Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease

This mini review provides an overview of the issues and challenges inherent in autosomal recessive polycystic kidney disease (ARPKD), with a particular focus on the neurological factors and neurocognitive functioning of this population. ARPKD typically is discovered at the end of pregnancy or during...

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Main Author: Stephen R. Hooper
Format: Article
Language:English
Published: Frontiers Media S.A. 2017-05-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:http://journal.frontiersin.org/article/10.3389/fped.2017.00107/full
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author Stephen R. Hooper
author_facet Stephen R. Hooper
author_sort Stephen R. Hooper
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description This mini review provides an overview of the issues and challenges inherent in autosomal recessive polycystic kidney disease (ARPKD), with a particular focus on the neurological factors and neurocognitive functioning of this population. ARPKD typically is discovered at the end of pregnancy or during the neonatal developmental period and occurs in approximately 1 in 20,000 live births. During the neonatal period, there is a relatively high risk of death, with many infants dying from respiratory failure. As the child ages, they experience progressive kidney disease and become increasingly vulnerable to liver disease, with many individuals eventually requiring dual organ transplants. This mini review provides a brief description of ARPKD and describes the various factors that place children with ARPKD at risk for neurological and neuropsychological impairment (e.g., a genetic condition leading to chronic kidney disease and eventual transplant; difficult-to-treat hypertension; eventual liver disease; possible dual transplantation of the kidneys and liver; chronic lung disease), including that these factors are present during a critical period of brain development. Further, the mini review discusses the available studies that have addressed the neurocognitive functioning in children with ARPKD. This paper concludes by providing the key clinical and research challenges that face the field of pediatric nephrology with respect to the clinical and scientific study of the neurocognitive functioning of children with ARPKD. Selected directions are offered in both the clinical and research arenas for this multiorgan chronic condition.
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spelling doaj.art-6bb3c84871584c8d8151356a500f55642022-12-21T23:35:52ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602017-05-01510.3389/fped.2017.00107253653Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney DiseaseStephen R. Hooper0Department of Allied Health Sciences, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USAThis mini review provides an overview of the issues and challenges inherent in autosomal recessive polycystic kidney disease (ARPKD), with a particular focus on the neurological factors and neurocognitive functioning of this population. ARPKD typically is discovered at the end of pregnancy or during the neonatal developmental period and occurs in approximately 1 in 20,000 live births. During the neonatal period, there is a relatively high risk of death, with many infants dying from respiratory failure. As the child ages, they experience progressive kidney disease and become increasingly vulnerable to liver disease, with many individuals eventually requiring dual organ transplants. This mini review provides a brief description of ARPKD and describes the various factors that place children with ARPKD at risk for neurological and neuropsychological impairment (e.g., a genetic condition leading to chronic kidney disease and eventual transplant; difficult-to-treat hypertension; eventual liver disease; possible dual transplantation of the kidneys and liver; chronic lung disease), including that these factors are present during a critical period of brain development. Further, the mini review discusses the available studies that have addressed the neurocognitive functioning in children with ARPKD. This paper concludes by providing the key clinical and research challenges that face the field of pediatric nephrology with respect to the clinical and scientific study of the neurocognitive functioning of children with ARPKD. Selected directions are offered in both the clinical and research arenas for this multiorgan chronic condition.http://journal.frontiersin.org/article/10.3389/fped.2017.00107/fullautosomal recessive polycystic kidney diseasepediatric autosomal recessive polycystic kidney diseaseneurocognition in autosomal recessive polycystic kidney diseaserisk factors for neurocognitive dysfunction in autosomal recessive polycystic kidney diseasepolycystic kidney diseases
spellingShingle Stephen R. Hooper
Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease
Frontiers in Pediatrics
autosomal recessive polycystic kidney disease
pediatric autosomal recessive polycystic kidney disease
neurocognition in autosomal recessive polycystic kidney disease
risk factors for neurocognitive dysfunction in autosomal recessive polycystic kidney disease
polycystic kidney diseases
title Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease
title_full Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease
title_fullStr Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease
title_full_unstemmed Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease
title_short Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive Polycystic Kidney Disease
title_sort risk factors for neurocognitive functioning in children with autosomal recessive polycystic kidney disease
topic autosomal recessive polycystic kidney disease
pediatric autosomal recessive polycystic kidney disease
neurocognition in autosomal recessive polycystic kidney disease
risk factors for neurocognitive dysfunction in autosomal recessive polycystic kidney disease
polycystic kidney diseases
url http://journal.frontiersin.org/article/10.3389/fped.2017.00107/full
work_keys_str_mv AT stephenrhooper riskfactorsforneurocognitivefunctioninginchildrenwithautosomalrecessivepolycystickidneydisease