Acute Kidney Injury in Multisystem Inflammatory Syndrome in Children (MIS-C): a Case Report.

A 15-year-old female with no significant past medical history who presented with abdominal pain, vomiting, and diarrhea, found to be in acute renal failure and was subsequently diagnosed with multisystem inflammatory syndrome in children (MIS-C). Critical manifestations seen in pediatric COVID-19 can be varied affecting different organ systems. Pediatric providers, during a pandemic with imperfect testing, must be keenly aware of how varied the pathogenesis of COVID-19 can be in children.

Association of a de novo nonsense mutation of the TRIM8 gene with childhood-onset focal segmental glomerulosclerosis.

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is an etiologically heterogeneous disorder. Genetic FSGS may be either limited to the kidney or part of a genetic syndrome with other systemic involvement. At least 21 and 34 genes have been reported for renal-limited and syndromic FSGS, respectively. The TRIM8 gene encodes a tripartite motif protein, which is an E3 ubiquitin-protein ligase that promotes proteasomal degradation of the suppressor of cytokine signaling 1 (SOCS1) and participates in the activation of interferon-gamma signaling. The TRIM8 gene is expressed in various tissues including the kidney and the central nervous system (CNS). An association between a mutation in the TRIM8 gene and childhood-onset FSGS has not been well established. CASE-DIAGNOSIS: We describe an 8-year-old Hispanic male with infantile onset motor and developmental delay, seizures, and proteinuria secondary to FSGS. Next generation sequencing revealed a heterozygous de novo pathogenic variant in the TRIM8 gene (C1380T>A, p.Tyr460*). Immunohistochemical staining using anti-TRIM8 and anti-SOCS1 antibodies showed no significant TRIM8 expression and strong expression of SOCS1 in the renal biopsy tissue. TREATMENT AND CONCLUSIONS: De novo truncating mutations of TRIM8 have been previously reported in childhood-onset epileptic encephalopathy. A molecular analysis of TRIM8 should be considered in children with FSGS and clinical abnormalities of the central nervous system.

Discontinuation of Antimicrobial Prophylaxis (AP) in Children With Spina Bifida: A Case Series Analysis.

BACKGROUND: Spina bifida increases the risk for urinary tract infections (UTI). Antimicrobial prophylaxis (AP) reduces symptomatic UTI's but selects resistant organisms. Measures to ensure regular and complete emptying of the bladder combined with treatment of constipation reduce the risk for UTI. OBJECTIVES: Demonstrate that close adherence to a catheterization regimen in children with spina bifida (Selective Treatment - ST) reduces the need for antimicrobial prophylaxis. METHODS: Case series analysis of pediatric spina bifida clinic patients where routine antimicrobial prophylaxis was replaced by clean-catch catheterization and daily bowel regimen (ST). Retrospective chart review of 67 children (mean entry age: 24 months, median age: 4 months; 32 Males, 35 Females) enrolled between 1986 - 2004. Mean follow-up was 128.6 months (range 3 - 257 months). Asymptomatic and symptomatic UTI incidences were noted on AP and ST protocols. Creatinine clearance at study entry and follow-up was calculated by the age appropriate method. A multivariable regression model with delta Glomerular Filtration Rate (GFR) as the dependent variable, independent sample t-test and Wilcoxon rank sum were performed with SAS v. 9.2. RESULTS: The mean number of infections while on AP was 8.7 (95% CI 5.72, 11.68) and was 1.0 on ST (95% CI 0.48, 1.43). 5 infections on the AP protocol required intravenous (IV) antibiotics due to resistance to oral therapy, but none on ST. Comparing change in GFR between both protocols (AP vs. ST) found a significant difference in the change of GFR by treatment protocol. CONCLUSIONS: AP did not prevent UTIs and resulted in more resistant organisms requiring IV antibiotics. Discontinuing AP allowed the return of susceptibility to oral antimicrobials and significantly improved GFR in those children who had previously been on AP. Adherence to a catheterization regimen with prompt treatment of symptomatic UTI conserved renal function and prevented selection of resistant organisms.

Redefining Quality in Medical Education Research: A Consumer's View.

BACKGROUND: Despite an explosion of medical education research and publications, it is not known how medical educator consumers decide what to read or apply in their practice. OBJECTIVE: To determine how consumers of medical education research define quality and value. METHODS: Journal of Graduate Medical Education editors performed a literature search to identify articles on medical education research quality published between 2000 and 2013, surveyed medical educators for their criteria for judging quality, and led a consensus-building workshop at a 2013 Association of American Medical Colleges meeting to further explore how users defined quality in education research. The workshop used standard consensus-building techniques to reach concept saturation. Attendees then voted for the 3 concepts they valued most in medical education research. RESULTS: The 110 survey responses generated a list of 37 overlapping features in 10 categories considered important aspects of quality. The literature search yielded 27 articles, including quality indexes, systematic and narrative reviews, and commentaries. Thirty-two participants, 12 facilitators, and 1 expert observer attended the workshop. Participants endorsed the following features of education research as being most valuable: (1) provocative, novel, or challenged established thinking; (2) adhered to sound research principles; (3) relevant to practice, role, or needs; (4) feasible, practical application in real-world settings; and (5) connection to a conceptual framework. CONCLUSIONS: Medical educators placed high value on rigorous methods and conceptual frameworks, consistent with published quality indexes. They also valued innovative or provocative work, feasibility, and applicability to their setting. End-user opinions of quality may illuminate how educators translate knowledge into practice.

Achieving and maintaining asthma control in an urban pediatric disease management program: the Breathmobile Program.

BACKGROUND: National guidelines suggest that, with appropriate care, most patients can control their asthma. The probabilities of children achieving and maintaining control with ongoing care are unknown. OBJECTIVE: We sought to evaluate the degree to which children in a lower socioeconomic urban setting achieve and maintain control of asthma with regular participation in a disease management program that provides guideline-based care. METHODS: Interdisciplinary teams of asthma specialists use mobile clinics to offer ongoing care at schools and county clinics. A guideline-derived construct of asthma control is recorded at each visit. RESULTS: Two thousand one hundred eighty-five enrollees were eligible to evaluate the time to first achieve control, and 1591 patients were eligible to evaluate subsequent control maintenance. Depending on severity, 70% to 87% of patients with persistent asthma achieved control by visit 3, and 89% to 98% achieved control by visit 6. Subsequent control maintenance was highly variable. Thirty-nine percent of patients displayed well-controlled asthma (control at >90% of subsequent visits), whereas 13% displayed difficult-to-control asthma (<50% of subsequent visits). Patients from each baseline severity category were found in each group. Maintenance of control was influenced by physician-estimated compliance with the treatment plan, baseline severity, and the interval between clinic visits. CONCLUSIONS: Many children can achieve asthma control with regular visit intervals and guideline-based care; however, long-term control can be highly variable among patients in all severity categories. CLINICAL IMPLICATIONS: These findings highlight the need and feasibility for systematically tracking each patient's clinical response to individualize therapy and guide the use of population management strategies.

The Breathmobile Program: structure, implementation, and evolution of a large-scale, urban, pediatric asthma disease management program.

Despite more than a decade of education and research-oriented intervention programs, inner city children with asthma continue to engage in episodic "rescue" patterns of healthcare and experience a disproportionate level of morbidity. The aim of this study was to establish and evaluate a sustainable community-wide pediatric asthma disease management program designed to shift inner city children in Los Angeles from acute episodic care to regular preventive care in accordance with national standards. In 1995 the Southern California Chapter of the Asthma and Allergy Foundation of America (AAFA), the Los Angeles County Department of Health Services (LAC DHS), and the Los Angeles Unified School District (LAUSD) established an agreement to initiate and sustain the Breathmobile Program. This program includes automated case identification, mobile school-based clinics, and highly structured clinical encounters supported by an advanced information technology solution. Interdisciplinary teams of asthma care specialists provide regular and ongoing care to children at school and county clinic sites over a wide geographic area of urban Los Angeles. Each team operates in a specially equipped mobile clinic (Breathmobile), efficiently moving a structured healthcare process to school and county clinic sites with large numbers of children. Demographic, clinical, and participation data is tracked carefully in an electronic medical record system. Program operations, clinical oversight, and patient tracking are centralized at a care coordination center. Clinical operations and methods have been replicated in fixed specialty clinic sites at the Los Angeles County + University of Southern California Medical Center. Clinical and process measures are regularly evaluated to assure quality, plan iterative improvement, and support evidence-based care. Four Breathmobiles deliver ongoing care at more than 90 school sites. The program has engaged over five thousand patients and their families in a continuity care model that has demonstrated efficacy over usual episodic care. More than 90% of patients in all asthma severity categories achieved clinical control of asthma with significant reductions in inpatient (IP) and emergency department (ED) use. On February 14, 2002, the program became the first program in the United States to receive the award of disease-specific care certification by the Joint Commission on Accreditation of Healthcare Organizations (JCAHO). Proper design and resource allocation can sustain a school-based community-wide pediatric asthma disease management program and shift a population of inner city children from acute episodic care to routine preventive care in accordance with national standards. An evidence-based approach to evaluating and maintaining quality, coupled with stratified care delivery, can assure the efficient use of safety net healthcare resources.