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
Hauptverfasser: Windyga, J., Zbikowski, P., Ambroziak, P., Baran, B., Kotela, I., Stefanska-Windyga, E.
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container_issue 1
container_start_page 89
container_title Haemophilia : the official journal of the World Federation of Hemophilia
container_volume 19
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.
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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. 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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 &amp; 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 &amp; 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 &amp; 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 &amp; 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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