ABSTRACT

Cyst formation is seen in a variety of kidney diseases (Table 22.1). Many of these diseases are genetically inherited, and many are associated with extrarenal manifestations. They are generally relatively rare diseases, although a few are common enough to cause considerable pediatric morbidity and mortality. Autosomal dominant polycystic kidney disease (ADPKD) is actually one of the most common human genetic diseases, but its manifestations are usually fairly subtle in pediatric patients. It does cause considerable morbidity for adults, and it is likely that future therapy of this disease will focus on the pediatric patient.