Alport syndrome
Alternative names:
hematuria - nephropathy - deafness; hemorrhagic familial nephritis; hereditary deafness and nephropathy; hereditary nephritis
Treatment:
Treatment goals include monitoring and controlling progression of the disease and treatment of symptoms. The most important task is strict control of high blood pressure.
Treatment of chronic renal failure will become necessary. This may include dietary modifications, fluid restriction, and other treatments. Ultimately, chronic renal failure progresses to end-stage renal disease, requiring dialysis or transplantation.
Surgical repair of cataracts (cataract extraction) or repair of the anterior lenticonus is possible.
Loss of hearing is likely to be permanent. Counseling and education to increase coping skills, and learning new skills such as lip reading or sign language may be of some benefit. Hearing aids are helpful. Young men with Alport syndrome should use hearing protection in noisy environments.
Genetic counseling may be recommended because of the inherited pattern of the disorder.
Expectations (prognosis):
In women, there is usually a normal life span with no manifestation except for blood in the urine. Rarely, women exhibit hypertension, edema, and nerve deafness as a complication of pregnancy.
In men, deafness, visual difficulties, and renal failure are likely by age 50.
Complications:
Calling your health care provider:
Call for an appointment with your health care provider if symptoms indicate Alport syndrome may be present or if there is a history of Alport syndrome and children are planned.
Call your health care provider if urine output decreases or stops (this may be a symptom of chronic renal failure).
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