43 research outputs found

    Phase structure of a two-fluid bosonic system

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    The phase diagram of a two-fluid bosonic system is investigated. The proton-neutron interacting boson model (IBM-2) possesses a rich phase structure involving three control parameters and multiple order parameters. The surfaces of quantum phase transition between spherical, axially-symmetric deformed, and SU*(3) triaxial phases are determined, and the evolution of classical equilibrium properties across these transitions is investigated. Spectroscopic observables are considered in relation to the phase diagram.Comment: LaTeX (elsart), 46 pages, as published in Ann. Phys. (N.Y.

    A detailed description of the phenotypic spectrum of North Sea Progressive Myoclonus Epilepsy in a large cohort of seventeen patients

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    Introduction: In 2011, a homozygous mutation in GOSR2 (c.430G > T; p. Gly144Trp) was reported as a novel cause of Progressive Myoclonus Epilepsy (PME) with early-onset ataxia. Interestingly, the ancestors of patients originate from countries bound to the North Sea, hence the condition was termed North Sea PME (NSPME). Until now, only 20 patients have been reported in literature. Here, we provide a detailed description of clinical and neurophysiological data of seventeen patients. Methods: We collected clinical and neurophysiological data from the medical records of seventeen NSPME patients (5–46 years). In addition, we conducted an interview focused on factors influencing myoclonus severity. Results: The core clinical features of NSPME are early-onset ataxia, myoclonus and seizures, with additionally areflexia and scoliosis. Factors such as fever, illness, heat, emotions, stress, noise and light (flashes) all exacerbated myoclonic jerks. Epilepsy severity ranged from the absence of or incidental clinical seizures to frequent daily seizures and status epilepticus. Some patients made use of a wheelchair during their first decade, whereas others still walked independently during their third decade. Neurophysiological features suggesting neuromuscular involvement in NSPME were variable, with findings ranging from indicative of sensory neuronopathy and anterior horn cell involvement to an isolated absent H-reflex. Conclusion: Although the sequence of symptoms is rather homogeneous, the severity of symptoms and rate of progression varied considerably among individual patients. Common triggers for myoclonus can be identified and myoclonus is difficult to treat; to what extent neuromuscular involvement contributes to the phenotype remains

    A screening tool to quickly identify movement disorders in patients with inborn errors of metabolism

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    BackgroundMovement disorders are frequent in patients with inborn errors of metabolism (IEMs) but poorly recognized, particularly by nonmovement disorder specialists. We propose an easy-to-use clinical screening tool to help recognize movement disorders.ObjectiveThe aim is to develop a user-friendly rapid screening tool for nonmovement disorder specialists to detect moderate and severe movement disorders in patients aged ≥4 years with IEMs.MethodsVideos of 55 patients with different IEMs were scored by experienced movement disorder specialists (n = 12). Inter-rater agreements were determined on the presence and subtype of the movement disorder. Based on ranking and consensus, items were chosen to be incorporated into the screening tool.ResultsA movement disorder was rated as present in 80% of the patients, with a moderate inter-rater agreement (κ =0.420, P P ConclusionsWe designed a screening tool to recognize movement disorders in patients with IEMs. We propose that this screening tool can contribute to select patients who should be referred to a movement disorder specialist for further evaluation and, if necessary, treatment of the movement disorder. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.Neurological Motor Disorder

    International Lower Limb Collaborative (INTELLECT) study : a multicentre, international retrospective audit of lower extremity open fractures

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    Effectiveness of a tailored return to work program for cancer survivors with job loss: results of a randomized controlled trial

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    Contains fulltext : 172426.pdf (publisher's version ) (Open Access)BACKGROUND: Up to 53% of cancer survivors (CSs) experiences job loss during or after treatment. To support CSs with job loss in the Netherlands, a tailored return to work (RTW) program was developed. The objective of this study was to assess the effectiveness of the program on duration until sustainable RTW in CSs with job loss. MATERIAL AND METHODS: This study employed a two-armed (intervention/control) randomized controlled design with one-year follow-up. The primary outcome measure was duration until sustainable RTW. The secondary outcome measures were: rate of RTW, fatigue, quality of life, and participation in society. Descriptive analyses, Kaplan-Meier estimators and Cox regression analyses were conducted. RESULTS: Participants (N = 171) had a mean age of 48.4 years (SD = 8.6). The majority was female (69%) and breast cancer survivor (40%). The crude hazard ratio (HR) for duration until sustainable RTW was 0.86 (95% CI 0.46-1.62; p = 0.642). In the adjusted model, the intervention group had a slight, but statistically non-significant, improvement in duration until sustainable RTW compared to the control group (HR 1.16; 95% CI 0.59-2.31; p = 0.663). The program did not have any significant effects on secondary outcome measures. CONCLUSION: As the tailored RTW program did not demonstrate a statistically significant effect on duration until sustainable RTW in CSs with job loss, implementation of the program in its current form is not recommended

    Preoperative functional status is not associated with postoperative surgical complications in low risk patients undergoing esophagectomy

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    Preoperative functional status is a risk factor for developing postoperative complications (POC) in major abdominal and thoracic surgery, but this has hardly been evaluated in esophageal cancer patients undergoing esophagectomy. The aim of this prospective cohort study was to determine if preoperative functional status in esophageal cancer patients is associated with POC. From March 2012 to October 2014, esophageal cancer patients scheduled for esophagectomy at the outpatient clinic of a large tertiary referral center were eligible for the study. We measured inspiratory muscle strength, hand grip strength, physical activities, and health related quality of life as indicators of functional status one day before surgery. POC were scored according to the Clavien-Dindo Classification. We used univariate and multivariate backward regression analysis to determine the association between functional status and POC. We included 94 patients in the study and esophagectomy was performed in 90 patients from which 55 developed POC (61.1%). After multivariate analysis, none of the indicators of preoperative functional status were independently associated with POC (inspiratory muscle strength [OR 1.00; P = 0.779], hand grip strength [OR 0.99; P = 0.250], physical activities [OR 1.00; P = 0.174], and health related quality of life [OR 1.02; P = 0.222]). We concluded that preoperative functional status in our study cohort is not associated with POC after esophagectomy
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