Patient and Parent Characteristics Related to Quality of Life and Self-esteem in Healthy Youth Undergoing Provocative Growth Hormone Testing.

2023

113460

05/2023

1097-6833

OBJECTIVE: To examine how height and youth as well as parenting characteristics associate with quality of life (QoL) and self-esteem among healthy youth undergoing growth evaluation with growth hormone (GH) testing .

STUDY DESIGN: Healthy youth, age 8-14 years, undergoing provocative GH testing, and a parent completed surveys at or around the time of testing. Surveys collected demographic data; youth and parent reports of youth health-related QoL; youth reports of self-esteem, coping skills, social support, and parental autonomy support; and parent reports of perceived environmental threats and achievement goals for their child. Clinical data were extracted from electronic health records. Univariate models and multivariable linear regressions were used to identify factors associated with QoL and self-esteem.

RESULTS: Sixty youth (mean height Z-score -2.18 ± 0.61) and their parents participated. On multivariable modeling, youth perceptions of their physical QoL associated with higher grade in school, greater friend and classmate support, and older parent age; youth psychosocial QoL with greater friend and classmate support, and with less disengaged coping; and youth height-related QoL and parental perceptions of youth psychosocial QoL with greater classmate support. Youth self-esteem associated with greater classmate support and taller mid-parental height. Youth height was not associated with QoL or self-esteem outcomes in multivariable regression.

CONCLUSIONS: Perceived social support and coping skills, rather than height, were related to QoL and self-esteem in healthy short youth and may serve as an important potential area for clinical intervention.

10.1016/j.jpeds.2023.113460

J Pediatr

37172805

Opioid use in children with inflammatory bowel disease-related arthritis.

2023

04/2023

0392-856X

OBJECTIVES: Concomitant arthritis may increase risk of chronic opioid use in youngsters with IBD. We aimed to assess trends and clinical features associated with opioid use in children with IBD-related arthritis.

METHODS: Adolescents under 18 years of age with IBD-related arthritis, at least 1 year of continuous enrolment, and at least 1 pharmacy claim in the Truven Health MarketScan Claims and Encounter Database were included. Subjects were identified using previously validated algorithms consisting of ICD codes, pharmacy claims and procedure codes. The primary outcome was chronic opioid exposure. Temporal trends in opioid exposure were tested using the Cuzick-Wilcoxon test. The association of chronic opioid use and baseline covariates in the IBD and IBD-arthritis cohorts were examined using multivariable logistic regression models.

RESULTS: 14,943 adolescents with IBD, 480 of whom had arthritis, were included. Chronic opioid use was non-trivial in youngsters with IBD-related arthritis, higher than that of total IBD cohort (12.3% vs. 5%) and remained stable over the years of study. Using multivariable regression, joint pain and arthritis were significantly associated with chronic opioid exposure in young people with IBD. Among IBD-related arthritis patients older age, public insurance, gastrointestinal surgery, hospitalisation and psychiatric comorbidities were significantly associated with chronic opioid use.

CONCLUSIONS: Chronic opioid use in adolescents with IBD-related arthritis was higher than that of total IBD cohort but stable over the years of study. Future study is needed to explore ways to optimise non-narcotic pain management strategies and ensuring appropriate use of opioids when necessary.

10.55563/clinexprheumatol/3bu1sf

Clin Exp Rheumatol

37083174

Asthma medication adherence during the coronavirus disease 2019 pandemic in children at high risk of exacerbation.

2023

04/2023

1534-4436

10.1016/j.anai.2023.04.007

Ann Allergy Asthma Immunol

37080458

Nighttime sleep duration and variability in children with obstructive sleep apnea syndrome: Sociodemographic disparities and neurobehavioral outcomes.

2023

165-172

02/2023

1878-5506

OBJECTIVES: This study examined differences in sleep patterns by race, ethnicity, and socioeconomic status (SES) among children with Obstructive Sleep Apnea Syndrome (OSAS), and linkages between sleep patterns and neurobehavioral functioning.

METHOD: We used baseline data from the Childhood Adenotonsillectomy Study (CHAT), a multicenter, single-blind, randomized controlled trial designed to evaluate the efficacy of early adenotonsillectomy versus watchful waiting with supportive care for children with OSAS. Participants included children with OSAS (ages 5.0-9.9 years). SES indicators were obtained via questionnaire and geocoding (ArcGIS version 10.1). Caregivers and teachers reported on child inattention/impulsivity and executive functioning. Nighttime sleep duration and variability were measured using five-night sleep diaries.

RESULTS: Black children experienced shorter nighttime sleep duration than White children, by about 25 min, as well as greater sleep duration variability, while sleep duration was more variable in children of "other" racial and ethnic backgrounds versus White children. Of the socioeconomic correlates, only lower family income was associated with sleep duration variability. A short and more variable nighttime sleep duration were each associated with caregiver-rated child inattention and impulsivity. Greater sleep duration variability was linked to greater teacher-rated, but not caregiver-rated, executive functioning impairments.

CONCLUSIONS: Compared to White children with OSAS, Black children with OSAS experience a shorter and more variable nighttime sleep duration. Having a short and/or variable sleep duration may increase risk for neurobehavioral impairments in youth with OSAS, underscoring the potential benefits of sleep health promotion in the context of OSAS care.

10.1016/j.sleep.2023.01.003

Sleep Med

36682144

Biologic Abatement and Capturing Kids' Outcomes and Flare Frequency in Juvenile Spondyloarthritis (BACK-OFF JSpA): study protocol for a randomized pragmatic trial.

2023

100

02/2023

1745-6215

BACKGROUND: The effectiveness of biologic therapies, primarily tumor necrosis factor inhibitors (TNFi), for children with spondyloarthritis (SpA) has made inactive disease a realistic patient outcome. However, biologic therapies are costly, primarily delivered by subcutaneous or intravenous route, and have non-trivial side effects. Many patients and families want to know if biologic medications can be discontinued after inactive disease is achieved. It remains unclear whether medication dose should remain unchanged, tapered (increase the time between doses), or discontinued once when inactive disease is attained.

METHODS: The Biologic Abatement and Capturing Kids' Outcomes and Flare Frequency in Juvenile SpA (BACK-OFF JSpA) trial is a multicenter pragmatic trial that will randomize 198 participants ages 8-21 years old with SpA and sustained inactive disease on standard TNFi dosing to (1) continue standard TNFi dosing, (2) fixed longer dosing intervals of TNFi, or (3) stop TNFi. The trial will compare the hazard rate of protocol-defined flare and participants' emotional health among the 3 groups over 12 months. Innovative aspects of this trial are the involvement of patient and parent stakeholders in the design and conduct of the study as well as an electronic health record-based enhanced recruitment strategy.

DISCUSSION: This is the first randomized pragmatic trial to assess the efficacy of TNFi de-escalation strategies in children with JSpA with sustained inactive disease. This research will improve the evidence base that patients, caregivers, and rheumatologists use to make shared decisions about continued treatment versus de-escalation of TNFi therapy in this population.

TRIAL REGISTRATION: ClinicalTrials.gov NCT04891640. Registered on 18 May 2021.

10.1186/s13063-022-07038-6

Trials

36755328

Sustainable deimplementation of continuous pulse oximetry monitoring in children hospitalized with bronchiolitis: study protocol for the Eliminating Monitor Overuse (EMO) type III effectiveness-deimplementation cluster-randomized trial.

2022

72

10/2022

1748-5908

BACKGROUND: Methods of sustaining the deimplementation of overused medical practices (i.e., practices not supported by evidence) are understudied. In pediatric hospital medicine, continuous pulse oximetry monitoring of children with the common viral respiratory illness bronchiolitis is recommended only under specific circumstances. Three national guidelines discourage its use for children who are not receiving supplemental oxygen, but guideline-discordant practice (i.e., overuse) remains prevalent. A 6-hospital pilot of educational outreach with audit and feedback resulted in immediate reductions in overuse; however, the best strategies to optimize sustainment of deimplementation success are unknown.

METHODS: The Eliminating Monitor Overuse (EMO) trial will compare two deimplementation strategies in a hybrid type III effectiveness-deimplementation trial. This longitudinal cluster-randomized design will be conducted in Pediatric Research in Inpatient Settings (PRIS) Network hospitals and will include baseline measurement, active deimplementation, and sustainment phases. After a baseline measurement period, 16-19 hospitals will be randomized to a deimplementation strategy that targets unlearning (educational outreach with audit and feedback), and the other 16-19 will be randomized to a strategy that targets unlearning and substitution (adding an EHR-integrated clinical pathway decision support tool). The primary outcome is the sustainment of deimplementation in bronchiolitis patients who are not receiving any supplemental oxygen, analyzed as a longitudinal difference-in-differences comparison of overuse rates across study arms. Secondary outcomes include equity of deimplementation and the fidelity to, and cost of, each deimplementation strategy. To understand how the deimplementation strategies work, we will test hypothesized mechanisms of routinization (clinicians developing new routines supporting practice change) and institutionalization (embedding of practice change into existing organizational systems).

DISCUSSION: The EMO trial will advance the science of deimplementation by providing new insights into the processes, mechanisms, costs, and likelihood of sustained practice change using rigorously designed deimplementation strategies. The trial will also advance care for a high-incidence, costly pediatric lung disease.

TRIAL REGISTRATION: ClinicalTrials.gov, NCT05132322 . Registered on November 10, 2021.

10.1186/s13012-022-01246-z

Implement Sci

36271399

Use of renin angiotensin aldosterone system inhibitors in children with lupus and time to glucocorticoid discontinuation.

2022

395-404

05/2022

1523-1755

There is little data to inform use of renin angiotensin aldosterone system (RAAS) inhibitors in pediatric patients with systemic lupus erythematosus (SLE). Here, we sought to characterize RAAS inhibitor use in pediatric SLE and determine whether early RAAS inhibitor initiation among children with incident lupus nephritis is associated with decreased duration of chronic glucocorticoid exposure. A retrospective cohort study was performed of children (ages 5-18) with SLE and/or lupus nephritis in the Truven MarketScan™ Medicaid and Commercial databases (2013-2018) and estimated RAAS inhibitor use. Among incident nephritis cases, we used competing risk hazard models with inverse probability of treatment weighting to estimate the association between RAAS inhibitor initiation less than 180 days after diagnosis and time to glucocorticoid discontinuation with kidney failure as a competing event. Among 592 children with nephritis and 1407 children with non-kidney SLE, 67% and 15% ever received RAAS inhibitors, respectively. Median duration of RAAS inhibitor use among 323 incident users was 14 and 9 months in children with and without nephritis, respectively. Medicaid enrollment was independently associated with greater likelihood of RAAS inhibitor use, irrespective of nephritis. Among 158 incident nephritis cases, early RAAS inhibitor initiation was significantly associated with a faster rate of glucocorticoid discontinuation (adjusted sub-distribution hazard ratio 1.81, 95% confidence interval [1.09 - 3.00]). Thus, early initiation of RAAS inhibitors may have a role in children newly diagnosed with lupus nephritis; not only those with refractory proteinuria after induction therapy. Hence, integrated health systems data could be leveraged to confirm these findings and optimize adjunctive therapies in pediatric lupus.

10.1016/j.kint.2022.04.023

Kidney Int

35618096

The current state of adult metabolic medicine in the United States: Results of a nationwide survey.

2022

1722-1731

08/2022

1530-0366

PURPOSE: Patients with inherited metabolic disorders (IMDs) now have improved health outcomes and increased survival into adulthood. There is scant evidence on managing adults with IMDs. We present an analysis of current care practices for adults with IMDs in the United States.

METHODS: We created and distributed an online survey to US members of the Society of Inherited Metabolic Disorders. The survey addressed ambulatory care, acute management, and health care transition (HCT) practices of adults with IMDs.

RESULTS: The survey was completed by 91 providers from 73 institutions. Most adult patients with IMDs receive lifelong care from a single metabolic clinician, predominantly in pediatric clinic settings. Adults receive comprehensive ambulatory metabolic care, but fewer trainees participate compared with pediatric visits. Most acute IMD management occurs in pediatric hospitals. Clinician comfort with HCT increased the frequency of HCT planning. Overall, all respondents felt that providing specialized care to adults with IMDs is high value.

CONCLUSION: Our survey demonstrates the paucity of clinical resources dedicated to adult metabolic medicine. Care is fragmented and varies by medical system. Interest in HCT is robust but would benefit from standardized practices. Our findings reinforce the need for greater focus on adult metabolic medicine in the United States.

10.1016/j.gim.2022.04.018

Genet Med

35543711

Characteristics of Neonates with Cardiopulmonary Disease Who Experience Seizures: A Multicenter Study.

2022

63-73

2022 03

1097-6833

<p><strong>OBJECTIVE: </strong>To compare key seizure and outcome characteristics between neonates with and without cardiopulmonary disease.</p>

<p><strong>STUDY DESIGN: </strong>The Neonatal Seizure Registry is a multicenter, prospectively acquired cohort of neonates with clinical or electroencephalographic (EEG)-confirmed seizures. Cardiopulmonary disease was defined as congenital heart disease, congenital diaphragmatic hernia, and exposure to extracorporeal membrane oxygenation. We assessed continuous EEG monitoring strategy, seizure characteristics, seizure management, and outcomes for neonates with and without cardiopulmonary disease.</p>

<p><strong>RESULTS: </strong>We evaluated 83 neonates with cardiopulmonary disease and 271 neonates without cardiopulmonary disease. Neonates with cardiopulmonary disease were more likely to have EEG-only seizures (40% vs 21%, P&nbsp;&lt;&nbsp;.001) and experience their first seizure later than those without cardiopulmonary disease (174 vs 21&nbsp;hours of age, P&nbsp;&lt;&nbsp;.001), but they had similar seizure exposure (many-recurrent electrographic seizures 39% vs 43%, P&nbsp;=&nbsp;.27). Phenobarbital was the primary initial antiseizure medication for both groups (90%), and both groups had similarly high rates of incomplete response to initial antiseizure medication administration (66% vs 68%, P&nbsp;=&nbsp;.75). Neonates with cardiopulmonary disease were discharged from the hospital later (hazard ratio 0.34, 95% CI 0.25-0.45, P&nbsp;&lt;&nbsp;.001), although rates of in-hospital mortality were similar between the groups (hazard ratio 1.13, 95% CI 0.66-1.94, P&nbsp;=&nbsp;.64).</p>

<p><strong>CONCLUSION: </strong>Neonates with and without cardiopulmonary disease had a similarly high seizure exposure, but neonates with cardiopulmonary disease were more likely to experience EEG-only seizures and had seizure onset later in the clinical course. Phenobarbital was the most common seizure treatment, but seizures were often refractory to initial antiseizure medication. These data support guidelines recommending continuous EEG in neonates with cardiopulmonary disease and indicate a need for optimized therapeutic strategies.</p>

10.1016/j.jpeds.2021.10.058

J Pediatr

34728234

Incidence Rates of Psoriasis in Children with Inflammatory Bowel Disease and Juvenile Arthritis Treated with TNFi Agents and DMARDs.

2022

2022 Apr 15

0315-162X

<p><strong>OBJECTIVE: </strong>To estimate the differential effect of tumor necrosis factor inhibitor (TNFi) therapies and presence/absence of conventional synthetic disease-modifying antirheumatic drug (DMARD) on the incidence of psoriasis in children with inflammatory bowel disease (IBD), juvenile idiopathic arthritis (JIA), and chronic nonbacterial osteomyelitis (CNO).</p>

<p><strong>METHODS: </strong>This was a retrospective cohort study from 2008 to 2020. TNFi and DMARD exposures were dichotomized as ever/never. The primary outcome was incident psoriasis. Incidence rates (IRs) of psoriasis were stratified by underlying diagnosis, TNFi agent, and DMARD use. Poisson regression was used to assess the IR ratios (IRR) between exposure groups.</p>

<p><strong>RESULTS: </strong>5088 children met inclusion criteria - 3794 (75%) had IBD, 1189 (23%) had JIA, and 105 (2%) had CNO. 613 (30%) and 1410 (70%) had TNFi exposure with or without a DMARD, respectively. IRR of developing psoriasis in patients exposed to adalimumab was 2.70 times higher (95% CI: 1.52-4.75; p&lt;0.001) than those who did not receive any TNFi treatment, when controlling for DMARD, sex, and family history of psoriasis. IRR was lower, but not significantly different, for patients exposed to infliximab (IRR=2.34; 95% CI: 1.56-3.51; p&lt;0.001) and etanercept (IRR=2.22; 95% CI: 1.17-4.21; p=0.006) compared to TNFi unexposed patients. IRR of TNFi exposure was lower by 0.25 (p&lt;0.001) in DMARD exposed patients compared to non- DMARD exposed patients.</p>

<p><strong>CONCLUSION: </strong>IRR of TNFi-induced psoriasis was not significantly different amongst adalimumab, infliximab, and etanercept. However, for patients with exposure to any of the TNFi evaluated, the IRR was significantly lower in those also exposed to a DMARD.</p>

10.3899/jrheum.211359

J Rheumatol

35428721