JAMA Clinical Reviews

Diagnosis, Treatment, and Prognosis of Autosomal Dominant Polycystic Kidney Disease (ADPKD) in Adults

28 snips
Mar 24, 2025
Nerra Dahl, a renowned professor at Mayo Clinic and director of the Polycystic Kidney Disease Foundation Center of Excellence, dives into the complexities of Autosomal Dominant Polycystic Kidney Disease (ADPKD). She discusses the genetic underpinnings of the disease and its impact on kidney function, emphasizing the importance of genetic testing for effective diagnosis. The conversation also highlights innovative treatments like Tolvaptan, the significance of monitoring intracranial aneurysms, and promising advances in kidney transplantation outcomes for ADPKD patients.
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INSIGHT

PKD Natural History

  • PKD progression involves hypertension, urologic events, cyst complications, and kidney failure risk by age 62.
  • Monitor blood pressure, kidney function, and extra-renal manifestations like aneurysms and liver cysts.
INSIGHT

Liver Cysts in PKD

  • Liver cysts, common in PKD, are usually asymptomatic.
  • However, 5% of patients develop large livers causing various complications.
ADVICE

Intracranial Aneurysm Screening

  • Screen patients with a family history of intracranial aneurysms.
  • For others, discuss screening risks and benefits due to slow growth.
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