![]() ![]() On the admission to the hospital with acute appendicitis, the management of her nutrition to increase the anabolism and prevent the accumulation of BCAA was challenging she was not able to tolerate oral food but only a fraction of the total volume of prescribed supplements administered via nasogastic tube. As a result she was at increased risk of refeeding syndrome and acute metabolic decompensation. Due to poor food intake an eating disorder was suspected. ![]() Her recurrent episodes of vomiting might have been also caused by an oesophageal polyp. Frequent increases in leucine >1000 μmol/L were associated with vomiting, reduced oral intake and general illness. We present a case of a female adult patient with Maple Syrup Urine Disease (MSUD) who had recurrent episodes of raised Branched Chain Amino Acids levels (BCAA). This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. The Management Of a Case With Maple Syrup Urine Disease With Haemofiltration and Nutritional Support in the Perioperative Period. The Mark Holland Metabolic Unit, Salford Royal Foundation NHS Trust, Ladywell, UK Tel: 0044 1 Fax: 0044 1 E-mail: Stepien KM, Wilcox G, Green D, et al. Specialty Registrar in Adult Inherited Metabolic Disorders, Adult Inherited Metabolic Disorders Karolina M Stepien 1 *, Gisela Wilcox 2, Diane Green 3, Stephen Fletcher 4, Prof Chris J Hendriksz 5ġ Specialty Registrar in Adult Inherited Metabolic Disorders, Adult Inherited Metabolic Disorders, The Mark Holland Metabolic Unit, Salford Royal Foundation NHS Trust, Ladywell, UKĢ Consultant in Adult Inherited Metabolic Disorders Salford Royal NHS Foundation Trust, UKģ Metabolic Salford Royal NHS Foundation Trust Dietician, UKĤ Consultant Intensivist, Bradford Teaching Hospitals NHS Foundation Trust, UKĥ Consultant Transitional Metabolic Medicine, Salford Royal NHS Foundation Trust, UK *Corresponding Author: Karolina M Stepien
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