Alport syndrome

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Male urinary system

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

The goals of treatment include monitoring and controlling progression of the disease and treating the 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 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.

In women, there is usually 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 renal failure are likely by age 50.

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

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

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

Review Date: 12/09/2005
Reviewed By: Colm C. Magee, MD, MPH, Medical Director, Renal Transplant, Brigham & Women's Hospital, and Assistant Professor, Harvard Medical School, Boston, MA. Review provided by VeriMed Healthcare Network.