| 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.
|
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.
|
Clinical Correlation
•Cases are discovered antenatally if prenatal care includes ultrasonographic
studies.
•Most cases that go to term die shortly after birth from
respiratory difficulties due to the enlarged kidneys that have resulted in
developmental pulmonary hypoplasia.
•This disease is not invariably
fatal. Those cases that survive infancy may subsequently develop hepatic
portal fibrosis, portal hypertension and splenomegaly.
|
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.
|
| Infantile Polycystic Kidney Disease
|
| Synopsis by: Harold Yamase M.D. (T71000M26740)[231]
|
Search Medline at National Library of Medicine
Please be patient during transfer. Medline will open in a new window. To return, close the Medline Window
|