Adult PKU Clinics in the UK-Users' Experiences and Perspectives.

Adults with PKU require life-long management, and ideally, their care should be in a specialised adult metabolic clinic. Their outcomes and co-morbidities have received much attention, but data are lacking on their experience, satisfaction and expectations about the care they receive. This survey reports the experiences and care adults with PKU receive from specialist metabolic clinics in the UK. The online survey developed by the UK NSPKU (National Society for Phenylketonuria), was placed on the NSPKU website from February 2021 to December 2022, and was completed by adults with PKU (≥18 years) or their carers/family members. Sixty-five adult PKU patients and 9 caregivers of adult patients completed the questionnaire (63% female in total). Only 32% of respondents were following a Phe-restricted diet with protein substitute intake as prescribed; the rest were partially adherent or not on dietary restrictions. Nineteen per cent (n = 14/74) had not been reviewed in clinic for two years. Half of the respondents (50%) described their experience in adult clinics as "good". Half of the patients were unable to contact their dietitians with questions or concerns, and only 24% considered that they received adequate support. Clinic reviews usually included anthropometric (82%) and dietary assessments (64%), discussion on management of PKU in daily life (78%) and a blood test (71%). Eighty-eight per cent reported they had at least one neurocognitive, mental health or behavioural co-morbidity but less than half of the patients reported an assessment on their neurocognitive functioning or mental health issues. Adult male patients appeared to have less detailed clinic review than females. Less than half (44%) of the respondents reported that they performed a blood spot for blood Phe at least monthly, but only 32% considered they had been informed about the risk of high Phe levels in adulthood. Although time, cost and stress related to travelling were barriers to a face-to-face review, more than 40% of patients had concerns about remote appointments. The frequency and extent of monitoring of adults with PKU, attending specialist adult services, were less than those specified by the PKU European guidelines. The care of women of reproductive age is prioritised over men. Adult metabolic health services require further attention, development and resources to provide a high standard and equitable service to patients with PKU.

Evidence-based management of pulmonary embolism in the emergency department.

Patients with pulmonary emboli present both diagnostic and therapeutic challenges to the emergency clinician, because initial symptoms can be variable and overlap with other medical conditions. This issue reviews treatment options for patients with pulmonary emboli based on risk stratification scores of low, intermediate-low, intermediate-high, and high risk classifications. The evidence on laboratory testing and imaging is presented, as well as treatment strategies that include anticoagulation, thrombolytics, and mechanical or surgical thrombectomy. Management decisions regarding pregnancy and COVID-19 are discussed, as well as considerations for outpatient treatment of low-risk patients.

Drug-Eluting Bead, Irinotecan Therapy of Unresectable Intrahepatic Cholangiocarcinoma (DELTIC) with Concomitant Systemic Gemcitabine and Cisplatin.

BACKGROUND: Unresectable intrahepatic cholangiocarcinoma (ICC) carries a poor prognosis, and currently there are moderately established chemotherapeutic [gemcitabine/cisplatin (Gem/Cis)] treatments to prolong survival. The purpose of this study was to assess the efficacy of irinotecan drug-eluting beads (DEBIRI) therapy by transarterial infusion in combination with systemic therapy in unresectable ICC. PATIENTS AND METHODS: This is a prospective, multicenter, open-label, randomized phase II study (Clin Trials: NCT01648023-DELTIC trial) of patients with ICC randomly assigned to Gem/Cis with DEBIRI or Gem/Cis alone. The primary endpoint was response rate. RESULTS: The intention-to-treat population comprised 48 patients: 24 treated with Gem/Cis and DEBIRI and 22 with Gem/Cis alone (2 screen failures). The two groups were similar with respect to the extent of liver involvement (35% versus 38%) and presence of extrahepatic disease (29% versus 14%, p = 0.12). Median numbers of chemotherapy cycles were similar (6 versus 6), as were rates of grade 3/4 adverse events (34% for the Gem/Cis-DEBIRI group versus 36% for the Gem/Cis group). The overall response rate was significantly greater in the Gem/Cis-DEBIRI arm versus the Gem/Cis arm at 2 (p < 0.04), 4 (p < 0.03), and 6 months (p < 0.05). There was significantly more downsizing to resection/ablation in the Gem/Cis-DEBIRI arm versus the Gem/Cis arm (25% versus 8%, p < 005), and there was improved median progression-free survival [31.9 (95% CI 8.5-75.3) months versus 10.1 (95% CI 5.3-13.5) months, p = 0.028] and improved overall survival [33.7 (95% CI 13.5-54.5) months versus 12.6 (95% CI 8.7-33.4) months, p = 0.048]. CONCLUSION: Combination Gem/Cis with DEBIRI is safe, and leads to significant improvement in downsizing to resection, improved progression-free survival, and overall survival.

Coastal Tourism and Its Influence on Wastewater Nitrogen Loading: A Barrier Island Case Study.

Package treatment plants (PTPs) are facilities designed to treat onsite wastewater for small communities, commercial, and residential developments. PTPs are being utilized in a growing number of coastal communities. This study estimated the effects of coastal tourism on onsite wastewater nitrogen (N) inputs to a barrier island surficial aquifer (Bogue Banks, NC). The N-removal effectiveness was assessed for seven PTPs that treated wastewater from vacation properties using a range of technologies: extended aeration; sequencing batch reactor; and advanced media filtration. Influent and effluent wastewater samples were collected monthly from Feb. 2014 to Jan. 2015 and analyzed for particulate and dissolved N. Increased summer visitation associated with coastal tourism resulted in an increase in water use, wastewater inputs, and PTP N loading to the surficial aquifer. However, extended aeration systems did not have significantly elevated TN loads during the summer months because their treatment efficiency increased. N inputs associated with coastal tourism made up approximately 51% of the annual wastewater-related N load to the surficial aquifer. Onsite wastewater N-loading to the surficial aquifer (6.7 kg-N/ha/yr) appeared to be the dominant source of N loading on the island. Water quality data indicated that these N inputs have resulted in increased groundwater NO3 concentrations in the surficial aquifer. Overall, wastewater inputs added approximately 4.6 cm of groundwater recharge annually to the island. Coastal tourism can result in measurable increases in wastewater N loading, groundwater nitrogen concentrations, and groundwater recharge.

Should We Always Biopsy in Clinically Evident Lichen Sclerosus?

The aims of the study were to review cases of clinically diagnosed lichen sclerosus (LS) and to compare the histological features found on biopsy to clinical features seen on examination. METHODS: A retrospective chart review was undertaken of patients attending a specialist vulval service between 2013 and 2015 with a clinical diagnosis of LS. Patients in whom there was clinical diagnostic uncertainty or those with features of lichen planus or lichen planus/LS overlap were excluded. We determined the proportion of these patients who underwent vulval biopsy and reviewed their histology. RESULTS: One hundred fifteen charts were reviewed. Sixty-nine (69/115, 60%) had a firm diagnosis of established LS, and of these, 39 (39/69, 56.5%) had a biopsy performed in the previous 5 years. Thirteen (13/39, 33.3%) of these biopsies fell short of a diagnosis of LS histologically with some suggestive but nondiagnostic and some showing nonspecific features. CONCLUSIONS: Reliance on biopsies solely to establish or exclude the diagnosis of LS is inadvisable. A good level of knowledge of the characteristic clinical features is imperative among gynecologists, dermatologists, and general practitioners.

Moderating Role of Acculturation in a Mediation Model of Work-Family Conflict among Chinese Immigrants in New Zealand.

This study examined the antecedents of work-family conflict (WFC) and the mediation effects of WFC on well-being consequences among Chinese immigrants to New Zealand, along with the moderating role of acculturation. Four types of WFC were explored: time-based and strain-based work interference with family, and time-based and strain-based family interference with work. Data were collected from 577 Chinese immigrants in New Zealand, who had full-time or part-time work and lived with family members in New Zealand. The four types of WFC were differentially related to the antecedents and well-being consequences, providing some evidence that both Chinese and New Zealand cultures may exert influences on Chinese immigrants' experiences of WFC. Both directions of WFC (work interference with family, and family interference with work) were related to job satisfaction and family satisfaction, and strain-based WFC influenced their well-being more than time-based WFC. Most importantly, we found immigrants who were proficient in English perceived greater WFC and psychological strain. Copyright © 2016 John Wiley & Sons, Ltd.

Phosphate treatment by onsite wastewater systems in nutrient-sensitive watersheds of North Carolina's Piedmont.

The goal of this study was to gain a better understanding of the PO4-P treatment efficiency of onsite wastewater systems (OWS) installed in nutrient-sensitive watersheds of the North Carolina Piedmont. Four OWS including two conventional and two single-pass sand filter (SF) systems were evaluated at sites with clay-rich soils. Piezometers were installed near all of the OWS, and down-gradient from the conventional OWS for groundwater collection and characterization. Septic tanks, groundwater, SF effluent, and surface waters were sampled each season during 2015 (five times) and analyzed for PO4-P and Cl concentrations and for various environmental parameters. The conventional and SF OWS reduced PO4-P concentrations by an average of 99% and 90%, respectively, before discharge to surface waters. Mass-load reductions of PO4-P were also greater for the conventional OWS (mean 95%), relative to SF (83%) systems. The effluents discharged by SF OWS were influencing surface water quality. Additional treatment of the effluent from single-pass SF with reactive media is suggested, along with monitoring of the final effluent for PO4-P concentrations. This research provides important information that is absent from the published literature concerning PO4-P contributions to water resources from OWS in clay soils.

Are senior nurses on Clinical Commissioning Groups in England inadvertently supporting the devaluation of their profession?: A critical integrative review of the literature.

In this study, we discuss the role of senior nurses who sit on clinical commissioning groups that now plan and procure most health services in England. These nurses are expected to bring a nursing view to all aspects of clinical commissioning group business. The role is a senior level appointment and requires experience of strategic commissioning. However, little is known about how nurses function in these roles. Following Barrientos' methodology, published policy and literature were analysed to investigate these roles and National Health Service England's claim that nursing can influence and advance a nursing perspective in clinical commissioning groups. Drawing on work by Berg, Barry and Chandler on 'new public management', we discuss how nurses on clinical commissioning groups work at the alignment of the interests of biomedicine and managerialism. We propose that the way this nursing role is being implemented might paradoxically offer further evidence of the devaluing of nursing rather than the emergence of a strong professional nursing voice at the level of strategic commissioning.

Predictors of Workplace Bullying and Cyber-Bullying in New Zealand.

BACKGROUND: The negative effects of in-person workplace bullying (WB) are well established. Less is known about cyber-bullying (CB), in which negative behaviours are mediated by technology. Drawing on the conservation of resources theory, the current research examined how individual and organisational factors were related to WB and CB at two time points three months apart. METHODS: Data were collected by means of an online self-report survey. Eight hundred and twenty-six respondents (58% female, 42% male) provided data at both time points. RESULTS: One hundred and twenty-three (15%) of participants had been bullied and 23 (2.8%) of participants had been cyber-bullied within the last six months. Women reported more WB, but not more CB, than men. Worse physical health, higher strain, more destructive leadership, more team conflict and less effective organisational strategies were associated with more WB. Managerial employees experienced more CB than non-managerial employees. Poor physical health, less organisational support and less effective organisational strategies were associated with more CB. CONCLUSION: Rates of CB were lower than those of WB, and very few participants reported experiencing CB without also experiencing WB. Both forms of bullying were associated with poorer work environments, indicating that, where bullying is occurring, the focus should be on organisational systems and processes.

Psychometric properties of the General Health Questionnaire-12 in a sample of Hong Kong employees.

The General Health Questionnaire-12 (GHQ-12) is a widely used instrument for measuring psychological strain, but the factor structure of the GHQ-12 is inconclusive. The present study examined one-factor, two- and three-factor models of the GHQ-12 using structural equations modelling in a longitudinal data-set of Hong Kong employees. The findings supported a two-factor model consisting of a 'Social Dysfunction' factor measured by three items, and an 'Anxiety/Depression' factor measured by four items. Implications and limitations are discussed.

Does the degree of coarctation of the aorta influence wall shear stress focal heterogeneity?

The development of atherosclerosis in the aorta is associated with low and oscillatory wall shear stress for normal patients. Moreover, localized differences in wall shear stress heterogeneity have been correlated with the presence of complex plaques in the descending aorta. While it is known that coarctation of the aorta can influence indices of wall shear stress, it is unclear how the degree of narrowing influences resulting patterns. We hypothesized that the degree of coarctation would have a strong influence on focal heterogeneity of wall shear stress. To test this hypothesis, we modeled the fluid dynamics in a patient-specific aorta with varied degrees of coarctation. We first validated a massively parallel computational model against experimental results for the patient geometry and then evaluated local shear stress patterns for a range of degrees of coarctation. Wall shear stress patterns at two cross sectional slices prone to develop atherosclerotic plaques were evaluated. Levels at different focal regions were compared to the conventional measure of average circumferential shear stress to enable localized quantification of coarctation-induced shear stress alteration. We find that the coarctation degree causes highly heterogeneous changes in wall shear stress.

Groundwater and stream E. coli concentrations in coastal plain watersheds served by onsite wastewater and a municipal sewer treatment system.

The goal of this study was to determine if onsite wastewater treatment systems (OWS) were influencing groundwater and surface water Escherichia coli concentrations in a coastal plain watershed. Piezometers for groundwater monitoring were installed at four residences served by OWS and five residences served by a municipal wastewater treatment system (MWS). The residences were located in two different, but nearby (<3 km), watersheds. Effluent from the four septic tanks, groundwater from piezometers, and the streams draining the OWS and MWS watersheds were sampled on five dates between September 2011 and May 2012. Groundwater E. coli concentrations and specific conductivity were elevated within the flow path of the OWS and near the stream, relative to other groundwater sampling locations in the two watersheds. Groundwater discharge in the OWS watershed could be a contributor of E. coli to the stream because E. coli concentrations in groundwater at the stream bank and in the stream were similar. Stream E. coli concentrations were higher for the OWS in relation to MWS watersheds on each sampling date. Water quality could be improved by ensuring OWS are installed and operated to maintain adequate separation distances to water resources.

A Three-wave Study of Antecedents of Work-Family Enrichment: The Roles of Social Resources and Affect.

On the basis of conservation of resources theory (Hobfoll, ) and the resource-gain-development perspective (Wayne, Grzywacz, Carlson, & Kacmar, ), this paper examines the differential impact of specific social resources (supervisory support and family support) on specific types of affect (job satisfaction and family satisfaction, respectively), which, in turn, influence work-to-family enrichment and family-to-work enrichment, respectively. A sample of 276 Chinese workers completed questionnaires in a three-wave survey. The model was tested with structural equation modelling. Job satisfaction at time 2 partially mediated the relationship between time 1 supervisory support and time 3 work-to-family enrichment (capital), and the effect of supervisory support on work-to-family enrichment (affect) was fully mediated by job satisfaction. Family satisfaction at time 2 fully mediated the relationship between time 1 family support and time 3 family-to-work enrichment (affect, efficiency). Implications for theory, practice and future research are discussed.

Fate and transport of enteric microbes from septic systems in a coastal watershed.

Onsite wastewater treatment systems (OWTS) are commonly used in coastal areas to treat household wastewater. These systems represent potential sources of fecal pollution of groundwater and nearby surface water. OWTS are expected to reduce microbial concentrations in wastewater; however, system and environmental factors can affect treatment efficiency and impacts on ground and surface water. In the study of OWTS described in this article, the authors sampled septic tanks and groundwater at two households in coastal North Carolina between October 2009 and October 2011. Samples were tested for the fecal indicator microbes E. coli, enterococci, and Clostridium perfringens. Microbial source tracking was also performed in year two. Results showed that enteric microbe concentrations in groundwater significantly decreased with distance from the OWTS. Human markers of fecal contamination were also detected in the OWTS and downgradient groundwater, indicating that OWTS can impact the microbial quality of shallow groundwater.

Detection of pharmaceuticals and other personal care products in groundwater beneath and adjacent to onsite wastewater treatment systems in a coastal plain shallow aquifer.

Onsite wastewater treatment systems (OWTS) are the predominant disposal method for human waste in areas without municipal sewage treatment alternatives. Relatively few studies have addressed the release of pharmaceuticals and personal care products (PPCPs) from OWTS to groundwater. PPCP fate and transport from OWTS are important, particularly where these systems are adjacent to sensitive aquatic ecosystems such as coastal areas or wetlands. The objectives of this study were to identify PPCPs in residential wastewater and groundwater beneath OWTS and to characterize the environmental conditions affecting the OWTS discharge of PPCPs to nearby streams. The study sites are in coastal plain aquifers, which may be considered vulnerable "end-members" for subsurface PPCP transport. The PPCPs most commonly detected in the OWTS, at concentrations ranging from 0.12 µg L(-1) to 12.04 µg L(-1) in the groundwater, included: caffeine, ibuprofen, DEET, and homosalate. Their presence was related to particulate and dissolved organic carbon abundance.

Nitrogen and carbon dynamics beneath on-site wastewater treatment systems in Pitt County, North Carolina.

On-site wastewater treatment systems (OWS) are a potentially significant non-point source of nutrients to groundwater and surface waters, and are extensively used in coastal North Carolina. The goal of this study was to determine the treatment efficiency of four OWS in reducing total dissolved nitrogen (TDN) and dissolved organic carbon (DOC) concentrations before discharge to groundwater and/or adjacent surface water. Piezometers were installed for groundwater sample collection and nutrient analysis at four separate residences that use OWS. Septic tank effluent, groundwater, and surface water samples (from an adjacent stream) were collected four times during 2012 for TDN and DOC analysis and pH, temperature, electrical conductivity, and dissolved oxygen measurements. Treatment efficiencies from the tank to the groundwater beneath the drainfields ranged from 33 to 95% for TDN and 45 to 82% for DOC, although dilution accounted for most of the concentration reductions. There was a significant positive correlation between nitrate concentration and separation distance from trench bottom to water table and a significant negative correlation between DOC concentration and separation distance. The TDN and DOC transport (>15 m) from two OWS with groundwater saturated drainfield trenches was significant.

Development of a nondestructive leak testing method utilizing the head space analyzer for ampoule products containing ethanol-based solutions.

The application of a head space analyzer for oxygen concentration was examined to develop a novel ampoule leak test method. Studies using ampoules filled with ethanol-based solution and with nitrogen in the headspace demonstrated that the head space analysis (HSA) method showed sufficient sensitivity in detecting an ampoule crack. The proposed method is the use of HSA in conjunction with the pretreatment of an overpressurising process known as bombing to facilitate the oxygen flow through the crack in the ampoule. The method was examined in comparative studies with a conventional dye ingress method, and the results showed that the HSA method exhibits sensitivity superior to the dye method. The results indicate that the HSA method in combination with the bombing treatment provides potential application as a leak test for the detection of container defects not only for ampoule products with ethanol-based solutions, but also for testing lyophilized products in vials with nitrogen in the head space. LAY ABSTRACT: The application of a head space analyzer for oxygen concentration was examined to develop a novel ampoule leak test method. The proposed method is the use of head space analysis (HSA) in conjunction with the pretreatment of an overpressurising process known as bombing to facilitate oxygen flow through the crack in the ampoule for use in routine production. The result of the comparative study with a conventional dye leak test method indicates that the HSA method in combination with the bombing treatment can be used as a leak test method, enabling detection of container defects.