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CONTEXT: Current literature suggests that contact with specialist palliative care for patients diagnosed with hematological malignancy is infrequent. As part of an investigation into patterns of care, the symptom profile of this patient group required elucidation. OBJECTIVES: The purpose of this study was to determine the patterns of symptoms and level of distress in patients diagnosed with a hematological malignancy. METHODS: One hundred eighty patients diagnosed with a hematological malignancy attending a tertiary referral hospital completed the Memorial Symptom Assessment Scale-Short Form. Comparisons were made to published symptom prevalence studies of those with nonhematological malignancies. RESULTS: Patients with hematological malignancy had a considerable physical and psychological symptom burden, with an overall mean of 8.8 (±5.9) symptoms. The mean number of symptoms was significantly greater in those on treatment (P<0.05), those with poorer performance status (P<0.001), inpatients (P<0.01), and those with a more advanced disease stage (P<0.001) than their respective counterparts. Symptom prevalence ranged from 69% for fatigue to 9% for vomiting. Global, physical, and psychological distress scores were high and varied significantly according to disease stage, Eastern Cooperative Oncology Group status, and patient location. The mean number of symptoms and level of distress were comparable to those patients with metastatic nonhematological malignancy. CONCLUSION: Patients with hematological malignancy are likely to have symptom control needs similar to those with metastatic cancer. Because such symptom burden appears to affect those at all phases of illness, comprehensive symptom assessment is suggested throughout. The introduction of palliative care services during times of increased symptom burden may assist hematologists and other carers in the management of their patients' distress and quality of life. [ABSTRACT FROM AUTHOR]

Aim: To explore the benefits and challenges of a recently introduced Registered Undergraduate Student of Nursing workforce from the perspective of Nurses and Registered Undergraduate Students of Nursing, in a major metropolitan hospital in Australia in 2020., Design: A qualitative descriptive study was undertaken using individual interviews and focus groups., Methods: Purposively selected employed Registered Undergraduate Students of Nursing and nurses who worked with them were interviewed, using a semi-structured format. Recordings were transcribed and coded using NVivo software. Reflexive thematic analysis using an inductive approach was undertaken., Results: Four major themes were revealed: (i) Navigating the programme, (ii) Belonging and integration; (iii) Patient care; and (iv) Continuing Development. Initial challenges were common, often related to clarifying the scope of practice for the new role. Ongoing issues were associated with gaps in understanding the role and lack of integration into the team. Mostly, nurses and Registered Undergraduate Students of Nursing built positive, professional relationships. Nurses valued the Registered Undergraduate Student of Nursing knowledge and skill level, reporting improved workload and work experiences when the Registered Undergraduate Student of Nursing was on shift. Nurses believed that the Registered Undergraduate Students of Nursing enhanced patient care. Registered Undergraduate Students of Nursing described positive, therapeutic relationships with patients. Registered Undergraduate Student of Nursing employment provided opportunities for new learning, leading to increased efficiency and confidence on clinical placement., Conclusions: This employment model benefited the Registered Undergraduate Students of Nursing and nurses who worked with them. In the absence of adequate training and support, challenges remained unresolved and negatively impacted the experience for nurses. In addition to university-level education and clinical placement, the employment model can create a third space for student learning via on-the-job training. The study supports the ongoing employment of student nurses through the Registered Undergraduate Student of Nursing model., Implications for the Profession And/or Patient Care: Impact This study contributes to the very small body of literature investigating Registered Undergraduate Student of Nursing workforces in Australian hospitals. It is the first to explore the experiences of both nurses and students working together in a major metropolitan setting and also the first in the context of the COVID-19 pandemic. This study reflected a mostly positive experience for Registered Undergraduate Student of Nursing and the nurses who worked with them and highlighted the importance of adequate oversight and support in the implementation and maintenance of a Registered Undergraduate Student of Nursing workforce. Employed Registered Undergraduate Students of Nursing reported improved confidence, skills, and felt like they started clinical placement at an advantage, ready to step up and learn the Registered Nurse scope of practice. In addition to university-level education and clinical placement, this employment model creates a third space for learning via on-the-job training., Reporting Method: COREQ guidelines were followed in the reporting of this study., Patient or Public Contribution: No patient or public contribution., (© 2024 John Wiley & Sons Ltd.)

As a result of climate change heatwaves are expected to increase in frequency and intensity and will have detrimental impacts on human health globally. EDs are often the critical point of care for acute heat illnesses and other conditions associated with heat exposure. Existing literature has focused on heatwave-related hospitalisation and mortality. This scoping review aimed to identify, evaluate and summarise current literature regarding patient characteristics and outcomes of ED admissions from heatwaves. A scoping review of the literature was conducted using six databases: Medline, EMBASE, EMCARE, CINAHL, PsycINFO, and Scopus, using MeSH terms and keywords related to 'heatwave' and 'Emergency Department'. Articles were included if they were: published in English from January 2000 to August 2021, related to ED, and examined high temperature periods consistent with heatwave criteria. Articles were appraised using the Mixed Methods Appraisal Tool (MMAT). Thirty-one studies were included, mostly from the United States, Australia, and France. The study designs include retrospective case analysis, case-control, and time-series analysis. Eight studies examined known heatwaves, 21 used different criteria to identify heatwave occurrence, and two focused on heat-related illness. The selected articles display a moderate-high quality on MMAT. ED admissions for both heat-related illnesses and other conditions increased during heatwaves, with up to 18.5 times risk increase. The risk was elevated for all population groups, and substantially in the elderly, male patients with certain comorbidities, medications, or lower socioeconomic status. Outcomes including hospitalisation and mortality rates after ED admissions showed positive associations with heatwaves. The heatwaves resulting from climate change will place increasing demands on EDs providing care for increasingly susceptible populations. Significant public heatwave planning across multiple sectors is required to reduce the risk of overwhelming EDs with these patients., (© 2023 The Authors. Emergency Medicine Australasia published by John Wiley & Sons Australia, Ltd on behalf of Australasian College for Emergency Medicine.)

Purpose: The purposes of this study are to, firstly, develop techniques to accurately identify extensor mechanism malalignment by measuring the alignment of the quadriceps tendon (QTA) with computerized tomography (CT) scans. Secondly, to investigate correlations between QTA and lower limb bony anatomical variations within a representative normal population. Lastly, to evaluate the clinical significance of QTA by establishing its potential connection with lateral facet patellofemoral joint osteoarthritis (LFPFJOA)., Method: CT scans were orientated to a mechanical axis reference frame and three techniques developed to measure the alignment of the quadriceps tendon. Multiple measurement of bony alignment from the hip to the ankle were performed on each scan. A series of 110 cadaveric CT scans were measured to determine normal values, reproducibility, and correlations with bony anatomy. Secondly, a comparison between 2 groups of 25 patients, 1 group with LFPFJOA and 1 group with isolated medial OA and no LFPFJOA., Results: From the cadaveric study, it was determined that the alignment of the quadriceps tendon is on average 4.3° (SD 3.9) varus and the apex of the tendon is 9.1 mm (SD 7.7 mm) lateral to the trochlear groove and externally rotated 1.9° (SD 12.4°) from the centre of the femoral shaft. There was no association between the quadriceps tendon alignment and any other bony measurements including tibial tubercle trochlear groove distance (TTTG), coronal alignment, trochlear groove alignment and femoral neck anteversion. A lateralized QTA was significantly associated with LFPFJOA. QTA in the LFPFJOA group was 9.6° varus (SD 2.8°), 21.3 mm (SD 6.6) lateralised and 17.3° ER (SD 11°) compared to 5.5° (SD 2.3°), 10.7 mm (SD 4.9) and 3.3° (SD 7.2°), respectively, in the control group (p < 0.001). A significant association with LFPFJOA was also found for TTTG (17.2 mm (SD 5.7) vs 12.1 mm (SD 4.3), p < 0.01). Logistic regression analysis confirmed the QTA as having the stronger association with LFPFJOA than TTTG (AUC 0.87 to 0.92 for QTA vs 0.79 for TTTG)., Conclusion: These studies have confirmed the ability to accurately determine QTA on CT scans. The normal values indicate that the QTA is highly variable and unrelated to bony anatomy. The comparative study has determined that QTA is clinically relevant and a lateralised QTA is the dominant predictor of severe LFPFJOA. This deformity should be considered when assessing patella maltracking associated with patella osteoarthritis, patella instability and arthroplasty., Level of Evidence: III (retrospective cohort study)., (© 2023. The Author(s) under exclusive licence to European Society of Sports Traumatology, Knee Surgery, Arthroscopy (ESSKA).)

Objective: This study aimed to compare the heart rate response to stress during airway intubations in clinical practice and a simulated environment., Methods: Twenty-five critical care registrars participated in the study over a 3-month period. Heart rate data during intubations was recorded by a FitBit® Charge 2 worn by each participant during their clinical practice, and during a single simulated airway management scenario. The heart rate range was calculated by subtracting the baseline working heart rate (BWHR) from the maximum functional heart rate (MFHR). For each airway intubation performed participants recorded an airway diary entry. Data from intubations performed in the clinical environment was compared to data from a simulated environment. Heart rate changes were observed in two ways: percentage rise (median) across the 20-min intubation period and; percentage rise at point of intubation (median)., Results: Eighteen critical care registrars completed the study, mean age 31.8 years (SD = 2.015, 95% CI = 30.85-32.71). Throughout the 20-min peri-intubation recording period there was no significant difference in the median change in heart rates between the clinical (14.72%) and simulation (15.96%) environment (p = 0.149). At the point of intubation there was no significant difference in the median change in heart rate between the clinical (16.03%) and the simulation (25.65%) environment groups (p = 0.054)., Conclusion: In this small population of critical care trainees, a simulation scenario induced a comparable heart rate response to the clinical environment during intubation. This provides evidence that simulation scenarios are able to induce a comparable physiological stress response to the clinical environment and thus facilitates effective teaching of a high-risk procedure in a safe manner., (© 2023. The Author(s).)

Background: People experiencing homelessness have an increased risk of mortality. The association between being at risk of homelessness and premature mortality is unclear. We aimed to determine all-cause and cause-specific mortality in patients who were homeless, at risk of homelessness (marginally housed), or housed., Methods: This retrospective longitudinal cohort study compared mortality patterns in adult patients identified in 2003/04 by linking data from an Australian metropolitan emergency department to national mortality data. We used Cox proportional hazards models to estimate associations between housing status and mortality. To address competing risks, cause-specific hazards were modelled and transformed into stacked cumulative incidence functions., Findings: Data from 6290 patients (homeless deceased = 382/1050, marginally housed deceased = 259/518, housed deceased = 1204/4722) found increased risk of mortality in homeless [hazard ratio (HR) = 4.0, 95% confidence interval (CI) = 2.0-3.3) and marginally housed (HR = 2.6, 95% CI = 3.4-4.8) patients. Homeless patients had an excess risk from external causes (HR = 6.1, 95% CI = 4.47-8.35), cardiovascular disease (HR = 4.9, 95% CI = 2.78-8.70) and cancer (HR = 1.5, 95% CI = 1.15-2.09). Marginally housed patients had increased risk from external causes (HR = 3.6, 95% CI = 2.36-5.40) and respiratory diseases (HR = 4.7, 95% CI = 1.82-12.05). Taking account of competing risk, marked inequality was observed, with homeless, marginally housed and housed patients having probabilities of death by 55 years of 0.2, 0.1 and 0.02, respectively., Conclusions: Mortality rates were elevated in patients who were homeless or at risk of homelessness. Increasing numbers of people are at risk of homelessness, and the effect of this on mortality is relatively unrecognized. Marginal housing may assuage some risk of premature mortality associated with homelessness; however, it is not equivalent to stable housing., (© The Author(s) 2023. Published by Oxford University Press on behalf of the International Epidemiological Association.)

Objective: Using a strength-based framework, we aimed to describe and compare First Nations patients who completed care in an ED to those who took their own leave., Methods: Routinely collected adult patient data from a metropolitan ED collected over a 5-year period were analysed., Results: A total of 6446 presentations of First Nations patients occurred from 2016 to 2020, constituting 3% of ED presentations. Of these, 5589 (87%) patients waited to be seen and 857 (13%) took their own leave. Among patients who took their own leave, 624 (73%) left not seen and 233 (27%) left at own risk after starting treatment. Patients who were assigned a triage category of 4-5 were significantly more likely to take their own leave (adjusted odds ratio [OR] 3.17, 95% confidence interval [CI] 2.67-3.77, P < 0.001). Patients were significantly less likely to take their own leave if they were >60 years (adjusted OR 0.69, 95% CI 1.01-1.36, P = 0.014) and had private health insurance (adjusted OR 0.61, 95% CI 0.45-0.84, P < 0.001). Patients were more likely to leave if they were women (adjusted OR 1.17, 95% CI 1.01-1.36, P = 0.04), had an unknown housing status (adjusted OR 1.76, 95% CI 1.44-2.15, P < 0.001), were homeless (adjusted OR 1.50, 95% CI 1.22-1.93, P < 0.001) or had a safety alert (adjusted OR 1.60, 95% CI 1.35-1.90, P < 0.001)., Conclusion: A lower triage category is a strong predictor of First Nations patients taking their own leave. It has been documented that First Nations patients are under-triaged. One proposed intervention in the metropolitan setting is to introduce practices which expediate the care of First Nations patients. Further qualitative studies with First Nations patients should be undertaken to determine successful approaches to create equitable access to emergency healthcare for this population., (© 2022 The Authors. Emergency Medicine Australasia published by John Wiley & Sons Australia, Ltd on behalf of Australasian College for Emergency Medicine.)

Objectives: Alcohol is the most widely consumed psychoactive substance in Australia and the consequences of alcohol consumption have enormous personal and social impacts. This study aimed to describe the principal diagnoses of emergency department (ED) presentations involving alcohol use in the previous 12 hours at eight hospitals in Victoria and the Australian Capital Territory, Australia., Methods: Twelve months' data (1 July 2018 - 30 June 2019) were collected from eight EDs, including demographics, ICD-10 codes, hospital location and self-reported drinking in the preceding 12 hours. The ten most common ICD-10 discharge codes were analysed based on age, sex and hospital geographic area., Results: ICD codes pertaining to mental and behavioural disorders due to alcohol use accounted for the highest proportion in most EDs. Suicide ideation/attempt was in the five highest ICD codes for all but one hospital. It was the second most common alcohol-related presentation for both males and females., Conclusions: Alcohol plays a major role in a range of presentations, especially in relation to mental health and suicide., Implications for Public Health: The collection of alcohol involvement in ED presentations represents a major step forward in informing the community about the burden of alcohol on their health resources., (© 2022 The Authors.)

Background: The chance of hospital staff encountering a patient with a trauma history is high. The way health services are offered and carried out are important when engaging with people who have experienced trauma. Implementing training in trauma-informed care (TIC) is part of a cultural change of benefit to both patients and staff. Simulation-based training is a well-accepted method to reduce staff fear and anxiety when working with individuals in distress and to address issues relating to bias and stigma., Objectives: To provide simulation-based TIC training to graduate nurses., Design: A three-phase process was undertaken to i) create the intervention, ii) determine feasibility, and iii) evaluate the developed training., Setting: A 600-bed inner-city tertiary hospital in Melbourne, Victoria., Participants: Graduate nurses undertaking their first year of employment (n = 23)., Methods: The content of the training was created using evidence derived from a literature review, a scoping study of available resources, and expert consensus. A pre/post-test within-groups design to assess the safety, acceptability, and effectiveness of the training was undertaken., Results: The Trauma Informed-Simulation Based Training (TI-SBT) aims to increase TIC knowledge and promote TIC behaviours. It is delivered face-to-face over one day and encompasses an education component followed by three immersive patient simulations using professional actors. Analysis found significant improvement in TIC knowledge (p ≤ 0.001, 95% CI = -3.53, -0.47) and behaviours (p = 0.013, 95% CI = -8.88, -5.03). No significant differences were found in measures of anxiety and confidence. Satisfaction with all aspects of the training was high. Qualitatively, participants provided concrete examples of changes to their practice to facilitate TIC., Conclusions: The developed and novel TI-SBT is a feasible (safe, acceptable, and effective) way of introducing TIC to graduate nurses. These findings provide strong evidence to support a more rigorous evaluation of the training by randomised controlled trial. The TI-SBT has the capacity to not only improve patient care but the experience of hospital staff., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Purpose: Patellar dislocation is associated with a range of anatomical abnormalities affecting the trochlea, extensor mechanism and the tibia. The relationship between patellofemoral instability and rotational abnormalities of the posterior condyles, trochlear groove and proximal tibia has not been adequately determined. This study aimed to identify the frequency and severity of anatomical risk factors to determine their relative contribution to patellofemoral instability., Methods: A retrospective morphological study was undertaken comparing multiple anatomical measurements with magnetic resonance imaging of 50 patients with patellofemoral instability to an age- and gender-matched Control group (n = 50). Several techniques were assessed measuring both femoral and tibial axial asymmetry. A new measurement, tibial rotational asymmetry, comparing a line between the midpoints of the collateral ligaments to the axis between the patellar tendon and posterior cruciate ligament, was assessed for its association with patellofemoral instability., Results: Compared to the controls, the patellofemoral instability group demonstrated a significant difference in tibial rotational asymmetry, with a mean of 2.9° (SD 3.2°) externally rotated vs - 1.6° (SD 2.2°) in the control group. Significant differences were also demonstrated regarding the sulcus angle, tibial tubercle-trochlear groove distance, tibial tubercle-posterior cruciate ligament distance, patellar size and the Insall-Salvati ratio. There were no differences between groups regarding the lengths of the posterior condyles, the heights of the trochlear ridges or lateralisation of the trochlear groove. Further analysis of the patellofemoral instability group revealed a subgroup of males with normal anatomy (7/50) and a subgroup of females with isolated patella alta (7/50)., Conclusion: Patellofemoral instability is associated with tibial rotational asymmetry due to lateralisation of the tibial tubercle. It is also associated with patella alta and reduced trochlear groove depth. The femoral axial shape is otherwise unchanged., Level of Evidence: III., (© 2021. The Author(s) under exclusive licence to European Society of Sports Traumatology, Knee Surgery, Arthroscopy (ESSKA).)

Opioid-related harms have been increasing in Australia over the last 5 years. Patients with opioid use disorder are over-represented in ED presentations. Opioid agonist treatment is the most effective community-based treatment. Buprenorphine is considered the safest of these treatments to use in the ED setting. This rapid review investigated the effectiveness of initiating buprenorphine in the ED setting. Medline, Embase, Emcare, PSYCinfo, CINAHL and Cochrane Central Register of Controlled Trials databases were searched. Randomised and non-randomised studies published in peer-reviewed journals that involved the initiation of buprenorphine in the ED setting were considered eligible. The search revealed 350 articles of which 11 were included in the review; three articles representing two randomised controlled trials (RCTs) and eight observational studies. Data were extracted from included papers and risk of bias assessed on the RCTs. One well-conducted RCT showed that buprenorphine initiated in the ED does improve treatment engagement up to 2 months after an ED visit. Eight observational studies, one with a comparator group reported positive results for this intervention. There is strong evidence that clinicians should consider commencing buprenorphine in the ED for patients with opioid use disorder when combined with a direct and supported referral or 'warm handover' to community care. Further implementation studies and investigation of long-acting injectable buprenorphine treatment are required., (© 2020 Australasian College for Emergency Medicine.)

Objective: To examine the effect of homelessness on mortality., Methods: This 15-year retrospective longitudinal cohort study compared mortality outcomes of homeless and non-homeless adults attending the emergency department of an inner-city public hospital in Melbourne, Victoria between 1 January 2003 and 31 December 2004. Homeless individuals had ≥1 recorded episodes of homelessness within the recruitment period, categorised by type: primary, secondary, tertiary, marginally housed. Non-homeless individuals were stably housed throughout., Results: Over 15 years, homeless individuals had a higher mortality rate (11.89 vs. 8.10 per 1,000 person-years), significantly increased mortality risk (rate ratio 1.47, 95% confidence interval [CI] 1.26-1.71) and younger median age at death (66.60 vs. 78.19 years) compared to non-homeless individuals. Using adjusted Cox proportional hazards models, primary (hazard ratio [HR] 2.05, 95%CI 1.67-2.50), secondary (HR 1.60, 95%CI 1.23-2.10) and tertiary (HR 1.72, 95%CI 1.16-2.56) homelessness were independent risk factors for premature mortality., Conclusion: At least one recorded episode of primary, secondary, or tertiary homelessness was associated with premature mortality and younger age at death over a 15-year period. Implications for public health: Accurately identifying individuals experiencing primary, secondary or tertiary homelessness at the emergency department may enable targeted interventions that could potentially reduce their risk of premature mortality., (© 2020 The Authors.)

Multiple acyl-CoA dehydrogenase deficiency, or glutaric aciduria type II, is an autosomal recessive disorder of fatty acid oxidation due to defects in electron transfer flavoprotein (ETF) encoded by ETFA and ETFB, or in electron transfer flavoprotein dehydrogenase (ETFDH) encoded by the ETFDH gene. The disease may present as a severe neonatal onset form and a mild late-onset form which is heterogeneous for the age at onset and clinical presentation. We describe two patients in their seventies, referred for a nonspecific myopathy, which resulted to manifest carriers of ETFDH gene mutation. Treatment with riboflavin and L-carnitine improved the clinical picture and the biochemical profile. This condition should be included in the differential diagnosis of myopathies even at an old age.

Objective: To determine the prevalence, predictors, and characteristics of health-related internet searches by adult emergency department (ED) patients; to examine the effect of searching on the doctor-patient relationship and treatment compliance., Design: A multi-centre, observational, cross-sectional study; a purpose-designed 51-item survey, including tools for assessing e-health literacy (eHEALS) and the effects of internet searching on the doctor-patient relationship (ISMII). Setting, participants: 400 adult patients presenting to two large tertiary referral centre emergency departments in Melbourne, February-May 2017., Outcome Measures: Descriptive statistics for searching prevalence and characteristics, doctor-patient interaction, and treatment compliance; predictors of searching; effect of searching on doctor-patient interaction., Results: 400 of 1056 patients screened for eligibility were enrolled; their mean age was 47.1 years (SD, 21.1 years); 51.8% were men. 196 (49.0%) regularly searched the internet for health information; 139 (34.8%) had searched regarding their current problem before presenting to the ED. The mean ISMII score was 30.3 (95% CI, 29.6-31.0); searching improved the doctor-patient interaction for 150 respondents (77.3%). Younger age (per 10-year higher age band: odds ratio [OR], 0.74; 95% CI, 0.61-0.91) and greater e-health literacy (per one-point eHEALS increase: OR, 1.11; 95% CI, 1.06-1.17) predicted searching the current problem prior to presentation; e-health literacy predicted ISMII score (estimate, 0.39; 95% CI, 0.20-0.39). Most patients would never or rarely doubt their diagnosis (79%) or change their treatment plan (91%) because of conflicting online information., Conclusion: Online health care information was frequently sought before presenting to an ED, especially by younger and e-health literate patients. Searching had a positive impact on the doctor-patient interaction and was unlikely to reduce adherence to treatment.

Background: Lysosomal storage diseases (LSDs) are inborn errors of metabolism resulting from 50 different inherited disorders. The increasing availability of treatments and the importance of early intervention have stimulated newborn screening (NBS) to diagnose LSDs and permit early intervention to prevent irreversible impairment or severe disability. We present our experience screening newborns in North East Italy to identify neonates with Mucopolysaccharidosis type I (MPS I) and Pompe, Fabry, and Gaucher diseases., Methods: Activities of acid β-glucocerebrosidase (ABG; Gaucher), acid α-glucosidase (GAA; Pompe), acid α-galactosidase (GLA; Fabry), and acid α-L-iduronidase (IDUA; MPS-I) in dried blood spots (DBS) from all newborns during a 17-month period were determined by multiplexed tandem mass spectrometry (MS/MS) using the NeoLSD ® assay system. Enzymatic activity cutoff values were determined from 3500 anonymous newborn DBS. In the screening study, samples were retested if the value was below cutoff and a second spot was requested, with referral for confirmatory testing and medical evaluation if a low value was obtained., Results: From September 2015 to January 2017, 44,411 newborns were screened for the four LSDs. We recalled 40 neonates (0.09%) for collection of a second DBS. Low activity was confirmed in 20, who had confirmatory testing. Ten of 20 had pathogenic mutations: two Pompe, two Gaucher, five Fabry, and one MPS-I. The incidences of Pompe and Gaucher diseases were similar (1/22,205), with Fabry disease the most frequent (1/8882) and MPS-I the rarest (1/44411). The combined incidence of the four disorders was 1/4411 births., Conclusions: Simultaneously determining multiple enzyme activities by MS/MS, with a focus on specific biochemical markers, successfully detected newborns with LSDs. The high incidence of these disorders supports this screening program.

Coenzyme Q (CoQ, or ubiquinone) is a remarkable lipid that plays an essential role in mitochondria as an electron shuttle between complexes I and II of the respiratory chain, and complex III. It is also a cofactor of other dehydrogenases, a modulator of the permeability transition pore and an essential antioxidant. CoQ is synthesized in mitochondria by a set of at least 12 proteins that form a multiprotein complex. The exact composition of this complex is still unclear. Most of the genes involved in CoQ biosynthesis (COQ genes) have been studied in yeast and have mammalian orthologues. Some of them encode enzymes involved in the modification of the quinone ring of CoQ, but for others the precise function is unknown. Two genes appear to have a regulatory role: COQ8 (and its human counterparts ADCK3 and ADCK4) encodes a putative kinase, while PTC7 encodes a phosphatase required for the activation of Coq7. Mutations in human COQ genes cause primary CoQ(10) deficiency, a clinically heterogeneous mitochondrial disorder with onset from birth to the seventh decade, and with clinical manifestation ranging from fatal multisystem disorders, to isolated encephalopathy or nephropathy. The pathogenesis of CoQ(10) deficiency involves deficient ATP production and excessive ROS formation, but possibly other aspects of CoQ(10) function are implicated. CoQ(10) deficiency is unique among mitochondrial disorders since an effective treatment is available. Many patients respond to oral CoQ(10) supplementation. Nevertheless, treatment is still problematic because of the low bioavailability of the compound, and novel pharmacological approaches are currently being investigated. This article is part of a Special Issue entitled 'EBEC 2016: 19th European Bioenergetics Conference, Riva del Garda, Italy, July 2-6, 2016', edited by Prof. Paolo Bernardi., (Copyright © 2016. Published by Elsevier B.V.)

Purpose: The sulcus line (SL) is a three-dimensional curve produced from multiple points along the trochlear groove. Whiteside's Line, also known as the anteroposterior axis (APA), is derived from single anterior and posterior points. The purposes of the two studies presented in this paper are to (1) assess the results from the clinical use of the SL in a large clinical series, (2) measure the SL and the APA on three-dimensional CT reconstructions, (3) demonstrate the effect of parallax error on the use of the APA and (4) determine the accuracy of an axis derived by combining the SL and the posterior condylar axis (PCA)., Methods: In the first study, we assessed the SL using a large, single surgeon series of consecutive patients undergoing primary total knee arthroplasties. The post-operative CT scans of patients (n = 200) were examined to determine the final rotational alignment of the femoral component. In the second study, measurements were taken in a series of 3DCT reconstructions of osteoarthritic knees (n = 44)., Results: The mean position of the femoral component in the clinical series was 0.6° externally rotated to the surgical epicondylar axis, with a standard deviation of 2.9° (ranges from -7.2° to 6.7°). On the 3DCT reconstructions, the APA (88.2° ± 4.2°) had significantly higher variance than the SL (90.3° ± 2.7°) (F = 5.82 and p = 0.017). An axis derived by averaging the SL and the PCA+3° produced a significant decrease in both the number of outliers (p = 0.03 vs. PCA and p = 0.007 vs. SL) and the variance (F = 6.15 and p = 0.015 vs. SL). The coronal alignment of the SL varied widely relative to the mechanical axis (0.4° ± 3.8°) and the distal condylar surface (2.6° ± 4.3°)., Conclusions: The multiple points used to determine the SL confer anatomical and geometrical advantages, and therefore, it should be considered a separate rotational landmark to the APA. These findings may explain the high degree of variability in the measurement of the APA which is documented in the literature. Combining a geometrically correct SL and the PCA is likely to further improve accuracy.

Background: Respite services are recommended as an important support for caregivers of children with life-threatening conditions. However, the benefits of respite have not been convincingly demonstrated through quantitative research., Aim: To determine the impact of out-of home respite care on levels of fatigue, psychological adjustment, quality of life and relationship satisfaction among caregivers of children with life-threatening conditions., Design: A mixed-methods, pre-test and post-test study, Setting/participants: A consecutive sample of 58 parental caregivers whose children were admitted to a children's hospice for out-of-home respite over an average of 4 days., Results: Caregivers had below-standard levels of quality of life compared to normative populations. Paired t-tests demonstrated that caregivers' average psychological adjustment scores significantly improved from pre-respite (mean = 13.9, standard error = 0.71) to post-respite (mean = 10.7, standard error = 1); p < 0.001, 95% confidence interval: 1.25-5.11). Furthermore, caregivers' average fatigue scores significantly improved from pre-respite (mean = 14.3, standard error = 0.85) to post-respite (mean = 10.9, standard error = 1.01; p < 0.001, 95% confidence interval: 1.69-7.94), and caregivers' average mental health quality of life scores significantly improved from pre-respite (mean = 44.2, standard error = 1.8) to post-respite (mean = 49.1, standard error = 1.6; p < 0.01, 95% confidence interval: -9.56 to 0.36). Qualitative data showed caregivers sought respite for relief from intensive care provision and believed this was essential to their well-being., Conclusion: Findings indicate the effectiveness of out-of-home respite care in improving the fatigue and psychological adjustment of caregivers of children with life-threatening conditions. Study outcomes inform service provision and future research efforts in paediatric palliative care., (© The Author(s) 2015.)

Objectives: To investigate the quality of end-of-life care for patients with metastatic non-small cell lung cancer (NSCLC)., Design and Participants: Retrospective cohort study of patients from first hospitalisation for metastatic disease until death, using hospital, emergency department and death registration data from Victoria, Australia, between 1 July 2003 and 30 June 2010., Main Outcome Measures: Emergency department and hospital use; aggressiveness of care including intensive care and chemotherapy in last 30 days; palliative and supportive care provision; and place of death., Results: Metastatic NSCLC patients underwent limited aggressive treatment such as intensive care (5%) and chemotherapy (< 1%) at the end of life; however, high numbers died in acute hospitals (42%) and 61% had a length of stay of greater than 14 days in the last month of life. Although 62% were referred to palliative care services, this occurred late in the illness. In a logistic regression model adjusted for year of metastasis, age, sex, metastatic site and survival, the odds ratio (OR) of dying in an acute hospital bed compared with death at home or in a hospice unit decreased with receipt of palliative care (OR, 0.25; 95% CI, 0.21-0.30) and multimodality supportive care (OR, 0.65; 95% CI, 0.56-0.75)., Conclusion: Because early palliative care for patients with metastatic NSCLC is recommended, we propose that this group be considered a benchmark of quality end-of-life care. Future work is required to determine appropriate quality-of-care targets in this and other cancer patient cohorts, with particular focus on the timeliness of palliative care engagement.

Background: Palliative care incorporates comprehensive support of family caregivers because many of them experience burden and distress. However, evidence-based support initiatives are few., Purpose: We evaluated a one-to-one psychoeducational intervention aimed at mitigating the distress of caregivers of patients with advanced cancer receiving home-based palliative care. We hypothesised that caregivers would report decreased distress as assessed by the General Health Questionnaire (GHQ)., Method: A randomised controlled trial comparing two versions of the delivery of the intervention (one face-to-face home visit plus telephone calls versus two visits) plus standard care to a control group (standard care only) across four sites in Australia., Results: Recruitment to the one visit condition was 57, the two visit condition 93, and the control 148. We previously reported non-significant changes in distress between times 1 (baseline) and 2 (1-week post-intervention) but significant gains in competence and preparedness. We report here changes in distress between times 1 and 3 (8-week post-death). There was significantly less worsening in distress between times 1 and 3 in the one visit intervention group than in the control group; however, no significant difference was found between the two visit intervention and the control group., Conclusions: These results are consistent with the aim of the intervention, and they support existing evidence demonstrating that relatively short psychoeducational interventions can help family caregivers who are supporting a dying relative. The sustained benefit during the bereavement period may also have positive resource implications, which should be the subject of future inquiry., (© 2014 The Authors. Psycho-Oncology published by John Wiley & Sons Ltd.)

Background: Palliative care is expected to incorporate comprehensive support for family caregivers given that many caregivers suffer psychological morbidity. However, systematically implemented evidence-based psychological support initiatives are lacking., Aim: The objective of this study was to prepare caregivers for the role of supporting a patient with advanced cancer receiving home-based palliative care by offering a one-to-one psycho-educational intervention. We hypothesised that primary family caregivers who participated in the intervention would report decreased psychological distress (primary outcome), fewer unmet needs and increased levels of perceived preparedness, competence and positive emotions., Methods: A three-arm randomised controlled trial was conducted comparing two versions of the intervention (one face-to-face visit versus two visits) plus standard care to a control group (standard care) across four sites in Australia., Results: A total of 298 participants were recruited; 148 were in the Control condition, 57 in Intervention 1 (one visit) and 93 in Intervention 2 (two visits). Relative to participants in the control group; the psychological well-being of participants in the intervention condition was improved by a small amount but non-significantly. No significant reduction in unmet needs or improvements in positive aspects of caregiving amongst the intervention group were identified. However, the intervention demonstrated significant improvements in participants' levels of preparedness and competence for Intervention 2., Conclusion/implications: This research adds to accumulating body of evidence demonstrating that relatively short psycho-educational interventions can enable family caregivers to feel more prepared and competent in the role of supporting a dying relative. Further investigation is required to determine the longer term outcomes of such interventions., (Copyright © 2013 John Wiley & Sons, Ltd.)

Background: Many family caregivers of palliative care patients experience poor health and have other unmet needs, requiring health professionals' support. However, there are few evidence-based supportive interventions to address these issues., Purpose: The purpose of this project was to undertake preliminary testing of a psychoeducational group education programme, delivered in an in-patient setting, designed to prepare family caregivers for the role of supporting a relative currently receiving hospital-based palliative care., Methods: A pilot phase was conducted to develop the intervention and explore its utility. Thereafter the single session intervention was delivered in five palliative care units in three states of Australia and its effectiveness was examined using a pre-post design. Outcome variables included caregiver preparedness, competence and unmet needs. Psychological wellbeing was measured in order to determine if there were any deleterious psychological outcomes., Results: One hundred and twenty-six participants completed Time 1 data and 107 (84.9%) completed Time 2 data (post-intervention). There were statistically significant improvements in caregivers' sense of preparedness (p=<.001; effect size (ES) 0.43) and a significant reduction in unmet caregiver needs (p=014; ES 0.22). There was no significant effect on psychological wellbeing and the improvement on competence fell short of statistical significance., Conclusions: This study reinforces the notion that psychoeducational interventions for this population can potentially be applicable, acceptable and effective. However, the number of participants who were recruited and attended each session was fewer than anticipated, resulting in methodological implications. It is recommended that the intervention undergo further empirical inquiry, such as via a controlled trial.

Background: Support for family caregivers, including bereavement follow-up, is a core function of palliative care. Many caregivers acknowledge positive aspects associated with the role; however a considerable proportion will experience poor psychological, social, financial, spiritual, and physical well-being and some will suffer from complicated grief. Many family caregivers have unmet needs and would like more information, preparation, and support to assist them in the caregiving role. There is a shortage of evidence-based strategies to guide health professionals in providing optimal support while the caregiver is providing care and after the patient's death., Purpose: To develop clinical practice guidelines for the psychosocial and bereavement support of family caregivers of palliative care patients., Methods: (1) Literature review; (2) focus groups and structured interviews with key stakeholders within Australia; (3) national and international expert opinion to further develop and refine the guidelines using a modified Delphi process; and (4) endorsement of the guidelines from key palliative care, caregiver, and bereavement organizations (national and international)., Results: The guidelines were developed for multidisciplinary health care professionals and clinical services commonly involved in caring for adult patients receiving palliative care in a variety of care sites throughout Australia. These consensus-based guidelines have been endorsed key Australian and international organizations., Conclusions: The guidelines may prove valuable for the international palliative care community and for generalist health care providers who occasionally care for palliative care patients. Research is recommended to explore the uptake, implementation, and effectiveness of the guidelines.

Background and Purpose: Reviews of the literature have consistently highlighted significant gaps with regard to research associated with family caregivers within the context of palliative care. We sought to determine a priority driven research agenda for this field of inquiry., Methods: A Web-based survey was sent to 80 people who had previously expressed interest in, or were members of The International Palliative Care Family Carer Research Collaboration (http://centreforpallcare.org/index.php/research/ipcfcrc/)., Results: Fifty-five participants completed the survey (response rate, 70%) from 12 countries. Priority research areas included: intervention development and testing; underresearched caregiver groups; access to services; unmet needs; bereavement; experience and implications of the caregiver role; and development of assessment tools. Qualitative responses complemented these data and also acknowledged the importance of collaboration and development of a critical mass of researchers focusing in this area in order to progress knowledge., Conclusions: These results reinforce the findings of systematic reviews that have demonstrated a need for the evolution of intervention development focused on improving family caregiver support. However, there are other key areas that also warrant comprehensive attention, including marginalized family caregivers and strategies to assist health professionals to identify family caregivers who have significant psychosocial issues.

The High-Activity Arthroplasty Score (HAAS) was specifically developed to assess subtle variations in functional ability after lower limb arthroplasty with particular regard to highly functioning individuals. The score was a 4-item self-assessment measure covering the 4 domains of walking, running, stair climbing, and general activities, with a possible score ranging from 0 to 18 points. The score was validated in 22 patients (total hip arthroplasty [THA], n = 11; total knee arthroplasty [TKA], n = 11) by comparison with the Oxford, Knee Society, Harris Hip, and Short WOMAC scores. The HAAS was then administered to 152 high-functioning arthroplasty patients (THA, n = 99; TKA, n = 53), all younger than 66 years. The HAAS produced a much wider range of scores, allowing greater differentiation of level of function between patients in assessing performance after TKA or THA., (2010 Elsevier Inc. All rights reserved.)

Rewards derived from leading a cancer support group are poorly understood yet may be crucial to offset the challenges and difficulties of this role. This study sought to obtain the views of a representative sample of Australian cancer support group leaders (CSGLs) concerning the perceived rewards and challenges of their role. All CSGLs identified by the state-based Cancer Councils were invited to participate by postal questionnaire. Qualitative methods were used to analyze responses to open-ended questions concerning rewards and challenges. A total of 300 CSGLs returned the questionnaire (response rate = 66%) with 272 providing qualitative comments. Four parallel themes emerged from the qualitative analysis: (i) Personal, (ii) Relationship, (iii) Group, and (iv) Community rewards and challenges. These were integrated into a model depicting key positive and negative aspects of the CSGL's role, to provide direction for future training and ongoing support of CSGLs.

Aims: To establish whether tolerance to the QT effect could ensue during maintenance treatment with rac-sotalol., Methods: The effect of rac-sotalol on QT interval duration was studied in 10 patients after single oral administration (160 mg) and after 6-day multiple oral dosing (80 mg two or three times daily). In order to separate the pure Class III effect from the bradycardia-related QT prolongation, heart rate/QT relationship was preliminarly assessed in each patient after the administration of a pure beta-adrenoceptor blocker (propranolol, 80 mg orally). Repolarization changes were quantified as percent difference between the measured QT and the expected QT on the basis of the individual heart rate/QT relationship., Results: In all patients QT interval prolongation was linearly correlated with rac-sotalol log plasma concentration. The maximal QT prolongation and peak plasma concentration were not significantly different following acute and chronic administrations (QT effect: +18.1+/-6.3% vs +14.2+/-3.3%; peak concentration: 1.64+/-0.49 mg l(-1) vs 1.83+/-0.66 mg l(-1)). Line slopes were also unchanged following chronic treatment (21.8+/-8.9 vs 21.1+/-9.2). In four cases a significant rightward shift of the line occurred during repeated administrations, consistent with the appearance of pharmacodynamic tolerance. The inconstancy of this change in responsiveness may either be ascribed to a genetically determined individual susceptibility or to a variable interplay between Class III effect, gradual QT prolongation due to long-term beta-adrenoceptor blockade and tolerance development., Conclusions: During maintenance treatment with rac-solatol, partial loss of repolarization effects occurred in some patients suggesting pharmacological tolerance.