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hereditary nephritis with deafness

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Alport's syndrome is a hereditary disease of basement membranes which is characterised by sensorineural deafness and renal failure.

Inheritance can be X-linked dominant, autosomal dominant and, rarely, autosomal recessive. More information about the genetics of this condition is available in the linked menu item.

Prevalence is about 1:5000.

Pathological renal changes in Alport's syndrome include thickening of the glomerular basement membrane and splitting of the lamina densa.


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