhdNh5j6ExIPj1lm8xCwiziEOJ4T24baBOuL70ih7fXw3aX7z_fP7abfX5UvMotB8alMygbcBRKa9AJapQA6pBbYQpTcmuROwGGguCNErYuZM0aWaqq5iuSn7VHaPUQfQfxpAN4vdvs9QBpxCnqeVBBVS1_2cw_nvkhhp8J06g7nyy2LfQYpqQrIaUsBecz-XAhJ9Ohu8r_L-F_QGRb6A</recordid><startdate>201010</startdate><enddate>201010</enddate><creator>Kraiem, Imen</creator><creator>Guermazi, Sami</creator><creator>Ben Abid, Héla</creator><creator>Meddeb, Balkis</creator><general>Maghreb-Editions; 1999</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope><scope>1XC</scope></search><sort><creationdate>201010</creationdate><title>Dysfibrinogenemia and thrombosis. A case report</title><author>Kraiem, Imen ; Guermazi, Sami ; Ben Abid, Héla ; Meddeb, Balkis</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-h937-c3a136dbe6fad0a4cbed50b95a0de3c5b2b43cce3d5ab0a53f95c82681f649783</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>fre</language><creationdate>2010</creationdate><topic>Adult</topic><topic>Afibrinogenemia</topic><topic>Afibrinogenemia - complications</topic><topic>Biochemistry, Molecular Biology</topic><topic>Fatal Outcome</topic><topic>Female</topic><topic>Fibrinogens, Abnormal</topic><topic>Fibrinogens, Abnormal - genetics</topic><topic>Humans</topic><topic>Life Sciences</topic><topic>Pulmonary Embolism</topic><topic>Pulmonary Embolism - etiology</topic><topic>Thrombophilia</topic><topic>Thrombophilia - complications</topic><topic>Venous Thrombosis</topic><topic>Venous Thrombosis - blood</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kraiem, Imen</creatorcontrib><creatorcontrib>Guermazi, Sami</creatorcontrib><creatorcontrib>Ben Abid, Héla</creatorcontrib><creatorcontrib>Meddeb, Balkis</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><jtitle>Tunisie Medicale</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kraiem, Imen</au><au>Guermazi, Sami</au><au>Ben Abid, Héla</au><au>Meddeb, Balkis</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Dysfibrinogenemia and thrombosis. A case report</atitle><jtitle>Tunisie Medicale</jtitle><addtitle>Tunis Med</addtitle><date>2010-10</date><risdate>2010</risdate><volume>88</volume><issue>10</issue><spage>757</spage><epage>760</epage><pages>757-760</pages><issn>0041-4131</issn><abstract>Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia.
To report a Tunisian case of the association dysfibrinogenemia and thrombosis.
A woman with inherited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significantly prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerisation. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded.
Although it is rare, this cause of thrombophilia must not be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplastin time and functional fibrinogen might be helpful.</abstract><cop>Tunisia</cop><pub>Maghreb-Editions; 1999</pub><pmid>20890827</pmid><tpages>4</tpages></addata></record> |
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| ispartof | Tunisie Medicale, 2010-10, Vol.88 (10), p.757-760 |
| issn | 0041-4131 |
| language | fre |
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| source | MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection |
| subjects | Adult Afibrinogenemia Afibrinogenemia - complications Biochemistry, Molecular Biology Fatal Outcome Female Fibrinogens, Abnormal Fibrinogens, Abnormal - genetics Humans Life Sciences Pulmonary Embolism Pulmonary Embolism - etiology Thrombophilia Thrombophilia - complications Venous Thrombosis Venous Thrombosis - blood |
| title | Dysfibrinogenemia and thrombosis. A case report |
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