Familial hematuria; Hereditary nephritis; Alport syndrome; Alport syndrome–diffuse leiomyomatosis complex; Alport syndrome–mental retardation complex; Thin basement membrane nephropathy; Epstein syndrome; Fechtner syndrome

Hematuria (blood in the urine) is a common symptom of diseases of the urinary tract, including the kidneys. For the purposes of this essay, familial hematuria is defined as a group of genetic disorders of glomerular capillaries that are characterized clinically by the onset of persistent hematuria during childhood. Hematuria may be the presenting feature of other heritable diseases, including polycystic kidney disease, hypercalciuria and other forms of hereditary urolithiasis, which will not be discussed here.

Alport Syndrome:Alport syndrome is a generalized disorder of basement membranes resulting from defective incorporation of a type IV collagen network composed of α3, α4 and α5 type IV collagen chains. Although basement...