Sample records for rapidly progressive form

  1. Rapidly progressive periodontitis. A distinct clinical condition. (United States)

    Page, R C; Altman, L C; Ebersole, J L; Vandesteen, G E; Dahlberg, W H; Williams, B L; Osterberg, S K


    We report radiographic, clinical, historical, and laboratory observations on seven patients selected to illustrate the features and characteristics of rapidly progressive periodontitis, with the aim of establishing this disease as a distinct clinical entity. This form of periodontitis is seen most commonly in young adults in their twenties, but it can occur in postpubertal individuals up to approximately 35 years of age. During the active phase, the gingival tissues are extremely inflamed and there is hemorrhage, proliferation of the marginal gingiva, and exudation. Destruction is very rapid, with loss of much of the alveolar bone occurring within a few weeks or months. This phase may be accompanied by general malaise, weight loss, and depression, although these symptoms are not seen in all patients. The disease may progress, without remission, to tooth loss, or alternatively, it may subside and become quiescent with or without therapy. The quiescent phase is characterized by the presence of clinically normal gingiva that may be tightly adapted to the roots of teeth with very advanced bone loss and deep periodontal pockets. The quiescent phase may be permanent, it may persist for an indefinite period, or the disease activity may return. Most patients with rapidly progressive periodontitis have serum antibodies specific for various species of Bacteroides, Actinobacillus, or both, and manifest defects in either neutrophil or monocyte chemotaxis. Affected patients generally respond favorably to treatment by scaling and open or closed curettage, especially when accompanied by standard doses of antibiotics for conventional time periods. A small minority of patients do not respond to any treatment, including antibiotics, and the disease progresses inexorably to tooth loss even in the presence of aggressive periodontal therapy and maintenance. At the present time it is not possible to distinguish prior to treatment which individuals will respond to therapy and which will

  2. Rapidly progressive post-transplant lymphoproliferative disease ...

    African Journals Online (AJOL)

    Sirolimus, a potent inhibitor of B- and T-cell activation, is a commonly used immunosuppressant after renal transplantation. Withdrawal of sirolimus from the immunosuppression regimen may reduce B-cell surveillance. We present a case of rapidly progressive central nervous system (CNS) polymorphic Epstein-Barr virus ...

  3. Rapidly progressive young-onset dementia. (United States)

    Kelley, Brendan J; Boeve, Bradley F; Josephs, Keith A


    To characterize a cohort of individuals who have experienced rapidly progressive dementia with onset before age 45. Very little data regarding the clinical features or clinical spectrum of rapidly progressive young-onset dementia (RP-YOD) is available, primarily consisting of case reports or small series. A search of the Mayo Clinic medical record was employed to identify patients who had onset before age 45 of rapidly progressive dementia. All available medical records, laboratory data, neuroimaging studies, and pathologic data were reviewed. Twenty-two patients met the predefined inclusion and exclusion criteria. Behavioral and affective disorders, cerebellar dysfunction, and visual and/or oculomotor dysfunction were common early clinical features within the cohort, as were clinical features often associated with Creutzfeldt-Jakob disease. Diagnostic testing identified an etiology in most patients. Presentations of RP-YOD result from a variety of etiologies and significant overlap in clinical features is observed. Clinical features often associated with Creutzfeldt-Jakob disease seem to be common within the entire cohort of RP-YOD patients. Diagnostic studies aided in establishing a diagnosis in most patients, however 5 had uncertain diagnoses despite exhaustive evaluation.

  4. An Unusual Case of Rapidly Progressive Hyperbilirubinemia

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    Kimberly M. Thornton


    Full Text Available We present an unusual case of hyperbilirubinemia with rapid early progression leading to bilirubin encephalopathy in a term neonate. Despite early recognition and intervention, the total serum bilirubin reached a maximum level of 39 mg/dL at 32 hours of life. Prior to an emergent exchange transfusion, the patient’s diagnostic evaluation was significant for Coombs-negative microangiopathic hemolytic anemia and thrombocytopenia. Further testing revealed a deficiency of ADAMTS13 protein, or von Willebrand factor-cleaving protease, a finding diagnostic of congenital thrombotic thrombocytopenic purpura, or Upshaw-Schulman syndrome. This rare disease is often misdiagnosed, especially in the newborn period.

  5. Sheet metal forming using rapid prototyped tooling (United States)

    Park, Young-Bin

    The demand for rapid, low-cost die fabrication and modification technology is greater than ever in sheet metal forming industry. One category of rapid tooling technology involves the application of advanced polymers and composites to fabricate metal forming dies. Despite their advantages in lead time and cost reductions, polymer dies for sheet metal forming applications have several drawbacks. Due to their lack of strength as compared to conventional die materials, the use of polymer dies is often limited to prototype or short-run production. In addition, because the mechanisms by which they fail are not fully understood, the dies are designed on the basis of experience and intuition. The research (1) characterized the mechanical behavior of an advanced polymer composite tooling material, (2) developed a method to predict the failure mode and the life of a polymer die, and (3) established optimal die design guidelines. The focus was on rapid prototyped, aluminum trihydrate(ATH)-filled, polyurethane-based dies in sheet metal forming. The study involved the determination of dominant process parameters based on the finite element analyses of 90° V-die bending and cylindrical cup drawing processes. The effects of process parameters on stress distribution in the die provided guidelines to the modification of die design for achieving the desired die life. The presented parametric study lays the groundwork for providing reliable tool failure prediction and design optimization guidelines for advanced polymer tooling materials in metal forming. In addition, the failure mechanisms were investigated to predict the failure mode and the fatigue life of the die. To establish a fundamental understanding of the fatigue behavior of the polyurethane-based die material, extensive material tests were performed, the microstructure was studied, and the fatigue properties were identified experimentally. The test data were incorporated into the local stress-based fatigue analysis to

  6. Relapsing and Progressive Tumefactive Demyelinating Form of Central Nervous System Involvement in a Patient with Progressive Systemic Sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ho Kyun [Dept. of Radiology, Catholic University of Daegu School of Medicine, Daegu (Korea, Republic of); Lee, Hui Joong [Dept. of Kyungpook National University Hospital, Daegu (Korea, Republic of)


    White matter hyper intensities (WMHI) on MRI are not rare in patients with progressive systemic sclerosis (PSS). In this presentation, WMHI were developed in both middle cerebellar peduncles and temporal white matter in a patient with PSS, and regressed after medication of high dose steroid. However, new lesions were developed in the subcortices of both precentral gyri, and progressed rapidly to tumefactive hyperintensity on MRI. We report an unusual relapsing and progressive tumefactive demyelinating form of central nervous system involvement in PSS.

  7. The usefulness and significance of assessing rapidly progressive spermatozoa (United States)

    Björndahl, Lars


    It is possible and clinically relevant to distinguish between slow and rapid progressive spermatozoa in basic semen analysis. This is discussed in light of the different purposes of semen analysis for the subfertile couple and the male patient. The two groups of progressive spermatozoa should be distinguished to help ensure that pertinent information available in the semen sample is not neglected. PMID:20111079

  8. Technical progress, market forms and unemployment

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    Full Text Available The purpose of the present paper is threefold. The first purpose is to emphasise the importance of the insights to be found in Sylos Labini’s work. The second purpose is to expand upon it by distinguishing  between the different types of technical progress. Finally, the third purpose is to comment on Labini’s comparison of Ricardo and Keynes on the possibility of technological unemployment. Regarding his description of technological progress as “labour saving”, the author shows that a more comprehensive description would be more useful for his purposes.

  9. Technetium Waste Form Development - Progress Report

    Energy Technology Data Exchange (ETDEWEB)

    Gelles, David S.; Ermi, Ruby M.; Buck, Edgar C.; Seffens, Rob J.; Chamberlin, Clyde E.


    Analytical electron microscopy using SEM and TEM has been used to analyze a ~5 g. ingot with composition 71.3 wt% 316SS-5.3 wt% Zr-13.2 wt% Mo-4.0 wt% Rh-6.2 wt% Re prepared at the Idaho National Laboratory. Four phase fields have been identified two of which are lamellar eutectics, with a fifth possibly present. A Zr rich phase was found distributed as fine precipitate, ~10µm in diameter, often coating large cavities. A Mo-Fe-Re-Cr lamellar eutectic phase field appears as blocky regions ~30µm in diameter, surrounded by a Fe-Mo-Cr lamellar eutectic phase field, and that in turn is surrounded by a Zr-Fe-Rh-Mo-Ni phase field. The eutectic phase separation reactions are different. The Mo-Fe-Re-Cr lamellar eutectic appears a result of austenitic steel forming at lower volume fraction within an Mo-Fe-Re intermetallic phase, whereas the Fe-Mo-Cr lamellar eutectic may be a result of the same intermetallic phase forming within a ferritic steel phase. Cavitation may have arisen either as a result of bubbles, or from loss of equiaxed particles during specimen preparation.

  10. Rapidly Progressive Atrioventricular Block in a Patient with Sarcoidosis

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    Nagham Saeed Jafar


    Full Text Available Cardiac sarcoidosis is a major cause of death in patients with systemic sarcoidosis. Cardiac manifestations are seen in 2.3% of the patients. Atrioventricular (AV block is one of the common manifestations of cardiac sarcoidosis. Other presentations of cardiac involvement include congestive heart failure, ventricular arrhythmias, and sudden cardiac death. The presence of AV block in young patients should raise the suspicion of sarcoidosis. AV block may be the only manifestation and patients may not have clinical evidence of pulmonary involvement. Here we describe a young male presented with exercise induced AV block rapidly progressing to complete heart block with recurrent syncope needing urgent pacemaker implantation. Factors that suggested an infiltrative process included his young age, rapidly progressive conduction abnormalities in the ECG in the absence of coronary disease, and previous history of cutaneous sarcoidosis.

  11. Rapid progress on the vertebrate tree of life

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    Shaffer H Bradley


    Full Text Available Abstract Background Among the greatest challenges for biology in the 21st century is inference of the tree of life. Interest in, and progress toward, this goal has increased dramatically with the growing availability of molecular sequence data. However, we have very little sense, for any major clade, of how much progress has been made in resolving a full tree of life and the scope of work that remains. A series of challenges stand in the way of completing this task but, at the most basic level, progress is limited by data: a limited fraction of the world's biodiversity has been incorporated into a phylogenetic analysis. More troubling is our poor understanding of what fraction of the tree of life is understood and how quickly research is adding to this knowledge. Here we measure the rate of progress on the tree of life for one clade of particular research interest, the vertebrates. Results Using an automated phylogenetic approach, we analyse all available molecular data for a large sample of vertebrate diversity, comprising nearly 12,000 species and 210,000 sequences. Our results indicate that progress has been rapid, increasing polynomially during the age of molecular systematics. It is also skewed, with birds and mammals receiving the most attention and marine organisms accumulating far fewer data and a slower rate of increase in phylogenetic resolution than terrestrial taxa. We analyse the contributors to this phylogenetic progress and make recommendations for future work. Conclusions Our analyses suggest that a large majority of the vertebrate tree of life will: (1 be resolved within the next few decades; (2 identify specific data collection strategies that may help to spur future progress; and (3 identify branches of the vertebrate tree of life in need of increased research effort.

  12. Syndromes of rapidly progressive cognitive decline-our experience

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    Sadanandavalli Retnaswami Chandra


    Full Text Available Background: Dementias are fairly slowly progressive degenerative diseases of brain for which treatment options are very less and carry a lot of burden on family and society. A small percentage of them are rapidly progressive and mostly carry a different course outcome. However, there are no definite criteria other than the time line for these patients. Aims: The aim of this was to identify and categorize the causes and course of rapidly progressive dementias seen in our center. Settings and Design: Patients who presented with rapid deterioration of cognitive functions within weeks to 1 year between 2011 and December 2016 were evaluated. Patients and Methods: All patients underwent all mandatory tests for dementia including brain imaging. Complete vasculitis workup, autoimmune encephalitis profile including Voltage Gated Potassium Channel, N-methyl-D-aspartic acid receptor, glutamic acid-decarboxylase, thyroid-peroxidase antibody, cerebrospinal fluid, and other special tests such as duodenal biopsy and paraneoplastic workup were done based on clinical indications. Results and Conclusions: Out of 144 patients 42 had immune-mediated encephalopathy, 18 had Creutzfeldt-Jakob disease, 3 had Vitamin B12 deficiency, 63 had infection with neurocysticercosis, 7 had tuberculosis, 2 had HIV, 1 had herpes simplex encephalitis, 1 had neurosyphilis, 1 Whipples disease, 1 had Subacute Sclerosing Panencephalitis, 1 had Mass lesion, 3 had Frontotemporal dementia, and 3 had small vessel disease. Good majority of these patients have infective and immune-mediated causes and less number belong to degenerative group. Therefore, caution is needed to look for treatable cause as it carries a different treatment options and outcome.

  13. Adult-onset phenylketonuria with rapidly progressive dementia and parkinsonism. (United States)

    Tufekcioglu, Zeynep; Cakar, Arman; Bilgic, Basar; Hanagasi, Hasmet; Gurvit, Hakan; Emre, Murat


    Phenylketonuria (PKU) is an autosomal recessive metabolic disorder due to mutations in the phenylalanine hydroxylase (PAH) gene, which converts phenylalanine (PHE) to tyrosine. Although it is principally a childhood disorder, in rare cases, the first signs of PKU may develop in late adulthood resembling common neurological diseases. Here we report a 59-year-old, previously normal functioning man who was admitted with blurred vision, cognitive problems, and gait difficulty that began 8 months before. He had brisk reflexes and left side dominant parkinsonism. His Mini-Mental State Examination (MMSE) score was 25/30, and neuropsychological evaluation revealed a dysexecutive syndrome with simultanagnosia and constructional apraxia. His Clinical Dementia Rating score (CDR) was 1. Cranial MRI revealed bilateral diffuse hyperintense lesions in parietal and occipital white matter in T2, fluid-attenuated inversion recovery, and diffusion weighted images. Diagnostic workup for rapidly progressive dementias was all normal except PHE level which was found to be highly elevated (1075 μmol/L, normal 39-240 μmol/L) with normal tyrosine level (61.20 μmol/L, normal 35-100 μmol/L). Three months after PHE-restricted diet, his cognitive impairment and signs of parkinsonism significantly improved, with MRI scan unchanged. This case demonstrates that late-onset PKU is a rare, treatable cause of rapidly progressive dementia and parkinsonism with certain constellations such as consanguinity and white matter abnormalities (WMAs) in imaging.

  14. Rapidly progressive cryptogenic organising pneumonia presenting as a lung mass (United States)

    Akram, Saeed; Irfan, Muhammad; Aftab, Kanwal


    A very rare case of a rapidly progressive variant of cryptogenic organising pneumonia (COP) presenting as a focal mass-like lesion with compression of the large airways leading to respiratory failure is described. A 60-year-old lady presented to the Aga Khan University Hospital Emergency Department in hypoxaemic respiratory failure with a 6-day history of dyspnoea, productive cough and fever. Chest x ray showed a right upper lobe mass-like lesion compressing the large airways and right pleural effusion. She deteriorated in the Emergency Department and was intubated due to worsening hypoxaemic respiratory failure. The pleural fluid and bronchoscopic specimens were negative on microbiological and cytological examination. CT-guided right lung biopsy revealed chronic non-specific inflammation without granuloma and malignancy. COP was diagnosed on video-assisted thoracoscopic (VATS) lung biopsy. She was successfully treated with high dose steroids and discharged in a stable condition; her 3-month follow-up chest x rays showed complete resolution of the lung lesion with some residual fibrosis. PMID:21686529

  15. Case of Rapid Progression of Hemiatrophy on the Face: A New Clinical Entity?

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    Hisashi Nomura


    Full Text Available A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.

  16. Heavy metals processing near-net-forming summary progress report

    Energy Technology Data Exchange (ETDEWEB)

    Watson, L.D. [Custom Spray Technologies, Inc., Rigby, ID (United States); Thompson, J.E. [Martin Marietta Energy Systems, Inc., Oak Ridge, TN (United States)


    This study utilized a converging-diverging nozzle to spray-form an alloy having a weight percent composition of 49.6% iron, 49.6% tungsten, and 0.8% carbon into samples for analysis. The alloy was a surrogate that displayed metallurgical characteristics similar to the alloys used in the heavy metals processing industry. US DOE facilities are evaluating advanced technologies which can simplify component fabrication, reduce handling steps, and minimize final machining. The goal of producing net-shaped components can be approached from several directions. In spray forming, molten metal is converted by a nozzle into a plume of fine droplets which quickly cool in flight and solidify against a substrate. The near-final dimension product that is formed receives additional benefits from rapid solidification. This single-step processing approach would aid the heavy metals industry by streamlining fabrication, improving production yields, and minimizing the generation of processing wastes. This Program effort provided a large selection of as-sprayed specimens. These samples were sprayed with gas-to-metal mass ratios ranging from 0.8:1 to 4:1. Samples targeted for analysis were produced from different spray conditions. Metallography on some samples revealed areas that were fully dense and homogeneous at 5,000X. These areas averaged grain sizes of 1 micron diameter. Other samples when viewed at 2,000X were highly segregated in the 10 micron diameter range. Deposit efficiencies of greater than 90% were demonstrated using the untailored spray system. Discharge gases were analyzed and two categories of particles were identified. One category of particle had a chemical composition characteristic of the alloy being sprayed and the second type of particle had a chemical composition characteristic of the ceramics used in the spray system component fabrication. Particles ranged in size from 0.07 to 3 microns in diameter. 8 refs., 67 figs., 20 tabs.

  17. Clinical reasoning: rapidly progressive quadriparesis in a forgetful patient. (United States)

    Mantoan Ritter, Laura; Isaacs, Jeremy D; McEntagart, Meriel; O'Dwyer, John P


    A 50-year-old right-handed retired family business manager developed progressive left-sided weakness over 5 days after a mechanical fall. She remembered catching her foot on the carpet and falling down a flight of stairs, followed by severe neck pain over C4-C5 and inability to get up for nearly an hour. Over the subsequent month her symptoms progressed and she presented to hospital with an asymmetric spastic paraparesis, loss of pinprick sensation in her arms and legs, loss of vibration sense to both hips, and double incontinence.

  18. Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis. (United States)

    Ozkurt, Sultan; Acikalin, Mustafa Fuat; Temiz, Gokhan; Akay, Olga Meltem; Soydan, Mehmet


    Hereditary hemochromatosis leads to the accumulation of iron in many organs including the liver, spleen and heart and results in injury and dysfunction of these organs. On the other hand, iron accumulation and functional impairment in kidney is extremely rare. We report a 61-year-old male patient with hereditary hemochromatosis, in whom the renal function was deteriorated rapidly. Renal biopsy revealed crescentic glomeruli and hemosiderin accumulation in tubular epithelial cells.

  19. Treatment of nephrotic syndrome associated with idiopathic rapidly progressive glomerulonephritis and cyclosporin A. (United States)

    Maduell, F; Sánchez-Alcaraz, A; Sigüenza, F; Caridad, A; Sangrador, G


    Recent reports suggest that cyclosporin A is beneficial in inducing remission of idiopathic nephrotic syndrome. Nephrotic syndrome is seen in 10-30% of patients with rapidly progressive glomerulonephritis. We report a case of a 69-year-old man with nephrotic syndrome, associated with idiopathic rapidly progressive glomerulonephritis, who was treated initially with corticosteroid and cyclophosphamide. Three months later he developed thrombophlebitis and leucopenia and cyclophosphamide was suspended. Relapse of nephrotic syndrome associated with rapidly progressive glomerulonephritis developed and therapy with cyclosporin A was used with a good response.

  20. Correlation of the Type and Form of Hip Osteoarthritis Progression with Forms of the Hip-Spine Syndrome

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    H.V. Gaiko


    Slow-progressing form and hyperplastic type of hip osteoarthritis by Bombelli corresponds to the true form of hip-spine syndrome. Fast-progressing form and hypoplastic type of hip osteoarthritis by Bombelli corresponds to the reversible form of hip-spine syndrome. Such relationships contribute to a better understanding of the pathogenesis of hip-spine syndrome and the development of effective methods for the prevention and treatment of this severe orthopedic pathology.

  1. Progress on high-performance rapid prototype aluminum mirrors (United States)

    Woodard, Kenneth S.; Myrick, Bruce H.


    Near net shape parts can be produced using some very old processes (investment casting) and the relatively new direct metal laser sintering (DMLS) process. These processes have significant advantages for complex blank lightweighting and costs but are not inherently suited for producing high performance mirrors. The DMLS process can provide extremely complex lightweight structures but the high residual stresses left in the material results in unstable mirror figure retention. Although not to the extreme intricacy of DMLS, investment casting can also provide complex lightweight structures at considerably lower costs than DMLS and even conventional wrought mirror blanks but the less than 100% density for casting (and also DMLS) limits finishing quality. This paper will cover the progress that has been made to make both the DMLS and investment casting processes into viable near net shape blank options for high performance aluminum mirrors. Finish and figure results will be presented to show performance commensurate with existing conventional processes.

  2. Acute HIV infection with rapid progression to AIDS

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    Marcio de Oliveira Silva

    Full Text Available Acute HIV infection is rarely recognized as the signs and symptoms are normally unspecific and can persist for days or weeks. The normal HIV course is characterized by a progressive loss of CD4+ cells, which normally leads to severe immunodeficiency after a variable time interval. The mean time from initial infection to development of clinical AIDS is approximately 8-10 years, but it is variable among individuals and depends on a complex interaction between virus and host. Here we describe an extraordinary case of a man who developed Pneumocisits jiroveci pneumonia within one month after sexual exposure to HIV-1, and then presented with 3 consecutive CD4 counts bellow 200 cells/mm³ within 3 months, with no other opportunistic disease. Although antiretroviral therapy (AZT+3TC+ATZ/r was started, with full adherence of the patient, and genotyping indicating no primary antiretroviral resistance mutations, he required more than six months to have a CD4 restoration to levels above 200 cells/mm³ and 10 months to HIV-RNA to become undetectable.

  3. Development of a definition for Rapid Progression (RP) of renal function in HIV-positive persons

    DEFF Research Database (Denmark)

    Kamara, David A; Nielsen, Lene Ryom; Ross, Michael


    No consensus exists on how to define abnormally rapid deterioration in renal function (Rapid Progression, RP). We developed an operational definition of RP in HIV-positive persons with baseline estimated glomerular filtration rate (eGFR) >90ml/min/1.73m2 (using Cockcroft Gault) in the Data Collec...

  4. B-Cell Depletion Salvage Therapy in Rapidly Progressive Dermatomyositis Related Interstitial Lung Disease. (United States)

    Eissa, Khaled; Palomino, Jaime


    Interstitial lung disease (ILD) is a major cause of morbidity and mortality in patients with idiopathic inflammatory myopathies (IIM). Glucocorticoids are the initial standard treatment. However, many patients fail to respond and continue to progress despite treatment with high dose glucocorticoids. The efficacy of rituximab has been suggested in case reports and case series of refractory antisynthetase (AS) syndrome, but data on patients without auto-antibodies or with rapidly progressive ILD are scarce. We report a case of rapidly progressive dermatomyositis (DM) associated ILD treated successfully with B-cell depletion therapy.

  5. Rapid progression of gliomatosis cerebri to secondary glioblastoma, factors that affects the progression rate: A case report

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    Kim, Hee Kyung; Yu, In Kyu; Kim, Seung Min; Kim, Joo Heon; Lee, Seung Hoon; Lee, Seung Yeon [Eulji University Hospital, Daejeon (Korea, Republic of)


    Glioblastomas may develop de novo or through progression from low-grade or anaplastic astrocytomas. The term 'primary glioblastoma' refers to a glioblastoma that lacks a precursor lesion and has a clinical history of less than three months. On the other hand, the term 'secondary glioblastoma' indicates that the glioblastoma has progressed from a low-grade tumor after a long latency period and often manifests in younger patients. These subtypes of glioblastoma develop via different genetic pathways, and they differ in prognosis and response to therapy. Thus, differential diagnosis of these subtypes and prediction of the factors that affect the progression from low-grade diffuse astrocytoma to secondary glioblastoma would be clinically very important. We present a rare case of secondary glioblastoma, which developed only three months after the follow up imaging evaluations, with a history of low grade glioma, and present the factors that cause rapid progression.

  6. Peranan Terapi Awal dan Terapi Pemeliharaan pada Rapidly Progressive Periodontitis Type 1

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    Anita H. Joedo


    Full Text Available Rapidly Progressive Periodontitis (RPP is a severe form of a periodontal disease which starts since a puberty age. The disease if a generalized periodontal destruction without a specific distribution mode: it develops more progressively but does not in accordance to local factors. The first step to the RPP treatment is initial therapy: i.e. DHE, scaling and root planing, and eliminating predisposing local factors and continued with a maintence therapy which will support the success of a surgery later. A study case: a 21-year old RPP woman showed hyperaemia, an abscess, a 10 mm mesial pocket depth, a 5 mm distal pocker depth, a 5 mm buccal pocket depth, a 2nd degree tooth mobility and a 3 mm buccal recession on 25. In the initial therapy she was given an amoxicillin, a metronidazole for killing a supra and subgingival baterial, vitamins B and C, and also a chlorhexidine 0.2% mouth wash for a week. After a week the abscess and the inflammation decreased, but the mobility was still in the same condition and the DHE was still evaluated because of the patient's social factor, the FO was delayed. The next visit was done every 2 monts for a year for maintenance care. The clinical result showed the gingival inflammation and the tooth mobility disappeared. Radiographically, the alveolar bone showed more radiopaque and the lamina dura was seen. In conclusio, the initial and the maintenance therapy was seen to heal the RPP.

  7. Wegener's granulomatosis: report of a case with rapidly progressive glomerulonephritis and diabetes mellitus. (United States)

    Kakoi, H; Hiraide, F; Nishizawa, S; Inouye, T; Yoshizawa, N


    A patient with the classic form of Wegener's granulomatosis with severe dabetes mellitus and rapidly progressive glomerulonephritis is described. This 61-year-old male presented with epistaxis and nasal pain and obstruction. The nasal cavities were filled with crusts covering eroded mucosa. The diagnosis was made by biopsy of nasal and bronchial mucosa, and laboratory data. The epistaxis was stopped by 10 Gy irradiation over the nasal cavities. The patient had severe diabetes mellitus. His blood sugar was not controlled by diet and insulin injection. His general condition worsened rapidly as the growth of granuloma in the nose and lung. Accordingly, prednisolone therapy reinitiated to suppress the granuloma although it has a reverse effect on diabetes mellitus. Approximately one month after admission, he died of acute renal failure. Autopsy was carried out. Granulomatous lesions were noted in the nasal cavities, lungs and spleen. Many petechiae were found macroscopically over the cortex of the kidney. Hyalinization or sclerosis with crescent formation was found microscopically in estimated 85% of the glomeruli. Immunohistologic analysis of the renal tissue demonstrated an irregular linear pattern deposition of IgG, IgA and C3 and a granular pattern deposition of IgM and C1q.

  8. Primary Cutaneous Peripheral T-Cell Lymphoma Not Otherwise Specified: A Rapidly Progressive Variant of Cutaneous T-Cell Lymphoma

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    Kimberly Aderhold


    Full Text Available Primary Cutaneous Peripheral T-Cell Lymphoma NOS (PTL-NOS is a rare, progressive, fatal dermatologic disease that presents with features similar to many common benign plaque-like skin conditions, making recognition of its distinguishing features critical for early diagnosis and treatment (Bolognia et al., 2008. A 78-year-old woman presented to ambulatory care with a single 5 cm nodule on her shoulder that had developed rapidly over 1-2 weeks. Examination was suspicious for malignancy and a biopsy was performed. Biopsy results demonstrated CD4 positivity, consistent with Mycosis Fungoides with coexpression of CD5, CD47, and CD7. Within three months her cancer had progressed into diffuse lesions spanning her entire body. As rapid progression is usually uncharacteristic of Mycosis Fungoides, her diagnosis was amended to PTL-NOS. Cutaneous T-Cell Lymphoma (CTCL should be suspected in patients with patches, plaques, erythroderma, or papules that persist or multiply despite conservative treatment. Singular biopsies are often nondiagnostic, requiring a high degree of suspicion if there is deviation from the anticipated clinical course. Multiple biopsies are often necessary to make the diagnosis. Physicians caring for patients with rapidly progressive, nonspecific dermatoses with features described above should keep more uncommon forms of CTCL in mind and refer for early biopsy.

  9. Subacute sclerosing panencephalitis presenting as rapidly progressive young-onset dementia. (United States)

    Chakor, Rahul Tryambak; Santosh, Nandanavana Subbareddy


    Onset of dementia before 65 years of age is termed as young-onset dementia (YOD). Very little literature exists regarding the clinical features and diagnoses of dementia in younger individuals. We present a case series of four patients of age 10 to 23 years with severe dementia within 18 months of clinical onset (rapidly progressive dementia). Three patients had generalised periodic complexes typical of subacute sclerosing panencephalitis (SSPE) on electroencephalogram (EEG). All patients had elevated cerebrospinal fluid (CSF) IgG measles antibodies. Our case series highlights that SSPE is an important cause of rapidly progressive YOD in developing countries like India.

  10. Obesity induced rapid melanoma progression is reversed by orlistat treatment and dietary intervention: Role of adipokines‡


    Malvi, Parmanand; Chaube, Balkrishna; Pandey, Vimal; Vijayakumar, Maleppillil Vavachan; Boreddy, Purushotham Reddy; Mohammad, Naoshad; Singh, Shivendra Vikram; Bhat, Manoj Kumar


    Obesity, owing to adiposity, is associated with increased risk and development of various cancers, and linked to their rapid growth as well as progression. Although a few studies have attempted to understand the relationship between obesity and melanoma, the consequences of controlling body weight by reducing adiposity on cancer progression is not well understood. By employing animal models of obesity, we report that controlling obesity either by orlistat treatment or by restricting caloric i...

  11. Rapidly progressing and resistant warts in an immuno-competent male

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    Md. Shahidullah Sikder


    Full Text Available Cutaneous warts are common skin conditions caused by different specific strains of the human papilloma virus (HPV, mostly affect children as localized lesion on the hands and feet. They are slowly progressing and disseminated lesions are found in immuno-compromised situations. Usually majority of warts disappear by few months to two years. This is a case of extensive, giant, rapidly progressing and resistant warts in an immuno-competent adult male. 

  12. Progress in forming bottom barriers under waste sites

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    Carter, E.E. [Carter Technologies, Sugar Land, TX (United States)


    The paper describes an new method for the construction, verification, and maintenance of underground vaults to isolate and contain radioactive burial sites without excavation or drilling in contaminated areas. The paper begins with a discussion of previous full-scale field tests of horizontal barrier tools which utilized high pressure jetting technology. This is followed by a discussion of the TECT process, which cuts with an abrasive cable instead of high pressure jets. The new method is potentially applicable to more soil types than previous methods and can form very thick barriers. Both processes are performed from the perimeter of a site and require no penetration or disturbance of the active waste area. The paper also describes long-term verification methods to monitor barrier integrity passively.

  13. [Rapidly progressive puberty in a patient with mosaic Turner syndrome: a case report and literature review]. (United States)

    Liang, Y; Wei, H; Yu, X; Huang, W; Luo, X P


    Objective: To explore the clinical characteristics of diagnosis and treatment in patients with Turner syndrome and rapidly progressive puberty. Method: A rare case of rapidly progressive puberty in Turner syndrome with a mosaic karyotype of 45, X/46, X, del(X)(p21)(80%/20%)was diagnosed at Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology in January. 2015. Clinical characteristics and the related literature were reviewed. Original papers on precocious puberty or rapidly progressive puberty in Turner syndrome, published until Apr. 2016 were retrieved at PubMed and CNKI databases by the use of the key words "Turner syndrome" , "precocious puberty" and "rapidly progressive puberty" . Result: The patient was born at term with birth weight of 2 450 g and was diagnosed with SGA at 3 years of age for the first evaluating of growth and development. Then recombined human growth hormone (rhGH )was given at 4 years of age due to short stature (heightpuberty in a 45, X/46, X, del(X)(p21) mosaic Turner syndrome is reported. Although short stature and ovarian dysgenesis are common in TS, precocious puberty may occur in TS, which is liable to cause delayed diagnosis and misdiagnosis. Careful examination is recommended for patients with unusual growth pattern, even though girls have normal height in accord with standard growth curve or spontaneous puberty. Evaluation for TS and subsequent investigation should be prompted.

  14. Rapidly progressive systemic sclerosis with a fatal outcome in male patients

    Directory of Open Access Journals (Sweden)

    Małgorzata Widuchowska


    Full Text Available Objectives: Retrospective analysis of clinical outcomes of malepatients with particularly severe and rapidly progressive diffusesystemic sclerosis (SSc with a fatal outcome with emphasis onorgan involvement and results of diagnostic tests, and tentativedistinction of a subgroup of especially progressive SSc. Material and methods: In the last few years among patients withSSc hospitalized in our centres, five patients with particularlyrapidly progressive disease were distinguished. Despiteaggressive treatment, the disease led to a fatal outcome ina short time. Their clinical history and results of diagnostic testswere evaluated. Results: All of them were smokers and three of them did not stopsmoking after the diagnosis. Laboratory findings revealed hightitres of Scl70 antibodies and enhanced erythrocytesedimentation rate (ESR in all of the patients. Most of them hadincreased serum creatine kinase (CK values. During the diseaserapidly progressive severe organ involvement was observed(pulmonary fibrosis, renal failure, cardiac failure, pulmonaryarterial hypertension. The skin thickening increased rapidly andthey died within 12-24 months after the first signs of skinthickening. Acute cardiac failure was the cause of death. Conclusions: The described cases suggest possible distinction ofa subset of a subgroup of patients with a particularly severe and rapidly progressive disease. It might be a population of patientswith the following characteristics: males over 40 years of agewith high titres of anti-Scl70 antibodies and elevated serum CKlevels. This is consistent with the presently published data onfactors associated with fatal prognosis in patients with SSc.

  15. Twin-cutter form turning of commutator rings using cutters’ progressive motion piezoelectric engines

    Directory of Open Access Journals (Sweden)

    Vozhzhov Andrey


    Full Text Available The option of realization of the technological scheme of twin-cutter form turning via use of piezoelectric engines of progressive motion is hereby proposed. The cutting force vector constitutes the external perturbation effect. The scheme, design, transfer function of the cutter's piezoelectric engine of progressive motion is hereby presented.

  16. [Features of biochemical changes in blood serum according to the form of progression of idiopathic and dysplastic coxarthrosis]. (United States)

    Gaĭko, G V; Magomedov, A M; Kalashnikov, A V; Kuzub, T A; Polishchuk, L V


    Carried out biochemical studies of blood serum (collagenase activity, glycosamineglicans and hydroxyproline fractions) of 72 patients with hip idiopathic osteoarthrosis and 30 patients with dysplastic osteoarthrosis of the iv-th stage in.accordance with J. H. Kellgren and J. S. Lavrence depending on the form of pathologic process progression. It has been proved that both with idiopathic and dysplastic coxartrosis metabolism of basic protein of osteochonrous tissue was broken both in catabolic and in synthetic phase of this process. The most deep changes 1 of biochemical values (collagenase, free and proteinbinded hydroxyprolines, the content of glycosamines) have been observed with rapid form of course progression of idiopathic and dysplastic coxarthrosis. Definite appropriateness promotesin.patients better understanding of coxarthrosis pathogenese, development of diagnostic and medical measures for patients with this severe orthopedic pathology.

  17. [A young man with rapidly progressing dyspnoea and a mediastinal mass]. (United States)

    van Spronsen, D J; van den Berkmortel, F W P J; Bootsma, G P; de Mulder, P H M


    An 18-year-old male presented with a 2-week history of rapid progressive dyspnoea and dry cough due to a large mediastinal mass with compression of the trachea. Based on the raised serum values of the tumour markers chorionogonadotrophin and alphafoetoprotein the diagnosis of germ-cell tumour was made. Because of the severity of his symptoms chemotherapy with bleomycin, etoposide and cisplatin was begun on the same day and before the results of the histology investigations were known. The next day the symptoms were diminished and after completing four courses of chemotherapy there was complete remission. The differential diagnosis of a rapid progressive mediastinal mass is limited and mainly relates to malignant lymphoma and germ-cell tumours. In emergency situations if tumour markers are raised then anti-tumour therapy may be begun before any histological confirmation is available.

  18. Case report of rapidly progressive proliferative verrucous leukoplakia and a proposal for aetiology in mainland China

    Directory of Open Access Journals (Sweden)

    Zeng Xin


    Full Text Available Abstract Proliferative verrucous leukoplakia (PVL is a rare oral leukoplakia and has four features such as chronic proliferation, multiple occurrences, refractoriness to treatment and high rate of malignant transformation. As mentioned above, most PVL cases processed to malignancy over many years, sometimes 20 years. However, this report described a case of rapid progress, which had malignant transformation in a short period. Additionally, the aetiology of PVL was discussed and immunity was proposed as the possible cause.

  19. Rapidly Progressive Frontotemporal Dementia Associated with MAPT Mutation G389R. (United States)

    Sun, Lin; Chen, Kathryn; Li, Xia; Xiao, Shifu


    Frontotemporal dementia includes a large spectrum of neurodegenerative disorders. Here, we report the case of a young patient with MAPT mutation G389R, who was 27 years old when he progressively developed severe behavioral disturbances. Initially, he presented with slowly progressive personality change. After 1 year, he exhibited moderate dementia with extrapyramidal and pyramidal symptoms. MRI showed frontotemporal atrophy. He rapidly progressed to severe dementia 3 years after onset. Genetic testing revealed a heterozygous guanine to cytosine mutation at the first base of codon 389 (c.1165G>A) of MAPT, the tau gene, resulting in a glycine to arginine substitution in the patient and two unaffected relatives. We predicted the model of mutant tau protein through I-TASSER software, and speculated the structural change of tau protein caused by mutant site. We also detected the MAPT gene transcript and methylation of samples from peripheral blood leucocytes in an attempt to explain the possible mechanisms of incomplete penetrance, although there were not positive findings. This case is remarkable because of the early onset and rapid progression of the disease.

  20. Obesity induced rapid melanoma progression is reversed by orlistat treatment and dietary intervention: role of adipokines. (United States)

    Malvi, Parmanand; Chaube, Balkrishna; Pandey, Vimal; Vijayakumar, Maleppillil Vavachan; Boreddy, Purushotham Reddy; Mohammad, Naoshad; Singh, Shivendra Vikram; Bhat, Manoj Kumar


    Obesity, owing to adiposity, is associated with increased risk and development of various cancers, and linked to their rapid growth as well as progression. Although a few studies have attempted to understand the relationship between obesity and melanoma, the consequences of controlling body weight by reducing adiposity on cancer progression is not well understood. By employing animal models of obesity, we report that controlling obesity either by orlistat treatment or by restricting caloric intake significantly slows down melanoma progression. The diminished tumor progression was correlated with decreased fat mass (adiposity) in obese mice. Obesity associated factors contributing to tumor progression were decreased in the experimental groups compared to respective controls. In tumors, protein levels of fatty acid synthase (FASN), caveolin (Cav)-1 and pAkt, which are tumor promoting molecules implicated in melanoma growth under obese state, were decreased. In addition, increased necrosis and reduction in angiogenesis as well as proliferative markers PCNA and cyclin D1 were observed in tumors of the orlistat treated and/or calorically restricted obese mice. We observed that growth of melanoma cells cultured in conditioned medium (CM) from orlistat-treated adipocytes was reduced. Adipokines (leptin and resistin), via activating Akt and modulation of FASN as well as Cav-1 respectively, enhanced melanoma cell growth and proliferation. Together, we demonstrate that controlling body weight reduces adipose mass thereby diminishing melanoma progression. Therefore, strategic means of controlling obesity by reduced caloric diet or with antiobesity drugs treatment may render obesity-promoted tumor progression in check and prolong survival of patients. Copyright © 2014 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.

  1. Rapidly in situ forming biodegradable robust hydrogels by combining stereocomplexation and photopolymerization

    NARCIS (Netherlands)

    Hiemstra, C.; Zhou, W.; Zhong, Zhiyuan; Wouters, Marielle; Feijen, Jan


    Our previous studies have shown that stereocomplexed hydrogels can be rapidly formed in vitro as well as in vivo upon mixing aqueous solutions of eight-arm poly(ethylene glycol)−poly(l-lactide) (PEG−PLLA) and poly(ethylene glycol)−poly(d-lactide) (PEG−PDLA) star block copolymers. In this study,

  2. Rapidly progressive atherosclerosis after domino liver transplantation from a teenage donor with homozygous familial hypercholesterolemia. (United States)

    Golbus, Jessica R; Farhat, Linda; Fontana, Robert J; Rubenfire, Melvyn

    Familial hypercholesterolemia (FH) is an autosomal dominant disorder characterized by impaired clearance of low-density lipoprotein cholesterol. Given limitations in pharmacologic therapy and the significant morbidity and mortality associated with this disease, liver transplantation may be offered to select homozygous FH patients in childhood in an effort to slow progression of atherosclerotic cardiovascular disease. In rare cases, domino liver transplantation can be performed, transplanting the livers of patients with various metabolic disorders into elderly recipients whose projected survival precludes prolonged waiting on the transplant list. Herein, we report a case of domino liver transplantation using the liver of a 14-year-old boy with homozygous FH into a 65-year-old man with primary sclerosing cholangitis and cirrhosis who developed rapidly progressive atherosclerotic cardiovascular disease involving the arteries of his proximal bilateral lower extremities, carotid arteries and superior mesenteric artery. Copyright © 2017 National Lipid Association. Published by Elsevier Inc. All rights reserved.

  3. Research progress on the pathogenesis of rapid eye movement sleep behavior disorder and neurodegenerative diseases

    Directory of Open Access Journals (Sweden)

    Hai-yang JIANG


    Full Text Available Rapid eye movement sleep behavior disorder (RBD is a sleep disorder characterized by the disappearance of muscle relaxation and enacting one's dreams during rapid eye movement (REM, with most of the dreams being violent or aggressive. Prevalence of RBD, based on population, is 0.38%-2.01%, but it becomes much higher in patients with neurodegenerative diseases, especially α - synucleinopathies. RBD may herald the emergence of α-synucleinopathies by decades, thus it may be used as an effective early marker of neurodegenerative diseases. In this review, we summarized the progress on the pathogenesis of RBD and its relationship with neurodegenerative diseases. DOI: 10.3969/j.issn.1672-6731.2017.10.003

  4. Rapidly progressing dual infection with Aspergillus and Rhizopus: when soil inhabitants become deadly invaders. (United States)

    Bhagat, Milind; Rapose, Alwyn


    We present a case report of a 61-year-old patient with acute pulmonary and cerebral infections with Aspergillus and Rhizopus. The only risk factor for invasive fungal disease was high-dose corticosteroids used to treat her chronic obstructive pulmonary disease exacerbation. She had rapid progression and succumbed to her infections within 2 weeks of diagnosis in spite of aggressive antifungal therapy and surgery. To the best of our knowledge, this is the first reported case of rapidly fatal dual infection with Aspergillus and Rhizopus Our case highlights the role of high-dose corticosteroids as a risk factor for invasive fungal disease in patients without traditional risk factors like haematological malignancies, solid organ transplantation or uncontrolled diabetes. 2016 BMJ Publishing Group Ltd.

  5. Rapidly progressive subacute sclerosing panencephalitis presenting with acute loss of vision. (United States)

    Ekici, Bariş; Calişkan, Mine; Tatli, Burak; Aydinli, Nur; Ozmen, Meral


    A 10-year-old male presented with vision loss and behavioral changes. He had midpoint pupils with no reaction to light and normal funduscopic examination. Cranial magnetic resonance imaging revealed bilateral cortical lesions at parieto-occipital lobes. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Despite oral inosiplex and supportive care, patient developed generalized seizures with frequent myoclonic jerks and rapidly progressed into coma. Cortical blindness in subacute sclerosing panencephalitis can be an early indicator for fulminant course.

  6. Rapidly Progressive Encephalopathy: Initial Diagnosis of Creutzfeldt Jakob Disease in an Intensive Care Unit

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    Patrícia Afonso Mendes


    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare, incurable and fatal condition that can only be confirmed through neuropathological investigation, such as brain biopsy or post-mortem study. However, a probable diagnosis can be made using clinical criteria. CJD manifests as rapidly progressive dementia with myoclonus and to a lesser extent visual impairment and cerebellar and pyramidal/extrapyramidal signs. We report the case of a previously independent adult male that met all the clinical criteria. Taken together, the investigation results suggested probable CJD.

  7. Rapid detection of malto-oligosaccharide-forming bacterial amylases by high performance anion-exchange chromatography

    DEFF Research Database (Denmark)

    Duedahl-Olesen, Lene; Larsen, K. L.; Zimmermann, W.


    High performance anion-exchange chromatography with pulsed amperometric detection was applied for the rapid analysis of malto-oligosaccharides formed by extracellular enzyme preparations from 49 starch-degrading bacterial strains isolated from soil and compost samples. Malto-oligosaccharide-formi......High performance anion-exchange chromatography with pulsed amperometric detection was applied for the rapid analysis of malto-oligosaccharides formed by extracellular enzyme preparations from 49 starch-degrading bacterial strains isolated from soil and compost samples. Malto......-oligosaccharide-forming amylases, indicated by a predominant formation of maltohexaose from starch, were produced by enzyme preparations from four of the isolates growing at pH 7.0 and 10....

  8. Rapid changes in gene expression direct rapid shifts in intestinal form and function in the Burmese python after feeding. (United States)

    Andrew, Audra L; Card, Daren C; Ruggiero, Robert P; Schield, Drew R; Adams, Richard H; Pollock, David D; Secor, Stephen M; Castoe, Todd A


    Snakes provide a unique and valuable model system for studying the extremes of physiological remodeling because of the ability of some species to rapidly upregulate organ form and function upon feeding. The predominant model species used to study such extreme responses has been the Burmese python because of the extreme nature of postfeeding response in this species. We analyzed the Burmese python intestine across a time series, before, during, and after feeding to understand the patterns and timing of changes in gene expression and their relationship to changes in intestinal form and function upon feeding. Our results indicate that >2,000 genes show significant changes in expression in the small intestine following feeding, including genes involved in intestinal morphology and function (e.g., hydrolases, microvillus proteins, trafficking and transport proteins), as well as genes involved in cell division and apoptosis. Extensive changes in gene expression occur surprisingly rapidly, within the first 6 h of feeding, coincide with changes in intestinal morphology, and effectively return to prefeeding levels within 10 days. Collectively, our results provide an unprecedented portrait of parallel changes in gene expression and intestinal morphology and physiology on a scale that is extreme both in the magnitude of changes, as well as in the incredibly short time frame of these changes, with up- and downregulation of expression and function occurring in the span of 10 days. Our results also identify conserved vertebrate signaling pathways that modulate these responses, which may suggest pathways for therapeutic modulation of intestinal function in humans. Copyright © 2015 the American Physiological Society.

  9. Imiquimod 5% cream treatment for rapidly progressive genital condyloma in a 3-year-old girl. (United States)

    Leclair, Emily; Black, Amanda; Fleming, Nathalie


    The incidence of genital warts in children has increased in the last 50 years. Although pediatric genital warts may resolve spontaneously, the treatment of extensive perianal genital warts in children can be challenging. Imiquimod, although not approved in the pediatric population, may avoid the pain or extensive scarring associated with other treatment modalities. A 3-year-old female was scheduled for surgical resection of genital warts. At surgery, she had extensive condylomas that had progressed rapidly from initial presentation. They were not amenable to surgical treatment due to concerns of incomplete resection, post-operative pain, and genital scarring. After 6 weeks of imiquimod treatment, the condylomatous lesions had completely resolved with minimal side effects. Imiquimod 5% cream is an effective treatment option for children with extensive and rapidly progressive perianal warts and is associated with minimal side effects. Its use should be considered in children with extensive condyloma in order to avoid the pain and possible scarring associated with other approved treatment modalities. Copyright © 2012 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  10. Rapidly progressive osteoarthritis after arthroscopic labral repair in patients with hip dysplasia. (United States)

    Matsuda, Dean K; Khatod, Monti


    Recent reports of poor clinical outcomes after arthroscopic surgery in hips with marked dysplasia have emerged. Arthroscopic resection of the hypertrophic labrum in cases of dysplasia, especially in the absence of periacetabular osteotomy (PAO), has been implicated. Some patients will refuse PAO because it is a major open procedure, opting for a less invasive arthroscopic procedure. We present the cases of 2 young adults with marked dysplasia who had rapidly progressive osteoarthrosis despite arthroscopic labral repair. Though perhaps beneficial as an isolated procedure in borderline or mild dysplasia cases, arthroscopic hip surgery, even labral repair, may best be performed with PAO in cases with more severe dysplasia. Albeit attractive as a less invasive labral-preserving surgery, arthroscopic labral repair not only may fail to provide symptomatic improvement but may compromise or preclude a later PAO if rapidly progressive osteoarthrosis ensues. Hip arthroscopy may best be performed concurrently with or after PAO but not proceeding PAO in patients requiring both procedures. Copyright © 2012 Arthroscopy Association of North America. Published by Elsevier Inc. All rights reserved.

  11. A rapidly progressive defective spermatogenesis in a Mexican family affected by spino-bulbar muscular atrophy. (United States)

    Piña-Aguilar, Raul Eduardo; Regalado-Hernández, Miguel Ángel; Moreno-García, Jesús Daniel; Buentello-Volante, Beatriz; Chacón-Camacho, Oscar Francisco; Gallegos-Rivas, Mayra Celina; Kazakova, Ekaterina; Santillán-Hernández, Yuritzi; Zenteno, Juan Carlos


    Spino-bulbar muscular atrophy (SBMA) is an X-linked recessive adult progressive disorder affecting motor neurons. It is caused by a poly-glutamine tract expansion in the androgen receptor (AR) which generates protein aggregates that cannot be processed by proteasomes. A secondary mild androgen resistance is developed by AR dysfunction and patients present endocrine abnormalities including gynecomastia and poor function of testosterone in tissues; however, normally they are fertile. In this report we describe a Mexican family with three affected brothers with primary infertility caused by a progressive impairment of spermatogenesis leading to azoospermia before 40 years of age. They presented common features associated to patients affected by SMBA, such as gynecomastia, high level of CPK, muscle cramps, fasciculations, muscle wastage, and impaired swallowing. Two intracytoplasmic sperm injection (ICSI) cycles were performed in one of the patients resulting in fertilization failure. Molecular analysis of AR gene exon 1 revealed 54 CAG repeats in DNA extracted from leukocytes in affected patients and 22 repeats in the fertile non-affected brother. Severe impaired spermatogenesis of rapid progression has not been associated before to SBMA. This is the first report of assisted reproduction techniques indicated by male infertility in patients with this rare disorder. Further studies are required to confirm the unusual result of intracytoplasmic sperm injection cycles. We discuss the implications and possible pathogenesis of these unique features of SBMA in this family.

  12. Activation of Notch3 in Glomeruli Promotes the Development of Rapidly Progressive Renal Disease. (United States)

    El Machhour, Fala; Keuylian, Zela; Kavvadas, Panagiotis; Dussaule, Jean-Claude; Chatziantoniou, Christos


    Notch3 expression is found in the glomerular podocytes of patients with lupus nephritis or focal segmental GN but not in normal kidneys. Here, we show that activation of the Notch3 receptor in the glomeruli is a turning point inducing phenotypic changes in podocytes promoting renal inflammation and fibrosis and leading to disease progression. In a model of rapidly progressive GN, Notch3 expression was induced by several-fold in podocytes concurrently with disease progression. By contrast, mice lacking Notch3 expression were protected because they exhibited less proteinuria, uremia, and inflammatory infiltration. Podocyte outgrowth from glomeruli isolated from wild-type mice during the early phase of the disease was higher than outgrowth from glomeruli of mice lacking Notch3. In vitro studies confirmed that podocytes expressing active Notch3 reorganize their cytoskeleton toward a proliferative/migratory and inflammatory phenotype. We then administered antisense oligodeoxynucleotides targeting Notch3 or scramble control oligodeoxynucleotides in wild-type mice concomitant to disease induction. Both groups developed chronic renal disease, but mice injected with Notch3 antisense had lower values of plasma urea and proteinuria and inflammatory infiltration. The improvement of renal function was accompanied by fewer deposits of fibrin within the glomeruli and by decreased peritubular inflammation. Finally, abnormal Notch3 staining was observed in biopsy samples of patients with crescentic GN. These results demonstrate that abnormal activation of Notch3 may be involved in the progression of renal disease by promoting migratory and proinflammatory pathways. Inhibiting Notch3 activation could be a novel, promising approach to treat GN. Copyright © 2015 by the American Society of Nephrology.

  13. An unusual case of rapidly progressive contractures: Case report and brief review

    Directory of Open Access Journals (Sweden)

    Subasree R


    Full Text Available An 8-year-old boy, diagnosed as cervical dystonia, was referred to our tertiary center. After a trivial trauma he had developed painful lumps in the axial region, which was followed by restricted movements of neck, shoulder, and abdominal muscles over 4 months. He had kyphoscoliosis, torticollis, rigid abdomen, and multiple muscle contractures. He also had short great toes. A detailed skeletal survey showed calcification in the soft tissues surrounding the shoulder anterior chest wall, thorax, and paraspinal muscles; there was also beaking of vertebrae, which was confirmed by CT thorax. This report showcases the diagnostic challenge posed by myositis ossificans progressiva, which can rarely cause rapidly progressing muscle contractures. A brief review of literature is also presented.

  14. Non-inflammatory cerebral amyloid angiopathy as a cause of rapidly progressive dementia: A case study

    Directory of Open Access Journals (Sweden)

    Leonel Tadao Takada

    Full Text Available Abstract A 77 year-old men developed a subacute-onset, rapidly progressive cognitive decline. After 6 months of evolution, he scored 6 on the Mini-Mental State Examination and had left hemiparesis and hemineglect. The patient died 11 months after the onset of cognitive symptoms. Brain MRI showed microhemorrhages on gradient-echo sequence and confluent areas of white matter hyperintensities on T2-weighted images. Brain biopsy revealed amyloid-b peptide deposition in vessel walls, some of them surrounded by micro-bleeds. In this case report, we discuss the role of cerebral amyloid angiopathy (CAA in cognitive decline, due to structural lesions associated with hemorrhages and infarcts, white matter lesions and co-morbidity of Alzheimer's disease, as well as the most recently described amyloid angiopathy-related inflammation.

  15. Male patients presenting with rapidly progressive puberty associated with malignant tumors

    Directory of Open Access Journals (Sweden)

    Soo Jung Kim


    Full Text Available In males, precocious puberty (PP is defined as the development of secondary sexual characteristics before age 9 years. PP is usually idiopathic; though, organic abnormalities including tumors are more frequently found in male patients with PP. However, advanced puberty in male also can be an important clinical manifestation in tumors. We report 2 cases of rapidly progressive puberty in males, each associated with a germ-cell tumor. First, an 11-year-old boy presented with mild fever and weight loss for 1 month. Physical examination revealed a pubertal stage of G3P3 with 10-mL testes. Investigations revealed advanced bone age (16 years with elevated basal luteinizing hormone and testosterone levels. An anterior mediastinal tumor was identified by chest radiography and computed tomography, and elevated α-fetoprotein (AFP and β-human chorionic gonadotropin (β-hCG levels were noted. Histopathologic analysis confirmed a yolk-sac tumor. Second, a 12-year-old boy presented with diplopia, polydipsia, and polyuria for 4 months. Physical examination revealed a pubertal stage of G3P3 with 8-mL testes. Bone age was advanced (16 years and laboratory tests indicated panhypopituitarism with elevated testosterone level. A mixed germ-cell tumor was diagnosed with elevated AFP and β-hCG levels. Of course, these patients also have other symptoms of suspecting tumors, however, rapidly progressive puberty can be the more earlier screening sign of tumors. Therefore, in male patients with accelerated or advanced puberty, malignancy should be considered, with evaluation of tumor markers. In addition, advanced puberty in male should be recognized more widely as a unique sign of neoplasm.

  16. Chimeric antigen receptor T cells form nonclassical and potent immune synapses driving rapid cytotoxicity. (United States)

    Davenport, A J; Cross, R S; Watson, K A; Liao, Y; Shi, W; Prince, H M; Beavis, P A; Trapani, J A; Kershaw, M H; Ritchie, D S; Darcy, P K; Neeson, P J; Jenkins, M R


    Chimeric antigen receptor T (CAR-T) cells are effective serial killers with a faster off-rate from dying tumor cells than CAR-T cells binding target cells through their T cell receptor (TCR). Here we explored the functional consequences of CAR-mediated signaling using a dual-specific CAR-T cell, where the same cell was triggered via TCR (tcrCTL) or CAR (carCTL). The carCTL immune synapse lacked distinct LFA-1 adhesion rings and was less reliant on LFA to form stable conjugates with target cells. carCTL receptors associated with the synapse were found to be disrupted and formed a convoluted multifocal pattern of Lck microclusters. Both proximal and distal receptor signaling pathways were induced more rapidly and subsequently decreased more rapidly in carCTL than in tcrCTL. The functional consequence of this rapid signaling in carCTL cells included faster lytic granule recruitment to the immune synapse, correlating with faster detachment of the CTL from the target cell. This study provides a mechanism for how CAR-T cells can debulk large tumor burden quickly and may contribute to further refinement of CAR design for enhancing the quality of signaling and programming of the T cell. Copyright © 2018 the Author(s). Published by PNAS.

  17. Semi-solid dosage form of clonazepam for rapid oral mucosal absorption. (United States)

    Sakata, Osamu; Machida, Yoshiharu; Onishi, Hiraku


    In order to obtain an alternative to the intravenous (i.v.) dosage form of clonazepam (CZ), an oral droplet formulation of CZ was developed previously; however, the droplet was physically unstable. Therefore, in the present study, it was attempted to develop an easily-handled dosage form, which was more physically stable and allowed rapid drug absorption from oral mucosa. A semi-solid dosage form, composed of polyethylene glycol 1500 (PEG), CZ, and oleic acid (OA) at 37/1/2 (w/w) and named PEG/CZ/OA, and a semi-solid dosage form containing PEG and CZ at 39/1 (w/w), called PEG/CZ, were prepared. Their physical stability in air at room temperature and oral mucosal absorption in rats were investigated. The semi-solid dosage forms were much more stable physically than the droplet, that is, no recrystallization of CZ was observed for at least 8 days. The effective concentration for humans and rats (20 ng/mL or more) was achieved within 30 min after buccal administration for both PEG/CZ/OA and PEG/CZ. The plasma concentration increased gradually and less varied at each time point for PEG/CZ/OA. PEG/CZ/OA was found to show more rapid and higher absorption of CZ in buccal administration than in sublingual administration. Buccal administration with the semi-solid dosage PEG/CZ with or without OA was suggested to be a possibly useful novel dosage form as an alternative to i.v. injection.

  18. Rapid self-healable poly(ethylene glycol) hydrogels formed by selective metal-phosphate interactions. (United States)

    Sato, Takeshi; Ebara, Mitsuhiro; Tanaka, Shinji; Asoh, Taka-Aki; Kikuchi, Akihiko; Aoyagi, Takao


    Rapid self-healable and biocompatible hydrogels were prepared using the selective formation of metal-ligand interactions between selected metal ions and phosphate end groups of poly(ethylene glycol) (PEG). The phosphate-terminated branch of PEG was synthesized via a substitution reaction of the hydroxyl end groups using phosphoryl chloride. The gelation and gel properties including rheological properties can be tuned by the careful selection of metal ions, branch numbers, and temperature. Especially, the gels rapidly formed by trivalent metal ions such as Fe(3+), V(3+), Al(3+), Ti(3+), and Ga(3+) have relatively small ionic radii. The ligand substitution rates also affected the repeatable autonomic healing ability. We have also demonstrated a gel-sol/sol-gel transition by switching the redox states of Fe(3+)/Fe(2+) ions. Learning from biological systems, the proposed phosphate-metal ion based self-healable hydrogels could become an attractive candidate for various biomedical and environmental applications.

  19. Amorphous salts formed from rapid dehydration of multicomponent chloride and ferric sulfate brines: Implications for Mars (United States)

    Sklute, Elizabeth C.; Rogers, A. Deanne; Gregerson, Jason C.; Jensen, Heidi B.; Reeder, Richard J.; Dyar, M. Darby


    Salts with high hydration states have the potential to maintain high levels of relative humidity (RH) in the near subsurface of Mars, even at moderate temperatures. These conditions could promote deliquescence of lower hydrates of ferric sulfate, chlorides, and other salts. Previous work on deliquesced ferric sulfates has shown that when these materials undergo rapid dehydration, such as that which would occur upon exposure to present day Martian surface conditions, an amorphous phase forms. However, the fate of deliquesced halides or mixed ferric sulfate-bearing brines are presently unknown. Here we present results of rapid dehydration experiments on Ca-, Na-, Mg- and Fe-chloride brines and multicomponent (Fe2(SO4)3 ± Ca, Na, Mg, Fe, Cl, HCO3) brines at ∼21 °C, and characterize the dehydration products using visible/near-infrared (VNIR) reflectance spectroscopy, mid-infrared attenuated total reflectance spectroscopy, and X-ray diffraction (XRD) analysis. We find that rapid dehydration of many multicomponent brines can form amorphous solids or solids with an amorphous component, and that the presence of other elements affects the persistence of the amorphous phase under RH fluctuations. Of the pure chloride brines, only Fe-chloride formed an amorphous solid. XRD patterns of the multicomponent amorphous salts show changes in position, shape, and magnitude of the characteristic diffuse scattering observed in all amorphous materials that could be used to help constrain the composition of the amorphous salt. Amorphous salts deliquesce at lower RH values compared to their crystalline counterparts, opening up the possibility of their role in potential deliquescence-related geologic phenomena such as recurring slope lineae (RSLs) or soil induration. This work suggests that a wide range of aqueous mixed salt solutions can lead to the formation of amorphous salts and are possible for Mars; detailed studies of the formation mechanisms, stability and transformation

  20. Rapidly progressive polyneuropathy due to dry beriberi in a man: a case report

    Directory of Open Access Journals (Sweden)

    Lekwuwa Godwin


    Full Text Available Abstract Introduction We describe a case of rapidly progressive and severely debilitating polyneuropathy in a patient with confirmed hypovitaminosis B1, consistent with dry beriberi. Crucially, this is a treatable condition, although sometimes with incomplete recovery, but it is probably under-recognized yet increasingly common given increasing levels of alcohol abuse in the western world. Case presentation A 49-year-old Caucasian British man presented with progressive weakness of both lower limbs of approximately seven months' duration. He noted difficulty climbing stairs. He also complained of lethargy, and loss of muscle bulk, including his thighs. He had a history of type 2 diabetes mellitus and admitted prior alcohol abuse but denied excessive alcohol intake in the five years prior to presentation. Initial clinical and neurophysiological examinations were consistent with a mild peripheral neuropathy and probable proximal myopathy. However, over the subsequent four months he evolved a marked tetraparesis, with profound sensory disturbance of all limbs. Repeat neurophysiology revealed a widespread polyneuropathy with extensive acute and sub-acute denervation changes in all four limbs, and reduced or absent sensory nerve action potentials. Hypovitaminosis B1 was confirmed (45 nmol/L, reference range 66-200 nmol/L. His rapid clinical deterioration was in keeping with dry beriberi. He was treated with thiamine. Subsequent follow-up revealed slow but significant improvement, such that by 15-16 months from the initial onset of symptoms, and approximately six months after the onset of his marked tetraparesis, he was able to stand independently and was gradually gaining confidence in walking pending a period of in-patient neurorehabilitation. Conclusion A potentially wide differential diagnosis exists for this type of presentation. Confirming hypovitaminosis B1 by requesting the assay prior to vitamin replacement ensures accurate diagnosis and

  1. Critical Role for Monocytes/Macrophages in Rapid Progression to AIDS in Pediatric Simian Immunodeficiency Virus-Infected Rhesus Macaques. (United States)

    Sugimoto, Chie; Merino, Kristen M; Hasegawa, Atsuhiko; Wang, Xiaolei; Alvarez, Xavier A; Wakao, Hiroshi; Mori, Kazuyasu; Kim, Woong-Ki; Veazey, Ronald S; Didier, Elizabeth S; Kuroda, Marcelo J


    Infant humans and rhesus macaques infected with the human or simian immunodeficiency virus (HIV or SIV), respectively, express higher viral loads and progress more rapidly to AIDS than infected adults. Activated memory CD4(+) T cells in intestinal tissues are major primary target cells for SIV/HIV infection, and massive depletion of these cells is considered a major cause of immunodeficiency. Monocytes and macrophages are important cells of innate immunity and also are targets of HIV/SIV infection. We reported previously that a high peripheral blood monocyte turnover rate was predictive for the onset of disease progression to AIDS in SIV-infected adult macaques. The purpose of this study was to determine if earlier or higher infection of monocytes/macrophages contributes to the more rapid progression to AIDS in infants. We observed that uninfected infant rhesus macaques exhibited higher physiologic baseline monocyte turnover than adults. Early after SIV infection, the monocyte turnover further increased, and it remained high during progression to AIDS. A high percentage of terminal deoxynucleotidyltransferase dUTP nick end label (TUNEL)-positive macrophages in the lymph nodes (LNs) and intestine corresponded with an increasing number of macrophages derived from circulating monocytes (bromodeoxyuridine positive [BrdU(+)] CD163(+)), suggesting that the increased blood monocyte turnover was required to rapidly replenish destroyed tissue macrophages. Immunofluorescence analysis further demonstrated that macrophages were a significant portion of the virus-producing cells found in LNs, intestinal tissues, and lungs. The higher baseline monocyte turnover in infant macaques and subsequent macrophage damage by SIV infection may help explain the basis of more rapid disease progression to AIDS in infants.IMPORTANCE HIV infection progresses much more rapidly in pediatric cases than in adults; however, the mechanism for this difference is unclear. Using the rhesus macaque model

  2. HPLC method for rapidly following biodiesel fuel transesterification reaction progress using a core-shell column

    Energy Technology Data Exchange (ETDEWEB)

    Allen, Samuel J.; Ott, Lisa S. [California State University, Chico, CA (United States)


    There are a wide and growing variety of feedstocks for biodiesel fuel. Most commonly, these feedstocks contain triglycerides which are transesterified into the fatty acid alkyl esters (FAAEs) which comprise biodiesel fuel. While the tranesterification reaction itself is simple, monitoring the reaction progress and reaction products is not. Gas chromatography-mass spectrometry is useful for assessing the FAAE products, but does not directly address either the tri-, di-, or monoglycerides present from incomplete transesterification or the free fatty acids which may also be present. Analysis of the biodiesel reaction mixture is complicated by the solubility and physical property differences among the components of the tranesterification reaction mixture. In this contribution, we present a simple, rapid HPLC method which allows for monitoring all of the main components in a biodiesel fuel transesterification reaction, with specific emphasis on the ability to monitor the reaction as a function of time. The utilization of a relatively new, core-shell stationary phase for the HPLC column allows for efficient separation of peaks with short elution times, saving both time and solvent. (orig.)

  3. A rapidly progressing Pancoast syndrome due to pulmonary mucormycosis: a case report

    Directory of Open Access Journals (Sweden)

    Hiatt Kim M


    Full Text Available Abstract Introduction Pancoast syndrome is characterized by Horner syndrome, shoulder pain radiating down the arm, compression of the brachial blood vessels, and, in long-standing cases, atrophy of the arm and hand muscles. It is most commonly associated with lung carcinoma but rarely is seen with certain infections. Case presentation We present the case of a 51-year-old Caucasian man who had acute myeloid leukemia and who developed a rapidly fulminating pneumonia along with signs and symptoms of acute brachial plexopathy and left Horner syndrome. Also, a purpuric plaque developed over his left chest wall and progressed to skin necrosis. The skin biopsy and bronchoalveolar lavage showed a Rhizopus species, leading to a diagnosis of mucormycosis. This is a rare case of pneumonia due to mucormycosis associated with acute Pancoast syndrome. Conclusions According to our review of the literature, only a few infectious agents have been reported to be associated with Pancoast syndrome. We found only three case reports of mucormycosis associated with acute Pancoast syndrome. Clinicians should consider mucormycosis in their differential diagnosis in a patient with pulmonary lesions and chest wall invasion with or without neurological symptoms, especially in the setting of neutropenia or other immunosuppressed conditions. It is important to recognize this condition early in order to target therapy and interventions.

  4. Compact form fitting small antennas using three-dimensional rapid prototyping (United States)

    Willis, Bryan Jon

    Three-dimensional (3D) rapid prototyping holds significant promise for future antenna designs. Many complex designs that would be unmanufacturable or costly are realizable on a 3D printing machine. The ability to create 3D designs of virtually any configuration makes it possible to build compact antennas that can form fit to any space. These antennas build on the concept that small antennas can best reach the ideal operating limit when utilizing the entire 3D space in a sphere surrounding the antenna. Antennas require a combination of dielectric and conductive materials. 3D rapid prototyping is already well advanced for plastics and dielectric materials (with more options coming online). Prototyping with conductive materials has lagged behind; due mainly to their higher melting points, but this is advancing as well. This dissertation focuses on 3D rapid prototyping for antenna design. A 3D antenna made from small cubical cells is optimized for 2.4--3GHz using a genetic algorithm (GA). The antennas are built using 3D printing of plastic covered by conductive paint. The effects of the conductivity of the paint and number of layers on the resonance and gain of the antenna are evaluated. These results demonstrate the feasibility of using 3D rapid prototyping for antenna design. A 3D dipole is also optimized using a GA to function from 510--910MHz. The antenna was built using 3D rapid prototyping from plastic. The 3D antenna was covered with a conductive coating and measured, showing good agreement with simulation. The 3D GA is used to design 3D antennas of random shape to fit inside the empty space in a cell phone case and optimized for cell phone bands 800--900MHz and 1.6--3.7GHz. The research also evaluates methods and materials that can be used to produce 3D antennas. In addition to the flexibility that 3D prototyping brings to antenna design, this paper describes how this new and emerging method for building antennas can provide fast and affordable antennas for

  5. Structural and compositional properties of CZTS thin films formed by rapid thermal annealing of electrodeposited layers (United States)

    Lehner, J.; Ganchev, M.; Loorits, M.; Revathi, N.; Raadik, T.; Raudoja, J.; Grossberg, M.; Mellikov, E.; Volobujeva, O.


    In this work Cu2ZnSnS4 (CZTS) thin films were formed by rapid thermal annealing (RTA) of sequentially electrodeposited Cu-Zn and Sn films in 5% H2S containing atmosphere. Six different thermal profiles were used in the experiments. In three of these, the temperature ramping up was varied, while the variable in the other three profiles was the cooling down rate. The optimising parameters for RTA of electrodeposited films were found and annealed films were characterised by X-ray diffraction (XRD), micro-Raman spectroscopy, scanning electron microscopy and energy dispersive X-ray spectroscopy (SEM+EDS). The material parameters such as lattice strain and crystallite size were also determined and the influence of annealing temperature and heating rate on these parameters was discussed.The pathway of MoS2 formation was investigated.

  6. A Rapid Determination of Cinnarizine in Bulk and Pharmaceutical Dosage Form by LC

    Directory of Open Access Journals (Sweden)

    A. A. Heda


    Full Text Available A simple, selective, rapid and precise reverse phase high pressure liquid chromatographic method has been developed for the estimation of cinnarizine from pharmaceutical formulation. The method was developed using MICRA-NPS C18 (length×OD×ID =33×8.0×6.0 mm, 1.5 μm column with a mobile phase consisting of acetonitrile, triethylamine buffer (adjusted to pH 4.5 with 10% w/v potassium hydroxide and tetrahydrofuran in the ratio 30:66:4 respectively, at a flow rate of 0.5 mL/min. Wavelength was fixed at 253 nm. The developed method was validated for linearity, accuracy, precision, limit of detection and limit of quantitation. The proposed method can be used for the routine estimation of cinnarizine in pharmaceutical dosage form.

  7. Injury Markers but not Amyloid Markers are Associated with Rapid Progression from Mild Cognitive Impairment to Dementia in Alzheimer's Disease

    NARCIS (Netherlands)

    van Rossum, I.A.; Visser, P.J.; Knol, D.L.; van der Flier, W.M.; Teunissen, C.E.; Barkhof, F.; Blankenstein, M.A.; Scheltens, P.


    Alzheimer's disease (AD) is a common cause of mild cognitive impairment (MCI). However, the time between the diagnosis of MCI and the diagnosis of dementia is highly variable. In this study we investigated which known risk factors and biomarkers of AD pathology were associated with rapid progression

  8. Cortical plasticity induced by rapid Hebbian learning of novel tonal word-forms : Evidence from mismatch negativity

    NARCIS (Netherlands)

    Yue, Jinxing; Bastiaanse, Roelien; Alter, Kai


    Although several experiments reported rapid cortical plasticity induced by passive exposure to novel segmental patterns, few studies have devoted attention to the neural dynamics during the rapid learning of novel tonal word-forms in tonal languages, such as Chinese. In the current study, native

  9. Pseudotumoral form of primary progressive tuberculosis: a diagnosis to be considered

    Directory of Open Access Journals (Sweden)

    Franco Rosana


    Full Text Available The diversity of clinical presentations of primary progressive tuberculosis (TB and the difficulty in establishing the diagnosis of paucibacillary forms is the subject of painstaking research, as well as a cause of delay in therapy. We report the case of a 10-year-old black child who presented with chest pain and progressive widening of the upper mediastinum. Computerized tomography of the chest revealed multiple calcifications that were not identified with X-rays. Biopsy through mediastinoscopy was compatible with a diagnosis of tuberculosis. Despite exhaustive investigation that included direct examination, culture for mycobacteria and PCR (Polymerase Chain Reaction of tissue samples, the etiologic agent was not revealed. Tuberculin conversion was observed during the follow-up and resolution period of the lesion, after administration of isoniazid, rifampicin and pyrazinamide. The nodal pseudotumoral form of tuberculosis is rare in immunocompetent children and it may simulate neoplastic disease; therefore, it should be included in the list of differential diagnoses of masses located in the anterosuperior mediastinum.

  10. A novel form of progressive retinal atrophy in Swedish vallhund dogs.

    Directory of Open Access Journals (Sweden)

    Ann E Cooper

    Full Text Available Inherited retinal degenerations, such as retinitis pigmentosa (RP and age-related macular degeneration (AMD, represent leading causes of incurable blindness in humans. This is also true in dogs, where the term progressive retinal atrophy (PRA is used to describe inherited photoreceptor degeneration resulting in progressive vision loss. Because of the similarities in ocular anatomy, including the presence of a cone photoreceptor-rich central retinal region, and the close genotype-phenotype correlation, canine models contribute significantly to the understanding of retinal disease mechanisms and the development of new therapies. The screening of the pure-bred dog population for new forms of PRA represents an important strategy to establish new large animal models. By examining 324 dogs of the Swedish vallhund breed in seven countries and across three continents, we were able to describe a new and unique form of PRA characterized by the multifocal appearance of red and brown discoloration of the tapetal fundus followed over time by thinning of the retina. We propose three stages of the disease based on the appearance of the ocular fundus and associated visual deficits. Electroretinography revealed a gradual loss of both rod and cone photoreceptor-mediated function in Stages 2 and 3 of the disease. In the few dogs that suffered from pronounced vision loss, night-blindness occurred first in late Stage 2, followed by decreased day-vision in Stage 3. Histologic examinations confirmed the loss of photoreceptor cells at Stage 3, which was associated with the accumulation of autofluorescent material in the adjacent retinal pigment epithelium. Pedigree analysis was suggestive of an autosomal-recessive mode of inheritance. Mutations in six known canine retinal degeneration genes as well as hypovitaminosis E were excluded as causes of the disease. The observed variability in the age of disease onset and rate of progression suggest the presence of genetic and

  11. A novel form of progressive retinal atrophy in Swedish vallhund dogs. (United States)

    Cooper, Ann E; Ahonen, Saija; Rowlan, Jessica S; Duncan, Alison; Seppälä, Eija H; Vanhapelto, Päivi; Lohi, Hannes; Komáromy, András M


    Inherited retinal degenerations, such as retinitis pigmentosa (RP) and age-related macular degeneration (AMD), represent leading causes of incurable blindness in humans. This is also true in dogs, where the term progressive retinal atrophy (PRA) is used to describe inherited photoreceptor degeneration resulting in progressive vision loss. Because of the similarities in ocular anatomy, including the presence of a cone photoreceptor-rich central retinal region, and the close genotype-phenotype correlation, canine models contribute significantly to the understanding of retinal disease mechanisms and the development of new therapies. The screening of the pure-bred dog population for new forms of PRA represents an important strategy to establish new large animal models. By examining 324 dogs of the Swedish vallhund breed in seven countries and across three continents, we were able to describe a new and unique form of PRA characterized by the multifocal appearance of red and brown discoloration of the tapetal fundus followed over time by thinning of the retina. We propose three stages of the disease based on the appearance of the ocular fundus and associated visual deficits. Electroretinography revealed a gradual loss of both rod and cone photoreceptor-mediated function in Stages 2 and 3 of the disease. In the few dogs that suffered from pronounced vision loss, night-blindness occurred first in late Stage 2, followed by decreased day-vision in Stage 3. Histologic examinations confirmed the loss of photoreceptor cells at Stage 3, which was associated with the accumulation of autofluorescent material in the adjacent retinal pigment epithelium. Pedigree analysis was suggestive of an autosomal-recessive mode of inheritance. Mutations in six known canine retinal degeneration genes as well as hypovitaminosis E were excluded as causes of the disease. The observed variability in the age of disease onset and rate of progression suggest the presence of genetic and/or environmental

  12. Rapid Progression of Metastatic Pulmonary Calcification and Alveolar Hemorrhage in a Patient with Chronic Renal Failure and Primary Hyperparathyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Eun Joo; Kim, Dong Hun; Yoon, Seong Ho [Chosun University College of Medicine, Gwangju (Korea, Republic of); Suk, Eun Ha [Dept. of Anaesthesiology and Pain Medicine, Seonam University College of Medicine, Namwon (Korea, Republic of)


    Metastatic pulmonary calcification (MPC) is common in patients with chronic renal failure. The authors experienced a patient with chronic renal failure and primary hyperparathyroidism by parathyroid adenoma accompanied with rapid progressions of MPC and alveolar hemorrhage. Recent chest radiographs, compared with previous chest radiographs, showed rapid accumulation of calcification in both upper lungs. Following up on the high-resolution CT scan after five years demonstrates more increased nodules in size and ground glass opacity. The patient was diagnosed with MPC and alveolar hemorrhage by transbronchial lung biopsy. We assumed rapid progression of MPC and alveolar hemorrhage in underlying chronic renal failures could be a primary hyperparathyroidism which may be caused by parathyroid adenoma detected incidentally. Therefore parathyroid adenoma was treated with ethanol injections. Herein, we have reported on CT findings of MPC with alveolar hemorrhage and reviewed our case along with other articles.

  13. Neurocognitive features distinguishing primary central nervous system lymphoma from other possible causes of rapidly progressive dementia. (United States)

    Deutsch, Mariel B; Mendez, Mario F


    Define the neurocognitive features of primary central nervous system lymphoma (PCNSL) presenting with dementia, and compare with other causes of rapidly progressive dementia (RPD). PCNSL can present as an RPD. Differentiating PCNSL from other RPDs is critical because lymphomatous dementia may be reversible, and untreated PCNSL is fatal. We performed a meta-analysis of case reports of dementia from PCNSL (between 1950 and 2013); 20 patients (14 with lymphomatosis cerebri) met our criteria. We compared these patients to a case series of patients with RPD from Creutzfeldt-Jakob disease and other non-PCNSL etiologies (Sala et al, 2012. Alzheimer Dis Assoc Disord. 26:267-271). Median age was 66 years (range 41 to 81); 70% were men. Time from symptom onset to evaluation was <6 months in 65%. No patients had seizures; 5% had headaches; 45% had non-aphasic speech difficulty. There was significantly more memory impairment in patients with PCNSL than other RPDs and significantly less myoclonus and parkinsonism. Behavioral changes and cerebellar signs were not significantly different. Significantly more patients with PCNSL than other RPDs had white matter changes; significantly fewer had atrophy. Elevated CSF protein and pleocytosis were more frequent in PCNSL; patients with other RPDs tended to have normal CSF±14-3-3 protein. Unlike patients with RPD from other causes, those with PCNSL commonly present with impaired memory, apathy, and abnormal speech and gait, without headache, seizure, or myoclonus. White matter changes and CSF abnormalities predominate. Improved clinical awareness of PCNSL can prompt earlier diagnosis and treatment.

  14. Simulation of Microstructure during Laser Rapid Forming Solidification Based on Cellular Automaton

    Directory of Open Access Journals (Sweden)

    Zhi-jian Wang


    Full Text Available The grain microstructure of molten pool during the solidification of TC4 titanium alloy in the single point laser cladding was investigated based on the CAFE model which is the cellular automaton (CA coupled with the finite element (FE method. The correct temperature field is the prerequisite for simulating the grain microstructure during the solidification of the molten pool. The model solves the energy equation by the FE method to simulate the temperature distribution in the molten pool of the single point laser cladding. Based on the temperature field, the solidification microstructure of the molten pool is also simulated with the CAFE method. The results show that the maximum temperature in the molten pool increases with the laser power and the scanning rate. The laser power has a larger influence on the temperature distribution of the molten pool than the scanning rate. During the solidification of the molten pool, the heat at the bottom of the molten pool transfers faster than that at the top of the molten pool. The grains rapidly grow into the molten pool, and then the columnar crystals are formed. This study has a very important significance for improving the quality of the structure parts manufactured through the laser cladding forming.

  15. Clinical assessment of a customized free-form progressive add lens spectacle. (United States)

    Han, Susan C; Graham, Andrew D; Lin, Meng C


    To determine whether there are significant differences in standard clinical measures of vision, progressive addition lens (PAL)-specific vision tests, or subjective ratings and preferences between customized free-form and standard non-free-form PALs in an experienced wearing population. In addition, we aim to determine whether subjective or objective clinical outcomes depend on demographic, PAL usage, spectacle prescription, or frame fitting characteristics. In a randomized, double-masked cross-over trial, 95 experienced wearers wore Zeiss Individual customized free-form PAL spectacles (test) and standard non-free-form PAL spectacles (control) for 1 week each. At dispensing and after 1 week of wear, subjects were tested for distance and near visual acuity under both high and low contrast; in addition, 30° off-axis visual acuity was measured using a novel apparatus, as was the horizontal extent of clear, undistorted vision at reading distance. Subjects also completed a set of questionnaires detailing their satisfaction levels, adaptation times, and preferences for test or control spectacles for different visual tasks. The test spectacles were preferred overall and for distance, midrange, transitional and active vision, and rated higher in overall satisfaction (p = 0.006). There were no clinically important differences between test and control spectacles in standard clinical vision assessments. In the PAL-specific assessments, however, the horizontal extent of clear vision at reading distance was significantly greater with the test spectacles (p = 0.004). There were statistically significant preferences for the optically customized free-form lenses over the non-free-form lenses. Subjects also reported a wider field of undistorted vision when looking through the reading zone of the test spectacles. Although standard clinical vision assessments are not sufficiently refined to detect important objective differences between the spectacle types, customization taking into

  16. Rapid solidification of an Al-5Ni alloy processed by spray forming

    Directory of Open Access Journals (Sweden)

    Conrado Ramos Moreira Afonso


    Full Text Available Recently, intermetallic compounds have attracted much attention due to their potential technological applications as high-temperature materials. In particular the intermetallic compounds, associated with the Al-Ni binary system stand out as promising candidates for high-temperature materials for the use in harsh environments. It is expected that a bulk Al-Ni alloy may exceed the strength of many commercial materials. The great challenge in developing these alloys is to manipulate the solidification thermal parameters in order to obtain the desired microstructural features. One of the indicated routes to obtain very refined intermetallic phases dispersed in the microstructure is the spray forming process. The dendritic and eutectic growth dependences on cooling rate are already known for directionally solidified (DS hypoeutectic Al-Ni alloys. In the case of rapidly solidified (RS samples, extrapolations of such experimental laws are needed, which can be very helpful to estimate realistic values of high cooling rates imposed during the spray forming process. The present study aims to compare directionally solidified and spray-formed Al-5wt. (%Ni alloy samples with a view to providing a basis for understanding how to control solidification parameters and the as-cast microstructure. The Al-5.0wt. (%Ni alloy was shown to have a cellular morphology for the overspray powder size range examined (up to 500 µm. The mean cell spacing decreased from 5.0 to 1.1 µm with the decrease in the powder average diameter. It was found that the experimental cooling rates imposed during the atomization step of the overspray powder solidification varied from 10³ to 2.10(4 K/s. The DSC trace depicted a crystallization peak of an amorphous structure fraction in the smallest Al-5.0wt. (%Ni alloy powder size range (<32 µm estimating a 15 µm critical diameter of amorphous powder in the binary Al97.5Ni2.5 (at% alloy.

  17. Connexin 43 astrocytopathy linked to rapidly progressive multiple sclerosis and neuromyelitis optica.

    Directory of Open Access Journals (Sweden)

    Katsuhisa Masaki

    Full Text Available BACKGROUND: Multiple sclerosis (MS and neuromyelitis optica (NMO occasionally have an extremely aggressive and debilitating disease course; however, its molecular basis is unknown. This study aimed to determine a relationship between connexin (Cx pathology and disease aggressiveness in Asian patients with MS and NMO. METHODS/PRINCIPAL FINDINGS: Samples included 11 autopsied cases with NMO and NMO spectrum disorder (NMOSD, six with MS, and 20 with other neurological diseases (OND. Methods of analysis included immunohistochemical expression of astrocytic Cx43/Cx30, oligodendrocytic Cx47/Cx32 relative to AQP4 and other astrocytic and oligodendrocytic proteins, extent of demyelination, the vasculocentric deposition of complement and immunoglobulin, and lesion staging by CD68 staining for macrophages. Lesions were classified as actively demyelinating (n=59, chronic active (n=58 and chronic inactive (n=23. Sera from 120 subjects including 30 MS, 30 NMO, 40 OND and 20 healthy controls were examined for anti-Cx43 antibody by cell-based assay. Six NMO/NMOSD and three MS cases showed preferential loss of astrocytic Cx43 beyond the demyelinated areas in actively demyelinating and chronic active lesions, where heterotypic Cx43/Cx47 astrocyte oligodendrocyte gap junctions were extensively lost. Cx43 loss was significantly associated with a rapidly progressive disease course as six of nine cases with Cx43 loss, but none of eight cases without Cx43 loss regardless of disease phenotype, died within two years after disease onset (66.7% vs. 0%, P=0.0090. Overall, five of nine cases with Cx43 loss and none of eight cases without Cx43 loss had distal oligodendrogliopathy characterized by selective myelin associated glycoprotein loss (55.6% vs. 0.0%, P=0.0296. Loss of oligodendrocytic Cx32 and Cx47 expression was observed in most active and chronic lesions from all MS and NMO/NMOSD cases. Cx43-specific antibodies were absent in NMO/NMOSD and MS patients. CONCLUSIONS

  18. Spray forming -- Aluminum: Third annual report (Phase 2). Technical progress -- Summary

    Energy Technology Data Exchange (ETDEWEB)

    Kozarek, R.L.


    Commercial production of aluminum sheet and plate by spray atomization and deposition is a potentially attractive manufacturing alternative to conventional ingot metallurgy/hot-milling and to continuous casting processes because of reduced energy requirements and reduced cost. To realize the full potential of the technology, the Aluminum Company of America (Alcoa), under contract by the US Department of Energy, is investigating currently available state-of-the-art atomization devices to develop nozzle design concepts whose spray characteristics are tailored for continuous sheet production. This third technical progress report will summarize research and development work conducted during the period 1997 October through 1998 March. Included are the latest optimization work on the Alcoa III nozzle, results of spray forming runs with 6111 aluminum alloy and preliminary rolling trials of 6111 deposits.

  19. Setting Mechanical Properties of High Strength Steels for Rapid Hot Forming Processes. (United States)

    Löbbe, Christian; Hering, Oliver; Hiegemann, Lars; Tekkaya, A Erman


    Hot stamping of sheet metal is an established method for the manufacturing of light weight products with tailored properties. However, the generally-applied continuous roller furnace manifests two crucial disadvantages: the overall process time is long and a local setting of mechanical properties is only feasible through special cooling techniques. Hot forming with rapid heating directly before shaping is a new approach, which not only reduces the thermal intervention in the zones of critical formability and requested properties, but also allows the processing of an advantageous microstructure characterized by less grain growth, additional fractions (e.g., retained austenite), and undissolved carbides. Since the austenitization and homogenization process is strongly dependent on the microstructure constitution, the general applicability for the process relevant parameters is unknown. Thus, different austenitization parameters are analyzed for the conventional high strength steels 22MnB5, Docol 1400M, and DP1000 in respect of the mechanical properties. In order to characterize the resulting microstructure, the light optical and scanning electron microscopy, micro and macro hardness measurements, and the X-ray diffraction are conducted subsequent to tensile tests. The investigation proves not only the feasibility to adjust the strength and ductility flexibly, unique microstructures are also observed and the governing mechanisms are clarified.


    Directory of Open Access Journals (Sweden)

    I.N. Karpenko


    Full Text Available In modern stomatology the problem ofatypicalforms ofinflammatoryperiodontaldiseases origination, namely of rapidly progressive periodontitis (RPP, has got special importance due to its widespread. The article presents one of the impotant parts of the pathogenesis- the disturbance of microcirculation processes caused by the decrease of blood clot resistencyofa vascularwall in pathogenesis ofmicrocirculatori disorders in patients with RPP. These disturbances are predetermined by endothelial dysfunction with the subsequent degradation of the clinical presentation of disease, the stomatologic status and quality of patients life.

  1. Stratification by Genetic and Demographic Characteristics Improves Diagnostic Accuracy of Cerebrospinal Fluid Biomarkers in Rapidly Progressive Dementia. (United States)

    Karch, André; Llorens, Franc; Schmitz, Matthias; Arora, Amandeep Singh; Zafar, Saima; Lange, Peter; Schmidt, Christian; Zerr, Inga


    Cerebrospinal fluid (CSF) biomarkers are routinely used for the differential diagnosis of rapidly progressive dementia, but are also affected by patients' characteristics. To assess if stratification by age, sex, and genetic risk factors improves the accuracy of cerebrospinal fluid (CSF) biomarkers in patients with rapidly progressive dementia. 1,538 individuals with sporadic Creutzfeldt-Jakob disease (CJD), 173 with classic Alzheimer's disease (cAD), 37 with rapidly progressive Alzheimer's disease (rpAD), and 589 without signs of dementia were included in this retrospective diagnostic study. The effect of age, sex, PRNP codon 129, and APOE genotype on CSF levels of tau, p-tau, Aβ1-42, and Aβ1-40 values measured at time of diagnostic work-up was assessed. Tau was a better marker for the differentiation of CJD and rpAD in older (AUC:0.97; 95% CI:0.96-1.00) than in younger (AUC:0.91; 95% CI:0.87-0.94) patients as tau levels increased with age in CJD patients, but not in rpAD patients. PRNP codon 129 and APOE genotype had complex effects on biomarkers in all diseases, making stratification by genotype a powerful tool. In females (AUC:0.78; 95% CI:0.65-0.91) and patients older than 70 (AUC:0.78; 95% CI:0.62-0.93), tau was able to differentiate with moderate accuracy between cAD and rpAD patients. Implementation of stratum-specific reference ranges improves the diagnostic accuracy of CSF biomarkers for the differential diagnosis of rapidly progressive dementia. Diagnostic criteria developed for this setting have to take this into account.

  2. Progression of CKD form pre-dialysis : natural course, risk factors, and outcomes

    NARCIS (Netherlands)

    Appeldoorn- de Jager, Dina Jezina (Dinanda) van


    Chronic kidney disease (CKD) is a progressive disease associated with increased morbidity and mortality. Different therapeutic interventions in the course of CKD are shown to be effective in slowing or preventing disease progression. This thesis focused on the progression of CKD from pre-dialysis to

  3. Lower corneal hysteresis is associated with more rapid glaucomatous visual field progression. (United States)

    De Moraes, Carlos V Gustavo; Hill, Victoria; Tello, Celso; Liebmann, Jeffrey M; Ritch, Robert


    We investigated the correlation between central corneal thickness (CCT) and corneal hysteresis (CH) and their relationship with the rate of visual field (VF) change. Glaucoma patients who underwent complete ophthalmic examination and tonometry using both the Goldmann applanation tonometer and the Ocular Response Analyzer were prospectively enrolled. Only eyes with ≥5 SITA Standard 24-2 VF tests were included. Automated pointwise linear regression analysis was used to determine VF progression. One hundred fifty-three eyes (153 patients; mean age, 61.3 ± 14.0 y; mean number of VF, 8.5 ± 3.4; mean follow-up time, 5.3 ± 2.0 y) met the enrollment criteria. The mean global rate of VF change was -0.34 ± 0.7 dB/y. Twenty-five eyes (16%) reached a progression endpoint. Progressing eyes had lower CCT (525.0 ± 34.2 vs 542.3 ± 3 8.5 μm, P=0.04) and lower CH (7.5 ± 1.4 vs 9.0 ± 1.8 mm Hg, PCorneal biomechanical and physical properties, such as CH and CCT, are highly correlated and associated with VF progression. As CH may describe corneal properties more completely than thickness alone, it may be a parameter that is better associated with progression.

  4. Progress towards Rapid Detection of Measles Vaccine Strains: a Tool To Inform Public Health Interventions. (United States)

    Hacker, Jill K


    Rapid differentiation of vaccine from wild-type strains in suspect measles cases is a valuable epidemiological tool that informs the public health response to this highly infectious disease. Few public health laboratories sequence measles virus-positive specimens to determine genotype, and the vaccine-specific real-time reverse transcriptase PCR (rRT-PCR) assay described by F. Roy et al. (J. Clin. Microbiol. 55:735-743, 2017, offers a rapid, easily adoptable method to identify measles vaccine strains in suspect cases. Copyright © 2017 American Society for Microbiology.

  5. A rapidly progressing, deadly disease ofActias selene (Indian moon ...

    Indian Academy of Sciences (India)

    A mixed baculoviral–bacterial infection observed among Actias selene (Hübner 1807), the Indian moon moth (Insecta: Lepidoptera: Saturniidae), larvae was characterized and followed by a photographic documentation of the disease progression. The etiological agents were determined using mass spectrometry and ...

  6. Papillary tumor of the pineal region: report of a rapidly progressive tumor with possible multicentric origin

    Energy Technology Data Exchange (ETDEWEB)

    Sato, Takashi S. [University of Iowa, Carver College of Medicine, Iowa City, IA (United States); Kirby, Patricia A. [University of Iowa, Department of Pathology, Iowa City, IA (United States); Buatti, John M. [University of Iowa, Department of Radiation Oncology, Iowa City, IA (United States); Moritani, Toshio [University of Iowa Hospitals and Clinics, Department of Radiology, Iowa City, IA (United States)


    Papillary tumor of the pineal region (PTPR) is an uncommon tumor recently added to the WHO classification of CNS tumors. We report a case of PTPR in a young boy that was noteworthy for early CSF dissemination and relentless progression. In spite of intensive chemotherapy and comprehensive radiotherapy, the boy died. The neuroimaging appearance is unique with possible multicentric origin of the tumor and intense uptake of {sup 111}In-DTPA-pentetreotide. (orig.)

  7. A Case of Immunotactoid Glomerulopathy with Rapid Progression to End-Stage Renal Disease

    Directory of Open Access Journals (Sweden)

    Shikha Jain


    Full Text Available Immunotactoid glomerulopathy (IGN is a rare immunoglobulin deposition disease. It is often mistaken for cryoglobulinemia or amyloidosis due to the similarities on biopsy findings. The disease progresses to end-stage renal disease (ESRD within 7 months to 10 years. This is the first case reported of a patient with a diagnosis of IGN who developed acute kidney injury (AKI and ESRD within 1 week of initial presentation.

  8. Technical Progress Report on Single Pass Flow Through Tests of Ceramic Waste Forms for Plutonium Immobilization

    Energy Technology Data Exchange (ETDEWEB)

    Zhao, P; Roberts, S; Bourcier, W


    This report updates work on measurements of the dissolution rates of single-phase and multi-phase ceramic waste forms in flow-through reactors at Lawrence Livermore National Laboratory. Previous results were reported in Bourcier (1999). Two types of tests are in progress: (1) tests of baseline pyrochlore-based multiphase ceramics; and (2) tests of single-phase pyrochlore, zirconolite, and brannerite (the three phases that will contain most of the actinides). Tests of the multi-phase material are all being run at 25 C. The single-phase tests are being run at 25, 50, and 75 C. All tests are being performed at ambient pressure. The as-made bulk compositions of the ceramics are given in Table 1. The single pass flow-through test procedure [Knauss, 1986 No.140] allows the powdered ceramic to react with pH buffer solutions traveling upward vertically through the powder. Gentle rocking during the course of the experiment keeps the powder suspended and avoids clumping, and allows the system to behave as a continuously stirred reactor. For each test, a cell is loaded with approximately one gram of the appropriate size fraction of powdered ceramic and reacted with a buffer solution of the desired pH. The buffer solution compositions are given in Table 2. All the ceramics tested were cold pressed and sintered at 1350 C in air, except brannerite, which was sintered at 1350 C in a CO/CO{sub 2} gas mixture. They were then crushed, sieved, rinsed repeatedly in alcohol and distilled water, and the desired particle size fraction collected for the single pass flow-through tests (SPFT). The surface area of the ceramics measured by BET ranged from 0.1-0.35 m{sup 2}/g. The measured surface area values, average particle size, and sample weights for each ceramic test are given in the Appendices.

  9. Association of AIDS and Bipolar Mania with Rapid Progression to Dementia and Death

    Directory of Open Access Journals (Sweden)

    Chin-Yi Yang


    Full Text Available Neuropsychiatric complications of human immunodeficiency virus (HIV infection or acquired immune deficiency syndrome (AIDS may present clinically as acute or chronic organic brain syndrome, or mimic functional psychiatric diseases. Among such psychiatric diseases, mania tends to occur with increased frequency after the onset of AIDS. We report a case in which manic manifestations were noted before the diagnosis of AIDS. The patient had no past or family history of mood disorders, but had risk factors for HIV infection. He had a rapid downhill course from initial manic symptoms to depression, dementia and then death within 10 months. Such rapid cognitive deterioration into AIDS dementia after mania is consistent with previous reports. Cases like this will become more common with spread of the AIDS pandemic in Asian regions, including Taiwan. Clinicians should be mindful of HIV infection/AIDS as a differential diagnosis in patients with manic episodes and risk factors for HIV infection.

  10. A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease

    Directory of Open Access Journals (Sweden)

    Shinji Sato


    Full Text Available Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM with rapidly progressive interstitial lung disease (RP-ILD was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis.

  11. A Case of Sarcoidosis with Interstitial Lung Disease Mimicking Clinically Amyopathic Dermatomyositis and Rapidly Progressive Interstitial Lung Disease (United States)

    Nogi, Shinichi; Sasaki, Noriko; Chinen, Naofumi; Honda, Kiri; Saito, Eiko; Wakabayashi, Takayuki; Yamada, Chiho; Suzuki, Yasuo


    Here, we report a patient with sarcoidosis who developed edematous erythema and interstitial lung disease. At the initial visit, clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP-ILD) was suspected because he had progressive dyspnea but no muscle weakness. The presence of anti-CADM-140/MDA5 autoantibodies was immediately assessed to facilitate a precise diagnosis, with negative results. Thereafter, skin and transbronchial lung biopsies revealed noncaseating granuloma with Langhans giant cells in both specimens, leading to a diagnosis of sarcoidosis. In this case, clinical features of skin and lung were unable to distinguish DM (including CADM) from sarcoidosis, but the lack of anti-CADM-140/MDA5 antibody was useful for differentiating CADM with RP-ILD mimicking sarcoidosis from bona fide sarcoidosis. PMID:25431723

  12. Hydrothermal vents in Lake Tanganyika harbor spore-forming thermophiles with extremely rapid growth

    DEFF Research Database (Denmark)

    Elsgaard, Lars; Prieur, Daniel


    and peptone. The optimum temperature for growth was 60 °C, while minimum and maximum temperatures were 40 and 75 °C. The pH response was alkalitolerant with optimum pH at 7.4 and 8.5 depending on the growth medium. The distinct feature of rapid proliferation and endospore formation may allow the novel...

  13. A Case of Adventitial Cystic Disease of the Popliteal Artery Progressing Rapidly after Percutaneous Ultrasound-guided Aspiration. (United States)

    Seo, Hiroyuki; Fujii, Hiromichi; Aoyama, Takanobu; Sasako, Yoshikado


    Adventitial cystic disease is a rare non-atherosclerotic vascular disease. We report a 36-year-old man with right intermittent claudication by adventitial cystic disease. computed tomography (CT) and magnetic resonance imaging (MRI) revealed an ovoid cystic mass compressing the right popliteal artery and causing severe stenosis of the lumen. Percutaneous aspiration was performed, which improved his symptoms. However, he complained of identical intermittent claudication two weeks later. Radiographic findings revealed that the cystic lesion had progressed rapidly. The cystic lesion was resected and the affected arterial segment was interposed. We consider that conventional surgical intervention remains the favored treatment option in the management of adventitial cystic disease.

  14. Rapid progression of mediastinal tumor within a few days: A case report of T cell lymphoblastic lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Ahn, Tae Ran; Lee, Young Kyung; Jun, Hyun Jung; Jung, Eun Ah; Son, Jin Sung [Seoul Medical Center, Seoul (Korea, Republic of)


    T-cell lymphoblastic lymphoma is a highly aggressive tumor derived from lymphocyte of the thymus, which accounts for 2% of non-Hodgkin's lymphoma. The disease occurs most commonly in adolescent and young adult males. It often results in respiratory emergency because of high proliferation rate. In this case, we confirmed the rapid progression of T-cell lymphoblastic lymphoma through the chest CT scan with one week interval. Three days of empirical chemotherapy resulted in substantial reduction of mediastinal mass, pleural thickening and pleural effusion.

  15. CRF19_cpx is an Evolutionary fit HIV-1 Variant Strongly Associated With Rapid Progression to AIDS in Cuba. (United States)

    Kouri, Vivian; Khouri, Ricardo; Alemán, Yoan; Abrahantes, Yeissel; Vercauteren, Jurgen; Pineda-Peña, Andrea-Clemencia; Theys, Kristof; Megens, Sarah; Moutschen, Michel; Pfeifer, Nico; Van Weyenbergh, Johan; Pérez, Ana B; Pérez, Jorge; Pérez, Lissette; Van Laethem, Kristel; Vandamme, Anne-Mieke


    Clinicians reported an increasing trend of rapid progression (RP) (AIDS within 3 years of infection) in Cuba. Recently infected patients were prospectively sampled, 52 RP at AIDS diagnosis (AIDS-RP) and 21 without AIDS in the same time frame (non-AIDS). 22 patients were sampled at AIDS diagnosis (chronic-AIDS) retrospectively assessed as > 3 years infected. Clinical, demographic, virological, epidemiological and immunological data were collected. Pol and env sequences were used for subtyping, transmission cluster analysis, and prediction of resistance, co-receptor use and evolutionary fitness. Host, immunological and viral predictors of RP were explored through data mining. Subtyping revealed 26 subtype B strains, 6 C, 6 CRF18_cpx, 9 CRF19_cpx, 29 BG-recombinants and other subtypes/URFs. All patients infected with CRF19 belonged to the AIDS-RP group. Data mining identified CRF19, oral candidiasis and RANTES levels as the strongest predictors of AIDS-RP. CRF19 was more frequently predicted to use the CXCR4 co-receptor, had higher fitness scores in the protease region, and patients had higher viral load at diagnosis. CRF19 is a recombinant of subtype D (C-part of Gag, PR, RT and nef), subtype A (N-part of Gag, Integrase, Env) and subtype G (Vif, Vpr, Vpu and C-part of Env). Since subtypes D and A have been associated with respectively faster and slower disease progression, our findings might indicate a fit PR driving high viral load, which in combination with co-infections may boost RANTES levels and thus CXCR4 use, potentially explaining the fast progression. We propose that CRF19 is evolutionary very fit and causing rapid progression to AIDS in many newly infected patients in Cuba.

  16. Advanced and rapidly progressing head and neck cancer: good palliation following intralesional bleomycin.

    LENUS (Irish Health Repository)

    Quintyne, Keith Ian


    The authors herein report the case of a 61-year-old man undergoing adjuvant therapy for locally advanced laryngeal cancer, who developed parastomal recurrence in his radiation field around his tracheotomy site, while he was undergoing radiation therapy, and compromised the secure placement of his tracheotomy tube and maintenance of his upper airway. MRI restaging and biopsy confirmed recurrence and progressive disease in his mediastinum. He underwent local therapy with intralesional bleomycin with good palliation, and ability to maintain the patency of his upper airway.

  17. Undercooling of Rapidly Solidified Droplets and Spray Formed Strips of Al-Cu (Sc) (United States)

    Bogno, A.; Natzke, P.; Yin, S.; Henein, H.

    Impulse Atomization (IA) (a single fluid atomization technique) was used to rapidly solidify Al-4.5wt%Cu and Al-4.5wt%Cu-0.4wt%Sc under argon atmosphere. In addition to the IA-generated droplets, the same technique was used to produce strips by Spray Deposition (SD) of the same alloys on a copper substrate with and without oil coating. The rapid solidification microstructures were analyzed using Scanning Electron Microscopy (SEM). From the SEM images, the amount of eutectic and the secondary dendrite arm spacing (SDAS) were measured. These SDAS results lead to the estimation of cooling rate. The eutectic fraction coupled with the metastable extension of the solidus and liquidus lines of Al-Cu (Sc) phase diagram lead to the estimation of primary and eutectic undercoolings. A comparison of the solidification path of the droplets and the strips was done as well as the analysis of the effects of scandium.

  18. A Novel Form of Progressive Retinal Atrophy in Swedish Vallhund Dogs


    Cooper, Ann E.; Ahonen, Saija; Rowlan, Jessica S.; Duncan, Alison; Seppala, Eija H.; Vanhapelto, Paivi; Lohi, Hannes; Komaromy, Andras M.


    Inherited retinal degenerations, such as retinitis pigmentosa (RP) and age-related macular degeneration (AMD), represent leading causes of incurable blindness in humans. This is also true in dogs, where the term progressive retinal atrophy (PRA) is used to describe inherited photoreceptor degeneration resulting in progressive vision loss. Because of the similarities in ocular anatomy, including the presence of a cone photoreceptor-rich central retinal region, and the close genotype-phenotype ...

  19. Acute Q Fever Presenting as Fever of Unknown Origin with Rapidly Progressive Hepatic Failure in a Patient with Alcoholism

    Directory of Open Access Journals (Sweden)

    Po-Han Lin


    Full Text Available We report a case of fulminant acute Q fever presenting as fever of unknown origin with rapidly progressive hepatic failure in a patient with alcoholism. A 51-year-old electrician, who was a habitual drinker, presented with a 2-week history of intermittent high fever, acute hepatomegaly and rapidly progressive jaundice after being accidentally exposed to dust from bird nests when he was repairing electrical equipment and circuitry at an abandoned factory in Taipei County. Ascites and prolonged prothrombin time were noted at admission. Transjugular liver biopsy and bone marrow biopsy found multiple small fibrinoid-ring granulomas in liver parenchyma and bone marrow. Doxycycline therapy was empirically started. The fever gradually subsided over a 2-week period, along with the recovery of liver function. The diagnosis of acute Q fever was confirmed by high titers of antibodies against Coxiella burnetii (phase I IgM 1:160 and IgG 1:2560, phase II IgM > 1:320 and IgG 1:5120 and a four-fold elevation of phase II IgG titer in the paired serum. The experience of this case shows that the possibility of Q fever should not be overlooked in patients who have an unexplained febrile illness and severe liver function impairment following exposure to a contaminated environment in Taiwan.

  20. Application of morphing technique with mesh-merging in rapid hull form generation

    Directory of Open Access Journals (Sweden)

    Ju Young Kang


    Full Text Available Morphing is a geometric interpolation technique that is often used by the animation industry to transform one form into another seemingly seamlessly. It does this by producing a large number of ‘intermediate’ forms between the two ‘extreme’ or ‘parent’ forms. It has already been shown that morphing technique can be a powerful tool for form design and as such can be a useful addition to the armoury of product designers. Morphing procedure itself is simple and consists of straightforward linear interpolation. However, establishing the correspondence between vertices of the parent models is one of the most difficult and important tasks during a morphing process. This paper discusses the mesh-merging method employed for this process as against the already established mesh-regularising method. It has been found that the merging method minimises the need for manual manipulation, allowing automation to a large extent.

  1. Application of morphing technique with mesh-merging in rapid hull form generation (United States)

    Kang, Ju Young; Lee, Byung Suk


    Morphing is a geometric interpolation technique that is often used by the animation industry to transform one form into another seemingly seamlessly. It does this by producing a large number of `intermediate' forms between the two `extreme' or `parent' forms. It has already been shown that morphing technique can be a powerful tool for form design and as such can be a useful addition to the armoury of product designers. Morphing procedure itself is simple and consists of straightforward linear interpolation. However, establishing the correspondence between vertices of the parent models is one of the most difficult and important tasks during a morphing process. This paper discusses the mesh-merging method employed for this process as against the already established mesh-regularising method. It has been found that the merging method minimises the need for manual manipulation, allowing automation to a large extent.

  2. Cobalamin C Deficiency Shows a Rapidly Progressing Maculopathy With Severe Photoreceptor and Ganglion Cell Loss (United States)

    Bonafede, Lucas; Ficicioglu, Can H.; Serrano, Leona; Han, Grace; Morgan, Jessica I. W.; Mills, Monte D.; Forbes, Brian J.; Davidson, Stefanie L.; Binenbaum, Gil; Kaplan, Paige B.; Nichols, Charles W.; Verloo, Patrick; Leroy, Bart P.; Maguire, Albert M.; Aleman, Tomas S.


    Purpose To describe in detail the retinal structure and function of a group of patients with cobalamin C (cblC) disease. Methods Patients (n = 11, age 4 months to 15 years) with cblC disease (9/11, early onset) diagnosed by newborn screening underwent complete ophthalmic examinations, fundus photography, near-infrared reflectance imaging, and spectral-domain optical coherence tomography (SD-OCT). Electroretinograms (ERGs) were performed in a subset of patients. Results Patients carried homozygous or compound heterozygote mutations in the methylmalonic aciduria and homocystinuria type C (MMACHC) gene. Late-onset patients had a normal exam. All early-onset patients showed a maculopathy; older subjects had a retina-wide degeneration (n = 4; >7 years of age). In general, retinal changes were first observed before 1 year of age and progressed within months to a well-established maculopathy. Pseudocolobomas were documented in three patients. Measurable visual acuities ranged from 20/200 to 20/540. Nystagmus was present in 8/11 patients; 5/6 patients had normal ERGs; 1/6 had reduced rod-mediated responses. Spectral-domain OCT showed macular thinning, with severe ganglion cell layer (GCL) and outer nuclear layer (ONL) loss. Inner retinal thickening was observed in areas of total GCL/ONL loss. A normal lamination pattern in the peripapillary nasal retina was often seen despite severe central and/or retina-wide disease. Conclusions Patients with early-onset cblC and MMACHC mutations showed an early-onset, unusually fast-progressing maculopathy with severe central ONL and GCL loss. An abnormally thickened inner retina supports a remodeling response to both photoreceptor and ganglion cell degeneration and/or an interference with normal development in early-onset cblC. PMID:26658511

  3. Difficult preoperative diagnosis in a case of rapidly progressive carcinomatous pericarditis. (United States)

    Wada, Tomoyuki; Anai, Hirofumi; Shuto, Takashi; Okamoto, Keitaro; Kawano, Madoka; Kozaki, Satoshi; Hirota, Jun; Miyamoto, Shinji


    A 54-year-old woman initially diagnosed with stage IIIb squamous cell carcinoma of the uterine cervix was treated with chemotherapy and radiation therapy. After 8 months, she developed dyspnea, leg edema, pleural effusion, pericardial effusion, and liver congestion. Her cardiac ejection fraction was normal and cardiomegaly was not evident. Metastatic carcinomatous pericarditis or pleurisy was suspected, but laboratory findings, including tumor markers, were normal. She was transferred to our hospital for the repair a cardiac injury caused by a pericardial drainage procedure. Emergency surgery was performed for the misplaced drainage catheter in the right atrium and for an abnormal mass in her right and left atria. The clinical diagnosis of carcinomatous pericarditis was made; however, her condition rapidly deteriorated, and she died 6 days postoperatively. At autopsy, metastasis was identified in a large area of the pericardium and myocardium.

  4. Systemic and rapidly progressive light-chain deposition disease initially presenting as tubulointerstitial nephritis. (United States)

    Takahashi, Satoko; Soma, Jun; Nakaya, Izaya; Yahata, Mayumi; Sakuma, Tsutomu; Yaegashi, Hiroshi; Sato, Akiyoshi; Wano, Masaharu; Sato, Hiroshi


    A 42-year-old woman was admitted to a hospital after first-time detection of proteinuria and hematuria during a routine medical check-up. Because her serum creatinine level had rapidly increased from 0.9 to 3.2 mg/dl since measurement 3 months earlier, she was referred to our hospital. Renal biopsy revealed extensive tubular atrophy and interstitial fibrosis with mild leukocyte infiltration. Glomeruli showed minimal changes, and no immunoglobulin or complement deposition was observed by immunofluorescence. Oral prednisolone was commenced under the diagnosis of chronic tubulointerstitial nephritis, and she discharged once. However, its effects were transient; her renal function deteriorated rapidly and hemodialysis was initiated 5 months after her initial check-up. On readmission, urinary Bence-Jones protein κ-type was detected, and examination of bone marrow led to a diagnosis of Bence-Jones κ-type multiple myeloma. Light-chain staining using a renal biopsy specimen obtained 2 months earlier showed κ-light-chain deposition on tubular basement membranes but not glomeruli. Despite undergoing chemotherapy with vincristine, doxirubicin, and dexamethasone, the patient died suddenly from a cardiac arrhythmia. Autopsy showed κ-light-chain deposition in the heart, thyroid, liver, lungs, spleen, and ovaries. Congo red staining yielded negative results. Typical light-chain deposition disease (LCDD) characterized by nodular glomerulosclerosis was observed in the kidneys. This case demonstrates that tubulointerstitial nephritis can be an early pathological variant of LCDD, which may be followed by accelerated and massive light-chain deposition in glomeruli.

  5. Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in a 10-year-old girl

    Directory of Open Access Journals (Sweden)

    Fermin Blanco Filho


    Full Text Available CONTEXT: The term pulmonary-renal syndrome has been used frequently to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. The classic example of this type of vasculitis is Goodpasture´s syndrome, a term used to describe the association of pulmonary hemorrhage, glomerulonephritis and the presence of circulating antiglomerular basement membrane antibodies (anti-GBM. Among the several types of systemic vasculitides that can present clinical manifestations of the pulmonary-renal syndrome, we focus the discussion on two types more frequently associated with antineutrophil cytoplasm antibodies (ANCA, microscopic polyangiitis and Wegener´s granulomatosis, concerning a 10 year old girl with clinical signs and symptoms of pulmonary-renal syndrome, with positive ANCA and rapidly progressive evolution. CASE REPORT: We describe the case of a 10-year-old girl referred to our hospital for evaluation of profound anemia detected in a primary health center. Five days before entry she had experienced malaise, pallor and began to cough up blood-tinged sputum that was at first attributed to dental bleeding. She was admitted to the infirmary with hemoglobin = 4 mg/dL, hematocrit = 14%, platelets = 260,000, white blood cells = 8300, 74% segmented, 4% eosinophils, 19% lymphocytes and 3% monocytes. Radiographs of the chest revealed bilateral diffuse interstitial alveolar infiltrates. There was progressive worsening of cough and respiratory distress during the admission day, when she began to cough up large quantities of blood and hematuria was noted. There was rapid and progressive loss of renal function and massive lung hemorrhage. The antineutrophil cytoplasm antibody (ANCA test with antigen specificity for myeloperoxidase (anti-MPO was positive and the circulating anti-GBM showed an indeterminate result.

  6. Rapid progression to glioblastoma in a subset of IDH-mutated astrocytomas: a genome-wide analysis. (United States)

    Richardson, Timothy E; Snuderl, Matija; Serrano, Jonathan; Karajannis, Matthias A; Heguy, Adriana; Oliver, Dwight; Raisanen, Jack M; Maher, Elizabeth A; Pan, Edward; Barnett, Samuel; Cai, Chunyu; Habib, Amyn A; Bachoo, Robert M; Hatanpaa, Kimmo J


    According to the recently updated World Health Organization (WHO) classification (2016), grade II-III astrocytomas are divided into IDH-wildtype and IDH-mutant groups, the latter being significantly less aggressive in terms of both progression-free and total survival. We identified a small cohort of WHO grade II-III astrocytomas that harbored the IDH1 R132H mutation, as confirmed by both immunohistochemistry and molecular sequence analysis, which nonetheless had unexpectedly rapid recurrence and subsequent progression to glioblastoma. Among these four cases, the mean time to recurrence as glioblastoma was only 16 months and the mean total survival among the three patients who have died during the follow-up was only 31 months. We hypothesized that these tumors had other, unfavorable genetic or epigenetic alterations that negated the favorable effect of the IDH mutation. We applied genome-wide profiling with a methylation array (Illumina Infinium Human Methylation 450k) to screen for genetic and epigenetic alterations in these tumors. As expected, the methylation profiles of all four tumors were found to match most closely with IDH-mutant astrocytomas. Compared with a control group of four indolent, age-similar WHO grade II-III astrocytomas, the tumors showed markedly increased levels of overall copy number changes, but no consistent specific genetic alterations were seen across all of the tumors. While most IDH-mutant WHO grade II-III astrocytomas are relatively indolent, a subset may rapidly recur and progress to glioblastoma. The precise underlying cause of the increased aggressiveness in these gliomas remains unknown, although it may be associated with increased genomic instability.

  7. Rapid In Vivo Validation of Tumor Suppressor Gene Function in Prostate Cancer Progression (United States)


    cancer is critical to distinguish lethal forms of the disease to those that are indolent. Our goal was to establish CRISPR / Cas9 -based methods to...platform to systematically interrogate genes that are significantly mutated in human prostate cancer. Keywords CRISPR / Cas9 , Genetically engineered...goal with the following specific aims: Aim1: Develop in vitro methods to identify effective guide RNAs to quantify CRISPR / Cas9 efficiency We have

  8. The Troy Microneedle: A Rapidly Separating, Dissolving Microneedle Formed by Cyclic Contact and Drying on the Pillar (CCDP.

    Directory of Open Access Journals (Sweden)

    Miroo Kim

    Full Text Available In dissolving microneedle (DMN-mediated therapy, complete and rapid delivery of DMNs is critical for the desired efficacy. Traditional patch-based DMN delivery, however, may fail due to incomplete delivery from insufficient skin insertion or rapid separation of microneedles due to their strong bond to the backing film. Here, we introduce the Troy microneedle, which was created by cyclic contact and drying on the pillar (CCDP, and which enabled simultaneous complete and rapid delivery of DMN. This CCDP process could be flexibly repeated to achieve a specific desired drug dose in a DMN. We evaluated DMN separation using agarose gel, and the Troy microneedle achieved more complete and rapid separation than other, more deeply dipped DMN, primarily because of the Troy's minimal junction between the DMN and pillar. When Troy microneedles were applied to pig cadaver skin, it took only 15 s for over 90% of encapsulated rhodamine B to be delivered, compared to 2 h with application of a traditional DMN patch. In vivo skin penetration studies demonstrated rapid DMN-separation of Troy microneedles still in solid form before dissolution. The Troy microneedle overcomes critical issues associated with the low penetration efficiency of flat patch-based DMN and provides an innovative route for DMN-mediated therapy, combining patient convenience with the desire drug efficacy.

  9. Acute gouty arthritis and rapidly progressive renal failure as manifestation of multiple myeloma: clinical case description

    Directory of Open Access Journals (Sweden)

    O.V. Gudym


    Full Text Available The article describes a clinical case of multiple myeloma in 78-year-old man, its clinical onset was as an acute attack of gout. The patient was admitted to hospital due to the development of the first acute attack of gout. The attack was characterized by polyarthricular joint lesion of the upper and lower extremities, pronounced inflammatory reaction, insufficient response to the use of non-steroidal anti-inflammatory drugs, and a high level of hyperuricemia. The serum uric acid concentration ranged from 636 to 712 μmol/l. The study of the synovial fluid of the inflamed knee joint made it possible to reveal uric acid crystals and to confirm the diagnosis of acute gouty arthritis. Simultaneously, the patient had significant renal impairment: creatinine was 574 μmol/l, urea — 39.9 mmol/l, glomerular filtration rate according to CKD-EPI — 8 ml/min. The daily proteinuria was 1.8 g. A retrospective assessment of laboratory parameters allowed to reveal completely normal indicators of renal function 6 months ago. Considering the development of acute gouty arthritis, its polyarticular nature, persistent course, rapid involvement of new joints, high uric acid levels during an acute attack exceeding 600 μmol/l (10 mg/dL, rapid development of renal failure within 6 months until the terminal stage, it was suggested the secondary nature of gout on the background of kidney damage by another pathological process. Further clinical, laboratory and instrumental studies allowed verifying multiple myeloma with renal damage. Bence Jones protein in the urine was not detected, there was also no evidence of hyperproteinemia. However, pain in the spine, ribs and chest was the basis for carrying out an X-ray study of the bones of the skeleton. Changes in the skeleton typical for multiple myeloma have been identified. Myelogram showed a high content of plasma cells (21.1 %, electrophoresis of blood proteins showed a high M-gradient (30.42 %, and a cytochemical

  10. Large cross-sectional study of presbycusis reveals rapid progressive decline in auditory temporal acuity. (United States)

    Ozmeral, Erol J; Eddins, Ann C; Frisina, D Robert; Eddins, David A


    The auditory system relies on extraordinarily precise timing cues for the accurate perception of speech, music, and object identification. Epidemiological research has documented the age-related progressive decline in hearing sensitivity that is known to be a major health concern for the elderly. Although smaller investigations indicate that auditory temporal processing also declines with age, such measures have not been included in larger studies. Temporal gap detection thresholds (TGDTs; an index of auditory temporal resolution) measured in 1071 listeners (aged 18-98 years) were shown to decline at a minimum rate of 1.05 ms (15%) per decade. Age was a significant predictor of TGDT when controlling for audibility (partial correlation) and when restricting analyses to persons with normal-hearing sensitivity (n = 434). The TGDTs were significantly better for males (3.5 ms; 51%) than females when averaged across the life span. These results highlight the need for indices of temporal processing in diagnostics, as treatment targets, and as factors in models of aging. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome

    Directory of Open Access Journals (Sweden)

    Wei-Ching Lin


    Full Text Available Pancreatic lipomatosis is defined as deposition of fat cells in pancreatic parenchyma. Although the etiology of this condition is still unclear, it is not uncommon in the elderly obese individuals, and a variety of transfusion-dependent hematologic diseases such as β-thalassemia major. Pancreatic lipomatosis associated with transfusion-dependent myelodysplastic syndrome (MDS has never been reported. We present a 17-year-old male patient with transfusion-dependent MDS. He received transfusion of a total of 345 units of blood in a period of 18 months but without iron chelating agent. Progressive fatty replacement of the pancreas parenchyma was found by a series of computed tomography images over seven hospital admissions due to repeated infections. Bone marrow biopsy revealed hemosiderin deposition. Because of his poor response to induction chemotherapy, stem cell transplantation was suggested, but the patient died of sepsis before the therapeutic procedure could take place. Although most patients with pancreatic lipomatosis have neither clinical symptoms nor abnormal laboratory data, it may cause endocrine and exocrine pancreas dysfunction. In this reported case, mild exocrine dysfunction was noted on the last admission. Clinicians should be cautious of hemosiderin deposition after large amount of blood transfusion and chelating therapy should be given to avoid iron overload.

  12. Primary pleural leiomyosarcoma with rapid progression and fatal outcome: a case report

    Directory of Open Access Journals (Sweden)

    Rais Ghizlane


    Full Text Available Abstract Introduction Leiomyosarcomas are neoplasms of smooth muscles that most commonly arise from the uterus, gastrointestinal tract, or soft tissue. Primary pleural leiomyosarcoma is extremely rare. To the best of our knowledge, only nine cases have been published to date. Because of the rarity of pleural leiomyosarcoma and its similarity (clinical and histological to other pleural neoplasms, particularly sarcomatous mesothelioma, diagnosis is often difficult. Case presentation A 58-year-old North African man was admitted with complaints of dyspnea and chest pain to our hospital. Chest computed tomography revealed right pleural effusion and pleural thickening. A transthoracic needle biopsy yielded a diagnosis of leiomyosarcoma, and tumor cells were strongly and uniformly positive for vimentin, a smooth muscle actin at immunohistochemical analysis. A general examination did not show any metastatic lesions in other areas. One month after diagnosis, the tumor grew rapidly, with pulmonary invasion, and therefore he was treated only by palliative care. He died from respiratory failure one month later. Because no organ of origin of the leiomyosarcoma, other than the pleura, was detected, this case was diagnosed as a primary pleural leiomyosarcoma. Conclusions Although leiomyosarcoma originating from the pleura is rare, this entity is increasingly described. The purpose of presenting this case report is to raise awareness among clinicians to consider this clinical entity as a differential diagnosis when a pleural mass is identified.

  13. Development of a definition for Rapid Progression (RP) of renal function in HIV-positive persons: the D:A:D study

    NARCIS (Netherlands)

    Kamara, David A.; Ryom, Lene; Ross, Michael; Kirk, Ole; Reiss, Peter; Morlat, Philippe; Moranne, Olivier; Fux, Christoph A.; Mocroft, Amanda; Sabin, Caroline; Lundgren, Jens D.; Smith, Colette J.; Powderly, B.; Shortman, N.; Moecklinghoff, C.; Reilly, G.; Franquet, X.; Ryom, L.; Sabin, C. A.; Kamara, D.; Smith, C.; Phillips, A.; Mocroft, A.; Tverland, J.; Mansfeld, M.; Nielsen, J.; Raben, D.; Lundgren, J. D.; Brandt, R. Salbøl; Rickenbach, M.; Fanti, I.; Krum, E.; Hillebregt, M.; Geffard, S.; Sundström, A.; Delforge, M.; Fontas, E.; Torres, F.; McManus, H.; Wright, S.; Kjær, J.; Sjøl, A.; Meidahl, P.; Helweg-Larsen, J.; Iversen, J. Schmidt; Kirk, O.; Ross, M.; Fux, C. A.; Morlat, P.; Moranne, O.; Kesselring, A. M.; Kamara, D. A.; Weber, R.; Pradier, C.; Friis-Møller, N.; Kowalska, J.; Sabin, C.; Law, M.; d'Arminio Monforte, A.; Dabis, F.; Bruyand, M.; Bower, M.; Fätkenheuer, G.; Donald, A.; Grulich, A.; Zaheri, S.; Gras, L.; Prins, J. M.; Kuijpers, T. W.; Scherpbier, H. J.; van der Meer, J. T. M.; Wit, F. W. M. N.; Godfried, M. H.; van der Poll, dr T.; Nellen, F. J. B.; Lange, J. M. A.; Geerlings, S. E.; van Vugt, M.; Pajkrt, D.; Bos, Drs J. C.; van der Valk, Drs M.; Grijsen, M. L.; Wiersinga, W. J.; Goorhuis, A.; Hovius, J. W. R.; Lowe, S.; Oude Lashof, A.; Posthouwer, D.; Ammerlaan, H. S. M.; Pronk, M. J. H.; van der Ende, M. E.; de Vries-Sluijs, T. E. M. S.; Schurink, C. A. M.; Nouwen, J. L.; Verbon, A.; Rijnders, B. J. A.; van Gorp, E. C. M.; van der Feltz, M.; Driessen, G. J. A.; van Rossum, A. M. C.; Branger, J.; Schippers, F.; van Nieuwkoop, C.; van Elzakker, E. P.; Groeneveld, P. H. P.; Bouwhuis, J. W.; Soetekouw, R.; ten Kate, R. W.; Kroon, F. P.; van Dissel, J. T.; Arend, S. M.; de Boer, M. G. J.; Jolink, H.; ter Vollaard, H. J. M.; Bauer, M. P.; den Hollander, J. G.; Pogany, K.; van Twillert, Drs G.; Kortmann, W.; Stuart, J. W. T. Cohen; Diederen, B. M. W.; Leyten, M. S.; Gelinck, L. B. S.; Kootstra, G. J.; Delsing, C. E.; Brinkman, K.; Blok, W. L.; Frissen, P. H. J.; Schouten, W. E. M.; van den Berk, G. E. L.; van Kasteren, M. E. E.; Brouwer, A. E.; Veenstra, J.; Lettinga, K. D.; Mulder, J. W.; Vrouenraets, S. M. E.; Lauw, F. N.; van Eeden, A.; Verhagen, D. W. M.; Sprenger, H. G.; Doedens, R.; Scholvinck, E. H.; van Assen, S.; Bierman, W. F. W.; Koopmans, P. P.; Keuter, M.; van der Ven, A. J. A. M.; ter Hofstede, H. J. M.; Dofferhoff, A. S. M.; Warris, A.; van Crevel, R.; Hoepelman, A. I. M.; Mudrikova, T.; Schneider, M. M. E.; Ellerbroek, P. M.; Oosterheert, J. J.; Arends, J. E.; Wassenberg, M. W. M.; Barth, R. E.; van Agtmael, M. A.; Perenboom, R. M.; Claessen, F. A. P.; Bomers, M.; Peters, E. J. G.; Geelen, S. P. M.; Wolfs, T. F. W.; Bont, L. J.; Richter, C.; van der Berg, J. P.; Gisolf, E. H.; van den Berge, M.; Stegeman, A.; van Vonderen, M. G. A.; van Houte, D. P. F.; Weijer, S.; el Moussaoui, R.; Winkel, C.; Muskiet, F.; Durand, N. N.; Voigt, R.; Chêne, G.; Lawson-Ayayi, S.; Thiébaut, R.; Bonnal, F.; Bonnet, F.; Bernard, N.; Caunègre, L.; Cazanave, C.; Ceccaldi, J.; Chambon, D.; Chossat, I.; Dauchy, F. A.; de Witte, S.; Dupon, M.; Duffau, P.; Dutronc, H.; Farbos, S.; Gaborieau, V.; Gemain, M. C.; Gerard, Y.; Greib, C.; Hessamfar, M.; Lacoste, D.; Lataste, P.; Lafarie, S.; Lazaro, E.; Malvy, D.; Meraud, J. P.; Mercié, P.; Monlun, E.; Neau, D.; Ochoa, A.; Pellegrin, J. L.; Pistone, T.; Ragnaud, J. M.; Receveur, M. C.; Tchamgoué, S.; Vandenhende, M. A.; Viallard, J. F.; Moreau, J. F.; Pellegrin, I.; Fleury, H.; Lafon, M. E.; Masquelier, B.; Trimoulet, P.; Breilh, D.; Haramburu, F.; Miremont-Salamé, G.; Blaizeau, M. J.; Decoin, M.; Delaune, J.; Delveaux, S.; D'Ivernois, C.; Hanapier, C.; Leleux, O.; Uwamaliya-Nziyumvira, B.; Sicard, X.; Palmer, G.; Touchard, D.; Petoumenos, K.; Bendall, C.; Moore, R.; Edwards, S.; Hoy, J.; Watson, K.; Roth, N.; Nicholson, J.; Bloch, M.; Franic, T.; Baker, D.; Vale, R.; Carr, A.; Cooper, D.; Chuah, J.; Ngieng, M.; Nolan, D.; Skett, J.; Calvo, G.; Mateu, S.; Domingo, P.; Sambeat, M. A.; Gatell, J.; del Cacho, E.; Cadafalch, J.; Fuster, M.; Codina, C.; Sirera, G.; Vaqué, A.; de Wit, S.; Clumeck, N.; Necsoi, C.; Gennotte, A. F.; Gerard, M.; Kabeya, K.; Konopnicki, D.; Libois, A.; Martin, C.; Payen, M. C.; Semaille, P.; van Laethem, Y.; Neaton, J.; Bartsch, G.; El-Sadr, W. M.; Thompson, G.; Wentworth, D.; Luskin-Hawk, R.; Telzak, E.; Abrams, D. I.; Cohn, D.; Markowitz, N.; Arduino, R.; Mushatt, D.; Friedland, G.; Perez, G.; Tedaldi, E.; Fisher, E.; Gordin, F.; Crane, L. R.; Sampson, J.; Baxter, J.; Lundgren, J.; Cozzi-Lepri, A.; Grint, D.; Podlekareva, D.; Peters, L.; Reekie, J.; Fischer, A. H.; Losso, M.; Elias, C.; Vetter, N.; Zangerle, R.; Karpov, I.; Vassilenko, A.; Mitsura, V. M.; Suetnov, O.; Colebunders, R.; Vandekerckhove, L.; Hadziosmanovic, V.; Kostov, K.; Machala, L.; Begovac, J.; Jilich, D.; Sedlacek, D.; Kronborg, G.; Gerstoft, J.; Benfield, T.; Larsen, M.; Katzenstein, T.; Hansen, A.-B. E.; Skinhøj, P.; Pedersen, C.; Ostergaard, L.; Zilmer, K.; Smidt, Jelena; Ristola, M.; Katlama, C.; Viard, J.- P.; Girard, P.- M.; Livrozet, J. M.; Vanhems, P.; Rockstroh, J.; Schmidt, R.; van Lunzen, J.; Degen, O.; Stellbrink, H. J.; Staszewski, S.; Bickel, M.; Kosmidis, J.; Gargalianos, P.; Xylomenos, G.; Perdios, J.; Panos, G.; Filandras, A.; Karabatsaki, E.; Sambatakou, H.; Banhegyi, D.; Mulcahy, F.; Yust, I.; Turner, D.; Burke, M.; Pollack, S.; Hassoun, G.; Maayan, S.; Vella, S.; Esposito, R.; Mazeu, I.; Mussini, C.; Arici, C.; Pristera, R.; Mazzotta, F.; Gabbuti, A.; Vullo, V.; Lichtner, M.; Chirianni, A.; Montesarchio, E.; Gargiulo, M.; Antonucci, G.; Testa, A.; Narciso, P.; Vlassi, C.; Zaccarelli, M.; Lazzarin, A.; Castagna, A.; Gianotti, N.; Galli, M.; Ridolfo, A.; Rozentale, B.; Zeltina, I.; Chaplinskas, S.; Hemmer, R.; Staub, T.; Ormaasen, V.; Maeland, A.; Bruun, J.; Knysz, B.; Gasiorowski, J.; Horban, A.; Bakowska, E.; Grzeszczuk, A.; Flisiak, R.; Boron-Kaczmarska, A.; Pynka, M.; Parczewski, M.; Beniowski, M.; Mularska, E.; Trocha, H.; Jablonowska, E.; Malolepsza, E.; Wojcik, K.; Antunes, F.; Doroana, M.; Caldeira, L.; Mansinho, K.; Maltez, F.; Duiculescu, D.; Rakhmanova, A.; Babes, Victor; Zakharova, N.; Jevtovic, D.; Mokráš, M.; Staneková, D.; Tomazic, J.; González-Lahoz, J.; Soriano, V.; Labarga, P.; Medrano, J.; Moreno, S.; Rodriguez, J. M.; Clotet, B.; Jou, A.; Paredes, R.; Tural, C.; Puig, J.; Bravo, I.; Gatell, J. M.; Miró, J. M.; Gutierrez, M.; Karlsson, A.; Mateo, G.; Flamholc, L.; Ledergerber, B.; Francioli, P.; Cavassini, M.; Hirschel, B.; Boffi, E.; Kravchenko, E.; Furrer, H.; Battegay, M.; Elzi, L.; Chentsova, N.; Frolov, V.; Kutsyna, G.; Servitskiy, S.; Krasnov, M.; Barton, S.; Johnson, A. M.; Mercey, D.; Johnson, M. A.; Murphy, M.; Weber, J.; Scullard, G.; Fisher, M.; Leen, C.; Morfeldt, L.; Thulin, G.; Åkerlund, B.; Koppel, K.; Håkangård, C.; Moroni, M.; Angarano, G.; Antinori, A.; Armignacco, O.; Castelli, F.; Cauda, R.; Di Perri, G.; Iardino, R.; Ippolito, G.; Perno, C. F.; von Schloesser, F.; Viale, P.; Ceccherini-Silberstein, F.; Girardi, E.; Lo Caputo, S.; Puoti, M.; Andreoni, Massimo; Ammassari, Adriana; Antinori, Andrea; Balotta, Claudia; Bonfanti, Paolo; Bonora, Stefano; Borderi, Marco; Capobianchi, M. Rosaria; Castagna, Antonella; Ceccherini-Silberstein, Francesca; Cingolani, Antonella; Cinque, Paola; Cozzi-Lepri, Alessandro; de Luca, Andrea; Di Biagio, Antonio; Girardi, Enrico; Gianotti, Nicola; Gori, Andrea; Guaraldi, Giovanni; Lapadula, Giuseppe; Lichtner, Miriam; Madeddu, Giordano; Maggiolo, Franco; Marchetti, Giulia; Marcotullio, Simone; Monno, Laura; Mussini, Cristina; Puoti, Massimo; Quiros, Eugenia; Rusconi, Stefano; Cicconi, P.; Formenti, T.; Galli, L.; Lorenzini, P.; Giacometti, A.; Costantini, A.; Monno, L.; Santoro, C.; Maggiolo, F.; Suardi, C.; Vanino, E.; Verucchi, G.; Quiros Roldan, E.; Minardi, C.; Quirino, T.; Abeli, C.; Manconi, P. E.; Piano, P.; Vecchiet, J.; Falasca, K.; Sighinolfi, L.; Segala, D.; Cassola, G.; Viscoli, G.; Alessandrini, A.; Piscopo, R.; Mazzarello, G.; Mastroianni, C.; Belvisi, V.; Bonfanti, P.; Caramma, I.; Castelli, A. P.; Rizzardini, G.; Ridolfo, A. L.; Piolini, R.; Salpietro, S.; Carenzi, L.; Moioli, M. C.; Marchetti, G.; Puzzolante, C.; Gori, A.; Onofrio, M.; Lapadula, G.; Abrescia, N.; Guida, M. G.; Baldelli, F.; Francisci, D.; Parruti, G.; Ursini, T.; Magnani, G.; Ursitti, M. A.; Andreoni, M.; Cingolani, A.; d' Avino, A.; Ammassari, A.; Gallo, L.; Nicastri, E.; Acinapura, R.; Capozzi, M.; Libertone, R.; Tebano, G.; Cattelan, A.; Mura, M. S.; Madeddu, G.; Caramello, P.; Orofino, G. C.; Bonora, S.; Sciandra, M.; Pellizzer, G.; Manfrin, V.; Caissotti, C.; Dellamonica, P.; Bernard, E.; Cua, E.; de Salvador- Guillouet, F.; Durant, J.; Ferrando, S.; Mondain-Miton, V.; Naqvi, A.; Perbost, I.; Prouvost-Keller, B.; Pillet, S.; Pugliese, P.; Rahelinirina, V.; Roger, P. M.; Dollet, K.; Aubert, V.; Barth, J.; Bernasconi, E.; Böni, J.; Bucher, H. C.; Burton- Jeangros, C.; Calmy, A.; Egger, M.; Fehr, J.; Fellay, J.; Gorgievski, M.; Günthard, H.; Haerry, D.; Hasse, B.; Hirsch, H. H.; Hösli, I.; Kahlert, C.; Kaiser, L.; Keiser, O.; Klimkait, T.; Kovari, H.; Martinetti, G.; de Tejada, B. Martinez; Metzner, K.; Müller, N.; Nadal, D.; Pantaleo, G.; Rauch, A.; Regenass, A.; Rudin, C.; Schmid, P.; Schultze, D.; Schöni-Affolter, F.; Schüpbach, J.; Speck, R.; Taffé, P.; Tarr, P.; Telenti, A.; Trkola, A.; Vernazza, P.; Yerly, S.


    No consensus exists on how to define abnormally rapid deterioration in renal function (Rapid Progression, RP). We developed an operational definition of RP in HIV-positive persons with baseline estimated glomerular filtration rate (eGFR) >90 ml/min/1.73 m2 (using Cockcroft Gault) in the Data

  14. Development of a definition for Rapid Progression (RP) of renal function in HIV-positive persons : the D:A:D study

    NARCIS (Netherlands)

    Kamara, David A; Ryom, Lene; Ross, Michael; Kirk, Ole; Reiss, Peter; Morlat, Philippe; Moranne, Olivier; Fux, Christoph A; Mocroft, Amanda; Sabin, Caroline; Lundgren, Jens D; Smith, Colette J; Schölvinck, Elisabeth H.


    BACKGROUND: No consensus exists on how to define abnormally rapid deterioration in renal function (Rapid Progression, RP). We developed an operational definition of RP in HIV-positive persons with baseline estimated glomerular filtration rate (eGFR) >90 ml/min/1.73 m2 (using Cockcroft Gault) in the

  15. Development of a definition for Rapid Progression (RP) of renal function in HIV-positive persons: the D:A:D study

    NARCIS (Netherlands)

    Kamara, D.A.; Ryom, L.; Ross, M.; Kirk, O.; Reiss, P.; Morlat, P.; Moranne, O.; Fux, C.A.; Mocroft, A.; Sabin, C.; Lundgren, J.D.; Smith, C.J.; Koopmans, P.P.; Keuter, M.; Ven, A.J.A.M. van der; Hofstede, H.J.M. ter; Dofferhoff, A.S.M.; Warris, A.; Crevel, R. van


    BACKGROUND: No consensus exists on how to define abnormally rapid deterioration in renal function (Rapid Progression, RP). We developed an operational definition of RP in HIV-positive persons with baseline estimated glomerular filtration rate (eGFR) >90 ml/min/1.73 m2 (using Cockcroft Gault) in

  16. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes. (United States)

    Broussolle, E; Bakchine, S; Tommasi, M; Laurent, B; Bazin, B; Cinotti, L; Cohen, L; Chazot, G


    We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6-10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal lobe cognitive slowing without generalised intellectual deterioration. Most disabled patients presented with an anterior opercular syndrome (Foix-Chavany-Marie syndrome), and pyramidal or extrapyramidal signs. CT and MRI findings disclosed asymmetric (left > right) progressive cortical atrophy of the frontal lobes predominating in the posterior inferior frontal region, notably the operculum. SPECT and PET revealed a decreased cerebral blood flow and metabolism, prominent in the left posterior-inferior frontal gyrus and premotor cortex, extending bilaterally in the most advanced cases. Pathological study of two cases showed non-specific neuronal loss, gliosis, and spongiosis of superficial cortical layers, mainly confined to the frontal lobes, with no significant abnormalities in the basal ganglia, thalamus, cerebellum, brain stem (except severe neuronal loss in the substantia nigra in one case), and spinal cord. We propose to call this peculiar syndrome Slowly Progressive Anarthria (SPA), based on its specific clinical presentation, and its metabolic and pathological correlates. SPA represents another clinical expression of focal cortical degeneration syndromes, that may overlap with other similar syndromes, specially primary progressive aphasia and the various frontal lobe dementias.

  17. Clinical follow-up data and the rate of development of precocious and rapidly progressive puberty in patients with premature thelarche. (United States)

    Çiçek, Dilek; Savas-Erdeve, Senay; Cetinkaya, Semra; Aycan, Zehra


    We aimed to evaluate the clinical follow-up data of patients with premature thelarche and determine the rate of development of precocious and early puberty in these patients. The charts of 158 girls with premature thelarche who were followed-up in our pediatric endocrinology polyclinic were reviewed. The patients were divided into three groups according to the age at onset: group 1 (0-1 month) (n=12), group 2 (1-24 months) (n=40) and group 3 (2-8 years) (n=106). At admission, the mean height standard deviation score (SDS), body weight (BW)-SDS, body mass index (BMI) and BMI-SDS were significantly higher in group 3 than in group 1 and group 2. At admission, 8.8% of the patients were obese and 24% of the patients were overweight. The majority of patients who were obese and overweight were in group 3. At the end of the follow-up, thelarche regressed in 24.7%, persisted in 32.9%, progressed in 25.9% and had a cyclic pattern in 16.5% of the patients. Precocious or rapidly progressive puberty developed in 47 of the 158 patients (29.7%). The mean age at progression to early or rapidly progressive puberty was 98.1±17.6 months. A total of 89.3% of the patients who progressed to early or rapidly progressive puberty were in group 3. Precocious or rapidly progressive puberty developed in 29.7% of subjects with premature thelarche. As patients who developed rapidly progressive puberty had a higher BW-SDS and BMI-SDS than those who did not, it is suggested that the increase in weight could stimulate rapidly progressive puberty in cases with premature thelarche.

  18. Rapid quantification of low level polymorph content in a solid dose form using transmission Raman spectroscopy. (United States)

    Griffen, Julia A; Owen, Andrew W; Burley, Jonathan; Taresco, Vincenzo; Matousek, Pavel


    This proof of concept study demonstrates the application of transmission Raman spectroscopy (TRS) to the non-invasive and non-destructive quantification of low levels (0.62-1.32% w/w) of an active pharmaceutical ingredient's polymorphic forms in a pharmaceutical formulation. Partial least squares calibration models were validated with independent validation samples resulting in prediction RMSEP values of 0.03-0.05% w/w and a limit of detection of 0.1-0.2% w/w. The study further demonstrates the ability of TRS to quantify all tablet constituents in one single measurement. By analysis of degraded stability samples, sole transformation between polymorphic forms was observed while excipient levels remained constant. Additionally, a beam enhancer device was used to enhance laser coupling to the sample, which allowed comparable prediction performance at 60 times faster rates (0.2s) than in standard mode. Copyright © 2016 Elsevier B.V. All rights reserved.

  19. Amyotrophic form of Creutzfeldt-Jakob disease with rapid course in 82-year-old man. (United States)

    Nowacki, P; Kulczycki, J; Narolewska, A; Grzelec, H


    The authors present a case of Creutzfeldt-Jakob disease in 82-year-old man. Besides the onset of the disease in the elderly and short survival time (8 weeks), other uncommon clinical and morphological features also characterized our case. An evident amyotrophic syndrome, confirmed in morphological findings, developed soon after the CJD onset. The spongiform change also observed within the white matter of cerebral hemispheres allowed us to diagnose the 'panencephalopathic' form of CJD.

  20. Rapid anaerobic benzene oxidation with a variety of chelated Fe(III) forms (United States)

    Lovley, D.R.; Woodward, J.C.; Chapelle, F.H.


    Fe(III) chelated to such compounds as EDTA, N-methyliminodiacetie acid, ethanol diglycine, humic acids, and phosphates stimulated benzene oxidation coupled to Fe(III) reduction in anaerobic sediments from a petroleum- contaminated aquifer as effectively as or more effectively than nitrilotriacetic acid did in a previously demonstrated stimulation experiment. These results indicate that many forms of chelated Fe(III) might be applicable to aquifer remediation.

  1. Peroxynitrate is formed rapidly during decomposition of peroxynitrite at neutral pH. (United States)

    Gupta, Deepali; Harish, B; Kissner, Reinhard; Koppenol, Willem H


    The decomposition of peroxynitrite near neutral pH leads ultimately to the formation of dioxygen and nitrite via an intermediate with an absorbance maximum at 284 nm. The intermediate oxidises I(-) with a rate constant of (78 +/- 8) x 10 M(-1) s(-1) and decays near pH 7 with a rate constant of (0.58 +/- 0.02) s(-1) at 22 degrees C, but is longer-lived at lower pH. On the basis of experiments performed with a tandem-quenching flow reactor, we tentatively identify this intermediate as peroxynitric acid, formed during the proposed reaction sequence ONOOH + ONOO(-) --> NO(2)(-) + O(2)NOO(-) + H(+) --> 2 NO(2)(-) + O(2). These products are those expected from a peracid. The rate constant for the first reaction is ca. 3 x 10(4) M(-1) s(-1). Part of the dioxygen formed is in the (1)Delta(g) state (S. Miyamoto, G. E. Ronsein, T. C. Corréa, G. R. Martinez, M. H. G. Medeiros and P. Di Mascio, Dalton Trans., 2009, DOI: 10.1039/b905560f). The decay of peroxynitrous acid at concentrations higher than 0.1 mM near neutral pH is best described by the simultaneous process of isomerisation (k = 1.2 s(-1)) and decomposition to peroxynitrate. The rate of formation and the amount of peroxynitrate formed are much larger than can be accounted for by homolysis reactions.

  2. Radiation cataracts: mechanisms involved in their long delayed occurrence but then rapid progression. (United States)

    Wolf, Norman; Pendergrass, William; Singh, Narendra; Swisshelm, Karen; Schwartz, Jeffrey


    This study was directed to assess the DNA damage and DNA repair response to X-ray inflicted lens oxidative damage and to investigate the subsequent changes in lens epithelial cell (LEC) behavior in vivo that led to long delayed but then rapidly developing cataracts. Two-month-old C57Bl/6 female mice received 11 Grays (Gy) of soft x-irradiation to the head only. The animals' eyes were examined for cataract status in 30 day intervals by slit lamp over an 11 month period post-irradiation. LEC migration, DNA fragment, free DNA retention, and reactive oxygen species (ROS) presence were established in the living lenses with fluorescent dyes using laser scanning confocal microscopy (LSCM). The extent and removal of initial LEC DNA damage were determined by comet assay. Immunohistochemistry was used to determine the presence of oxidized DNA and the response of a DNA repair protein in the lenses. This treatment resulted in advanced cortical cataracts that developed 5-11 months post-irradiation but then appeared suddenly within a 30 day period. The initially incurred DNA strand breaks were repaired within 30 min, but DNA damage remained as shown 72 h post-irradiation by the presence of the DNA adduct, 8-hydroxyguanosine (8-OHG), and a DNA repair protein, XRCC1. This was followed months later by abnormal behavior by LEC descendant cells with abnormal differentiation and migration patterns as seen with LSCM and fluorescent dyes. The sudden development of cortical cataracts several months post-irradiation coupled with the above findings suggests an accumulation of damaged descendants from the initially x-irradiated LECs. As these cells migrate abnormally and leave acellular lens surface sites, eventually a crisis point may arrive for lens entry of environmental O(2) with resultant ROS formation that overwhelms protection by resident antioxidant enzymes and results in the coagulation of lens proteins. The events seen in this study indicate the retention and transmission of

  3. Extranodal NK/T-cell lymphoma, nasal type, manifesting as rapidly progressive dementia without any mass or enhancing brain lesion. (United States)

    Shimatani, Yoshimitsu; Nakano, Yuta; Tsuyama, Naoko; Murayama, Shigeo; Oki, Ryosuke; Miyamoto, Ryosuke; Murakami, Nagahisa; Fujita, Koji; Watanabe, Syunsuke; Uehara, Hisanori; Abe, Takashi; Nodera, Hiroyuki; Kawarai, Toshitaka; Izumi, Yuishin; Kaji, Ryuji


    Among the many potential etiologies for rapidly progressive dementia (RPD), primary central nervous system extranodal NK/T-cell lymphoma, nasal-type (ENKL) is a rare entity. We present the first reported case of autopsy-proven RPD due to ENKL without any mass or enhancing lesion of the brain. A 54-year-old immunocompetent man presented with RPD, myoclonus and ataxia. The mini-mental state examination (MMSE) score was 22/30. His brain MRI revealed progressive brain atrophy without gadolinium enhancement or mass lesion. Five months after the initial evaluation, cognitive impairment further worsened with an MMSE score of 3/30. At the advanced stage, lumbar MRI showed swollen cauda equina with gadolinium enhancement. The number of Epstein-Barr virus (EBV) DNA in cerebrospinal fluid had gradually increased. Twelve months after onset, the patient died of respiratory failure. Pathological findings revealed that lymphoma cells had diffusely invaded the meninges, parenchyma of the brain, spinal cord and cauda equina. Cells were positive for CD3, CD56 and EBV-encoded small RNAs and negative for CD20. No evidence of malignancy was identified in the visceral organs. This report indicates that ENKL should be recognized as one of the rare causes of RPD. Early testing for EBV-DNA in cerebrospinal fluid and imaging of cauda equina would be useful diagnostic tools. © 2016 Japanese Society of Neuropathology.

  4. Rapidly progressive psychotic symptoms triggered by infection in a patient with methylenetetrahydrofolate reductase deficiency: a case report. (United States)

    Iida, Shin; Nakamura, Masataka; Asayama, Shinya; Kunieda, Takenobu; Kaneko, Satoshi; Osaka, Hitoshi; Kusaka, Hirofumi


    Methylenetetrahydrofolate reductase (MTHFR) deficiency is a rare inborn error of metabolism inherited in autosomal recessive pattern and is associated with a wide spectrum of neurological abnormalities. We herein describe a 15-year-old boy with MTHFR deficiency who presented with a slowly progressive decline of school performance and a spastic gait. Rapidly deteriorating psychosis and repetitive seizures triggered by a febrile infection prompted neurological investigation. He had significantly elevated total plasma homocysteine and urinary homocystine levels, as well as a decreased plasma methionine level. Brain magnetic resonance imaging (MRI) revealed leukoencephalopathy. DNA gene sequencing showed c.446_447 del GC ins TT and c.137G > A, and c.665C > T heterozygous mutations in the MTHFR gene of the patient. Oral administration of betaine drastically improved his clinical symptoms within a few months. After 8 months of treatment, his total plasma homocysteine level moderately decreased; and the plasma methionine concentration became normalized. Furthermore, the white matter lesions on MRI had disappeared. This patient demonstrates the possibility that MTHFR deficiency should be considered in mentally retarded adolescents who display an abnormally elevated plasma level of homocysteine in association with progressive neurological dysfunction and leukoencephalopathy. Febrile infections may be an aggravating factor in patients with MTHFR deficiency.

  5. Rapid therapeutic response onset of a new pharmaceutical form of chloroquine phosphate 300 mg: effervescent tablets. (United States)

    Yanze, M F; Duru, C; Jacob, M; Bastide, J M; Lankeuh, M


    To compare the efficiency, safety and taste of two pharmaceutical forms of chloroquine phosphate 300 mg: effervescent tablets against uncoated tablets. An open randomized study with 60 adults who suffered from acute uncomplicated Plasmodium falciparum malaria in three health centres in Nkongsamba health district, Cameroon. Mean times to fever clearance, symptoms clearance and asexual parasites clearance were longer in the uncoated tablets group: 36 h (range 24-48 h, SD = 16.8) vs. 60 h (range 24-96 h, SD = 31.2, P = 0.001) for fever clearance, 36 h (24-48 h, SD = 16.8) vs. 48 h (24-72, SD = 24, P = 0.001) for symptoms clearance and 48 h (24-72, SD = 1) vs. 72 h (48-96, SD = 24, P = 0.001) for parasitaemia clearance. Uncoated tablets took significantly longer to achieve 50% reduction of the initial asexual parasite density: (mean/SD) 19.2 h/7 vs. 52.8 h/16.8, P 0.05. The cure rate at day 7 in the two groups was similar, P > 0.05. There was no chloroquine resistance in the effervescent tablets group but one RI and one RII resistance in the uncoated tablets group. The taste of the two pharmaceutical forms was significantly different, P Effervescent tablets tasted sweet (score = 7.93), whereas uncoated tablets were bitter (score = 2.07). Effervescent tablets of chloroquine phosphate 300 mg work faster than uncoated tablets and because of their safe use and sweet taste achieve good therapeutic compliance.

  6. Immunological Basis for Rapid Progression of Diabetes in Older NOD Mouse Recipients Post BM-HSC Transplantation.

    Directory of Open Access Journals (Sweden)

    Nan Wang

    Full Text Available Type I diabetes (T1D, mediated by autoreactive T cell destruction of insulin-producing islet beta cells, has been treated with bone marrow-derived hematopoietic stem cell (BM-HSC transplantation. Older non-obese diabetic (NOD mice recipients (3m, at disease-onset stage receiving syngeneic BM-HSC progressed more rapidly to end-stage diabetes post-transplantation than younger recipients (4-6w, at disease-initiation stage. FACS analyses showed a higher percentage and absolute number of regulatory T cells (Treg and lower proportion of proliferating T conventional cells (Tcon in pancreatic lymph nodes from the resistant mice among the younger recipients compared to the rapid progressors among the older recipients. Treg distribution in spleen, mesenteric lymph nodes (MLN, blood and thymus between the two groups was similar. However, the percentage of thymic Tcon and the proliferation of Tcon in MLN and blood were lower in the young resistants. These results suggest recipient age and associated disease stage as a variable to consider in BM-HSC transplantation for treating T1D.

  7. The analysis of the clinical and tool parameters characterizing a cardiomyopathyat various forms of the progressing muscular dystrophies

    Directory of Open Access Journals (Sweden)

    Poverennova I.E.


    Full Text Available Purpose: studying of clinical and tool characteristics of cardiomyopathies at various forms of the progressing muscular dystrophies. Material and methods. There had been 103 patients with hereditary forms of the progressing muscular dystrophies examined, among which 35 persons were with Duchenne muscular dystrophy, 28 with an atrophic myo-tonia, and 40 with a limb girdle dystrophy is conducted. Assessment of clinical and tool features of cardiomyopathies at these types of the hereditary progressing muscular dystrophies had been carried out. Results. In the group of patients with diffusion damage of a myocardium in the form of dystrophic violations had been revealed. Existence of a negative tooth of T in some assignments and lengthenings of an interval of QT is noted. With a dystrophic myotonia violation of a warm rhythm occurred at patients by 87 times more often than in the group of comparison. Violation of a rhythm of heart in group of patients with a limb girdle dystrophy came to light 91 times more often in reference to the group of comparison. Conclusion. Violations in a cardiovascular system at Duchenne muscular dystrophy are preferentially diffusion changes in a myocardium. At a dystrophic myotonia and a limb girdle dystrophy cardial violations concern mainly excitability and the conductivity of heart which are the main reason of developing of this disease at these patients.

  8. Rapid growth of black holes in massive star-forming galaxies. (United States)

    Alexander, D M; Smail, I; Bauer, F E; Chapman, S C; Blain, A W; Brandt, W N; Ivison, R J


    The tight relationship between the masses of black holes and galaxy spheroids in nearby galaxies implies a causal connection between the growth of these two components. Optically luminous quasars host the most prodigious accreting black holes in the Universe, and can account for greater than or approximately equal to 30 per cent of the total cosmological black-hole growth. As typical quasars are not, however, undergoing intense star formation and already host massive black holes (> 10(8)M(o), where M(o) is the solar mass), there must have been an earlier pre-quasar phase when these black holes grew (mass range approximately (10(6)-10(8))M(o)). The likely signature of this earlier stage is simultaneous black-hole growth and star formation in distant (redshift z > 1; >8 billion light years away) luminous galaxies. Here we report ultra-deep X-ray observations of distant star-forming galaxies that are bright at submillimetre wavelengths. We find that the black holes in these galaxies are growing almost continuously throughout periods of intense star formation. This activity appears to be more tightly associated with these galaxies than any other coeval galaxy populations. We show that the black-hole growth from these galaxies is consistent with that expected for the pre-quasar phase.

  9. EU-approved rapid tests for bovine spongiform encephalopathy detect atypical forms: a study for their sensitivities.

    Directory of Open Access Journals (Sweden)

    Daniela Meloni

    Full Text Available Since 2004 it become clear that atypical bovine spongiform encephalopthies (BSEs exist in cattle. Whenever their detection has relied on active surveillance plans implemented in Europe since 2001 by rapid tests, the overall and inter-laboratory performance of these diagnostic systems in the detection of the atypical strains has not been studied thoroughly to date. To fill this gap, the present study reports on the analytical sensitivity of the EU-approved rapid tests for atypical L- and H-type and classical BSE in parallel. Each test was challenged with two dilution series, one created from a positive pool of the three BSE forms according to the EURL standard method of homogenate preparation (50% w/v and the other as per the test kit manufacturer's instructions. Multilevel logistic models and simple logistic models with the rapid test as the only covariate were fitted for each BSE form analyzed as directed by the test manufacturer's dilution protocol. The same schemes, but excluding the BSE type, were then applied to compare test performance under the manufacturer's versus the water protocol. The IDEXX HerdChek ® BSE-scrapie short protocol test showed the highest sensitivity for all BSE forms. The IDEXX® HerdChek BSE-scrapie ultra short protocol, the Prionics®--Check WESTERN and the AJ Roboscreen® BetaPrion tests showed similar sensitivities, followed by the Roche® PrionScreen, the Bio-Rad® TeSeE™ SAP and the Prionics®--Check PrioSTRIP in descending order of analytical sensitivity. Despite these differences, the limit of detection of all seven rapid tests against the different classes of material set within a 2 log(10 range of the best-performing test, thus meeting the European Food Safety Authority requirement for BSE surveillance purposes. These findings indicate that not many atypical cases would have been missed surveillance since 2001 which is important for further epidemiological interpretations of the sporadic character of


    Energy Technology Data Exchange (ETDEWEB)

    Amorin, R.; Vilchez, J. M.; Perez-Montero, E. [Instituto de Astrofisica de Andalucia-CSIC, Glorieta de la Astronomia S/N, E-18008 Granada (Spain); Haegele, G. F.; Firpo, V. [Facultad de Ciencias Astronomicas y Geofisicas, Universidad de la Plata, Paseo del Bosque S/N, 1900 La Plata (Argentina); Papaderos, P., E-mail: [Centro de Astrofisica and Faculdade de Ciencias, Universidade do Porto, Rua das Estrelas, 4150-762 Porto (Portugal)


    Deep, high-resolution spectroscopic observations have been obtained for six compact, strongly star-forming galaxies at redshift z {approx} 0.1-0.3, most of them also known as green peas. Remarkably, these galaxies show complex emission-line profiles in the spectral region including H{alpha}, [N II] {lambda}{lambda}6548, 6584, and [S II] {lambda}{lambda}6717, 6731, consisting of the superposition of different kinematical components on a spatial extent of few kiloparsecs: a very broad line emission underlying more than one narrower component. For at least two of the observed galaxies some of these multiple components are resolved spatially in their two-dimensional spectra, whereas for another one a faint detached H{alpha} blob lacking stellar continuum is detected at the same recessional velocity {approx}7 kpc away from the galaxy. The individual narrower H{alpha} components show high intrinsic velocity dispersion ({sigma} {approx} 30-80 km s{sup -1}), suggesting together with unsharped masking Hubble Space Telescope images that star formation proceeds in an ensemble of several compact and turbulent clumps, with relative velocities of up to {approx}500 km s{sup -1}. The broad underlying H{alpha} components indicate in all cases large expansion velocities (full width zero intensity {>=}1000 km s{sup -1}) and very high luminosities (up to {approx}10{sup 42} erg s{sup -1}), probably showing the imprint of energetic outflows from supernovae. These intriguing results underline the importance of green peas for studying the assembly of low-mass galaxies near and far.

  11. Classifying Glioblastoma Multiforme Follow-Up Progressive vs. Responsive Forms Using Multi-Parametric MRI Features. (United States)

    Ion-Mărgineanu, Adrian; Van Cauter, Sofie; Sima, Diana M; Maes, Frederik; Sunaert, Stefan; Himmelreich, Uwe; Van Huffel, Sabine


    Purpose: The purpose of this paper is discriminating between tumor progression and response to treatment based on follow-up multi-parametric magnetic resonance imaging (MRI) data retrieved from glioblastoma multiforme (GBM) patients. Materials and Methods: Multi-parametric MRI data consisting of conventional MRI (cMRI) and advanced MRI [i.e., perfusion weighted MRI (PWI) and diffusion kurtosis MRI (DKI)] were acquired from 29 GBM patients treated with adjuvant therapy after surgery. We propose an automatic pipeline for processing advanced MRI data and extracting intensity-based histogram features and 3-D texture features using manually and semi-manually delineated regions of interest (ROIs). Classifiers are trained using a leave-one-patient-out cross validation scheme on complete MRI data. Balanced accuracy rate (BAR)-values are computed and compared between different ROIs, MR modalities, and classifiers, using non-parametric multiple comparison tests. Results: Maximum BAR-values using manual delineations are 0.956, 0.85, 0.879, and 0.932, for cMRI, PWI, DKI, and all three MRI modalities combined, respectively. Maximum BAR-values using semi-manual delineations are 0.932, 0.894, 0.885, and 0.947, for cMRI, PWI, DKI, and all three MR modalities combined, respectively. After statistical testing using Kruskal-Wallis and post-hoc Dunn-Šidák analysis we conclude that training a RUSBoost classifier on features extracted using semi-manual delineations on cMRI or on all MRI modalities combined performs best. Conclusions: We present two main conclusions: (1) using T1 post-contrast (T1pc) features extracted from manual total delineations, AdaBoost achieves the highest BAR-value, 0.956; (2) using T1pc-average, T1pc-90th percentile, and Cerebral Blood Volume (CBV) 90th percentile extracted from semi-manually delineated contrast enhancing ROIs, SVM-rbf, and RUSBoost achieve BAR-values of 0.947 and 0.932, respectively. Our findings show that AdaBoost, SVM-rbf, and RUSBoost

  12. The performance of matrices in daily clinical practice to predict rapid radiologic progression in patients with early RA. (United States)

    De Cock, D; Vanderschueren, G; Meyfroidt, S; Joly, J; Van der Elst, K; Westhovens, R; Verschueren, P


    To compare in daily clinical practice the reliability of matrices that forecast rapid radiologic progression (RRP) at year one, at year two, and over 2 years in patients with early rheumatoid arthritis (RA). Overall, 74 early RA patients with X-rays of hands and feet at baseline, year one, and year two were included. Initial DMARD combination therapy with steroids (ICTS) or DMARD monotherapy (IMT) was initiated according to patients' RA severity, based on rheumatologist opinion. The images were scored via the modified Sharp/van der Heijde (SvH) method. A total Sharp score progression of equal or higher than five per year was considered RRP. Six matrices were tested: ASPIRE CRP/ESR matrices, the BEST matrix, two SWEFOT matrices, and the ESPOIR matrix. Patients were placed in each of them yielding a RRP probability. The performance was tested by Area Under the Curve analysis reflecting the predictive value. Four patients developed RRP in year one, five in year two, and four over 2 years. With regard to face validity, the predicted probability did not correspond to the risk in reality: the one ICTS patient who developed RRP over 2 years was always found in the lowest RRP categories of all matrices. The ASPIRE CRP matrix yielded at least a moderate predicting value for the three time points. The other matrices showed moderate to no predicting value. The performance of all matrices was disappointing and it is impossible to fully rely on the existing matrices in daily clinical practice. © 2013 Published by Elsevier Inc.


    Directory of Open Access Journals (Sweden)

    N. S. Krylova


    Full Text Available Objective of work: to describe the apical form of hypertrophic cardiomyopathy (AFHC developing under the "mask" of the ischemic heart disease not diagnosed for a long period.Materials and methods. Patient B., 73 y.o., female, was brought to the cardiology department with complains of severe pressing pain behind the breastbone caused with no apparent reason and lasting for over 4 hours. The following examination of the patient was performed: electrocardiography (ECG, echocardiography (EchoCG, Holter ECG monitoring, coronary angiography (CAG, ventriculography.Results. The final diagnosis for the patient was set on the basis of the following readings: ECG data (basic rhythm – atrial fibrillation, left ventricle (LV hypertrophy, negative T-waves in leads V1–6, ST segment depression up to 1–2 mm in leads V4–6, EchoCG (hypertrophy of apical segments of the LV with decreasing of its cavity, moderate dilatation of the left atrium, intraventricular obstruction in the apical third of the LV with the maximum pressure gradient of up to 48 mm Hg., CAG (stenotic lesions of coronary arteries were found, ventriculography (LV volume is not increased, no violations of local contractility, narrowing of the LV cavity in the lower third is observed with thinning in the apex, which indicatesexpressed apical hypertrophy of the LV myocard. AFHC, apical form with moderate obstruction in the lower third of the left ventricle. Stress angina syndrome. CAG and ventriculography were main diagnostic methods that allowed setting the final diagnosis.Conclusion. The clinical case sets forth the peculiarities of diagnostics, therapy, and post-therapy management of patients with this form of AFHC.

  14. Waste-Form Development Program. Annual progress report, October 1981-September 1982

    Energy Technology Data Exchange (ETDEWEB)

    Neilson, R.M. Jr.; Colombo, P.


    Low-level wastes (LLW) at nuclear facilities have traditionally been solidified using portland cement (with and without additives). Urea-formaldehyde has been used for LLW solidification while bitumen (asphalt) and thermosetting polymers will be applied to domestic wastes in the near future. Operational difficulties have been observed with each of these solidification agents. Such difficulties include incompatibility with waste constitutents inhibiting solidification, premature setting, free standing water and fires. Some specific waste types have proven difficult to solidify with one or more of the contemporary agents. Similar problems are also anticipated for the solidification of new wastes, which are generated using advanced volume reduction technologies, and with the application of additional agents which may be introduced in the near future for the solidification of LLW. In the Waste Form Development program, contemporary solidification agents are being investigated relative to their potential applications to major fuel cycle and non-fuel cycle LLW streams. The range of conditions under which these solidification agents can be satisfactorily applied to specific LLW streams is being determined. These studies are primarily directed towards defining operating parameters for both improved solidification of problem wastes such as ion exchange resins, organic liquids and oils for which prevailing processes, as currently employed, appear to be inadequate, and solidification of new LLW streams including high solids content evaporator concentrates, dry solids, and incinerator ash generated from advanced volume reduction technologies. Solidified waste forms are tested and evaluated to demonstrate compliance with waste form performance and shallow land burial (SLB) acceptance criteria and transportation requirements (both as they currently exist and as they are anticipated to be modified with time).

  15. [Primary progressive forms of multiple sclerosis: application of the new diagnostic criteria in French]. (United States)

    De Seze, J; Mackowiak, A; Stojkovic, T; Ferriby, D; Hautecoeur, P; Vermersch, P


    Approximately 15 p.100 of all cases of multiple sclerosis (MS) are primary progressive multiple sclerosis (PPMS). Diagnosis is however frequently delayed due to the lack of relapse. The commonly used Poser criteria established in 1983 are not directly applicable to this subgroup of MS patients. In 2000, Thompson et al proposed new diagnostic criteria for PPMS. The aim of our study was to apply retrospectively these new criteria to a cohort of patients from northern France (G-SEP cohort). The cohort included 184 patients (94 women and 90 men, sex ratio=1.04). Mean age at disease onset was 40.3 years (18-67 years) and mean follow-up was 9.9 (1-39 years). Only one patient was older than 65 years but 13 patients (7.1 p.100) were younger than 25 years. Patients were classed as having definite, probable or possible PPMS on the basis of clinical, MRI, cerebrospinal fluid and visual evoked potential (VEP) data. Most of the patients (61.4 p.100) had one symptom at onset; spastic paraparesis was frequent (79 p.100). Mean delay to diagnosis was 4.4 years. Ten patients (5.4 p.100) were surgically treated for cervic arthrosic myelopathy. Mean EDSS score at the end of follow-up was 5.8 (3-10). MRI was positive in 87.3 p.100 of the patients. Oligoclonal bands were found in 78.2 p.100. VEPs ware abnormal in 79.9 p.100 of the patients. Applying the Thompson criteria, 57.7 p.100 of the patients had definite PPMS, 38.7 p.100 probable PPMS. Our results are very similar to previous studies and confirm the usefulness of the new proposed criteria, which however should be prospectively tested to determine sensitivity and specificity in a new cohort of patients.

  16. Loss of the proprioception and touch sensation channel PIEZO2 in siblings with a progressive form of contractures. (United States)

    Mahmud, A A; Nahid, N A; Nassif, C; Sayeed, M S B; Ahmed, M U; Parveen, M; Khalil, M I; Islam, M M; Nahar, Z; Rypens, F; Hamdan, F F; Rouleau, G A; Hasnat, A; Michaud, J L


    Dominant mutations in PIEZO2, which codes for the principal mechanotransduction channel for proprioception and touch sensation, have been found to cause different forms of distal arthrogryposis. Some observations suggest that these dominant mutations induce a gain-of-function effect on the channel. Here, we report a consanguineous family with three siblings who showed short stature, scoliosis, gross motor impairment, and a progressive form of contractures involving the distal joints that is distinct from that found in patients with dominant mutations in PIEZO2. These siblings also displayed deficits in proprioception and touch sensation. Whole-exome sequencing performed in the three affected siblings revealed the presence of a rare homozygous variant (c.2708C>G; p.S903*) in PIEZO2. This variant is predicted to disrupt PIEZO2 function by abolishing the pore domain. Sanger sequencing confirmed that all three siblings are homozygous whereas their parents and an unaffected sibling are heterozygous for this variant. Recessive mutations in PIEZO2 thus appear to cause a progressive phenotype that overlaps with, while being mostly distinct from that associated with dominant mutations in the same gene. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  17. Rapidly progressive renal disease as part of Wolfram syndrome in a large inbred Turkish family due to a novel WFS1 mutation (p.Leu511Pro)

    DEFF Research Database (Denmark)

    Yuca, Sevil Ari; Rendtorff, Nanna Dahl; Boulahbel, Houda


    in a large inbred Turkish family. The patients showed early onset of IDDM, diabetes insipidus, optic atrophy, sensorineural hearing impairment and very rapid progression to renal failure before age 12 in three females. Ectopic expression of the wolframin mutant in HEK cells results in greatly reduced levels...

  18. The performance of the progressive resolution optimizer (PRO) for RapidArc planning in targets with low-density media. (United States)

    Kan, Monica W K; Leung, Lucullus H T; Yu, Peter K N


    A new version of progressive resolution optimizer (PRO) with an option of air cavity correction has been implemented for RapidArc volumetric-modulated arc therapy (RA). The purpose of this study was to compare the performance of this new PRO with the use of air cavity correction option (PRO10_air) against the one without the use of the air cavity correction option (PRO10_no-air) for RapidArc planning in targets with low-density media of different sizes and complexities. The performance of PRO10_no-air and PRO10_air was initially compared using single-arc plans created for four different simple heterogeneous phantoms with virtual targets and organs at risk. Multiple-arc planning of 12 real patients having nasopharyngeal carcinomas (NPC) and ten patients having non-small cell lung cancer (NSCLC) were then performed using the above two options for further comparison. Dose calculations were performed using both the Acuros XB (AXB) algorithm with the dose to medium option and the analytical anisotropic algorithm (AAA). The effect of using intermediate dose option after the first optimization cycle in PRO10_air and PRO10_no-air was also investigated and compared. Plans were evaluated and compared using target dose coverage, critical organ sparing, conformity index, and dose homogeneity index. For NSCLC cases or cases for which large volumes of low-density media were present in or adjacent to the target volume, the use of the air cavity correction option in PRO10 was shown to be beneficial. For NPC cases or cases for which small volumes of both low- and high-density media existed in the target volume, the use of air cavity correction in PRO10 did not improve the plan quality. Based on the AXB dose calculation results, the use of PRO10_air could produce up to 18% less coverage to the bony structures of the planning target volumes for NPC cases. When the intermediate dose option in PRO10 was used, there was negligible difference observed in plan quality between

  19. Rapid progressing allele HLA-B35 Px restricted anti-HIV-1 CD8+ T cells recognize vestigial CTL epitopes.

    Directory of Open Access Journals (Sweden)

    Christian B Willberg

    Full Text Available The HLA-B*35-Px allele has been associated with rapid disease progression in HIV-1 infection, in contrast to the HLA-B*35-Py allele.Immune responses to two HLA-B*35 restricted HIV-1 specific CTL epitopes and their variants were followed longitudinally during early HIV-1 infection in 16 HLA-B*35+ individuals. Subjects expressing HLA-B*35-Px alleles showed no difference in response to the consensus epitopes compared to individuals with HLA-B*35-Py alleles. Surprisingly, all the HLA-B*35-Px+ individuals responded to epitope-variants even in the absence of a consensus response. Sequencing of the viral population revealed no evidence of variant virus in any of the individuals.This demonstrates a novel phenomenon that distinguishes individuals with the HLA-B*35-Px rapid progressing allele and those with the HLA-B*35-Py slower progressing allele.

  20. Using SAR and GPS for Hazard Management and Response: Progress and Examples from the Advanced Rapid Imaging and Analysis (ARIA) Project (United States)

    Owen, S. E.; Simons, M.; Hua, H.; Yun, S. H.; Agram, P. S.; Milillo, P.; Sacco, G. F.; Webb, F.; Rosen, P. A.; Lundgren, P.; Milillo, G.; Manipon, G. J. M.; Moore, A. W.; Liu, Z.; Polet, J.; Cruz, J.


    ARIA is a joint JPL/Caltech project to automate synthetic aperture radar (SAR) and GPS imaging capabilities for scientific understanding, hazard response, and societal benefit. We have built a prototype SAR and GPS data system that forms the foundation for hazard monitoring and response capability, as well as providing imaging capabilities important for science studies. Together, InSAR and GPS have the ability to capture surface deformation in high spatial and temporal resolution. For earthquakes, this deformation provides information that is complementary to seismic data on location, geometry and magnitude of earthquakes. Accurate location information is critical for understanding the regions affected by damaging shaking. Regular surface deformation measurements from SAR and GPS are useful for monitoring changes related to many processes that are important for hazard and resource management such as volcanic deformation, groundwater withdrawal, and landsliding. Observations of SAR coherence change have a demonstrated use for damage assessment for hazards such as earthquakes, tsunamis, hurricanes, and volcanic eruptions. These damage assessment maps can be made from imagery taken day or night and are not affected by clouds, making them valuable complements to optical imagery. The coherence change caused by the damage from hazards (building collapse, flooding, ash fall) is also detectable with intelligent algorithms, allowing for rapid generation of damage assessment maps over large areas at fine resolution, down to the spatial scale of single family homes. We will present the progress and results we have made on automating the analysis of SAR data for hazard monitoring and response using data from the Italian Space Agency's (ASI) COSMO-SkyMed constellation of X-band SAR satellites. Since the beginning of our project with ASI, our team has imaged deformation and coherence change caused by many natural hazard events around the world. We will present progress on our

  1. Plasma Leucine-Rich α-2-Glycoprotein 1 Predicts Rapid eGFR Decline and Albuminuria Progression in Type 2 Diabetes Mellitus. (United States)

    Liu, Jian-Jun; Pek, Sharon Li Ting; Ang, Kevin; Tavintharan, Subramaniam; Lim, Su Chi


    Abnormal angiogenesis plays an important role in pathogenesis of diabetic kidney disease (DKD). Leucine-rich α-2 glycoprotein 1 (LRG1) is a newly identified angiogenic factor. To study whether plasma LRG1 may independently predict progression of DKD in individuals with type 2 diabetes mellitus (T2DM). Prospective cohort study in a regional hospital. In total, 1226 T2DM participants were followed for a mean ± standard deviation (SD) of 3.1 ± 0.4 years. Albuminuria progression was defined as elevation in albuminuria level to a higher category. Chronic kidney disease (CKD) progression [rapid estimated glomerular filtration rate (eGFR) decline] was defined as a 40% or greater deterioration in eGFR in 3 years. Both participants with albuminuria progression and those with CKD progression had higher plasma LRG1 levels at baseline. LRG1 independently predicted albuminuria progression above traditional risk factors, including baseline eGFR and urine albumin to creatinine ratio. A 1-SD increment in LRG1 was associated with a 1.26-fold [95% confidence interval (CI), 1.04 to 1.53, P = 0.018] higher adjusted risk for albuminuria progression. The association of LRG1 with microalbuminuria to macroalbuminuria progression was stronger than its association with normoalbuminuria to microalbuminuria progression [odds ratio (OR), 1.51; 95% CI, 1.04 to 2.18, P = 0.029 vs OR, 1.09; 95% CI, 0.86 to 1.37, P = 0.486, per 1-SD LRG1 increment]. Also, LRG1 independently predicted CKD progression above traditional risk factors. A 1-SD increment in LRG1 was associated with a 1.48-fold (95% CI, 1.04 to 2.11, P = 0.032) higher adjusted risk for CKD progression. Plasma LRG1 predicts both albuminuria and CKD progression beyond traditional risk factors. It may play a role in the pathologic pathway leading to progression of DKD in T2DM.

  2. Progression in the Fountain Pen Approach: From 2D Writing to 3D Free-Form Micro/Nanofabrication. (United States)

    Je, Jung Ho; Kim, Jong-Man; Jaworski, Justyn


    The fountain pen approach, as a means for transferring materials to substrates, has shown numerous incarnations in recent years for creating 2D micro/nanopatterns and even generating 3D free-form nanostructures using a variety of material "inks". While the idea of filled reservoirs used to deliver material to a substrate via a capillary remains unchanged since antiquity, the advent of precise micromanipulation systems and functional material "inks" allows the extension of this mechanism to more high-tech applications. Herein, the recent growth in meniscus guided fountain pen approaches for benchtop micro/nanofabrication, which has occurred in the last decade, is discussed. Particular attention is given to the theory, equipment, and experimentation encompassing this unique direct writing approach. A detailed exploration of the diverse ink systems and functional device applications borne from this strategy is put forth to reveal its rapid expansion to a broad range of scientific and engineering disciplines. As such, this informative review is provided for researchers considering adoption of this recent advancement of a familiar technology. © 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  3. Rapid disease progression in human immunodeficiency virus type 1-infected individuals with adverse reactions to trimethoprim-sulfamethoxazole prophylaxis

    NARCIS (Netherlands)

    Veenstra, J.; Veugelers, P. J.; Keet, I. P.; van der Ven, A. J. A. M.; Miedema, F.; Lange, J. M.; Coutinho, R. A.


    We studied the relation between the occurrence of adverse reactions to trimethoprim-sulfamethoxazole (TMP-SMZ) prophylaxis and the subsequent course of human immunodeficiency virus (HIV) infection in a cohort of homosexual men. Adverse reactions to TMP-SMZ were associated with a more rapid

  4. Preparation of a Rapidly Forming Poly(ferrocenylsilane)-Poly(ethylene glycol)-based Hydrogel by a Thiol–Michael Addition Click Reaction

    NARCIS (Netherlands)

    Sui, Xiaofeng; van Ingen, Lennard; Hempenius, Mark A.; Vancso, Gyula J.


    The synthesis of a rapidly forming redox responsive poly(ferrocenylsilane)-poly(ethylene glycol) (PFS-PEG)-based hydrogel is described, achieved by a thiol-Michael addition click reaction. PFS bearing acrylate side groups (PFS-acryl) was synthesized by side group modification of

  5. Optic neuritis and rapidly progressive necrotizing retinitis as the initial signs of subacute sclerosing panencephalitis: a case report with clinical and histopathologic findings. (United States)

    Oray, Merih; Tuncer, Samuray; Kir, Nur; Karacorlu, Murat; Tugal-Tutkun, Ilknur


    We report a case of subacute sclerosing panencephalitis (SSPE) presenting first with optic neuritis and rapidly progressive necrotizing retinitis at the posterior pole. We reviewed the clinical, laboratory, photographic, angiographic, and histopathologic records of a patient with SSPE. A 15-year-old girl was referred after rapid loss of vision due to optic neuritis and macular necrosis in the right eye. She had a history of cardiac valve surgery, but had no systemic symptoms and extensive work-up was unrewarding. Contralateral involvement with rapidly progressive optic neuritis and macular necrotizing retinitis prompted retinochoroidal biopsy of the right eye, which revealed necrosis of inner retinal layers and perivascular lymphoplasmocytic infiltration with intact choroid and outer retina without any findings of inclusion bodies, microorganisms, or atypical cells. The diagnosis was based on histopathologic findings consistent with SSPE, and detection of elevated measles antibody titers in cerebrospinal fluid and serum. It was further confirmed by development of typical electroencephalography pattern at 6 months and neurological symptoms at 4-year follow-up. Clinicians need to be aware that optic neuritis and necrotizing retinitis at the posterior pole may be the presenting features of SSPE.

  6. Distinguishing autonomous and directive forms of goal support: their effects on goal progress, relationship quality, and subjective well-being. (United States)

    Koestner, Richard; Powers, Theodore A; Carbonneau, Noémie; Milyavskaya, Marina; Chua, Sook Ning


    Three studies examined the relations of autonomy support and directive support to goal progress over 3 months. Autonomy support was defined in terms of empathic perspective-taking, whereas directive support was defined in terms of the provision of positive guidance. Results from Study 1 revealed that autonomy support between romantic partners was significantly positively related to goal progress over 3 months, and that the beneficial effect of autonomy support was mediated by enhanced autonomous goal motivation. Study 2 involved female friend dyads and extended the goal progress results to include both self-reports and reports by peers. Study 3 showed that autonomy support similarly promoted progress at vicarious goals. Across three studies, autonomy support was also significantly associated with improved relationship quality and subjective well-being. Directive support was marginally associated with better goal progress across the three studies and unrelated to relationship quality or well-being.

  7. Headache attack followed by rapid disease progression in pediatric moyamoya disease--how should we manage it? (United States)

    Vuignier, Sandra; Akioka, Naoki; Hamada, Hideo; Kashiwazaki, Daina; Kuroda, Satoshi


    A 4-year-old female was presented at our hospital with frequent right frontal headache attack. She was diagnosed with moyamoya disease and was conservatively followed up. One year later, the frequency of headache gradually decreased. However, follow-up MR imaging revealed that the disease stage markedly progressed in the right side and cerebral infarction occurred in the temporal lobe with atrophy of the right frontal lobe. She underwent direct and indirect revascularization on the right side. Aware of this case, we would like to emphasize that headache may be one subtype of ischemic attacks and require frequent MR follow-up to see the disease course. If there is any sign of disease progression, immediate surgical intervention should be indicated to avoid irreversible brain damage.

  8. EU-Approved Rapid Tests for Bovine Spongform Encephalopathy Detect Atypical Forms: A Study for Their Sensitivities

    NARCIS (Netherlands)

    Meloni, D.; Davidse, A.; Langeveld, J.P.M.; varello, K.; Casalone, C.; Corona, C.; Balkema-Buschmann, A.; Groschup, M.; Ingravalle, F.; Bozzetta, E.


    Since 2004 it become clear that atypical bovine spongiform encephalopthies (BSEs) exist in cattle. Whenever their detection has relied on active surveillance plans implemented in Europe since 2001 by rapid tests, the overall and inter-laboratory performance of these diagnostic systems in the

  9. Rapid Mechanistic Evaluation and Parameter Estimation of Putative Inhibitors in a Single-Step Progress-Curve Analysis: The Case of Horse Butyrylcholinesterase

    Directory of Open Access Journals (Sweden)

    Jure Stojan


    Full Text Available Highly efficient and rapid lead compound evaluation for estimation of inhibition parameters and type of inhibition is proposed. This is based on a single progress-curve measurement in the presence of each candidate compound, followed by the simultaneous analysis of all of these curves using the ENZO enzyme kinetics suite, which can be implemented as a web application. In the first step, all of the candidate ligands are tested as competitive inhibitors. Where the theoretical curves do not correspond to the experimental data, minimal additional measurements are added, with subsequent processing according to modified reaction mechanisms.

  10. Rapid Mechanistic Evaluation and Parameter Estimation of Putative Inhibitors in a Single-Step Progress-Curve Analysis: The Case of Horse Butyrylcholinesterase. (United States)

    Stojan, Jure


    Highly efficient and rapid lead compound evaluation for estimation of inhibition parameters and type of inhibition is proposed. This is based on a single progress-curve measurement in the presence of each candidate compound, followed by the simultaneous analysis of all of these curves using the ENZO enzyme kinetics suite, which can be implemented as a web application. In the first step, all of the candidate ligands are tested as competitive inhibitors. Where the theoretical curves do not correspond to the experimental data, minimal additional measurements are added, with subsequent processing according to modified reaction mechanisms.

  11. Efficacy of concurrent treatments in idiopathic pulmonary fibrosis patients with a rapid progression of respiratory failure: an analysis of a national administrative database in Japan. (United States)

    Oda, Keishi; Yatera, Kazuhiro; Fujino, Yoshihisa; Ishimoto, Hiroshi; Nakao, Hiroyuki; Hanaka, Tetsuya; Ogoshi, Takaaki; Kido, Takashi; Fushimi, Kiyohide; Matsuda, Shinya; Mukae, Hiroshi


    Some IPF patients show a rapid progression of respiratory failure. Most patients are treated with high-dose corticosteroids. However, no large clinical studies have investigated the prognosis or efficacy of combined treatments including high-dose corticosteroids in IPF patients with a rapid progression of respiratory failure. We enrolled IPF patients who received mechanical ventilation and high-dose corticosteroids between April 2010 and March 2013. Records were extracted from a Japanese nationwide inpatient database. We conducted a retrospective epidemiologic and prognostic analysis. Two hundred nine patients receiving an average of 12.8 days of ventilatory support were enrolled. There were 138 (66 %) fatal cases; the median survival was 21 days. The short-term (within 30 days) and long-term (within 90 days) survival rates were 44.6 and 24.6 %, respectively. The average monthly admission rate among the IPF patients with the rapid progression of respiratory failure in the winter was significantly higher than that in spring (p = 0.018). Survival did not differ to a statistically significant extent in the different geographic areas of Japan. Survivors were significantly younger (p = 0.002) with higher rates of mild dyspnea on admission (p = 0.012), they more frequently underwent bronchoscopy (p 80 years of age (OR = 2.94, 95 % Cl 1.044-8.303; p = 0.041) and the intravenous administration of high-dose cyclophosphamide (OR = 3.17, 95 % Cl 1.101-9.148; p = 0.033). Undergoing bronchoscopy during intubation (OR = 0.25, 95 % Cl 0.079-0.798; p = 0.019) and the administration of co-trimoxazole (OR = 0.28, 95 % Cl 0.132-0.607; p = 0.001) and macrolides (OR = 0.37, 95 % Cl 0.155-0.867; p = 0.033) were significantly associated with a good prognosis. The dosage of co-trimoxazole significantly correlated with survival. Co-trimoxazole and macrolides may be a good addition to high-dose corticosteroids in the

  12. Emergence of CXCR4-tropic HIV-1 variants followed by rapid disease progression in hemophiliac slow progressors.

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    Tsunefusa Hayashida

    Full Text Available The association between emergence of CXCR4-tropic HIV-1 variants (X4 variants and disease progression of HIV-1 infection has been reported. However, it is not known whether the emergence of X4 variants is the cause or result of HIV-1 disease progression. We tried to answer this question.HIV-1 env sequences around the V3 region were analyzed in serially stocked samples in order to determine whether X4 variants emerged before or after the fall in CD4+ T-cell count.The study subjects were five HIV-1-infected hemophiliac slow progressors. Deep sequencing around the HIV-1 env V3 region was conducted in duplicate. Tropism was predicted by geno2pheno [coreceptor] 2.5 with cutoff value of false positive ratio at <5%. When X4 variant was identified in the latest stocked sample before the introduction of antiretroviral therapy, we checked viral genotype in previously stocked samples to determine the time of emergence of X4 variants.Emergence of X4 variants was noted in two of the five patients when their CD4+ T-cell counts were still high. The rate of decrease of CD4+ T-cell count or of rise of HIV-1 load accelerated significantly after the emergence of X4 variants in these two cases. Phylogenetic analysis showed that these X4 variants emerged from CCR5-tropic HIV-1 viruses with several amino acid changes in the V3 region.The emergence of X4 variants preceded HIV-1 disease progression in two hemophiliac slow progressors.

  13. EMAST is a Form of Microsatellite Instability That is Initiated by Inflammation and Modulates Colorectal Cancer Progression

    Directory of Open Access Journals (Sweden)

    John M. Carethers


    Full Text Available DNA mismatch repair (MMR function is critical for correcting errors coincident with polymerase-driven DNA replication, and its proteins are frequent targets for inactivation (germline or somatic, generating a hypermutable tumor that drives cancer progression. The biomarker for defective DNA MMR is microsatellite instability-high (MSI-H, observed in ~15% of colorectal cancers, and defined by mono- and dinucleotide microsatellite frameshift mutations. MSI-H is highly correlated with loss of MMR protein expression, is commonly diploid, is often located in the right side of the colon, prognosticates good patient outcome, and predicts poor efficacy with 5-fluorouracil treatment. Elevated microsatellite alterations at selected tetranucleotide repeats (EMAST is another form of MSI at tetranucleotide repeats that has been observed in multiple cancers, but its etiology and clinical relevance to patient care has only been recently illuminated. Specifically, EMAST is an acquired somatic defect observed in up to 60% of colorectal cancers and caused by unique dysfunction of the DNA MMR protein MSH3 (and its DNA MMR complex MutSβ, a heterodimer of MSH2-MSH3, and in particular a loss-of-function phenotype due to a reversible shift from its normal nuclear location into the cytosol in response to oxidative stress and the pro-inflammatory cytokine interleukin-6. Tumor hypoxia may also be a contributor. Patients with EMAST colorectal cancers show diminished prognosis compared to patients without the presence of EMAST in their cancer. In addition to defective DNA MMR recognized by tetranucleotide (and di- and tri-nucleotide frameshifts, loss of MSH3 also contributes to homologous recombination-mediated repair of DNA double stranded breaks, indicating the MSH3 dysfunction is a complex defect for cancer cells that generates not only EMAST but also may contribute to chromosomal instability and aneuploidy. Areas for future investigation for this most common DNA MMR

  14. EMAST is a Form of Microsatellite Instability That is Initiated by Inflammation and Modulates Colorectal Cancer Progression. (United States)

    Carethers, John M; Koi, Minoru; Tseng-Rogenski, Stephanie S


    DNA mismatch repair (MMR) function is critical for correcting errors coincident with polymerase-driven DNA replication, and its proteins are frequent targets for inactivation (germline or somatic), generating a hypermutable tumor that drives cancer progression. The biomarker for defective DNA MMR is microsatellite instability-high (MSI-H), observed in ~15% of colorectal cancers, and defined by mono- and dinucleotide microsatellite frameshift mutations. MSI-H is highly correlated with loss of MMR protein expression, is commonly diploid, is often located in the right side of the colon, prognosticates good patient outcome, and predicts poor efficacy with 5-fluorouracil treatment. Elevated microsatellite alterations at selected tetranucleotide repeats (EMAST) is another form of MSI at tetranucleotide repeats that has been observed in multiple cancers, but its etiology and clinical relevance to patient care has only been recently illuminated. Specifically, EMAST is an acquired somatic defect observed in up to 60% of colorectal cancers and caused by unique dysfunction of the DNA MMR protein MSH3 (and its DNA MMR complex MutSβ, a heterodimer of MSH2-MSH3), and in particular a loss-of-function phenotype due to a reversible shift from its normal nuclear location into the cytosol in response to oxidative stress and the pro-inflammatory cytokine interleukin-6. Tumor hypoxia may also be a contributor. Patients with EMAST colorectal cancers show diminished prognosis compared to patients without the presence of EMAST in their cancer. In addition to defective DNA MMR recognized by tetranucleotide (and di- and tri-nucleotide) frameshifts, loss of MSH3 also contributes to homologous recombination-mediated repair of DNA double stranded breaks, indicating the MSH3 dysfunction is a complex defect for cancer cells that generates not only EMAST but also may contribute to chromosomal instability and aneuploidy. Areas for future investigation for this most common DNA MMR defect among

  15. Polymerization of Vinylpyrrolidone to Form a Neutral Coating on Anionic Nanomaterials in Aqueous Suspension for Rapid Sedimentation


    Lai, Edward P. C.; Zafar Iqbal; Sherif Nour


    Nanomaterials in water present an array of identifiable potential hazards to ecological and human health. There is no general consensus about the influence of anionic or cationic charge on the toxicity of nanomaterials on environmental ecology. One challenge is the limited number of scalable technologies available for the removal of charged nanomaterials from water. A new method based on polymer coating has been developed in our laboratory for rapid sedimentation of nanomaterials in aqueous s...

  16. Case report of a 28-year-old male with the rapid progression of steroid-resistant central nervous system vasculitis diagnosed by a brain biopsy. (United States)

    Takahashi, Keigo; Sato, Hideki; Hattori, Hidenori; Takao, Masaki; Takahashi, Shinichi; Suzuki, Norihiro


    A 28-year-old Japanese male without a significant past medical history presented with new-onset generalized clonic seizure and headache. A brain MRI revealed multiple enhanced lesions on both cerebral hemispheres. Laboratory exams showed no evidence of systemic inflammation or auto-immune antibodies such as ANCAs. Despite four courses of high-dose methylprednisolone pulse therapy and five treatments with plasmapheresis, his symptoms worsened and the MRI lesions progressed rapidly. During these treatments, we performed a targeted brain biopsy, that revealed histological findings consistent with a predominant angiitis of parenchymal and subdural small vessels. He was provided with diagnosis of central nervous system vasculitis (CNSV). Subsequent cyclophosphamide pulse therapy enabled a progressive successful improvement of his symptoms. While diagnostic methods for CNSV remain controversial, histological findings are thought to be more useful in obtaining a more definitive diagnosis than findings in image studies, such as MRI and angiography. We suggest that a brain biopsy should be considered during the early period of cases with suspected CNSV and rapid clinical deterioration. We also detected human herpesvirus 7 (HHV-7) using PCR technology in brain biopsy specimens, however the relationship between CNSV and HHV-7 infection is unknow.

  17. Outcome analysis of aromatase inhibitor therapy to increase adult height in males with predicted short adult stature and/or rapid pubertal progress: a retrospective chart review. (United States)

    Shams, Kim; Cameo, Tamara; Fennoy, Ilene; Hassoun, Abeer A; Lerner, Shulamit E; Aranoff, Gaya S; Sopher, Aviva B; Yang, Christine; McMahon, Donald J; Oberfield, Sharon E


    Aromatase inhibitors (AIs) have been used off-label to increase adult height in short adolescent males. Studies have shown that AIs increase the predicted adult height (PAH) while delaying bone age (BA) maturation. We sought to determine whether AI therapy increases PAH in boys with short stature or rapid pubertal progression, and to evaluate any untoward effects. The charts of 27 boys with BA ≥ 13 and short stature [height ≥ 2 standard deviation (SD) below the mean or ≥ 2 SD below mid-parental target height (MPTH)] or rapid pubertal progress, treated with anastrozole were reviewed. Outcome measures included anthropomorphic, hormonal, and metabolic data. The AI therapy averaged 21 months (range 14-30 months) for all, with Rx group 1 receiving height SDS, or BA/chronological age (CA). In Rx group 2, there was a small, nonsignificant increase in PAH, no change in height SDS, and a small decrease in BA/CA. Post-therapy PAH was different from MPTH in all and in both Rx groups 1 and 2, pheight, averaging 6.73 ± 1.40 cm less than MPTH and 1.91 ± 0.86 cm less than the pre-therapy PAH. Post-therapy, the initially decreased estradiol did not persist but mildly increased testosterone and decreased high-density lipoprotein were noted, as was an increase in hematocrit, and decrease in growth velocity. We suggest that although bone age progression may be slightly delayed with longer duration of therapy, an overall short-term AI therapy does not lead to a final height that is greater than the predicted pre-therapy height.

  18. Development of a definition for Rapid Progression (RP) of renal function in HIV-positive persons: the D:A:D study. (United States)

    Kamara, David A; Ryom, Lene; Ross, Michael; Kirk, Ole; Reiss, Peter; Morlat, Philippe; Moranne, Olivier; Fux, Christoph A; Mocroft, Amanda; Sabin, Caroline; Lundgren, Jens D; Smith, Colette J


    No consensus exists on how to define abnormally rapid deterioration in renal function (Rapid Progression, RP). We developed an operational definition of RP in HIV-positive persons with baseline estimated glomerular filtration rate (eGFR) >90 ml/min/1.73 m2 (using Cockcroft Gault) in the Data Collection on Adverse Events of Anti-HIV Drugs (D:A:D) study from 2004 to 2011. Two definitions were evaluated; RP definition A: An average eGFR decline (slope) ≥5 ml/min/1.73 m2/year over four years of follow-up with ≥3 eGFR measurements/year, last eGFR definition B: An absolute annual decline ≥5 ml/min/1.73 m2/year in each year and last eGFR definition A; similar proportions were observed when considering follow-up periods of three (n=195/6375; 3.1%) and two years (n=355/10756; 3.3%). In contrast under RP definition B, greater proportions experienced RP when considering two years (n=476/10756; 4.4%) instead of three (n=48/6375; 0.8%) or four (n=15/3655; 0.4%) years' follow-up. For RP definition A, 13 (12%) individuals who experienced RP progressed to CKD, and only (21) 0.6% of those without RP progressed to CKD (sensitivity 38.2% and specificity 97.4%); whereas for RP definition B, fewer RP individuals progressed to CKD. Our results suggest using three years' follow-up and at least two eGFR measurements per year is most appropriate for a RP definition, as it allows inclusion of a reasonable number of individuals and is associated with the known risk factors. The definition does not necessarily identify all those that progress to incident CKD, however, it can be used alongside other renal measurements to early identify and assess those at risk of developing CKD. Future analyses will use this definition to identify other risk factors for RP, including the role of antiretrovirals.


    NARCIS (Netherlands)


    When cyanide poisoning is treated with a methemoglobin-forming agent, oxidative metabolism is protected at the expense of the oxygen capacity of the blood.The affinity of methemoglobin for CN- is high enough to compete with cytochrome oxidase, which protects the latter from becoming blocked, but all

  20. Rapidly progressing subperiosteal orbital abscess: an unexpected complication of a group-A streptococcal pharyngitis in a healthy young patient

    Directory of Open Access Journals (Sweden)

    Costantinides Fulvia


    Full Text Available Abstract Introduction Complications associated to group-A streptococcal pharyingitis include non-suppurative complications such as acute rheumatic fever and glomerulonephritis and suppurative complications such as peritonsillar or retropharyngeal abscess, sinusitis, mastoiditis, otitis media, meningitis, brain abscess, or thrombosis of the intracranial venous sinuses. Case presentation We described a case of a 15-year-old patient with a history of acute pharyngodinia early followed by improvise fever and a progressive formation of a diffuse orbital edema, corneal hyperaemia, diplopia and severe decrease of visual acuity. The patient was surgically treated with functional endoscopic sinus surgery (FESS after the response of a maxillofacial computed tomography scans that showed a pansinusitis complicated by a left orbital cellulites. Numerous colonies of Streptococcus pyogenes were found in the samples of pus and an antibiotic therapy with meropenem was initiated on the basis of the sensitivity test to antibiotics. The patient was finally discharged with diagnosis of left orbital cellulites with periorbital abscess, endophtalmitis and acute pansinusitis as a consequence of streptococcal pharyngitis. Conclusion The case highlights the possible unusual complication of a group-A streptococcal pharyingitis in a immunocompetent child and the needing of a prompt surgical and medical approach toward the maxillofacial complications associated to the infection.

  1. Rapid progressive visual decline and visual field defects in two patients with the Heidenhain variant of Creutzfeld-Jakob disease. (United States)

    Lenk, Janine; Engellandt, Kay; Terai, Naim; Bottesi, Antonia; Matthé, Egbert


    Heidenhain variant of Creutzfeldt-Jakob (HvCJD) is a rare disease, patients presenting with loss of visual acuity and a decline in visual fields. Two patients with rapid loss of visual acuity and declining visual fields presented with homonymic hemianopsia over several weeks. Cranial MRI showed neither stroke nor other morphological changes explaining the severe visual field defects. Neurological examination revealed no pathologies. However, lumbar puncture showed an increase in total protein in cerebrospinal fluid (CSF). Visual field testing revealed further deterioration during follow-up. Several weeks later, patients' behaviour changed markedly, exhibiting aggression, declining memory function and physical degeneration. The suspected diagnosis was the Heidenhain variant of Creutzfeld-Jakob disease (HvCJD). CSF analysis showed evidence of PrP Sc and 14-3-3 protein. Both patients died within 8 weeks of the CJD diagnosis. Loss of visual acuity and a decline in visual fields without corresponding MRI findings and marked changes in behaviour should lead to a diagnosis of HvCJD. Corresponding diagnostic tests should be performed for confirmation. The prognosis for survival is poor and should be immediately communicated to affected patients and their relatives. Copyright © 2018 Elsevier Ltd. All rights reserved.

  2. Atypical rapid progression of osteoarticular amyloidosis involving the hip in a patient on hemodialysis using polyacrylonitrile membranes

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kenneth S. [University of Wisconsin School of Medicine and Public Health, Department of Radiology, University of Wisconsin Hospital and Clinics, Madison, WI (United States); Holsbeeck, Marnix T. van [Wayne State School of Medicine, Department of Radiology, Henry Ford Hospital, Detroit, MI (United States); Abbud, Alexander [Wayne State School of Medicine, Department of Pathology, Henry Ford Hospital, Detroit, MI (United States)


    Amyloidosis related to dialysis is a well-known complication affecting many organ systems, in particular the musculoskeletal system. In 1985 Shirahama et al. (Biochem Biophys Res Commun 53:705-709, 1985) identified beta-2 microglobulin (MG) as the offending constituent by using protein purification techniques. Amyloidosis has been increasing in prevalence because of longer life spans and increased chronic medical conditions such as end-stage renal disease. When dialysis-related amyloidosis involves the musculoskeletal system, it affects the shoulder girdle, the so called shoulder pad sign, the wrist, hip, knee, and spine (Resnick, Diagnosis of bone and joint disorders, 4th edn., pp. 2054-2058 and 2176-2183, 2002). Other osteoarticular manifestations of amyloidosis include osteoporosis, lytic lesions, and pathologic fractures. It has been well documented that the prevalence of amyloid is dependent on duration of dialysis - over 90% in patients on dialysis for over 7 years (Jadoul, Nephrol Dial Transplant 13:61-64, 1998). However, a recent changeover to high-flux membranes used in hemofiltration has been reported to delay its onset (Campistol et al., Contrib Nephrol 125:76-85, 1999). We report on the radiographic, nuclear medicine, and computed tomography (CT) findings of osteoarticular amyloidosis involving the hip, and sequence its atypical rapid onset. The imaging, histopathological findings, and differential diagnosis are discussed. (orig.)

  3. Formulation and experimental evaluation of closed-form control laws for the rapid maneuvering of reactor neutronic power

    Energy Technology Data Exchange (ETDEWEB)

    Bernard, J.A. (Massachusetts Inst. of Tech., Cambridge, MA (USA). Nuclear Reactor Lab.)


    This report describes both the theoretical development and the experimental evaluation of a novel, robust methodology for the time-optimal adjustment of a reactor's neutronic power under conditions of closed-loop digital control. Central to the approach are the MIT-SNL Period-Generated Minimum Time Control Laws' which determine the rate at which reactivity should be changed in order to cause a reactor's neutronic power to conform to a specified trajectory. Using these laws, reactor power can be safely raised by five to seven orders of magnitude in a few seconds. The MIT-SNL laws were developed to facilitate rapid increases of neutronic power on spacecraft reactors operating in an SDI environment. However, these laws are generic and have other applications including the rapid recovery of research and test reactors subsequent to an unanticipated shutdown, power increases following the achievement of criticality on commercial reactors, power adjustments on commercial reactors so as to minimize thermal stress, and automated startups. The work reported here was performed by the Massachusetts Institute of Technology under contract to the Sandia National Laboratories. Support was also provided by the US Department of Energy's Division of University and Industry Programs. The work described in this report is significant in that a novel solution to the problem of time-optimal control of neutronic power was identified, in that a rigorous description of a reactor's dynamics was derived in that the rate of change of reactivity was recognized as the proper control signal, and in that extensive experimental trials were conducted of these newly developed concepts on actual nuclear reactors. 43 refs., 118 figs., 11 tabs.

  4. A rapid molecular method for differentiating two special forms (lycopersici and radicis-lycopersici) of Fusarium oxysporum. (United States)

    Attitalla, Idress H; Fatehi, Jamshid; Levenfors, Jens; Brishammar, Sture


    Two pathogenic special forms (f. sp.) of the Fusarium oxysporum species complex f. sp. lycopersici (Fol) and f. sp. radicis-lycopersici (Forl) are morphologically indistinguishable. Although they are pathogenic to the same host genus Lycopersicon (tomato), and infect the same tomato cultivar, they form distinct diseases; Fol causes wilt and Forl causes crown rot and root rot. These two special forms apparently exist as genetically isolated populations, based on vegetative compatibility and molecular variation at the DNA level. In seeking efficient diagnostic tools for differentiating Fol and Forl isolates, we examined three techniques: isozyme analysis, mitochondrial DNA (mtDNA) RFLP by HaeIII-digestion of total genomic DNA, and an osmotic method using high performance liquid chromatography (HPLC) to detect fungal pigments. The isolates were collected from geographically widespread locations. Distinct HPLC-profile differences were found between an endophytic non-pathogenic isolate and the other pathogenic isolates. However, the direct mtDNA RFLP technique proved to be an efficient diagnostic tool for routine differentiation of Fol and Forl isolates.

  5. Potential for energy conservaton in the metal forming industries. Progress report, July 1, 1978-August 15, 1979

    Energy Technology Data Exchange (ETDEWEB)

    Avitzur, B.; Beidleman, C.R.; Smackey, B.M.


    Reduced energy consumption and improved product attributes are realizable benefits that are achievable through the adoption of optimal metal forming techniques. With the meteoric rise in energy costs, certain segments of the metal forming industries have accelerated their efforts in switching from energy intensive manufacturing techniques, e.g., casting, to metal forming, and, furthermore, from hot forming and machined components to cold forming, notably the automotive industry. The first year results of a two year study that will identify and document potential energy and cost savings associated with the adoption of low energy consumption techniques are presented. When compared with techniques requiring energy for hot forming, heat treatments, and excess or scrap material, the utilizaton of alternative metal forming processes offer considerable promise for energy savings. Descriptions of savings achieved by a combination of analytical methods and imaginative new processes are provided in the form of specific industrial case studies. The elimination of defects through the use of an analytical criteria for the prevention of the central burst is presented. Such available criteria for central burst serve as a justification for the desirability to develop criteria for the prevention of fishskin and other defects. Other savings which may be possible through the development of new technologies are included in an Appendix entitled: Recent Developments in Wire Making. One specific new process, Continuous Hydrostatic Extrusion, has been developed at Western Electric and is described in detail.

  6. [Language deficits in major forms of dementia and primary progressive aphasias: an update according to new diagnostic criteria]. (United States)

    Macoir, Joël; Laforce, Robert; Monetta, Laura; Wilson, Maximiliano


    In this review, we report current data on spoken and written language disorders in the most frequent dementia syndromes, namely Alzheimer' disease, vascular cognitive impairment and dementia with Lewy bodies. Language deficits are also the core features of three variants of primary progressive aphasia, namely the nonfluent/agrammatic, semantic and logopenic variants. This review reveals that, like other cognitive functions, language is highly vulnerable to neurodegenerative diseases. For some, language deficits result from impairment in linguistic processes per se, while for others, they are the direct consequence of impairments affecting working memory and executive functions. Language deficits in Alzheimer's disease and in nonfluent/agrammatic and semantic variants of primary progressive aphasia are well documented. By contrast, those about vascular cognitive impairment and dementia with Lewy bodies remain scarce and limited to large cognitive domains. The identification of logopenic variant of primary progressive aphasia is very recent, and more research is needed to complete the clinical description and identification of the functional origin of the disorders. Finally, knowledge on the impairment of written language in neurodegenerative diseases is less well documented than those on spoken language deficits. Other studies are therefore needed to improve the description of linguistic profiles and to provide additional elements to help in the differential diagnosis.

  7. Polymerization of Vinylpyrrolidone to Form a Neutral Coating on Anionic Nanomaterials in Aqueous Suspension for Rapid Sedimentation

    Directory of Open Access Journals (Sweden)

    Edward P. C. Lai


    Full Text Available Nanomaterials in water present an array of identifiable potential hazards to ecological and human health. There is no general consensus about the influence of anionic or cationic charge on the toxicity of nanomaterials on environmental ecology. One challenge is the limited number of scalable technologies available for the removal of charged nanomaterials from water. A new method based on polymer coating has been developed in our laboratory for rapid sedimentation of nanomaterials in aqueous suspension. Using colloidal silica as a model inorganic oxide, coating of polyvinylpyrrolidone (PVP around the SiO2 nanoparticles produced SiO2@PVP particles, as indicated by a linear increase of nephelometric turbidity. Purification of the water sample was afforded by total sedimentation of SiO2@PVP particles when left for 24 h. Characterization by capillary electrophoresis (CE revealed nearly zero ionic charge on the particles. Further coating of polydopamine (PDA around those particles in aqueous suspension produced an intense dark color due to the formation of SiO2@PVP@PDA. The SiO2@PVP@PDA peak appeared at a characteristic migration time of 4.2 min that allowed for quantitative CE-UV analysis to determine the original SiO2 concentration with enhanced sensitivity and without any ambiguous identity.

  8. Rapid degradation of an active formylglycine generating enzyme variant leads to a late infantile severe form of multiple sulfatase deficiency. (United States)

    Schlotawa, Lars; Radhakrishnan, Karthikeyan; Baumgartner, Matthias; Schmid, Regula; Schmidt, Bernhard; Dierks, Thomas; Gärtner, Jutta


    Multiple sulfatase deficiency (MSD) is a rare inborn error of metabolism affecting posttranslational activation of sulfatases by the formylglycine generating enzyme (FGE). Due to mutations in the encoding SUMF1 gene, FGE's catalytic capacity is impaired resulting in reduced cellular sulfatase activities. Both, FGE protein stability and residual activity determine disease severity and have previously been correlated with the clinical MSD phenotype. Here, we report a patient with a late infantile severe course of disease. The patient is compound heterozygous for two so far undescribed SUMF1 mutations, c.156delC (p.C52fsX57) and c.390A>T (p.E130D). In patient fibroblasts, mRNA of the frameshift allele is undetectable. In contrast, the allele encoding FGE-E130D is expressed. FGE-E130D correctly localizes to the endoplasmic reticulum and has a very high residual molecular activity in vitro (55% of wildtype FGE); however, it is rapidly degraded. Thus, despite substantial residual enzyme activity, protein instability determines disease severity, which highlights that potential MSD treatment approaches should target protein folding and stabilization mechanisms.

  9. Chronic Subdural Hematoma development in Accelerated phase of Chronic Myeloid Leukaemia presenting with seizure and rapid progression course with fatal outcome

    Directory of Open Access Journals (Sweden)

    Raheja Amol


    Full Text Available Occurrence of chronic subdural hematoma (CSDH in leukemia is rare, and most reported cases occurred in relation with acute myeloid leukaemia; however, occurrence is extremely rare in accelerated phase of chronic myelogenous leukaemia (CML. Seizure as presentation of SDH development in CML cases is not reported in literature. Authors report an elderly male, who was diagnosed as CML, accelerated phase of developing SDH. Initially presented to local physician with seizure; urgent CT scan head was advised, but ignored and sensorium rapidly worsened over next day and reported to our emergency department in deeply comatose state, where imaging revealed chronic subdural hematoma with hypoxic brain injury with fatal outcome. Seizure, progressive worsening of headache, vomiting and papilloedema are harbinger of intracranial space occupying lesion and requires CT head in emergency medical department for exclusion, who are receiving treatment of haematological malignancy

  10. A galaxy rapidly forming stars 700 million years after the Big Bang at redshift 7.51. (United States)

    Finkelstein, S L; Papovich, C; Dickinson, M; Song, M; Tilvi, V; Koekemoer, A M; Finkelstein, K D; Mobasher, B; Ferguson, H C; Giavalisco, M; Reddy, N; Ashby, M L N; Dekel, A; Fazio, G G; Fontana, A; Grogin, N A; Huang, J-S; Kocevski, D; Rafelski, M; Weiner, B J; Willner, S P


    Of several dozen galaxies observed spectroscopically that are candidates for having a redshift (z) in excess of seven, only five have had their redshifts confirmed via Lyman α emission, at z = 7.008, 7.045, 7.109, 7.213 and 7.215 (refs 1-4). The small fraction of confirmed galaxies may indicate that the neutral fraction in the intergalactic medium rises quickly at z > 6.5, given that Lyman α is resonantly scattered by neutral gas. The small samples and limited depth of previous observations, however, makes these conclusions tentative. Here we report a deep near-infrared spectroscopic survey of 43 photometrically-selected galaxies with z > 6.5. We detect a near-infrared emission line from only a single galaxy, confirming that some process is making Lyman α difficult to detect. The detected emission line at a wavelength of 1.0343 micrometres is likely to be Lyman α emission, placing this galaxy at a redshift z = 7.51, an epoch 700 million years after the Big Bang. This galaxy's colours are consistent with significant metal content, implying that galaxies become enriched rapidly. We calculate a surprisingly high star-formation rate of about 330 solar masses per year, which is more than a factor of 100 greater than that seen in the Milky Way. Such a galaxy is unexpected in a survey of our size, suggesting that the early Universe may harbour a larger number of intense sites of star formation than expected.

  11. Higher body mass index in adults at diagnosis of the slowly progressive form of type 1 diabetes mellitus is associated with lower risk HLA genes. (United States)

    Fourlanos, S; Elkassaby, S; Varney, M D; Colman, P G; Harrison, L C


    We hypothesised that higher body weight, a proposed risk factor for type 1 diabetes mellitus, would be associated with increased penetrance of lower risk genes. In adults at diagnosis of the slowly progressive form of type 1 diabetes mellitus we found that higher body mass index was associated with the absence of the highest risk HLA genes. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  12. Identification de programmes d'activation macrophagique et microgliale dans les formes progressives de la sclérose en plaques


    Lhuillier, Alice


    Multiple sclerosis (MS) is a chronic neuro-inflammatory disease and the most common cause of chronic neurological disability in young adults. No treatment is currently available to prevent the aggravation of symptoms in the progressive forms of the disease. The involvement of macrophages and microglia in demyelination and axonal injury is well known but need to be further characterized. Especially, the high level of functional plasticity harboured by macrophages/ microglia suggests that these...

  13. Psychometric Study of the advanced form of the Raven’s Progressive Matrices Test in university students


    Delgado Vasquez, Ana; Facultad de Psicología, Universidad Nacional Mayor de San Marcos, Lima, Perú; Escurra Mayaute, Luis M.; Facultad de Psicología, Universidad Nacional Mayor de San Marcos, Lima, Perú; Bulnes Bedón, Mario; Facultad de Psicología, Universidad Nacional Mayor de San Marcos, Lima, Perú; Quesada Murillo, María Rosario; Facultad de Psicología, Universidad Nacional Mayor de San Marcos, Lima, Perú


    It is analized the psychometric properties of Raven’s Advanced Progressive Matrices. The participants were 501 Freshmen students of 5 professional careers of the Universidad Nacional Mayor de San Marcos (U.N.M.S.M.), a public university located at Lima, Perú. The reliability analysis shows that the test is reliable. Also, the construct validity analysis meets the requirements of test making. The norms were elaborated considering the professional careers of the U.N.M.SM. students. It was find ...

  14. Form and function of damselfish skulls: rapid and repeated evolution into a limited number of trophic niches (United States)

    Cooper, W James; Westneat, Mark W


    biomechanically defined link between structure and the functional ecology of fish skulls, and indicate that certain mechanisms for transmitting motion through their jaw linkages may require particular anatomical configurations, a conclusion that contravenes the concept of "many-to-one mapping" for fish jaw mechanics. Damselfish trophic evolution is characterized by rapid and repeated shifts between a small number of eco-morphological states, an evolutionary pattern that we describe as reticulate adaptive radiation. PMID:19183467

  15. Form and function of damselfish skulls: rapid and repeated evolution into a limited number of trophic niches

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    Cooper W James


    data support a tight and biomechanically defined link between structure and the functional ecology of fish skulls, and indicate that certain mechanisms for transmitting motion through their jaw linkages may require particular anatomical configurations, a conclusion that contravenes the concept of "many-to-one mapping" for fish jaw mechanics. Damselfish trophic evolution is characterized by rapid and repeated shifts between a small number of eco-morphological states, an evolutionary pattern that we describe as reticulate adaptive radiation.

  16. Considering Valproate as a Risk Factor for Rapid Exacerbation of Complex Movement Disorder in Progressed Stages of Late-Infantile CLN2 Disease. (United States)

    Johannsen, Jessika; Nickel, Miriam; Schulz, Angela; Denecke, Jonas


    Neuronal ceroid lipofuscinosis type 2 (CLN2 disease, OMIM 204500) is a rare autosomal-recessive lysosomal storage disorder. It is one of the most common neurodegenerative disorders in childhood. Symptoms include epilepsy, rapid motor and language regression, dementia, visual loss, and a complex movement disorder in later stages of the disease. We report on two children with genetically confirmed late-infantile CLN2 disease who developed a severe exacerbation of their complex movement disorder leading to hyperthermia, hyper-CK-emia and decreased level of consciousness over several weeks despite different therapeutic approaches. Both patients were on long-term antiepileptic treatment with valproate and only after the withdrawal of valproate, the movement disorder disappeared and level of consciousness improved. These observations emphasize that valproate has to be considered as a possible risk factor in patients in later stages of late-infantile CLN2 disease who develop a rapidly progressive complex movement disorder. Georg Thieme Verlag KG Stuttgart · New York.


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    A. S. Kalinichenko


    Full Text Available The variety of requirements for friction pairs requires the development of different technologies for the production of tribological materials with reference to the operation modes. Composite materials obtained by the casting technology have been successfully applied for the normalization of the thermomechanical state of the steam turbines. These composites consist of the matrix based on copper alloys reinforced with cast iron granules. Because the structure and properties of cast iron are determined by the conditions of their production studies have been conducted on determination of preparation conditions on grain structure and properties of the synthesized composite material. Using an upgraded unit for production of granules technological regimes were determined providing narrow fractional composition. It has been found that granules formed are characterized with typical microstructure of white cast iron containing perlite and ledeburite. Microhardness of pilot cast iron granules is characterized by high values (from 7450 up to 9450 MPa and depends on the size of the fraction. Composite materials obtained using experimental granules had a microhardness of the reinforcing cast iron granules about 3500 MPa, and a bronze matrix – 1220 MPa, which is higher than the hardness of the composite material obtained by using the annealed DCL-1granules (2250 MPa. Metal base of experimental granules in the composite material has the structure of perlitic ductile iron with inclusions of ferrite not exceeding 10–15% and set around a flocculent graphite. As a result, the increase of physical-mechanical properties of finished products made of composite material is observed. 

  18. Muscle-Derived Proteins as Serum Biomarkers for Monitoring Disease Progression in Three Forms of Muscular Dystrophy (United States)

    Burch, Peter M.; Pogoryelova, Oksana; Goldstein, Richard; Bennett, Donald; Guglieri, Michela; Straub, Volker; Bushby, Kate; Lochmüller, Hanns; Morris, Carl


    Abstract Background: Identifying translatable, non-invasive biomarkers of muscular dystrophy that better reflect the disease pathology than those currently available would aid the development of new therapies, the monitoring of disease progression and the response to therapy. Objective: The goal of this study was to evaluate a panel of serum protein biomarkers with the potential to specifically detect skeletal muscle injury. Method: Serum concentrations of skeletal troponin I (sTnI), myosin light chain 3 (Myl3), fatty acid binding protein 3 (FABP3) and muscle-type creatine kinase (CKM) proteins were measured in 74 Duchenne muscular dystrophy (DMD), 38 Becker muscular dystrophy (BMD) and 49 Limb-girdle muscular dystrophy type 2B (LGMD2B) patients and 32 healthy controls. Results: All four proteins were significantly elevated in the serum of these three muscular dystrophy patient populations when compared to healthy controls, but, interestingly, displayed different profiles depending on the type of muscular dystrophy. Additionally, the effects of patient age, ambulatory status, cardiac function and treatment status on the serum concentrations of the proteins were investigated. Statistical analysis revealed correlations between the serum concentrations and certain clinical endpoints including forced vital capacity in DMD patients and the time to walk ten meters in LGMD2B patients. Serum concentrations of these proteins were also elevated in two preclinical models of muscular dystrophy, the mdx mouse and the golden-retriever muscular dystrophy dog. Conclusions: These proteins, therefore, are potential muscular dystrophy biomarkers for monitoring disease progression and therapeutic response in both preclinical and clinical studies. PMID:26870665

  19. Application of narrow-bore HPLC columns in rapid determination of sildenafil citrate in its pharmaceutical dosage forms. (United States)

    Ghodsi, Razieh; Kobarfard, Farzad; Tabatabai, Sayyed Abbas


    A special type of silica-based columns has been recently introduced into the market which is called narrow-bore columns. They have lower internal volume than the standard high-performance liquid chromatography (HPLC) columns and thus reduce the solvent consumption by almost 80%. A simple, accurate and environmentally friendly reversed phase- HPLC (RP-HPLC method) which could be used in fast and high throughput analyses has been developed for the purpose of determining the sildenafil in bulk and pharmaceutical dosage forms, using narrow-bore C18 column (50 × 3.2 mm, 5 µm particle size) in isocratic mode, with mobile phase comprising of buffer (pH = 3) and acetonitrile in the ratio of 75:25 v/v. The flow rate was 0.7 mL/min and the detection was monitored through Ultraviolet detector (UV detector) at 292 nm. Clonazepam was used as the internal standard and the run time was 4 min. The proposed method has permitted the quantification of sildenafil over the linearity in the range of 30-4000 ng/mL and its percentage recovery was found to be 99-105%. Limit of quantitation (LOQ) is determined as 30 ng/mL. The intra-day and inter-day precisions were found 1.2-2.2% and 1.56-3.4% respectively. The solvent consumption was 2.8 mL per sample of which ca 0.7 mL was acetonitrile. This study shows that the application of narrow-bore column instead of the conventional reversed phase column in HPLC analyses has the advantages of shorter run time and less organic solvent consumption. This method is highly sensitive with excellent recoveries and precision and there is no need for special column and pre-column or post-column treatment of the sample. Moreover, the method is free from interference by common additives and excipients, suggesting applications in routine quality control analyses.

  20. A rapid validated UV-HPLC method for the simultaneous determination of the antiretroviral compounds darunavir and raltegravir in their dosage form. (United States)

    Estan-Cerezo, G; García-Monsalve, A; Soriano-Irigaray, L; Rodríguez-Lucena, F J; Navarro-Ruiz, A


    A rapid, simple and sensitive high-performance liquid chromatography (HPLC) method with ultraviolet detection has been developed for quantification of darunavir and raltegravir in their pharmaceutical dosage form. The assay enables the measurement of both drugs with a linear calibration curve (R2= 0.999) over the concentration range 5-100 mg/L. The determination was performed on an analytical Tracer Excel 120 ODSB (15x0.4.6 cm) column at 35ºC. The selected wavelength was 254 nm. The mobile phase was a mixture of 0.037 M sodium dihydrogen phosphate buffer, acetonitrile and methanol (40:50:10, v/v/v) at a flow rate of 2.0 mL/min Nevirapine (50 mg/L) was used as internal standard. Accuracy, intra-day repeatability (n = 5), and inter-day precision (n = 3) were found to be satisfactory, being the accuracy from -4.33 to 3.88% and precisions were intra-day and inter-day, 0.25% and 4.42% respectively in case of darunavir. Raltegravir intra-day and inter-day precisions lower of 1.01 and 2.36%, respectively and accuracy values bet from -4.02 to 1.06%. Determination of the darunavir and raltegravir in their dosage form was done with a maximum deviation of 4%. This analytical method is rapid, easily implantable and offers good results.

  1. Rapid, progressive neuropathic arthropathy of the hip in a patient co-infected with human immunodeficiency virus, hepatitis C virus and tertiary syphilis: case report (United States)


    Background Syphilis is a chronic infection that is classified into three stages. In its tertiary stage, syphilis spreads to the brain, heart and other organs; the lesions may involve the skin, mucous membranes and bones. Neuropathic arthropathy associated with tertiary syphilis has rarely been described in Europe and its association with HIV-HCV co-infection has not been reported so far. This article reports the case of a man with tertiary syphilis presenting with rapidly evolving neuropathic arthropathy of the hip and extensive bone destruction. Case presentation On initial presentation, the patient complained of progressively worsening left-sided coxalgia without localized or generalized inflammation. The patient reported to have no history of previous infections, trauma or cancer. Plain x-ray films of the left coxofemoral joint showed marked degeneration with necrosis of the proximal epiphysis of femur and morphological alterations of the acetabulum without protrusion. Primary coxarthrosis was diagnosed and hip arthroplasty was offered, but the patient declined treatment. Three months later, the patient presented a marked deterioration of his general condition. He disclosed that he was seropositive for HCV and HIV, as confirmed by serology. Syphilis serology testing was also positive. A Girdlestone's procedure was performed and samples were collected for routine cultures for bacteria and acid fast bacilli, all resulting negative. Although histological findings were inconclusive, confirmed positive serology for syphilis associated with progressive arthropathy was strongly suggestive of tertiary syphilis, probably exacerbated by HIV-HCV co-infection. The patient partially recovered the ability to walk. Conclusions Due to the resurgence of syphilis, this disease should be considered as a possible cause of neuropathic arthropathy when other infectious causes have been ruled out, particularly in patients with HIV and/or HCV co-infection. PMID:21645338

  2. Rapid, progressive neuropathic arthropathy of the hip in a patient co-infected with human immunodeficiency virus, hepatitis C virus and tertiary syphilis: case report

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    Pasqualini Marco


    Full Text Available Abstract Background Syphilis is a chronic infection that is classified into three stages. In its tertiary stage, syphilis spreads to the brain, heart and other organs; the lesions may involve the skin, mucous membranes and bones. Neuropathic arthropathy associated with tertiary syphilis has rarely been described in Europe and its association with HIV-HCV co-infection has not been reported so far. This article reports the case of a man with tertiary syphilis presenting with rapidly evolving neuropathic arthropathy of the hip and extensive bone destruction. Case presentation On initial presentation, the patient complained of progressively worsening left-sided coxalgia without localized or generalized inflammation. The patient reported to have no history of previous infections, trauma or cancer. Plain x-ray films of the left coxofemoral joint showed marked degeneration with necrosis of the proximal epiphysis of femur and morphological alterations of the acetabulum without protrusion. Primary coxarthrosis was diagnosed and hip arthroplasty was offered, but the patient declined treatment. Three months later, the patient presented a marked deterioration of his general condition. He disclosed that he was seropositive for HCV and HIV, as confirmed by serology. Syphilis serology testing was also positive. A Girdlestone's procedure was performed and samples were collected for routine cultures for bacteria and acid fast bacilli, all resulting negative. Although histological findings were inconclusive, confirmed positive serology for syphilis associated with progressive arthropathy was strongly suggestive of tertiary syphilis, probably exacerbated by HIV-HCV co-infection. The patient partially recovered the ability to walk. Conclusions Due to the resurgence of syphilis, this disease should be considered as a possible cause of neuropathic arthropathy when other infectious causes have been ruled out, particularly in patients with HIV and/or HCV co-infection.

  3. Fundus Autofluorescence and SD-OCT Document Rapid Progression in Autosomal Dominant Vitreoretinochoroidopathy (ADVIRC) Associated with a c.256G > A Mutation in BEST1. (United States)

    Kellner, Simone; Stöhr, Heidi; Fiebig, Britta; Weinitz, Silke; Farmand, Ghazaleh; Kellner, Ulrich; Weber, Bernhard H F


    To report the variability of clinical findings, rapid concentric progression, and successful treatment of macular edema in autosomal dominant vitreoretinochoroidopathy (ADVIRC) associated with a heterozygous c.256G > A missense mutation in the bestrophin-1 (BEST1) gene. Three affected members of a four-generation ADVIRC family were examined with fundus autofluorescence (FAF), near-infrared autofluorescence (NIA) and spectral domain optical coherence tomography (SD-OCT). Direct sequence analysis of coding and flanking intronic regions of the BEST1 gene was performed. Disease manifestations presented with high variability with visual problems manifesting between 10 and 40 years of age. Two probands showed marked signs of peripheral degeneration, while this retinal area was not noticeably affected in the third. Cystoid macular edema was present in one proband, which responded to long-term treatment with topic dorzolamide with improved visual acuity. FAF and NIA revealed mid-peripheral retinal degeneration in areas that appeared normal on ophthalmoscopy. The full-field ERG was markedly reduced in two probands. Within a 5-year period a marked increase in concentric progression of degeneration including the posterior pole was documented with FAF, NIA and SD-OCT in one proband after the age of 63 years. Direct sequence analysis of the BEST1 gene revealed a heterozygous c.256G > A missense mutation in the three affected probands. The findings in this family emphasize the previously noted variability of clinical manifestations in BEST1-associated ADVIRC and the relevance of FAF and NIA imaging. Cystoid macular edema and vascular leakage can be successfully treated using dorzolamide.

  4. Tc-99m-bicisate (ECD)-brain-SPECT in rapidly progressive dementia; Hirn-SPECT mit Tc-99m-Bicisat (ECD) bei rasch progredientem dementiellen Syndrom

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    Marienhagen, J.; Eilles, C. [Regensburg Univ. (Germany). Abt. fuer Nuklearmedizin; Weingaertner, U.; Blaha, L. [Bezirkskrankenhaus Mainkofen (Germany). Psychiatrische Klinik; Zerr, I.; Poser, S. [Goettingen Univ. (Germany). Klinik und Poliklinik fuer Neurologie


    We present a 61-year-old male patient with progressive dementia. A brain SPECT with Tc-99m-bicisate was performed for confirmation of clinically suspected Alzheimer-dementia. At the time of the SPECT-investigation marked apraxia and aphasia besides severe dementia were present. Electrophysiological as well as anatomical neuroimaging findings showed non-diagnostic alterations. SPECT revealed distinct perfusion defects, which made Alzheimer Dementia unlikely. The further course of the patient was determined by rapidly progressive deterioration with development of akinetic mutism. Thereafter, increased levels of neuron-specific enolase as well as 14-3-3 proteins were found in the cerebro-spinal fluid (CSF). The patient finally died with signs of cerebral decortication. Due to the clinical course and the CSF-findings the patient's final diagnosis was Creutzfeld-Jakob-disease, nevertheless no autopsy was performed. The presented case report underscores the clinical utility of perfusion brain SPECT in the differential diagnosis of dementias. (orig.) [German] Wir berichten ueber einen 61jaehrigen Patienten mit progredientem dementiellen Syndrom, der unter der Verdachtsdiagnose einer Demenz vom Alzheimer-Typ (DAT) zur Hirn-SPECT-Untersuchung mit TC-99m-Bicisat (ECD) vorgestellt wurde. Zum Untersuchungszeitpunkt bestanden neben dem Vollbild einer Demenz eine ausgepraegte Apraxie und Aphasie bei unspezifischen Veraenderungen im EEG sowie der neuroradiologischen Bildgebung. In der Hirn-SPECT-Untersuchung fanden sich fuer eine DAT untypische ausgedehnte, vorwiegend rechtshemisphaerische Perfusionsstoerungen. Im weiteren Verlauf rasche Progredienz des Krankheitsbildes mit Entwicklung eines akinetischen Mutismus sowie Nachweis erhoehter Werte der neuronspezifischen Enolase und des 14-3-3-Proteins im Liquor. Der Patient verstarb schliesslich unter dem Bild einer Decortication. Aufgrund des klinischen Verlaufs sowie der Liquorbefunde wurde, da eine autoptische Befundsicherung

  5. Frequent overlap of active hepatitis in recurrent primary sclerosing cholangitis after living-donor liver transplantation relates to its rapidly progressive course. (United States)

    Miyagawa-Hayashino, Aya; Egawa, Hiroto; Yoshizawa, Atsusi; Ueda, Yoshihide; Ichida, Takafumi; Ueno, Yoshiyuki; Uemoto, Shinji; Harada, Kenichi; Nakanuma, Yasuni


    Recurrence of primary sclerosing cholangitis after liver transplantation is a challenging issue. Liver pathologies of recurrent primary sclerosing cholangitis after living-donor liver transplantation have not been reported. Here, liver pathologies of explanted grafts and biopsies of 9 patients who underwent retransplantation for recurrent primary sclerosing cholangitis were compared with those of native livers. Recurrence was diagnosed in 13 of 36 patients for primary sclerosing cholangitis post-living-donor liver transplantation, and 9 of them underwent retransplantation. All explanted grafts revealed biliary cirrhosis with sclerosing cholangitis, and 6 patients had additional features of active hepatitis. Liver biopsies showed that 3 had active hepatitis in addition to fibrous cholangitis at recurrence of primary sclerosing cholangitis. Two developed active hepatitis later after the diagnosis of recurrence. In explanted grafts, in addition to extensive hilar lymphoplasmacytic cholangitis, 4 cases showed hilar xanthogranulomatous cholangitis. The latter was not evident in 7 native livers. Ductopenia was extensive in all native livers, although such changes were relatively mild in explanted grafts at retransplantation. Patients with recurrent primary sclerosing cholangitis developed progressive graft failure, and the interval between diagnosis of recurrence and retransplantation (mean, 3.2 years) was shorter than that between diagnosis of primary sclerosing cholangitis and first transplantation (mean, 7.7 years). The rather rapid deterioration of recurrent primary sclerosing cholangitis after liver transplantation may be related to the frequent overlap of active hepatitis. Copyright © 2011 Elsevier Inc. All rights reserved.

  6. Decreased phasic EMG activity during rapid eye movement sleep in treatment-naïve Parkinson's disease: effects of treatment with levodopa and progression of illness. (United States)

    Garcia-Borreguero, Diego; Caminero, Ana B; De La Llave, Yolanda; Larrosa, Oscar; Barrio, Soledad; Granizo, Juan J; Pareja, Juan A


    Rapid eye movement (REM) sleep behavior disorder (RBD) is frequently associated with Parkinson's disease (PD) and may anticipate its diagnosis by several years. We assessed the presence of motor dyscontrol during REM sleep in treatment-naïve PD patients and investigated the putative effect of levodopa (L-dopa) treatment on motor activity. Overnight sleep studies were performed on 15 previously untreated PD patients and 14 controls at baseline, again after a 3- to 9-month treatment period with a low dose of L-dopa, and 2 to 5 days after treatment discontinuation (in 8 patients). No differences in sleep parameters were observed across groups or treatment conditions. None of the patients met criteria for RBD at baseline, whereas 5 patients were symptomatic at the time of the second sleep study. A quantitative analysis of electromyographic (EMG) activity during REM sleep showed a lower phasic twitching activity in untreated PD than in controls. However, an increase in both phasic twitching and tonic activity was found after treatment with L-dopa. Discontinuation of treatment resulted in a return to pretreatment values of phasic but not of tonic EMG activity. Thus, the increase in phasic activity seems to depend on the effects of L-dopa, whereas the increase in tonic EMG activity during REM sleep might be caused by other factors such as the progression of disease. Potential implications for the understanding of the relationship between RBD and PD are discussed. Copyright 2002 Movement Disorder Society

  7. Case of streptococcal toxic shock syndrome caused by rapidly progressive group A hemolytic streptococcal infection during postoperative chemotherapy for cervical cancer. (United States)

    Nogami, Yuya; Tsuji, Kousuke; Banno, Kouji; Umene, Kiyoko; Katakura, Satomi; Kisu, Iori; Tominaga, Eiichiro; Aoki, Daisuke


    Streptococcal toxic shock syndrome (STSS) is a severe infectious disease caused by group A hemolytic streptococcus (Streptococcus pyogenes). This condition is a serious disease that involves rapidly progressive septic shock. We experienced a case of STSS caused by primary peritonitis during treatment with paclitaxel and cisplatin (TP therapy) as postoperative chemotherapy for cervical cancer. STSS mostly develops after extremity pain, but initial influenza-like symptoms of fever, chill, myalgia and gastrointestinal symptoms may also occur. TP therapy is used to treat many cancers, including gynecological cancer, but may cause adverse reactions of neuropathy and nephrotoxicity and sometimes fever, arthralgia, myalgia, abdominal pain and general malaise. The case reported here indicates that development of STSS can be delayed after chemotherapy and that primary STSS symptoms may be overlooked because they may be viewed as adverse reactions to chemotherapy. To our knowledge, this is the first report of a case of STSS during chemotherapy. © 2013 The Authors. Journal of Obstetrics and Gynaecology Research © 2013 Japan Society of Obstetrics and Gynecology.

  8. Rapid tumor progression in a patient with HPV type 16 associated anal squamous cell carcinoma suffering from long-standing Crohn's disease: A case report

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    Fischer AK


    Full Text Available Background and aim: Squamous cell carcinoma (SCC is the most common cancer of the anal region, typically associated with high-risk (hr HPV infection. Furthermore, there is evidence that Crohn's disease predisposes to adenocarcinoma in patients with perianal disease. Materials and methods: A 57-year old patient presenting with long history of Crohn's disease since the age of mid-twenties, went through several surgeries including ileocolectomy and anal fistula resection, combined with immunosuppressive therapy additionally periodically since 2008. One year before death (in 2015 a painful fistula was diagnosed with extensive high grade anal intraepithelial neoplasia (AIN-HG and evidence of invasive growth as non-keratinizing SCC. Tissue samples from several previous and current resection specimens were re-evaluated and extensively investigated for Crohn´s type inflammation, dysplasia and HPV both by immunohistochemistry (p16/Ki67 and molecular subtyping of HPV. Results: AIN-HG and invasive anal squamous cell carcinoma turned out to be strongly positive for p16/Ki67 staining and molecular analysis disclosed a HPV-16 subtype. In contrast, HPV-analysis was negative in all available previous tissue samples including one anal fistula resected five years before (in 2009 which was lined by non-keratinized squamous epithelium without any evidence of dysplasia. Thus, the patient was diagnosed as Crohn's disease with hr-HPV infection that rapidly (< 5ys progressed to AIN-HG and anal SCC. Finally, osseous metastases occurred and the patient died shortly after. Conclusions: This case of a patient diagnosed with SCC of the anal canal in combination with Crohn's disease as well as HPV Type 16 infection, points to the pathomechanism leading to dysplasia and finally cancer. We assume that immunosuppressive therapy in Crohn's disease may predispose to both persistent HPV infection and HPV related invasive anal carcinoma. The accelerated progression of HPV

  9. Development and Validation of a Stability-Indicating RP-HPLC Method for Rapid Determination of Doxycycline in Pharmaceutical Bulk and Dosage Forms

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    Shabnam Pourmoslemi, Soroush Mirfakhraee, Saeid Yaripour, Ali Mohammadi


    Full Text Available Background: A rapid stability-indicating RP-HPLC method for analysis of doxycycline in the presence of its degradation products was developed and validated. Methods: Forced degradation studies were carried out on bulk samples and capsule dosage forms of doxycycline using acid, base, H2O2, heat, and UV light as described by ICH for stress conditions to demonstrate the stability-indicating power of the method. Separations were performed on a Perfectsil® Target ODS column (3-5µm, 125 mm×4 mm, using a mobile phase consisting of methanol-50 mM ammonium acetate buffer (containing 0.1% v/v trifluoroacetic acid and 0.1% v/v triethylamine, pH 2.5 (50:50 v/v at room temperature. The flow rate was 0.8 mL/min. Results: The method linearity was investigated in the range of 25–500 µg/mL (r > 0.9999. The LOD and LOQ were 5 and 25 µg/mL, respectively. The method selectivity was evaluated by peak purity test using a diode array detector. There was no interference among detection of doxycycline and its stressed degradation products. Total peak purity numbers were in the range of 0.94-0.99, indicating the homogeneity of DOX peaks. Conclusion: These data show the stability-indicating nature of the method for quality control of doxycycline in bulk samples and capsule dosage forms.

  10. Grade 3 ischemia on the admission electrocardiogram predicts rapid progression of necrosis over time and less myocardial salvage by primary angioplasty. (United States)

    Billgren, Therese; Maynard, Charles; Christian, Timothy F; Rahman, Mohmmad A; Saeed, Mahammad; Hammill, Stephen C; Wagner, Galen S; Birnbaum, Yochai


    Among patients with ST-elevation acute myocardial infarction, those with terminal QRS distortion (grade 3 ischemia) have higher mortality and larger infarct size (IS) than patients without QRS distortion (grade 2 ischemia). We assessed the relation of baseline electrocardiographic ischemia grades to area at risk (AR) and myocardial salvage [100 (AR-IS)/AR] in 79 patients who underwent primary angioplasty for first ST-elevation acute myocardial infarction and had technetium Tc 99m sestamibi single-photon emission computed tomography before angioplasty (AR) and at predischarge (IS). Patients were classified as having grade 2 ischemia (ST elevation without terminal QRS distortion in any of the leads, n = 48), grade 2.5 ischemia (ST elevation with terminal QRS distortion in 1 lead, n = 16), or grade 3 ischemia (ST elevation with terminal QRS distortion in >2 adjacent leads, n = 15). Time to treatment was comparable among groups. AR was comparable among groups (38% +/- 20%, 33% +/- 23%, and 34% +/- 23%, respectively; P = .70). There were no differences among groups in residual myocardial perfusion (severity index 0.28 +/- 0.12, 0.29 +/- 0.16, and 0.30 +/- 0.15 in grades 2, 2.5, and 3 ischemia, respectively; P = .97). In contrast, there was a trend toward lower myocardial salvage (45% +/- 32%) in the grade 3 group than in the grade 2 (65% +/- 33%) and grade 2.5 (65% +/- 40%) groups ( P = .16). Salvage was dependent on time only in the grade 3 group. Spearman rank correlation coefficients between time to treatment and percentage salvage were 0.003 ( P = .99), -0.24 ( P = .38), and -0.63 ( P = .022) for grades 2, 2.5, and 3, respectively. Patients with grade 3 ischemia have rapid progression of necrosis over time and less myocardial salvage. This admission pattern is a predictor of myocardial salvage by primary angioplasty.

  11. Defective Proteolytic Processing of Fibrillar Procollagens and Prodecorin Due to Biallelic BMP1 Mutations Results in a Severe, Progressive Form of Osteogenesis Imperfecta. (United States)

    Syx, Delfien; Guillemyn, Brecht; Symoens, Sofie; Sousa, Ana Berta; Medeira, Ana; Whiteford, Margo; Hermanns-Lê, Trinh; Coucke, Paul J; De Paepe, Anne; Malfait, Fransiska


    Whereas the vast majority of osteogenesis imperfecta (OI) is caused by autosomal dominant defects in the genes encoding type I procollagen, mutations in a myriad of genes affecting type I procollagen biosynthesis or bone formation and homeostasis have now been associated with rare autosomal recessive OI forms. Recently, homozygous or compound heterozygous mutations in BMP1, encoding the metalloproteases bone morphogenetic protein-1 (BMP1) and its longer isoform mammalian Tolloid (mTLD), were identified in 5 children with a severe autosomal recessive form of OI and in 4 individuals with mild to moderate bone fragility. BMP1/mTLD functions as the procollagen carboxy-(C)-proteinase for types I to III procollagen but was also suggested to participate in amino-(N)-propeptide cleavage of types V and XI procollagens and in proteolytic trimming of other extracellular matrix (ECM) substrates. We report the phenotypic characteristics and natural history of 4 adults with severe, progressive OI characterized by numerous fractures, short stature with rhizomelic shortening, and deformity of the limbs and variable kyphoscoliosis, in whom we identified novel biallelic missense and frameshift mutations in BMP1. We show that BMP1/mTLD-deficiency in humans not only results in delayed cleavage of the type I procollagen C-propeptide but also hampers the processing of the small leucine-rich proteoglycan prodecorin, a regulator of collagen fibrillogenesis. Immunofluorescent staining of types I and V collagen and transmission electron microscopy of the dermis show impaired assembly of heterotypic type I/V collagen fibrils in the ECM. Our study thus highlights the severe and progressive nature of BMP1-associated OI in adults and broadens insights into the functional consequences of BMP1/mTLD-deficiency on ECM organization. © 2015 American Society for Bone and Mineral Research.

  12. Rapid detection of human and canine visceral leishmaniasis: assessment of a latex agglutination test based on the A2 antigen from amastigote forms of Leishmania infantum. (United States)

    Akhoundi, Behnaz; Mohebali, Mehdi; Shojaee, Saeedeh; Jalali, Mahmoud; Kazemi, Bahram; Bandehpour, Mojgan; Keshavarz, Hossein; Edrissian, Gholam Hossein; Eslami, Mohammad Bagher; Malekafzali, Hossein; Kouchaki, Ameneh


    The diagnosis of visceral leishmaniasis (VL) in humans and animal reservoir hosts is difficult, particularly in rural areas where the disease is endemic and laboratory facilities are limited. This study aimed to develop a latex agglutination test (LAT) for the rapid detection of anti-Leishmania antibodies against the A2 antigen derived from the amastigote form as well as those against crude antigens derived from the promastigote form of an Iranian strain of Leishmania (Leishmania) infantum. The A2 antigen (42-100 kDa) was prepared from the amastigote form of L. infantum, purified with electroelution and compared with the crude antigen from the promastigote form of L. infantum. Both antigens showed appropriate intensity reactions, were selected using dot blotting of positive and negative pooled sera and used to sensitize 0.9-μm latex beads. The tests were carried out on sera from 43 symptomatic, human patients with VL confirmed by parasitological examination and direct agglutination test (DAT), 30 healthy controls and 32 patients with other infections but without VL. Canine sera were collected from 63 domestic dogs with VL confirmed using parasitological examinations and DAT and 31 healthy dogs from areas non-endemic for VL. Compared with the controls, human sera from DAT-confirmed patients yielded a sensitivity of 88.4% (95% CI, 82.1-94.5%) and specificity of 93.5% (95% CI, 87.0-99.7%) on A2-LAT (amastigote) when 1:3200 was used as the cut-off titre. A good degree of agreement was found between A2-LAT and DAT (0.914). LAT required 3-5 min to complete, versus the 12-18 h needed for DAT. Compared with the controls, A2-LAT of canine sera from DAT-confirmed cases yielded a sensitivity of 95.2% (95% CI, 95.0-95.4%) and specificity of 100% (95% CI 100%) when 1:320 was used as the cut-off titre. A good degree of agreement was found between A2-LAT and DAT (0.968). Similarly, the sensitivity and specificity of Pro.-LAT (promastigote) was calculated to be 88.4% and 91

  13. Qualitative and quantitative analysis of diffusion-weighted imaging of gestational trophoblastic disease: Can it predict progression of molar pregnancy to persistent form of disease? (United States)

    Sefidbakht, Sepideh; Hosseini, Fatemeh; Bijan, Bijan; Hamedi, Bahareh; Azizi, Tayyebeh


    To describe the diffusion-weighted imaging (DWI) appearance of gestational trophoblastic disease (GTD) and to determine its apparent diffusion coefficient (ADC) values. To evaluate the feasibility of DWI to predict progression of hydatidiform mole (HM) to persistent disease. During a period of 6 months, women with preliminary diagnosis of GTD, based on ultrasound and ßhCG levels, underwent 1.5T MRI (T2 high-resolution and DWI; b values 50, 400, 800; sagittal and perpendicular to the endometrium; and T1, T2 Turbo Spin Echo [TSE] axial images). Patients were followed for 6-12 months to monitor progression to persistent form of the disease. ADC values and image characteristics were compared between HM and persistent neoplasia and between GTD and non-molar pregnancy using Mann-Whitney U and Fisher's exact tests, respectively. Among the 23 studied patients, 19 (83%) were classified as molar and 4 (17%) as non-molar, based on pathology reports. After 6-12 months of follow-up, 5 (26%) cases progressed to persistent disease and 14 (74%) cases were benign HM. There was no significant difference between ADC values for HM (1.93±0.33×10-3mm2/s) and persistent neoplasia (2.03±0.28×10-3mm2/s) (P=0.69). The ADC of non-molar pregnancies was (0.96±0.46×10-3mm2/s), which was significantly different from GTD (1.96 ±0.32×10-3mm2/s) (P=0.001). Heterogeneous snowstorm appearance, focal intratumoral hemorrhage, myometrial contraction, and prominent myometrial vascularity were more common in GTD compared to non-molar pregnancy (Psnowstorm appearance, focal intratumoral hemorrhage, myometrial contraction, and prominent myometrial vascularity are among the imaging characteristics of GTD. We cannot use ADC values to predict progression to persistent disease. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  14. A Case of Adrenoleukodystrophy Presenting as Progressive Cerebellar Dysfunction

    Directory of Open Access Journals (Sweden)

    Seunguk Jung


    Full Text Available X-linked adrenoleukodystrophy (X-ALD is a hereditary neurological disorder affecting the nervous system and adrenal cortex. The phenotype of X-ALD ranges from the rapidly progressive cerebral form to milder adrenomyeloneuropathy. However, cerebellar manifestations are rare. We report a case of adrenoleukodystrophy presenting as progressive cerebellar dysfunction resembling olivopontocerebellar degeneration, with a review of the literature

  15. Progressive Multifocal Leuoencephalopathy(PML) in HIV Patient ...

    African Journals Online (AJOL)

    ... parasitic, and neoplastic causes. Human immunodeficiency virus leucoencephalopathy (HIVL) is an uncommon and rapidly progressive form of AIDS dementia complex (ADC) that has remained poorly understood. Here we report a rare case of Progressive Multifocal Leuoencephalopathy (PML) in a case of HIV/AIDS who ...

  16. Progress in the establishment and strengthening of national immunization technical advisory groups: analysis from the 2013 WHO/UNICEF joint reporting form, data for 2012. (United States)

    Duclos, Philippe; Dumolard, Laure; Abeysinghe, Nihal; Adjagba, Alex; Janusz, Cara Bess; Mihigo, Richard; Mosina, Liudmila; Takashima, Yashohiro; Oztürk, Murat Hakan


    The majority of industrialized and some developing countries have established National Immunization Technical Advisory Groups (NITAGs). To enable systematic global monitoring of the existence and functionality of NITAGs, in 2011, WHO and UNICEF included related questions in the WHO/UNICEF Joint Reporting Form (JRF) that provides an official means to globally collect indicators of immunization program performance. These questions relate to six basic process indicators. According to the analysis of the 2013 JRF, data for 2012, notable progress was achieved between 2010 and 2012 and by the end of 2012, 99 countries (52%) reported the existence of a NITAG with a formal legislative or administrative basis (with a high of 86% in the Eastern Mediterranean Region - EMR), among the countries that reported data in the NITAG section of the JRF. There were 63 (33%) countries with a NITAG that met six process indicators (47% increase over the 43 reported in 2010) including a total of 38 developing countries. 11% of low income countries reported a NITAG that meets all six process criteria, versus 29% of middle income countries and 57% of the high income ones. Countries with smaller populations reported the existence of a NITAG that meets all six process criteria less frequently than more populated countries (23% for less populated countries versus 43% for more populated ones). However, progress needs to be accelerated to reach the Global Vaccine Action Plan (GVAP) target of ensuring all countries have support from a NITAG. The GVAP represents a major opportunity to boost the institutionalization of NITAGs. A special approach needs to be explored to allow small countries to benefit from sub-regional or other countries advisory groups. Copyright © 2013 The Authors. Published by Elsevier Ltd.. All rights reserved.

  17. A Coding Variant in the Gene Bardet-Biedl Syndrome 4 (BBS4 Is Associated with a Novel Form of Canine Progressive Retinal Atrophy

    Directory of Open Access Journals (Sweden)

    Tracy Chew


    Full Text Available Progressive retinal atrophy is a common cause of blindness in the dog and affects >100 breeds. It is characterized by gradual vision loss that occurs due to the degeneration of photoreceptor cells in the retina. Similar to the human counterpart retinitis pigmentosa, the canine disorder is clinically and genetically heterogeneous and the underlying cause remains unknown for many cases. We use a positional candidate gene approach to identify putative variants in the Hungarian Puli breed using genotyping data of 14 family-based samples (CanineHD BeadChip array, Illumina and whole-genome sequencing data of two proband and two parental samples (Illumina HiSeq 2000. A single nonsense SNP in exon 2 of BBS4 (c.58A > T, p.Lys20* was identified following filtering of high quality variants. This allele is highly associated (PCHISQ = 3.425e−14, n = 103 and segregates perfectly with progressive retinal atrophy in the Hungarian Puli. In humans, BBS4 is known to cause Bardet–Biedl syndrome which includes a retinitis pigmentosa phenotype. From the observed coding change we expect that no functional BBS4 can be produced in the affected dogs. We identified canine phenotypes comparable with Bbs4-null mice including obesity and spermatozoa flagella defects. Knockout mice fail to form spermatozoa flagella. In the affected Hungarian Puli spermatozoa flagella are present, however a large proportion of sperm are morphologically abnormal and <5% are motile. This suggests that BBS4 contributes to flagella motility but not formation in the dog. Our results suggest a promising opportunity for studying Bardet–Biedl syndrome in a large animal model.

  18. Seeking sunlight: rapid phototactic motility of filamentous mat-forming cyanobacteria optimize photosynthesis and enhance carbon burial in Lake Huron’s submerged sinkholes

    Directory of Open Access Journals (Sweden)

    Bopaiah A Biddanda


    Full Text Available We studied the motility of filamentous mat-forming cyanobacteria consisting primarily of Oscillatoria-like cells growing under low-light, low-oxygen and high-sulfur conditions in Lake Huron’s submerged sinkholes using in situ observations, in vitro measurements and time-lapse microscopy. Gliding movement of the cyanobacterial trichomes (100-10,000 µm long filaments, composed of cells ~10 µm wide and ~3 µm tall revealed individual as well as group-coordinated motility. When placed in a petri dish and dispersed in ground water from the sinkhole, filaments re-aggregated into defined colonies within minutes, then dispersed again. Speed of individual filaments increased with temperature from ~50 µm minute-1 or ~15 body lengths minute-1 at 10°C to ~215 µm minute-1 or ~70 body lengths minute-1 at 35°C – rates that are rapid relative to non-flagellated/ciliated microbes. Filaments exhibited precise and coordinated positive phototaxis towards pinpoints of light and congregated under the light of foil cutouts. Such light-responsive clusters showed an increase in photosynthetic yield – suggesting phototactic motility aids in light acquisition as well as photosynthesis. Once light source was removed, filaments slowly spread out evenly and re-aggregated, demonstrating coordinated movement through inter-filament communication regardless of light. Pebbles and pieces of broken shells placed upon intact mat were quickly covered by vertically motile filaments within hours and became fully buried in the anoxic sediments over 3-4 diurnal cycles – likely facilitating the preservation of falling debris. Coordinated horizontal and vertical filament motility optimize mat cohesion and dynamics, photosynthetic efficiency and sedimentary carbon burial in modern-day sinkhole habitats that resemble the shallow seas in Earth’s early history. Analogous cyanobacterial motility may have played a key role in the oxygenation of the planet by optimizing

  19. Seeking sunlight: rapid phototactic motility of filamentous mat-forming cyanobacteria optimize photosynthesis and enhance carbon burial in Lake Huron's submerged sinkholes. (United States)

    Biddanda, Bopaiah A; McMillan, Adam C; Long, Stephen A; Snider, Michael J; Weinke, Anthony D


    We studied the motility of filamentous mat-forming cyanobacteria consisting primarily of Oscillatoria-like cells growing under low-light, low-oxygen, and high-sulfur conditions in Lake Huron's submerged sinkholes using in situ observations, in vitro measurements and time-lapse microscopy. Gliding movement of the cyanobacterial trichomes (100-10,000 μm long filaments, composed of cells ∼10 μm wide and ∼3 μm tall) revealed individual as well as group-coordinated motility. When placed in a petri dish and dispersed in ground water from the sinkhole, filaments re-aggregated into defined colonies within minutes, then dispersed again. Speed of individual filaments increased with temperature from ∼50 μm min(-1) or ∼15 body lengths min(-1) at 10°C to ∼215 μm min(-1) or ∼70 body lengths min(-1) at 35°C - rates that are rapid relative to non-flagellated/ciliated microbes. Filaments exhibited precise and coordinated positive phototaxis toward pinpoints of light and congregated under the light of foil cutouts. Such light-responsive clusters showed an increase in photosynthetic yield - suggesting phototactic motility aids in light acquisition as well as photosynthesis. Once light source was removed, filaments slowly spread out evenly and re-aggregated, demonstrating coordinated movement through inter-filament communication regardless of light. Pebbles and pieces of broken shells placed upon intact mat were quickly covered by vertically motile filaments within hours and became fully buried in the anoxic sediments over 3-4 diurnal cycles - likely facilitating the preservation of falling debris. Coordinated horizontal and vertical filament motility optimize mat cohesion and dynamics, photosynthetic efficiency and sedimentary carbon burial in modern-day sinkhole habitats that resemble the shallow seas in Earth's early history. Analogous cyanobacterial motility may have played a key role in the oxygenation of the planet by optimizing photosynthesis while favoring

  20. Interagency partnering for weed prevention--progress on development of a National Early Detection and Rapid Response System for Invasive Plants in the United States (United States)

    Westbrooks, R.; Westbrooks, R.


    Over the past 50 years, experience has shown that interagency groups provide an effective forum for addressing various invasive species issues and challenges on multiple land units. However, more importantly, they can also provide a coordinated framework for early detection, reporting, identification and vouchering, rapid assessment, and rapid response to new and emerging invasive plants in the United States. Interagency collaboration maximizes the use of available expertise, resources, and authority for promoting early detection and rapid response (EDRR) as the preferred management option for addressing new and emerging invasive plants. Currently, an interagency effort is underway to develop a National EDRR System for Invasive Plants in the United States. The proposed system will include structural and informational elements. Structural elements of the system include a network of interagency partner groups to facilitate early detection and rapid response to new invasive plants, including the Federal Interagency Committee for the Management of Noxious and Exotic Weeds (FICMNEW), State Invasive Species Councils, State Early Detection and Rapid Response Coordinating Committees, State Volunteer Detection and Reporting Networks, Invasive Plant Task Forces, and Cooperative Weed Management Areas. Informational elements and products being developed include Regional Invasive Plant Atlases, and EDRR Guidelines for EDRR Volunteer Network Training, Rapid Assessment and Rapid Response, and Criteria for Selection of EDRR Species. System science and technical support elements which are provided by cooperating state and federal scientists, include EDRR guidelines, training curriculum for EDRR volunteers and agency field personnel, plant identification and vouchering, rapid assessments, as well as predictive modeling and ecological range studies for invasive plant species.

  1. Rapid, simple and stability-indicating determination of polyhexamethylene biguanide in liquid and gel-like dosage forms by liquid chromatography with diode-array detection

    National Research Council Canada - National Science Library

    Küsters, Markus; Beyer, Sören; Kutscher, Stephan; Schlesinger, Harald; Gerhartz, Michael


    A rapid and simple method for the determination of potyhexamethylene biguanide (polyhexanide, PHMB) in liquid and gel-like pharmaceutical formulations by means of high performance liquid chromatography coupled to diode-array detection...

  2. Seeking sunlight: rapid phototactic motility of filamentous mat-forming cyanobacteria optimize photosynthesis and enhance carbon burial in Lake Huron’s submerged sinkholes (United States)

    Biddanda, Bopaiah A.; McMillan, Adam C.; Long, Stephen A.; Snider, Michael J.; Weinke, Anthony D.


    We studied the motility of filamentous mat-forming cyanobacteria consisting primarily of Oscillatoria-like cells growing under low-light, low-oxygen, and high-sulfur conditions in Lake Huron’s submerged sinkholes using in situ observations, in vitro measurements and time-lapse microscopy. Gliding movement of the cyanobacterial trichomes (100–10,000 μm long filaments, composed of cells ∼10 μm wide and ∼3 μm tall) revealed individual as well as group-coordinated motility. When placed in a petri dish and dispersed in ground water from the sinkhole, filaments re-aggregated into defined colonies within minutes, then dispersed again. Speed of individual filaments increased with temperature from ∼50 μm min-1 or ∼15 body lengths min-1 at 10°C to ∼215 μm min-1 or ∼70 body lengths min-1 at 35°C – rates that are rapid relative to non-flagellated/ciliated microbes. Filaments exhibited precise and coordinated positive phototaxis toward pinpoints of light and congregated under the light of foil cutouts. Such light-responsive clusters showed an increase in photosynthetic yield – suggesting phototactic motility aids in light acquisition as well as photosynthesis. Once light source was removed, filaments slowly spread out evenly and re-aggregated, demonstrating coordinated movement through inter-filament communication regardless of light. Pebbles and pieces of broken shells placed upon intact mat were quickly covered by vertically motile filaments within hours and became fully buried in the anoxic sediments over 3–4 diurnal cycles – likely facilitating the preservation of falling debris. Coordinated horizontal and vertical filament motility optimize mat cohesion and dynamics, photosynthetic efficiency and sedimentary carbon burial in modern-day sinkhole habitats that resemble the shallow seas in Earth’s early history. Analogous cyanobacterial motility may have played a key role in the oxygenation of the planet by optimizing photosynthesis while

  3. Progression of mouse skin carcinogenesis is associated with increased ERα levels and is repressed by a dominant negative form of ERα.

    Directory of Open Access Journals (Sweden)

    Stella Logotheti

    Full Text Available Estrogen receptors (ER, namely ERα and ERβ, are hormone-activated transcription factors with an important role in carcinogenesis. In the present study, we aimed at elucidating the implication of ERα in skin cancer, using chemically-induced mouse skin tumours, as well as cell lines representing distinct stages of mouse skin oncogenesis. First, using immunohistochemical staining we showed that ERα is markedly increased in aggressive mouse skin tumours in vivo as compared to the papilloma tumours, whereas ERβ levels are low and become even lower in the aggressive spindle tumours of carcinogen-treated mice. Then, using the multistage mouse skin carcinogenesis model, we showed that ERα gradually increases during promotion and progression stages of mouse skin carcinogenesis, peaking at the most aggressive stage, whereas ERβ levels only slightly change throughout skin carcinogenesis. Stable transfection of the aggressive, spindle CarB cells with a dominant negative form of ERα (dnERα resulted in reduced ERα levels and reduced binding to estrogen responsive elements (ERE-containing sequences. We characterized two highly conserved EREs on the mouse ERα promoter through which dnERα decreased endogenous ERα levels. The dnERα-transfected CarB cells presented altered protein levels of cytoskeletal and cell adhesion molecules, slower growth rate and impaired anchorage-independent growth in vitro, whereas they gave smaller tumours with extended latency period of tumour onset in vivo. Our findings suggest an implication of ERα in the aggressiveness of spindle mouse skin cancer cells, possibly through regulation of genes affecting cell shape and adhesion, and they also provide hints for the effective targeting of spindle cancer cells by dnERα.

  4. The fumarate sensor DcuS: progress in rapid protein fold elucidation by combining protein structure prediction methods with NMR spectroscopy (United States)

    Meiler, Jens; Baker, David


    We illustrate how moderate resolution protein structures can be rapidly obtained by interlinking computational prediction methodologies with un- or partially assigned NMR data. To facilitate the application of our recently described method of ranking and subsequent refining alternative structural models using unassigned NMR data [Proc. Natl. Acad. Sci. USA 100 (2003) 15404] for such "structural genomics"-type experiments it is combined with protein models from several prediction techniques, enhanced to utilize partial assignments, and applied on a protein with an unknown structure and fold. From the original NMR spectra obtained for the 140 residue fumarate sensor DcuS, 1100 1H, 13C, and 15N chemical shift signals, 3000 1H- 1H NOESY cross peak intensities, and 209 backbone residual dipolar couplings were extracted and used to rank models produced by de novo structure prediction and comparative modeling methods. The ranking proceeds in two steps: first, an optimal assignment of the NMR peaks to atoms is found for each model independently, and second, the models are ranked based on the consistency between the NMR data and the model assuming these optimal assignments. The low-resolution model selected using this ranking procedure had the correct overall fold and a global backbone RMSD of 6.0Å, and was subsequently refined to 3.7 Å RMSD. With the incorporation of a small number of NOE and residual dipolar coupling constraints available very early in the traditional spectral assignment process, a model with an RMSD of 2.8 Å could rapidly be built. The ability to generate moderate resolution models within days of NMR data collection should facilitate large scale NMR structure determination efforts.

  5. Significant progression of load on the musculoskeletal system with extremely high loads, with rapid weekly weight gains, using the Anatoly Gravitational System, in a 10-week training period

    Directory of Open Access Journals (Sweden)

    Burke DT


    Full Text Available David T Burke,1 David Tran,1 Di Cui,1 Daniel P Burke,2 Samir Al-Adawi,3 Atsu SS Dorvlo41Emory University Medical School, Atlanta, GA, USA; 2Georgia College and State University, GA, USA; 3Department of Behavioral Medicine, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman; 4Department of Mathematics and Statistics, College of Science, Sultan Qaboos University, Muscat, OmanAbstract: In an age of increasing numbers of lifestyle diseases and plasticity of longevity, exercise and weight training have been increasingly recognized as both preventing and mitigating the severity of many illnesses. This study was designed to determine whether significant weight-lifting gains could be realized through the Anatoly Gravitational System. Specifically, this study sought to determine whether this once-weekly weight-training system could result in significant weekly strength gains during a 10-week training period. A total of 50 participants, ranging in age from 17 to 67 years, completed at least 10 weekly 30-minute training sessions. The results suggest participants could, on average, double their weight-lifting capacity within 10 sessions. This preliminary study, which would require further scrutiny, suggests the Anatoly Gravitational System provides a rather unique opportunity to load the musculoskeletal system with extremely high loads, with rapid weekly weight gains, using only short weekly training sessions. More studies are warranted to scrutinize these findings.Keywords: Anatoly Gravitational System, weight training, musculoskeletal system

  6. A rapid and robust assay for detection of S-phase cell cycle progression in plant cells and tissues by using ethynyl deoxyuridine

    Directory of Open Access Journals (Sweden)

    Horváth Gábor V


    Full Text Available Abstract Background Progress in plant cell cycle research is highly dependent on reliable methods for detection of cells replicating DNA. Frequency of S-phase cells (cells in DNA synthesis phase is a basic parameter in studies on the control of cell division cycle and the developmental events of plant cells. Here we extend the microscopy and flow cytometry applications of the recently developed EdU (5-ethynyl-2'-deoxyuridine-based S-phase assay to various plant species and tissues. We demonstrate that the presented protocols insure the improved preservation of cell and tissue structure and allow significant reduction in assay duration. In comparison with the frequently used detection of bromodeoxyuridine (BrdU and tritiated-thymidine incorporation, this new methodology offers several advantages as we discuss here. Results Applications of EdU-based S-phase assay in microscopy and flow cytometry are presented by using cultured cells of alfalfa, Arabidopsis, grape, maize, rice and tobacco. We present the advantages of EdU assay as compared to BrdU-based replication assay and demonstrate that EdU assay -which does not require plant cell wall digestion or DNA denaturation steps, offers reduced assay duration and better preservation of cellular, nuclear and chromosomal morphologies. We have also shown that fast and efficient EdU assay can also be an efficient tool for dual parameter flow cytometry analysis and for quantitative assessment of replication in thick root samples of rice. Conclusions In plant cell cycle studies, EdU-based S-phase detection offers a superior alternative to the existing S-phase assays. EdU method is reliable, versatile, fast, simple and non-radioactive and it can be readily applied to many different plant systems.

  7. Rapid Chondrocyte Isolation for Tissue Engineering Applications: The Effect of Enzyme Concentration and Temporal Exposure on the Matrix Forming Capacity of Nasal Derived Chondrocytes. (United States)

    Vedicherla, Srujana; Buckley, Conor Timothy


    Laboratory based processing and expansion to yield adequate cell numbers had been the standard in Autologous Disc Chondrocyte Transplantation (ADCT), Allogeneic Juvenile Chondrocyte Implantation (NuQu®), and Matrix-Induced Autologous Chondrocyte Implantation (MACI). Optimizing cell isolation is a key challenge in terms of obtaining adequate cell numbers while maintaining a vibrant cell population capable of subsequent proliferation and matrix elaboration. However, typical cell yields from a cartilage digest are highly variable between donors and based on user competency. The overall objective of this study was to optimize chondrocyte isolation from cartilaginous nasal tissue through modulation of enzyme concentration exposure (750 and 3000 U/ml) and incubation time (1 and 12 h), combined with physical agitation cycles, and to assess subsequent cell viability and matrix forming capacity. Overall, increasing enzyme exposure time was found to be more detrimental than collagenase concentration for subsequent viability, proliferation, and matrix forming capacity (sGAG and collagen) of these cells resulting in nonuniform cartilaginous matrix deposition. Taken together, consolidating a 3000 U/ml collagenase digest of 1 h at a ratio of 10 ml/g of cartilage tissue with physical agitation cycles can improve efficiency of chondrocyte isolation, yielding robust, more uniform matrix formation.

  8. Nucleon Electromagnetic Form Factors

    Energy Technology Data Exchange (ETDEWEB)

    Marc Vanderhaeghen; Charles Perdrisat; Vina Punjabi


    There has been much activity in the measurement of the elastic electromagnetic proton and neutron form factors in the last decade, and the quality of the data has greatly improved by performing double polarization experiments, in comparison with previous unpolarized data. Here we review the experimental data base in view of the new results for the proton, and neutron, obtained at JLab, MAMI, and MIT-Bates. The rapid evolution of phenomenological models triggered by these high-precision experiments will be discussed, including the recent progress in the determination of the valence quark generalized parton distributions of the nucleon, as well as the steady rate of improvements made in the lattice QCD calculations.

  9. Rapidly progressive post-transplant lymphoproliferative disease ...

    African Journals Online (AJOL)

    He presented with ataxia and acute confusion. In the 3 months preceding presentation he had experienced unexplained, asymptomatic weight loss. Clinical examination was unremarkable. Sirolimus had been stopped. 1 month before the onset of symptoms, owing to an apparent functional iron deficiency in the absence of ...

  10. Rapidly Progressive and Almost Lethal Pneumonia. (United States)

    Martínez-González, Juancarlo; Robles-Arias, Carlos; Rodríguez-Cintrón, William


    We herein describe the case of a 65-year-old male patient who presented with Osler's triad, which is the combination of endocarditis, pneumonia, and meningitis. This report is even more unusual since the pathogen isolated was the invasive and virulent strain of Streptococcus pneumoniae serotype 3. The clinical entity described is also called Austrian syndrome. Even though rare in this antibiotic era, the syndrome remains one of high morbidity and mortality. This particular case is of paramount importance for the clinician reader. First, it documents the clinical features associated with invasive pneumococcal disease and the Austrian syndrome. Second, and equally important, it highlights why following the Surviving Sepsis Campaign guidelines saves lives. For this case, the following steps were taken: 1. As a surrogate for perfusion, early and aggressive fluid resuscitation therapy (guided by lactic acid levels) was instituted; 2. also early in the treatment, broad spectrum antibiotics were administered; 3. to guide antibiotic therapy, microbiological cultures were obtained. The patient subsequently improved and was transferred to the internal medicine ward to complete 4 weeks of antibiotic therapy.

  11. [Variants of changes in the psychopathological phenomenology of the long-term remission in stable patients with attack-like progressive form of schizophrenia: a longitudinal study]. (United States)

    Morozova, M A; Burminskiy, D S; Rupchev, G E


    An analysis of literature data on schizophrenia shows the change of interest from acute psychosis to phenomenology of remission. The separate objects of research become the impact of symptoms, observed in remission, on everyday life of patients and factors, which influence rapidity and comprehension of reintegration of patients into society. Authors aimed to describe the changes in psychopathological phenomenology and personality functioning during long-term (no less than three years) remission in patients with schizophrenia treated with atypical antipsychotics. One hundred and thirty stable patients with schizophrenia underwent regular clinical examination during no less than three years. All patients were constantly medicated with atypical antipsychotics. Long-term observation of patients with the similar diagnosis and phase of disease, similar treatment and good treatment response (at least without psychotic exacerbation during the observation period) and tolerability revealed difference in changes of psychopathological phenomenology and personality functioning, which could be grouped into four distinct variants. The description of specificity of each variant is presented. A role of the initial level of personality functioning and its changes during observation period were analyzed. A new concept is introduced in order to name the ability of patients to use rationally recourses, delivered by treatment. The connection of this concept with the level of personality functioning was demonstrated. The hypothesis of mechanisms which underlie the four variants is offered. Long-term observation of the changes in the psychopathological phenomenology in the period of disease reveals the variants of the development of remission. The fluctuation of adaptive and regressive phases is registered. More objective factors, which impact on prognosis, are elucidated.

  12. Teaching Form as Form

    DEFF Research Database (Denmark)

    Keiding, Tina Bering


    , this book offers both inspiration to teaching form and a systematic framework for pedagogical and didactical reflection on this topic. In this sense, it shapes and professionalizes design teaching, and contributes to the development of the double-professionalism, which is so essential for teachers in modern...... means that form serves both as the connective value and as the concept for reflection. In other words, form is observed as form, not anything else. The didactical challenge of teaching form as form is accentuated by students’ everyday-based pre-orientation towards function at the expense of form...... in this book that they are highly interested in both the declarative and formative dimension of making form. Methodologically, the courses described in the contributions have a strong focus on student-centered experiential activities, thereby implicitly claiming that students must learn to make form...

  13. A matrix risk model for the prediction of rapid radiographic progression in patients with rheumatoid arthritis receiving different dynamic treatment strategies: post hoc analyses from the BeSt study. (United States)

    Visser, K; Goekoop-Ruiterman, Y P M; de Vries-Bouwstra, J K; Ronday, H K; Seys, P E H; Kerstens, P J S M; Huizinga, T W J; Dijkmans, B A C; Allaart, C F


    To develop a matrix model for the prediction of rapid radiographic progression (RRP) in subpopulations of patients with recent-onset rheumatoid arthritis (RA) receiving different dynamic treatment strategies. Data from 465 patients with recent-onset RA randomised to receive initial monotherapy or combination therapy were used. Predictors for RRP (increase in Sharp-van der Heijde score > or =5 after 1 year) were identified by multivariate logistic regression analysis. For subpopulations, the estimated risk of RRP per treatment group and the number needed to treat (NNT) were visualised in a matrix. The presence of autoantibodies, baseline C-reactive protein (CRP) level, erosion score and treatment group were significant independent predictors of RRP in the matrix. Combination therapy was associated with a markedly reduced risk of RRP. The positive and negative predictive values of the matrix were 62% and 91%, respectively. The NNT with initial combination therapy to prevent one patient from RRP with monotherapy was in the range 2-3, 3-7 and 7-25 for patients with a high, intermediate and low predicted risk, respectively. The matrix model visualises the risk of RRP for subpopulations of patients with recent-onset RA if treated dynamically with initial monotherapy or combination therapy. Rheumatologists might use the matrix for weighing their initial treatment choice.

  14. Around the laboratories: Dubna: Physics results and progress on bubble chamber techniques; Stanford (SLAC): Operation of a very rapid cycling bubble chamber; Daresbury: Photographs of visitors to the Laboratory; Argonne: Charge exchange injection tests into the ZGS in preparation for a proposed Booster

    CERN Multimedia


    Around the laboratories: Dubna: Physics results and progress on bubble chamber techniques; Stanford (SLAC): Operation of a very rapid cycling bubble chamber; Daresbury: Photographs of visitors to the Laboratory; Argonne: Charge exchange injection tests into the ZGS in preparation for a proposed Booster

  15. Primary Progressive Aphasia (United States)

    ... include: Having difficulty retrieving words Frequently pausing in speech while searching for words Having difficulty repeating phrases or sentences Nonfluent-agrammatic variant primary progressive aphasia Symptoms include: Having difficulty forming words Being hesitant ...

  16. Predictive value of autoantibodies from anti-CCP2, anti-MCV and anti-human citrullinated fibrinogen tests, in early rheumatoid arthritis patients with rapid radiographic progression at 1 year: results from the ESPOIR cohort. (United States)

    Degboé, Yannick; Constantin, Arnaud; Nigon, Delphine; Tobon, Gabriel; Cornillet, Martin; Schaeverbeke, Thierry; Chiocchia, Gilles; Nicaise-Roland, Pascale; Nogueira, Leonor; Serre, Guy; Cantagrel, Alain; Ruyssen-Witrand, Adeline


    We compared the ability of antibodies against cyclic citrullinated peptides (anti-CCP2), against mutated citrullinated vimentin (anti-MCV) and against citrullinated fibrinogen (AhFibA) to predict 1 year rapid radiographic progression (RRP; total Sharp score variation ≥5 points), in early rheumatoid arthritis (RA). We analysed 566 patients from the ESPOIR cohort with early RA fulfilling the 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria at year 1. We assayed the 3 anticitrullinated peptide antibodies (ACPA) tests on baseline sera. We compared the performance of these 3 ACPA tests to predict first-year RRP, by comparing areas under the receiver operating characteristic curves (ROCs). We assessed the 1 year RRP risk by ACPA titres. We used a logistic multivariate regression to analyse RRP risk in terms either of ACPA positivity or titre: high (>3 times the N cut-off) and low (1 to 3N). 145 patients displayed RRP. Areas under the ROCs were similar (0.60) for the 3 tests. High ACPA titres were associated with 1 year RRP, whatever the test was, and with similar ORs. Low+ anti-MCV titres were not associated with 1-year RRP, whereas low+ anti-CCP2 titres (p=0.0226) and low+ AhFibA titres (p=0.0332) were significantly associated. In multivariate analysis, 1 year RRP was associated with anti-CCP2 positivity (p1), AhFibA positivity (p1) and high anti-MCV titres (p1). Anti-CCP2 antibodies and AhFibA were predictive of 1 year RRP in early RA whatever their titre was, whereas only high anti-MCV antibody titres were predictive, potentially making them more discriminant to predict 1 year RRP risk.

  17. Iatrogenic keratectasia after LASIK in a case of forme fruste keratoconus. (United States)

    Seiler, T; Quurke, A W


    We report an iatrogenic corneal ectasia that developed after laser in situ keratomileusis (LASIK) in a case of forme fruste keratoconus. One month postoperatively, corneal topography revealed a central steep area that showed rapid progression. Visual acuity in the eye decreased from 20/20 to 20/80. Corneas with forme fruste keratoconus may have altered biomechanical properties compared with normal corneas. Forme fruste keratoconus may be a contraindication for LASIK.


    Energy Technology Data Exchange (ETDEWEB)

    Nelson, Erica June; Van Dokkum, Pieter G.; Skelton, Rosalind E.; Bezanson, Rachel; Lundgren, Britt [Astronomy Department, Yale University, New Haven, CT 06511 (United States); Brammer, Gabriel [European Southern Observatory, Alonson de Cordova 3107, Casilla 19001, Vitacura, Santiago (Chile); Foerster Schreiber, Natascha [Max-Planck-Institut fuer extraterrestrische Physik, Giessenbachstrasse, D-85748 Garching (Germany); Franx, Marijn; Fumagalli, Mattia; Patel, Shannon; Labbe, Ivo [Leiden Observatory, Leiden University, Leiden (Netherlands); Rix, Hans-Walter; Da Cunha, Elisabete; Schmidt, Kasper B. [Max Planck Institute for Astronomy (MPIA), Koenigstuhl 17, 69117 Heidelberg (Germany); Kriek, Mariska [Department of Astronomy, University of California, Berkeley, CA 94720 (United States); Quadri, Ryan [Carnegie Observatories, 813 Santa Barbara Street, Pasadena, CA 91101 (United States)


    We investigate the buildup of galaxies at z {approx} 1 using maps of H{alpha} and stellar continuum emission for a sample of 57 galaxies with rest-frame H{alpha} equivalent widths >100 A in the 3D-HST grism survey. We find that the H{alpha} emission broadly follows the rest-frame R-band light but that it is typically somewhat more extended and clumpy. We quantify the spatial distribution with the half-light radius. The median H{alpha} effective radius r{sub e} (H{alpha}) is 4.2 {+-} 0.1 kpc but the sizes span a large range, from compact objects with r{sub e} (H{alpha}) {approx} 1.0 kpc to extended disks with r{sub e} (H{alpha}) {approx} 15 kpc. Comparing H{alpha} sizes to continuum sizes, we find =1.3 {+-} 0.1 for the full sample. That is, star formation, as traced by H{alpha}, typically occurs out to larger radii than the rest-frame R-band stellar continuum; galaxies are growing their radii and building up from the inside out. This effect appears to be somewhat more pronounced for the largest galaxies. Using the measured H{alpha} sizes, we derive star formation rate surface densities, {Sigma}{sub SFR}. We find that {Sigma}{sub SFR} ranges from {approx}0.05 M{sub Sun} yr{sup -1} kpc{sup -2} for the largest galaxies to {approx}5 M{sub Sun} yr{sup -1} kpc{sup -2} for the smallest galaxies, implying a large range in physical conditions in rapidly star-forming z {approx} 1 galaxies. Finally, we infer that all galaxies in the sample have very high gas mass fractions and stellar mass doubling times <500 Myr. Although other explanations are also possible, a straightforward interpretation is that we are simultaneously witnessing the rapid formation of compact bulges and large disks at z {approx} 1.

  19. Collaborative form(s)

    DEFF Research Database (Denmark)

    Gunn, Wendy

    Gunn asks us to consider beauty as collaborative forms of action generated by moving between design by means of anthropology and anthropology by means of design. Specifically, she gives focus to play-like reflexions on practices of designing energy products, systems and infrastructure. Design...... anthropology engages groups of people within collaborative, interdisciplinary, inter-organizational design processes and co-analytic activities vs. the individual anthropologist conducting studies of people. In doing anthropology by means of design as Gatt and Ingold (2013) have shown, design is considered...

  20. RAPID3? Aptly named! (United States)

    Berthelot, J-M


    The RAPID3 score is the sum of three 0-10 patient self-report scores: pain, functional impairment on MDHAQ, and patient global estimate. It requires 5 seconds for scoring and can be used in all rheumatologic conditions, although it has mostly been used in rheumatoid arthritis where cutoffs for low disease activity (12/30) have been set. A RAPID3 score of ≤ 3/30 with 1 or 0 swollen joints (RAPID3 ≤ 3 + ≤ SJ1) provides remission criteria comparable to Boolean, SDAI, CDAI, and DAS28 remission criteria, in far less time than a formal joint count. RAPID3 performs as well as the DAS28 in separating active drugs from placebos in clinical trials. RAPID3 also predicts subsequent structural disease progression. RAPID3 can be determined at short intervals at home, allowing the determination of the area under the curve of disease activity between two visits and flare detection. However, RAPID3 should not be seen as a substitute for DAS28 and face to face visits in routine care. Monitoring patient status with only self-report information without a rheumatologist's advice (including joints and physical examination, and consideration of imaging and laboratory tests) may indeed be as undesirable for most patients than joint examination without a patient questionnaire. Conversely, combining the RAPID3 and the DAS28 may consist in faster or more sensitive confirmation that a medication is effective. Similarly, better enquiring of most important concerns of patients (pain, functional status and overall opinion on their disorder) should reinforces patients' confidence in their rheumatologist and treatments.

  1. Volcanoes drive climate variability by emitting ozone weeks before eruptions, by forming lower stratospheric aerosols, by causing sustained ozone depletion, and by causing rapid changes in regional ozone concentrations affecting temperature and pressure differences driving atmospheric oscillations (United States)

    Ward, P. L.


    Total column ozone observed by satellite on February 19, 2010, increased 75% in a plume from Eyjafjallajökull volcano in southern Iceland eastward past Novaya Zemlya, extending laterally from northern Greenland to southern Norway ( Contemporaneous ground deformation and rapidly increasing numbers of earthquakes imply magma began rising from a sill 4-6 km below the volcano, erupting a month later. Whether the ozone formed from the magma or from very hot gases rising through cracks in the ground is unclear. On February 20-22, 1991, similar increases in ozone were observed north of Pinatubo volcano before its initial eruption on April 2 ( Annual average total column ozone during the year of most moderate to large explosive volcanic eruptions since routine observations of ozone began in 1927 has been substantially higher than normal. Increased total column ozone absorbs more solar ultraviolet-B radiation, warming the ozone layer and cooling Earth. Most major volcanic eruptions form sulfuric-acid aerosols in the lower part of the ozone layer providing aqueous surfaces on which heterogeneous chemical reactions enhance ozone depletion. Within a year, aerosol droplets grew large enough to reflect and scatter high-frequency solar radiation, cooling Earth 0.5oC for 2-3 years. Temperature anomalies in the northern hemisphere rose 0.7oC in 28 years from 1970 to 1998 (HadCRUT4), while annual average ozone at Arosa dropped 27 DU because of manufactured CFC gases. Beginning in August 2014, temperature anomalies in the northern hemisphere rose another 0.6oC in less than two years apparently because of the 6-month eruption of Bárðarbunga volcano in central Iceland, the highest rate of basaltic lava extrusion since 1783. Large extrusions of basaltic lava are typically contemporaneous with the greatest periods of warming throughout Earth history. Ozone concentrations at Arosa change by season typically from 370 DU during

  2. Internationalisering og progression

    DEFF Research Database (Denmark)

    Wilken, Lisanne; Tange, Hanne


    education followed by two years of specialization within the same discipline. This idea is now being challenged on several fronts. For instance, it is becoming more common for Danish universities to offer interdisciplinary master programs. Also, the trend for greater internationalization in higher education...... sig til progression. Artiklen er skrevet på baggrund af semistrukturerede interviews med undervisere fra tværfaglige, internationale uddannelser ved Aarhus Universitet. University programs in Denmark have traditionally been perceived as a continuous education consisting of three years of basic...... universities reflect on progression in education. The article distinguishes between different forms of progression....

  3. Progress Report

    DEFF Research Database (Denmark)

    Duer, Karsten


    Progress report describing the work carried out by the Danish participant in the ALTSET project in the period January 1999 to July 1999.......Progress report describing the work carried out by the Danish participant in the ALTSET project in the period January 1999 to July 1999....

  4. Rapid small lot manufacturing

    Energy Technology Data Exchange (ETDEWEB)

    Harrigan, R.W.


    The direct connection of information, captured in forms such as CAD databases, to the factory floor is enabling a revolution in manufacturing. Rapid response to very dynamic market conditions is becoming the norm rather than the exception. In order to provide economical rapid fabrication of small numbers of variable products, one must design with manufacturing constraints in mind. In addition, flexible manufacturing systems must be programmed automatically to reduce the time for product change over in the factory and eliminate human errors. Sensor based machine control is needed to adapt idealized, model based machine programs to uncontrolled variables such as the condition of raw materials and fabrication tolerances.

  5. Maior sobrevida em pacientes com marcadores imunogenéticos de rápida progressão para a AIDS: subsídios para a assistência de enfermagem Mayor supervivencia en pacientes presentando marcadores inmunogenéticos de rápida progresión para el SIDA: apoyo para la atención de enfermería Greater survival among patients with immunogenetic markers of rapid progression to AIDS: subsidies for nursing care

    Directory of Open Access Journals (Sweden)

    Ana Paula M. Fernandes


    ón de salud con enfoque en el comportamiento positivo de salud son herramientas que la enfermería debe utilizar con portadores del VIH, buscando la mejor calidad de vida y mayor supervivencia de esos individuos, incluso en aquellos que tienen predisposición genética a la rápida progresión de la enfermedad.This study sought subsidies for improving nursing care programs for AIDS patients and aimed to verify the influence of changes in sexual behavior, including the adoption of safe sex practices, associated with the survival of AIDS patients with immunogenetic markers of rapid disease progression. 27 AIDS patients were interviewed, with genetic predisposition to rapid progression to AIDS. Genes were typified through the polymerase chain reaction. In spite of the presence of immunogenetic factors, associated with individual predisposition to a rapid evolution of the disease, changes in sexual behavior, including safe sex practices and antiretroviral therapy, may be related to greater survival. This suggests that counseling, detection of risk attitudes and health education, focusing on positive health behavior, are tools nursing must use with HIV-positive patients, with a view to better quality of life and greater survival among these individuals, even among those with genetic predisposition to rapid disease progression.

  6. Rapid Prototyping (United States)


    Javelin, a Lone Peak Engineering Inc. Company has introduced the SteamRoller(TM) System as a commercial product. The system was designed by Javelin during a Phase II NASA funded small commercial product. The purpose of the invention was to allow automated-feed of flexible ceramic tapes to the Laminated Object Manufacturing rapid prototyping equipment. The ceramic material that Javelin was working with during the Phase II project is silicon nitride. This engineered ceramic material is of interest for space-based component.

  7. Progressive Business

    DEFF Research Database (Denmark)

    Christiansen, Christian O.


    Guest Post to the Society for U.S. Intellectual History Blog. Brief introduction to the book Progressive Business: An Intellectual History of the Role of Business in American Society, Oxford U.P., 2015.......Guest Post to the Society for U.S. Intellectual History Blog. Brief introduction to the book Progressive Business: An Intellectual History of the Role of Business in American Society, Oxford U.P., 2015....

  8. Rapid thermal processing of semiconductors

    CERN Document Server

    Borisenko, Victor E


    Rapid thermal processing has contributed to the development of single wafer cluster processing tools and other innovations in integrated circuit manufacturing environments Borisenko and Hesketh review theoretical and experimental progress in the field, discussing a wide range of materials, processes, and conditions They thoroughly cover the work of international investigators in the field

  9. Progressive osseous heteroplasia

    African Journals Online (AJOL)

    ous and deep connective tissue including muscle and fascia during childhood. It is at the severe end of a spectrum of Guanine ... and extensive bone formation in deep muscle and fascia. Dermal lesions co- alesce rapidly to form ... web-like calcification surrounding all the upper and lower limbs and sparing only the soft ...

  10. Modular forms

    NARCIS (Netherlands)

    Edixhoven, B.; van der Geer, G.; Moonen, B.; Edixhoven, B.; van der Geer, G.; Moonen, B.


    Modular forms are functions with an enormous amount of symmetry that play a central role in number theory, connecting it with analysis and geometry. They have played a prominent role in mathematics since the 19th century and their study continues to flourish today. Modular forms formed the

  11. [Specificities of the logopenic variant of primary progressive aphasia]. (United States)

    Magnin, E; Teichmann, M; Martinaud, O; Moreaud, O; Ryff, I; Belliard, S; Pariente, J; Moulin, T; Vandel, P; Démonet, J-F


    The logopenic variant of primary progressive aphasia is a syndrome with neuropsychological and linguistic specificities, including phonological loop impairment for which diagnosis is currently mainly based on the exclusion of the two other variants, semantic and nonfluent/agrammatic primary progressive aphasia. The syndrome may be underdiagnosed due (1) to mild language difficulties during the early stages of the disease or (2) to being mistaken for mild cognitive impairment or Alzheimer's disease when the evaluation of episodic memory is based on verbal material and (3) finally, it is not uncommon that the disorders are attributed to psychiatric co-morbidities such as, for example, anxiety. Moreover, compared to other variants of primary progressive aphasia, brain abnormalities are different. The left temporoparietal junction is initially affected. Neuropathology and biomarkers (cerebrospinal fluid, molecular amyloid nuclear imaging) frequently reveal Alzheimer's disease. Consequently this variant of primary progressive aphasia does not fall under the traditional concept of frontotemporal lobar degeneration. These distinctive features highlight the utility of correct diagnosis, classification, and use of biomarkers to show the neuropathological processes underlying logopenic primary progressive aphasia. The logopenic variant of primary progressive aphasia is a specific form of Alzheimer's disease frequently presenting a rapid decline; specific linguistic therapies are needed. Further investigation of this syndrome is needed to refine screening, improve diagnostic criteria and better understand the epidemiology and the biological mechanisms involved. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  12. The Application Trends of Rapid Prototyping Manufacturing

    Directory of Open Access Journals (Sweden)

    Qiu Xiao Lin


    characteristics of laser stero lithography (LSL selective laser sintering (SLS, three-dimensional printing (DP, fused deposition modeling (FDM, computer numerical control (CNC and other rapid prototyping technologies. After discussed these five rapid prototyping technology materials, we presented the hotspot and direction of rapid prototyping technology and look forward to the development of its technique, the expansion of its field and the progress of its academic ideology.

  13. Rapid chemical separations

    CERN Document Server

    Trautmann, N


    A survey is given on the progress of fast chemical separation procedures during the last few years. Fast, discontinuous separation techniques are illustrated by a procedure for niobium. The use of such techniques for the chemical characterization of the heaviest known elements is described. Other rapid separation methods from aqueous solutions are summarized. The application of the high speed liquid chromatography to the separation of chemically similar elements is outlined. The use of the gas jet recoil transport method for nuclear reaction products and its combination with a continuous solvent extraction technique and with a thermochromatographic separation is presented. Different separation methods in the gas phase are briefly discussed and the attachment of a thermochromatographic technique to an on-line mass separator is shown. (45 refs).

  14. Rapid Airplane Parametric Input Design (RAPID) (United States)

    Smith, Robert E.


    RAPID is a methodology and software system to define a class of airplane configurations and directly evaluate surface grids, volume grids, and grid sensitivity on and about the configurations. A distinguishing characteristic which separates RAPID from other airplane surface modellers is that the output grids and grid sensitivity are directly applicable in CFD analysis. A small set of design parameters and grid control parameters govern the process which is incorporated into interactive software for 'real time' visual analysis and into batch software for the application of optimization technology. The computed surface grids and volume grids are suitable for a wide range of Computational Fluid Dynamics (CFD) simulation. The general airplane configuration has wing, fuselage, horizontal tail, and vertical tail components. The double-delta wing and tail components are manifested by solving a fourth order partial differential equation (PDE) subject to Dirichlet and Neumann boundary conditions. The design parameters are incorporated into the boundary conditions and therefore govern the shapes of the surfaces. The PDE solution yields a smooth transition between boundaries. Surface grids suitable for CFD calculation are created by establishing an H-type topology about the configuration and incorporating grid spacing functions in the PDE equation for the lifting components and the fuselage definition equations. User specified grid parameters govern the location and degree of grid concentration. A two-block volume grid about a configuration is calculated using the Control Point Form (CPF) technique. The interactive software, which runs on Silicon Graphics IRIS workstations, allows design parameters to be continuously varied and the resulting surface grid to be observed in real time. The batch software computes both the surface and volume grids and also computes the sensitivity of the output grid with respect to the input design parameters by applying the precompiler tool

  15. Measuring progress

    DEFF Research Database (Denmark)

    Wahlberg, Ayo


    In recent years, sociological examinations of genetics, therapeutic cloning, neuroscience and tissue engineering have suggested that 'life itself' is currently being transformed through technique with profound implications for the ways in which we understand and govern ourselves and others...... in much the same way that mortality rates, life expectancy or morbidity rates can. By analysing the concrete ways in which human progress has been globally measured and taxonomised in the past two centuries or so, I will show how global stratifications of countries according to their states...

  16. Rapidity resummation for B-meson wave functions

    Directory of Open Access Journals (Sweden)

    Shen Yue-Long


    Full Text Available Transverse-momentum dependent (TMD hadronic wave functions develop light-cone divergences under QCD corrections, which are commonly regularized by the rapidity ζ of gauge vector defining the non-light-like Wilson lines. The yielding rapidity logarithms from infrared enhancement need to be resummed for both hadronic wave functions and short-distance functions, to achieve scheme-independent calculations of physical quantities. We briefly review the recent progress on the rapidity resummation for B-meson wave functions which are the key ingredients of TMD factorization formulae for radiative-leptonic, semi-leptonic and non-leptonic B-meson decays. The crucial observation is that rapidity resummation induces a strong suppression of B-meson wave functions at small light-quark momentum, strengthening the applicability of TMD factorization in exclusive B-meson decays. The phenomenological consequence of rapidity-resummation improved B-meson wave functions is further discussed in the context of B → π transition form factors at large hadronic recoil.

  17. Rapid prototype and test

    Energy Technology Data Exchange (ETDEWEB)

    Gregory, D.L.; Hansche, B.D.


    In order to support advanced manufacturing, Sandia has acquired the capability to produce plastic prototypes using stereolithography. Currently, these prototypes are used mainly to verify part geometry and ``fit and form`` checks. This project investigates methods for rapidly testing these plastic prototypes, and inferring from prototype test data actual metal part performance and behavior. Performances examined include static load/stress response, and structural dynamic (modal) and vibration behavior. The integration of advanced non-contacting measurement techniques including scanning laser velocimetry, laser holography, and thermoelasticity into testing of these prototypes is described. Photoelastic properties of the epoxy prototypes to reveal full field stress/strain fields are also explored.

  18. Multiple gingival pregnancy tumors with rapid growth

    Directory of Open Access Journals (Sweden)

    Wei-Lian Sun


    Full Text Available Pregnancy gingivitis is an acute form of gingivitis that affects pregnant women, with a prevalence of 30%, possibly ranging up to 100%. Sometimes, pregnancy gingivitis shows a tendency toward a localized hyperplasia called gingival pyogenic granuloma. Pregnancy tumor is a benign gingival hyperplasia with the gingiva as the most commonly involved site, but rarely it involves almost the entire gingiva. A 22-year-old woman was referred to our clinic with a chief complaint of gingival swelling that had lasted for 2 days. The lesions progressed rapidly and extensively, and almost all the gingiva was involved a week later. Generalized erythema, edema, hyperplasia, a hemorrhagic tendency, and several typical hemangiomatous masses were noted. Pregnancy was denied by the patient at the first and second visits, but was confirmed 2 weeks after the primary visit. The patient was given oral hygiene instructions. She recovered well, and the mass gradually regressed and had disappeared completely at the end of 12 weeks of pregnancy, without recurrence. The gingival lesions were finally diagnosed as multiple gingival pregnancy tumors. The patient delivered a healthy infant. An extensive and rapid growth of gingival pregnancy tumors during the early first month of pregnancy is a rare occurrence that is not familiar to dentists, gynecologists, and obstetricians. Those practitioners engaged in oral medicine and periodontology, primary care obstetrics, and gynecology should be aware of such gingival lesions to avoid misdiagnosis and overtreatment.

  19. Progress in Controlled Gastroretentive Delivery Systems | Garg ...

    African Journals Online (AJOL)

    Progress in Controlled Gastroretentive Delivery Systems. ... Abstract. Controlled release (CR) dosage forms have been extensively used to improve therapy with several important drugs. However, the ... Keywords: Gastroretention, Oral controlled release, Swelling, Narrow absorption window, Floating dosage form. Tropical ...

  20. Potential of Progressive Construction Systems in Slovakia (United States)

    Kozlovska, Maria; Spisakova, Marcela; Mackova, Daniela


    Construction industry is a sector with rapid development. Progressive technologies of construction and new construction materials also called modern methods of construction (MMC) are developed constantly. MMC represent the adoption of construction industrialisation and the use of prefabrication of components in building construction. One of these modern methods is also system Varianthaus, which is based on, insulated concrete forms principle and provides complete production plant for wall, ceiling and roof elements for a high thermal insulation house construction. Another progressive construction system is EcoB, which represents an insulated precast concrete panel based on combination of two layers, insulation and concrete, produced in a factory as a whole. Both modern methods of construction are not yet known and wide-spread in the Slovak construction market. The aim of this paper is focused on demonstration of MMC using potential in Slovakia. MMC potential is proved based on comparison of the selected parameters of construction process – construction costs and construction time. The subject of this study is family house modelled in three material variants – masonry construction (as a representative of traditional methods of construction), Varianthaus and EcoB (as the representatives of modern methods of construction). The results of this study provide the useful information in decision-making process for potential investors of construction.

  1. EDITORIAL: Catalysing progress Catalysing progress (United States)

    Demming, Anna


    Examples of the merits of blue-sky research in the history of science are legion. The invention of the laser, celebrating its 50th anniversary this year, is an excellent example. When it was invented it was considered to be 'a solution waiting for a problem', and yet the level to which it has now infiltrated our day-to-day technological landscape speaks volumes. At the same time it is also true to say that the direction of research is also at times rightly influenced by the needs and concerns of the general public. Over recent years, growing concerns about the environment have had a noticeable effect on research in nanotechnology, motivating work on a range of topics from green nanomaterial synthesis [1] to high-efficiency solar cells [2] and hydrogen storage [3]. The impact of the world's energy consumption on the welfare of the planet is now an enduring and well founded concern. In the face of an instinctive reluctance to curtail habits of comfort and convenience and the appendages of culture and consumerism, research into renewable and more efficient energy sources seem an encouraging approach to alleviating an impending energy crisis. Fuel cells present one alternative to traditional combustion cells that have huge benefits in terms of the efficiency of energy conversion and the limited harmful emissions. In last week's issue of Nanotechnology, Chuan-Jian Zhong and colleagues at the State University of New York at Binghamton in the USA presented an overview of research on nanostructured catalysts in fuel cells [4]. The topical review includes insights into the interactions between nanoparticles and between nanoparticles and their substrate as well as control over the composition and nanostructure of catalysts. The review also serves to highlight how the flourishing of nanotechnology research has heralded great progress in the exploitation of catalysts with nanostructures ingeniously controlled to maximize surface area and optimize energetics for synthesis

  2. Rapidly progressive course of primary renal synovial sarcoma: Case report

    Directory of Open Access Journals (Sweden)

    Marković-Lipkovski Jasmina


    Full Text Available Introduction. Primary kidney sarcoma, especially synovial sarcoma (SS, is a very rare neoplasm. Pre-operative signs and symptoms are very similar to renal cell carcinoma, therefore, the proper diagnosis is very difficult and usually made after nephrectomy. This is a case report of primary renal SS. Case Outline. A 38-year-old man presented with a history of fever and hematuria, and right flank pain 3 weeks ago. Abdominal computerized tomography revealed a heterogeneous well-marginated soft tissue mass arising in the lower part of the right kidney. Right nephrectomy was performed. A cystic tumor of 120x85 mm in size with soft solid growth, and with the extensive areas of hemorrhage and necrosis was seen on gross examination. Histopathology revealed a neoplasm composed of solid monomorphic sheets of spindle cells. Immunohistochemistry showed tumor cells strongly positive for BCL2, CD99, CD56 and vimentin, and focally positive for epithelial membrane antigen (EMA. The histological diagnosis of primary renal SS was based on morphology and immunohistochemistry. FISH analysis and RT-PCR was carried out on formalin-fixed paraffin-embedded tissue sections. The molecular analysis demonstrated translocation of SYT gene on chromosome 18 and SSX2 gene on chromosome X. The findings were consistent with diagnosis of SS. Conclusion. Our case shows that histopathological diagnosis of primary kidney SS, although difficult, is possible to be made on the basis of morphological and immunohistochemical analysis. However, this diagnosis should be corroborated by molecular techniques confirming SYT-SSX translocation on chromosome 18 and chromosome X. Here we present visceral monophasic SS with aggressive clinical course and poor outcome. [Projekat Ministarstva nauke Republike Srbije, br. OI 175047

  3. Rapidly progressive periodontal disease associated with human immunodeficiency virus. (United States)

    Al-Hezaimi, Khalid; Javed, Fawad; Ali, Tazeen Saeed; Al-Askar, Mansour; Al-Rasheed, Abdulaziz


    Severe periodontal inflammation with generalized dental plaque accumulation, spontaneous and severe gingival bleeding, fungal infection, and interdental papillae necrosis are presented in a patient infected with human immunodeficiency virus (HIV). Bite-wing radiographs revealed a generalized horizontal alveolar bone loss of 7-8 millimetres in both arches. Erythematous patches were noted on the gingival mucosa in both jaws. DNA testing was performed to indentify the periodontopathogens. The patient had no signs or symptoms of acquired immunodeficiency syndrome. This case-report presents the massive periodontal destruction that occurred in a patient infected with HIV. Therefore, it is highly recommended that patients infected with HIV should be regularly monitored to aid in early detection and to provide proper management of periodontal inflammatory conditions to minimize its destruction.

  4. Tracking Clean Energy Progress 2013

    Energy Technology Data Exchange (ETDEWEB)



    Tracking Clean Energy Progress 2013 (TCEP 2013) examines progress in the development and deployment of key clean energy technologies. Each technology and sector is tracked against interim 2020 targets in the IEA Energy Technology Perspectives 2012 2°C scenario, which lays out pathways to a sustainable energy system in 2050. Stark message emerge: progress has not been fast enough; large market failures are preventing clean energy solutions from being taken up; considerable energy efficiency remains untapped; policies need to better address the energy system as a whole; and energy-related research, development and demonstration need to accelerate. Alongside these grim conclusions there is positive news. In 2012, hybrid-electric vehicle sales passed the 1 million mark. Solar photovoltaic systems were being installed at a record pace. The costs of most clean energy technologies fell more rapidly than anticipated.

  5. Spray-formed tooling

    Energy Technology Data Exchange (ETDEWEB)

    McHugh, K.M.; Key, J.F.


    The United States Council for Automotive Research (USCAR) has formed a partnership with the Idaho National Engineering Laboratory (INEL) to develop a process for the rapid production of low-cost tooling based on spray forming technology developed at the INEL. Phase 1 of the program will involve bench-scale system development, materials characterization, and process optimization. In Phase 2, prototype systems will be de signed, constructed, evaluated, and optimized. Process control and other issues that influence commercialization will be addressed during this phase of the project. Technology transfer to USCAR, or a tooling vendor selected by USCAR, will be accomplished during Phase 3. The approach INEL is using to produce tooling, such as plastic injection molds and stamping dies, combines rapid solidification processing and net-shape materials processing into a single step. A bulk liquid metal is pressure-fed into a de Laval spray nozzle transporting a high velocity, high temperature inert gas. The gas jet disintegrates the metal into fine droplets and deposits them onto a tool pattern made from materials such as plastic, wax, clay, ceramics, and metals. The approach is compatible with solid freeform fabrication techniques such as stereolithography, selective laser sintering, and laminated object manufacturing. Heat is extracted rapidly, in-flight, by convection as the spray jet entrains cool inert gas to produce undercooled and semi-solid droplets. At the pattern, the droplets weld together while replicating the shape and surface features of the pattern. Tool formation is rapid; deposition rates in excess of 1 ton/h have been demonstrated for bench-scale nozzles.

  6. Internationalisering og progression

    Directory of Open Access Journals (Sweden)

    Lisanne Wilken


    article summarizes their opinions and distinguishes In this article we explore how Danish university professors teaching at interdisciplinary international master programs at Danish universities reflect on progression in education. The article distinguishes between different forms of progression.

  7. Predicting progression of Alzheimer's disease. (United States)

    Doody, Rachelle S; Pavlik, Valory; Massman, Paul; Rountree, Susan; Darby, Eveleen; Chan, Wenyaw


    Clinicians need to predict prognosis of Alzheimer's disease (AD), and researchers need models of progression to develop biomarkers and clinical trials designs. We tested a calculated initial progression rate to see whether it predicted performance on cognition, function and behavior over time, and to see whether it predicted survival. We used standardized approaches to assess baseline characteristics and to estimate disease duration, and calculated the initial (pre-progression) rate in 597 AD patients followed for up to 15 years. We designated slow, intermediate and rapidly progressing groups. Using mixed effects regression analysis, we examined the predictive value of a pre-progression group for longitudinal performance on standardized measures. We used Cox survival analysis to compare survival time by progression group. Patients in the slow and intermediate groups maintained better performance on the cognitive (ADAScog and VSAT), global (CDR-SB) and complex activities of daily living measures (IADL) (P values < 0.001 slow versus fast; P values < 0.003 to 0.03 intermediate versus fast). Interaction terms indicated that slopes of ADAScog and PSMS change for the slow group were smaller than for the fast group, and that rates of change on the ADAScog were also slower for the intermediate group, but that CDR-SB rates increased in this group relative to the fast group. Slow progressors survived longer than fast progressors (P = 0.024). A simple, calculated progression rate at the initial visit gives reliable information regarding performance over time on cognition, global performance and activities of daily living. The slowest progression group also survives longer. This baseline measure should be considered in the design of long duration Alzheimer's disease clinical trials.

  8. Rapid Prototyping by Single Point Incremental Forming of Sheet Metal

    DEFF Research Database (Denmark)

    Skjødt, Martin


    explains a lot of experimental observation seen in the literature. SPIF of tailored blanks produced by friction stir welding. It is demonstrated that SPIF of tailored sheets produced by friction stir welding is possible and a promising way of combining two innovative manufacturing processes. Multi stage...

  9. Using the MCF10A/MCF10CA1a Breast Cancer Progression Cell Line Model to Investigate the Effect of Active, Mutant Forms of EGFR in Breast Cancer Development and Treatment Using Gefitinib.

    Directory of Open Access Journals (Sweden)

    Darrell C Bessette

    Full Text Available Basal-like and triple negative breast cancer (TNBC share common molecular features, poor prognosis and a propensity for metastasis to the brain. Amplification of epidermal growth factor receptor (EGFR occurs in ~50% of basal-like breast cancer, and mutations in the epidermal growth factor receptor (EGFR have been reported in up to ~ 10% of Asian TNBC patients. In non-small cell lung cancer several different mutations in the EGFR tyrosine kinase domain confer sensitivity to receptor tyrosine kinase inhibitors, but the tumourigenic potential of EGFR mutations in breast cells and their potential for targeted therapy is unknown.Constructs containing wild type, G719S or E746-A750 deletion mutant forms of EGFR were transfected into the MCF10A breast cells and their tumorigenic derivative, MCF10CA1a. The effects of EGFR over-expression and mutation on proliferation, migration, invasion, response to gefitinib, and tumour formation in vivo was investigated. Copy number analysis and whole exome sequencing of the MCF10A and MCF10CA1a cell lines were also performed.Mutant EGFR increased MCF10A and MCF10CA1a proliferation and MCF10A gefitinib sensitivity. The EGFR-E746-A750 deletion increased MCF10CA1a cell migration and invasion, and greatly increased MCF10CA1a xenograft tumour formation and growth. Compared to MCF10A cells, MCF10CA1a cells exhibited large regions of gain on chromosomes 3 and 9, deletion on chromosome 7, and mutations in many genes implicated in cancer.Mutant EGFR enhances the oncogenic properties of MCF10A cell line, and increases sensitivity to gefitinib. Although the addition of EGFR E746-A750 renders the MCF10CA1a cells more tumourigenic in vivo it is not accompanied by increased gefitinib sensitivity, perhaps due to additional mutations, including the PIK3CA H1047R mutation, that the MCF10CA1a cell line has acquired. Screening TNBC/basal-like breast cancer for EGFR mutations may prove useful for directing therapy but, as in non

  10. Progressive Multifocal Leukoencephalopathy (United States)

    ... clumsiness; progressive weakness; and visual, speech, and sometimes personality changes. The progression of deficits leads to life-threatening disability and (frequently) death. A diagnosis of PML can ...

  11. Genetics of primary progressive multiple sclerosis


    Cree, BAC


    Multiple sclerosis (MS) patients are classified as either having relapsing onset or progressive onset disease, also known as primary progressive MS (PPMS). Relative to relapsing onset patients, PPMS patients are older at disease onset, are equally likely to be men or women, and have more rapid accumulation of disability that does not respond well to treatments used in relapsing onset MS. Although estimates vary, 5-15% of all MS patients have a PPMS disease course. Genetic variance is a propos...

  12. Information Loss from Technological Progress (United States)

    Townsend, P. D.


    Progress in electronics and optics offers faster computers, and rapid communication via the internet that is matched by ever larger and evolving storage systems. Instinctively one assumes that this must be totally beneficial. However advances in software and storage media are progressing in ways which are frequently incompatible with earlier systems and the economics and commercial pressures rarely guarantee total compatibility with earlier systems. Instead, the industries actively choose to force the users to purchase new systems and software. Thus we are moving forward with new technological variants that may have access to only the most recent systems and we will have lost earlier alternatives. The reality is that increased processing speed and storage capacity are matched by an equally rapid decline in the access and survival lifetime of older information. This pattern is not limited to modern electronic systems but is evident throughout history from writing on stone and clay tablets to papyrus and paper. It is equally evident in image systems from painting, through film, to magnetic tapes and digital cameras. In sound recording we have variously progressed from wax discs to vinyl, magnetic tape and CD formats. In each case the need for better definition and greater capacity has forced the earlier systems into oblivion. Indeed proposed interactive music systems could similarly relegate music CDs to specialist collections. The article will track some of the examples and discuss the consequences as well as noting that this information loss is further compounded by developments in language and changes in cultural views of different societies.

  13. Contributor Form

    Directory of Open Access Journals (Sweden)

    Chief Editor


    to produce preprints or reprints and translate into languages other than English for sale or free distribution; and 4 the right to republish the work in a collection of articles in any other mechanical or electronic format. We give the rights to the corresponding author to make necessary changes as per the request of the journal, do the rest of the correspondence on our behalf and he/she will act as the guarantor for the manuscript on our behalf. All persons who have made substantial contributions to the work reported in the manuscript, but who are not contributors, are named in the Acknowledgment and have given me/us their written permission to be named. If I/we do not include an Acknowledgment that means I/we have not received substantial contributions from non-contributors and no contributor has been omitted.S NoAuthors' NamesContribution (IJCME Guidelines{1 substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; 2 drafting the article or revising it critically for important intellectual content; and 3 final approval of the version to be published. Authors should meet conditions 1, 2, and 3}.SignatureDate                              Note: All the authors are required to sign independently in this form in the sequence given above. In case an author has left the institution/country and whose whereabouts are not known, the senior author may sign on his/her behalf taking the responsibility.No addition/deletion/ or any change in the sequence of the authorship will be permissible at a later stage, without valid reasons and permission of the Editor.If the authorship is contested at any stage, the article will be either returned or will not be processed for publication till the issue is solved.Maximum up to 4 authors for short communication and up to 6 authors for original article.

  14. Contributors Form

    Directory of Open Access Journals (Sweden)

    Chief Editor


    to produce preprints or reprints and translate into languages other than English for sale or free distribution; and 4 the right to republish the work in a collection of articles in any other mechanical or electronic format. We give the rights to the corresponding author to make necessary changes as per the request of the journal, do the rest of the correspondence on our behalf and he/she will act as the guarantor for the manuscript on our behalf. All persons who have made substantial contributions to the work reported in the manuscript, but who are not contributors, are named in the Acknowledgment and have given me/us their written permission to be named. If I/we do not include an Acknowledgment that means I/we have not received substantial contributions from non-contributors and no contributor has been omitted.S NoAuthors' NamesContribution (IJCME Guidelines{1 substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; 2 drafting the article or revising it critically for important intellectual content; and 3 final approval of the version to be published. Authors should meet conditions 1, 2, and 3}.SignatureDate                              Note: All the authors are required to sign independently in this form in the sequence given above. In case an author has left the institution/country and whose whereabouts are not known, the senior author may sign on his/her behalf taking the responsibility.No addition/deletion/ or any change in the sequence of the authorship will be permissible at a later stage, without valid reasons and permission of the Editor.If the authorship is contested at any stage, the article will be either returned or will not be processed for publication till the issue is solved.Maximum up to 4 authors for short communication and up to 6 authors for original article.

  15. [Rapidly developing vertebral eosinophilic granuloma. Apropos of a case]. (United States)

    Laburthe-Tolra, Y; Boutillier, J B; Chome, J


    Radiographs taken at an interval of one week showed collapse of the twelfth dorsal vertebra, very suggestive of an eosinophilic granuloma. A biopsy performed during surgery, because of the rapid progression of the lesion, was able to confirm the diagnosis.

  16. Primary progressive aphasia and apraxia of speech. (United States)

    Jung, Youngsin; Duffy, Joseph R; Josephs, Keith A


    Primary progressive aphasia is a neurodegenerative syndrome characterized by progressive language dysfunction. The majority of primary progressive aphasia cases can be classified into three subtypes: nonfluent/agrammatic, semantic, and logopenic variants. Each variant presents with unique clinical features, and is associated with distinctive underlying pathology and neuroimaging findings. Unlike primary progressive aphasia, apraxia of speech is a disorder that involves inaccurate production of sounds secondary to impaired planning or programming of speech movements. Primary progressive apraxia of speech is a neurodegenerative form of apraxia of speech, and it should be distinguished from primary progressive aphasia given its discrete clinicopathological presentation. Recently, there have been substantial advances in our understanding of these speech and language disorders. The clinical, neuroimaging, and histopathological features of primary progressive aphasia and apraxia of speech are reviewed in this article. The distinctions among these disorders for accurate diagnosis are increasingly important from a prognostic and therapeutic standpoint. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  17. Geothermal progress monitor. Progress report No. 1

    Energy Technology Data Exchange (ETDEWEB)


    Progress is reported on the following: electrical uses, direct-heat uses, drilling activities, leases, geothermal loan guarantee program, general activities, and legal, institutional, and regulatory activites. (MHR)

  18. Technological progress in ultrasonography. (United States)

    Chou, Y H


    Technical progress in ultrasonography (US) is especially rapid now, due to continuing advances in transducer design, signal processing techniques, and Doppler technology. A number of important technical breakthroughs have been made in the past decade. Among these, multidimensional array transducers, harmonic imaging, miniaturized transducers, extended field-of-view imaging, hand-carried ultrasound units, three-dimensional (3-D) US, and ultrasound contrast agents are the most remarkable innovations. Improved spatial and contrast resolution allows delineation of anatomic details and increases diagnostic accuracy and confidence. Miniaturized transducers can be used to image tiny or superficial structures in the human body, and can guide the surgeon to the problem site. Extended field-of-view imaging provides a larger field for demonstration of pathology in certain anatomic locations. Hand-carried US units are used widely in a physician's office or in remote areas, and bring high-quality medical imaging to the bedside. 3-D US has already shown significant benefits in perinatology; with further improvements in real-time imaging technology, 3-D US will emerge as an important adjunct to conventional 2-D US. In conjunction with harmonic imaging technology and Doppler technology, US contrast agents allow more powerful anatomic and functional evaluation of human organs. They may also play important therapeutic roles in the near future. Advancing computer technology is expected to lead to more important break-throughs in the next 5 to 10 years.

  19. Progress in neuromorphic photonics (United States)

    Ferreira de Lima, Thomas; Shastri, Bhavin J.; Tait, Alexander N.; Nahmias, Mitchell A.; Prucnal, Paul R.


    As society's appetite for information continues to grow, so does our need to process this information with increasing speed and versatility. Many believe that the one-size-fits-all solution of digital electronics is becoming a limiting factor in certain areas such as data links, cognitive radio, and ultrafast control. Analog photonic devices have found relatively simple signal processing niches where electronics can no longer provide sufficient speed and reconfigurability. Recently, the landscape for commercially manufacturable photonic chips has been changing rapidly and now promises to achieve economies of scale previously enjoyed solely by microelectronics. By bridging the mathematical prowess of artificial neural networks to the underlying physics of optoelectronic devices, neuromorphic photonics could breach new domains of information processing demanding significant complexity, low cost, and unmatched speed. In this article, we review the progress in neuromorphic photonics, focusing on photonic integrated devices. The challenges and design rules for optoelectronic instantiation of artificial neurons are presented. The proposed photonic architecture revolves around the processing network node composed of two parts: a nonlinear element and a network interface. We then survey excitable lasers in the recent literature as candidates for the nonlinear node and microring-resonator weight banks as the network interface. Finally, we compare metrics between neuromorphic electronics and neuromorphic photonics and discuss potential applications.

  20. Recent Progress in Terahertz Metasurfaces (United States)

    Al-Naib, Ibraheem; Withayachumnankul, Withawat


    In the past decade, the concept of metasurfaces has gradually dominated the field of metamaterials owing to their fascinating optical properties and simple planar geometries. At terahertz frequencies, the concept has been driven further by the availability of advanced micro-fabrication technologies that deliver sub-micron accuracy, well below the terahertz wavelengths. Furthermore, terahertz spectrometers with high dynamic range and amplitude and phase sensitivity provide valuable information for the study of metasurfaces in general. In this paper, we review recent progress in terahertz metasurfaces mainly in the last 5 years. The first part covers nonuniform metasurfaces that perform beamforming in reflection and transmission. In addition, we briefly overview four different methodologies that can be utilized in realizing high-quality-factor metasurfaces. We also describe two recent approaches to tuning the frequency response of terahertz metasurfaces using graphene as an active medium. Finally, we provide a brief summary and outlook for future developments in this rapidly progressing field.

  1. Gastroretentive dosage forms. (United States)

    Moës, A J


    This article begins with a review of gastric emptying, small intestine transit, and colonic transit of drug delivery systems with special attention paid to the different physiological processes involved in stomach emptying and to the cut-off size of nondigestible solids for passage through the gastroduodenal junction during the digestive phase. Then, the proposed means for prolonging the gastric residence time (GRT) of drug delivery systems are reviewed and analyzed with special emphasis on floating (F) dosage forms. The following means are discussed: the use of passage-delaying agents, large single-unit dosage forms, bioadhesive drug delivery systems, "heavy" pellets, and buoyant forms. In the section devoted to bioadhesive forms, the influence of the turnover time of the intestinal mucus gel layer on the performance of mucoadhesive preparations is pointed out to explain the poor results obtained in humans with such peroral products. The use of a specifically designed apparatus for measuring the total force acting vertically on an object immersed in a liquid is presented as a methodology for selecting optimized buoyant formations in vitro. Scintigraphic studies are described in nonfasting human volunteers either in upright or in supine posture, who concurrently were given one optimized F and one nonfloating (NF) hydrophilic matrix capsules of the same size, for three different sizes (small, medium, and large). In upright subjects, the F forms stayed continuously above the gastric contents irrespective of their size, whereas the NF ones sank rapidly after administration and never rose back to the surface thereafter. Consequently, the F forms show prolonged and more reproducible GRTs compared to the NF ones. The significance and extent of this prolongation are the most marked for the small size units (p 0.05). Moreover, there is no significant difference between the mean GRTs of the small, medium, and large F units (p > 0.05). This indirectly confirms that the

  2. Some Less Commonly Used Forms of Grouping (United States)

    Miller, Wilma H.


    This article describes some of the less commonly used forms of grouping; namely, needs groups, interest groups, research groups, tutorial groups, the Joplin Plan, departmentalized teaching, the ungraded primary plan, multigrade and multiage grouping, and the dual progress plan. (Author)

  3. Rapid Prototyping Laboratory (United States)

    Federal Laboratory Consortium — The ARDEC Rapid Prototyping (RP) Laboratory was established in December 1992 to provide low cost RP capabilities to the ARDEC engineering community. The Stratasys,...

  4. Infrared Colloidal Quantum Dots for Photovoltaics: Fundamentals and Recent Progress

    KAUST Repository

    Tang, Jiang


    Colloidal quantum dots (CQDs) are solution-processed semiconductors of interest in low-cost photovoltaics. Tuning of the bandgap of CQD films via the quantum size effect enables customization of solar cells\\' absorption profile to match the sun\\'s broad visible- and infrared-containing spectrum reaching the earth. Here we review recent progress in the realization of low-cost, efficient solar cells based on CQDs. We focus in particular on CQD materials and approaches that provide both infrared and visible-wavelength solar power conversion CQD photovoltaics now exceed 5% solar power conversion efficiency, achieved by the introduction of a new architecture, the depleted-heterojunction CQD solar cell, that jointly maximizes current, voltage, and fill factor. CQD solar cells have also seen major progress in materials processing for stability, recently achieving extended operating lifetimes in an air ambient. We summarize progress both in device operation and also in gaining new insights into materials properties and processing - including new electrical contact materials and deposition techniques, as well as CQD synthesis, surface treatments, film-forming technologies - that underpin these rapid advances. Infrared colloidal quantum dots that absorb most of the solar radiation enable potential efficient and low-cost photovoltaic devices. Careful optimization of quantum dot passivation and device configuration leads to solar cells with AM1.5G efficiency as high as 5.1% Copyright © 2011 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  5. Digitalization in roll forming manufacturing (United States)

    Sedlmaier, A.; Dietl, T.; Ferreira, P.


    Roll formed profiles are used in automotive chassis production as building blocks for the body-in-white. The ability to produce profiles with discontinuous cross sections, both in width and in depth, allows weight savings in the final automotive chassis through the use of load optimized cross sections. This has been the target of the 3D Roll Forming process. A machine concept is presented where a new forming concept for roll formed parts in combination with advanced robotics allowing freely positioned roll forming tooling in 3D space enables the production of complex shapes by roll forming. This is a step forward into the digitalization of roll forming manufacturing by making the process flexible and capable of rapid prototyping and production of small series of parts. Moreover, data collection in a large scale through the control system and integrated sensors lead to an increased understanding of the process and provide the basis to develop self-optimizing roll forming machines, increasing the productivity, quality and predictability of the roll-forming process. The first parts successfully manufactured with this new forming concept are presented.

  6. Progressive familial intrahepatic cholestasis. (United States)

    Srivastava, Anshu


    Progressive familial intrahepatic cholestasis (PFIC) is a group of rare disorders which are caused by defect in bile secretion and present with intrahepatic cholestasis, usually in infancy and childhood. These are autosomal recessive in inheritance. The estimated incidence is about 1 per 50,000 to 1 per 100,000 births, although exact prevalence is not known. These diseases affect both the genders equally and have been reported from all geographical areas. Based on clinical presentation, laboratory findings, liver histology and genetic defect, these are broadly divided into three types-PFIC type 1, PFIC type 2 and PFIC type 3. The defect is in ATP8B1 gene encoding the FIC1 protein, ABCB 11 gene encoding BSEP protein and ABCB4 gene encoding MDR3 protein in PFIC1, 2 and 3 respectively. The basic defect is impaired bile salt secretion in PFIC1/2 whereas in PFIC3, it is reduced biliary phospholipid secretion. The main clinical presentation is in the form of cholestatic jaundice and pruritus. Serum gamma glutamyl transpeptidase (GGT) is normal in patients with PFIC1/2 while it is raised in patients with PFIC3. Treatment includes nutritional support (adequate calories, supplementation of fat soluble vitamins and medium chain triglycerides) and use of medications to relieve pruritus as initial therapy followed by biliary diversion procedures in selected patients. Ultimately liver transplantation is needed in most patients as they develop progressive liver fibrosis, cirrhosis and end stage liver disease. Due to the high risk of developing liver tumors in PFIC2 patients, monitoring is recommended from infancy. Mutation targeted pharmacotherapy, gene therapy and hepatocyte transplantation are being explored as future therapeutic options.

  7. Densified waste form and method for forming (United States)

    Garino, Terry J.; Nenoff, Tina M.; Sava Gallis, Dorina Florentina


    Materials and methods of making densified waste forms for temperature sensitive waste material, such as nuclear waste, formed with low temperature processing using metallic powder that forms the matrix that encapsulates the temperature sensitive waste material. The densified waste form includes a temperature sensitive waste material in a physically densified matrix, the matrix is a compacted metallic powder. The method for forming the densified waste form includes mixing a metallic powder and a temperature sensitive waste material to form a waste form precursor. The waste form precursor is compacted with sufficient pressure to densify the waste precursor and encapsulate the temperature sensitive waste material in a physically densified matrix.

  8. Progression of Liver Disease (United States)

    ... The Progression of Liver Disease Diagnosing Liver Disease – Liver Biopsy and Liver Function Tests Clinical Trials Liver Transplant ... The Progression of Liver Disease Diagnosing Liver Disease: Liver Biopsy and Liver Function Tests Clinical Trials Liver Transplant ...

  9. Progressive Pigmentary Purpura (United States)

    ... Name: Category: Share: Yes No, Keep Private Progressive Pigmentary Purpura Share | Progressive pigmentary purpura (we will call it PPP) is a ... blood "rusts" (turns into hemosiderin) giving the distinct color. A biopsy may be done to confirm the ...

  10. Bimodal magmatism produced by progressively inhibited crustal assimilation. (United States)

    Meade, F C; Troll, V R; Ellam, R M; Freda, C; Font, L; Donaldson, C H; Klonowska, I


    The origin of bimodal (mafic-felsic) rock suites is a fundamental question in volcanology. Here we use major and trace elements, high-resolution Sr, Nd and Pb isotope analyses, experimental petrology and thermodynamic modelling to investigate bimodal magmatism at the iconic Carlingford Igneous Centre, Ireland. We show that early microgranites are the result of extensive assimilation of trace element-enriched partial melts of local metasiltstones into mafic parent magmas. Melting experiments reveal the crust is very fusible, but thermodynamic modelling indicates repeated heating events rapidly lower its melt-production capacity. Granite generation ceased once enriched partial melts could no longer form and subsequent magmatism incorporated less fertile restite compositions only, producing mafic intrusions and a pronounced compositional gap. Considering the frequency of bimodal magma suites in the North Atlantic Igneous Province, and the ubiquity of suitable crustal compositions, we propose 'progressively inhibited crustal assimilation' (PICA) as a major cause of bimodality in continental volcanism.

  11. Bimodal magmatism produced by progressively inhibited crustal assimilation (United States)

    Meade, F. C.; Troll, V. R.; Ellam, R. M.; Freda, C.; Font, L.; Donaldson, C. H.; Klonowska, I.


    The origin of bimodal (mafic-felsic) rock suites is a fundamental question in volcanology. Here we use major and trace elements, high-resolution Sr, Nd and Pb isotope analyses, experimental petrology and thermodynamic modelling to investigate bimodal magmatism at the iconic Carlingford Igneous Centre, Ireland. We show that early microgranites are the result of extensive assimilation of trace element-enriched partial melts of local metasiltstones into mafic parent magmas. Melting experiments reveal the crust is very fusible, but thermodynamic modelling indicates repeated heating events rapidly lower its melt-production capacity. Granite generation ceased once enriched partial melts could no longer form and subsequent magmatism incorporated less fertile restite compositions only, producing mafic intrusions and a pronounced compositional gap. Considering the frequency of bimodal magma suites in the North Atlantic Igneous Province, and the ubiquity of suitable crustal compositions, we propose ‘progressively inhibited crustal assimilation’ (PICA) as a major cause of bimodality in continental volcanism.

  12. Reconstructing Progressive Education (United States)

    Kaplan, Andy


    The work of Colonel Francis W. Parker, the man whom Dewey called "the father of progressive education," provides a starting point for reconstructing the loose ambiguities of progressive education into a coherent social and educational philosophy. Although progressives have claimed their approach is more humane and sensitive to children, we need…

  13. New developments in rapidly solidified magnesium alloys

    Energy Technology Data Exchange (ETDEWEB)

    Das, S.K. [Allied-Signal, Inc., Morristown, NJ (United States); Chang, C.F. [Allied-Signal, Inc., Morristown, NJ (United States); Raybould, D. [Allied-Signal, Inc., Morristown, NJ (United States); King, J.F. [Magnesium Elektron Ltd., Manchester (United Kingdom); Thistlethwaite, S. [Magnesium Elektron Ltd., Manchester (United Kingdom)


    In the present paper, we will examine the new developments in the rapidly solidified Mg-Al-Zn-Nd (EA55RS) alloy. We shall first briefly review the process scale-up currently employed for producing rapidly solidified magnesium alloys in large quantities, and then discuss the effect of billet size and processing parameters on the mechanical properties of various mill product forms such as extrusions and sheets. The superplastic behavior of EA55RS extrusions and rolled sheets are also discussed. Finally, some results on magnesium metal-matrix composites using rapidly solidified EA55RS matrix powders and SiC particulates are presented. (orig.)

  14. Rapidly Developing Toxic Epidermal Necrolysis

    Directory of Open Access Journals (Sweden)

    Viktoria Oline Barrios Poulsen


    Full Text Available Severe cutaneous reactions with potentially fatal outcomes can have many different causes. The Stevens-Johnson syndrome (SJS and toxic epidermal necrolysis (TEN are rare. They are characterized by a low incidence but high mortality, and drugs are most commonly implicated. Urgent active therapy is required. Prompt recognition and withdrawal of suspect drug and rapid intervention can result in favourable outcome. No further international guidelines for treatment exist, and much of the treatment relies on old or experimental concepts with no scientific evidence. We report on a 54-year-old man experiencing rapidly developing drug-induced severe TEN and presented multiorgan failure involving the respiratory and circulatory system, coagulopathy, and renal insufficiency. Detachment counted 30% of total body surface area (TBSA. SCORTEN = 5, indicating a mortality rate >90%. The patient was sedated and mechanically ventilated, supported with fluids and inotropes to maintain a stable circulation. Component therapy was guided by thromboelastography (TEG. The patient received plasmapheresis, and shock reversal treatment was initiated. He was transferred to a specialized intensive care burn unit within 24 hours from admittance. The initial care was continued, and hemodialysis was started. Pulmonary, circulatory, and renal sequelae resolved with intensive care, and re-epithelialization progressed slowly. The patient was discharged home on hospital day 19.

  15. Predicting periodontitis progression? (United States)

    Ferraiolo, Debra M


    Cochrane Library, Ovid, Medline, Embase and LILACS were searched using no language restrictions and included information up to July 2014. Bibliographic references of included articles and related review articles were hand searched. On-line hand searching of recent issues of key periodontal journals was performed (Journal of Clinical Periodontology, Journal of Dental Research, Journal of Periodontal Research, Journal of Periodontology, Oral Health and Preventive Dentistry). Prospective and retrospective cohort studies were used for answering the question of prediction since there were no randomised controlled trials on this topic. Risk of bias was assessed using the validated Newcastle-Ottawa quality assessment scale for non-randomised studies. Cross-sectional studies were included in the summary of currently reported risk assessment tools but not for risk of progression of disease, due to the inability to properly assess bias in these types of studies. Titles and abstracts were scanned by two reviewers independently.Full reports were obtained for those articles meeting inclusion criteria or those with insufficient information in the title to make a decision. Any published risk assessment tool was considered. The tool was defined to include any composite measure of patient-level risk directed towards determining the probability for further disease progression in adults with periodontitis. Periodontitis was defined to include both chronic and aggressive forms in the adult population. Outcomes included changes in attachment levels and/or deepening of periodontal pockets in millimeters in study populations undergoing supportive periodontal therapy. Data extraction was performed independently and in collaboration by two reviewers; completed evidence tables were reviewed by three reviewers. Studies were each given a descriptive summary to assess the quantity of data as well as further assessment of study variations within study characteristics. This also allowed for

  16. Rapid shallow breathing (United States)

    ... the smallest air passages of the lungs in children ( bronchiolitis ) Pneumonia or other lung infection Transient tachypnea of the newborn Anxiety and panic Other serious lung disease Home Care Rapid, shallow breathing should not be treated at home. It is ...

  17. Rapid Strep Test (United States)

    ... worse than normal. Your first thoughts turn to strep throat. A rapid strep test in your doctor’s office ... your suspicions.Viruses cause most sore throats. However, strep throat is an infection caused by the Group A ...

  18. Fear of progression. (United States)

    Herschbach, Peter; Dinkel, Andreas


    Fear of progression (or fear of recurrence) is an appropriate, rational response to the real threat of cancer and cancer treatments. However, elevated levels of fear of progression can become dysfunctional, affecting well-being, quality of life, and social functioning. Research has shown that fear of progression is one of the most frequent distress symptoms of patients with cancer and with other chronic diseases. As a clear consensus concerning clinically relevant states of fear of progression is currently lacking, it is difficult to provide a valid estimate of the rate of cancer patients who clearly suffer from fear of progression. However, recent systematic reviews suggest that probably 50 % of cancer patients experience moderate to severe fear of progression. Furthermore, many patients express unmet needs in dealing with the fear of cancer spreading. These results underline the necessity to provide effective psychological treatments for clinical levels of fear of progression. A few psychosocial interventions for treating fear of progression have been developed so far. Our own, targeted intervention study showed that dysfunctional fear of progression can be effectively treated with a brief group therapy.

  19. Progress in biomaterials

    National Research Council Canada - National Science Library


    "Progress in Biomaterials is a multidisciplinary, English-language publication of original contributions and reviews concerning studies of the preparation, performance and evaluation of biomaterial...

  20. Solar lease grant program. Technical progress report

    Energy Technology Data Exchange (ETDEWEB)


    Progress on a lease program for the installation of a solar water heater with no installation charge is reported. Information on the announcement of the program, the selection of participants, the contractural agreement, progress on installation of equipment, monitoring, and evaluation is summarized. The status of the budget concerned with the program is announced. Forms used for applications for the program and an announcement from Resource Alternatives for Cilco customers are presented.

  1. Slowly progressive aphasia associated with surface dyslexia. (United States)

    Chiacchio, L; Grossi, D; Stanzione, M; Trojano, L


    We report an Italian patient affected by slowly progressive aphasia (SPA) lasting since four years when he first came to our observation. During the successive four years, we documented a progressive language decline resembling transcortical sensory aphasia, associated with a reading disorder corresponding to surface dyslexia, a form extremely rare in patients with native transparent language. His performance at standard intelligence tasks remained in the normal range, without any variation. CT scan showed left temporal atrophy. We emphasize the heterogeneity of the syndrome of SPA and suggest that it can represent one of the pictures of focal cortical degenerative disease, with variable onset, progression, and evolution.

  2. Preparation, Evaluation and Optimization of Rapidly Disintegrating ...

    African Journals Online (AJOL)

    The unit dose content uniformity, dissolution rate, assay values and other tablet characteristics evaluated were all within the acceptable limits. Thus, it was possible to formulate an RDT of artemether-lumefantrine FDC using CPVP as a disintegrant and camphor as a pore forming agent. Keywords: rapidly disintegrating tablet ...

  3. Rapid Cycling and Its Treatment (United States)

    ... Announcements Public Service Announcements Partnering with DBSA Rapid Cycling and its Treatment What is bipolar disorder? Bipolar ... to Depression and Manic Depression . What is rapid cycling? Rapid cycling is defined as four or more ...



    Saxena Vaibhav; Khinchi Mahaveer Pr; Gupta M.K.; Agarwal Dilip; Sharma Natasha


    Orally disintegrating tablets (ODTs) have emerged as one of the popular and widely accepted dosage forms, especially for the paediatric and geriatric patients. In recent decades, a variety of pharmaceutical research has been conducted to develop new dosage forms. Among the dosage forms developed to facilitate ease of medication, the rapid disintegrating tablet (RDT) is one of the most widely employed commercial products.1 As our society is becoming increasingly aged, the development of Fast-...

  5. Progressive osseous heteroplasia

    African Journals Online (AJOL)

    Progressive osseous heteroplasia is a rare genetic disorder characterized by cu- taneous ossification during infancy and progressive ossification of subcutane- ous and deep connective tissue including muscle and fascia during childhood. It is at the severe end of a spectrum of Guanine Nucleotide-binding protein,.

  6. White matter lesion progression

    DEFF Research Database (Denmark)

    Hofer, Edith; Cavalieri, Margherita; Bis, Joshua C


    BACKGROUND AND PURPOSE: White matter lesion (WML) progression on magnetic resonance imaging is related to cognitive decline and stroke, but its determinants besides baseline WML burden are largely unknown. Here, we estimated heritability of WML progression, and sought common genetic variants asso...

  7. Rapid manufacturing for microfluidics

    CSIR Research Space (South Africa)

    Land, K


    Full Text Available . Microfluidics is at the forefront of developing solutions for drug discovery, diagnostics (from glucose tests to malaria and TB testing) and environmental diagnostics (E-coli monitoring of drinking water). In order to quickly implement new designs, a rapid...

  8. Rapid Prototyping in PVS (United States)

    Munoz, Cesar A.; Butler, Ricky (Technical Monitor)


    PVSio is a conservative extension to the PVS prelude library that provides basic input/output capabilities to the PVS ground evaluator. It supports rapid prototyping in PVS by enhancing the specification language with built-in constructs for string manipulation, floating point arithmetic, and input/output operations.

  9. Rapid Prototyping Reconsidered (United States)

    Desrosier, James


    Continuing educators need additional strategies for developing new programming that can both reduce the time to market and lower the cost of development. Rapid prototyping, a time-compression technique adapted from the high technology industry, represents one such strategy that merits renewed evaluation. Although in higher education rapid…

  10. Pharmacokinetics and pharmacodynamics of MD1003 (high-dose biotin) in the treatment of progressive multiple sclerosis. (United States)

    Peyro Saint Paul, Laure; Debruyne, Danièle; Bernard, Delphine; Mock, Donald M; Defer, Gilles L


    Multiple sclerosis (MS) is a chronic, potentially highly disabling neurological disorder. No disease-modifying treatments are approved in the progressive and not active forms of the disease. High doses of biotin were tested in an open-label pilot study involving 23 patients with progressive MS and reported positive results. A randomized, double-blind, placebo-controlled trial in 154 progressive MS patients confirmed the beneficial effect of MD1003 (high-dose biotin) on reversing or stabilizing disability progression, with a good safety profile. It is proposed that MD1003 in progressive MS 1) increases energy production in demyelinated axons and/or 2) enhances myelin synthesis in oligodendrocytes. Biotin is highly bioavailable; absorption and excretion are rapid. The major route of elimination is urinary excretion. A high oral dose of biotin seems generally well tolerated but a few important safety concerns were identified: 1) teratogenicity in one species and 2) interference with some biotin-based laboratory immunoassays. The animal toxicity data are limited at such high doses. Further preclinical studies would be useful to address the mechanism of action of MD1003. Assessment of clinical benefit duration in responders will be also very important to set. Results of randomized, placebo-controlled trial are reassuring and provide hope for the treatment of progressive MS.

  11. Pectus excavatum, pectus carinatum and other forms of thoracic deformities

    Directory of Open Access Journals (Sweden)

    Saxena Amulya


    Full Text Available This review article covers the spectrum of congenital thoracic wall deformities along with their historical background. Willital′s classification divides these deformities into 11 types - funnel chest (4 types, pigeon chest (4 types, and combination of funnel and pigeon chest, chest wall aplasia and cleft sternum. Records of patients at our center comprised 90% depression deformities, 6% protrusion deformities, 3% combined depression-protrusion deformities and 1% other forms. Mild forms of abnormalities warrant the wait- and-watch approach during the first 4-5 years. The deformities manifest primarily during the pubertal spurt often with rapid progression with subjective complaints like dyspnea, cardiac dysthesia, limited work performance and secondary changes. Operative correction in young adults is more favorable in mild cases. The Willital technique has been the standard technique for the correction of pectus excavatum, pectus carinatum and other combined forms of deformities at our center with excellent long term results. The Nuss procedure and the Pectus Less Invasive Extrapleural Repair (PLIER technique for pectus excavatum and pectus carinatum have also been described in this article. Surgical correction for Poland′s syndrome is reserved for patients with severe aplasia of the ribs with major depression deformity. Sternal defects including various types of ectopia cordis are discussed. Even after surgical correction, there is significant reduction in the total capacity and inspiratory vital capacity of the lungs, probably a result of the decreased compliance of the chest wall. However, the efficiency of breathing at maximal exercise improves significantly after operation.

  12. Progress in research on Tourette syndrome (United States)

    Black, Kevin J.; Jankovic, Joseph; Hershey, Tamara; McNaught, Kevin St. P.; Mink, Jonathan W.; Walkup, John


    Tourette syndrome (TS) is a heritable neuropsychiatric disorder commonly complicated by obsessions and compulsions, but defined by frequent unwanted movements (motor tics) and vocalizations (phonic tics) that develop in childhood or adolescence. In recent years, research on TS has progressed rapidly on several fronts. Inspired by the Fifth International Scientific Symposium on Tourette Syndrome, the articles in this special issue review advances in the phenomenology, epidemiology, genetics, pathophysiology, and treatment of TS. PMID:25436182

  13. On good ETOL forms

    DEFF Research Database (Denmark)

    Skyum, Sven


    This paper continues the study of ETOL forms and good EOL forms done by Maurer, Salomaa and Wood. It is proven that binary very complete ETOL forms exist, good synchronized ETOL forms exist and that no propagating or synchronized ETOL form can be very complete.......This paper continues the study of ETOL forms and good EOL forms done by Maurer, Salomaa and Wood. It is proven that binary very complete ETOL forms exist, good synchronized ETOL forms exist and that no propagating or synchronized ETOL form can be very complete....

  14. Physicians’ progress notes

    DEFF Research Database (Denmark)

    Bansler, Jørgen P.; Havn, Erling C.; Mønsted, Troels


    care, they have not dealt specifically with the role, structure, and content of the progress notes. As a consequence, CSCW research has not yet taken fully into account the fact that progress notes are coordinative artifacts of a rather special kind, an open-ended chain of prose texts, written...... sequentially by cooperating physicians for their own use as well as for that of their colleagues. We argue that progress notes are the core of the medical record, in that they marshal and summarize the overwhelming amount of data that is available in the modern hospital environment, and that their narrative...

  15. Rapid manufacturing facilitated customisation


    Tuck, Christopher John; Hague, Richard; Ruffo, Massimiliano; Ransley, Michelle; Adams, Paul Russell


    Abstract This paper describes the production of body-fitting customised seat profiles utilising the following digital methods: three dimensional laser scanning, reverse engineering and Rapid Manufacturing (RM). The seat profiles have been manufactured in order to influence the comfort characteristics of an existing ejector seat manufactured by Martin Baker Aircraft Ltd. The seat, known as Navy Aircrew Common Ejection Seat (NACES), was originally designed with a generic profile. ...

  16. Rapid Detection of Pathogens

    Energy Technology Data Exchange (ETDEWEB)

    David Perlin


    Pathogen identification is a crucial first defense against bioterrorism. A major emphasis of our national biodefense strategy is to establish fast, accurate and sensitive assays for diagnosis of infectious diseases agents. Such assays will ensure early and appropriate treatment of infected patients. Rapid diagnostics can also support infection control measures, which monitor and limit the spread of infectious diseases agents. Many select agents are highly transmissible in the early stages of disease, and it is critical to identify infected patients and limit the risk to the remainder of the population and to stem potential panic in the general population. Nucleic acid-based molecular approaches for identification overcome many of the deficiencies associated with conventional culture methods by exploiting both large- and small-scale genomic differences between organisms. PCR-based amplification of highly conserved ribosomal RNA (rRNA) genes, intergenic sequences, and specific toxin genes is currently the most reliable approach for bacterial, fungal and many viral pathogenic agents. When combined with fluorescence-based oligonucleotide detection systems, this approach provides real-time, quantitative, high fidelity analysis capable of single nucleotide allelic discrimination (4). These probe systems offer rapid turn around time (<2 h) and are suitable for high throughput, automated multiplex operations that are critical for clinical diagnostic laboratories. In this pilot program, we have used molecular beacon technology invented at the Public health Research Institute to develop a new generation of molecular probes to rapidly detect important agents of infectious diseases. We have also developed protocols to rapidly extract nucleic acids from a variety of clinical specimen including and blood and tissue to for detection in the molecular assays. This work represented a cooperative research development program between the Kramer-Tyagi/Perlin labs on probe development

  17. Rapid Airplane Parametric Input Design(RAPID) (United States)

    Smith, Robert E.; Bloor, Malcolm I. G.; Wilson, Michael J.; Thomas, Almuttil M.


    An efficient methodology is presented for defining a class of airplane configurations. Inclusive in this definition are surface grids, volume grids, and grid sensitivity. A small set of design parameters and grid control parameters govern the process. The general airplane configuration has wing, fuselage, vertical tail, horizontal tail, and canard components. The wing, tail, and canard components are manifested by solving a fourth-order partial differential equation subject to Dirichlet and Neumann boundary conditions. The design variables are incorporated into the boundary conditions, and the solution is expressed as a Fourier series. The fuselage has circular cross section, and the radius is an algebraic function of four design parameters and an independent computational variable. Volume grids are obtained through an application of the Control Point Form method. Grid sensitivity is obtained by applying the automatic differentiation precompiler ADIFOR to software for the grid generation. The computed surface grids, volume grids, and sensitivity derivatives are suitable for a wide range of Computational Fluid Dynamics simulation and configuration optimizations.

  18. Tiber Personal Rapid Transit

    Directory of Open Access Journals (Sweden)

    Diego Carlo D'agostino


    Full Text Available The project “Tiber Personal Rapid Transit” have been presented by the author at the Rome City Vision Competition1 2010, an ideas competition, which challenges architects, engineers, designers, students and creatives individuals to develop visionary urban proposals with the intention of stimulating and supporting the contemporary city, in this case Rome. The Tiber PRT proposal tries to answer the competition questions with the definition of a provocative idea: a Personal Rapid transit System on the Tiber river banks. The project is located in the central section of the Tiber river and aims at the renewal of the river banks with the insertion of a Personal Rapid Transit infrastructure. The project area include the riverbank of Tiber from Rome Transtevere RFI station to Piazza del Popolo, an area where main touristic and leisure attractions are located. The intervention area is actually no used by the city users and residents and constitute itself a strong barrier in the heart of the historic city.

  19. Progressive Finland sees progress with nuclear projects

    Energy Technology Data Exchange (ETDEWEB)

    Dalton, David [NucNet, Brussels (Belgium)


    The Finnish Hanhikivi-1 reactor project is firmly on track and a licence has been granted for construction of a final disposal facility for spent nuclear fuel - the first final repository in the world to enter the construction phase. Significant progress has been made with plans for Finland to build its sixth nuclear reactor unit at Hanhikivi. Fennovoima's licensing manager Janne Liuko said the company expects to receive the construction licence for the Generation III+ Hanhikivi-1 plant in late 2017. The application was submitted to the Finnish Ministry of Employment and the Economy in June 2015.

  20. Modular Forms and Weierstrass Mock Modular Forms

    Directory of Open Access Journals (Sweden)

    Amanda Clemm


    Full Text Available Alfes, Griffin, Ono, and Rolen have shown that the harmonic Maass forms arising from Weierstrass ζ-functions associated to modular elliptic curves “encode” the vanishing and nonvanishing for central values and derivatives of twisted Hasse-Weil L-functions for elliptic curves. Previously, Martin and Ono proved that there are exactly five weight 2 newforms with complex multiplication that are eta-quotients. In this paper, we construct a canonical harmonic Maass form for these five curves with complex multiplication. The holomorphic part of this harmonic Maass form arises from the Weierstrass ζ-function and is referred to as the Weierstrass mock modular form. We prove that the Weierstrass mock modular form for these five curves is itself an eta-quotient or a twist of one. Using this construction, we also obtain p-adic formulas for the corresponding weight 2 newform using Atkin’s U-operator.

  1. Analysis and documentation of progression of Fuchs corneal dystrophy with retroillumination photography. (United States)

    Gottsch, John D; Sundin, Olof H; Rencs, Erik V; Emmert, David G; Stark, Walter J; Cheng, Clement J; Schmidt, Gregory W


    Fuchs corneal dystrophy (FCD) is a degenerative disorder of the cornea that is characterized by the progressive accumulation of guttae, which are small excrescences of Descemet's membrane. We describe a method for documenting the location and number of guttae, and ask whether disease progression can be observed during relatively short periods. Patients with FCD were imaged by standard retroillumination photography with a slit lamp. Scanned photographs were analyzed by using NIH ImageJ software to determine the number of individual guttae and areas of confluence. In 4 FCD patients, photographs taken 23 to 30 months apart revealed that, once formed, individual guttae and their relative positions persisted during this period. Very few guttae disappeared, and the emergence of many new guttae was observed. Determination of the area with confluent guttae was used to quantify disease stage. Computer-assisted analysis of retroillumination photographs is proposed as an effective way to document the number and distribution of individual guttae. Although the disease typically progresses slowly during decades, we have been able to detect the formation of new guttae within only 2 years. This rapid assessment of disease progression could be used to measure phenotypic differences between genetic subtypes of FCD. It also could provide important baseline information and methodology for clinical trials of therapeutic options, should these become available.

  2. Antimicrobial and Antioxidant Activity of Chitosan/Hydroxypropyl Methylcellulose Film-Forming Hydrosols Hydrolyzed by Cellulase

    Directory of Open Access Journals (Sweden)

    Anna Zimoch-Korzycka


    Full Text Available The aim of this study was to evaluate the impact of cellulase (C on the biological activity of chitosan/hydroxypropyl methylcellulose (CH/HPMC film-forming hydrosols. The hydrolytic activity of cellulase in two concentrations (0.05% and 0.1% was verified by determination of the progress of polysaccharide hydrolysis, based on viscosity measurement and reducing sugar-ends assay. The 2,2-diphenyl-1-picrylhydrazyl (DPPH free radical scavenging effect, the ferric reducing antioxidant power (FRAP, and microbial reduction of Pseudomonas fluorescens, Yersinia enterocolitica, Bacillus cereus, and Staphylococcus aureus were studied. During the first 3 h of reaction, relative reducing sugar concentration increased progressively, and viscosity decreased rapidly. With increasing amount of enzyme from 0.05% to 0.1%, the reducing sugar concentration increased, and the viscosity decreased significantly. The scavenging effect of film-forming solutions was improved from 7.6% at time 0 and without enzyme to 52.1% for 0.1% cellulase after 20 h of reaction. A significant effect of cellulase addition and reaction time on antioxidant power of the tested film-forming solutions was also reported. Film-forming hydrosols with cellulase exhibited a bacteriostatic effect on all tested bacteria, causing a total reduction.

  3. [Progressive visual agnosia]. (United States)

    Sugimoto, Azusa; Futamura, Akinori; Kawamura, Mitsuru


    Progressive visual agnosia was discovered in the 20th century following the discovery of classical non-progressive visual agnosia. In contrast to the classical type, which is caused by cerebral vascular disease or traumatic injury, progressive visual agnosia is a symptom of neurological degeneration. The condition of progressive visual loss, including visual agnosia, and posterior cerebral atrophy was named posterior cortical atrophy (PCA) by Benson et al. (1988). Progressive visual agnosia is also observed in semantic dementia (SD) and other degenerative diseases, but there is a difference in the subtype of visual agnosia associated with these diseases. Lissauer (1890) classified visual agnosia into apperceptive and associative types, and it in most cases, PCA is associated with the apperceptive type. However, SD patients exhibit symptoms of associative visual agnosia before changing to those of semantic memory disorder. Insights into progressive visual agnosia have helped us understand the visual system and discover how we "perceive" the outer world neuronally, with regard to consciousness. Although PCA is a type of atypical dementia, its diagnosis is important to enable patients to live better lives with appropriate functional support.

  4. A scoping review of rapid review methods. (United States)

    Tricco, Andrea C; Antony, Jesmin; Zarin, Wasifa; Strifler, Lisa; Ghassemi, Marco; Ivory, John; Perrier, Laure; Hutton, Brian; Moher, David; Straus, Sharon E


    Rapid reviews are a form of knowledge synthesis in which components of the systematic review process are simplified or omitted to produce information in a timely manner. Although numerous centers are conducting rapid reviews internationally, few studies have examined the methodological characteristics of rapid reviews. We aimed to examine articles, books, and reports that evaluated, compared, used or described rapid reviews or methods through a scoping review. MEDLINE, EMBASE, the Cochrane Library, internet websites of rapid review producers, and reference lists were searched to identify articles for inclusion. Two reviewers independently screened literature search results and abstracted data from included studies. Descriptive analysis was conducted. We included 100 articles plus one companion report that were published between 1997 and 2013. The studies were categorized as 84 application papers, seven development papers, six impact papers, and four comparison papers (one was included in two categories). The rapid reviews were conducted between 1 and 12 months, predominantly in Europe (58 %) and North America (20 %). The included studies failed to report 6 % to 73 % of the specific systematic review steps examined. Fifty unique rapid review methods were identified; 16 methods occurred more than once. Streamlined methods that were used in the 82 rapid reviews included limiting the literature search to published literature (24 %) or one database (2 %), limiting inclusion criteria by date (68 %) or language (49 %), having one person screen and another verify or screen excluded studies (6 %), having one person abstract data and another verify (23 %), not conducting risk of bias/quality appraisal (7 %) or having only one reviewer conduct the quality appraisal (7 %), and presenting results as a narrative summary (78 %). Four case studies were identified that compared the results of rapid reviews to systematic reviews. Three studies found that the conclusions between

  5. Stability of tubulin polymers formed with dideoxyguanosine nucleotides in the presence and absence of microtubule-associated proteins. (United States)

    Hamel, E; del Campo, A A; Lin, C M


    We have examined the effects of dilution, Ca2+, reduced temperature, and triphosphate depletion on microtubules formed from purified tubulin, heat-treated microtubule-associated proteins (MAPs), and either GTP, 2',3'-dideoxyguanosine 5'-diphosphate (ddGDP), or 2',3'-dideoxyguanosine 5'-triphosphate (ddGTP). The stability of the polymer formed with tubulin plus ddGTP without MAPs was also examined. In all cases dilution resulted in rapid depolymerization of polymer until a new turbidity plateau was established. These experiments yielded estimates of the critical concentration of tubulin of 0.09 mg/ml with GTP plus MAPs, 0.04 mg/ml with either ddGDP or ddGTP plus MAPs, and 0.07 mg/ml with ddGTP minus MAPs. Addition of CaCl2 to polymer resulted in depolymerization of microtubules formed with either GTP or ddGDP plus MAPs; but both with and without MAPs the polymer formed with ddGTP was stable to Ca2+. The polymer formed with ddGTP minus MAPs was the most cold-labile, major depolymerization occurring at 25 degrees C. With MAPs, microtubules were progressively less cold-labile when formed with GTP, ddGDP, or ddGTP. Depolymerization with GTP was virtually complete at 15 degrees C, with ddGDP at 5 degrees C, and with ddGTP at 0 degrees C. Rapid triphosphate depletion was achieved with phosphofructokinase. GTP-formed tubules were rapidly and completely depolymerized at all GTP concentrations after the enzyme was added to the reaction mixture. Both with and without MAPs polymer formed with ddGTP was progressively more stable upon enzyme addition the higher the initial ddGTP concentration. At specific ddGTP concentrations, however, less depolymerization was observed following enzyme addition if MAPs were present. Microtubules formed with ddGDP plus MAPs were unaffected by phosphofructokinase addition. This comparison of the properties of microtubules formed with MAPs and either ddGDP or ddGTP demonstrates that their stability is enhanced rather than reduced following

  6. Genetic Association Analysis of Drusen Progression

    NARCIS (Netherlands)

    Hoffman, J.D.; Grinsven, M.J.J.P. van; Li, C.; Brantley, M., Jr.; McGrath, J.; Agarwal, A.; Scott, W.K.; Schwartz, S.G.; Kovach, J.; Pericak-Vance, M.; Sanchez, C.I.; Haines, J.L.


    PURPOSE: Age-related macular degeneration is a common form of vision loss affecting older adults. The etiology of AMD is multifactorial and is influenced by environmental and genetic risk factors. In this study, we examine how 19 common risk variants contribute to drusen progression, a hallmark of

  7. Laser ablation/ionization characterization of solids: Second interim progress report of the strategic environmental research development program

    Energy Technology Data Exchange (ETDEWEB)

    Hess, W.P.; Bushaw, B.A.; McCarthy, M.I.; Campbell, J.A.; Colson, S.D. [Pacific Northwest National Lab., Richland, WA (United States); Dickinson, J.T. [Washington State Univ., Pullman, WA (United States). Dept. of Physics


    The Department of Energy is undertaking the enormous task of remediating defense wastes and environmental insults which have occurred over 50 years of nuclear weapons production. It is abundantly clear that significant technology advances are needed to characterize, process, and store highly radioactive waste and to remediate contaminated zones. In addition to the processing and waste form issues, analytical technologies needed for the characterization of solids, and for monitoring storage tanks and contaminated sites do not exist or are currently expensive labor-intensive tasks. This report describes progress in developing sensitive, rapid, and widely applicable laser-based mass spectrometry techniques for analysis of mixed chemical wastes and contaminated soils.

  8. Comparison of spectacle classical progressive and office progressive lenses. (United States)

    Kozlík, Marek; Knollová, Libuse Nováková


    This paper elaborates on analysis of progressive spectacle lenses, to correct presbyopia, which are nowadays offered at the market. The paper describes different types of progressive lenses, their parameters, length and width of their progressive segments. It also describes degressive spectacles lenses--progressive lenses on middle and near distance. The main part of the paper is a comparison of functional differences among different types of progressive spectacles lenses. The paper also addresses correctness of choice of progressive lenses for different works and professions. Lastly, it elaborates on differences of centration of different types of progressive lenses and parameters for correct choice of glasses frame for progressive spectacles lenses.

  9. Rapidly variable relatvistic absorption (United States)

    Parker, M.; Pinto, C.; Fabian, A.; Lohfink, A.; Buisson, D.; Alston, W.; Jiang, J.


    I will present results from the 1.5Ms XMM-Newton observing campaign on the most X-ray variable AGN, IRAS 13224-3809. We find a series of nine absorption lines with a velocity of 0.24c from an ultra-fast outflow. For the first time, we are able to see extremely rapid variability of the UFO features, and can link this to the X-ray variability from the inner accretion disk. We find a clear flux dependence of the outflow features, suggesting that the wind is ionized by increasing X-ray emission.

  10. Right-Rapid-Rough (United States)

    Lawrence, Craig


    IDEO (pronounced 'eye-dee-oh') is an international design, engineering, and innovation firm that has developed thousands of products and services for clients across a wide range of industries. Its process and culture attracted the attention of academics, businesses, and journalists around the world, and are the subject of a bestselling book, The Art of Innovation by Tom Kelley. One of the keys to IDEO's success is its use of prototyping as a tool for rapid innovation. This story covers some of IDEO's projects, and gives reasons for why they were successful.

  11. Rapid solidification of Nb-base alloys (United States)

    Gokhale, A. B.; Javed, K. R.; Abbaschian, G. J.; Lewis, R. E.


    New Nb-base alloys are of interest for aerospace structural applications at high temperatures, viz, 800 to 1650 C. Fundamental information regarding the effects of rapid solidification in achieving greatly refined microstructures, extended solid solubility, suppression of embrittling equilibrium phases, and formation of new phases is desired in a number of Nb-X alloys. The microstructures and selected properties of Nb-Si and other Nb-base alloys are presented for materials both rapidly quenched from the equilibrium liquidus and rapidly solidified following deep supercooling. Electromagnetic levitation was used to achieve melting and supercooling in a containerless inert gas environment. A variety of solidification conditions were employed including splatting or drop casting of supercooled samples. The morphology and composition of phases formed are discussed in terms of both solidification history and bulk composition.


    Energy Technology Data Exchange (ETDEWEB)

    Vienna, John D.; Todd, Terry A.; Peterson, Mary E.


    This Separations and Waste Forms Campaign Implementation Plan provides summary level detail describing how the Campaign will achieve the objectives set-forth by the Fuel Cycle Reasearch and Development (FCRD) Program. This implementation plan will be maintained as a living document and will be updated as needed in response to changes or progress in separations and waste forms research and the FCRD Program priorities.

  13. Rapid mineralocorticoid receptor trafficking. (United States)

    Gekle, M; Bretschneider, M; Meinel, S; Ruhs, S; Grossmann, C


    The mineralocorticoid receptor (MR) is a ligand-dependent transcription factor that physiologically regulates water-electrolyte homeostasis and controls blood pressure. The MR can also elicit inflammatory and remodeling processes in the cardiovascular system and the kidneys, which require the presence of additional pathological factors like for example nitrosative stress. However, the underlying molecular mechanism(s) for pathophysiological MR effects remain(s) elusive. The inactive MR is located in the cytosol associated with chaperone molecules including HSP90. After ligand binding, the MR monomer rapidly translocates into the nucleus while still being associated to HSP90 and after dissociation from HSP90 binds to hormone-response-elements called glucocorticoid response elements (GREs) as a dimer. There are indications that rapid MR trafficking is modulated in the presence of high salt, oxidative or nitrosative stress, hypothetically by induction or posttranslational modifications. Additionally, glucocorticoids and the enzyme 11beta hydroxysteroid dehydrogenase may also influence MR activation. Because MR trafficking and its modulation by micro-milieu factors influence MR cellular localization, it is not only relevant for genomic but also for nongenomic MR effects. Copyright © 2013 Elsevier Inc. All rights reserved.

  14. Rapid response manufacturing (RRM)

    Energy Technology Data Exchange (ETDEWEB)

    Cain, W.D. [Lockheed Martin Energy Systems, Inc., Oak Ridge, TN (United States); Waddell, W.L. [National Centers for Manufacturing Sciences, Ann Arbor, MI (United States)


    US industry is fighting to maintain its competitive edge in the global market place. Today markets fluctuate rapidly. Companies, to survive, have to be able to respond with quick-to-market, improved, high quality, cost efficient products. The way products are developed and brought to market can be improved and made more efficient through the proper incorporation of emerging technologies. The RRM project was established to leverage the expertise and resources of US private industries and federal agencies to develop, integrate, and deploy new technologies that meet critical needs for effective product realization. The RRM program addressed a needed change in the US Manufacturing infrastructure that will ensure US competitiveness in world market typified by mass customization. This project provided the effort needed to define, develop and establish a customizable infrastructure for rapid response product development design and manufacturing. A major project achievement was the development of a broad-based framework for automating and integrating the product and process design and manufacturing activities involved with machined parts. This was accomplished by coordinating and extending the application of feature-based product modeling, knowledge-based systems, integrated data management, and direct manufacturing technologies in a cooperative integrated computing environment. Key technological advancements include a product model that integrates product and process data in a consistent, minimally redundant manner, an advanced computer-aided engineering environment, knowledge-based software aids for design and process planning, and new production technologies to make products directly from design application software.

  15. Manufacturing processes 4 forming

    CERN Document Server

    Klocke, Fritz


    This book provides essential information on metal forming, utilizing a practical distinction between bulk and sheet metal forming. In the field of bulk forming, it examines processes of cold, warm and hot bulk forming, as well as rolling and a new addition, the process of thixoforming. As for the field of sheet metal working, on the one hand it deals with sheet metal forming processes (deep drawing, flange forming, stretch drawing, metal spinning and bending). In terms of special processes, the chapters on internal high-pressure forming and high rate forming have been revised and refined. On the other, the book elucidates and presents the state of the art in sheet metal separation processes (shearing and fineblanking). Furthermore, joining by forming has been added to the new edition as a new chapter describing mechanical methods for joining sheet metals. The new chapter “Basic Principles” addresses both sheet metal and bulk forming, in addition to metal physics, plastomechanics and computational basics; ...

  16. Arachnoiditis ossificans with progressive syringomyelia and spinal arachnoid cyst. (United States)

    Papavlasopoulos, F; Stranjalis, G; Kouyialis, A T; Korfias, S; Sakas, D


    We present a 30-year-old man with progressive spastic paraparesis. Spinal imaging revealed extensive calcification of the thoracic cord and cauda equina arachnoid, an intradural extramedullary cyst and evidence of rapidly progressing syringomyelia. Radiological diagnosis was arachnoiditis ossificans and an attempt at surgical decompression was made because of progressive neurologic deterioration. Due to tenacious adhesion of the calcified plaques to the cord and roots, only cyst drainage was achieved; the patient had no clinical improvement. A literature review revealed only two other cases reported in the literature with co-existence of arachnoiditis ossificans and syringomyelia. In none of the previous cases was there an intradural extramedullary arachnoid cyst, nor did the syrinx progress in such a rapid fashion. An attempt is made to explain possible pathophysiological mechanisms leading to this unusual pathology.

  17. Rapid mortality of Populus tremuloides in southwestern Colorado, USA (United States)

    James J. Worrall; Leanne Egeland; Thomas Eager; Roy A. Mask; Erik W. Johnson; Philip A. Kemp; Wayne D. Shepperd


    Concentrated patches of recent trembling aspen (Populus tremuloides) mortality covered 56,091 ha of Colorado forests in 2006. Mortality has progressed rapidly. Area affected increased 58% between 2005 and 2006 on the Mancos-Dolores Ranger District, San Juan National Forest, where it equaled nearly 10% of the aspen cover type. In four stands that were...

  18. Climate economics in progress 2011; Climate economics in progress 2011

    Energy Technology Data Exchange (ETDEWEB)

    De Perthuis, Christian [Paris-Dauphine University (France); Jouvet, Pierre-Andre [Paris-Ouest University (France); Trotignon, Raphael; Simonet, Gabriela; Boutueil, Virginie [Climate Economics Chair, Paris-Dauphine University (France)


    Climate Economics in Progress offers a global overview of the present status of action on climate change. Drawing on the most recent data, it analyzes the development of carbon markets in Europe and other parts of the world. It also examines the conditions for including major players such as China and new sectors such as agriculture, forestry and transport in the fight against global warming. The book is essential reading for anyone wishing to understand current advances in climate control, which could pave the way for a new form of economic growth. The book brings together a group of researchers whose goal is to make the link between academic research on the economics of climate change and the implementation of operational tools, thereby allowing the climate issue to be integrated into the functioning of the real economy

  19. Chronic progressive external ophthalmoplegia. (United States)

    Lee, Andrew G; Brazis, Paul W


    Chronic progressive external ophthalmoplegia (CPEO) is a descriptive term for a heterogenous group of disorders characterized by chronic, progressive, bilateral, and usually symmetric ocular motility deficit and ptosis. Significant pain, proptosis, or pupil involvement are not features of CPEO and should prompt evaluation for alternative etiologies. Mitochondrial DNA mutations are increasingly being recognized as the etiology for CPEO syndromes. Clinicians should recognize the specific syndromes associated with CPEO, characterized by variable systemic, neurologic, or other findings. Treatment is limited, but newer therapies are being investigated.

  20. Progress in physical chemistry

    CERN Document Server

    Hempelmann, Rolf


    Progress in Physical Chemistry is a collection of recent ""Review Articles"" published in the ""Zeitschrift für Physikalische Chemie"". The second volume of Progress in Physical Chemistry is a collection of thematically closely related minireview articles written by the members of the Collaborative Research Centre (SFB) 277 of the German Research Foundation (DFG). These articles are based on twelve years of intense coordinated research efforts. Central topics are the synthesis and the characterization of interface-dominated, i.e. nanostructured materials, mainly in the solid state but also as

  1. Rapid Refresh (RAP) [13 km (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Rapid Refresh (RAP) numerical weather model took the place of the Rapid Update Cycle (RUC) on May 1, 2012. Run by the National Centers for Environmental...

  2. Rapid Refresh (RAP) [20 km (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Rapid Refresh (RAP) numerical weather model took the place of the Rapid Update Cycle (RUC) on May 1, 2012. Run by the National Centers for Environmental...

  3. Sensor Webs: Autonomous Rapid Response to Monitor Transient Science Events (United States)

    Mandl, Dan; Grosvenor, Sandra; Frye, Stu; Sherwood, Robert; Chien, Steve; Davies, Ashley; Cichy, Ben; Ingram, Mary Ann; Langley, John; Miranda, Felix


    To better understand how physical phenomena, such as volcanic eruptions, evolve over time, multiple sensor observations over the duration of the event are required. Using sensor web approaches that integrate original detections by in-situ sensors and global-coverage, lower-resolution, on-orbit assets with automated rapid response observations from high resolution sensors, more observations of significant events can be made with increased temporal, spatial, and spectral resolution. This paper describes experiments using Earth Observing 1 (EO-1) along with other space and ground assets to implement progressive mission autonomy to identify, locate and image with high resolution instruments phenomena such as wildfires, volcanoes, floods and ice breakup. The software that plans, schedules and controls the various satellite assets are used to form ad hoc constellations which enable collaborative autonomous image collections triggered by transient phenomena. This software is both flight and ground based and works in concert to run all of the required assets cohesively and includes software that is model-based, artificial intelligence software.

  4. Maass Forms and Quantum Modular Forms (United States)

    Rolen, Larry

    This thesis describes several new results in the theory of harmonic Maass forms and related objects. Maass forms have recently led to a flood of applications throughout number theory and combinatorics in recent years, especially following their development by the work of Bruinier and Funke the modern understanding Ramanujan's mock theta functions due to Zwegers. The first of three main theorems discussed in this thesis concerns the integrality properties of singular moduli. These are well-known to be algebraic integers, and they play a beautiful role in complex multiplication and explicit class field theory for imaginary quadratic fields. One can also study "singular moduli" for special non-holomorphic functions, which are algebraic but are not necessarily algebraic integers. Here we will explain the phenomenon of integrality properties and provide a sharp bound on denominators of symmetric functions in singular moduli. The second main theme of the thesis concerns Zagier's recent definition of a quantum modular form. Since their definition in 2010 by Zagier, quantum modular forms have been connected to numerous different topics such as strongly unimodal sequences, ranks, cranks, and asymptotics for mock theta functions. Motivated by Zagier's example of the quantum modularity of Kontsevich's "strange" function F(q), we revisit work of Andrews, Jimenez-Urroz, and Ono to construct a natural vector-valued quantum modular form whose components. The final chapter of this thesis is devoted to a study of asymptotics of mock theta functions near roots of unity. In his famous deathbed letter, Ramanujan introduced the notion of a mock theta function, and he offered some alleged examples. The theory of mock theta functions has been brought to fruition using the framework of harmonic Maass forms, thanks to Zwegers. Despite this understanding, little attention has been given to Ramanujan's original definition. Here we prove that Ramanujan's examples do indeed satisfy his

  5. Gleason Grade Progression Is Uncommon (United States)

    Penney, Kathryn L.; Stampfer, Meir J.; Jahn, Jaquelyn L; Sinnott, Jennifer A.; Flavin, Richard; Rider, Jennifer R.; Finn, Stephen; Giovannucci, Edward; Sesso, Howard D.; Loda, Massimo; Mucci, Lorelei A.; Fiorentino, Michelangelo


    Gleason grade is universally used for pathologic scoring the differentiation of prostate cancer. However, it is unknown whether prostate tumors arise well-differentiated and then progress to less differentiated forms or if Gleason grade is an early and largely unchanging feature. Prostate Specific Antigen (PSA) screening has reduced the proportion of tumors diagnosed at advanced stage, which allows assessment of this question on a population level. If Gleason grade progresses as stage does, one would expect a similar reduction in high grade tumors. We studied 1,207 Physicians’ Health Study and Health Professionals Follow-up Study participants diagnosed with prostate cancer 1982–2004 and treated with prostatectomy. We compared the distribution of grade and clinical stage across the pre-PSA and PSA screening eras. We re-reviewed grade using the ISUP 2005 revised criteria. The proportion of advanced stage tumors dropped more than six-fold, from the earliest period (12/1982–1/1993), 19.9% stage ≥T3, to the latest (5/2000–12/2004), 3% stage T3, none T4. The proportion of Gleason score ≥8 decreased substantially less, from 25.3% to 17.6%. A significant interaction between stage and diagnosis date predicting grade (p=0.04) suggests the relationship between grade and stage varies by time period. As the dramatic shift in stage since the introduction of PSA screening was accompanied by a more modest shift in Gleason grade, these findings suggest grade may be established early in tumor pathogenesis. This has implications for the understanding of tumor progression and prognosis, and may help patients diagnosed with lower grade disease feel more comfortable choosing active surveillance. PMID:23946472


    Directory of Open Access Journals (Sweden)

    Khaled Nassar


    Full Text Available The practice of architecture is continuously changing, mirroring the paradigm shifts in the world it builds for. With increasing use of digital technology, we need to ensure that learning and teaching do not shift from the fundamental skill set required of an architect. Architectural problems are unique in their nature, requiring volumetric visualization and problem solving skills, and although many of these skills can be replicated using digital technology, can digital technology replace the cognitive development, which occurs through manual problem solving? Over the last three decades we have seen the almost ubiquitous use of computers in the design practice and professional studios with increasingly more complex forms being thought of and turned into buildings. This development obviously raises challenging questions of architectural theory and perplexing issues for those concerned with the future of architectural education and its effect on the design process. But how can this effect be analyzed subjectively remains an open question. Recent research efforts have shown that perception and visualization abilities reflect the quality of a design outcome. Very limited research however exists which attempts to understand or document the spatial analysis and visualization abilities of new generations of architects. This paper reports on a novel scalable test that could be used to investigate the processing and synthesis of visual information related to the new kinds of free form encountered in today’s architecture. Unlike traditional missing views and orthographic projection problems, proposed test can be used to accurate assess freeform visualization. A number of 2-mainfold very high genus surfaces were selected. Physical models of these surfaces are manufactured from a durable thermoplastic material by Fused Deposition Modeling rapid prototyping machines. Students are then asked to position a digital model of the surface to match that of the

  7. [Domestic violence: any progress?]. (United States)

    Henrion, Roger


    Since the publication of the French national survey of violence against women in 2000, the fight against domestic violence has made steady progress. Knowledge of the phenomenon has significantly improved. A nationwide study of murders and manslaughters perpetrated by one partner of a couple against the other has been published annually since 2006. In 2012, domestic violence resulted in the deaths of 314 persons: 166 women, 31 men, 25 children, 9 collateral victims, 14 rivals, and two former spouses killed by their ex-fathers in law. In addition, 67 perpetrators committed suicide (51 men and3 women). The number of victims fluctuates from year to year but has remained fairly stable since 2006 (n=168). Legislation has improved significantly: eight new laws have been passed since 2004, all designed to protect women and to ensure that violent men are restrained and treated. New measures to inform and protect women have been implemented and others have been improved, such as the anonymous helpline (phone no 3919, "domestic violence information"). An inter-ministerial committee on the protection of women from violence and the prevention of human trafficking (MIPROF) was created on 3 January 2013. A website entitled "Stop violence against women " (Stop violences faites aux femmes) is now available. The "Imminent Danger" mobile phone system, designed to alert police if a suspected or known perpetrator breaches restraint conditions, will be extended to the entire country from January 2014. Referees charged with coordinating comprehensive long-tern care of women victims have been deployed at the county level. Information centers on the rights of women and families (CIDFF) now form a local nationwide network. Routine interviews with a midwife during the fourth month of pregnancy, focusing on the woman's emotional, economic and social conditions, have been implemented in 21 % of maternity units and should gradually be generalized. The authorities who have enforced the law have

  8. Rapid, sensitive, and specific detection of Clostridium tetani by loop-mediated isothermal amplification assay. (United States)

    Jiang, Dongneng; Pu, Xiaoyun; Wu, Jiehong; Li, Meng; Liu, Ping


    Tetanus is a specific infectious disease, which is often associated with catastrophic events such as earthquakes, traumas, and war wounds. The obligate anaerobe Clostridium tetani is the pathogen that causes tetanus. Once the infection of tetanus progresses to an advanced stage within the wounds of limbs, the rates of amputation and mortality increase manifold. Therefore, it is necessary to devise a rapid and sensitive point-of-care detection method for C. tetani so as to ensure an early diagnosis and clinical treatment of tetanus. In this study, we developed a detection method for C. tetani using loop-mediated isothermal amplification (LAMP) assay, wherein the C. tetani tetanus toxin gene was used as the target gene. The method was highly specific and sensitive, with a detection limit of 10 colony forming units (CFU)/ml, and allowed quantitative analysis. While detecting C. tetani in clinical samples, it was found that the LAMP results completely agreed with those of the traditional API 20A anaerobic bacteria identification test. As compared with the traditional API test and PCR assay, LAMP detection of C. tetani is simple and rapid, and the results can be identified through naked-eye observation. Therefore, it is an ideal and rapid point-of-care testing method for tetanus.

  9. Rapid Tooling via Investment Casting and Rapid Prototype Patterns

    Energy Technology Data Exchange (ETDEWEB)

    Baldwin, Michael D.


    The objective of this work to develop the materials processing and design technologies required to reduce the die development time for metal mold processes from 12 months to 3 months, using die casting of Al and Mg as the example process. Sandia demonstrated that investment casting, using rapid prototype patterns produced from Stereo lithography or Selective laser Sintering, was a viable alternative/supplement to the current technology of machining form wrought stock. A demonstration die insert (ejector halt) was investment cast and subsequently tested in the die casting environment. The stationary half of the die insert was machined from wrought material to benchmark the cast half. The two inserts were run in a die casting machine for 3,100 shots of aluminum and at the end of the run no visible difference could be detected between the cast and machined inserts. Inspection concluded that the cast insert performed identically to the machined insert. Both inserts had no indications of heat checking or degradation.

  10. Progressive Retirement Programme

    CERN Multimedia

    HR Department


    Following discussion at the Standing Concertation Committee at its meeting on 30 January 2007, the Director-General has approved the extension of the Progressive Retirement Programme with effect from 1 April 2007 until 31 March 2008. Human Resources Department Tel. 74484/74128

  11. Are Forecast Updates Progressive?

    NARCIS (Netherlands)

    C-L. Chang (Chia-Lin); Ph.H.B.F. Franses (Philip Hans); M.J. McAleer (Michael)


    textabstractMacro-economic forecasts typically involve both a model component, which is replicable, as well as intuition, which is non-replicable. Intuition is expert knowledge possessed by a forecaster. If forecast updates are progressive, forecast updates should become more accurate, on average,

  12. Are Forecast Updates Progressive?

    NARCIS (Netherlands)

    C-L. Chang (Chia-Lin); Ph.H.B.F. Franses (Philip Hans); M.J. McAleer (Michael)


    textabstractMany macroeconomic forecasts and forecast updates like those from IMF and OECD typically involve both a model component, which is replicable, as well as intuition, which is non-replicable. Intuition is expert knowledge possessed by a forecaster. If forecast updates are progressive,

  13. The storm of progress

    African Journals Online (AJOL)


    The famous Eiffel tower was erected for the 1889 World Fair in Paris. The theme was Manufacturing and. Transformation. This construction still symbolises the romance of tech- nology and progress. I do not intend to recall the chain of ..... Surely we cannot simply ignore the pathology. The interventional radiologist has an.

  14. Progressive dysarthria and ataxia

    African Journals Online (AJOL)

    support from the Community Stroke Team. Over the following eight days there was a progressive deterioration in his clinical condition. Community stroke nurses raised concerns over his marked dysarthria and severely unsteady gait; they found that he was unable to walk independently and was having recurrent falls. They.

  15. Progress in optics

    CERN Document Server

    Wolf, Emil


    The Progress in Optics series contains more than 300 review articles by distinguished research workers, which have become permanent records for many important developments, helping optical scientists and optical engineers stay abreast of their fields. Comprehensive, in-depth reviewsEdited by the leading authority in the field

  16. Progressive Web applications

    CERN Multimedia

    CERN. Geneva


    Progressive Web Applications are native-like applications running inside of a browser context. In my presentation I would like describe their characteristics, benchmarks and building process using a quick and simple case study example with focus on Service Workers api.

  17. Learning Progressions & Climate Change (United States)

    Parker, Joyce M.; de los Santos, Elizabeth X.; Anderson, Charles W.


    Our society is currently having serious debates about sources of energy and global climate change. But do students (and the public) have the requisite knowledge to engage these issues as informed citizenry? The learning-progression research summarized here indicates that only 10% of high school students typically have a level of understanding…

  18. Progression og underviserkompetencer

    Directory of Open Access Journals (Sweden)

    Lene Tortzen Bager


    Full Text Available På baggrund af en kvalitativ interviewundersøgelse af undervisere ved Aarhus Universitet lavet i 2012, tematiserer artiklen, hvordan undervisere udvikler deres faglige og pædagogiske kompetencer i forhold til at kunne skabe progression inden for innovation og entreprenørskab forstået enten som didaktik, arbejdsformer i faglige forløb eller som fag på universitetet. I arbejdet med progression er det en udfordring at integrere de nye faglige dimensioner i det kernefaglige felt. Den seneste model for progression inden for innovation og entreprenør-skab siger, at det er den lærendes generelle erfaringsniveau, der er den afgørende progressionsskabende faktor (Progressionsmodellen, Fonden for Entreprenørskab, 2013b. Samtidig skelner international forskning inden for studiekompetenceområdet mellem niveauer, hvor indlejret viden er det mest avancerede kompetenceniveau (Barrie, 2002.Ifølge progressionsmodellen og den nævnte kompetenceforskning er erfaring og dybt integreret læring altså centrale dimensioner i progression. Men hvad er underviserens rolle heri? Underviserens professionelle udviklingsarbejde forekommer at være underbelyst i forhold til, at underviseren er den legitime garant for integrationen af nye faglige dimensioner og for den studerendes kompetenceniveau. Interviewundersøgelsen forholder sig til spørgsmålet om progression gennem de deltagende underviseres beskrivelse af betydningslag i entreprenørskabsbegrebet koblet til de praksisformer i undervisningen, der knytter sig hertil samt et indblik i undervisernes refleksioner over deres kompetenceudviklingsprocesser. Artiklens bidrag til progression er at se underviserens motivation og kompetenceudvikling som forudsætninger herfor.     Based on a qualitative study of five teachers in the Faculty of Arts at Aarhus University that took place during 2012, the article thematizes how teachers develop their professional and educational qualifications in innovation and

  19. Progression og underviserkompetencer

    Directory of Open Access Journals (Sweden)

    Lene Tortzen Bager


    Full Text Available På baggrund af en kvalitativ interviewundersøgelse af undervisere ved Aarhus Universitet lavet i 2012, tematiserer artiklen, hvordan undervisere udvikler deres faglige og pædagogiske kompetencer i forhold til at kunne skabe progression inden for innovation og entreprenørskab forstået enten som didaktik, arbejdsformer i faglige forløb eller som fag på universitetet. I arbejdet med progression er det en udfordring at integrere de nye faglige dimensioner i det kernefaglige felt. Den seneste model for progression inden for innovation og entreprenør-skab siger, at det er den lærendes generelle erfaringsniveau, der er den afgørende progressionsskabende faktor (Progressionsmodellen, Fonden for Entreprenørskab, 2013b. Samtidig skelner international forskning inden for studiekompetenceområdet mellem niveauer, hvor indlejret viden er det mest avancerede kompetenceniveau (Barrie, 2002.Ifølge progressionsmodellen og den nævnte kompetenceforskning er erfaring og dybt integreret læring altså centrale dimensioner i progression. Men hvad er underviserens rolle heri? Underviserens professionelle udviklingsarbejde forekommer at være underbelyst i forhold til, at underviseren er den legitime garant for integrationen af nye faglige dimensioner og for den studerendes kompetenceniveau. Interviewundersøgelsen forholder sig til spørgsmålet om progression gennem de deltagende underviseres beskrivelse af betydningslag i entreprenørskabsbegrebet koblet til de praksisformer i undervisningen, der knytter sig hertil samt et indblik i undervisernes refleksioner over deres kompetenceudviklingsprocesser. Artiklens bidrag til progression er at se underviserens motivation og kompetenceudvikling som forudsætninger herfor.  Based on a qualitative study of five teachers in the Faculty of Arts at Aarhus University that took place during 2012, the article thematizes how teachers develop their professional and educational qualifications in innovation and

  20. Forms of Arthritis (United States)

    ... this page please turn Javascript on. Forms of Arthritis Past Issues / Fall 2006 Table of Contents Today, ... of Linda Saisselin Osteoarthritis (OA) — the form of arthritis typically occurring during middle or old age, this ...

  1. Form postponement: a reconceptualization




    Form postponement has been widely acknowledged as one of the main avenues to mitigate the adverse effects of product proliferation or customization on operational performance. As it often happens with long debated concepts, the proposed definitions of form postponement sometimes display substantial differences. This paper aims at moving a step forward towards a more precise definition of form postponement in the domain of tangible products. A first result on this way is that form postponement...

  2. Characterization of an Early-Onset, Autosomal Recessive, Progressive Retinal Degeneration in Bengal Cats (United States)

    Ofri, Ron; Reilly, Christopher M.; Maggs, David J.; Fitzgerald, Paul G.; Shilo-Benjamini, Yael; Good, Kathryn L.; Grahn, Robert A.; Splawski, Danielle D.; Lyons, Leslie A.


    Purpose A form of retinal degeneration suspected to be hereditary was discovered in a family of Bengal cats. A breeding colony was established to characterize disease progression clinically, electrophysiologically, and morphologically, and to investigate the mode of inheritance. Methods Affected and related cats were donated by owners for breeding trials and pedigree analysis. Kittens from test and complementation breedings underwent ophthalmic and neuro-ophthalmic examinations and ERG, and globes were evaluated using light microscopy. Results Pedigree analysis, along with test and complementation breedings, indicated autosomal recessive inheritance and suggested that this disease is nonallelic to a retinal degeneration found in Persian cats. Mutation analysis confirmed the disease is not caused by CEP290 or CRX variants found predominantly in Abyssinian and Siamese cats. Ophthalmoscopic signs of retinal degeneration were noted at 9 weeks of age and became more noticeable over the next 4 months. Visual deficits were behaviorally evident by 1 year of age. Electroretinogram demonstrated reduced rod and cone function at 7 and 9 weeks of age, respectively. Rod responses were mostly extinguished at 14 weeks of age; cone responses were minimal by 26 weeks. Histologic degeneration was first observed at 8 weeks, evidenced by reduced photoreceptor numbers, then rapid deterioration of the photoreceptor layer and, subsequently, severe outer retinal degeneration. Conclusions A recessively inherited primary photoreceptor degeneration was characterized in the Bengal cat. The disease is characterized by early onset, with histologic, ophthalmoscopic, and electrophysiological signs evident by 2 months of age, and rapid progression to blindness. PMID:26258614

  3. Unified form language

    DEFF Research Database (Denmark)

    Alnæs, Martin S.; Logg, Anders; Ølgaard, Kristian Breum


    We present the Unied Form Language (UFL), which is a domain-specic language for representing weak formulations of partial dierential equations with a view to numerical approximation. Features of UFL include support for variational forms and functionals, automatic dierentiation of forms and expres...... libraries to generate concrete low-level implementations. Some application examples are presented and libraries that support UFL are highlighted....

  4. Modular forms on Schiermonnikoog

    NARCIS (Netherlands)

    Edixhoven, B.; van der Geer, G.; Moonen, B.


    Modular forms are functions with an enormous amount of symmetry that play a central role in number theory, connecting it with analysis and geometry. They have played a prominent role in mathematics since the 19th century and their study continues to flourish today. Modular forms formed the

  5. Mesonic Form Factors

    Energy Technology Data Exchange (ETDEWEB)

    Frederic D. R. Bonnet; Robert G. Edwards; George T. Fleming; Randal Lewis; David Richards


    We have started a program to compute the electromagnetic form factors of mesons. We discuss the techniques used to compute the pion form factor and present preliminary results computed with domain wall valence fermions on MILC asqtad lattices, as well as Wilson fermions on quenched lattices. These methods can easily be extended to rho-to-gamma-pi transition form factors.

  6. Building a rapid response team. (United States)

    Halvorsen, Lisa; Garolis, Salomeja; Wallace-Scroggs, Allyson; Stenstrom, Judy; Maunder, Richard


    The use of rapid response teams is a relatively new approach for decreasing or eliminating codes in acute care hospitals. Based on the principles of a code team for cardiac and/or respiratory arrest in non-critical care units, the rapid response teams have specially trained nursing, respiratory, and medical personnel to respond to calls from general care units to assess and manage decompensating or rapidly changing patients before their conditions escalate to a full code situation. This article describes the processes used to develop a rapid response team, clinical indicators for triggering a rapid response team call, topics addressed in an educational program for the rapid response team members, and methods for evaluating effectiveness of the rapid response team.

  7. Stillbirths: progress and unfinished business. (United States)

    Frøen, J Frederik; Friberg, Ingrid K; Lawn, Joy E; Bhutta, Zulfiqar A; Pattinson, Robert C; Allanson, Emma R; Flenady, Vicki; McClure, Elizabeth M; Franco, Lynne; Goldenberg, Robert L; Kinney, Mary V; Leisher, Susannah Hopkins; Pitt, Catherine; Islam, Monir; Khera, Ajay; Dhaliwal, Lakhbir; Aggarwal, Neelam; Raina, Neena; Temmerman, Marleen


    This first paper of the Lancet Series on ending preventable stillbirths reviews progress in essential areas, identified in the 2011 call to action for stillbirth prevention, to inform the integrated post-2015 agenda for maternal and newborn health. Worldwide attention to babies who die in stillbirth is rapidly increasing, from integration within the new Global Strategy for Women's, Children's and Adolescents' Health, to country policies inspired by the Every Newborn Action Plan. Supportive new guidance and metrics including stillbirth as a core health indicator and measure of quality of care are emerging. Prenatal health is a crucial biological foundation to life-long health. A key priority is to integrate action for prenatal health within the continuum of care for maternal and newborn health. Still, specific actions for stillbirths are needed for advocacy, policy formulation, monitoring, and research, including improvement in the dearth of data for effective coverage of proven interventions for prenatal survival. Strong leadership is needed worldwide and in countries. Institutions with a mandate to lead global efforts for mothers and their babies must assert their leadership to reduce stillbirths by promoting healthy and safe pregnancies. Copyright © 2016 Elsevier Ltd. All rights reserved.

  8. Laboratory markers associated with progression of HIV infection

    Directory of Open Access Journals (Sweden)

    Gupta V


    Full Text Available Infection with HIV may develop to AIDS at different rates in different individuals, with a spectrum varying from rapid progression to long term non-progression. The variable course of HIV-1 infection causes emotional trauma for the infected person and complicates the design and interpretation of therapeutic trials because of unrecognized differences in prognosis. Thus it is essential to have tests which can accurately assess the stage of infection in an individual, as well as predict its course and monitor its progression. These laboratory tests are very valuable during the period of clinical latency and subsequently supplement various clinical parameters.

  9. Problems of rapid growth. (United States)

    Kim, T D


    South Korea's export-oriented development strategy has achieved a remarkable growth record, but it has also brought 2 different problems: 1) since the country's exports accounted for about 1% of total world export volume, the 1st world has become fearful about Korea's aggressive export drive; and 2) the fact that exports account for over 30% of its total gross national product (GNP) exposes the vulnerability of South Korea's economy itself. South Korea continues to be a poor nation, although it is rated as 1 of the most rapidly growing middle income economies. A World Bank 1978 report shows Korea to be 28th of 58 middle income countries in terms of per capita GNP in 1976. Of 11 newly industrializing countries (NIC), 5 in the European continent are more advanced than the others. A recent emphasis on the basic human needs approach has tended to downgrade the concept of GNP. Korea has only an abundant labor force and is without any natural resources. Consequently, Korea utilized an export-oriented development strategy. Oil requirements are met with imports, and almost all raw materials to be processed into exportable products must be imported. To pay import bills Korea must export and earn foreign exchange. It must be emphasized that foreign trade must always be 2-way traffic. In order to export more to middle income countries like Korea, the countries of the 1st world need to ease their protectionist measures against imports from developing countries.

  10. Rapid Polymer Sequencer (United States)

    Stolc, Viktor (Inventor); Brock, Matthew W (Inventor)


    Method and system for rapid and accurate determination of each of a sequence of unknown polymer components, such as nucleic acid components. A self-assembling monolayer of a selected substance is optionally provided on an interior surface of a pipette tip, and the interior surface is immersed in a selected liquid. A selected electrical field is impressed in a longitudinal direction, or in a transverse direction, in the tip region, a polymer sequence is passed through the tip region, and a change in an electrical current signal is measured as each polymer component passes through the tip region. Each of the measured changes in electrical current signals is compared with a database of reference electrical change signals, with each reference signal corresponding to an identified polymer component, to identify the unknown polymer component with a reference polymer component. The nanopore preferably has a pore inner diameter of no more than about 40 nm and is prepared by heating and pulling a very small section of a glass tubing.

  11. Rapidly rotating red giants (United States)

    Gehan, Charlotte; Mosser, Benoît; Michel, Eric


    Stellar oscillations give seismic information on the internal properties of stars. Red giants are targets of interest since they present mixed modes, wich behave as pressure modes in the convective envelope and as gravity modes in the radiative core. Mixed modes thus directly probe red giant cores, and allow in particular the study of their mean core rotation. The high-quality data obtained by CoRoT and Kepler satellites represent an unprecedented perspective to obtain thousands of measurements of red giant core rotation, in order to improve our understanding of stellar physics in deep stellar interiors. We developed an automated method to obtain such core rotation measurements and validated it for stars on the red giant branch. In this work, we particularly focus on the specific application of this method to red giants having a rapid core rotation. They show complex spectra where it is tricky to disentangle rotational splittings from mixed-mode period spacings. We demonstrate that the method based on the identification of mode crossings is precise and efficient. The determination of the mean core rotation directly derives from the precise measurement of the asymptotic period spacing ΔΠ1 and of the frequency at which the crossing of the rotational components is observed.

  12. Progress in molecular precursors for electronic materials

    Energy Technology Data Exchange (ETDEWEB)

    Buhro, W.E. [Washington Univ., St. Louis, MO (United States)


    Molecular-precursor chemistry provides an essential underpinning to all electronic-materials technologies, including photovoltaics and related areas of direct interest to the DOE. Materials synthesis and processing is a rapidly developing field in which advances in molecular precursors are playing a major role. This article surveys selected recent research examples that define the exciting current directions in molecular-precursor science. These directions include growth of increasingly complex structures and stoichiometries, surface-selective growth, kinetic growth of metastable materials, growth of size-controlled quantum dots and quantum-dot arrays, and growth at progressively lower temperatures. Continued progress in molecular-precursor chemistry will afford precise control over the crystal structures, nanostructures, and microstructures of electronic materials.

  13. Progressive posterior cortical dysfunction

    Directory of Open Access Journals (Sweden)

    Fábio Henrique de Gobbi Porto

    Full Text Available Abstract Progressive posterior cortical dysfunction (PPCD is an insidious syndrome characterized by prominent disorders of higher visual processing. It affects both dorsal (occipito-parietal and ventral (occipito-temporal pathways, disturbing visuospatial processing and visual recognition, respectively. We report a case of a 67-year-old woman presenting with progressive impairment of visual functions. Neurologic examination showed agraphia, alexia, hemispatial neglect (left side visual extinction, complete Balint's syndrome and visual agnosia. Magnetic resonance imaging showed circumscribed atrophy involving the bilateral parieto-occipital regions, slightly more predominant to the right . Our aim was to describe a case of this syndrome, to present a video showing the main abnormalities, and to discuss this unusual presentation of dementia. We believe this article can contribute by improving the recognition of PPCD.

  14. Progress in Scientific Visualization

    Energy Technology Data Exchange (ETDEWEB)

    Max, N


    Visualization of observed data or simulation output is important to science and engineering. I have been particularly interested in visualizing 3-D structures, and report here my personal impressions on progress in the last 20 years in visualizing molecules, scalar fields, and vector fields and their associated flows. I have tried to keep the survey and list of references manageable, so apologize to those authors whose techniques I have not mentioned, or have described without a reference citation.

  15. Progress in optics

    CERN Document Server

    Wolf, Emil


    In the fourty-seven years that have gone by since the first volume of Progress in Optics was published, optics has become one of the most dynamic fields of science. The volumes in this series which have appeared up to now contain more than 300 review articles by distinguished research workers, which have become permanent records for many important developments.- Backscattering and Anderson localization of light- Advances in oliton manipulation in optical lattices- Fundamental quantum noise in optical amplification- Invisibility cloaks

  16. Clean Energy Progress Report

    Energy Technology Data Exchange (ETDEWEB)



    For the past several years, the IEA and others have been calling for a clean energy revolution to achieve global energy security, economic growth and climate change goals. This report analyses for the first time progress in global clean energy technology deployment against the pathways that are needed to achieve these goals. It provides an overview of technology deployment status, key policy developments and public spending on RDD&D of clean energy technologies.

  17. Recent progress in microcalorimetry

    CERN Document Server

    Calvet, E; Skinner, H A


    Recent Progress in Microcalorimetry focuses on the methodologies, processes, and approaches involved in microcalorimetry, as well as heat flow, temperature constancy, and chemistry of alumina and cements.The selection first offers information on the different types of calorimeters; measurement of the heat flow between the calorimeter and jacket boundaries by means of a thermoelectric pile; and constructional details of the microcalorimeter. Discussions focus on classification of calorimeters, use of thermoelectric piles as thermometers, correct measurement of heat flow from a calorimeter conta

  18. Progress in optics

    CERN Document Server

    Wolf, Emil


    In the fourty-six years that have gone by since the first volume of Progress in Optics was published, optics has become one of the most dynamic fields of science. The volumes in this series which have appeared up to now contain more than 300 review articles by distinguished research workers, which have become permanent records for many important developments.- Metamaterials- Polarization Techniques- Linear Baisotropic Mediums- Ultrafast Optical Pulses- Quantum Imaging- Point-Spread Funcions- Discrete Wigner Functions

  19. Progress in nanophotonics 1

    CERN Document Server

    Ohtsu, Motoichi


    This book focuses on the recent progress in nanophotonics technology to be used to develop novel nano-optical devices, fabrication technology, and security systems. It begins with a review of the concept of dressed photons and applications to devices, fabrication, and systems; principles and applications. Further topics include: DNA process for quantum dot chain, photon enhanced emission microscopy, near field spectroscopy of metallic nanostructure, self-organized fabrication of composite semiconductor quantum dots, formation of metallic nanostructure, and nanophotonic information systems with

  20. Fatal Metformin Overdose Presenting with Progressive Hyperglycemia

    Directory of Open Access Journals (Sweden)

    Suchard, Jeffrey R


    Full Text Available A 29-year-old man with no history of diabetes ingested over 60 grams of metformin in a suicide attempt. He presented to the emergency department with acute renal insufficiency, severe lactic acidosis, and rapidly-progressive hyperglycemia. The patient’s peak serum glucose level of 707 mg/dL is the highest yet reported in a case of metformin toxicity. Treatment included sodium bicarbonate infusion and hemodialysis, but the patient suffered several cardiac arrests with pulseless electrical activity and ultimately expired 25 hours after the ingestion.

  1. Geothermal Progress Monitor: Report No. 14

    Energy Technology Data Exchange (ETDEWEB)


    This issue of the Geothermal Progress Monitor, the 14th since its inception in 1980, highlights the anticipated rapid growth in the use of geothermal heat pumps and documents the continued growth in the use of geothermal energy for power generation, both in this country and abroad. In countries with a relatively large demand for new generation capacity, geothermal, if available, is being called on as a preferable alternative to the use of domestic or imported oil. On the other hand, in this country where current demand for new capacity is less, geothermal energy is commonly being put to use in small power generation units operating on the hot water resource.

  2. [Progress in digital PCR technology and application]. (United States)

    Lin, Jiaqi; Su, Guocheng; Su, Wenjin; Zhou, Changyi


    Digital PCR is an emerging analysis technology for absolute quantification after realtime-PCR. Through digital PCR, single DNA molecules are distributed into isolated reactions, and the product with fluorescence signal can be detected and analyzed after amplification. With the advantages of higher sensitivity and accuracy, digital PCR, independent of a standard curve, is developing rapidly and applied widely to the next generation sequencing and detection fields, such as gene mutation, copy number variation, microorganism, and genetically modified food. In this article, we reviewed the quantitative method and research progress of digital PCR technology in the main application fields.

  3. Progressive pseudorheumatoid dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Mampaey, S.; De Schepper, A. [Dept. of Radiology, University Hospital Antwerp, Edegem (Belgium); Vanhoenacker, F. [Dept. of Radiology, University Hospital Antwerp, Edegem (Belgium); Dept. of Radiology, St. Maarten Hospital, Duffel (Belgium); Boven, K. [Dept. of Pediatrics, University Hospital Antwerp, Edegem (Belgium); Hul, W. van [Dept. of Medical Genetics, University of Antwerp (Belgium)


    A rare case of progressive pseudorheumatoid dysplasia (PPD) in a 9-year-old girl is presented. Clinically, chronic painless swollen joints, accompanied by progressive motion restriction and progressive walking difficulties, were found. Radiologically, there was enlargement of the epimetaphyseal portions of the large joints, metacarpal heads, and phalanges, and generalized platyspondyly with irregular delineation of the endplates of the vertebral bodies. The radioclinical features at the peripheral joints were originally misdiagnosed as juvenile rheumatoid arthritis (JRA), and the structural spinal abnormalities were neglected and interpreted as Scheuermann's disease. However, the absence of active inflammatory parameters argues against JRA, whereas the low age of onset of the irregularities at the vertebral endplates is an argument against the diagnosis of Scheuermann's disease. The combination of the dysplastic abnormalities of the spine, with platyspondyly and Scheuermann-like lesions at an unusually low age of onset, and radiological features mimicking JRA of the peripheral joints, is the clue to the diagnosis of this rare autosomal-recessive disease. This case is the first to document the MRI features of PPD of the spine. (orig.)

  4. Simple and rapid spectrophotometric assay of levocetirizine in ...

    African Journals Online (AJOL)

    Simple, rapid, selective and fairly sensitive method is described for the determination of levocetirizine (LCTZ) in pure form and in its dosage forms. The method is based on the formation of intensely colored charge-transfer (CT) complexes between LCTZ as donor with two π acceptors, chloranilic acid (CAA) and 2 ...

  5. The spectrum of progressive derecho formation environments (United States)

    Guastini, Corey T.

    Progressive derechos are severe mesoscale convective systems that often form east of the Rocky Mountains during the warm season (May--August) and cause, by definition, straight-line wind damage along paths upwards of 400 km long. This study develops a subjective, seven-category classification scheme that spans the spectrum of progressive derecho formation environments from those dominated by robust upper-level ridges to those characterized by vigorous upper-level troughs. A climatology of 256 progressive derecho events is created for 1996--2013 and is categorized according to the developed classification scheme. Derecho initiation-relative composites are constructed for each of the seven groups using 0.5° Climate Forecast System Reanalysis data to document the environmental characteristics unique to each group as well as those shared among them. Finally, two in-depth case studies and five cursory case studies provide examples of the seven categories and reveal important nuances in mesoscale dynamic and thermodynamic structure inherent to all derecho cases. Results of the climatology show progressive derecho activity increases from 1 May through 1 July before decreasing again through the end of August and follows a northward trend in latitude from 1 May through 1 August before shifting slightly southward through the end of the warm season. Upslope flow in the vicinity of the Rocky Mountains initiates 28 percent of progressive derechos, upper-level troughs initiate 20 percent, 47 percent form in benign synoptic environments, and 5 percent are unclassifiable. Composite results show all progressive derecho initiation environments are marked by a long axis of instability caused by the overlap of high atmospheric moisture content and steep midlevel lapse rates, but the relative positions and strengths of upper-level troughs and ridges are crucial in determining how the instability axis develops and what its orientation in space will be. Case studies reveal instability

  6. Rapid mixing kinetic techniques. (United States)

    Martin, Stephen R; Schilstra, Maria J


    Almost all of the elementary steps in a biochemical reaction scheme are either unimolecular or bimolecular processes that frequently occur on sub-second, often sub-millisecond, time scales. The traditional approach in kinetic studies is to mix two or more reagents and monitor the changes in concentrations with time. Conventional spectrophotometers cannot generally be used to study reactions that are complete within less than about 20 s, as it takes that amount of time to manually mix the reagents and activate the instrument. Rapid mixing techniques, which generally achieve mixing in less than 2 ms, overcome this limitation. This chapter is concerned with the use of these techniques in the study of reactions which reach equilibrium; the application of these methods to the study of enzyme kinetics is described in several excellent texts (Cornish-Bowden, Fundamentals of enzyme kinetics. Portland Press, 1995; Gutfreund, Kinetics for the life sciences. Receptors, transmitters and catalysis. Cambridge University Press, 1995).There are various ways to monitor changes in concentration of reactants, intermediates and products after mixing, but the most common way is to use changes in optical signals (absorbance or fluorescence) which often accompany reactions. Although absorbance can sometimes be used, fluorescence is often preferred because of its greater sensitivity, particularly in monitoring conformational changes. Such methods are continuous with good time resolution but they seldom permit the direct determination of the concentrations of individual species. Alternatively, samples may be taken from the reaction volume, mixed with a chemical quenching agent to stop the reaction, and their contents assessed by techniques such as HPLC. These methods can directly determine the concentrations of different species, but are discontinuous and have a limited time resolution.

  7. Forms of Life, Forms of Reality

    Directory of Open Access Journals (Sweden)

    Piergiorgio Donatelli


    Full Text Available The article explores aspects of the notion of forms of life in the Wittgensteinian tradition especially following Iris Murdoch’s lead. On the one hand, the notion signals the hardness and inexhaustible character of reality, as the background needed in order to make sense of our lives in various ways. On the other, the hardness of reality is the object of a moral work of apprehension and deepening to the point at which its distinctive character dissolves into the family of connections we have gained for ourselves. The two movements of thought are connected and necessary.

  8. Micro metal forming

    CERN Document Server


    Micro Metal Forming, i. e. forming of parts and features with dimensions below 1 mm, is a young area of research in the wide field of metal forming technologies, expanding the limits for applying metal forming towards micro technology. The essential challenges arise from the reduced geometrical size and the increased lot size. In order to enable potential users to apply micro metal forming in production, information about the following topics are given: tribological behavior: friction between tool and work piece as well as tool wear mechanical behavior: strength and formability of the work piece material, durability of the work pieces size effects: basic description of effects occurring due to the fact, that the quantitative relation between different features changes with decreasing size process windows and limits for forming processes tool making methods numerical modeling of processes and process chains quality assurance and metrology All topics are discussed with respect to the questions relevant to micro...

  9. Gravitation and quadratic forms (United States)

    Ananth, Sudarshan; Brink, Lars; Majumdar, Sucheta; Mali, Mahendra; Shah, Nabha


    The light-cone Hamiltonians describing both pure ( N = 0) Yang-Mills and N = 4 super Yang-Mills may be expressed as quadratic forms. Here, we show that this feature extends to theories of gravity. We demonstrate how the Hamiltonians of both pure gravity and N = 8 supergravity, in four dimensions, may be written as quadratic forms. We examine the effect of residual reparametrizations on the Hamiltonian and the resulting quadratic form.

  10. Gravitation and quadratic forms

    Energy Technology Data Exchange (ETDEWEB)

    Ananth, Sudarshan [Indian Institute of Science Education and Research,Pune 411008 (India); Brink, Lars [Department of Physics, Chalmers University of Technology,S-41296 Göteborg (Sweden); Institute of Advanced Studies and Department of Physics & Applied Physics,Nanyang Technological University,Singapore 637371 (Singapore); Majumdar, Sucheta [Indian Institute of Science Education and Research,Pune 411008 (India); Mali, Mahendra [School of Physics, Indian Institute of Science Education and Research,Thiruvananthapuram, Trivandrum 695016 (India); Shah, Nabha [Indian Institute of Science Education and Research,Pune 411008 (India)


    The light-cone Hamiltonians describing both pure (N=0) Yang-Mills and N=4 super Yang-Mills may be expressed as quadratic forms. Here, we show that this feature extends to theories of gravity. We demonstrate how the Hamiltonians of both pure gravity and N=8 supergravity, in four dimensions, may be written as quadratic forms. We examine the effect of residual reparametrizations on the Hamiltonian and the resulting quadratic form.

  11. Electronic Capitalization Asset Form - (United States)

    Department of Transportation — National Automated Capitalization Authorization Form used by ATO Engineering Services, Logistics, Accounting for the purpose of identifying and capturing FAA project...

  12. Cooperative Station History Forms (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — Various forms, photographs and correspondence documenting the history of Cooperative station instrumentation, location changes, inspections, and...

  13. Monitoring Radiographic Brain Tumor Progression

    Directory of Open Access Journals (Sweden)

    John H. Sampson


    Full Text Available Determining radiographic progression in primary malignant brain tumors has posed a significant challenge to the neuroncology community. Glioblastoma multiforme (GBM, WHO Grade IV through its inherent heterogeneous enhancement, growth patterns, and irregular nature has been difficult to assess for progression. Our ability to detect tumor progression radiographically remains inadequate. Despite the advanced imaging techniques, detecting tumor progression continues to be a clinical challenge. Here we review the different criteria used to detect tumor progression, and highlight the inherent challenges with detection of progression.

  14. Effect of phosphorus fertilizer application on some forms of ...

    African Journals Online (AJOL)

    Effect of phosphorus fertilizer application on some forms of phosphorus in ... The addition of organic litter alone had little effect on bicarbonate-extractable ... When litter and phosphorus fertilizer were added together, there was an initial rapid ...

  15. 77 FR 69484 - Agency Forms Undergoing Paperwork Reduction Act Review (United States)


    ... HUMAN SERVICES Centers for Disease Control and Prevention Agency Forms Undergoing Paperwork Reduction...--Scientific Education and Professional Development Program Office (SEPDPO), Office of Surveillance... public health problems rapidly ensures cost-effective health care and enhances health promotion and...

  16. Rapid Active Sampling Package (United States)

    Peters, Gregory


    A field-deployable, battery-powered Rapid Active Sampling Package (RASP), originally designed for sampling strong materials during lunar and planetary missions, shows strong utility for terrestrial geological use. The technology is proving to be simple and effective for sampling and processing materials of strength. Although this originally was intended for planetary and lunar applications, the RASP is very useful as a powered hand tool for geologists and the mining industry to quickly sample and process rocks in the field on Earth. The RASP allows geologists to surgically acquire samples of rock for later laboratory analysis. This tool, roughly the size of a wrench, allows the user to cut away swaths of weathering rinds, revealing pristine rock surfaces for observation and subsequent sampling with the same tool. RASPing deeper (.3.5 cm) exposes single rock strata in-situ. Where a geologist fs hammer can only expose unweathered layers of rock, the RASP can do the same, and then has the added ability to capture and process samples into powder with particle sizes less than 150 microns, making it easier for XRD/XRF (x-ray diffraction/x-ray fluorescence). The tool uses a rotating rasp bit (or two counter-rotating bits) that resides inside or above the catch container. The container has an open slot to allow the bit to extend outside the container and to allow cuttings to enter and be caught. When the slot and rasp bit are in contact with a substrate, the bit is plunged into it in a matter of seconds to reach pristine rock. A user in the field may sample a rock multiple times at multiple depths in minutes, instead of having to cut out huge, heavy rock samples for transport back to a lab for analysis. Because of the speed and accuracy of the RASP, hundreds of samples can be taken in one day. RASP-acquired samples are small and easily carried. A user can characterize more area in less time than by using conventional methods. The field-deployable RASP used a Ni

  17. Psychological functioning in primary progressive versus secondary progressive multiple sclerosis

    DEFF Research Database (Denmark)

    Vleugels, L; Pfennings, L E; Pouwer, F


    Psychological functioning in two types of multiple sclerosis (MS) patients is assessed: primary progressive (PP) and secondary progressive (SP) patients. On the basis of differences in clinical course and underlying pathology we hypothesized that primary progressive patients and secondary......-MS scored significantly better on 5 out of 14 subscales than patients with SP-MS (p diagnosis of MS, amongst patients attending an MS unit, primary progressive...

  18. Rapid Robot Design Validation Project (United States)

    National Aeronautics and Space Administration — Energid Technologies will create a comprehensive software infrastructure for rapid validation of robotic designs. The software will support push-button validation...

  19. Rapid Robot Design Validation Project (United States)

    National Aeronautics and Space Administration — Energid Technologies will create a comprehensive software infrastructure for rapid validation of robot designs. The software will support push-button validation...

  20. A Modified Sesamol Derivative Inhibits Progression of Atherosclerosis (United States)

    Ying, Zhekang; Kherada, Nisharahmed; Kampfrath, Thomas; Mihai, Georgeta; Simonetti, Orlando; Desikan, Rajagopal; Selvendiran, Karuppaiyah; Sun, Qinghua; Ziouzenkova, Ouiliana; Parthasarathy, Sampath; Rajagopalan, Sanjay


    Objective Sesamol, a phenolic component of lignans, has been previously shown to reduce lipopolysaccharide-induced oxidative stress and upregulate phosphatidylinositol 3-kinase/Akt/endothelial nitric oxide synthase pathways. In the present study, we synthesized a modified form of sesamol (INV-403) to enhance its properties and assessed its effects on atherosclerosis. Methods and Results Watanabe heritable hyperlipidemic rabbits were fed with high-cholesterol chow for 6 weeks and then randomized to receive high-cholesterol diet either alone or combined with INV-403 (20 mg/kg per day) for 12 weeks. Serial MRI analysis demonstrated that INV-403 rapidly reduced atherosclerotic plaques (within 6 weeks), with confirmatory morphological analysis at 12 weeks posttreatment revealing reduced atherosclerosis paralleled by reduction in lipid and inflammatory cell content. Consistent with its effect on atherosclerosis, INV-403 improved vascular function (decreased constriction to angiotensin II and increased relaxation to acetylcholine), reduced systemic and plaque oxidative stress, and inhibited nuclear factor–κB activation via effects on nuclear factor of kappa light polypeptide gene enhancer in B-cells inhibitor, alpha (IκBα) phosphorylation with coordinate reduction in key endothelial adhesion molecules. In vitro experiments in cultured endothelial cells revealed effects of INV-403 in reducing IκBα phosphorylation via inhibition of IκB kinase 2 (IKK2). Conclusion INV-403 is a novel modified lignan derivative that potently inhibits atherosclerosis progression via its effects on IKK2 and nuclear factor–κB signaling. PMID:21183734

  1. Monthly progress report summary, September 1994

    Energy Technology Data Exchange (ETDEWEB)



    This report consists of numerous progress reports on program support, systems analysis, materials handling, chemical and physical treatments, waste destruction and stabilization, off-gas treatment, and final waste form studies. The Mixed Waste Integrated Program has responsibility for the Department of Energy`s low-level radioactive waste and hazardous material mixture characterization, treatment, and disposal. The program is undergoing transition to the Mixed Waste Focus Area.

  2. Recent Progress in Baryogenesis

    CERN Document Server

    Riotto, Antonio; Riotto, Antonio; Trodden, Mark


    We provide an up to date account of progress in understanding the origin of the observed baryon asymmetry of the universe. While our primary goal is to be current, we have attempted to give a pedagogical introduction to the primary areas of research in this field, giving a detailed description of the different scenarios. The very recent developments in GUT baryogenesis, leptogenesis, electroweak baryogenesis and the Affleck-Dine mechanism are presented. In particular, we focus on specific particle physics implementations, mostly in the context of supersymmetry, which lead to specific testable predictions.


    Energy Technology Data Exchange (ETDEWEB)



    Progress is reported on the Staged Electron Laser Acceleration (STELLA) experiment, which has been assembled on the BNL Accelerator Test Facility (ATF). The primary goal of STELLA is to demonstrate staging of the laser acceleration process by using the BNL inverse free electron laser (IFEL) as a prebuncher, which generates {approx} 1-{micro}m long microbunches, and accelerating these microbunches using an inverse Cerenkov acceleration (ICA) stage. Experimental runs are underway to recommission the IFEL and ICA systems separately, and reestablish the: microbunching process. Staging will then be examined by running both the IFEL and ICA systems together.

  4. Progress in optics

    CERN Document Server

    Wolf, Emil


    In the thirty-seven years that have gone by since the first volume of Progress in Optics was published, optics has become one of the most dynamic fields of science. At the time of inception of this series, the first lasers were only just becoming operational, holography was in its infancy, subjects such as fiber optics, integrated optics and optoelectronics did not exist and quantum optics was the domain of only a few physicists. The term photonics had not yet been coined. Today these fields are flourishing and have become areas of specialisation for many science and engineering students and n

  5. Progress in optics

    CERN Document Server

    Wolf, Emil


    In the thirty-seven years that have gone by since the first volume of Progress in Optics was published, optics has become one of the most dynamic fields of science. At the time of inception of this series, the first lasers were only just becoming operational, holography was in its infancy, subjects such as fiber optics, integrated optics and optoelectronics did not exist and quantum optics was the domain of only a few physicists. The term photonics had not yet been coined. Today these fields are flourishing and have become areas of specialisation for many science and engineering students and n

  6. [Progresses on Neandertal genomics]. (United States)

    Bi, Cai-Li; Guo, Guang-Yan; Zhang, Xiao; Tian, Yan-Hui; Shen, Yin-Zhu


    Neandertal is our closest known relative and also an archaic hominid reserving the richest fossils. Whether the Neandertals exchanged their DNA with modern human or not is a matter of debate on the modern human origin. The progresses on the mitochondrial and nuclear genomes of Neandertals in recent years were reviewed in this paper. Recent study has revealed possible genetic contribution of Neandertals to the modern human to some extent, which arose the rethinking of modern human origin. The experiences gained in the research on Neandertals will benefit the study on archaic hominids, unravel the mystery of modern human origin, and enrich the relative theoretical systems in evolutionary biological field.

  7. Isotropy of quadratic forms

    Indian Academy of Sciences (India)

    V. Suresh University Of Hyderabad Hyderabad


    Oct 31, 2008 ... Polynomials f (X1,··· ,Xn) − −− polynomial. V. Suresh University Of Hyderabad Hyderabad. Isotropy of quadratic forms ..... Historically, the study of quadratic forms was part of number theory; Minkowski, Siegel, Hasse, Eichler, Kneser and .... For example Z/pZ, p a prime number. V. Suresh University Of ...

  8. Mastering HTML5 forms

    CERN Document Server

    Gupta, Gaurav


    This tutorial will show you how to create stylish forms, not only visually appealing, but interactive and customized, in order to gather valuable user inputs and information.Enhance your skills in building responsive and dynamic web forms using HTML5, CSS3, and related technologies. All you need is a basic understanding of HTML and PHP.

  9. PowerForms

    DEFF Research Database (Denmark)

    Brabrand, Claus; Møller, Anders; Ricky, Mikkel


    All uses of HTML forms may benefit from validation of the specified input field values. Simple validation matches individual values against specified formats, while more advanced validation may involve interdependencies of form fields. There is currently no standard for specifying or implementing...

  10. Rapid prototyping: An innovative technique in dentistry

    Directory of Open Access Journals (Sweden)

    Shakeba Quadri


    Full Text Available Emergence of advanced digital technology has opened up new perspectives for design and production in the field of dentistry. Rapid prototyping (RP is a technique to quickly and automatically construct a three-dimensional (3D model of a part or product using 3D printers or stereolithography machines. RP has various dental applications, such as fabrication of implant surgical guides, zirconia prosthesis and molds for metal castings, maxillofacial prosthesis and frameworks for fixed and removable partial dentures, wax patterns for the dental prosthesis and complete denture. Rapid prototyping presents fascinating opportunities, but the process is difficult as it demands a high level of artistic skill, which means that the dental technicians should be able to work with the models obtained after impression to form a mirror image and achieve good esthetics. This review aims to focus on various RP methods and its application in dentistry.

  11. Progress in computational toxicology. (United States)

    Ekins, Sean


    Computational methods have been widely applied to toxicology across pharmaceutical, consumer product and environmental fields over the past decade. Progress in computational toxicology is now reviewed. A literature review was performed on computational models for hepatotoxicity (e.g. for drug-induced liver injury (DILI)), cardiotoxicity, renal toxicity and genotoxicity. In addition various publications have been highlighted that use machine learning methods. Several computational toxicology model datasets from past publications were used to compare Bayesian and Support Vector Machine (SVM) learning methods. The increasing amounts of data for defined toxicology endpoints have enabled machine learning models that have been increasingly used for predictions. It is shown that across many different models Bayesian and SVM perform similarly based on cross validation data. Considerable progress has been made in computational toxicology in a decade in both model development and availability of larger scale or 'big data' models. The future efforts in toxicology data generation will likely provide us with hundreds of thousands of compounds that are readily accessible for machine learning models. These models will cover relevant chemistry space for pharmaceutical, consumer product and environmental applications. Copyright © 2013 Elsevier Inc. All rights reserved.

  12. Ghrelin and cancer progression. (United States)

    Lin, Tsung-Chieh; Hsiao, Michael


    Ghrelin is a small peptide with 28 amino acids, and has been characterized as the ligand of the growth hormone secretagogue receptor (GHSR). In addition to its original function in stimulating pituitary growth hormone release, ghrelin is multifunctional and plays a role in the regulation of energy balance, gastric acid release, appetite, insulin secretion, gastric motility and the turnover of gastric and intestinal mucosa. The discovery of ghrelin and GHSR expression beyond normal tissues suggests its role other than physiological function. Emerging evidences have revealed ghrelin's function in regulating several processes related to cancer progression, especially in metastasis and proliferation. We further show the relative GHRL and GHSR expression in pan-cancers from The Cancer Genome Atlas (TCGA), suggesting the potential pathological role of the axis in cancers. This review focuses on ghrelin's biological function in cancer progression, and reveals its clinical significance especially the impact on cancer patient outcome. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  13. Periodontal disease's contribution to Alzheimer's disease progression in Down syndrome. (United States)

    Kamer, Angela R; Fortea, Juan O; Videla, Sebastià; Mayoral, Angela; Janal, Malvin; Carmona-Iragui, Maria; Benejam, Bessy; Craig, Ronald G; Saxena, Deepak; Corby, Patricia; Glodzik, Lidia; Annam, Kumar Raghava Chowdary; Robbins, Miriam; de Leon, Mony J


    People with Down syndrome (DS) are at an increased risk for Alzheimer's disease (AD). After 60 years of age, >50% of DS subjects acquire dementia. Nevertheless, the age of onset is highly variable possibly because of both genetic and environmental factors. Genetics cannot be modified, but environmental risk factors present a potentially relevant intervention for DS persons at risk for AD. Among them, inflammation, important in AD of DS type, is potential target. Consistent with this hypothesis, chronic peripheral inflammation and infections may contribute to AD pathogenesis in DS. People with DS have an aggressive form of periodontitis characterized by rapid progression, significant bacterial and inflammatory burden, and an onset as early as 6 years of age. This review offers a hypothetical mechanistic link between periodontitis and AD in the DS population. Because periodontitis is a treatable condition, it may be a readily modifiable risk factor for AD.

  14. Rapid prototyping in medical sciences

    Directory of Open Access Journals (Sweden)

    Ákos Márk Horváth


    Full Text Available Even if it sound a bit incredible rapid prototyping (RPT as production method has been used for decades in other professions. Nevertheless medical science just started discover the possibilities of this technology and use the offered benefits of 3D printing. In this paper authors have investigated the pharmaceutical usage of rapid prototyping.

  15. Caffeine, creatine, GRIN2A and Parkinson's disease progression. (United States)

    Simon, David K; Wu, Cai; Tilley, Barbara C; Lohmann, Katja; Klein, Christine; Payami, Haydeh; Wills, Anne-Marie; Aminoff, Michael J; Bainbridge, Jacquelyn; Dewey, Richard; Hauser, Robert A; Schaake, Susen; Schneider, Jay S; Sharma, Saloni; Singer, Carlos; Tanner, Caroline M; Truong, Daniel; Wei, Peng; Wong, Pei Shieen; Yang, Tianzhong


    Caffeine is neuroprotective in animal models of Parkinson's disease (PD) and caffeine intake is inversely associated with the risk of PD. This association may be influenced by the genotype of GRIN2A, which encodes an NMDA-glutamate-receptor subunit. In two placebo-controlled studies, we detected no association of caffeine intake with the rate of clinical progression of PD, except among subjects taking creatine, for whom higher caffeine intake was associated with more rapid progression. We now have analyzed data from 420 subjects for whom DNA samples and caffeine intake data were available from a placebo-controlled study of creatine in PD. The GRIN2A genotype was not associated with the rate of clinical progression of PD in the placebo group. However, there was a 4-way interaction between GRIN2A genotype, caffeine, creatine and the time since baseline. Among subjects in the creatine group with high levels of caffeine intake, but not among those with low caffeine intake, the GRIN2A T allele was associated with more rapid progression (p=0.03). These data indicate that the deleterious interaction between caffeine and creatine with respect to rate of progression of PD is influenced by GRIN2A genotype. This example of a genetic factor interacting with environmental factors illustrates the complexity of gene-environment interactions in the progression of PD. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. NOAA Form 370 Database (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The data set contains information from submitted NOAA Form 370s, also known as the Fisheries Certificate of Origin, for imported shipments of frozen and/or processed...

  17. Forms of Dystonia (United States)

    ... Fellowship Training Program Dystonia Coalition Dystonia Study Group Global Dystonia Registry About Us Mission & History How Will We Find a Cure? People Newsletters & Press Releases Dystonia Dialogue Financials Contact What is Dystonia? Symptoms & Diagnosis Forms ...

  18. VMS forms Output Tables (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — These output tables contain parsed and format validated data from the various VMS forms that are sent from any given vessel, while at sea, from the VMS devices on...

  19. Benchtop Energetics Progress (United States)


    C – Ionization Region D – Deflection Region E – MCP Ion Detector Use skimmers and differential pumping to form molecular beam. N.C. Blais, H.A. Fry...Simulations Ionization Region MCP Ion Detector Flight Tube Acceleration Grid Fast Neutrals Deflection Electrodes “Vxy” Ionization Region 17DISTRIBUTION A

  20. Rapid eye movement sleep behavior disorder

    DEFF Research Database (Denmark)

    Zoetmulder, Marielle; Jennum, Poul


    and neurological disease. RBD is related to brainstem pathology. Furthermore, it is increasingly recognized that RBD is frequently related to Parkinsonian disorders and narcolepsy. This article reviews recent knowledge about RBD with focus on the diagnostic process and management. Udgivelsesdato: 2009-May......Rapid eye movement (REM) sleep behaviour disorder (RBD) is characterized by loss of REM sleep and related electromyographic atonia with marked muscular activity and dream enactment behaviour. RBD is seen in 0.5% of the population. It occurs in an idiopathic form and secondarily to medical...

  1. Forms of global governence

    Directory of Open Access Journals (Sweden)

    Maxim V. Kharkevich


    Full Text Available Global governance as a concept defines the meaning of contemporary world politics both as a discipline and as reality. Interdependent and globalized world requires governance, and a global government has not been formed yet. The theoretical possibility of global governance without global government is proved and justified. The purpose of this article is to analytically identify possible forms of global governance. Three such forms of global governance are identified: hierarchical, market and network. In a hierarchy the governance is due to the asymmetry of power between the parties. Market control happens via anonymous pricing mechanism. Network, in contrast to the market is characterized by a closer value link between the actors, but unlike the hierarchical relationship actors are free to leave the network. Global governance takes three forms and is being implemented by different actors. To determine the most efficient form of global governance is impossible. Efficiency depends on the match between a form and an object of government. It should be noted that meta governance is likely to remain a monopoly of institutionally strong states in global governance.

  2. Progress in Induction Linacs

    Energy Technology Data Exchange (ETDEWEB)

    Caporaso, G J


    This presentation will be a broad survey of progress in induction technology over the past four years. Much work has been done on accelerators for hydrodynamic test radiography and other applications. Solid-state pulsers have been developed which can provide unprecedented flexibility and precision in pulse format and accelerating voltage for both ion and electron induction machines. Induction linacs can now be built which can operate with MHz repetition rates. Solid-state technology has also made possible the development of fast kickers for precision control of high current beams. New insulator technology has been developed which will improve conventional induction linacs in addition to enabling a new class of high gradient induction linacs.

  3. Progress in nanophotonics 3

    CERN Document Server

    Yatsui, Takashi


    This book focuses on the recent progress in nanophotonics technology to be used to develop novel nano-optical devices, fabrication technology and advanced systems. It reviews light-emitting diodes and lasers made of silicon bulk crystals in which the light emission principle is based on dressed-photon-phonons. Further topics include: theoretical studies of optoelectronic properties of molecular condensates for organic solar cells and light-emitting devices, the basics of topological light beams together with their important properties for laser spectroscopy, spatially localized modes emerging in nonlinear discrete dynamic systems and theoretical methods to explore the dynamics of nanoparticles by the light-induced force of tailored light fields under thermal fluctuations. These topics are reviewed by leading scientists. This overview is a variable resource for engineers and scientists working in the field of nanophotonics.

  4. Muon collider progress

    Energy Technology Data Exchange (ETDEWEB)

    Noble, Robert J. FNAL


    Recent progress in the study of muon colliders is presented. An international collaboration consisting of over 100 individuals is involved in calculations and experiments to demonstrate the feasibility of this new type of lepton collider. Theoretical efforts are now concentrated on low-energy colliders in the 100 to 500 GeV center-of-mass energy range. Credible machine designs are emerging for much of a hypothetical complex from proton source to the final collider. Ionization cooling has been the most difficult part of the concept, and more powerful simulation tools are now in place to develop workable schemes. A collaboration proposal for a muon cooling experiment has been presented to the Fermilab Physics Advisory Committee, and a proposal for a targetry and pion collection channel experiment at Brookhaven National Laboratory is in preparation. Initial proton bunching and space-charge compensation experiments at existing hadron facilities have occurred to demonstrate proton driver feasibility.

  5. Progress in nanophotonics 4

    CERN Document Server

    Yatsui, Takashi


    This book presents the recent progress in the field of nanophotonics. It contains review-like chapters focusing on various but mutually related topics in nanophotonics written by the world’s leading scientists. Following the elaboration of the idea of nanophotonics, much theoretical and experimental work has been carried out, and several novel photonic devices, high-resolution fabrication, highly efficient energy conversion, and novel information processing have been developed in these years. Novel theoretical models describing the nanometric light-matter interaction, nonequilibrium statistical mechanical models for photon breeding processes and near-field‐assisted chemical reactions as well as light‐matter interaction are also explained in this book. It describes dressed photon technology and its applications, including implementation of nanophotonic devices and systems, fabrication methods and performance characteristics of ultrathin, ultraflexible organic light‐emitting diodes, organic solar cells ...

  6. Progress in nanophotonics 2

    CERN Document Server


    This book focuses the recent progress in nanophotonics technology to be used to develop novel nano-optical devices, fabrication technology, and advanced systems. It begins with a review of near-field excitation dynamics in molecules. Further topics include: wavelength up-converting a phonon-assisted excitation process with degenerate beams and non-degenerate beams in dye grains, a fabrication method of semiconductor quantum dots including self-assembly of InAs quantum dots based on the Stranski-Krastanov growth mode, single-nanotube spectroscopy and time-resolved spectroscopy for studying novel excitonic properties of single-walled carbon nanotubes. The striking features of ecxitons in the carbon nanotube, multiple-exciton states, and microfluidic and extended-nano fluidic techniques. These topics are reviewed by nine leading scientists. This overview is a variable resource for engineers and scientists working in the field of nanophotonics.

  7. Current research on progressive familial intrahepatic cholestasis

    Directory of Open Access Journals (Sweden)

    DENG Baocheng


    Full Text Available Progressive familial intrahepatic cholestasis (PFIC refers to a heterogeneous group of autosomal-recessive disorders. The estimated incidence varies between 1/50,000 and 1/100,000 births. Three types of PFIC have been identified and related to mutations in hepatocellular transport system genes involved in bile formation. PFIC-1, PFIC-2, and PFIC-3 are due to mutations in ATP8B1, ABCB11, and ABCB4 genes involved in bile secretion, respectively. Serum gamma-glutamyl transpeptidase is normal in patients with PFIC-1 and PFIC-2, while it is raised in patients with PFIC3. The main clinical manifestation of PFIC is severe intrahepatic cholestasis. PFIC usually appears in infancy or childhood and rapidly progresses to end-stage liver disease before adulthood. Diagnosis of this disease is based on clinical manifestations, liver function tests, liver ultrasonography, liver histology, and genetic testing. Ursodeoxycholic acid therapy is the initial treatment in all PFIC patients to prevent liver damage. In some PFIC1 and PFIC2 patients, biliary diversion may also relieve pruritus and slow disease progression. However, most PFIC patients are ultimately candidates for liver transplantation.

  8. How Rapid is Rapid Prototyping? Analysis of ESPADON Programme Results

    Directory of Open Access Journals (Sweden)

    Ian D. Alston


    Full Text Available New methodologies, engineering processes, and support environments are beginning to emerge for embedded signal processing systems. The main objectives are to enable defence industry to field state-of-the-art products in less time and with lower costs, including retrofits and upgrades, based predominately on commercial off the shelf (COTS components and the model-year concept. One of the cornerstones of the new methodologies is the concept of rapid prototyping. This is the ability to rapidly and seamlessly move from functional design to the architectural design to the implementation, through automatic code generation tools, onto real-time COTS test beds. In this paper, we try to quantify the term “rapid” and provide results, the metrics, from two independent benchmarks, a radar and sonar beamforming application subset. The metrics show that the rapid prototyping process may be sixteen times faster than a conventional process.


    Directory of Open Access Journals (Sweden)

    G.L. Balyasinskaya


    Full Text Available Author discusses advantages of topical antibacterial treatment of inflammatory diseases of upper airways. Advantages of new form of Bioparox (fusafungine are rapid effect from the 1st day of treatment, optimized dosing regimen, additional children’s attachment for nose and increased adherence to therapy. Key words: sinusitis, acute respiratory infections, pharyngitis, treatment, fusafungine.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(5:107-110

  10. Four Decades of Progress (United States)

    Alshishtawy, Moeness M


    Since 1970, Oman has witnessed rapid and significant improvements in health made possible largely because of the country’s success in building a state-of-the-art health care infrastructure. This paper presents Oman’s experience in health development, analyses the whole system, highlights its strengths and delineates the factors to which Oman’s success could be attributed. Furthermore, health care financing and expenditures, health care workforce, the delivery system, quality and other issues are discussed in detail. The paper intends also to study thoroughly the organisation of Oman’s health system and discuss health policy, and planning in Oman. This analysis could serve as a source of information for future investigation and development of the health system in Oman. PMID:21509077

  11. Genetics of monegenic forms of diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Tamara Leonidovna Kuraeva


    Full Text Available It is universally recognized that autoimmune type 1 diabetes mellitus (DM is not the only form of this disease in children. Increasingly more children and adolescents present with DM2, MODY, and rarer syndromal forms of DM. The actual prevalence of DM other than DM1 in children and adolescents is unknown but may be estimated at 10%. Despite rare occurrence of genetic syndromes, they collectively account for almost 5% of DM cases among children. The rapid upgrowth of molecular biology opens up a wide range of possibilities for designating various symptom complexes as nosologically selfconsistent forms. New genetic syndromes associated with DM are annually described. It is important both to adequately identify and treat manifestations and complications of these syndromes in children and to provide relevant medico-genetic counseling and recommendations to the parents. Key words: non-immune diabetes mellitus, MODY, Wolfram syndrome, neonatal, syndromal forms

  12. Rapid-Response Impulsivity: Definitions, Measurement Issues, and Clinical Implications


    Hamilton, Kristen R.; Littlefield, Andrew K.; Anastasio, Noelle C.; Cunningham, Kathryn A.; Fink, Latham H.; Wing, Victoria C.; Mathias, Charles W.; Lane, Scott D; Schutz, Christian; Swann, Alan C.; Lejuez, C.W.; Clark, Luke; Moeller, F. Gerard; Potenza, Marc N.


    Impulsivity is a multi-faceted construct that is a core feature of multiple psychiatric conditions and personality disorders. However, progress in understanding and treating impulsivity in the context of these conditions is limited by a lack of precision and consistency in its definition and assessment. Rapid-response-impulsivity (RRI) represents a tendency toward immediate action that occurs with diminished forethought and is out of context with the present demands of the environment. Expert...

  13. Efficient 3D frequency response modeling with spectral accuracy by the rapid expansion method

    KAUST Repository

    Chu, Chunlei


    Frequency responses of seismic wave propagation can be obtained either by directly solving the frequency domain wave equations or by transforming the time domain wavefields using the Fourier transform. The former approach requires solving systems of linear equations, which becomes progressively difficult to tackle for larger scale models and for higher frequency components. On the contrary, the latter approach can be efficiently implemented using explicit time integration methods in conjunction with running summations as the computation progresses. Commonly used explicit time integration methods correspond to the truncated Taylor series approximations that can cause significant errors for large time steps. The rapid expansion method (REM) uses the Chebyshev expansion and offers an optimal solution to the second-order-in-time wave equations. When applying the Fourier transform to the time domain wavefield solution computed by the REM, we can derive a frequency response modeling formula that has the same form as the original time domain REM equation but with different summation coefficients. In particular, the summation coefficients for the frequency response modeling formula corresponds to the Fourier transform of those for the time domain modeling equation. As a result, we can directly compute frequency responses from the Chebyshev expansion polynomials rather than the time domain wavefield snapshots as do other time domain frequency response modeling methods. When combined with the pseudospectral method in space, this new frequency response modeling method can produce spectrally accurate results with high efficiency. © 2012 Society of Exploration Geophysicists.

  14. Progressive multiple sequence alignments from triplets

    Directory of Open Access Journals (Sweden)

    Stadler Peter F


    Full Text Available Abstract Background The quality of progressive sequence alignments strongly depends on the accuracy of the individual pairwise alignment steps since gaps that are introduced at one step cannot be removed at later aggregation steps. Adjacent insertions and deletions necessarily appear in arbitrary order in pairwise alignments and hence form an unavoidable source of errors. Research Here we present a modified variant of progressive sequence alignments that addresses both issues. Instead of pairwise alignments we use exact dynamic programming to align sequence or profile triples. This avoids a large fractions of the ambiguities arising in pairwise alignments. In the subsequent aggregation steps we follow the logic of the Neighbor-Net algorithm, which constructs a phylogenetic network by step-wisely replacing triples by pairs instead of combining pairs to singletons. To this end the three-way alignments are subdivided into two partial alignments, at which stage all-gap columns are naturally removed. This alleviates the "once a gap, always a gap" problem of progressive alignment procedures. Conclusion The three-way Neighbor-Net based alignment program aln3nn is shown to compare favorably on both protein sequences and nucleic acids sequences to other progressive alignment tools. In the latter case one easily can include scoring terms that consider secondary structure features. Overall, the quality of resulting alignments in general exceeds that of clustalw or other multiple alignments tools even though our software does not included heuristics for context dependent (mismatch scores.

  15. Autonomous motivation, controlled motivation, and goal progress. (United States)

    Koestner, Richard; Otis, Nancy; Powers, Theodore A; Pelletier, Luc; Gagnon, Hugo


    Although the self-concordance of goals has been repeatedly shown to predict better goal progress, recent research suggests potential problems with aggregating autonomous and controlled motivations to form a summary index of self-concordance (Judge, Bono, Erez, & Locke, 2005). The purpose of the present investigation was to further examine the relations among autonomous motivation, controlled motivation, and goal progress to determine the relative importance of autonomous motivation and controlled motivation in the pursuit of personal goals. The results of three studies and a meta-analysis indicated that autonomous motivation was substantially related to goal progress whereas controlled motivation was not. Additionally, the relation of autonomous motivation to goal progress was shown to involve implementation planning. Together, the three studies highlight the importance for goal setters of having autonomous motivation and developing implementation plans, especially ones formulated in terms of approach strategies rather than avoidance strategies. The present research suggests that individuals pursuing goals should focus relatively greater attention on enhancing their autonomous motivation rather than reducing their controlled motivation.

  16. A Rapid Coliform Detector Project (United States)

    National Aeronautics and Space Administration — ORBITEC, in collaboration with Lucigen, proposes a rapid genetic detector for spaceflight water systems to enable real-time detection of E-coli with minimal...

  17. Rapid Multiplex Microbial Detector Project (United States)

    National Aeronautics and Space Administration — ORBITEC, in collaboration with Lucigen, proposes a rapid nucleic acid-based detector for spaceflight water systems to enable simultaneous quantification of multiple...

  18. Sixth form pure mathematics

    CERN Document Server

    Plumpton, C


    Sixth Form Pure Mathematics, Volume 1, Second Edition, is the first of a series of volumes on Pure Mathematics and Theoretical Mechanics for Sixth Form students whose aim is entrance into British and Commonwealth Universities or Technical Colleges. A knowledge of Pure Mathematics up to G.C.E. O-level is assumed and the subject is developed by a concentric treatment in which each new topic is used to illustrate ideas already treated. The major topics of Algebra, Calculus, Coordinate Geometry, and Trigonometry are developed together. This volume covers most of the Pure Mathematics required for t

  19. Circle of Form

    DEFF Research Database (Denmark)

    Jaeger, Thomas Arvid


    of architecture, design, art, art theory, and perception. It was then obvious in related areas to look for systems or models which organized opposites. The colour system has the same fundamental dualistic structure based on complementary colours, on light and dark (black and white), cold and warm. Inspired...... by this model, and using the knowledge gathered from the other areas, especially perception psychology, it showed to be a possible way to organize contrasts in form, a system of 4 different opposites, geometric, organic, mass and structure: The Circle of Form....

  20. PVUSA progress report, 1991

    Energy Technology Data Exchange (ETDEWEB)

    Ellyn, W. [ed.] [Nesbit (William) and Associates, Santa Rosa, CA (United States); Jennings, C. [ed.] [Pacific Gas and Electric Co., San Ramon, CA (United States)


    Photovoltaics for Utility Scale Applications (PVUSA) is a national public-private partnership that is assessing and demonstrating the viability of utility-scale photovoltaic (PV) electric generating systems. PVUSA participants include Pacific Gas & Electric (PG&E), the US Department of Energy (DOE), the Electric Power Research Institute (EPRI), the California Energy Commission (CEC), and eight utilities and other agencies. This report updates the progress of the PVUSA project, reviews the status and performance of the various PV installations during 1991, and summarizes key findings and conclusions from work to date. PVUSA offers utilities hands-on experience needed to evaluate and utilize maturing PV technology. The project also provides manufacturers a test bed for their products, encourages technology improvement and cost reductions in PV modules and other system components, and establishes communication channels between utilities and the PV industry. The project consists of two types of demonstrations: Emerging Module Technology (EMT) arrays, which are unproven but promising state-of-the-art PV technologies in 20-kW (nominal) arrays; and Utility Scale (US) systems, which represent more mature PV technologies in 200- to 500-kW turnkey systems.

  1. Technical progress report

    Energy Technology Data Exchange (ETDEWEB)



    This report summarizes experimental and theoretical work in basic nuclear physics carried out between October 1, 1995, the closing of our last Progress Report, and September 30, 1996 at the Nuclear Physics Laboratory of the University of Colorado, Boulder, under contracts DE-FG03-93ER-40774 and DE-FG03-95ER-40913 with the United States Department of Energy. The experimental contract supports broadly-based experimental research in intermediate energy nuclear physics. This report includes results from studies of Elementary Systems involving the study of the structure of the nucleon via polarized high-energy positron scattering (the HERMES experiment) and lower energy pion scattering from both polarized and unpolarized nucleon targets. Results from pion- and kaon-induced reactions in a variety of nuclear systems are reported under the section heading Meson Reactions; the impact of these and other results on understanding the nucleus is presented in the Nuclear Structure section. In addition, new results from scattering of high-energy electrons (from CEBAF/TJNAF) and pions (from KEK) from a broad range of nuclei are reported in the section on Incoherent Reactions. Finally, the development and performance of detectors produced by the laboratory are described in the section titled Instrumentation.

  2. Geometrical precision of 3DP casting form for founding gears

    Directory of Open Access Journals (Sweden)

    T. Markowski


    Full Text Available The article presents analysis of geometrical precision of casting form made by means of 3DP method. Three dimentional printing is a universal method of rapid prototyping which can be used to make tools – castings forms as a Rapid Tooling method. By means of this method in direct incremental process a casting form of spur gear was created. Precision of creating the form by 3DP method depends on different factors. Technological factors depending on machine operator include: the thickness of the layer and placing the model in working space. The precision of creating the model also depends on preparing the printer for work: the condition and calibration of printing heads, the condition of the printing unit slide guides and preparing the working space. Three dimentional printer Z510 Spectrum was prepared for work in a way assuring the greatest precision of created models. Technological parameters responsible for precision were set on maximum values. The aim of the research was defining the precision of casting form generated by 3DP Rapid Prototyping Method. The research was made by means of coordinate measuring machine Wenzel LH87. The measuring machine software was used to compare the outcome of the measurement to nominal model 3D-CAD. The problem of tool precision of casting forms generated by Rapid Tooling is rarely considered in literature covering rapid prototyping and casting technologies. That is why the research presented in this article isan original work in the technological and practical aspect.

  3. Disability progression in multiple sclerosis is slower than previously reported. (United States)

    Tremlett, Helen; Paty, Donald; Devonshire, Virginia


    To investigate disease progression and risk factors in a large geographically based population with multiple sclerosis (MS), using two different inception points--clinical onset and date of birth. The authors reviewed a database of subjects with definite MS and symptom onset prior to July 1988. The main outcome was sustained progression to Expanded Disability Status Scale (EDSS) 6 (requires a cane), using the date of birth and date of MS onset as inception points in separate analyses. Risk factors examined were sex, relapsing vs primary progressive course, onset age, and onset symptoms. The study included 2,837 patients, followed prospectively for 22,723 patient years. The median time to EDSS 6 was 27.9 years, 15 years after onset; only 21% reached EDSS 6, and by age 50, 28% required a cane. Men progressed 38% more quickly than women from onset (p men and 60.1 for women (p = 0.082). A younger onset age predicted a slower progression, but those older at onset were consistently older when reaching EDSS 6. A primary progressive course predicted a more rapid progression from both onset (p multiple sclerosis (MS) accrued more slowly than found in earlier longitudinal studies. The authors also challenged two fundamental concepts in MS, demonstrating that neither male sex nor older onset age was associated with worse disease outcome.

  4. Compacted Solid Dosage Form

    NARCIS (Netherlands)

    Frijlink, Henderik W.; Hinrichs, Wouter; Grasmeijer, Floris


    The present invention relates to dosage forms comprising a compressed blend of a biologically active ingredient, one or more polymers like a poly(α-hydroxy carboxylic acid) in which optionally is incorporated a glass transition modifying agent, and optional further ingredients, wherein the polymer

  5. Fra tanke til form

    DEFF Research Database (Denmark)

    Der er gået 20 år siden Aalborg og Nordjylland i 1997 fik sin egen, unikke civilingeniøruddannelse i form af Arkitektur & Design-uddannelsen ved Aalborg Universitet. Hele forløbet er nu dokumenteret i en jubilæumsbog, der fortæller om hvordan uddannelsen i sin tid blev etableret; hvordan undervis...

  6. Metal forming and lubrication

    DEFF Research Database (Denmark)

    Bay, Niels


    Lubrication is essential in most metal forming processes. The lubricant film has two basic functions, [1]: i. to separate the work piece and tool surfaces and ii. to cool the workpiece and the tool. Separation of the two surfaces implies lower friction facilitating deformation and lowering the tool...

  7. Many Forms of Culture (United States)

    Cohen, Adam B.


    Psychologists interested in culture have focused primarily on East-West differences in individualism-collectivism, or independent-interdependent self-construal. As important as this dimension is, there are many other forms of culture with many dimensions of cultural variability. Selecting from among the many understudied cultures in psychology,…

  8. Sesotho Address Forms

    Directory of Open Access Journals (Sweden)

    Akindele, Dele Femi


    Full Text Available Address forms constitute an integral part of Basotho sociolinguistic etiquette. They are regarded as a kind of emotional capital that may be invested in putting others at ease. They are indicators of deference, politeness and markers of social distance. (Fasold 1990, Akindele 1990, 1991, 1993 This paper examines the address forms used by the Basotho people. It analyzes and discusses the various types and the factors determining their use. The discussion of address forms in Sesotho focuses on First Name, Title plus First Name, Title plus Last Name, Nickname, Multiple Names, and Teknonym. Drawing data from semi-literate and literate urban and rural population of Maseru district of Lesotho, it was found that the commonest form of address used by the Basotho people is title plus first name. e.g. ntate Thabo (father Thabo, 'm'e Puleng (mother Puleng, ausi Maneo (sister Maneo, abuti Mahao (brother Mahao. It is used by close relations, associates, and familiar people in both formal and informal situations.

  9. Measuring urban form

    NARCIS (Netherlands)

    Berghauser Pont, M.Y.


    For many, the notion of ‘measuring urban form’ will sound disturbing. Urban form is about visual images of cities, experiences, feelings, memories of place, thoughts and intellectual constructs anchored in the realm of the arts and the humanities. Anne Vernez Moudon however gives in the paper

  10. Nanomaterial-enabled Rapid Detection of Water Contaminants. (United States)

    Mao, Shun; Chang, Jingbo; Zhou, Guihua; Chen, Junhong


    Water contaminants, e.g., inorganic chemicals and microorganisms, are critical metrics for water quality monitoring and have significant impacts on human health and plants/organisms living in water. The scope and focus of this review is nanomaterial-based optical, electronic, and electrochemical sensors for rapid detection of water contaminants, e.g., heavy metals, anions, and bacteria. These contaminants are commonly found in different water systems. The importance of water quality monitoring and control demands significant advancement in the detection of contaminants in water because current sensing technologies for water contaminants have limitations. The advantages of nanomaterial-based sensing technologies are highlighted and recent progress on nanomaterial-based sensors for rapid water contaminant detection is discussed. An outlook for future research into this rapidly growing field is also provided. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  11. Final Technical Progress Report

    Energy Technology Data Exchange (ETDEWEB)

    J.Y. Hwang; R.C. Greenlund


    Michigan Technological University has demonstrated major inroads in establishing the viability of utilizing aluminum smelting by-product waste materials in lightweight concrete product applications. The research identified key elements of producing various forms of lightweight concrete products through utilizing various procedures and mixture components with the by-product materials. A process was developed through pilot plant testing that results in additional aluminum recovery at finer sizes, a clean returnable salt product through spray drying technology, and a low-salt-content oxide product with enough aluminum metal content that it can be used to form lightweight cementitious mixtures. Having three distinct products aids in generating favorable process economics. Revenue projections from aluminum recovery and salt recovery are enough to cover processing costs and create a cost-free oxide product to market for lightweight concrete applications. This supply side commercialization strategy offers aluminum by-product recyclers a potentially no cost product, which has been demonstrated through this project to create desirable and marketable lightweight concrete products of various forms. Environmental benefits to the public are tremendous. At best, all dross and salt cake materials have the potential to be completely recycled and utilized. At worst, disposal sites would see a reduced amount of material: a post processed oxide product with little salt and no hydrogen sulfide or ammonia gas generating capability, which, if isolated from high alkali conditions, would pose no reactivity concerns. The US aluminum industry has historically, along with the steel industry, been a leader in recycling metal. The findings from this project, increased metal recovery, improved salt recycling, and demonstrated end uses for oxide residues, will go a long way in helping the aluminum industry obtain 100% material utilization and zero discharge.

  12. Progress on plutonium stabilization

    Energy Technology Data Exchange (ETDEWEB)

    Hurt, D. [Defense Nuclear Facilities Safety Board, Washington, DC (United States)


    The Defense Nuclear Facilities Safety Board has safety oversight responsibility for most of the facilities where unstable forms of plutonium are being processed and packaged for interim storage. The Board has issued recommendations on plutonium stabilization and has has a considerable influence on DOE`s stabilization schedules and priorities. The Board has not made any recommendations on long-term plutonium disposition, although it may get more involved in the future if DOE develops plans to use defense nuclear facilities for disposition activities.

  13. Progressive inhibition of neuromuscular structures (PINS) technique. (United States)

    Dowling, D J


    Progressive inhibition of neuromuscular structures (PINS) is a technique that can be included in the osteopathic manipulative treatment repertoire. It relies on knowledge of anatomy and neuromuscular physiologic features as well as on standard forms of osteopathic palpatory diagnosis and treatment. It is a variant of the inhibition technique that has been taught as an osteopathic manipulative technique for many years, and it bears some resemblance to other manual medicine techniques. The emphasis of the approach is the determination of the alteration of the tissues due to dysfunction, delivering treatment based on palpatory evaluation and patient feedback. Two related points are initially chosen, followed by a progression from one to the other. Relationships to similar techniques are also discussed. Theoretical as well as selected practical applications are presented.

  14. The progression of aortic aneurysms. (United States)

    Mastracci, Tara M


    Aortic aneurysm is a progressive disease that can involve any territory of the aorta. This has ramifications on endovascular repair, because progressive dilation of sealing zones can compromise the integrity of the repair and lead to long term failure. This article describes the evidence behind and some of the predictors of progressive disease of the aorta. Knowledge of predictors of progressive disease may help prevent it from causing failure in the longterm by helping to tailor surveillance routines or have influence on initial graft design.

  15. Populism in Iceland: Has the Progressive Party turned populist?

    Directory of Open Access Journals (Sweden)

    Eiríkur Bergmann


    Full Text Available Though nationalism has always been strong in Iceland, populist political parties did not emerge as a viable force until after the financial crisis of 2008. On wave of the crisis a completely renewed leadership took over the country’s old agrarian party, the Progressive Party (PP, which was rapidly transformed in a more populist direction. Still the PP is perhaps more firmly nationalist than populist. However, when analyzing communicational changes of the new postcrisis leadership it is unavoidable to categorize the party amongst at least the softer version of European populist parties, perhaps closest to the Norwegian Progress Party.

  16. 2004 Progress Report

    Energy Technology Data Exchange (ETDEWEB)

    Batistoni, Paola; De Marco, Francesco; Pieroni, Leonardo (ed.)


    Fusion research is undertaken all over the world with the objective of realising an environmentally responsible source of energy with essentially unlimited and widely distributed fuel reserves. The results of the worldwide efforts made in recent years are now embodied in ITER, the International Thermonuclear Experimental Reactor, designed to produce at least 500 MW of fusion power with a power gain of ten. ITER will test for the first time the interaction of fusion plasma physics with power station technology. In this international framework, during 2004 Fusion Technical and Scientific Unit of ENEA obtained important results in several keys areas. At the Frascati Tokamak Upgrade the lower hybrid microwave system was fully exploited to study the generation and control of the plasma current, and the electron cyclotron heating system reached full power (1.5 MW). With the simultaneous injection of the two waves, good energy confinement regimes with internal transport barriers were obtained at the highest plasma densities ever achieved. Advanced scenario regimes were also addressed in the activities of ENEA at JET. The engineering design of the IGNITOR machine was finalised, and significant progress was made in understanding the plasma physics regimes. Among the technology activities, the qualification of the deposition process of a copper liner on carbon fibre composite (CFC) hollow tiles may be mentioned as the most important achievement. This innovative pre brazed casting process is a competitive candidate for the fabrication of the CFCbased ITER divertor components. ENEA participated in the European activity for the definition and production on an industrial scale of an advanced Nb3Sn strand for the ITER superconducting central solenoid and toroidal field coils. Contributions were also made to the design of the final conductor layout and the characterisation tests. Inertial fusion studies continued along the previous lines, namely, the study of the implosion

  17. The Thermochronologist's Progress (United States)

    Zeitler, P. K.


    We owe our current understanding of thermochronology less to a series of revolutionary insights than to a somewhat uneven intellectual pilgrimage that over fifty years has progressed in fits and starts. Though hampered at times by overenthusiasm, oversimplification, and misunderstandings, on balance the field advanced thanks to a blend of curiosity-driven research, tool-building motivated by new ideas about Earth science, and improvements in technology. But now that we've exploited most radiogenic systems and the major minerals that host them, and now that our models can devour CPU time along with the best of them, are we done? Have we reached peak thermochron? The answer of course is no, and papers in this session will demonstrate what new technologies and techniques might have to offer in the coming years. However, I will argue that the discipline as a whole has matured to a point where if thermochronology is to remain a mainstream tool as opposed to a weekend sport, we need to get serious about several challenges. The most fundamental challenge is that current geodynamic models (and even more complex models we can envision coding) have outpaced our meagre stockpile of kinetic calibrations, our understanding of detailed isotope systematics, and our ability to generate data with sufficient throughput. These issues will not be addressed adequately through the business-as-usual approach that brought us to our current knowledge, and some community effort will probably be needed to coordinate the hard work that will be required. But any serious attempt to answer important questions with accurate thermal histories that have low and well-defined uncertainties will require that we actually know the kinetics for the specific samples we are analyzing, that we fully understand scatter in the data, that we work with the large sample numbers that are required for some problems like landscape evolution, and that inversion tools fully explore the important aspects of both the

  18. 1997 Progress report

    Energy Technology Data Exchange (ETDEWEB)

    Cecchini, M.; Crescentini, L.; Ghezzi, L. [ENEA, Centro Ricerche Frascati, Rome (Italy). Nuclear fusion division


    1997 was another year of intense activity for the ENEA Nuclear Fusion Division in the evolving scenario of fusion research. With respect to the International Thermonuclear Experimental Reactor (ITER), a major review process has started, originate by the wide perception that the difficult financial situation affecting some of the parties would make it very difficult, practically impossible, to secure funding for the project as it stands. To scale down the size and cost of the machine by reducing the technical objectives, while keeping to the programmatic goal of constructing a demonstration reactor (DEMO) as the following step, appears achievable. Progress in physics was substantial during 1997. Analysis of the huge existing database, complemented by the latest results, had led to a better, more accurate scaling for the confinement time on which to base extrapolation to ITER. Studies of the very promising advanced regimes have been pursued on many tokamaks. The Frascati Tokamak Upgrade (FTU) is well placed in this respect since it is equipped with the right tools, lower hybrid and electron cyclotron radiofrequency heating and current drive systems, to explore the new promised land of low transport from the plasma core. The main highlights of FTU operation in 1997 were related to providing information relevant to these future developments. Transient production of low transport regimes with electron temperatures of 8-9 keV at the plasma center was obtained by taking advantage of the plasma skin effect and precise electron cyclotron power deposition. High-efficiency current drive at high density using lower hybrid waves was demonstrated. High-confinement pellet-enhanced modes of operation and good ion Bernstein wave coupling through the waveguide-type coupler were also achieved. Concerning the IGNITOR experiment, funds were made available only for continuation of the engineering design activities, and nothing has been released so far for manufacturing the additional

  19. Progress Report for 1947

    Energy Technology Data Exchange (ETDEWEB)

    Authors, Various


    The year 1947 has witnessed the dawn of a new era of atomic science, a flowering of fundamental knowledge of the nature of matter which appears to be unsurpassed even by that period of the 1930's which led to the age of plutonium. A great new cyclotron, an atom-smasher ten times more powerful than the one which brought plutonium into the world, has carried mankind over a new horizon of sub-atomic space. It has brought scientists at last to grips with the infinitely small and rapid forces, until now beyond reach, which operate within the incredibly tiny distances of nuclear space. On the new energy frontier created by the giant machine, now laws govern nuclear reactions. methods are at hand, heretofore unavailable, which permit the measurement and determination of the nature of sub-atomic forces. Under ultra-high energy bombardment, the nucleus presents a different appearance from the nucleus of Bohr and Rutherford, the nucleus of atomic energy fission. The new exploration of the atom has been sponsored by the Atomic Energy Commission with the giant, new 4000-ton cyclotron in the Radiation Laboratory of the University of California. This is the thirdmajor machine built by the Director of the Laboratory and inventor of the cyclotron, Professor Ernest O. Lawrence. Whether the new knowledge will be of immediate practical consequence cannot now be predicted. Nor could Professor Lawrence predict, when in 1934 he established a new atomic energy range for that day with his first cyclotron, that the fundamental knowledge he pursued would be climaxed with the discovery of plutonium. What can be predicted is this: without the new basic knowledge, practical atomic developments of the future would be limited to the applicability of the fundamental information which made possible the initial release of atomic energy. In short, the nation's atomic potential has been greatly expanded.

  20. Progressive transcortical sensory aphasia and progressive ideational apraxia owing to temporoparietal cortical atrophy. (United States)

    Funayama, Michitaka; Nakajima, Asuka


    In contrast to frontotemporal lobar degeneration, atrophy of the focal posterior lateral cortex has not been thoroughly studied. Three clinical types of focal cortical atrophy have been described: 1) logopenic variant of primary progressive aphasia, which presents with impaired repetition despite normal articulation; 2) posterior cortical atrophy, which presents with prominent visuospatial deficits; and 3) primary progressive apraxia. All three clinical types are characterized by specific patterns of hypometabolism/hypoperfusion: the left posterior perisylvian area in the logopenic variant of primary progressive aphasia, bilateral parietooccipital areas in posterior cortical atrophy, and the parietal cortex in primary progressive apraxia. However, not every patient clearly fits into one of these categories. Here we describe two patients with atypical focal cortical presentations. They presented with a history of a few years of progressive transcortical sensory aphasia characterized by fluent output with normal grammar and syntax, normal repetition, sentence comprehension deficits, and anomia without loss of word meaning. They also presented with progressive apraxia that began at the initial stages. Some forms of posterior symptoms including acalculia, agraphia, and visuospatial deficits were also observed. Hypoperfusion was noted mainly in the left temporoparietal region, which is slightly posterior to the perisylvian area. Although our cases lack in CSF findings and PIB scan, these two cases and previous reports might suggest the existence of a subgroup of patients presenting with transcortical sensory aphasia, apraxia, and posterior symptoms (acalculia, agraphia, and visuospatial deficits) in the setting of Alzheimer's disease. This subgroup may reflect the spectrum of clinical manifestations between logopenic variant of primary progressive aphasia and posterior cortical atrophy.

  1. [Typical forms of schizophrenia in old age]. (United States)

    Sternberg, E; Konzewoi, V


    Aside from typical forms of late schizophrenia which generally conform to the definition given by M. Bleuler, there also are psychoses appearing in old age which differ significantly from the atypical symptoms and consequently present certain diagnostic difficulties. This report contains descriptions of late manifestations in schizophrenic psychoses, which develop with a continuous or assault-like course with a prevalence of parnoial disorders. Paranoid delusions, in such cases, are characterized by aging traits (concrete and short-term delusions, exaggeration of the degree of superficial persecution and prejudice, and a limited number of people involved in the delusions). The development of such forms of late schizophrenia takes a slowly progressing course. The results of these studies, especially the psychopathological symptomatology, the genetic-constitutional background and the development and outcome of these psychoses are analyzed in detail. The data permit to consider such forms of psychose as atypical variants of late schizophrenia.

  2. The Form of Gentrification

    CERN Document Server

    Venerandi, Alessandro; Romice, Ombretta; Porta, Sergio


    Many socioeconomic studies have been carried out to explain the phenomenon of gentrification. Although results of these works shed light on the process around this phenomenon, a perspective which focuses on the relationship between city form and gentrification is still missing. With this paper we try to address this gap by studying and comparing, through classic methods of mathematical statistics, morphological features of five London gentrified neighbourhoods. Outcomes confirm that areas which have undergone gentrification display similar and recognizable morphological patterns in terms of urban type and geographical location of main and local roads as well as businesses. These initial results confirm findings from previous research in urban sociology, and highlight the role of urban form in contributing to shape dynamics of non-spatial nature in cities.

  3. Formed HIP Can Processing

    Energy Technology Data Exchange (ETDEWEB)

    Clarke, Kester Diederik [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)


    The intent of this report is to document a procedure used at LANL for HIP bonding aluminum cladding to U-10Mo fuel foils using a formed HIP can for the Domestic Reactor Conversion program in the NNSA Office of Material, Management and Minimization, and provide some details that may not have been published elsewhere. The HIP process is based on the procedures that have been used to develop the formed HIP can process, including the baseline process developed at Idaho National Laboratory (INL). The HIP bonding cladding process development is summarized in the listed references. Further iterations with Babcock & Wilcox (B&W) to refine the process to meet production and facility requirements is expected.

  4. Wars of Forms

    DEFF Research Database (Denmark)

    Clausen, Lars


    , both political and military, war between the two forms, the post-napoleonic, Fichtean notion of nationality (1807-8) and the historical notion of imperium. “Nationality” entered the political semantics witch such a force and shook the existing political order of empires to the ground because of its...... households and fragments of earlier political empires, such as the Danish Realm (rigsfællesskab) with Greenland, The Faroe Islands and the Danish State, poses the important question: how did the replacement and re-formation of the European political system happen? I argue, that we can observe a semantic...... and apply appropriate forms of response. To unravel the two complex cases of political ‘management’ of the rise of german nationality, I have to open up the semantic complex of “crown”, “state”, “kingdom”, prince and government, as they are used in governmental, internal communications, dispatches...

  5. Tube-Forming Assays. (United States)

    Brown, Ryan M; Meah, Christopher J; Heath, Victoria L; Styles, Iain B; Bicknell, Roy


    Angiogenesis involves the generation of new blood vessels from the existing vasculature and is dependent on many growth factors and signaling events. In vivo angiogenesis is dynamic and complex, meaning assays are commonly utilized to explore specific targets for research into this area. Tube-forming assays offer an excellent overview of the molecular processes in angiogenesis. The Matrigel tube forming assay is a simple-to-implement but powerful tool for identifying biomolecules involved in angiogenesis. A detailed experimental protocol on the implementation of the assay is described in conjunction with an in-depth review of methods that can be applied to the analysis of the tube formation. In addition, an ImageJ plug-in is presented which allows automatic quantification of tube images reducing analysis times while removing user bias and subjectivity.

  6. How Stars Form (United States)

    McKee, Christopher F.


    Stars are the atoms of the universe. The process by which stars form is at the nexus of astrophysics since they are believed to be responsible for the re-ionization of the universe, they created the heavy elements, they play a central role in the formation and evolution of galaxies, and their formation naturally leads to the formation of planets. Whereas early work on star formation was based on the assumption that it is a quiescent process, it is now believed that turbulence plays a dominant role. In this overview, I shall discuss the evolution of our understanding of how stars form and current ideas about the stellar initial mass function and the rate of star formation.

  7. Incremental Forming Process

    Directory of Open Access Journals (Sweden)

    Adrian Blaga


    Full Text Available This paper comprises an experimental study for a complex geometry part obtained by incremental forming. Due to the process complexity (the presence of forces on three directions—a vertical one and two in the blank's plane, a three axes CNC milling machine, capable of describing the complex paths covered by the punch for obtaining the truncated cone-shaped parts, has been chosen. To obtain a truncated cone, three different trajectories were selected: in first and second variants after each vertical press having a constant step, the punch covers a circular path. The differences show that the following circular trajectory can start at the same point or can be shifted at an angle of 90° from the previous press point. In the last variant, the punch performs a spatial spiral trajectory. The main objective of our study was to determine the optimal forming strategy, by shifting the press position of the punch and the path it follows to obtain a truncated cone through single point incremental forming. Thus, the strain distribution can be homogeneous, and the thickness reduction and the process forces are minimal.

  8. Forms of Life

    Directory of Open Access Journals (Sweden)

    Peter Hacker


    Full Text Available The phrase ‘Lebensform’ (form of life had a long and varied history prior to Wittgenstein’s use of it on a mere three occasions in the Philosophical Investigations. It is not a pivotal concept in Wittgenstein’s philosophy. But it is a minor signpost of a major reorientation of philosophy, philosophy of language and logic, and philosophy of mathematics that Wittgenstein instigated. For Wittgenstein sought to replace the conception of a language as a meaning calculus (Frege, Russell, the Tractatus by an anthropological or ethnological conception. A language is not a class of sentences that can be formed from a set of axioms (definitions, formation and transformation rules and the meanings of which is given by their truth-conditions, but an open-ended series of interlocking language-games constituting a form of life or way of living (a culture. Wittgenstein’s uses of ‘Lebensform’ and its cognates, both in the Investigations and in his Nachlass are severally analysed, and various exegetical misinterpretations are clarified.

  9. Progressive dysarthria and ataxia

    Directory of Open Access Journals (Sweden)

    Lynsey McAlpine


    Full Text Available ‘Guillain-Barre syndrome’ (GBS is a broad term used to describe a collection of clinical syndromes which manifest as acute immune-mediated demyelinating diseases or more rarely axonal diseases of the peripheral nervous system. The most commonly recognised form is ‘acute inflammatory demyelinating polyradiculoneuropathy’ (AIDP, which classically presents as a proximal and distal weakness with diminished reflexes, often involving the cranial nerves and muscles of respiration [1]. It is a neurological emergency as these patients are at risk of developing respiratory failure; one third will require admission to the Intensive Care Unit (ICU for ventilatory support, and mortality rates of 3-10% have been reported

  10. [Research progresses on nutrigenomics]. (United States)

    Chen, Qin; Wang, Wen-Jun; Shangguan, Xin-Chen; Xu, Ming-Sheng


    Nutrition science is an age-old subject, and offers important theoretic instructions for human health protection and disease prevention. With the development of molecular biology, it will be a key technique in the 21 century. Combination of molecular biology and nutrition, bio-nutrition is formed. While the combination of genome and nutrition, nutrigenomics is developed. Nutrigenomics covers a wide range of areas, which studies the interactive effects between nutrients and genes expression, and predicts the expressive response to the nutrients. Genomics technology can help us to identify some dis-ease-related genes, thereby people establish individual diet and make their heath attained the best status through adjusting diet. This paper focuses on the main research technology of nutrigenomics and its application.

  11. Process for Forming a High Temperature Single Crystal Canted Spring (United States)

    DeMange, Jeffrey J (Inventor); Ritzert, Frank J (Inventor); Nathal, Michael V (Inventor); Dunlap, Patrick H (Inventor); Steinetz, Bruce M (Inventor)


    A process for forming a high temperature single crystal canted spring is provided. In one embodiment, the process includes fabricating configurations of a rapid prototype spring to fabricate a sacrificial mold pattern to create a ceramic mold and casting a canted coiled spring to form at least one canted coil spring configuration based on the ceramic mold. The high temperature single crystal canted spring is formed from a nickel-based alloy containing rhenium using the at least one coil spring configuration.

  12. Arithmetic of p-adic modular forms

    CERN Document Server

    Gouvêa, Fernando Quadros


    The central topic of this research monograph is the relation between p-adic modular forms and p-adic Galois representations, and in particular the theory of deformations of Galois representations recently introduced by Mazur. The classical theory of modular forms is assumed known to the reader, but the p-adic theory is reviewed in detail, with ample intuitive and heuristic discussion, so that the book will serve as a convenient point of entry to research in that area. The results on the U operator and on Galois representations are new, and will be of interest even to the experts. A list of further problems in the field is included to guide the beginner in his research. The book will thus be of interest to number theorists who wish to learn about p-adic modular forms, leading them rapidly to interesting research, and also to the specialists in the subject.

  13. The Form Factors of the Nucleons

    Energy Technology Data Exchange (ETDEWEB)

    Perdrisat, Charles F. [William and Mary College, JLAB


    There has been much activity in the measurement of the elastic electromagnetic proton and neutron form factors in the last decade, and the quality of the data has been greatly improved by performing double-polarization experiments, in comparison with with pre-vious unpolarized cross section data. Here we will review the experimental data base in view of the new results for the proton and the neutron, obtained at MIT-Bates, JLab and MAMI. The rapid evolution of phenomenological models triggered by these high- precision experiments will be discussed. In particular, the possibility that the proton is non-spherical in its ground state, and that the transverse charge density are model in- dependently defined in the infinite momentum frame. Likewise, flavor decomposition of the nucleon form factors into dressed u and d quark form factors, may give information about the quark-diquark structure of the nucleon. The current proton radius "crisis" will also be discussed.

  14. Structural MRI correlates of amyotrophic lateral sclerosis progression. (United States)

    Senda, Joe; Atsuta, Naoki; Watanabe, Hirohisa; Bagarinao, Epifanio; Imai, Kazunori; Yokoi, Daichi; Riku, Yuichi; Masuda, Michihito; Nakamura, Ryoichi; Watanabe, Hazuki; Ito, Mizuki; Katsuno, Masahisa; Naganawa, Shinji; Sobue, Gen


    Amyotrophic lateral sclerosis (ALS) presents with varying degrees of brain degeneration that can extend beyond the corticospinal tract (CST). Furthermore, the clinical course and progression of ALS varies widely. Brain degeneration detected using structural MRI could reflect disease progression. On study registration, 3-Tesla volumetric MRI and diffusion tensor imaging scans were obtained at baseline in 38 healthy controls and 67 patients with sporadic ALS. Patients had Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) scores of ≥36 and did not have the chromosome 9, open reading frame 72 repeat expansion. Six months later, changes in ALSFRS-R (ΔALSFRS-R) scores were calculated and patients were grouped into three categories, namely, patients with slow progression with ΔALSFRS-R scores ≤3 (n=19), intermediate progression with ΔALSFRS-R scores =4, 5 and 6 (n=36) and rapid progression with ΔALSFRS-R scores ≥7 (n=12). We analysed voxel-based morphometry and tract-based spatial statistics among these subgroups and controls. In comparison with controls, patients with ALS showed grey matter atrophy and decreased fractional anisotropy beyond the motor cortex and CST, especially in the frontotemporal lobes and basal ganglia. Moreover, the degree of change was highly proportional to ΔALSFRS-R at the 6-month assessment. A more rapid disease progression and poorer functional decline were associated with greater involvement of the extra-motor cortex and basal ganglia, suggesting that the spatial extent of brain involvement can be an indicator of the progression in ALS. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  15. [Progress and countermeasures of Dendrobium officinale breeding]. (United States)

    Si, Jin-Ping; He, Bo-wei; Yu, Qiao-xian


    The standandized cultivation of Chinese medicinal materials is based on variety. With the rapid development of Dendrobium officinale industry and increasing demand of improved varieties, many studies have concentrated on the variety breeding of D. officinale and subsequently achieved remarkable success. This paper systematically expounds the research progress of D. officinale breeding, e. g. the collection and differentiated evaluation for germplasm, theory and practice for variety breeding, tissue culture and efficient production with low-carbon for germchit, and DNA molecular marker-assisted breeding, and then indicates the main problems of the current breeding of D. officinale. Furthermore, the priorities and keys for the further breeding of D. officinale have been pointed out.

  16. Recent Progress in Understanding Solar Magnetic Reconnection

    Directory of Open Access Journals (Sweden)

    Jeongwoo Lee


    Full Text Available Magnetic reconnection is a fundamental process occurring in a wide range of astrophysical, heliospheric and laboratory plasmas. This process alters magnetic topology and triggers rapid conversion of magnetic energy into thermal heating and nonthermal particle acceleration. Efforts to understand the physics of magnetic reconnection have been made across multiple disciplines using remote observations of solar flares and in-situ measurements of geomagnetic storms and substorms as well as laboratory and numerical experiments. This review focuses on the progress achieved with solar flare observations in which most reconnection-related signatures could be resolved in both space and time. The emphasis is on various observable emission features in the low solar atmosphere which manifest the coronal magnetic reconnection because these two regions are magnetically connected to each other. The research and application perspectives of solar magnetic reconnection are briefly discussed and compared with those in other plasma environments.

  17. Saudi National Assessment of Educational Progress (SNAEP

    Directory of Open Access Journals (Sweden)

    Abdullah Saleh Al Sadaawi


    Full Text Available To provide a universal basic education, Saudi Arabia initially employed a rapid quantitative educational strategy, later developing a qualitative focus to improve standards of education delivery and quality of student outcomes. Despite generous resources provided for education, however, there is no national assessment system to provide statistical evidence on students’ learning outcomes. Educators are querying the curricula and quality of delivery for Saudi education, especially following low student performances on the Trends in International Mathematics and Science Study (TIMSS in 2003 and 2007. There is a growing demand for national assessment standards for all key subject areas to monitor students’ learning progress. This study acknowledges extant research on this important topic and offers a strategy of national assessment to guide educational reform.

  18. Recent progress on the ATHENA Positron Accumulator

    CERN Document Server

    Jørgensen, L V; Watson, T L; Charlton, M; Collier, M


    The Positron Accumulator for the ATHENA anti-hydrogen experiment at CERN, Geneva has recently been upgraded with a new 50 mCi /sup 22/Na beta /sup +/-radioactive source. Following this, rapid progress has been made in optimizing and characterizing the properties of the positron plasma. The rotating wall technique has also been implemented in the accumulation region and has been shown to lead to compression of better than a factor of 10 in density and markedly increased lifetimes, even when using the N/sub 2/ buffer gas as a cooling gas. Using these techniques we have routinely accumulated up to 2 * 10/sup 8/ positrons in a few minutes. The positron plasma has a FWHM of only 3-4 mm when using the rotating wall which compares with a FWHM of 15 mm without the rotating wall. (13 refs).


    Directory of Open Access Journals (Sweden)

    Mallamaci F


    Conclusions: Plasma procalcitonin is a more sensitive biomarker of innate immunity than CRP in CKD patients and in part reflects excessive adiposity. High PCT in CKD patients predicts progression toward kidney failure. These results are compatible with the hypothesis that alterations in innate immunity play a role in the progression of CKD in humans.

  20. Scientific Progress in Strategic Management

    DEFF Research Database (Denmark)

    Foss, Nicolai

    Does the RBV represent a case of scientific progress? And has it emerged as the dominant approach to the analysis of competitive advantage for this reason? Conventional criteria for scientific progress, notably those of the growth of knowledge literature, are not particularly helpful for understa...

  1. Composites by rapid prototyping technology

    CSIR Research Space (South Africa)

    Kumar, S


    Full Text Available powder is a fiber, problems of manufacturing occur. The method has also been used to make Metal Matrix Composite (MMC), e.g Fe and graphite [17], WC-Co [18,19], WC-Co and Cu [20,21], Fe, Ni and TiC [22] etc and Ceramic Matrix Composite (CMC) e.g. Si... of various materials used. Key words: : Rapid Prototyping (RP), Laser, Composites 1 Introduction Rapid Prototyping (RP) initially focussed on polymers. These were later re- placed/supplemented by ceramics, metals and composites. Composites are used in RP...

  2. Progressive multifocal leukoencephalopathy limited to the brain stem

    Energy Technology Data Exchange (ETDEWEB)

    Kastrup, O.; Maschke, M.; Diener, H.C. [Neurologische Universitaetsklinik, University of Essen (Germany); Wanke, I. [Department of Neuroradiology, University of Essen (Germany)


    Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating slow-virus encephalitis caused by the JC polyomavirus in 2-5% of patients with AIDS. MRI typically shows multiple lesions in the cerebral hemispheres. We present a rare case of rapidly evolving and lethal PML with a severe bulbar syndrome and spastic tetraparesis in a patient with AIDS. MRI showed high-signal lesions on T2-weighted images confined to the brain stem, extending from the medulla oblongata to the midbrain. JC virus polymerase chain reaction in cerebrospinal fluid was positive, and neuropathology showed the findings of PML. This case was also notable because of the rapid progression despite improved immune status with antiretroviral therapy. (orig.)

  3. Progressive outer retinal necrosis presenting as cherry red spot. (United States)

    Yiu, Glenn; Young, Lucy H


    To report a case of progressive outer retinal necrosis (PORN) presenting as a cherry red spot. Case report. A 53-year-old woman with recently diagnosed HIV and varicella-zoster virus (VZV) aseptic meningitis developed rapid sequential vision loss in both eyes over 2 months. Her exam showed a "cherry red spot" in both maculae with peripheral atrophy and pigmentary changes, consistent with PORN. Due to her late presentation and the rapid progression of her condition, she quickly developed end-stage vision loss in both eyes. PORN should be considered within the differential diagnosis of a "cherry red spot." Immune-deficient patients with a history of herpetic infection who present with visual loss warrant prompt ophthalmological evaluation.

  4. Constitutive integrin activation on tumor cells contributes to progression of leptomeningeal metastases1 (United States)

    Brandsma, Dieta; Ulfman, Laurien; Reijneveld, Jaap C.; Bracke, Madelon; Taphoorn, Martin J.B.; Zwaginga, Jaap Jan; Gebbink, Martijn F.B.; de Boer, Hetty; Koenderman, Leo; Voest, Emile E.


    Leptomeningeal metastases are a serious neurological complication in cancer patients and associated with a dismal prognosis. Tumor cells that enter the subarachnoid space adhere to the leptomeninges and form tumor deposits. It is largely unknown which adhesion molecules mediate tumor cell adhesion to leptomeninges. We studied the role of integrin expression and activation in the progression of leptomeningeal metastases. For this study, we used a mouse acute lymphocytic leukemic cell line that was grown in suspension (L1210-S cell line) to develop an adherent L1210 cell line (L1210-A) by selectively culturing the few adherent cells in the cell culture. β1, β2, and β3 integrins were in a constitutively high active state on L1210-A cells and in a low, but inducible, active state on L1210-S cells. Expression levels of these integrins were comparable in the two cell lines. Static adhesion levels of L1210-A cells on a leptomeningeal cell layer were significantly higher than those of L1210-S cells. All mice that were injected intrathecally with L1210-A cells died rapidly of leptomeningeal leukemia. In contrast, 45% long-term survival was seen after intrathecal injection of mice with L1210-S cells. Our data indicate that constitutive integrin activation on leukemic cells promotes progression of leptomeningeal leukemia by increased tumor cell adhesion to the leptomeninges. We argue that an aberrantly regulated inside-out signaling pathway underlies constitutive integrin activation on the adherent leukemic cell population. PMID:16533879

  5. Progression of High Anisometropia in Children. (United States)

    Zedan, Rasha H; El-Fayoumi, Dina; Awadein, Ahmed


    To investigate the onset and rate of progression of high anisometropia in myopic children younger than 13 years. A retrospective study was performed on children with anisometropia younger than 13 years with myopia of more than 4.00 diopters (D) in the more ametropic eye and a difference in spherical equivalent refraction of 4.00 D between both eyes. All children had a complete ophthalmologic examination, including measurement of visual acuity and cycloplegic refraction every 3 to 6 months for at least 5 years. Change in the spherical equivalent and the cylindrical error for both eyes and changes in the difference in spherical equivalent refraction between both eyes were calculated for each patient at each visit. Linear, polynomial, logarithmic, and exponential fitting models were tested for both eyes and for the anisometropic difference between both eyes. The regression line with the greatest R2 value was considered best fit. Sixty-three patients fulfilled the inclusion criteria. The more ametropic eye grew in a regular fashion during the first 2 years of life, followed by a rapid decrease in the rate of growth to become almost stable after 4 years of age. The increase in myopia best fit a third-degree polynomial (cubic) model (R2 = 0.98). The less ametropic eye showed only a small increase in myopia during the follow-up period. The anisometropic difference between both eyes increased gradually during the first 2 years, then remained stable. High anisometropic myopia progresses rapidly in the first few years of life before becoming stable. [J Pediatr Ophthalmol Strabismus. 2017;54(5):282-286.]. Copyright 2017, SLACK Incorporated.

  6. Autologous hematopoietic stem cell transplantation in combination with immunoablative protocol in secondary progressive multiple sclerosis: A 10-year follow-up of the first transplanted patient

    Directory of Open Access Journals (Sweden)

    Obradović Dragana


    Full Text Available Introduction. Multiple sclerosis (MS is an immunemediated disease of the central nervous system that affects young individuals and leads to severe disability. High dose immunoablation followed by autologous hemopoietic stem cell transplantation (AHSCT has been considered in the last 15 years as potentialy effective therapeutic approach for agressive MS. The most recent long-time follow-up results suggest that AHSCT is not only effective for highly aggressive MS, but for relapsing-remitting MS as well, providing long-term remission, or maybe even cure. We presented a 10- year follow-up of the first MS patient being treated by immunoablation therapy and AHSCT. Case report. A 27-year-old male experienced the first symptoms - intermitent numbness and paresthesia of arms and legs of what was treated for two years by psychiatrist as anxiety disorder. After he developed severe paraparesis he was admitted to the Neurology Clinic and diagnosed with MS. Our patient developed aggressive MS with frequent relapses, rapid disability progression and transition to secondary progressive form 6 years after MS onset [the Expanded Disability Status Scale (EDSS 7.0 Ambulation Index (AI 7]. AHSCT was performed, cyclophosphamide was used for hemopoietic stem cell mobilization and the BEAM protocol was used as conditionig regimen. No major adverse events followed the AHSCT. Neurological impairment improved, EDSS 6.5, AI 6 and during a 10-year followup remained unchanged. Brain MRI follow-up showed the absence of gadolinium enhancing lesions and a mild progression of brain atrophy. Conclusion. The patient with rapidly evolving, aggressive, noninflammatory MS initialy improved and remained stable, without disability progression for 10 years, after AHSCT. This kind of treatment should be considered in aggressive MS, or in disease modifying treatment nonresponsive MS patients, since appropriately timed AHSCT treatment may not only prevent disability progression but reduce

  7. Research of Automatic Progress Report Generation for Railway Construction Projects in China

    Directory of Open Access Journals (Sweden)

    Qing Li


    Full Text Available The rapid construction of railways in China has posed tremendous challenges for managing railway construction projects, especially their progress. Frequently changed schedule, complicated contents, and error-prone data have practically hampered the efforts to automatically generate progress reports on railway construction projects within the country. In this paper, we set out to explore the linkages among current data from Chinese railway construction units on inspection lots, construction drawings, and construction schedules, which are used to establish an engineering quantity computation model and automatic project progress report model. An automatic progress report generation system for railway construction projects was developed to generate a wide range of standard progress reports. Practical applications showed that the proposed system offers an alternative to hardware-based methods of progress report generation and can significantly improve the accuracy of data and the quality of management regarding project progress.

  8. Influence of quench rates on the properties of rapidly solidified ...

    Indian Academy of Sciences (India)


    A K PANDA, I CHATTORAJ, S BASU* and A MITRA. National Metallurgical Laboratory, Jamshedpur 831 007, India. *Materials Science Centre, Indian Institute of Technology, Kharagpur 721 302, India. Abstract. FeNbCuSiB based materials were produced in the form of ribbons by rapid solidification techniques.

  9. Quantum mechanics of rapidly and periodically driven systems

    Indian Academy of Sciences (India)

    Rapid forcing; Kapitza pendulum; effective Hamiltonian; quadrupole and. Paul traps; strong-field excitations. PACS Nos 42.50.Hz; 42.50.Ct; 32.80.Lg. 1. Introduction .... (10) in mind, eq. (13) provides a surprisingly simple derivation of the form of the effective potential, as given in eq. (8). Although the treatment here is.

  10. Rapid neutron capture process in supernovae and chemical element formation

    NARCIS (Netherlands)

    Baruah, Rulee; Duorah, Kalpana; Duorah, H. L.


    The rapid neutron capture process (r-process) is one of the major nucleosynthesis processes responsible for the synthesis of heavy nuclei beyond iron. Isotopes beyond Fe are most exclusively formed in neutron capture processes and more heavier ones are produced by the r-process. Approximately half

  11. A rapid and convergent synthesis of the integrastatin core

    KAUST Repository

    Tadross, Pamela M.


    The tetracyclic core of the integrastatin natural products has been prepared in a convergent and rapid manner. Our strategy relies upon a palladium(ii)-catalyzed oxidative cyclization to form the central [3.3.1]-dioxabicycle of the natural product core. Overall, the core has been completed in only 4 linear steps from known compounds. © 2011 The Royal Society of Chemistry.

  12. Managing in the rapidly changing context of higher education: a ...

    African Journals Online (AJOL)

    Higher education is one of the most rapidly changing sectors of our society. Besides the rate of change in the sector there are also, as seen from the continuous media coverage, a number of universities and technikons in some form of financial or leadership crisis. Over the past years one of the main reasons given for these ...

  13. Rapid urbanisation and slum upgrading : what can land surveyors do?

    NARCIS (Netherlands)

    van der Molen, P.


    This paper aims at understanding the domain of rapid urbanisation and slum upgrading. Therefore it collects facts in order to clarify the status quo. The paper highlights relevant aspects, such as development of new forms of spatial planning, modern slum upgrading methods, provision of security to

  14. Influence of quench rates on the properties of rapidly solidified ...

    Indian Academy of Sciences (India)

    FeNbCuSiB based materials were produced in the form of ribbons by rapid solidification techniques. The crystallization, magnetic, mechanical and corrosion behaviour were studied for the prepared materials as a function of quenching rate from liquid to the solid state. Higher quench rates produced a more amorphous ...

  15. Rapid mimicry and emotional contagion in domestic dogs. (United States)

    Palagi, Elisabetta; Nicotra, Velia; Cordoni, Giada


    Emotional contagion is a basic form of empathy that makes individuals able to experience others' emotions. In human and non-human primates, emotional contagion can be linked to facial mimicry, an automatic and fast response (less than 1 s) in which individuals involuntary mimic others' expressions. Here, we tested whether body (play bow, PBOW) and facial (relaxed open-mouth, ROM) rapid mimicry is present in domestic dogs (Canis lupus familiaris) during dyadic intraspecific play. During their free playful interactions, dogs showed a stronger and rapid mimicry response (less than 1 s) after perceiving PBOW and ROM (two signals typical of play in dogs) than after perceiving JUMP and BITE (two play patterns resembling PBOW and ROM in motor performance). Playful sessions punctuated by rapid mimicry lasted longer that those sessions punctuated only by signals. Moreover, the distribution of rapid mimicry was strongly affected by the familiarity linking the subjects involved: the stronger the social bonding, the higher the level of rapid mimicry. In conclusion, our results demonstrate the presence of rapid mimicry in dogs, the involvement of mimicry in sharing playful motivation and the social modulation of the phenomenon. All these findings concur in supporting the idea that a possible linkage between rapid mimicry and emotional contagion (a building-block of empathy) exists in dogs.

  16. Forms of Inattentiveness

    DEFF Research Database (Denmark)

    Knudsen, Morten


    Research dealing with governmental and managerial ideals and tools for transparency has observed how these tools co-create new types of blindness. It has documented the existence of three different types of blindness: blindness caused by power games, by cognitive limitations and blindness as a side...... and it points to activities that produce this blindness as they reduce the probability that potentially destructive subjects are actualized. Information is not only something organizations need, but may also be something they protect themselves against. In that case, the forms of inattentiveness may...

  17. Progress in Genome Editing Technology and Its Application in Plants


    Zhang, Kai; Raboanatahiry, Nadia; Zhu, Bin; Li, Maoteng


    Genome editing technology (GET) is a versatile approach that has progressed rapidly as a mechanism to alter the genotype and phenotype of organisms. However, conventional genome modification using GET cannot satisfy current demand for high-efficiency and site-directed mutagenesis, retrofitting of artificial nucleases has developed into a new avenue within this field. Based on mechanisms to recognize target genes, newly-developed GETs can generally be subdivided into three cleavage systems, pr...

  18. CBM progress report 2007

    Energy Technology Data Exchange (ETDEWEB)

    Herrmann, N.; Rami, F.; Roehrich, D.; Stroth, J.; Wessels, J.; Zaitsev, Yu


    This report documents the activities within the CBM project in 2007. Significant progress has been made in the optimization of the simulation software, the layout and development of detectors, the design of front-end electronics, and the concepts for data acquisition. The simulation and analysis routines have been completely integrated into the software framework (FAIRoot and CBMroot), and can be used now easily by users outside GSI. A breakthrough has been achieved in the development of fast algorithms for track and vertex reconstruction which have been improved in speed by a factor of 10{sup 5}. These fast routines permit to perform high-statistics simulations for detailed detector layout optimization. Full event reconstruction based on realistic detector properties and particle multiplicities as given by microscopic transport models are routinely used in the feasibility studies. A version of the Silicon Tracking System is now implemented in the simulation software comprising 8 detector layers based on microstrip technology only, including the readout cables, and the mechanical detector structure. The studies of open charm detection have been extended to D{sub s}{sup +} and {lambda}{sub c}, taking into account a realistic layout of the Silicon Pixel Microvertex detector. The identification of electrons has been optimized by improved ring recognition algorithms and transition radiation simulations. The Ring Imaging Cherenkov (RICH) detector has been redesigned, resulting in a reduction by a factor of two in mirror size and number of readout channels without reducing the pion rejection capability. The muon detection system has been optimized with respect to the number of detector layers. The muon simulations take into account detector inefficiencies and a segmentation of the muon chambers into pads according to a nominal occupancy of 5% for central Au+Au collisions. Studies for a dimuon trigger show promising results. Radiation dose simulations using the FLUKA

  19. Free form fabrication of thermoplastic composites

    Energy Technology Data Exchange (ETDEWEB)

    Kaufman, S.G.; Spletzer, B.L.; Guess, T.R.


    This report describes the results of composites fabrication research sponsored by the Laboratory Directed Research and Development (LDRD) program at Sandia National Laboratories. They have developed, prototyped, and demonstrated the feasibility of a novel robotic technique for rapid fabrication of composite structures. Its chief innovation is that, unlike all other available fabrication methods, it does not require a mold. Instead, the structure is built patch by patch, using a rapidly reconfigurable forming surface, and a robot to position the evolving part. Both of these components are programmable, so only the control software needs to be changed to produce a new shape. Hence it should be possible to automatically program the system to produce a shape directly from an electronic model of it. It is therefore likely that the method will enable faster and less expensive fabrication of composites.

  20. A Dextral Primary Progressive Aphasia Patient with Right Dominant Hypometabolism and Tau Accumulation and Left Dominant Amyloid Accumulation. (United States)

    Jang, Young Kyoung; Park, Seongbeom; Kim, Hee Jin; Cho, Hanna; Lyoo, Chul Hyoung; Seo, Sang Won; Na, Duk L


    Primary progressive aphasia (PPA) is a degenerative disease that presents as progressive decline of language ability with preservation of other cognitive functions in the early stages. Three subtypes of PPA are known: progressive nonfluent aphasia, semantic dementia, and logopenic aphasia (LPA). We report the case of a 77-year-old patient with PPA whose clinical findings did not correspond to the three subtypes but mainly fit LPA. Unlike other LPA patients, however, this patient showed a right hemisphere predominant glucose hypometabolism and tau accumulation and a left hemisphere predominant amyloid deposition. The right-handed patient presented with comprehension difficulty followed by problems naming familiar objects. This isolated language problem had deteriorated rapidly for 2 years, followed by memory difficulties and impairment of daily activities. Using a Korean version of the Western Aphasia Battery, aphasia was consistent with a severe form of Wernicke's aphasia. According to the brain magnetic resonance imaging and (18)F-fludeoxyglucose positron emission tomography results, right hemisphere atrophy and hypometabolism, more predominant on the right hemisphere than the left, were apparent despite the fact that Edinburgh Handedness Questionnaire scores indicated strong right-handedness. On Pittsburgh compound B-PET, amyloid accumulation was asymmetrical with the left hemisphere being more predominant than the right, whereas (18)F-T807-PET showed a right dominant tau accumulation. This is the first report of atypical PPA, in which the patient exhibited crossed aphasia and asymmetrical amyloid accumulation.

  1. Primary progressive multiple sclerosis in the Polish population

    Directory of Open Access Journals (Sweden)

    Waldemar Brola


    Full Text Available Objectives: The aim of the study was the epidemiological analysis and evaluation of selected clinical and sociodemographic factors in Polish patients with primary progressive multiple sclerosis. Methods: The study included patients from 7 provinces in central and eastern Poland registered in the Registry of Patients with Multiple Sclerosis on 31 December 2016. The incidence of various forms of the disease was compared, and clinical, demographic and social disparities between relapsing-remitting and primary progressive multiple sclerosis were analysed. Results: Of 3,199 registered patients, 2,188 persons (66.2% had the relapsing-remitting form of multiple sclerosis, 774 (24.2% had the secondary progressive type and 307 (9.6% suffered from primary progressive disease. The first symptoms of primary progressive multiple sclerosis appeared almost 10 years later than in patients with the relapsing-remitting type (39.2 ± 11.4 vs. 29.8 ± 9.8. The period from the first symptoms to diagnosis was more than twice as long in patients with primary progressive multiple sclerosis (5.8 ± 3.4 as in those with relapsing-remitting disease (2.4 ± 1.6. The average degree of disability in the Expanded Disability Status Scale was similar and amounted to 3.2 ± 2.1 for relapsing-remitting and 3.6 ± 2.4 for primary progressive multiple sclerosis. The relapsing-remitting form was observed more often in women (2.4:1, and the primary progressive form appeared with equal frequency in both sexes (1:1. Disease-modifying treatment was received by 34% of patients with relapsing-remitting and in only 1.9% of patients with primary progressive multiple sclerosis. Conclusions: The primary progressive form affects approximately 10% of Polish patients with multiple sclerosis. The first symptoms appear at about 40 years of age with equal frequency in both sexes, and its diagnosis takes more than twice as much time as in the case of relapsing-remitting multiple

  2. Expandable gastroretentive dosage forms. (United States)

    Klausner, Eytan A; Lavy, Eran; Friedman, Michael; Hoffman, Amnon


    Expandable gastroretentive dosage forms (GRDFs) have been designed for the past 3 decades. They were originally created for possible veterinary use, but later the design was modified for enhanced drug therapy in humans. These GRDFs are easily swallowed and reach a significantly larger size in the stomach due to swelling or unfolding processes that prolong their gastric retention time (GRT). After drug release, their dimensions are minimized with subsequent evacuation from the stomach. Gastroretentivity is enhanced by the combination of substantial dimensions with high rigidity of the dosage form to withstand the peristalsis and mechanical contractility of the stomach. Positive results were obtained in preclinical and clinical studies evaluating GRT of expandable GRDFs. Narrow absorption window drugs compounded in such systems have improved in vivo absorption properties. These findings are an important step towards the implementation of expandable GRDFs in the clinical setting. The current review deals with expandable GRDFs reported in articles and patents, and describes the physiological basis of their design. Using the dog as a preclinical screening model prior to human studies, relevant imaging techniques and pharmacokinetic-pharmacodynamic aspects of such delivery systems are also discussed.

  3. Bipolar pulse forming line (United States)

    Rhodes, Mark A.


    A bipolar pulse forming transmission line module for linear induction accelerators having first, second, third, fourth, and fifth planar conductors which form an interleaved stack with dielectric layers between the conductors. Each conductor has a first end, and a second end adjacent an acceleration axis. The first and second planar conductors are connected to each other at the second ends, the fourth and fifth planar conductors are connected to each other at the second ends, and the first and fifth planar conductors are connected to each other at the first ends via a shorting plate adjacent the first ends. The third planar conductor is electrically connectable to a high voltage source, and an internal switch functions to short a high voltage from the first end of the third planar conductor to the first end of the fourth planar conductor to produce a bipolar pulse at the acceleration axis with a zero net time integral. Improved access to the switch is enabled by an aperture through the shorting plate and the proximity of the aperture to the switch.

  4. Regulation of Tumor Progression by Programmed Necrosis

    Directory of Open Access Journals (Sweden)

    Su Yeon Lee


    Full Text Available Rapidly growing malignant tumors frequently encounter hypoxia and nutrient (e.g., glucose deprivation, which occurs because of insufficient blood supply. This results in necrotic cell death in the core region of solid tumors. Necrotic cells release their cellular cytoplasmic contents into the extracellular space, such as high mobility group box 1 (HMGB1, which is a nonhistone nuclear protein, but acts as a proinflammatory and tumor-promoting cytokine when released by necrotic cells. These released molecules recruit immune and inflammatory cells, which exert tumor-promoting activity by inducing angiogenesis, proliferation, and invasion. Development of a necrotic core in cancer patients is also associated with poor prognosis. Conventionally, necrosis has been thought of as an unregulated process, unlike programmed cell death processes like apoptosis and autophagy. Recently, necrosis has been recognized as a programmed cell death, encompassing processes such as oncosis, necroptosis, and others. Metabolic stress-induced necrosis and its regulatory mechanisms have been poorly investigated until recently. Snail and Dlx-2, EMT-inducing transcription factors, are responsible for metabolic stress-induced necrosis in tumors. Snail and Dlx-2 contribute to tumor progression by promoting necrosis and inducing EMT and oncogenic metabolism. Oncogenic metabolism has been shown to play a role(s in initiating necrosis. Here, we discuss the molecular mechanisms underlying metabolic stress-induced programmed necrosis that promote tumor progression and aggressiveness.

  5. On the progressive nature of grain crushing (United States)

    Ciantia, Matteo O.; Piñero, Gema; Zhu, Jian; Shire, Tom


    In this work acoustic emission (AE) is used as experimental evidence of the progressive nature of grain crushing. Stress controlled high pressure oedometric compression test are carried out on 1.2 mm monodisperse samples of glass beads. It was observed that the granular assembly starts to experience particle breakage at a vertical stress of about 25MPa. When this yield pressure is exceeded the glass beads start to break emitting loud impulsive sound and the vertical displacement increases rapidly. The load was increased beyond the yield stress and at each increment while the vertical stress remained constant the sample continued to emit sound. The emission of sound at a constant vertical stress indicates that crushing is a progressive failure mechanism; once the first crushing event occurs, the structure starts to rearrange causing other crushing events to occur and additional settlement. In particular, two signal processing algorithms are used on the samples of the acoustic signal to obtain two additional metrics of the crushing evolution. The first is the cumulative energy versus time. The second is the number of crushing events versus time, which is based on the automatic detection of the peaks of the sound signal envelope. There is a clear correlation between the cumulative acoustic energy emitted and the observed sample displacement. Using laser scanning, the evolution of the particle size distribution and particle shape are measured in detail so that a link between the acoustic data and the crushing intensity is established. The crushing intensity was controlled using materials with different strengths.

  6. Developmental evolution facilitates rapid adaptation. (United States)

    Lin, Hui; Kazlauskas, Romas J; Travisano, Michael


    Developmental evolution has frequently been identified as a mode for rapid adaptation, but direct observations of the selective benefits and associated mechanisms of developmental evolution are necessarily challenging to obtain. Here we show rapid evolution of greatly increased rates of dispersal by developmental changes when populations experience stringent selection. Replicate populations of the filamentous fungus Trichoderma citrinoviride underwent 85 serial transfers, under conditions initially favoring growth but not dispersal. T. citrinoviride populations shifted away from multicellular growth toward increased dispersal by producing one thousand times more single-celled asexual conidial spores, three times sooner than the ancestral genotype. Conidia of selected lines also germinated fifty percent faster. Gene expression changed substantially between the ancestral and selected fungi, especially for spore production and growth, demonstrating rapid evolution of tight regulatory control for down-regulation of growth and up-regulation of conidia production between 18 and 24 hours of growth. These changes involved both developmentally fixed and plastic changes in gene expression, showing that complex developmental changes can serve as a mechanism for rapid adaptation.

  7. Multigrade Teaching Rapid Appraisal Procedure. (United States)

    Nielsen, Dean

    Multigrade classes have been recognized as part of elementary education for many years, but their special needs have been largely ignored. This manual focuses on the survey research that should predate the design of instructional management strategies in multigrade classrooms. It describes rapid and reliable ways to collect information about the…

  8. Furnace for rapid thermal processing

    NARCIS (Netherlands)

    Roozeboom, F.; Duine, P.A.; Sluis, P. van der


    A Method (1) for Rapid Thermal Processing of a wafer (7), wherein the wafer (7) is heated by lamps (9), and the heat radiation is reflected by an optical switching device (15,17) which is in the reflecting state during the heating stage. During the cooling stage of the wafer (7), the heat is

  9. Rapid Energy Modeling Workflow Demonstration (United States)


    BIM Building Information Modeling BPA Building Performance Analysis BTU British Thermal Unit CBECS Commercial Building ...geometry, orientation, weather, and materials, generates 3D Building Information Models ( BIM ) guided by satellite views of building footprints and...Rapid Energy Modeling (REM) workflows that employed building information modeling ( BIM ) approaches and conceptual energy analysis.

  10. Portable Diagnostics and Rapid Germination

    Energy Technology Data Exchange (ETDEWEB)

    Dunn, Zachary Spencer [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)


    In the Bioenergy and Defense Department of Sandia National Laboratories, characterization of the BaDx (Bacillus anthracis diagnostic cartridge) was performed and rapid germination chemistry was investigated. BaDx was tested with complex sample matrixes inoculated with Bacillus anthracis, and the trials proved that BaDx will detect Bacillus anthracis in a variety of the medium, such as dirt, serum, blood, milk, and horse fluids. The dimensions of the device were altered to accommodate an E. coli or Listeria lateral flow immunoassay, and using a laser printer, BaDx devices were manufactured to identify E. coli and Listeria. Initial testing with E. coli versions of BaDx indicate that the device will be viable as a portable diagnostic cartridge. The device would be more effective with faster bacteria germination; hence studies were performed the use of rapid germination chemistry. Trials with calcium dipicolinic acid displayed increased cell germination, as shown by control studies using a microplate reader. Upon lyophilization the rapid germination chemistry failed to change growth patterns, indicating that the calcium dipicolinic acid was not solubilized under the conditions tested. Although incompatible with the portable diagnostic device, the experiments proved that the rapid germination chemistry was effective in increasing cell germination.

  11. Aluminum involvement in the progression of Alzheimer's disease. (United States)

    Walton, J R


    The neuroanatomic specificity with which Alzheimer's disease (AD) progresses could provide clues to AD etiopathology. Magnetic resonance imaging studies of AD clinical progression have confirmed general conclusions from earlier studies of AD neuropathological progression wherein neurofibrillary tangle pathology was observed to spread along a well-defined sequence of corticocortical and corticosubcortical connections, preferentially affecting certain cell types, while sparing others. Identical and non-identical twin studies have consistently shown AD has mixed (environmental and genetic) etiopathogenesis. The decades-long prodromal phase over which AD develops suggests slow but progressive accumulation of a toxic or infective agent over time. Major environmental candidates are reviewed to assess which best fits the profile of an agent that slowly accrues in susceptible cell types of AD-vulnerable brain regions to toxic levels by old age, giving rise to AD neuropathology without rapid neuronal lysis. Chronic aluminum neurotoxicity best matches this profile. Many humans routinely ingest aluminum salts as additives contained in processed foods and alum-treated drinking water. The physical properties of aluminum and ferric iron ions are similar, allowing aluminum to use mechanisms evolved for iron to enter vulnerable neurons involved in AD progression, accumulate in those neurons, and cause neurofibrillary damage. The genetic component of AD etiopathogenesis apparently involves a susceptibility gene, yet to be identified, that increases aluminum absorption because AD and Down syndrome patients have higher than normal plasma, and brain, aluminum levels. This review describes evidence for aluminum involvement in AD neuropathology and the clinical progression of sporadic AD.

  12. Risk of progression in macula-on rhegmatogenous retinal detachment. (United States)

    Callizo, Josep; Pfeiffer, Sebastian; Lahme, Eva; van Oterendorp, Christian; Khattab, Mohammed; Bemme, Sebastian; Kulanga, Miroslav; Hoerauf, Hans; Feltgen, Nicolas


    To identify factors that may lead to a rapid progression in macula-on rhegmatogenous retinal detachment (RRD), in particular, those that may lead to macular involvement. Observational, prospective, single-center study. Patients referred for surgery due to primary rhegmatogenous retinal detachment with the macula on between 2009 and 2013 were included. Relevant factors analyzed included age, time delay until surgery, lens status, myopia, the detachment's location and configuration as well as number, size and type of retinal breaks. Eyes underwent optical coherence tomography to detect macular detachment. A multivariate analysis was performed to investigate the effect of several factors in the progression of retinal detachment. A total of 116 eyes of 116 patients were included. Mean time delay between admission and surgery was 1.8 ± 1.4 days. Progression was observed in 19.8% of the eyes. Of those, 47.8% presented macular detachment. Ten of the 11 (90.9%) eyes presenting progression involving the macula also exhibited a bullous configuration, which was the only parameter that correlated significantly with detachment progression in patients with (p = 0.0036) and without (p = 0.0014) macular involvement. For the first time in a prospective trial, a bullous configuration was found to be a highly significant predictor for progression in macula-on detachments. Our data support prompt surgery in patients diagnosed with bullous macula-on RRD.

  13. Progress in the ecology and conservation of giant pandas. (United States)

    Wei, Fuwen; Swaisgood, Ronald; Hu, Yibo; Nie, Yonggang; Yan, Li; Zhang, Zejun; Qi, Dunwu; Zhu, Lifeng


    Giant panda (Ailuropoda melanoleuca) conservation is a possible success story in the making. If extinction of this iconic endangered species can be avoided, the species will become a showcase program for the Chinese government and its collaborators. We reviewed the major advancements in ecological science for the giant panda, examining how these advancements have contributed to panda conservation. Pandas' morphological and behavioral adaptations to a diet of bamboo, which bear strong influence on movement ecology, have been well studied, providing knowledge to guide management actions ranging from reserve design to climate change mitigation. Foraging ecology has also provided essential information used in the creation of landscape models of panda habitat. Because habitat loss and fragmentation are major drivers of the panda population decline, efforts have been made to help identify core habitat areas, establish where habitat corridors are needed, and prioritize areas for protection and restoration. Thus, habitat models have provided guidance for the Chinese governments' creation of 67 protected areas. Behavioral research has revealed a complex and efficient communication system and documented the need for protection of habitat that serves as a communication platform for bringing the sexes together for mating. Further research shows that den sites in old-growth forests may be a limiting resource, indicating potential value in providing alternative den sites for rearing offspring. Advancements in molecular ecology have been revolutionary and have been applied to population census, determining population structure and genetic diversity, evaluating connectivity following habitat fragmentation, and understanding dispersal patterns. These advancements form a foundation for increasing the application of adaptive management approaches to move panda conservation forward more rapidly. Although the Chinese government has made great progress in setting aside protected areas

  14. Recent Progress on Spherical Torus Research

    Energy Technology Data Exchange (ETDEWEB)

    Ono, Masayuki [PPPL; Kaita, Robert [PPPL


    The spherical torus or spherical tokamak (ST) is a member of the tokamak family with its aspect ratio (A = R0/a) reduced to A ~ 1.5, well below the normal tokamak operating range of A ≥ 2.5. As the aspect ratio is reduced, the ideal tokamak beta β (radio of plasma to magnetic pressure) stability limit increases rapidly, approximately as β ~ 1/A. The plasma current it can sustain for a given edge safety factor q-95 also increases rapidly. Because of the above, as well as the natural elongation κ, which makes its plasma shape appear spherical, the ST configuration can yield exceptionally high tokamak performance in a compact geometry. Due to its compactness and high performance, the ST configuration has various near term applications, including a compact fusion neutron source with low tritium consumption, in addition to its longer term goal of attractive fusion energy power source. Since the start of the two megaampere class ST facilities in 2000, National Spherical Torus Experiment (NSTX) in the US and Mega Ampere Spherical Tokamak (MAST) in UK, active ST research has been conducted worldwide. More than sixteen ST research facilities operating during this period have achieved remarkable advances in all of fusion science areas, involving fundamental fusion energy science as well as innovation. These results suggest exciting future prospects for ST research both near term and longer term. The present paper reviews the scientific progress made by the worldwide ST research community during this new mega-ampere-ST era.

  15. Genetics of primary progressive multiple sclerosis. (United States)

    Cree, Bruce A C


    Multiple sclerosis (MS) patients are classified as either having relapsing onset or progressive onset disease, also known as primary progressive MS (PPMS). Relative to relapsing onset patients, PPMS patients are older at disease onset, are equally likely to be men or women, and have more rapid accumulation of disability that does not respond well to treatments used in relapsing onset MS. Although estimates vary, 5-15% of all MS patients have a PPMS disease course. Genetic variance is a proposed determinant of MS disease course. If distinct genes associated with PPMS were identified study of these genes might lead to an understanding of the biology underlying disease progression and neural degeneration that are the hallmarks of PPMS. These genes and their biological pathways might also represent therapeutic targets. This chapter systematically reviews the PPMS genetic literature. Despite the intuitively appealing notion that differences between PPMS and relapsing onset MS are due to genetics, definite differences associated with these phenotypes at the major histocompatibility complex or elsewhere in the genome have not been found. Recent large-scale genome wide screens identified multiple genes associated with MS susceptibility outside the MHC. The genetic variants identified thus far make only weak individual contributions to MS susceptibility. If the genetic effects that contribute to the differences between PPMS and relapsing MS are similar in magnitude to those that distinguish MS from healthy controls then, given the relative scarcity of the PPMS phenotype, very large datasets will be needed to identify PPMS associated genes. International collaborative efforts could provide the means to identify such genes. Alternately, it is possible that factors other than genetics underlie the differences between these clinical phenotypes. Copyright © 2014 Bruce Cree. Published by Elsevier B.V. All rights reserved.

  16. Progressive Taxation and Tax Morale


    Doerrenberg, Philipp; Peichl, Andreas


    As the link between tax compliance and tax morale is found to be robust, finding the determinants of tax morale can help to understand and fight tax evasion. In this paper we analyze the effect of progressive taxation on tax morale in a cross-country approach - which has not been investigated before. Our theoretical analysis leads to two testable predictions. First, an individual's tax morale is higher, the more progressive the tax schedule is. Second, the impact of tax progressivity on tax m...

  17. Pion form factor

    Energy Technology Data Exchange (ETDEWEB)

    Ryong Ji, C.; Pang, A.; Szczepaniak, A. [North Carolina State Univ., Raleigh, NC (United States)


    It is pointed out that the correct criterion to define the legal PQCD contribution to the exclusive processes in the lightcone perturbative expansion should be based on the large off-shellness of the lightcone energy in the intermediate states. In the lightcone perturbative QCD calculation of the pion form factor, the authors find that the legal PQCD contribution defined by the lightcone energy cut saturates in the smaller Q{sup 2} region compared to that defined by the gluon four-momentum square cut. This is due to the contribution by the highly off-energy-shell gluons in the end point regions of the phase space, indicating that the gluon four-momentum-square cut may have cut too much to define the legal PQCD.


    Directory of Open Access Journals (Sweden)

    Maximiliano Emanuel Korstanje


    Full Text Available Modern studies emphasized on the needs of researching the hospitality as relevant aspects of tourism and hospitality fields. Anyway, these approaches are inextricably intertwined to the industry of tourism and do not take seriously the anthropological and sociological roots of hospitality. In fact, the hotel seems to be a partial sphere of hospitality at all. Under this context, the present paper explores the issue of hospitality enrooted in the political and economic indo-European principle of free-transit which is associated to a much broader origin.  Starting from the premise etymologically hostel and hospital share similar origins, we follow the contributions of J Derrida to determine the elements that formed the hospitality up to date.

  19. Progressive outer retinal necrosis: manifestation of human immunodeficiency virus infection (United States)

    Lo, Phey Feng; Lim, Rongxuan; Antonakis, Serafeim N; Almeida, Goncalo C


    We present the case of a 54-year-old man who developed progressive outer retinal necrosis (PORN) as an initial manifestation of HIV infection without any significant risk factors for infection with HIV. PORN is usually found as a manifestation of known AIDS late in the disease. Our patient presented with transient visual loss followed by decrease in visual acuity and facial rash. Subsequent investigation revealed anterior chamber tap positive for varicella zoster virus (VZV), as well as HIV positivity, with an initial CD4 count of 48 cells/µL. Systemic and intravitreal antivirals against VZV, and highly active antiretroviral therapy against HIV were started, which halted further progression of retinal necrosis. This case highlights the importance of suspecting PORN where there is a rapidly progressive retinitis, and also testing the patient for HIV, so appropriate treatment can be started. PMID:25948844

  20. Progressive outer retinal necrosis: manifestation of human immunodeficiency virus infection. (United States)

    Lo, Phey Feng; Lim, Rongxuan; Antonakis, Serafeim N; Almeida, Goncalo C


    We present the case of a 54-year-old man who developed progressive outer retinal necrosis (PORN) as an initial manifestation of HIV infection without any significant risk factors for infection with HIV. PORN is usually found as a manifestation of known AIDS late in the disease. Our patient presented with transient visual loss followed by decrease in visual acuity and facial rash. Subsequent investigation revealed anterior chamber tap positive for varicella zoster virus (VZV), as well as HIV positivity, with an initial CD4 count of 48 cells/µL. Systemic and intravitreal antivirals against VZV, and highly active antiretroviral therapy against HIV were started, which halted further progression of retinal necrosis. This case highlights the importance of suspecting PORN where there is a rapidly progressive retinitis, and also testing the patient for HIV, so appropriate treatment can be started. 2015 BMJ Publishing Group Ltd.