Literature DB >> 25255238

A case of congenital afibrinogenemia complicated with thromboembolic events that required repeated amputations.

Mehmet A Ozdemir1, Bilgen Işik, Turkan Patiroglu, Musa Karakukcu, Fatma T Mutlu, Ebru Yilmaz, Ekrem Unal.   

Abstract

Although congenital afibrinogenemia is a rare autosomal recessive bleeding disorder, it can be more frequently encountered in countries where consanguineous marriages are common. Congenital afibrinogenemia is characterized by the undetectable low level of fibrinogen, which causes hemorrhagic diathesis. Paradoxically, arterial and venous thromboembolic complications can develop in patients with afibrinogenemia, which may cause a diagnostic problem to anyone unfamiliar with its clinical features. We report a case of congenital afibrinogenemia presenting with bilateral ischemic lesions of bilateral foot and ankle that required amputations. The patient was treated with fibrinogen concentrate, low-molecular-weight heparin, aspirin, and nifedipine. In conclusion, arterial and venous thromboembolic complications are rare, but severe complications of afibrinogenemia. The management of thromboembolic complications in patients with afibrinogenemia is a balance game. At one end of the scale, there is a bleeding disorder, and at the other end, there is a thrombosis. This fine adjustment is a job of mastery.

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Year:  2015        PMID: 25255238     DOI: 10.1097/MBC.0000000000000200

Source DB:  PubMed          Journal:  Blood Coagul Fibrinolysis        ISSN: 0957-5235            Impact factor:   1.276


  4 in total

Review 1.  Thrombosis in Inherited Fibrinogen Disorders.

Authors:  Wolfgang Korte; Man-Chiu Poon; Alfonso Iorio; Michael Makris
Journal:  Transfus Med Hemother       Date:  2017-03-14       Impact factor: 3.747

Review 2.  Genetic Variants in the FGB and FGG Genes Mapping in the Beta and Gamma Nodules of the Fibrinogen Molecule in Congenital Quantitative Fibrinogen Disorders Associated with a Thrombotic Phenotype.

Authors:  Tomas Simurda; Monika Brunclikova; Rosanna Asselta; Sonia Caccia; Jana Zolkova; Zuzana Kolkova; Dusan Loderer; Ingrid Skornova; Jan Hudecek; Zora Lasabova; Jan Stasko; Peter Kubisz
Journal:  Int J Mol Sci       Date:  2020-06-29       Impact factor: 5.923

Review 3.  Women With Congenital Hypofibrinogenemia/Afibrinogenemia: From Birth to Death.

Authors:  Yan Zhang; Xiaohang Zuo; Yue Teng
Journal:  Clin Appl Thromb Hemost       Date:  2020 Jan-Dec       Impact factor: 2.389

4.  Endovascular Treatment for Lower-extremity Arterial Thrombosis in a Patient with Congenital Afibrinogenemia and a History of Bleeding Complications.

Authors:  Daisuke Hiramatsu; Yoshito Ogihara; Takeshi Matsumoto; Kei Sato; Akihiro Takasaki; Tairo Kurita; Ryuji Okamoto; Hideo Wada; Kaoru Dohi
Journal:  Intern Med       Date:  2021-07-30       Impact factor: 1.271

  4 in total

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