bluuebunny/crossref_metadata_2025_split huggingface dataset:
083 Response to immunosuppressive therapy in cerebral amyloid angiopathy-related inflammation
Authors: Tilling Jasmin , Trewin Benjamin , Levy Stanley 
Published: May 2018

IntroductionCerebral amyloid angiopathy (CAA) is a common age-related condition characterised by amyloid beta-peptide deposition affecting the medium sized cortical and leptomeningeal arteries, arterioles and capillaries. CAA-related Inflammation (CAA-I) is an increasingly recognised variant of CAA, which is thought to be due to perivascular auto-inflammation in response to amyloid deposition. We describe the clinical course of two cases of probable CAA-I.CasesA 71 year old man presented with new-onset seizures, headaches and subacute cognitive decline. MRI of the brain demonstrated confluent subcortical T2 white matter hyperintensities and cerebral oedema, with predominantly superimposed widespread cortico-subcortical micro-haemorrhages, in keeping with the diagnosis of CAA-I. A course of immunosuppresive therapy was commenced with five days of intravenous methylprednisolone, resulting in marked radiological and clinical improvement within two weeks.A 76 year old female presented with subacute cognitive dysfunction and apraxia, and transient left-sided weakness. MRI scan of the brain initially demonstrated a right temporo-occipital infarct, leading to primary treatment for stroke, but subsequently evolved to reveal diffuse multi-lobar T2 white matter hyperintensities with leptomeningeal involvement. A provisional diagnosis of CAA-I was made and following a poor clinical response to a trial of corticosteroid therapy, treatment with intravenous cyclophosphamide was commenced.ConclusionThese cases emphasise the importance of CAA-I as part of the differential diagnosis in patients presenting with symptoms of subacute cognitive decline, seizures, headaches and focal neurological deficits, given the potential for dramatic improvement with readily accessible immunosuppressive therapies.
091 Poems syndrome treated with autologous stem cell transplant
Authors: Devlin Michael , Costello Craig , Hodges Georgina , Urkude Ravindra 
Published: May 2018

IntroductionAcquired demyelinating neuropathies comprise a diverse spectrum of individual diseases and pathophysiological processes. Differential diagnoses can be distinguished through assessment of region of involvement, time course, neurophysiology and ancillary testing. Where an atypical presentation of chronic inflammatory demyelinating polyradiculoneuropathy arises, further investigation and changes to management are required. We present a single case report from the Townsville Hospital.CaseA 45 year old man presented with two months of altered sensation in the distal lower extremities. Lower limb weakness developed, and three months after symptoms onset the patient had bilateral foot drop, and developed sensory disturbance in the upper limbs. Electrophysiological testing revealed severely reduced lower limb CMAPs with demyelinating range conduction velocity without conduction block; upper limb SNAPs were normal in amplitude with conduction velocity slowing. A lumbar puncture revealed elevated CSF protein 870 mg/L without raised white cells. A trace lambda IgG band of uncertain significance was detected. IVIG was commenced and symptoms initially stabilised. After four months of monthly IVIG, symptoms worsened and neurophysiology revealed further neurogenic changes. Skeletal survey and whole Spine MR STIR sequences did not reveal any bony lesions, and bone marrow biopsy revealed 5% plasmacytosis. The patient’s functional status deteriorated to full-time wheelchair use despite escalation of therapy. Sural nerve biopsy revealed axonal loss and demyelination without inflammation. A final diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes) syndrome was confirmed with vascular endothelial growth factor elevation, and the patient underwent an autologous stem cell transplant with significant improvement in symptoms and functional status by day 100.ConclusionPOEMS syndrome is a rare disorder and should be suspected in atypical cases of CIDP particularly when treatment resistance is present. Extensive investigation is often required to meet diagnostic criteria for POEMS.
092 Early-onset dementia with white matter changes
Authors: Trewin Benjamin P , Baskin Jono , Satgunaseelan Laveniya , Levy Stanley 
Published: May 2018

IntroductionWe report a rare case of early-onset dementia with leukoencephalopathy in which the diagnosis was unclear until autopsy. As previously seen in case reports of this condition, there were a number of working diagnoses made, prefixed with ‘atypical’, including cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and Huntington disease.1CaseA sixty-one-year old Caucasian female initially presented with 2 years of progressive chorea of her right upper limb, anxiety and memory difficulties. These symptoms steadily progressed over the next 5 years as she developed dementia and a progressive gait disturbance with pyramidal weakness. In the terminal stages of her illness, at sixty-six years of age, she had recurrent hospital admissions for pyrexia of unknown origin and finally refractory seizures and death. Her serum investigations were non-diagnostic; she was negative for both CADASIL and Huntington disease mutations. Her MRI demonstrated diffuse confluent white matter changes involving the corpus callosum and the vermis. She also had an abnormal electroencephalogram late in the course of her disease, with lateralised periodic discharges recorded during episodes of non-convulsive status epilepticus. Her autopsy revealed a leukoencephalopathy with pathognomonic histologic features of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP). Post-mortem genetic testing is currently underway.ConclusionOur case demonstrates the possibility of establishing an accurate diagnosis with pathology and genetic testing in cases of early-onset dementia with white matter changes. Recently elucidated entities offer diagnostic closure to patients, families and clinicians and can inform prognosis and genetic counselling. Furthermore, potential therapeutic options for leukodystrophies appear to be on the horizon.2 Our case report is the first to our knowledge to present with chorea and to exhibit vermian hyperintensity on T2-weighted MRI.References. Meyer-Ohlendorf M, Braczynski A, Al-Qaisi O, et al. Comprehensive diagnostics in a case of hereditary diffuse leukodystrophy with spheroids. BMC Neurology2015;15:103.. van der Knaap MS, Wolf NI, Heine VM. Leukodystrophies: Five new things. Neurol Clin Pract2016;6:506–514.
098 A case of myasthenia gravis lambert eaton overlap syndrome (MLOS) in seronegative myasthenia gravis
Authors: Saleem Sameer , Patel Ronak , Gawarikar Yash 
Published: May 2018

IntroductionMyasthenia gravis (MG) is an antibody-mediated autoimmune disease of neuromuscular transmission. 6 to 12 percent of MG are negative for acetyl choline receptor (AChR) and MuSK antibodies and are defined as seronegative MG. Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune condition with antibodies to presynaptic voltage-dependent calcium channelCaseA 39 year old male presented with blurred vision and right-sided ptosis. Initial examination showed a pupil-sparing left complete third nerve palsy. Demyelination and intracranial aneurysms were ruled out with gadolinium-enhanced MRI/MRA. Outpatient follow-up 2 weeks later showed new onset proximal muscle weakness of the upper limbs with fatigability and a complex ophthalmoplegia with almost complete paralysis of gaze. A repeat MRI with gadolinium and CSF analysis were normal. His AChR and MuSK antibodies were negative; however, voltage-dependent calcium channel antibodies, ANA, dsDNA, and SSA were positive. Initial nerve conduction tests were normal, but repeat NCS on two separate occasions showed decrement on repetitive stimulation in the right trapezius with no evidence of facilitation post exercise. CT chest, abdomen and pelvis was normal. He improved with pulsed steroids and was discharged on a tapering dose of oral steroids, pyridostigmine and regular IVIG infusions as a steroid-sparing agent.ConclusionLambert-Eaton myasthenic syndrome shares the same pathologic site and similar pathophysiology with MG but has a markedly different clinical and electro-physiological picture. There are reports of MG and LEMS overlap syndrome, however, they exhibit phenotypic characteristics of both LEMS and MG. Voltage-dependent calcium channel antibodies have not been described in patients with seronegative MG. Ours is potentially the first reported case of seronegative myasthenia with voltage-dependent calcium channel antibodies and only clinical and neurophysiological features of MG.
099 A case of possible Fahr’s disease
Authors: Saleem Sameer , Gawarikar Yash , Patel Ronak 
Published: May 2018

IntroductionIdiopathic basal ganglia calcification (IBGC) or Fahr’s disease is an autosomal dominant, rare progressive neurological disorder, of unknown aetiology. It is characterised by abnormal deposition of calcium in the basal ganglia and other brain regions. We present the case of a woman with Fahr’s disease.Case63 year old woman presented with rapid cognitive decline on resuming work after a long holiday. Her co-morbidities included type 1 diabetes, Crohn’s disease and hypertension. She was slow at work, had difficulty concentrating, tired and forgetful in paying important bills. On initial clinical examination, she was fidgety without any choreiform movements or parkinsonian features. Addenbrooke’s cognitive assessment scored 90/100 but lost points in fluency and visuospatial. MRI brain showed bilateral symmetrical blooming artefacts suggestive of calcifications involving basal ganglia, thalami, cerebellar hemispheres and occipital cortices and also extensive white matter disease. CT brain confirmed intracerebral calcification involving the same areas in MRI. She had an ANA of 1280, dsDNA of 40, antiphospholipid Abs with b2GP1 IgG of 20 U/mL and a normal lumbar puncture. During follow-up, she had developed choreiform movements of her trunk and legs, bilateral dysdiadochokinesia, and bradykinesia on repetitive fine finger movements. Her Montreal cognitive assessment score was 24/30.ConclusionFahr’s disease is a very rare condition with unknown prevalence. Typical age of presentation is between the 4th – 6th decades but early onset is reported. Symptoms include Parkinsonism, chorea, dystonia, cognitive impairment, seizures, headache or ataxia. The familial form of IBGC is genetically heterogeneous and both sporadic and familial forms are not associated with disorders of calcium or parathyroid hormone metabolism. Our case illustrates the high index of clinical suspicion necessary to diagnose rare conditions such as Fahr’s disease.
The effect of iodopovidone versus bleomycin in chemical pleurodesis
Authors: Bagheri Reza , Noori Marzieh , Rajayi Maryam , Attaran Davood , Mohammad Hashem Asna Ashari Amir , Mohammadzadeh Lari Shahrzad , Basiri Reza , Rezaeetalab Fariba , Afghani Reza , Salehi Maryam 
Published: May 2018

Background Malignant pleural effusion continues to be a common problem in patients with metastatic disease. This study was conducted to compare the efficacy and safety of bleomycin pleurodesis with povidone-iodine pleurodesis through a chest drain as palliative treatment for recurrent malignant pleural effusion. Methods Sixty cancer patients (36 males and 24 females) with recurrent malignant pleural effusion were enrolled in a prospective randomized trial. Thirty patients received povidone-iodine pleurodesis and 30 received bleomycin pleurodesis. Age, sex, side of the primary pathology, treatment outcome (recurrence and relapse time), and complications were analyzed. Results The mean age was 59.63 ± 7.68 years in the povidone-iodine group and 57.97 ± 9.27 years in the bleomycin group ( p = 0.452). The complications were identical in both groups: 2 (6.7%) patients had chest pain, 2 (6.7%) had fever, and one (3.3%) had hypotension. There was a good response to therapy in 20 (66.7%) patients in the bleomycin group and 25 (83.3%) in the povidone-iodine group ( p = 0.136). Conclusion The results of this study indicate that povidone-iodine should be considered as a selective chemical agent to perform pleurodesis in patients with recurrent malignant pleural effusion because it has the same effect but costs less than bleomycin.
A systematic review and meta-analysis of the association between vitamin A intake, serum vitamin A, and risk of liver cancer
Authors: Leelakanok Nattawut , D’Cunha Ronilda R. , Sutamtewagul Grerk , Schweizer Marin L. 
Published: May 2018

Background: Previous evidence supports that vitamin A decreases the risk of several types of cancer. However, the association between vitamin A and liver cancer is inconclusive. Aim: This systematic review and meta-analysis summarizes the existing literature, discussing the association between vitamin A intake, serum vitamin A, and liver cancer in adult populations. Methods: A systematic literature review was performed by searching the EMBASE, PubMed, Scopus and International Pharmaceutical Abstract databases using terms related to vitamin A (e.g. retinol, α-carotene, β-carotene, and β-cryptoxanthin) and hepatic cancer without applying any time restriction. A meta-analysis was performed using random effect models. Results: The meta-analysis of five studies showed no association between serum retinol and liver cancer (pooled risk ratio = 1.90 (0.40–9.02); n = 5 studies, I2 = 92%). In addition, the systematic review of studies from 1955 to July 2017 found studies that indicated no association between the intake and serum level of α-carotene ( n = 2) and β-cryptoxanthin ( n = 1) and the risk of liver cancer. Further, the associations between retinol intake ( n = 3), β-carotene intake ( n = 3), or serum β-carotene ( n = 3) and liver cancer were inconclusive. Conclusions: Current information on the association between vitamin A intake and liver cancer or serum vitamin A and liver cancer are limited. Most studies demonstrated no association between dietary vitamin A and the risk of liver cancer. However, the finding was based on a small number of studies with potential publication bias. Therefore, large observational studies should be conducted to confirm these associations.
The Impact of New-Product R&D on the Circular Flow
Authors: McClure James E. , Thomas David Chandler 
Published: May 2018

Closed-system circular flow models have been ubiquitous in economics for at least half a century. Because these models account for neither new-product R&D, nor the attendant leakages and injections, they obscure understanding of (a) the distinction between new-product and process R&D, (b) the circular flow leakages and injections attending new-product R&D, and (c) Schumpeterian “creative destruction” and “swarming.” To shed light upon these matters, we present an open-system circular flow model and a price theoretic explanation of the swarming overinvestment that characterizes business cycle downturns. JEL Classifications: B20, O31, E30
A comparative study of anterior cruciate ligament reconstruction with double, single, or selective bundle techniques
Authors: Chia Zi-Yang , Chee Jade N , Bin-Abd-Razak Hamid Rahmatullah , Lie Denny TT , Chang Paul CC 
Published: May 2018

Purpose: Reconstruction of the anterior cruciate ligament (ACL) is the most frequently performed reconstructive surgery in the knee. Biomechanical studies have shown that double bundle (DB) reconstruction is better than single bundle (SB) reconstruction with regard to rotational stability. It is postulated that resection of ACL fibres that remain in continuity may be counterproductive for the knee as these fibres have the capacity to produce collagen. In this study, we aimed to evaluate the efficacy among selective bundle, DB and SB ACL reconstructions over a 2-year post-operative follow-up period. Methods: A retrospective comparative study was conducted for comparison between selective bundle, DB and SB reconstructions. Between 2012 and 2014, 291 ACL reconstructions were performed. Of these, 68 patients had selective ACL reconstructions (group SLB), 147 had DB ACL reconstructions (group DB) and 76 had SB ACL reconstructions (group SB). Institutional Review Board approval was obtained, and all patients provided informed consent. Clinical results were assessed with the International Knee Documentation Committee (IKDC), Lysholm and Tegner scores. Stability was measured using Lachman, pivot shift and anterior drawer stress tests using the KT1000 at 30° of knee flexion. Results: There was no significant difference in ligament grade, function grade, IKDC grade, as well as Tegner and Lysholm means among all three groups after a 2-year follow-up period. Conclusion: Selective bundle reconstruction provides comparable results to DB and SB reconstruction techniques. It is a viable alternative for patients with partial tears.
Melodramatic Tactics for Survival in the Neoliberal Era: Excess and Justice in The Heirs and My Love from the Star
Authors: Yi We Jung 
Published: May 2018

AbstractThis article examines two 2013 TV miniseries, The Heirs and My Love from the Star, by relating their melodramatic aesthetics to the survivalist imperative under neoliberal governance. From the colonial sinp’a theater to Golden Age films, melodrama has operated as a popular mode of imagination that expresses inarticulate experiences generated over the course of Korea’s modernization. To extend and complicate existing scholarship on modern melodrama, this study approaches recent K-dramas’ melodramatic modes as both an affective response to and an everyday tactic for coping with failing economic democracy in contemporary Korea. In this light, the intensifying fantastic elements of the genre are deemed not so much anachronistic as tactical, as they are deployed to reclaim the justice and equality that are felt to be hopelessly disappearing in daily lives. As I look at their excessive aesthetics within the context of diminishing social mobility in the neoliberal era, my analysis of the two miniseries further notes the gendered structure of these melodramatic fantasies in which the survival of women, who have fewer privileges, is achieved through the reform of male elites.
The meaning of money laundering in business life and prevention of it
Authors: Türkössy Anikó 
Published: May 2018

Money laundering is the process whereby the proceeds of crime are transformed into ostensibly legitimate money or other assets. The actuality of the subject derives from the Select Committee on the Evaluation of Anti Money Laundering Measures aided by the Financial Action Task Force. Money obtained from certain crimes, such as extortion, drug trafficking, illegal gambling and tax evasion trough off shore companies as "dirty". The reason of the Committee program is to give aides to those countries which wore not FATF members as money laundering is the most profit yielding business on the world with it’s 2800 milliard USD turnover. This organization controls Hungary by supervising the law and overall actions giving a so called Progress Report about the achievements. In aspect of criminal affairs Money laundering includes all activities which achieve to transform the origin of funds coming from criminal activity as well as tax evasion activity into a legalized form. Money laundering as a phenomenon became a global problem in the second half of the 20th century parallel to sudden increase of drug trafficking. In the past few decades money laundering and the chain of criminal activities as underlying offences got into the scope of the leading economic states. Those activities of money laundering maximally exploit the free movement of capital and financial services. In both the economy and political life there is a need for having laws and regulations against money laundering which rigorously regulate the different financial, bank supervisory activities. According to estimations in the nineties three hundred billion dollars were circulating annually across the world in order to be laundered. Nowadays this figure is well over thousand billion dollars.
Enzime recovery by ultrafiltration from broth
Authors: Zeitoune Jessica Florinda 
Published: May 2018

Recycling waste products of food industry is important: one side because of the environmental aspects and the other side because of the economic reasons. The one of the most preferred basic material for second generation bio-fuels might be the tobacco (Ábel et al. 2011). The cost of the process depends on the cost of the hydrolysis of cellulose/lignocelluloses i.e. and the cost of the enzymes. These enzymes are very expensive and that is why it is so important to find a good enzyme recovery method. In my research the membrane separation was used for enzyme recovery. Different polyether-sulphone membranes with cut-off value of 7 kDa (PES7) and 10 kDa (PES10) were used for separation the hydrolyzate.
How can we spread the quality virus in domestic organizations?
Authors: Szabó Krisztián 
Published: May 2018

There is no room for debate about that the quality is one of the most important competitive factor and nobody against that this quality virus has to be spread. The paper would like to help with methods and techniques -what are not well known and used in the domestic organizations- to spread this virus in different fields of the organization. Its strategic motto is: Instead of quality management development and improvement the quality of the management has to be developed in the interest of quality management. Today, that idea has to be given up what says more and more modern quality systems development start is needed instead of it the whole quality of the management has to be developed. High level of the quality system is not equal with the high quality of the management. It has to be turned. The high level of the management quality will ensure the possibility of well operating high level quality management. The paper wants to present this from different aspects on the one hand by comparing the classical development of quality management systems on the other hand by new (volume, profit and quality) approaches of certain management elements and methods.