Hereditary Nephritis in Children

Abstract

Hereditary nephritis usually refers to Alport syndrome and thin basement membrane nephropathy. Hematuria occurs in about 6% of the pediatric population and is mainly transient and nonglomerular, from, for example, cystitis or familial hypercalciuria. The most common cause of persistent glomerular hematuria is thin basement membrane nephropathy, and Alport syndrome is much less frequent. In children, Alport syndrome is one of the commonest causes of end-stage kidney failure after focal and segmental glomerulosclerosis and structural renal disease. Genetic testing has the advantage of not only confirming the diagnosis but also identifying the mode of inheritance. Newer agents undergoing evalua..