QIing your QI: a 13-year experience of a paediatric residency QI programme.

Introduction: QI education is essential for resident physicians with established requirements from the Accreditation Council for Graduate Medical Education outlining the necessary components. Literature supports the inclusion of both didactic and experiential learning, however, most studies review knowledge and attitude based assessments of residency QI programs. In 2012, our pediatric residency program identified a gap in resident engagement in QI, which led to the formalization of a QI education program grounded in the Institute for Healthcare Improvement (IHI) Model for improvement with objective measures of QI projects.

Disease activity at two consecutive registry visits and subsequent medication escalation for patients with juvenile idiopathic arthritis in the CARRA registry.

Objective: To account for the chronic time course of juvenile idiopathic arthritis (JIA), we assessed medication changes by disease activity patterns across 2 sequential timepoints.

Methods: Patients with non-systemic JIA enrolled in the Childhood Arthritis and Rheumatology Research Alliance Registry with complete clinical Juvenile Arthritis Disease Activity Scores (cJADAS) at 6 and 12-month registry visits were included. Disease activity was classified by cJADAS categories (inactive/minimal, moderate/high).

Multicenter Study of Associations Between Area-Level Child Opportunity, Initial Disease Severity, and Outcomes Among Children with Lupus.

Objective: Child opportunity encompasses neighborhood resources and conditions that influence healthy childhood development. We determined whether area-level opportunity is associated with disease severity or disease control in a geographically and socioeconomically diverse multicenter cohort of patients with childhood-onset systemic lupus erythematosus (cSLE).

Methods: We linked medical records of patients with cSLE at three tertiary centers (2016-2022) to the Child Opportunity Index (COI) 2.

Electronic health record modification and dashboard development to improve clinical care in pediatric rheumatology.

Objective: This report describes our experience in electronic health record (EHR) note modification and creation of an external dashboard to create a local learning health system that contributes to quality improvement and patient care within our pediatric rheumatology clinic.

Methods: We applied quality improvement methodology to develop a more reliable and accurate system to identify patients with juvenile idiopathic arthritis and track important measures that aide in improving patient care and performance outcomes. From 2019 to 2021, we iteratively modified our outpatient clinic EHR note to include structured data elements to improve longitudinal monitoring.

Racial and Ethnic Composition of Populations Served by Freestanding Children's Hospitals and Disparities in Outcomes of Pediatric Lupus.

Objective: Health disparities may be driven by hospital-level factors. We assessed whether racial and ethnic composition of populations hospitals serve explain or modify disparities in hospital outcomes of children with systemic lupus erythematosus (SLE).

Methods: In this retrospective cohort study of patients 5 to 26 years old with SLE at 47 children's hospitals in the Pediatric Health Information System (2006-2021), race and ethnicity were assessed at the patient level and hospital level (proportion of total admissions composed of Black or Hispanic patients, respectively).

Majority of new patient referrals to a large pediatric rheumatology center result in non-rheumatic diagnosis.

Objective: Pediatric rheumatology faces a looming supply-demand crisis. While strategies have been proposed to address the supply shortfall, investigation into the increased demand for pediatric rheumatic care has been limited. Herein, we analyze new patient visits to a large tertiary care pediatric rheumatology center to identify emerging trends in referrals and areas for potential intervention to meet this increased demand.

Real-world use and outcomes of belimumab in childhood-onset lupus: A single-center retrospective study.

Background: Studies of real-world effectiveness of belimumab in adults with systemic lupus erythematosus have shown improved disease control and decreased oral glucocorticoid use. However, belimumab use outside of clinical trial settings has not been well studied in childhood-onset systemic lupus erythematosus (cSLE). We aimed to characterize indications for belimumab use and evaluate oral glucocorticoid doses and disease activity scores in the year following belimumab initiation at a single, large pediatric rheumatology center.

A multi-site pilot randomized clinical trial of the Treatment and Education Approach for Childhood-onset Lupus (TEACH) program: study design and COVID-19 adaptations.

Background: Childhood-onset Systemic Lupus Erythematosus (cSLE) is an autoimmune disease associated with fatigue, mood symptoms, and pain. Fortunately, these symptoms are potentially modifiable with psychological intervention such as cognitive-behavioral therapy (CBT). The Treatment and Education Approach for Childhood-onset Lupus (TEACH) program is a CBT intervention developed to target these symptoms for adolescents and young adults with cSLE.

Childhood-Onset Lupus Nephritis in the Childhood Arthritis and Rheumatology Research Alliance Registry: Short-Term Kidney Status and Variation in Care.

Objective: The goal was to characterize short-term kidney status and describe variation in early care utilization in a multicenter cohort of patients with childhood-onset systemic lupus erythematosus (cSLE) and nephritis.

Methods: We analyzed previously collected prospective data from North American patients with cSLE with kidney biopsy-proven nephritis enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry from March 2017 through December 2019. We determined the proportion of patients with abnormal kidney status at the most recent registry visit and applied generalized linear mixed models to identify associated factors.

Patient-Reported Outcomes Among Transition-Age Young Adults With Juvenile Idiopathic Arthritis in the Childhood Arthritis and Rheumatology Research Alliance Registry.

Objective: To evaluate patient-reported care utilization and outcomes among young adults with juvenile idiopathic arthritis (JIA), including factors associated with complete transfer to adult rheumatology.

Methods: We included young adults with JIA enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry from 2015 to 2019 with age ≥ 18 years at their last clinical site visit. We used data from the CARRA Registry Long-term Follow-up program, which follows inactive CARRA Registry patients and collects patient-reported information through phone surveys.

Pediatric to Adult Transition Literature: Scoping Review and Rheumatology Research Prioritization Survey Results.

The transition from pediatric to adult care is the focus of growing research. It is important to identify how to direct future research efforts for maximum effect. Our goals were to perform a scoping review of the transition literature, highlight gaps in transition research, and offer stakeholder guidance on the importance and feasibility of research questions designed to fill identified gaps.

A Rare STXBP2 Mutation in Severe COVID-19 and Secondary Cytokine Storm Syndrome.

Background: Primary (familial) hemophagocytic lymphohistiocytosis (pHLH) is a potentially lethal syndrome of infancy, caused by genetic defects in natural killer (NK) cell and CD8 T cell cytotoxicity, leading to hyperinflammation, elevated cytokine levels, and a disorganized immune response resulting in multi-organ system failure and frequently death. Secondary HLH (sHLH) can be triggered in the setting of malignances, diseases of chronic immune system activation, or by infectious etiologies. While pHLH is usually a result of homozygous gene mutations, monoallelic hypomorphic and dominant-negative mutations in pHLH genes have been implicated in sHLH.

Leukocyte Telomere Length and Childhood Onset of Systemic Lupus Erythematosus in the Black Women's Experiences Living with Lupus Study.

Objective: The study objective was to compare leukocyte telomere length (LTL) among patients with systemic lupus erythematosus (SLE) diagnosed in childhood versus adulthood.

Methods: Data are from the Black Women's Experiences Living with Lupus (BeWELL) study. Multivariable linear regression analyses that examined childhood diagnosis of SLE (diagnosed before 18 years of age), age, and their interaction in relationship to LTL were conducted, adjusting for a range of demographic, socioeconomic, and health-related covariates.

Implementation Science in Pediatric Rheumatology: A Path to Health Equity.

Implementation science is the study of processes that promote reliable uptake of evidence-based practices into clinical care. The integration of implementation science and health disparities research approaches has been proposed as a method to reduce health inequity through detection, understanding, and implementation of health equity-focused interventions. In this review, we provide an argument for the study of implementation science in pediatric rheumatology in light of previously observed health disparities, present a framework for the study of health equity and implementation science in pediatric rheumatology, and propose next steps to accelerate action.

Principles of pediatric lupus nephritis in a prospective contemporary multi-center cohort.

Lupus nephritis (LN) is a life-threatening manifestation of systemic lupus erythematosus (SLE) and is more common in children than adults. The epidemiology and management of childhood-onset SLE (cSLE) have changed over time, prompting the need to reassess expected outcomes. The purpose of this study is to use the Childhood Arthritis and Rheumatology Research Alliance (CARRA) prospective registry to validate historical principles of LN in a contemporary, real-world cohort.

Maintaining Hepatitis B Protection in Immunocompromised Pediatric Rheumatology and Inflammatory Bowel Disease Patients.

Objective: Hepatitis B virus (HBV) infection remains a significant public health challenge, particularly for immunocompromised patients. Our aim was to evaluate the serologic immunity in immunocompromised rheumatology and inflammatory bowel disease (IBD) patients, assess factors for serologic nonimmunity, and evaluate their response to 1 HBV booster dose.

Methods: Immunocompromised rheumatology and IBD patients with completed HBV screening were identified.

Quality of Care in Childhood-onset Systemic Lupus Erythematosus: Report of an Intervention to Improve Cardiovascular and Bone Health Screening.

Objective: Initial benchmarking of childhood-onset systemic lupus erythematosus (cSLE) quality indicators revealed suboptimal performance across multiple centers. Our aim was to improve cardiovascular and bone health screenings at a tertiary treatment center for cSLE. This included annual measurements of vitamin D, lipid profiles, and bone mineral density through dual-energy x-ray absorptiometry (DXA).

Improving a process to obtain hepatitis B serology among patients treated with infliximab at a large urban children's hospital.

Background: Hepatitis B infection is a significant public health challenge despite improvements in vaccination efforts. Patients such as those on chronic immunosuppressive therapy for inflammatory bowel disease (IBD) or rheumatic disease may incur greater risk. The risk of reactivation of hepatitis B while on immunosuppressive therapy may have mortality rates up to 25%.

Self-Reported Ageism Across the Lifespan: Role of Aging Knowledge.

The authors examined the prevalence of self-reported ageist behaviors in a lifespan sample ranging in age from 13 to 91 years. Participants completed the Relating to Older People Evaluation (Cherry & Palmore). Results indicated that adolescents and young adults reported fewer ageist behaviors overall than did middle-aged and older adults.

Knowledge of memory aging across the lifespan.

The authors examined knowledge of normal and pathological memory aging in a lifespan sample of 198 individuals who ranged in age from 13 to 88 years. Participants completed the Knowledge of Memory Aging Questionnaire (Cherry, Brigman, Hawley, & Reese, 2003). The authors hypothesized that high school students would be less knowledgeable about memory aging issues than college students, middle-aged, and community-dwelling older adults.

Semantic encoding enhances the pictorial superiority effect in the oldest-old.

ABSTRACT We examined the effect of a semantic orienting task during encoding on free recall and recognition of simple line drawings and matching words in middle-aged (44-59 years), older (60-89 years), and oldest-old (90+ years) adults. Participants studied line drawings and matching words presented in blocked order. Half of the participants were given a semantic orienting task and the other half received standard intentional learning instructions.

Working memory in the oldest-old: evidence from output serial position curves.

In the present study, we examined adult age differences in short-term and working memory performance in middle-aged (45-64 years), young-old (65-74 years), old-old (75-89 years), and oldest-old adults (90 years and over) in the Louisiana Healthy Aging Study. Previous research suggests that measures of working memory are more sensitive to age effects than are simple tests of short-term memory Bopp and Verhaeghen (Journal of Gerontology: Psychological Sciences 60:223-233, 2005), Myerson, Emery, White, and Hale, (Aging, Neuropsychology, and Cognition 10:20-27, 2003). To test this hypothesis, we examined output serial position curves of recall data from three span tasks: forward and backward digit span and size judgment span.