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Infantile Polycystic Kidney Disease
Etiology

Genetic, autosomal recessive.
Pathogenesis

The pelvis, calyces, papillae as well as the nephrons are believed to develop normally in the initial stages of renal genesis.
The cystic development of the collecting ducts are believed to occur subsequently by a hyperplastic process.,
Epidemiology

Developmental disorder of the kidneys discovered in developing fetuses (by ultrasound) or in the newborn period (enlarged kidneys).
General Gross Description

The kidneys are bilateral and symmetrically enlarged but retain a reniform shape.
Cut surface of the kidneys shows elongate cylindrical spaces radially arranged from the medulla into the cortex. This feature blurs the normal distinct cortico-medullary junction.
•Examples:
Infantile Polycystic Kidney
General Microscopic Description

On microscopic examination, the cysts are elongate and cylindrical and radiate from the medulla to the cortex.
The cysts are lined by a single layer of cuboidal epithelium similar to that of the collecting ducts.
•Examples:
Infantile Polycystic Kidney Disease
References

Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. 936.
Zerres K, Volpel MC, Weib H: Cystic kidneys. Human Genetics (1984) 8:104-135.

For Most Current Information Search Medline at National Library of Medicine
This link will directly take you to the relevant new literature
Infantile Polycystic Kidney Disease
Synopsis by: Harold Yamase M.D. (T71000M26740)[231]
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