Citations

Objective: To compare voice-related outcomes of type 1 thyroplasty using Gore-Tex® between patients with and without augmentation injection laryngoplasty (IL) prior to surgery. Methods: Forty-five patients who underwent Gore-Tex® thyroplasty at a single institution by a single surgeon between November 2016 and February 2019 were identified as those who previously had IL (n = 20) and those without IL (n = 25). Pre- and post-operative voice-related primary outcomes were evaluated using the GRBAS, and CAPE-V auditory-perceptual rating scales and secondary outcome were evaluated using the VRQOL. Pre- and post-operative voice samples were blinded, randomized, and analyzed by 3 voice-specialized speech pathologists to obtain CAPE-V scores. The VRQOL and GRBAS scores were obtained from retrospective chart review. Student’s t test with a paired one-tailed distribution was used for comparisons within groups and 2-sample equal variance for comparisons between groups. Intraclass correlation coefficient determined interrater agreement. Results: GRBAS, and VRQOL significantly improved post Gore-Tex® thyroplasty. There was no difference in improvement between patients who received pre-surgery IL and those who did not in either GRBAS or VRQOL scores, but CAPE-V showed significant improvement in the IL group. A strongly positive correlation was demonstrated between the severity of CAPE-V pre-op score and the overall improvement following surgery for both groups combined. Conclusion: Patients with vocal fold paralysis have a significantly better voice after Gore-Tex® thyroplasty by self-report (VRQOL) and assessment by trained voice professionals (GRBAS). Having IL prior to surgery does not adversely affect later surgical outcomes. This paper represents one of the largest analyses of voice quality outcomes of Gore-Tex® thyroplasty using validated patient scales and randomized blinded analyses.

Background: Acute myocarditis is an inflammatory condition that may herald the onset of dilated cardiomyopathy (DCM) or arrhythmogenic cardiomyopathy (ACM). We investigated the frequency and clinical consequences of DCM and ACM genetic variants in a population-based cohort of patients with acute myocarditis. Methods: This was a population-based cohort of 336 consecutive patients with acute myocarditis enrolled in London and Maastricht. All participants underwent targeted DNA sequencing for well-characterized cardiomyopathy-associated genes with comparison to healthy controls (n=1053) sequenced on the same platform. Case ascertainment in England was assessed against national hospital admission data. The primary outcome was all-cause mortality. Results: Variants that would be considered pathogenic if found in a patient with DCM or ACM were identified in 8% of myocarditis cases compared with <1% of healthy controls ( P =0.0097). In the London cohort (n=230; median age, 33 years; 84% men), patients were representative of national myocarditis admissions (median age, 32 years; 71% men; 66% case ascertainment), and there was enrichment of rare truncating variants (tv) in ACM-associated genes (3.1% of cases versus 0.4% of controls; odds ratio, 8.2; P =0.001). This was driven predominantly by DSP -tv in patients with normal LV ejection fraction and ventricular arrhythmia. In Maastricht (n=106; median age, 54 years; 61% men), there was enrichment of rare truncating variants in DCM-associated genes, particularly TTN -tv, found in 7% (all with left ventricular ejection fraction <50%) compared with 1% in controls (odds ratio, 3.6; P =0.0116). Across both cohorts over a median of 5.0 years (interquartile range, 3.9–7.8 years), all-cause mortality was 5.4%. Two-thirds of deaths were cardiovascular, attributable to worsening heart failure (92%) or sudden cardiac death (8%). The 5-year mortality risk was 3.3% in genotype-negative patients versus 11.1% for genotype-positive patients ( P adjusted =0.08). Conclusions: We identified DCM- or ACM-associated genetic variants in 8% of patients with acute myocarditis. This was dominated by the identification of DSP -tv in those with normal left ventricular ejection fraction and TTN -tv in those with reduced left ventricular ejection fraction. Despite differences between cohorts, these variants have clinical implications for treatment, risk stratification, and family screening. Genetic counseling and testing should be considered in patients with acute myocarditis to help reassure the majority while improving the management of those with an underlying genetic variant.

Abstract We present the case of a 46-year-old male with medically complicated class III obesity who presented with new-onset and persistent dysphagia after undergoing a successful laparoscopic Roux-en-Y gastric bypass surgery. An esophagogastroduodenoscopy was unrevealing, however, further workup with the endoluminal functional lumen imaging probe (EndoFLIP) device was suggestive of esophageal dysmotility with decreased distensibility index of the GE junction and absent/disorganized secondary esophageal peristalsis. Thorough chart review was performed to elicit details of the case presentation. Further, a literature review was performed to supplement background knowledge and discussion. We present a case of symptomatic esophageal dysphagia in a post-procedural bariatric surgery patient with normal EGD yet positive findings on functional lumen imaging, consistent with the newly described entity termed Post Obesity Surgery Esophageal Dysfunction (POSED). POSED is defined by aperistalsis and increased intragastric pressure seen after bariatric surgery. It is thought to occur due to a high-pressure gastric zone created in the noncompliant postoperative stomach after Roux-en-Y gastric bypass, or an increased esophageal afterload by a restrictive laparoscopic sleeve gastrectomy. No treatment guidelines exist, though options include botulinum toxin, dilation, and per-oral esophageal myotomy. This case provides further insight into the role that functional lumen imaging has in the bariatric patient, as it may be able to more accurately detect patients who will develop esophageal dysmotility following bariatric surgery, or perhaps even those who may be predisposed prior to undergoing intervention for weight loss.

ABSTRACT Model organism (MO) research provides a basic understanding of biology and disease due to the evolutionary conservation of the molecular and cellular language of life. MOs have been used to identify and understand the function of orthologous genes, proteins, cells and tissues involved in biological processes, to develop and evaluate techniques and methods, and to perform whole-organism-based chemical screens to test drug efficacy and toxicity. However, a growing richness of datasets and the rising power of computation raise an important question: How do we maximize the value of MOs? In-depth discussions in over 50 virtual presentations organized by the National Institutes of Health across more than 10 weeks yielded important suggestions for improving the rigor, validation, reproducibility and translatability of MO research. The effort clarified challenges and opportunities for developing and integrating tools and resources. Maintenance of critical existing infrastructure and the implementation of suggested improvements will play important roles in maintaining productivity and facilitating the validation of animal models of human biology and disease.

Vaccination is critical to control the ongoing COVID-19 pandemic, but despite the availability of safe and effective vaccine in children over 5 years, vaccination rates remain low. There is paucity of data about vaccine acceptance and factors influencing parents’ hesitancy about the COVID-19 vaccine for young children. Aims and objectives To estimate vaccine acceptance by parents of children 6 months through 4 years, and to evaluate the factors influencing vaccine hesitancy. Methods Electronic survey was sent to parents of children 6 months through 4 years through an online portal account at Mayo Clinic Health System, Northwest-Wisconsin. Data were captured via Research Electronic Data Capture secured data collection software. Bivariate and multivariate regression was used to determine most pertinent factors influencing parents’ decisions against the outcome, ‘Intent to Vaccinate’. Results 39.7% of the parents were ‘very likely’ or ‘somewhat likely ’ to vaccinate their children once the vaccine became available, while 49.8% were not likely or highly unlikely to vaccinate. Routine childhood vaccination, receiving seasonal influenza vaccine, parents' perception of COVID-19 severity in children and safety and effectiveness of the vaccine were all associated with more vaccine acceptance. 71.4% of parents who will likely not vaccinate their children indicated that they are unlikely to change their decision. The need for more research on the vaccine and more information from the PCP office were the most common reasons behind the vaccine decision-making. Conclusions Vaccine hesitancy remains a major issue regarding uptake of the upcoming COVID-19 vaccine. Strong and clear evidence-based recommendations from primary care provider and more information from trusted websites such as Centers for Disease Control and Prevention can decrease vaccine hesitancy in parents. Further research targeted at understanding beliefs and perspectives of parents from different demographics can assist policy-makers in implementing measures to improve vaccination rates in children and tailor our dialogue to match the needs of our patients and families.

Abstract Primary leptomeningeal lymphoma is exceedingly rare. We describe 2 rare lymphoma cases with exclusive leptomeningeal disease: 1 ALK-positive (ALK+) anaplastic large cell lymphoma (ALCL) and 1 primary effusion lymphoma (PEL). Case 1: A 19-year-old man presented with symptoms concerning for leptomeningitis. Cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis. Spine MRI demonstrated pial enhancement from T10 through the conus medullaris and cauda equina enhancement/thickening. A biopsy showed leptomeningeal involvement by large lymphoma cells with hallmark cells and brisk mitotic activity. By immunohistochemistry, cells were CD7/CD30-positive with cytoplasmic ALK staining. No systemic disease was identified. The diagnosis of primary leptomeningeal ALK+ ALCL was made. Despite 2 CSF relapses requiring systemic therapy and autologous bone marrow transplant, the patient was in complete clinical remission 9 years after the diagnosis. Case 2: A 60-year-old, human immunodeficiency virus-positive man presented with symptoms suggestive of leptomeningitis. Brain MRIs revealed multifocal, supratentorial, and infratentorial leptomeningeal enhancement. A right frontal biopsy demonstrated leptomeningeal involvement by large lymphoma cells negative for B-cell immunostains, but CD138, MUM-1, and HHV8-positive, with aberrant CD3 expression. EBV-encoded RNA in situ hybridization was positive. In absence of solid lesions/extracranial involvement, the diagnosis of leptomeningeal PEL was rendered. Despite initial complete remission after chemotherapy, the patient died 9 months later.

Objective We investigated prolonged COVID-19 symptom duration, defined as lasting 28 days or longer, among people with systemic autoimmune rheumatic diseases (SARDs). Methods We analysed data from the COVID-19 Global Rheumatology Alliance Vaccine Survey (2 April 2021–15 October 2021) to identify people with SARDs reporting test-confirmed COVID-19. Participants reported COVID-19 severity and symptom duration, sociodemographics and clinical characteristics. We reported the proportion experiencing prolonged symptom duration and investigated associations with baseline characteristics using logistic regression. Results We identified 441 respondents with SARDs and COVID-19 (mean age 48.2 years, 83.7% female, 39.5% rheumatoid arthritis). The median COVID-19 symptom duration was 15 days (IQR 7, 25). Overall, 107 (24.2%) respondents had prolonged symptom duration (≥28 days); 42/429 (9.8%) reported symptoms lasting ≥90 days. Factors associated with higher odds of prolonged symptom duration included: hospitalisation for COVID-19 vs not hospitalised and mild acute symptoms (age-adjusted OR (aOR) 6.49, 95% CI 3.03 to 14.1), comorbidity count (aOR 1.11 per comorbidity, 95% CI 1.02 to 1.21) and osteoarthritis (aOR 2.11, 95% CI 1.01 to 4.27). COVID-19 onset in 2021 vs June 2020 or earlier was associated with lower odds of prolonged symptom duration (aOR 0.42, 95% CI 0.21 to 0.81). Conclusion Most people with SARDs had complete symptom resolution by day 15 after COVID-19 onset. However, about 1 in 4 experienced COVID-19 symptom duration 28 days or longer; 1 in 10 experienced symptoms 90 days or longer. Future studies are needed to investigate the possible relationships between immunomodulating medications, SARD type/flare, vaccine doses and novel viral variants with prolonged COVID-19 symptoms and other postacute sequelae of COVID-19 among people with SARDs.

ObjectiveTo describe demographics and imaging and compare findings and symptoms at presentation in a large cohort of persons with cricopharyngeus muscle dysfunction (CPMD) with and without hypopharyngeal diverticula.MethodologyProspective, multicenter cohort study of all individuals enrolled in the Prospective OUtcomes of Cricopharyngeal Hypertonicity (POUCH) Collaborative. Patient survey, comorbidities, radiography, laryngoscopy findings, and patient‐reported outcome measures (e.g., Eating Assessment Tool [EAT‐10]) data were abstracted from a REDCap database and summarized using means, medians, percentages, and frequencies. Diagnostic categories were compared using analysis of variance.ResultsA total of 250 persons were included. The mean age (standard deviation [SD]) of the cohort was 69.0 (11.2). Forty‐two percent identified as female. Zenker diverticula (ZD) was diagnosed in 85.2%, 9.2% with CPMD without diverticula, 4.4% with a Killian Jamieson diverticula (KJD), and 1.2% traction‐type diverticula. There were no differences between diagnostic categories in regard to age, gender, and duration of symptoms (p = 0.25, 0.19, 0.45). The mean (SD) EAT‐10 score for each group was 17.1 (10.1) for ZD, 20.2 (9.3) for CPMD, and 10.3 (9.4) for KJD. Patients with isolated CPMD had significantly greater EAT‐10 scores compared to the other diagnostic groups (p = 0.03).ConclusionZD is the most common, followed by CPMD without diverticula, KJD, and traction‐type. Patients with isolated obstructing CPMD may be more symptomatic than persons with ZD or KJD.Level of Evidence4 Laryngoscope, 133:1349–1355, 2023

Abstract Tγδ large granular lymphocyte leukemia (LGLL) is a rare variant of T-cell LGLL (T-LGLL) that has been less investigated as compared with the more frequent Tαβ LGLL, particularly in terms of frequency of STAT3 and STAT5b mutations. In this study, we characterized the clinical and biological features of 137 patients affected by Tγδ LGLL; data were retrospectively collected from 1997 to 2020 at 8 referral centers. Neutropenia and anemia were the most relevant clinical features, being present in 54.2% and 49.6% of cases, respectively, including severe neutropenia and anemia in ∼20% of cases each. Among the various treatments, cyclosporine A was shown to provide the best response rates. DNA samples of 97 and 94 cases were available for STAT3 and STAT5b mutation analysis, with 38.1% and 4.2% of cases being mutated, respectively. Clinical and biological features of our series of Tγδ cases were also compared with a recently published Tαβ cohort including 129 cases. Though no differences in STAT3 and STAT5b mutational frequency were found, Tγδ cases more frequently presented with neutropenia (P = .0161), anemia (P &lt; .0001), severe anemia (P = .0065), and thrombocytopenia (P = .0187). Moreover, Vδ2− cases displayed higher frequency of symptomatic disease. Overall, Tγδ cases displayed reduced survival with respect to Tαβ cases (P = .0017). Although there was no difference in STAT3 mutation frequency, our results showed that Tγδ LGLL represents a subset of T-LGLL characterized by more frequent symptoms and reduced survival as compared with Tαβ LGLL.

Introduction The COVID 19 pandemic increased the need for rapid and accurate diagnostic testing for COVID. When testing became available, a systems response was needed to efficiently accommodate the high-volume flow of patients who needed testing. Self-scheduling of COVID testing was developed to help patients safely and efficiently schedule their COVID testing online or with a mobile app. Methods We captured the counts of COVID test appointments, time patients spent in scheduling COVID test appointments, appointment lead times, and no-shows for COVID test appointments. For 17 months of self-scheduling, we retrospectively compared self-scheduling with the concurrent staff scheduling of COVID tests. Results From November 2020 through March 2022 there were 619 104 scheduled appointments for COVID testing with 22% (136 252) being self-scheduled. For asymptomatic self-scheduled COVID tests, accounting for 10.3% (63 605/619 104) of total COVID tests scheduled, median time to self-schedule was 3.1 min, interquartile range (IQR) [2.4,4.7]. For symptomatic self-schedulers accounting for 11.7% (72 647/619 104) of total COVID tests scheduled, the median time to self-triage and self-schedule was 5.8 min, IQR[4.3,8.9]. Self-scheduled COVID appointments increased to 44% (42 387/97 086) of the total COVID appointments during the peak month of January 2022. Median appointment lead time for symptomatic self-scheduled COVID test appointments was 6.6 h compared to 2.9 h ( P < .0001) for symptomatic staff scheduled appointments. However, adjusting for the 24% (32 194/135 252) that self-scheduled during hours when testing was unavailable, the median appointment lead time for symptomatic self-scheduled patients dropped to 3.6 h. No-shows were 2.5% for self-scheduled appointments compared to 3.0% no-shows that were staff scheduled (odds ratio 0.83, P < .0001). Conclusion COVID testing was self-scheduled for a large percent of scheduled COVID tests, taking patients only a few minutes to complete. Self-scheduling use increased over time, associated with a decreasing use of staff scheduled appointments and lower no-shows.