Congenital Prothrombin Deficiency

Congenital prothrombin deficiency is an extremely rare hemorrhagic disorder with estimated prevalence of 1 per 2,000,000 in the general population. Since the disorder is an autosomal recessive disorder, the disorder is more frequent in areas with high rate of consanguinity. Clinical manifestations o...

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Main Authors: Maryam Daneshi, Tohid Naderi, Shadi Tabibian, Mahmood Shams, Jamal Rashidpanah, Akbar Dorgalaleh
Format: Article
Language:English
Published: Shahid Beheshti University of Medical Sciences 2019-02-01
Series:Journal of Cellular and Molecular Anesthesia
Online Access:http://journals.sbmu.ac.ir/jcma/article/view/23494
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spelling doaj-e4d79e0230364d3e882856dae20489e42020-11-25T01:24:11ZengShahid Beheshti University of Medical SciencesJournal of Cellular and Molecular Anesthesia2476-51202019-02-013414615410.22037/jcma.v3i4.2349411354Congenital Prothrombin DeficiencyMaryam Daneshi0Tohid Naderi1Shadi Tabibian2Mahmood Shams3Jamal Rashidpanah4Akbar Dorgalaleh5Department of Hematology and Blood transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, IranDepartment of Hematology and Blood transfusion, School of Allied Medicine, Shahid Beheshti University of Medical Sciences, Tehran, IranDepartment of Hematology and Blood transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, IranDepartment of Hematology and Blood transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, IranShariati hospital, Tehran University of Medical Sciences, Tehran, IranDepartment of Hematology and Blood transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, IranCongenital prothrombin deficiency is an extremely rare hemorrhagic disorder with estimated prevalence of 1 per 2,000,000 in the general population. Since the disorder is an autosomal recessive disorder, the disorder is more frequent in areas with high rate of consanguinity. Clinical manifestations of disorder are highly variable ranging from mild bleeding episodes to severe life-threatening hemorrhages. The disorder can be diagnosed based on routine and specific tests. No specific factor II concentrate is available, but patients can receive fresh frozen plasma and prothrombin complex concentrate (PCC). Traditionally patients with prothrombin deficiency receive on-demand therapy, but secondary prophylaxis can be used for those patients with high risk of severe life-threatening bleeding. With timely diagnosis and appropriate management of disorder, the quality of life in these patients can significantly improve.    Keywords: Prothrombin deficiency, Clinical manifestations, Diagnosis, Treatmenthttp://journals.sbmu.ac.ir/jcma/article/view/23494
collection DOAJ
language English
format Article
sources DOAJ
author Maryam Daneshi
Tohid Naderi
Shadi Tabibian
Mahmood Shams
Jamal Rashidpanah
Akbar Dorgalaleh
spellingShingle Maryam Daneshi
Tohid Naderi
Shadi Tabibian
Mahmood Shams
Jamal Rashidpanah
Akbar Dorgalaleh
Congenital Prothrombin Deficiency
Journal of Cellular and Molecular Anesthesia
author_facet Maryam Daneshi
Tohid Naderi
Shadi Tabibian
Mahmood Shams
Jamal Rashidpanah
Akbar Dorgalaleh
author_sort Maryam Daneshi
title Congenital Prothrombin Deficiency
title_short Congenital Prothrombin Deficiency
title_full Congenital Prothrombin Deficiency
title_fullStr Congenital Prothrombin Deficiency
title_full_unstemmed Congenital Prothrombin Deficiency
title_sort congenital prothrombin deficiency
publisher Shahid Beheshti University of Medical Sciences
series Journal of Cellular and Molecular Anesthesia
issn 2476-5120
publishDate 2019-02-01
description Congenital prothrombin deficiency is an extremely rare hemorrhagic disorder with estimated prevalence of 1 per 2,000,000 in the general population. Since the disorder is an autosomal recessive disorder, the disorder is more frequent in areas with high rate of consanguinity. Clinical manifestations of disorder are highly variable ranging from mild bleeding episodes to severe life-threatening hemorrhages. The disorder can be diagnosed based on routine and specific tests. No specific factor II concentrate is available, but patients can receive fresh frozen plasma and prothrombin complex concentrate (PCC). Traditionally patients with prothrombin deficiency receive on-demand therapy, but secondary prophylaxis can be used for those patients with high risk of severe life-threatening bleeding. With timely diagnosis and appropriate management of disorder, the quality of life in these patients can significantly improve.    Keywords: Prothrombin deficiency, Clinical manifestations, Diagnosis, Treatment
url http://journals.sbmu.ac.ir/jcma/article/view/23494
work_keys_str_mv AT maryamdaneshi congenitalprothrombindeficiency
AT tohidnaderi congenitalprothrombindeficiency
AT shaditabibian congenitalprothrombindeficiency
AT mahmoodshams congenitalprothrombindeficiency
AT jamalrashidpanah congenitalprothrombindeficiency
AT akbardorgalaleh congenitalprothrombindeficiency
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