Congenital Prothrombin Deficiency

Maryam Daneshi; Tohid Naderi; Shadi Tabibian; Mahmood Shams; Jamal Rashidpanah; Akbar Dorgalaleh

doi:10.22037/jcma.v3i4.23494

Journal of Cellular & Molecular Anesthesia

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https://doi.org/10.22037/jcma.v3i4.23494

Congenital Prothrombin Deficiency

Author(s):

Maryam Daneshi^1,*,

Tohid Naderi²,

Shadi Tabibian¹,

Mahmood Shams¹,

Jamal Rashidpanah³,

Akbar Dorgalaleh¹

1Department of Hematology and Blood transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran

2Department of Hematology and Blood transfusion, School of Allied Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran

3Shariati hospital, Tehran University of Medical Sciences, Tehran, Iran

Journal of Cellular & Molecular Anesthesia:Vol. 3, issue 4; e149569

Published online:Feb 22, 2019

Article type:Review

How to Cite:Maryam DaneshiTohid NaderiShadi TabibianMahmood ShamsJamal RashidpanahAkbar Dorgalalehet al.Congenital Prothrombin Deficiency.J Cell Mol Anesth.2019;3(4):e149569.https://doi.org/10.22037/jcma.v3i4.23494.

Abstract

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

Keywords

Prothrombin deficiency

Clinical manifestations

Diagnosis

Treatment

References

1.
References are in the PDF file of the article.

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Author(s):

Maryam Daneshi:[PubMed][Scholar]
Tohid Naderi:[PubMed][Scholar]
Shadi Tabibian:[PubMed][Scholar]
Mahmood Shams:[PubMed][Scholar]
Jamal Rashidpanah:[PubMed][Scholar]
Akbar Dorgalaleh:[PubMed][Scholar]