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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.

Objectives: To correlate the clinical severity of laryngomalacia (LM) with endoscopic findings, swallowing evaluations and polysomnography in a cohort of patients . Methods: We conducted a retrospective analysis between 2017-2018 on a cohort of patients diagnosed with upper airway obstruction (UAO), stridor, noisy breathing or laryngomalacia. This study took place at the Pediatric Pulmonology Department, Riley Children’s Hospital, Indianapolis, United States of America. Results: There were 157 patients with laryngomalacia included in the study. Patients with severe LM were significantly younger than those with mild LM (p=0.0214) and moderate LM (p=0.0220). Subjects with type I of LM were significantly older than type III (p=0.0051). When associations were tested between polysomnogram (PSG) variables and clinical severity, there were significant associations with age at PSG. The overall apnea-hypopnea index (AHI) in mild (p=0.0103) and moderate (p=0.0242) were significantly lower than the severe group. The rapid eye movement (REM) AHI was significantly lower in moderate cases than severe (p=0.0134). The end-tidal carbon dioxide (EtCO2) peak was significantly lower in mild cases than severe (p=0.0141). The total sleep time (TST) peripheral capillary oxygen saturation (SpO2) 90% occurs in both mild (p=0.0197) and moderate (p=0.0498)) were significantly lower than the severe group. Conclusions: The severity of the clinical manifestations of LM did not correlate with the different endoscopic types in our study. The presence of cyanosis was associated with type III LM. Rapid eye movement AHI and EtCO2 in polysomnogram were remained significantly associated with clinical severity.

Rationale Sputum and bronchoalveolar lavage fluid (BALF) are often obtained to elucidate the lower airway microbiota in adults. Acquiring sputum samples from children is difficult and obtaining samples via bronchoscopy in children proves challenging due to the need for anesthesia and specialized procedural expertise; therefore nasopharyngeal (NP) swabs are often used as surrogates when investigating the pediatric airway microbiota. In adults, the airway microbiota differs significantly between NP and BALF samples however, minimal data exist in children. Objectives To compare NP and BALF samples in children undergoing clinically indicated bronchoscopy. Methods NP and BALF samples were collected during clinically indicated bronchoscopy. Bacterial DNA was extracted from 72 samples (36 NP/BALF pairs); the bacterial V1-V3 region of the 16S rRNA gene was amplified and sequenced on the Illumina Miseq platform. Analysis was performed using mothur software. Results Compared to NP samples, BALF had increased richness and diversity. Similarity between paired NP and BALF (intra-subject) samples was greater than inter-subject samples (P = 0.0006). NP samples contained more Actinobacteria (2.2% vs 21%; adjusted P = 1.4 × 10-6 ), while BALF contained more Bacteroidetes (29.5% vs 3.2%; adjusted P = 1.2 × 10-9 ). At the genus level several differences existed, however Streptococcus abundance was similar in both sample types (NP 37.3% vs BAL 36.1%; adjusted P = 0.8). Conclusion Our results provide evidence that NP samples can be used to distinguish differences between children, but the relative abundance of organisms may differ between the nasopharynx and lower airway in pediatric patients. Studies utilizing NP samples as surrogates for the lower airway should be interpreted with caution.

Ready access to physiologic measures, including respiratory mechanics, lung volumes, and ventilation/perfusion inhomogeneity, could optimize the clinical management of the critically ill pediatric or neonatal patient and minimize lung injury. There are many techniques for measuring respiratory function in infants and children but very limited information on the technical ease and applicability of these tests in the pediatric and neonatal intensive care unit (PICU, NICU) environments. This report summarizes the proceedings of a 2011 American Thoracic Society Workshop critically reviewing techniques available for ventilated and spontaneously breathing infants and children in the ICU. It outlines for each test how readily it is performed at the bedside and how it may impact patient management as well as indicating future areas of potential research collaboration. From expert panel discussions and literature reviews, we conclude that many of the techniques can aid in optimizing respiratory support in the PICU and NICU, quantifying the effect of therapeutic interventions, and guiding ventilator weaning and extubation. Most techniques now have commercially available equipment for the PICU and NICU, and many can generate continuous data points to help with ventilator weaning and other interventions. Technical and validation studies in the PICU and NICU are published for the majority of techniques; some have been used as outcome measures in clinical trials, but few have been assessed specifically for their ability to improve clinical outcomes. Although they show considerable promise, these techniques still require further study in the PICU and NICU together with increased availability of commercial equipment before wider incorporation into daily clinical practice.

Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.

We report the first case of a previously healthy term 5 week old infant with no known risk factors who developed a frontal lobe abscess from community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA). To date, only 16 cases of invasive CA-MRSA with central nervous system involvement have been previously described, but the incidence of CA-MRSA has surged within the last decade, creating a growing impact on public health. Increasing numbers of invasive infections caused by CA-MRSA in previously healthy individuals is of particular concern, and the highest rates of these infections are in children. As CA-MRSA continues to spread and develop further antibiotic resistance, cases of invasive diseases as described in this report may become more common.

Summary Background Data supporting the clinical use of infant lung function (ILF) tests are limited making the interpretation of clinical ILF measures difficult. Objectives To evaluate current ILF testing practices and to survey users regarding the indications, limitations and perceived clinical benefits of ILF testing. Methods We created a 26-item survey hosted on the European Respiratory Society (ERS) website between January and May 2010. Notifications were sent to members of the ERS, American Thoracic Society and the Asian Pacific Society of Respirology. Responses were sought from ILF laboratory directors and pediatric respirologists. The survey assessed the clinical indications, patient populations, equipment and reference data used, and perceived limitations of ILF testing. Results We received 148 responses with 98 respondents having ILF equipment and performing testing in a clinical capacity. Centers in North America were less likely to perform ≥50 studies/year than centers in Europe or other continents (13% vs. 41%). Most respondents used ILF data to either “start a new therapy” (78%) or “help decide about initiation of further diagnostic workup such as bronchoscopy, chest CT or serological testing” (69%). Factors reported as limiting clinical ILF testing were need for sedation, uncertainty regarding clinical impact of study results and time intensive nature of the study. Conclusions Clinical practices associated with ILF testing vary significantly; centers that perform more studies are more likely to use the results for clinical purposes and decision making. The future of ILF testing is uncertain in the face of the limitations perceived by the survey respondents. Pediatr Pulmonol. 2014; 49:126–131. © 2013 Wiley Periodicals, Inc.

Whether defined as chronically critically ill, long-term mechanical ventilator dependent (or otherwise chronically medically supported), or medically fragile, a population of infants and children with chronic illness clearly exists. Infants and children with chronic healthcare needs are at an increased risk for physical, developmental, behavioral, and/or emotional conditions and generally require healthcare services of a type or amount beyond that of a general pediatric or adult population. This review will focus on the specific management and psychosocial needs associated with the healthcare of this subgroup of infants and children with chronic illness. Attention will be paid to defining the population, describing trends over time, reviewing their special needs, and discussing outcomes. Increased focus and an increasing quantity of resources for this subgroup of infants and children are needed, as the number of such pediatric patients continues to grow.

BACKGROUND: Respiratory failure and acute respiratory distress syndrome secondary to H1N1 influenza infection is a source of substantial morbidity and mortality, having caused over 265,000 hospitalizations in the United States in 2009. During the H1N1 pandemic, up to 31% of the H1N1 patients required intensive care unit admission, and many were refractory to maximal conventional therapies. These most critically ill patients may require extracorporeal membrane oxygenation (ECMO) for survival. METHODS: We retrospectively reviewed the medical records of the 7 patients with refractory hypoxemia due to H1N1 influenza who were treated with ECMO in our pediatric intensive care unit. RESULTS: Five of the 7 patients survived to hospital discharge. The cohort's mean age was 21 years, and 4 were female. At admission to the pediatric intensive care unit, 6 had at least one comorbid condition, 6 were mechanically ventilated, and one was in shock. All 7 patients were treated with oral oseltamivir, high-frequency oscillatory ventilation, and inhaled nitric oxide prior to ECMO. Five received intravenous steroids, and 2 were treated with compassionate-use intravenous zanamivir. The mean duration of pre-ECMO ventilation was 8.7 days (range 14 h to 25 d). Mean oxygenation index was 50 (range 26–73) at ECMO cannulation. Six received venovenous ECMO, and one received venoarterial ECMO. The mean duration of ECMO was 432 hours (range 192–890 h). CONCLUSIONS: This series suggests that ECMO is a viable treatment for refractory hypoxemia secondary to H1N1 influenza infection in both pediatric and adult patients.

Summary. Background: The relationship between lower airway markers of inflammation and infection with physiologic findings is poorly understood in young children with cystic fibrosis (CF). The goal of this study was to evaluate the association of bronchoalveolar lavage fluid (BALF) markers of infection and inflammation, including mediators linked to airway remodeling, to infant lung function values in young children with CF undergoing clinically indicated bronchoscopy. Methods: Plethysmography and the raised volume rapid thoracoabdominal compression (RVRTC) technique were performed in 16 sedated infants and young children with CF prior to bronchoscopy. BALF was collected and analyzed for pathogen density, cell count, % neutrophils, transforming growth factor beta 1 (TGF-b1), matrix metalloproteinases (MMP), and interleukin-8 (IL-8). Results: There was a significant direct correlation between functional residual capacity (FRC), the ratio of residual volume to total lung capacity (RV/TLC) and FRC/TLC with % neutrophils (P < 0.05). Forced expiratory flows were inversely correlated to % neutrophils (P < 0.01). Lung function parameters did not differentiate those with and without lower airway infection; however, pathogen density directly correlated with FRC and inversely correlated with flows (P < 0.05). In a subset of the population, MMP-2 directly correlated with RV/TLC and inversely correlated with flows (P < 0.05) and TGF-b1 directly correlated with FRC (P < 0.05). Conclusions: Results from this study suggest that lower airway inflammation as well as mediators linked to airway remodeling play an active role in pulmonary deterioration in CF infants and young children undergoing clinically indicated bronchoscopy. Pediatr Pulmonol. 2009; 44:503–511.

In developed countries, bacterial tracheitis is an uncommon cause of acute upper airway obstruction in children, but the majority (57–91%) of patients who present with this illness require invasive mechanical ventilation.[1][1]–[3][2] Bacterial tracheitis is also associated with numerous

The delivery of oxygen is a vital component of patient care within pediatric and neonatal intensive care units. Clinicians are constantly altering the support provided to each patient in an attempt to optimize oxygen delivery as defined by the following equation: $$ {\mathrm{DO}}_2=CO\times \mathrm{arterial}\ {\mathrm{O}}_2\mathrm{content} $$ $$ \mathrm{arterial}\ {\mathrm{O}}_2\mathrm{content}=\left({SaO}_2\ \mathrm{saturation}\times 1.39\ \left(\mathrm{mL}/\mathrm{g}\right)\times Hb\right)+\left(0.003\times {PaO}_2\right) $$ where DO2 represents oxygen delivery; CO, cardiac output; SaO2, arterial oxygen saturation; Hb, hemoglobin; and PaO2, partial pressure of oxygen in arterial blood. Thus, decreased oxygen delivery can result from inadequate cardiac output, low arterial oxygen saturation, inadequate hemoglobin concentration, or low PaO2 as a minor contributor (secondary to the factor of 0.003 in the formula outlined above). In this chapter, we will review the variety of mechanisms by which clinicians can improve the supply of oxygen to the patient to meet the metabolic demands involved with various pathophysiologic processes.

There are numerous malignant conditions that place the pediatric patient at great risk requiring the need of critical care services. Therefore, it is essential that the pediatric critical care provider possess a sound understanding of these conditions such that they may be anticipated, recognized and treated effectively. Of these conditions, perhaps none requires the prompt, well conceived care of a pediatric intensivist as much as a malignant mediastinal mass. A definitive diagnosis must be established balancing the likelihood of a definitive result with the risk of the diagnostic procedure and the associated sedation. A clear understanding of the findings that suggest a patient is at high risk for airway compromise is essential. Hyperleukocytosis is another malignant condition associated with significant morbidity and mortality as a result of leukostasis. Leukostasis is a clinical condition characterized by progressive and potentially severe neurologic or respiratory disease attributable to small vessel infiltration and occlusion by leukemic blasts. Hypercalcemia is another disorder that may be associated with a variety of malignant processes. Although relatively rare in children, it may result in a number of life-threatening conditions including cardiac dysrhythmias, neurologic impairment, and renal failure. Additionally, it is well-established that varying degrees of coagulopathy are present in most patients with advanced malignancy. Perhaps most notable, acute promyelocytic leukemia is associated with such a high incidence of death near the time of presentation secondary to intracranial and pulmonary hemorrhages that it is viewed as a medical emergency. Other forms of leukemia may be associated with other pathophysiological consequences that may also be life-threatening. For example, lactic acidosis resulting from a high rate of glycolysis is a very rare, but life-threatening complication of hematologic malignancies. A heightened sense of alertness for these rare conditions may result in earlier detection, more effective therapy, and better outcomes for these children.

Introduction This is the first published report of a young girl with co-inherited sickle cell-β+ thalassemia and cystic fibrosis. Although a small subset of patients with co-inherited cystic fibrosis and other hemoglobinopathies have been reported, this patient developed early hematologic and pulmonary complications that were more severe than the previous cases. To assess pulmonary co-morbidities, we used infant pulmonary function testing through the raised volume rapid thoracoabdominal compression technique as both an established study of early cystic fibrosis and also as a newer study of mechanism for early sickle cell lung disease. This further serves as the first report of the raised volume rapid thoracoabdominal compression technique to determine raised volume forced expiratory flows and fractional lung volumes in a patient with a hemoglobinopathy. Case presentation A 2-year-old African-American girl with co-inherited cystic fibrosis and sickle cell-β+ thalassemia developed severe hematologic complications (recurrent vaso-occlusive events, hepatic sequestration, and acute chest syndrome) during periods of cystic fibrosis pulmonary exacerbations and weight loss. Because cystic fibrosis and sickle cell-β+ thalassemia both confer distinct patterns of pulmonary disease, infant pulmonary function testing with the raised volume rapid thoracoabdominal compression technique was used to define respiratory pathophysiology and guide treatment options. Infant pulmonary function testing data demonstrated moderate-to-severe lower airways obstruction, moderate air trapping, and no evidence of restrictive lung disease. Conclusions Infant pulmonary function testing with the raised volume rapid thoracoabdominal compression technique guided therapy in this patient with cystic fibrosis and sickle cell-β+ thalassemia. Although this is an original case report on a unique patient, this case highlights the need to evaluate early respiratory pathophysiology in a broader population of young patients with hemoglobinopathies and screen those at risk for early pulmonary co-morbidities.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare and lethal developmental disorder of the lung defined by a constellation of characteristic histopathological features. Non-pulmonary anomalies involving organs of gastrointestinal, cardiovascular, and genitourinary systems have been identified in approximately 80% of patients with ACD/MPV. We have collected DNA and pathological samples from more than 90 infants with ACD/MPV and their family members. Since the publication of our initial report of four point mutations and ten deletions, we have identified an additional thirty eight novel nonsynonymous mutations of FOXF1 (nine nonsense, seven frameshift, one inframe deletion, twenty missense, and one no stop). This report represents an up to date list of all known FOXF1 mutations to the best of our knowledge. Majority of the cases are sporadic whereas four familial cases with three showing maternal inheritance, consistent with paternal imprinting of the gene. Twenty five mutations (60%) are located within the putative DNA binding domain, indicating its plausible role in gene regulation. Five mutations map to the second exon. We identified two additional genic and eight genomic deletions upstream to FOXF1. These results corroborate and extend our previous observations and further establish involvement of FOXF1 in ACD/MPV and lung organogenesis.

Data supporting the clinical use of infant lung function (ILF) tests are limited making the interpretation of clinical ILF measures difficult.To evaluate current ILF testing practices and to survey users regarding the indications, limitations and perceived clinical benefits of ILF testing.We created a 26-item survey hosted on the European Respiratory Society (ERS) website between January and May 2010. Notifications were sent to members of the ERS, American Thoracic Society and the Asian Pacific Society of Respirology. Responses were sought from ILF laboratory directors and pediatric respirologists. The survey assessed the clinical indications, patient populations, equipment and reference data used, and perceived limitations of ILF testing.We received 148 responses with 98 respondents having ILF equipment and performing testing in a clinical capacity. Centers in North America were less likely to perform ≥50 studies/year than centers in Europe or other continents (13% vs. 41%). Most respondents used ILF data to either "start a new therapy" (78%) or "help decide about initiation of further diagnostic workup such as bronchoscopy, chest CT or serological testing" (69%). Factors reported as limiting clinical ILF testing were need for sedation, uncertainty regarding clinical impact of study results and time intensive nature of the study.Clinical practices associated with ILF testing vary significantly; centers that perform more studies are more likely to use the results for clinical purposes and decision making. The future of ILF testing is uncertain in the face of the limitations perceived by the survey respondents.

Extracorporeal membrane oxygenation as a bridge to lung transplantation has traditionally been associated with substantial morbidity and mortality. A major contributor to these complications may be weakness and overall deconditioning secondary to pretransplant critical illness and immobility. In an attempt to address this issue, we developed a collaborative program to allow for active rehabilitation and physical therapy for patients requiring life support with extracorporeal membrane oxygenation before lung transplantation.An interdisciplinary team responded to an acute need to develop a mechanism for active rehabilitation and physical therapy for patients awaiting lung transplantation while being managed with extracorporeal membrane oxygenation. We describe a series of three patients who benefited from this new approach.A quaternary care pediatric intensive care unit in a children's hospital set within an 800-bed university academic hospital with an active lung transplantation program for adolescent and adult patients. PATIENTS, INTERVENTIONS, AND MAIN RESULTS: Three patients (ages 16, 20, and 24 yrs) with end-stage respiratory failure were rehabilitated while on extracorporeal membrane oxygenation awaiting lung transplantation. These patients were involved in active rehabilitation and physical therapy and, ultimately, were ambulatory on extracorporeal membrane oxygenation before successful transplantation. Following lung transplantation, the patients were liberated from mechanical ventilation, weaned to room air, transitioned out of the intensive care unit, and ambulatory less than 1 wk posttransplant.A comprehensive, multidisciplinary system can be developed to safely allow for active rehabilitation, physical therapy, and ambulation of patients being managed with extracorporeal membrane oxygenation. Such programs may lead to a decreased threshold for the utilization of extracorporeal membrane oxygenation before transplant and have the potential to improve conditioning, decrease resource utilization, and lead to better outcomes in patients who require extracorporeal membrane oxygenation before lung transplantation.

Introduction: Acute febrile neutrophilic dermatosis, or Sweet syndrome, is a rare inflammatory disorder in children that presents with cutaneous lesions with dermal neutrophilic infiltrate, fever, and leukocytosis. Extracutaneous manifestations usually include ocular, musculoskeletal, oral, and cent