<p><strong>Background</strong> Despite excellent operative survival, correction of tetralogy of Fallot frequently is accompanied by residual lesions that may affect health beyond the incident hospitalization. Measuring resource utilization, specifically cost and length of stay, provides an integrated measure of morbidity not appreciable in traditional outcomes.</p>

<p><strong>Methods and Results</strong> We conducted a retrospective cohort study, using de-identified commercial insurance claims data, of 269 children who underwent operative correction of tetralogy of Fallot from January 2004 to September 2015 with ≥2&nbsp;years of continuous follow-up (1) to describe resource utilization for the incident hospitalization and subsequent 2&nbsp;years, (2) to determine whether prolonged length of stay (&gt;7&nbsp;days) in the incident hospitalization was associated with increased subsequent resource utilization, and (3) to explore whether there was regional variation in resource utilization with both direct comparisons and multivariable models adjusting for known covariates. Subjects with prolonged incident hospitalization length of stay demonstrated greater resource utilization (total cost as well as counts of outpatient visits, hospitalizations, and catheterizations) after hospital discharge (&lt;0.0001 for each), though the number of subsequent operative and transcatheter interventions were not significantly different. Regional differences were observed in the cost of incident hospitalization as well as subsequent hospitalizations, outpatient visits, and the costs associated with each.</p>

<p><strong>Conclusions</strong> This study is the first to report short- and medium-term resource utilization following tetralogy of Fallot operative correction. It also demonstrates that prolonged length of stay in the initial hospitalization is associated with increased subsequent resource utilization. This should motivate research to determine whether these differences are because of modifiable factors.</p>

<p><strong>BACKGROUND: </strong>Loss of the normal nocturnal decline in blood pressure (BP), known as non-dipping, is a potential measure of cardiovascular risk identified by ambulatory blood pressure monitoring (ABPM). We sought to determine whether non-dipping is a useful marker of abnormal vascular function and subclinical atherosclerosis in pediatric-onset systemic lupus erythematosus (pSLE).</p>

<p><strong>METHODS: </strong>Twenty subjects 9-19 years of age with pSLE underwent ABPM, peripheral endothelial function testing, carotid-femoral pulse wave velocity/analysis for aortic stiffness, and carotid intima-media thickness. We assessed the prevalence of non-dipping and other ABPM abnormalities. Pearson or Spearman rank correlation tests were used to evaluate relationships between nocturnal BP dipping, BP load (% of abnormally elevated BPs over 24-h), and vascular outcome measures.</p>

<p><strong>RESULTS: </strong>The majority (75%) of subjects had inactive disease, with mean disease duration of 3.2 years (± 2.1). The prevalence of non-dipping was 50%, which occurred even in the absence of nocturnal or daytime hypertension. Reduced diastolic BP dipping was associated with poorer endothelial function (r 0.5, p = 0.04). Intima-media thickness was significantly greater in subjects with non-dipping (mean standard deviation score of 3.0 vs 1.6, p = 0.02). In contrast, higher systolic and diastolic BP load were associated with increased aortic stiffness (ρ 0.6, p = 0.01 and ρ 0.7, p &lt; 0.01, respectively), but not with endothelial function or intima-media thickness.</p>

<p><strong>CONCLUSION: </strong>In a pSLE cohort with low disease activity, isolated nocturnal BP non-dipping is prevalent and associated with endothelial dysfunction and atherosclerotic changes. In addition to hypertension assessment, ABPM has a promising role in risk stratification and understanding heterogeneous mechanisms of cardiovascular disease in pSLE.</p>

<p>We present a series of six critically ill children with Multisystem Inflammatory Syndrome in Children (MIS-C). Key findings of this syndrome include fever, diarrhea, shock, and variable presence of rash, conjunctivitis, extremity edema, and mucous membrane changes.</p>

<p><strong>OBJECTIVES: </strong>The increased relative risk of heart failure (HF) from systemic lupus erythematosus (SLE) is greatest at younger ages, but the etiology remains unclear. We identified risk factors for HF in children and adults with SLE and evaluated associations between SLE manifestations and HF.</p>

<p><strong>METHODS: </strong>Incident SLE cases without preceding HF were identified using Clinformatics DataMart® (OptumInsight, Eden Prairie, MN) US claims data (2000-2015), and categorized by age of SLE onset (children 5-17, young adults 18-24, adults 25-44 years old). The primary outcome was the first HF ICD-9-CM diagnosis code (428.x), categorized as early-onset (&lt; 6 months) or delayed-onset. Multivariable logistic regression was used to identify factors associated with early or delayed-onset HF. Cox proportional hazards regression was used to identify time-dependent associations between the onset of SLE manifestations and incident HF.</p>

<p><strong>RESULTS: </strong>There were 523 (2.3%) HF cases among 1,466 children, 2,163 young adults and 19,349 adults age 25-44 with SLE. HF in children and young adults was early-onset in 50% and 60% of cases, respectively, compared to 35% of cases in adults 25-44 years old. There was a temporal association between incident myopericarditis and valvular disease diagnoses and early-onset HF, whereas nephritis and hypertension were more strongly associated with delayed-onset HF. Black race remained independently associated with a 1.5-fold increased HF risk at any time.</p>

<p><strong>CONCLUSION: </strong>Hypertension remains an important traditional CV risk factor across all ages and should be managed aggressively even in younger patients with SLE. Cardiac dysfunction due to acute cardiac manifestations of SLE may contribute to the very high relative incidence of early HF diagnoses among younger SLE patients. Therefore, future prospective studies will need to address heterogeneity in the types and severity of heart failure in order to determine etiology and which patients should be monitored.</p>

<p><strong>OBJECTIVE: </strong>Youth with systemic lupus erythematosus (SLE) transferring from pediatric to adult care are at risk for poor outcomes. We describe patterns of rheumatology/nephrology care and changes in health care utilization and medication adherence during transfer.</p>

<p><strong>METHODS: </strong>We identified youth ages 15-25 with SLE using US private insurance claims from Optum's de-identified Clinformatics® Data Mart. Rheumatology/nephrology visit patterns were categorized as 1) unilateral transfers to adult care within 12 months, 2) overlapping pediatric and adult visits, 3) lost to follow-up, or 4) continuing pediatric care. We used negative binomial regression and paired t-tests to estimate changes in health care utilization and medication possession ratios (MPR) after the last pediatric (index) visit. We compared MPRs between youth who transferred and age-matched peers continuing pediatric care.</p>

<p><strong>RESULTS: </strong>184 youth transferred out of pediatric care, of which 41.8% transferred unilaterally, 31.5% had overlapping visits over a median of 12 months before final transfer, and 26.6% were lost to follow-up. We matched 107 youth continuing pediatric care. Overall ambulatory utilization decreased among those lost to follow-up. Acute care utilization decreased across all groups. MPRs after the index date were lower in youth lost to follow-up (mean 0.24) compared to peers in pediatric care (0.57, p&lt;0.001).</p>

<p><strong>CONCLUSION: </strong>Youth with SLE with continuous private insurance coverage do not use more acute care after transfer to adult care. However, a substantial proportion fail to see adult subspecialists within 12 months and have worse medication adherence, placing them at higher risk for adverse outcomes.</p>

<p>The success of rare disease research relies heavily on robust partnerships with clinicians to help identify new patients and collect samples. Many studies for paediatric rheumatic diseases requiring pretreatment samples have suffered from slow enrolment rates due to the low incidence of disease and relative urgency to treat. Therefore, timely identification of all potentially eligible patients is crucial. The objective of this project was to apply quality improvement methods to increase the frequency and timeliness of identification of eligible patients with new paediatric rheumatic diagnoses to approach for research studies. A retrospective chart review was undertaken in our paediatric rheumatology clinic to measure the number of eligible patients identified for potential research recruitment between missed recruitment opportunities. Improvement methodology was used to integrate standardised communication between clinicians and the research team into clinic workflow, to leverage social feedback as positive reinforcement for good communication and to measure change in response to the interventions. The number of eligible patients identified between missed recruitment opportunities increased from every 0-1 patient to every 14 patients during the project period, corresponding to an increase in the overall identification rate from 32% to 91% of all eligible patients. Quality improvement methods can be used to successfully integrate research recruitment into routine clinical care and accelerate advances necessary to improve health outcomes.</p>

<p>The timing and etiology of diastolic impairment in pediatric-onset systemic lupus erythematosus (SLE) are poorly understood. We compared echocardiographic metrics of left ventricular diastolic function in children at SLE diagnosis to controls and identified factors associated with diastolic indices. Echocardiograms of children aged 5-18&nbsp;years within 1&nbsp;year of SLE diagnosis and age-/sex-matched controls were retrospectively read by blinded cardiologists. Clinical characteristics were abstracted separately. Z-scores for diastolic indices (E/A, e', E/e', and isovolumetric relaxation time (IVRT)) were calculated using published normative data and study controls, and compared using linear mixed-effects models adjusted for blood pressure. Pericardial effusions and valvular disease were also evaluated. Linear regression was used to identify factors associated with diastolic measures. 85 children with incident SLE had echocardiograms performed a median of 6&nbsp;days after diagnosis (interquartile range (IQR) 1-70). Prior cumulative prednisone exposure was minimal (median 60&nbsp;mg, IQR 0-1652). SLE cases had lower E/A, lower e', higher E/e', and longer IVRT compared to controls. Though none met criteria for Grade I diastolic dysfunction, Z-scores for e', E/e', and IVRT were abnormal in 30%, 25%, and 6% of SLE cases, respectively. Greater disease activity was associated with lower septal e' (p &lt; 0.01), higher E/e' (p = 0.02), and longer IVRT (p &lt; 0.01). Children with incident SLE have worse diastolic indices at diagnosis compared to peers without SLE, independent of blood pressure and prior to significant prednisone exposure. Longitudinal studies will determine whether diastolic dysfunction develops in this population over time.</p>

<p><strong>OBJECTIVE: </strong>Childhood-onset systemic lupus erythematosus (SLE) is associated with high risk for organ damage, which may be mitigated by early diagnosis and treatment. We characterized health care utilization for youth in the year preceding SLE diagnosis compared to controls.</p>

<p><strong>METHODS: </strong>Using Clinformatics™ DataMart (OptumInsight, Eden Prairie, MN) de-identified administrative data from 2000 to 2013, we identified 682 youth ages 10-24 years with new-onset SLE (≥3 International Classification of Diseases, Ninth Revision (ICD-9) codes for SLE 710.0, each &gt;30 days apart), and 1,364 age and sex-matched healthy controls. We compared the incidence of ambulatory, emergency, and inpatient visits 12 months before SLE diagnosis, and frequency of primary diagnoses. We examined subject characteristics associated with utilization preceding SLE diagnosis.</p>

<p><strong>RESULTS: </strong>Youth with SLE had significantly more visits in the year preceding diagnosis than controls across ambulatory (incidence rate ratio (IRR) 2.48, p&lt;0.001), emergency (IRR 3.42, p&lt;0.001) and inpatient settings (IRR 3.02, p&lt;0.001). The most frequent acute care diagnoses and median days to SLE diagnosis were: venous thromboembolism (313, interquartile range (IQR) 18-356), thrombocytopenia (278, IQR 39-354), chest pain (73, IQR 29.5-168), fever (52, IQR 17-166), and acute kidney failure (14, IQR 5-168). Having a psychiatric diagnosis prior to SLE diagnosis was strongly associated with increased utilization across all settings.</p>

<p><strong>CONCLUSION: </strong>Youth with SLE have high health care utilization throughout the year preceding SLE diagnosis. Examining variable diagnostic trajectories of youth presenting for acute care preceding SLE diagnosis, and increased attention to psychiatric morbidity may help improve care for youth with new-onset SLE. This article is protected by copyright. All rights reserved.</p>

<p><strong>BACKGROUND: </strong>Inner-city, minority children with asthma have the highest rates of morbidity and death from asthma and the lowest rates of asthma controller medication adherence. Some recent electronic medication monitoring interventions demonstrated dramatic improvements in adherence in lower-risk populations. The feasibility and acceptability of such an intervention in the highest-risk children with asthma has not been studied.</p>

<p><strong>OBJECTIVE: </strong>Our objective was to assess the feasibility and acceptability of a community health worker-delivered electronic adherence monitoring intervention among the highest utilizers of acute asthma care in an inner-city practice.</p>

<p><strong>METHODS: </strong>This was a prospective cohort pilot study targeting children with the highest frequency of asthma-related emergency department and hospital care within a local managed care Medicaid plan. The 3-month intervention included motivational interviewing, electronic monitoring of controller and rescue inhaler use, and outreach by a community health worker for predefined medication alerts. We measured acceptability by using a modified technology acceptability model and changes in asthma control using the Asthma Control Test (ACT). Given prominent feasibility issues, we describe qualitative patterns of medication use at baseline only.</p>

<p><strong>RESULTS: </strong>We enrolled 14 non-Hispanic black children with a median age of 3.5 years. Participants averaged 7.8 emergency or hospital visits in the year preceding enrollment. We observed three distinct patterns of baseline controller use: 4 patients demonstrated sustained use, 5 patients had periodic use, and 5 patients lapsed within 2 weeks. All participants initiated use of the electronic devices; however, no modem signal was transmitted for 5 or the 14 participants after a mean of 45 days. Of the 9 (64% of total) caregivers who completed the final study visit, all viewed the electronic monitoring device favorably and would recommend it to friends, and 5 (56%) believed that the device helped to improve asthma control. ACT scores improved by a mean of 2.7 points (P=.05) over the 3-month intervention.</p>

<p><strong>CONCLUSIONS: </strong>High-utilizer, minority families who completed a community health worker-delivered electronic adherence intervention found it generally acceptable. Prominent feasibility concerns, however, such as recruitment, data transmission failure, and lost devices, should be carefully considered when designing interventions in this setting.</p>