Characterizing the Cellular Constituents of Proximal Airway Disease in Granulomatosis With Polyangiitis.

Objective: Granulomatosis with polyangiitis (GPA) is a rare multisystem autoimmune vasculitis. 10-20% of patients suffer life-threatening obstruction of their proximal airways. Although progress has been made in the treatment of systemic disease, ameliorating airway disease in GPA remains an unmet need arising from limited understanding of disease pathogenesis.

Idiopathic Subglottic Stenosis and the Epithelial Interface of Host and Environment.

Background: Idiopathic subglottic stenosis (iSGS) is a rare fibrotic disease of the proximal airway affecting adult Caucasian women nearly exclusively. Life-threatening ventilatory obstruction occurs secondary to pernicious subglottic mucosal scar. Disease rarity and wide geographic patient distribution has previously limited substantive mechanistic investigation into iSGS pathogenesis.

Third Proceedings of The North American Airway Collaborative (NoAAC): Consensus Statement on Trial Design for Airway Stenosis.

Importance: Airway stenosis is a rare but debilitating disorder that significantly degrades the quality of life in affected patients. Treatments are primarily surgical, and disease management lacks established medical therapies. The North American Airway Collaborative held its third symposium at The Johns Hopkins Hospital in Baltimore, Maryland, on April 15, 2024, focused on strategies to advance the care of these patients.

Social network analysis as a new tool to measure academic impact of physicians.

Introduction: H-index is a widely used metric quantifying a researcher's productivity and impact based on an author's publications and citations. Though convenient to calculate, h-index fails to incorporate collaborations and interrelationships between physicians into its assessment of academic impact, leading to limited insight into grouped networks. We present social network analysis as a tool to measure relationships between physicians and quantify their academic impact.

Serum S100A8/A9 Correlates to Surgery-Free Interval in Idiopathic Subglottic Stenosis.

Objective: Idiopathic subglottic stenosis (iSGS) is a progressive fibrotic condition of the subglottis that presents in women of northern European descent. Endoscopic dilation is a common surgical approach to management of iSGS. The surgery-free interval, or the time between endoscopic dilation procedures is considered an indicator of disease severity.

Laryngeal Surgery as It Pertains to the Thoracic Surgeon.

A surgeon managing complex airway disease benefits from knowledge of laryngeal physiology and pathology. The Larynx provides phonation and protection for the lower airway via closure and cough reflex. Disruption of these functions or development of paralysis or stenosis can lead to respiratory failure.

HIV persists in late coronary atheroma and is associated with increased local inflammation and disease progression.

Chronic inflammation contributes to the prevalence of cardiovascular disease in people living with HIV (PLWH). The immune mechanisms driving atherosclerosis progression in PLWH remain unclear. This study conducted comprehensive assessments of medium-sized coronary arteries and aorta from deceased PLWH and controls without HIV using DNA/RNA assays, spatial transcriptomics, and high-resolution mass spectrometry.

PD-L1 Acts Independently of PD-1 as a Marker of Pathologic Fibroblasts in Laryngotracheal Stenosis.

Objective: Laryngotracheal stenosis (LTS) describes fibrotic airway obstruction that is life-threatening without treatment. Targeted therapies are needed as an adjunct to surgical management. We have previously observed the upregulation of immune checkpoint programmed cell death (PD)-1 and its ligand, PD-L1, in patients with LTS.

Cost Analysis of Avoiding Gastrostomy Tube in Robin Sequence Neonates that Undergo Mandibular Distraction.

Objectives/hypothesis: To evaluate costs associated with perioperative gastrostomy tube (G-tube) placement for neonates with Robin sequence (PRS) that undergo mandibular distraction osteogenesis (MDO).

Methods: Retrospective chart review was performed to examine the medical records of neonates with RS who received treatment at our institution between 2012 and 2021. Patients under 6 months of age that underwent MDO for RS were included.

Oral Everolimus Following Dilation in Idiopathic Subglottic Stenosis: A Phase 1 Nonrandomized Clinical Trial.

Importance: Current medical therapies in idiopathic subglottic stenosis (iSGS) are insufficient in preventing the development and progression of scar tissue. An inhibitor of mammalian target of rapamycin, everolimus is an immunosuppressive medication shown to be effective in reducing fibrosis across a variety of fibroproliferative disorders, including preclinical models of iSGS.

Objective: To evaluate the effect of oral everolimus on postoperative recurrence of stenosis in iSGS.

Primary tracheal adenoid cystic carcinoma: A case report and analysis of the tumor immune microenvironment using single cell RNA sequencing.

Background: Tracheal adenoid cystic carcinoma (ACC) is a slow growing yet aggressive malignancy with high rates of local recurrence as well as distant metastasis. Tracheal ACC exhibit a low mutation burden along with high mutational diversity, and generally do not respond well to chemotherapeutics.

Methods: We present a rare case of primary tracheal ACC initially presenting with nonspecific cervicalgia and globus sensation that was ultimately treated with tracheal resection followed by chemoradiation.

A Comprehensive Flow Cytometry Panel for Analysis of Idiopathic Subglottic Stenosis.

Objective: To present a comprehensive flow cytometry panel for idiopathic subglottic stenosis (iSGS).

Study Design: Controlled ex vivo cohort study.

Setting: Tertiary care academic hospital in a metropolitan area.

Removal of Metal Tracheal Stent: A Difficult Foreign Body.

Introduction: With its introduction in 1986, the use of metal tracheal stents gained favor due to relative ease of deployment and reduced risk of stent migration and mucus plugging. However, adverse events associated with metal stenting led the FDA to publish a recommendation against its use for benign airway stenosis in 2005. We present a case which illustrates the difficulty in removal of a metal stent from the airway.

#OtoTwitter: The Top 75 Twitter Influencers in Otolaryngology and Association With Academic Impact.

Objectives: Social media has enabled discussion of relevant topics within otolaryngology. With increasing academic discourse occurring on virtual platforms, it is important to examine who is influencing these discussions. This study thus aims to: (1) identify the top Twitter influencers in otolaryngology and (2) assess the relationship between Twitter influence and academic impact.

Similarity Network Analysis of the Adaptive Immune Response in the Proximal Airway.

Objectives: Recent immunologic study of the adaptive immune repertoire in the subglottic airway demonstrated high-frequency T cell clones that do not overlap between individuals. However, the anatomic distribution and antigenic target of the T cell repertoire in the proximal airway mucosa remain unresolved.

Methods: Single-cell RNA sequencing of matched scar and unaffected mucosa from idiopathic subglottic stenosis patients (iSGS, n = 32) was performed and compared with airway mucosa from healthy controls (n = 10).

A Single Center Description of Adult and Pediatric Endoscopic Posterior Costal Cartilage Grafting.

Objective: Posterior glottis impairment alters breathing, voicing, and swallowing. Bilateral vocal fold movement impairment (BVFMI) occurs when the vocal cords are fixed/remain midline. Studies inadequately assessed endoscopic posterior costal cartilage grafting (enPCCG) for BVFMI across broad ages.

Mucosal Microbiome Disruption in Acute Laryngeal Injury Following Intubation.

The objective of this study was to characterize mucosal microbial shifts in patients with acute laryngeal injury (ALgI) after intubation. This cross-sectional study included 20 patients with ALgI who underwent early endoscopic intervention with tissue culture, 20 patients with idiopathic subglottic stenosis (iSGS) who underwent tissue culture during the routine endoscopic intervention, and 3 control patients who underwent mucosal swab culture. 70% of the ALgI patients had a positive culture compared to 5% of the iSGS patients and none of the controls.

Characterizing the T Cell Repertoire in the Proximal Airway in Health and Disease.

Objectives: Recent translational scientific efforts in subglottic stenosis (SGS) support a disease model where epithelial alterations facilitate microbiome displacement, dysregulated immune activation, and localized fibrosis. Given the observed immune cell infiltrate in SGS, we sought to test the hypothesis that SGS cases possessed a low diversity (highly clonal) adaptive immune response when compared with healthy controls.

Methods: Single cell RNA sequencing (scRNA-seq) of subglottic mucosal scar in iSGS (n = 24), iLTS (n = 8), and healthy controls (n = 7) was performed.

Navigating Pathways to Diagnosis in Idiopathic Subglottic Stenosis: A Qualitative Study.

Objective: Idiopathic subglottic stenosis is a rare disease, and time to diagnosis is often prolonged. In the United States, some estimate it takes an average of 9 years for patients with similar rare disease to be diagnosed. Patient experience during this period is termed the diagnostic odyssey.

Association between Estrogen Exposure and Idiopathic Subglottic Stenosis.

Objective: Idiopathic subglottic stenosis (iSGS) is a rare, recurrent, fibroinflammatory disease affecting the larynx and proximal trachea. Given it occurs primarily in adult females, estrogen is speculated to play a central pathophysiological role. This study aimed to evaluate relationships between estrogen exposure, disease progression, and recurrence.

Decision-Making in the Treatment of Idiopathic Subglottic Stenosis: A Survey of Laryngologists.

Objective: Idiopathic subglottic stenosis (iSGS) is a rare condition involving the subglottic larynx and upper trachea, commonly affecting Caucasian females between 30 and 50 years of age. Despite its homogeneous presentation, clinical management for iSGS is yet to be standardized, leading to variability in outcomes between predominant interventions. In recognition of the heterogenicity in iSGS treatment and the need to improve patient outcomes, this study aimed to survey laryngologists to understand the factors influencing clinical decision-making and the incorporation of new treatment modalities for iSGS.

scDemultiplex: An iterative beta-binomial model-based method for accurate demultiplexing with hashtag oligos.

Single-cell sequencing have been widely used to characterize cellular heterogeneity. Sample multiplexing where multiple samples are pooled together for single-cell experiments, attracts wide attention due to its benefits of increasing capacity, reducing costs, and minimizing batch effects. To analyze multiplexed data, the first crucial step is to demultiplex, the process of assigning cells to individual samples.