Search

Best treatments for initial presentation and relapses in children with nephrotic syndrome (NS) are still to be defined. The PROPINE study, published in this issue of Kidney International, demonstrates for relapse of childhood NS, the non-inferiority of a short taper (over 36 days) after remission with steroids. This study reinforces the need for more well-designed studies and the incorporation of predictive biomarkers, genetic studies, and other details to personalize treatment for each child with idiopathic NS., (Copyright © 2020 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Background: Excessive inpatient administrative tasks can lead to adverse consequences for residents and their patients. Furthermore, this burden has been linked to depersonalization, a major component of physician burnout., Objective: To describe the development, implementation, feasibility, acceptability, and early outcomes of Resident Team Assistant (RTA) programs., Methods: Three large academic medical centers created RTA programs in which administrative assistants are incorporated into inpatient medical teams. First steps included a needs assessment and driver diagram creation to identify key issues and to solidify goals. Program directors were assigned, and RTAs were hired, trained, and incorporated into inpatient teams at each institution (2003, 2016, 2018). Program leadership and institutional stakeholders met regularly to discuss development and quality assurance. Surveys and direct interviews were performed to evaluate impact and acceptability. Institutional goals in accordance to RTAs tasks were also investigated., Results: Resident surveys and interviews have shown acceptability with RTAs completing a large percentage of resident administrative tasks while promoting time spent in direct clinical care and job satisfaction. Hospital-specific improvements have included increase in referring physician communication rate and decrease in work hour violations. The programs have maintained high feasibility and sustainability with a relatively low time commitment from leadership and cost for the institutions., Conclusions: The RTA programs at the 3 institutions have continued to be sustained over time with perceived improvements in administrative task burden and job satisfaction for the residents. They have maintained high acceptability and feasibility in terms of effort and costs for the hospitals., Competing Interests: Conflict of interest: The authors declare they have no competing interests., (© 2020.)

Introduction: Patients receiving pediatric tracheostomy have significant risk for mortality due to compromised airway. Timely management of airway emergencies in children with tracheostomies is an important clinical skill for pediatricians. We developed this curriculum to improve residents' self-efficacy with tracheostomy management., Methods: We collected baseline data on 67 residents from two hospitals while creating a blended curriculum with video-based instruction on routine tracheostomy change and team management of tracheostomy emergency. Forty residents enrolled in the curriculum. During an ICU rotation, they received face-to-face instruction on routine tracheostomy change in small groups, followed by assessment of managing a tracheostomy emergency during a simulation. A video completed prior to the simulation took 9 minutes, the routine tracheostomy change didactic session took 15 minutes, and the simulation instruction was completed in 10-15 minutes. We collected feedback on the effectiveness of the curriculum from the participants., Results: All 107 residents from the baseline and intervention groups completed the self-efficacy survey. The intervention group had significantly higher changes in scores across all self-efficacy domains than the baseline group. On the curriculum feedback survey, residents rated the curriculum very highly, between 4.4 and 4.8 on a 5-point Likert scale., Discussion: Our blended curriculum increased learners' self-efficacy and promoted learner competence in tracheostomy management. Residents scored more than 80% across all aspects of simulation assessment and reported higher self-efficacy scores following our curricular intervention., (© 2020 Benjamin et al.)

Objective: Describe the demographics of pediatric and internal medicine/pediatric residents participating in global health (GH) experiences and examine relationships between GH involvement and self-perceived burnout, resilience, mindfulness, empathy, and spirituality., Methods: The Pediatric Resident Burnout and Resilience Study Consortium developed a national longitudinal study through collaboration with the Association of Pediatric Program Directors' Longitudinal Educational Assessment Research Network. Electronic surveys were administered to pediatric trainees annually (2016-2018). GH and well-being data were extracted. Descriptive statistics were calculated., Results: Of 9653 eligible pediatric and medicine/pediatric residents from 55 institutions, 6150 responded to the survey in 1 or more years, with average completion rate of 63.7% over a 3-year period. Controlling for repeat survey-takers, 12.7% (536/4213) of residents reported involvement in a GH-specific pathway, curricula, or track. GH participants were significantly more likely to be unmarried (P < .001), childless (P = .003), and medicine/pediatric trainees (P < .001). Controlling for repeated measures and demographic factors, GH participants demonstrated higher levels of empathic concern (P < .001) and higher spirituality scores in 2 of 3 domains (P < .01/<.05). GH involvement was not associated with lower reports of burnout or improved resilience/mindfulness., Conclusion: Although GH involvement is associated with increased levels of empathy and spirituality, it was not protective against burnout in this study. This highlights the need to study and promote the well-being of all residents, and perhaps especially those experiencing the challenges of working in low-resource settings. Future efforts should determine the impact of predeparture training, programmatic support, and post-trip debriefing on resident well-being., (Copyright © 2020 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Objectives: Fellowship program directors (FPD) and Clinical Competency Committees (CCCs) both assess fellow performance. We examined the association of entrustment levels determined by the FPD with those of the CCC for 6 common pediatric subspecialty entrustable professional activities (EPAs), hypothesizing there would be strong correlation and minimal bias between these raters., Methods: The FPDs and CCCs separately assigned a level of supervision to each of their fellows for 6 common pediatric subspecialty EPAs. For each EPA, we determined the correlation between FPD and CCC assessments and calculated bias as CCC minus FPD values for when the FPD was or was not a member of the CCC. In addition, we examined the effect of program size, FPD understanding of EPAs, and subspecialty on the correlations. Data were obtained in fall 2014 and spring 2015., Results: A total of 1040 fellows were assessed in the fall and 1048 in the spring. In both periods and for each EPA, there was a strong correlation between FPD and CCC supervision levels ( P  < .001). The correlation was somewhat lower when the FPD was not a CCC member ( P  < .001). Overall bias in both periods was small., Conclusions: The correlation between FPD and CCC assignment of EPA supervision levels is strong. Although slightly weaker when the FPD is not a CCC member, bias is small, so this is likely unimportant in determining fellow entrustment level. The similar performance ratings of FPDs and CCCs support the validity argument for EPAs as competency-based assessment tools., Competing Interests: Declaration of conflicting interests:The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2020.)

Introduction: Prior cross-sectional studies suggest that health-related quality of life (HRQOL) worsens with more severe glomerular disease. This longitudinal analysis was conducted to assess changes in HRQOL with changing disease status., Methods: Cure Glomerulonephropathy (CureGN) is a cohort of patients with minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, IgA vasculitis, or IgA nephropathy. HRQOL was assessed at enrollment and follow-up visits 1 to 3 times annually for up to 5 years with the Patient-Reported Outcomes Measurement Information System (PROMIS). Global health, anxiety, and fatigue domains were measured in all; mobility was measured in children; and sleep-related impairment was measured in adults. Linear mixed effects models were used to evaluate HRQOL responsiveness to changes in disease status., Results: A total of 469 children and 1146 adults with PROMIS scores were included in the analysis. HRQOL improved over time in nearly all domains, though group-level changes were modest. Edema was most consistently associated with worse HRQOL across domains among children and adults. A greater number of symptoms also predicted worse HRQOL in all domains. Sex, age, obesity, and serum albumin were associated with some HRQOL domains. The estimated glomerular filtration rate (eGFR) was only associated with fatigue and adult physical health; proteinuria was not associated with any HRQOL domain in adjusted models., Conclusion: HRQOL measures were responsive to changes in disease activity, as indicated by edema. HRQOL over time was not predicted by laboratory-based markers of disease. Patient-reported edema and number of symptoms were the strongest predictors of HRQOL, highlighting the importance of the patient experience in glomerular disease. HRQOL outcomes inform understanding of the patient experience for children and adults with glomerular diseases., (© 2020 Published by Elsevier, Inc., on behalf of the International Society of Nephrology.)

Objective The American Board of Pediatrics Certifying Exam (ABP CE) is a high stakes exam and is important for employment and fellowship opportunities in pediatrics. Although research has suggested interventions which may improve scores, little research has focused on resident perception of preparation for the ABP CE. In this study, we aimed to better define pediatric residents' attitudes and preferences regarding preparation for the ABP CE. Methods Pediatric residents from one residency program were invited to participate in focus groups to discuss their attitudes and preferences on board exam preparation for the ABP CE. Focus groups were audio recorded and transcribed. Conventional content analysis was used to analyze qualitative data. From the transcripts, authors developed codes through an iterative process, which were then organized into categories. These categories were then grouped into themes. Results Nineteen residents participated in three focus groups. Focus group transcription analysis resulted in 49 codes, which were sorted into 26 categories. The categories were then grouped into four key themes: 1) the ABP CE is not immediately important early in residency; 2) more personalized guidance is preferred; 3) consistent board preparation focus from the residency program is valued; 4) learning format is important. Conclusions Residents believe preparation for the ABP CE increases in importance as they progress through residency, and they desire more personalized, consistent, and structured focus from their training program related to ABP CE preparation. Attention to these perceptions can help guide pediatric residency program leadership in developing effective board exam preparation strategies and curricula for their residents., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Liu et al.)

Applying for a pediatric residency position has become an increasingly stressful event and recently medical educators have described it as a "Match Frenzy." Match statistics demonstrate increased competition for pediatric residency positions and a record number of applications. Faculty who mentor medical students are now challenged to counsel them through the intensified process and pediatric residency programs are now forced to navigate the rising number of applications. We define the Match Frenzy and its implications, review historical and current match statistics, and describe a data-driven approach to the problem. Through mitigating the frenzy surrounding this process, we can help students and residency programs better allocate their precious resources., (Copyright © 2019 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Background: Kidney transplant (KT) recipients have higher incidence of malignancies, including Human Papillomavirus (HPV)-associated cancers. Thus, HPV vaccines may have an important role in preventing HPV-related disease in this population; however, immunogenicity and safety data are lacking. Objective: To examine the immunological response and tolerability to HPV vaccination in pediatric KT recipients compared to future KT candidates. Methods: The quadrivalent HPV vaccine was administered to girls and boys age 9-18 recruited from seven centers part of the Pediatric Nephrology Research Consortium. Subjects were recruited for three groups: (1) CKD: chronic kidney disease stages 3, 4, and 5 not on dialysis; (2) Dialysis; (3) KT recipients. The outcome consisted of antibody concentrations against HPV 6, 11, 16, and 18. Geometric mean titers (GMTs) and seroconversion rates were compared. Vaccine tolerability was assessed. Results: Sixty-five participants were recruited: 18 in the CKD, 18 in the dialysis, and 29 into the KT groups. KT patients had significantly lower GMTs after vaccination for all serotypes. The percentages of subjects who reached seroconversion were overall lower for the KT group, reaching statistical significance for HPV 6, 11, and 18. Comparing immunosuppressed subjects (anyone taking immunosuppression medications, whether KT recipient or not) with the non-immunosuppressed participants, the former had significantly lower GMTs for all the HPV serotypes and lower seroconversion rates for HPV 6, 11, and 18. KT females had higher GMTs and seroconversion rates for certain serotypes. There were no adverse events in either group. Conclusions: HPV vaccine was well-tolerated in this population. Pediatric KT recipients had in general lower GMTs and seroconversion rates compared to their peers with CKD or on dialysis. Immunosuppression played a role in the lack of seroconversion. Our results emphasize the importance of advocating for HPV vaccination prior to KT and acknowledge its safety post transplantation. Future studies are needed to investigate the effect of a supplemental dose of HPV vaccine in KT recipients who do not seroconvert and to evaluate the long-term persistence of antibodies post-KT., (Copyright © 2020 Nailescu, Nelson, Verghese, Twombley, Chishti, Mills, Mahan, Slaven and Shew.)

Background: We evaluated the epidemiology of fluid balance (FB) over the first postnatal week and its impact on outcomes in a multi-center cohort of premature neonates from the AWAKEN study., Methods: Retrospective analysis of infants <36 weeks' gestational age from the AWAKEN study (N = 1007). FB was defined by percentage of change from birth weight., Outcome: Mechanical ventilation (MV) at postnatal day 7., Results: One hundred and forty-nine (14.8%) were on MV at postnatal day 7. The median peak FB was 0% (IQR: -2.9, 2) and occurred on postnatal day 2 (IQR: 1,5). Multivariable models showed that the peak FB (aOR 1.14, 95% CI 1.10-1.19), lowest FB in first postnatal week (aOR 1.12, 95% CI 1.07-1.16), and FB on postnatal day 7 (aOR 1.10, 95% CI 1.06-1.13) were independently associated with MV on postnatal day 7. In a similar analysis, a negative FB at postnatal day 7 protected against the need for MV at postnatal day 7 (aOR 0.21, 95% CI 0.12-0.35)., Conclusions: Positive peak FB during the first postnatal week and more positive FB on postnatal day 7 were independently associated with MV at postnatal day 7. Those with a negative FB at postnatal day 7 were less likely to require MV.

Background and Objectives: Congenital or primary nephrogenic diabetes insipidus (NDI) is a rare genetic disorder that severely impairs renal concentrating ability, resulting in massive polyuria. There is limited information about prognosis or evidence guiding the management of these patients, either in the high-risk period after diagnosis, or long-term. We describe the clinical presentation, genetic etiology, treatment and renal outcomes in a large group of children <21 years with NDI. Design: A multi-center retrospective chart review. Results: We report on 66 subjects from 16 centers. They were mainly male (89%) and white (67%). Median age at diagnosis was 4.2 months interquartile range (IQR 1.1, 9.8). A desmopressin acetate loading test was administered to 46% of children at a median age of 4.8 months (IQR 2.8, 7.6); only 15% had a water restriction test. Genetic testing or a known family history was present in 70% of the patients; out of those genetically tested, 89 and 11% had mutations in AVPR2 and AQP2 , respectively. No positive family history or genetic testing was available for 30%. The most common treatments were thiazide diuretics (74%), potassium-sparing diuretics (67%) and non-steroidal anti-inflammatory drugs (42%). At the time of first treatment, 70 and 71% of children were below -2 standard deviations (SD) for weight and height, respectively. At last follow-up, median age was 72.3 months (IQR 40.9, 137.2) and the percentage below -2 SD improved to 29% and 38% for weight and height, respectively. Adverse outcomes included inpatient hospitalizations (61%), urologic complications (37%), and chronic kidney disease (CKD) stage 2 or higher in 23%. Conclusion: We found the majority of patients were treated with thiazides with either a potassium sparing diuretic and/or NSAIDs. Hospitalizations, urologic complications, short stature, and CKD were common. Prospective trials to evaluate different treatment strategies are needed to attempt to improve outcomes., (Copyright © 2020 D'Alessandri-Silva, Carpenter, Ayoob, Barcia, Chishti, Constantinescu, Dell, Goodwin, Hashmat, Iragorri, Kaspar, Mason, Misurac, Muff-Luett, Sethna, Shah, Weng, Greenbaum and Mahan.)

Background: We aimed to describe the national epidemiology of burnout in pediatric residents., Methods: We conducted surveys of residents at 34 programs in 2016, 43 programs in 2017, and 49 programs in 2018. Survey items included the Maslach Burnout Inventory, demographics, program characteristics, personal qualities, experiences, and satisfaction with support, work-life balance, and learning environment. Analyses included cross-sectional comparisons and cross-sectional and longitudinal regression., Results: More than 60% of eligible residents participated; burnout rates were >50% in all years and not consistently associated with any demographic or residency characteristics. Cross-sectional associations were significant between burnout and stress, sleepiness, quality of life, mindfulness, self-compassion, empathy, confidence in providing compassionate care (CCC), being on a high-acuity rotation, recent major medical error, recent time off, satisfaction with support and career choice, and attitudes about residency. In cross-sectional logistic regression analyses, 4 factors were associated with an increased risk of burnout: stress, sleepiness, dissatisfaction with work-life balance, and recent medical error; 4 factors were associated with lower risk: empathy, self-compassion, quality of life, and CCC. Longitudinally, after controlling for 2017 burnout and 2018 risk factors (eg, recent error, sleepiness, rotation, and time off), 2017 quality of life was associated with 2018 burnout; 2017 self-compassion was associated with lower 2018 stress; and 2017 mindfulness, empathy, and satisfaction with learning environment and career choice were associated with 2018 CCC., Conclusions: A majority of residents met burnout criteria. Several identified factors (eg, stress, sleepiness, medical errors, empathy, CCC, and self-compassion) suggest targets for interventions to reduce burnout in future studies., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2020 by the American Academy of Pediatrics.)

Physician burnout is a highly complex phenomenon whose origins are multifactorial. As the medical profession works to better understand and reduce physician burnout, conceptual models can offer a framework to guide research and practice in the field of physician well-being. Conceptual models represent complex systems in a simplified fashion that facilitates understanding of and communication about those systems. This paper reviews seven conceptual models of physician well-being and discusses their strengths and limitations., (Copyright © 2019 Elsevier Inc. All rights reserved.)

It is clear that graduates of osteopathic medical schools desire to maintain their Osteopathic Manipulative Medicine (OMM) and Osteopathic Principles and Practice (OPP) skills and mindset of their professional identity. In a recent survey, 68% of 1,523 third year osteopathic medical students indicated that it would be more appealing to attend a residency with osteopathic recognition, with more than half indicating this would play an important role in how they made their rank list. There are currently few options available to DO students that would like to maintain their OMM and OPP skills during pediatric residency programs, and with an increasing number of DO graduates each year, there may be a need to provide more opportunities for them. In this article we describe our pediatric Osteopathic Recognition Track, which has the goal of providing an appropriate level of osteopathic focused training to our small number of residents in the track (4 annually) while incorporating them fully into a large and very busy pediatric program. We use Bloom's taxonomy as the framework upon which to provide details about our approach., (Copyright © 2019 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Purpose: Burnout symptoms are common among health professionals. Gaps remain in understanding both the stability of burnout and compassion over time and relationships among burnout, self-compassion, stress, and mindfulness in pediatric residents., Method: The authors conducted a prospective cohort study of residents at 31 U.S. residency programs affiliated with the Pediatric Resident Burnout-Resilience Study Consortium. Residents completed online cross-sectional surveys in spring 2016 and 2017. The authors assessed demographic characteristics and standardized measures of mindfulness, self-compassion, stress, burnout, and confidence in providing compassionate care., Results: Of 1,108 eligible residents, 872 (79%) completed both surveys. Of these, 72% were women. The prevalence of burnout was 58% and the level of mindfulness was 2.8 in both years; levels of stress (16.4 and 16.2) and self-compassion (37.2 and 37.6) were also nearly identical in both years. After controlling for baseline burnout levels in linear mixed-model regression analyses, mindfulness in 2016 was protective for levels of stress and confidence in providing compassionate care in 2017. Self-compassion in 2016 was protective for burnout, stress, and confidence in providing compassionate care in 2017; a one-standard-deviation increase in self-compassion score was associated with a decrease in the probability of burnout from 58% to 48%., Conclusions: Burnout and stress were prevalent and stable over at least 12 months among pediatric residents. Mindfulness and self-compassion were longitudinally associated with lower stress and greater confidence in providing compassionate care. Future studies are needed to evaluate the effectiveness of training that promotes mindfulness and self-compassion in pediatric residents.

Childhood nephrotic syndrome is a challenging and often persistent renal disorder, and its incidence varies between different ethnicities and regions. Corticosteroids have been the main treatment for decades and are effective in most children with idiopathic NS, although 10-15% of these children become steroid resistant. Furthermore, some initially steroid sensitive children follow a steroid dependent or frequently relapsing course and are therefore at increased risk for developing steroid toxicity. In such children, alternative immunosuppressive medications are used to induce and/or maintain remission of NS. One such drug, rituximab, is a monoclonal antibody directed against the B lymphocyte CD20 marker which induces depletion of B cells, and has shown promising results in the management of NS in children. In this review, we summarize recent studies on the efficacy and safety of rituximab in the different types of childhood nephrotic syndrome, the known and potential mechanisms of action of rituximab, its possible complications and side effects, and the available and potential biomarkers of rituximab activity.

Objective: Nephrotic syndrome (NS) is a kidney disease known to adversely impact health-related quality of life (HRQOL). Patient-reported outcome (PRO) measures are commonly used to characterize HRQOL and the patient disease experience. This study aims to improve the interpretability and clinical utility of the Patient-Reported Outcomes Measurement Information System® (PROMIS®) by identifying distinct meaningful HRQOL profiles in children and adults with NS., Method: Patients were from 2 prospective NS cohort studies (PROMIS-II®: 121 children; NEPTUNE: 40 children and 219 adults) with data from 6 PROMIS® domains. Latent Profile Analysis was used to identify subgroups of patients based on PROMIS® score patterns. A 3-step analysis of latent profile predictors was used to determine how clinical parameters predicted HRQOL profile membership., Results: We identified 3 HRQOL profiles (Good, Average, and Poor) with strong indicators of membership classification (entropy >0.86). Complete proteinuria remission, reduction in symptoms, and shorter disease duration, were significant predictors of better HRQOL profile membership., Conclusions: Patients with NS can be classified by HRQOL into clinically meaningful categories. Integrating this approach into clinic may help in the identification of individuals with poor HRQOL will help clinicians better manage their symptoms and researchers study the causes and possible interventions for these patients. PROMIS® HRQOL profiles were reproducible in replication cohorts. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

There is scant literature describing the effect of glomerular disease on health-related quality of life (HRQOL). The Cure Glomerulonephropathy study (CureGN) is an international longitudinal cohort study of children and adults with four primary glomerular diseases (minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy). HRQOL is systematically assessed using items from the Patient-Reported Outcomes Measurement Informative System (PROMIS). We assessed the relationship between HRQOL and demographic and clinical variables in 478 children and 1115 adults at the time of enrollment into CureGN. Domains measured by PROMIS items included global assessments of health, mobility, anxiety, fatigue, and sleep impairment, as well as a derived composite measure incorporating all measured domains. Multivariable models were created that explained 7 to 32% of variance in HRQOL. Patient-reported edema consistently had the strongest and most robust association with each measured domain of HRQOL in multivariable analysis (adjusted β [95% CI] for composite PROMIS score in children, -5.2 [-7.1 to -3.4]; for composite PROMIS score in adults, -6.1 [-7.4 to -4.9]). Female sex, weight (particularly obesity), and estimated glomerular filtration rate were also associated with some, but not all, domains of HRQOL. Primary diagnosis, disease duration, and exposure to immunosuppression were not associated with HRQOL after adjustment. Sensitivity analyses and interaction testing demonstrated no significant association between disease duration or immunosuppression and any measured domain of HRQOL. Thus, patient-reported edema has a consistent negative association with HRQOL in patients with primary glomerular diseases, with substantially greater impact than other demographic and clinical variables., (Copyright © 2019 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Background: Measuring burnout symptoms is important, but the Maslach Burnout Inventory (MBI) has 22 items. This project compared 3 single-item measures with the MBI and other factors related to burnout., Methods: Data were analyzed from the 2016 and 2017 Pediatric Resident Burnout-Resilience Study Consortium surveys, which included standard measures of perceived stress, mindfulness, resilience, and self-compassion; the MBI; and the 1- and 2-item screening questions., Results: In 2016 and 2017, data were collected from 1785/2723 (65%) and 2148/3273 (66%) eligible pediatric residents, respectively. Burnout rates on the MBI were 56% in 2016 and 54% in 2017. The Physician Work Life Study item generated estimates of burnout prevalence of 43% to 49% and, compared with the MBI for 2016 and 2017, had sensitivities of 69% to 72%, specificities of 79% to 82%, positive likelihood ratios of 3.4 to 3.8, and negative likelihood ratios of 0.35 to 0.38. The combination of an emotional exhaustion item and a depersonalization item generated burnout estimates of 53% in both years and, compared with the full MBI, had sensitivities of 85% to 87%, specificities of 84% to 85%, positive likelihood ratios of 5.7 to 6.4, and negative likelihood ratios of 0.18 for both years. Both items were significantly correlated with their parent subscales. The single items were significantly correlated with stress, mindfulness, resilience, and self-compassion., Conclusions: The 1- and 2-item screens generated prevalence estimates similar to the MBI and were correlated with variables associated with burnout. The 1- and 2-item screens may be useful for pediatric residency training programs tracking burnout symptoms and response to interventions., (Copyright © 2018 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Introduction: Children with neuromuscular disabilities (NMD) receive care in a wide variety of clinical settings. Residents lack training to develop physical examination skills for evaluating patients with NMD. We devised a curriculum to teach residents how to examine patients with NMD using a systematic and simplified approach., Methods: Creation of this resource was a response to a survey of final-year residents that revealed the need for education focused on developing physical examination skills. The curriculum has four components-multimedia PowerPoint with embedded video, knowledge assessment, clinical exam (CEX) assessment, and module feedback-and was completed by 37 residents over an 8-month period from January to September 2016. We utilized knowledge assessment, direct clinical skills observation using the CEX, and module-feedback responses as part of the evaluation., Results: All 37 residents completed the curriculum, with an overall knowledge score of greater than 80%. Residents demonstrated most of the desired patient care behaviors on the CEX assessment and provided positive feedback on the quality, usefulness, and applicability of the module, in addition to requesting more curricula to develop their physical examination skills., Discussion: The CEX assessment provided a unique opportunity for faculty feedback on residents' physical exam performance. After completing the module, residents achieved high scores in most areas of the standardized CEX and were able to conduct the NMD physical exam in a sensitive manner. The assessment highlighted the need to improve residents' skills of detecting abnormal clinical findings and communicating with the patient during the physical exam., Competing Interests: None to report.

Background: In sick neonates admitted to the NICU, improper fluid balance can lead to fluid overload. We report the impact of fluid balance in the first postnatal week on outcomes in critically ill near-term/term neonates., Methods: This analysis includes infants ≥36 weeks gestational age from the Assessment of Worldwide Acute Kidney injury Epidemiology in Neonates (AWAKEN) study (N = 645). Fluid balance: percent weight change from birthweight., Primary Outcome: mechanical ventilation (MV) on postnatal day 7., Results: The median peak fluid balance was 1.0% (IQR: -0.5, 4.6) and occurred on postnatal day 3 (IQR: 1, 5). Nine percent required MV at postnatal day 7. Multivariable models showed the peak fluid balance (aOR 1.12, 95%CI 1.08-1.17), lowest fluid balance in 1st postnatal week (aOR 1.14, 95%CI 1.07-1.22), fluid balance on postnatal day 7 (aOR 1.12, 95%CI 1.07-1.17), and negative fluid balance at postnatal day 7 (aOR 0.3, 95%CI 0.16-0.67) were independently associated with MV on postnatal day 7., Conclusions: We describe the impact of fluid balance in critically ill near-term/term neonates over the first postnatal week. Higher peak fluid balance during the first postnatal week and higher fluid balance on postnatal day 7 were independently associated with MV at postnatal day 7.

Background: Traditionally, quizzes have been applied as a tool for summative assessment, though literature suggests their use as a formative assessment can improve motivation and content retention. With this premise, we implemented a series of intermittent, online quizzes known as the Board Examination Simulation Exercise (BESE). We sought to demonstrate an association between BESE participation and scores and performance on the American Board of Pediatrics (ABP) Certifying Examination (CE)., Methods: Residents were assigned online quizzes on a single topic at 2 week intervals that consisted of 20 multiple choice questions written by the study authors. This analysis includes graduates of 3 Pediatric and Internal Medicine-Pediatrics residency programs., Results: Data were available for 329 residents. The overall BESE score weakly correlated with ABP CE score (n = 287; r = 0.39, p < 0.0001). ABP CE pass rates increased from 2009 to 2016 at all programs combined (p = 0.0001). A composite BESE score ≤ 11 had sensitivity of 54% and specificity of 80% for predicting ABP CE failure on the first attempt. There was no difference in ABP CE failure rates or scores by number of completed quizzes., Conclusion: Intermittent online quizzes implemented at three pediatric residency programs were associated with overall increasing ABP CE pass rates. BESE increased program emphasis on board preparation. Residents with lower BESE scores more often failed ABP CE. Though additional data are needed, BESE is a promising tool for pediatric resident learning and board preparation. It may also aid in earlier identification of residents at higher risk of failing the ABP CE and facilitate targeted interventions.

Background: The Accreditation Council for Graduate Medical Education requires semiannual Milestone reporting on all residents. Milestone expectations of performance are unknown., Objective: To determine pediatric program director (PD) minimum Milestone expectations for residents before being ready to supervise and before being ready to graduate., Methods: Mixed methods survey of pediatric PDs on their programs' Milestone expectations before residents are ready to supervise and before they are ready to graduate, and in what ways PDs use Milestones to make supervision and graduation decisions. If programs had no established Milestone expectations, PDs indicated expectations they considered for use in their program. Mean minimum Milestone level expectations were adjusted for program size, region, and clustering of Milestone expectations by program were calculated for before supervise and before graduate. Free-text questions were analyzed using thematic analysis., Results: The response rate was 56.8% (113 of 199). Most programs had no required minimum Milestone level before residents are ready to supervise (80%; 76 of 95) or ready to graduate (84%; 80 of 95). For readiness to supervise, minimum Milestone expectations PDs considered establishing for their program were highest for humanism (2.46; 95% confidence interval [CI], 2.21-2.71) and professionalization (2.37; 95% CI, 2.15-2.60). Minimum Milestone expectations for graduates were highest for help-seeking (3.14; 95% CI, 2.83-3.46). Main themes included the use of Milestones in combination with other information to assess learner performance and Milestones are not equally weighted when making advancement decisions., Conclusions: Most PDs have not established program minimum Milestones, but would vary such expectations according to competency., (Copyright © 2018 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Despite increasing numbers of patients with neurodisability, residents lack training to develop physical examination skills. Following a blended educational intervention combining online and bedside teaching, residents demonstrated desired patient-care behaviors on standardized clinical exam assessment., (Copyright © 2018 Academic Pediatric Association. Published by Elsevier Inc. All rights reserved.)

Background: Congenital nephrogenic diabetes insipidus (NDI) is a rare genetic disorder affecting urinary concentration. Clinicians have varied medication regimens as well as nutritional plan approaches for these children., Materials and Methods: An electronic survey was distributed to member pediatric nephrologists of the Midwest Pediatric Nephrology Consortium via email (n = 179). Questions included types of drugs prescribed, factors contributing to drug choice, common drug combinations given, and dietary/failure to thrive interventions used., Results: We analyzed results from 72 respondents (42% overall response rate). 72% treated only 1 - 3 patients with NDI per year, 12% treated 4 or more, and 17% had no NDI patients. Of providers treating NDI patients, almost all prescribed thiazides (93%), 62% prescribed amiloride, and 55% reported prescribing nonsteroidal anti-inflammatory drugs (NSAIDs) as part of their drug regimen. gastrointestinal (GI) and renal side effects (43%) were given as reasons for not prescribing indomethacin. For 70%, drug choice was determined by severity of failure to thrive (FTT). Physicians were asked to define the most common drug combinations they prescribed. 48% reported prescribing indomethacin with hydrochlorothiazide. 84% of respondents have a renal dietitian on staff, and half included appointments with a dietitian as part of FTT therapy. The most common intervention for FFT was gastrostomy tube placement (78%)., Conclusion: Our results suggest consensus on the use of thiazides, while the use of indomethacin is limited by GI and renal side effect profile. Our results revealed that multiple drug combinations are frequently used without one specific preferred regimen.
.

Background: Interventions to address burnout include mind-body skills training (MBST), but few studies have evaluated the feasibility of MBST for busy pediatric residents., Objective: In this pilot study, we tested the feasibility of a brief MBST intervention, using in-person peer-led training supported by online modules, to decrease stress and burnout in pediatric resident physicians., Methods: Of 99 (10%) residents, 10 residents at Nationwide Children's Hospital in Ohio participated in up to four 90-minute MBST sessions more than 1 month, led by a co-resident with 5 years of informal training in mind-body skills. Participants were offered 8 assigned online modules through OSU Center for Integrative Health and Wellness. Measures including Maslach Burnout Inventory (MBI), Cohen's Perceived Stress, Smith's Brief Resilience, Cognitive and Affective Mindfulness Scale-Revised, and Neff's Self-Compassion Scale (NSS) were administered before (T1) and after (T2) the course. Participants were offered optional monthly "maintenance" sessions for 6 months and completed a third set of measures at this follow-up (T3)., Results: The residents completed an average of 4.3/8 online modules and attended an average of 2.8/4 in-person sessions. There was significant improvement in positive attitude, perceived stress, and resilience post intervention (T2). Follow-up evaluation (T3) also demonstrated significant improvement in burnout (depersonalization) and mindfulness. More than 75% of participants found the course worthwhile., Conclusions: A short mixed-method mindfulness-based skills course may be a practical way to offer resilience and stress management training to busy resident physicians., Competing Interests: Declaration of conflicting interests:The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To perform a derivation study to determine in which subcompetencies marginal/unsatisfactory pediatric residents had the greatest deficits compared with their satisfactorily performing peers and which subcompetencies best discriminated between marginal/unsatisfactory and satisfactorily performing residents., Method: Multi-institutional cohort study of all 21 milestones (rated on four or five levels) reported to the Accreditation Council for Graduate Medical Education, and global marginal/unsatisfactory versus satisfactory performance reported to the American Board of Pediatrics. Data were gathered in 2013-2014. For each level of training (postgraduate year [PGY] 1, 2, and 3), mean differences between milestone levels of residents with marginal/unsatisfactory and satisfactory performance adjusted for clustering by program and C-statistics (area under receiver operating characteristic curve) were calculated. A Bonferroni-corrected significance threshold of .0007963 was used to account for multiple comparisons., Results: Milestone and overall performance evaluations for 1,704 pediatric residents in 41 programs were obtained. For PGY1s, two subcompetencies had almost a one-point difference in milestone levels between marginal/unsatisfactory and satisfactory trainees and outstanding discrimination (≥ 0.90): organize/prioritize (0.93; C-statistic: 0.91) and transfer of care (0.97; C-statistic: 0.90). The largest difference between marginal/unsatisfactory and satisfactory PGY2s was trustworthiness (0.78). The largest differences between marginal/unsatisfactory and satisfactory PGY3s were ethical behavior (1.17), incorporating feedback (1.03), and professionalization (0.96). For PGY2s and PGY3s, no subcompetencies had outstanding discrimination., Conclusions: Marginal/unsatisfactory pediatric residents had different subcompetency gaps at different training levels. While PGY1s may have global deficits, senior residents may have different performance deficiencies requiring individualized counseling and targeted performance improvement plans.

Residency is a high-risk period for physician burnout. We aimed to determine the short-term stability of factors associated with burnout, application of these data to previous conceptual models, and the relationship of these factors over 3 months. Physician wellness questionnaire results were analyzed at 2 time points 3 months apart. Associations among variables within and across time points were analyzed. Logistic regression was used to predict burnout and compassionate care. A total of 74% of residents completed surveys. Over 3 months, burnout ( P = .005) and empathy ( P = .04) worsened. The most significant cross-sectional relationship was between stress and emotional exhaustion (time 1 r = 0.61, time 2 r = 0.68). Resilience was predictive of increased compassionate care and decreased burnout ( P < .05). Mindfulness was predictive of decreased burnout ( P < .05). Mitigating stress and fostering mindfulness and resilience longitudinally may be key areas of focus for improved wellness in pediatric residents. Larger studies are needed to better develop targeted wellness interventions.

Background: There is a high rate of burnout among health professionals, driving diverse attempts to promote resilience and well-being to counter this trend. The purpose of this project was to assess the dose-response relationship between the number of hours of online mind-body skills training for health professionals and relevant outcomes a year later., Methods: Among 1438 registrants for online training (including up to 12 hours of training on mind-body practices) between December 2013 and November 2015, we analyzed responses from the first 10% who responded to an anonymous online survey between December 1, 2015 and February 1, 2016. Questions included the type and frequency of mind-body practice in the past 30 days and whether the online training had any impact on personal life or professional practice. Standardized measures were used to assess stress, mindfulness, confidence in providing compassionate care, and burnout., Results: The 149 respondents represented a variety of ages and health professions; 55% completed one or more mind-body training modules an average of 14 months previously. Most (78%) engaged in one or more mind-body practices in the 30 days before the survey; 79% reported changes in self-care and 71% reported changes in the care of others as a result of participating. Increasing number of hours of training were significantly associated with practicing mind-body skills more frequently; increasing practice frequency was associated with less stress and burnout, which were associated with missing less work. Greater practice frequency was also associated with improvements in stress, mindfulness, and resilience, which were associated with increased confidence in providing compassionate care., Conclusion: Online training in mind-body therapies is associated with changes in self-reported behavior one year later; increasing doses of training are associated with more frequent practice which is associated with less stress, burnout, and missing work, and higher levels of mindfulness, resilience, and confidence in providing compassionate care. Additional studies are needed to compare mind-body skills training with other interventions designed to improve resilience and compassion while decreasing burnout in health professionals.

Zoledronic acid (ZA), a highly potent intravenous bisphosphonate (BP), has been increasingly used in children with primary and secondary osteoporosis due to its convenience of shorter infusion time and less frequent dosing compared to pamidronate. Many studies have also demonstrated beneficial effects of ZA in other conditions such as hypercalcemia of malignancy, fibrous dysplasia (FD), chemotherapy-related osteonecrosis (ON) and metastatic bone disease. This review summarizes pharmacologic properties, mechanism of action, dosing regimen, and therapeutic outcomes of ZA in a variety of metabolic bone disorders in children. Several potential novel uses of ZA are also discussed. Safety concerns and adverse effects are also highlighted., Competing Interests: Conflicts of Interest: SA Bowden is a member of the advisory board for Novartis. JD Mahan has no conflicts of interest to declare.

Introduction: Traditional normative Likert-type evaluations of faculty teaching have several drawbacks, including lack of granular feedback, potential for inflation, and the halo effect. To provide more meaningful data to faculty on their teaching skills and encourage educator self-reflection and skill development, we designed and implemented a milestone-based faculty clinical teaching evaluation tool., Methods: The evaluation tool contains 10 questions that assess clinical teaching skills with descriptive milestone behavior anchors. Nine of these items are based on the Stanford Faculty Development Clinical Teaching Model and annual Accreditation Council for Graduate Medical Education (ACGME) resident survey questions; the tenth was developed to address professionalism at our institution. The tool was developed with input from residency program leaders, residents, and the faculty development committee and piloted with graduate medical education learners before implementation., Results: More than 7,200 faculty evaluations by learners and 550 faculty self-evaluations have been collected. Learners found the form easy to use and preferred it to previous Likert-based evaluations. Over the 2 years that faculty self-evaluations have been collected, their scores have been similar to the learner evaluation scores. The feedback provided faculty with more meaningful data on teaching skills and opportunities for reflection and skill improvement and was used in constructing faculty teaching skills programs at the institutional level., Discussion: This innovation provides an opportunity to give faculty members more meaningful teaching evaluations and feedback. It should be easy for other institutions and programs to implement. It leverages a familiar milestone construct and incorporates important ACGME annual resident survey information., Competing Interests: None to report.

Background: Nephrotic syndrome represents a condition in pediatric nephrology typified by a relapsing and remitting course, proteinuria and the presence of edema. The PROMIS measures have previously been studied and validated in cross-sectional studies of children with nephrotic syndrome. This study was designed to longitudinally validate the PROMIS measures in pediatric nephrotic syndrome., Methods: One hundred twenty seven children with nephrotic syndrome between the ages of 8 and 17 years participated in this prospective cohort study. Patients completed a baseline assessment while their nephrotic syndrome was active, a follow-up assessment at the time of their first complete proteinuria remission or study month 3 if no remission occurred, and a final assessment at study month 12. Participants completed six PROMIS measures (Mobility, Fatigue, Pain Interference, Depressive Symptoms, Anxiety, and Peer Relationships), the PedsQL version 4.0, and two global assessment of change items., Results: Disease status was classified at each assessment: nephrotic syndrome active in 100% at baseline, 33% at month 3, and 46% at month 12. The PROMIS domains of Mobility, Fatigue, Pain Interference, Depressive Symptoms, and Anxiety each showed a significant overall improvement over time (p < 0.001). When the PROMIS measures were compared to the patients' global assessment of change, the domains of Mobility, Fatigue, Pain Interference, and Anxiety consistently changed in an expected fashion. With the exception of Pain Interference, change in PROMIS domain scores did not correlate with changes in disease activity. PROMIS domain scores were moderately correlated with analogous PedsQL domain scores., Conclusion: This study demonstrates that the PROMIS Mobility, Fatigue, Pain Interference, and Anxiety domains are sensitive to self-reported changes in disease and overall health status over time in children with nephrotic syndrome. The lack of significant anchoring to clinically defined nephrotic syndrome disease active and remission status may highlight an opportunity to improve the measurement of HRQOL in children with nephrotic syndrome through the development of a nephrotic syndrome disease-specific HRQOL measure.

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., (© American Academy of Pediatrics, 2017. All rights reserved.)

Objective: Our objective was to evaluate the utility of an educational program consisting of a workshop based on the Take the HEAT communication strategy, designed specifically for addressing patients who are angry, using a novel tool to evaluate residents' skills in employing this method., Methods: 33 first-year pediatric and internal medicine-pediatrics residents participated in the study. The workshop presented the Take the HEAT (Hear, Empathize, Apologize, Take action) strategy of communication. Communication skills were assessed through standardized patient encounters at baseline and post-workshop. Encounters were scored using a novel assessment tool., Results: After the workshop, residents' Take the HEAT communication improved from baseline total average score 23.15 to total average score 25.36 (Z=-3.428, p<0.001). At baseline, empathy skills were the lowest. Intraclass Correlation Coefficient demonstrated substantial agreement (0.60 and 0.61) among raters using the tool., Conclusion: First-year pediatric trainees' communication with angry families improved with education focused on the Take the HEAT strategy. Poor performance by residents in demonstrating empathy should be explored further., Practice Implications: This study demonstrates the utility of a brief communications curriculum aimed at improving pediatric residents' ability to communicate with angry families., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: Growth impairment remains common in children with chronic kidney disease (CKD). Available literature indicates low level of recombinant human growth hormone (rhGH) utilization in short children with CKD. Despite efforts at consensus guidelines, lack of high-level evidence continues to complicate rhGH therapy decision-making and the level of practice variability in rhGH treatment by pediatric nephrologists is unknown., Methods: Cross-sectional online survey electronically distributed to pediatric nephrologists through the Midwest Pediatric Nephrology Consortium and American Society of Pediatric Nephrology., Results: Seventy three pediatric nephrologists completed the survey. While the majority (52.1%) rarely involve endocrinology in rhGH management, 26.8% reported that endocrinology managed most aspects of rhGH treatment in their centers. The majority of centers (68.5%) have a dedicated renal dietitian, but 20.6% reported the nephrologist as the primary source of nutritional support for children with CKD. Children with growth failure did not receive rhGH most commonly because of family refusal. Differences in initial work-up for rhGH therapy include variable use of bone age (95%), thyroid function (58%), insulin-like growth factor-1 (40%), hip/knee X-ray (36%), and ophthalmologic evaluation (7%). Most pediatric nephrologists (95%) believe that rhGH treatment improves quality of life, but only 24% believe that it improves physical function; 44% indicated that rhGH improves lean body mass., Conclusions: There is substantial variation in pediatric nephrology practice in addressing short stature and rhGH utilization in children with CKD. Hence, there may be opportunities to standardize care to study and improve growth outcomes in short children with CKD.

Purpose: To describe clinical skills progression during pediatric residency using the distribution of pediatric milestone assessments by subcompetency and year of training and to determine reasonable milestone expectations at time of graduation., Method: Multi-institutional cohort study of the milestones reported to the Accreditation Council for Graduate Medical Education for all 21 pediatric subcompetencies. Most subcompetencies were measured using five milestone levels (1 = novice, 2 = advanced beginner, 3 = competent, 4 = proficient, 5 = master); 3 subcompetencies had only four levels defined., Results: Milestone assessments for 2,030 pediatric residents in 47 programs during academic year 2013-2014 were obtained. There was significant variation in end-of-year milestone ratings for residents within each level of training, which decreased as training level increased. Most (78.9%; 434/550) graduating third-year pediatric residents received a milestone rating of ≥ 3 in all 21 subcompetencies; fewer (21.1%; 116/550) received a rating of ≥ 4 in all subcompetencies. Across all training levels, professionalism and interpersonal communication skills were rated highest; quality improvement was rated lowest., Conclusions: Trainees entered residency with a wide range of skills. As they advanced, skill variability within a training level decreased. Most graduating pediatric residents were still advancing on the milestone continuum toward proficiency and mastery, and an expectation of milestone ratings ≥ 4 in all categories upon graduation is unrealistic; milestone ratings ≥ 3 upon graduation may be more realistic. Understanding current pediatric residents' and graduates' skills can help to identify key areas that should be specifically targeted during training.

The aim of this study was to evaluate whether the current state of child neurology education during pediatric residency provides adequate preparation for pediatric practice. A survey was sent to recent graduates from 3 pediatric residency programs to assess graduate experience, perceived level of competence, and desire for further education in child neurology. Responses from generalists versus subspecialists were compared. The response rate was 32%, half in general pediatric practice. Only 22% feel very confident in approaching patients with neurologic problems. This may represent the best-case scenario as graduates from these programs had required neurology experiences, whereas review of Accreditation Council of Graduate Medical Education-accredited residency curricula revealed that the majority of residencies do not. Pediatric neurologic problems are common, and pediatric residency graduates do encounter such problems in practice. The majority of pediatricians report some degree of confidence; however, some clear areas for improvement are apparent.

Objective: To evaluate the preparedness of pediatric residents entering accredited neonatal-perinatal medicine (NPM) fellowships in the United States., Study Design: A multi-domain, validated survey was distributed to Program Directors (PDs) of US NPM fellowship programs. The 47-item survey explored 5 domains: professionalism, independent practice, psychomotor ability, clinical evaluation, and academia. A systematic, qualitative analysis on free-text comments was also performed., Results: Sixty-one PDs completed the survey, for a response rate of 62% (61/98). For entering fellows, PDs assessed performance in professionalism positively, including 76% as communicating effectively with parents and 90% treating residents/house-staff with respect. In contrast, most PDs rated performance in psychomotor abilities negatively, including 59% and 79% as deficient in bag-and-mask ventilation and neonatal endotracheal intubation, respectively. Although 62% of PDs assessed entering fellows positively for genuine interest in academic projects, fewer than 10% responded positively that entering fellows understood research protocol design, basic statistics, or were capable of writing a cohesive manuscript well. Thematic clustering of qualitative data revealed deficits in psychomotor ability and academia/scholarship., Conclusions: On the basis of the perspective of front line educators, graduating pediatric residents are underprepared for subspecialty fellowship training in NPM. To provide the best preparation for pediatric graduates who pursue advanced training, changes to residency education to address deficiencies in these important competencies are warranted.

The host and bacterial factors that lead to development of pneumococcal haemolytic uraemic syndrome (pHUS) remain poorly defined; however, it is widely believed that pneumococcal exposure of the Thomsen-Friedenreich antigen (T-antigen) on host surfaces is a key step in pathogenesis. Two enzymatic activities encoded by pneumococci determine the level of T-antigen exposed. Neuraminidases cleave terminal sialic acid to expose the T-antigen which is subsequently cleaved by O-glycosidase Eng. While a handful of studies have examined the role of neuraminidases in T-antigen exposure, no studies have addressed the potential role of O-glycosidase. This study used 29 pHUS isolates from the USA and 31 serotype-matched controls. All isolates contained eng, and no significant correlation between enzymatic activity and disease state (pHUS and blood non-pHUS isolates) was observed. A prior study from Taiwan suggested that neuraminidase NanC contributes to the development of pHUS. However, we observed no difference in nanC distribution. Similar to previously published data, we found no significant correlation between neuraminidase activity and disease state. Accurate quantification of these enzymatic activities from bacteria grown in whole blood is currently impossible, but we confirmed that there were no significant correlations between disease state and neuraminidase and O-glycosidase transcript levels after incubation in blood. Genomic sequencing of six pHUS isolates did not identify any genetic elements possibly contributing to haemolytic uraemic syndrome. These findings support the hypothesis that while exposure of T-antigen may be an important step in disease pathogenesis, host factors likely play a substantial role in determining which individuals develop haemolytic uraemic syndrome after pneumococcal invasive disease.

Purpose: Medical education aims to equip physicians for lifelong learning, an objective supported by the conceptual framework of self-regulated learning (SRL). Learning goals have been used to develop SRL skills in learners across the medical education continuum. This study's purpose was to elicit residents' perspectives on learning goal use and to develop explanations suggesting how aspects of the learning environment may facilitate or hinder the meaningful use of learning goals in residency., Method: Resident focus groups and program director interviews were conducted in 2012-2013, audio-recorded, and transcribed. Programs were selected to maximize diversity of size, geographic location, type of program, and current use of learning goals. Data were analyzed using the constant comparative method associated with grounded theory. Further analysis compared themes frequently occurring together to strengthen the understanding of relationships between the themes. Through iterative discussions, investigators built a grounded theory., Results: Ninety-five third-year residents and 12 program directors at 12 pediatric residency programs participated. The analysis identified 21 subthemes grouped into 5 themes: program support, faculty roles, goal characteristics and purposes, resident attributes, and accountability and goal follow-through. Review of relationships between the themes revealed a pyramid of support with program support as the foundation that facilitates the layers above it, leading to goal follow-through., Conclusions: Program support facilitates each step of the SRL process that leads to meaningful use of learning goals in residency. A strong foundation of program support should include attention to aspects of the implicit curriculum as well as the explicit curriculum.

Background: The role of biochemical bone turnover markers (BTMs) in assessing low bone mass and monitoring bisphosphonate treatment in pediatric patients with clinical bone fragility is not well established. The aim of the study was to examine the correlations of BTMs and the bone mineral density (BMD), and evaluate the effects of bisphosphonates therapy on BTMs in children with clinical bone fragility., Methods: Clinical data of 115 patients with clinical bone fragility (mean age 9.7±5.8 years), 102 of whom received bisphosphonates, were studied. Serum alkaline phosphatase (ALP), osteocalcin (OC), urine pyridinoline (PD) and deoxypyridinoline (DPD), BMD at baseline and subsequent years were analyzed., Results: There was a significant negative correlation between urine PD and lumbar BMD (slope=-0.29, p<0.001). There were no correlations between BTMs and lumbar BMD Z-score. There was a significant positive correlation between serum OC and serum ALP, urine PD and DPD (p<0.001). Serum OC, urine PD and DPD index, as expressed as measured value/upper limit of normal value for age, decreased during the first 3 years of bisphosphonate therapy., Conclusions: In children with clinical bone fragility, BTMs correlated with each other, but not with lumbar BMD Z-score. While they were not reliable predictors of degree of low BMD, the bone markers showed suppression during bisphosphonate therapy and may be helpful in monitoring the response to therapy.

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.