Urine Odor

Aka: Urine Odor

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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. Urinary Tract Infection (including infected Kidney Stone)
  3. Fecal odor
    1. Enterovesical fistula (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
    2. Sulfa medications
    3. Asparagus

III. Causes: Food and medications

  1. Asparagus
  2. Vitamin B6 Supplementation

IV. Causes: Inborn Errors of Metabolism

  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

V. References

  1. Hitzeman (2022) Am Fam Physician 106(1): 27-35 [PubMed]
  2. Simerville (2005) Am Fam Physician 71(6): 1153-62 [PubMed]

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