Abstract
Over the past 15 years, it has become clear that autosomal-dominant polycystic kidney disease (ADPKD) (previously known as "adult polycystic kidney disease") is an important clinical entity in the pediatric population. Indeed, 60% of children younger than 5 years of age, and 75 to 80% of children 5 to 18 years of age with a PKD-1 mutation have renal cysts detectable by ultrasound1. Renal cysts in children with ADPKD have been associated with a wide clinical spectra, ranging from totally asymptomatic patients to those who present as newborns with massive renal enlargement, hypertension, oliguria, and pulmonary hypoplasia2. These latter patients may be clinically indistinguishable from those with autosomal-recessive PKD.
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