Child Health Needs and the Pediatric Nephrology Subspecialty Workforce: 2020-2040.

Pediatric nephrology is dedicated to caring for children with kidney disease, a unique blend of acute care and chronic longitudinal patient relationships. Though historically a small field, trainee interest has declined over the past 2 decades. This has led to growing alarm about the health of the pediatric nephrology workforce, although concerns have been hampered by a lack of available data to enable feasible projections. This article is part of a supplement that anticipates the future pediatric subspecialty workforce supply. It draws on existing literature, data from the American Board of Pediatrics, and findings from a model that estimates the future supply of pediatric subspecialists developed by the Carolina Health Workforce Research Center at the University of North Carolina Chapel Hill's Cecil G. Sheps Center for Health Services Research and Strategic Modeling Analytics & Planning Ltd. The workforce projections from 2020 to 2040 incorporate population growth, clinical effort, and geographic trends and model alternate scenarios adjusting for changes in trainee interest, clinical efforts, and workforce attrition. The baseline model predicts growth of clinical work equivalents by 26% by 2040, but further widening geographic disparities worsen the existing mismatch between supply, clinical need, and market demand. The worst-case scenario projects 13% growth by 2040 which, at best, maintains the status quo of an already strained workforce. The models do not account for many factors expected to heighten demand over the coming decades. Urgent reforms are necessary now. Proposed solutions require multipronged changes in education and training pathways, remuneration, clinical practice models, and government policy.

The impact of rural status on pediatric chronic kidney disease.

Children and adolescents in rural areas with chronic kidney disease (CKD) face unique challenges related to accessing pediatric nephrology care. Challenges to obtaining care begin with living increased distances from pediatric health care centers. Recent trends of increasing centralization of pediatric care mean fewer locations have pediatric nephrology, inpatient, and intensive care services. In addition, access to care for rural populations expands beyond distance and encompasses domains of approachability, acceptability, availability and accommodation, affordability, and appropriateness. Furthermore, the current literature identifies additional barriers to care for rural patients that include limited resources, including finances, education, and community/neighborhood social resources. Rural pediatric kidney failure patients have barriers to kidney replacement therapy options that may be even more limited for rural pediatric kidney failure patients when compared to rural adults with kidney failure. This educational review identifies possible strategies to improve health systems for rural CKD patients and their families: (1) increasing rural patient and hospital/clinic representation and focus in research, (2) understanding and mediating gaps in the geographic distribution of the pediatric nephrology workforce, (3) introducing regionalization models for delivering pediatric nephrology care to geographic areas, and (4) employing telehealth to expand the geographic reach of services and reduce family time and travel burden.

Interpretation of ambiguous TP53 test results: Mosaicism, clonal hematopoiesis, and variants of uncertain significance.

The increased use of next-generation sequencing has led to the detection of pathogenic TP53 variants in the germline setting in patients without a personal or family history consistent with Li-Fraumeni syndrome (LFS). These variants can represent low-penetrance LFS, mosaic LFS, or clonal hematopoiesis of indeterminate potential. Additionally, TP53 variants of uncertain significance can be detected in patients with a history suspicious for LFS. The interpretation of the significance of these variants can be challenging but is crucial for an accurate diagnosis and appropriate medical management. This retrospective case review provides illustrative examples of the interpretation of challenging TP53 results through multidisciplinary expertise and use of a flowchart. The authors describe eight patients with TP53 variants associated with ambiguous diagnoses and, for each case, describe how the results were interpreted and the medical care that was implemented. This report presents illustrative cases to help guide clinicians to reach definitive diagnoses for patients when confronted with TP53 variants that are inconsistent with the clinical picture and to add to the body of literature regarding interpretation and medical management of TP53 variants discovered on germline testing.

Trends in the representation of women in the American Society of Pediatric Nephrology program at the Pediatric Academic Society annual meetings 2012-2021.

Introduction: Women are under-represented in virtually all fields of academic medicine. Even in pediatrics, a field that historically attracts a workforce with a majority of women physicians, substantial gender disparities persist in leadership positions. However, previous studies of gender representation in various academic settings are limited to small studies or aggregate pediatric subspecialties, thereby omitting important granularity within each subspecialty. No prior studies have investigated potential gender disparities in pediatric nephrology. The aim of this study is to determine the representation of women physicians in leadership and speaking roles in the annual American Society of Pediatric Nephrology (ASPN) meeting. Methods: Data were analyzed from the 2012-2022 ASPN annual scientific meetings at the Pediatric Academic Society (PAS). Data were abstracted regarding gender and roles: speaker, chair/moderator, and lifetime achievement awardee. We performed a time series analysis using linear regression, with the year as the independent variable and the proportion of women as the dependent variable. Results: Overall, there were statistically significant increases in the proportion of women speakers per year and percentage of women chairs or moderators. There were no specific trends noted for lifetime achievement awards and no statistically significant changes in the number of lifetime achievement awards. Discussion: We found proportionate representations of gender representation with regards to speakers and chairs or moderators, although our data was limited by comparison to the American Board of Pediatrics (ABP) workforce cumulative "ever certified" data. The ABP data include a disproportionate representation of faculty who are men from earlier certification periods who may no longer be actively practicing pediatric nephrology.

Mothers' and fathers' engagement in math activities with their toddler sons and daughters: The moderating role of parental math beliefs.

Parents' beliefs about the importance of math predicts their math engagement with their children. However, most work focuses on mothers' math engagement with preschool- and school-aged children, leaving gaps in knowledge about fathers and the experiences of toddlers. We examined differences in mothers' and fathers' (N = 94) engagement in math- and non-math activities with their two-year-old girls and boys. Parents reported their beliefs about the importance of math and literacy for young children and their frequency of home learning activities. Parents of sons did not differ in their engagement in math activities from parents of daughters. Mothers reported engaging more frequently in math activities with their toddlers than fathers did, but the difference reduced when parents endorsed stronger beliefs about the importance of math for children. Even at very early ages, children experience vastly different opportunities to learn math in the home, with math-related experiences being shaped by both parent gender and parents' beliefs.

Six case reports of NTHL1-associated tumor syndrome further support it as a multi-tumor predisposition syndrome.

NTHL1-associated tumor syndrome (NATS) is an autosomal recessive condition characterized by an increased risk for colorectal polyposis and colorectal cancer (CRC). Only 46 case reports have been previously published. In a retrospective review, we analyzed the clinical histories of six patients found to have NATS after genetic counseling and testing. NATS appears to be associated with an increased risk for colorectal polyposis, CRC, female breast cancer, meningiomas, and endometrial cancer. Although research is limited, prior publications have reported a multi-tumor predisposition for individuals with biallelic pathogenic or likely pathogenic variants in NTHL1. Additional data are necessary to further define the cancer risks so affected individuals can be appropriately managed.

Influences of Adult Gender and Parenthood on Adult-Child Interaction Style.

Previous studies report differences between mothers and fathers during parent-child interactions. However, the origins of these differences remain unknown. We address this gap by examining the impact of adult gender and gender perceptions on adult-child interactions. Unlike previous studies, we observed both parent and non-parent adults during one-on-one interactions with a child. Further, for non-parent adults the child's identity was held constant while the child's assumed gender was actively manipulated using clothing cues. Results reveal systematic differences between parents and non-parents, but also between male and female adults in language quantity, quality, and engagement strategies during adult-child interactions. Adults' perceptions of gender roles partially explain these findings. In contrast, the child's gender did not impact adult-child interactions. Together, our results support the notion that male and female adults offer unique contributions to a child's development.

The Early Motor Questionnaire revisited: Starting points, standardized scores, and stability.

Motor skills are an important aspect of development during infancy and have been found to predict development in other domains. Therefore, fast and reliable assessments of infant motor skills are needed. The current study revisited a time and cost-effective parent-report measure of infants' motor skills-the Early Motor Questionnaire (EMQ)-and aimed to improve the utility of the EMQ as a tool to examine variability, stability, and individual differences in early motor development. A sample of 446 parents of infants provided a total of 775 EMQ responses for analyses. Using this large sample, regression was used to create age-independent scores for global, gross motor, fine motor, and perception-action scores on the EMQ. Age-adjusted scores were then converted to t-scores to facilitate score interpretation for past and future studies using the EMQ. Finally, starting flags for different age groups were created to decrease the time it takes parents to complete the EMQ. Together, these changes to the EMQ will improve the utility and interpretability of the measure. The EMQ is free to use and available in the supplemental materials or via www.onlinebabylab.com/emq.

Moving the Needle Toward Fair Compensation in Pediatric Nephrology.

Remuneration issues are a substantial threat to the long-term stability of the pediatric nephrology workforce. It is uncertain whether the pediatric nephrology workforce will meet the growing needs of children with kidney disease without a substantial overhaul of the current reimbursement policies. In contrast to adult nephrology, the majority of pediatric nephrologists practice in an academic setting affiliated with a university and/or children's hospital. The pediatric nephrology service line is crucial to maintaining the financial health and wellness of a comprehensive children's hospital. However, in the current fee-for-service system, the clinical care for children with kidney disease is neither sufficiently valued, nor appropriately compensated. Current compensation models derived from the relative value unit (RVU) system contribute to the structural biases inherent in the current inequitable payment system. The perceived negative financial compensation is a significant driver of waning trainee interest in the field which is one of the least attractive specialties for students, with a significant proportion of training spots going unfilled each year and relatively stagnant growth rate as compared to the other pediatric subspecialties. This article reviews the current state of financial compensation issues plaguing the pediatric nephrology subspecialty. We further outline strategies for pediatric nephrologists, hospital administrators, and policy-makers to improve the landscape of financial reimbursement to pediatric subspecialists. A physician compensation model is proposed which aligns clinical activity with alternate metrics for current non-RVU producing activities that harmonizes hospital and personal mission statements.

A review of ferric citrate clinical studies, and the rationale and design of the Ferric Citrate and Chronic Kidney Disease in Children (FIT4KiD) trial.

Pediatric chronic kidney disease (CKD) is characterized by many co-morbidities, including impaired growth and development, CKD-mineral and bone disorder, anemia, dysregulated iron metabolism, and cardiovascular disease. In pediatric CKD cohorts, higher circulating concentrations of fibroblast growth factor 23 (FGF23) are associated with some of these adverse clinical outcomes, including CKD progression and left ventricular hypertrophy. It is hypothesized that lowering FGF23 levels will reduce the risk of these events and improve clinical outcomes. Reducing FGF23 levels in CKD may be accomplished by targeting two key stimuli of FGF23 production-dietary phosphate absorption and iron deficiency. Ferric citrate is approved for use as an enteral phosphate binder and iron replacement product in adults with CKD. Clinical trials in adult CKD cohorts have also demonstrated that ferric citrate decreases circulating FGF23 concentrations. This review outlines the possible deleterious effects of excess FGF23 in CKD, summarizes data from the adult CKD clinical trials of ferric citrate, and presents the Ferric Citrate and Chronic Kidney Disease in Children (FIT4KiD) study, a randomized, placebo-controlled trial to evaluate the effects of ferric citrate on FGF23 in pediatric patients with CKD stages 3-4 (ClinicalTrials.gov Identifier NCT04741646).

Revising the FOCUS framework through a qualitative study assessing self-reported counseling skills of genetic counselors.

Frameworks have been developed to help conceptualize clinical genetic counseling (GC), and observational studies have helped understand the process and content of GC sessions. However, additional research is needed to identify GC skills (behaviors or strategies) that practicing genetic counselors report consciously using to meet certain GC goals and determine what common terminology, if any, is being used to describe the various skills. Nineteen practicing genetic counselors in prenatal, pediatric, or cancer specialties were interviewed to elicit how they achieve session goals. Interview recordings were transcribed, coded thematically, and categorized using process categories from the communication strategy domain of the Framework for Outcomes in Clinical Communication Services (FOCUS). Reported skills largely fit within FOCUS, though findings prompted minor modifications of several FOCUS process categories and consolidation of the categories from 13 into 10. Although genetic counselor respondents reported a broad range of strategies and behaviors, they rarely had terms for skills they described. Results reveal concrete examples of GC skills, provide evidence for refinement of FOCUS, and highlight the need for establishing common terminology to describe these skills.

Variability in CKD Biomarker Studies: Soluble Urokinase Plasminogen Activator Receptor (suPAR) and Kidney Disease Progression in the Chronic Kidney Disease in Children (CKiD) Study.

RATIONALE & OBJECTIVE: Biomarker studies are important for generating mechanistic insight and providing clinically useful predictors of chronic kidney disease (CKD) progression. However, variability across studies can often muddy the evidence waters. Here we evaluated real-world variability in biomarker studies using two published studies, independently conducted, of the novel plasma marker soluble urokinase-type plasminogen activator receptor (suPAR) for predicting CKD progression in children with CKD. STUDY DESIGN: A comparison of 2 prospective cohort studies. SETTING & PARTICIPANTS: 541 children from the Chronic Kidney Disease in Children (CKiD) study, median age 12 years, median glomerular filtration rate (GFR) of 54 mL/min/1.73m2. OUTCOME: The first occurrence of either a 50% decline in GFR from baseline or incident end-stage kidney disease. ANALYTICAL APPROACH: The suPAR plasma marker was measured using the Quantikine ELISA immunoassay in the first study and Meso Scale Discovery (MSD) platform in the second. The analytical approaches varied. We used suPAR data from the 2 assays and mimicked each analytical approach in an overlapping subset. RESULTS: We found that switching assays had the greatest impact on inferences, resulting in a 38% to 66% change in the magnitude of the effect estimates. Covariate and modeling choices resulted in an additional 8% to 40% variability in the effect estimate. The cumulative variability led to different inferences despite using a similar sample of CKiD participants and addressing the same question. LIMITATIONS: The estimated variability does not represent optimal repeatability but instead illustrates real-world variability that may be present in the CKD biomarker literature. CONCLUSIONS: Our results highlight the importance of validation, avoiding conclusions based on P value thresholds, and providing comparable metrics. Further transparency of data and equal weighting of negative and positive findings in explorations of novel biomarkers will allow investigators to more quickly weed out less promising biomarkers.

Flexibility in an emergency life-history stage: acute food deprivation prevents sickness behaviour but not the immune response.

The emergency life-history stage (ELHS) can be divided into two subcategories that describe distinct, coordinated responses to disease- or non-disease-related physiological challenges. Whether an individual can simultaneously express aspects of both subcategories when faced with multiple challenges is poorly understood. Emergency life-history theory suggests that disease- and non-disease-related responses are coordinated at the level of the whole organism and therefore cannot be expressed simultaneously. However, the reactive scope and physiological regulatory network models suggest that traits can be independently regulated, allowing for components of both disease- and non-disease-related responses to be simultaneously expressed within a single organism. To test these ideas experimentally, we subjected female zebra finches to food deprivation, an immune challenge, both, or neither, and measured a suite of behavioural and physiological traits involved in the ELHS. We examined whether the trait values expressed by birds experiencing simultaneous challenges resembled trait values of birds experiencing a single challenge or if birds could express a mixture of trait values concurrently. We find that birds can respond to simultaneous challenges by regulating components of the behavioural and immune responses independently of one another. Modularity within these physio-behavioural networks adds additional dimensions to how we evaluate the intensity or quality of an ELHS. Whether modularity provides fitness advantages or costs in nature remains to be determined.

A comparison of recreational skiing- and snowboarding-related injuries at a Colorado ski resort, 2012/13-2016/17.

The purpose of this study was to compare injury patterns between recreational skiers and snowboarders. Injured skiers (n = 3,961) and snowboarders (n = 2,428) presented to a mountainside medical clinic, 2012/13-2016/17. Variables investigated for analysis included demographics/characteristics, injury event information, and injury information. Skiers were older than snowboarders (34.3 ± 19.3 vs. 23.2 ± 10.5 years, p < 0.001); a greater proportion of skiers were female (46.3% vs. 27.8%, p < 0.001). Most skiers (84.4%) and snowboarders (84.5%) were helmeted at the time of injury (p = 0.93). Snowboarders were most frequently beginners (38.9%), skiers were intermediates (37.8%). Falls to snow (skiers = 72.3%, snowboarders = 84.8%) and collisions with natural objects (skiers = 9.7%, snowboarders = 7.4%) were common injury mechanisms. Common skiing injuries were knee sprains (20.5%) and head trauma (8.9%); common snowboarding injuries were wrist fractures (25.7%), shoulder separations (9.1%), and head trauma (9.0%). Given that injury patterns significantly differ between sports, it is important for clinicians, ski patrollers, and resorts to develop and deliver sport-specific injury prevention interventions to most effectively decrease injury burden.