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A 40-year-old member asked:

Besides maple syrup urine disease, are there some other causes of urine that smells sweet like syrup?

2 doctor answers6 doctors weighed in
Dr. Jon Ahrendsen
Family Medicine 40 years experience
Possibly diabetes: To my knowledge, uncontrolled diabetes might also cause this, but i would expect the person to also have weight loss, and frequent urination besides just a sweet smell to the urine.
Dr. Sandra Lora Cremers
Ophthalmology 26 years experience
Diabetes Mellitus: Patients with Diabetes Mellitus can have urine that smells sweet.

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A 34-year-old member asked:

Please tell me what causes maple syrup urine disease?

1 doctor answer3 doctors weighed in
Dr. James Ferguson
Pediatrics 47 years experience
Recessive defect: Msud is an inherited defect in in metabolism.This results when two asymptomatic carrier parents give their fetus a defective gene. After birth, the baby will lack an enzyme needed to break down branch chained protein structures.These build up in the blood ; spill into the urine producing the characteristic odor.The parents are unaffected because a single copy of the normal gene do the work.
A 41-year-old member asked:

Hows maple syrup urine disease diagnosed?

1 doctor answer2 doctors weighed in
Dr. Quresh Khairullah
Nephrology and Dialysis 43 years experience
See below: The diagnostic test for this condition is the measurement of blood Amino Acid concentrations. Affected patients have elevated levels of Amino Acids (leucine, isoleucine, and valine). Urine organic acid measurement will detect elevated levels of branched-chain ketoacids, lactate, and pyruvate.
CA
A 33-year-old male asked:

What is the definition or description of: Maple syrup urine disease?

1 doctor answer2 doctors weighed in
Dr. Pierrette Mimi Poinsett
Pediatrics 39 years experience
Maple Syrup Urine: disease is an inherited metabolic disorder in which the body is unable to process certain protein building blocks (amino acids) . Also known as branched chain ketoaciduria. People with this disorder have urine that smells like maple syrup as a result. The smell is from the buildup of certain amino acids and toxic byproducts in the urine and sweat.

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Last updated Sep 29, 2016

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