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Maple syrup urine disease (MSUD) is an autosomal recessive inherited disorder. Despite the advances in medical nutrition therapies, classical phenotype causes severe neurological disorders and sudden death. It is known… Click to show full abstract
Maple syrup urine disease (MSUD) is an autosomal recessive inherited disorder. Despite the advances in medical nutrition therapies, classical phenotype causes severe neurological disorders and sudden death. It is known that MSUD patients do not experience metabolic attacks despite their free diet after liver transplantation (LT). This study aims to reveal the long‐term results, development, mental, motor, intellectual and nutritional status of MSUD patients who underwent LT.
Keywords:
long term;
maple syrup;
syrup urine;
transplantation;
liver transplantation;
urine disease
Journal Title: Pediatric Transplantation
Year Published: 2023
Link to full text (if available)
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Journal Title: Pediatric Transplantation
Year Published: 2023
Link to full text (if available)
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recommendations!