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Miracles, like London buses, just seem to come along. The truth is, there are no miracles, just lots of hard work behind the scenes, minds open to opportunity, serendipity, and possibly a little luck. In my time as a pediatric respiratory physician, I have borne witness to remarkable advances in treatment that have changed patients' fortunes overnight. Examples of these include artificial surfactant replacement for premature newborns, conjugate Haemophilus influenzae type b vaccination, propranolol for infants with subglottic haemangiomas, mandibular distraction for babies with micrognathia, cystic fibrosis transmembrane conductance regulator modulators therapy for patients with cystic fibrosis, and antisense oligonucleotide therapy for infants with spinal muscular atrophy. There are lessons to be learned from reflection upon these life-transforming treatments, and perhaps it is a good time just to pause and wonder.

In 2018-2020, The American Thoracic Society's Pediatric Pulmonology Division Directors Association (PPDDA) and Pediatric Pulmonology Training Directors Association (PEPTDA) convened a series of workshops to analyze workforce issues from the perspectives of current leaders within the discipline. In this, the report from the 3rd workgroup, we examine future workforce issues from several perspectives. We describe a PPDDA survey regarding current state and future needs of the discipline; an assessment of the current NIH research funding portfolio for major children's respiratory disorders; and an estimate of workforce trends in training, based mainly on ABP data. We conclude that current fellowship graduation rates appear to be sufficient to maintain and even grow the total number of pulmonologists, but demand for pediatric pulmonologists may still be greater than supply, and there are geographical disparities in access to care. New treatments for CF, growth of hospitalist services, care of technology dependent children, and multidisciplinary care for complex chronic disease, will likely change our practice over the next 10 years. Research funding appears to be robust for asthma, CF, BPD, microbiome, and pulmonary hypertension, compared to other chronic respiratory disorders. Few of these grants are led by pediatric pulmonologists. Thus, our discipline needs to adjust our training of fellows and junior faculty to take advantage of existing funding opportunities as well as advocate for greater funding for the other disorders we manage. Finally, we highlight opportunities for the discipline to enhance its roles in addressing emerging respiratory pathogens and expanding the diversity of its workforce. This article is protected by copyright. All rights reserved.

Understanding of coronavirus disease 2019 is rapidly evolving with new articles on the subject daily. This flood of articles can be overwhelming for busy practicing clinicians looking for key pieces of information that can be applied in daily practice. This review article synthesizes the reported imaging findings in pediatric COVID‐19 across the literature, offers imaging differential diagnostic considerations and useful radiographic features to help differentiate these entities from COVID‐19, and provides recommendations for ordering imaging studies to evaluate suspected cases of pediatric COVID‐19. This article is protected by copyright. All rights reserved.

Climate change (CC) is expected to negatively impact respiratory health due to air pollution and increased aeroallergen exposure. Children are among the most vulnerable populations due to high ventilation rates, small peripheral airways, and developing respiratory and immunological systems. To assess the current knowledge among Italian pediatric pulmonologists on the potential effects of CC on pediatric respiratory allergic diseases, a national survey was launched online from February 2020 to February 2021. The members of the Italian Pediatric Respiratory Society (SIMRI) were contacted by email and 117 questionnaires were returned (response rate 16.4%). 72.6% of respondents were females, 53.8% were academic pediatricians, 42.7% had been working >10 years. Most of the participants were aware of the potential health effects of CC and stated that they had noticed an increase in the incidence (90.6%) and severity (67.5%) of allergic respiratory diseases among their patients. About 61% and 41% of participants respectively felt that there had been an increase in the number of children sensitized to pollen and molds. When applying latent class analysis to identify the features characterizing participants with greater awareness and knowledge of CC-related health effects, two classes were identified: almost 60% of the participants were labeled as “poor knowledge” and those with greater awareness were older, had longer work experience, and were those using the Internet to gather information about CC. There is urgent need to increase pediatricians' awareness of the detrimental effects of CC on children's respiratory health and integrate them in the educational programs of healthcare professionals.

Aim Children with respiratory conditions benefit from care provided by pediatric pulmonologists. As these physicians are a small portion of the overall pediatric workforce, it is necessary to understand the practices and career plans of these specialists. Methods An internet survey was developed by the American Academy of Pediatrics Division of Workforce and Medical Education Policy and sent to members of the American Academy of Pediatrics and American College of Chest Physicians who identified as pediatric pulmonary physicians. Results Responses were received from 485 physicians and were compared to the results of a similar survey done in 1997. Of those completing the survey, 63% were male and 37% female, with increased number of females since the earlier poll. The average calculated age was 56 years. They worked 54 h per week, down from 59 h in the prior survey. Pediatric pulmonologists are overwhelmingly clinicians (92%) with major responsibilities for administration (79%), teaching (78%), and research. Basic science research was rarely reported (7%). Pediatric pulmonologists felt that referrals had become more complicated in the recent past. Nearly all planned to maintain Pediatric Pulmonology Sub-board certification, though one third planned to cut back on clinical workload in the next decade. Many were concerned that the number needed in the profession in a decade would be inadequate with significant concerns about funding for those positions. Conclusion Overall, these results reflect the current state of the workforce and the need to monitor the supply of practitioners in the future.

Pediatric pulmonologists have been involved in the care of adult COVID‐19 patients in a variety of ways, particularly in areas with a high concentration of cases. This invited commentary is a series of questions to Dr Mikhail Kazachkov, a pediatric pulmonologist at New York University, about his experiences to date in a major COVID‐19 “hotspot” and his thoughts about how other pediatric pulmonologists facing this situation can best support their colleagues.

Unprecedented opportunities and daunting difficulties are anticipated in the future of pediatric pulmonary medicine. To address these issues and optimize pediatric pulmonary training, a group of faculty from various institutions met in 2019 and proposed specific, long-term solutions to the emerging problems in the field. Input on these ideas was then solicited more broadly from faculty with relevant expertise and from recent trainees. This proposal is a synthesis of these ideas. Pediatric pulmonology was among the first pediatric specialties to be grounded deliberately in science, requiring its fellows to demonstrate expertise in scientific inquiry (1). In the future, we will need more training in science, not less. Specifically, the scope of scientific inquiry will need to be broader. The proposal outlined below is designed to help optimize the practices of current providers and to prepare the next generation to be leaders in pediatric care in the future. We are optimistic that this can be accomplished. Our broad objectives are (a) to meet the pediatric subspecialty workforce demand by increasing interest and participation in pediatric pulmonary training; (b) to modernize training to ensure that future pediatric pulmonologists will be prepared clinically and scientifically for the future of the field; (c) to train pediatric pulmonologists who will add value in the future of pediatric healthcare, complemented by advanced practice providers and artificial intelligence systems that are well-informed to optimize quality healthcare delivery; and (d) to decrease the cost and improve the quality of care provided to children with respiratory diseases.

PURPOSE Children's interstitial lung disease (chILD) is often associated with multiple burdens and chronic impairment of health-related quality of life. Patient reported outcomes describe the patients' perspective on medical conditions and their treatments. We aimed to develop and evaluate the psychometric properties of a chILD-specific PRO (chILD-QoL) as an instrument for monitoring the patients' health status. METHODS Items were generated through focus groups with parents, patients, and interviews with pediatric pulmonologists. After a pretest of the German pilot version, the questionnaire was refined and translated into four European languages. Psychometric properties of the questionnaire were analyzed within a multi-center collaborative throughout Europe involving 180 parents of children with an interstitial lung disease and 65 pediatric patients. RESULTS The final instrument is available in different developmentally adapted versions from infancy to adolescence, comprising between 5 and 11 items. The scales showed high internal consistency (Cronbachs'α between 0.85 and 0.94). Convergent validity was indicated by moderate to high correlations (r = 0.43-0.91) with the Pediatric Quality of Life Questionnaire (PedsQL™ 4.0 Core module). Lower scores were significantly associated with dyspnoea (tproxy = 3.18, P = 0.002), tachypnoea (tproxy = 2.95, P = 0.002), and with worse clinical course of lung disease (tself = 3.96, P

INTRODUCTION Flexible bronchoscopy (FB) is the current gold standard for diagnosing tracheobronchomalacia. However, it is not always feasible and virtual bronchoscopy (VB), acquired from chest multi-detector CT (MDCT) scan is an alternative diagnostic tool. We determined the sensitivity, specificity, and positive and negative predictive values of VB compared to FB in diagnosing tracheobronchomalacia. METHODS Children aged

There has been a dramatic increase in the past few decades in the number of children receiving noninvasive positive airway pressure (PAP) therapy at home. Although PAP therapy was first prescribed for children with obstructive sleep apnea, the indications have rapidly widened to include treatment for central hypoventilation syndromes, neuromuscular and chest wall disorders as well as primary respiratory diseases. Given the rapidly expanding use of PAP therapy in children, pediatric pulmonologists need to be familiar with the indications, technical and safety considerations as well as potential complications and challenges that may arise when caring for children using PAP therapy. This review article covers the definition of PAP therapy, modes, interfaces, devices, indications, contraindications, suggested settings, complications as well as the factors influencing the adherence.

Summary Objectives First, create a clinical severity score for patients with chronic lung disease of infancy (CLDi) following neonatal intensive care unit (NICU) stay. Second, using California wide population-based data, identify factors associated with clinical severity of CLDi at 4–9 months corrected gestational age (CGA). Study Design Pediatric pulmonologists ranked and weighted eight factors reflecting clinical severity of CLDi. Utilizing these data we scored and assigned these to 4–9 month old CGA moderate/severe bronchopulmonary dysplasia (BPD) infants, born

An 18-year-old male patient was referred to the department of pediatric pulmonology with a history of recurrent pneumothorax. Initial pneumothorax occurred at the age of 10. Following diagnosis of congenital lobar emphysema, he had five episodes of pneumothorax and subsequently underwent right-lower lobe anterobasal segmentectomy. Based on thoracic computed tomography (CT) and clinical manifestation, Birt-Hogg-Dube (BHD) syndrome was suspected and confirmed following genetic testing. BHD syndrome is a rare tumor predisposition syndrome first described in 1977. The syndrome is characterized by skin fibrofolliculomas, lung cysts, recurrent spontaneous pneumothorax, and renal cell cancer. The underlying cause is a germline mutation in the folliculin (FLCN) gene located on chromosome 17p11.2. Clinical manifestation usually appears after the age of 20 years. In this case, we report a case of BHD with episodes of recurrent pneumothorax, the first of which occurred at the age of 10 years. Pulmonologists should be aware of this syndrome in patients with a personal and family history of pneumothoraces and CT findings of multiple pulmonary cysts as additional evaluation and testing may be warranted. Pediatr Pulmonol. 2016;51:E41-E43. © 2016 Wiley Periodicals, Inc.

Summary Objective Assessment of asthma control in children by physicians, patients and their parents was compared, assuming parents may underestimate symptoms in asthmatic children and exploring whether physicians tend to agree with them. Design Asthma control perception was assessed in 4- to 11-year-old asthmatic children and their parents, using C-ACT, during 2011–2012. Pediatric pulmonologists used GINA guidelines for their assessment; pediatricians, not having spirometry, used the information given in addition to physical examination. The C-ACT scores given by the children and their parents were further analyzed separately, and compared with their physicians' assessment. Statistical methods, which also measured possible influence of different variables, included kappa, Chi-square, linear-by-linear association, McNemar test and logistic regression. Patient Selection The study comprised 354 parents and children aged 4–11 years with moderate-severe asthma; 129 (36.4%) were treated by 23 pediatricians; 225 (63.6%) by 11 pediatric pulmonologists. Results The C-ACT was generally found valid in assessing asthma control (P

A multi-disciplinary scientific conference focused on diffuse and interstitial lung diseases in children was held in La Jolla, CA in June 2012. The conference brought together clinicians (including Pediatric and Adult Pulmonologists, Neonatologists, Pathologists, and Radiologists), clinical researchers, basic scientists, government agency representatives, patient advocates, as well as children affected by diffuse lung disease (DLD) and their families, to review recent scientific developments and emerging concepts in the pathophysiology of childhood DLD. Invited speakers discussed translational approaches, including genetics and proteomics, epigenetics and epigenomics, models of DLD, including animal models and induced pluripotent stem cells, and regenerative medicine approaches. The presentations of the invited speakers are summarized here.

Objective To examine cystic fibrosis (CF) physician adherence to the 2007 CF Foundation (CFF) Pulmonary Guidelines for Chronic Medications. Specifically adherence and barriers to prescribing level A medication recommendations (i.e., inhaled tobramycin and dornase alfa) and level B medication recommendations (i.e., macrolide antibiotics and hypertonic saline) were studied. Methods During Spring 2010, the CFF emailed survey invitations to directors of 136 accredited CF care centers treating 50+ CF patients. Directors were asked to forward the invitations to their physician colleagues. One hundred thirty-three surveys were included in the analyses, representing 92 centers. Barriers were conceptualized based on Cabana et al.'s framework for adherence to guidelines. Adherence was assessed via a case vignette. Results Logistic regression analysis revealed that higher outcome expectancy (OR = 1.099, CI 1.010–1.196) and fewer environmental/system barriers (OR = 1.484, CI 1.158–1.902) were significantly associated with Vignette Adherence. A trend for an association between Familiarity and Vignette Adherence (OR = 1.642, CI 0.953–2.828) was evident, while no demographic variables were significantly associated with Vignette Adherence. Conclusion Targeting outcome expectancy and external barriers with multifaceted, ongoing interventions may improve guideline adherence. Pulmonologists are clearly looking for empirical evidence that these medications benefit their patients over the long-term and offset patient treatment burden with improved health. Pediatr Pulmonol. 2012; 47:434–440. © 2012 Wiley Periodicals, Inc.

The ethics of invasive mechanical ventilation for children with the neurodegenerative disease Spinal Muscular Atrophy Type I (SMA I) is highly debated, and wide variability in clinical outcomes exists internationally. We conducted this international survey to identify physician characteristics associated with recommendation for tracheostomy and ventilation for SMA I. A cross-sectional online survey was distributed to 1,772 pediatric pulmonologists and pediatric intensivists from online membership directories of American Thoracic Society, American College of Chest Physicians, and European Respiratory Society. Questions explored physician demographics, attitudes and experience with SMA and end-of-life care, knowledge of consensus guidelines, and recommendations for respiratory care of SMA I. A logistic regression model assessed the independent effects of physician variables on the recommendation for invasive ventilation for SMA I. A total of 367 (21%) physicians completed the survey; 82% were pediatric pulmonologists; and 16% pediatric intensivists. Seventy percent of respondents were from the U.S. Fifty percent of physicians were aware of SMA consensus guidelines. Physicians from Commonwealth countries (U.K., Canada, Australia, etc.) were less likely to recommend tracheostomy/ventilation than U.S. physicians (7% vs. 25%, P = 0.005). Logistic regression modeling identified years of experience, pediatric pulmonology specialty, agreement with a pro-life statement, and recommendation for non-invasive ventilation as predictive of recommendation for long-term invasive ventilation for SMA I. In the largest international survey on this topic, we identified regional differences in physician recommendation for invasive ventilation for children with SMA I. Our data demonstrate a need for increased awareness of consensus guidelines and further dialog about the physician role in variability of care for children with SMA I.

Background Flexible bronchoscopy (FB) is the gold standard method of diagnosing tracheomalacia but it is not always feasible in settings with limited resources. Fluoroscopy is sometimes performed as an alternative diagnostic tool but there are no prospective studies that have evaluated the diagnostic accuracy of airway fluoroscopy for tracheomalacia using a-priori definitions. We determined the sensitivity, specificity, and likelihood predictive ratio of airway fluoroscopy compared with FB in children suspected of having an airway abnormality. Methods: Airway fluoroscopic examination was undertaken within 2-weeks of a FB in children aged < 18-years and reported by a pediatric radiologist blinded to FB data. Fluoroscopic and FB methods and diagnostic criteria were standardized and defined a-priori. Tracheomalacia diagnosed by FB were independently scored (mild, moderate, severe) by 2 pulmonologists in a blinded manner. Results: In 22 children (median age 33 months, range 1-187) evaluated for airway abnormality, tracheomalacia was found in 21 children at bronchoscopy. Of these, fluoroscopy detected tracheomalacia in five children. Airway fluoroscopy was poorly sensitive (23.8%) but highly specific (100%), positive likelihood ratio was 8.6. However, in moderatesevere tracheomalacia, the sensitivity improved to 57.1% but the specificity reduced (93.3%). The agreement between bronchoscopists for tracheomalacia severity was excellent, weighted kappa 0.74 (95% CI 0.77, 0.98). Conclusion: Airway fluoroscopy cannot replace FB which remains the tool for definitively diagnosing airway malacia. However, in absence of other modalities for diagnosis fluoroscopy should be considered in the setting of persistent respiratory symptoms compatible with the clinical picture of tracheomalacia. Pediatr Pulmonol. 2012; 47: 63-67. (C) 2011 Wiley Periodicals, Inc.

Summary. Many patients with pediatric neuromuscular diseases (NMDs) are now achieving prolonged survival through advances in management of the cardiopulmonary complications of their illnesses. Because respiratory complications are among the main causes of mortality and morbidity in these diseases, pulmonologists are in a unique position to observe and describe the largely unanticipated medical, social, and ethical problems generated when patients with progressive NMDs achieve prolonged survival. For example, prolonged survivors of pediatric NMDsarenowexperiencingpreviouslyrareorunknownmedicalcomplications,anunprecedented severity of burden of disease and the potential for prolonged impairment of quality of life. As the patients age, their families must cope with a high level of burden of care. Society’s acceptance of theeligibilityofthesepatientstoutilizecriticalcareresources,andissuesrelatedtothetransitionof prolongedsurvivorsfrompediatrictoadultmedicalprovidersandvenueshaveresultedincomplex practical and ethical issues. In this article, the author, a pediatric pulmonologist closely involved in the care of patients with NMDs, will identify and discuss some of the major medical, social, and ethical implications of prolonged survival among these patients, with an emphasis on Duchenne muscular dystrophy (DMD), the most common of the pediatric NMDs. Pediatr Pulmonol. 2006

We report the unusual case of a child referred for persistent pneumonia and possible pulmonary nodules who was found to have a cardiac fibroma and diagnosed with Gorlin syndrome. Pulmonologists should be aware of the potential for vascular anomalies associated with this syndrome as well as the risk for pulmonary metastases from primary skin tumors.

Spinal muscular atrophy with respiratory distress (SMARD1) is a rare autosomal recessive disease that is caused by mutations in the gene encoding for immunoglobulin µ-binding protein 2. Unlike the relatively more common spinal muscular atrophy, patients with SMARD1 have respiratory distress prior to manifestation of distal muscle weakness making the diagnosis a challenge. Because respiratory distress is a hallmark feature of this disease, pediatric pulmonologists should recognize its features, and consider it as a diagnostic entity. Pediatr. Pulmonol. 2011; 46:817–819. © 2011 Wiley-Liss, Inc.

This study was designed to achieve a final modeling, validation, and standardization plan for the Wisconsin cystic fibrosis (CF) chest radiographic scoring system. Sixty chest radiographs were selected to reflect a range of severity of lung pathology in children with CF. Seven experienced volunteer raters (three radiologists and four pediatric pulmonologists) from five institutions were recruited to evaluate and score the films. Analysis of scores revealed that the subcomponents of the Wisconsin system showed considerable variation from rater to rater, but reliability assessment indicated satisfactory Cronbach's alpha coefficients (0.83-0.90) among the seven raters. It was found that an additive method of total score computation is significantly more reliable (P < 0.05) than either the original multiplicative model or the traditional Brasfield scoring system. Comparison of radiologists and pulmonologists revealed a marked, systematic difference in scoring with the former group being more conservative in interpretation of abnormalities than the pulmonologists, and some of the raters showing very limited sensitivity. Quantitative chest radiology applied to children with cystic fibrosis studied long-term in longitudinal research projects requires the careful use of sensitive scoring methods and careful selection and training of multiple raters. This is particularly important since pulmonologists and radiologists can differ systematically in interpreting/scoring abnormalities.

In 1996, the Future of Pediatric Education (FOPE) Project of the American Academy of Pediatrics (AAP) developed surveys to describe the nature of pediatric practices, recent trends in clinical practice, and anticipated workforce needs for both pediatric generalists and pediatric sub-specialists. A survey was specifically developed to describe the features of pediatric pulmonology as self-reported by pediatric pulmonologists. The survey was distributed to members of the AAP Pulmonology Section, the Pediatric Assembly of the American Thoracic Society, and certified pediatric pulmonologists recognized by the American Board of Pediatrics. Of the 535 respondents (67% of those invited to respond), the responses of 388 certified and 94 trained but not board-certified pulmonologists were included in the results. The characteristics of certified and non-certified respondents were the same for most survey questions. Clinical activities occupy 73 +/- 29% of professional time. Most pulmonologists work in urban, inner city, or suburban settings and 85% are affiliated with a medical school. One third are in private practice. As a group, research activities occupy less than 15% of their time. Most pediatric pulmonologists maintain a referral practice and use physician extenders to provide care. Patients with asthma and cystic fibrosis comprise 60-70% of patient volume. Both the volume and complexity of patients are increasing, as is competition for pediatric sub-specialty services. Pediatric pulmonary practices vary in size and in volume of patients that they manage in various settings. Forty percent of respondents identify allergists and other pediatric pulmonologists as sources of competition. Sixty-nine percent of respondents do not believe that there is a current need for additional pediatric pulmonologists in their respective communities. Only 15% of respondents plan to retire in the next decade.

Few studies have addressed use of written treatment plans (WTPs) for asthma by specialist physicians. The purpose of this study is to characterize the attitudes, beliefs, and self-reported practice behaviors regarding asthma WTP use among specialist physicians. Structured interviews were conducted with pulmonologists and allergists who provide direct patient asthma care in two New York City medical centers. The interview covered five areas: (1) demographic information; (2) experiences with WTPs; (3) reported clinical practice behaviors; (4) factors influencing use of WTPs; and (5) physician-patient communication. Forty-five physicians were eligible to participate in the study. Sixty-eight percent of physicians treated adult patients while 32% were pediatric specialists. Forty-four physicians completed interviews, (response rate of 98%). Eighty-six percent indicated they use WTPs with at least some of their patients (71% of their patients had received a plan from them). Most reported handwriting plans on a blank piece of paper (66%). Most plans were symptom-based (47%) or combined symptoms with peak flow measurement (50%). Most plans supported patient autonomy. More than 80% of physicians believe the use of a WTP improves patient outcomes. The results suggest that asthma specialists in this survey utilize WTPs more frequently than reported in other studies. Physicians encourage patient autonomy and believe asthma self-management by patients improves their outcomes. Controlled studies of the efficacy of asthma management plans are needed to assess the impact of WTPs as used in clinical practice.

We present a patient that is the index case of pulmonary arterial hypertension (PAH) in a child due to the bone morphogenetic protein type II (BMPR2) missense mutation p.K230N, also known as c.690A>T. Missense mutations typically have earlier onset and more severe disease in PAH, so pulmonologists should be aware of this in the evaluation of PAH in children.

The defense of the respiratory system against infection involves a complex interplay of the humoral immune system and the cellular immune system with complement and phagocytic cells. The respiratory tract is an important portal of entry for infection, and it is therefore not surprising that many immune deficiencies present initially with a respiratory infection. Thus, pediatric pulmonologists must be familiar with the pulmonary manifestation of immune deficiency. In this article we review current concepts concerning pulmonary disease in children with primary immune deficiency diseases and acquired immune deficiency syndrome (AIDS).