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Neurocognition in children with autosomal recessive polycystic kidney disease in the CKiD cohort study

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Abstract

Background

Autosomal recessive polycystic kidney disease (ARPKD) is an inherited disorder characterized by enlarged, cystic kidneys with progressive chronic kidney disease (CKD), systemic hypertension, and congenital hepatic fibrosis. Children with ARPKD can have early onset CKD and severe hypertension, both of which are known to have adverse neurocognitive effects. The objectives of this study were (1) to determine whether ARPKD patients have greater neurocognitive deficits compared to that of children with other causes of CKD, and (2) to examine the relative prevalence of hypertension in ARPKD, a known risk factor for neurocognitive dysfunction.

Methods

We performed a cross-sectional, control-matched analysis of 22 ARPKD patients with mild-to-moderate CKD in the Chronic Kidney Disease in Children (CKiD) cohort study, compared with a control group of 44 children with other causes of CKD, matched based on glomerular filtration rate, age at study entry, and age at diagnosis.

Results

Children with ARPKD in this cohort had neurocognitive functioning comparable to children with other causes of CKD in domains of intellectual functioning, academic achievement, attention regulation, executive functioning, and behavior. Blood pressure parameters were similar between the two groups; however, ARPKD patients required a significantly greater number of antihypertensive medications to achieve similar BP levels.

Conclusions

ARPKD patients are potentially at risk for neurocognitive dysfunction due to early onset CKD and more severe hypertension. However, this study of children with mild-to-moderate CKD in the CKiD cohort did not demonstrate increased risk in children with ARPKD compared to children with other causes of CKD. Further studies are needed to determine if these findings are applicable to children with more severe manifestations of ARPKD.

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Acknowledgments

The authors thank Lynn Pereira for assistance with statistical analysis.

Dr. Hartung is supported by the National Center for Advancing Translational Sciences (NCATS) of the National Institutes of Health (NIH) under Award Number KL2TR000139. The content is solely the responsibility of the authors and does not necessarily represent the official view of NCATS or the NIH.

Data in this manuscript was collected by the Chronic Kidney Disease in Children prospective cohort study (CKiD) with clinical coordinating centers (Principal Investigators) at Children’s Mercy Hospital and the University of Missouri – Kansas City (Bradley Warady, MD) and Children’s Hospital of Philadelphia (Susan Furth, MD, Ph.D.), Central Biochemistry Laboratory (George Schwartz, MD) at the University of Rochester Medical Center, and data coordinating center (Alvaro Muñoz Ph. D.) at the Johns Hopkins Bloomberg School of Public Health. The CKiD Study is supported by grants from the National Institute of Diabetes and Digestive and Kidney Diseases, with additional funding from the Eunice Kennedy Shriver National Institute of Child Health and Human Development, and the National Heart, Lung, and Blood Institute (U01-DK-66143, U01-DK-66174, U01DK-082194, U01-DK-66116). The CKID website is located at http://www.statepi.jhsph.edu/ckid.

Author information

Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, Children’s Hospital of Philadelphia, 34th & Civic Center Boulevard, Philadelphia, PA, 19104, USA

    Erum A. Hartung & Susan L. Furth

  2. Department of Epidemiology, Johns Hopkins Bloomberg School of Public Heath, Baltimore, MD, USA

    Matthew Matheson

  3. Division of Nephrology, Department of Pediatrics, University of Rochester Medical Center, Rochester, NY, USA

    Marc B. Lande

  4. Department of Pediatrics, Case Western Reserve University and Cleveland Clinic Children’s, Cleveland, OH, USA

    Katherine M. Dell

  5. Center for Translational Science, Children’s National Health System, Washington, DC, USA

    Lisa M. Guay-Woodford

  6. Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD, USA

    Arlene C. Gerson

  7. Division of Pediatric Nephrology, Children’s Mercy Hospital, Kansas City, MO, USA

    Bradley A. Warady

  8. Department of Allied Health Sciences and Department of Psychiatry, University of North Carolina School of Medicine, Chapel Hill, NC, USA

    Stephen R. Hooper

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  1. Erum A. Hartung
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Correspondence to Erum A. Hartung.

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Hartung, E.A., Matheson, M., Lande, M.B. et al. Neurocognition in children with autosomal recessive polycystic kidney disease in the CKiD cohort study. Pediatr Nephrol 29, 1957–1965 (2014). https://doi.org/10.1007/s00467-014-2816-5

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  • DOI: https://doi.org/10.1007/s00467-014-2816-5

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