Hereditary ciliopathy characterized by multiple renal cysts & various other systemic manifestations
Pei Ravine criteria: ≥ 3 uni- or bilateral cysts if ≥ 15-years-old; <15-years-old with positive family history, ≥ 1 kidney cyst &/or renal enlargement is considered highly suggestive of ADPKD
Aneurysms: Cerebral berry aneurysms (5-10%); aortic or coronary aneurysms are less common
IMAGING
General Features
Radiographic Findings
Ultrasonographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Benz EG et al: Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease. Pediatr Nephrol. 36(9):2639-58, 2021
Li D et al: MicroRNAs and polycystic kidney disease. Kidney Med. 2(6):762-70, 2020
Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
Thomas CC et al: Ultrasound imaging of renal cysts in children. J Ultrasound Med. 40(3):621-35, 2020
Botwin A et al: Clinical significance of incidentally discovered renal cysts in pediatric patients. Abdom Radiol (NY). 44(8):2835-40., 2019
De Rechter S et al: ADPedKD: A global online platform on the management of children with ADPKD. Kidney Int Rep. 4(9):1271-84, 2019
Garel J et al: Prenatal ultrasonography of autosomal dominant polycystic kidney disease mimicking recessive type: case series. Pediatr Radiol. 49(7):906-12, 2019
Gimpel C et al: Imaging of kidney cysts and cystic kidney diseases in children: an international working group consensus statement. Radiology. 290(3):769-82, 2019
Gimpel C et al: International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol. 15(11):713-26, 2019
De Rechter S et al: Is autosomal dominant polycystic kidney disease becoming a pediatric disorder? Front Pediatr. 5:272, 2017
Strzelczyk M et al: Grayscale ultrasound characteristics of autosomal dominant polycystic kidney disease severity - an adult and pediatric cohort study. J Ultrason. 17(69):85-90, 2017
Cadnapaphornchai MA et al: Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 9(5):889-96, 2014
Chung EM et al: From the radiologic pathology archives: pediatric polycystic kidney disease and other ciliopathies: radiologic-pathologic correlation. Radiographics. 34(1):155-78, 2014
Sweeney WE Jr et al: Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 26(5):675-92, 2011
Barua M et al: Diagnosis of autosomal-dominant polycystic kidney disease: an integrated approach. Semin Nephrol. 30(4):356-65, 2010
Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
Walz G et al: Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40. Epub 2010 Jun 26. Erratum in: N Engl J Med. 2010 Sep 16;363(12):1190. N Engl J Med. 363(20):1977, 2010
de Mattos AM et al: Autosomal-dominant polycystic kidney disease as a risk factor for diabetes mellitus following renal transplantation. Kidney Int. 67(2):714-20, 2005
Lang EK et al: Autosomal dominant polycystic disease with renal cell carcinoma. J Urol. 173(3):987, 2005
Nagaba Y et al: Spontaneous rupture of a left gastroepiploic artery aneurysm in a patient with autosomal-dominant polycystic kidney disease. Clin Nephrol. 63(2):163-6, 2005
Paterson AD et al: Progressive loss of renal function is an age-dependent heritable trait in type 1 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 16(3):755-62, 2005
Hereditary ciliopathy characterized by multiple renal cysts & various other systemic manifestations
Pei Ravine criteria: ≥ 3 uni- or bilateral cysts if ≥ 15-years-old; <15-years-old with positive family history, ≥ 1 kidney cyst &/or renal enlargement is considered highly suggestive of ADPKD
Aneurysms: Cerebral berry aneurysms (5-10%); aortic or coronary aneurysms are less common
IMAGING
General Features
Radiographic Findings
Ultrasonographic Findings
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Benz EG et al: Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease. Pediatr Nephrol. 36(9):2639-58, 2021
Li D et al: MicroRNAs and polycystic kidney disease. Kidney Med. 2(6):762-70, 2020
Meyers ML et al: Imaging of fetal cystic kidney disease: multicystic dysplastic kidney versus renal cystic dysplasia. Pediatr Radiol. 50(13):1921-33, 2020
Thomas CC et al: Ultrasound imaging of renal cysts in children. J Ultrasound Med. 40(3):621-35, 2020
Botwin A et al: Clinical significance of incidentally discovered renal cysts in pediatric patients. Abdom Radiol (NY). 44(8):2835-40., 2019
De Rechter S et al: ADPedKD: A global online platform on the management of children with ADPKD. Kidney Int Rep. 4(9):1271-84, 2019
Garel J et al: Prenatal ultrasonography of autosomal dominant polycystic kidney disease mimicking recessive type: case series. Pediatr Radiol. 49(7):906-12, 2019
Gimpel C et al: Imaging of kidney cysts and cystic kidney diseases in children: an international working group consensus statement. Radiology. 290(3):769-82, 2019
Gimpel C et al: International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat Rev Nephrol. 15(11):713-26, 2019
De Rechter S et al: Is autosomal dominant polycystic kidney disease becoming a pediatric disorder? Front Pediatr. 5:272, 2017
Strzelczyk M et al: Grayscale ultrasound characteristics of autosomal dominant polycystic kidney disease severity - an adult and pediatric cohort study. J Ultrason. 17(69):85-90, 2017
Cadnapaphornchai MA et al: Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol. 9(5):889-96, 2014
Chung EM et al: From the radiologic pathology archives: pediatric polycystic kidney disease and other ciliopathies: radiologic-pathologic correlation. Radiographics. 34(1):155-78, 2014
Sweeney WE Jr et al: Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol. 26(5):675-92, 2011
Barua M et al: Diagnosis of autosomal-dominant polycystic kidney disease: an integrated approach. Semin Nephrol. 30(4):356-65, 2010
Katabathina VS et al: Adult renal cystic disease: a genetic, biological, and developmental primer. Radiographics. 30(6):1509-23, 2010
Walz G et al: Everolimus in patients with autosomal dominant polycystic kidney disease. N Engl J Med. 2010 Aug 26;363(9):830-40. Epub 2010 Jun 26. Erratum in: N Engl J Med. 2010 Sep 16;363(12):1190. N Engl J Med. 363(20):1977, 2010
de Mattos AM et al: Autosomal-dominant polycystic kidney disease as a risk factor for diabetes mellitus following renal transplantation. Kidney Int. 67(2):714-20, 2005
Lang EK et al: Autosomal dominant polycystic disease with renal cell carcinoma. J Urol. 173(3):987, 2005
Nagaba Y et al: Spontaneous rupture of a left gastroepiploic artery aneurysm in a patient with autosomal-dominant polycystic kidney disease. Clin Nephrol. 63(2):163-6, 2005
Paterson AD et al: Progressive loss of renal function is an age-dependent heritable trait in type 1 autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 16(3):755-62, 2005
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