Management of factor VII-deficient patients undergoing joint surgeries - preliminary results of locally developed treatment regimen
Summary Inherited factor VII (FVII) deficiency is a rare coagulation disorder with variable haemorrhagic manifestations. In severely affected cases spontaneous haemarthroses leading to advanced arthropathy have been observed. Such cases may require surgery. Therapeutic options for bleeding preventio...
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Veröffentlicht in: | Haemophilia : the official journal of the World Federation of Hemophilia 2013-01, Vol.19 (1), p.89-93 |
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creator | Windyga, J. Zbikowski, P. Ambroziak, P. Baran, B. Kotela, I. Stefanska-Windyga, E. |
description | Summary
Inherited factor VII (FVII) deficiency is a rare coagulation disorder with variable haemorrhagic manifestations. In severely affected cases spontaneous haemarthroses leading to advanced arthropathy have been observed. Such cases may require surgery. Therapeutic options for bleeding prevention in FVII deficient patients undergoing surgery comprise various FVII preparations but the use of recombinant activated factor VII (rFVIIa) seems to be the treatment of choice. To present the outcome of orthopaedic surgery under haemostatic coverage of rFVIIa administered according to the locally established treatment regimen in five adult patients with FVII baseline plasma levels below 10 IU dL−1. Two patients required total hip replacement (THR); three had various arthroscopic procedures. Recombinant activated factor VII was administered every 8 h on day of surgery (D0) followed by every 12–24 h for the subsequent 9–14 days, depending on the type of surgery. Factor VII plasma coagulation activity (FVII:C) was determined daily with no predefined therapeutic target levels. Doses of rFVIIa on D0 ranged from 18 to 37 μg kg−1 b.w. and on the subsequent days – from 13 to 30 μg kg−1 b.w. Total rFVIIa dose per procedure ranged from 16 to 37.5 mg, and the total number of doses per procedure was 16–31. None of our patients developed excessive bleeding including those in whom FVII:C trough levels returned nearly to the baseline level on the first post‐op day. Preliminary results demonstrate that rFVIIa administered according to our treatment regimen is an effective and safe haemostatic agent for hypoproconvertinaemia patients undergoing orthopaedic surgery. |
doi_str_mv | 10.1111/j.1365-2516.2012.02921.x |
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Inherited factor VII (FVII) deficiency is a rare coagulation disorder with variable haemorrhagic manifestations. In severely affected cases spontaneous haemarthroses leading to advanced arthropathy have been observed. Such cases may require surgery. Therapeutic options for bleeding prevention in FVII deficient patients undergoing surgery comprise various FVII preparations but the use of recombinant activated factor VII (rFVIIa) seems to be the treatment of choice. To present the outcome of orthopaedic surgery under haemostatic coverage of rFVIIa administered according to the locally established treatment regimen in five adult patients with FVII baseline plasma levels below 10 IU dL−1. Two patients required total hip replacement (THR); three had various arthroscopic procedures. Recombinant activated factor VII was administered every 8 h on day of surgery (D0) followed by every 12–24 h for the subsequent 9–14 days, depending on the type of surgery. Factor VII plasma coagulation activity (FVII:C) was determined daily with no predefined therapeutic target levels. Doses of rFVIIa on D0 ranged from 18 to 37 μg kg−1 b.w. and on the subsequent days – from 13 to 30 μg kg−1 b.w. Total rFVIIa dose per procedure ranged from 16 to 37.5 mg, and the total number of doses per procedure was 16–31. None of our patients developed excessive bleeding including those in whom FVII:C trough levels returned nearly to the baseline level on the first post‐op day. Preliminary results demonstrate that rFVIIa administered according to our treatment regimen is an effective and safe haemostatic agent for hypoproconvertinaemia patients undergoing orthopaedic surgery.</description><identifier>ISSN: 1351-8216</identifier><identifier>EISSN: 1365-2516</identifier><identifier>DOI: 10.1111/j.1365-2516.2012.02921.x</identifier><identifier>PMID: 22845882</identifier><language>eng</language><publisher>England: Blackwell Publishing Ltd</publisher><subject>Adult ; Aged ; Blood Loss, Surgical - prevention & control ; Coagulants - therapeutic use ; factor VII deficiency ; Factor VII Deficiency - complications ; Factor VII Deficiency - drug therapy ; Factor VII Deficiency - surgery ; Factor VIIa - therapeutic use ; Female ; Hemostasis, Surgical - methods ; Humans ; joint surgery ; Male ; Middle Aged ; Orthopedics - methods ; Postoperative Hemorrhage - prevention & control ; recombinant activated factor VII ; Recombinant Proteins - therapeutic use ; Surgical Procedures, Operative - methods ; treatment regimen ; Young Adult</subject><ispartof>Haemophilia : the official journal of the World Federation of Hemophilia, 2013-01, Vol.19 (1), p.89-93</ispartof><rights>2012 Blackwell Publishing Ltd</rights><rights>2012 Blackwell Publishing Ltd.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5231-fc154f8dbdf42aa7a64af7f0b9ace9de226a4523806ef337c754644e7924031f3</citedby><cites>FETCH-LOGICAL-c5231-fc154f8dbdf42aa7a64af7f0b9ace9de226a4523806ef337c754644e7924031f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1365-2516.2012.02921.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1365-2516.2012.02921.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27903,27904,45553,45554</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22845882$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Windyga, J.</creatorcontrib><creatorcontrib>Zbikowski, P.</creatorcontrib><creatorcontrib>Ambroziak, P.</creatorcontrib><creatorcontrib>Baran, B.</creatorcontrib><creatorcontrib>Kotela, I.</creatorcontrib><creatorcontrib>Stefanska-Windyga, E.</creatorcontrib><title>Management of factor VII-deficient patients undergoing joint surgeries - preliminary results of locally developed treatment regimen</title><title>Haemophilia : the official journal of the World Federation of Hemophilia</title><addtitle>Haemophilia</addtitle><description>Summary
Inherited factor VII (FVII) deficiency is a rare coagulation disorder with variable haemorrhagic manifestations. In severely affected cases spontaneous haemarthroses leading to advanced arthropathy have been observed. Such cases may require surgery. Therapeutic options for bleeding prevention in FVII deficient patients undergoing surgery comprise various FVII preparations but the use of recombinant activated factor VII (rFVIIa) seems to be the treatment of choice. To present the outcome of orthopaedic surgery under haemostatic coverage of rFVIIa administered according to the locally established treatment regimen in five adult patients with FVII baseline plasma levels below 10 IU dL−1. Two patients required total hip replacement (THR); three had various arthroscopic procedures. Recombinant activated factor VII was administered every 8 h on day of surgery (D0) followed by every 12–24 h for the subsequent 9–14 days, depending on the type of surgery. Factor VII plasma coagulation activity (FVII:C) was determined daily with no predefined therapeutic target levels. Doses of rFVIIa on D0 ranged from 18 to 37 μg kg−1 b.w. and on the subsequent days – from 13 to 30 μg kg−1 b.w. Total rFVIIa dose per procedure ranged from 16 to 37.5 mg, and the total number of doses per procedure was 16–31. None of our patients developed excessive bleeding including those in whom FVII:C trough levels returned nearly to the baseline level on the first post‐op day. Preliminary results demonstrate that rFVIIa administered according to our treatment regimen is an effective and safe haemostatic agent for hypoproconvertinaemia patients undergoing orthopaedic surgery.</description><subject>Adult</subject><subject>Aged</subject><subject>Blood Loss, Surgical - prevention & control</subject><subject>Coagulants - therapeutic use</subject><subject>factor VII deficiency</subject><subject>Factor VII Deficiency - complications</subject><subject>Factor VII Deficiency - drug therapy</subject><subject>Factor VII Deficiency - surgery</subject><subject>Factor VIIa - therapeutic use</subject><subject>Female</subject><subject>Hemostasis, Surgical - methods</subject><subject>Humans</subject><subject>joint surgery</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Orthopedics - methods</subject><subject>Postoperative Hemorrhage - prevention & control</subject><subject>recombinant activated factor VII</subject><subject>Recombinant Proteins - therapeutic use</subject><subject>Surgical Procedures, Operative - methods</subject><subject>treatment regimen</subject><subject>Young Adult</subject><issn>1351-8216</issn><issn>1365-2516</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2013</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkU1v1DAQhi0Eakvbv4B87CXBX3GyBw5VKe2iAkKiH-rF8jrjyFvnAzuB3TN_HKdb9owPnlf2-8xoZhDClOQ0nffrnHJZZKygMmeEspywBaP55hU62n-8nnVBs4pReYjexrgmhHJG5AE6ZKwSRVWxI_Tni-50Ay10I-4tttqMfcB3y2VWg3XGze-DHucY8dTVEJredQ1ep3vEcQoNBAcRZ3gI4F3rOh22OECcfAJSRt8b7f0W1_ALfD9AjccAenwuGKBxSZygN1b7CKcv8Rjdfrr8cXGd3Xy7Wl6c32SmYJxm1tBC2Kpe1VYwrUsthbalJauFNrCogTGpRXJWRILlvDRlIaQQUC6YIJxafozOdnmH0P-cII6qddGA97qDfoqKJpYSURGRrNXOakIfYwCrhuDa1JqiRM0rUGs1T1rNk1bzCtTzCtQmoe9eqkyrFuo9-G_myfBhZ_jtPGz_O7G6Pr-cVeKzHe_iCJs9r8OTkiUvC3X_9Uo9fvz--eHxXijC_wLwS6bB</recordid><startdate>201301</startdate><enddate>201301</enddate><creator>Windyga, J.</creator><creator>Zbikowski, P.</creator><creator>Ambroziak, P.</creator><creator>Baran, B.</creator><creator>Kotela, I.</creator><creator>Stefanska-Windyga, E.</creator><general>Blackwell Publishing Ltd</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>201301</creationdate><title>Management of factor VII-deficient patients undergoing joint surgeries - preliminary results of locally developed treatment regimen</title><author>Windyga, J. ; Zbikowski, P. ; Ambroziak, P. ; Baran, B. ; Kotela, I. ; Stefanska-Windyga, E.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5231-fc154f8dbdf42aa7a64af7f0b9ace9de226a4523806ef337c754644e7924031f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2013</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Blood Loss, Surgical - prevention & control</topic><topic>Coagulants - therapeutic use</topic><topic>factor VII deficiency</topic><topic>Factor VII Deficiency - complications</topic><topic>Factor VII Deficiency - drug therapy</topic><topic>Factor VII Deficiency - surgery</topic><topic>Factor VIIa - therapeutic use</topic><topic>Female</topic><topic>Hemostasis, Surgical - methods</topic><topic>Humans</topic><topic>joint surgery</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Orthopedics - methods</topic><topic>Postoperative Hemorrhage - prevention & control</topic><topic>recombinant activated factor VII</topic><topic>Recombinant Proteins - therapeutic use</topic><topic>Surgical Procedures, Operative - methods</topic><topic>treatment regimen</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Windyga, J.</creatorcontrib><creatorcontrib>Zbikowski, P.</creatorcontrib><creatorcontrib>Ambroziak, P.</creatorcontrib><creatorcontrib>Baran, B.</creatorcontrib><creatorcontrib>Kotela, I.</creatorcontrib><creatorcontrib>Stefanska-Windyga, E.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Haemophilia : the official journal of the World Federation of Hemophilia</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Windyga, J.</au><au>Zbikowski, P.</au><au>Ambroziak, P.</au><au>Baran, B.</au><au>Kotela, I.</au><au>Stefanska-Windyga, E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Management of factor VII-deficient patients undergoing joint surgeries - preliminary results of locally developed treatment regimen</atitle><jtitle>Haemophilia : the official journal of the World Federation of Hemophilia</jtitle><addtitle>Haemophilia</addtitle><date>2013-01</date><risdate>2013</risdate><volume>19</volume><issue>1</issue><spage>89</spage><epage>93</epage><pages>89-93</pages><issn>1351-8216</issn><eissn>1365-2516</eissn><abstract>Summary
Inherited factor VII (FVII) deficiency is a rare coagulation disorder with variable haemorrhagic manifestations. In severely affected cases spontaneous haemarthroses leading to advanced arthropathy have been observed. Such cases may require surgery. Therapeutic options for bleeding prevention in FVII deficient patients undergoing surgery comprise various FVII preparations but the use of recombinant activated factor VII (rFVIIa) seems to be the treatment of choice. To present the outcome of orthopaedic surgery under haemostatic coverage of rFVIIa administered according to the locally established treatment regimen in five adult patients with FVII baseline plasma levels below 10 IU dL−1. Two patients required total hip replacement (THR); three had various arthroscopic procedures. Recombinant activated factor VII was administered every 8 h on day of surgery (D0) followed by every 12–24 h for the subsequent 9–14 days, depending on the type of surgery. Factor VII plasma coagulation activity (FVII:C) was determined daily with no predefined therapeutic target levels. Doses of rFVIIa on D0 ranged from 18 to 37 μg kg−1 b.w. and on the subsequent days – from 13 to 30 μg kg−1 b.w. Total rFVIIa dose per procedure ranged from 16 to 37.5 mg, and the total number of doses per procedure was 16–31. None of our patients developed excessive bleeding including those in whom FVII:C trough levels returned nearly to the baseline level on the first post‐op day. Preliminary results demonstrate that rFVIIa administered according to our treatment regimen is an effective and safe haemostatic agent for hypoproconvertinaemia patients undergoing orthopaedic surgery.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>22845882</pmid><doi>10.1111/j.1365-2516.2012.02921.x</doi><tpages>5</tpages><oa>free_for_read</oa></addata></record> |
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subjects | Adult Aged Blood Loss, Surgical - prevention & control Coagulants - therapeutic use factor VII deficiency Factor VII Deficiency - complications Factor VII Deficiency - drug therapy Factor VII Deficiency - surgery Factor VIIa - therapeutic use Female Hemostasis, Surgical - methods Humans joint surgery Male Middle Aged Orthopedics - methods Postoperative Hemorrhage - prevention & control recombinant activated factor VII Recombinant Proteins - therapeutic use Surgical Procedures, Operative - methods treatment regimen Young Adult |
title | Management of factor VII-deficient patients undergoing joint surgeries - preliminary results of locally developed treatment regimen |
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