Nasogastric drip feeding as the only treatment of neonatal maple syrup urine disease
1993
Parini, R. | Sereni, L.P. | Bagossi, D.C. | Corbetta, C. | Rabier, D. | Narcy, C. | Hubert, P. | Saudubray, J.M.
The authors present their experience with five patients with maple syrup urine disease who were treated successfully by nutritional therapy alone during eight neonatal and subsequent episodes of acute metabolic decompensation. The therapy aimed at achieving maximal stimulation of protein anabolism, using branched-chain amino acid (BCAA) free formula, adequate hydration and caloric intake, followed by timely reintroduction of BCCAAs based on daily plasma levels.
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