絞り込み

16766

広告

Long-term outcome of methylmalonic aciduria after kidney, liver or combined liver-kidney transplantation: the French experience.

著者 Brassier A , Krug P , Lacaille F , Pontoizeau C , Krid S , Sissaoui S , Servais A , Arnoux JB , Legendre C , Charbit M , Scemla A , Francoz C , Benoist JF , Schiff M , Mochel F , Touati G , Broué P , Cano A , Tardieu M , Querciagrossa S , Grévent D , Boye
J Inherit Metab Dis.2019 Sep 16 ; ():.
この記事をPubMed上で見るPubMedで表示
この記事をGoogle翻訳上で見る Google翻訳で開く

スターを付ける スターを付ける     (8view , 0users)

Full Text Sources

Organ transplantation is discussed in methylmalonic aciduria (MMA) for renal failure, and poor quality of life and neurological outcome. We retrospectively evaluated 23 French MMA patients after kidney (KT), liver-kidney (LKT) and liver transplantation (LT). Two patients died, one after LKT, one of hepatoblastoma after KT. One graft was lost early after KT. Of 18 evaluable patients, 12 previously on dialysis, 8 underwent KT (mean 12.5 years), 8 LKT (mean 7 years), and 2 LT (7 and 2.5 years). At a median follow-up of 7.3 (KT), 2.3 (LKT) and 1.0 years (LT), no metabolic decompensation occurred except in 1 KT. Plasma and urine MMA levels dramatically decreased, more after LKT. Protein intake was increased more significantly after LKT than KT. Enteral nutrition was stopped in 7/8 LKT, 1/8 KT. Early complications were frequent after LKT. Neurological disorders occurred in 4 LKT, reversible in one. Five years after KT, 4 patients had renal failure. The metabolic outcomes were much better after LKT than KT. LKT in MMA is difficult but improves the quality of life. KT will be rarely indicated. We need more long term data to indicate early LT, in the hope to delay renal failure and prevent neurodevelopmental complications. This article is protected by copyright. All rights reserved.
PMID: 31525265 [PubMed - as supplied by publisher]
印刷用ページを開く Endnote用テキストダウンロード