Two mutations in ACE have been described which were linked to premature fetal death due to autosomal recessive renal tubular dysgenesis -RTD [24]–[25], a severe disorder of renal tubular development characterized by persistent fetal anuria and perinatal death, probably due to pulmonary hypoplasia from early-onset oligohydramnios (Potter phenotype). This evidence concerns the gene ACE and renal tubular dysgenesis of genetic origin.