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Wilson disease (WD) is a chronic disorder of copper metabolism which may affect patient's quality of life (QOL).Our aim was to assess the relationship between mental QOL (M-QOL) and physical QOL (P-QOL) and severity of the liver, neurological disease and mental health in patients with WD.At enrollment into our multisite international WD registry, adults (n = 62) were administered examinations assessing QOL (Short-Form 12-Item Health Survey), cognition, and mood. Patients also underwent hepatology and neurological assessments.Patients had lower M-QOL than P-QOL scores, P = 0.0006. Patients with major depressive disorder (n = 22) had worse M-QOL scores, P = 0.0017 but not P-QOL. We found no association with impaired cognition (n = 37) and QOL. The P-QOL scores have a moderate negative association with neurological disease severity based on the Unified Wilson Disease Rating Scale score (total [r = -0.38, P0.003], part 2 [r = -0.50, P0.0001], and part 3 [r = -0.37, P = 0.004]). M-QOL was not associated with Unified Wilson Disease Rating Scale scores. Worse P-QOL, but not M-QOL, was found in higher cirrhosis severity indicated by Child-Pugh (r = -0.80, P = 0.002) and Model for End Stage Liver Disease scores (r = -0.64, P = 0.03).M-QOL was associated with depression but not cognitive impairment, neurological disease, or liver disease severity, suggesting that mental health issues may affect overall QOL independent of the degree of liver or neurological disease. P-QOL was affected by the severity of neurological and liver disease but not mental health but also contributes to overall QOL in WD. An appreciation of the range of problems that affect QOL in adults with WD will help health care providers address issues that could improve overall well-being. The Short-Form 12-Item Health Survey may provide a useful instrument for QOL surveillance in WD.

Objective: We previously demonstrated that plasma levels of F-actin and Thymosin Beta 4 differs among patients with septic shock, non-infectious systemic inflammatory syndrome and healthy controls ...

Objectives Adoption of emergency department (ED) initiation of buprenorphine (BUP) for opioid use disorder (OUD) into routine emergency care has been slow, partly due to clinicians’ unfamiliarity with this practice and perceptions that it is complicated and time‐consuming. To address these barriers and guide emergency clinicians through the process of BUP initiation, we implemented a user‐centered computerized clinical decision support system (CDS). This study was conducted to assess the feasibility of implementation and to evaluate the preliminary efficacy of the intervention to increase the rate of ED‐initiated BUP. Methods An interrupted time series study was conducted in an urban, academic ED from April 2018 to February 2019 (preimplementation phase), March 2019 to August 2019 (implementation phase), and September 2019 to December 2019 (maintenance phase) to study the effect of the intervention on adult ED patients identified by a validated electronic health record (EHR)‐based computable phenotype consisting of structured data consistent with potential cases of OUD who would benefit from BUP treatment. The intervention offers flexible CDS for identification of OUD, assessment of opioid withdrawal, and motivation of readiness to start treatment and automates EHR activities related to ED initiation of BUP (including documentation, orders, prescribing, and referral). The primary outcome was the rate of ED‐initiated BUP. Secondary outcomes were launch of the intervention, prescription for naloxone at ED discharge, and referral for ongoing addiction treatment. Results Of the 141,041 unique patients presenting to the ED over the preimplementation and implementation phases (i.e., the phases used in primary analysis), 906 (574 preimplementation and 332 implementation) met OUD phenotype and inclusion criteria. The rate of BUP initiation increased from 3.5% (20/574) in the preimplementation phase to 6.6% (22/332) in the implementation phase (p = 0.03). After the temporal trend of the number of physician's with X‐waiver training and other covariates were adjusted for, the relative risk of BUP initiation rate was 2.73 (95% confidence interval [CI] = 0.62 to 12.0, p = 0.18). Similarly, the number of unique attendings who initiated BUP increased modestly 7/53 (13.0%) to 13/57 (22.8%, p = 0.10) after offering just‐in‐time training during the implementation period. The rate of naloxone prescribed at discharge also increased (6.5% preimplementation and 11.5% implementation; p

Emergency physicians express concern administering a 30-cc/kg fluid bolus to septic shock patients with pre-existing congestive heart failure (CHF), end-stage renal disease (ESRD), or obesity, due to the perceived risk of precipitating a fluid overload state.Our aim was to determine whether there is a difference in fluid administration to septic shock patients with these pre-existing conditions in the emergency department (ED). Secondary objectives focused on whether compliance impacts mortality, need for intubation, and length of stay.We conducted a retrospective chart review of 470,558 ED patient encounters at a single urban academic center during a 5-year period.Of 847 patients with septic shock, 308 (36.36%) had no pre-existing condition and 199 (23.49%), 17 (2.01%), and 154 (18.18%) had the single pre-existing condition of CHF, ESRD, and obesity, respectively, and 169 (19.95%) had multiple pre-existing conditions. Weight-based fluid compliance was achieved in 460 patients (54.31%). There was a lower likelihood of compliance among patients with CHF (adjusted odds ratio [aOR] 0.35; 95% confidence interval [CI] 0.24-0.52; p0.001), ESRD (aOR 0.11, 95% CI 0.04-0.32; p0.001), and obesity (aOR 0.29, 95% CI 0.19-0.44; p0.001) compared with patients with no pre-existing conditions. Compliance decreased further in patients with multiple pre-existing conditions (aOR 0.49, 95% CI 0.33-0.72; p0.001). Compliance was not associated with mortality in patients with CHF and ESRD, but was protective in patients with obesity and those with no pre-existing conditions.Septic shock patients with pre-existing CHF, ESRD, or obesity are less likely to achieve compliance with a 30-cc/kg weight-based fluid goal compared with those without these pre-existing conditions.

Background Wilson Disease (WD) can result in a number of physical and mental health issues due to copper toxicity affecting the liver and brain. It is no longer acceptable to look at survival alone as an outcome measure in chronic disease. Our aim was to a) assess quality of life (QOL) in WD b) assess the relationship between mental health QOL (M-QOL) and physical health QOL (P-QOL) and severity of liver and neurological disease and mental health issues (major depressive disorder (MDD) and cognitive impairment). Methods Adult WD patients in the WD registry study (n=52), were evaluated at enrollment over 1.5 y using questionnaires and administered exams assessing QOL (SF-12), cognition (MOCA) and mood (MINI-7). Patients also underwent hepatology and neurological assessments (UWDRS). Results Adult patients with WD had lower M-QOL scores compared to P-QOL scores median 50.2 (range 19.3–62.2) vs. 56.0 (range 23.9–64.7), p=0.0018 indicating that the burden of mental health issues in WD is greater than that of physical health issues on quality of life. Eleven patients had cirrhosis based on review of imaging, APRI and Fib4 scores. There was no significant difference in M-QOL scores in patients with cirrhosis vs. those without (median 54.5 (range 26.2–59.7) vs 46.5 (range 19.3–62.2), p = 0.11. Similarly, there was no significant difference in P-QOL in patients with cirrhosis vs. those without (median 55.7 (range 44.4–59.9) vs. 56.3 (range 23.9–64.7), p = 1.00. In those with cirrhosis, higher Child-Pugh scores were associated with a worse P-QOL (r=-0.84, p=-.0011) and M-QOL (r=-0.60, p=0.0488). Patients with lifetime MDD (n=22) had worse M-QOL scores compared to those without MDD (median 42.3 vs 54.6, p The P-QOL scores have a moderate negative association with the neurological UWDRS II Score (r=-0.44, p=0.001), UWDRS III Score (r=-0.42, p= 0.002) and total UWDRS score (r=-0.44, p=0.001). There are no associations with M-QOL and neurological UWDRS scores. Conclusions While overall QOL in WD is affected by both mental and physical health, patients with WD have worse M-QOL than P-QOL scores and mental health issues may affect WD patient’s QOL independent of their degree of liver or neurologic disease. Multivariate regression will be performed to evaluate if mental health issues are independently associated with QOL.

Introduction Mental health problems are underappreciated in Wilson disease (WD) (Schaefer et al. 2016). We aimed to determine: i) the prevalence and clinical features of major depressive disorder (MDD) in adults with WD ii) whether MDD correlates with liver and neurological disease and quality of life (QOL). Method A multi-site international WD registry was developed and initiated in December 2017. At enrolment adults (n=55) were evaluated using questionnaires and administered exams assessing cognition, mood, psychosis, substance use, anxiety, perceived stress, personality change and QOL. MDD was determined using the investigator led MINI-7 questionnaire. Patients also underwent hepatology and neurological assessments (UWDRS). We reviewed patient reported depression at first presentation and diagnosis. Statistical analysis using SAS9.4 (SAS Institute; Cary, NC) included summary statistics, Wilcoxon Rank Sum test and categorical comparisons using Chi-squared or Fisher’s exact test. Results Depression was reported in 26% at first presentation of WD (33% at diagnosis). At enrolment 35% had a lifetime history of MDD. At evaluation 5 patients with lifetime MDD took antidepressants; 1 without. At enrolment 36.33% were suffering from symptoms of current depression based on the PHQ-9 (self-administered). Cirrhosis was present in 24% based on imaging, APRI and Fib4 scores. There was no association between cirrhosis and depression symptomology (PHQ-9 score >9). In those with depression symptomology, 1 had cirrhosis and 6 did not. Liver disease severity did not differ in cirrhotics with MDD and those without (median Child-Pugh 5 (5–9) vs. 5 (5–9), p=0.13). Two patients (1 with MDD) took medication for hepatic encephalopathy. No significant difference in neurological total UWDRS scores was found in those with MDD vs. without (median 8 (0–73) vs. 4 (0–40), p=0.22). A neurological UWDRS total score >0 was not associated with lifetime MDD status (79% vs. 81%, p=1.00). Patients with MDD had worse mental health QOL (median 43 vs. 52.7 p=0.042), more suicidal ideation (32% vs. 8%, p=0.05), higher anxiety (p=0.008), higher perceived stress (median 19 vs. 9, p=0.007), and more neuroticism (median 7 vs. 4.5, p=0.006). We found no significant difference in physical health QOL in MDD (median 55.6 vs. 56.3, p=0.69). Conclusion MDD is highly prevalent in WD and associated with worse mental health QOL. The risk appears higher than other liver diseases (Lee et al. 2013. Psychosomatics, 4:52–9). We did not find an association between the severity of liver, neurological disease and MDD. Screening for depression should be considered in patients with WD.

Buprenorphine (BUP) can safely and effectively reduce craving, overdose, and mortality rates in people with opioid use disorder (OUD). However, adoption of ED-initiation of BUP has been slow partly due to physician perception this practice is too complex and disruptive. We report progress of the ongoing EMBED (EMergency department-initiated BuprenorphinE for opioid use Disorder) project. This project is a five-year collaboration across five healthcare systems with the goal to develop, integrate, study, and disseminate user-centered Clinical Decision Support (CDS) to promote the adoption of Emergency Department (ED)-initiation of buprenorphine/naloxone (BUP) into routine emergency care. Soon to enter its third year, the project has already completed multiple milestones to achieve its goals including (1) user-centered design of the CDS prototype, (2) integration of the CDS into an automated electronic health record (EHR) workflow, (3) data coordination including derivation and validation of an EHR-based computable phenotype, (4) meeting all ethical and regulatory requirements to achieve a waiver of informed consent, (5) pilot testing of the intervention at a single site, and (6) launching a parallel group-randomized 18-month pragmatic trial in 20 EDs across 5 healthcare systems. Pilot testing of the intervention in a single ED was associated with increased rates of ED-initiated BUP and naloxone prescribing and a doubling of the number of unique physicians adopting the practice. The ongoing multi-center pragmatic trial will assess the intervention’s effectiveness, scalability, and generalizability with a goal to shift the emergency care paradigm for OUD towards early identification and treatment. Trial Registration: Clinicaltrials.gov # NCT03658642.