Pleuroparenchymal fibroelastosis in an 8-year old treated for neuroblastoma.

Pleuroparenchymal fibroelastosis (PPFE), which is primarily diagnosed in adults, is a progressive lung pathology associated with significant morbidity and mortality. PPFE is characterized by pleural and subpleural parenchymal disease causing dyspnea, cough, and recurrent pneumothoraces. PPFE can be precipitated by autoimmune disorders, recurrent respiratory infections, chemotherapy, and transplant. We describe the youngest recorded patient to develop PPFE, whose symptoms began several years after treatment for neuroblastoma. Her symptoms were initially mistaken for worsening asthma, and multiple comorbidities developed during the prolonged time to recognition of PPFE and she progressed to fatal lung disease before potentially curative lung transplantation could occur.

Flexible Bronchoscopy Under Bronchoscopist-Administered Moderate Sedation Versus General Anesthesia: A Comparative Study in Children.

Background: Flexible bronchoscopy (FB) can be performed under bronchoscopist administered moderate sedation (BAMS) with a midazolam/fentanyl combination or general anesthesia (GA). However, the outcome of BAMS has not been well established in children. Currently, most of the centers prefer FB under GA. Both techniques have their advantages and disadvantages with implications for safety, complications, and diagnostic yield. The primary objective of our study was to evaluate the safety, time efficiency, and cost-effectiveness of FB under BAMS as compared with FB under GA in a similar setting. Methods: We performed a retrospective chart review to compare BAMS versus GA for FB in children. We recruited BAMS children (n = 295) from University of Florida (UF) Health Shands Children's Hospital, and GA children (n = 100) from Penn State Children's Hospital (PSHCH). Both the groups had similar indications, complexities, and procedural environments. Comparisons of various time-intervals including preprocedure time, sedation-induction time, scope time, and post-procedure time among different BAMS versus GA age-groups were the primary outcomes. The secondary outcomes were the determination of the rates of complications, the dosages of sedative/anesthetic, cost-effectiveness, and sedation patterns under BAMS. Results: FB under BAMS required significantly higher preprocedure times and sedation-induction times (P < 0.001** and P < 0.001** respectively) but shorter scope and post-procedure times compared with the GA group times (P < 0.001** and P < 0.001** respectively). Younger children had a deeper level of sedation for an extended period under BAMS. The costs for the sedation services and the complication rates were lower in the BAMS group compared with the GA group. Conclusion: Our study demonstrated the feasibility of BAMS in children. FB under BAMS had an advantage of lower cost and fewer procedural complications compared with FB under GA. Despite that, the safety of BAMS could not be conclusively established from this retrospective study. Moreover, BAMS can potentially compromise the diagnostic yield because the bronchoscopist is also responsible for monitoring sedation and managing the airway.

Optimizing an Internal Airway Percussion Device for Facilitating Exhalate Diagnostics of the Human Respiratory System.

PURPOSE: There is an urgent need for simple, inexpensive, noninvasive, and repeatable technique for the diagnosis of pulmonary diseases. Bronchoalveolar lavage, which is the gold standard diagnostic method for pulmonary diseases, does not meet any of these criteria. This study seeks to develop and optimize a novel technique of Internal Airway Percussion (IAP) to facilitate the collection and characterization of human respiratory system exhalates. METHODS: The IAP device transmits sound waves into the respiratory tract, thereby increasing the release of aerosolized particles within exhaled breath by vibrating both lungs. Nine combinations of sound wave frequencies and amplitudes were studied to determine optimal frequency and amplitude combination for maximum aerosol particle gain in healthy human subjects. RESULTS: Square-shaped sound waves generated at 15 Hz and 3 cm H2O resulted in 15 times greater total mass of collected particles in the first 2 min of sampling, and 1.2 to 1.5 times increase in count median diameter of the particles. CONCLUSIONS: IAP, optimized at the frequency of 15 Hz and the pressure amplitude of 3 cm H2O, increased the total mass of particles exhaled from the human respiratory system. IAP has a broad range of potential clinical applications for noninvasive diagnosis of lung diseases including asthma, cystic fibrosis, pneumonia, and lung cancer, along with improvement of mucus clearance.

Ivacaftor Therapy in CF Patients: Single Center Experience.

Ivacaftor is the first novel cystic fibrosis pharmaceutical that acts at the molecular level to potentiate cystic fibrosis transmembrane conductance regulator (CFTR) function and was first approved for clinical use in 2012. We are sharing our single center experience of five patients: four from pediatric age group and one adult patient. All patients had both subjective and objective improvements in their health. Despite established lung disease, our patients had significant improvement in both their FEV1 (forced expiratory volume in 1 second) and FEF25-75 and BMI (body mass index). Larger studies demonstrated only 6.7% improvement in mean FEV1 after starting Ivacaftor therapy but their patient population had normal lung function to begin with. In contrast our case series demonstrates that, in patients with established lung disease and diminished lung function, Ivacaftor can be expected to result in much higher recovery in lung function. Mean FEV1 improved by 35% in our case series. Ivacaftor is extremely expensive, costing $300,000 per patient per year requiring lifelong therapy, hence requiring prior authorizations from most third-party payers in the USA. The knowledge shared from our experience will be useful for other clinicians to petition healthcare policymakers on behalf of their patients.

Serious air leak syndrome complicating high-flow nasal cannula therapy: a report of 3 cases.

Despite the absence of clinical safety data, heated, humidified high-flow nasal cannula (HHFNC) therapy is increasingly being used as an alternative to positive-pressure ventilation in pediatrics. This use of HHFNC is "off label" because the US Food and Drug Administration's approval for these devices was only for air humidification and not as a modality to provide positive distending pressure. For the first time we describe 3 cases who developed serious air leaks related to HHFNC therapy. The first child was a previously healthy 2-month-old male infant with respiratory syncytial virus bronchiolitis who developed a right pneumothorax on day 5 of his illness at 8 liters per minute (lpm). He subsequently required intubation and ventilation for 14 days. The second case involved an otherwise healthy 16-year-old boy with cerebral palsy who developed pneumomediastinum and died of its complications. He was receiving 20 lpm HHFNC therapy when he developed pneumomediastinum. The third case involved a 22-month-old, previously healthy boy who developed subdural hematoma secondary to abuse. He developed a right pneumothorax while receiving HHFNC at a flow of 6 lpm, requiring chest tube placement. These cases emphasize the need for extreme caution while using HHFNC for the off-label indication of providing positive distending pressure in children, especially at flows higher than the patient's minute ventilation. A more detailed study to specifically look at the serious adverse events related to HHFNC is urgently needed.