TY - JOUR
T1 - Volume Progression and Imaging Classification of Polycystic Liver in Early Autosomal Dominant Polycystic Kidney Disease
AU - CRISP and HALT PKD Consortium
AU - Bae, Kyongtae T.
AU - Tao, Cheng
AU - Feldman, Robert
AU - Yu, Alan S.L.
AU - Torres, Vicente E.
AU - Perrone, Ronald D.
AU - Chapman, Arlene B.
AU - Brosnahan, Godela
AU - Steinman, Theodore I.
AU - Braun, William E.
AU - Mrug, Michal
AU - Bennett, William M.
AU - Harris, Peter C.
AU - Srivastava, Avantika
AU - Landsittel, Douglas P.
AU - Abebe, Kaleab Z.
N1 - Funding Information:
K.T. Bae reports consultancy agreements with Kadmon, Otsuka, and Sanofi-Genzyme and honoraria from Kadmon and Otsuka. W.M. Bennett reports honoraria from the American Society of Nephrology and serving as an Editor-in-Chief, Emeritus of CJASN. A.B. Chapman reports consultancy agreements with Guidepoint Global, Janssen, NovusMed, Otsuka Pharmaceuticals, Pfizer, Inc., Pfizer Pharmaceuticals, Reata, and Sanofi-Genzyme; research funding from Boston Scientific, the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), Otsuka, Reata, and Sanofi-Genzyme; honoraria from Guidepoint Global, Otsuka, Reata, and UpToDate; serving as an external advisor to the O’Brien Center, Northwestern University; speakers bureau for Otsuka; and other interests/relationships with the Department of Defense Review Committee and the Special Emphasis Panel and Review Panel, the National Institutes of Health/NIDDK and Small Business Innovation Research. P.C. Harris reports consultancy agreements with Mitobridge, Otsuka, Regulus, and Vertex; research funding from Acceleron, Jemincare, Navitor, and Otsuka Pharmaceuticals; and patents and inventions with Amgen, Bayer, Gen-zyme, GlaxoSmithKline, Millipore, Mitobridge, and Vertex. D.P. Landsittel reports consultancy agreements with the Society for Critical Care Medicine (not related to this project); research funding from Eli Lilly & Company Pharmaceuticals and GlaxoSmith-Kline Pharmaceuticals to his university; and serving as chair of the Centers for Disease Control and Prevention/National Institute For Occupational Safety and Health Safety and Occupational Study Section, a member of the external expert panel for the “Prevention of Lower Urinary Tract Symptoms in Women” project funded through NIDDK, and a member of the data safety monitoring board for the Improving Chronic Disease Management with Pieces trial funded through NIDDK. M. Mrug reports employment with the Department of Veterans Affairs Medical Center (Birmingham, AL); consultancy agreements with Caraway Therapeutics, Chinook, Goldilocks Therapeutics, Natera, Otsuka Corp., Reata, and Sanofi-Genzyme; research funding from Chinook, Goldilocks Therapeutics, Otsuka Corp., and Sanofi-Genzyme; honoraria from Chinook, Otsuka Corp., Natera, Reata, and Sanofi; serving as a scientific advisor or member of the Polycystic Kidney Disease Foundation and the Medical Research Study Designed to Determine if Venglustat Can be a Future Treatment for ADPKD Patients steering committee (Sanofi); and serving on the advisory boards of Carraway Therapeutics, Goldilocks Therapeutics, and Santa Barbara Nutrients. R.D. Perrone reports consultancy agreements with Caraway, Navitor, Otsuka, Palladiobio, Reata, and Sanofi-Genzyme; research funding from Kadmon, Otsuka, Palladiobio, Reata, and Sanofi-Genzyme; honoraria from Otsuka and Sanofi-Genzyme; serving as a scientific advisor or member of Otsuka, PalladioBio, Sanofi-Genzyme, and UpToDate; and other interests/relationships with the PKD Foundation and UpToDate. T.I. Steinman reports research funding from Kadmon, Reata, Regulus, and Travere; honoraria from Mallinkrodt and Otsuka; serving on the Nephrology News and Issues editorial board; serving on the medical advisory board of National Kidney Foundation; serving as a committee member for transforming dialysis safety, International Society of Nephrology; and other interests or relationships with the National Kidney Foundation and the Polycystic Kidney Foundation. V.E. Torres reports consultancy agreements with Blueprint Medicines, Mironid, Otsuka Pharmaceuticals, Palladio, Reata, Regulus, and Sanofi; research funding from Blueprint Medicines, Mironid, Otsuka Pharmaceuticals, Palladio Biosciences, Reata, Regulus (all preclinical trial, preclinical research, or clinical trials), and Sanofi-Genzyme; honoraria from Otsuka Pharmaceuticals (to institution); and serving as a scientific advisor or member of Mironid, Otsuka Pharmaceuticals, Palladio, Reata, and Sanofi-Genzyme. A.S.L. Yu reports consultancy agreements with Calico, Otsuka, Navitor, Palladio, and Regulus Therapeutics; ownership interest in Amgen Corp., Gilead Sciences, Pfizer, and Prothena; research funding from Regulus and Sanofi; honoraria from Elsevier, Otsuka, and Wolters Kluwer; and served on an advisory board for Otsuka. All remaining authors have nothing to disclose.
Funding Information:
This work was supported by National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health grants DK056943, DK056956, DK056957, DK056961, and R01 DK113111. This study was also supported in part by the National Institute of Diabetes and Digestive and Kidney Diseases; PKD grant DK106912; Mayo Translational PKD Center grant DK090728; Emory University; National Center for Research Resources grant RR000039; Kansas University Medical Center grants RR033179, RR23940, and TR000001; National Center for Advancing Translational Sciences grants RR025008 and TR000454; Mayo College of Medicine grants RR00585, RR024150, and TR000135; University of Alabama at Birmingham grants RR000032, RR025777, TR000165, and TR001417; and University of Pittsburgh School of Medicine grants RR024153 and TR000005.
Publisher Copyright:
© 2022 by the American Society of Nephrology.
PY - 2022/3
Y1 - 2022/3
N2 - Background and objectives The progression of polycystic liver disease is not well understood. The purpose of the study is to evaluate the associations of polycystic liver progression with other disease progression variables and classify liver progression on the basis of patient’s age, height-adjusted liver cystic volume, and height-adjusted liver volume. Design, setting, participants, & measurements Prospective longitudinal magnetic resonance images from 670 patients with early autosomal dominant polycystic kidney disease for up to 14 years of follow-up were evaluated to measure height-adjusted liver cystic volume and height-adjusted liver volume. Among them, 245 patients with liver cyst volume >50 ml at baseline were included in the longitudinal analysis. Linear mixed models on log-transformed height-adjusted liver cystic volume and height-adjusted liver volume were fitted to approximate mean annual rate of change for each outcome. The association of sex, body mass index, genotype, baseline height-adjusted total kidney volume, and Mayo imaging class was assessed. We calculated height-adjusted liver cystic volume ranges for each specific age and divided them into five classes on the basis of annual percentage increase in height-adjusted liver cystic volume. Results The mean annual growth rate of height-adjusted liver cystic volume was 12% (95% confidence interval, 11.1% to 13.1%; P<0.001), whereas that for height-adjusted liver volume was 2% (95% confidence interval, 1.9% to 2.6%; P<0.001). Women had higher baseline height-adjusted liver cystic volume than men, but men had higher height-adjusted liver cystic volume growth rate than women by 2% (95% confidence interval, 0.4% to 4.5%; P=0.02). Whereas the height-adjusted liver cystic volume growth rate decreased in women after menopause, no decrease was observed in men at any age. Body mass index, genotype, and baseline height-adjusted total kidney volume were not associated with the growth rate of height-adjusted liver cystic volume or height-adjusted liver volume. According to the height-adjusted liver cystic volume growth rate, patients were classified into five classes (number of women, men in each class): A (24, six); B (44, 13); C (43, 48); D (28, 17); and E (13, nine). Conclusions Compared with height-adjusted liver volume, the use of height-adjusted liver cystic volume showed greater separations in volumetric progression of polycystic liver disease. Similar to the Mayo imaging classification for the kidney, the progression of polycystic liver disease may be categorized on the basis of patient’s age and height-adjusted liver cystic volume.
AB - Background and objectives The progression of polycystic liver disease is not well understood. The purpose of the study is to evaluate the associations of polycystic liver progression with other disease progression variables and classify liver progression on the basis of patient’s age, height-adjusted liver cystic volume, and height-adjusted liver volume. Design, setting, participants, & measurements Prospective longitudinal magnetic resonance images from 670 patients with early autosomal dominant polycystic kidney disease for up to 14 years of follow-up were evaluated to measure height-adjusted liver cystic volume and height-adjusted liver volume. Among them, 245 patients with liver cyst volume >50 ml at baseline were included in the longitudinal analysis. Linear mixed models on log-transformed height-adjusted liver cystic volume and height-adjusted liver volume were fitted to approximate mean annual rate of change for each outcome. The association of sex, body mass index, genotype, baseline height-adjusted total kidney volume, and Mayo imaging class was assessed. We calculated height-adjusted liver cystic volume ranges for each specific age and divided them into five classes on the basis of annual percentage increase in height-adjusted liver cystic volume. Results The mean annual growth rate of height-adjusted liver cystic volume was 12% (95% confidence interval, 11.1% to 13.1%; P<0.001), whereas that for height-adjusted liver volume was 2% (95% confidence interval, 1.9% to 2.6%; P<0.001). Women had higher baseline height-adjusted liver cystic volume than men, but men had higher height-adjusted liver cystic volume growth rate than women by 2% (95% confidence interval, 0.4% to 4.5%; P=0.02). Whereas the height-adjusted liver cystic volume growth rate decreased in women after menopause, no decrease was observed in men at any age. Body mass index, genotype, and baseline height-adjusted total kidney volume were not associated with the growth rate of height-adjusted liver cystic volume or height-adjusted liver volume. According to the height-adjusted liver cystic volume growth rate, patients were classified into five classes (number of women, men in each class): A (24, six); B (44, 13); C (43, 48); D (28, 17); and E (13, nine). Conclusions Compared with height-adjusted liver volume, the use of height-adjusted liver cystic volume showed greater separations in volumetric progression of polycystic liver disease. Similar to the Mayo imaging classification for the kidney, the progression of polycystic liver disease may be categorized on the basis of patient’s age and height-adjusted liver cystic volume.
KW - ADPKD
KW - Diagnostic imaging
KW - Disease progression
KW - Genetic renal disease
KW - Kidney volume
KW - Liver
KW - Liver cysts
KW - Polycystic kidney disease
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U2 - 10.2215/CJN.08660621
DO - 10.2215/CJN.08660621
M3 - Article
C2 - 35217526
AN - SCOPUS:85125964928
VL - 17
SP - 374
EP - 384
JO - Clinical Journal of the American Society of Nephrology
JF - Clinical Journal of the American Society of Nephrology
SN - 1555-9041
IS - 3
ER -