Resolution of fibrinogen deficiency in a patient with congenital afibrinogenemia after liver transplantation

Gallastegui, Nicolas; Kimble, Erik; Harrington, Thomas J.

doi:10.1111/hae.12802

Cited by 8 publications

(5 citation statements)

References 12 publications

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“…To our knowledge, this case represents the only reported pediatric patient with congenital afibrinogenemia successfully treated with OLT, with only four reported cases to date in adult patients 17‐20 . These patients also suffered from other liver‐related comorbidities including Budd‐Chiari syndrome, liver cirrhosis, and hepatocellular carcinoma.…”

Section: Discussionmentioning

confidence: 85%

“…To our knowledge, this case represents the only reported pediatric patient with congenital afibrinogenemia successfully treated with OLT, with only four reported cases to date in adult patients. [17][18][19][20] These patients also suffered from other liver-related comorbidities including Budd-Chiari syndrome, liver cirrhosis, and hepatocellular carcinoma. Liver transplantation has been very successful in treating children with end-stage liver disease, offering the opportunity for a long, healthy life.…”

Section: Discussionmentioning

confidence: 99%

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Liver transplantation as a novel strategy for resolution of congenital afibrinogenemia in a pediatric patient

Corrales-Medina

Miloh

O’Farrell

et al. 2020

Journal of Thrombosis and Haemostasis

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Fibrinogen plays a central role in hemostasis, specifically in clot formation and stabilization. 1 Congenital fibrinogen disorders are rare, affecting ~1 to 2 in every million people worldwide. 2 Although healthy individuals have a plasma fibrinogen level ranging from 150 to 450 mg/dL, those with afibrinogenemia have undetectable levels of circulating fibrinogen. 3,4 Symptoms, including frequent and/or severe bleeding episodes, typically present in the neonatal period. 5,6 A study of 100 patients with congenital fibrinogen deficiency found that among patients with severe deficiency, hemarthroses were the most common type of bleed, and came with a risk of progressive musculoskeletal damage. 2 In patients with congenital afibrinogenemia, fibrinogen replacement therapy is recommended in case of acute hemorrhagic episodes and/or when undergoing surgical procedures. 7,8 Although not routinely used, long-term prophylaxis is recommended for those presenting with recurrent and severe bleeding episodes, and for female patients during pregnancy. 9

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Section: Discussionmentioning

confidence: 85%

“…To our knowledge, this case represents the only reported pediatric patient with congenital afibrinogenemia successfully treated with OLT, with only four reported cases to date in adult patients. [17][18][19][20] These patients also suffered from other liver-related comorbidities including Budd-Chiari syndrome, liver cirrhosis, and hepatocellular carcinoma. Liver transplantation has been very successful in treating children with end-stage liver disease, offering the opportunity for a long, healthy life.…”

Section: Discussionmentioning

confidence: 99%

Liver transplantation as a novel strategy for resolution of congenital afibrinogenemia in a pediatric patient

Corrales-Medina

Miloh

O’Farrell

et al. 2020

Journal of Thrombosis and Haemostasis

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“…8 Previous reports have documented donor-to-recipient transmission of protein C deficiency with dysfibrinogenemia, protein S, factor I (fibrinogen), factor VII, factor VIII, and factor XI deficiencies. [9][10][11][12][13][14][15][16] Indeed, OLT may correct hepatic coagulation abnormalities, including protein C, protein S, 10 hemophilia, 17,18,19 and antithrombin III deficiencies. In contrast, transplantation of livers with a synthetic defect of a particular factor such as protein C, protein S, or antithrombin III deficiency may impose a coagulation abnormality upon these recipients that is not benign ( Table 2).…”

Section: Discussionmentioning

confidence: 99%

Donor-to-recipient transmission of factor XII deficiency by orthotopic liver transplantation

Elsiesy

Shawakat

Al–Hamoudi

et al. 2019

Baylor University Medical Center Proceedings

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Transmission of congenital clotting factor deficiencies following orthotopic liver transplantation is rare. There has been one reported case of donor-to-recipient transmission of factor XII deficiency in a transplant, and we report the second case. KEYWORDS Coagulation; factor XII deficiency; liver transplantation; primary hyperoxaluria O rthotopic liver transplantation (OLT) may be associated with donor-transmitted deficiencies of coagulation factors due to lack of proteins synthesized by the liver. Some of these deficiencies do not have clinical significance, whereas others can lead to bleeding or thrombotic complications after OLT.

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“…La prise en charge est basée sur la prévention des traumatismes et une thérapeutique de substitution en période hémorragique ainsi qu’en prophylaxie [ 1 , 17 ]. Il a été observé après transplantation hépatique une résolution de l’afibrinogénémie congénitale [ 18 ]. La thérapie génique est une approche thérapeutique d’avenir.…”

Section: Discussionunclassified

Afibrinogénémie congénitale: à propos d’une observation

Assani¹,

Karboubi²,

Dakhama³

2016

Pan Afr Med J

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L’afibrinogénémie est une dyscrasie rare caractérisée par un déficit constitutionnel en fibrinogène. Elle se transmet sur un mode autosomique récessif. Les manifestations hémorragiques sont variables et peuvent mettre en jeu le pronostic vital. Un peu plus de 250 cas ont été publiés à nos jours. Nous rapportons un nouveau cas d’afibrinogénémie congénitale chez une enfant de 3 ans et demi hospitalisée pour hématémèse de moyenne abondance. A travers ce cas, nous rappelons les différents aspects de cette affection sur les plans clinique, biologique, génétique ainsi que thérapeutique.

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Resolution of fibrinogen deficiency in a patient with congenital afibrinogenemia after liver transplantation

Cited by 8 publications

References 12 publications

Liver transplantation as a novel strategy for resolution of congenital afibrinogenemia in a pediatric patient

Liver transplantation as a novel strategy for resolution of congenital afibrinogenemia in a pediatric patient

Donor-to-recipient transmission of factor XII deficiency by orthotopic liver transplantation

Afibrinogénémie congénitale: à propos d’une observation

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