{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,14]],"date-time":"2024-07-14T12:59:01Z","timestamp":1720961941785},"reference-count":15,"publisher":"Index Copernicus","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pol Przegl Chir"],"published-print":{"date-parts":[[2020,4,16]]},"abstract":"<b>Introduction: <\/b>Gastrointestinal bleeding is a common disease that surgeons encounter in everyday clinical practice. It is most often easy to diagnose and treat. However, rare causes of bleeding can lead to delayed diagnosis and ineffective treatment. Dysfibrinogenemia is a qualitative fibrinogen disorder in which functional fibrinogen level is reduced with normal antigenic level. <br><b> Case report:<\/b> Herein we present the case of a 59-year-old female with recurrent gastrointestinal bleeds, that turned out to be an unusual manifestation of congenital dysfibrinogenemia. Detailed imaging and endoscopic diagnostics revealed portal hypertension with a non-bleeding 1-cm gastrointestinal stromal tumor and multiple angiodysplastic lesions in close proximity.\n\n<\/jats:p>","DOI":"10.5604\/01.3001.0014.0948","type":"journal-article","created":{"date-parts":[[2020,5,14]],"date-time":"2020-05-14T07:41:30Z","timestamp":1589442090000},"page":"1-5","source":"Crossref","is-referenced-by-count":2,"title":["Congenital dysfibrinogenemia as a rare cause\nof recurrent gastrointestinal bleeding"],"prefix":"10.5604","volume":"93","author":[{"given":"Weronika","family":"Lebowa","sequence":"first","affiliation":[{"name":"Klinika Chirurgii Og\u00f3lnej, Onkologicznej i Geriatrycznej, Collegium Medicum Uniwersytetu Jagiello\u0144skiego, Krak\u00f3w, Polska"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jakub","family":"Kenig","sequence":"additional","affiliation":[{"name":"Klinika Chirurgii Og\u00f3lnej, Onkologicznej i Geriatrycznej, Collegium Medicum Uniwersytetu Jagiello\u0144skiego, Krak\u00f3w, Polska"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna","family":"Zdziarska","sequence":"additional","affiliation":[{"name":"Katedra Hematologii, Collegium Medicum Uniwersytetu Jagiello\u0144skiego, Krak\u00f3w, Polska"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3689","reference":[{"key":"202640r1","unstructured":"Castaman G., Giacomelli S.H., Biasoli C., Contino L., Radossi P.: Risk of Bleeding and Thrombosis inInherited Qualitative Fibrinogen Disorders. Eur JHaematol., 2019; 103(4): 379\u2013384."},{"key":"202640r2","unstructured":"Caimia G., Rasoa S., Napolitanoa M., Hoppsa E., Lo Prestib R., Siragusa S.:Haemorheological profile in congenital afibrinogenemia and in congenitaldysfibrinogenemia: A clinical case report. Clin Hemorheol Microcirc., 2019;73(4): 523\u2013530."},{"key":"202640r3","unstructured":"Neerman-Arbez M., de Moerloose P., Casini A.: Laboratory and Genetic Investigation of Mutations Accounting for Congenital Fibrinogen Disorders.Semin Thromb Hemost., 2016; 42(4): 356\u2013365."},{"key":"202640r4","unstructured":"Toskes P.P., Wagger G.M., Raskin H.F., Attar S.: Coagulation Defects in Undiagnosed Recurrent Gastrointestinal Bleeding. Ann. Surg., 1970; 172(1): 98\u2013108."},{"key":"202640r5","unstructured":"Shapiro S.E., Phillips E., Manning R.A., Morse C.V., Murden S.L., Laffan M.A.et al.: Clinical phenotype, laboratory features and genotype of 35 patients withheritable dysfibrinogenaemia. Br J Haematol., 2013; 160(2): 220\u2013227."},{"key":"202640r6","unstructured":"Zhou J., Zhu P., Zhang X.: A Chinese family with congenital Dysfirinogenemia carries a heterozygous missense mutation in FGA: Concerning the genetic abnormality and clinical treatment. Pak J Med Sci., 2017; 33(4): 968\u2013972."},{"key":"202640r7","unstructured":"Casini A., de Moerloose P., Neerman-Arbez M.: Clinical features and management of congenital firinogen deficiencies. Semin Thromb Hemost., 2016;42(4): 366\u2013374."},{"key":"202640r8","unstructured":"Wypasek E., Klukowska A., Zdziarska J. et al.: Genetic and clinical characterization of congenital firinogen disorders in Polish patients: Identifiation of threenovel firinogen gamma chain mutations. Thromb Res., 2019; 182: 133\u2013140."},{"key":"202640r9","unstructured":"Casini A., Neerman-Arbez M., Ariens R.A., de Moerloose P.: Dysfibrinogenemia: from molecular anomalies to clinical manifestations and management. JThromb Haemost., 2015; 13(6): 909\u2013919."},{"key":"202640r10","unstructured":"Kim B.S., Li B.T., Engel A., Samara J.S.: Diagnosis of gastrointestinal bleeding: A practical guide for clinicians. World J Gastrointest Pathophysiol., 2014; 5(4): 467\u2013478."},{"key":"202640r11","unstructured":"Undas A., Zabczyk M., Iwaniec T.: Dysfibrinogenemia: from bleeding tendencyto thromboembolic disorders. Boletim da SPHM, 2011; 26: 5\u201317."},{"key":"202640r12","unstructured":"Morris E.S., Hampton K.K., Nesbitt I.M., Preston F.E., Thomas W.E.G., MakrisM.: The management of von Willebrand\u2019s disease-associated gastrointestinalangiodysplasia. Blood Coagul Fibrinolysis., 2001; 12(2): 143\u2013148."},{"key":"202640r13","unstructured":"Robert F., Desroches-Castan A., Bailly S., Dupuis-Girod S., Feige J.J.: Future treatments for hereditary hemorrhagic telangiectasia. Orphanet. J Rare Dis., 2020; 15(1): 4."},{"key":"202640r14","unstructured":"Ou G., Galorport C., Enns R.: Bevacizumab and gastrointestinal bleedingin hereditary hemorrhagic telangiectasia. World J Gastrointest Sur., 2016;8(12): 792\u2013795."},{"key":"202640r15","unstructured":"de Moerloose P., Neerman-Arbez M.: Treatment of congenital fibrinogen disorders. Expert Opin Biol Ther., 2008; 8(7): 979\u2013992."}],"container-title":["Polish Journal of Surgery"],"original-title":[],"link":[{"URL":"https:\/\/ppch.pl\/gicid\/pdf\/01.3001.0014.0948","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/ppch.pl\/gicid\/pdf\/01.3001.0014.0948","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,16]],"date-time":"2021-03-16T15:51:22Z","timestamp":1615909882000},"score":1,"resource":{"primary":{"URL":"https:\/\/ppch.pl\/gicid\/01.3001.0014.0948"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4,16]]},"references-count":15,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,4,3]]}},"URL":"https:\/\/doi.org\/10.5604\/01.3001.0014.0948","relation":{},"ISSN":["0032-373X","2299-2847"],"issn-type":[{"value":"0032-373X","type":"print"},{"value":"2299-2847","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,4,16]]}}}