Neurocognitive Dysfunction in Children, Adolescents, and Young Adults With CKD

Ruebner, Rebecca L.; Laney, Nina; Kim, Ji Young; Hartung, Erum A.; Hooper, Stephen R.; Radcliffe, Jerilynn; Furth, Susan L.

doi:10.1053/j.ajkd.2015.08.025

Cited by 74 publications

(41 citation statements)

References 39 publications

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“…Contrary to the literature describing lower cognitive function in individuals with renal [Ruebner et al, 2016] and liver disease [Stewart et al, 1992], neuropsychological performance was not correlated with presence of kidney dysfunction or portal hypertension in our sample. This lack of correlation may be due to the fact that genes and/or brain abnormalities play a dominant role in cognitive function in individuals with JS, potentially obscuring the relatively minor contribution from kidney and/or liver disease.…”

Section: Discussioncontrasting

confidence: 99%

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Summers

Snow

Wiggs

et al. 2017

American J of Med Genetics Pt A

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Objectives Joubert Syndrome (JS) is a genetically heterogeneous ciliopathy characterized by hypo-dysplasia of the cerebellar vermis, a distinct hindbrain/midbrain malformation (molar tooth sign), and intellectual disability. We evaluated the neuropsychological profiles of 76 participants with JS in the context of molecular genetics and clinical covariates. Methods Evaluations included neuropsychological testing, structured parental interviews, DNA sequencing, brain magnetic resonance imaging (MRI), electroencephalography (EEG), ophthalmologic examination, and assessment for renal and hepatic disease. Results On average, participants manifested Full Scale Intelligence Quotients (FSIQ) in the moderately to profoundly low range (M=64.3±15.3). Of the Wechsler index scores, verbal comprehension was least affected and processing speed was most affected. Receptive language was rated as better than expressive language on the Vineland Adaptive Behavior Scales-Second Edition. Those with abnormal EEG had a significantly lower FSIQ (n=15; M=50.7±12.9) compared to participants with normal EEG (n=39; M=64.7±16.3; p=.004). Participants taking psychiatric medications manifested a lower FSIQ (n=20; M=54.8±13.2) than those not taking them (n=42; M=65.0±17.2; p=.022). These correlations were also present in the TMEM67-related JS sub-cohort (n=14). Based on parental assessment, psychiatric and behavioral problems were significantly more common than in the general population for all measures (p<.004 for all). Conclusions The majority (65%) of individuals with JS have some degree of intellectual disability. Abnormal EEG is associated with lower neuropsychological function. Processing speed is a weakness, while verbal comprehension and receptive language are relative strengths. These findings may guide parents, teachers, therapists, and doctors to determine appropriate therapies, accommodations and academic goals for individuals with JS.

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Section: Discussioncontrasting

confidence: 99%

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Summers

Snow

Wiggs

et al. 2017

American J of Med Genetics Pt A

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“…39 Interestingly, a longitudinal study of patients receiving renal allografts did show that patients with impaired cognitive function prior to transplant improved after transplantation such that post-transplant test scores were not appreciably different from healthy norms. 60 Although we are not including children in this review, we note that the presence of neurocognitive deficits even in children 61, 62 also supports a possible effect of CKD or its sequelae on neurologic function.…”

Section: Cognitive Dysfunctionmentioning

confidence: 97%

Consequences of CKD on Functioning

Kittiskulnam

Sheshadri

Johansen

2016

Seminars in Nephrology

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CKD is highly prevalent in the U.S. and throughout the world,1 with approximately 13% of adults affected.2 In addition, according to recent estimates, almost half of patients with CKD stage 3-5 are 70 years of age or older.2 In the United States, the number of prevalent ESRD cases continues to rise in patients aged over 65. In light of the demographic characteristics of patients with CKD and ESRD, there has been considerable focus on associations between CKD and cardiovascular outcomes.3 Until recently, less attention had been paid to other consequences of CKD in general and among older individuals with CKD in particular, but there is now solid evidence linking CKD with impairments of physical function, cognitive function, and emotional function and quality of life. This review will summarize available literature on these topics, focusing specifically on physical functioning and frailty; cognitive function; emotional health, including depression and anxiety; and health-related quality of life.

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“…The uncertainty about the relevance of trial outcomes to patients, caregivers, and clinicians has important implications for children with CKD, as treatment can be highly intrusive and onerous, with profound impacts on health and lifestyle. Also, shared decision-making can be particularly challenging in this vulnerable population given their younger age and higher risk of neurocognitive dysfunction [ 12 , 13 , 30 ]. These challenges highlight the need for systematic, collaborative and concerted efforts to engage children with CKD and their families in explicitly identifying the outcomes that they regard as important for research, and to understand the reasons for their preferences.…”

Section: Introductionmentioning

confidence: 99%

Standardised Outcomes in Nephrology—Children and Adolescents (SONG-Kids): a protocol for establishing a core outcome set for children with chronic kidney disease

Tong

Samuel

Zappitelli

et al. 2016

Trials

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BackgroundChildren with chronic kidney disease (CKD), requiring dialysis or kidney transplantation, have a mortality rate of up to 30-fold higher than the general aged-matched population, and severely impaired quality of life. Symptoms such as fatigue and pain are prevalent and debilitating. Children with CKD are at risk of cognitive impairment, and poorer educational, vocational, and psychosocial outcomes compared with their well peers, which have consequences through to adulthood. Treatment regimens for children with CKD are long-term, complex, and highly intrusive. While many trials have been conducted to improve outcomes in children with CKD, the outcomes measured and reported are often not relevant to patients and clinicians, and are highly variable. These problems can diminish the value of trials as a means to improve the lives of children with CKD. The Standardised Outcomes in Nephrology—Children and Adolescents (SONG-Kids) study aims to develop a core outcome set for trials in children and adolescents with any stage of CKD that is based on the shared priorities of all stakeholders.Methods/DesignSONG-Kids involves five phases: a systematic review to identify outcomes (both domains and measures) that have been reported in randomised controlled trials involving children aged up to 21 years with CKD; focus groups (using nominal group technique) with adolescent patients and caregivers of paediatric patients (all ages) to identify outcomes that are relevant and important to patients and their family and the reasons for their choices; semistructured key informant interviews with health professionals involved in the care of children with CKD to ascertain their views on establishing core outcomes in paediatric nephrology; an international three-round online Delphi survey with patients, caregivers, clinicians, researchers, policy-makers, and members from industry to develop consensus on important outcome domains; and a stakeholder workshop to review and finalise the set of core outcome domains for trials in children with CKD (including nondialysis-dependent, dialysis, and kidney transplantation).DiscussionEstablishing a core outcome set to be reported in all trials conducted in children with any stage of CKD will enhance the relevance, transparency, and impact of research to improve the lives of children and adolescents with CKD.

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Neurocognitive Dysfunction in Children, Adolescents, and Young Adults With CKD

Cited by 74 publications

References 39 publications

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Neuropsychological phenotypes of 76 individuals with Joubert syndrome evaluated at a single center

Consequences of CKD on Functioning

Standardised Outcomes in Nephrology—Children and Adolescents (SONG-Kids): a protocol for establishing a core outcome set for children with chronic kidney disease

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