Kidney Disease Progression in Autosomal Recessive Polycystic Kidney Disease - 24/03/16

Doi : 10.1016/j.jpeds.2015.12.079

Katherine M. Dell, MD ^1,^⁎ , Matthew Matheson, MS ², Erum A. Hartung, MD ³, Bradley A. Warady, MD ⁴, Susan L. Furth, MD, PhD ³
on behalf of the
Chronic Kidney Disease in Children (CKiD) Study^{^∗}
List of additional members of CKiD Study is available at www.jpeds.com (Appendix).
Alvaro Muñoz, PhD, Allison Dart, MD, MSc, FRCPC, Larry Greenbaum, MD, PhD, Jens Goebel, MD, Mark Mitsnefes, MD, Joseph Flynn, MD, Craig Wong, MD, Sahar Fathallah, MD, Isidro Salusky, MD, Ora Yadin, MD, Bruce Morgenstern, MD, Tom Blydt-Hansen, MD, FRCPC, Keefe Davis, MD, Cynthia Pan, MD, Amira Al-Uzri, MD, Randall Jenkins, MD, Anthony Portale, MD, Mouin Seikaly, MD, Martin Turman, MD, PhD, Cynthia Wong, MD, Steven Alexander, MD, Colleen Hastings, MD, Randall Jenkins, MD, Nancy Rodig, MD, William Harmon, MD, Sharon Bartosh, MD, Nadine Benador, MD, Robert Mak, MD, PhD, Ellen Wood, MD, Randall Jenkins, MD, Gary Lerner, MD, Susan Massengill, MD, Guillermo Hidalgo, MD, Meredith Atkinson, MD, Debbie Gipson, MD, Poyyapakkam Srivaths, MD, Joshua Samuels, MD, Frederick Kaskel, MD, PhD, Debora Mattosian, MD, Yi Cai, MD, Sharon Andreoli, MD, PhD, Jeffrey Saland, MD, Hiren Patel, MD, Victoria Norwood, MD, Rulan Parekh, MD, Lisa Robinson, MD, Susan Mendley, MD, Marc Lande, MD, George Schwartz, MD, Patrick Brophy, MD, Eunice John, MD, Kiran Upadhyay, MD, Maria Ferris, MD, Tej Matoo, MD, Juan Kupferman, MD, Lynne Weiss, MD, Craig Langman, MD, Patricia Seo-Mayer, MD, Kanwal Kher, MD, Dmitry Samsonov, MD

¹ Center for Pediatric Nephrology, Cleveland Clinic Children's, Department of Pediatrics, Case Western Reserve University, Cleveland, OH
² Department of Epidemiology, Johns Hopkins University, Baltimore, MD
³ Division of Nephrology, Children's Hospital of Philadelphia, Philadelphia, PA
⁴ Division of Pediatric Nephrology, Children's Mercy Hospital, Kansas City, MO

^∗Reprint requests: Katherine M. Dell, MD, 9500 Euclid Avenue, A12-800E, Cleveland, OH 44195.9500 Euclid AvenueA12-800EClevelandOH44195

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 m² (−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.

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Keyword : A/H/D, ACEI, ARPKD, CKD, CKiD, CT, eGFR, GFR, HTN, iGFR, LVH, OU, UPC

Plan

Methods

Statistical Analyses

Results

Renal Function (GFR) Decline

HTN, LVH, and Proteinuria

Discussion

CKiD Study is supported by the National Institute of Diabetes and Digestive and Kidney Diseases (U01-DK-66143, U01-DK-66174, U01DK-082194, U01-DK-66116), the Eunice Kennedy Shriver National Institute of Child Health and Human Development (U01-DK-66143, U01-DK-66174) and the National Heart, Lung, and Blood Institute (U01-DK-66143, U01-DK-66174). K.D. receives support from the NIDDK (R01-DK-085099). The authors declare no conflicts of interest.

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Kidney Disease Progression in Autosomal Recessive Polycystic Kidney Disease - 24/03/16

Abstract

Objective

Study design

Results

Conclusions

Plan

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