Limitations of U25 CKiD and CKD-EPI eGFR formulae in patients 2-20 years of age with measured GFR > 60 mL/min/1.73 m2-a cross-sectional study.

BACKGROUND: When applying Pierce U25 formula for estimating glomerular filtration rate (eGFR), we observed a higher proportion of eGFR < 90 mL/min/1.73 m2 (chronic kidney disease (CKD) stage 2). We compared agreement and accuracy of the Pierce U25 (ages 2-25), Pottel (ages 2-100), and CKD-EPI (ages 18-100) formulae to GFR measurements. METHODS: Post hoc analysis of the three eGFRs compared to 367 99m technetium-diethylene-triamine penta-acetic acid (99Tc DTPA) GFR measurements (240 patients) using 3 sampling points and Brockner/Mørtensen correction (body surface area calculation based on ideal weight) on simultaneous serum creatinine and cystatin C measurements. RESULTS: Overall, the U25 formula performed well with a Spearman r of 0.8102 (95% confidence interval 0.7706 to 0.8435, p < 0.0001) while diagnostic accuracy was low in patients with normal mGFR. The U25 formula reclassified 29.5% of patients with normal mGFR as CKD stage 2; whereas the average of the modified Schwartz formula based on serum creatinine and the Filler formula based on cystatin C, only over-diagnosed CKD stage 2 in 8.5%, 24.5% within 10% and 62.7% within 30%. We therefore combined both. The average Schwartz/Filler eGFR had 36.5% of results within 10%, 84.7% within 30%, and normal mGFR accuracy was 26.8%, 63.9% for 10% and 30%, respectively, outperforming the CKD-EPI and Pottel formulae. CONCLUSIONS: The Pierce U25 formula results correlated well with mGFR < 75 mL/min/1.73 m2. Over the entire GFR range, accuracy was better for patients with a higher mGFR, when averaging the combined Schwartz/Filler formulae. More work is needed to prospectively confirm our findings in other centers.

Trends and Disparities in Health Care Transition Preparation from 2016 to 2019: Findings from the US National Survey of Children's Health.

OBJECTIVES: To evaluate national trends in health care transition preparation over a 4-year period using the National Survey of Children's Health (NSCH) and to identify and examine disparities in receipt of health care transition preparation over the study period. STUDY DESIGN: Data from the NSCH, an annual serial cross-sectional survey conducted from 2016 to 2019, were examined. Caregivers answered questions regarding one of their children within each of a random sample of households across the US. The primary analysis examined trends in health care transition preparation based on the year of survey completion. A secondary analysis examined the associations of race/ethnicity, primary household language, insurance type, and children with special health care needs (CSHCN) with receipt of health care transition preparation. RESULTS: We included data from 54 434 youths (20 708 in 2016, 8909 in 2017, 12 587 in 2018, and 12 230 in 2019) aged 12-17 years whose caregivers completed the NSCH between 2016 and 2019. The sample was weighted to be nationally representative based on weights provided by the NSCH. The proportion of youths receiving necessary health care transition preparation increased over the study period, from 14.8% in 2016 to 20.5% in 2019 (P < .001). Multivariable logistic regression demonstrated increased odds of receiving health care transition preparation in 2018 and 2019, as well as for White non-Hispanic youths, those with English or Spanish as a primary household language, those with private insurance, and CSHCN. CONCLUSIONS: Although the proportion of youths receiving health care transition preparation has increased since 2016, the need for ongoing improvement and elimination of disparities in health care transition preparation remains.

A.L.L. Y.O.U. N.E.E.D. I.S. L.O.V.E. Manual on health self-management and patient-reported outcomes among low-income young adult Mexicans on chronic dialysis: Feasibility study.

PURPOSE: We evaluated disease knowledge/self-management skills among low-income Mexican young adults maintained on dialysis and to test the effectiveness of the A.L.L. Y.O.U. N.E.E.D. I.S. L.O.V.E (AYNIL) Manual - Spanish Version on patient-reported outcomes. This is a low literacy teaching tool designed with patients and educators' input. DESIGN AND METHODS: A quasi-experimental study was conducted in 17 chronic dialysis patients at Mexico City's Hospital General de México, Dr. Eduardo Liceaga. Ages 18-30-year-old completed disease knowledge/self-management and quality of life measures before the intervention and 6 weeks later. RESULTS: Significant increases were observed on disease knowledge/self-management scores in the STARx questionnaire from 47 (IQ: 40,51) to 50 (IQ: 48,54) p = 0.04. The UNC-TRxANSITION Index increased significantly from 4.8 (IQ: 3.9,5.7) to 7.7 (IQ: 7.5,8.2) p ≤0.001. Significant increases in scores were detected in the "Burden of kidney disease" (p = 0.008), "Effects of kidney disease" " (p = 0.03) and " Dialysis staff encouragement" (p = 0.027) based on the KDQoL survey. CONCLUSIONS: In this vulnerable population, the Spanish version of the A.L.L. Y.O.U. N.E.E.D. I.S. L.O.V.E. - AYNIL Manual improved CKD/ESRD disease knowledge/self-management skills and HRQoL. This study highlighted the need for low-literacy educational tools to improve patient-reported outcomes. PRACTICE IMPLICATIONS: Young adults with CKD/ESRD can benefit from patient-centered educational interventions to enhance their autonomy and the development of self-management behaviors that improve patient-reported outcomes and potential complications of the disease. Special attention is needed in low-income patients with low rates of adherence to treatments and poor self-management skills.

Marginal parent donors-Process and ethics.

BACKGROUND: Pre-emptive kidney transplantation for end-stage kidney disease in children has many advantages and may lead to the consideration of marginal parent donors. METHODS: Using the example of the transplant of a kidney with medullary sponge disease from a parent to the child, we review the ethical framework for working up such donors. RESULTS: The four principles of health ethics include autonomy (the right of the patient to retain control over his/her own body); beneficence (healthcare providers must do all they can do to benefit the patient in each situation); non-maleficence ("first do no harm"-providers must consider whether other people or society could be harmed by a decision made, even if it is made for the benefit of an individual patient) and justice (there should be an element of fairness in all medical decisions). Highly motivated donors may derive significant psychological benefit from their donation and may thus be willing to incur more risk. The transplantation team and, ideally, an independent donor advocate team must make a judgment about the acceptability of the risk-benefit ratio for particular potential donors, who must also make their own assessment. The transplantation team and donor advocate team must be comfortable with the risk-benefit ratio before proceeding. CONCLUSIONS: An independent donor advocacy team that focuses on the donor needs is needed with sufficient multidisciplinary ethical, social, and psychological expertise. The decision to accept or reject the donor should be within the authority of the independent donor advocacy team and not the providers or the donor.

Management of severe polyuria in idiopathic Fanconi syndrome.

BACKGROUND: Polyuria is a common problem in patients with tubular diseases, especially for those with CKD and high-output Fanconi syndrome. There are currently no guidelines on how to treat debilitating polyuria, in children or adults, and vasopressin is usually not effective. CASE-DIAGNOSIS/TREATMENT: A 13-year-old female with idiopathic Fanconi syndrome and an eGFR of 69 mL/min/1.73 m2 was severely affected by polyuria of 5 L per day (voiding at least 11 times during the day and up to 8 times at night), impacting her mood (measured by the RCADS-child) and academic performance at school. In the absence of guidelines and with literature discouraging the use of indomethacin in this condition, we attempted indomethacin treatment at a dose of 2 mg/kg divided in two doses with substantial success. Urine output dropped to 2.5L and this was accompanied by a substantial decrease of her sodium wasting from 24.6 to 7.7 mmol/kg/day. Over the course of 18 months, the patient's eGFR dropped temporarily to 60 mL/min/1.73 m2 and was 68 mL/min/1.73 m2 at last follow-up. However, a sodium-23 (23Na) MRI of her thigh revealed ongoing moderate sodium decrease in her skin and substantial Na+ decrease in her muscle when compared to age-matched peers with normal kidney function. CONCLUSIONS: Indomethacin may be a safe and effective treatment option for polyuria in idiopathic Fanconi syndrome.

Health-related quality of life in children with chronic kidney disease is affected by the number of medications.

INTRODUCTION: The number of medications could serve as a surrogate for burden of care at home and may affect health-related quality of life (HRQoL) in children with chronic kidney disease (CKD). METHODS: Using baseline data from the Chronic Kidney Disease in Children (CKiD) Study, we modeled HRQoL scores, self-reported by the child (if ≥ 8 years old) and/or caregiver (all children) on unique counts and administrations of CKD- and non-CKD-related medications, using multivariate linear regression. Heterogeneity of associations between HRQoL and medication burden by age group (≥ 8 vs. < 8 years old) were explored. RESULTS: 734 participants median age 11 years, disease duration 8 years, median eGFR 53 mL/min/1.73 m2, 61% male, 22% African-American, 31% glomerular disease were prescribed median 3 unique CKD-related medications. Regarding HRQoL assessment, 201 children were < 8 years old and had only parent-proxy HRQoL score; 533 children ≥ 8 years of age had both child and parent-proxy scores. Overall, parents of children < 8 years old reported higher HRQoL scores than parents of older children: 84 vs. 76. However, in a unified multivariate regression model, HRQoL scores of children < 8 years showed greater decreases as the number of CKD-related medications increased compared to scores for children ≥ 8 years old. CONCLUSION: Average HRQoL scores reported by parents of younger CKD children were higher than those of older CKD children but decreased more with increased CKD medication counts than scores of older children. Considerations of HRQoL may be of particular importance for clinicians and caregivers when managing chronic disease comorbidities in younger children.

How should we assess renal function in neonates and infants?

AIM: Review of current knowledge on assessing renal function in term and preterm neonates. METHODS: Literature review and analysis of own data. RESULTS: Prematurity, genetic, environmental and maternal factors may alter peak nephron endowment and life-long renal function. Nephrogenesis continues until 34-36 weeks of gestation, but it is altered with premature delivery. Variability of nephron endowment has a substantial impact on the clearance of renally excreted drugs. Postnatally, glomerular function rate (GFR) increases daily, doubles by two weeks, and slowly reaches full maturity at 18 months of age. Ideally, renal function biomarkers should be expressed as age-independent z-scores, and evidence suggests indexing these values to post-conceptual age rather than chronological age. Newborn and maternal serum creatinine correlate tightly for more than 72 hours after delivery, rendering this biomarker unsuitable for the assessment of neonatal renal function. Cystatin C does not cross the placenta and may be the preferred biomarker in the neonate. Here, we provide preliminary data on the natural evolution of the cystatin C eGFR in infancy. CONCLUSION: Cystatin C may be superior for GFR estimation in neonates, but the best approach to drug dosing of renally excreted drugs remains to be established.

School Nurses Practices Promoting Self-Management and Healthcare Transition Skills for Adolescents with Chronic Conditions in Urban Public Schools: A Mixed Methods Study.

Background: Adolescents and young adults (AYA) with chronic conditions should acquire self-management skills as part of their healthcare transition (HCT) from pediatric to adult-focused care. HCT/self-management skills have the potential to help mitigate health disparities among minority AYA with chronic conditions. This study investigated school nurses' practices promoting HCT/self-management skills in urban public schools. Methods: Seventy-nine nurses from three urban school districts in Massachusetts completed a survey of 32 Likert-type questions on HCT/self-management skills, eight demographic questions, and five open-ended practice questions assessing how often they have asked students with chronic conditions about HCT/self-management skills, based on the UNC TRxANSITION IndexTM. Results: Among the 79 school nurses who participated (response rate 76%), 67% never or rarely assessed students' knowledge of HCT/self-management, and 90% would use a tool that promotes/measures HCT/self-management skills. Conclusion: In our study sample, most school nurses acknowledged the importance of assessing HCT/self-management skills. The majority favored using a tool to promote these skills.

Longitudinal Self-Management and/or Transition Readiness per the TRxANSITION Index among Patients with Chronic Conditions in Pediatric or Adult Care Settings.

OBJECTIVE: To evaluate the roles of key individual, family, and illness characteristics on the levels of and gains in longitudinal healthcare transition (HCT) readiness in the pediatric setting and/or self-management skills (SMS) in the adult-focused setting, we used a large dataset with longitudinal measurements from 2006 to 2015. STUDY DESIGN: This longitudinal observational study followed 566 adolescents and young adults with chronic conditions at University of North Carolina Hospitals. TRxANSITION Index measurements, which represent learning outcomes rather than health outcomes, were collected multiple times per patient and analyzed using a novel application of an education-based approach. RESULTS: Levels of and gains in HCT/SMS scores increased with age (P < .001) with smaller increases at older ages. Mastery of skills varied by age with self-management achieved after 20 years of age. Scores varied positively by father's education and negatively by mother's education and duration of diagnosis. Gains in scores further varied positively with private insurance and negatively with mother's education and duration of diagnosis. CONCLUSIONS: We found diminishing positive increases in HCT/SMS scores as patients become older and smaller levels of and gains in readiness among younger patients with more educated mothers. Risk factors for absolute level of HCT/SMS readiness and inadequate longitudinal gains are not always the same, which motivates a deeper understanding of this dynamic process through additional research. This information can guide providers to focus HCT/SMS preparation efforts on skills mastered at particular ages and to identify patients at risk for inadequate development of HCT/SMS skills.

A Cross-Sectional Study of Growth and Metabolic Bone Disease in a Pediatric Global Cohort Undergoing Chronic Hemodialysis.

OBJECTIVE: We sought to assess worldwide differences among pediatric patients undergoing hemodialysis. Because practices differ widely regarding nutritional resources, treatment practice, and access to renal replacement therapy, investigators from the Pediatric Investigation and Close Collaboration to examine Ongoing Life Outcomes, the pediatric subset of the MONitoring Dialysis Outcomes Cohort (PICCOLO MONDO) performed this cross-sectional study. We hypothesized that growth would be better in developed countries, possibly at the expense of bone mineral disease. STUDY DESIGN: In this cross-sectional study, we analyzed growth by height z score and recommended age-specific bone mineral metabolism markers from 225 patients <18 years of age maintained on hemodialysis, between the years of 2000 to 2012 from 21 countries in different regions. RESULTS: The patients' median age was 16 (IQR 14-17) years, and 45% were females. A height z score less than the third percentile was noted in 34% of the cohort, whereas >66% of patients reported normal heights, with patients from North America having the greatest proportion (>80%). More than 70% of the entire cohort had greater than the age-recommended levels of phosphorus, particularly in the Asia-Pacific and North America, where we also observed the greatest body mass index z score (0.99 ± 1.6) and parathyroid hormone levels (557.1 [268.4-740.5]). Below-recommended parathyroid hormone levels were noted in 26% and elevated levels in 61% of the entire sample, particularly in the Asia Pacific region. Lower-than-recommended calcium levels were noted in 36% of the entire cohort, particularly in Latin America. CONCLUSIONS: We found regional differences in growth- and age-adjusted bone mineral metabolism markers. Children from North America had the best growth, received the most dialysis, but also had the worst phosphate control and body mass index z scores.

Low agreement between modified-Schwartz and CKD-EPI eGFR in young adults: a retrospective longitudinal cohort study.

BACKGROUND: While there is a great deal of research updating methods for estimating renal function, many of these methods are being developed in either adults with CKD or younger children. Currently, there is limited understanding of the agreement between the modified new bedside Schwartz estimated glomerular filtration rate (eGFR) formula and the adult CKD-EPI formula in adolescents and young adults (AYAs) with chronic kidney disease (CKD) measured longitudinally. METHODS: Longitudinal cohort study of 242 patients (10-30 years) with CKD, followed retrospectively in a single tertiary centre as they transitioned from the paediatric- to adult-focused settings. The study population came from a longitudinal cohort of AYAs undergoing healthcare transition at the STARx Program at the University of North Carolina, in the South-Eastern USA, from 2006 to 2015. We calculated and compared the eGFR using the new bedside Schwartz formula and the CKD-EPI eGFR. Measurements were repeated for each age in years. Agreement was tested using Bland & Altman analysis. Subgroup analysis was performed using the following age groups 10-15, 15-20, 20-25 and 25-30 years, glomerular and non-glomerular causes of CKD and height z-score. RESULTS: Using repeated measures, concordance between the new Schwartz and CKD-EPI eGFR was low at 0.74 (95% C.I. 0.67, 0.79) at the lowest age range of 10-15, 0.78 (95% C.I. 0.71, 0.84) at age 15-20, 0.80 (0.70, 0.87) at ages 20-25, and 0.82 (95% C.I. 0.70, 0.90) at age 25-30. Discordance was worse in males and largest in the 10-15 year-old age group, and in patients with stunted growth. CONCLUSIONS: The Schwartz and CKD-EPI equations exhibit poor agreement in patients before and during the transition period with CKD-EPI consistently yielding higher eGFRs, especially in males. Further studies are required to determine the appropriate age for switching to the CKD-EPI equation after age 18.

Lack of Knowledge and Low Readiness for Health Care Transition in Eosinophilic Esophagitis and Eosinophilic Gastroenteritis.

OBJECTIVES: A growing population of adolescents/young adults with eosinophilic esophagitis (EoE) and eosinophilic gastroenteritis (EGE) will need to transition from pediatric to adult health providers. Measuring health care transition (HCT) readiness is critical, but no studies have evaluated this process in EoE/EGE. We determined the scope and predictors of HCT knowledge in patients and parents with EoE/EGE and measured HCT readiness in adolescents/young adults. METHODS: We conducted an online survey of patients 13 years or older and parents of patients with EoE/EGE who were diagnosed when 25 years or younger. Parents answered questions regarding their children and their own knowledge of HCT. HCT readiness was assessed in adolescents/young adults aged 13 to 25 years with the Self-Management and Transition to Adulthood with Rx Questionnaire (a 6-domain self-report tool) with a score range of 0 to 90. RESULTS: Four hundred fifty participants completed the survey: 205 patients and 245 parents. Included in the analysis (those diagnosed with EoE/EGE at age 25 years or younger) were 75 of 205 patients and children of 245 parent respondents. Overall, 78% (n = 52) of the patients and 76% (n = 187) of parents had no HCT knowledge. Mean HCT readiness score in adolescents/young adults (n = 50) was 30.4 ±â€Š11.3 with higher scores in domains of provider communication and engagement during appointments. Mean parent-reported (n = 123) score was 35.6 ±â€Š9.7 with higher scores in medication management and disease knowledge. CONCLUSIONS: There was a significant deficit in HCT knowledge, and HCT readiness scores were lower than other chronic health conditions. HCT preparation and readiness assessments should become a priority for adolescents/young adults with EoE/EGE and their parents.

Cognitive remediation in pediatric chronic kidney disease and end-stage kidney disease: rationale, candidate interventions, and applicability.

The purpose of this paper is to address the potential use of cognitive remediation interventions for children and adolescents with chronic kidney disease (CKD) and end-stage kidney disease (ESKD). The prevalence and risk for neurocognitive dysfunction in children with this condition remains high, but, to date, interventions targeting these challenges have not been attempted either individually or as part of a larger treatment program. This is the next logical step in addressing the neurocognitive dysfunction that can be present in pediatric CKD/ESKD, with the field needing to determine the efficacy of cognitive remediation approaches for this population. To our knowledge, this paper is the first to raise this possibility by identifying candidate treatments addressing the neurocognitive challenges observed in children and adolescents with CKD/ESKD. Initially, we present the rationale for the importance of addressing the cognitive difficulties in this population, including an overview of the literature documenting the neurocognitive deficits associated with pediatric-onset CKD/ESKD. This is followed by a review of five candidate cognitive remediation programs that may be applicable to patients with this condition, and associated factors that could affect such treatment. The paper concludes with suggestions for both clinical and research initiatives that could be implemented to examine cognitive remediation as potential components of a larger treatment program for children and adolescents with CKD/ESKD.

Self-management and Transition to Adult Health Care in Adolescents and Young Adults: A Team Process.

As health care continues to evolve, the need for more effective health care transition (HCT) for all youth, but particularly children with chronic conditions and special health care needs, becomes even more important. With more than 90% of adolescents with chronic medical conditions now surviving into adulthood, suboptimal transition can lead to poorer quality of life and less successful adulthood.Through a series of clinical vignettes, the challenges of HCT are presented herein and accompanied by comments that underscore how these adolescents can best be helped to transition to successful adulthood. Several methods are presented to assess the readiness of adolescents and young adults (AYA) for transition. The process of transition can be divided into 3 stages: 1) setting the stage: initiation of HCT services and transition readiness assessment, 2) moving forward: ongoing provision of HCT services, and 3) reaching the goal: transfer of care and transition to adulthood.Several valuable suggestions for incorporating the HCT process into the health care system and improving HCT programs through a quality improvement (QI) approach are outlined. Future challenges in HCT include developing more precise assessments of transition status or transition readiness, better understanding the status and specific needs of AYA with chronic health care needs, continued program evaluation and QI efforts, and more reliance on patients and families to teach us about the challenges and methods in HCT that most effectively work for them.

Vitamin D in incident nephrotic syndrome: a Midwest Pediatric Nephrology Consortium study.

BACKGROUND: Cross-sectional studies of children with prevalent nephrotic syndrome (NS) have shown 25-vitamin D (25(OH)D) deficiency rates of 20-100 %. Information on 25(OH)D status in incident patients or following remission is limited. This study aimed to assess 25(OH)D status of incident idiopathic NS children at presentation and longitudinally with short-term observation. METHODS: Multicenter longitudinal study of children (2-18 years old) from 14 centers across the Midwest Pediatric Nephrology Consortium with incident idiopathic NS. 25(OH)D levels were assessed at diagnosis and 3 months later. RESULTS: Sixty-one children, median age 5 (3, 11) years, completed baseline visit and 51 completed second visit labs. All 61 (100 %) had 25(OH)D < 20 ng/ml at diagnosis. Twenty-seven (53 %) had 25(OH)D < 20 ng/ml at follow-up. Fourteen (28 %) children were steroid resistant. Univariate analysis showed that children prescribed vitamin D supplements were less likely to have 25(OH)D deficiency at follow-up (OR 0.2, 95 % CI 0.04, 0.6). Steroid response, age, and season did not predict 25(OH)D deficiency. Multivariable linear regression modeling showed higher 25(OH)D levels at follow-up by 13.2 ng/ml (SE 4.6, p < 0.01) in children supplemented with vitamin D. CONCLUSIONS: In this incident idiopathic NS cohort, all children at diagnosis had 25(OH)D deficiency and the majority continued to have a deficiency at 2-4 months. Supplemental vitamin D decreased the odds of 25(OH)D deficiency at follow-up, supporting a role for supplementation in incident NS.

Adolescents and Young Adults with Chronic or End-Stage Kidney Disease.

BACKGROUND: Adolescents and young adults face unique and complex physical, psychological, and family challenges. Despite improvements in care for chronic kidney disease (CKD) and end-stage kidney disease (ESKD), long-term mortality for children, adolescents, and young adults with CKD remains substantially higher than their healthy counterparts. SUMMARY: In this article, we discuss the complex challenges that adolescent and young adult CKD/ESKD patients face. Adolescents have different CKD etiologies and progress along a course dissimilar to the adult population, but have similar multifarious comorbidities. In the setting of puberty and learning to become self-sufficient, adolescence is a critical time for growth and psychosocial development. Physiological complications of CKD underlie many of the long-term outcomes. CKD-mineral and bone disorder and anemia are particularly challenging given that they are exacerbated by the rapid growth of adolescents. Endocrine imbalances and malnutrition can delay and limit growth. All of these factors, together with family dynamics and socioeconomic status, contribute to the poor long-term outcomes and decreased quality of life (QoL) for these patients and their families. KEY MESSAGES: Care for the adolescent CKD/ESKD population is uniquely challenging, but research has identified ways in which we can continue to improve long-term outcomes and QoL for adolescents with CKD/ESKD.