Pediatric Pulmonology最新文献

{"title":"A call to action: An urgent need for national efforts to expand access to pediatric home healthcare.","authors":"Carolyn Foster, Holly Hòa Võ, Avani V Shah","doi":"10.1002/ppul.27127","DOIUrl":"10.1002/ppul.27127","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141311385","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Burkholderia cepacia complex in primary ciliary dyskinesia.","authors":"José Muñiz-Hernández, Kimberley R Kaspy, Jennifer S Landry, Adam J Shapiro, Michael G O'Connor, Margaret W Leigh, Wilfredo De Jesús-Rojas","doi":"10.1002/ppul.27119","DOIUrl":"10.1002/ppul.27119","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141458666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Is there an increased number of community-acquired pneumonia requiring drainage placement in children after COVID-19 pandemic in Italy?","authors":"Luca Barchi, Egidio Barbi, Giulia Zamagni, Alessandro De Fanti, Lorenzo Iughetti, Andrea Trombetta","doi":"10.1002/ppul.27150","DOIUrl":"10.1002/ppul.27150","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Refractory Mycoplasma pneumoniae pneumonia complicated by massive pyopneumothorax in children: A case series.","authors":"Cheng Beilei, Huang Yuan, Ke Liqin, Huang Meixia, Wu Xiling, Tang Lanfang","doi":"10.1002/ppul.27151","DOIUrl":"10.1002/ppul.27151","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141470111","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study.","authors":"Nagehan Emiralioğlu, Banu Çakır, Ahmet Sertçelik, Ebru Yalçın, Nural Kiper, Velat Şen, Derya Ufuk Altıntaş, Mahir Serbes, Haluk Çokuğraş, Ayşe Ayzıt Kılınç, Azer Kılıç Başkan, Evrim Hepkaya, Hakan Yazan, Özden Türel, Hale Molla Kafi, Aslı İmran Yılmaz, Gökçen Ünal, Tuğçe Çağlar, Ebru Damadoğlu, İlim Irmak, Esen Demir, Gökçen Öztürk, Ayşen Bingöl, Erdem Başaran, Nihat Sapan, Ayşe Tana Aslan, Pelin Asfuroğlu, Koray Harmancı, Mehmet Köse, Melih Hangül, Ali Özdemir, Gökçen Tuğcu, Sanem Eryılmaz Polat, Gizem Özcan, Zeynep Gökçe Gayretli, Özlem Keskin, Sevgi Bilgiç, Hasan Yüksel, Şebnem Özdoğan, Erdem Topal, Gönül Çaltepe, Demet Can, Pervin Korkmaz Ekren, Mehmet Kılıç, Ayşe Süleyman, Tuğba Şişmanlar Eyüboğlu, Güzin Cinel, Sevgi Pekcan, Nazan Çobanoğlu, Erkan Çakır, Uğur Özçelik, Deniz Doğru","doi":"10.1002/ppul.27165","DOIUrl":"10.1002/ppul.27165","url":null,"abstract":"<p><strong>Background: </strong>The decline in pulmonary function is a predictor of disease progression in patients with cystic fibrosis (CF). This study aimed to determine the decline rate of percent predicted forced expiratory volume in 1 s (ppFEV1) based on the data of the CF Registry of Turkey. The secondary aim was to investigate the risk factors related to the decline in ppFEV1.</p><p><strong>Methods: </strong>A retrospective cohort study of CF patients over 6 years old, with pulmonary function data over at least 2 years of follow-up was extracted from the national CF registry for years 2017-2019. Patients were classified according to disease severity and age groups. Multivariate analysis was used to predict the decline in ppFEV1 and to investigate the associated risk factors.</p><p><strong>Results: </strong>A total of 1722 pulmonary function test results were available from 574 patients over the study period. Mean diagnostic age was older and weight for age, height for age, and body mass index z scores were significantly lower in the group of ppFEV1 < 40, while chronic Pseudomonas aeruginosa (p < .001) and mucoid P. aeruginosa colonization (p < .001) were significantly higher in this group (p < .001). Overall mean annual ppFEV1 decline was -0.97% (95% confidence interval [CI] = -0.02 to -1.92%). The mean change of ppFEV1 was significantly higher in the group with ppFEV1 ≥ 70 compared with the other (ppFEV1 < 40 and ppFEV1: 40-69) two groups (p = .004). Chronic P. aeruginosa colonization (odds ratio [OR] = 1.79 95% CI = 1.26-2.54; p = .01) and initial ppFEV1 ≥ 70 (OR = 2.98 95% CI = 1.06-8.36), p = .038) were associated with significant ppFEV1 decline in the whole cohort.</p><p><strong>Conclusions: </strong>This data analysis recommends close follow-up of patients with normal initial ppFEV1 levels at baseline; advocates for early interventions for P. aeruginosa; and underlines the importance of nutritional interventions to slow down lung disease progression.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141559469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Top 5 research priorities in asthma -parents perspective.","authors":"Adnan Zafar","doi":"10.1002/ppul.27138","DOIUrl":"10.1002/ppul.27138","url":null,"abstract":"","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141311392","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Improved detection of cystic fibrosis by the California Newborn Screening Program for all races and ethnicities.","authors":"Meghan E McGarry, Stanley Sciortino, Steve Graham, Tracey Bishop, Elizabeth R Gibb","doi":"10.1002/ppul.27155","DOIUrl":"10.1002/ppul.27155","url":null,"abstract":"<p><strong>Background: </strong>Newborn screening (NBS) for cystic fibrosis (CF) is universal in the United States. Protocols vary but include an immunoreactive trypsinogen (IRT) level and CFTR variant panel. California CF NBS has a 3-step screening: IRT level, variant panel, and CFTR sequencing if only one variant identified on panel.</p><p><strong>Methods: </strong>This was a cohort study of infants with CF born in California (2007-2021) to examine racial and ethnic differences in having a false-negative NBS result for CF and at which step the false-negative occurred. We examined how different CFTR variant panels would improve detection of variants by race and ethnicity: original 39-variant panel, current 75-variant panel, and all 402 disease-causing CFTR variants in the CFTR2 database.</p><p><strong>Results: </strong>Of the 912 infants born in California with CF, 84 had a false-negative result: 38 due to low IRT level and 46 with a high IRT value (but incomplete variant detection). Asian (OR 6.3) and Black infants (OR 2.5) were more likely to have a false-negative screening result than non-Hispanic white infants. The majority of false-negative screening (but CF diagnosis) cases among American Indian/Native Alaskan and non-Hispanic White infants were due to low IRT levels. The majority of Asian and Hispanic infants with false-negative screening had no variants detected. Detection of two CFTR variants was improved with the 75-variant panel in Black, Hispanic, and non-Hispanic White infants and with the 402-variant panel in Black, Hispanic, non-Hispanic White, and other race infants.</p><p><strong>Conclusions: </strong>Larger CFTR panels in NBS improved the detection of CF in all races and ethnicities.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141470110","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Validation of the Integrated Palliative Care Outcome Scale (IPOS) in adults with cystic fibrosis.","authors":"Anna M Georgiopoulos, Stephanie DiFiglia, Elizabeth K Seng, Russell Portenoy, Nivedita Chaudhary, Ruobin Wei, Maria N Berdella, Deborah Friedman, Catherine Kier, Rachel W Linnemann, Brandi Middour-Oxler, Teresa Stables-Carney, Patricia Walker, Janice Wang, Lael M Yonker, Lara Dhingra","doi":"10.1002/ppul.27143","DOIUrl":"10.1002/ppul.27143","url":null,"abstract":"<p><strong>Background: </strong>A primary palliative care model for cystic fibrosis (CF) recommends using the Integrated Palliative Care Outcome Scale (IPOS) for screening. Validation of the IPOS is needed.</p><p><strong>Methods: </strong>This secondary analysis utilized baseline data from a multisite trial of the palliative care model, Improving Life with CF. Adults with CF completed the IPOS, the Memorial Symptom Assessment Scale-CF (MSAS-CF), the CF Questionnaire-Revised (CFQ-R), the Patient Health Questionnaire (PHQ-8), the Generalized Anxiety Disorder (GAD-7), and the Perceived Stress Scale (PSS). IPOS structure was assessed using Cronbach α coefficients and a factor analysis. Construct validity was evaluated through bivariate relationships between IPOS scores and other questionnaire scores, and linear regressions assessing the extent to which the IPOS explains variance in quality-of-life domains.</p><p><strong>Results: </strong>The sample comprised 256 adults with complete IPOS data. α coefficients were .86 for the IPOS total score, .81 for the Physical Symptoms subscale, .79 for the Emotional Symptoms subscale, and .63 for the Communication/Practical Issues subscale. A two-component factor structure best aligned with the current subscales. IPOS scores were significantly associated with other measures; associations with MSAS-CF and CFQ-R subscales differentiated the IPOS Physical and Emotional subscales. The IPOS total score provided unique information about the variance in the CFQ-R Physical Functioning and Respiratory Symptoms domain scores.</p><p><strong>Conclusions: </strong>In adults with CF, the IPOS has acceptable internal consistency and there is evidence of construct validity. These findings support adoption of the IPOS in the primary palliative care model for CF.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141458674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Role of body mass index in unbalanced (dysanaptic) lung growth of healthy infants.","authors":"Manuel Sanchez-Solis, Erick Forno, Eva Morales, Luis Garcia-Marcos","doi":"10.1002/ppul.27161","DOIUrl":"10.1002/ppul.27161","url":null,"abstract":"<p><strong>Rationale: </strong>Imbalance between forced expiratory volume in the first second (FEV₁) and forced vital capacity (FVC) (dysanapsis) has been reported in children who are obese. This dysanaptic growth might begin at an early age, although there are no data on children younger than 6 years.</p><p><strong>Objetives: </strong>To assess whether body mass index (BMI) and early weight gain, in healthy infants born at term, plays a significant role in the imbalance between FEV₁ and FVC, even in the absence of obesity.</p><p><strong>Methods: </strong>Lung function was measured by means of raised volume rapid thoracic compression in 69 healthy infants born at term from the Nutrition in Early Life and Asthma cohort. Dysanapsis was defined as zFVC >0.674, zFEV₀ _.5 ≥-1.645, and FEV₀ _.5/FVC ≤-1.645. Weight gain (g/day) and growth rate (cm/year) were calculated as the difference between weight and length on the test date and those at birth. To assess the relationship between zBMI and dysanapsis, a receiver operating characteristic curve was performed. Multivariable analysis was carried out by means of linear regressions (one for each lung function index) and by logistic regression for dysanapsis (yes/no).</p><p><strong>Results: </strong>Higher zBMI was associated with risk of dysanapsis (odds ratio: 3.53, [95% confidence interval: 1.30; 9.66]; p = .014): Each additional zBMI unit was associated with ~10 mL higher FVC and with ~3.5% lower FEV_0.5/FVC. Weight gain was associated with lower FEV_0.5/FVC ratio.</p><p><strong>Conclusion: </strong>Dysanaptic development of lung function begins very early in infancy and is related with weight gain and body mass index, even in the absence of obesity.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535046","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"New York cystic fibrosis consortium newborn screening quality improvement: Development and implementation of a statewide consensus recommendations for management of infants with CFTR-related metabolic syndrome.","authors":"Saroj Choudhary, Eleanor D Muise, Soumia Hammouda, Danielle Goetz, Robert Giusti","doi":"10.1002/ppul.27160","DOIUrl":"10.1002/ppul.27160","url":null,"abstract":"<p><strong>Background: </strong>New York (NY) State implemented a new cystic fibrosis (CF) newborn screen (NBS) algorithm in December 2017 with improvement in positive predictive value and unanticipated increased identification of infants with cystic fibrosis transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS). Repeat sweat testing is recommended in infants with CRMS. During the COVID-19 pandemic infants with CRMS were lost to follow up. With this quality improvement (QI) initiative, we aimed to perform repeat sweat testing in 25% of infants lost to follow up. We also describe consensus recommendations for CRMS from the NY CF NBS Consortium.</p><p><strong>Methods: </strong>Our QI team identified the primary drivers contributing to absent follow up, outreached to families, and created a questionnaire to evaluate parental understanding of CRMS using QI-based strategies.</p><p><strong>Results: </strong>Of 350 infants diagnosed with CRMS during the study period, 179 (51.1%) infants were lost to follow up. A total of 31 (17.3%) were scheduled for repeat sweat tests and followed up at CF Centers. Families reported high satisfaction with the CRMS knowledge questionnaire.</p><p><strong>Conclusions: </strong>With this QI-based approach, we effectively recaptured infants with CRMS previously lost to follow up during the COVID-19 pandemic. Ongoing concerns about infection risk and lack of understanding on the part of families and pediatricians likely contributed to patients with CRMS lost to follow up. Consensus recommendations for CRMS include annual visits with repeat sweat testing until 2-6 years of age and education for adolescents about clinical and reproductive implications of CRMS.</p>","PeriodicalId":19932,"journal":{"name":"Pediatric Pulmonology","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141580471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}