{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T17:29:48Z","timestamp":1783531788964,"version":"3.55.0"},"reference-count":59,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2019,4,11]],"date-time":"2019-04-11T00:00:00Z","timestamp":1554940800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>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\u2010, peri\u2010, and post\u2010transplant course of all children with MMA who underwent liver or combined liver\u2010kidney transplant in our centers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design and methods<\/jats:title><jats:p>Retrospective chart review of all cases of MMA who underwent organ transplantation over the last 10\u00a0years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Five children with MMA underwent liver transplant (4\/5) and combined liver\u2010kidney transplant (1\/5). Three were Mut<jats:sup>0<\/jats:sup> and two had a cobalamin B disorder. Four of five were transplanted between ages 3 and 5\u00a0years. Renal dysfunction prior to transplant was seen in 2\/5 patients. Post\u2010transplant (one liver transplant and one combined transplant) renal function improved slightly when using creatinine\u2010based GFR formula. We noticed in 2 patients a big discrepancy between creatinine\u2010 and cystatin C\u2010based GFR calculations. One patient with no renal disease developed renal failure post\u2013liver transplantation. Serum MMA levels have decreased in all to &lt;300\u00a0\u03bcmol\/L. Four patients remain on low protein diet, carnitine, coenzyme Q, and vitamin E post\u2010transplant.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>MMA is a complex metabolic disorder. Renal disease can continue to progress post\u2013liver transplant and close follow\u2010up 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\u2010transplant can halt the progression of renal disease remains unclear.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.13407","type":"journal-article","created":{"date-parts":[[2019,4,12]],"date-time":"2019-04-12T02:52:02Z","timestamp":1555037522000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Kidney disease and organ transplantation in methylmalonic acidaemia"],"prefix":"10.1111","volume":"23","author":[{"given":"Damien","family":"Noone","sequence":"first","affiliation":[{"name":"Division of Nephrology The Hospital for Sick Children Toronto Ontario Canada"},{"name":"Department of Paediatrics University of Toronto Toronto Ontario 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