Towards a Diagnosis of Cardiac Amyloidosis: Single Center Experience with (99m) Technetium Pyrophosphate Planar Imaging and Opportunities for Standardization of Diagnostic Workflow

Background and Objectives: Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. Almost all forms of clinical cardiac amyloidosis are transthyretin amyloidosis (ATTR) or light chain amyloidosis. (99m) technetium pyrophosphate ((99m)Tc PYP scan) has changed the landscape of the non-biopsy diagnosis of ATTR cardiac amyloidosis (ATTR-CA) by providing remarkably high diagnostic accuracy. We examined our experience with PYP scans in patients undergoing workup for ATTR-CA and evaluated the diagnostic workflow in patients with intermediate PYP scan results. Materials and Methods: Retrospective chart review study in which we analyzed data of 84 patients who underwent c-99m pyrophosphate (PYP) SPECT scan for the diagnosis of ATTR-CA from 2017 till 2021 at our institution. We identified three groups: Low uptake (PYPL uptake ratio \u3c 1.2 + visual grade 1/0), n = 30, Intermediate uptake (PYPI uptake ratio 1.2-1.49, visual grade 2/3), n = 25 and High uptake (PYPH uptake ratio ≥ 1.5 + visual grade 2/3), n = 29. We reviewed patients\u27 demographics, medical histories, echo parameters and diagnostic testing including light chain analysis, cardiac magnetic resonance results, and biopsies. Results: Mean patients\u27 age was 73, male-to=female ratio 3:1, 59% of patients were African American. Cardiovascular comorbidities, cardiac biomarkers (BNP and Troponin) and amyloid-related neuropathy were similar in all groups. A statistically significant difference in septal thickness/posterior wall thickness and final diagnosis were found between the groups. The distribution of overall diagnostic testing ratios for the PYPI group included serum protein electrophoresis 92%, urine protein electrophoresis 65%, free light chain 80%, CMR 32%, tissue biopsy done in 20% and BM biopsy in 16%, which are similar to the ratios of other groups. Overall, 25% (n = 5, 4 TTR-CA and 1 AL Amyloid) of patients in the PYPI group had a final diagnosis of CA established with additional testing (p = 0.001 vs. other groups). Conclusions: The (99m)PYP scan is an accurate noninvasive test for cardiac ATTR-CA. Importantly, 25% of the PYPI group had a final diagnosis of ATTR-CA reiterating that diagnosis needs to be pursued in PYPI cases based on clinical suspicion. Routine evaluation and exclusion of light chain disease and establishing a consistent workflow for amyloid diagnosis and continued education for technologists and readers of PYP scans is key to a successful amyloidosis workup

Socioeconomic Determinants of Survival in Laryngeal Squamous Cell Carcinoma Patients

Objective: To determine whether comorbidities, socioeconomic factors, and race affect median survival times and overall survival outcomes for patients with laryngeal squamous cell carcinoma treated at a single institution with a large African American population. Study Design: Retrospective cohort study Setting: Tertiary care medical center. Subjects and Methods: Six-hundred nineteen patients treated for laryngeal squamous cell carcinoma at a single institution between 1999 and 2013. Kaplan-Meier survival curve analysis and Cox proportional hazard models were used. Results: Median survival time decreased with age ≥ 65 years (P \u3c .001), stage 3 or 4 (P \u3c .001),clinical T stage 3 or 4 (P \u3c .001), and clinical N stage 1, 2, or 3 (P \u3c .001). Living in zip code with increasing proportion of residents with high school degree or less (P \u3c .001),increasing proportion of residents living below the poverty level (P \u3c .001), and increasing Charlson Comorbidity Index (P \u3c .001) were associated with increased risk of death. Increasing median household income was associated with decreased increased risk of death (P \u3c .001). Caucasian, rather than African American, race was associated with decreased median survival time (P = .01). Conclusion: Socioeconomic factors and medical comorbidities were negative prognostic indicators of survival in patients with laryngeal squamous cell carcinoma. Contrary to previous reports, African American race was associated with improved overall survival in univariate analysis. However, once socioeconomic factors were controlled for, race conferred no effect on survival. Rather, living in neighborhoods with higher median household income was protective.https://scholarlycommons.henryford.com/merf2019hcd/1001/thumbnail.jp

Effect of Socioeconomic Status and Comorbidities on Thyroid Cancer Survival Outcomes

Purpose/Objectives: Thyroid cancer ranks among the most common head and neck malignancies and makes up 3% of new cancer cases per year, having increased in overall incidence in the last several decades. This study investigates patient characteristics, socioeconomic status (SES), and medical comorbidities as independent predictors of survival in patients with thyroid cancer, treated at a tertiary care hospital with a diverse, primarily low SES patient population. Materials/Methods: The Henry Ford Health System’s Virtual Data Warehouse Tumor Registry was used to identify patients with thyroid cancers from 1997 to 2016. Socioeconomic data was obtained from 2010 US Census. Comorbidities were quantified using the Charlson Comorbidity Index (CCI). Statistical analysis was performed using Kaplan-Meier estimator and Cox proportional hazards models. Results: There were 1042 patients with thyroid cancer. 5- and 10-year survival probabilities decreased with age \u3e=60 years (p\u3c0.001), stage 3/4 (p\u3c0.001), clinical N stage 1-3 (p\u3c0.001), black race (p=0.001), CCI\u3e1 (p\u3c0.001), and lowest quartile median household income (p\u3c0.001). In multivariate analysis, age (1-year increase HR: 1.97, 95% CI: 1.06-1.09), sex (female vs. male HR: 0.67, 95% CI: 0.48-0.95), and CCI (1-point increase HR: 1.26, 95% CI: 1.19-1.34) were significantly associated with survival outcomes. A CCI cutoff of \u3e1 was a reliable predictor of mortality (AUC: 0.759, 95% CI: 0.716-0.801). CCI was significantly correlated with stage (r=0.166, p=0.004) and median household income (r=-0.175, p\u3c0.001). Conclusion: Increased age, male sex, and medical comorbidities predicted significantly worse survival in thyroid cancer. Race and median household income were not independent significant prognostic indicators

Class III β-tubulin expression as a predictor of docetaxel-resistance in metastatic castration-resistant prostate cancer

About half of the patients treated with docetaxel in the setting of metastatic castration-resistant prostate cancer (CRPC) are non-responders. Therefore, a marker of response would be beneficial for clinical decision-making. We evaluated class III β-tubulin (βIII-tubulin) expression as a predictor of resistance in this setting, which previously has been correlated with lack of response to taxanes in other cancers. Patients with CRPC were included if they were treated with at least 3 cycles of docetaxel between 1990 and 2011. βIII-tubulin expression was assessed by immunostaining, which was performed in tissue samples obtained either via biopsy or prostatectomy at the time of diagnosis. Rates of prostate-specific antigen (PSA) response and overall survival (OS) following docetaxel treatment were compared between patients with high (2+ or 3+ staining) vs. low (0 or 1+ staining) βIII-tubulin expression. Of 73 patients, 26 (35%) had a high expression of βIII-tubulin. A PSA decline of 10% or greater occurred in 65% of patients with a high βIII-tubulin expression vs. 89% with a low βIII-tubulin expression (p = 0.0267). The median OS for patients with a high βIII-tubulin expression was 17.4 (95% CI 8.7-21.0) months vs. 19.8 (95% CI 16.6-23.6) months for patients with a low expression (p = 0.039). Our results show that a high βIII-tubulin expression is a negative prognostic factor in metastatic CRPC patients treated with docetaxel

Conversational Vocal Intensity in Parkinson\u27s Disease: Treatment and Environmental Comparisons

BACKGROUND: Vibrotactile Feedback (VF) using wearable devices is an emerging treatment option for hypophonia in Individuals with Parkinson\u27s disease (IwPD). Studies evaluating the effectiveness of VF in improving conversational vocal intensity in real-life environment in IwPD are limited. OBJECTIVE: To determine the effect of VF on conversational vocal intensity and compare vocal intensity between a) clinic and real-life environment b) VF and Lee Silverman Voice Treatment (LSVT LOUD®)vs. VF alone in IwPD using a portable voice monitor (VocaLog2). METHODS: Eight individuals with hypophonia secondary to PD were randomly assigned to two treatment groups- VF and LSVT LOUD® (Group 1) and VF (Group 2). VF was provided using VocaLog2 device. Duration of treatment was 4 weeks for both groups. Vocal intensity was measured in the real-life environment at baseline, during treatment, and at one-month follow-up. Vocal intensity in clinic was obtained at baseline and one-month follow-up. Voice Handicap Index (VHI) questionnaire was administered at baseline and one-month follow-up. RESULTS: There was no significant difference in conversational vocal intensity between a) clinic and real-life environment at any point of time b) baseline and follow up for both treatment groups c) the two treatment groups at baseline, during each of the 4 weeks of treatment and at follow up d) VHI baseline and one month follow up scores. CONCLUSION: VF, including when combined with LSVT LOUD®, is limited in improving conversational vocal intensity in real-life in IwPD. The effects of frequency and duration of VF on conversational vocal intensity must be systematically investigated using large scale studies in IwPD

Towards a Diagnosis of Cardiac Amyloidosis: Single Center Experience with ^99m Technetium Pyrophosphate Planar Imaging and Opportunities for Standardization of Diagnostic Workflow

Background and Objectives: Cardiac amyloidosis is a disorder caused by amyloid fibril deposition in the extracellular space of the heart. Almost all forms of clinical cardiac amyloidosis are transthyretin amyloidosis (ATTR) or light chain amyloidosis. 99m technetium pyrophosphate (99mTc PYP scan) has changed the landscape of the non-biopsy diagnosis of ATTR cardiac amyloidosis (ATTR-CA) by providing remarkably high diagnostic accuracy. We examined our experience with PYP scans in patients undergoing workup for ATTR-CA and evaluated the diagnostic workflow in patients with intermediate PYP scan results. Materials and Methods: Retrospective chart review study in which we analyzed data of 84 patients who underwent c-99m pyrophosphate (PYP) SPECT scan for the diagnosis of ATTR-CA from 2017 till 2021 at our institution. We identified three groups: Low uptake (PYPL uptake ratio n = 30, Intermediate uptake (PYPI uptake ratio 1.2–1.49, visual grade 2/3), n = 25 and High uptake (PYPH uptake ratio ≥ 1.5 + visual grade 2/3), n = 29. We reviewed patients’ demographics, medical histories, echo parameters and diagnostic testing including light chain analysis, cardiac magnetic resonance results, and biopsies. Results: Mean patients’ age was 73, male-to=female ratio 3:1, 59% of patients were African American. Cardiovascular comorbidities, cardiac biomarkers (BNP and Troponin) and amyloid-related neuropathy were similar in all groups. A statistically significant difference in septal thickness/posterior wall thickness and final diagnosis were found between the groups. The distribution of overall diagnostic testing ratios for the PYPI group included serum protein electrophoresis 92%, urine protein electrophoresis 65%, free light chain 80%, CMR 32%, tissue biopsy done in 20% and BM biopsy in 16%, which are similar to the ratios of other groups. Overall, 25% (n = 5, 4 TTR-CA and 1 AL Amyloid) of patients in the PYPI group had a final diagnosis of CA established with additional testing (p = 0.001 vs. other groups). Conclusions: The 99mPYP scan is an accurate noninvasive test for cardiac ATTR-CA. Importantly, 25% of the PYPI group had a final diagnosis of ATTR-CA reiterating that diagnosis needs to be pursued in PYPI cases based on clinical suspicion. Routine evaluation and exclusion of light chain disease and establishing a consistent workflow for amyloid diagnosis and continued education for technologists and readers of PYP scans is key to a successful amyloidosis workup

Middle turbinate resection is unlikely to cause empty nose syndrome in first year postoperatively

PURPOSE: Empty nose syndrome (ENS) is characterized by nasal dryness, crusting, and paradoxical nasal obstruction most commonly after inferior turbinate resection. ENS has also been reported to occur after middle turbinate resection (MTR), and concern for causing ENS is a possible reason surgeons preserve the MT during endoscopic sinus surgery (ESS). The objective was to determine whether MTR during ESS led to ENS. MATERIALS AND METHODS: This was a prospective case series of 95 consecutive patients that underwent bilateral subtotal MTR during ESS with either Draf IIB or Draf III frontal sinusotomies, for chronic rhinosinusitis with or without nasal polyps, and frontal sinus inverted papillomas. Demographic data and postoperative Empty Nose Syndrome 6-item Questionnaire (ENS6Q) scores were obtained. Nasal crusting was also documented on last postoperative nasal endoscopy. RESULTS: Pathologies included chronic rhinosinusitis with nasal polyps (69), without nasal polyps (12), and inverted papillomas (14). Fifty-six patients underwent subtotal MTRs during ESS with Draf IIB, and 39 with Draf III. Mean follow-up was 19.4 months (range 12-49). Mean postoperative ENS6Q score was 2.1. Only 2.1% had ENS6Q scores ≥ 11, and 6.3% had nasal crusting at last follow-up. None of the patients with ENS6Q scores ≥ 11 had nasal crusting at last follow-up. There were no significant differences in outcomes between ages, genders, surgery types, or pathologies. CONCLUSIONS: Patients who underwent bilateral subtotal MTR during ESS were unlikely to develop ENS by at least 1 year postoperatively, based on patients rarely experiencing ENS6Q scores ≥ 11 or persistent nasal crusting

Parathyroid Gland Autofluorescence Characteristics in Patients With Primary Hyperparathyroidism

OBJECTIVE: Near-infrared imaging for intraoperative parathyroid gland (PG) detection has recently commanded significant attention. The PTeye (Medtronic, Minneapolis, MN) is a probe-based system for near-infrared autofluorescent evaluation of PGs. This study was designed to evaluate the capabilities of the PTeye in the setting of surgery for primary hyperparathyroidism. STUDY DESIGN: Prospective, Cohort study. METHODS: This single-institution, prospective cohort study included all patients undergoing parathyroidectomy for primary hyperparathyroidism with presumed single gland disease from June 2020 to December 2020. Absolute intensity and intensity ratios, with the thyroid as the control tissue, were obtained for the adenoma, ipsilateral normal PG, and adjacent tissue. The ability of the PTeye to function when not in direct contact with tissue was measured. RESULTS: Twenty-two patients were included. The median intensity ratio for the in situ adenomas was 4.38 (interquartile range [IQR]: 2.03-5.87), ipsilateral normal PGs 6.17 (IQR: 3.83-7.67), strap muscle 0.47 (IQR: 0.30-0.60), and fat 0.20 (IQR: 0.17-0.47). All normal PGs and 21/22 adenomas demonstrated autofluorescence above the detection threshold. The PTeye functioned at a maximum distance of separation of 10 mm through saline medium and 6 mm through clear solid medium. CONCLUSION: This study confirms the PTeye\u27s ability to recognize PGs with a high degree of precision. The device was found to function properly even with the probe not in direct contact with the tissue. Although adenomatous PGs appear to demonstrate altered autofluorescent properties from normal PGs, additional research is required to determine if these differences are clinically useful. LEVEL OF EVIDENCE: 3 Laryngoscope, 2021

Clinical Characteristics, Diagnostic and Outcome Differences in Patients with Optic Neuritis

Objective: To identify differences in demographics, clinical characteristics, diagnostic studies and outcomes in patients with optic neuritis. Background: Optic neuritis (ON) can be the initial presenting symptom of a variety of immunemediated neurological diseases such as multiple sclerosis(MS), neuromyelitis optica(NMO), neurosarcoidosis(NS) lupus and myelin oligodendrocyte glycoprotein-IgG-associated(MOG) optic neuritis. Design/Methods: Retrospective chart review was performed on patients with the diagnosis of optic neuritis seen between January 2016 through December 2017. Patients demographics, etiology, clinical presentation, final diagnosis, laboratory, imaging data, recurrence rate, and visual outcome were recorded. Univariate two-group comparisons are carried out using chi-square or Fisher\u27s exact tests for categorical variables and using Wilcoxon rank-sum tests for continuous variables. Results: 184 patients with diagnosis of ON were included. MS was the most common etiology 88(48%) followed by isolated optic neuritis (ION) 48(26%), NMO 17(9.2%), clinically isolated syndrome(CIS) 16(8.7%), NS 5(2.7%), chronic relapsing inflammatory optic neuropathy (CRION) 5(2.7%) and MOG 5(2.7%). There were significantly more patients on oral steroids(96% vs 46%, p\u3c0.001), more recurrence (81% vs 27%, p\u3c0.001), more MRI orbit enhancement(67% vs 42%, p=0.039), lower CSF WBC count(3.7±3.9 versus 11.4±15.4, p=0.003), lower CSF IgG index(0.7±0.7 vs 1.0±0.7, p=0.005), fewer positive CSF O bands(17% vs 54%, p=0.005), and more normal optic coherence tomography(OCT)(58% vs 29%, p=0.015) in the NMO/CRION/MOG group as compared to the MS/Sarcoid/ION/CIS group. Patients with intracanalicular segment of ON(25) were compared to non-intracanalicular segment of ON(117)and there were significantly more patients positive for MRI orbit enhancement in the Intracanalicular group(80% versus 38%, p\u3c0.001) with high rate of recurrence(64% vs 36%, p=0.009). Conclusions: MS remains the most common etiology for ON followed by NMO. A High rate of recurrence and MRI orbit enhancement with initial normal OCT can be seen in, patient with ON associated with NMO/CRION/MOG. The intracanalicular segment of ON can be associated with optic nerve enhancement and high risk of recurrence