Cardiac Sarcoidosis: A Unique Presentation.

Isolated cardiac sarcoidosis is a rare subset of sarcoidosis, a systemic autoimmune condition primarily found in African American females. The manifestations of cardiac sarcoidosis include atrioventricular and bundle branch blocks, arrhythmias, heart failure, and pericardial effusions, although these complications occur at varying prevalence. The diagnosis of cardiac sarcoidosis requires several different criteria; however, recent literature has focused heavily on imaging modalities such as cardiac magnetic resonance imaging. We present a case of a 42-year-old Caucasian male who was found to have unexplained cardiac arrhythmias and ultimately diagnosed with cardiac sarcoidosis by imaging modalities.

Neuromyelitis Optica Spectrum Disorder: A Rare Cause of Spinal Cord and Optic Nerve Involvement.

Neuromyelitis optica spectrum disorder (NMOSD) is defined as a rare central nervous system, demyelinating, autoimmune disorder with autoantibodies against aquaporin-4. Commonly affecting females, NMOSD is known to also be a relapsing disease and can increase in severity during each episode. Diagnostic criteria include ruling out multiple sclerosis, spinal magnetic resonance imaging, and autoantibody detection. While management focuses on relapse treatment and prevention, high-dose steroids and plasma exchange have proven to be useful options. We present a case of a young female presenting with NMOSD relapse appropriately managed with plasma exchange.

Barriers to Physiotherapists' Use of Professional Development Tools for Chronic Pain: A Knowledge Translation Study.

Purpose: The Pain Science Division (PSD) is a special interest group of the Canadian Physiotherapy Association that serves physiotherapists who have an interest in better understanding and managing patients' pain. The PSD developed evidence-based resources for its members with the goal of improving patient care by supporting professional development. However, online metrics tracking access to these resources indicated that access was low. The purpose of this study was to identify the barriers PSD members encountered to the use of PSD resources and to recommend interventions to address these barriers guided by the Theory and Techniques Tool (TTT). Method: We distributed an online survey to PSD members across Canada. We used the TTT, a knowledge translation tool, to guide the design of the questionnaire and identify actionable findings. Results: Response rates from 621 non-student members and 1,470 student members were 26.9% and 1.4%, respectively. Based on the frequency of practising physiotherapists' (n = 167) agreement with items in the TTT, the primary barriers to use of the PSD resources were forgetting that the resources were available and forgetting to use them. Conclusions: The TTT can be used to identify barriers to use of professional development tools.

Objectif : la division science de la douleur (DSD) est un groupe d'intérêt de l'Association canadienne de physiothérapie destiné aux physiothérapeutes qui souhaitent mieux comprendre et gérer la douleur des patients. La DSD a préparé des ressources fondées sur des données probantes pour ses membres afin d'améliorer les soins aux patients grâce au perfectionnement professionnel. Cependant, les mesures virtuelles qui surveillent l'accès à ces ressources ont révélé que cet accès était limité. La présente étude visait à établir les obstacles auxquels se sont heurtés les membres de la DSD pour utiliser les ressources de la DSD et à recommander des interventions afin d'écarter ces obstacles en fonction de l'outil Theory and Techniques (TTT). Méthodologie : les chercheurs ont distribué un sondage en ligne aux membres de la DSD du Canada. Ils ont utilisé le TTT, un outil d'application des connaissances, pour orienter la conception du questionnaire et déterminer les résultats réalisables. Résultats : le taux de réponse des 621 membres non étudiants et des 1 470 membres étudiants s'établissait à 26,9 % et à 1,4 %, respectivement. D'après la fréquence d'accord des physiothérapeutes en exercice (n = 167) avec les points du TTT, l'oubli de l'existence des ressources ou l'oubli de les utiliser étaient les principaux obstacles à l'utilisation des ressources de la DSD. Conclusions : le TTT peut être utilisé pour déterminer les obstacles à l'utilisation d'outils de perfectionnement professionnel.

Brain Vital Signs in Elite Ice Hockey: Towards Characterizing Objective and Specific Neurophysiological Reference Values for Concussion Management.

Background: Prior concussion studies have shown that objective neurophysiological measures are sensitive to detecting concussive and subconcussive impairments in youth ice-hockey. These studies monitored brain vital signs at rink-side using a within-subjects design to demonstrate significant changes from pre-season baseline scans. However, practical clinical implementation must overcome inherent challenges related to any dependence on a baseline. This requires establishing the start of normative reference data sets. Methods: The current study collected specific reference data for N = 58 elite, youth, male ice-hockey players and compared these with a general reference dataset from N = 135 of males and females across the lifespan. The elite hockey players were recruited to a select training camp through CAA Hockey, a management agency for players drafted to leagues such as the National Hockey League (NHL). The statistical analysis included a test-retest comparison to establish reliability, and a multivariate analysis of covariance to evaluate differences in brain vital signs between groups with age as a covariate. Findings: Test-retest assessments for brain vital signs evoked potentials showed moderate-to-good reliability (Cronbach's Alpha > 0.7, Intraclass correlation coefficient > 0.5) in five out of six measures. The multivariate analysis of covariance showed no overall effect for group (p = 0.105), and a significant effect of age as a covariate was observed (p < 0.001). Adjusting for the effect of age, a significant difference was observed in the measure of N100 latency (p = 0.022) between elite hockey players and the heterogeneous control group. Interpretation: The findings support the concept that normative physiological data can be used in brain vital signs evaluation in athletes, and should additionally be stratified for age, skill level, and experience. These can be combined with general norms and/or individual baseline assessments where appropriate and/or possible. The current results allow for brain vital sign evaluation independent of baseline assessment, therefore enabling objective neurophysiological evaluation of concussion management and cognitive performance optimization in ice-hockey.

Staphylococcus capitis Endocarditis of a Native Valve.

Described as primarily an opportunistic pathogen, Staphylococcus capitis is primarily found as normal flora of the human skin but has been defined in literature as being a rare cause of infective endocarditis. We present a case of an otherwise healthy 65-year-old male who presented to our institution with symptoms similar to septic emboli. Blood cultures were obtained and ultimately grew Staphylococcus capitis in both bottles with repeat cultures one day later confirming the growth. A transthoracic echocardiogram was performed showing an ejection fraction of 60-65% and a thickened mitral value with mild-to-moderate mitral regurgitation. He was ultimately treated with IV cefazolin and improved with symptom resolution in outpatient follow-up. Staphylococcus capitis pathogenesis continues to be poorly understood, yet aggressive management with surgery and antibiotics has proven to decrease morbidity and mortality.

Improving Hospital Length of Stay: Results of a Retrospective Cohort Study.

(1) Background: Jersey Shore University Medical Center (JSUMC) is a 646-bed tertiary medical center located in central New Jersey. Over the past several years, development and maturation of tertiary services at JSUMC has resulted in tremendous growth, with the inpatient volume increasing by 17% between 2016 and 2018. As hospital floors functioned at maximum capacity, the medical center was frequently forced into crisis mode with substantial increases in emergency department (ED) waiting times and a paradoxical increase in-hospital length of stay (hLOS). Prolonged hLOS can contribute to worse patient outcomes and satisfaction, as well as increased medical costs. (2) Methods: A root cause analysis was conducted to identify the factors leading to delays in providing in-hospital services. Four main bottlenecks were identified by the in-hospital phase sub-committee: incomplete orders, delays in placement to rehabilitation facilities, delays due to testing (mainly imaging), and delays in entering the discharge order. Similarly, the discharge process itself was analyzed, and obstacles were identified. Specific interventions to address each obstacle were implemented. Mean CMI-adjusted hospital LOS (CMI-hLOS) was the primary outcome measure. (3) Results: After interventions, CMI-hLOS decreased from 2.99 in 2017 to 2.84 and 2.76 days in 2018 and 2019, respectively. To correct for aberrations due to the COVID pandemic, we compared June-August 2019 to June-August 2020 and found a further decrease to 2.42 days after full implementation of all interventions. We estimate that the intervention led to an absolute reduction in costs of USD 3 million in the second half of 2019 and more than USD 7 million in 2020. On the other hand, the total expenses, represented by salaries for additional staffing, were USD 2,103,274, resulting in an estimated net saving for 2020 of USD 5,400,000. (4) Conclusions: At JSUMC, hLOS was found to be a complex and costly issue. A comprehensive approach, starting with the identification of all correctable delays followed by interventions to mitigate delays, led to a significant reduction in hLOS along with significant cost savings.

Portable neuromodulation induces neuroplasticity to re-activate motor function recovery from brain injury: a high-density MEG case study.

BACKGROUND: In a recent high-profile case study, we used functional magnetic resonance imaging (fMRI) to monitor improvements in motor function related to neuroplasticity following rehabilitation for severe traumatic brain injury (TBI). The findings demonstrated that motor function improvements can occur years beyond current established limits. The current study extends the functional imaging investigation to characterize neuromodulation effects on neuroplasticity to further push the limits. METHODS: Canadian Soldier Captain (retired) Trevor Greene (TG) survived a severe open-TBI when attacked with an axe during a 2006 combat tour in Afghanistan. TG has since continued intensive daily rehabilitation to recover motor function, experiencing an extended plateau using conventional physical therapy. To overcome this plateau, we paired translingual neurostimulation (TLNS) with the continuing rehabilitation program. RESULTS: Combining TLNS with rehabilitation resulted in demonstrable clinical improvements along with corresponding changes in movement evoked electro-encephalography (EEG) activity. High-density magneto-encephalography (MEG) characterized cortical activation changes in corresponding beta frequency range (27 Hz). MEG activation changes corresponded with reduced interhemispheric inhibition in the post-central gyri regions together with increased right superior/middle frontal activation suggesting large scale network level changes. CONCLUSIONS: The findings provide valuable insight into the potential importance of non-invasive neuromodulation to enhance neuroplasticity mechanisms for recovery beyond the perceived limits of rehabilitation.

Prevalence of abnormalities in electrocardiogram conduction in dialysis patients: a comparative study / Prevalência de anormalidades de condução no eletrocardiograma de pacientes em diálise: um estudo comparativo

ABSTRACT Background: The electrocardiogram (ECG) can aid in identification of chronic kidney disease (CKD) patients at high risk for cardiovascular diseases. Cohort studies describe ECG abnormalities in patients on hemodialysis (HD), but we did not find data comparing ECG abnormalities among patients with normal kidney function or peritoneal dialysis (PD) to those on hemodialysis. We hypothesized that ECG conduction abnormalities would be more common, and cardiac conduction interval times longer, among patients on hemodialysis vs. those on peritoneal dialysis and CKD 1 or 2. Methods: Retrospective review of adult inpatients' charts, comparing those with billing codes for "Hemodialysis" vs. inpatients without those charges, and an outpatient peritoneal dialysis cohort. Patients with CKD 3 or 4 were excluded. Results: One hundred and sixty-seven charts were reviewed. ECG conduction intervals were consistently and statistically longer among hemodialysis patients (n=88) vs. peritoneal dialysis (n=22) and CKD stage 1 and 2 (n=57): PR (175±35 vs 160±44 vs 157±22 msec) (p=0.009), QRS (115±32 vs. 111±31 vs 91±18 msec) (p=0.001), QT (411±71 vs. 403±46 vs 374±55 msec) (p=0.006), QTc (487±49 vs. 464±38 vs 452±52 msec) (p=0.0001). The only significantly different conduction abnormality was prevalence of left bundle branch block: 13.6% among HD patients, 5% in PD, and 2% in CKD 1 and 2 (p=0.03). Conclusion: To our knowledge, this is the first study to report that ECG conduction intervals are significantly longer as one progresses from CKD Stage 1 and 2, to PD, to HD. These and other data support the need for future research to utilize ECG conduction times to identify dialysis patients who could potentially benefit from proactive cardiac evaluations and risk reduction.

RESUMO Introdução: O eletrocardiograma (ECG) pode auxiliar na identificação de pacientes com doença renal crônica (DRC) e alto risco para doenças cardiovasculares. Estudos de coorte descrevem anormalidades no ECG de pacientes em hemodiálise (HD), mas não encontramos dados comparando anormalidades no ECG entre pacientes com função renal normal ou aqueles em diálise peritoneal (DP), com aqueles em hemodiálise. Nossa hipótese foi de que as anormalidades de condução no ECG seriam mais comuns, e o intervalo de condução cardíaca seria mais longo entre os pacientes em hemodiálise comparados àqueles em diálise peritoneal e DRC 1 ou 2. Métodos: revisão retrospectiva dos prontuários de pacientes adultos internados, comparando aqueles com códigos de cobrança para "Hemodiálise" versus pacientes internados sem esses encargos, e uma coorte de pacientes em diálise peritoneal ambulatorial. Pacientes com DRC 3 ou 4 foram excluídos. Resultados: Cento e sessenta e sete prontuários foram revisados. Os intervalos de condução no ECG foram consistente- e estatisticamente mais longos entre os pacientes em hemodiálise (n = 88) vs. em diálise peritoneal (n = 22) e DRC estágios 1 e 2 (n = 57): PR (175 ± 35 vs 160 ± 44 vs 157 ± 22 msec) (p = 0,009); QRS (115 ± 32 vs. 111 ± 31 vs 91 ± 18 ms) (p = 0,001); QT (411 ± 71 vs. 403 ± 46 vs 374 ± 55 ms) (p = 0,006 ), QTc (487 ± 49 vs. 464 ± 38 vs 452 ± 52 ms) (p = 0,0001). A única anormalidade de condução significativamente diferente foi a prevalência de bloqueio do ramo esquerdo: 13,6% nos pacientes em HD, 5% em DP e 2% na DRC 1 e 2 (p = 0,03). Conclusão: Pelo que sabemos, este é o primeiro estudo a relatar que os intervalos de condução no ECG são significativamente maiores à medida que se progride das DRC Estágios 1 e 2, para DP, e para HD. Esses e outros dados corroboram a necessidade de estudos futuros para utilizar os tempos de condução no ECG para identificar pacientes em diálise que poderiam se beneficiar de avaliações cardíacas proativas e assim redução de risco.

Brain Vital Signs Detect Cognitive Improvements During Combined Physical Therapy and Neuromodulation in Rehabilitation From Severe Traumatic Brain Injury: A Case Report.

Using a longitudinal case study design, we have tracked the recovery of motor function following severe traumatic brain injury (TBI) through a multimodal neuroimaging approach. In 2006, Canadian Soldier Captain (retired) Trevor Greene (TG) was attacked with an axe to the head while on tour in Afghanistan. TG continues intensive daily rehabilitation, which recently included the integration of physical therapy (PT) with neuromodulation using translingual neurostimulation (TLNS) to facilitate neuroplasticity. Recent findings with PT + TLNS demonstrated that recovery of motor function occurred beyond conventional time limits, currently extending past 14-years post-injury. To investigate whether PT + TLNS similarly resulted in associated cognitive function improvements, we examined event-related potentials (ERPs) with the brain vital signs framework. In parallel with motor function improvements, brain vital signs detected significant increases in basic attention (as measured by P300 response amplitude) and cognitive processing (as measured by contextual N400 response amplitude). These objective cognitive improvements corresponded with TG's self-reported improvements, including a noteworthy and consistent reduction in ongoing symptoms of post-traumatic stress disorder (PTSD). The findings provide valuable insight into the potential importance of non-invasive neuromodulation in cognitive rehabilitation, in addition to initial indications for physical rehabilitation.

Brain Vital Signs Detect Information Processing Differences When Neuromodulation Is Used During Cognitive Skills Training.

Background: Neuromodulation through translingual neurostimulation (TLNS) has been shown to initiate long-lasting processes of neuronal reorganization with a variety of outcomes (i.e., neuroplasticity). Non-invasive TLNS is increasingly accessible through the Portable Neuromodulation Stimulator (PoNS®), a medical device that delivers electrical stimulation to the tongue to activate the trigeminal (V) and facial (VII) cranial nerves. Anecdotal reports from previous clinical studies have suggested incidental improvements in cognitive function. To objectively explore this observation, we examined TLNS-related effects on the semantic N400 brain vital sign cognitive response during cognitive skills training in healthy individuals. Methods: Thirty-seven healthy volunteers were randomized to receive simultaneous TLNS (treatment) or no TLNS (control) while undergoing cognitive skills training. Cognitive training was conducted for two 20-min sessions (morning and afternoon/evening) over 3 consecutive days. Brain vital signs were evaluated at baseline, Day 1, and Day 3. Analyses focused on cognitive processing as measured by N400 changes in amplitude and latency. Results: Over the 3-day course of cognitive training, the N400 amplitude decreased significantly in the control group due to habituation (p = 0.028). In contrast, there was no significant change in the TLNS treatment group. Conclusion: TLNS led to a sustained N400 response during cognitive skills training, as measured by the brain's vital signs framework. The study findings suggest differential learning effects due to neuromodulation, consistent with increased attention and cognitive vigilance.

Prevalence of abnormalities in electrocardiogram conduction in dialysis patients: a comparative study.

BACKGROUND: The electrocardiogram (ECG) can aid in identification of chronic kidney disease (CKD) patients at high risk for cardiovascular diseases. Cohort studies describe ECG abnormalities in patients on hemodialysis (HD), but we did not find data comparing ECG abnormalities among patients with normal kidney function or peritoneal dialysis (PD) to those on hemodialysis. We hypothesized that ECG conduction abnormalities would be more common, and cardiac conduction interval times longer, among patients on hemodialysis vs. those on peritoneal dialysis and CKD 1 or 2. METHODS: Retrospective review of adult inpatients' charts, comparing those with billing codes for "Hemodialysis" vs. inpatients without those charges, and an outpatient peritoneal dialysis cohort. Patients with CKD 3 or 4 were excluded. RESULTS: One hundred and sixty-seven charts were reviewed. ECG conduction intervals were consistently and statistically longer among hemodialysis patients (n=88) vs. peritoneal dialysis (n=22) and CKD stage 1 and 2 (n=57): PR (175±35 vs 160±44 vs 157±22 msec) (p=0.009), QRS (115±32 vs. 111±31 vs 91±18 msec) (p=0.001), QT (411±71 vs. 403±46 vs 374±55 msec) (p=0.006), QTc (487±49 vs. 464±38 vs 452±52 msec) (p=0.0001). The only significantly different conduction abnormality was prevalence of left bundle branch block: 13.6% among HD patients, 5% in PD, and 2% in CKD 1 and 2 (p=0.03). CONCLUSION: To our knowledge, this is the first study to report that ECG conduction intervals are significantly longer as one progresses from CKD Stage 1 and 2, to PD, to HD. These and other data support the need for future research to utilize ECG conduction times to identify dialysis patients who could potentially benefit from proactive cardiac evaluations and risk reduction.

Acute Fulminant Hepatic Failure and Renal Failure Induced by Oral Amiodarone: A Case Report and Literature Review.

Amiodarone is a class III antiarrhythmic agent that inhibits adrenergic stimulation by blocking alpha and beta receptors. It prolongs action potential and refractory period in myocardial tissue. Its remarkably long half-life is associated with a myriad of adverse events. Here, we present an 85-year-old male patient who was started on amiodarone for atrial flutter. After three oral doses, he developed fulminant hepatic failure and acute renal failure, which resolved after stopping amiodarone. While fulminant hepatic failure is rare, it has been seen in less than 2% of patients. Alternative theories behind susceptibility to amiodarone-induced hepatic injury and acute kidney injury are discussed here.

Protein-Losing Enteropathy as the Initial Presentation of Gastrointestinal Kaposi's Sarcoma in Previously Undiagnosed HIV Disease.

Occult Kaposi's sarcoma (KS) presenting as a protein-losing gastroenteropathy is a rare occurrence. We report the case of a 23-year-old male presenting with leg bilateral swelling and epigastric discomfort. A workup revealed human immunodeficiency virus seropositivity, hypoalbuminemia, and small bowel wall thickening on computed tomography scan. Initially there were no mucosal or cutaneous lesions visible. An upper endoscopy demonstrated subepithelial lesions with a reddish appearance involving the palate, cardia, duodenum, and jejunum, consistent with KS. Gastrointestinal involvement is the most common extracutaneous site of KS and is found in about half of the acquired immune deficiency syndrome (AIDS)-related cases. However, only one out of 5 patients are symptomatic in the absence of skin lesions. Antiretroviral therapy along with anthracycline chemotherapy must be promptly initiated to improve chances of survival.

Carcinoid tumor of lung and BRCA mutation: a case report.

BACKGROUND: A BRCA mutation is a mutation in either of the BRCA1 or BRCA2 genes, which are tumor suppressor genes. Hundreds of different types of mutations in these genes have been identified, some of which have been determined to be harmful, whereas others have no proven impact. BRCA mutations are well known to be associated with breast, uterine, and ovarian cancers along with some nongynecological malignancies involving the peritoneum, prostate, pancreas, skin, stomach, and rectum. However, there are no reported cases to date of an association between carcinoid tumors and a BRCA mutation. CASE PRESENTATION: Our patient was a 33-year-old White woman with BRCA2 mutation who presented to her primary care physician for evaluation of abdominal pain. She underwent computed tomography of her abdomen and pelvis, which showed an incidental finding of infrahilar mass along with renal stones. Further workup with bronchoscopy and biopsy of the mass confirmed it to be a carcinoid tumor of the lung. CONCLUSIONS: No literature thus far exists describing a connection between BRCA mutations and carcinoid tumors. Early diagnosis and prompt treatment of carcinoid tumors are proven to have impact on survival and prognosis of these patients.

Varicella Zoster Meningitis with Hypoglycorrhachia on Cerebrospinal Fluid (CSF) Analysis in a Young Immunocompetent Host without a Rash.

BACKGROUND Varicella zoster virus (VZV) is a common viral infection, with primary infection presenting as fevers and pruritic vesicular rash. After staying dormant in the dorsal root ganglia, reactivation can lead to secondary infection. Meningitis is a rare a complication of VZV infection. CASE REPORT We report a case of a 44-year-old woman with no past medical history, presenting with severe frontal headache without meningeal signs or fevers, found to have VZV meningitis. CSF analysis revealed hypoglycorrhachia and she was treated successfully with combination of intravenous acyclovir and oral valacyclovir. CONCLUSIONS VZV meningitis can present with subtle clinical signs and symptoms and should be considered as a possible etiology for headaches without identifiable cause.

Recurrent optic neuritis as the only manifestation of chronic hepatitis B virus flare: a case report.

BACKGROUND: Autoimmune reactions have been associated with acute hepatitis B virus infection. Among these optic neuritis is a rare presentation with only a handful of cases reported in the literature thus far. The pathophysiologic mechanism governing this phenomenon includes high levels of circulating immune complexes, tissue deposit, and complement activation cascade. CASE PRESENTATION: In this report, we present the case of a 46-year-old African American man with a past medical history of untreated chronic hepatitis B virus, diagnosed 5 years ago, who presented to our facility on two occasions with the chief compliant of blurry vision. He was diagnosed with optic neuritis associated with acute on chronic hepatitis B virus infection, where the recurrent visual impairment was the main presenting symptom. Because hepatitis constituted a relative contraindication for steroid therapy, our patient was solely treated with antiviral medication. Antiviral therapy resulted in complete resolution of his symptoms and improvement in his liver function. CONCLUSIONS: Further studies are necessary to conclusively establish whether antiviral therapy can be employed as the sole therapy in immune complex-mediated optic neuritis, in the setting of active recurrent hepatitis B infection.

Practical strategies and perceptions from community pharmacists following their experiences with conducting pharmacy practice research: a qualitative content analysis.

OBJECTIVES: While prior research identified barriers to conducting research in community pharmacies, there remains a need to better understand facilitators to ensure successful collaborations between academic researchers and pharmacists. Our objective was to determine the experiences and perspectives of community pharmacists who have recently conducted a pharmacy practice-based research study to gain in-depth understanding of challenges as well as facilitators and identify strategies and solutions. METHODS: We conducted a qualitative study involving one-on-one semi-structured telephone interviews with community pharmacists following the completion of a practice-based research study in their pharmacies. Interview transcripts were analysed using inductive content analysis involving open coding, creating categories and abstraction into final themes. KEY FINDINGS: Eleven pharmacists participated in the qualitative interviews. We identified six major themes including: (1) barriers (e.g. time constraints); (2) facilitators (e.g. ideal pharmacy layout); (3) support and resources from academic researchers (e.g. helpfulness of training, easy-to-use study materials); (4) pharmacist-initiated strategies for conducting research (beyond prior suggestions from researchers); (5) suggestions for future pharmacy practice research; and (6) motivation for conducting pharmacy practice research. These findings informed practical strategies targeted at academic researchers and pharmacists, respectively, to facilitate the conduct of research in community pharmacists across various stages of the research process. CONCLUSIONS: Our study adds to better understanding of community pharmacists' perspectives on conducting research and identifies practical solutions that can be readily implemented by academic researchers and pharmacists participating in research.

The Duality of Economic Issues With Medication Non-adherence in Patients With Inflammatory Arthritis.

PURPOSE OF REVIEW: In this review, we synthesize current data on non-adherence across inflammatory arthritides and explore (1) the effects of economic factors on non-adherence and (2) the impacts of non-adherence on economic outcomes. RECENT FINDINGS: Recent evidence demonstrates medication non-adherence rates as high as 74% in ankylosing spondylitis (AS), 90% in gout, 50% in psoriatic arthritis (PsA), 75% in systemic lupus erythematosus (SLE), and 82% in rheumatoid arthritis (RA). The effects of socioeconomic factors have been studied most in RA and SLE but with inconsistent findings. Nonetheless, the evidence points to having prescription coverage and costs of treatment as important factors in RA and education as an important factor in SLE. Limited data in AS and gout, and no studies of the effects of socioeconomic factors in PsA, show knowledge gaps for future research. Finally, there is a dearth of data with respect to the impacts of non-adherence on economic outcomes.

Reevaluating Muscle Biopsies in the Diagnosis of Pompe Disease: A Corroborative Report.

BACKGROUND: Previous reports suggest that although a diagnostic muscle biopsy can confirm the presence of Pompe disease, the absence of a definitive biopsy result does not rule out the diagnosis. METHODS: In this study, we reviewed patients with a limb-girdle syndrome who demonstrated nonspecific abnormalities of muscle, without evidence of the classical changes of acid maltase deficiency. These patients were rescreened for Pompe disease using dried blood spot (DBS) testing. RESULTS: Twenty-seven patients provided blood samples for the DBS test. Four patients underwent subsequent genetic testing. Genetic analysis demonstrated that one patient tested positive for Pompe disease and one patient had one copy of a pathogenic variant. CONCLUSIONS: In conclusion, the ability of a diagnostic muscle biopsy to definitively rule out the presence of Pompe disease is limited. There is a role for a screening DBS in all patients presenting with a limb-girdle syndrome without a clear diagnosis.