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Fibrinogen deficiency

MedGen UID:
1372663
•Concept ID:
C4316812
•
Disease or Syndrome
Synonyms: Deficiency, Fibrinogen; Fibrinogen Deficiencies; Fibrinogen Deficiency
SNOMED CT: Fibrinogen deficiency (359727008)

Definition

A usually inherited blood coagulation disorder characterized by the partial or complete absence of fibrinogen in the blood, resulting in bleeding. [from NCI]

Professional guidelines

PubMed

Novel approaches in management of perioperative coagulopathy.
Tanaka KA, Bader SO, Görlinger K
Curr Opin Anaesthesiol 2014 Feb;27(1):72-80. doi: 10.1097/ACO.0000000000000025. PMID: 24263685
Epidemiology and treatment of congenital fibrinogen deficiency.
Peyvandi F
Thromb Res 2012 Dec;130 Suppl 2:S7-11. doi: 10.1016/S0049-3848(13)70004-5. PMID: 23439004
Treatment of congenital fibrinogen deficiency: overview and recent findings.
Tziomalos K, Vakalopoulou S, Perifanis V, Garipidou V
Vasc Health Risk Manag 2009;5:843-8. Epub 2009 Oct 12 doi: 10.2147/vhrm.s5305. PMID: 19851522Free PMC Article

Recent clinical studies

Etiology

Early and Empirical High-Dose Cryoprecipitate for Hemorrhage After Traumatic Injury: The CRYOSTAT-2 Randomized Clinical Trial.
Davenport R, Curry N, Fox EE, Thomas H, Lucas J, Evans A, Shanmugaranjan S, Sharma R, Deary A, Edwards A, Green L, Wade CE, Benger JR, Cotton BA, Stanworth SJ, Brohi K; CRYOSTAT-2 Principal Investigators
JAMA 2023 Nov 21;330(19):1882-1891. doi: 10.1001/jama.2023.21019. PMID: 37824155Free PMC Article
Haemostatic support in postpartum haemorrhage: A review of the literature and expert opinion.
Hofer S, Blaha J, Collins PW, Ducloy-Bouthors AS, Guasch E, Labate F, Lança F, Nyfløt LT, Steiner K, Van de Velde M
Eur J Anaesthesiol 2023 Jan 1;40(1):29-38. Epub 2022 Sep 22 doi: 10.1097/EJA.0000000000001744. PMID: 36131564Free PMC Article
Congenital Fibrinogen Deficiency in India and Role of Human Fibrinogen Concentrate.
John MJ, Byreddy P, Modak K, Makkar M
Acta Haematol 2021;144(6):595-602. Epub 2021 Jun 4 doi: 10.1159/000516339. PMID: 34091452
Fibrinogen Supplementation and Its Indications.
Grottke O, Mallaiah S, Karkouti K, Saner F, Haas T
Semin Thromb Hemost 2020 Feb;46(1):38-49. Epub 2019 Oct 1 doi: 10.1055/s-0039-1696946. PMID: 31574543
Afibrinogenemia Acquired by Liver Transplant.
Milani S, Aliakbarian M, Amouian S
Exp Clin Transplant 2019 Oct;17(5):692-694. Epub 2017 Sep 26 doi: 10.6002/ect.2016.0338. PMID: 28952920

Diagnosis

Haemostatic support in postpartum haemorrhage: A review of the literature and expert opinion.
Hofer S, Blaha J, Collins PW, Ducloy-Bouthors AS, Guasch E, Labate F, Lança F, Nyfløt LT, Steiner K, Van de Velde M
Eur J Anaesthesiol 2023 Jan 1;40(1):29-38. Epub 2022 Sep 22 doi: 10.1097/EJA.0000000000001744. PMID: 36131564Free PMC Article
Congenital afibrinogenemia in a 4-year-old girl complicated with acute lymphoblastic leukemia.
Özcan A, Samur B, Akyol Ş, Erdoğmuş NA, Patıroğlu T, Karakükçü M, Ünal E
Turk J Pediatr 2020;62(2):289-292. doi: 10.24953/turkjped.2020.02.017. PMID: 32419423
Unexpected uterine leiomyosarcoma in a woman with multiple myomas treated with ulipristal acetate: case report and literature review.
Modaffari P, D'alonzo M, Garbagnati M, Pecchio S, Menato G, Biglia N
Gynecol Endocrinol 2018 Mar;34(3):192-194. Epub 2017 Sep 21 doi: 10.1080/09513590.2017.1380186. PMID: 28933575
Thrombin clotting time.
Ignjatovic V
Methods Mol Biol 2013;992:131-8. doi: 10.1007/978-1-62703-339-8_10. PMID: 23546710
Rare bleeding disorders.
Peyvandi F, Cattaneo M, Inbal A, De Moerloose P, Spreafico M
Haemophilia 2008 Jul;14 Suppl 3:202-10. doi: 10.1111/j.1365-2516.2008.01751.x. PMID: 18510543

Therapy

Early and Empirical High-Dose Cryoprecipitate for Hemorrhage After Traumatic Injury: The CRYOSTAT-2 Randomized Clinical Trial.
Davenport R, Curry N, Fox EE, Thomas H, Lucas J, Evans A, Shanmugaranjan S, Sharma R, Deary A, Edwards A, Green L, Wade CE, Benger JR, Cotton BA, Stanworth SJ, Brohi K; CRYOSTAT-2 Principal Investigators
JAMA 2023 Nov 21;330(19):1882-1891. doi: 10.1001/jama.2023.21019. PMID: 37824155Free PMC Article
Haemostatic support in postpartum haemorrhage: A review of the literature and expert opinion.
Hofer S, Blaha J, Collins PW, Ducloy-Bouthors AS, Guasch E, Labate F, Lança F, Nyfløt LT, Steiner K, Van de Velde M
Eur J Anaesthesiol 2023 Jan 1;40(1):29-38. Epub 2022 Sep 22 doi: 10.1097/EJA.0000000000001744. PMID: 36131564Free PMC Article
Fibrinogen Supplementation and Its Indications.
Grottke O, Mallaiah S, Karkouti K, Saner F, Haas T
Semin Thromb Hemost 2020 Feb;46(1):38-49. Epub 2019 Oct 1 doi: 10.1055/s-0039-1696946. PMID: 31574543
Afibrinogenemia Acquired by Liver Transplant.
Milani S, Aliakbarian M, Amouian S
Exp Clin Transplant 2019 Oct;17(5):692-694. Epub 2017 Sep 26 doi: 10.6002/ect.2016.0338. PMID: 28952920
Hemostatic and thrombotic issues in cardiac surgery.
Ranucci M
Semin Thromb Hemost 2015 Feb;41(1):84-90. Epub 2015 Jan 15 doi: 10.1055/s-0034-1398383. PMID: 25590527

Prognosis

Trials and Tribulations of Viscoelastic-Based Determination of Fibrinogen Concentration.
Ranucci M, Di Dedda U, Baryshnikova E
Anesth Analg 2020 Mar;130(3):644-653. doi: 10.1213/ANE.0000000000004522. PMID: 31725023
Thromboelastography and Thromboelastometry in Assessment of Fibrinogen Deficiency and Prediction for Transfusion Requirement: A Descriptive Review.
Peng HT, Nascimento B, Beckett A
Biomed Res Int 2018;2018:7020539. Epub 2018 Nov 25 doi: 10.1155/2018/7020539. PMID: 30596098Free PMC Article
Inherited Bleeding Disorders in the Obstetric Patient.
Bannow BS, Konkle BA
Transfus Med Rev 2018 Oct;32(4):237-243. Epub 2018 Jul 12 doi: 10.1016/j.tmrv.2018.06.003. PMID: 30097224
Managing major obstetric haemorrhage: Pharmacotherapy and transfusion.
Collis R, Guasch E
Best Pract Res Clin Anaesthesiol 2017 Mar;31(1):107-124. Epub 2017 Feb 9 doi: 10.1016/j.bpa.2017.02.001. PMID: 28625299
Fibrinogen in craniosynostosis surgery.
Haas T, Fries D, Velik-Salchner C, Oswald E, Innerhofer P
Anesth Analg 2008 Mar;106(3):725-31, table of contents. doi: 10.1213/ane.0b013e318163fb26. PMID: 18292409

Clinical prediction guides

Congenital fibrinogen disorders: a retrospective clinical and genetic analysis of the Prospective Rare Bleeding Disorders Database.
Mohsenian S, Palla R, Menegatti M, Cairo A, Lecchi A, Casini A, Neerman-Arbez M, Asselta R, Scardo S, Siboni SM, Blatny J, Zapletal O, Schved JF, Giansily-Blaizot M, Halimeh S, Daoud MA, Platokouki H, Pergantou H, Schutgens REG, Van Haaften-Spoor M, Brons P, Laros-van Gorkom B, Van Pinxten E, Borhany M, Fatima N, Mikovic D, Saracevic M, Özdemir GN, Ay Y, Makris M, Lockley C, Mumford A, Harvey A, Austin S, Shapiro A, Williamson A, McGuinn C, Goldberg I, De Moerloose P, Peyvandi F
Blood Adv 2024 Mar 26;8(6):1392-1404. doi: 10.1182/bloodadvances.2023012186. PMID: 38286442Free PMC Article
Early and Empirical High-Dose Cryoprecipitate for Hemorrhage After Traumatic Injury: The CRYOSTAT-2 Randomized Clinical Trial.
Davenport R, Curry N, Fox EE, Thomas H, Lucas J, Evans A, Shanmugaranjan S, Sharma R, Deary A, Edwards A, Green L, Wade CE, Benger JR, Cotton BA, Stanworth SJ, Brohi K; CRYOSTAT-2 Principal Investigators
JAMA 2023 Nov 21;330(19):1882-1891. doi: 10.1001/jama.2023.21019. PMID: 37824155Free PMC Article
Trials and Tribulations of Viscoelastic-Based Determination of Fibrinogen Concentration.
Ranucci M, Di Dedda U, Baryshnikova E
Anesth Analg 2020 Mar;130(3):644-653. doi: 10.1213/ANE.0000000000004522. PMID: 31725023
Thromboelastography and Thromboelastometry in Assessment of Fibrinogen Deficiency and Prediction for Transfusion Requirement: A Descriptive Review.
Peng HT, Nascimento B, Beckett A
Biomed Res Int 2018;2018:7020539. Epub 2018 Nov 25 doi: 10.1155/2018/7020539. PMID: 30596098Free PMC Article
New developments in the treatment of pediatric hemophilia and bleeding disorders.
Branchford BR, Monahan PE, Di Paola J
Curr Opin Pediatr 2013 Feb;25(1):23-30. doi: 10.1097/MOP.0b013e32835bf77e. PMID: 23274430

Recent systematic reviews

Bone cysts in patients with afibrinogenaemia: a literature review and two new cases.
van Meegeren ME, de Rooy JW, Schreuder HW, Brons PP
Haemophilia 2014 Mar;20(2):244-8. Epub 2013 Nov 29 doi: 10.1111/hae.12337. PMID: 24533951
Systemic treatments for the prevention of venous thrombo-embolic events in paediatric cancer patients with tunnelled central venous catheters.
Schoot RA, Kremer LC, van de Wetering MD, van Ommen CH
Cochrane Database Syst Rev 2013 Sep 11;(9):CD009160. doi: 10.1002/14651858.CD009160.pub2. PMID: 24026801
Fibrinogen replacement therapy for congenital fibrinogen deficiency.
Bornikova L, Peyvandi F, Allen G, Bernstein J, Manco-Johnson MJ
J Thromb Haemost 2011 Sep;9(9):1687-704. doi: 10.1111/j.1538-7836.2011.04424.x. PMID: 21711446

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