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. The aim of this study is to use qualitative methods grounded in behavioral-ecological conceptual frameworks to identify drivers of diagnostic odyssey length that can help inform efforts to improve health care for iSGS patients. METHODS: Qualitative study using semi-structured interviews. Setting consisted of participants who were recruited from those enrolled in a large, prospective multicenter trial. We use directed content analysis to analyze qualitative semi-structured interviews with iSGS patients focusing on their pathways to diagnosis. RESULTS: Overall, 30 patients with iSGS underwent semi-structured interviews. The patient-reported median time to diagnosis was 21 months. On average, the participants visited four different health care providers. Specialists were most likely to make an appropriate referral to otolaryngology that ended in diagnosis. However, when primary care providers referred to otolaryngology, patients experienced a shorter diagnostic odyssey. The most important behavioral-ecological factors in accelerating diagnosis were strong social support for the patient and providers' willingness to refer. CONCLUSION: Several factors affected time to diagnosis for iSGS patients. Patient social capital was a catalyst in decreasing time to diagnosis. Patient-reported medical paternalism and gatekeeping limited specialty care referrals extended diagnostic odysseys. Additional research is needed to understand the effect of patient-provider and provider-provider relationships on time to diagnosis for patients with iSGS. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:815-824, 2024.

Learning Health Systems Research Competencies: A Survey of the Rehabilitation Research Community.

OBJECTIVE: The Learning Health Systems Rehabilitation Research Network (LeaRRn), an NIH-funded rehabilitation research resource center, aims to advance the research capacity of learning health systems (LHSs) within the rehabilitation community. A needs assessment survey was administered to inform development of educational resources. METHODS: The online survey included 55 items addressing interest in and knowledge of 33 LHS research core competencies in 7 domains and additional items on respondent characteristics. Recruitment targeting rehabilitation researchers and health system collaborators was conducted by LeaRRn, LeaRRn health system partners, rehabilitation professional organizations, and research university program directors using email, listservs, and social media announcements. RESULTS: Of the 650 people who initiated the survey, 410 respondents constituted the study sample. Respondents indicated interest in LHS research and responded to at least 1 competency item and/or demographic question. Two-thirds of the study sample had doctoral research degrees, and one-third reported research as their profession. The most common clinical disciplines were physical therapy (38%), communication sciences and disorders (22%), and occupational therapy (10%). Across all 55 competency items, 95% of respondents expressed "a lot" or "some" interest in learning more, but only 19% reported "a lot" of knowledge. Respondents reported "a lot" of interest in a range of topics, including selecting outcome measures that are meaningful to patients (78%) and implementing research evidence in health systems (75%). "None" or "some" knowledge was reported most often in Systems Science areas such as understanding the interrelationships between financing, organization, delivery, and rehabilitation outcomes (93%) and assessing the extent to which research activities will improve the equity of health systems (93%). CONCLUSION: Results from this large survey of the rehabilitation research community indicate strong interest in LHS research competencies and opportunities to advance skills and training. IMPACT: Competencies where respondents indicated high interest and limited knowledge can inform development of LHS educational content that is most needed.

Comparative Treatment Outcomes for Idiopathic Subglottic Stenosis: 5-Year Update.

The North American Airway Collaborative (NoAAC) previously published a 3-year multi-institutional prospective cohort study showing variation in treatment effectiveness between 3 primary surgical techniques for idiopathic subglottic stenosis (iSGS). In this report, we update these findings to include 5 years of data evaluating treatment effectiveness. Patients in the NoAAC cohort were re-enrolled for 2 additional years and followed using the prespecified published protocol. Consistent with prior data, prospective observation of 487 iSGS patients for 5 years showed treatment effectiveness differed by modality. Cricotracheal resection maintained the lowest rate of recurrent operation (5%), followed by endoscopic resection with adjuvant medical therapy (30%) and endoscopic dilation (50%). These data support the initial observations and continue to provide value to providers and patients navigating longitudinal decision-making. Level of evidence: 2-prospective cohort study.

A survey of patients with laryngotracheal stenosis on future clinical trial design.

BACKGROUND/AIMS: Laryngotracheal stenosis is a rare but devastating proximal airway fibrosis that restricts a patient's ability to breathe. Treatment is primarily surgical and to date, there has never been a multi-institutional, randomized, prospective, and interventional clinical trial for a medical therapy to treat laryngotracheal stenosis. Therefore, we aimed to obtain patient feedback to guide successful trial design, recruitment, retention, and for identifying potential barriers to study participation. METHODS: Over 1000 members of an international laryngotracheal stenosis online support community (the Living with Idiopathic Subglottic Stenosis Facebook group) were sent two questionnaires for a proposed interventional double-blinded, randomized, placebo-controlled clinical trial. RESULTS: A total of 317 and 558 participants responded to the first and second surveys, respectively. The majority of participants (77%) were willing to consider enrollment, regardless of having a 50% chance of receiving placebo versus treatment (78%). The majority (84%) of participants were willing to travel 200 miles to participate for up to six in-person visits over 50 days. Specific side effects, including anemia/thrombocytopenia (72%) or risk of infection (69.3%) had the greatest impact on clinical trial participation with other side effects (peripheral edema (53%), oral ulcers (51%), and gastrointestinal side effects (41%)) having less impact. CONCLUSION: Patients with laryngotracheal stenosis possess nuanced insight into their disease and treatment options. As a group, they are extremely motivated for better therapies. Future laryngotracheal stenosis clinical trials should focus on providing excellent side effect -related education and utilizing feedback from online advocacy groups to optimize recruitment and retention.

"A Major Quality of Life Issue": A Survey-Based Analysis of the Experiences of Adults With Laryngotracheal Stenosis with Mucus and Cough.

OBJECTIVES: To investigate how the symptoms of mucus and cough impact adults living with laryngotracheal stenosis, and to use this information to guide future research and treatment plans. METHODS: A survey was developed with the support of patient advisors and distributed to people suffering with laryngotracheal stenosis. The survey comprised 15 closed and open questions relating to mucus and cough and included the Leicester Cough Questionnaire (LCQ). Descriptive statistics, X2 and thematic analyses were completed. RESULTS: In total, 641 participants completed the survey, with 83.62% (n = 536) reporting problems with mucus; 79% having daily issues of varying severity that led to difficulties with cough (46.18%) and breathing (20.90%). Mucus affected voice and swallowing to a lesser degree. Respondents described a range of triggers; they identified smoky air as the worst environmental trigger. Strategies to manage mucus varied widely with drinking water (72.26%), increasing liquid intake in general (49.35%) and avoiding or reducing dairy (45.32%) the most common approaches to control symptoms. The LCQ showed a median total score of 14 (interquartile range 11-17) indicative of cough negatively affecting quality of life. Thematic analysis of free text responses identified 4 key themes-the Mucus Cycle, Social impact, Psychological impact, and Physical impact. CONCLUSION: This study shows the relevance of research focusing on mucus and cough and its negative impact on quality of life, among adults with laryngotracheal stenosis. It demonstrates the inconsistent advice and management strategies provided by clinicians for this issue. Further research is required to identify clearer treatment options and pathways.

GAD65Cre Drives Reporter Expression in Multiple Taste Cell Types.

In taste buds, Type I cells represent the majority of cells (50-60%) and primarily have a glial-like function in taste buds. However, recent studies suggest that they have additional sensory and signaling functions including amiloride-sensitive salt transduction, oxytocin modulation of taste, and substance P mediated GABA release. Nonetheless, the overall function of Type I cells in transduction and signaling remains unclear, primarily because of the lack of a reliable reporter for this cell type. GAD65 expression is specific to Type I taste cells and GAD65 has been used as a Cre driver to study Type I cells in salt taste transduction. To test the specificity of transgene-driven expression, we crossed GAD65Cre mice with floxed tdTomato and Channelrhodopsin (ChR2) lines and examined the progeny with immunochemistry, chorda tympani recording, and calcium imaging. We report that while many tdTomato+ taste cells express NTPDase2, a specific marker of Type I cells, we see some expression of tdTomato in both Gustducin and SNAP25-positive taste cells. We also see ChR2 in cells just outside the fungiform taste buds. Chorda tympani recordings in the GAD65Cre/ChR2 mice show large responses to blue light. Furthermore, several isolated tdTomato-positive taste cells responded to KCl depolarization with increases in intracellular calcium, indicating the presence of voltage-gated calcium channels. Taken together, these data suggest that GAD65Cre mice drive expression in multiple taste cell types and thus cannot be considered a reliable reporter of Type I cell function.

Association of Social Determinants of Health with Time to Diagnosis and Treatment Outcomes in Idiopathic Subglottic Stenosis.

OBJECTIVES: To examine whether social determinants of health (SDH) factors are associated with time to diagnosis, treatment selection, and time to recurrent surgical intervention in idiopathic subglottic stenosis (iSGS) patients. METHODS: Adult patients with diagnosed iSGS were recruited prospectively (2015-2017) via clinical providers as part of the North American Airway Collaborative (NoAAC) and via an online iSGS support community on Facebook. Patient-specific SDH factors included highest educational attainment (self-reported), median household income (matched from home zip code via U.S. Census data), and number of close friends (self-reported) as a measure of social support. Main outcomes of interest were time to disease diagnosis (years from symptom onset), treatment selection (endoscopic dilation [ED] vs cricotracheal resection [CTR] vs endoscopic resection with adjuvant medical therapy [ERMT]), and time to recurrent surgical intervention (number of days from initial surgical procedure) as a surrogate for disease recurrence. RESULTS: The total 810 participants were 98.5% female, 97.2% Caucasian, and had a median age of 50 years (IQR, 43-58). The cohort had a median household income of $62 307 (IQR, $50 345-$79 773), a median of 7 close friends (IQR, 4-10), and 64.7% of patients completed college or graduate school. Education, income, and number of friends were not associated with time to diagnosis via multivariable linear regression modeling. Univariable multinominal logistic regression demonstrated an association between education and income for selecting ED versus ERMT, but no associations were noted for CTR. No associations were noted for time to recurrent surgical procedure via Kaplan Meier modeling and Cox proportional hazards regression. CONCLUSIONS: Patient education, income, and social support were not associated with time to diagnosis or time to disease recurrence. This suggests additional patient, procedure, or disease-specific factors contribute to the observed variations in iSGS surgical outcomes.

Idiopathic Subglottic Stenosis during Pregnancy: A Support Group Survey.

OBJECTIVES: (1) To determine how pregnancy affects idiopathic subglottic stenosis (iSGS) symptoms. (2) To determine treatments utilized (including operating room (OR) and in-office procedures) for iSGS before, during, and after pregnancy. METHODS: A 24-question survey was distributed to an international iSGS support group to assess the patient experience among individuals who have been pregnant with diagnosis of iSGS. Descriptive statistics and chi2 analyses were completed. RESULTS: A total of 413 iSGS patients participated; 84.7% (n = 350) of patients were diagnosed prior to menopause. A total of 25.5% patients reported being pregnant when they had airway stenosis; 71.1% of those reported more severe airway symptoms during pregnancy. The proportion of patients that reported requiring ≥1 OR interventions (microlaryngoscopy and bronchoscopy, laser, balloon dilation, or steroid injection) before, during and after pregnancy was the following, respectively: 37.3%, 35.6%, 51.3%. Whereas the proportion of patients that reported requiring ≥1 in-office interventions (awake balloon dilation or steroid injection) before, during and after pregnancy was the following, respectively: 13.6%, 11.8%, 15.8%. Number of pregnancies and age of diagnosis was not related to severity of symptoms or requiring more airway interventions (P > .05). CONCLUSIONS: This survey reveals worsening of symptoms during pregnancy, however, this did not lead to increase in operative or clinic interventions to improve airway symptoms during pregnancy. Future avenues for research include optimizing management of airway symptoms during pregnancy to limit OR-based interventions. LEVEL OF EVIDENCE: Level IV.

Utilization and Influence of Online Support Communities in Idiopathic Subglottic Stenosis Patients.

OBJECTIVES: To determine the factors that shape utilization of social media-based online support communities (OSCs) and study the influence of these communities on medical decision-making in patients with Idiopathic Subglottic Stenosis (iSGS). STUDY DESIGN: Survey study. METHODS: A survey investigating OSC use was sent to the 1,056 members of the North American Airway Collaborative (NoAAC) iSGS1000 cohort in January 2018. Responses were merged with the existing NoAAC data set containing extensive demographic data, disease-specific history, and responses to validated patient-reported outcome measures. RESULTS: A total of 755 individuals with iSGS and mean age of 51.8 ± 11.6 years were included (99% female, 98% white, 63% college educated) and 58% were OSC users. Younger age, female gender, and college education were each associated with OSC use (P < .05). Users spent 2.5 ± 3.3 hours per week on the platforms. Time spent on OSC was not associated with total number of prior treatments. Higher disease anxiety (FoP-Q, R = 0.26, P < .001), lower social support (MOS, R = -0.12, P = .037), and lower level of shared-decision-making with the treating physician (SDM-Q9, R = -0.16, P = .007) were weakly associated with more hours spent engaging an OSC. OSC use influenced treatment and physician choice in 35% and 26% of users, respectively. Increased time spent on OSC use was associated with increased influence on patient medical decisions regarding treatment, surgery, and physician choice (P < .05). CONCLUSION: OSC engagement is common in patients with iSGS. Disease anxiety, social support, and relationship with the physician may influence OSC utilization. More OSC engagement weakly associated with greater OSC influence on patient medical decision-making. LEVEL OF EVIDENCE: NA. Laryngoscope, 131:1821-1827, 2021.

Impact of the COVID-19 Pandemic on Patients With Idiopathic Subglottic Stenosis.

OBJECTIVES: To determine the number of patients with idiopathic subglottic stenosis (iSGS) who have contracted coronavirus disease 2019 (COVID-19), the impact of the pandemic on patients' attitudes on seeking help and attending hospitals, as well as the delays in the offer of treatment from the local health care systems. METHODS: A 29-question survey was distributed to an international mailing list of patients with subglottic stenosis to assess the patient experience during the COVID-19 pandemic. RESULTS: A total of 543 patients with iSGS participated. Fewer than 1 in 10 patients with iSGS have experienced COVID-19 symptoms, which were predominantly mild to moderate, with only 2 hospitalizations. Most patients with iSGS (80.0%) have not been advised they are high risk for COVID-19, despite 36.5% of patients with iSGS being obese (body mass index of 30+). Delays to surgeries and in-office procedures have impacted 40.1% of patients currently receiving treatment, with 38.8% of patients increasingly struggling to breathe as a result. Anxiety and stress are increasing among patients, with 3 in 4 (75.2%) reporting they are anxious about travelling by public transport, contracting the virus in hospital and infecting loved ones (69.0% and 71.9%, respectively). Of greater concern is that 23.1% with increasing dyspnea state they are staying away from hospital despite their deteriorating health. CONCLUSIONS: The COVID-19 pandemic has had an impact on the physical and psychological health of patients with iSGS. Surgeons managing cases of laryngotracheal stenosis need to offer appropriate support and communication to these high risk patients. During the pandemic, this should include self-isolation if they are dyspneic or on treatments that may have reduced their immunity. In addition, they should offer safe clinical pathways to airway assessment and treatments, if they become necessary. To minimize unnecessary travel, much of the clinical monitoring can be carried out remotely, using telephone or video-based consultations, in conjunction with local health professionals.

Company Characteristics, Disability Inclusion Practices, and Employment of People with Disabilities in the Post COVID-19 Job Economy: A Cross Sectional Survey Study.

Purpose In the post coronavirus disease 2019 (COVID-19) recession economy, rehabilitation counselors, transition specialists, and other disability service providers must redouble their efforts to connect with employers to create employment opportunities for people with physical and mental impairments. The purpose of the present study was to investigate company characteristics and effective disability inclusion practices that are related to employment of people with disabilities. Methods Four hundred sixty-six employers completed a demographic questionnaire and the Disability Inclusion Profiler. Results Results indicated company characteristics and disability practices were positively related to employment of people with disabilities. Conclusions Findings of the present study can be used by transition specialists, rehabilitation counselors, and other disability service providers to engage and connect with employers to increase employment opportunity for people with disabilities in the post COVID-19 economy. Future research and practice implications are provided.

Function, Innervation, and Neurotransmitter Signaling in Mice Lacking Type-II Taste Cells.

The Skn-1a transcription factor (Pou2f3) is required for Type II taste cell differentiation in taste buds. Taste buds in Skn-1a-/- mice lack Type II taste cells but have a concomitant expansion of Type III cells, providing an ideal model to determine the relative role of taste cell types in response specificity. We confirmed that chorda tympani responses to sweet, bitter, and umami stimuli were greatly reduced in the knock-outs (KOs) compared with wild-type (WT) littermates. Skn-1a-/- mice also had reductions to NaCl that were partially amiloride-insensitive, suggesting that both Type II and Type III cells contribute to amiloride-insensitive salt detection in anterior tongue. We also confirmed that responses to sour stimuli are equivalent in the KOs, despite the large increase in the number of Type III taste cells. To examine their innervation, we crossed the Htr3a-GFP (5-HT3A-GFP) reporter mouse with the Skn-1a-/- mice and examined geniculate ganglion neurons for GFP expression and responses to 5-HT. We found no change in the number of 5-HT3A-expressing neurons with KO of Skn-1a Calcium imaging showed that only 5-HT3A-expressing neurons respond to exogenous 5-HT, while most neurons respond to ATP, similar to WT mice. Interestingly, despite loss of all Type II cells, the P2X3 antagonist AF353 blocked all chorda tympani responses. These data collectively raise questions pertaining the source of ATP signaling in the absence of Type II taste cells and whether the additional Type III cells are innervated by fibers that would have normally innervated Type II cells.

Comparative Treatment Outcomes for Patients With Idiopathic Subglottic Stenosis.

Importance: Surgical treatment comparisons in rare diseases are difficult secondary to the geographic distribution of patients. Fortunately, emerging technologies offer promise to reduce these barriers for research. Objective: To prospectively compare the outcomes of the 3 most common surgical approaches for idiopathic subglottic stenosis (iSGS), a rare airway disease. Design, Setting, and Participants: In this international, prospective, 3-year multicenter cohort study, 810 patients with untreated, newly diagnosed, or previously treated iSGS were enrolled after undergoing a surgical procedure (endoscopic dilation [ED], endoscopic resection with adjuvant medical therapy [ERMT], or cricotracheal resection [CTR]). Patients were recruited from clinician practices in the North American Airway Collaborative and an online iSGS community on Facebook. Main Outcomes and Measures: The primary end point was days from initial surgical procedure to recurrent surgical procedure. Secondary end points included quality of life using the Clinical COPD (chronic obstructive pulmonary disease) Questionnaire (CCQ), Voice Handicap Index-10 (VHI-10), Eating Assessment Test-10 (EAT-10), the 12-Item Short-Form Version 2 (SF-12v2), and postoperative complications. Results: Of 810 patients in this cohort, 798 (98.5%) were female and 787 (97.2%) were white, with a median age of 50 years (interquartile range, 43-58 years). Index surgical procedures were ED (n = 603; 74.4%), ERMT (n = 121; 14.9%), and CTR (n = 86; 10.6%). Overall, 185 patients (22.8%) had a recurrent surgical procedure during the 3-year study, but recurrence differed by modality (CTR, 1 patient [1.2%]; ERMT, 15 [12.4%]; and ED, 169 [28.0%]). Weighted, propensity score-matched, Cox proportional hazards regression models showed ED was inferior to ERMT (hazard ratio [HR], 3.16; 95% CI, 1.8-5.5). Among successfully treated patients without recurrence, those treated with CTR had the best CCQ (0.75 points) and SF-12v2 (54 points) scores and worst VHI-10 score (13 points) 360 days after enrollment as well as the greatest perioperative risk. Conclusions and Relevance: In this cohort study of 810 patients with iSGS, endoscopic dilation, the most popular surgical approach for iSGS, was associated with a higher recurrence rate compared with other procedures. Cricotracheal resection offered the most durable results but showed the greatest perioperative risk and the worst long-term voice outcomes. Endoscopic resection with medical therapy was associated with better disease control compared with ED and had minimal association with vocal function. These results may be used to inform individual patient treatment decision-making.

Expression of Bitter Taste Receptors and Solitary Chemosensory Cell Markers in the Human Sinonasal Cavity.

Some bitter taste receptors (TAS2R gene products) are expressed in the human sinonasal cavity and may function to detect airborne irritants. The expression of all 25 human bitter taste receptors and their location within the upper airway is not yet clear. The aim of this study is to characterize the presence and distribution of TAS2R transcripts and solitary chemosensory cells (SCCs) in different locations of the human sinonasal cavity. Biopsies were obtained from human subjects at up to 4 different sinonasal anatomic sites. PCR, microarray, and qRT-PCR were used to examine gene transcript expression. The 25 human bitter taste receptors as well as the sweet/umami receptor subunit, TAS1R3, and canonical taste signaling effectors are expressed in sinonasal tissue. All 25 human bitter taste receptors are expressed in the human upper airway, and expression of these gene products was higher in the ethmoid sinus than nasal cavity locations. Fluorescent in situ hybridization demonstrates that epithelial TRPM5 and TAS2R38 are expressed in a rare cell population compared with multiciliated cells, and at times, consistent with SCC morphology. Secondary analysis of published human sinus single-cell RNAseq data did not uncover TAS2R or canonical taste transduction transcripts in multiciliated cells. These findings indicate that the sinus has higher expression of SCC markers than the nasal cavity in chronic rhinosinusitis patients, comprising a rare cell type. Biopsies obtained from the ethmoid sinus may serve as the best location for study of human upper airway taste receptors and SCCs.

A2ML1 and otitis media: novel variants, differential expression, and relevant pathways.

A genetic basis for otitis media is established, however, the role of rare variants in disease etiology is largely unknown. Previously a duplication variant within A2ML1 was identified as a significant risk factor for otitis media in an indigenous Filipino population and in US children. In this report exome and Sanger sequencing was performed using DNA samples from the indigenous Filipino population, Filipino cochlear implantees, US probands, Finnish, and Pakistani families with otitis media. Sixteen novel, damaging A2ML1 variants identified in otitis media patients were rare or low-frequency in population-matched controls. In the indigenous population, both gingivitis and A2ML1 variants including the known duplication variant and the novel splice variant c.4061 + 1 G>C were independently associated with otitis media. Sequencing of salivary RNA samples from indigenous Filipinos demonstrated lower A2ML1 expression according to the carriage of A2ML1 variants. Sequencing of additional salivary RNA samples from US patients with otitis media revealed differentially expressed genes that are highly correlated with A2ML1 expression levels. In particular, RND3 is upregulated in both A2ML1 variant carriers and high-A2ML1 expressors. These findings support a role for A2ML1 in keratinocyte differentiation within the middle ear as part of otitis media pathology and the potential application of ROCK inhibition in otitis media.

Nebulizer Use in Adults With Subglottic Stenosis: A Survey Study.

OBJECTIVE:: People with subglottic stenosis (SGS) may experience laryngeal symptoms that pose significant challenges to management. This study surveyed the use and effects of nebulized treatments on laryngeal symptoms in a large cohort of adults with SGS. METHODS:: A survey of nebulized treatment practices was distributed to an online international community of over 2000 members; 316 individuals (298 female, 1 male, 17 declined to specify; age 25+ years) completed the survey. RESULTS:: Nearly half (144) of participants had tried a nebulizer in the past. Among those, half currently used nebulized treatments; the majority used these treatments regularly and for greater than 1 year. The most frequently reported treatments included isotonic saline (0.9% Na+Cl-) or hypertonic saline (3% Na+Cl-). Symptoms improved by these treatments included thick mucus (69%), cough (57%), throat dryness (31%), stridor (28%), and voice (15%). The most frequently reported limitations to nebulizer use included time, noise, and portability. CONCLUSION:: Among those individuals with SGS who have tried nebulized treatments, nearly half experienced relief from secondary laryngeal symptoms. Additional efficacy research is needed, particularly with respect to treatment type and dosage. The results are encouraging given the adverse impact these laryngeal symptoms can have on activities of daily living and quality of life.

The Online Support Group as a Community: A Thematic Content Analysis of an Online Support Group for Idiopathic Subglottic Stenosis.

OBJECTIVE:: Idiopathic subglottic stenosis (iSGS) is a rare disease with few local resources for individuals to use. With the explosive growth of online social networking, platforms such as Facebook possess compelling potential to facilitate user-driven sharing of health information and peer support. This study was performed to better understand the content shared in a busy online community for individuals with iSGS. METHODS:: The largest online community (OC) for individuals with iSGS, Living With Idiopathic Subglottic Stenosis (LwiSGS), was examined. A thematic content analysis of the communications shared in February of 2018 was performed. A conventional qualitative analysis model was employed to analyze aggregated data. The data were then codified. RESULTS:: Analysis demonstrated that communications primarily encompassed three major thematic elements: (1) information sharing; (2) emotional support, expression, and experience sharing; and (3) community building. Positively toned posts grossly overshadowed negatively toned posts by almost a factor of 3. A significant portion of group members requested information from their peers, suggesting a high level of trust toward the resources provided in this group, even those involving a surgical procedure or medication. CONCLUSION:: LwiSGS is a forum for patients with a rare chronic condition to share informational resources, personal experiences, and emotional support, as well as a community with their peers. These data suggest that LwiSGS could be a powerful resource for individuals with iSGS to share information, personal experiences, or emotional support.

RNA sequencing and pathway analysis identify tumor necrosis factor alpha driven small proline-rich protein dysregulation in chronic rhinosinusitis.

BACKGROUND: Chronic rhinosinusitis (CRS) is a heterogeneous inflammatory disorder in which many pathways contribute to end-organ disease. Small proline-rich proteins (SPRR) are polypeptides that have recently been shown to contribute to epithelial biomechanical properties relevant in T-helper type 2 inflammation. There is evidence that genetic polymorphism in SPRR genes may predict the development of asthma in children with atopy and, correlatively, that expression of SPRRs is increased under allergic conditions, which leads to epithelial barrier dysfunction in atopic disease. METHODS: RNAs from uncinate tissue specimens from patients with CRS and control subjects were compared by RNA sequencing by using Ingenuity Pathway Analysis (n = 4 each), and quantitative polymerase chain reaction (PCR) (n = 15). A separate cohort of archived sinus tissue was examined by immunohistochemistry (n = 19). RESULTS: A statistically significant increase of SPRR expression in CRS sinus tissue was identified that was not a result of atopic presence. SPRR1 and SPRR2A expressions were markedly increased in patients with CRS (p < 0.01) on RNA sequencing, with confirmation by using real-time PCR. Immunohistochemistry of archived surgical samples demonstrated staining of SPRR proteins within squamous epithelium of both groups. Pathway analysis indicated tumor necrosis factor (TNF) alpha as a master regulator of the SPRR gene products. CONCLUSION: Expression of SPRR1 and of SPRR2A is increased in mucosal samples from patients with CRS and appeared as a downstream result of TNF alpha modulation, which possibly resulted in epithelial barrier dysfunction.

Case Report: Diagnosis of hypogeusia after oral exposure to commercial cleaning agent and considerations for clinical taste testing.

Few reports in the literature document acute taste disturbance following exposure to toxic chemicals. We describe the case of a 54-year-old man who presented with primary complaint of tongue numbness and persistent problems with taste 1.5 years following oral exposure to a commercial cleaning agent. A test of olfaction revealed normosmia for age and gender. Lingual tactile two-point discrimination testing showed reduced somatosensation. Taste threshold testing using a 3-drop method demonstrated severe hypogeusia, though the patient was able to discriminate tastants at lower concentrations with a whole mouth swish and spit test. We conclude that clinical evaluation of dysgeusia can be performed using a number of previously published testing methods, however, determining causative factors may be confounded by duration since exposure, lack of knowledge of baseline taste function, and medications. Although many testing options exist, basic taste testing can be performed with minimal expertise or specialized equipment, depending on the patient history and goals of evaluation.

Associations between children's diet quality and watching television during meal or snack consumption: A systematic review.

Studies have identified an association between watching television (TV) and childhood obesity. This review adds context to existing research by examining the associations between TV viewing, whilst eating, and children's diet quality. Web of Science and PubMed databases were searched from January 2000 to June 2014. Cross-sectional trials of case control or cohort studies, which included baseline data, measuring the associations between eating whilst watching TV and children's food and drink intake. Quality of selected papers was assessed. Thirteen studies, representing 61,674 children aged 1-18 yrs, met inclusion criteria. Of six studies reporting overall food habits, all found a positive association between TV viewing and consumption of pizza, fried foods, sweets, and snacks. Of eight studies looking at fruit and vegetable consumption, seven identified a negative association with eating whilst watching TV (p < .0001). Four out of five studies identified a positive association between watching TV whilst eating and servings of sugar-sweetened beverages (p < .0001). Four studies identified an association between low socioeconomic status and increased likelihood of eating whilst watching TV (p ≤ .01). Family meals did not overcome the adverse impact on diet quality of having the TV on at mealtimes. Eating whilst watching television is associated with poorer diet quality among children, including more frequent consumption of sugar-sweetened beverages and high-fat, high-sugar foods and fewer fruits and vegetables. Although these differences in consumption are small, the cumulative effect may contribute to the positive association between eating whilst watching TV and childhood obesity.