SARS-CoV-2 pandemic effect on pediatric asthma health care utilization in an urban hospital.

Background: The "September epidemic" is a well-described phenomenon of increased pediatric asthma-related health care utilization from August to September each year. The coronavirus disease 2019 (COVID-19) pandemic has brought about significant changes in health care utilization, warranting an investigation into its impact on the September epidemic.

Objective: Our aim was to identify the impact of COVID-19 in asthma-related health care utilization, specifically in the September epidemic.

The motivation and process for developing a consortium-wide time and motion study to estimate resource implications of innovations in the use of genome sequencing to inform patient care.

Costs of implementing genomic testing innovations extend beyond the cost of sequencing, affecting personnel and infrastructure for which little data are available. We developed a time and motion (T&M) study within the Clinical Sequencing Evidence-Generating Research (CSER) consortium to address this gap, and herein describe challenges of conducting T&M studies within a research consortium and the approaches we developed to overcome them. CSER investigators created a subgroup to carry out the T&M study (authors).

Continued improvement in disease manifestations of acid sphingomyelinase deficiency for adults with up to 2 years of olipudase alfa treatment: open-label extension of the ASCEND trial.

Background: Olipudase alfa is a recombinant human acid sphingomyelinase enzyme replacement therapy for non-central-nervous-system manifestations of acid sphingomyelinase deficiency (ASMD). The ASCEND randomized placebo-controlled trial in adults with ASMD demonstrated reductions in sphingomyelin storage, organomegaly, interstitial lung disease and impaired diffusion capacity of the lung (DL), during the first year of olipudase alfa treatment. In an ongoing open-label extension of the ASCEND trial, individuals in the placebo group crossed over to olipudase alfa, and those in the olipudase alfa group continued treatment.

Screening, patient identification, evaluation, and treatment in patients with Gaucher disease: Results from a Delphi consensus.

Several guidelines are available for identification and management of patients with Gaucher disease, but the most recent guideline was published in 2013. Since then, there have been significant advances in newborn screening, phenotypic characterization, identification of biomarkers and their integration into clinical practice, and the development and approval of new treatment options. Accordingly, the goal of this Delphi consensus exercise was to extend prior initiatives of this type by addressing issues related to newborn screening, diagnostic evaluations, and treatment (both disease directed and adjunctive).

Endotoxin Acts Synergistically With Clostridioides difficile Toxin B to Increase Interleukin 1β Production: A Potential Role for the Intestinal Biome in Modifying the Severity of C. difficile Colitis.

Background: Inflammation is a crucial driver of host damage in patients with Clostridioides difficile colitis. We examined the potential for the intestinal microbiome to modify inflammation in patients with C. difficile colitis via the effects of gut-derived endotoxin on cytokine production.

Efficient Clinical Counseling for Sickle Cell Disease.

Sickle cell anemia (SCA) is a chronic illness that requires frequent health care visits for preventative management. Adherence to national guidelines such as the National Heart Lung and Blood Institute (NHLBI) Expert Panel Report on the Evidence-Based Management of Sickle Cell Disease can be challenging to both the clinician and the patient. Utilizing effective communication strategies with patients and their families can improve clinician/patient relationships, as well as adherence to national guidelines.

GUÍA: a digital platform to facilitate result disclosure in genetic counseling.

Purpose: Use of genomic sequencing is increasing at a pace that requires technological solutions to effectively meet the needs of a growing patient population. We developed GUÍA, a web-based application, to enhance the delivery of genomic results and related clinical information to patients and families.

Methods: GUÍA development occurred in five overlapping phases: formative research, content development, stakeholder/community member input, user interface design, and web application development.

Contributing Factors for Pediatric Ambulatory Diagnostic Process Errors: Project RedDE.

Background: Pediatric ambulatory diagnostic errors (DEs) occur frequently. We used root cause analyses (RCAs) to identify their failure points and contributing factors.

Methods: Thirty-one practices were enrolled in a national QI collaborative to reduce 3 DEs occurring at different stages of the diagnostic process: missed adolescent depression, missed elevated blood pressure (BP), and missed actionable laboratory values.

Project RedDE: Cluster Randomized Trial to Reduce Missed or Delayed Abnormal Laboratory Value Actions.

Unlabelled: Failure of timely abnormal laboratory result follow-up is relatively common and may lead to harm. This study hypothesized that a quality improvement collaborative (QIC) could reduce the frequency of missed or delayed action on abnormal laboratory values.

Methods: A national cohort of pediatric practices was cluster-randomized to sequentially receive a QIC intervention: video conferences, transparent data sharing, a "focus on failures," QI coaching, and tools to help reduce missed or delayed action on abnormal laboratory values.

Increasing Recognition and Diagnosis of Adolescent Depression: Project RedDE: A Cluster Randomized Trial.

Unlabelled: Adolescent depression causes appreciable morbidity and is underdiagnosed in primary care. This study investigated whether a quality improvement collaborative (QIC) increases the frequency of adolescent depression diagnoses, thus reducing missed diagnoses.

Methods: During a cluster-randomized clinical trial, a national cohort of primary care pediatric practices worked in different orders based on randomization to improve performance on each of three different diagnoses; one was increasing adolescent depression diagnoses.

Cluster Randomized Trial Reducing Missed Elevated Blood Pressure in Pediatric Primary Care: Project RedDE.

Unlabelled: Recognition of childhood hypertension is essential, but pediatricians routinely fail to identify elevated blood pressure (BP). This study investigated if a quality improvement collaborative (QIC) reduces missed elevated BP in primary care.

Methods: During a cluster-randomized clinical trial, a national cohort worked sequentially to reduce each of three different errors, including missed elevated BP.

The Other Side of the Coin: Anti-inflammatory Antibody Therapy for Infectious Diseases.

The inflammatory response to the fungus plays a central role in the respiratory failure associated with pneumonia. To help ameliorate the inflammatory response, corticosteroids are used as an adjuvant to standard antimicrobial therapy. Corticosteroids, however, can have a wide range of effects (including deleterious effects) on the host immune response.

Pediatric Hospitalizations: Are We Missing an Opportunity to Immunize?

Objectives: Fewer than half of children receive all recommended immunizations on time. Hospitalizations may be opportunities to address delayed immunizations. Our objectives were to assess (1) prevalence of delayed immunizations among hospitalized patients, (2) missed opportunities to administer delayed immunizations, and (3) time to catch up after discharge.

Diagnostic Errors in Primary Care Pediatrics: Project RedDE.

Objective: Diagnostic errors (DEs), which encompass failures of accuracy, timeliness, or patient communication, cause appreciable morbidity but are understudied in pediatrics. Pediatricians have expressed interest in reducing high-frequency/subacute DEs, but their epidemiology remains unknown. The objective of this study was to investigate the frequency of two high-frequency/subacute DEs and one missed opportunity for diagnosis (MOD) in primary care pediatrics.

Psychological and behavioural impact of returning personal results from whole-genome sequencing: the HealthSeq project.

Providing ostensibly healthy individuals with personal results from whole-genome sequencing could lead to improved health and well-being via enhanced disease risk prediction, prevention, and diagnosis, but also poses practical and ethical challenges. Understanding how individuals react psychologically and behaviourally will be key in assessing the potential utility of personal whole-genome sequencing. We conducted an exploratory longitudinal cohort study in which quantitative surveys and in-depth qualitative interviews were conducted before and after personal results were returned to individuals who underwent whole-genome sequencing.

Surgical Site Infections Following Pediatric Ambulatory Surgery: An Epidemiologic Analysis.

OBJECTIVE To identify surgical site infection (SSI) rates following pediatric ambulatory surgery, SSI outcomes and risk factors, and sensitivity and specificity of SSI administrative billing codes. DESIGN Retrospective chart review of pediatric ambulatory surgeries with International Classification of Disease, Ninth Revision (ICD-9) codes for SSI, and a systematic random sampling of 5% of surgeries without SSI ICD-9 codes, all adjudicated for SSI on the basis of an ambulatory-adapted National Healthcare Safety Network definition. SETTING Urban pediatric tertiary care center April 1, 2009-March 31, 2014.

The Armstrong Institute Resident/Fellow Scholars: A Multispecialty Curriculum to Train Future Leaders in Patient Safety and Quality Improvement.

The objective was to determine if a year-long, multispecialty resident and fellow quality improvement (QI) curriculum is feasible and leads to improvements in QI beliefs and self-reported behaviors. The Armstrong Institute Resident/Fellow Scholars (AIRS) curriculum incorporated (a) a 2-day workshop in lean sigma methodology, (b) year-long interactive weekly small-group lectures, (c) mentored QI projects, and (d) practicum-based components to observe frontline QI efforts. Pre-post evaluation was performed with the Quality Improvement Knowledge Application Tool (QIKAT) and the Systems Thinking Scale (STS) using the Wilcoxon matched-pairs signed-rank test.

HER-2 expression is not prognostic in osteosarcoma; a Children's Oncology Group prospective biology study.

Background: Since the initial reports of human epidermal growth factor receptor 2 (HER-2) expression as being prognostic in osteosarcoma, numerous small studies varying in the interpretation of the immunohistochemical (IHC) staining patterns have produced conflicting results. The Children's Oncology Group therefore embarked on a prospective biology study in a larger sample of patients to define in osteosarcoma the prognostic value of HER-2 expression using the methodology employed in the initial North American study describing an association between HER-2 expression and outcome.

Procedure: The analytic patient population was comprised of 149 patients with newly diagnosed osteosarcoma, 135 with localized disease and 14 with metastatic disease, all of whom had follow up clinical data.

Cardiac manifestations of neuromuscular disorders in children.

Cardiac abnormalities occur in association with many of the neuromuscular disorders that present in childhood. Genetic defects involving the cytoskeleton, nuclear membrane, and mitochondrial function have all been described in patients with skeletal myopathy and cardiac involvement. The most common classes of neuromuscular disorders with cardiac manifestations are the muscular dystrophies- Duchenne, Becker, limb-girdle and Emery Dreifuss.