Maple Syrup Urination Disease (MSUD)

 

MSUD Diagram

Recently, I had a student diagnosed with MSUD. It was the first time I had heard of the disease. This post will review all that I learned about MSUD.

Defined

Maple syrup urine disease is an inherited disorder in which the body is unable to process certain protein building blocks (amino acids) properly. The condition gets its name from the distinctive sweet odor of affected infants’ urine. It is also characterized by poor feeding, vomiting, lack of energy (lethargy), abnormal movements, and delayed development. If untreated, maple syrup urine disease can lead to seizures, coma, and death.

Maple syrup urine disease is often classified by its pattern of signs and symptoms. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. Variant forms of the disorder become apparent later in infancy or childhood and are typically milder, but they still lead to delayed development and other health problems if not treated. Source

MSUD means that the person’s body is unable to break down protein in the usual way. This condition is a rare, non-contagious condition, which, left untreated, can result in irreversible brain damage. Fortunately, the condition can be treated by a special diet, medications and careful management during illness.

Great resource- The ASIEM Low Protein Handbook for MSUD

 

 

Books

MSUD related books published by both casual and professional authors.

 
MSUD Food List Booklet
Recipes your whole family will enjoy
MSUD & Me
Glossary of Terms pertaining to Maple Syrup Urine Disease

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