Improving Outcomes of Pediatric Lupus Care Delivery With Provider Goal-Setting Activities and Multidisciplinary Care Models.

OBJECTIVE: The present study was undertaken to evaluate high-quality care delivery in the context of provider goal-setting activities and a multidisciplinary care model using an electronic health record (EHR)-enabled pediatric lupus registry. We then determined associations between care quality and prednisone use among youth with systemic lupus erythematosus (SLE). METHODS: We implemented standardized EHR documentation tools to autopopulate a SLE registry. We compared pediatric Lupus Care Index (pLCI) performance (range 0.0-1.0; 1.0 representing perfect metric adherence) and timely follow-up 1) before versus during provider goal-setting activities and population management, and 2) in a multidisciplinary lupus nephritis versus rheumatology clinic. We estimated associations between pLCI and subsequent prednisone use adjusted for time, current medication, disease activity, clinical features, and social determinants of health. RESULTS: We analyzed 830 visits by 110 patients (median 7 visits per patient [interquartile range 4-10]) over 3.5 years. The provider-directed activity was associated with improved pLCI performance (adjusted ß 0.05 [95% confidence interval (95% CI) 0.01, 0.09]; mean 0.74 versus 0.69). Patients with nephritis in multidisciplinary clinic had higher pLCI scores (adjusted ß 0.06 [95% CI 0.02, 0.10]) and likelihood of timely follow-up than those in rheumatology (adjusted relative risk [RR] 1.27 [95% CI 1.02, 1.57]). A pLCI score of ≥0.50 was associated with 0.72-fold lower adjusted risk of subsequent prednisone use (95% CI 0.53, 0.93). Minoritized race, public insurance, and living in areas with greater social vulnerability were not associated with reduced care quality or follow-up, but public insurance was associated with higher risk of prednisone use. CONCLUSION: Greater attention to quality metrics is associated with better outcomes in childhood SLE. Multidisciplinary care models with population management may additionally facilitate equitable care delivery.

Multicenter Study of Utility and Acceptability of Depression and Anxiety Screening in Adolescents and Young Adults With Childhood-Onset Systemic Lupus.

OBJECTIVE: To determine the utility and acceptability for depression and anxiety screening of adolescents and young adults (AYA) with childhood-onset systemic lupus erythematosus (cSLE) in the pediatric rheumatology setting. METHODS: AYA with cSLE, ages 12-21 years, from 8 collaborating sites, were consecutively screened for depression and anxiety with the Patient Health Questionnaire-9 (PHQ-9) and Generalized Anxiety Disorder 7-item scale (GAD-7). Demographic and disease characteristics were collected, as well as patient-reported outcome measures using the Patient Reported Outcomes Measurement Information System (PROMIS) pediatric profile-25. Acceptability of screening was assessed with postscreening surveys completed by AYA and parents. Chi-square and Wilcoxon rank sum tests examined the relationship between patient characteristics and history of previous screening. Spearman correlations examined relationships between screening scores, PROMIS domains, and other disease factors. RESULTS: Among 106 AYA screened, 64 (60%) had been previously screened, 25 (24%) by general pediatricians. Thirty-two (30%) AYA screened positive, including 24% for depression, 17% for anxiety, and 14% for suicidal ideation. Depression and anxiety symptom severity were highly correlated with increased PROMIS domain scores for fatigue and pain interference and moderately correlated with increased pain severity, decreased mobility, and decreased peer relationships. Eighty-six percent of AYA and 95% of parents expressed comfort with screening in the pediatric rheumatology setting. CONCLUSION: Depression, anxiety, and suicidal ideation are common among AYA with cSLE, and symptoms are correlated with important patient-reported outcomes. Mental health screening in the pediatric rheumatology setting was highly acceptable among AYA with cSLE and their parents.

Validation of coding algorithms for the identification of herpes zoster among children.

PURPOSE: To examine the validity of International Classification of Diseases, 10th Revision, (ICD-10) code-based algorithms for herpes zoster (HZ) in the electronic medical record (EMR) of a large, integrated pediatric healthcare network and to examine baseline demographics and chronic comorbidities associated with HZ in a representative pediatric population. METHODS: We reviewed the electronic charts of all patients with a single ICD-10 for HZ (B02.xx) as their primary or secondary diagnosis in the EMR of the Children's Hospital of Philadelphia (CHOP) healthcare network from January 2010-March 2019. The positive predictive value (PPV) for a single code for HZ was calculated and alternative algorithms were examined to determine which method resulted in the highest PPV. RESULTS: The PPV for a single ICD-10 code was 91.7% (95% CI 80.8-95.4) for definitive and/or probable cases of HZ and 63.9% (95% CI 53.4%-75.5%) for definitive cases alone. Adding a prescription for an antiviral did not improve the PPV. However, adding a new code for rash entered within 1 week of the HZ code increased the PPV to 100% for definitive and/or probable cases but with substantial loss of sensitivity. A high proportion of children with HZ who required inpatient hospitalization had chronic disease (70%) and were on systemic immunomodulatory therapy (50%). CONCLUSIONS: HZ can be identified with a high PPV in electronic medical records of children using ICD-10 code alone. These findings lay the foundation for future pharmacoepidemiologic research to better understand risk factors for HZ infection.

BRCA1 deficient mouse models to study pathogenesis and therapy of triple negative breast cancer.

Genetically engineered mice along with allograft and xenograft models can be used to effectively model triple negative breast cancer both for studies of pathophysiology as well as preclinical prevention and therapeutic drug studies. In this review eight distinct genetically engineered mouse models of BRCA1 deficiency are discussed in relationship to the generation of triple negative mammary cancer. Allograft models derived from some of these genetically engineered mice are considered and xenograft models derived from breast cancers that developed from BRCA1 mutation are presented. Examples of the use of genetically engineered, allograft and xenografts models for preventive and therapeutic studies are presented.