Evidence map›Paper›PMID 33232939›Full record

ArticleBlood research2020

Mining of mortality-related findings in rare bleeding disorders: a retrospective study from two centers.

Hassan Rafieemehr, Akbar Dorgalaleh, Hassan Mansouritorghabeh

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Article in Blood research, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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3 · Its place in the literature

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1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

3 authors.

Hassan RafieemehrDepartment of Medical Laboratory Sciences, School of Paramedicine, Hamadan University of Medical Sciences, Hamadan, Iran.
Akbar DorgalalehDepartment of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran.
Hassan MansouritorghabehImmunology Research Center, Mashhad University of Medical Sciences, Mashhad, Iran.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundRare bleeding disorders include inherited coagulation disorders except for von Willebrand disease and hemophilia A and B. These disorders affect both men and women worldwide and mainly have an autosomal recessive pattern of inheritance. Given the paucity of cases of rare bleeding disorders, there are limited data regarding some topics among bleeding disorders.

methodsThis retrospective study from 2005-2019 collected demographic data and the causes of death among cases with rare bleeding disorders from 2 provinces of Iran.

resultsOverall, 5 deaths were reported, including 3 cases with factor V deficiency, a case with factor XIII deficiency, and a case with combined factor V and factor VIII deficiencies. The main causes of death were bleeding in the central nervous system (2 cases; 1 with factor V deficiency and 1 with combined factor XIII deficiency). Post-partum hemorrhage was the cause of death in a woman with factor V deficiency while anaphylaxis shock was the cause of death in the case with combined factor V and factor VIII deficiencies. A woman with factor V deficiency died from an internal bleeding episode.

conclusionGathering data on the causes of death in rare bleeding disorders through worldwide registries can be helpful for the management of this rare group of bleeding disorders.

Indexed as

Anaphylactic shockCentral venous system hemorrhageDeathFactor V deficiencyFactor XIII deficiencyPost-partum hemorrhagerare bleeding disorders

Identifiers

PMID33232939
PMCPMC7784127

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