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BACKGROUNDAcute liver failure (ALF) can be a primary presentation of Wilson disease (WD). Mortality rates are high in WD with ALF (WDALF). Predictions of mortality in WDALF vary by model and are sometimes contradictory, perhaps because few patients are studied or WD diagnoses are questionable. AIMTo determine the outcomes among well-documented WDALF patients and assess mortality model performance in this cohort.METHODSWe reviewed the medical records of our pediatric WDALF patients (n = 41 over 6-years-old, single-center retrospective study) and compared seven prognostic models (King’s College Hospital Criteria, model for end-stage liver disease/pediatric end-stage liver disease scoring systems, Liver Injury Unit [LIU] using prothrombin time [PT] or international normalized ratio [INR], admission LIU using PT or INR, and Devarbhavi model) with one another.RESULTSAmong the 41 Han Chinese patients with ALF, WD was established by demonstrating ATP7B variants in 36. In 5 others, Kayser-Fleischer rings and Coombs-negative hemolytic anemia permitted diagnosis. Three died during hospitalization and three underwent liver transplantation (LT) within 1 mo of presentation and survived (7.3% each); 35 (85.4%) survived without LT when given enteral D-penicillamine and zinc-salt therapy with or without urgent plasmapheresis. Parameters significantly correlated with mortality included encephalopathy, coagulopathy, and gamma-glutamyl transpeptidase activity, bilirubin, ammonia, and serum sodium levels. Area under the receiver operating curves varied among seven prognostic models from 0.981 to 0.748 with positive predictive values from 0.214 to 0.429.CONCLUSIONWDALF children can survive and recover without LT when given D-penicillamine and Zn with or without plasmapheresis, even after enlisting for LT.  相似文献   
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ABSTRACT

This study focused on factors associated with antiretroviral therapy (ART) adherence and quality of life among transgenderwomen in Sao Paulo, Brazil, using univariable and adjusted analysis. Adherence was evaluated with a self-report tool and with HIV viral load (VL) measurement. PROQOL-HIV was used to assess quality of life. 106 TGW with median 41 years old were included; most were white (56%) and had >10 years of education (57%). Median time since HIV/AIDS diagnosis was 10 years. Overall, participants had high T CD4+ counts (median 659 cells/mm3) and most (75%) had undetectable HIV VL. 85% were considered adherent using self-report (95%CI 77-91), whereas 72% (95%CI 62-80) were considered adherent when self-report and undetectable HIV VL were analyzed jointly. Older age was associated with higher ART adherence; each year increase in age was associated with 5% higher odds of adherence (p?=?0.021). Quality of life ranged from good-excellent in 5 of 8 domains. Younger age, lower education, higher time since HIV diagnosis, comorbidities, illicit drugs use and depression were associated with lower PROQOL scores in specific domains in univariable analysis, while depression was also associated with lower total PROQOL score even after adjustment for age, comorbidities and time since HIV diagnosis (p?=?0.048).  相似文献   
24.
AimsSelf-expanding metal stents provide rapid improvement of dysphagia in oesophageal cancer but are associated with complications. The aim of the present study was to test the effectiveness of an alternative treatment of combining biodegradable stents with radiotherapy.Materials and methodsA Simon two-stage single-arm prospective phase II trial design was used to determine the efficacy of biodegradable stents plus radiotherapy in patients with dysphagia caused by oesophagus cancer who were unsuitable for radical treatment. Fourteen patients were recruited and data from 12 were included in the final analyses.ResultsFive of 12 patients met the primary end point: one stent-related patient death; four further interventions for dysphagia within 16 weeks of stenting (41.7%, 95% confidence interval 15.2–72.3%). The median time to a 10-point deterioration of quality of life was 2.7 weeks. Nine patients died within 52 weeks of registration. The median time to death from any cause was 15.0 weeks (95% confidence interval 9.6–not reached).ConclusionThe high re-intervention observed, which met the pre-defined early stopping criteria, meant that the suggested alternative treatment was not sufficiently effective to be considered for a larger scale trial design. Further work is needed to define the place of biodegradable stents in the management of malignant oesophageal strictures.  相似文献   
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Background

Nursing Home Compare (NHC) ratings, created and maintained by Medicare, are used by both hospitals and consumers to aid in the skilled nursing facility (SNF) selection process. To date, no studies have linked NHC ratings to actual episode-based outcomes. The purpose of this study was to evaluate whether NHC ratings are valid predictors of 90-day complications, readmission, and bundle costs for patients discharged to an SNF after primary total joint arthroplasty (TJA).

Methods

All SNF-discharged primary TJA cases in 2017 at a multihospital academic health system were queried. Demographic, psychosocial, and clinical variables were manually extracted from the health record. Medicare NHC ratings were then collected for each SNF. For patients in the Medicare bundle, postacute and total bundle cost was extracted from claims.

Results

Four hundred eighty-eight patients were discharged to a total of 105 unique SNFs. In multivariate analysis, overall NHC rating was not predictive of 90-day readmission/major complications, >75th percentile postacute cost, or 90-day bundle cost exceeding the target price. SNF health inspection and quality measure ratings were also not predictive of 90-day readmission/major complications or bundle performance. A higher SNF staffing rating was independently associated with a decreased odds for >75th percentile 90-day postacute spend (odds ratio, 0.58; P = .01) and a 90-day bundle cost exceeding the target price (odds ratio = 0.69; P = .02) but was similarly not predictive of 90-day readmission/complications.

Conclusion

Results of our study suggest that Medicare's NHC tool is not a useful predictor of 90-day costs, complications, or readmissions for SNFs within our health system.  相似文献   
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In cases of suspected violations of rules, regulations or the law by armed forces personnel, investigations are invariably mandatory. Military investigations differ from well-researched civilian criminal investigations. Differing from civilian police detectives, most military investigators – as disciplinary supervisors and military police personnel – have a number of tasks to accomplish, which include leading in combat and ensuring military readiness. Military investigations can lead to substantive negative or positive consequences for military readiness, including mental health, unit cohesion and subjective legal certainty. This impact on unit cohesion and mental health is influenced by any prior history of distress or trauma; military investigations are often preceded by contravention of internal disciplinary acts, complaints and traumatic events. This study explores factors in the differing military and legal systems of Germany and the United Kingdom (UK) that might help military personnel to successfully conduct investigations while ensuring deployment readiness and maintaining human rights.  相似文献   
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Nephrolithiasis is a common pathology encountered in the primary care and emergency department (ED) setting. In 2009 alone, there were over one million ED visits related to nephrolithiasis Higa et al. (2017) [1]. Emergent treatment options range from non-invasive pain control and patient education to lithotripsy and other invasive urologic procedures depending on stone location and related pathology. Urethral calculi are estimated to represent 0.3% of all urinary stone diseases Verit et al. (2006) [2]. There are very few case reports documenting distal urethral stone removal in the ED. Here we present a case of distal urethral stone impaction and the removal of this stone by ED providers, leading to expedited care and prevention of consultation and possible admission.  相似文献   
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