ABSTRACT

CLINICAL HISTORY The disease is characterized by multiple renal and extrarenal manifestations, but there is significant variability in the clinical presentation. Renal size increases with age with renal enlargement occurring in virtually all patients (182-184). Manifestations of renal involvement include pain, hematuria, hypertension, and renal insufficiency. Acute flank pain may occur as a result of cyst hemorrhage, infection, or stone. Macroscopic hematuria occurs in over 40% of patients with ADPKD and may be the presenting symptom. Cyst hemorrhage is frequent. It can present as macroscopic hematuria, or with pain and fever simulating infection of the cyst. In the majority of patients, symptoms resolve within 1 week. Persistence of hematuria, especially if the initial episode occurs after age 50 years, should prompt exclusion of underlying neoplasm. Urinary tract infection may present as cystitis, pyelonephritis, cyst infection, or a perinephric abscess. If cyst infection is suspected, cyst aspiration under ultrasound or CT guidance may need to be undertaken to confirm the diagnosis and guide selection of appropriate antimicrobial therapy.