Further Outpatient Care

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

Further Inpatient Care

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

Inpatient & Outpatient Medications

Medications are not needed, although large doses of supplemental vitamin C may be beneficial. Administration of daily nitinosone should be considered.

Transfer

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

Deterrence/Prevention

High-protein diets should be avoided.

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

Complications

Potential complications include the following:

Calcification of the ear cartilage
Calcified lumbar discs
Severe arthritis
Ankylosis
Aortic or mitral valvulitis

Prognosis

Life expectancy is normal.

Risk of myocardial infarction later in life is increased.

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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Upon microscopic examination, amber-colored, oval-shaped structures are detected in the mid-to-upper dermal tissues (hematoxylin and eosin, original magnification X40).

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