{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T22:16:17Z","timestamp":1789251377676,"version":"build-2803163510"},"reference-count":38,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2015,5,8]],"date-time":"2015-05-08T00:00:00Z","timestamp":1431043200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2015,11]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>The spectrum of bleeding problems in <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> deficiency is highly variable and <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> levels and causative genetic mutations correlate poorly with the bleeding risk. Long\u2010term prophylaxis is generally initiated in order to prevent subsequent <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content> bleeding after a first event or in patients with other major\/ life threatening\/ frequent bleeding symptoms as gastrointestinal bleeding or hemarthrosis. However few data are available in the literature regarding <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> prophylaxis and clinical decisions cannot be based on evidence.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>We report the data available in the literature on <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> prophylaxis and our personal experience regarding three patients affected by severe <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> deficiency.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Specific papers on long\u2010term prophylaxis in severe <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> deficiency were identified using the database, <jats:styled-content style=\"fixed-case\">PUBMED<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The most frequent indications for long\u2010term prophylaxis were <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content> bleeding (58%), hemartrosis (15%) and <jats:styled-content style=\"fixed-case\">GI<\/jats:styled-content> bleeding (9%). Patients were treated with various dosages and frequency. Prophylactic treatment with 10\u201030U\/kg (pd<jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content>) or 20\u201030mcg\/kg (<jats:styled-content style=\"fixed-case\">rFVII<\/jats:styled-content>a) twice or three times\/weeks was described to be effective.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In the literature and in our experience, prophylaxis can be considered in patients with severe <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> deficiency and severe bleeding phenotype. A dose of 10\u201030U\/kg (pd<jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content>) or 20\u201030 microg\/kg (<jats:styled-content style=\"fixed-case\">rFVII<\/jats:styled-content>a) twice or three times\/week is usually administrated, but dose and frequency can be tailored based on the clinical follow\u2010up of the patients. Since hemarthrosis is a frequent manifestation, a suggestion to improve the outcomes of patients with severe <jats:styled-content style=\"fixed-case\">FVII<\/jats:styled-content> deficiency is to monitor joint condition in order to identify early arthropathy that could be another indication to start secondary prophylaxis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/hae.12702","type":"journal-article","created":{"date-parts":[[2015,5,8]],"date-time":"2015-05-08T09:42:51Z","timestamp":1431078171000},"page":"812-819","source":"Crossref","is-referenced-by-count":22,"title":["Long\u2010term prophylaxis in severe factor <scp>VII<\/scp> deficiency"],"prefix":"10.1111","volume":"21","author":[{"given":"S. M.","family":"Siboni","sequence":"first","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Dipartimento delle Units Multispecialistiche e dei Trapianti Unit\u00e0 Operativa Complessa di Ematologia non Tumorale e Coagulopatie Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Biguzzi","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Dipartimento delle Units Multispecialistiche e dei Trapianti Unit\u00e0 Operativa Complessa di Ematologia non Tumorale e Coagulopatie Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Mistretta","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Dipartimento delle Units Multispecialistiche e dei Trapianti Unit\u00e0 Operativa Complessa di Ematologia non Tumorale e Coagulopatie Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Garagiola","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Dipartimento delle Units Multispecialistiche e dei Trapianti Unit\u00e0 Operativa Complessa di Ematologia non Tumorale e Coagulopatie Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Peyvandi","sequence":"additional","affiliation":[{"name":"Angelo Bianchi Bonomi Hemophilia and Thrombosis Center Dipartimento delle Units Multispecialistiche e dei Trapianti Unit\u00e0 Operativa Complessa di Ematologia non Tumorale e Coagulopatie Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico and University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,5,8]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0029-1225762"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00944.x"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02118.x"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-5-23"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04653.x"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.1997.00137.x"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.03545.x"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830100112"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02545.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/00001721-200302000-00016"},{"issue":"4","key":"e_1_2_9_12_1","first-page":"S1059","article-title":"Outcome of intracranial hemorrhage in infants with congenital factor VII deficiency","volume":"85","author":"Chuansumrit A","year":"2002","journal-title":"J Med Assoc Thai"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-07-0476"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.2830500408"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2008.03.010"},{"key":"e_1_2_9_16_1","first-page":"795","article-title":"Successful prophylaxis of intracranial hemorrhage in infants with severe congenital factor VII deficiency","volume":"31","author":"Kankirawatana S","year":"2000","journal-title":"Southeast Asian J Trop Med Public Health"},{"key":"e_1_2_9_17_1","first-page":"912","article-title":"Recombinant factor VIIa for long\u2010term replacement therapy in patients with congenital factor VII deficiency","volume":"10","author":"Huth\u2010K\u00fchne A","year":"2007","journal-title":"Thromb Haemost"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2007.02318.x"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2004.00922.x"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1080\/08880010500457939"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2004.04942.x"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00885.x"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20688"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2012.074039"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02471.x"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1177\/1076029611435091"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13106"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2008.01910.x"},{"key":"e_1_2_9_29_1","doi-asserted-by":"crossref","first-page":"5085","DOI":"10.1182\/blood.V87.12.5085.bloodjournal87125085","article-title":"Thr359Met mutation in factor VI1 of a patient with a hereditary deficiency causes defective secretion of the molecule","volume":"87","author":"Arbini AA","year":"1996","journal-title":"Blood"},{"key":"e_1_2_9_30_1","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1055\/s-0037-1614004","article-title":"Molecular characterisation and three\u2010dimensional structural analysis of mutations in 21 unrelated families with inherited factor VII deficiency","volume":"84","author":"Peyvand F","year":"2000","journal-title":"Thromb Haemost"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cca.2009.09.007"},{"key":"e_1_2_9_32_1","doi-asserted-by":"crossref","first-page":"1136","DOI":"10.1055\/s-0037-1615030","article-title":"Characterization of a factor VII molecule carrying a mutation in the second epidermal growth factor\u2010like domain","volume":"79","author":"Kavlie A","year":"1998","journal-title":"Thromb Haemost"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13106"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04844.x"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-02-0595"},{"key":"e_1_2_9_36_1","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1055\/s-0037-1613258","article-title":"The thrombogram in rare inherited coagulation disorders: its relation to clinical bleeding","volume":"88","author":"Al DR","year":"2002","journal-title":"Thromb Haemost"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.thromres.2013.05.027"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1179\/1607845412Y.0000000027"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12376"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12702","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12702","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T13:28:10Z","timestamp":1695562090000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12702"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,5,8]]},"references-count":38,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2015,11]]}},"alternative-id":["10.1111\/hae.12702"],"URL":"https:\/\/doi.org\/10.1111\/hae.12702","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,5,8]]}}}