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A call to action: An urgent need for national efforts to expand access to pediatric home healthcare. 行动呼吁:迫切需要全国努力扩大儿科家庭医疗服务的覆盖面。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-06-13 DOI: 10.1002/ppul.27127
Carolyn Foster, Holly Hòa Võ, Avani V Shah
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引用次数: 0
Burkholderia cepacia complex in primary ciliary dyskinesia. 原发性睫状肌运动障碍中的伯克霍尔德氏菌复合体
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-06-24 DOI: 10.1002/ppul.27119
José Muñiz-Hernández, Kimberley R Kaspy, Jennifer S Landry, Adam J Shapiro, Michael G O'Connor, Margaret W Leigh, Wilfredo De Jesús-Rojas
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引用次数: 0
Is there an increased number of community-acquired pneumonia requiring drainage placement in children after COVID-19 pandemic in Italy? 意大利 COVID-19 大流行后,需要放置引流管的社区获得性肺炎患儿数量是否有所增加?
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-07-03 DOI: 10.1002/ppul.27150
Luca Barchi, Egidio Barbi, Giulia Zamagni, Alessandro De Fanti, Lorenzo Iughetti, Andrea Trombetta
{"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}
引用次数: 0
Refractory Mycoplasma pneumoniae pneumonia complicated by massive pyopneumothorax in children: A case series. 儿童难治性肺炎支原体肺炎并发大面积脓胸:病例系列。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-06-28 DOI: 10.1002/ppul.27151
Cheng Beilei, Huang Yuan, Ke Liqin, Huang Meixia, Wu Xiling, Tang Lanfang
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引用次数: 0
Factors associated with pulmonary function decline of patients in the cystic fibrosis registry of Turkey: A retrospective cohort study. 土耳其囊性纤维化登记患者肺功能下降的相关因素:回顾性队列研究
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-07-09 DOI: 10.1002/ppul.27165
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

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

背景:肺功能下降是预测囊性纤维化(CF)患者病情发展的一个指标。本研究旨在根据土耳其 CF 登记处的数据确定 1 秒内用力呼气容积(ppFEV1)预测百分比的下降率。次要目的是调查与ppFEV1下降有关的风险因素:一项回顾性队列研究从国家CF登记处提取了2017-2019年6岁以上CF患者的肺功能数据,并进行了至少2年的随访。根据疾病严重程度和年龄组对患者进行了分类。采用多变量分析预测ppFEV1的下降,并研究相关风险因素:在研究期间,共有574名患者提供了1722份肺功能测试结果。ppFEV1组的平均诊断年龄较大,体重年龄比、身高年龄比和体重指数z评分明显较低:本数据分析建议对初始ppFEV1水平基线正常的患者进行密切随访;提倡对铜绿假单胞菌进行早期干预;强调营养干预对延缓肺部疾病进展的重要性。
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引用次数: 0
Top 5 research priorities in asthma -parents perspective. 哮喘病研究的五大重点--家长视角。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-06-13 DOI: 10.1002/ppul.27138
Adnan Zafar
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引用次数: 0
Improved detection of cystic fibrosis by the California Newborn Screening Program for all races and ethnicities. 加州新生儿筛查计划提高了对所有种族和族裔的囊性纤维化检测率。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-06-28 DOI: 10.1002/ppul.27155
Meghan E McGarry, Stanley Sciortino, Steve Graham, Tracey Bishop, Elizabeth R Gibb

Background: 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.

Methods: 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.

Results: 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.

Conclusions: Larger CFTR panels in NBS improved the detection of CF in all races and ethnicities.

背景:囊性纤维化(CF)新生儿筛查(NBS)在美国非常普遍。筛查方案各不相同,但都包括免疫反应性胰蛋白酶原(IRT)水平和 CFTR 变体面板。加利福尼亚州的 CF NBS 筛查分为 3 个步骤:免疫反应胰蛋白酶原(IRT)水平、变异体检测和 CFTR 测序(如果检测中仅发现一个变异体):这是一项对加利福尼亚州出生的 CF 婴儿(2007-2021 年)进行的队列研究,目的是检查在出现 CF NBS 假阴性结果时的种族和民族差异,以及假阴性发生在哪个步骤。我们研究了不同的 CFTR 变异面板如何提高不同种族和族裔的变异检测率:原始的 39 变异面板、当前的 75 变异面板以及 CFTR2 数据库中所有 402 个致病 CFTR 变异:在加利福尼亚州出生的 912 名 CF 婴儿中,有 84 人的结果为假阴性:38 人的 IRT 值低,46 人的 IRT 值高(但变异体检测不全)。与非西班牙裔白人婴儿相比,亚裔婴儿(OR 6.3)和黑人婴儿(OR 2.5)更容易出现筛查结果为假阴性的情况。在美国印第安人/阿拉斯加原住民和非西班牙裔白人婴儿中,大多数假阴性筛查结果(但 CF 诊断结果)是由于 IRT 水平过低造成的。大多数筛查结果为假阴性的亚裔和西班牙裔婴儿没有检测到变异体。在黑人、西班牙裔和非西班牙裔白人婴儿中,使用75个变异株的筛查组提高了两个CFTR变异株的检测率;在黑人、西班牙裔、非西班牙裔白人和其他种族婴儿中,使用402个变异株的筛查组提高了两个CFTR变异株的检测率:结论:在 NBS 中使用较大的 CFTR 染色体可提高所有种族和民族的 CF 检出率。
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引用次数: 0
Validation of the Integrated Palliative Care Outcome Scale (IPOS) in adults with cystic fibrosis. 在囊性纤维化成人患者中验证综合姑息治疗结果量表(IPOS)。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-06-27 DOI: 10.1002/ppul.27143
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

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

背景:一种针对囊性纤维化(CF)的初级姑息治疗模式建议使用综合姑息治疗结果量表(IPOS)进行筛查。需要对 IPOS 进行验证:这项二次分析利用了姑息治疗模式 "改善 CF 患者的生活 "多站点试验的基线数据。成年 CF 患者填写了 IPOS、纪念症状评估量表-CF (MSAS-CF)、CF 问卷-修订版 (CFQ-R)、患者健康问卷 (PHQ-8)、广泛性焦虑症 (GAD-7) 和感知压力量表 (PSS)。使用 Cronbach α 系数和因子分析评估了 IPOS 的结构。通过IPOS得分与其他问卷得分之间的双变量关系,以及评估IPOS对生活质量领域差异解释程度的线性回归,对结构有效性进行了评估:样本包括 256 名具有完整 IPOS 数据的成年人。IPOS 总分的 α 系数为 0.86,身体症状分量表的 α 系数为 0.81,情感症状分量表的 α 系数为 0.79,沟通/实际问题分量表的 α 系数为 0.63。双成分因子结构与当前的分量表最为吻合。IPOS得分与其他测量指标有明显关联;与MSAS-CF和CFQ-R分量表的关联区分了IPOS的身体和情感分量表。IPOS总分提供了有关CFQ-R身体功能和呼吸症状领域得分差异的独特信息:在成年 CF 患者中,IPOS 具有可接受的内部一致性,并有证据表明其具有构建效度。这些研究结果支持在CF的初级姑息治疗模式中采用IPOS。
{"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}
引用次数: 0
Role of body mass index in unbalanced (dysanaptic) lung growth of healthy infants. 体重指数在健康婴儿肺部生长不平衡(发育不良)中的作用。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-07-05 DOI: 10.1002/ppul.27161
Manuel Sanchez-Solis, Erick Forno, Eva Morales, Luis Garcia-Marcos

Rationale: 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.

Objetives: 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.

Methods: 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).

Results: 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 FEV0.5/FVC. Weight gain was associated with lower FEV0.5/FVC ratio.

Conclusion: 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.

理论依据:据报道,肥胖儿童的第一秒用力呼气容积(FEV1)和用力肺活量(FVC)之间存在失衡现象(失调)。虽然目前还没有关于 6 岁以下儿童的数据,但这种发育障碍可能在幼年时期就已开始:评估健康足月儿的体重指数(BMI)和早期体重增加是否对 FEV1 和 FVC 之间的不平衡起到重要作用,即使没有肥胖症:方法:对 69 名足月儿(来自生命早期营养和哮喘队列)的肺功能进行了测量,测量方法为快速胸腔挤压法。肺功能障碍的定义是 zFVC >0.674,zFEV0 .5 ≥-1.645,FEV0 .5/FVC ≤-1.645。体重增加(克/天)和生长速度(厘米/年)按测试日期的体重和身长与出生时的体重和身长之差计算。为评估 zBMI 与发育不良之间的关系,进行了接收器操作特征曲线分析。多变量分析是通过线性回归(每个肺功能指数一个)和肺发育不良(是/否)的逻辑回归进行的:较高的 zBMI 与呼吸困难的风险相关(几率比:3.53,[95% 置信区间:1.30; 9.66];P = .014):每增加一个 zBMI 单位,FVC 会增加 ~10 mL,FEV0.5/FVC 会降低 ~3.5%。体重增加与较低的 FEV0.5/FVC 比率有关:结论:肺功能的失调发育在婴儿期很早就开始了,与体重增加和体重指数有关,即使没有肥胖症也是如此。
{"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 (FEV<sub>1</sub>) 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 FEV<sub>1</sub> 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, zFEV<sub>0</sub> <sub>.5</sub> ≥-1.645, and FEV<sub>0</sub> <sub>.5</sub>/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<sub>0.5</sub>/FVC. Weight gain was associated with lower FEV<sub>0.5</sub>/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}
引用次数: 0
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. 纽约囊性纤维化联盟新生儿筛查质量改进:制定和实施全州范围内关于 CFTR 相关代谢综合征婴儿管理的共识建议。
IF 2.7 3区 医学 Q1 PEDIATRICS Pub Date : 2024-11-01 Epub Date: 2024-07-11 DOI: 10.1002/ppul.27160
Saroj Choudhary, Eleanor D Muise, Soumia Hammouda, Danielle Goetz, Robert Giusti

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

背景:纽约州(NY)于 2017 年 12 月实施了新的囊性纤维化(CF)新生儿筛查(NBS)算法,提高了阳性预测值,并意外增加了囊性纤维化跨膜传导调节器(CFTR)相关代谢综合征(CRMS)婴儿的识别率。建议对患有 CRMS 的婴儿进行重复汗液检测。在 COVID-19 大流行期间,患有 CRMS 的婴儿失去了随访机会。通过这项质量改进(QI)计划,我们的目标是对 25% 失去随访的婴儿进行重复汗液检测。我们还介绍了纽约 CF NBS 联合会针对 CRMS 提出的共识建议:我们的 QI 团队确定了导致随访缺失的主要原因,并与家庭进行了接触,还制作了一份调查问卷,利用基于 QI 的策略评估家长对 CRMS 的理解:结果:在研究期间被诊断为 CRMS 的 350 名婴儿中,有 179 名(51.1%)失去了随访机会。共有 31 名婴儿(17.3%)被安排在 CF 中心进行重复出汗测试和随访。家属对 CRMS 知识问卷的满意度很高:通过这种基于 QI 的方法,我们有效地找回了在 COVID-19 大流行期间失去随访的 CRMS 婴儿。家属和儿科医生对感染风险的持续担忧以及缺乏了解很可能是导致 CRMS 患者失去随访的原因。针对CRMS的共识建议包括每年进行随访,并在2-6岁前重复进行汗液检测,以及对青少年进行有关CRMS的临床和生殖影响的教育。
{"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}
引用次数: 0
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Pediatric Pulmonology
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