Renal stone disease in autosomal dominant polycystic kidney disease

VE Torres, DM Wilson, RR Hattery… - American Journal of Kidney …, 1993 - Elsevier
VE Torres, DM Wilson, RR Hattery, JW Segura
American Journal of Kidney Diseases, 1993Elsevier
Nephrolithiasis is an important manifestation of autosomal dominant polycystic kidney
disease (ADPKD), which occurs in approximately 20% of patients. It should always enter the
differential diagnosis of flank pain in patients with ADPKD. The diagnosis is hindered by the
distorted anatomy of the polycystic kidneys and the frequent occurrence of parenchymal and
cyst wall calcifications, and requires demonstration of the relationship to the collecting
system by intravenous urography and/or computed tomography. Computed tomography is …
Nephrolithiasis is an important manifestation of autosomal dominant polycystic kidney disease (ADPKD), which occurs in approximately 20% of patients. It should always enter the differential diagnosis of flank pain in patients with ADPKD. The diagnosis is hindered by the distorted anatomy of the polycystic kidneys and the frequent occurrence of parenchymal and cyst wall calcifications, and requires demonstration of the relationship to the collecting system by intravenous urography and/or computed tomography. Computed tomography is the most sensitive imaging technique for detection of stones or calcifications, whereas intravenous urography is the most sensitive for visualization of the intrarenal collecting system. Precaliceal tubular ectasia can be detected in 15% of patients with ADPKD and nephrolithiasis, but this association may not be specific to ADPKD. The composition of the stones is most frequently uric acid and/or calcium oxalate. Metabolic factors are important in their pathogenesis. Distal acidification defects may be important in a few patients, while an abnormal transport of ammonium, low urine pH, and hypocitruria are the most common abnormalities. The treatment of nephrolithiasis in patients with ADPKD is not different from that in patients without ADPKD. Extracorporeal shock wave lithotripsy and percutaneous nephrostolithotomy in patients with early disease and normal renal function are not contraindicated.
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