Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of kidney disease. Enlarging cysts within the kidneys are the clinical hallmark of the disease. Renal manifestations include varying degrees of kidney injury, urinary tract infections, kidney stones, and hematuria. Extrarenal manifestations can include pain, hypertension, left ventricular hypertrophy, hepatic cysts, intracranial aneurysm, diverticulosis, and abdominal and inguinal hernias.
Radiographic Diagnostic Criteria for ADPKD
| Age (Years) | Number of Cysts | |
| Ultrasonography (at-risk of ADPKD type 1) | < 30 | ≥ 2 in one or both kidneys |
| 30 to 59 | ≥ 2 in each kidney | |
| ≥ 60 | ≥ 4 in each kidney | |
| Ultrasonography (at risk and unknown genotype) | 15 to 39 | ≥ 3 in one or both kidneys |
| 40 to 59 | ≥ 2 in each kidney | |
| ≥ 60 | ≥ 4 in each kidney | |
| Magnetic resonance imaging (at risk) | < 30 | ≥ 5 in each kidney |
| 30 to 44 | ≥ 6 in each kidney | |
| 45 to 59 (females) | > 6 in each kidney | |
| 45 to 59 (males) | > 9 in each kidney |
References:
- Gradzik M, Niemczyk M, Gołębiowski M, Pączek L. Diagnostic Imaging of Autosomal Dominant Polycystic Kidney Disease. Pol J Radiol. 2016;81:441-453. Published 2016 Sep 17. [Medline]
- Srivastava A, Patel N. Autosomal dominant polycystic kidney disease. Am Fam Physician. 2014;90(5):303-307. [Medline]
- Pei Y, Obaji J, Dupuis A, et al. Unified criteria for ultrasonographic diagnosis of ADPKD. J Am Soc Nephrol. 2009;20(1):205-212. [Medline]
- Gimpel C, Bergmann C, Bockenhauer D, et al. International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol. 2019;15(11):713-726. [Medline]
Created Aug 18, 2020.
