Alkaptonuria (Black Urine Disease) Follow-up

Updated: Jul 12, 2017
  • Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD  more...
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Follow-up

Further Outpatient Care

Carefully monitor diet with periodic measurement of plasma amino acid levels to avoid phenylalanine deficiency.

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Further Inpatient Care

If treatment for alkaptonuria is successful, the patient does not need to be admitted.

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Inpatient & Outpatient Medications

Medications are not needed, although large doses of supplemental vitamin C may be beneficial.

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Transfer

Transfer is not required if appropriate outpatient consultation and follow-up are obtained.

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Deterrence/Prevention

High-protein diets should be avoided.

No exercise restriction is necessary in the absence of cardiovascular disease.

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Complications

See the list below:

  • Calcification of the ear cartilage

  • Calcified lumbar discs

  • Severe arthritis

  • Ankylosis

  • Aortic or mitral valvulitis

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Prognosis

Life expectancy is normal.

Risk of myocardial infarction later in life is increased.

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Patient Education

Family members should be referred for genetic counseling.

The need for rigorous follow-up with a biochemical geneticist should be emphasized.

Patients and family members should be counseled regarding a likely decreased quality of life.

For excellent patient education resources, see eMedicineHealth's patient education article Arthritis.

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