Congenital Prothrombin Deficiency

AuthorMaryam Daneshien
AuthorTohid Naderien
AuthorShadi Tabibianen
AuthorMahmood Shamsen
AuthorJamal Rashidpanahen
AuthorAkbar Dorgalalehen
Issued Date2018-12-31en
AbstractCongenital 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, Treatmenten
DOIhttps://doi.org/10.22037/jcma.v3i4.23494en
KeywordProthrombin deficiencyen
KeywordClinical manifestationsen
KeywordDiagnosisen
KeywordTreatmenten
PublisherBrieflandsen
TitleCongenital Prothrombin Deficiencyen
TypeReviewen

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