This is linked to ARPKD, found in young infants and some children before birth. It is caused by the inability to release hydrogen ions in the distal convoluted tubule. It occurs because of the affect that this genetic condition has on amniotic fluid concentration in the amniotic sac. The reduced amniotic fluid results in the amniotic sac compressing the childs skull and resulting in polycystic kidneys. These two factors affect development and often result in the death of these young infants