Pediatric combined liver–kidney transplantation: a single-center experience of 18 cases

It has been proposed that the liver protects the kidney in CLKT. However, few studies have examined long‐term renal function after CLKT and contrasted renal function of CLKT patients to KT patients beyond one year after transplantation. We studied long‐term renal function of CLKT patients and compared renal function of CLKT patients to KT patients between one and five years after transplantation. Patients who underwent CLKT between 1993 and 2011 were included (n = 34; 11 children and 23 adults). Ninety‐six (27 children and 69 adults) KT patients were selected as controls. GFR was estimated (eGFR) and measured (mGFR) with 51Cr‐EDTA clearance. Mean mGFR was 63 at one and 70 at ten years after pediatric CLKT. Mean eGFR was 75 at one and 50 at ten years after adult CLKT. Difference in mean mGFR between pediatric CLKT and KT patients was 8 (95% CI −7 to 23) and 11 (95% CI −4 to 26) at one and five years after transplantation, respectively. Difference in mean eGFR between adult CLKT and KT patients was 8 (95% CI −5 to 20) and 1 (95% CI −10 to 12) at one and five years after transplantation, respectively. Longitudinal changes in GFRs were somewhat similar in CLKT and KT patients in both age‐groups but pediatric CLKT patients had on average higher GFRs than pediatric KT patients. In long‐term follow‐up, renal function remains stable in pediatric CLKT patients but declines in adult CLKT patients.

Section: Discussionsupporting

confidence: 76%

Section: Other Resultsmentioning

confidence: 99%

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Renal function after combined liver‐kidney transplantation: A longitudinal study of pediatric and adult patients

Kivelä

Lempinen

Holmberg

et al. 2019

“…Most of the CLKT reports have been on cases from single deceased donors. For SLKT cases, most often one of the organs is procured from a living donor and the other from a deceased donor . Reports about CLKT and SLKT from a single living donor are limited from countries where a shortage of deceased donors remains a problem .…”

Section: Discussionmentioning

confidence: 99%

The experience of combined and sequential liver and kidney transplantation from a single living donor in patients with primary hyperoxaluria type 1

Ozer

Aktaş

Bulum

et al. 2019

LKT is the only effective treatment for PH1 because it replaces both the source (liver) and the target (kidney) of the disease. Most studies report on LKT in patients with PH1 from deceased donors. This study reports on five patients who underwent LKT from a single living donor between April 2017 and March 2018. Combined LKT was performed for 1 patient and sequential LKT for the remainder. The median age of the patients at the time of diagnosis and transplantation was 5.5 (0.3‐18) and 10 (6‐21) years, respectively. All patients received left lateral liver segment transplantation, except one patient who received right liver lobe transplantation. No liver graft loss was observed, and liver function tests were stable at the final evaluation of all patients. Renal function tests of the patients were also stable at the final assessment, except for the young adult patient. None of the patients suffered from acute rejection. One patient died at the second month following liver transplantation due to severe pneumonia and sepsis. This study concludes that combined or sequential LKT from a single living donor can be safely performed and provides encouraging results for even the youngest and smallest patients with PH1.

“…Success with combined liver‐kidney transplant has significantly improved recently, where an overall survival rate of 78.6% has been reported in those in whom the combined transplant was performed for an underlying metabolic condition . Even in the very small pediatric patients, an excellent survival rate approaching 90% and comparable to that in larger children can be attained in specialized centers . Others, including Niemi et al, have already suggested that LKT is effective and should be recommended in patients with chronic kidney disease .…”

Section: Discussionmentioning

confidence: 99%

Kidney disease and organ transplantation in methylmalonic acidaemia

Noone

Riedl

Atkison

et al. 2019

Objectives MMA is associated with chronic tubulointerstitial nephritis and a progressive decline in GFR. Optimal management of these children is uncertain. Our objectives were to document the pre‐, peri‐, and post‐transplant course of all children with MMA who underwent liver or combined liver‐kidney transplant in our centers. Design and methods Retrospective chart review of all cases of MMA who underwent organ transplantation over the last 10 years. Results Five children with MMA underwent liver transplant (4/5) and combined liver‐kidney transplant (1/5). Three were Mut0 and two had a cobalamin B disorder. Four of five were transplanted between ages 3 and 5 years. Renal dysfunction prior to transplant was seen in 2/5 patients. Post‐transplant (one liver transplant and one combined transplant) renal function improved slightly when using creatinine‐based GFR formula. We noticed in 2 patients a big discrepancy between creatinine‐ and cystatin C‐based GFR calculations. One patient with no renal disease developed renal failure post–liver transplantation. Serum MMA levels have decreased in all to <300 μmol/L. Four patients remain on low protein diet, carnitine, coenzyme Q, and vitamin E post‐transplant. Conclusions MMA is a complex metabolic disorder. Renal disease can continue to progress post–liver transplant and close follow‐up is warranted. More research is needed to clarify best screening GFR method in patients with MMA. Whether liver transplant alone, continued protein restriction, or the addition of antioxidants post‐transplant can halt the progression of renal disease remains unclear.