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an autosomal dominant disorder marked by progressive nerve deafness, progressive pyelonephritis or glomerulonephritis, and occasionally ocular defects.
Etymology: A.C. Alport, South African physician, 1880-1959
a form of hereditary nephritis (autosomal-dominant, autosomal-recessive, and x-linked) with symptoms of glomerulonephritis, hematuria, progressive sensorineural hearing loss, and occasionally, ocular disorders such as cataracts, drusen, and lenticonus. The trait is transmitted most often through females, who are often asymptomatic. In males, kidney impairment tends to develop in the third decade; death from renal complications occurs in middle age. Treatment is directed toward the relief of uremia or other kidney disorders. Kidney transplantation and dialysis are sometimes successful treatments.