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Alport syndrome - Treatment

Alternative Names

Hereditary nephritis; Hematuria - nephropathy - deafness; Hemorrhagic familial nephritis; Hereditary deafness and nephropathy

Treatment:

The goals of treatment include monitoring and controlling progression of the disease and treating the symptoms. Most important is to strictly control 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 in the eye is possible.

Loss of hearing is likely to be permanent. Counseling and education to increase coping skills can be helpful. 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):

Women usually have a normal life span with no signs of the disease except for blood in the urine. Rarely, women will have high blood pressure, swelling, and nerve deafness as a complication of pregnancy.

In men, deafness, visual difficulties, and kidney failure are likely by age 50.

Complications:

  • Chronic renal failure
  • Decrease or loss of vision
  • End-stage renal disease
  • Permanent deafness

Calling your health care provider:

Call for an appointment with your health care provider if your symptoms suggest Alport syndrome, or if you have a family history of Alport syndrome and you are planning to have children.

Call your health care provider if your urine output decreases or stops. This may be a symptom of chronic kidney failure.

  • Reviewed last on: 11/12/2007
  • Charles Silberberg, D.O., Private Practice specializing in Nephrology, Affiliated with NY Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network.

References

Behrman RE, Kliegman RM, Jenson HB. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, Pa: Saunders; 2003.