Faculty Bibliography

OBJECTIVES/OBJECTIVE:To compare lung function in a representative sample of World Trade Center (WTC)-exposed children with matched comparisons, and examine relationships with reported exposures. STUDY DESIGN/METHODS:Study population consisted of 402 participants. Oscillometry, spirometry, and plethysmography were performed on WTC Health Registry (WTCHR) respondents who were ≤8 years of age on September 11, 2001 (n = 180) and a sociodemographically matched group of New York City residents (n = 222). We compared lung function by study arm (WTCHR and comparison group) as well as dust cloud (acute); home dust (subchronic); and other traumatic, nondust exposures. RESULTS:In multivariable models, post-9/11 risk of incident asthma was higher in the WTCHR participants than in the comparison group (OR 1.109, 95% CI 1.021, 1.206; P = .015). Comparing by exposure rather than by group, dust cloud (OR 1.223, 95% CI 1.095, 1.365; P < .001) and home dust (OR 1.123, 95% CI 1.029, 1.226; P = .009) exposures were also associated with a greater risk of incidence of post-9/11 asthma. No differences were identified for lung function measures. CONCLUSIONS:Although we cannot exclude an alternative explanation to the null findings, these results may provide some measure of reassurance to exposed children and their families regarding long-term consequences. Further study with bronchodilation and/or methacholine challenge may be needed to identify and further evaluate effects of WTC exposure. Biomarker studies may also be more informative in delineating exposure-outcome relationships. TRIAL REGISTRATION/BACKGROUND:ClinicalTrials.gov: NCT02068183.

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.

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.

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/METHODS: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/CONCLUSIONS:monitoring during polysomnography in all patients with FD to detect SDB.

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.

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 patients with medical complexity, inpatient polysomnographies give invaluable information to guide immediate medical decision making and should be strongly considered if resources allow this.

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.

INTRODUCTION:: Distal airway secretions can be sampled by bronchoscopic bronchoalveolar lavage (B-BAL), blind protected BAL (BP-BAL) and tracheal aspirates (TA). We quantitatively compared the cultures of distal airway secretions from BP-BAL, B-BAL, and TA and assessed the efficacy of the three above methods in diagnosing bronchitis in tracheostomized children. METHOD:: Twenty children with tracheostomies underwent BP-BAL, B-BAL and TA. Samples were sent for quantitative bacterial cultures. Diagnosis of bronchitis was made based on a validated visual grading system as well as on positive quantitative culture in the BAL fluid. Diagnostic agreement between cultures obtained by the three methods and the visual grading scores was determined by kappa statistics. RESULTS:: Diagnosis of bronchitis by visual grading score had substantial agreement with BP-BAL, moderate agreement with B-BAL, and fair agreement with TA. BP-BAL had significantly lower pathogenic colonies (P<0.05) than either B-BAL or TA. CONCLUSION:: 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.

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 patients were studied for 12 months before and 12 months after initiation of HFCWC therapy, 15 patients were followed up for additional 12 months. The threshold of compliance with HFCWC therapy was 70%. The amounts of pulmonary exacerbations, which required hospitalization, were recorded. RESULTS:: Forty five percent of study patients required hospital admission before initiation of HFCWC therapy. This rate decreased to 36% after the first year of treatment and to 13% after the second year of treatment. There was a statistically significant reduction of the number of hospital days at follow-up relative to before treatment. Use of either the cough assist device or tracheostomy did not significantly interact over time with hospitalization days. CONCLUSIONS:: Prolonged HFCWC therapy may reduce the number of hospitalizations in neurologically impaired children.