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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2021.1
- 수록면
- 59 - 65 (7page)
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초록· 키워드
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Congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into systemic circulation. Herein, we report a case of pediatric deceased donor liver transplantation (DDLT) for symptomatic CAPV with whole liver graft from a hepatitis B surface antigen (HBsAg)-positive donor. A 4-year-old boy suffered from CAPV and secondary portal hypertension. He was also diagnosed with DiGeorge syndrome and heart anomalies. After waiting for 4 months, a 5-year-old donor weighing 19 kg with positive HBsAg was allocated to this 4-year-old patient weighing 15 kg. Recipient operation was performed according to the standard procedures of pediatric DDLT. Portal vein reconstruction was performed using interposition of a vascular homograft conduit to the superior mesenteric vein-splenic vein confluence. The patient recovered uneventfully from DDLT. He has been administered with lamivudine to prevent hepatitis B virus infection. This patient has been doing well for 5 years after DDLT without growth retardation. In conclusion, CAPV patients can have various vascular anomalies, thus combined vascular anomalies should be thoroughly assessed before and during liver transplantation operation. The most effective reconstruction techniques should be used to achieve satisfactory results following liver transplantation.
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등록된 정보가 없습니다.
참고문헌 (15)
참고문헌 신청
Shinkai M / 2001 / Congenital absence of the portal vein and role of liver transplantation in children / J Pediatr Surg 36:1026~1031
Sumida W / 2006 / Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure / J Pediatr Surg 41:e9~e12
Watanabe A / 2000 / Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment / J Gastroenterol Hepatol 15:969~979
Morgan G / 1994 / Congenital absence of the portal vein : two cases and a proposed classification system for portasystemic vascular anomalies / J Pediatr Surg 29:1239~1241
Mistinova J / 2010 / Congenital absence of the portal vein : case report and a review of literature / Clin Anat 23:750~758
Sumida W / 2006 / Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure / J Pediatr Surg 41:e9~e12
Watanabe A / 2000 / Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment / J Gastroenterol Hepatol 15:969~979
Morgan G / 1994 / Congenital absence of the portal vein : two cases and a proposed classification system for portasystemic vascular anomalies / J Pediatr Surg 29:1239~1241
Mistinova J / 2010 / Congenital absence of the portal vein : case report and a review of literature / Clin Anat 23:750~758
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