Pathology of the urinary system

5b) Polycystic kidney disease (PKD)

 

   ∙ 

inherited in humans, in at least two genetically distinguishable forms which can be associated with 

extrarenal manifestations, including biliary and pancreatic cysts: 

        -  the recessive form is often diagnosed in early infancy by massive nephromegaly and is 

rapidly progressive; cysts are small, 

∼

1-5 mm in diameter, and involve both cortex and 

medulla; 

        -  the dominant form can lead to death from renal failure in late adulthood; cysts can be up to 3-

4 cm in diameter, from gradual increase in size and, perhaps, number over time, and, grossly, 

appear to replace the entire renal parenchyma; 

   ∙ 

in animals, the most common form resembles human recessive PKD, with stillbirths or death within 

the first few weeks of life; syndromes resembling human dominant PKD have also been described; 

   ∙ 

Persian cats appear to be disproportionately affected by PKD; 

---

 

 

8

 

   ∙ 

the basic defect may be a failure in terminal differentiation of renal collecting and biliary ducts. 

 

 

6) Familial (hereditary) renal disease

 

(= progressive juvenile nephropathy)

 

   ∙