Kidney Disease Progression in Autosomal Recessive Polycystic Kidney Disease
Katherine M Dell, Matthew Matheson, Erum A Hartung, Bradley A Warady, Susan L Furth, Chronic Kidney Disease in Children (CKiD) Study, Alvaro Muñoz, Allison Dart, Larry Greenbaum, Jens Goebel, Mark Mitsnefes, Joseph Flynn, Craig Wong, Sahar Fathallah, Isidro Salusky, Ora Yadin, Bruce Morgenstern, Tom Blydt-Hansen, Keefe Davis, Cynthia Pan, Amira Al-Uzri, Randall Jenkins, Anthony Portale, Mouin Seikaly, Martin Turman, Cynthia Wong, Steven Alexander, Colleen Hastings, Randall Jenkins, Nancy Rodig, William Harmon, Sharon Bartosh, Nadine Benador, Robert Mak, Ellen Wood, Randall Jenkins, Gary Lerner, Susan Massengill, Guillermo Hidalgo, Meredith Atkinson, Debbie Gipson, Poyyapakkam Srivaths, Joshua Samuels, Frederick Kaskel, Debora Mattosian, Yi Cai, Sharon Andreoli, Jeffrey Saland, Hiren Patel, Victoria Norwood, Rulan Parekh, Lisa Robinson, Susan Mendley, Marc Lande, George Schwartz, Patrick Brophy, Eunice John, Kiran Upadhyay, Maria Ferris, Tej Matoo, Juan Kupferman, Lynne Weiss, Craig Langman, Patricia Seo-Mayer, Kanwal Kher, Dmitry Samsonov, Katherine M Dell, Matthew Matheson, Erum A Hartung, Bradley A Warady, Susan L Furth, Chronic Kidney Disease in Children (CKiD) Study, Alvaro Muñoz, Allison Dart, Larry Greenbaum, Jens Goebel, Mark Mitsnefes, Joseph Flynn, Craig Wong, Sahar Fathallah, Isidro Salusky, Ora Yadin, Bruce Morgenstern, Tom Blydt-Hansen, Keefe Davis, Cynthia Pan, Amira Al-Uzri, Randall Jenkins, Anthony Portale, Mouin Seikaly, Martin Turman, Cynthia Wong, Steven Alexander, Colleen Hastings, Randall Jenkins, Nancy Rodig, William Harmon, Sharon Bartosh, Nadine Benador, Robert Mak, Ellen Wood, Randall Jenkins, Gary Lerner, Susan Massengill, Guillermo Hidalgo, Meredith Atkinson, Debbie Gipson, Poyyapakkam Srivaths, Joshua Samuels, Frederick Kaskel, Debora Mattosian, Yi Cai, Sharon Andreoli, Jeffrey Saland, Hiren Patel, Victoria Norwood, Rulan Parekh, Lisa Robinson, Susan Mendley, Marc Lande, George Schwartz, Patrick Brophy, Eunice John, Kiran Upadhyay, Maria Ferris, Tej Matoo, Juan Kupferman, Lynne Weiss, Craig Langman, Patricia Seo-Mayer, Kanwal Kher, Dmitry Samsonov
Abstract
Objective: To define glomerular filtration rate (GFR) decline, hypertension (HTN), and proteinuria in subjects with autosomal recessive polycystic kidney disease (ARPKD) and compare with 2 congenital kidney disease control groups in the Chronic Kidney Disease in Children cohort.
Study design: GFR decline (iohexol clearance), rates of HTN (ambulatory/casual blood pressures), antihypertensive medication usage, left ventricular hypertrophy, and proteinuria were analyzed in subjects with ARPKD (n = 22) and 2 control groups: aplastic/hypoplastic/dysplastic disorders (n = 44) and obstructive uropathies (n = 44). Differences between study groups were examined with the Wilcoxon rank sum test.
Results: Annualized GFR change in subjects with ARPKD was -1.4 mL/min/1.73 m(2) (-6%), with greater decline in subjects age ≥ 10 years (-11.5%). However, overall rates of GFR decline did not differ significantly in subjects with ARPKD vs controls. There were no significant differences in rates of HTN or left ventricular hypertrophy, but subjects with ARPKD had a greater percent on ≥ 3 blood pressure medications (32% vs 0%, P < .0001), more angiotensin-converting enzyme inhibitor use (82% vs 27% vs 36%, P < .0005), and less proteinuria (urine protein: creatinine = 0.1 vs 0.6, P < .005).
Conclusions: This study reports rates of GFR decline, HTN, and proteinuria in a small but well-phenotyped ARPKD cohort. The relatively slow rate of GFR decline in subjects with ARPKD and absence of significant proteinuria suggest that these standard clinical measures may have limited utility in assessing therapeutic interventions and highlight the need for other ARPKD kidney disease progression biomarkers.
Copyright © 2016 Elsevier Inc. All rights reserved.
Source: PubMed