Riccardo Magistroni1, Cristiana Corsi2, Teresa Martí3,4, Roser Torra5. 1. Surgical, Medical and Dental Department of Morphological Sciences related to Transplant, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy. 2. Department of Electrical, Electronic, and Information Engineering "Guglielmo Marconi," University of Bologna, Cesena, Italy. 3. Department of Radiology, Fundació Puigvert, Barcelona, Spain. 4. Department of Radiology, Fundació Hospital Esperit Sant, Barcelona, Spain. 5. Department of Nephrology, Inherited Renal Disorders, Fundació Puigvert, REDINREN, IIB Sant Pau, Universitat Autónoma de Barcelona, Barcelona, Spain.
Abstract
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited renal disorder; it is defined by progressive renal cyst formation and subsequent renal enlargement that leads to end-stage renal disease. Until recently, only symptomatic treatments for ADPKD existed. However, therapies that address the underlying pathophysiology of ADPKD are now available and accurate identification of the rate of disease progression is essential. SUMMARY: Published data on the different imaging modalities for measuring kidney and cyst volumes in ADPKD are reviewed. The advantages and drawbacks of the different techniques for calculating kidney volume from renal imaging are also examined, including the use of manual planimetry, stereology, and the ellipsoid equation, as well as the prospect of semi- and fully automatic techniques. The translation of these approaches into clinical practice and their role in informing treatment decisions is discussed. Key Messages: These new therapies require the accurate monitoring of disease progression, which along with diagnosis and prognosis, relies on the effective use of renal imaging techniques. There is growing support for the use of total kidney volume as a measure of cyst burden and as a prognostic predictor of renal function in ADPKD, showing promise as a marker of disease progression.
BACKGROUND:Autosomal dominant polycystic kidney disease (ADPKD) is the commonest inherited renal disorder; it is defined by progressive renal cyst formation and subsequent renal enlargement that leads to end-stage renal disease. Until recently, only symptomatic treatments for ADPKD existed. However, therapies that address the underlying pathophysiology of ADPKD are now available and accurate identification of the rate of disease progression is essential. SUMMARY: Published data on the different imaging modalities for measuring kidney and cyst volumes in ADPKD are reviewed. The advantages and drawbacks of the different techniques for calculating kidney volume from renal imaging are also examined, including the use of manual planimetry, stereology, and the ellipsoid equation, as well as the prospect of semi- and fully automatic techniques. The translation of these approaches into clinical practice and their role in informing treatment decisions is discussed. Key Messages: These new therapies require the accurate monitoring of disease progression, which along with diagnosis and prognosis, relies on the effective use of renal imaging techniques. There is growing support for the use of total kidney volume as a measure of cyst burden and as a prognostic predictor of renal function in ADPKD, showing promise as a marker of disease progression.
Authors: Nathalie Demoulin; Victoria Nicola; Nicolas Michoux; Valentine Gillion; Thien Anh Ho; Caroline Clerckx; Yves Pirson; Laurence Annet Journal: Kidney Int Rep Date: 2021-08-19
Authors: Roslyn J Simms; Trushali Doshi; Peter Metherall; Desmond Ryan; Peter Wright; Nicolas Gruel; Maatje D A van Gastel; Ron T Gansevoort; Wendy Tindale; Albert C M Ong Journal: Eur Radiol Date: 2019-01-21 Impact factor: 5.315