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Genetic disorder frequently producing renal failure in childhood. It is the most common genetically determined childhood cystic disease of the kidneys. Congenital malformation of the collecting tubules.

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PCKD.

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A polycystic kidney disease classification has been described by E.L. Potter in 1964 (see Table P-1).

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Table Graphic Jump Location
P-1 Potter's Classification of Polycystic Kidney Disease
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1:10,000 to 1:40,000 live births. The age distribution of cases has two peaks, one at birth and one between the ages of 30 and 60 years.

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Autosomal recessive PKD is a neonatal disease that has been associated to a gene map locus at 6p21.1-p12. The later form is believed to be a familial pattern of an autosomal dominant inheritance. Both sexes are affected. There is also a form of renal cystic disease not inherited and described as the acquired cystic kidney disease, which develops in those individuals with long-term kidney problems.

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There is distention of the renal collecting tubules caused by localized proliferation and aberrant secretion of epithelial cells. The expanding structures develop into cysts that are filled with fluid containing biologically active ligands for the epidermal growth factor receptor (EGFR), such as epidermal growth factor and transforming growth factor-alpha. The EGFR, normally localized at the basolateral surfaces of the collecting tubule epithelium, becomes mislocalized to the apical surface on the cells lining cystic structures. This mislocalization of EGFR is a common end point associated with several different forms of PKD ...

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