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Urine Odor

Aka: Urine Odor

Medical Causes of abnormal Urine Odor
1. Sweet or fruity odor
  1. Diabetic Ketoacidosis
  2. Maple syrup urine disease (infants, rare)
2. Ammonia odor
  1. Bladder retention
  2. Urine at room temperature for prolonged period
3. Fecal odor
  1. Bladder-Intestinal fistula
4. Pungent odor
  1. Urinary Tract Infection
5. Strong odor
  1. Concentrated urine
6. Musty odor
  1. Phenylketonuria
7. Sulfur odor
  1. Cystine decomposition
Food and medication causes of abnormal Urine Odor
1. Asparagus
2. Vitamin B6 Supplementation
Inborn Errors of Metabolism causing Urine Odor
1. Phenylketonuria
2. Maple syrup urine disease (infants, rare)
3. Glutaric acidemia
4. Isovaleric acidemia
5. Hawkinsinuria
6. Hypermethioninemia
7. Multiple carboxylase deficiency
8. Oasthouse urine disease
9. Trimethylaminuria
10. Tyrosinemia
11. References
  1. Rezvani in Behrman (2004) Nelson Pediatrics, p. 397
  2. (2002) Harriet Lane, p. 275

Family Practice Notebook

Patients should address specific medical concerns with their physicians.

Although access to this page is not restricted, the information found here is intended for use by medical providers.

This page was written by Scott Moses, MD, last revised on 10/21/2007 and last published on 5/14/2013.

This is one of 5887 pages in the Family Practice Notebook