Combined en bloc liver pancreas transplantation for children with CF

Mekeel, Kristin L.; Langham, Max R.; Gonzalez-Perralta, Regino; Reed, Alan; Hemming, Alan W.

doi:10.1002/lt.21070

Cited by 43 publications

(35 citation statements)

References 24 publications

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“…Presumably with the use of immunosuppressive drugs after transplantation (mainly corticosteroids and calcineurin inhibitors), the prevalence of diabetes has been observed to be even higher 13. Interestingly, combined en bloc liver and pancreas transplantation in patients with CF has been reported to lower posttransplant insulin requirements in comparison with those patients receiving a liver transplant alone 23…”

Section: Discussionmentioning

confidence: 99%

Liver transplantation in patients with cystic fibrosis: Analysis of united network for organ sharing data

et al. 2011

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The improved life expectancy of patients with cystic fibrosis (CF) has led to a change in the impact of liver disease on the prognosis of this population. Liver transplantation has emerged as the procedure of choice for patients with CF and features of hepatic decompensation and for intractable variceal bleeding as a major manifestation. We retrospectively reviewed the United Network for Organ Sharing database to analyze the outcomes of 55 adults and 148 children with CF who underwent liver transplantation, and we compared them to patients who underwent transplantation for other etiologies. We additionally compared the benefits of liver transplantation among patients who underwent transplantation for cystic fibrosis-related liver disease (CFLD) and those who remained on the waiting list. The 5-year survival rates for children and adults undergoing liver transplantation were 85.8% and 72.7%, respectively (P ¼ 0.016). A multivariate Cox regression analysis comparing pediatric and adult CF patients to patients who underwent transplantation for other etiologies noted lower 5-year survival rates (P < 0.0001). However, compared to those remaining on the waiting list, pediatric transplant recipients with CF (hazard ratio ¼ 0.33, 95% confidence interval ¼ 0.16-0.70, P ¼ 0.004) and adult transplant recipients with CF (hazard ratio ¼ 0.25, 95% confidence interval ¼ 0.11-0.57, P ¼ 0.001) gained a significant survival benefit. In conclusion, long-term outcomes in patients with CFLD are acceptable but are inferior in comparison with the outcomes of those undergoing transplantation for other etiologies. Despite such observations, a survival benefit was noted in transplant patients versus those who remained on the waiting list.

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Section: Discussionmentioning

confidence: 99%

Liver transplantation in patients with cystic fibrosis: Analysis of united network for organ sharing data

et al. 2011

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“…There is an almost universal requirement for insulin in the immediate critical care postoperative period in CF patients undergoing transplantation procedures, and many have long-term insulin requirements after transplantation (34–36). A diagnosis of CFRD prior to transplantation may increase complications of surgery and has a negative impact on survival, at least in the early postoperative period when infection, bleeding, and multiorgan failure are the most common causes of death (34,37).…”

Section: Screeningmentioning

confidence: 99%

Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes

et al. 2010

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“…Since 1991, 13 LT procedures have been performed on average for CFLD each year in the United States (range = 7‐19) 1. The 1‐year survival rates are 75% or greater, whereas the 5‐year survival rates are 60% to 80% 19‐30. Despite the increase in LT for patients with CF, many questions about long‐term pulmonary outcomes remain unanswered.…”

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confidence: 99%

Pulmonary function in individuals who underwent liver transplantation: From the US cystic fibrosis foundation registry

et al. 2012

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Severe liver disease affects 4.5% to 10% of individuals with cystic fibrosis (CF) and is the third-leading cause of death. Liver transplantation (LT) is an accepted therapy, but the effects of liver disease and LT on pulmonary function in patients with CF are controversial. Our aim was to characterize changes in pulmonary function in LT patients with CF. Using mixed effect models, we analyzed pulmonary function before and after transplantation in 168 LT patients and 840 non-LT patients with CF who were matched by age, sex, pancreatic status, infections with US CF Foundation Patient Registry data (1989)(1990)(1991)(1992)(1993)(1994)(1995)(1996)(1997)(1998)(1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007). The primary outcome was the change in the forced expiratory volume in 1 second (FEV 1 ; percent predicted) in LT and non-LT in the 3-years periods before or after transplantation; second we compared FEV 1 changes. In the 3 years before transplantation, LT had lower initial FEV 1 values (71.5% 6 1.9%, P < 0.001) and a slower decline (þ0.1% 6 0.4%/year, P < 0.001) than non-LT (79.6% 6 1.3% and À1.3% 6 0.2%/year, respectively). There was no difference in the FEV 1 decline after transplantation (À1.4% 6 0.4%/year for LT versus À2.1% 6 0.2%/year for non-LT, P ¼ 0.14). Both the (P ¼ 0.003) and (P ¼ 0.001) had a slower FEV 1 decline in the period before transplantation versus after transplantation. In conclusion, pulmonary function is lower and declines more slowly in patients with CF before LT versus, but parallels the decline in non-LT after transplantation. LT is neither beneficial nor detrimental to pulmonary function in CF but returns FEV 1 decline to the same trajectory found for matched non-LT individuals with CF.

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Combined en bloc liver pancreas transplantation for children with CF

Cited by 43 publications

References 24 publications

Liver transplantation in patients with cystic fibrosis: Analysis of united network for organ sharing data

Liver transplantation in patients with cystic fibrosis: Analysis of united network for organ sharing data

Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes

Pulmonary function in individuals who underwent liver transplantation: From the US cystic fibrosis foundation registry

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