The US national registry for childhood interstitial and diffuse lung disease: Report of study design and initial enrollment cohort.

INTRODUCTION: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare disorders. The Children's Interstitial and Diffuse Lung Disease Research Network (chILDRN) established a prospective registry to advance knowledge regarding etiology, phenotype, natural history, and management of these disorders. METHODS: This longitudinal, observational, multicenter registry utilizes single-IRB reliance agreements, with participation from 25 chILDRN centers across the U.S. Clinical data are collected and managed using the Research Electronic Data Capture (REDCap) electronic data platform. RESULTS: We report the study design and selected elements of the initial Registry enrollment cohort, which includes 683 subjects with a broad range of chILD diagnoses. The most common diagnosis reported was neuroendocrine cell hyperplasia of infancy, with 155 (23%) subjects. Components of underlying disease biology were identified by enrolling sites, with cohorts of interstitial fibrosis, immune dysregulation, and airway disease being most commonly reported. Prominent morbidities affecting enrolled children included home supplemental oxygen use (63%) and failure to thrive (46%). CONCLUSION: This Registry is the largest longitudinal chILD cohort in the United States to date, providing a powerful framework for collaborating centers committed to improving the understanding and treatment of these rare disorders.

Inhaled antibiotics in children with tracheostomy tubes: A descriptive study.

INTRODUCTION: Respiratory tract infections (RTIs) are common in children with tracheostomy tubes. Anecdotally, inhaled antibiotics are commonly prescribed, although to date there are no studies describing their use in this patient population. The objective of this study was to assess the variability of this practice at a single tertiary care children's hospital. METHODS: All children admitted to our hospital with a tracheostomy tube who were prescribed inhaled antibiotics between 2013 and 2020 were included. Patient characteristics and data regarding inhaled antibiotic use were obtained retrospectively from the electronic medical record. RESULTS: A total of 424 courses of inhaled antibiotics were prescribed during the study period. 296 (69.8%) courses were prescribed to treat an acute RTI, whereas 128 (30.2%) were prescribed prophylactically to prevent RTIs. 58.9% of children with tracheostomy tubes hospitalized during the study period received at least one course of inhaled antibiotics. The most common antibiotics prescribed were tobramycin and gentamicin; several different doses were used. In 53.2% of treatment courses, inhaled antibiotics were co-prescribed with systemic antibiotics. Therapy duration for treatment varied from 3 to 28 days. Respiratory cultures were used variably and antimicrobial susceptibility was often not taken into account when prescribing inhaled antibiotics. CONCLUSIONS: Inhaled antibiotics were frequently prescribed as treatment and prophylaxis in children with tracheostomy tubes at our center, with significant variation in the prescribed antibiotic type, dose, frequency, duration, and co-prescription with systemic antibiotics. Prospective studies are needed to define best practice regarding inhaled antibiotics in this patient population.

Tracheobronchitis in children with tracheostomy tubes: Overview of a challenging problem.

Tracheobronchitis is common in children with tracheostomy tubes. These children are predisposed to respiratory infections due to the bypassing of normal upper airway defense mechanisms by the tracheostomy, bacterial colonization of the tracheostomy tube itself, and underlying medical conditions. Diagnosis of bacterial tracheobronchitis is challenging due to the difficulty in differentiating between bacterial colonization and infection, as well as between viral and bacterial etiologies. Difficulty in diagnosis complicates management decisions, and there are currently no consensus guidelines to assist clinicians in the treatment of these patients. Frequent administration of systemic antibiotics causes adverse effects and leads to the emergence of resistant organisms. Topical administration of antibiotics via nebulization or direct instillation may lead to a significantly higher concentration of drug in the upper and lower airways without causing systemic side effects, although therapeutic trials in children with tracheostomy tubes are lacking. Several preventative measures such as regular airway clearance and the use of a speaking valve may mitigate the risk of developing respiratory infections.

Randomized pilot trial of ipratropium versus placebo in children with critical asthma.

OBJECTIVE: To test the effects of inhaled ipratropium on clinical outcomes of critical asthma in the first randomized trial of this adjunctive therapy in critically ill children. DESIGN: Pilot, placebo-controlled, double-blinded, and randomized-controlled trial PATIENTS: Thirty children (15 per group) with critical asthma receiving high-intensity albuterol per a standardized pathway utilizing objective assessments to wean patients to less frequent albuterol administration. INTERVENTIONS: Subjects were randomized to receive either nebulized ipratropium bromide (500 µg in 0.9% saline per dose) or an equivalent volume of nebulized 0.9% saline every 6 h until the patient was successfully weaned to albuterol doses every 2 h ("q2 albuterol"). MEASUREMENTS AND MAIN RESULTS: Demographics, initial clinical severity score, and asthma histories were similar between groups. There was no significant difference in the median duration of high-intensity albuterol between the treatment group (17.5 [10.3-22.1] h) and placebo group (14.6 [12.7-24.5] days; p = .56). Similarly, there was no significant difference in pediatric intensive care unit length of stay (22.6 [21.1-33.6] vs. 21.4 [16.1-35.8] h; p = .74) or hospital length of stay (48.0 [41.8-59.8] vs. 47.3 [37.2-63.1] h; p = .67). In multivariate linear regression adjusting for identified confounders, treatment with ipratropium was not significantly associated with any of the three outcomes. Side effects were rare and occurred with equally between both groups CONCLUSIONS: Adjunctive therapy with ipratropium was not associated with decreased duration of high-intensity albuterol or shortened length of stay when compared to placebo. A larger, multicenter trial is warranted to confirm that ipratropium does not improve clinical outcomes.

Obstructive sleep apnea and sickle cell anemia.

OBJECTIVE: To ascertain the prevalence of and risk factors for obstructive sleep apnea syndrome (OSAS) in children with sickle cell anemia (SCA). METHODS: Cross-sectional baseline data were analyzed from the Sleep and Asthma Cohort Study, a multicenter prospective study designed to evaluate the contribution of sleep and breathing abnormalities to SCA-related morbidity in children ages 4 to 18 years, unselected for OSAS symptoms or asthma. Multivariable logistic regression assessed the relationships between OSAS status on the basis of overnight in-laboratory polysomnography and putative risk factors obtained from questionnaires and direct measurements. RESULTS: Participants included 243 children with a median age of 10 years; 50% were boys, 99% were of African heritage, and 95% were homozygous for ß(S) hemoglobin. OSAS, defined by obstructive apnea hypopnea indices, was present in 100 (41%) or 25 (10%) children at cutpoints of ≥1 or ≥5, respectively. In univariate analyses, OSAS was associated with higher levels of habitual snoring, lower waking pulse oxygen saturation (Spo2), reduced lung function, less caretaker education, and non-preterm birth. Lower sleep-related Spo2 metrics were also associated with higher obstructive apnea hypopnea indices. In multivariable analyses, habitual snoring and lower waking Spo2 remained risk factors for OSAS in children with SCA. CONCLUSIONS: The prevalence of OSAS in children with SCA is higher than in the general pediatric population. Habitual snoring and lower waking Spo2 values, data easily obtained in routine care, were the strongest OSAS risk factors. Because OSAS is a treatable condition with adverse health outcomes, greater efforts are needed to screen, diagnose, and treat OSAS in this high-risk, vulnerable population.

Wheezing symptoms and parental asthma are associated with a physician diagnosis of asthma in children with sickle cell anemia.

OBJECTIVE: To identify factors associated with asthma associated with increased sickle cell anemia (SCA). STUDY DESIGN: Children with SCA (N = 187; mean age 9.6 years, 48% male) were classified as having "asthma" based on parent report of physician diagnosis plus prescription of asthma medication (n = 53) or "no asthma" based on the absence of these features (n = 134). Pain and acute chest syndrome (ACS) events were collected prospectively. RESULTS: Multiple variable logistic regression model identified 3 factors associated with asthma: parent with asthma (P = .006), wheezing causing shortness of breath (P = .001), and wheezing after exercise (P < .001). When ≥2 features were present, model sensitivity was 100%. When none of the features were present, model sensitivity was 0%. When only 1 feature was present, model sensitivity was also 0%, and presence of ≥2 of positive allergy skin tests, airway obstruction on spirometry, and bronchodilator responsiveness did not improve clinical utility. ACS incident rates were significantly higher in individuals with asthma than in those without asthma (incident rate ratio 2.21, CI 1.31-3.76), but pain rates were not (incident rate ratio 1.28, CI 0.78-2.10). CONCLUSIONS: For children with SCA, having a parent with asthma and specific wheezing symptoms are the best features to distinguish those with and without parent report of a physician diagnosis of asthma and to identify those at higher risk for ACS events. The value of treatment for asthma in the prevention of SCA morbidity needs to be studied.

Complicaciones pulmonares del síndrome de Down durante la infancia / No disponible

Los niños con síndrome de Down se encuentran predispuestos a una amplia variedad de problemas respiratorios que pueden tener su origen en cualquier nivel del aparato respiratorio. Los problemas pulmonares que vemos a menudo consisten en: a) infecciones recurrentes y más graves de las vías respiratorias, b) las anomalías de estas vías, c) la enfermedad vascular pulmonar, d) la enfermedad pulmonar quística, y e) la apnea del sueño (AOS). Todos los problemas corrientes que se asocian con el síndrome de Down, como son la hipotonía, la disfagia y las anomalías de carácter inmune, contribuyen a la aparición de estos problemas respiratorios recurrentes, por lo que su evaluación ha de ser muy tenida en cuenta a la hora de evaluar y tratar a los niños con síndrome de Down cuando presentan estas dificultades respiratorias frecuentes o persistentes. Las cardiopatías congénitas, la patología gastrointestinal o la necesidad de cirugía acrecientan más aún la morbilidad y mortalidad derivadas de la patología respiratoria en estos niños. Las anomalías de la estructura de las vías respiratorias pueden ser complejas y requieren intervención quirúrgica en centros con amplia experiencia. Incluso problemas cuya asociación con el síndrome de Down es bien conocida, como es el caso de la AOS, son más difíciles de tratar que en el resto de la población (AU)

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