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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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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