Nasogastric Drip Feeding as the Only Treatment of Neonatal Maple Syrup Urine Disease

Abstract

The ideal management for severe metabolic decompensation in maple syrup urine disease (MSUD) is still debated. Most papers published in the past 20 years suggest the use of extracorporeal techniques combined with adequate caloric intake1-5 As renal clearance is poor,6 repeated and prolonged blood exchange transfusions (ETs), peritoneal dialysis, and hemodialysis have proved useful in removing branched-chain amino and keto acids (BCAAs, BCKAs) from plasma.3,7,8 However, single case reports have described good results in patients given nutritional treatment alone,9-11 and recently it has been suggested that extracorporeal techniques should be used only in selected cases, when diagnosis is made after 8 to 10 days of life and the condition is presumably life-threatening.12,13