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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2020.1
- 수록면
- 213 - 216 (4page)
이용수
초록· 키워드
오류제보하기
Background Rare 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. Methods This retrospective study from 2005‒2019 collected demographic data and the causes of death among cases with rare bleeding disorders from 2 provinces of Iran. Results Overall, 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. Conclusion Gathering 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.
목차
등록된 정보가 없습니다.
참고문헌 (20)
참고문헌 신청
Peyvandi F / 2006 / Genetic diagnosis of haemophilia and other inherited bleeding disorders / Haemophilia 12(Suppl 3):82~89
WFH Network / Rare clotting factor deficiencies
Mansouritorghabeh H / 2013 / An investigation of the spectrum of common and rare inherited coagulation disorders in north-eastern Iran / Blood Transfus 11:233~240
Peyvandi F / 2010 / European registry of rare bleeding disorders / InEuropean Hematology Association 4(Suppl):63~68
Soucie JM / 2010 / The universal data collection surveillance system for rare bleeding disorders / Am J Prev Med 38(4 Suppl):S475~481
WFH Network / Rare clotting factor deficiencies
Mansouritorghabeh H / 2013 / An investigation of the spectrum of common and rare inherited coagulation disorders in north-eastern Iran / Blood Transfus 11:233~240
Peyvandi F / 2010 / European registry of rare bleeding disorders / InEuropean Hematology Association 4(Suppl):63~68
Soucie JM / 2010 / The universal data collection surveillance system for rare bleeding disorders / Am J Prev Med 38(4 Suppl):S475~481
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