Which genetic disorder predisposes to bilateral renal cysts and early-onset hypertension?

Prepare for the Urology and Nephrology Test with focused study materials. Utilize flashcards and multiple-choice questions featuring hints and explanations. Ensure your readiness for the examination.

Multiple Choice

Which genetic disorder predisposes to bilateral renal cysts and early-onset hypertension?

Explanation:
Autosomal dominant polycystic kidney disease is the one that best fits this pattern. It arises from mutations such as PKD1 or PKD2, leading to the development of multiple cysts in both kidneys. As these cysts grow and replace normal renal parenchyma, blood pressure tends to rise early because of activation of the renin-angiotensin-aldosterone system and reduced nephron quality. This combination—bilateral renal cysts with early-onset hypertension—is a classic clue for ADPKD. By comparison, autosomal recessive polycystic kidney disease usually presents in infancy with very enlarged kidneys and congenital hepatic fibrosis; von Hippel-Lindau can involve renal cysts but is better known for associated tumors, not the hallmark early hypertension; Alport syndrome causes nephritis with hearing loss rather than bilateral cysts.

Autosomal dominant polycystic kidney disease is the one that best fits this pattern. It arises from mutations such as PKD1 or PKD2, leading to the development of multiple cysts in both kidneys. As these cysts grow and replace normal renal parenchyma, blood pressure tends to rise early because of activation of the renin-angiotensin-aldosterone system and reduced nephron quality. This combination—bilateral renal cysts with early-onset hypertension—is a classic clue for ADPKD.

By comparison, autosomal recessive polycystic kidney disease usually presents in infancy with very enlarged kidneys and congenital hepatic fibrosis; von Hippel-Lindau can involve renal cysts but is better known for associated tumors, not the hallmark early hypertension; Alport syndrome causes nephritis with hearing loss rather than bilateral cysts.

Subscribe

Get the latest from Passetra

You can unsubscribe at any time. Read our privacy policy