Enamel-Renal syndrome is caused by a defect in the gene FAM20A (not the other genes listed on this site). The dental defects include lack of enamel for the teeth, failure of the teeth to erupt, misshapen roots, large pulp chambers, with calcifications in the pulp. The nephrocalcinosis may be asymptomatic until early 20s, but is probably bilateral and visible on ultrasound early on (10 years old?). My daughter has this, but we are still trying to understand whether there could be other associated problems and what the prognosis is for her kidneys, long term. Does anyone else have experience with this?