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Introduction Idiopathic inflammatory myopathies (IIMs) excluding inclusion body myositis (IBM) are a group of heterogeneous autoimmune disorders characterised by subacute-onset and progressive proximal muscle weakness, which are frequently part of a multisystem autoimmune disorder. Reaching the diagnosis can be challenging, and no gold standard for the diagnosis of IIM exists. Diagnostic modalities include serum creatine kinase activity, muscle imaging (MRI or ultrasound (US)), electromyography (EMG), myositis autoantibody testing and muscle biopsy. Several diagnostic criteria have been developed for IIMs, varying in reported sensitivity and specificity.Hypothesis We hypothesise that an evidence-based diagnostic strategy, using fewer and preferably the least invasive diagnostic modalities, can achieve the accuracy of a complete panel of diagnostic tests, including MRI, US, EMG, myositis-specific autoantibody testing and muscle biopsy.Methods and analysis The OptimizAtion of Diagnostic Accuracy in idioPathic inflammaTory myopathies study is a prospective diagnostic accuracy study with an over-complete study design. 100 patients suspected of an IIM excluding IBM will be included. A reference diagnosis will be assigned by an expert panel using all clinical information and all results of all ancillary tests available, including 6 months of follow-up. Several predefined diagnostic strategies will be compared against the reference diagnosis to find the optimal diagnostic strategy.Ethics and dissemination Ethical approval was obtained from the medical ethics committee of the Academic Medical Centre, University of Amsterdam, The Netherlands (2019-814). The results will be distributed through conference presentations and peer-reviewed publications.Trial registration number Netherlands trial register; NL8764.

Objectives To develop and validate a protocol for MRI assessment of the distal radial and ulnar periphyseal area in gymnasts and non-gymnasts.Methods Twenty-four gymnasts with wrist pain, 18 asymptomatic gymnasts and 24 non-gymnastic controls (33 girls) underwent MRI of the wrist on a 3T scanner. Sequences included coronal proton density-weighted images with and without fat saturation, and three-dimensional water-selective cartilage scan and T2 Dixon series. Skeletal age was determined using hand radiographs. Three experienced musculoskeletal radiologists established a checklist of possible (peri)physeal abnormalities based on literature and clinical experience. Five other musculoskeletal radiologists and residents evaluated 30 MRI scans (10 from each group) using this checklist and reliability was determined using the intraclass correlation coefficient (ICC) and Fleiss’ kappa. A final evaluation protocol was established containing only items with fair to excellent reliability.Results Twenty-seven items were assessed for reliability. Intra-rater and inter-rater agreement was good to excellent (respective ICCs 0.60–0.91 and 0.60–0.78) for four epiphyseal bone marrow oedema-related items, physeal signal intensity, metaphyseal junction and depth of metaphyseal intrusions. For physeal thickness, thickness compared with proximal physis of first metacarpal, metaphyseal intrusions, physeal connection of intrusions and metaphyseal bone marrow signal intensity, intra-rater agreement was fair to excellent (ICC/kappa 0.55–0.85) and inter-rater agreement was fair (ICC/kappa 0.41–0.59). Twelve items were included in the final protocol.Conclusion The Amsterdam MRI assessment of the Physis protocol facilitates patient-friendly and reliable assessment of the (peri)physeal area in the radius and ulna.

Objectives: Determine obstetrical and neonatal outcomes in neonates with major CHD delivered at a level IV neonatal intensive care units (NICU) center lacking onsite pediatric cardiac surgery., Methods: A 10- year retrospective review of all neonates admitted to our level IV NICU, with CHD between January 1st, 2011 and December 31st, 2021. Births and NICU charts were cross queried with those from our perinatal center which include pediatric cardiology records. Terminations and stillbirths were excluded., Results: A total of 285 neonates with major CHD and 78 with minor defects were included. In the major CHD group, 82.8 % had an isolated cardiac anomaly and 17.2 % had an extracardiac anomaly. Type of extracardiac anomaly had no impact on neonatal survival. Prenatal diagnosis of aneuploidy did not impact survival in major CHD. Truncus arteriosus had the highest NICU mortality at 34.0 % followed by hypoplastic left heart syndrome (HLHS) at 31.6 %. Double outlet right ventricle with transposition of the great vessels and interrupted aortic arch (both types) had a 25 % mortality. Neonates with truncus arteriosus and total anomalous pulmonary venous returns were likely to have 5-min Apgar score<7. Transfer rate of neonates with major CHD for cardiac surgery was 58.6 %. Of those 81.5 % were discharged home, 14.3 % expired before discharge, and 1 % were transferred elsewhere post-operatively for higher level of care., Conclusions: Neonates with major CHD can deliver safely at a level IV NICU lacking onsite pediatric cardiac surgery. Our neonatal mortality was high for HLHS and truncus arteriosus, however comparable to other centers with proximate pediatric cardiac surgery., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

Introduction: Double outlet left ventricle (DOLV) is a rare congenital heart anomaly, and cases of DOLV with an intact ventricular septum are uncommon. To date, only four such cases have been reported in the medical literature., Case Presentation: This report presents a case of prenatally diagnosed DOLV. A fetal echocardiogram at 21 weeks of gestation demonstrated both great arteries, aorta and pulmonary artery, arising from the left ventricle with severely dysplastic tricuspid valve and severe hypoplasia of the right ventricle. Subsequent echocardiograms demonstrated no ventricular septal defect. The patient required balloon atrial septostomy in the first week of life, underwent pulmonary artery banding at 5 weeks of life, and is currently status post-bidirectional Glenn, and is awaiting final Fontan palliation., Conclusion: Prenatal diagnosis aided in predicting and guiding postnatal management., (© 2024 S. Karger AG, Basel.)

Coronary arteries arising from the pulmonary artery have an incidence of 0.002% in the general population. We present a 29-year-old woman who presented to our hospital with acute decompensated heart failure and atrial fibrillation with a rapid ventricular rate. She underwent a cardiac catheterization to rule out ischemic disease, which revealed retrograde contrast flow through the left coronary artery from the right coronary artery. A coronary computed tomography (CT) angiogram was pursued which showed the presence of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA). For the management of her atrial fibrillation, she was electrically cardioverted. She was discharged on guideline-directed medical therapy for her heart failure, with a cardiac surgery referral for the surgical fixation of her ALCAPA.

Objective: Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS)., Background: Long-term data following BPV is limited to small, single center studies., Methods: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis., Results: Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention., Conclusion: Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV., (© 2019 Wiley Periodicals, Inc.)

This paper considers a novel dynamical behaviour of two microbial populations, competing in a chemostat over a single substrate, that is only possible through the use of population balance equations (PBEs). PBEs are partial integrodifferential equations that represent a distribution of cells according to some internal state, mass in our case. Using these equations, realistic parameter values and the assumption that one population can deploy an emergency mechanism, where it can change the mean mass of division and hence divide faster, we arrive at two different steady states, one oscillatory and one non-oscillatory both of which seem to be stable. A steady state of either form is normally either unstable or only attainable through external control (cycling the dilution rate). In our case no external control is used. Finally, in the oscillatory case we attempt to explain how oscillations appear in the biomass without any explicit dependence on the division rate (the function that oscillates) through the approximation of fractional moments as a combination of integer moments. That allows an implicit dependence of the biomass on the number of cells which in turn is directly dependent on the division rate function., Competing Interests: The authors have declared that no competing interests exist.

Objective To examine the job accommodation and benefit needs of young adults with disabilities as they transition into employment, and their perceived barriers to meeting support needs. Methods An online survey was conducted of 155 Canadian young adults with disabilities (mean age = 25.8 years). Respondents were either employed or seeking employment, and were asked about their need for health benefits, and soft (e.g., flexible scheduling) and hard accommodations (e.g., ergonomic interventions), and perceived accommodation barriers. Disability characteristics (e.g., disability type), demographic details and work context information were collected. Multivariable logistic analyses were conducted to examine the factors associated with a greater need for health benefits and hard and soft accommodations. Result Participants reported having a physical (79%), psychological (79%) or cognitive/learning disability (77%); 68% had > 1 disability. Over half (55%) were employed. Health benefits and soft accommodations were most needed by participants. Also, an average of six perceived accommodation barriers were indicated; difficulty with disability disclosure was most frequently reported. More perceived accommodation barriers were associated with a greater need for health benefits (OR 1.17, 95% CI 1.04-1.31) and soft accommodations (OR 1.13, 95% CI 1.01-1.27). A psychological disability was a associated with a greater need for health benefits (OR 2.91, 95% CI 1.09-7.43) and soft accommodations (OR 3.83, 95% CI 1.41-10.42). Discussion Employers can support the employment of young adults with disabilities through provision of extended health benefits and soft accommodations. Addressing accommodation barriers could minimize unmet workplace need, and improve employment outcomes for young adults with disabilities as they begin their career and across the life course.

Background: Agent-based models provide a formidable tool for exploring complex and emergent behaviour of biological systems as well as accurate results but with the drawback of needing a lot of computational power and time for subsequent analysis. On the other hand, equation-based models can more easily be used for complex analysis in a much shorter timescale., Methods & Objective: This paper formulates an ordinary differential equations and stochastic differential equations model to capture the behaviour of an existing agent-based model of tumour cell reprogramming and applies it to optimization of possible treatment as well as dosage sensitivity analysis., Results: For certain values of the parameter space a close match between the equation-based and agent-based models is achieved. The need for division of labour between the two approaches is explored., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.)

Circulatory arrest (CA) is traditionally utilized during the repair of total anomalous pulmonary venous return (TAPVR). Since 2005, we have exclusively repaired all types of TAPVR using continuous cardiopulmonary bypass. We present our technique using continuous cardiopulmonary bypass throughout the duration of the repair, by temporarily occluding the vertical vein and placing a pump sucker within the pulmonary venous confluence. This technique has been used on 29 consecutive patients and resulted in limited morbidity and absence of pulmonary vein stenosis from most recent follow-up., (© The Author(s) 2016.)

Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) can result in left-ventricular distension and the development of pulmonary edema. We present the case of a 13-year-old girl who presented with cardiogenic shock. VA-ECMO was initiated, but after 6 days, severe left-ventricular distension resulted in decreased VA-ECMO flows. With guidance by bedside transesophageal echocardiography, a percutaneous atrial transseptal cannula was placed and connected to the venous circuit, thus decompressing the left ventricle. The patient improved, was weaned from VA-ECMO 5 days later, and was discharged from the hospital. Bedside transseptal catheter insertion is an effective method of left-ventricular decompression.

Methylamphetamine, ephedrine, and pseudoephedrine were derivatized using trifluoroacetic anhydride and enantiomers of each were analyzed using gas chromatography coupled to mass spectrometry (GC/MS) fitted with a γ-cyclodextrin (Chiraldex™ G-PN) chiral column. A temperature-programmed method was developed and optimized and the results compared with those obtained using a previously published isothermal GC method applied to GC/MS analysis. Trifluoroacetylated 3-(trifluoromethyl)phenethylamine hydrochloride was used as an internal standard, and mass fragmentation patterns are proposed for all derivatives analyzed. Qualitative validation of the optimized chromatographic conditions was completed in accordance with the guidelines published by the United Nations Office on Drugs and Crime (UNODC). Under conditions of repeatability and reproducibility, the method gave relative retention times with a relative standard deviation of less than 0.02% for all six analytes of interest. This surpasses the UNODC's acceptance criteria of 2% for validation of qualitative precision. Ephedrine and pseudoephedrine are common precursors in the clandestine manufacture of methylamphetamine. Seizures of illicit methylamphetamine therefore often contain mixtures of these optically active compounds. The simultaneous enantioseparation of these compounds to produce a profile would provide valuable information to law enforcement agencies regarding the provenance of a methylamphetamine seizure., (Copyright © 2011 Wiley-Liss, Inc.)

Fluoroquinolone (FLQ) drugs are a potent family of antibiotics used to treat infections including ocular infections. To determine if these antibiotics may be phototoxic to the eye, we exposed human lens epithelial cells to 0.125-1 mm FLQs (ciprofloxacin [Cipro], lomefloxacin [Lome], norfloxacin [Nor] and ofloxacin [Ofl]), the precursor quinolone nalidixic acid (Nalid) and UVA radiation (2.5 J cm(-2)). Based on fluorescence confocal microscopy, FLQs are diffused throughout the cytoplasm and preferentially located in the lysosomes of lens epithelial cells. Neither FLQ exposure alone nor UVA exposure alone reduced cell viability. However, with exposure to UVA radiation the FLQs studied (Cipro, Nor, Lome and Ofl) induced a phototoxic reaction that included necrosis, apoptosis, loss of cell viability as measured by MTS, and membrane damage as determined by the lactate dehydrogenase assay. Both Nalid and all FLQs studied (Cipro, Nor, Lome and Ofl) photopolymerized the lens protein alpha-crystallin. Phototoxic damage to lens epithelial cells and/or alpha-crystallin will lead to a loss of transparency of the human lens. However, if precautions are taken to filter all UV radiation from the eye while taking these antibiotics, eye damage may be prevented.

WHAT IS ALREADY KNOWN IN THIS AREA • Research suggests that traditional GP. SHO education has little or no contact with general practice during the hospital years, something viewed negatively by GP trainees. It also Suggests that the quality of training is variable, due, among other things, to heavy service commitment, and the lack of structured educational input and support. WHAT THIS WORK ADDS • This study reports a pilot of an innovative training programme that sought to address the problems previously identified with traditional GP SHO training. The posts were found to be popular and successful, offering integrated training between primary and secondary care' during the SHO years. SUGGESTIONS FOR FUTURE RESEARCH • Innovative training posts offer a much-needed way to extend the menu of training opportunities for general. practice, and further work is needed 18 explore-how they can be developed forther, as well as to continue to monitor the quality and development of the trust-attached posts now in existence in Severn and Wessex.

Study Design: Patients with symptomatic lumbar spinal stenosis underwent magnetic resonance imaging to study changes in the lumbar spine in various postures before and after implantation of the X STOP Interspinous Process Distraction Device (St. Francis Medical Technologies, Concord, CA)., Objective: To visualize the effect of the device in vivo., Summary of Background Data: Previous studies have shown vertebral canal and exit foraminal area to reduce from flexion to extension. Recently, reports on improved kinematics in vitro at the implantation level of the X STOP device have also been published., Methods: Using positional magnetic resonance imaging, patients were scanned before and 6 months after surgery. Images were taken with the patient in sitting flexed, extended, neutral, and standing positions. The total range of motion of the lumbar spine and individual segments were measured, along with changes in disc height, areas of the exit foramens, and dural sac., Results: In 12 patients with 17 distracted levels, the area of the dural sac at these levels increased from 77.8 to 93.4 mm after surgery in the standing position (P = 0.006), with increase in the exit foramens, but no change in lumbar posture., Conclusions: This study shows that the X STOP device increases the cross-sectional area of the dural sac and exit foramens without causing changes in posture.

Background: Previous studies have shown Graf ligament stabilisation procedure to give mixed results in the short to medium term. The aim of this study was to correlate the pre-operative state of the disc, multifidus muscles, age of the patient, levels operated and the clinical outcome after a mean follow-up of 47 months., Methods: Graf ligament stabilisation procedure was carried out in 38 patients between 1996 and 1999. Their post-operative status was assessed using MacNab criteria. The post-operative follow-up was by postal questionnaires and review of the clinical notes. Disc morphology and multifidus muscle wasting was graded blindly and independently. The intra- and interobserver reliability was measured with kappa score and classified using the kappa classification of Landis and Koch. Correlation was measured with the help of Spearman correlation coefficient., Results: Thirty-eight patients (100%) returned the questionnaires. Mean follow-up time was 47.55 months. Fifty-nine levels were operated on. Mean age was 39.68 years. The overall re-operation rate was 15.8%. The intra- and interobserver reliability was graded as good to substantial. Twenty-two patients (57.89%) were satisfied with the procedure. There was no statistically significant correlation between disc morphology, multifidus muscle wasting, sex, age, number of levels operated, the levels operated, and the satisfaction rate., Conclusions: The indications of Graf ligament stabilisation procedure are not clear. Further work is necessary to clearly identify the indication for the procedure.

A pilot study of production of respirable and inhalable dusts from sawing and sanding medium density fibreboard (MDF) and softwood in a typical cabinet-making workshop produced high but variable exposure levels at the bench and operator position. Exposure levels for the total inhalable fraction (approximately <100 microm) were 6.9-91 mg m(-3) for MDF and 2.5-45 mg m(-3) for softwood. For the respirable fraction (< 10 microm) levels were 0.4-13 mg m(-3) for MDF and 0.4-2.9 mg m(-3) for softwood. These results show significant dust loading is produced in the coarser fraction and that the material used has a significant impact on levels produced. It suggests that fuller evaluation of operator influence of fine dust production is needed and may question the common application of a single inhalable exposure standard for wood dust to all wood working scenarios.

Objective: To compare the frequency and outcome of upper gastrointestinal haemorrhage (UGH) patients who had undergone cardiac surgery with a control group of vascular surgery patients., Patients: Patients who had undergone cardiac or vascular surgery from January 1999 to December 2000 were identified from departmental records. The inclusion criteria used were haematemesis and/or melaena in the post-operative period., Results: Only 20 of the 2274 (0.9%) cardiac operations were complicated by UGH compared to eight of 708 (1.1%) vascular operations. Among those with UGH, 90% of the cardiac and 43% of the vascular patients were taking aspirin, warfarin or both. The mean interval between surgery and the UGH was 9.6 days (range 1-30) for the cardiac and 6 days (range 0-15) for the vascular patients. Duodenal and gastric ulcers were the most common cause of UGH (60%) in the cardiac group. Despite endoscopic intervention, more than one third of ulcer associated haemorrhages required surgical over-sewing, but none of the patients who had surgery died. The overall mortality on the cardiac surgery patients who experienced UGH was 15%, significantly higher than the 2.3% for the whole cardiac surgery group during the study period (P = 0.00075, OR = 8, 95% confidence interval 2.3-28). However, even this mortality is less than that of general inpatients who suffer UGH (33%)., Conclusions: Cardiac and vascular surgical patients have similar low post-operative rate of UGH. Post-operative UGH is associated with increased mortality after primary surgery. Early surgical intervention appears to be life saving in those patients who are too ill to compensate for the haemodynamic disturbance of untreated UGH.

The development of pulmonary arteriovenous malformations after cavopulmonary bypass in patients with congenital heart disease is well documented. We report successful management of pulmonary arteriovenous malformations after cavopulmonary bypass in a patient with an interrupted inferior vena cava (IVC) and multiple hepatic veins utilizing an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC. This technique, performed without circulatory arrest or an atriotomy, may limit morbidity associated with intracardiac procedures in patients with single ventricle morphology. Furthermore, this case suggests an alternative technique for completion Fontan in patients with an interrupted IVC and multiple hepatic venous drainage.

This paper reports the reliability in assessments of a series of portfolios assembled by a cohort of participants attending a course for prospective general practice trainers. Initial individual assessments are compared with open discussion between random pairs of assessors to produce paired composite scores, and analysed using kappa statistics. Overall reliability of a global pass/refer judgement improved from a kappa of 0.26 (fair) using individual assessment, to 0.5 (moderate) with paired discussants.

The long-term success of arterial bypass grafting with autologous saphenous veins is limited by neointimal hyperplasia (NIH), which seemingly develops preferentially at sites where hydrodynamic wall shear is low. Placement of a loose-fitting, porous stent around end-to-end, or end-to-side, autologous saphenous vein grafts on the porcine common carotid artery has been found significantly to reduce NIH, but the mechanism is unclear. In a preliminary study, we implanted autologous saphenous vein grafts bilaterally on the common carotid arteries of pigs, placing a stent around one graft and leaving the contralateral graft unstented. At sacrifice 1 month post implantation, the grafts were pressure fixed in situ and resin casts were made. Unstented graft geometry was highly irregular, with non-uniform dilatation, substantial axial lengthening, curvature, kinking, and possible long-pitch helical distortion. In contrast, stented grafts showed no major dilatation, lengthening or curvature, but there was commonly fine corrugation, occasional slight kinking or narrowing of segments, and possible long-pitch helical distortion. Axial growth of grafts against effectively tethered anastomoses could account for these changes. CFD studies are planned, using 3D MR reconstructions, on the effects of graft geometry on the flow. Abnormality of the flow could favour the development of vascular pathology, including NIH.

It is the aim of this to discuss the surgical aspects of a culturally determined, yet essentially preventible disease; to review the experience derived from 72 patients, in whom it was confirmed at operatin; and to draw conclusins which may assist in the future management of this problem.