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Pathology Polycystic Kidney Disease 4878f2bc

Which of the following statement are true regarding autosomal dominant polycystic kidney disease (ADPKD)?

A
Bilateral kidneys are enlarged
B
External surface show numerous cyst of 3-4 cm in diameter
C
Histology shows functioning nephrons dispersed between the cysts
D
All of the above
High-Yield Explanation
Autosomal dominant polycystic kidney disease (ADPKD) Kidneys are bilaterally enlarged External surface appears to be composed solely of a mass of cysts, up to 3 to 4 cm in diameter, with no intervening parenchyma. Microscopic examination - Functioning nephrons dispersed between the cysts. The cysts may be filled with a clear, serous fluid or with turbid, red to brown, sometimes hemorrhagic fluid. Cysts may arise at any level of the nephron, from tubules to collecting ducts, and therefore they have a variable, often atrophic lining. PKD1 gene implicated in 85% of cases.PKD1, on 16p, is the defective gene. This gene encodes polycystin-1. PKD2 gene, implicated in 10% to 15% of cases, resides on chromosome 4 and encodes polycystin-2.

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