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Virtual Pediatric Hospital: Paediapaedia: Adult Polycystic Kidney Disease (Autosomal Dominant Polycystic Kidney Disease) Paediapaedia: Genitourinary Diseases

Adult Polycystic Kidney Disease (Autosomal Dominant Polycystic Kidney Disease)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Normally presents in early adulthood as congestive heart failure, renal failure, and hypertension. Ten percent of cases present in first decade of life, as abdominal masses.

Etiology/Pathophysiology:
Multiple cysts of variable size with areas of intervening normal functioning renal parenchyma. Over time the cysts, which don't communicate with renal tubules or collecting structures, keep enlarging and continue to compress and therefore compromise the functioning renal parenchyma.

Pathology:
Not applicable

Imaging Findings:
Ultrasound and IVP shows bilateral enlarged kidneys composed of multiple large cysts of variable sizes. Forty percent have hepatic cysts, without hepatic fibrosis. Cysts are also seen in the pancreas, spleen, gonads, and lungs. Intracranial aneurysms are seen in 10%.

DDX:

References:
See References Chapter.

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