TY - JOUR
T1 - Renal structure in early autosomal-dominant polycystic kidney disease (ADPKD)
T2 - The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort
AU - Chapman, Arlene B.
AU - Guay-Woodford, Lisa M.
AU - Grantham, Jared J.
AU - Torres, Vicente E.
AU - Bae, Kyongtae T.
AU - Baumgarten, Deborah A.
AU - Kenney, Philip J.
AU - King, Bernard F.
AU - Glockner, James F.
AU - Wetzel, Louis H.
AU - Brummer, Marijn E.
AU - O'Neill, W. Charles
AU - Robbin, Michelle L.
AU - Bennett, William M.
AU - Klahr, Saulo
AU - Hirschman, Gladys H.
AU - Kimmel, Paul L.
AU - Thompson, Paul A.
AU - Miller, J. Philip
N1 - Funding Information:
This study was supported by the UO1-DK56956 grant for the National Institute of Diabetes and Digestive and Kidney Diseases and the General Clinical Research Center grants MO1-RR00039 (Emory University), MO1-RR00052 (University of Alabama at Birmingham), and MO1-RR00585 (the Mayo Foundation) for the Division of Research Resources, National Institutes of Health. The authors greatly appreciate the assistance of the study coordinators, Diane Watkins, MAS; Lorna Stevens, RN; Jody Mahan, RN; and Pam Truell, RN. These data were presented in part at the American Society of Nephrology 2002 Annual Meeting, Sunday November 3, 2002, Philadelphia, PA.
PY - 2003/9/1
Y1 - 2003/9/1
N2 - Background. Autosomal-dominant polycystic kidney disease (ADPKD) is characterized by gradual renal enlargement and cyst growth prior to loss of renal function. Standard radiographic imaging has not provided the resolution and accuracy necessary to detect small changes in renal volume or to reliably measure renal cyst volumes. The Consortium for Radiologic Imaging Studies in Polycystic Kidney Disease (CRISP) is longitudinally observing ADPKD individuals using high-resolution magnetic resonance (MR) imaging to determine if change in renal and cyst volumes can be detected over a short period of time, and if they correlate with decline in renal function early in disease. Methods. Standardization studies were conducted in phantoms and four subjects at each participating clinical center. After, in the full-scale protocol, healthy ADPKD individuals 15 to 45 years old with creatinine clearance >70 mL/min underwent standardized MR renal imaging, renal iothalamate clearance, comprehensive clinical evaluation, and determination of 24-hour urinary albumin and electrolyte excretion. Stereology was used from T1-weighted images to quantify renal volume, and region-growing thresholding was used from T2-weighted images to determine cyst volume. Renal structures were evaluated in relation to demographic, clinical, and biochemical variables using means/medians, standard deviations, and Pearson correlations. Results. Reliability coefficients for MR renal and cyst volume measurements in phantoms were 99.9% and 89.2%, respectively. In the full-scale protocol, 241 ADPKD individuals (145 women and 96 men) were enrolled. Total renal, cyst, and % cyst volume were significantly greater in each decade group. Hypertensive individuals demonstrated greater renal, cyst, and % cyst volume than normotensive subjects. Age-adjusted renal (r = -0.31, P < 0.0001), cyst (r = -0.36, P < 0.0001), and % cyst volume (r = -0.35, P < 0.0001) were inversely related to glomerular filtration rate (GFR). Age-adjusted renal volume (r = 0.42, P < 0.0001), cystic (r = 0.39, P < 0.0001, and % cyst volume (r = 0.41, P < 0.0001) were related with urinary albumin excretion. Conclusion. MR measures of renal and cyst volume are reliable and accurate in patients with ADPKD. ADPKD is characterized by significant cystic involvement that increases with age. Structure (renal and cyst volume) and function (GFR) are inversely related and directly related with the presence of hypertension and urinary albumin excretion in individuals with normal renal function.
AB - Background. Autosomal-dominant polycystic kidney disease (ADPKD) is characterized by gradual renal enlargement and cyst growth prior to loss of renal function. Standard radiographic imaging has not provided the resolution and accuracy necessary to detect small changes in renal volume or to reliably measure renal cyst volumes. The Consortium for Radiologic Imaging Studies in Polycystic Kidney Disease (CRISP) is longitudinally observing ADPKD individuals using high-resolution magnetic resonance (MR) imaging to determine if change in renal and cyst volumes can be detected over a short period of time, and if they correlate with decline in renal function early in disease. Methods. Standardization studies were conducted in phantoms and four subjects at each participating clinical center. After, in the full-scale protocol, healthy ADPKD individuals 15 to 45 years old with creatinine clearance >70 mL/min underwent standardized MR renal imaging, renal iothalamate clearance, comprehensive clinical evaluation, and determination of 24-hour urinary albumin and electrolyte excretion. Stereology was used from T1-weighted images to quantify renal volume, and region-growing thresholding was used from T2-weighted images to determine cyst volume. Renal structures were evaluated in relation to demographic, clinical, and biochemical variables using means/medians, standard deviations, and Pearson correlations. Results. Reliability coefficients for MR renal and cyst volume measurements in phantoms were 99.9% and 89.2%, respectively. In the full-scale protocol, 241 ADPKD individuals (145 women and 96 men) were enrolled. Total renal, cyst, and % cyst volume were significantly greater in each decade group. Hypertensive individuals demonstrated greater renal, cyst, and % cyst volume than normotensive subjects. Age-adjusted renal (r = -0.31, P < 0.0001), cyst (r = -0.36, P < 0.0001), and % cyst volume (r = -0.35, P < 0.0001) were inversely related to glomerular filtration rate (GFR). Age-adjusted renal volume (r = 0.42, P < 0.0001), cystic (r = 0.39, P < 0.0001, and % cyst volume (r = 0.41, P < 0.0001) were related with urinary albumin excretion. Conclusion. MR measures of renal and cyst volume are reliable and accurate in patients with ADPKD. ADPKD is characterized by significant cystic involvement that increases with age. Structure (renal and cyst volume) and function (GFR) are inversely related and directly related with the presence of hypertension and urinary albumin excretion in individuals with normal renal function.
KW - Autosomal-dominant polycystic kidney disease
KW - Cyst volume
KW - Magnetic resonance imaging
KW - Renal function
KW - Renal volume
UR - http://www.scopus.com/inward/record.url?scp=12444274302&partnerID=8YFLogxK
U2 - 10.1046/j.1523-1755.2003.00185.x
DO - 10.1046/j.1523-1755.2003.00185.x
M3 - Article
C2 - 12911554
AN - SCOPUS:12444274302
SN - 0085-2538
VL - 64
SP - 1035
EP - 1045
JO - Kidney International
JF - Kidney International
IS - 3
ER -