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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

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
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
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

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
Maternal fibrinogen is necessary for embryonic development.
Iwaki T, Castellino FJ
Curr Drug Targets 2005 Aug;6(5):535-9. doi: 10.2174/1389450054546006. PMID: 16026273

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
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

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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