Dietary Management of a Patient with Both Maple Syrup Urine Disease and Type I Diabetes

Özet

Maple Syrup Urine disease (MSUD) is caused by the deficiency of the branched chain 2-ketoacid dehydrogenase complex. Type I diabetes mellitus (TIDM) is a chronic illnesscharacterized by the body’s inability to produce insulin due to the autoimmune destruction of the beta cells in the pancreas. A case with both MSUD and TIDM has not beenreported previously. In this study, we presented a patient with both MSUD and TIDM, dietary management and the follow up period. Dietary management could be difficult inthe presence of two disorders affecting more than one macronutrient component such as both carbohydrate and protein. Our patient had good metabolic control in the followupperiod. Treatment was successful with intensive insulin, frequent feeding and a leucine restricted diet.