A daily regimen of a ceramide-dominant moisturizing cream and cleanser restores the skin permeability barrier in adults with moderate eczema: A randomized trial.

The dysfunctional skin barrier in eczema patients may be attributed to decreased levels of ceramides in the stratum corneum. The aim of this study was to determine whether a two-part system consisting of a ceramide-dominant physiological lipid-based moisturizing cream and cleanser could ameliorate the signs and symptoms of moderate eczema in adults over 28 days compared to placebo. Assessments were conducted at baseline and every 7 days thereafter. Eczema area severity index score decreased significantly across all time points in both groups compared to baseline (P < .0001), however, this decrease was not significant between groups at day 28 (P = .7804). In contrast, transepidermal water loss and skin hydration significantly improved over time in the active group, while it either stayed the same or worsened in the placebo group (P = .0342 and P < .0001, respectively). There was no difference in the use of mometasone furoate as rescue medication over time between groups (P = .1579). Dermatology life quality index scores improved significantly in both groups (P < .0001), with no difference between groups (P = .5256). However, patient satisfaction was greater in the active compared to the placebo group for several parameters including relief of itch, dry skin, skin softness and smoothness (all P < .05). No patients withdrew from the study due to adverse events (AEs) and there were no serious AEs. The ceramide-dominant moisturizing cream and cleanser safely restores skin permeability and improves the signs and symptoms of eczema in adults.

Recruitment of adults with moderate eczema for a randomised trial: Comparison of traditional versus modern methods.

BACKGROUND: Clinical trial recruitment is challenging for investigators who often overestimate the pool of qualified, willing subjects. Moreover, there is a paucity of literature, particularly in dermatology, regarding recruitment and the comparative success of advertising strategies. METHODS: Both 'traditional' (physician referral, newspaper and radio advertisements, letterbox drops, posters/flyers, word-of-mouth) and 'modern' (patient recruitment services, social media, Google advertisements, websites, email) recruitment methods were used to enrol 100 patients (>18 years) diagnosed with moderate eczema for a randomised, double-blind, placebo-controlled trial investigating the efficacy and safety of a topical eczema treatment over 4 weeks. The relationships between recruitment method and patient age, sex, race, study completion and costs were analysed. RESULTS: The majority of patients recruited were young, with millennials and Gen Z comprising 77% of the study population. Both traditional and modern recruitment methods were equally successful in recruiting younger patients, with older patients predominately recruited by traditional methods. Eighty per cent more men were recruited by traditional compared to modern methods, whilst 67% more women than men were recruited by modern methods. Recruitment method neither appeared to be influenced by race, nor did it effect whether patients completed the study. Costs per enrolment were similar for both methods. CONCLUSIONS: This study shows that despite the high proportion of young patients and the rising popularity of social media and increased internet use, a combination of both traditional and modern recruitment methods was required to successfully meet the trial enrolment target of 100 adult patients with moderate eczema.

Skin testing with ultra-heat-treated (UHT) cow's milk in children with cow's milk allergy.

BACKGROUND: A large proportion of cow's milk (CM)-allergic children are able to tolerate extensively heated forms of CM such as baked goods. Little is known about whether ultra-heat-treated (UHT) forms of cow's milk are immunologically similar to extensively heated cow's milk and therefore may be tolerated by these children. OBJECTIVE: To determine whether skin test wheal size using UHT CM was significantly different from other forms of CM and CM extracts. METHODS: Children presenting for oral food challenges with either extensively heated or unheated cow's milk underwent skin prick test (SPT) to commercial CM, UHT CM, evaporated CM, and fresh whole CM. The results were compared between groups of children. RESULTS: At study exit, only 14% of children were avoiding all forms of CM, compared with 70% at study entry. No difference was seen in the mean SPT results for UHT CM between those children that could tolerate heated CM compared with those that could not. The mean SPT result for casein was significantly lower in those that could tolerate heated CM. However, within the group of heated milk-tolerant children, the mean SPT for UHT CM was significantly lower than the SPT for fresh whole CM. CONCLUSION: Ultra-heat-treated CM does not behave significantly differently from other forms of CM when evaluated by SPT in heated milk-allergic vs heated milk-tolerant children. This suggests that UHT CM is not sufficiently immunologically different from unheated CM to be tolerated by heated CM-tolerant children.

Estimating Bacterial Concentrations in Fibrous Substrates Through a Combination of Scanning Electron Microscopy and ImageJ.

Conventional signal-based microanalytical techniques for estimating bacterial concentrations are often susceptible to false signals. A visual quantification, therefore, may compliment such techniques by providing additional information and support better management decisions in the event of outbreaks. Herein, we explore a method that combines electron microscopy (EM) and image-analysis techniques and allows both visualization and quantification of pathogenic bacteria adherent even to complex nonuniform substrates. Both the estimation and imaging parameters were optimized to reduce the estimation error ( E, %) to close to ±5%. The method was validated against conventional microbiological techniques such as the use of optical density, flow cytometry, and quantitative real-time PCR (qPCR). It could easily be tailored to estimate different species of pathogens, such as Escherichia coli O157, Listeria innocua, Staphylococcus aureus, Enterococcus faecalis, and Bacillus anthracis, on samples similar to those in real-time contamination scenarios. The present method is sensitive enough to detect ∼100 bacterial CFU/mL but has the potential to estimate even lower concentrations with increased imaging and computation times. Overall, this imaging-based method may greatly complement any signal-based pathogen-detection technique, especially in negating false signals, and therefore may significantly contribute to the field of analytical microbiology and biochemistry.

Bullying in Australian children and adolescents with food allergies.

BACKGROUND: Food allergy is an increasing concern worldwide. The significant impact of food allergies on quality of life and bullying has been well described in international studies. No studies have yet investigated the occurrence of bullying in children and adolescents with food allergies in the Australian population. This study aimed to characterize and examine the frequency of bullying and describe those most responsible and the effects of the bullying on the victims. METHODS: Questionnaires were developed based on those used in previous studies and were distributed throughout paediatric allergy clinics. Children and adolescents aged 10-19 with food allergies were recruited to complete the questionnaire independently, whilst parents completed the questionnaire on behalf of their children aged 5-9 with food allergies. The data were tabulated and analysed using descriptive statistics. RESULTS: Ninety-three questionnaires were completed. Overall, 39 (42%) children experienced some form of bullying. This was higher in the older children and adolescent group, where 53% (18/34) were bullied. In addition, 23% (21/93) of the cohort were bullied or teased specifically because of their allergy. Food allergens had been used in the bullying in 24 cases, but no child reported being forced to eat foods to which they were allergic. Two adolescents experienced allergic reactions. CONCLUSIONS: This study highlights that those with food allergy are subject to a high degree of bullying. This risk of bullying for children with food allergy indicates a significant social problem that requires addressing to positively assist these children.

Comparison of semiautomated tangential VMAT with 3DCRT for breast or chest wall and regional nodes.

Radiotherapy to the breast after surgery sometimes requires adjoining nodes to be included in the treatment volume. In these cases, the traditional approach has been a complex 3-Dimensional Conformal Radiotherapy (3DCRT) beam arrangement which can result in significant dose heterogeneity at the beam junctions. A Volumetric Modulated Arc Therapy (VMAT) beam arrangement has previously been proposed for breast cases, where the chest wall/breast is treated with a limited angle (partial arc) tangential VMAT technique (Virén et al. [2015] Radiat Oncol. 10:79). In our study, this approach is extended to breast and chest wall cases with adjoining nodes by adding a separate conventional VMAT arc field specifically limited to the superior nodes. This VMAT method was implemented using a semiautomated approach on 27 patients, and the resultant plan compared to a monoisocentric 3DCRT plan. Plan statistics, Dose-Volume Histogram (DVH) analysis and Radiation Oncologist (RO) preference were assessed. When compared to the 3DCRT technique, the VMAT planning method was found to result in better target volume coverage, high doses to organs at risk (OAR) were reduced but greater OAR volumes received low doses. Having said that, the volume receiving low doses with this tangential VMAT technique was less than that of other VMAT planning methods described in the literature, and the integral dose was less than the 3DCRT method. The VMAT technique also resulted in more robust junction doses that the 3DCRT method. RO review found that the VMAT technique was preferred in 81% of cases. Specifically, the VMAT plans were preferred in all categories of patients except left chest wall cases where the intermammary nodes were also treated. The VMAT technique described here is a useful addition to the treatment options available for breast/chest wall and nodal patients.

Bullying and quality of life in children and adolescents with food allergy.

Food allergy is an increasing phenomenon in Australia, with most recent reports of food allergy occurring in 1 in 12 children. A number of studies have demonstrated that children and adolescents with food allergies experience a decreased quality of life across a number of domains. More recent evidence suggests that this population also experiences an increased occurrence of bullying compared to similar school-aged children. Some individuals have reported being bullied because of their allergies, whilst others have reported specific allergy-related-bullying, such as being touched with foods that they are allergic to or having their food being intentionally contaminated with an allergen. Where there may be a risk of severe anaphylaxis, this is of great concern. This article reviews the current literature on bullying in populations of children and adolescents with food allergies. Several papers worldwide have investigated this, providing evidence of its occurrence in North America, Canada, Italy and Japan.

Quantitation of Chloramphenicol and Nitrofuran Metabolites in Aquaculture Products Using Microwave-Assisted Derivatization, Automated SPE, and LC-MS/MS.

This paper describes a rapid and robust method utilizing microwave-assisted derivatization, automated SPE, and LC-MS/MS for the quantitation and confirmation of chloramphenicol (CAP) and nitrofuran metabolites in various aquaculture matrixes. The use of equipment presented in this work allowed extractions to be completed on average within 6 h, with quantitation accuracy ranging from 89 to 107% and RSD≤8.3%. The demonstrated detection limits for all the nitrofuran metabolites of interest in three different matrixes were ≤0.06 ng/g, with a quantitation limit of ≤0.2 ng/g. Additionally, the method exhibited a CAP detection limit for all matrixes≤0.01 ng/g and an LOQ of ≤0.03 ng/g.

An integrin binding-defective mutant of insulin-like growth factor-1 (R36E/R37E IGF1) acts as a dominant-negative antagonist of the IGF1 receptor (IGF1R) and suppresses tumorigenesis but still binds to IGF1R.

Insulin-like growth factor-1 (IGF1) is a major therapeutic target for cancer. We recently reported that IGF1 directly binds to integrins (αvß3 and α6ß4) and induces ternary complex formation (integrin-IGF1-IGF1 receptor (IGF1R)) and that the integrin binding-defective mutant of IGF1 (R36E/R37E) is defective in signaling and ternary complex formation. These findings predict that R36E/R37E competes with WT IGF1 for binding to IGF1R and inhibits IGF signaling. Here, we described that excess R36E/R37E suppressed cell viability increased by WT IGF1 in vitro in non-transformed cells. We studied the effect of R36E/R37E on viability and tumorigenesis in cancer cell lines. We did not detect an effect of WT IGF1 or R36E/R37E in cancer cells under anchorage-dependent conditions. However, under anchorage-independent conditions, WT IGF1 enhanced cell viability and induced signals, whereas R36E/R37E did not. Notably, excess R36E/R37E suppressed cell viability and signaling induced by WT IGF1 under anchorage-independent conditions. Using cancer cells stably expressing WT IGF1 or R36E/R37E, we determined that R36E/R37E suppressed tumorigenesis in vivo, whereas WT IGF1 markedly enhanced it. R36E/R37E suppressed the binding of WT IGF1 to the cell surface and the subsequent ternary complex formation induced by WT IGF1. R36E/R37E suppressed activation of IGF1R by insulin. WT IGF1, but not R36E/R37E, induced ternary complex formation with the IGF1R/insulin receptor hybrid. These findings suggest that 1) IGF1 induces signals under anchorage-independent conditions and that 2) R36E/R37E acts as a dominant-negative inhibitor of IGF1R (IGF1 decoy). Our results are consistent with a model in which ternary complex formation is critical for IGF signaling.

An inverse relationship between asthma prevalence and medication dispensation trend: a 12-year spatial analysis of electronic health record data in Alberta, Canada.

Despite guideline-based asthma management in Alberta, an inverse trend between asthma prevalence and dispensation of asthma medications in the past 12 years possibly underscores the reason for a large number of emergency department visits https://bit.ly/3HQsBo8.

Temporal and Regional Trends in Obstructive Sleep Apnea Using Administrative Health Data in Alberta, Canada.

Rationale: Obstructive sleep apnea (OSA) is a common treatable condition with important health and societal consequences. Objectives: We aimed to assess the annual incidence and prevalence of clinically recognized and geographic clustering of OSA in Alberta, Canada, using administrative health data case definitions. Methods: We used two administrative health databases in Alberta to identify ICD-9 and ICD-10 (International Classification of Diseases, Ninth and 10th Revisions, respectively) diagnostic codes for adults and children at least 2 years old diagnosed with OSA between 2003 and 2020. We defined OSA using an algorithm developed and validated in Alberta: at least three claims or one hospitalization within 2 years. We mapped residential postal codes to 70 subregional health authorities (SRHAs). Crude, age group- and sex-specific incidence and prevalence, and age group- and sex-standardized rates were calculated for Alberta and SRHAs. Spatial scan statistics identified clusters of SRHAs in which OSA cases were higher (hot spots) or lower (cold spots) than expected. Results: Between 2003 and 2020, OSA prevalence increased from 0.14% to 4.59%. The annual incidence of OSA increased after 2013. Incidence and prevalence were higher in older adults and children aged 2-11 years compared with 12-17 years. Cluster analysis revealed regional variation in OSA incidence and prevalence over time with no consistent pattern except for cold spots in one large metropolitan center (Calgary). Conclusions: From 2003 to 2020, the incidence and prevalence of clinically recognized OSA increased but varied by geography. Administrative health data can be used to guide interventions aimed at improving health service delivery and the quality of OSA care.

MC1R signaling through the cAMP-CREB/ATF-1 and ERK-NFκB pathways accelerates G1/S transition promoting breast cancer progression.

MC1R, a G-protein coupled receptor, triggers ultraviolet light-induced melanin synthesis and DNA repair in melanocytes and is implicated in the pathogenesis of melanoma. Although widely expressed in different tissue types, its function in non-cutaneous tissue is relatively unknown. Herein, we demonstrate that disruptive MC1R variants associated with melanomagenesis are less frequently found in patients with several cancers. Further exploration revealed that breast cancer tissue shows a significantly higher MC1R expression than normal breast tissue, and knocking down MC1R significantly reduced cell proliferation in vitro and in vivo. Mechanistically, MC1R signaling through the MC1R-cAMP-CREB/ATF-1 and MC1R-ERK-NFκB axes accelerated the G1-S transition in breast cancer cells. Our results revealed a new association between MC1R and breast cancer, which could be potentially targeted therapeutically. Moreover, our results suggest that MC1R-enhancing/activating therapies should be used cautiously, as they might be pro-tumorigenic in certain contexts.

The Economic Burden of Food Allergy: What We Know and What We Need to Learn.

Purpose of Review: Food allergy management and treatment require dietary modification, are associated with significant burdens, and affect food choices and behaviours. Emerging therapies, such as oral immunotherapy (OIT), provide a glimmer of hope for those living with the condition. Some burdens have received substantial focus, whereas many knowledge gaps on the significance of other impacts, including economic burden, remain. Recent Findings: Evidence from many countries, but disproportionately from the United States, supports that food allergy carries significant healthcare and societal costs. Early introduction for the prevention of food allergies is theoretically cost-effective, but remains largely undescribed. Unique considerations, such as those to cow's milk protein allergy, which affects a substantial proportion of infants, and adrenaline autoinjectors, which have a high cost-per-use, require a balance between cost-effectiveness to the healthcare system and adverse outcomes. Household costs have largely been explored in two countries, but owing to different healthcare structures and costs of living, comparisons are difficult, as are generalisations to other countries. Stock epinephrine in schools may present a cost-effective strategy, particularly in economically disadvantaged areas. Costs relating to OIT must be examined within both immediate benefits, such as protection from anaphylaxis, and long-term benefits, such as sustained unresponsiveness. Summary: Although the absolute costs differ by region/country and type of food allergy, a consistent pattern persists: food allergy is a costly condition, to those who live with it, and the multiple stakeholders with which they interact. Supplementary Information: The online version contains supplementary material available at 10.1007/s40521-022-00306-5.

Health Care Utilization in the Last Year of Life in Parkinson Disease and Other Neurodegenerative Movement Disorders.

Background and Objectives: Neurodegenerative movement disorders are rising in prevalence and are associated with high health care utilization. Generally, health care resources are disproportionately expended in the last year of life. Health care utilization by those with neurodegenerative movement disorders in the last year of life is not well-understood. The goal of this study was to assess the utilization of acute care in the last year of life among individuals with neurodegenerative movement disorders and determine whether outpatient neurology or palliative care affected acute care utilization and place of death. Methods: We conducted a retrospective cross-sectional study including health system administrative data in Alberta, Canada, from 2011 to 2017. Administrative data were used to determine place of death and quantify emergency department (ED) visits, hospitalizations, intensive care unit admissions, and outpatient generalist and specialist visits. Diagnoses were classified by 10th revision of the International Classification of Diseases codes. Stata 16v was used for statistical analyses. Results: Among 1439 individuals (60% male), Parkinson disease (n = 1226), progressive supranuclear palsy (n = 78), multiple system atrophy (n = 47), and Huntington disease (n = 58) were the most common diagnoses. The most frequent place of death was in hospital (45.9%), followed by long-term care (36.3%), home (7.9%), and residential hospice (4.0%). Most (64.2%) had >1 ED visit, and 14.4% had >3 emergency department visits. Fifty-five percent had >1 hospitalization, and 23.3% spent >30 days in hospital. Few (2.6%) were admitted to ICU. Only 37.2% and 8.8% accessed outpatient neurologist and specialist palliative care services, respectively. Multivariate logistic regression found the odds of dying at home was higher for those who received outpatient palliative consultation (OR, 2.49, 95% confidence interval [CI], 1.48-4.21, p < 0.001) and were with a longer duration of home care support (OR, 1.0007, 95% CI, 1.0004-1.0009, p < 0.001). Discussion: There are high rates of in-hospital death and acute care utilization in the year before death among those with neurodegenerative movement disorders. Most did not access specialist palliative or neurologic care in the last year of life. Outpatient palliative care and home care services were associated with increased odds of dying at home. Our results indicate the need for further research into the causes, costs, and potential modifiers to inform public health planning.

Postoperative complications and mortality following colectomy for ulcerative colitis.

BACKGROUND & AIMS: Complications after colectomy for ulcerative colitis (UC) have not been well characterized in large, population-based studies. We characterized postoperative in-hospital complications, stratified them by severity, and assessed independent clinical predictors, including use of immunosuppressants. METHODS: We performed population-based surveillance using administrative databases to identify all adults (≥18 y) who had an International Classification of Diseases-9th/10th revisions code for UC and a colectomy from 1996 to 2009. All medical charts were reviewed. The primary outcome was severe postoperative complications, including in-hospital mortality. Logistic regression was used to assess predictors of complications after colectomy and then restricted to patients undergoing emergent or elective surgeries. RESULTS: Of the 666 UC patients who underwent a colectomy, a postoperative complication occurred in 27.0% and the mortality rate was 1.5%. Independent predictors of postoperative complications were age (for patients >64 vs 18-34 y: odds ratio [OR], 1.95; 95% confidence interval [CI], 1.07-3.54), comorbidities (>2 vs none: OR, 1.89; 95% CI, 1.06-3.37), and admission status (emergent vs elective colectomy: OR, 1.62; 95% CI, 1.14-2.30). Significant risk factors for an emergent colectomy included time from admission to colectomy (>14 vs 3-14 d: OR, 3.32; 95% CI, 1.62-6.80) and a preoperative complication (≥1 vs 0: OR, 3.04; 95% CI, 1.33-6.91). A prescription of immunosuppressants before colectomies did not increase the risk for postoperative complications. CONCLUSIONS: Postoperative complications frequently occur after colectomy for UC, predominantly among elderly patients with multiple comorbidities. Patients who were admitted to the hospital under emergency conditions and did not respond to medical treatment had worse outcomes when surgery was performed 14 or more days after admission.

Hospital-based acute care in the last 30 days of life among patients with chronic disease that received early, late or no specialist palliative care: a retrospective cohort study of eight chronic disease groups.

OBJECTIVE: For eight chronic diseases, evaluate the association of specialist palliative care (PC) exposure and timing with hospital-based acute care in the last 30 days of life. DESIGN: Retrospective cohort study using administrative data. SETTING: Alberta, Canada between 2007 and 2016. PARTICIPANTS: 47 169 adults deceased from: (1) cancer, (2) heart disease, (3) dementia, (4) stroke, (5) chronic lower respiratory disease (chronic obstructive pulmonary disease (COPD)), (6) liver disease, (7) neurodegenerative disease and (8) renovascular disease. MAIN OUTCOME MEASURES: The proportion of decedents who experienced high hospital-based acute care in the last 30 days of life, indicated by ≥two emergency department (ED) visit, ≥two hospital admissions,≥14 days of hospitalisation, any intensive care unit (ICU) admission, or death in hospital. Relative risk (RR) and risk difference (RD) of hospital-based acute care given early specialist PC exposure (≥90 days before death), adjusted for patient characteristics. RESULTS: In an analysis of all decedents, early specialist PC exposure was associated with a 32% reduction in risk of any hospital-based acute care as compared with those with no PC exposure (RR 0.69, 95% CI 0.66 to 0.71; RD 0.16, 95% CI 0.15 to 0.17). The association was strongest in cancer-specific analyses (RR 0.53, 95% CI 0.50 to 0.55; RD 0.31, 95% CI 0.29 to 0.33) and renal disease-specific analyses (RR 0.60, 95% CI 0.43 to 0.84; RD 0.22, 95% CI 0.11 to 0.34), but a~25% risk reduction was observed for each of heart disease, COPD, neurodegenerative diseases and stroke. Early specialist PC exposure was associated with reducing risk of four out of five individual indicators of high hospital-based acute care in the last 30 days of life, including ≥two ED visit,≥two hospital admission, any ICU admission and death in hospital. CONCLUSIONS: Early specialist PC exposure reduced the risk of hospital-based acute care in the last 30 days of life for all chronic disease groups except dementia.

Epidemiology and natural history of primary biliary cirrhosis in a Canadian health region: a population-based study.

UNLABELLED: The recent epidemiology and outcomes of primary biliary cirrhosis (PBC) in North America are incompletely described, partly due to difficulties in case ascertainment. In light of their availability, broad coverage, and limited expense, administrative databases may facilitate such investigations. We used population-based administrative data (inpatient, ambulatory care, and physician billing databases) and a validated International Classification of Diseases coding algorithm to describe the epidemiology and natural history of PBC in the Calgary Health Region (population approximately 1.1 million). Between 1996 and 2002, the overall age/sex-adjusted annual incidence of PBC was 30.3 cases per million (48.4 per million in women, 10.4 per million in men). Although the incidence remained stable, the prevalence increased from 100 per million in 1996 to 227 per million in 2002 (P < 0.0005). Among 137 incident cases with a total follow-up of 801 person-years from diagnosis (median 5.8 years), 27 patients (20%) died and six (4.4%) underwent liver transplantation. The estimated 10-year probabilities of survival, liver transplantation, and transplant-free survival were 73% (95% confidence interval [CI] 60%-83%), 6% (95% CI 2.5%-12.6%), and 68% (95% CI 55%-78%), respectively. Survival in PBC patients was significantly lower than that of the age/sex-matched Canadian population (standardized mortality ratio 2.87; 95% CI 1.89-4.17); male sex (hazard ratio [HR] 3.80; 95% CI 1.85-7.82) and an older age at diagnosis (HR per additional year, 1.06; 95% CI 1.03-1.10) were independent predictors of mortality. CONCLUSION: This population-based study demonstrates that the burden of PBC in Canada is high and growing. Survival of PBC patients is significantly lower than that of the general population, emphasizing the importance of developing new therapies for this condition.

Validation of coding algorithms for the identification of patients with primary biliary cirrhosis using administrative data.

BACKGROUND: Large-scale epidemiological studies of primary biliary cirrhosis (PBC) have been hindered by difficulties in case ascertainment. OBJECTIVE: To develop coding algorithms for identifying PBC patients using administrative data--a widely available data source. METHODS: Population-based administrative databases were used to identify patients with a diagnosis code for PBC from 1994 to 2002. Coding algorithms for confirmed PBC (two or more of antimitochondrial antibody positivity, cholestatic liver biochemistry and/or compatible liver histology) were derived using chart abstraction data as the reference. Patients with a recorded PBC diagnosis but insufficient confirmatory data were classified as 'suspected PBC'. RESULTS: Of 189 potential PBC cases, 119 (60%) had confirmed PBC and 28 (14%) had suspected PBC. The optimal algorithm including two or more uses of a PBC code had a sensitivity of 94% (95% CI 71% to 100%) and positive predictive values of 73% (95% CI 61% to 75%) for confirmed PBC, and 89% (95% CI 82% to 94%) for confirmed or suspected PBC. Sensitivity analyses revealed greater accuracy among women, and with the use of multiple data sources and one or more years of data. Inclusion of diagnosis codes for conditions frequently misclassified as PBC did not improve algorithm performance. CONCLUSIONS: Administrative databases can reliably identify patients with PBC and may facilitate epidemiological investigations of this condition.

Fatal refractory cardiac arrest as presentation of systemic amyloidosis.

Amyloidosis is a challenging diagnosis in the absence of clinical suspicion. The two patients reported here lacked any symptoms suggestive of amyloidosis. Instead, they presented with sudden, unexplained cardiac arrests that were persistent, treatment refractory, and ultimately fatal. Post-mortem examination unexpectedly revealed evidence of diffuse, systemic amyloidosis throughout multiple organ systems. We bring awareness to this unusual presentation of systemic amyloidosis that portends to poor outcome.