Alkaptonuria (patient information)
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What is Alkaptonuria?
Alkaptonuria is a rare condition in which a person's urine turns a dark brownish-black color when exposed to air.
What are the symptoms of Alkaptonuria?
Urine in an infant's diaper may darken and can turn almost black after several hours. However, many persons with this condition may not know they have it until mid-adulthood (around age 40), when joint and other problems occur.
Symptoms may include:
- Arthritis (especially of the spine) that gets worse over time
- Darkening of the ear
- Dark spots on the white of the eye (sclera) and cornea
How to know you have Alkaptonuria?
A urine test (urinalysis) is done to test for alkaptonuria. If ferric chloride is added to the urine, it will turn the urine a black color in patients with this condition.
When to seek urgent medical care
Call your health care provider if you notice that your own urine or your child's urine becomes dark brown or black when it is exposed to air.
Treatment options
Some patients benefit from high-dose vitamin C. This has been shown to decrease the build-up of brown pigment in the cartilage and may slow the development of arthritis.