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Long‐term outcome in 42 pediatric liver transplant patients with alpha 1‐antitrypsin deficiency: a single‐center experience
Author(s) -
Hughes Michael G.,
Khan Khalid M.,
Gruessner Angelika C.,
Sharp Harvey,
Hill Mark,
Jie Tun,
Kandaswamy Raja,
Humar Abhinav,
Payne William D.,
Gruessner Rainer W. G.
Publication year - 2010
Publication title -
clinical transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.918
H-Index - 76
eISSN - 1399-0012
pISSN - 0902-0063
DOI - 10.1111/j.1399-0012.2010.01371.x
Subject(s) - medicine , biliary atresia , liver transplantation , transplantation , gastroenterology , tacrolimus , single center , alpha 1 antitrypsin deficiency , surgery
Hughes MG Jr, Khan KM, Gruessner AC, Sharp H, Hill M, Jie T, Kandaswamy R, Humar A, Payne WD, Gruessner RWG. Long‐term outcome in 42 pediatric liver transplant patients with alpha 1‐antitrypsin deficiency: a single‐center experience.
Clin Transplant 2011: 25: 731–736. © 2010 John Wiley & Sons A/S. Abstract: Introduction: We examined the long‐term outcome of transplantation for alpha 1‐antitrypsin deficiency (A1ATD). Method: Data were reviewed on 42 transplants in 35 children with A1ATD over 42 yr and compared with 129 transplants in 116 children with biliary atresia (BA). Results: Over 50% of patients were followed up for >10 yr. A1ATD were older than BA at transplantation, median age, 6.0 vs. 1.0 yr (p < 0.0001), and transplanted earlier in the course of liver failure (total bilirubin, 2.7 mg/dL [1.4–6.9] vs. 9.7 mg/dL [2.9–15.4], p = 0.005). Patient survival was greater in A1ATD than BA: one‐yr post‐transplant, 82.7% vs. 67.9%; five yr, 76.5% vs. 60.2%; and 10 yr, 76.5% vs. 55.9% (p = 0.03). Death‐censored graft survival was similar: one‐yr post‐transplant, 68.4% vs. 66.2%; five yr, 68.4% vs. 55.8%; and 10 yr, 68.4% vs. 52.5% (p = 0.2). Deaths were from infection, hemorrhage, and graft failure <6 months post‐transplant. Patient survival improved at five yr from 33.3% pre‐cyclosporine (CSA) (1969–1984) (n = 6) to 76.5% in the CSA era (1985–1994) (n = 17) and 100% with tacrolimus (1995–2006) (n = 12) (p = 0.007). Conclusions: The age at transplantation and the degree of liver dysfunction were related to the differences in graft and patient survival between A1AT and BA.