Autosomal dominant polycystic kidney disease with situs inversus.

Situs inversus totalis and autosomal dominant polycystic kidney disease (ADPKD) occur with an incidence of 1/10,000 in the general population and 1 in 1000 live births, respectively. Association of the two conditions is rare and there are few pediatric case reports. Association of situs inversus totalis and autosomal recessive polycystic kidney disease has also been reported. Recent studies have revealed ciliary dysfunction as a cause of both conditions.