Impact of elexacaftor-tezacaftor-ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a multisystem disease with progressive deterioration. Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were introduced that repair underlying protein defects. Objective of this study was to determine the impact of elexacaftor-tezacaftor-ivacaftor (ETI) on clinical parameters and inflammatory responses in people with CF (pwCF). METHODS: Lung function (FEV1 ), body mass index (BMI) and microbiologic data were collected at initiation and 3-month intervals for 1 year. Blood was analyzed at baseline and 6 months for cytokines and immune cell populations via flow cytometry and compared to non-CF controls. RESULTS: Sample size was 48 pwCF, 28 (58.3%) males with a mean age of 28.8 ± 10.7 years. Significant increases in %predicted FEV1 and BMI were observed through 6 months of ETI therapy with no change thereafter. Changes in FEV1 and BMI at 3 months were significantly correlated (r = 57.2, p < 0.01). There were significant reductions in Pseudomonas and Staphylococcus positivity (percent of total samples) in pwCF through 12 months of ETI treatment. Healthy controls (n = 20) had significantly lower levels of circulating neutrophils, interleukin (IL)-6, IL-8, and IL-17A and higher levels of IL-13 compared to pwCF at baseline (n = 48). After 6 months of ETI, pwCF had significant decreases in IL-8, IL-6, and IL-17A levels and normalization of peripheral blood immune cell composition. CONCLUSIONS: In pwCF, ETI significantly improved clinical outcomes, reduced systemic pro-inflammatory cytokines, and restored circulating immune cell composition after 6 months of therapy.

U.S. active-duty service women's urogenital health and operational readiness through the lens of the IBM-WASH model: A systematic integrative review.

AIM: Identify literature regarding urogenital health and hygiene practices/behaviors of U.S. active-duty service women (ADSW) with attention to environmental conditions; access to water, sanitation, and hygiene (WASH) resources in austere environments. Synthesize relationships among the 3-dimensions and 5-levels of the Integrated Behavioral Model for Water, Sanitation, and Hygiene (IBM-WASH). BACKGROUND: ADSW face sex-specific urogenital health challenges due to decreased access to WASH resources in austere environments, leading to increased risk for urogenital infection-related outcomes (urinary tract infections, vulvovaginal candidiasis, and bacterial vaginosis). During military conflicts in Afghanistan and Iraq, urogenital infections in ADSW were reported as one of the top five medical encounters, and one of the top seven reasons for medical evacuation. METHODS: A systematic review was performed in MEDLINE, CINAHL, Embase, and ClinicalTrials.gov databases, between January 2007-November 2021, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses as a reporting guideline. Eligible literature was evaluated utilizing the Johns Hopkins Research Evidence Appraisal Tool. WASH interactions were mapped using the IBM-WASH interactive matrix. RESULTS: Evidence gaps include shifting focus from "deployment" to environmental austerity; lack of training/education; shifting male-centric culture; and innovative technologies for safety/security. CONCLUSIONS: The IBM-WASH framework allowed for quantification and interpretation of complex interactions occurring in real world austere environments. Some could be overcome individually, but in aggregate they lead to progressive urogenital conditions and potential mission failure. Prevention, diagnosis, and treatment can mitigate disease sequelae. Preventive knowledge and access to innovative technologies designed for ease and private use are critical to preserve operational readiness.

Impact of type of health care provider on long term asthma control.

OBJECTIVE: Asthma prevalence is high and adherence to asthma guidelines is still less than adequate. The main objective of this study was to determine if there were significant differences in outcome measures if asthma care was provided per guidelines either by physicians (pediatric pulmonologists) or specialty trained advance practice nurses (APNs). METHODS: This was a three-year, prospective cohort study of children referred by their primary care providers to a tertiary care center for better asthma control. Patients were provided asthma care per NAEPP guidelines including asthma education. Results were compared over time and between patients followed by physicians or APNs. Alpha level of significance was ≤0.05. RESULTS: The sample included 471 children, ages 2-17 years (mean = 6.4 ± 2.4 years). Physicians and APN's provided asthma care. Of the 471 children enrolled in the study, 176 (37%) were followed for the full three-year study period. At the initial visit, physician group reported more short courses of oral steroids and more unscheduled visits to PCP for acute asthma care in the past 6 months compared to those followed by APNs (<0.05 for all). Among the total cohort and both subgroups, there were significant improvements in mean Asthma Control Test (ACT), acute care need and mean days/month with asthma symptoms over a three-year period (p < 0.05). There was significantly more improvement in use of oral steroids and urgent care visits in physician group (p < 0.05). CONCLUSION: When asthma guidelines are followed, improvements in asthma control are achieved in children in both the MD and APN groups.

Impact of guideline adherence and race on asthma control in children.

BACKGROUND: Asthma control in African Americans (AA) is considered more difficult to achieve than in Caucasian Americans (CA). The aim of this study was to compare asthma control over time among AA and CA children whose asthma is managed per NAEPP (EPR-3) guidelines. METHODS: This was a one-year prospective study of children referred by their primary care physicians for better asthma care in a specialty asthma clinic. All children received asthma care per NAEPP guidelines. Results were compared between CA and AA children at baseline and then at three-month intervals for one year. RESULTS: Of the 345 children, ages 2-17 years (mean = 6.2 ± 4), 220 (63.8%) were CA and 125 (36.2%) were AA. There were no significant differences in demographics other than greater pet ownership in CA families. At baseline, AA children had significantly more visits to the Emergency Department for acute asthma symptoms (mean = 2.3 [Formula: see text] compared to CA (1.4 ± 2.3, P = 0.003). There were no other significant differences in acute care utilization, asthma symptoms (mean days/month), or mean asthma control test (ACT) scores at baseline. Within 3-6 months, in both groups, mean ACT scores, asthma symptoms and acute care utilization significantly improved (P < 0.05 for all) and change over time in both groups was comparable except for a significantly greater decrease in ED visits in AA children compared to CA children (P = 002). CONCLUSION: Overall, improvement in asthma control during longitudinal assessment was similar between AA and CA children because of  consistent use of NAEPP asthma care guidelines.

Impact of asthma severity on long-term asthma control.

Background: Asthma is a common childhood disease with significant morbidity. Severe asthma accounts for just 4-6% of patients, but this group is more difficult to treat and is responsible for up to 40% of asthma expenses.Objective: The relationship between asthma severity and control is not well characterized. The main objective of this study was to determine impact of asthma severity on asthma control over time.Methods: This was a three year, prospective observational cohort study at a tertiary care children's hospital. Results were compared over time and between patients with severe and non-severe persistent asthma. Intervention included therapy based on severity and control, accompanied by a NAEPP (EPR-3) guidelines based structured asthma education program.Results: The sample included 471 children referred from primary care offices with the diagnosis of persistent asthma, mean age 6.4 ± 2.4 years. Forty-one children (8.7%) had severe persistent asthma and 430 (91.3%) children had non-severe persistent asthma (mild-moderate persistent). Our sample size decreased over the three-year period and the number of patients completing the third year were 176 (38%) and among them 20 (11.4%) had severe asthma. At the initial visit, children with severe persistent asthma had significantly more acute care needs, more daily symptoms, and lower mean Asthma Control Test™ scores compared to children with non-severe persistent asthma. Differences between groups decreased within six months with significant improvements in most indicators persisting throughout three-year follow up in both groups (p < 0.05).Conclusion: Asthma control improves independent of severity if asthma guidelines are followed.

A Novel Approach to Assessing Head Injury Severity in Pediatric Patient Falls.

INTRODUCTION: Pediatric patient falls with head-to-floor impact have the greatest potential for injury. METHODS: An objective measure of head injury severity, the Head Injury Criterion (HIC15), was calculated from anthropometric and biomechanical components of patient falls. A secondary aim was to compare HIC15 levels with the hospital's subjective assignment of level of harm (1-9 scale) used for regulatory reports. RESULTS: Adverse event reports yielded a sample of 49 falls from heights of 72.5 to 1793.0 cm by children ages 11 months through 17 years. Contact velocity from beginning to end was 2.81 to 6.16 ms. Mean acceleration was 19.5 to 95.3g. HIC15 levels of impact ranged from 26.4 to 1,330.0, and mean force upon contact was 2.0 to 9.8 N/kg body mass. Seven (14.3%) children's HIC15 levels exceeded age-specific thresholds, with no follow-up scheduled. Hospital-assigned levels of harm were not correlated with HIC15 levels (r = .23, R2 = .05, p = .12). DISCUSSION: A point-of-care computerized HIC15 algorithm would be useful for diagnostic and follow-up decisions.

Erratum to: Racial differences in pet ownership in families of children with asthma.

In the article by Sheikh SI, et al, "Racial differences in pet ownership in families of children with asthma" in World Journal of Pediatrics 2016;12(3):343-346 (doi: 10.1007/s12519-016-0027-9), the last author's name was incorrectly listed as "Don Hayes". His name should have read "Don Hayes Jr".

Operational Failures Detected by Frontline Acute Care Nurses.

Frontline nurses encounter operational failures (OFs), or breakdowns in system processes, that hinder care, erode quality, and threaten patient safety. Previous research has relied on external observers to identify OFs; nurses have been passive participants in the identification of system failures that impede their ability to deliver safe and effective care. To better understand frontline nurses' direct experiences with OFs in hospitals, we conducted a multi-site study within a national research network to describe the rate and categories of OFs detected by nurses as they provided direct patient care. Data were collected by 774 nurses working in 67 adult and pediatric medical-surgical units in 23 hospitals. Nurses systematically recorded data about OFs encountered during 10 work shifts over a 20-day period. In total, nurses reported 27,298 OFs over 4,497 shifts, a rate of 6.07 OFs per shift. The highest rate of failures occurred in the category of Equipment/Supplies, and the lowest rate occurred in the category of Physical Unit/Layout. No differences in OF rate were detected based on hospital size, teaching status, or unit type. Given the scale of this study, we conclude that OFs are frequent and varied across system processes, and that organizations may readily obtain crucial information about OFs from frontline nurses. Nurses' detection of OFs could provide organizations with rich, real-time information about system operations to improve organizational reliability. © 2017 Wiley Periodicals, Inc.

Improved quality-of-life of caregivers of children with asthma through guideline-based management.

OBJECTIVE: The quality of life (QOL) of caregivers of children with asthma may be related to children's responses to asthma management. AIM: To evaluate change in QOL over time of caregivers of children with asthma through guideline-based management. DESIGN: This was a 3-year prospective cohort study of children with asthma referred to our pediatric asthma center. Families completed Pediatric Asthma Caregiver's Quality of Life Questionnaire (PACQLQ), the Asthma Control Test™ (ACT), and reported the number of days/month of albuterol use and wheezing at each clinic visit. RESULTS: We enrolled 143 children, ages 7-17 years (mean = 10.6 ± 2.9), 56.6% male, 70.6% Caucasian. Patients were managed by the same MD (n = 65,45.5%) or APN (n = 78,54.5%) over time. The mean total PACQLQ significantly increased over the 3-year period (F = 67.418, p < .001). Total scores at the first visit were 4.8 ± 1.6, which improved to 6.1 ± 1 at the 3-month follow-up visit. This improvement was sustained at the 1, 2, and 3-year clinic visits. PACQLQ emotional function (F = 60.798, p < .001) and activity limitation (F = 41.517, p < .001) domains significantly improved as well. PACQLQ scores were significantly associated with improved ACT scores (r = .37 to .47, p < .05), fewer days/month of albuterol use (r = -.25 to -.36., p < .05), and wheezing (r = -.28 to -.33, p < .05). There were no significant differences in PACQLQ, or asthma clinical outcome measures between MD and APN providers. CONCLUSION: Use of National Asthma Education and Prevention Program (NAEPP) guidelines significantly improved QOL of caregivers of children with asthma and in asthma-related symptoms. Improvements over time were independent of type of providers.

Novel Computed Tomography Scoring System for Sinus Disease in Adults With Cystic Fibrosis.

OBJECTIVE: There is no easy to use scoring system for computed tomography (CT) scans of the sinuses that is specific to cystic fibrosis (CF). We propose a simple and easily implemented scoring system to quantify severity of sinus disease in adults with CF. STUDY DESIGN: Case series with chart review. SETTING: Academic tertiary-care referral center. SUBJECTS: Sixty-nine adult patients with CF and 50 age-matched controls. METHODS: We validated a scoring system for CF sinus disease. The CT scans were interpreted by 3 physicians on 2 separate sittings. Parameters include maxillary opacification, nasal obstruction, lateral nasal wall displacement, uncinate process absence/demineralization, and presence/absence of mucocele. RESULTS: Patients with CF aged 21 to 30 years (mean = 24.7 ± 2.49). In CF cohort (n = 69), intrarater reliability for the 10 CT categories ranged from .70 to 1.00. Twenty-six (87%) were in the excellent range, and the remaining 4 (13%) were evaluated as good. In the non-CF cohort (n = 50), reliabilities ranged from .44 to 1.00. Twenty-seven (90%) were in the excellent range. For interrater reliability, in the CF cohort, 10 CT categories across the 3 raters ranged from .55 to 1.00. Excellent reliability was achieved in 15 (50%) of the observations. In the non-CF cohort, reliabilities ranged from .44 to 1.00. CONCLUSION: A novel and easy to use CT scoring system for CF sinus disease in adults was validated with inter- and intrarater reliability. This new CF sinus disease-specific scoring system can be used by clinicians, surgeons, and radiologists.

Racial differences in pet ownership in families of children with asthma.

BACKGROUND: Exposure to household domestic animals such as cats and dogs in early life may have some role in pathogenesis of asthma. Racial differences exist in the prevalence of asthma. We hypothesized that there may also be racial differences in pet ownership in families with asthma. METHODS: A cross sectional study was conducted from June 2011 to December 2014 on 823 of 850 (97%) families of children with asthma for pet ownership. Comparisons among racial groups were done using chi square analysis and one-way analysis of variance. RESULTS: The mean age of the cohort was 6.9±4.4 years. A total of 540 (65.62%) patients were Caucasian, 195 (23.7%) African American, 42 (5.1%) hispanics, and 26 (3.2%) biracial with one Caucasian parent. Pets in the home were reported by 470 (58.5%) households. Significantly fewer African American and hispanic families had pets in the home (26.9% and 44.7%) than biracial and Caucasian families (72% and 69.9%, P<0.001). Likewise, significantly more biracial and Caucasian families were noted to have dogs (52% and 54.4%) or cats (25.4% and 40%) or both cats and dogs (28% and 18%) than African Americans families (20.3%, P<0.001; 7.1%, P<0.001) and (4.6%, P<0.001), respectively. CONCLUSIONS: Among families with asthmatic children, pet ownership is significantly more likely in Caucasian families compared with African-American and Hispanic families, thus there is a racial diversity in pet ownership among families of children with asthma.