Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)

Nicole S Bartosh, Tara Tomlin, Christian Cable, Kathleen HalkaDepartment of Internal Medicine, Division of Medical Oncology, Scott and White Healthcare and Texas A and M Health Science Center College of Medicine, Temple, TX, USAAbstract: This case report presents a newly diagnosed congenital factor...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Bartosh NS, Tomlin T, Cable C, Halka K
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2013
Materias:	Therapeutics. Pharmacology RM1-950
Acceso en línea:	https://doaj.org/article/b4bf932b092349148cf13a2e0d2f0491
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:b4bf932b092349148cf13a2e0d2f0491
record_format	dspace
spelling	oai:doaj.org-article:b4bf932b092349148cf13a2e0d2f04912021-12-02T03:37:44ZNewly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)1179-1438https://doaj.org/article/b4bf932b092349148cf13a2e0d2f04912013-03-01T00:00:00Zhttp://www.dovepress.com/newly-diagnosed-congenital-factor-vii-deficiency-and-utilization-of-re-a12489https://doaj.org/toc/1179-1438Nicole S Bartosh, Tara Tomlin, Christian Cable, Kathleen HalkaDepartment of Internal Medicine, Division of Medical Oncology, Scott and White Healthcare and Texas A and M Health Science Center College of Medicine, Temple, TX, USAAbstract: This case report presents a newly diagnosed congenital factor VII deficiency treated with recombinant activated factor VII (rFVIIa). Congenital factor VII deficiency is a rare autosomal-recessive bleeding disorder that occurs in fewer than 1/500,000 persons. Its presentation can vary from epistaxis to hemarthroses and severe central nervous system bleeding, and correlates poorly with factor VII levels. Our patient had not had a significant hemostatic challenge prior to his presentation and therefore never had any symptomatology suggestive of this disease. He was treated with rFVIIa, and was able to undergo repair of his fractures without bleeding.Case report: A 19-year-old African-American male presented to the emergency room after an altercation that resulted in significant trauma. He sustained bilateral mandibular angle fractures and orbital floor fractures, requiring urgent surgical correction. On initial evaluation, he was noted to have a prolonged prothrombin time of 40.1 seconds, with an International Normalized Ratio of 4.0, a normal activated partial thromboplastin time of 29.9 seconds, and a platelet count of 241. After receiving vitamin K and fresh frozen plasma, he was taken to the operating room for a temporary rigid maxillomandibular fixation. A 1:1 mixing study with normal plasma corrected the prothrombin time (decreasing from 40.7 to 14.7 seconds) and a factor VII assay revealed 5% of the normal factor VII level. The patient was diagnosed with congenital factor VII deficiency. Due to his coagulopathy and the extensive surgical correction needed, rFVIIa was administered and surgery was accomplished without hemorrhagic sequelae.Conclusion: This case report and review describes a rare congenital disease, the history of rFVIIa use, and its mechanism. rFVIIA use in our patient provided a treatment option that allowed the necessary surgical correction, but further prospective studies on dose optimization would ensure adequate dosing with minimal risk of severe side effects.Keywords: factor VII deficiency, recombinant activated factor VII, coagulation cascadeBartosh NSTomlin TCable CHalka KDove Medical PressarticleTherapeutics. PharmacologyRM1-950ENClinical Pharmacology: Advances and Applications, Vol 2013, Iss Issue 1, Pp 53-58 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Therapeutics. Pharmacology RM1-950
spellingShingle	Therapeutics. Pharmacology RM1-950 Bartosh NS Tomlin T Cable C Halka K Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)
description	Nicole S Bartosh, Tara Tomlin, Christian Cable, Kathleen HalkaDepartment of Internal Medicine, Division of Medical Oncology, Scott and White Healthcare and Texas A and M Health Science Center College of Medicine, Temple, TX, USAAbstract: This case report presents a newly diagnosed congenital factor VII deficiency treated with recombinant activated factor VII (rFVIIa). Congenital factor VII deficiency is a rare autosomal-recessive bleeding disorder that occurs in fewer than 1/500,000 persons. Its presentation can vary from epistaxis to hemarthroses and severe central nervous system bleeding, and correlates poorly with factor VII levels. Our patient had not had a significant hemostatic challenge prior to his presentation and therefore never had any symptomatology suggestive of this disease. He was treated with rFVIIa, and was able to undergo repair of his fractures without bleeding.Case report: A 19-year-old African-American male presented to the emergency room after an altercation that resulted in significant trauma. He sustained bilateral mandibular angle fractures and orbital floor fractures, requiring urgent surgical correction. On initial evaluation, he was noted to have a prolonged prothrombin time of 40.1 seconds, with an International Normalized Ratio of 4.0, a normal activated partial thromboplastin time of 29.9 seconds, and a platelet count of 241. After receiving vitamin K and fresh frozen plasma, he was taken to the operating room for a temporary rigid maxillomandibular fixation. A 1:1 mixing study with normal plasma corrected the prothrombin time (decreasing from 40.7 to 14.7 seconds) and a factor VII assay revealed 5% of the normal factor VII level. The patient was diagnosed with congenital factor VII deficiency. Due to his coagulopathy and the extensive surgical correction needed, rFVIIa was administered and surgery was accomplished without hemorrhagic sequelae.Conclusion: This case report and review describes a rare congenital disease, the history of rFVIIa use, and its mechanism. rFVIIA use in our patient provided a treatment option that allowed the necessary surgical correction, but further prospective studies on dose optimization would ensure adequate dosing with minimal risk of severe side effects.Keywords: factor VII deficiency, recombinant activated factor VII, coagulation cascade
format	article
author	Bartosh NS Tomlin T Cable C Halka K
author_facet	Bartosh NS Tomlin T Cable C Halka K
author_sort	Bartosh NS
title	Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)
title_short	Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)
title_full	Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)
title_fullStr	Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)
title_full_unstemmed	Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&reg;)
title_sort	newly diagnosed congenital factor vii deficiency and utilization of recombinant activated factor vii (novoseven&reg;)
publisher	Dove Medical Press
publishDate	2013
url	https://doaj.org/article/b4bf932b092349148cf13a2e0d2f0491
work_keys_str_mv	AT bartoshns newlydiagnosedcongenitalfactorviideficiencyandutilizationofrecombinantactivatedfactorviinovosevenampreg AT tomlint newlydiagnosedcongenitalfactorviideficiencyandutilizationofrecombinantactivatedfactorviinovosevenampreg AT cablec newlydiagnosedcongenitalfactorviideficiencyandutilizationofrecombinantactivatedfactorviinovosevenampreg AT halkak newlydiagnosedcongenitalfactorviideficiencyandutilizationofrecombinantactivatedfactorviinovosevenampreg
_version_	1718401693384704000

Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven&amp;reg;)

Ejemplares similares

Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven®)