Marcus is a 5 years old boy with MSUD. His weight was plotted in the 4 th percen
ID: 126445 • Letter: M
Question
Marcus is a 5 years old boy with MSUD. His weight was plotted in the 4th percentile at his last check-up.
1. What are the patient’s caloric needs, Protein, Fat, and any other calculations (BMI, % weight change, UBW, % involuntary weight loss, % UBW) necessary to determine patient’s needs
2.Write a menu for each diet, taking into account patient disease state, and also color, texture and aesthetics
3.what are the RDA’s and AMDR’s are met for specific nutrients (CHO, Fat& Sat Fat, fiber, cholesterol, protein, Ca, Na,) and any other nutrient pertinent to the patient’s care
Explanation / Answer
Maple syrup urine disease is an inherited metabolic disorder caused by branched chain alpha ketoacid dehydogenase deficiency resulting in the accumulation of branched chain amino acids.
1.careful evaluation of leucine intake on an individual basis. Leucine is an essential amino acid and cannot be excluded completely from the diet,even though it is the most toxic of the branched chain amino acids (bcca)and is present in food in higher concentration. The patients tolerance of leucine must be calculated following measurements of BCCA levels and remeasure at appropriate intervals during the first 6-12months of life. Intake of protein substitutes that provides bcca free amino acids. Inclusion of supplements that provides necessary vitamins,minerals and trace elements. An adequate intake of calories from one of the three sources-foods naturally low in or free from protein,specifically formulated low protein foods,and protein free supplements containing glucose polymers and fats.
2. The diet should be followed under doctors guidance. The protein substitute formulation for children from 12months to 10 years of age is a unflavoured powder containing 8.4 gem of protein equivalent designed to be mixed with cold water to form either a gel or a drink. Special packets of black currant,orange,lemon,raspberry or tropical flavour are obtained in markets which are rich vitamins,minerals.
3 .Recommended dietary individuals RDA is the daily dietary intake level of a nutrient considered sufficient by the food and nutrition board. The definition implies that the intake level would cause a harmful nutrient deficiency in just 2.5%. It is calculated based on estimated average requirements. Acceptable macro nutrient distribution ranges is a range of intake specified as a percentage of total energy intake. Used for sources of energy such as fats and carbohydrates.