II. 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

III. Food and medication causes of abnormal Urine Odor

  1. Asparagus
  2. Vitamin B6 Supplementation

IV. 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

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