Approach to patient with cough by American and Chinese respiratory specialist.

Cough is a common pediatric condition. Acute cough is often considered to be self-limiting and not requiring clinical management. However, pediatric patients and their parents often seek remedies for an acute cough. Traditional Chinese Medicine (TCM) specialists have been treating pediatric cough for millennia. Here we present a case of pediatric cough and the approaches taken to it by a Western and Chinese respiratory specialist. We conclude that TCM may provide important and useful insights into the treatment of such pediatric respiratory disease.

The roles of a pediatric pulmonologist during the COVID-19 pandemic.

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.

Airway manifestations of sarcoidosis in adolescents.

Endobronchial sarcoid lesions have previously been described and visualized upon bronchoscopy in adult patients with pulmonary sarcoid involvement. Endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-TBNA) has come into favor as the preferred method of diagnosis, but it remains a novel technique in pediatric pulmonology. We describe the first two known cases of visualized endobronchial sarcoid lesions in the pediatric population with pathological confirmation of sarcoidosis with endobronchial and EBUS-TBNA biopsies.

Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula.

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on "strong", "moderate" or "weak" agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients' age were also developed.

Airway and esophageal eosinophils in children with severe uncontrolled asthma.

AIM: Children with severe uncontrolled asthma (SUA) have a high burden of symptoms and increased frequency of asthma exacerbations. Reflux esophagitis and eosinophilic esophagitis are important co-morbid factors for SUA. Both are associated with the presence of eosinophils in esophageal mucosa. We hypothesized that esophageal eosinophils are frequently present and correlate with the presence of airway eosinophils in children with SUA. METHOD: We performed a retrospective analysis of a prospective database of children who underwent "triple endoscopy" (sleep laryngoscopy, bronchoscopy with bronchoalveolar lavage [BAL] and endobronchial biopsy [EBB], and esophagogastroduodenoscopy with esophageal biopsy [EsB]) at our Aerodigestive Center for evaluation of SUA. Children with known cystic fibrosis, primary ciliary dyskinesia, and aspiration-related lung disease were excluded. RESULT: Twenty-four children (21 males) ages 2-16 years were studied. Elevated BAL eosinophils were found in 10 (42%) patients, endobronchial eosinophils in 16 (67%); 7 (29%) had endobronchial eosinophils without elevated BAL eosinophils. Esophageal eosinophils were found in 11 (46%) patients. There was a correlation between the amount of eosinophils in BAL and EBB (R = 0.43, P = 0.05) airway eosinophils, defined as elevated BAL and/or EBB eosinophils, correlated with esophageal eosinophils (R = 0.41, P = 0.047). CONCLUSION: We concluded that airway and esophageal eosinophils are frequently present in children with SUA.

Respiratory care in familial dysautonomia: Systematic review and expert consensus recommendations.

BACKGROUND: Familial dysautonomia (Riley-Day syndrome, hereditary sensory autonomic neuropathy type-III) is a rare genetic disease caused by impaired development of sensory and afferent autonomic nerves. As a consequence, patients develop neurogenic dysphagia with frequent aspiration, chronic lung disease, and chemoreflex failure leading to severe sleep disordered breathing. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of respiratory disorders in familial dysautonomia. METHODS: We performed a systematic review to summarize the evidence related to our questions. When evidence was not sufficient, we used data from the New York University Familial Dysautonomia Patient Registry, a database containing ongoing prospective comprehensive clinical data from 670 cases. The evidence was summarized and discussed by a multidisciplinary panel of experts. Evidence-based and expert recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system. RESULTS: Recommendations were formulated for or against specific diagnostic tests and clinical interventions. Diagnostic tests reviewed included radiological evaluation, dysphagia evaluation, gastroesophageal evaluation, bronchoscopy and bronchoalveolar lavage, pulmonary function tests, laryngoscopy and polysomnography. Clinical interventions and therapies reviewed included prevention and management of aspiration, airway mucus clearance and chest physical therapy, viral respiratory infections, precautions during high altitude or air-flight travel, non-invasive ventilation during sleep, antibiotic therapy, steroid therapy, oxygen therapy, gastrostomy tube placement, Nissen fundoplication surgery, scoliosis surgery, tracheostomy and lung lobectomy. CONCLUSIONS: Expert recommendations for the diagnosis and management of respiratory disease in patients with familial dysautonomia are provided. Frequent reassessment and updating will be needed.

Aerodigestive dysbiosis in children with chronic cough.

In pediatric patients with chronic cough, respiratory culture techniques commonly yield negative results. Studies using culture-independent methods have found a high relative abundance of oral microbes in the lower airways, suggesting that the topographical continuity, and dynamics of the intraluminal contents of the aerodigestive system likely influence the lower airway microbiota. We hypothesize that in subjects with chronic cough, clinical diagnosis will correlate with distinct microbial signatures detected using culture-independent methods. STUDY DESIGN AND METHODS: We enrolled 36 pediatric subjects with chronic cough in a cross-sectional study. Subjects were categorized into four clinical groups: asthma, bacterial bronchitis, neurologically impaired-orally fed, and neurologically impaired enterally fed. Samples from the aerodigestive tract were obtained through bronchoscopy and upper endoscopy. 16S rRNA gene sequencing compared the microbiota from bronchoalveolar lavage (BAL), tracheal, supraglottic, esophageal, gastric, and duodenal samples. RESULTS: We observed that the lower airway microbiota of asthma subjects had higher α diversity as compared with the other groups. ß diversity analysis of BAL samples revealed significant differences between the groups. Among the taxonomic differences found, most differentially enriched taxa were upper airway organisms such as Rothia, Gemellaceae (u.g. or uncharacterized genus), and Granulicatella in asthma, Prevotella in bacterial bronchitis, and Veillonella in neurologically impaired orally fed subjects. Greater dissimilarity between the upper airway and lower airway microbiota was associated with increased neutrophilic airway inflammation. CONCLUSIONS: Distinct dysbiotic signatures can be identified in the lower airway microbiota of pediatric subjects with chronic cough that relates to the degree and type of inflammation.

Prevalence and characteristics of sleep-disordered breathing in familial dysautonomia.

OBJECTIVE: Familial dysautonomia (FD) is an autosomal recessive disorder characterized by impaired development of sensory and afferent autonomic nerves. Untreated sleep-disordered breathing (SDB) has been reported to increase the risk of sudden unexpected death in FD. We aimed to describe the prevalence and characteristics of SDB in FD. PATIENTS/METHODS: Seventy-five patients with FD (20 adults and 55 children) underwent in-lab polysomnography, including peripheral capillary oxygen saturation (SpO2) and end-tidal capnography (EtCO2) measurements. A t-test and Spearman's correlation analysis were performed to evaluate the impact of age on sleep, occurrence of apneas, SpO2 and EtCO2 levels; and to determine the relationship between apneas and SpO2/EtCO2 measurements during different sleep stages. RESULTS: Overall, 85% of adults and 91% of pediatric patients had some degree of SDB. Obstructive sleep apneas were more severe in adults (8.5 events/h in adults vs. 3.5 events/h in children, p = 0.04), whereas central apneas were more severe (10.8 vs. 2.8 events/h, p = 0.04) and frequent (61.8% vs. 45%, p = 0.017) in children. Overall, a higher apnea-hypopnea index was associated with increased severity of hypoxia and hypoventilation, although in a significant fraction of patients (67% and 46%), hypoxemia and hypoventilation occurred independent of apneas. CONCLUSION: Most adult and pediatric patients with FD suffer from some degree of SDB. There was a differential effect of age in the pattern of SDB observed. In some FD patients, hypoventilation and hypoxia occurred independently of apneas. Therefore, we recommend including EtCO2 monitoring during polysomnography in all patients with FD to detect SDB.

Structure and Functions of Pediatric Aerodigestive Programs: A Consensus Statement.

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.

Foreign body removal is getting "cooler".

Foreign body removal is commonly performed with the rigid bronchoscope. However, based on recent literature, extraction with the flexible bronchoscope has increased in popularity. Here, we discuss a new tool - the flexible cryoprobe - that may enhance foreign body retrieval in select patients and comment on the methods of foreign body extraction in the view of common clinical practice. Pediatr Pulmonol. 2016; 51:886-888. © 2016 Wiley Periodicals, Inc.

Establishing a Role for Polysomnography in Hospitalized Children.

BACKGROUND: Children with medical complexity have a high prevalence of sleep disorders. However, outpatient polysomnography to evaluate for these conditions may be difficult to perform because of lack of skilled nursing care. The aim of this study was to explore polysomnography indications in hospitalized children and assess its impact on patient care. METHODS: Data from 85 inpatient polysomnographies of 70 children hospitalized between March and December 2014 were retrospectively collected. RESULTS: Sixty percent of patients were boys with ages 6.5 ± 6 years. Chronic respiratory failure was present in 33.8%, airway obstruction due to defects of the tracheobronchial tree or craniofacial abnormalities in 54.3%, neurological complications of the perinatal period in 22.9%, genetic syndromes and neurodegenerative disorders in 31.4%, congenital myopathies in 5.7%, metabolic diseases in 4.3% and congenital cyanotic heart defects in 4.3%. Indications for polysomnography included assessment of chronic pulmonary disease (60%), ventilator requirements (41.2%), apnea/desaturation (23.5%), and acute life-threatening events (1.2%). Abnormal results were found in 89.4%. The observed diagnosis was obstructive sleep apnea in 64.7%, signs of chronic lung disease in 34.1%, hypoventilation in 9.4%, periodic breathing in 3.5%, and periodic limb movement of sleep in 4.7%. The following interventions were performed: adjustment of ventilator parameters (45.8%), positive airway pressure initiation (24.7%), otorhinolaryngology referral (30.6%), supraglottoplasty (2.4%), tracheostomy decannulation (2.4%), and tracheostomy placement (3.5%). Nine patients had available follow-up polysomnograms, all showing improvement in sleep variables after adherence to recommended interventions. CONCLUSIONS: In individuals with complex medical disorders, inpatient polysomnographies give invaluable information to guide immediate medical decision making and should be strongly considered if resources allow this.

Anesthetic Management of an Extremely Premature, Extremely Low-Birth-Weight Infant Undergoing Bronchoscopy for Removal of an Aspirated Foreign Body.

We present a case of an ex-28-week, extremely low-birth-weight infant who was transferred to our institution for bronchoscopically assisted removal of an aspirated foreign body. This case presented several challenges because of the patient's extreme prematurity as well as the need for repeated tracheal extubations and reintubations during the procedure to accommodate surgical instruments in the patient's airway. We discuss the respiratory physiology, common comorbidities, and management of aspirated foreign bodies in the premature infant and emphasize the importance of clear communication in the operating room between the multidisciplinary team of physicians involved in this patient's care.

Bronchoscopic and nonbronchoscopic methods of airway culturing in tracheostomized children.

INTRODUCTION: Distal airway secretions can be sampled by bronchoscopic bronchoalveolar lavage (B-BAL), blind protected BAL (BP-BAL), and tracheal aspiration (TA). We quantitatively compared the cultures of distal airway secretions from BP-BAL, B-BAL, and TA specimens, and assessed the efficacy of the three above methods in diagnosing bronchitis in tracheostomized children. METHODS: Twenty children with tracheostomies underwent BP-BAL, B-BAL, and TA. Samples were sent for quantitative bacterial cultures. The diagnosis of bronchitis was made based on a validated visual grading system as well as on positive quantitative cultures from the BAL fluid. Diagnostic agreement between cultures obtained by the three methods and the visual grading scores was determined by kappa statistics. RESULTS: The diagnosis of bronchitis by visual grading score had substantial agreement with BP-BAL, moderate agreement with B-BAL, and fair agreement with TA results. BP-BAL specimens had significantly lower pathogenic colonies (P < .05) than either B-BAL or TA specimens. CONCLUSIONS: BP-BAL allows for more accurate sampling of lower airway secretions in tracheostomized children and is more accurate in the diagnosis of bronchitis in this group.

High-frequency chest wall compression therapy in neurologically impaired children.

BACKGROUND: Children with neurological impairment often suffer from insufficient airway secretion clearance, which substantially increases their respiratory morbidity. The goal of the study was to assess the clinical feasibility of high-frequency chest wall compression (HFCWC) therapy in neurologically impaired children with respiratory symptoms. METHODS: This was a single-center, investigator initiated, prospective study. Twenty-two subjects were studied for 12 months before and 12 months after initiation of HFCWC therapy, and 15 subjects were followed for an additional 12 months. The threshold of adherence to HFCWC therapy was 70%. The number of pulmonary exacerbations that required hospitalization was recorded. RESULTS: Forty-five percent of the subjects required hospital admission before initiation of HFCWC therapy. This rate decreased to 36% after the first year with HFCWC, and to 13% after the second year with HFCWC. There was a statistically significant reduction of the number of hospital days at follow-up, relative to before treatment. Use of an assisted-cough device or the presence of tracheostomy did not significantly affect hospitalization days. CONCLUSIONS: Regular HFCWC therapy may reduce the number of hospitalizations in neurologically impaired children.

Bronchoscopic findings in children with chronic wet cough.

OBJECTIVES: Protracted bacterial bronchitis is defined as the presence of more than 4 weeks of chronic wet cough that resolves with appropriate antibiotic therapy, in the absence of alternative diagnoses. The diagnosis of protracted bacterial bronchitis is not readily accepted within the pediatric community, however, and data on the incidence of bacterial bronchitis in children are deficient. The objective of this study was to determine the frequency of bacterial bronchitis in children with chronic wet cough and to analyze their bronchoscopic findings. METHODS: We performed a retrospective review of charts of children who presented with chronic wet cough, unresponsive to therapy, before referral to the pediatric pulmonary clinic. RESULTS: A total of 197 charts and bronchoscopy reports were analyzed. Of 109 children who were 0 to 3 years of age, 33 (30.3%) had laryngomalacia and/or tracheomalacia. The bronchoscopy showed purulent bronchitis in 56% (110) cases and nonpurulent bronchitis in 44% (87). The bronchoalveolar lavage bacterial cultures were positive in 46% (91) of the children and showed nontypable Haemophilus influenzae (49%), Streptococcus pneumoniae (20%), Moraxella catarrhalis (17%), Staphylococcus aureus (12%), and Klebsiella pneumoniae in 1 patient. The χ(2) analysis demonstrated that positive bacterial cultures occurred more frequently in children with purulent bronchitis (74, 69.8%) than in children with nonpurulent bronchitis (19, 19.8%) (P < .001). CONCLUSIONS: Children who present with chronic wet cough are often found to have evidence of purulent bronchitis on bronchoscopy. This finding is often indicative of a bacterial lower airway infection in these children.

The effect of surgically created gastroesophageal reflux on intrapleural pressures in dogs.

The causal relationship between gastroesophageal reflux (GER) and respiratory disorders is not well understood. Previous experimental studies that investigated this relationship were performed in anesthetized animals and used artificial acidification of esophagus for simulation of GER. In this study, we investigated the impact of GER on intrapleural pressures (IPP) in conscious, unanesthetized dogs. After the induction of appropriate anesthesia, 5 purpose-bred mongrel dogs underwent reflux-creating surgery (partial cardiomyectomy). The presence of GER was confirmed by determining the reflux index (RI) and the duration of longest reflux episode (DLRE) after 24-h intraesophageal pH-metry. IP was monitored before and after cardiomyectomy using a subcutaneously placed telemetric implant with its pressure-sensor catheter tip inserted into the pleural space. Partial cardiomyectomy resulted in a significant increase in RI from a preoperative mean value of 0.38 +/- 0.21 to 7.52 +/- 2.56%, and DLRE from 1.22 +/- 1.12 to 36.80 +/- 12.71 min, as recorded by the proximal sensor of the pH probe. A similar trend was observed at the distal sensor. After cardiomyectomy, the negative inspiratory IPP significantly increased from 17.2 +/- 7.9 to 28.4 +/- 9.7 mm Hg. A similar effect was observed in the negative expiratory IPP. The negative inspiratory IPP had a significant correlation with both RI (R = 0.932) and DLRE (R = 0.899). Cardiomyectomy causes GER, the severity of which correlates with negative inspiratory IPP in a dog model. The suggested model allows for the investigation of the pathologic association of GER with respiratory disorders in conscious animals.

Lipid laden macrophage indices and reflux finding score in canine gastroesophageal reflux model.

UNLABELLED: Laryngeal exposure to acid and aspiration of gastric contents may lead to severe respiratory disorders. This study utilizes the canine model of Gastroesophageal reflux (GER) to identify whether lower esophageal dysfunction is associated with upper and lower airway pathology. MATERIALS AND METHODS: Five mongrel dogs underwent GER-creating surgery (partial cardiomyectomy). Laryngeal reflux finding score (RFS), lipid-laden macrophage index (LLMI) and BAL fluid cell differential were obtained before and after surgery. RESULTS: Partial cardiomyectomy in dogs significantly increased the Reflux index (RI) from 0.38 +/- 0.21% to 7.56 +/- 2.89% (P = 0.048), the duration of the longest reflux episode (DLRE) from 1.22 +/- 1.19 min to 66.2 +/- 42.03 min postoperatively (P = 0.049) and the total number of episodes in 24 hr from 2.06 +/- 1.03 to a postoperative value of 19.24 +/- 4.79. There was no statistically significant change in values for RFS, LLMI, and BAL fluid cell differential after the induction of GER. CONCLUSIONS: Acid reflux to the proximal esophagus of this animal model did not cause laryngeal exposure to acid or aspiration of gastric content. The results of this study suggest that presence of GER, secondary to lower esophageal dysfunction is not necessarily associated with upper and lower airway pathology.

Telemetric recording of intrapleural pressure.

BACKGROUND: Monitoring of intrapleural pressure (IPP) is used for evaluation of lung function in a number of pathophysiological conditions. We describe a telemetric method of non-invasive monitoring of the IPP in conscious animals intermittently or continuously for a prolonged period of time. MATERIALS AND METHODS: After IACUC approval, six mongrel dogs were used for the study. After sedation, each dog was intubated and anesthetized using 0.5% Isoflurane. A telemetric implant model TL11M2-D70-PCT from Data Science International was secured subcutaneously. The pressure sensor tip of the catheter from the implant was inserted into the pleural space, and the catheter was secured with sutures. The IPP signals were recorded at a sampling rate of 100 points/second for 30 to 60 min daily for 4 days. From these recordings, the total mean negative IPP (mmHg), and the total mean negative IPP for a standard time of 30 min were calculated. In addition, the actual inspiratory and expiratory pressures were also measured from stable recording of the IPP waveforms. RESULTS: In six dogs, the total mean +/- SD negative IPP was -10.8 +/- 10.6 mmHg. After normalizing with respect to acquisition time it was -13.2 +/- 11.2 mmHg/min. The actual inspiratory pressure was -19.7 +/- 15.3, and the expiratory pressure was -11.0 +/- 12.9. CONCLUSIONS: Our study demonstrates that telemetric monitoring of IPP can be performed reliably and non-invasively in conscious experimental animals. The values for IPP in our study are compatible with the results of other investigators who used different methods of IPP measurement. Further work may show this method to be helpful in understanding the pathophysiology of various breathing disorders.