A new protein substitute for adolescents and adults with maple syrup urine disease (MSUD)

P Hallam, M Lilburn, P J Lee
Journal of Inherited Metabolic Disease 2005, 28 (5): 665-72

AIM: The aim was to study the efficacy and acceptability of MSUD Express in adolescent and adult patients with maple syrup urine disease (MSUD).

BACKGROUND: There are difficulties associated with current protein substitutes designed for adolescents and adults with MSUD. This can affect their metabolic control. MSUD Express is a new low-volume, nutritionally complete protein substitute, free from leucine, isoleucine and valine and designed specifically for older patients with MSUD.

METHODS: Four patients with MSUD were included in the trial (aged 16-41 years). Product dosage was 4 x 25 g sachets per day, providing 1264 kJ and 72 g amino acids: more than adequately meeting requirements for all micronutrients.

RESULTS: At the start of the study, the mean (range) of the previous eight leucine concentrations (on MSUD Aid III) was 564 micromol/L (430-817 micromol/L) and during the study (on MSUD Express) was 382 micromol/L (181-603 micromol/L). The average percentage fall in mean leucine concentrations was 32%. In patients 2, 3 and 4, low leucine levels resulted in an increase in the natural protein allowance per day. MSUD Express was rated as 'excellent' or 'good' by all patients on the basis of appearance, taste, smell and texture. All patients found the product 'very easy' to prepare and found it 'easy' or 'very easy' to take outside the home.

CONCLUSION: All patients thought the preparation of MSUD Express was 'very easy'. Metabolic control (mean leucine concentrations) improved in all of the patients. In three patients this meant that an increase in the amount of natural protein in the diet was possible. All patients elected to stay on MSUD Express after the trial period. MSUD Express appears to be an effective and acceptable product, offering an alternative to current protein substitutes available for older people with MSUD.

Full Text Links

Find Full Text Links for this Article


You are not logged in. Sign Up or Log In to join the discussion.

Related Papers

Remove bar
Read by QxMD icon Read

Save your favorite articles in one place with a free QxMD account.


Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.