Cognitive improvement in children with CKD after transplant

Icard P, Hooper SR, Gipson DS, Ferris ME. Cognitive improvement in children with CKD after transplant. Pediatr Transplantation 2010: 14:887–890. © 2010 John Wiley & Sons A/S.The primary purpose of this paper was to examine the cognitive functioning of children with CKD receiving transplantation to children with CKD not receiving transplantation, and a healthy control group. The sample included six children with CKD receiving transplant, 28 children with CKD being treated conservatively, and 23 healthy controls. All participants were administered intellectual (IQ) or developmental assessments at baseline and at a one-yr follow-up. Results revealed that children with CKD who had received transplant showed a significant increase in their intellectual/developmental functioning post transplant compared to children with CKD not receiving transplant. Although their overall intellectual/developmental level was not fully normalized, when compared with the healthy control group, the change scores for the transplant group reflected over a 12 point increase, moving the group from the borderline range to the low average range of functioning. In this regard, pediatric transplantation appears to have a positive impact on the intellectual and developmental functioning of children with CKD.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/79239/1/j.1399-3046.2010.01359.x.pd

The nervous system and chronic kidney disease in children

This paper provides a review of the literature on the nervous system involvement incurred by children and adolescents with chronic kidney disease (CKD), with a particular focus on neuropsychological functioning. In addition to an historical overview of earlier literature, published studies from the past 14 years that address both central and peripheral nervous system function in children with CKD are reviewed (1990–2003). These studies span work in neuroimaging, electrophysiology, and neuropsychology. A key focus for this review is on variables that might affect neurodevelopmental status in these children. The paper concludes with suggestions for achieving progress in the understanding of this complication of kidney disease in children

Differential Attention Functioning in Pediatric Chronic Kidney Disease

Objective To compare specific attention functions for school-age children with chronic kidney disease (CKD) to those of a typically developing control group. Methods A cross-sectional study examined attention dimensions for children and adolescents with CKD (n = 30) in comparison to a typically developing control group (n = 41). The CKD group consisted of those receiving maintenance dialysis (n = 15) and those with mild/moderate CKD treated conservatively (n = 15). Measures aligning with Mirsky’s conceptual multidimensional model of attention were selected to compare groups across five dimensions of attention: Focus/Execute, Sustain, Stability, Shift, and Encode. Results Significant group differences were revealed, with the CKD group performing worse than controls on the Focus/Execute, Sustain, and Encode dimensions. The CKD group also had a larger proportion of children with scores one standard deviation or more below the mean on the Shift and Encode domains, suggesting an at-risk level of functioning in these dimensions. Secondary analyses showed disease severity to be correlated with worse attention functions for children with CKD. Conclusion Children with CKD may be vulnerable to subtle, specific deficits in numerous attention dimensions relative to their typically developing peers, particularly for those with more severe disease

Cognitive improvement in children with CKD after transplant: Cognitive improvement in children with CKD

Icard P, Hooper SR, Gipson DS, Ferris ME. Cognitive improvement in children with CKD after transplant. Pediatr Transplantation 2010: 14:887–890. © 2010 John Wiley & Sons A/S. The primary purpose of this paper was to examine the cognitive functioning of children with CKD receiving transplantation to children with CKD not receiving transplantation, and a healthy control group. The sample included six children with CKD receiving transplant, 28 children with CKD being treated conservatively, and 23 healthy controls. All participants were administered intellectual (IQ) or developmental assessments at baseline and at a one-yr follow-up. Results revealed that children with CKD who had received transplant showed a significant increase in their intellectual/developmental functioning post transplant compared to children with CKD not receiving transplant. Although their overall intellectual/developmental level was not fully normalized, when compared with the healthy control group, the change scores for the transplant group reflected over a 12 point increase, moving the group from the borderline range to the low average range of functioning. In this regard, pediatric transplantation appears to have a positive impact on the intellectual and developmental functioning of children with CKD

Association between clinical risk factors and progression of chronic kidney disease in children

Background and objectives: Children with chronic kidney disease (CKD) have an increased risk of progression to ESRD. There is a need to identify treatments to slow the progression of CKD, yet there are limited data regarding clinical risk factors that may be suitable targets to slow progression. Design, setting, participants, & measurements: We performed a retrospective cohort study using the North American Pediatric Renal Trials and Cooperative Studies CKD database. There were 4166 pediatric subjects with CKD stages II to IV. Disease progression was defined as a GFR on follow-up of \u3c15 ml/min per 1.73 m 2 or termination in the registry because of dialysis or transplantation. We used Kaplan-Meier and Cox proportional hazards methods to describe progression rates and determine factors associated with CKD progression. Results: In the univariate analysis, CKD progression was associated with age, gender, race, primary disease, CKD stage, registration year, hematocrit, albumin, corrected calcium, corrected phosphorus, and use of certain medications. Factors that remained significant in the multivariate analysis were age, primary disease, CKD stage, registration year, hypertension, corrected phosphorus, corrected calcium, albumin, hematocrit, and medication proxies for anemia and short stature. Conclusions: There are multiple risk factors associated with disease progression in the pediatric CKD population. Factors that may be amenable to intervention include anemia, hypoalbuminemia, hyperphosphatemia, hypocalcemia, hypertension, and short stature. Because of the retrospective nature of our study, confirmation of our results from ongoing prospective studies is warranted before recommending prospective interventional trials. Copyright © 2010 by the American Society of Nephrology

The Effect of a Gluten-Free Diet in Children With Difficult-to-Manage Nephrotic Syndrome

Case reports have linked childhood nephrotic syndrome to food sensitivity, including gluten. We report our experience with 8 children (6 boys, 2 girls; age at implementation of special diet 2–14 years) with difficult-to-manage nephrotic syndrome who were placed on a gluten-free diet for 3.4 ± 4.3 years (range, 0.6–14 years) and who had clinical improvement enabling reduction or discontinuation in steroid dosage

Learning to live with nephrotic syndrome: experiences of adult patients and parents of children with nephrotic syndrome

People living with nephrotic syndrome (NS) need to develop an in-depth understanding of their condition in order to participate in treatment decisions, develop self-management skills and integrate illness into daily life. However, the learning needs of adult patients and parents of children with NS are unknown. We therefore explored patient and parent perspectives on learning needs related to NS as part of a larger study to develop a shared learning tool for NS

Provider perspectives on treatment decision-making in nephrotic syndrome

Managing patients with nephrotic syndrome (NS) remains difficult for the practicing nephrologist. This often young patient population is faced with a debilitating, relapsing and remitting disease with non-specific treatment options that are often poorly tolerated. Clinicians managing these complex patients must attempt to apply disease-specific evidence while considering the individual patient's clinical and personal situation

Neurocognitive, Social-Behavioral, and Adaptive Functioning in Preschool Children with Mild to Moderate Kidney Disease

The negative impact of End Stage Kidney Disease on cognitive function in children is well established, but no studies have examined the neurocognitive, social-behavioral, and adaptive behavior skills of preschool children with mild to moderate chronic kidney disease (CKD)

A phase 1, single-dose study of fresolimumab, an anti-TGF-β antibody, in treatment-resistant primary focal segmental glomerulosclerosis.

Primary focal segmental glomerulosclerosis (FSGS) is a disease with poor prognosis and high unmet therapeutic need. Here, we evaluated the safety and pharmacokinetics of single-dose infusions of fresolimumab, a human monoclonal antibody that inactivates all forms of transforming growth factor-β (TGF-β), in a phase I open-label, dose-ranging study. Patients with biopsy-confirmed, treatment-resistant, primary FSGS with a minimum estimated glomerular filtration rate (eGFR) of 25  ml/min per 1.73  m(2), and a urine protein to creatinine ratio over 1.8  mg/mg were eligible. All 16 patients completed the study in which each received one of four single-dose levels of fresolimumab (up to 4  mg/kg) and was followed for 112 days. Fresolimumab was well tolerated with pustular rash the only adverse event in two patients. One patient was diagnosed with a histologically confirmed primitive neuroectodermal tumor 2 years after fresolimumab treatment. Consistent with treatment-resistant FSGS, there was a slight decline in eGFR (median decline baseline to final of 5.85 ml/min per 1.73  m(2)). Proteinuria fluctuated during the study with the median decline from baseline to final in urine protein to creatinine ratio of 1.2  mg/mg with all three Black patients having a mean decline of 3.6  mg/mg. The half-life of fresolimumab was ∼14 days, and the mean dose-normalized Cmax and area under the curve were independent of dose. Thus, single-dose fresolimumab was well tolerated in patients with primary resistant FSGS. Additional evaluation in a larger dose-ranging study is necessary