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2017 ; 5
(ä): 107
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Risk Factors for Neurocognitive Functioning in Children with Autosomal Recessive
Polycystic Kidney Disease
#MMPMID28555180
Hooper SR
Front Pediatr
2017[]; 5
(ä): 107
PMID28555180
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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.