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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.1
- 수록면
- 66 - 70 (5page)
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초록· 키워드
오류제보하기
Congenital factor X (FX) deficiency is a rare autosomal-recessive disease that induces bleeding disorder. Herein, we present the 10-year posttransplant course of a pediatric patient who underwent liver transplantation (LT) with portal vein (PV) stenting for correction of severe congenital FX deficiency, with focus on long-term maintenance of coagulation function and patency of PV stenting. A 17-month-old infant with recurrent hemorrhagic episodes due to FX deficiency underwent split LT using a left lateral section graft. The graft-recipient weight ratio was 2.2%. The graft implantation procedures were performed by following the standard pediatric split LT procedure. Nevertheless, a wall stent was inserted due to PV anastomotic stenosis on posttransplant day 1. Graft function recovered slowly because of partial parenchyma infarct, and the patient was discharged at 46 days after LT operation. The FX activity started to increase soon after LT and gradually normalized; the coagulation profiles have been maintained well for the past 10 years. The patient has been doing well for the past 10 years after LT without any episodes of abnormal bleeding. Due to the risk of vascular complications owed to PV stenting, life-long follow-up is mandatory with special attention until attainment of complete physical growth to adolescent and adulthood.
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등록된 정보가 없습니다.
참고문헌 (12)
참고문헌 신청
Brown DL / 2008 / Diagnosis and treatment of inherited factor X deficiency / Haemophilia 14:1176~1182
Funk DM / 2010 / Factor X deficiency / Clin Lab Sci 23:131~133
Bang SH / 2012 / Successful liver transplantation for a child with life-threatening recurrent bleeding episodes due to congenital factor X deficiency : a case report / Transplant Proc 44:583~584
Girolami A / 2008 / Congenital bleeding disorders of the vitamin K-dependent clotting factors / Vitam Horm 78:281~374
Senturk S / 2010 / Factor X deficiency presenting with bilateral chronic subdural hematoma / Pediatr Neurosurg 46:54~57
Funk DM / 2010 / Factor X deficiency / Clin Lab Sci 23:131~133
Bang SH / 2012 / Successful liver transplantation for a child with life-threatening recurrent bleeding episodes due to congenital factor X deficiency : a case report / Transplant Proc 44:583~584
Girolami A / 2008 / Congenital bleeding disorders of the vitamin K-dependent clotting factors / Vitam Horm 78:281~374
Senturk S / 2010 / Factor X deficiency presenting with bilateral chronic subdural hematoma / Pediatr Neurosurg 46:54~57
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