{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:41:17Z","timestamp":1787020877363,"version":"build-2736575974"},"reference-count":10,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,5,7]],"date-time":"2021-05-07T00:00:00Z","timestamp":1620345600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,5,7]],"date-time":"2021-05-07T00:00:00Z","timestamp":1620345600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Med Case Reports"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      The plasma serine protease inhibitor alpha 2-antiplasmin (\u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP, otherwise known as \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -plasmin inhibitor) is a rapid-acting plasmin inhibitor recently found in human plasma, which seems to have a significant role in the regulation of\n                      <jats:italic>in vivo<\/jats:italic>\n                      fibrinolysis. Congenital deficiency of \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP is extremely uncommon.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>\n                      We report here a case of absolute deficiency of \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP in an 11-year-old Sudanese boy, who had a lifelong intermittent hemorrhagic tendency (gum bleeding, epistaxis, and exaggerated bleeding after trauma). Coagulation tests including prothrombin time, partial thromboplastin time, thrombin time, bleeding time, platelet count, clot retraction test, antithrombin, and factor VIII levels were within normal limits. Hepatic function tests and complete blood count were also normal. The main interesting finding in this patient was that the whole blood clot lysis was extremely fast, completed within 5\u20138 hours. The second abnormal finding is that the euglobulin clot lysis time was short. Nevertheless, the concentration of \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP in the patient's plasma was 0.2 IU\/ml (reference range is 0.80\u20131.20 IU\/ml). The addition of pooled plasma (with normal \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP) to the patient's whole blood corrected the accelerated fibrinolysis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      The study showed that \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP deficiency resulted in uninhibited fibrinolysis that caused the hemorrhagic tendency in this patient. Thus, this report demonstrates the significant role of \u03b1\n                      <jats:sub>2<\/jats:sub>\n                      -AP in coagulation.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13256-021-02813-6","type":"journal-article","created":{"date-parts":[[2021,5,6]],"date-time":"2021-05-06T19:05:54Z","timestamp":1620327954000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Alpha 2-antiplasmin deficiency in a Sudanese child: a case report"],"prefix":"10.1186","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5089-9531","authenticated-orcid":false,"given":"Bashir Abdrhman Bashir","family":"Mohammed","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,5,7]]},"reference":[{"key":"2813_CR1","doi-asserted-by":"publisher","first-page":"1250","DOI":"10.1111\/j.1365-2516.2008.01766.x","volume":"14","author":"SL Carpenter","year":"2008","unstructured":"Carpenter SL, Mathew P. Alpha2-antiplasmin and its deficiency: fibrinolysis out of balance. Haemophilia. 2008;14:1250\u20134.","journal-title":"Haemophilia"},{"issue":"2","key":"2813_CR2","first-page":"191","volume":"43","author":"GL Reed","year":"2017","unstructured":"Reed GL, Houng AK, Singh S, Wang D. Alpha2-antiplasmin: new insights and opportunities for ischemic stroke. SeminThrombHemost. 2017;43(2):191\u20139.","journal-title":"SeminThrombHemost."},{"issue":"5","key":"2813_CR3","doi-asserted-by":"publisher","first-page":"538","DOI":"10.1182\/blood-2015-09-670117","volume":"127","author":"S Abdul","year":"2016","unstructured":"Abdul S, Leebeek FW, Rijken DC, Willige SU. Natural heterogeneity of alpha2-antiplasmin: functional and clinical consequences. Blood. 2016;127(5):538\u201345.","journal-title":"Blood"},{"key":"2813_CR4","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1007\/s00277-016-2893-6","volume":"96","author":"J Gebhart","year":"2017","unstructured":"Gebhart J, Kepa S, Hofer S, Koder S, Kaider A, Wolberg AS, Haslacher H, Quehenberger P, Eigenbauer E, Panzer S, Mannhalter C, Pabinger I. Fibrinolysis in patients with a mild-to-moderate bleeding tendency of unknown cause. Ann Hematol. 2017;96:489\u201395.","journal-title":"Ann Hematol."},{"key":"2813_CR5","doi-asserted-by":"publisher","first-page":"1498","DOI":"10.1111\/jth.14160","volume":"16","author":"JL Saes","year":"2018","unstructured":"Saes JL, Schols SEM, van Heerde WL, Nijziel MR. Hemorrhagic disorders of fibrinolysis: aclinical review. J Thromb Haemost. 2018;16:1498\u2013509.","journal-title":"J Thromb Haemost."},{"key":"2813_CR6","doi-asserted-by":"publisher","first-page":"1209676","DOI":"10.1155\/2017\/1209676","volume":"2017","author":"DC Rijken","year":"2017","unstructured":"Rijken DC, Uitte de Willige S. Inhibition of fibrinolysis by coagulation factor XIII. Biomed Res Int. 2017;2017:1209676.","journal-title":"Biomed Res Int."},{"key":"2813_CR7","first-page":"93","volume":"71","author":"M Igala","year":"2013","unstructured":"Igala M, Oukkach B, Khoubila N, Faez S, Benchekroun S. A congenital alpha2-antiplasmin deficiency. Ann Biol Clin. 2013;71:93\u20135.","journal-title":"Ann Biol Clin"},{"key":"2813_CR8","doi-asserted-by":"publisher","first-page":"858293","DOI":"10.1155\/2014\/858293","volume":"2014","author":"S Yasar Yildiz","year":"2014","unstructured":"Yasar Yildiz S, Kuru P, Toksoy Oner E, Agirbasli M. Functionalstability of plasminogen activator inhibitor-1. Sci World J. 2014;2014:858293.","journal-title":"Sci World J."},{"key":"2813_CR9","doi-asserted-by":"publisher","first-page":"e237\u20139","DOI":"10.1111\/hae.12929","volume":"22","author":"J Hirose","year":"2016","unstructured":"Hirose J, Takedani H, Kubota M, Kinkawa J, Noguchi M. Total hip arthroplasty and total knee arthroplasty in a patientwith congenital deficiency of plasminogen activator inhibitor-1. Haemophilia. 2016;22:e237\u20139.","journal-title":"Haemophilia"},{"key":"2813_CR10","doi-asserted-by":"publisher","first-page":"1200","DOI":"10.1111\/j.1538-7836.2011.04288.x","volume":"9","author":"T Iwaki","year":"2011","unstructured":"Iwaki T, Tanaka A, Miyawaki Y, Suzuki A, Kobayashi T, Takamatsu J, Matsushita T, Umemura K, Urano T, Kojima T, Terao T, Kanayama N. Life-threatening hemorrhage and prolonged wound healing are remarkable phenotypes manifested bycomplete plasminogen activator inhibitor-1 deficiency inhumans. J ThrombHaemost. 2011;9:1200\u20136.","journal-title":"J ThrombHaemost"}],"container-title":["Journal of Medical Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-021-02813-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13256-021-02813-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13256-021-02813-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,6]],"date-time":"2021-05-06T19:35:02Z","timestamp":1620329702000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmedicalcasereports.biomedcentral.com\/articles\/10.1186\/s13256-021-02813-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,7]]},"references-count":10,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["2813"],"URL":"https:\/\/doi.org\/10.1186\/s13256-021-02813-6","relation":{},"ISSN":["1752-1947"],"issn-type":[{"value":"1752-1947","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,7]]},"assertion":[{"value":"24 August 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 March 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 May 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"This study did not include experiments on animals or humans. The patient gave consent to use his details for this case study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Written informed consent was obtained from the parents of the patient (the patient's legal guardians)\u00a0for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The author declares that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"238"}}