Predictors of Mortality in Veterans with Amyotrophic Lateral Sclerosis: Respiratory Status and Speech Disorder at Presentation.

BACKGROUND There is a lack of accurate models to predict amyotrophic lateral sclerosis (ALS) disease course and outcomes. As a result, risk assessment and counseling, the timing of interventions, and their stratification in clinical trials are difficult. This study aimed to evaluate the association between symptoms at presentation and mortality. MATERIAL AND METHODS A single veterans hospital reviewed the electronic records of 105 veterans with ALS who were periodically followed in our ALS clinic between 2010 and 2021. A survival decision tree (≤3 or >3 years) was generated based on the statistical median survival of our data. The variables known to influence survival when alive were compared to patients who died. RESULTS The (mean±SD) age at onset was 62±11 years, M/F ratio 101: 4, and 90% were non-Hispanic whites. The initial score for the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) was 31±8.3. Dysarthria and shortness of breath (SOB) were present on initial presentation in 52 (49.5%) and 32 (30.5%) patients, respectively. Deaths occurred in 80 (76.2%) patients during the study period. The main cause of death was respiratory disease (failure and pneumonia, n=43 53.75%). Patients survived for >3 years on initial presentation with normal respiration and speech, compared to ≤3 years of survival in patients with dysarthria and SOB, irrespective of age. CONCLUSIONS This study suggests that for veterans with ALS, the main predictors of shorter survival were respiratory status and speech disorder on initial presentation to the clinic.

Call to Action: The Need for a Standardized Emotional Debriefing Model for Clinical Settings.

Clinicians in high-acuity hospital settings experience chronic distress due to the secondhand trauma experienced at work. Chaplains are often responsible for providing staff support to address this distress. One form of staff support is emotional debriefing after critical events. There are few publications about emotional debriefings. It would benefit chaplains to engage in research and discovery regarding emotional debriefing and create a standard model for chaplains to use in staff support.

The Benefits and Limitations of Targeted Training in Flexible Transnasal Laryngoscopy Diagnosis.

Importance: Targeted laryngoscopy training can be used successfully in de novo learners. Objective: To determine the value of targeted laryngoscopy education in interns. Design, Setting, and Participants: This prospective study of fiberoptic laryngoscopy interpretations enrolled 13 participants in an academic hospital setting from August 1 to December 31, 2015. Participants included 10 postgraduate year 1 emergency and otolaryngology interns and 3 board-certified otolaryngology attending physicians. Interventions: Participants viewed 25 selected and digitally recorded fiberoptic laryngoscopies and were asked to rate 13 items relating to abnormalities in the pharynx, hypopharynx, larynx, and subglottis; the level of concern; and confidence with the diagnosis. A laryngoscopy teaching video was then administered to the interns before rating a second set of 25 videos. Improvement in diagnosis and intraclass correlation coefficients (ICC) were calculated for each question and compared between the first and second administration. Main Outcomes and Measures: Improvement in correct diagnosis of abnormalities in recorded laryngoscopies. Results: All 13 participants completed the interventions. The ICCs for all questions were generally low for the intern groups and higher for the attending group. For vocal cord mobility, a preintervention ICC of 0.25 (95% CI, 0.16-0.37) improved to 0.47 (95% CI, 0.36-0.59) among interns after the intervention. The ICCs for vocal cord mobility were higher among attendings for the preintervention (0.89; 95% CI, 0.84-0.93) and postintervention (0.89; 95% CI, 0.83-0.93) assessments. Minimal improvement was observed in intern scores for base of tongue abnormalities, subglottic stenosis, vocal cord abnormalities, level of comfort, level of concern, pharyngeal abnormalities, or laryngeal, pharyngeal, and hypopharyngeal masses. Conclusions and Relevance: Learning of flexible laryngoscopy can be improved with the use of a teaching video; however, additional interventions are needed to attain competence in accurately diagnosing upper airway lesions. Clinicians who seek to perform flexible laryngoscopy require robust training.

Perceptions of Burnout, Its Prevention, and Its Effect on Patient Care as Described by Oncology Nurses in the Hospital Setting
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PURPOSE/OBJECTIVES: To identify overall perceptions of burnout within the inpatient oncology nursing population, how they perceived that burnout affected the care they provided, and how they perceived that burnout could be decreased.
. DESIGN: A quantitative descriptive study using questionnaires to describe perceptions of burnout.
. SETTING: A university-affiliated hospital using inpatient oncology nurses from three nursing units at University of Pittsburgh Medical Center Presbyterian in Pennsylvania.
. SAMPLE: A convenience sample of 61 nurses. 
. METHODS: Two instruments were used to investigate various aspects about perceptions of burnout among inpatient oncology nurses. Nurses participated on an anonymous voluntary basis by completing these instruments. 
. MAIN RESEARCH VARIABLES: Perceived burnout, perception of how burnout affects care provided to patients, and strategies to relieve burnout.
. FINDINGS: Inpatient oncology nurses report a moderate level of perceived burnout. In addition, this nursing population perceived that this burnout had a negative impact on the care they provided. Nurses believed they experienced burnout because of increased nurse-patient ratios and skipped or shortened lunches or breaks. However, they perceived that burnout could be prevented when adequate resources, collaboration, teamwork, and the support of family and friends existed. 
. CONCLUSIONS: As a result of the level of care needed by inpatients with cancer, the association between burnout experienced by nurses and how it can affect care is important to recognize. One such association identified was that a relationship existed between the nurses' interactions with patients' family, friends, or visitors and increased perceptions of burnout and depersonalization. As a result, nurses can experience increased burnout and act in a manner that lacks compassion because of emotional detachment.
. IMPLICATIONS FOR NURSING: With extremely ill inpatients with cancer, nurses need to be able to manage high levels of demands from patients and their family members to provide quality and compassionate care. Nurses reported that they experience burnout because of emotional exhaustion and depersonalization as a result of missed, shortened, or skipped breaks and lunches. This perception can affect the nurse's ability to perform physically and mentally, resulting in negative effects on nurse-patient relationships.

Monoclonal gammopathy of undetermined significance: Using risk stratification to guide follow-up.

Varying combinations of 3 measurable factors determine a patient's risk of progressing toward multiple myeloma and influence monitoring decisions. This review--and accompanying algorithm--can guide your approach. For monoclonal gammopathy of undetermined significance (MGUS) patients at low risk, repeat serum protein electrophoresis (SPE) in 6 months. If no significant elevation of M-protein is found, repeat SPE every 2 to 3 years.

Time to competency, reliability of flexible transnasal laryngoscopy by training level: a pilot study.

OBJECTIVE: To determine the progression of flexible transnasal laryngoscopy reliability and competency in otolaryngology residency training. STUDY DESIGN: Prospective case control study. SETTING: Academic otolaryngology department. SUBJECTS: Medical students, otolaryngology residents, and otolaryngology attending physicians. METHODS: Fourteen otolaryngology residents from PGY-1 to PGY-5 and 3 attending otolaryngologists viewed 25 selected and digitally recorded flexible transnasal laryngoscopies. The evaluators were asked to rate 13 items relating to abnormalities in the oropharynx, hypopharynx, larynx, and subglottis. The level of concern and level of comfort with the diagnosis were assessed. Intraclass correlations were calculated for each topic and by level of training to determine reliability within each class and compare competency versus attending interpretations. RESULTS: Intraclass correlation of residents compared to attending physicians demonstrated significant improvements by year for left and right vocal fold immobility, subglottic stenosis, laryngeal mass, left and right vocal cord abnormalities, and level of concern. Additionally, pooled vocal cord mobility and pooled results in categories with good attending reliability demonstrated stepwise improvement as well. For these categories, resident reliability was found to be statistically similar to attending physicians in all categories by PGY-3. There were no trends for base of tongue abnormalities, pharyngeal abnormalities, and pharyngeal and hypopharyngeal masses. CONCLUSIONS: Resident competency for flexible transnasal laryngoscopy progresses during residency to reliability with attending otolaryngologists by the PGY-3 year over key facets of the examination.

Predictive findings of allergic disease in fiberoptic nasolaryngoscopy.

OBJECTIVES/HYPOTHESIS: To determine whether findings on fiberoptic nasolaryngoscopy beyond the nasal cavity can aid in diagnosis of atopy. STUDY DESIGN: Case control analysis of patients undergoing fiberoptic nasolaryngoscopy and allergy testing at a single academic institution. METHODS: Patients who underwent flexible nasolaryngoscopy for either laryngeal or nasal symptoms and allergy testing by in vitro methods were divided into an atopic group and a nonatopic control group based on results of allergy testing. Three board-certified otolaryngologists who were blinded to the atopic status and symptoms viewed 88 patient videos and filled out an 8-item endoscopic rating questionnaire for each. Correlation between rater scores, endoscopic findings, and atopic status was calculated using Randolph's multirater kappa values and Mann-Whitney test. RESULTS: Intrarater reliability was moderate to perfect for all physicians on all questions (kappa 0.545-1.0). Inter-rater reliability was slight to fair (kappa 0.143-0.399) for all questions and the overall impression of atopic disease. Abnormalities of the torus tubarius (P = .007) and increased nasopharyngeal secretions (P = .038) were predictive of atopic disease, whereas the presence of an adenoid (P = .08) and impression of atopic disease (P = .15) approached significance. All other endoscopic measures were not predictive of atopic status. CONCLUSIONS: Fiberoptic nasolaryngeal findings within the nasopharynx rather than the larynx are predictive of a positive atopic status. LEVEL OF EVIDENCE: 3b.

Influence of adolescence and obesity on the rate of stillbirth.

BACKGROUND: We hypothesise that the rate of stillbirth is increased in mothers younger than 18 years of age compared to adult mothers, and that obesity further increases the risk of stillbirth in this population. METHODS: We conducted a population-based cohort study comparing rates of stillbirth between adolescent, defined as young women under the age of 18 and adult women. We then compared the rate of stillbirth in normal weight vs. obese adolescents. These effects were stratified according to gestational age. Log-binomial regression models were used to estimate the effect of adolescence and obesity on stillbirth risk while adjusting for important confounders. Risk ratios (RR) with 95% confidence intervals [CI]were calculated. RESULTS: We reviewed data from 650 760 births in Missouri between 1998 and 2005. Stillbirth rates were 6.7 and 4.1 per 1000 in adolescents and adult women, respectively (RR 1.2, 95% CI 1.03-1.5). A higher proportion of stillbirths occurred prior to 28 weeks in adolescents vs. adults (53% vs. 37% respectively, P = 0.002). The risk of stillbirth in obese adolescents was further increased over normal weight adolescents (adjusted RR [aRR] 1.7, 95% CI 1.02-2.9). CONCLUSION: Adolescent pregnancies, particularly obese adolescents, are at an increased risk of stillbirth.

Acute profound thrombocytopenia associated with readministration of eptifibatide: case report and review of the literature.

The glycoprotein IIb-IIIa inhibitor eptifibatide has been shown to be beneficial in the treatment of acute coronary syndromes and during percutaneous coronary intervention (PCI). Case reports of acute profound thrombocytopenia have been reported with eptifibatide, yet the true incidence of this reaction is unknown. We describe a 50-year-old woman with severe coronary artery disease who developed acute profound thrombocytopenia after readministration of eptifibatide. Eptifibatide was administered through hospital day 3, when it was discontinued in preparation for coronary angiography and PCI; the drug was restarted on day 5. On hospital day 6, she was noted to have a platelet count below 5 x 10(3)/mm,(3) indicating a profound decrease from a baseline of 456 x 10(3)/mm(3) on admission. Eptifibatide, heparin, vancomycin, and clopidogrel were potential causative agents. Anticoagulation and vancomycin were stopped, and her platelet count increased to 30 x 10(3)/mm(3) on day 7. Subsequent reexposure to heparin and vancomycin yielded no adverse effects. The patient's platelet count increased over the remainder of her hospitalization, and she was discharged home on day 19. Based on clinical presentation and negative heparin platelet factor 4 antibody test, eptifibatide was the most likely cause of thrombocytopenia. Use of the Naranjo adverse drug reaction probability scale indicated that eptifibatide was the probable cause of thrombocytopenia (score of 5); scores of 1 (possible) or 0 (doubtful) were derived with heparin, vancomycin, and clopidogrel. We conducted a literature search and compiled information from published case reports to describe the pattern of onset and recovery of eptifibatide-induced thrombocytopenia. In all patients receiving eptifibatide, routine platelet counts should be monitored at baseline and within 2-6 hours after starting the drug.

Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis.

OBJECTIVE: Human neutrophil elastase (HNE) and proteinase 3 (PR3) are structurally and functionally related. PR3 is the prominent target antigen for antineutrophil cytoplasmic antibodies (ANCAs) in Wegener's granulomatosis (WG). Reported frequencies of HNE ANCAs in WG and other autoimmune diseases range from 0% to 20%. We previously detected HNE ANCAs in patients with cocaine-induced midline destructive lesions (CIMDL). We tested the hypothesis that discrepancies in the reported frequencies of HNE ANCAs in patients with vasculitis may be related to differences in detection methods, and that HNE ANCA may be a marker for CIMDL. METHODS: HNE ANCA reactivity in 25 patients with CIMDL was characterized and compared with that in a control cohort of 604 consecutive patients (64 with WG, 14 with microscopic polyangiitis [MPA], and 526 others) and 45 healthy volunteers. HNE ANCAs were measured by indirect immunofluorescence using a previously undescribed expression system for recombinant HNE and by direct and capture enzyme-linked immunosorbent assays using purified native HNE as target antigen. RESULTS: Among patients with CIMDL, HNE ANCAs were detectable by 1 assay in 84%, by 2 assays in 68%, and by all 3 assays in 36%. Fifty-seven percent of HNE ANCA-positive CIMDL sera were also PR3 ANCA-positive by at least 1 assay. In contrast, only 8 (1.3%) of 604 control sera reacted with HNE in at least 1 assay, 3 (0.5%) reacted in 2 assays, and only 1 serum sample (0.16%) reacted in all 3 assays. Sera obtained from patients with WG or MPA were universally HNE ANCA-negative, as were sera obtained from healthy controls. CONCLUSION: Optimal sensitivity for HNE ANCA requires multimodality testing. HNE ANCAs are frequent in CIMDL but not in other autoimmune diseases, including classic ANCA-associated vasculitis. HNE ANCAs may discriminate between CIMDL and WG, whereas a positive test result for PR3 ANCA may not.

Different gender-associated genotype risks of Wegener's granulomatosis and microscopic polyangiitis.

Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are systemic small vessel vasculitides associated with ANCA (AAV). Predominant Th1 and Th2 cytokine patterns have been reported for WG and MPA, respectively. Consequently, genotypes suppressing Th1 responses or augmenting Th2 responses may be more frequent in MPA than in WG. Transforming growth beta1 (TGF-beta1) and interleukin-10 (IL-10) genes may modify the course of vasculitis. Therefore, we investigated associations between genotype frequencies of functional polymorphisms of these cytokine genes and clinical manifestations in AAV. One hundred sixty-one AAV patients and 153 healthy blood donors were genotyped for the biallelic polymorphism in codon 25 of the TGF-beta1 gene and the biallelic polymorphism at position -1082 of the IL-10 gene. No difference was found for TGF-beta1 codon 25 polymorphism between control and patient groups. In contrast, a significant shift toward the homozygous AA genotype of the IL-10 (-1082) polymorphism was found in WG (25%, p<0.005) and MPA patients (39%; p<0.00001) compared to controls (10.5%). Furthermore, in MPA the AA homozygous genotype was significantly more frequent in females (62.5%) compared to males (20%, p<0.05). A contribution of the TGF-beta1 codon 25 polymorphism to the susceptibility-defining genetic backgrounds of AAV appears unlikely. In contrast, our findings suggest a role of the enhanced IL-10 (-1082) PM in WG and MPA with a significant gender difference in MPA.

Detection of anti-neutrophil cytoplasmic antibodies under actual clinical testing conditions.

Anti-neutrophil cytoplasmic antibodies (ANCA) are a useful diagnostic tool for Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA). To maximize diagnostic utility, current guidelines recommend dual testing by standard indirect immunofluorescence (IIF) and target antigen-specific assays. Most published data come from specialized research laboratories, not reflecting the performance of assays under routine clinical conditions. Therefore, we compared the performance of standard IIF, PR3-, and MPO-ANCA-specific direct ELISA, and a PR3-ANCA-specific capture ELISA used alone and in combination under routine clinical conditions. Consecutive serum samples (615) submitted for routine ANCA testing over a 10-month period were assayed. Diagnoses were WG/MPA (n = 86), other autoimmune disease (n = 118), and various others (n = 411). The combination of PR3-ANCA and MPO-ANCA ELISA had the highest sensitivity (72.1%), and C-ANCA determination using IIF, the highest specificity (99.6%). While maintaining maximal diagnostic accuracy, significant labor savings are achieved by screening for WG/MPA by ELISA followed by confirmatory IIF.