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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 저널정보
- 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제18권 제1호
- 발행연도
- 2015.1
- 수록면
- 48 - 54 (7page)
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Purpose: Metabolic liver disease (MLD) often progresses to life-threatening conditions. This study intends to describe the outcomes of liver transplantation (LTx) for MLD at a living donor-dominant transplantation center where potentially heterozygous carrier grafts are employed. Methods: We retrospectively evaluated the medical records of 54 patients with MLD who underwent LTx between November 1995 and February 2012 at Asan Medical Center in Seoul, Korea. The cumulative graft and patient survival rates were analyzed according to patient age, and living or deceased donor LTx. Recurrence of the original disease was also investigated.Results: The post-transplant cumulative patient survival rates at one, five, and 10 years were 90.7%, 87.5% and 87.5%, and the graft survival rates were 88.8%, 85.5%, and 85.5%, respectively. There were no differences in the patient survival rates according to the recipient age, human leukocyte antigen matching, and living or deceased donor LTx. There were also no differences in the patient survival rates between the MLD and the non-MLD groups for children. Recurrence of the original metabolic disease was not observed in any patient during the follow-up period.Conclusion: Our results suggest that the living donor-dominant transplantation program is well-tolerated in MLD with-out recurrence of the original MLD using all types of transplantation.
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참고문헌 (25)
참고문헌 신청
Angelis M / 2001 / En bloc heterotopic auxiliary liver and bilateral renal transplant in a patient with homozygous protein C deficiency / J Pediatr 138 : 120 ~ 122
Arnon R / 2010 / Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience / Pediatr Transplant 14 : 796 ~ 805
Asonuma K / 1999 / Living related liver transplantation from heterozygote genetic carriers to children with Wilson's disease / Pediatr Transplant 3 : 201 ~ 205
Bellary S / 1995 / Liver transplantation for Wilson's disease / J Hepatol 23 : 373 ~ 381
Franco LM / 2005 / Hepatocellular carcinoma in glycogen storage disease type Ia: a case series / J Inherit Metab Dis 28 : 153 ~ 162
Arnon R / 2010 / Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience / Pediatr Transplant 14 : 796 ~ 805
Asonuma K / 1999 / Living related liver transplantation from heterozygote genetic carriers to children with Wilson's disease / Pediatr Transplant 3 : 201 ~ 205
Bellary S / 1995 / Liver transplantation for Wilson's disease / J Hepatol 23 : 373 ~ 381
Franco LM / 2005 / Hepatocellular carcinoma in glycogen storage disease type Ia: a case series / J Inherit Metab Dis 28 : 153 ~ 162
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