rs12252 was not associated with susceptibility to influenza-related critical illness in children or with critical illness severity. Our data also do not support it being a splice site.

Influenza-MRSA coinfection is associated with high fatality in critically ill children. These data support early addition of a second anti-MRSA antibiotic to vancomycin in suspected severe cases.

Although cystic fibrosis (CF) is attributed to dysfunction of a single gene, the relationships between the abnormal gene product and the development of inflammation and progression of lung disease are not fully understood, which limits our ability to predict an individual patient’s clinical course and treatment response. To better understand CF progression, we characterized the molecular signatures of CF disease status with plasma-based functional genomics. Peripheral blood mononuclear cells (PBMCs) from healthy donors were cultured with plasma samples from CF patients ( n = 103) and unrelated, healthy controls ( n = 31). Gene expression levels were measured with an Affymetrix microarray (GeneChip Human Genome U133 Plus 2.0). Peripheral blood samples from a subset of the CF patients ( n = 40) were immunophenotyped by flow cytometry, and the data were compared with historical data for age-matched healthy controls ( n = 351). Plasma samples from another subset of CF patients ( n = 56) and healthy controls ( n = 16) were analyzed by multiplex enzyme-linked immunosorbent assay (ELISA) for numerous cytokines and chemokines. Principal component analysis and hierarchical clustering of induced transcriptional data revealed disease-specific plasma-induced PBMC profiles. Among 1,094 differentially expressed probe sets, 51 genes were associated with pancreatic sufficient status, and 224 genes were associated with infection with Pseudomonas aeruginosa. The flow cytometry and ELISA data confirmed that various immune modulators are relevant contributors to the CF molecular signature. This study provides strong evidence for distinct molecular signatures among CF patients. An understanding of these molecular signatures may lead to unique molecular markers that will enable more personalized prognoses, individualized treatment plans, and rapid monitoring of treatment response.

IMPORTANCE Family-centered care, which supports family presence (FP) during procedures, is now a widely accepted standard at health care facilities that care for children. However, there is a paucity of data regarding the practice of FP during tracheal intubation (TI) in pediatric intensive care units (PICUs). Family presence during procedures in PICUs has been advocated.OBJECTIVE To describe the current practice of FP during TI and evaluate the association with procedural and clinician (including physician, respiratory therapist, and nurse practitioner) outcomes across multiple PICUs. DESIGN, SETTING, AND PARTICIPANTS Prospective cohort study in which all TIs from July 2010 to March 2014 in the multicenter TI database (National Emergency Airway Registry for Children [NEAR4KIDS]) were analyzed. Family presence was defined as a family member present during TI. This study included all TIs in patients younger than 18 years in 22 international PICUs.EXPOSURES Family presence and no FP during TI in the PICU. MAIN OUTCOMES AND MEASURESThe percentage of FP during TIs. First attempt success rate, adverse TI-associated events, multiple attempts (Ն3), oxygen desaturation (oxygen saturation as measured by pulse oximetry <80%), and self-reported team stress level.RESULTS A total of 4969 TI encounters were reported. Among those, 81% (n = 4030) of TIs had documented FP status (with/without). The median age of participants with FP was 2 years and 1 year for those without FP. The average percentage of TIs with FP was 19% and varied widely across sites (0%-43%; P < .001). Tracheal intubations with FP (vs without FP) were associated with older patients (median, 2 years vs 1 year; P = .04), lower Paediatric Index of Mortality 2 score, and pediatric resident as the first airway clinician (23%, n = 179 vs 18%, n = 584; odds ratio [OR], 1.4; 95% CI, 1.2-1.7). Tracheal intubations with FP and without FP were no different in the first attempt success rate (OR, 1.00; 95% CI, 0.85-1.18), adverse TI-associated events (any events: OR, 1.06; 95% CI, 0.85-1.30 and severe events: OR, 1.04; 95% CI, 0.75-1.43), multiple attempts (Ն3) (OR, 1.03; 95% CI, 0.82-1.28), oxygen desaturation (oxygen saturation <80%) (OR, 0.97; 95% CI, 0.80-1.18), or self-reported team stress level (OR, 1.09; 95% CI, 0.92-1.31). This result persisted after adjusting for patient and clinician confounders.CONCLUSIONS AND RELEVANCE Wide variability exists in FP during TIs across PICUs. Family presence was not associated with first attempt success, adverse TI-associated events, oxygen desaturation (<80%), or higher team stress level. Our data suggest that FP during TI can safely be implemented as part of a family-centered care model in the PICU.

Of 296 observations of medication infusions and 231 observations of intravenous fluid infusions, the frequency of discrepancies between orders entered and pumps programming ranged from 24.3% for observed medications to 42.4% for observed fluids. Anti-infectives (100%), concentrated electrolytes (46.7%) and anticoagulants (46.2%) were associated with greatest discrepancy between orders and programmed doses.