Autosomal Dominant Polycystic Kidney Disease
Although it occurs in one in 400 people, autosomal dominant polycystic kidney disease (ADPKD) is relatively unknown outside of the renal medicine community. Discovery of the polycystin genes that code for ADPKD has revolutionized diagnosis of one of the most common genetic diseases. Back and/or flank pain with hematuria are the usual presenting symptoms, but long-term hypertension is the key objective finding. A timely diagnosis and aggressive treatment of hypertension can slow cyst growth and reduce end-stage organ damage, thus extending the life span of the patient and delaying the need for dialysis or transplantation.
60. Perico N, Antiga L, Caroli A, et al. Sirolimus therapy to halt the progression of ADPKD. J Am Soc Nephrol. 2010;21(6):1031-1040.
61. Mario Negri Institute for Pharmacological Research, sponsor. Somatostatin in Polycystic Kidney: a Long-term Three Year Follow up Study. https://clinicaltrials.gov/ct2/show/NCT00309283. Accessed July 22, 2011.
62. Hogan MC, Masyuk TV, Page LJ, et al. Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol. 2010; 21(6):1052-1061.
