{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:32:37Z","timestamp":1790814757693,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,11,29]],"date-time":"2013-11-29T00:00:00Z","timestamp":1385683200000},"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":[[2014,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Afibrinogenaemia is an autosomal recessive disease with an estimated prevalence of approximately one in a million. The most common symptoms of afibrinogenaemia are umbilical cord bleeding, bleeding into skin, mouth, muscles, gastrointestinal and genitourinary tracts and the central nervous system. Other recognized complications include; haemarthroses, spontaneous splenic rupture, epistaxis, menorrhagia, recurrent abortion and venous and arterial thromboembolism. Bone cysts have also been described as a rare complication of afibrinogenaemia. The aim of this study was to conduct a systematic literature review, summarize the reported cases and to report two new cases. Three electronic databases were searched for relevant publications: PubMed, Medline and <jats:styled-content style=\"fixed-case\">EMBASE<\/jats:styled-content>. The following search criteria were used: \u2018(bone cysts <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> intraosseous haematoma <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> intraosseous haemorrhage) <jats:styled-content style=\"fixed-case\">AND<\/jats:styled-content> (afibrinogenaemia <jats:styled-content style=\"fixed-case\">OR<\/jats:styled-content> fibrinogen deficiency)\u2019. The reference lists of the selected papers were searched for more relevant literature. In total, eight patients had bone cysts as complication of afibrinogenaemia and six of them suffered from pain in their extremities. Bone cysts were primarily located in the vicinity of the cortex or trabeculae in the diaphysis of the long bones, especially in the femora, tibiae and humeri. Some were regressive, probably due to reactive bone remodelling. A number of cysts were filled with serosanguinous fluid. It might be useful to check for bone cysts when patients with congenital afibrinogenaemia complain of \u2018rheumatic\u2019 pains in their extremities. Whole body magnetic resonance imaging is the diagnostic imaging technique of choice. Recurrent episodes of pain, but not radiological deterioration, appear to benefit from prophylactic therapy with fibrinogen concentrate.<\/jats:p>","DOI":"10.1111\/hae.12337","type":"journal-article","created":{"date-parts":[[2013,11,29]],"date-time":"2013-11-29T11:48:54Z","timestamp":1385725734000},"page":"244-248","source":"Crossref","is-referenced-by-count":13,"title":["Bone cysts in patients with afibrinogenaemia: a literature review and two new cases"],"prefix":"10.1111","volume":"20","author":[{"given":"M. E. R.","family":"van Meegeren","sequence":"first","affiliation":[{"name":"Haemophilia Treatment Center Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. W. J.","family":"de Rooy","sequence":"additional","affiliation":[{"name":"Department of Radiology Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. W. B.","family":"Schreuder","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic surgery Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P. P. T.","family":"Brons","sequence":"additional","affiliation":[{"name":"Haemophilia Treatment Center Radboud University Medical Center Nijmegen The Netherlands"},{"name":"Department of Paediatrics Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,11,29]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(13)70004-5"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1349222"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00944.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2004.04.003"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1159\/000204533"},{"key":"e_1_2_7_7_1","first-page":"1013","article-title":"[Bone manifestations of congenital afibrinogenemia. Anatomo\u2010radiologic study of a case]","volume":"75","author":"Strijthem N","year":"1967","journal-title":"La Presse medicale"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00580596"},{"key":"e_1_2_7_9_1","first-page":"463","article-title":"[Congenital afibrinemia in 2 brothers with bone and hepatic lesions]","volume":"20","author":"Lemoine P","year":"1963","journal-title":"Arch Fr Pediatr"},{"key":"e_1_2_7_10_1","first-page":"263","article-title":"[Intra\u2010osseus hemorrhagic lesions in congenital afibrinogenemia. A new case (author's transl)]","volume":"62","author":"Zenny JC","year":"1981","journal-title":"J Radiol"},{"key":"e_1_2_7_11_1","first-page":"291","article-title":"[Congenital afibrinogenemia with bleeding, bone cysts and antibodies to fibrinogen]","volume":"121","author":"Ra'anani P","year":"1991","journal-title":"Harefuah"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2009.02033.x"},{"key":"e_1_2_7_13_1","unstructured":"BronsP ThomsonR VerbruggenB DeRooyJ VethR.Results of treatment with long term haemocomplettan in two patients with congenital afibrinogenemia and multiple bone cysts. Conference abstract: 22nd Congress of the International Society of Thrombosis and Haemostasis Boston MA United States2009;7:1156."},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.183.1.1830055"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002560050268"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1159\/000208261"},{"key":"e_1_2_7_17_1","first-page":"46","article-title":"[Hemorrhage in the epiphyseal region of the long bones and its relation to the pathogenesis of isolated cysts and brown tumors of bone]","volume":"15","author":"Cottier H","year":"1952","journal-title":"Schweiz Z Pathol Bakteriol"},{"key":"e_1_2_7_18_1","first-page":"149","article-title":"Mutations in the fibrinogen A\u03b1 gene account for the majority of cases of congenital afibrinogenemia","volume":"96","author":"Neerman\u2010Arbez M","year":"2000","journal-title":"Blood"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2002.377910.x"}],"container-title":["Haemophilia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fhae.12337","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/hae.12337","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T15:26:26Z","timestamp":1695569186000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/hae.12337"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,11,29]]},"references-count":18,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2014,3]]}},"alternative-id":["10.1111\/hae.12337"],"URL":"https:\/\/doi.org\/10.1111\/hae.12337","archive":["Portico"],"relation":{},"ISSN":["1351-8216","1365-2516"],"issn-type":[{"value":"1351-8216","type":"print"},{"value":"1365-2516","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,11,29]]}}}