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- W2092600348 abstract "Methylmalonic acidemia (MMA) and propionic acidemia (PA) are inborn errors of metabolism, specifically types of organic acidemias. Individuals with MMA and PA are unable to catabolize branched-chain amino acids because of specific enzymatic defects. Nutritionally, patients are managed by intact protein-restricted diets. Despite strict medical and nutritional management, metabolic instability may continue. Liver or liver and kidney transplantation may be a treatment option. Transplant may result in improvement of laboratory values, increased oral intake, and developmental gains. The aim of medical nutrition therapy for MMA/PA-transplanted patients is promotion of metabolic stability peritransplant." @default.
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- W2092600348 date "2009-10-01" @default.
- W2092600348 modified "2023-09-26" @default.
- W2092600348 title "Nutritional Management of Methylmalonic and Propionic Acidemia" @default.
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- W2092600348 doi "https://doi.org/10.1097/tin.0b013e3181c622fb" @default.
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