Development and validation of the caregiver Burden from infant home NGT care instrument.

OBJECTIVE: To design and validate an age and condition-specific health status instrument to best reflect the parental experience caring for these children with complex needs and home Nasogastric Tube (NGT) placement. STUDY DESIGN: Combined Qualitative and Quantitative design, testing and implementation for item production and reduction, followed by formal validation by evaluating validity, reliability, and establishing a clinically meaningful change score. SETTINGS: Tertiary care, multi-disciplinary aerodigestive center. PARTICIPANTS: All caregivers whose infant met criteria for eligibility for discharge home from the NICU or Special Care Nursery (SCN) with NGT in place were offered inclusion in this group. Intervention/Exposure: Structured qualitative interviews of these caregivers to explore and define these concepts and domains, to item generate and then reduce, and then psychometric analyses. METHODS: Structured, moderated qualitative interviews with parents/caregivers of children who have undergone home NGT care of their children for item creation, design, and then reduction. Reliability was assessed by Cronbach alpha analysis. Construct validity and clinically meaningful change score was assessed using various query methods. MAIN OUTCOME MEASURES: Cronbach's alpha to assess reliability, a priori hypotheses validity analyses, and minimally important clinical difference calculation. RESULTS: Scaled scores of this condition specific instrument ranged from 14 to 74 where higher scores indicate better QOL related to managing the NGT. Cronbach's alpha with all 14 items was 0.93. Validity was assessed by a self-assessment question to discriminate between change (95% CI: 8.5-14.1; p < 0.0001) as well as by other comparators to identify the instrument's ability to discriminate among populations where parents felt a difference in experience. The minimally important difference was calculated at 18 points. CONCLUSION: This represents the initial validation of the first condition and age-specific health status instrument to assess parent experience of caring for infants requiring a home NGT for dysphagia.

Harnessing the power of electronic health records and open natural language data mining to capture meaningful patient experience during routine clinical care.

INTRODUCTION: Electronic health records (EHR) are a rich data source for both quality improvement and clinical research. Natural language processing can be harnessed to extract data from these previously difficult to access sources. OBJECTIVE: The objective of this study was to create and apply a natural language search query to extract EHR data to ask and answer quality improvement questions at a pediatric aerodigestive center. METHODS: We developed a combined natural language search query to extract clinically meaningful data along with International Statistical Classification of Diseases (ICD10) and Current Procedural Terminology (CPT) code data. This search query was applied to a single pediatric aerodigestive center to answer key clinical questions asked by families. Data were extracted from EHR data from first clinic visit, operative note, microbiology lab report, and pathology report for all new patients from 2020 to 2021. Included as three queries were: 1) if I bring my child to a pediatric aerodigestive center, how often will my child obtain a medical diagnosis without needing an intervention? 2) if my child has a diagnostic procedure, how often will a diagnosis be made? 3) if a diagnosis is made, can it be addressed during that endoscopic intervention? RESULTS: For the 711 new patients coming to the pediatric aerodigestive center from 2020 to 2021, only 26-32% required an interventional triple endoscopy (rigid/flexible bronchoscopy with esophagoduodenoscopy). Of these triple endoscopies, 75.7% resulted in a positive finding that enabled optimization of that child's care. Of the 221 patients who underwent diagnostic triple endoscopies, 40.7% underwent intervention at the same time for laryngeal cleft (injection or suture, dependent upon age). CONCLUSION: Here we created an effective model of open language search query to extract meaningful metrics of patient experience from EHR data. This model easily allows the EHR to be harnessed to create retrospective and prospective databases that can be readily queried to answer clinical questions important to patients. Such databases are widely applicable not just to pediatric aerodigestive centers but to any clinical care setting using an EHR.

Pediatric paradoxical vocal-fold motion: presentation and natural history.

OBJECTIVES: To describe (1) a cohort of children with paradoxical vocal-fold motion (PVFM) who were referred to a multidisciplinary airway center and (2) the outcomes of various treatment modalities including speech therapy, gastroesophageal reflux disease treatment, and psychiatric treatment. PATIENTS AND METHODS: This was a case series with chart review of children younger than 18 years with PVFM evaluated at a tertiary care pediatric airway center over a 36-month period. RESULTS: Fifty-nine children with PVFM were evaluated. The cohort had a mean age of 13.64 years (range: 8-18 years) and a female-to-male ratio of 3:1. Speech therapy as an initial treatment resulted in a 63% (24 of 38) success rate after an average of 3.7 treatment sessions. Speech therapy was a more successful treatment than antireflux therapy (P = .001). Ten percent (6 of 59) of the children presented with a known psychiatric diagnosis, and 30% (18 of 59) of children in the cohort were ultimately diagnosed with a psychiatric condition. Children with inspiratory stridor at rest had a lower initial success rate with speech therapy (56%), a higher rate of underlying psychiatric disorders (75%), and a high rate of success after psychiatric treatment (100%) that required, on average, 3 sessions over a 2-month period. CONCLUSIONS: To our knowledge, this is the largest study to date on pediatric PVFM. The majority of children with PVFM improve with speech therapy. Children with PVFM at rest may be better treated with psychiatric therapy than speech therapy. Furthermore, children who present with symptoms at rest may have a higher likelihood of underlying psychiatric disease.

Pathogen-derived effectors trigger protective immunity via activation of the Rac2 enzyme and the IMD or Rip kinase signaling pathway.

Although infections with virulent pathogens often induce a strong inflammatory reaction, what drives the increased immune response to pathogens compared to nonpathogenic microbes is poorly understood. One possibility is that the immune system senses the level of threat from a microorganism and augments the response accordingly. Here, focusing on cytotoxic necrotizing factor 1 (CNF1), an Escherichia coli-derived effector molecule, we showed the host indirectly sensed the pathogen by monitoring for the effector that modified RhoGTPases. CNF1 modified Rac2, which then interacted with the innate immune adaptors IMD and Rip1-Rip2 in flies and mammalian cells, respectively, to drive an immune response. This response was protective and increased the ability of the host to restrict pathogen growth, thus defining a mechanism of effector-triggered immunity that contributes to how metazoans defend against microbes with pathogenic potential.