Alport Syndrome, also known as chronic hereditary nephritis, is a hereditary renal disease that involves development abnormalities of the renal parenchyma. The basic defect in Alport Syndrome is altered synthesis of the glomerular basement membrane (GBM), and the patient commonly has hematuria (blood in urine) and proressive uremia (A toxic condition resulting from kidney disease in which there is retention in the bloodstream of waste products normally excreted in the urine. Also called azotemia).
There are two forms of Alport Syndrome:
Classic Alport syndrome - inherited as a sex-linked disorder with hematuria, sensorineural deafness, and deformities of the anterior surface of the lens.
Nonclassical Alport syndrome - inherited as an autosomal trait that causes hematuria but not deafness or lens deformities.
Treatment for Alport Syndrome is supportive, and if there is a kidney transplant the disease does not recur.