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Volume 16
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10.3390/nu16183145
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Article

The Impact of Diet on Body Composition in a Cohort of Pediatric and Adult Patients with Maple Syrup Urine Disease

by
Roberta Pretese
1,
Cristina Bonfanti
1,
Martha Caterina Faraguna
1,2,
Marialetizia Fantasia
1,2,
Viola Crescitelli
1,
Silvia Barzaghi
1,
Mara Botti
3,
Giulia Mezzanotti
4 and
Serena Gasperini
1,*
1
Department of Pediatrics, Fondazione IRCCS San Gerardo dei Tintori, 20900 Monza, Italy
2
Residency in Pediatrics, University of Milano Bicocca, 20126 Milano, Italy
3
Rare Disease Centre, Fondazione IRCCS San Gerardo dei Tintori, 20900 Monza, Italy
4
Deparment of Biology Applied to Nutrition Sciences, University of Milano Statale, 20122 Milano, Italy
*
Author to whom correspondence should be addressed.
Nutrients 2024, 16(18), 3145; https://doi.org/10.3390/nu16183145
Submission received: 30 August 2024 / Accepted: 10 September 2024 / Published: 18 September 2024
(This article belongs to the Section Nutrition and Diabetes)
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Abstract

The treatment for Maple Syrup Urine Disease (MSUD) consists of a hypoproteic diet with integration therapy to limit leucine intake, ensuring adequate energy, macronutrients, and micronutrients to prevent catabolism and promote anabolism. We conducted a retrospective cross-sectional study at the Metabolic Rare Disease Unit, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy. Patients with MSUD who were over 3 years old, not treated with liver transplantation, and who provided written consent, were included. The study aimed to describe the dietary treatment of patients with MSUD, evaluate growth data, and analyze the effect of a low-protein and semi-synthetic diet on body composition. Data on height, weight, BMI, waist circumference, food intake, physical activity, and DEXA scans were collected. Thirteen subjects (11 classic MSUD, 2 intermediate MSUD) were included, of which 5 < 18 years old. Results indicated that patients with MSUD follow a balanced diet and have body compositions like healthy subjects in terms of fat and lean mass. A high incidence of osteopenia was observed from a young age, with a positive correlation between protein intake and lean mass and a negative correlation between BCAA-free mixture consumption and bone mineral density z-score. The study highlights the positive effects and potential consequences of the semi-synthetic diet on the body composition of patients with MSUD. A similar study involving all Italian metabolic centers treating MSUD is recommended.
Keywords: maple syrup urine disease; branched-chain amino acids; body composition; hypoproteic diet; osteopenia; bone mineral density maple syrup urine disease; branched-chain amino acids; body composition; hypoproteic diet; osteopenia; bone mineral density
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MDPI and ACS Style

Pretese, R.; Bonfanti, C.; Faraguna, M.C.; Fantasia, M.; Crescitelli, V.; Barzaghi, S.; Botti, M.; Mezzanotti, G.; Gasperini, S. The Impact of Diet on Body Composition in a Cohort of Pediatric and Adult Patients with Maple Syrup Urine Disease. Nutrients 2024, 16, 3145. https://doi.org/10.3390/nu16183145

AMA Style

Pretese R, Bonfanti C, Faraguna MC, Fantasia M, Crescitelli V, Barzaghi S, Botti M, Mezzanotti G, Gasperini S. The Impact of Diet on Body Composition in a Cohort of Pediatric and Adult Patients with Maple Syrup Urine Disease. Nutrients. 2024; 16(18):3145. https://doi.org/10.3390/nu16183145

Chicago/Turabian Style

Pretese, Roberta, Cristina Bonfanti, Martha Caterina Faraguna, Marialetizia Fantasia, Viola Crescitelli, Silvia Barzaghi, Mara Botti, Giulia Mezzanotti, and Serena Gasperini. 2024. "The Impact of Diet on Body Composition in a Cohort of Pediatric and Adult Patients with Maple Syrup Urine Disease" Nutrients 16, no. 18: 3145. https://doi.org/10.3390/nu16183145

APA Style

Pretese, R., Bonfanti, C., Faraguna, M. C., Fantasia, M., Crescitelli, V., Barzaghi, S., Botti, M., Mezzanotti, G., & Gasperini, S. (2024). The Impact of Diet on Body Composition in a Cohort of Pediatric and Adult Patients with Maple Syrup Urine Disease. Nutrients, 16(18), 3145. https://doi.org/10.3390/nu16183145

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