Management of acute bleeding in a patient with congenital afibrinogenaemia

Vakalopoulou, Sofia; Rizopoulou, D.; Zafiriadou, E.; Perifanis, Vassilios; Τζιόμαλος, Κωνσταντίνος; Lefkou, Eleftheria; Hill, Marium; Dolan, Gerard; Garipidou, Vassilia

doi:10.1111/j.1365-2516.2006.01340.x

Cited by 13 publications

(11 citation statements)

References 7 publications

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“…Whilst efficacy of fibrinogen concentrates in treating bleeding episodes is well recognized, there is no evidence on the target fibrinogen level that should be achieved according to various clinical situations . In a recent systematic review including the management of 20 acute bleeds in afibrinogenaemic patients, fibrinogen level varied between >0.5 and 2.0 g L −1 .…”

Section: Discussionmentioning

confidence: 83%

Management of congenital quantitative fibrinogen disorders: a Delphi consensus

Casini

Moerloose

2016

Haemophilia

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Section: Discussionmentioning

confidence: 83%

Management of congenital quantitative fibrinogen disorders: a Delphi consensus

Casini

Moerloose

2016

Haemophilia

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“…Musculoskeletal bleeding (including hemarthroses) is observed in around half of the patients3,11 and in some series it was more frequent than mucosal bleeding 12. These discrepant results on the relative frequency of bleeding complications are partly due to the small number of patients included in the reported series 3,12.…”

Section: Clinical Manifestationsmentioning

confidence: 95%

“…The presence of modifier genes (yet unidentified) or the coexistence of thrombophilic disorders has been implicated as an explanation for this variability 21. Besides spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures are other principal manifestations of afibrinogenemia 2,3,11…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Treatment of congenital fibrinogen deficiency: overview and recent findings

Τζιόμαλος

Vakalopoulou²,

Perifanis³

et al. 2009

VHRM

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Afibrinogenemia is a rare bleeding disorder with an estimated prevalence of 1:1,000,000. It is an autosomal recessive disease resulting from mutations in any of the 3 genes that encode the 3 polypeptide chains of fibrinogen and are located on the long arm of chromosome 4. Spontaneous bleeding, bleeding after minor trauma and excessive bleeding during interventional procedures are the principal manifestations. We review the management of afibrinogenemia. Replacement therapy is the mainstay of treatment of bleeding episodes in these patients and plasma-derived fibrinogen concentrate is the agent of choice. Cryoprecipitate and fresh frozen plasma are alternative treatments that should be used only when fibrinogen concentrate is not available. Secondary prophylactic treatment may be considered after life-threatening bleeding whereas primary prophylactic treatment is not currently recommended. We also discuss alternative treatment options and the management of surgery, pregnancy and thrombosis in these patients. The development of new tests to identify higher risk patients and of safer replacement therapy will improve the management of afibrinogenemia in the future.

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“…Recurrent miscarriages are not uncommon. Management of haemorrhage in afibrinogenaemic patients is based on the administration of fibrinogen concentrates, if available, or of cryoprecipitates [2,3]. Inactivated plasma-derived concentrates of fibrinogen are available in France (Clottagen s ; LFB, Lille, France) and Germany (Haemocomplettan s P; ZLB Berhring, Marburg, Germany).…”

Section: Discussionmentioning

confidence: 99%

“…The risk of abnormal bleeding during a surgical procedure is high but can be avoided by the administration of fibrinogen concentrates [2,3]. The administration of coagulation proteins in patients deficient in coagulation factors can be complicated by venous or arterial thrombosis [1].…”

mentioning

confidence: 99%

Postoperative deep venous thrombosis in a woman with congenital afibrinogenaemia treated with fibrinogen concentrates

Haberer

Obstler

Samama

et al. 2008

European Journal of Anaesthesiology

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on heart rate and blood pressure following laryngoscopy and intubation. AANA J 2000; 68: 437-442. 3. Kovac AL, Bennets PS, Ohara S, LaGreca BA, Khan JA, Calkins JW. Effect of esmolol on hemodynamics and intraocular pressure response to succinylcholine and intubation following low-dose alfentanil premedication. J Clin Anesth 1992; 4: 315-320. 4. Jaakola ML, Ali-Melkkila T, Kanto J, Kallio A, Scheinin H, Scheinin M. Dexmedetomidine reduces intraocular pressure, intubation responses and anaesthetic requirements in patients undergoing ophthalmic surgery. Br J Anaesth 1992; 68: 570-575. 5. Dutta S, Karol MD, Cohen T, Jones RM, Mant T. Effect of dexmedetomidine on propofol requirements in healthy subjects. J Pharm Sci 2001; 90: 172-181. 6. Peden CJ, Cloote AH, Stratford N, Prys-Roberts C. The effect of intravenous dexmedetomidine premedication on the dose requirement of propofol to induce loss of consciousness in patients receiving alfentanil. Anaesthesia 2001; 56: 408-413.

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Management of acute bleeding in a patient with congenital afibrinogenaemia

Cited by 13 publications

References 7 publications

Management of congenital quantitative fibrinogen disorders: a Delphi consensus

Management of congenital quantitative fibrinogen disorders: a Delphi consensus

Treatment of congenital fibrinogen deficiency: overview and recent findings

Postoperative deep venous thrombosis in a woman with congenital afibrinogenaemia treated with fibrinogen concentrates

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