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1.
Pediatr Pulmonol ; 2024 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-38869166

RESUMO

BACKGROUND AND OBJECTIVES: The lung and sleep health of adults is heavily influenced by early factors, both genetic and environmental; therefore, optimizing respiratory health begins in childhood. Multiple barriers impede improvements in lung and sleep health for children. First, the traditional siloing between general pediatric care in the community, pediatric pulmonary and sleep subspecialty care, and the research community limits the translation of knowledge into practice. Additionally, identifying and addressing health disparities remains a challenge. The 2021 NHLBI-sponsored workshop "Defining and Promoting Pediatric Pulmonary Health (DAP3H)" was a first step in defining critical gaps in our current healthcare system in identifying and optimizing lung and sleep health in children. The workshop identified key opportunities including measuring pulmonary function in young children, sleep-focused outcomes, developing biomarkers, and longitudinal research cohorts. To expand on the work of DAP3H and continue initiatives to improve childhood lung and sleep health, the Pediatrics & Pulmonary Network: Improving Health Together conference was held in 2023. STUDY DESIGN: A modified Delphi process was applied to form consensus surrounding gaps, barriers, and action items, with the goal of identifying the most urgent opportnities for improving childhood lung and sleep health. RESULTS: Cross-cutting foundational principles were identified as: (1) Authentic Stakeholder Collaboration & Engagement, (2) Reach & Implementation in Real World Settings, (3) Understanding Current Landscape & Resources and (4) Purposeful Diversity, Equity, & Inclusion Initiatives. CONCLUSIONS: To improve lung and sleep health in children, these principles should be the foundation for research design, development, and implementation.

2.
Pediatr Pulmonol ; 2024 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-38804690

RESUMO

INTRODUCTION: This study compares home-based oscillometry and spirometry for characterizing respiratory system disease in school-aged children with bronchopulmonary dysplasia (BPD) in clinical research. We hypothesized higher rates of successful completion and abnormal cases identified through oscillometry, with correlations between device measurements. METHODS: Participants 6-12 years old with BPD in the ongoing Air Quality, Environment and Respiratory Outcomes in BPD (AERO-BPD) study performed oscillometry followed by spirometry at two separate home visits. Parameters measured included airway resistance at 5 Hz(R5), resistance from 5 to 19 Hz(R5-19), resonance frequency(Fres), reactance at 5 Hz(X5), area under the curve between Fres and X5(AX), forced expiratory volume in 1 second(FEV1), forced vital capacity(FVC), and FEV1/FVC. Descriptive statistics identified the proportion of successful tests, correlation in measurements, and rate of lung disease for each device. RESULTS: Among 76 subjects with 120 paired observations, 95% and 71% of participants successfully performed oscillometry and spirometry, respectively, at home visit one. 98% and 77% successfully performed oscillometry and spirometry, respectively, at home visit two. Odds ratios favored oscillometry (range 5.31-10.13, p < 0.01). FEV1 correlated with AX (correlation coefficient r = -0.27, p = 0.03); FEV1/FVC with AX (r = -0.32, p = 0.02); and FEV1/FVC with R5 (r = -0.37, p = 0.01). AX exhibited the highest prevalence of abnormality at 25%; other oscillometry parameters ranged from 5%-22%. Forty-five to sixty-four percent of participants had abnormal spirometry. Oscillometry assessments had significantly lower odds of capturing lung disease (odds ratios 0.07-0.24, p < 0.0001). CONCLUSIONS: School-aged children with BPD demonstrated higher success rates in field-based oscillometry than spirometry. Spirometry exhibited higher rates of abnormality than oscillometry. Moderate correlation exists between device measurements.

4.
Curr Allergy Asthma Rep ; 24(5): 253-260, 2024 05.
Artigo em Inglês | MEDLINE | ID: mdl-38498229

RESUMO

PURPOSE OF REVIEW: Ubiquitous environmental exposures, including ambient air pollutants, are linked to the development and severity of childhood asthma. Advances in our understanding of these links have increasingly led to clinical interventions to reduce asthma morbidity. RECENT FINDINGS: We review recent work untangling the complex relationship between air pollutants, including particulate matter, nitrogen dioxide, and ozone and asthma, such as vulnerable windows of pediatric exposure and their interaction with other factors influencing asthma development and severity. These have led to interventions to reduce air pollutant levels in children's homes and schools. We also highlight emerging environmental exposures increasingly associated with childhood asthma. Growing evidence supports the present threat of climate change to children with asthma. Environmental factors play a large role in the pathogenesis and persistence of pediatric asthma; in turn, this poses an opportunity to intervene to change the course of disease early in life.


Assuntos
Poluentes Atmosféricos , Asma , Exposição Ambiental , Material Particulado , Humanos , Asma/etiologia , Criança , Exposição Ambiental/efeitos adversos , Poluentes Atmosféricos/efeitos adversos , Material Particulado/efeitos adversos , Poluição do Ar/efeitos adversos , Ozônio/efeitos adversos , Mudança Climática , Dióxido de Nitrogênio/efeitos adversos
7.
Pediatr Qual Saf ; 8(6): e700, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38058470

RESUMO

Introduction: Asthma is the most common chronic disease among children. Asthma Action Plans (AAPs) enable asthma self-management tailored to each patient and should be updated annually. At our institution, providers face challenges in creating reliable processes to consistently complete AAPs for patients with asthma. This project's aim was to increase the percentage of patients across five hospital divisions who have an up-to-date AAP from 80% in May 2021 to 85% by October 1, 2021. Methods: We launched a quality improvement (QI) project using the Model for Improvement, focusing on improving AAP completion rates across five hospital divisions providing ambulatory care for asthma patients. The divisions (Adolescent/Young Adult Medicine, Allergy, Pulmonary, and two Primary Care sites) participated in the QI process using tools to understand the problem context. They implemented a cross-divisional AAP completion competition from June to October 2021. Each month during Action Periods, divisions trialed their interventions using Plan-Do-Study-Act cycles. We held monthly Learning Sessions for divisions to collaborate on successful intervention strategies. Results: Statistical process control chart analysis demonstrated that the overall AAP completion rate increased from a baseline of 80% to 87% with the initiation of the competition. All divisions showed improvement in AAP completion rates during the active intervention period, but sustainment varied. Conclusions: The cross-divisional competition motivated five divisions to improve processes to increase AAP completion rates. This approach effectively fostered engagement and idea sharing to boost performance, and may be considered for other QI projects.

8.
Pediatrics ; 152(Suppl 2)2023 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-37656029

RESUMO

Lifelong respiratory health is rooted in the structural and functional development of the respiratory system in early life. Exposures and interventions antenatally through childhood can influence lung development into young adulthood, the life stage with the highest achievable lung function. Because early respiratory health sets the stage for adult lung function trajectories and risk of developing chronic obstructive pulmonary disease, understanding how to promote lung health in children will have far reaching personal and population benefits. To achieve this, it is critical to have accurate and precise measures of structural and functional lung development that track throughout life stages. From this foundation, evaluation of environmental, genetic, metabolic, and immune mechanisms involved in healthy lung development can be investigated. These goals require the involvement of general pediatricians, pediatric subspecialists, patients, and researchers to design and implement studies that are broadly generalizable and applicable to otherwise healthy and chronic disease populations. This National Institutes of Health workshop report details the key gaps and opportunities regarding lung function and structure.


Assuntos
Nível de Saúde , National Institutes of Health (U.S.) , Estados Unidos , Adulto , Criança , Humanos , Adulto Jovem , Pediatras , Taxa Respiratória , Pulmão
9.
Pediatr Allergy Immunol Pulmonol ; 36(3): 115-118, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37703537

RESUMO

Introduction: Data on the use of remote spirometry are limited in the pediatric population. We sought to assess the feasibility and accuracy of a digital turbine spirometer, Medical International Research (MIR) Spirobank Smart (MIR, New Berlin, WI, USA), compared with a pneumotachography spirometer, Pneumotrac (Vitalograph Inc., Lenexa, KS, USA), in field-based clinical research. Methods: This is a cross-sectional study of a subgroup of school-aged participants enrolled in the Air quality, Environment, and Respiratory Outcomes in Bronchopulmonary Dysplasia (BPD) study, who performed same-day paired coached baseline spirometry measurements from the Pneumotrac and MIR devices. Proportion of successful tests was estimated for each device and compared using McNemar's test. Correlation between devices forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) was analyzed by Lin's concordance correlation, and Bland-Altman plots were generated. Results: Twenty-one participants with history of BPD completed home spirometry maneuvers on both devices. The mean age of participants was 8.7 years. The mean FEV1 and FVC measurement was 81% predicted and 90.4% predicted, respectively. The proportion of acceptable tests appeared higher using Pneumotrac (81%) than when using MIR (67%), although without evidence of discordance (P = 0.317). Among subjects with successful tests on both devices, Lin's concordance correlation demonstrated moderate agreement (FEV1 r = 0.955, 95% confidence interval [CI]: 0.87-0.98; FVC r = 0.971, CI: 0.91-0.99). The mean difference in FEV1 between Pneumotrac and MIR was 0.079 L (95% limits of agreement were -0.141 to 0.298 L) and FVC was 0.075 L (95% limits of agreement were -0.171 to 0.322 L). These were relatively small and without evidence of systematic or volume-dependent bias. Conclusions: Utilizing turbine spirometers may be a promising and feasible way to perform pulmonary function testing for field research in children.


Assuntos
Poluição do Ar , Pesquisa Biomédica , Neoplasias da Mama , Displasia Broncopulmonar , Lesões Pré-Cancerosas , Criança , Recém-Nascido , Humanos , Feminino , Displasia Broncopulmonar/diagnóstico , Estudos Transversais , Espirometria
10.
Pediatr Res ; 94(6): 2070-2076, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37438475

RESUMO

BACKGROUND: No previous study has evaluated the relationship between chronotype and adherence to the Mediterranean diet among adolescents. The aim of the present study was to assess the association between chronotype and adherence to the Mediterranean diet in a sample of Spanish adolescents aged 12-17 years. METHODS: This study examined secondary data from 820 adolescents (55.5% girls) from the Eating Healthy and Daily Life Activities (EHDLA) study, which included a representative sample of adolescents aged 12-17 years from the Valle de Ricote (Region of Murcia, Spain). Adolescents' chronotype was evaluated with the Morningness/Eveningness Scale in Children. Adherence to the Mediterranean diet was determined through the Mediterranean Diet Quality Index for Children and Teenagers. RESULTS: The proportion of adolescents with an optimal Mediterranean diet was 37.6%. In relation to chronotype, we found that 24.5%, 69.4%, and 6.1% of the adolescents were classified as morning, intermediate, and evening types, respectively. After adjusting for several covariates, both evening- and intermediate-type adolescents showed lower odds of having optimal adherence to the Mediterranean diet (evening-type: odds ratio [OR] = 0.30, confidence interval [CI] 95% 0.12-0.72; intermediate-type: OR = 0.62, CI 95% 0.44-0.87) than morning-type adolescents. CONCLUSIONS: Chronotype could affect adherence to the Mediterranean Diet in adolescents, so it should be a factor to be considered in future studies assessing eating habits. IMPACT: No previous study has evaluated the relationship between chronotype and adherence to the Mediterranean diet among adolescents. Both evening- and intermediate-type adolescents showed lower odds of having optimal Mediterranean diet adherence than morning-type adolescents. These findings may indicate a need to promote eating healthy habits based on a more holistic approach, not only on the total energy expenditure or in specific food groups but also on the chronotype.


Assuntos
Ritmo Circadiano , Dieta Mediterrânea , Criança , Feminino , Humanos , Adolescente , Masculino , Cronotipo , Inquéritos e Questionários , Comportamento Alimentar
11.
J Pediatr ; 261: 113548, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37301279

RESUMO

OBJECTIVE: To determine health-related quality of life (HRQOL) of school-aged children with bronchopulmonary dysplasia (BPD) using the standardized Patient-Reported Outcomes Measurement Information System (PROMIS) assessment tools. STUDY DESIGN: The Indoor Air Quality and Respiratory Morbidity in Children with BPD Study is an ongoing observational study of school-aged children with BPD. HRQOL is assessed at enrollment by 3 PROMIS questionnaires, Parent Proxy Scale-Global Health 7, Parent Proxy Psychological Stress Experiences-Short Form, and the Parent Proxy Profile-Profile-25. PROMIS data were tested for significant deviation from the standardized T-Score references for normative populations of children. RESULTS: Eighty-nine subjects enrolled in the AERO-BPD study had complete outcome data for HRQOL. The mean age was 9 (±2) years and 43% were female. Mean days on respiratory support totaled 96 (±40). Across all domains, school-aged children with BPD reported similar or slightly better outcomes than the reference sample. Statistically significant findings of lower depression (P < .0001), fatigue (P < .0001), and pain (P < .0001) scores were found; there was no difference in psychological stress experiences (P = .87), global health (P = .06), anxiety (P = .08), relationships (P = .80), and mobility (P = .59) domains. CONCLUSIONS: This study demonstrated that children with BPD may have less depression, fatigue, and pain HRQL than the general population. Once validated, these findings may offer reassurance to parents and providers caring for children with BPD.


Assuntos
Displasia Broncopulmonar , Criança , Feminino , Humanos , Masculino , Displasia Broncopulmonar/epidemiologia , Fadiga , Estudos Longitudinais , Dor , Qualidade de Vida/psicologia
12.
Pediatr Pulmonol ; 58(7): 2042-2049, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37083192

RESUMO

BACKGROUND: Radon may have a role in obstructive lung disease outside its known carcinogenicity. Little is known about radon's effects on asthma morbidity. OBJECTIVE: To determine the effect of radon on fractional exhaled nitric oxide (FE NO), asthma symptom-days, and lung function in inner-city asthmatic school children. METHODS: Two hundred ninety-nine school-aged asthmatic children enrolled in the School Inner-City Asthma Study (SICAS-1) were followed. One and two-month averaged radon was assessed using a spatiotemporal model predicting zip code-specific monthly exposures. FE NO and spirometry were measured twice during the academic year. Asthma symptoms were assessed four times during the academic year. The interaction between indoor radon exposure (Bq/m3 ) and seasonality predicting log-transformed FE NO, forced expiratory volume in 1 s (FEV1 ) % predicted, forced vital capacity (FVC) % predicted, FEV1 /FVC, and asthma symptom-days was evaluated. RESULTS: Participants with high radon exposure had greater change in FE NO from warm to cold periods compared to low radon exposure (interaction p = 0.0013). Participants with >50th percentile radon exposure experience significant FE NO increase from warm to cold weather ( ß $\beta $ = 0.29 [95% confidence interval [CI]: 0.04-0.54], p = 0.0240). We report a positive association between radon 1-month moving average (incidence rate ratio [IRR] = 1.01, p = 0.0273) and 2-month moving average (IRR = 1.01, p = 0.0286) with maximum asthma symptom-days (n = 299, obs = 1167). CONCLUSIONS: In asthmatic children, radon may be associated with increased asthma morbidity, suggesting radon may be a modifiable environmental risk factor for airway inflammation.


Assuntos
Asma , Radônio , Criança , Humanos , Asma/epidemiologia , Asma/etiologia , Asma/diagnóstico , Testes de Função Respiratória , Espirometria , Volume Expiratório Forçado , Morbidade , Radônio/efeitos adversos , Óxido Nítrico
13.
Curr Opin Pediatr ; 35(3): 337-343, 2023 06 01.
Artigo em Inglês | MEDLINE | ID: mdl-36861771

RESUMO

PURPOSE OF REVIEW: The current review provides an assessment of the recent pediatric literature evaluating socioeconomic drivers of asthma incidence and morbidity. The review addresses the specific social determinants of health related to housing, indoor and outdoor environmental exposures, healthcare access and quality, and the impact of systematic racism. RECENT FINDINGS: Many social risk factors are associated with adverse asthma outcomes. Children living in low-income, urban neighborhoods have greater exposure to both indoor and outdoor hazards, including molds, mice, second-hand smoke, chemicals, and air pollutants, all of which are associated with adverse asthma outcomes. Providing asthma education in the community - via telehealth, school-based health centers, or peer mentors - are all effective methods for improving medication adherence and asthma outcomes. The racially segregated neighborhoods created by the racist 'redlining' policies implemented decades ago, persist today as hotspots of poverty, poor housing conditions, and adverse asthma outcomes. SUMMARY: Routine screening for social determinants of health in clinical settings is important to identify the social risk factors of pediatric patients with asthma. Interventions targeting social risk factors can improve pediatric asthma outcomes, but more studies are needed related to social risk interventions.


Assuntos
Poluição do Ar , Asma , Exposição Ambiental , Determinantes Sociais da Saúde , Humanos , Criança , Acessibilidade aos Serviços de Saúde , Racismo Sistêmico , Fatores Socioeconômicos , Áreas de Pobreza , Poluição do Ar/efeitos adversos , Ciência da Implementação , Qualidade de Vida , Qualidade da Assistência à Saúde
14.
Curr Opin Allergy Clin Immunol ; 23(2): 100-110, 2023 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-36730122

RESUMO

PURPOSE OF REVIEW: Asthma is the most common chronic disease of childhood. Environmental exposures, such as allergens and pollutants, are ubiquitous factors associated with asthma development and asthma morbidity. In this review, we highlight the most recent studies relevant to childhood asthma risk, onset, and exacerbation related to air pollution exposure. RECENT FINDINGS: In this article, we review current research that has been published between 2021 and 2022, demonstrating the effects of early-life exposure to key air pollutants (e.g., particulate matter (PM), nitrogen dioxide (NO 2 ), sulfur dioxide (SO 2 ) and ground-level ozone (O 3 ), environmental tobacco smoke, radon, and volatile organic compounds (VOC) on respiratory health. SUMMARY: Air pollution continues to be a global burden with serious consequences related to respiratory health. Interventions aimed at reducing air pollution in the environment must be achieved in an effort to improve asthma outcomes and pediatric health.


Assuntos
Poluentes Atmosféricos , Poluição do Ar , Asma , Humanos , Criança , Exposição Ambiental , Material Particulado
15.
Curr Opin Allergy Clin Immunol ; 23(2): 179-184, 2023 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-36728241

RESUMO

PURPOSE OF REVIEW: Higher asthma prevalence and morbidity are seen in inner-city areas, disproportionately affecting low-income families living in substandard housing. Children within these families experience more frequent asthma exacerbations, acute care and emergency department visits, and hospitalizations, thus characterizing severe asthma. In this review, we assess recent published literature focused on indoor and outdoor exposures that contribute to the development and morbidity of asthma. RECENT FINDINGS: Many urban environmental exposures contribute to asthma burden, including tobacco/e-cigarette smoke, pest allergens, molds, and possibly synthetic chemicals such as phthalates and bisphenol A, radon, and volatile organic compounds. Individuals living in inner-city areas also experience higher levels of air pollutants and ambient heat, further perpetuating asthma incidence and severity. SUMMARY: This article summarizes the latest advances and provides direction for future research on risk factors, interventions, and public policy to help alleviate the burden of asthma due to urban environment exposures.


Assuntos
Poluição do Ar em Ambientes Fechados , Asma , Sistemas Eletrônicos de Liberação de Nicotina , Criança , Humanos , Asma/epidemiologia , Exposição Ambiental/efeitos adversos , Alérgenos/efeitos adversos , Morbidade , População Urbana , Poluição do Ar em Ambientes Fechados/efeitos adversos , Poluição do Ar em Ambientes Fechados/análise
16.
J Asthma ; 60(3): 479-486, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-35341426

RESUMO

OBJECTIVE: The School Inner-City Asthma Intervention Study 2 (SICAS 2) tested interventions to reduce exposures in classrooms of students with asthma. The objective of this post-hoc analysis was limited to evaluating the effect of high-efficiency particulate (HEPA) filtration interventions on mold levels as quantified using the Environmental Relative Moldiness Index (ERMI) and the possible improvement in the students' asthma, as quantified by spirometry testing. METHODS: Pre-intervention dust samples were collected at the beginning of the school year from classrooms and corresponding homes of students with asthma (n = 150). Follow-up dust samples were collected in the classrooms at the end of the HEPA or Sham intervention. For each dust sample, ERMI values and the Group 1 and Group 2 mold levels (components of the ERMI metric) were quantified. In addition, each student's lung function was evaluated by spirometry testing, specifically the percentage predicted forced expiratory volume at 1 sec (FEV1%), before and at the end of the intervention. RESULTS: For those students with a higher Group 1 mold level in their pre-intervention classroom than home (n = 94), the FEV1% results for those students was significantly (p < 0.05) inversely correlated with the Group 1 level in their classrooms. After the HEPA intervention, the average Group 1 and ERMI values were significantly lowered, and the average FEV1% test results significantly increased by an average of 4.22% for students in HEPA compared to Sham classrooms. CONCLUSIONS: HEPA intervention in classrooms reduced Group 1 and ERMI values, which corresponded to improvements in the students' FEV1% test results.


Assuntos
Poluição do Ar em Ambientes Fechados , Asma , Humanos , Asma/terapia , Habitação , Poeira/análise , Fungos , Espirometria , Poluição do Ar em Ambientes Fechados/prevenção & controle , Poluição do Ar em Ambientes Fechados/análise
17.
J Allergy Clin Immunol ; 151(1): 138-146.e9, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36041656

RESUMO

BACKGROUND: Children with asthma are at risk for low lung function extending into adulthood, but understanding of clinical predictors is incomplete. OBJECTIVE: We sought to determine phenotypic factors associated with FEV1 throughout childhood in the Severe Asthma Research Program 3 pediatric cohort. METHODS: Lung function was measured at baseline and annually. Multivariate linear mixed-effects models were constructed to assess the effect of baseline and time-varying predictors of prebronchodilator FEV1 at each assessment for up to 6 years. All models were adjusted for age, predicted FEV1 by Global Lung Function Initiative reference equations, race, sex, and height. Secondary outcomes included postbronchodilator FEV1 and prebronchodilator FEV1/forced vital capacity. RESULTS: A total of 862 spirometry assessments were performed for 188 participants. Factors associated with FEV1 include baseline Feno (B, -49 mL/log2 PPB; 95% CI, -92 to -6), response to a characterizing dose of triamcinolone acetonide (B, -8.4 mL/1% change FEV1 posttriamcinolone; 95% CI, -12.3 to -4.5), and maximal bronchodilator reversibility (B, -27 mL/1% change postbronchodilator FEV1; 95% CI, -37 to -16). Annually assessed time-varying factors of age, obesity, and exacerbation frequency predicted FEV1 over time. Notably, there was a significant age and sex interaction. Among girls, there was no exacerbation effect. For boys, however, moderate (1-2) exacerbation frequency in the previous 12 months was associated with -20 mL (95% CI, -39 to -2) FEV1 at each successive year. High exacerbation frequency (≥3) 12 to 24 months before assessment was associated with -34 mL (95% CI, -61 to -7) FEV1 at each successive year. CONCLUSIONS: In children with severe and nonsevere asthma, several clinically relevant factors predict FEV1 over time. Boys with recurrent exacerbations are at high risk of lower FEV1 through childhood.


Assuntos
Asma , Masculino , Feminino , Criança , Humanos , Adulto , Volume Expiratório Forçado , Asma/tratamento farmacológico , Broncodilatadores/uso terapêutico , Broncodilatadores/farmacologia , Testes de Função Respiratória , Espirometria , Pulmão
20.
Pediatr Pulmonol ; 57(10): 2363-2373, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35712759

RESUMO

BACKGROUND: Because of the heterogeneity in cystic fibrosis (CF) lung disease among young children, a clinical method to identify early-onset lung disease is needed. OBJECTIVE: To develop a CF early-onset lung disease (CFELD) scoring system by utilizing prospectively collected longitudinal data on manifestations in the first 3 years of life. DESIGN: We studied 145 infants born during 2012-2017, diagnosed through newborn screening by age 3 months, and followed to 36 months of age. Cough severity, pulmonary exacerbations (PEx), respiratory cultures, and hospitalizations were collected at each CF center visit (every 1-2 months in infancy and quarterly thereafter). These data were used to construct the CFELD system and to classify lung disease into five categories: asymptomatic, minimal, mild, moderate, and severe. RESULTS: The most frequent manifestation of CF early lung disease was MD-reported PEx episodes, PEx hospitalizations, and positive Pseudomonas aeruginosa cultures. Parent-reported cough severity was correlated with the number of respiratory hospitalizations (r = 0.48, p < 0.0001). The distribution of CFELD categories was 10% asymptomatic, 17% minimal, 29% mild, 33% moderate, and 12% severe. The moderate and severe categories occurred threefold higher in pancreatic insufficient (PI, 49%) versus sufficient subjects (16%), p < 0.0001. In addition to PI, gastrointestinal and nutrition-related hospitalizations, plasma cytokines interleukin (IL)-6 and IL-10, duration of CFTR modulator therapy, and type of health insurance were significant predictors of CFELD scores. CONCLUSION: The CFELD scoring system is novel, allows systematic evaluation of lung disease prognosis early, and may aid in therapeutic decision-making particularly in the initiation of CFTR modulator therapy.


Assuntos
Fibrose Cística , Pré-Escolar , Tosse , Fibrose Cística/complicações , Fibrose Cística/diagnóstico , Fibrose Cística/tratamento farmacológico , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/uso terapêutico , Humanos , Lactente , Recém-Nascido , Interleucina-10 , Pulmão
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