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Pediatric Nephrology
Published in: Pediatric Nephrology 10/2014

01-10-2014 | Original Article

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

Authors: Erum A. Hartung, Matthew Matheson, Marc B. Lande, Katherine M. Dell, Lisa M. Guay-Woodford, Arlene C. Gerson, Bradley A. Warady, Stephen R. Hooper, Susan L. Furth

Published in: Pediatric Nephrology | Issue 10/2014

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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.
Appendix
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Metadata
Title
Neurocognition in children with autosomal recessive polycystic kidney disease in the CKiD cohort study
Authors
Erum A. Hartung
Matthew Matheson
Marc B. Lande
Katherine M. Dell
Lisa M. Guay-Woodford
Arlene C. Gerson
Bradley A. Warady
Stephen R. Hooper
Susan L. Furth
Publication date
01-10-2014
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 10/2014
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-014-2816-5

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