Idiopathic hematuria as a familial, genetically controlled disease is unusual. Reyersbach et al.1 identified this condition in only eight patients in an 18-year period in a large pediatric clinic. Hematuria, per se, is a commonly encountered clinical finding, and that this finding can result from some undefined genetically controlled abnormality must be frequently considered if rather disastrous diagnostic errors are to be avoided.
Three persons with asymptomatic hematuria and one who was asymptomatic for about 15 years and eventually succumbed to nephritis have been found in the family to be discussed here. An attempt has been made to gain some insight into the nature of the genetic transmission of the abnormality. The natural history of the disturbance is of interest with particular regard to the long-term prognosis in children found to have this condition.
The propositus, Case 1, was seen by one of us (E. P. R.)