The Use of Extracorporeal Life Support in Children With Immune-Mediated Diseases.

OBJECTIVES: To describe the use and outcomes of extracorporeal membrane oxygenation support among children with immune-mediated conditions. DESIGN: Retrospective cohort study. SETTING: The Extracorporeal Life Support Organization registry. PATIENTS: Patients 1 month to 18 years old with International Classification of Diseases, 9th Edition and International Classification of Diseases, 10th Edition codes for immune-mediated conditions from 1989 to 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: During the study period, 207 patients with an immune-mediated condition received extracorporeal membrane oxygenation, and 50% survived to discharge. Most patients (63%) received extracorporeal membrane oxygenation for respiratory support with 53% survival, 21% received cardiac support (55% survival), and 15% received extracorporeal cardiopulmonary resuscitation (34% survival). The most common diagnosis among nonsurvivors was hemophagocytic lymphohistiocytosis/macrophage activation syndrome with 37% survival. Patients with juvenile idiopathic arthritis (23%) and dermatomyositis (25%) had the lowest survival. Nonsurvivors had a higher frequency of infections, neurologic complications, and renal replacement therapy use. Use of preextracorporeal membrane oxygenation corticosteroids was associated with mortality. CONCLUSIONS: Children with immune-mediated conditions can be successfully supported with extracorporeal membrane oxygenation. Extracorporeal membrane oxygenation use has increased over time, and survival varies considerably by diagnosis.

Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis.

OBJECTIVE: To describe patient demographics, interventions, and outcomes in hospitalized children with macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA). METHODS: We performed a retrospective cohort study using data recorded in the Pediatric Health Information System (PHIS) database from October 1, 2006 to September 30, 2010. Participants had International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for MAS and either SLE or JIA. The primary outcome was hospital mortality (for the index admission). Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use. RESULTS: A total of 121 children at 28 children's hospitals met the inclusion criteria, including 19 children with SLE and 102 children with JIA. The index admission mortality rate was 7% (8 of 121 patients). ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common. Compared to children with JIA, those with SLE had a similar mortality rate (6% versus 11%, respectively; exact P = 0.6). More patients with SLE than those with JIA received ICU care (63% versus 27%; P = 0.002), received mechanical ventilation (53% versus 21%; P = 0.003), and had cardiovascular dysfunction (47% versus 23% received inotrope/vasopressor therapy; P = 0.02). Children with SLE and those with JIA received cyclosporine at similar rates, but more children with SLE received cyclophosphamide and mycophenolate mofetil, and more children with JIA received interleukin-1 antagonists. CONCLUSION: Organ system dysfunction is common in children with rheumatic diseases complicated by MAS, and more organ system support is required in children with underlying SLE than in children with JIA. Current treatment of pediatric MAS varies based on the underlying rheumatic disease.

Very high serum ferritin levels are associated with increased mortality and critical care in pediatric patients.

OBJECTIVE: To determine whether an elevated serum ferritin level is independently associated with mortality and receipt of critical care in pediatric patients. DESIGN: Retrospective cohort study, open population. SETTING: Seattle Children's Hospital, Seattle, WA, from September 2, 2003, to February 15, 2008. PATIENTS: All patients tested for serum ferritin level from September 2, 2003, to August 16, 2007, with a level ≥1000 ng/mL. INTERVENTIONS: None. MAIN ANALYSIS: Cox regression. MEASUREMENTS AND MAIN RESULTS: The predictor of interest was the patient-specific peak serum ferritin level, dichotomized a priori at 3000 ng/mL. The outcomes were mortality and intensive care unit admission. A total of 171 patients met the inclusion criteria. The observation time without death or intensive care unit admission ranged from 184 to 1621 days. The hazard ratio of death with peak ferritin of >3000 ng/mL was 4.32 (95% confidence interval 2.21-8.47, p < .001) compared to peak ferritin of 1000-3000 ng/mL. The hazard ratio of intensive care unit admission with peak ferritin of >3000 ng/mL was 2.49 (95% confidence interval 1.53-4.05, p < .001) compared to peak ferritin of 1000-3000 ng/mL. Both estimates were adjusted for bone marrow transplant, solid organ transplant, hemoglobinopathy, and existing rheumatologic disease. CONCLUSION: In this pediatric population, with serum ferritin levels of >3000 ng/mL, there was increased risk for both receipt of critical care and subsequent death.

A Multi-Institution Collaboration to Define Core Content and Design Flexible Curricular Components for a Foundational Medical School Course: Implications for National Curriculum Reform.

Medical educators have not reached widespread agreement on core content for a U.S. medical school curriculum. As a first step toward addressing this, five U.S. medical schools formed the Robert Wood Johnson Foundation Reimagining Medical Education collaborative to define, create, implement, and freely share core content for a foundational medical school course on microbiology and immunology. This proof-of-concept project involved delivery of core content to preclinical medical students through online videos and class-time interactions between students and facilitators. A flexible, modular design allowed four of the medical schools to successfully implement the content modules in diverse curricular settings. Compared with the prior year, student satisfaction ratings after implementation were comparable or showed a statistically significant improvement. Students who took this course at a time point in their training similar to when the USMLE Step 1 reference group took Step 1 earned equivalent scores on National Board of Medical Examiners-Customized Assessment Services microbiology exam items. Exam scores for three schools ranged from 0.82 to 0.84, compared with 0.81 for the national reference group; exam scores were 0.70 at the fourth school, where students took the exam in their first quarter, two years earlier than the reference group. This project demonstrates that core content for a foundational medical school course can be defined, created, and used by multiple medical schools without compromising student satisfaction or knowledge. This project offers one approach to collaboratively defining core content and designing curricular resources for preclinical medical school education that can be shared.