Epidermolysis bullosa acquisita: clinical manifestations, microscopic findings, and surgical periodontal therapy. A case report.

Science.gov (United States)

Hakki, S S; Celenligil-Nazliel, H; Karaduman, A; Usubütün, A; Ertoy, D; Ayhan, A; Ruacan, S

2001-04-01

Epidermolysis bullosa acquisita (EBA) is an uncommon, acquired, chronic subepidermal bullous disease. This report describes a case of EBA with gingival involvement. A 43-year-old woman with EBA was referred to our clinic for periodontal therapy because of gingival tenderness and bleeding. She has been on cyclosporin A therapy for the last 2 years. Clinical findings were analyzed. Anterior gingivectomy operations were performed in 2 stages. The samples obtained during the surgery were examined using histopathologic, immunohistologic, and electronmicroscopic methods. Long-term effects of the surgical periodontal treatment on gingiva were evaluated both clinically and microscopically. The dentition displayed minimal enamel hypoplasia. Decayed, missing, and filled surfaces score was found to be elevated. Periodontal examination showed generalized diffuse gingival inflammation and gingival enlargement localized mainly to the anterior region. Nikolsky's sign was positive. However, wound healing was uneventful after the operations. Microscopic findings were similar to those obtained from the skin. Twenty-one months after the operations, Nikolsky's sign was negative and no remarkable gingival inflammation was noted. Microscopic examination revealed that the blisters were fewer in number and smaller in size. These results indicate that gingival tissues may also be involved in EBA. Uneventful wound healing after periodontal surgery in this case suggests that periodontal surgery can be performed in patients with EBA. Moreover, both our clinical and histopathologic findings imply that gingivectomy proves useful in maintaining gingival integrity in these patients. Our data may also suggest that the patients with EBA are highly likely to develop dental caries.

Three familial cases of Pasini variant of dominant dystrophic epidermolysis

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Seirafi H

1999-07-01

Full Text Available Epidermolysis bullosa (EB is the term applied to a group of disorders whose common primary feature is the formation of blisters following trivial trauma. Hereditary EB comprises 3 major classes: simplex, junctional and dystrophic, and includes more than 23 phenotypes. The albopapuloid pasini variant of dominant dystrophic EB is characterized by a distinctive clinical appearance. In this article, we report this disease in three members of a family (father and two sons.

Use of a novel laparoscopic gastrostomy technique in children with severe epidermolysis bullosa.

Science.gov (United States)

Patel, Kamlesh; Wells, Jonathan; Jones, Rosie; Browne, Fiona; Moss, Celia; Parikh, Dakshesh

2014-05-01

Supplementing nutrition in children with severe epidermolysis bullosa (EB) is challenging because of skin and mucosal fragility. Percutaneous endoscopic gastrostomy is contraindicated in EB, whereas more invasive open surgical gastrostomy placement can be complicated by chronic leakage. The aim of the study was to review the efficacy and acceptability, in children with severe EB, of our modified 2-port laparoscopic approach using the Seldinger technique with serial dilatation and tube insertion through a peel-away sheath. Retrospective review of children with EB who underwent laparoscopic feeding gastrostomy at our centre since 2009. Seven children (6 severe generalised recessive dystrophic EB, 1 non-Herlitz junctional EB; 2 girls, 5 boys) underwent modified laparoscopic gastrostomy placement at median age 4.85 years (range 1.0-8.8), with fundoplication for gastro-oesophageal reflux in 1 case, with follow-up for 0.3 to 3.9 years. The procedure was well tolerated with oral feeds usually given after 4 hours and whole protein gastrostomy feeds within 24 hours in 6 patients. Improved growth was reflected in mean weight and height z scores: -1.36 (range -2.6 to 0.5) to -0.61 (range -2.34 to 2.0) and -1.09 (range -2.42 to 1.0) to 0.71 (range -1.86 to 1.0), respectively. Postoperatively, 5 patients experienced minor local complications: minimal leakage without skin damage in 3 and transient peristomal granulation rapidly responsive to topical treatment in 2; this followed acute gastrostomy site infection in 1. There was no leakage after the immediate postoperative period. We conclude that our less-invasive laparoscopic gastrostomy technique is effective and better tolerated in children with severe EB, at least in the medium term, than open gastrostomy placement. Longer follow-up is required.

A suicide gene therapy approach to treat epidermolysis bullosa-associated skin cancer

International Nuclear Information System (INIS)

Gruber, C.

2009-01-01

Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited disease causing extensive blister formation within the basal membrane zone (BMZ) of the skin and mucous membranes. It is caused by premature STOP mutations in the COL7A1 gene, which is indispensable for proper skin assembling. RDEB is associated with the development of a highly malignant skin cancer (squamous cell carcinoma, SCC) in early adulthood that displays a life threatening complication within this patient group. To date, neither chemo- nor radiotherapies showed successful results and due to the high metastatic potential of RDEB SCC wide surgical excision is still favoured. In this study we could reveal a new promising cancer treatment using spliceosome mediated RNA trans-splicing (SMaRT) using a suicide gene therapy approach. First we identified the tumour marker gene MMP-9 expressed by RDEB SCC cells in cell culture which was used to generate various pre-mRNA trans-splicing molecules (PTM). PTMs are able to facilitate trans-splicing between a tumour target gene and a cell death inducing peptide/toxin, encoded by the PTM. As a consequence the toxin is expressed in cancer cells leading to the induction of cell death. This technique offers high specificity in cancer cell targeting compared to other conventional cDNA expression studies. Various trans-splicing molecules were pre-evaluated in a fluorescence screening model for their best trans-splicing efficiency with the target molecule. Herein we identified two potent PTMs (PTM BD0 and PTM BD6), that were further adapted for endogenous suicide studies by inserting the toxin streptolysin O. In two independent in vitro cell culture assays we were able to confirm that the trans-splicing molecules are able to induce expression of the toxin resulting in cell membrane permeabilization and increased cell death induction. The results indicate that SMaRT technology offers a new platform for a suicide gene therapy approach to treat malignant squamous cell

Clinical variability in dystrophic epidermolysis bullosa and findings with scanning electron microscopy Variabilidade clínica em epidermólise bolhosa distrófica e achados de microscopia eletrônica de varredura

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Hiram Larangeira de Almeida Jr

2012-02-01

Full Text Available In dystrophic epidermolysis bullosa, the genetic defect of anchoring fibrils leads to cleavage beneath the basement membrane and its consequent loss. A 46 year-old female patient presented blisters with a pretibial distribution associated with nail dystrophy. Her two children had hyponychia and anonychia, which affected all toe nails and the thumb, forefinger and middle finger. DNA sequencing identified in exon 75 of COL7A1 gene a pathologic mutation: c.6235G>A (p.Gly2079Arg. Immunomapping of a blister demonstrated collagen IV (basal membrane in the blister roof and collagen VII in its floor, confirming dystrophic epidermolysis bullosa. Scanning electron microscopy of an inverted blister showed net-forming collagen attached to the blister roof . The variability found in this family has already been reported and confirms, on a clinical basis, the nail subtype as a dystrophic variant.Na epidermólise bolhosa distrófica, o defeito genético das fibrilas de ancoragem leva à clivagem abaixo da membrana basal com sua consequente perda. Uma paciente de 46 anos apresentava bolhas pré-tibiais associadas à distrofia ungueal. Seus dois filhos apresentavam hipo e anoníquia, afetando todas as unhas dos pododáctilos e dos primeiros, segundos e terceiros quirodáctilos. O sequenciamento de DNA identificou no exon 75 do gene COL7A1 uma mutação patológica: c.6235G>A (p.Gly2079Arg. O imunomapeamento identificou o colágeno IV no teto e colágeno VII no assoalho de uma bolha , confirmando o diagnóstico de epidermólise bolhosa distrófica. A microscopia eletrônica de varredura de um teto invertido de bolha demonstrou rede de colágeno aderida ao mesmo. A variabilidade clínica encontrada nessa família já foi escrita e confirma, que o subtipo ungueal das epidermólises bolhosas é uma forma distrófica.

先天性大疱表皮松解症患儿的创面护理管理%Wound nursing management of children with congenital epidermolysis bullosa

Institute of Scientific and Technical Information of China (English)

任平; 陈劼; 张玉侠

2016-01-01

It reviewed the research status quo of wound care of children with congenital epidermolysis bullosa (EB),based on epidermolysis bullosa“wound bed preparation”(WBP)from the wound management steps inclu-ding the effective cleaning of the wound,the reasonable choice of wet dressing and the prevention of complica-tions and so on.The wet dressings and matters needing attention suitable for the different types of EB patients were not the same,it thought that drug and non drug intervention methods of pain and itching management were nursing important points.%以大疱表皮松解症“创面床准备”为基础，从创面管理步骤包括有效清洁创面病灶、合理选用湿性敷料以及预防并发症等方面综述了先天性大疱表皮松解症患儿的创面护理管理研究现状，不同类型的大疱表皮松解症患儿适用的湿性敷料和注意事项各不相同，认为药物和非药物干预方法的疼痛、瘙痒管理也是护理的重点。

Inherited epidermolysis bullosa: clinical and therapeutic aspects Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos

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Vanessa Lys Simas Yamakawa Boeira

2013-04-01

Full Text Available Inherited epidermolysis bullosa (EB is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.A epidermólise bolhosa hereditária (EBH compreende um grupo heterogêneo de desordens genéticas que têm em comum a fragilidade cutânea e, em alguns casos mucosa, predispondo ao desenvolvimento de bolhas e/ou erosões após fricção ou trauma mínimo. Crianças com história recorrente deste tipo de lesão ou neonatos que as apresentem na ausência de outra explicação plausível devem ser investigados. O diagnóstico deve se basear em achados clínicos e histopatológicos. Até o presente momento, o manejo da EBH consiste basicamente em evitar os traumas desencadeadores das lesões, bem como evitar a infecção e facilitar a cicatrização das feridas com o uso sistemático de curativos.

Case Report: Whole exome sequencing reveals a novel frameshift deletion mutation p.G2254fs in COL7A1 associated with autosomal recessive dystrophic epidermolysis bullosa [version 2; referees: 2 approved, 1 approved with reservations

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Shamsudheen Karuthedath Vellarikkal

2016-07-01

Full Text Available Dystrophic epidermolysis bullosa simplex (DEB is a phenotypically diverse inherited skin fragility disorder. It is majorly manifested by appearance of epidermal bullae upon friction caused either by physical or environmental trauma. The phenotypic manifestations also include appearance of milia, scarring all over the body and nail dystrophy. DEB can be inherited in a recessive or dominant form and the recessive form of DEB (RDEB is more severe. In the present study, we identify a novel p.G2254fs mutation in COL7A1 gene causing a sporadic case of RDEB by whole exome sequencing (WES. Apart from adding a novel frameshift Collagen VII mutation to the repertoire of known mutations reported in the disease, to the best of our knowledge, this is the first report of a genetically characterized case of DEB from India.

Case Report: Whole exome sequencing reveals a novel frameshift deletion mutation p.G2254fs in COL7A1 associated with autosomal recessive dystrophic epidermolysis bullosa [version 1; referees: 2 approved, 1 approved with reservations

Directory of Open Access Journals (Sweden)

Shamsudheen Karuthedath Vellarikkal

2016-05-01

Full Text Available Dystrophic epidermolysis bullosa simplex (DEB is a phenotypically diverse inherited skin fragility disorder. It is majorly manifested by appearance of epidermal bullae upon friction caused either by physical or environmental trauma. The phenotypic manifestations also include appearance of milia, scarring all over the body and nail dystrophy. DEB can be inherited in a recessive or dominant form and the recessive form of DEB (RDEB is more severe. In the present study, we identify a novel p.G2254fs mutation in COL7A1 gene causing a sporadic case of RDEB by whole exome sequencing (WES. Apart from adding a novel frameshift Collagen VII mutation to the repertoire of known mutations reported in the disease, to the best of our knowledge, this is the first report of a genetically characterized case of DEB from India.

Impact of inherited epidermolysis bullosa on parental interpersonal relationships, marital status and family size.

Science.gov (United States)

Fine, J-D; Johnson, L B; Weiner, M; Suchindran, C

2005-05-01

The presence in a family of a child or children with epidermolysis bullosa (EB) may have profound psychological implications for other family members. To assess the impact of the presence of EB in one or more children on the personal relationships between their parents. Standardized questionnaires were used. In general, the presence of a child severely affected with EB had profound effects on many aspects of marriage. This included a lack of interest in participating in activities as couples [junctional EB (JEB), 45%; recessive dystrophic EB (RDEB), 25%], a lack of energy to invest in such pursuits (JEB, 82%; RDEB, 50%), limitations in opportunities for sharing nonintimate physical activities (reported by most parents having children with some type of generalized EB), and negatively altered parental sex life (JEB, 55%; RDEB, 39%). This is consistent with the fact that 10%, 64%, 25% and 36% of parents of an affected child with EB simplex (EBS), JEB, dominant dystrophic EB (DDEB) and RDEB, respectively, characterized their relationships as couples as revolving almost exclusively around the day-to-day care of their affected children. The severity of disease in an affected child clearly influenced parental decisions about having more children: 24% and 64% of parents of children with JEB and RDEB, respectively, chose not to have additional children, compared with 26% and 54% of parents with children having EBS or DDEB. This choice was most often pursued via tubal ligation; less often, alternative means of surgical sterilization were chosen. Divorce was common among parents of children with EB (range: 17% in EBS to 31% in JEB) and, with the exception of parents of children with EBS, was usually directly attributed by one or both parents to the profound impact that this disease had exerted on their marriage. Physicians caring for children with EB need to give more consideration to the many psychological factors that may contribute to their patients' well being. They may

Systemic granulocyte colony-stimulating factor (G-CSF) enhances wound healing in dystrophic epidermolysis bullosa (DEB): Results of a pilot trial.

Science.gov (United States)

Fine, Jo-David; Manes, Becky; Frangoul, Haydar

2015-07-01

Chronic nonhealing wounds are the norm in patients with inherited epidermolysis bullosa (EB), especially those with dystrophic EB (DEB). A possible benefit in wound healing after subcutaneous treatment with granulocyte colony-stimulating factor (G-CSF) was suggested from an anecdotal report of a patient given this during stem cell mobilization before bone-marrow transplantation. We sought to determine whether benefit in wound healing in DEB skin might result after 6 daily doses of G-CSF and to confirm its safety. Patients were assessed for changes in total body blister and erosion counts, surface areas of selected wounds, and specific symptomatology after treatment. Seven patients with DEB (recessive, 6; dominant, 1) were treated daily with subcutaneous G-CSF (10 μg/kg/dose) and reevaluated on day 7. For all patients combined, median reductions of 75.5% in lesional size and 36.6% in blister/erosion counts were observed. When only the 6 responders were considered, there were median reductions of 77.4% and 38.8% of each of these measured parameters, respectively. No adverse side effects were noted. Limitations include small patient number, more than 1 DEB subtype included, and lack of untreated age-matched control subjects. Subcutaneous G-CSF may be beneficial in promoting wound healing in some patients with DEB when conventional therapies fail. Copyright © 2015 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

A cross sectional, observational survey to assess levels and predictors of psychological wellbeing in adults with epidermolysis bullosa

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Emma Dures

2013-01-01

Full Text Available Epidermolysis bullosa (EB describes a cluster of genetically determined skin disorders. Symptoms can be painful, disabling and disfiguring, yet there is little research on the psychological impact of the disease. The study aim was to measure psychological wellbeing in adults with EB; and to examine the association between psychological wellbeing and self efficacy, health locus of control and adjustment to appearance in an observational, cross sectional survey. Questionnaire packs comprising the General Health Questionnaire (GHQ-12, the General Self Efficacy Scale (GSE, the Multidimensional Health Locus of Control Scale (MHLOC, and the Derriford Appearance Scale (DAS-24, were sent to approximately 385 adults with EB. The data were analysed using SPSS. Eighty-seven participants responded. Scores on the GHQ-12 showed non-problematic psychological health in 36% of the sample; levels bordering on clinical disorder in 32.1% and severe psychological distress in 31.9%. No correlations were found between demographic factors (age and sex or clinical factors (EB type and perceived severity and psychological well-being. Scores on the GSE, the internal locus of control sub-scale of the MHLOC and the DAS-24 showed them to be statistically significant correlates of psychological wellbeing (P<0.001; P<0.018; and P<0.001 respectively. In a regression analysis, adjustment to appearance and self efficacy accounted for 24% of the variation in psychological wellbeing. Adults with EB might be at risk of experiencing poor psychological health. Interventions designed to enhance disease self management, self efficacy and improve body image are likely to be beneficial in this clinical group.

Genetic and bibliographic information: KRT5 [GenLibi

Lifescience Database Archive (English)

Full Text Available nd Neonatal Diseases and Abnormalities (C16) > Congenital Abnormalities (C16.131) > Skin Abnormalities (C16....ases, Inborn (C16.320) > Skin Diseases, Genetic (C16.320.850) > Epidermolysis Bullosa (C16.320.850.275) > Ep...idermolysis Bullosa Simplex (C16.320.850.275.180) Skin and Connective Tissue Diseases (C17) > Skin Diseases (C17.800) > Skin....493) > Epidermolysis Bullosa Simplex (C17.800.804.493.180) Skin and Connective Tissue Diseases (C17) > Skin Diseases (C17.800) > Ski...rmolysis Bullosa Simplex (C17.800.827.275.180) Skin and Connective Tissue Diseases (C17) > Skin

Cardiomyopathy in Patients With Hereditary Bullous Epidermolysis.

Science.gov (United States)

Batalla, A; Vicente, A; Bartrons, J; Prada, F; Fortuny, C; González-Enseñat, M A

In recent decades, an association has been reported between epidermolysis bullosa (EB) and dilated cardiomyopathy (DC). DC is typically in an advanced phase when detected, leading to a poorer prognosis. Our objective was to determine the prevalence of DC in patients with EB seen in Hospital San Joan de Déu in Barcelona, Spain, between May 1986 and April 2015. This was a descriptive, cross-sectional chart-review study in which we recorded the type and main subtypes of EB and the presence or absence of DC. Fifty-seven patients with EB were found, 19 with EB simplex, 10 with junctional EB, 27 with dystrophic EB (14 dominant dystrophic and 13 recessive dystrophic), and just 1 with Kindler syndrome. DC was detected in only 2 patients with recessive dystrophic EB. Twenty-three patients had presented factors that could have had a causal relationship with the potential onset of DC. DC is a possible complication of EB, particularly in recessive dystrophic EB. Periodic follow-up should be performed to make an early diagnosis and start treatment. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

Adopting a Sustainable Community of Practice Model when Developing a Service to Support Patients with Epidermolysis Bullosa (EB): A Stakeholder-Centered Approach.

Science.gov (United States)

Gowran, Rosemary Joan; Kennan, Avril; Marshall, Siobhán; Mulcahy, Irene; Ní Mhaille, Sile; Beasley, Sarah; Devlin, Mark

2015-02-01

Epidermolysis bullosa (EB) is a rare, genetic skin condition that is complicated, distressing, and painful and permeates every aspect of patients' lives. Support services are essential for meeting the primary needs of patients and families living with EB; however, provision is challenged by many complex issues. In collaboration with the patient organization DEBRA Ireland, this research conducted an in-depth analysis of Irish healthcare support services for EB, with a view to moving towards an improved and sustainable care pathway. A sustainable community of practice model (SCOP), as a core construct provided a simplified framework when studying this complex system. The research utilized mixed methods, comprising individual interviews, questionnaires, and a participatory action research workshop based on a soft systems approach. The study engaged patients, family members, service providers, and policy developers. Findings emphasized that the complexities of life with EB are more than 'skin deep'. The lived experience of stakeholders revealed many levels of emotion, both positive and heart-rending. Despite the positive efforts of specialists in this field, inadequacies to meet the primary needs of people with EB, such as bandages-fundamental for survival-were highlighted. Participants reported challenges relating to understanding patients' needs, access to consistent services within hospitals and the community, time constraints, and the strong emotions evoked by this severe and rare disease. The study identified several areas that can be targeted to bring about improvements in meeting primary needs. Education and research at public, policy, and practice levels need to be prioritized. It is imperative that citizens move beyond an awareness that EB exists and demonstrate a consciousness about the importance of advocating and enabling seamless and sustainable support services through collective action.

Collagen VII deficient mice show morphologic and histologic corneal changes that phenotypically mimic human dystrophic epidermolysis bullosa of the eye.

Science.gov (United States)

Chen, Vicki M; Shelke, Rajani; Nyström, Alexander; Laver, Nora; Sampson, James F; Zhiyi, Cao; Bhat, Najma; Panjwani, Noorjahan

2018-06-16

Absence of collagen VII causes blistering of the skin, eyes and many other tissues. This disease is termed dystrophic epidermolysis bullosa (DEB). Corneal fibrosis occurs in up to 41% and vision loss in up to 64% of patients. Standard treatments are supportive and there is no cure. The immune-histologic and morphologic changes in the corneas of the mouse model for this disease have not been described in the literature. Our purpose is to characterize the eyes of these mice to determine if this is an appropriate model for study of human therapeutics. Western blot analysis (WB) and immunohistochemistry (IHC) were performed to assess the relative collagen VII protein levels and its location within the cornea. Additional IHC for inflammatory and fibrotic biomarkers alpha-smooth muscle actin (α-SMA), transforming growth factor-beta (TGF-β), connective tissue growth factor (CTGF), proteinase 3, tenascin C and collagen III were performed. Clinical photographs documenting opacification of the corneas of animals of differing ages were assessed and scored independently by 2 examiners. Histology was then used to investigate morphologic changes. IHC and WB confirmed that these mice are deficient in collagen VII production at the level of the basement membrane when compared with wild-types. IHC showed anomalous deposition of collagen III throughout the stroma. Of the 5 biomarkers tested, TGF-β showed the strongest and most consistently staining. Photographs documented corneal opacities only in mice older than 10 weeks, opacities were not seen in younger animals. Histology showed multiple abnormalities, including epithelial hyperplasia, ulceration, fibrosis, edema, dysplasia, neovascularization and bullae formation. The collagen VII hypomorphic mouse shows reduced collagen VII production at the level of the corneal basement membrane. Corneal changes are similar to pathology seen in humans with this disease. The presence of anomalous stromal collagen III and TGF-β appear to be

Disease: H00585 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available lysis bullosa simplex, Ogna type (EBS-Ogna) Inherited epidermolysis bullosa is a diverse group of disorders characterized by mechanic...ally fragile skin that readily blister. The conditions i

Epidermolysis Bullosa

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... this research; and the dissemination of information on research progress in these diseases. Contact Us NIAMS Archive Viewers and Players Social Media Moderation Policy FOIA Privacy Statement Accessibility Disclaimer Digital Strategy ...

NIGERIAN VETERINARY JOURNAL

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ADEYEYE

b). Diagnosis of Epitheliogenesis imperfecta was based on the clinical observation of congenital skin discontinuity and histopathological findings. The major differential diagnosis for an animal presenting with congenital discontinuity of squamous epithelium include epidermolysis bullosa which is characterized by cutaneous.

Imaging findings in systemic childhood diseases presenting with dermatologic manifestations.

Science.gov (United States)

Fink, Adam Z; Gittler, Julia K; Nakrani, Radhika N; Alis, Jonathan; Blumfield, Einat; Levin, Terry L

Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. We review the imaging findings in childhood diseases associated with dermatologic manifestations. Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura). Familiarity with pertinent findings in childhood diseases presenting with dermatologic manifestations in childhood diseases aids the radiologist in confirming the diagnosis and guiding imaging workup. Copyright © 2017 Elsevier Inc. All rights reserved.

The Nursing Care And Education Plan For A Child With Epidermolys is Bullosa (A Case Report

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Esra Karaca Çiftçi

2016-05-01

Full Text Available Epidermolysis Bullosa (EBis a chronic disease, some types of which may have morbidity and mortality. That is why the patients must receive multidisciplinary care in case any complications arise. Families must be informed about the disease, about home care and receive genetic counselling. The importance of genetic counselling cannot be stressed enough, since both M.C. and his younger brother were diagnosed with EB. If the family had received genetic counselling, this tragic situation could have been prevented. Home care education for the families of EB patients is also of great importance. Relatives of the patient must also be educated about wound care, infection control, patient nutrition and physical treatment. They must also be educated about how to use the medications and about bandaging, dressing, Vaseline bandages, antiseptics, bathing procedures, and the use of antibiotic cream ointment. It was obvious, therefore, that the nursing care given at home enhanced both the patient’s and the family’s quality of life.

Epidermolysis bullosa simplex

African Journals Online (AJOL)

Prof Ezechukwu

2012-03-01

Mar 1, 2012 ... Yenagoa on the second day of life and was treated as a case of skin sepsis with ... and adulthood and is usually confined to the hands and feet. Later in life ... his clothing and diapers while the sores on the nail beds occurred ...

Pyloric atresia: A report of ten patients | Ksia | African Journal of ...

African Journals Online (AJOL)

Pyloric atresia (PA) is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB). The aim of this study was to evaluate the clinical presentation, ...

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Items 151 - 200 of 488 ... Vol 8, No 1 (2011), Genitourinary complications as initial presentation of inherited epidermolysis bullosa, Abstract. M Arifi, S Arifi, K Demni, MA Bouhafs, R Belkacem, M Barahioui. Vol 9, No 1 (2012), Giant bladder diverticulum : A rare cause of bladder outlet obstruction in children, Abstract.

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Demni, K. Vol 8, No 1 (2011) - Articles Genitourinary complications as initial presentation of inherited epidermolysis bullosa. Abstract. ISSN: 0189-6725. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL · AJOL's Partners · Terms and Conditions of ...

Disease: H00586 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available osa, junctional, with pyloric atresia (JEB-PA) Inherited epidermolysis bullosa is a diverse group of disorders characterized by mecha...nically fragile skin that readily blister. The junctiona

Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.

Science.gov (United States)

Amber, Kyle T; Murrell, Dedee F; Schmidt, Enno; Joly, Pascal; Borradori, Luca

2018-02-01

Autoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. These autoantigens represent structural proteins important for maintenance of dermo-epidermal integrity. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease of the skin and mucosae. Although the disease typically presents with a generalized blistering eruption associated with itch, atypical variants with either localized bullous lesions or "non-bullous" presentations are observed in approximately 20% of patients. A peculiar form of BP typically associated with pregnancy is pemphigoid gestationis. In anti-p200 pemphigoid, patients present with tense blisters on erythematosus or normal skin resembling BP, with a predilection for acral surfaces. These patients have antibodies targeting the 200-kDa basement membrane protein. Epidermolysis bullosa is a rare autoimmune blistering disease associated with autoantibodies against type VII collagen that can have several phenotypes including a classical form mimicking dystrophic epidermolysis bullosa, an inflammatory presentation mimicking BP, or mucous membrane pemphigoid-like lesions. Mucous membrane pemphigoid (MMP) is the term agreed upon by international consensus for an autoimmune blistering disorder, which affects one or more mucous membrane and may involve the skin. The condition involves a number of different autoantigens in the basement membrane zone. It may result in severe complications from scarring, such as blindness and strictures. Diagnosis of these diseases relies on direct immunofluorescence microscopy studies

Caspase-1-Independent IL-1 Release Mediates Blister Formation in Autoantibody-Induced Tissue Injury through Modulation of Endothelial Adhesion Molecules

NARCIS (Netherlands)

Sadeghi, Hengameh; Lockmann, Anike; Hund, Anna-Carina; Samavedam, Unni K. S. R. L.; Pipi, Elena; Vafia, Katerina; Hauenschild, Eva; Kalies, Kathrin; Pas, Hendrikus; Jonkman, Marcel F.; Iwata, Hiroaki; Recke, Andreas; Schon, Michael P.; Zillikens, Detlef; Schmidt, Enno; Ludwig, Ralf J.

2015-01-01

Although reports documented aberrant cytokine expression in autoimmune bullous dermatoses (AIBDs), cytokine-targeting therapies have not been established in these disorders. We showed previously that IL-6 treatment protected against tissue destruction in experimental epidermolysis bullosa acquisita

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African Journals Online (AJOL)

Coffin Lowry Sydrome. Diastrophic Dysplasia. Sydrome. E.E.C. syndrome. Epidermolysis Bullosa. Fanconi syndrome. Focal Dermal Hypoplasia. Goldenhar syndrome. Hand-Foot-genital syndrome. Hanhart syndrome. Holt-Oram syndrome. Klipple-Feil Sydrome. Laurence Moon Sydrome. Marfan syndrome. Meckel Sydrome.

Carriers with functional null mutations in LAMA3 have localized enamel abnormalities due to haploinsufficiency

NARCIS (Netherlands)

Gostynska, Katarzyna B.; Yuen, Wing Yan; Pasmooij, Anna Maria Gerdina; Stellingsma, Cornelius; Pas, Hendri H.; Lemmink, Henny; Jonkman, Marcel F.

2017-01-01

The hereditary blistering disease junctional epidermolysis bullosa (JEB) is always accompanied by structural enamel abnormalities of primary and secondary dentition, characterized as amelogenesis imperfecta. Autosomal recessive mutations in LAMA3, LAMB3 and LAMC2 encoding the heterotrimer laminin

Acrodystrophic neuropathy of Bureau and Barriere in Sudanese ...

African Journals Online (AJOL)

Our case here has been documented to be a mutilating palmoplantar Keratoderma. The case is histopathologically confirmed to show Keratinized tissue. The condition as mentioned is extremely rare, where misdiagnosed as Epidermolysis Bullosa Dystrophica. Keywords: Acrodystrophic neuropathy of Bureau and Barriere, ...

Jos Journal of Medicine - Vol 4, No 1 (2009)

African Journals Online (AJOL)

Recessive Epidermolysis Bullosa simplex- A case report · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. SM Yusuf, AE Uloko, 26-27. http://dx.doi.org/10.4314/jjm.v4i1.55101 ...

Congenital anomalies in black South African liveborn neonates at ...

African Journals Online (AJOL)

hypospadias the commonest urogenital anomalies (Table VI). The incidence of cleft lip/palate was only 0,23 per 1 000. Iivebirths (Table V). The integumentary conditions seen included congenital ichthyosis, epidermolysis bullosa, both disorders of. Mendelian inheritance, an unclassifiable bullous dermatitis and a naevus.

Newborn with severe epidermolysis bullosa

DEFF Research Database (Denmark)

Boesen, Martin Lehmann; Bygum, Anette; Hertz, Jens Michael

2016-01-01

of EB depends on the subtype, and therefore maximum treatment is necessary until the final diagnosis is known. In this case, it took 2 weeks before a final diagnosis was reached. In the meantime, we had several ethical discussions on the treatment level. The most important issues were management of pain...

a case report and literature review

African Journals Online (AJOL)

Pyloric atresia was surgically corrected. Electron microscopy of skin biopsies showed junctional epidermolysis bullosa. There was typical nail dystrophy and no corneal lesions; ultrasonography for the renal system was normal. At 4 months of age, the baby required gastrostomy and fundoplication for severe failure to thrive.

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Indian Academy of Sciences (India)

PRAKASH KUMAR

2007-04-04

Apr 4, 2007 ... appearance of keratinopathies, which have been shown to be the direct result of single-point mutations in keratin genes (Coulombe and Omary 2002). Some of the severe skin disorders which result from point mutations include epidermolysis bullosa simplex (mutations in keratin 5/14 genes), epidermolytic.

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African Journals Online (AJOL)

Hassan, ME. Vol 8, No 4 (2012) - Articles Quality of life and parents' satisfaction with Duhamel's versus transanal endorectal pull-through for the treatment of Hirschsprung's disease in children. Abstract PDF · Vol 9, No 2 (2013) - Articles Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case report and ...

Congenital pyloric atresia: clinical features, diagnosis, associated ...

African Journals Online (AJOL)

Epidermolysis bullosa was seen in eight (40%) patients and multiple intestinal atresias in five (25%). Three patients had associated esophageal atresia. Pyloric diaphragm was the most common and seen in 13 patients including double diaphragms in two followed by pyloric atresia with a gap in four and pyloric atresia ...

Conservative Treatment of a Patient with Epidermolysis Bullosa Presenting as Bart Syndrome: A Case Report

Directory of Open Access Journals (Sweden)

Samet Vasfi Kuvat

2010-01-01

Full Text Available We presented a case of a newborn male with aplasia cutis congenita on the lower limb. The case was treated with conservative method. As for the conservative treatment, daily hydrodebridement with 1/200 diluted povidone-iodine and serum physiologic was performed, followed by closure of the wound with a dexpanthenol + chlorhexidine + fusidic acid-impregnated sterile gauze bandage. the followup that occured after three weeks, the wound was completely epithelialized, but a hypopigmented scar remained in the limb.

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African Journals Online (AJOL)

Omene, J. Vol 39, No 4 (2012) - Articles Epidermolysis bullosa simplex: A case report. Abstract PDF · Vol 40, No 1 (2013) - Articles Congenital tuberculosis: A case report and review of the literature. Abstract PDF. ISSN: 0302-4660. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians ...

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African Journals Online (AJOL)

Items 151 - 200 of 483 ... Vol 39, No 4 (2012), Epidermolysis bullosa simplex: A case report, Abstract PDF. O Peterside, OE Kunle-Olowu, OO Adeyemi, FO Akinbami, J Omene. Vol 40, No 1 (2013), Erythrocyte indices of iron status in children with cyanotic congenital heart disease at the University College Hospital, Ibadan ...

There Is No Structural Relationship between Nasal Septal Deviation, Concha Bullosa, and Paranasal Sinus Fungus Balls

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Tung-Lung Tsai

2012-01-01

Full Text Available This study aims to determine the relationship between nasal septal deviation, concha bullosa, and chronic rhinosinusitis by using a definitive pathological and simplified model. Fifty-two consecutive sinus computed tomography scans were performed on patients who received endoscopic sinus surgery and whose final diagnosis was paranasal sinus fungus balls. The incidences of nasal septal deviation and concha bullosa for patients diagnosed with paranasal sinus fungus balls among the study group were 42.3% and 25%, respectively. About 63.6% sinuses with fungus balls were located on the ipsilateral side of the nasal septal deviation, and 46.2% were located on the ipsilateral side of the concha bullosa. When examined by Pearson’s chi-square test and the chi-squared goodness-of-fit test, no significant statistical difference for the presence of paranasal sinus fungus balls between ipsilateral and contralateral sides of nasal septal deviation and concha bullosa was noted (P=0.292 and P=0.593, resp.. In conclusion, we could not demonstrate any statistically significant correlation between the location of infected paranasal sinus, the direction of nasal septal deviation, and the location of concha bullosa, in location-limited rhinosinusitis lesions such as paranasal sinus fungal balls. We conclude that the anatomical variants discussed herein do not predispose patients to rhinosinusitis.

"EB, or Not EB?" Neonatal Desquamative Impetigo in a Degloving Pattern.

Science.gov (United States)

Nguyen, Tuyet A; Wang, Audrey S; Eichenfield, Lawrence F; Barrio, Victoria

2016-01-01

We present the case of a 7-day-old boy with significant, rapidly spreading blistering and desquamation in a "degloving" pattern on the hands that mimicked epidermolysis bullosa but was ultimately diagnosed as bullous impetigo caused by a clinically aggressive strain of Staphylococcus aureus. Bullous impetigo is a desquamating condition caused by local release of S. aureus exfoliative toxin A and is more commonly seen in children. This case highlights the fragility of newborn skin and reviews the major diagnoses that should be considered in an infant with significant blistering. © 2016 Wiley Periodicals, Inc.

Immunologically mediated oral diseases

OpenAIRE

Jimson, Sudha; Balachader, N.; Anita, N.; Babu, R.

2015-01-01

Immune mediated diseases of oral cavity are uncommon. The lesions may be self-limiting and undergo remission spontaneously. Among the immune mediated oral lesions the most important are lichen planus, pemphigus, erythema multiformi, epidermolysis bullosa, systemic lupus erythematosis. Cellular and humoral mediated immunity play a major role directed against epithelial and connective tissue in chronic and recurrent patterns. Confirmatory diagnosis can be made by biopsy, direct and indirect imm...

Stem-Cell Based Therapies for Epidermolysis Bullosa

Science.gov (United States)

2014-10-01

immunocompromised NSG mice. This task has not been initiated. Task 4.6 Derive mesenchymal cells from genetically corrected patient-specific JEB iPSC and...determine their ability to stably engraft long-term into the BM of immunocompromised NSG mice. This task has not been initiated. Key Research...differentiation protocol below (see Note 7). 1. Prewarm complete DKSFM (with antibiotics and DKSFM supplement) in the 37 C water bath. 2. Add 5 mL of prewarmed

Stem Cell-Based Therapies for Epidermolysis Bullosa

Science.gov (United States)

2015-12-01

Methods , 2014. 11(3): p. 291-3. Differentiation of Human Induced Pluripotent Stem Cells into a Keratinocyte Lineage Igor Kogut...discovery of methods for reprogramming adult somatic cells into induced pluripotent stem cells (iPSCs) has raised the possibility of producing truly...2013. Generation of functional mul- tipotent keratinocytes from mouse induced pluripotent stem cells . Methods Mol Biol 961: 337–350.

Erythrodermia Congenitalis Ichthyosiformis Bullosa of Brocq

Directory of Open Access Journals (Sweden)

Dorothea Sander

2016-01-01

Full Text Available A 50-year-old man presented with congenital scaling and hyperkeratosis on his palms, the soles of his feet and the extensor areas of his joints. The flexural areas were unaffected. His maternal grandmother, questionably his maternal uncle, his mother, all three brothers, one of his two sisters as well as two nephews and three nieces have or had similar skin changes. A punch biopsy was taken from the left palm. Clinical and histological signs led to the diagnosis of erythrodermia congenitalis ichthyosiformis bullosa of Brocq. We confirmed this genetically and found a heterozygous duplication (c.1752dupT in the keratin 1 gene (KRT-1. To our knowledge, this is the first case of this skin condition reported in the literature with a heterozygous duplication (c.1752dupT in KRT-1.

Vesicostomy as a Treatment Option for Epidermolisis Bullosa Case With Urethral and Meatal Involvement

Directory of Open Access Journals (Sweden)

Mahmoudreza Moradi

2016-07-01

Full Text Available Epidermolisis Bullosa (EB is a rare hereditary disorder that its junctional type is very rare one that involves epithelium, however, genitourinary epithelium involvement occurs so rarely. The present case is 5-year old boy; a known case of junctional EB whom had recurrent urinary retention due to meatal and urethral stenosis that was deteriorated by therapeutically interventions.

Peeling skin in newborn with abdominal distension

OpenAIRE

Sujay Kumar Maitra; Nilay Kanti Das; Niharika Ranjan Lal

2016-01-01

Epidermolysis bullosa (EB) is a rare hereditary disorder characterized by the formation of blisters following minor trauma. It has been traditionally categorized by the level of basement membrane zone separation into EB simplex, junctional EB, and dystrophic EB. Recently, hemidesmosomal EB has been proposed as a fourth category, which includes EB with muscular dystrophy and EB with pyloric atresia (PA). Among the subtypes, EB with PA is a rare form of EB. We report here a neonate with EB-PA, ...

Epidermolysis bullosa simplex: A case report | Peterside | Nigerian ...

African Journals Online (AJOL)

Nigerian Journal of Paediatrics. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 39, No 4 (2012) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register · Download this PDF file. The PDF file you selected should ...

Genetics Home Reference: epidermolysis bullosa with pyloric atresia

Science.gov (United States)

... caused by mutations in the ITGA6 , ITGB4 , and PLEC genes. These genes provide instructions for making proteins ... of EB-PA result from mutations in the PLEC gene. This gene provides instructions for making a ...

A Gene Gun-mediated Nonviral RNA trans-splicing Strategy for Col7a1 Repair

Directory of Open Access Journals (Sweden)

Patricia Peking

2016-01-01

Full Text Available RNA trans-splicing represents an auspicious option for the correction of genetic mutations at RNA level. Mutations within COL7A1 causing strong reduction or absence of type VII collagen are associated with the severe skin blistering disease dystrophic epidermolysis bullosa. The human COL7A1 mRNA constitutes a suitable target for this RNA therapy approach, as only a portion of the almost 9 kb transcript has to be delivered into the target cells. Here, we have proven the feasibility of 5′ trans-splicing into the Col7a1 mRNA in vitro and in vivo. We designed a 5′ RNA trans-splicing molecule, capable of replacing Col7a1 exons 1–15 and verified it in a fluorescence-based trans-splicing model system. Specific and efficient Col7a1 trans-splicing was confirmed in murine keratinocytes. To analyze trans-splicing in vivo, we used gene gun delivery of a minicircle expressing a FLAG-tagged 5′ RNA trans-splicing molecule into the skin of wild-type mice. Histological and immunofluorescence analysis of bombarded skin sections revealed vector delivery and expression within dermis and epidermis. Furthermore, we have detected trans-spliced type VII collagen protein using FLAG-tag antibodies. In conclusion, we describe a novel in vivo nonviral RNA therapy approach to restore type VII collagen expression for causative treatment of dystrophic epidermolysis bullosa.

Concha Bullosa Mucopyocele: a Case Report

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Kamran Sari

2016-03-01

Full Text Available Concha bullosa (CB is among the most common anatomic variations of sinonasal anatomy. Although usually asymptomatic, CB can occasionally cause nasal obstruction or headache. Obstructions within the mucociliary transport system can develop into a mucocele or mucopyocele. A 48-year-old female, with a history of progressive headache and nasal obstruction, was referred to our department. Paranasal sinus tomography revealed a nasal mass in the left nasal cavity resembling a mucopyocele in the middle turbinate. Under general anesthesia, the purulent material was aspirated, and the lateral part of the left turbinate was resected. Mucopyoceles are common within the paranasal sinuses, but uncommon with CB; thus, they should be considered in patients with a large hyperemic nasal mass.

Peeling skin in newborn with abdominal distension

Directory of Open Access Journals (Sweden)

Sujay Kumar Maitra

2016-01-01

Full Text Available Epidermolysis bullosa (EB is a rare hereditary disorder characterized by the formation of blisters following minor trauma. It has been traditionally categorized by the level of basement membrane zone separation into EB simplex, junctional EB, and dystrophic EB. Recently, hemidesmosomal EB has been proposed as a fourth category, which includes EB with muscular dystrophy and EB with pyloric atresia (PA. Among the subtypes, EB with PA is a rare form of EB. We report here a neonate with EB-PA, who, unfortunately, died on the 2nd day of life.

Merosin/laminin-2 and muscular dystrophy

DEFF Research Database (Denmark)

Wewer, U M; Engvall, E

1996-01-01

structural organization of domains, some of which have been assigned biological activities, including self-assembly and interactions with other proteins. The particular importance of laminins for the formation and stability of cell adhesion complexes is highlighted in severe inherited diseases of muscle...... and skin. Merosin is the collective name for laminins that share a common subunit, the laminin alpha 2 chain. Merosin-deficient congenital muscular dystrophy (CMD) is caused by mutations in the laminin alpha 2 chain gene. The skin disease Herlitz junctional epidermolysis bullosa is caused by mutations...

A Case Report of Angina Bullosa Haemorrhagica (ABH in Patient with Churg Strauss Syndrome

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M. Koopaie

2015-11-01

Full Text Available Bullosa Haemorrhagica (ABH is a rare oral blood blisters disorder. This recurrent disorder is restricted to oral mucosa and its difference with other oral vesiculobullos disorders is that blood vesicles are formed after minor trauma and in the absence of any blood dyscrasia, vesiculobullos disease and systemic disorder. This blood blisters are without pain, with color of dark red and full of bloods that rapidly enlarge and rapidly burst. The incidence of Angina Bullosa Haemorrhagica is unknown. Diagnosis of this disorder and differentiation from vesiculobullos disease like pemphigus and pemphiguid is important for patient's health. In this report we introduce a 55 years old woman with a 4­years history of oral blood blisters. These vesicles were in lateral border and ventral surface of tongue and on the ferenum. In medical history the patient had churg strauss syndrome and using inhalation corticosteroid for control of asthmatic attacks (An etiologic factor for appearing or aggravation of the disease.

Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa

NARCIS (Netherlands)

Jonkman, MF; Pasmooij, AMG; Pasmans, SGMA; van den Berg, MP; ter Horst, HJ; Timmer, A; Pas, HH

2005-01-01

The cytoplasmic plaque protein desmoplakin (DP), which is located in desmosomes, plays a major role in epithelial and muscle cell adhesion by linking the transmembrane cadherins to the cytoplasmic intermediate filament network. Mutations of DP may cause striate palmoplantar keratoderma,

Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007

Directory of Open Access Journals (Sweden)

Jennifer D Peterson

2008-11-01

Full Text Available Jennifer D Peterson1, Lawrence S Chan2,3,41Department of Dermatology, Texas Tech University Health Sciences Center at Lubbock, Lubbock, TX, USA; 2Department of Dermatology; 3Department of Microbiology/Immunology, University of Illinois at Chicago, Chicago, IL, USA; 4Medicine Service, Jesse Brown VA Medical Center, Chicago, IL, USAAbstract: In order to examine the efficacy and side effects of the monoclonal antibody anti-CD20 (rituximab on autoimmune blistering skin diseases, we performed a comprehensive survey of 71 consecutive patients from initial use up to 2007, using the PubMed database. A heterogeneous group of patients, including 51 patients with pemphigus vulgaris, one with pemphigus vegetans, nine with pemphigus foliaceus, five with paraneoplastic pemphigus, four with epidermolysis bullosa acquisita, and one with both bullous pemphigoid and graft vs host disease was included in this survey. Overall the monoclonal antibody seems to be effective in that 69% of patients showed complete response, 25% of patients showed partial response, whereas 6% of patients showed progressive disease. Six deaths occurred in association with the treatment, with four of these deaths in patients with paraneoplastic pemphigus, a disease characteristically resistant to conventional medication and with a high mortality rate. Of note, 11 patients who received combined rituximab and intravenous immune globulin treatments had the best outcome: complete response without any serious side effects. Therefore further investigation on rituximab with controlled clinical trial is a worthy pursuit.Keywords: blistering diseases, skin, anti-CD20, pemphigus, epidermolysis bullosa acquisita

Ichthyosis bullosa of Siemens: Response to topical tazarotene

Directory of Open Access Journals (Sweden)

Rajiv S

2006-01-01

Full Text Available In 1937, Siemens described a Dutch family with superficial blistering, flexural hyperkeratosis, and characteristic mauserung appearance. Since then, less than 20 kindreds with this condition have been described in the English dermatologic literature. A 14-year-old boy presented with history of recurrent blistering and peeling of skin since the age of 1 month, predominantly seen over limbs and trunk, often associated with secondary infection. His mother also had similar symptoms from childhood. On examination, the child had typical mauserung peeling of the skin and dirty gray hyperkeratosis in a rippled pattern over flexures. Skin biopsy from the boy showed intracorneal blistering with epidermolytic hyperkeratosis in the upper spinous layers. The typical history and clinical features along with characteristic histological findings confirmed our diagnosis of ichthyosis bullosa of Siemens. It must be differentiated from other conditions with epidermolytic hyperkeratosis and skin peeling, such as bullous ichthyosiform erythroderma of Brocq and peeling skin syndrome. Our patient responded well to 0.05% topical tazarotene gel over four weeks.

Use of Epidermolysis Bullosa Biomarkers in Models of Vesicant Injury

National Research Council Canada - National Science Library

Gerecke, Donald R

2006-01-01

.... The specific aim of the compound evaluation study was to determine the effectiveness of topically delivered synthetic MMP inhibitors, Ilomastat, GM1489, MMP- 2/MMP-9 Inhibitor I, and MMP-2/MMP-9...

NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeats.

Science.gov (United States)

Chen, M; Marinkovich, M P; Jones, J C; O'Toole, E A; Li, Y Y; Woodley, D T

1999-02-01

Type VII collagen, the major component of anchoring fibrils, consists of a central collagenous triple-helical domain flanked by two noncollagenous, globular domains, NC1 and NC2. Approximately 50% of the molecular mass of the molecule is consumed by the NC1 domain. We previously demonstrated that NC1 binds to various extracellular matrix components including a complex of laminin 5 and laminin 6 (Chen et al, 1997a). In this study, we examined the interaction of NC1 with laminin 5 (a component of anchoring filaments). Both authentic and purified recombinant NC1 bound to human and rat laminin 5 as measured by enzyme-linked immunosorbant assay and by binding of 125I-radiolabeled NC1 to laminin 5-coated wells, but not to laminin 1 or albumin. NC1 bound predominantly to the beta3 chain of laminin 5, but also to the gamma2 chain when examined by a protein overlay assay. The binding of 125I-NC1 to laminin 5 was inhibited by a 50-fold excess of unlabeled NC1 or de-glycosylated NC1, as well as a polyclonal antibody to laminin 5 or a monoclonal antibody to the beta3 chain. In contrast, the NC1-laminin 5 interaction was not affected by a monoclonal antibody to the alpha3 chain. Using NC1 deletion mutant recombinant proteins, a 285 AA (residues 760-1045) subdomain of NC1 was identified as the binding site for laminin 5. IgG from an epidermolysis bullosa acquisita serum containing autoantibodies to epitopes within NC1 that colocalized with the laminin 5 binding site inhibited the binding of NC1 to laminin 5. Thus, perturbation of the NC1-laminin 5 interaction may contribute to the pathogenesis of epidermolysis bullosa acquisita.

Immunologically mediated oral diseases.

Science.gov (United States)

Jimson, Sudha; Balachader, N; Anita, N; Babu, R

2015-04-01

Immune mediated diseases of oral cavity are uncommon. The lesions may be self-limiting and undergo remission spontaneously. Among the immune mediated oral lesions the most important are lichen planus, pemphigus, erythema multiformi, epidermolysis bullosa, systemic lupus erythematosis. Cellular and humoral mediated immunity play a major role directed against epithelial and connective tissue in chronic and recurrent patterns. Confirmatory diagnosis can be made by biopsy, direct and indirect immunoflouresence, immune precipitation and immunoblotting. Therapeutic agents should be selected after thorough evaluation of immune status through a variety of tests and after determining any aggravating or provoking factors. Early and appropriate diagnosis is important for proper treatment planning contributing to better prognosis and better quality of life of patient.

Molecular Characterization of Squamous Cell Carcinomas From Recessive Dystrophic Epidermolysis Bullosa

Science.gov (United States)

2006-09-01

8 A ppendices ...................................................................................... 8 3 Mahoney...activation after acute injury to stratified epithelia. Biochem. Biophys. Res. Commun. 236, 231-238. Cowin, P. (1994). Unraveling the cytoplasmic

A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies.

Science.gov (United States)

Hashimoto, T; Ishiko, A; Shimizu, H; Tanaka, T; Dodd, H J; Bhogal, B S; Black, M M; Nishikawa, T

1996-02-01

In this study we present a patient with the sublamina densa type of linear IgA bullous dermatosis (LABD), with IgA autoantibodies reactive with the 290-kDa type VII collagen (the epidermolysis bullosa acquisita (EBA) antigen) and with immunoblotting of normal human dermal extracts. The clinical and histological features of the present case were compatible with those of LABD but quite different from those of EBA. Although EBA sera reacted with the bacterial fusion protein of the N-terminal globular (NC1) domain of type VII collagen, this patient's serum did not show reactivity. Furthermore, ultrastructural localization of target epitopes on the anchoring fibrils in this patient was considerably different from EBA. These results indicate that, whereas EBA antibodies react with the NC1 domain of type VII collagen, the epitope in this case is different from that of EBA (and is most likely on the central triple helical domain). This difference may be responsible for the clinical presentation in this patient being distinct from that of EBA.

Immunologically mediated oral diseases

Directory of Open Access Journals (Sweden)

Sudha Jimson

2015-01-01

Full Text Available Immune mediated diseases of oral cavity are uncommon. The lesions may be self-limiting and undergo remission spontaneously. Among the immune mediated oral lesions the most important are lichen planus, pemphigus, erythema multiformi, epidermolysis bullosa, systemic lupus erythematosis. Cellular and humoral mediated immunity play a major role directed against epithelial and connective tissue in chronic and recurrent patterns. Confirmatory diagnosis can be made by biopsy, direct and indirect immunoflouresence, immune precipitation and immunoblotting. Therapeutic agents should be selected after thorough evaluation of immune status through a variety of tests and after determining any aggravating or provoking factors. Early and appropriate diagnosis is important for proper treatment planning contributing to better prognosis and better quality of life of patient.

Development of Genetic Therapies for the Hemidesmosol Subtypes of Junction Epidermolysis Bullosa

Science.gov (United States)

2003-11-01

Billoni et al, 2000). These compounds include activators of the nuclear hormone receptor PPAR, in particular, clofibrate (1 mg) and linoleic acid (1 mg...been shown to provide a variety of skin benefits. Prescription retinoic acid in icular has been shown to repair photodamaged skin and provide...R.A., Stolle, C.A. and Deak, S.B. (1991) Mammalian Tropoelastin: Multiple Domains of the Protein Define an Evolutionarily Divergent Amino Acid

Development of Genetic Therapies for the Hemidesmosol Subtypes of Junction Epidermolysis Bullosa

National Research Council Canada - National Science Library

Christiano, Angela

2003-01-01

... for in vitro epithelial reprogramming, in which we have begun to utilize different epithelial cell types as donor cells, in addition to keratinocytes, specifically, amnion and cornea cells. Collectively, we have shown that the use of gene delivery combined with epithelial cell-skin equivalent models, show significant promise toward developing a cellular.

Italian ethical committee intervention in a case of embryo-fetal medicine.

Science.gov (United States)

Cannovo, Nunzia; Buccelli, Paola; Bryce, Jane

2011-06-01

Embryo-fetal experimentation is intuitively associated with a therapeutic intent, according to a consolidated line of thought on the international and national levels. We report on a researcher's request for Ethics Committee approval to perform intrauterine transplantation of stem cells via cordocentesis on a fetus diagnosed with dystrophic epidermolysis bullosa, using stem cells obtained from a sibling's umbilical cord blood. The Ethics Committee rejected the request because of deontological issues and clinical judgments about the potential good to be derived from the procedure. In particular, in this case there was no preclinical or animal research on the procedure, the risk factors for mother and fetus were unknown, there was no way to guarantee compliance with Italian laws regarding safety and quality of the donor cells, and there was lack of clear informed consent.

Staphylococcal scalded skin syndrome in a 4-year-old child: a case report.

Science.gov (United States)

Haasnoot, P J; De Vries, A

2018-01-29

Staphylococcal scalded skin syndrome is an exfoliating skin disease which primarily affects children. Differential diagnosis includes toxic epidermal necrolysis, staphylococcal scalded skin syndrome, epidermolysis bullosa, and Stevens-Johnson syndrome. Staphylococcal scalded skin syndrome primarily affects children and can cause serious morbidity. In this case report we highlight the case of a 4-year-old Caucasian boy. Diagnostic and therapeutic challenges are discussed. Differential diagnoses are considered and therapy is described and discussed. The latest treatment options are used and described. Successful results are achieved in this case due to timely and correct management. Some therapeutic options are widely used without thorough research bases. This case report highlights staphylococcal scalded skin syndrome and its treatment, and future challenges. Further research is warranted and this case report aims to further research in exfoliating skin disorders.

Ectodermal Dysplasia Skin Fragility Syndrome

Directory of Open Access Journals (Sweden)

Ayça Alan Atalay

2014-06-01

Full Text Available Ectodermal dysplasia-skin fragility syndrome (EDSFS is a rare autosomal recessive genodermatosis first described in 1997 by Mc Grath. EDSFS results from loss of function mutations in plakophilin-1 (PKP1. PKP1 is a structural component of desmosomes, cellcell adhesion complexes. It is also found as a nuclear protein in several cell types that are lack of desmosomes. In skin, however, PKP1 expression is confined mainly to suprabasal keratinocytes and the outer root sheath of hair follicules. Loss of function mutation in PKP1 leads to extensive skin fragility, bullae and erosions following minor trauma, focal keratoderma with painful fissures, alopecia, and nail dystrophy. In some patients hypohidrosis may also be seen. EDSFS is now considered as a specific suprabasal form of epidermolysis bullosa simplex. In this report we describe a 20 year old EDSFS case.

CD1a, HAM56, CD68 and S-100 are present in lesional skin biopsies from patients affected by autoimmune blistering diseases

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Ana Maria Abreu Velez

2014-04-01

Full Text Available Introduction: Previous research on autoimmune skin blistering diseases (ABD has primarily focused on the humoral immune response; moreover, little attention has been given to the potential role of the antigen presenting cells (APCs in lesional skin. Aim: The purpose of our study was to immunophenotype selected APC in the lesional skin of ABDs, utilizing immunohistochemistry (IHC stains. Materials and Methods: We utilized IHC to stain for dendritic cells (DC, staining with CD1a, CD68, HAM56, and S-100 in lesional skin from 30 patients with endemic pemphigus foliaceus (EPF, 15 controls from the EPF endemic area, and 15 healthy controls from the USA. We also tested archival biopsies from patients with selected ABD, including 30 patients with bullous pemphigoid (BP, 20 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF and 14 with dermatitis herpetiformis (DH and 2 with epidermolysis bullosa acquisita (EBA. Results: Cells stained by CD68, HAM56 and S-100 were present in the majority of the ABD skin biopsies; these cells were located primarily in perivascular infiltrates surrounding dermal vessels subjacent to the blisters. However, these cells were also noted within the blisters, in vessels supplying dermal eccrine glands and ducts, and in areas of dermal endothelial-mesenchymal cell junction-like structures, especially in BP cases. In our CD1a staining, the number and location of positive staining cells varied with each disease, being abundant in most ABD in the epidermis suprajacent to the blisters, or in the epidermis surrounding the blister site if the blister site epidermis was missing. In the control biopsies, most did not display positive IHC staining, with the exception of a few CD1a positive cells in the epidermis Conclusion: Our findings confirm positive IHC staining for APCs in areas of the skin besides the disease blisters. Our findings suggest that the antigen presentation in ABD proceeds in areas distant from the blister site

Novel Molecular Therapies for Heritable Skin Disorders

Science.gov (United States)

Uitto, Jouni; Christiano, Angela M.; Irwin McLean, W. H.; McGrath, John A.

2013-01-01

Tremendous progress has been made in the past two decades in molecular genetics of heritable skin diseases, and pathogenic mutations have been identified in as many as 500 distinct human genes. This progress has resulted in improved diagnosis with prognostic implications, refined genetic counseling, and has formed the basis for prenatal and presymptomatic testing as well as preimplantation genetic diagnosis. However, there has been relatively little progress in developing effective and specific treatments for these often devastating diseases. Very recently, however, a number of novel molecular strategies, including gene therapy, cell-based approaches, and protein replacement therapy have been explored for treatment of these conditions. This overview will focus on the prototypic heritable blistering disorders, epidermolysis bullosa and related keratinopathies, in which significant progress has been recently made towards treatment, and illustrate how some of the translational research therapies have already entered the clinical arena. PMID:22158553

Electrocauterización de la membrana de Bowman para el tratamiento de la queratopatía bullosa dolorosa Electrocauterization of Bowman's membrane for the treatment of painful bullous keratopathy

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Elizabeth Escalona Leyva

2002-12-01

Full Text Available El propósito en este trabajo es evaluar la electrocauterización de la membrana de Bowman como alternativa quirúrgica en el tratamiento de la queratopatía bullosa dolorosa. Se estudiaron 30 ojos de 30 pacientes con diagnóstico de queratopatía bullosa dolorosa que no habían logrado mejoría con los tratamientos habituales. Se tomaron como variables fundamentales la edad, el sexo, la etiología, el tiempo de evolución, la epitelización, las complicaciones y el alivio sintomático, los datos se tabularon de forma manual teniendo en cuenta el universo de pacientes. Predominó la queratopatía bullosa posquirúrgica (afáquicas y pseudofáquicas en el 83,3 % de los pacientes. Las edades más afectadas fueron los mayores de 60 años (63,3 %; no se encontraron diferencias significativas en cuanto al sexo. El tiempo de epitelización fue de 7 a 14 días en el 93,3 % de los pacientes y solo se encontraron complicaciones en el 14 % que no fueron de gravedad. El alivio de los síntomas se produjo en el 66,6 % en la primera semana del posoperatorio. La electrocauterización de la membrana de Bowman constituye un método eficaz, sencillo y con resultados satisfactorios en el tratamiento de la queratopatía bullosa dolorosaThe purpose of this paper is to evaluate the electrocauterization of Bowman's membrane as a surgical alternative in the treatment of painful bullous keratopathy. 30 eyes from 30 patients who were diagnosed painful bullous keratopathy and had not improved with the habitual treatments were studied. Age, sex, ethiology, time of evolution, epithelialization, complications and the symptomatic relief were taken as fundamental variables. Data were manually tabulated, taking into account the number of patients. The psotsurgical bullous keratopathy (aphakic y pseudophakic prevailed in 83.3 % of the patients. The most affected were those over 60 (63.3 %. No significant differences were found in relation to sex. The time of epithelialization

Flame figures in linear IgA bullous dermatosis: a novel histopathologic finding.

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Fulton, E; Jan, F; Zimarowski, M J

2017-11-15

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal blistering disease usually with a neutrophil rich inflammatory infiltrate, and characterized by linear IgA deposition at the basement membrane zone (BMZ), and neutrophil predominant dermal inflammation. We report a case of LABD with numerous eosinophils and flame figure formation, a unique histopathologic finding not previously reported. A 69-year-old woman presented with a rapidly progressive, intensely pruritic rash over forearms, breasts, axillae, hips, and thighs. Thelesions were comprised of annular vesicles and bullae with hemorrhagic crusts and erosions. The clinical differential diagnosis included bullous pemphigoid(BP), LABD, and epidermolysis bullosa aquisita (EBA). A biopsy from a bullous plaque on the wrist revealed a subepidermal blister with neutrophils and numerous eosinophils with flame figure formation.Direct immunofluorescent (DIF) microscopy revealed linear deposition of IgA at the BMZ. Although unusual, the combined findings supported a diagnosis of LABD. Increased eosinophils may be associated with drug-induced LABD and may explain the numerous eosinophils in our case. It is important to be aware of this finding as the pathology may easily be misdiagnosed as BP, or possibly bullousWells syndrome. This case emphasizes that combined clinical, pathologic, and DIF findings are essential in the diagnosis of bullous dermatoses.

Biocompatibility of electrospun human albumin: a pilot study.

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Noszczyk, B H; Kowalczyk, T; Łyżniak, M; Zembrzycki, K; Mikułowski, G; Wysocki, J; Kawiak, J; Pojda, Z

2015-03-02

Albumin is rarely used for electrospinning because it does not form fibres in its native globular form. This paper presents a novel method for electrospinning human albumin from a solution containing pharmaceutical grade protein and 25% polyethylene oxide (PEO) used as the fibre-forming agent. After spontaneous cross-linking at body temperature, with no further chemicals added, the fibres become insoluble and the excess PEO can be washed out. Albumin deposited along the fibres retains its native characteristics, such as its non-adhesiveness to cells and its susceptibility for degradation by macrophages. To demonstrate this we evaluated the mechanical properties, biocompatibility and biodegradability of this novel product. After subcutaneous implantation in mice, albumin mats were completely resorbable within six days and elicited only a limited local inflammatory response. In vitro, the mats suppressed cell attachment and migration. As this product is inexpensive, produced from human pharmaceutical grade albumin without chemical modifications, retains its native protein properties and fulfils the specific requirements for anti-adhesive dressings, its clinical use can be expedited. We believe that it could specifically be used when treating paediatric patients with epidermolysis bullosa, in whom non-healing wounds occur after minor hand injuries which lead to rapid adhesions and devastating contractures.

Advanced skin, scar and wound care centre for children: A new era of care

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Andrew Burd

2012-01-01

Full Text Available Advanced wound care centres are now a well established response to the growing epidemic of chronic wounds in the adult population. Is the concept transferable to children? Whilst there is not the same prevalence of chronic wounds in children there are conditions affecting the integumentary system that do have a profound effect on the quality of life of both children and their families. We have identified conditions involving the skin, scars and wounds which contribute to a critical number of potential patients that can justify the setting up of an advanced skin, scar and wound care centre for children. The management of conditions such as giant naevi, extensive scarring and epidermolysis bullosa challenge medical professionals and lead to new and novel treatments to be developed. The variation between and within such conditions calls for a customizing of individual patient care that involves a close relationship between research scientists and clinicians. This is translational medicine of its best and we predict that this is the future of wound care particularly and specifically in children.

Mesenchymal Stem Cells for the Treatment of Skin Diseases

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Toshio Hasegawa

2017-08-01

Full Text Available Mesenchymal stem cell (MSC-based therapy involving both autologous and allogeneic MSCs shows great promise in treating several conditions. MSCs promote wound healing, and can differentiate into multiple cell lineages, including keratinocytes. Therefore, MSCs can be used for the treatment of congenital or acquired skin defects. Because of their immunomodulatory properties, MSCs may be useful for the treatment of inflammatory and autoimmune skin diseases. In particular, MSCs might be effective for the treatment of large vitiligo lesions as immunosuppressant or cultured grafts. MSCs can also be a novel cell source for regenerating hair in the treatment of scarring alopecia and androgenic alopecia. MSCs might also be an effective treatment for alopecia areata, which is associated with autoimmunity. Stem cell therapies with topical administration of MSCs and bone marrow transplantation were shown to alleviate recessive dystrophic epidermolysis bullosa in both animal models and human subjects. In addition to cell transplantation, the mobilization of endogenous MSCs has been attempted for skin regeneration. Overall, this review highlights the great potential of MSCs for the treatment of skin diseases in the near future.

Whole-exome sequencing, without prior linkage, identifies a mutation in LAMB3 as a cause of dominant hypoplastic amelogenesis imperfecta.

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Poulter, James A; El-Sayed, Walid; Shore, Roger C; Kirkham, Jennifer; Inglehearn, Chris F; Mighell, Alan J

2014-01-01

The conventional approach to identifying the defective gene in a family with an inherited disease is to find the disease locus through family studies. However, the rapid development and decreasing cost of next generation sequencing facilitates a more direct approach. Here, we report the identification of a frameshift mutation in LAMB3 as a cause of dominant hypoplastic amelogenesis imperfecta (AI). Whole-exome sequencing of three affected family members and subsequent filtering of shared variants, without prior genetic linkage, sufficed to identify the pathogenic variant. Simultaneous analysis of multiple family members confirms segregation, enhancing the power to filter the genetic variation found and leading to rapid identification of the pathogenic variant. LAMB3 encodes a subunit of Laminin-5, one of a family of basement membrane proteins with essential functions in cell growth, movement and adhesion. Homozygous LAMB3 mutations cause junctional epidermolysis bullosa (JEB) and enamel defects are seen in JEB cases. However, to our knowledge, this is the first report of dominant AI due to a LAMB3 mutation in the absence of JEB.

Diagnóstico pré-natal das genodermatoses Prenatal diagnosis of genodermatoses

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Maria Carolina de Abreu Sampaio

2007-08-01

Full Text Available O diagnóstico pré-natal está indicado para algumas genodermatoses graves, como a epidermólise bolhosa distrófica recessiva e a epidermólise bolhosa juncional. A biópsia de pele fetal foi introduzida em 1980, mas não pode ser realizada antes da 15a semana de gestação. A análise do DNA fetal é método preciso e pode ser realizado mais precocemente na gestação. No entanto, deve-se conhecer a base molecular da genodermatose, e é essencial determinar a mutação e/ou marcadores informativos nas famílias com criança previamente afetada. O DNA fetal pode ser obtido pela biópsia da vilosidade coriônica ou amniocentese. O diagnóstico genético pré-implantação tem surgido como alternativa que dispensa a interrupção da gestação. Essa técnica, que envolve fertilização in vitro e teste genético do embrião. vem sendo realizada para genodermatoses em poucos centros de referência. A ultra-sonografia é exame não invasivo, mas tem uso limitado no diagnóstico pré-natal de genodermatoses. A ultrasonografia tridimensional geralmente estabelece o diagnóstico tardiamente na gestação, e há apenas relatos anedóticos de diagnóstico pré-natal de genodermatoses usando esse método.Prenatal diagnostic testing is indicated for some severe genodermatoses, such as recessive dystrophic epidermolysis bullosa and junctional epidermolysis bullosa. Fetal skin biopsy was introduced in 1980, but it cannot be performed before 15th gestational week. Fetal DNA analysis is a precise method and can be performed earlier in pregnancy. However, the molecular basis of the genodermatoses must be known and it is essential to determine the gene mutations and/or informative markers in the families with a previously affected child. Fetal DNA can be obtained by chorionic villus sampling or amniocentesis. Preimplantation genetic diagnosis is an alternative approach obviating the need for termination of pregnancy. It involves in vitro fertilization and

Innate sensing of microbial products promotes wound-induced skin cancer

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Hoste, Esther; Arwert, Esther N.; Lal, Rohit; South, Andrew P.; Salas-Alanis, Julio C.; Murrell, Dedee F.; Donati, Giacomo; Watt, Fiona M.

2015-01-01

The association between tissue damage, chronic inflammation and cancer is well known. However, the underlying mechanisms are unclear. Here we characterize a mouse model in which constitutive epidermal extracellular-signal-regulated kinase-MAP-kinase signalling results in epidermal inflammation, and skin wounding induces tumours. We show that tumour incidence correlates with wound size and inflammatory infiltrate. Ablation of tumour necrosis factor receptor (TNFR)-1/-2, Myeloid Differentiation primary response gene 88 or Toll-like receptor (TLR)-5, the bacterial flagellin receptor, but not other innate immune sensors, in radiosensitive leukocytes protects against tumour formation. Antibiotic treatment inhibits, whereas injection of flagellin induces, tumours in a TLR-5-dependent manner. TLR-5 is also involved in chemical-induced skin carcinogenesis in wild-type mice. Leukocytic TLR-5 signalling mediates upregulation of the alarmin HMGB1 (High Mobility Group Box 1) in wound-induced papillomas. HMGB1 is elevated in tumours of patients with Recessive Dystrophic Epidermolysis Bullosa, a disease characterized by chronic skin damage. We conclude that in our experimental model the combination of bacteria, chronic inflammation and wounding cooperate to trigger skin cancer. PMID:25575023

BP180 dysfunction triggers spontaneous skin inflammation in mice.

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Zhang, Yang; Hwang, Bin-Jin; Liu, Zhen; Li, Ning; Lough, Kendall; Williams, Scott E; Chen, Jinbo; Burette, Susan W; Diaz, Luis A; Su, Maureen A; Xiao, Shengxiang; Liu, Zhi

2018-06-04

BP180, also known as collagen XVII, is a hemidesmosomal component and plays a key role in maintaining skin dermal/epidermal adhesion. Dysfunction of BP180, either through genetic mutations in junctional epidermolysis bullosa (JEB) or autoantibody insult in bullous pemphigoid (BP), leads to subepidermal blistering accompanied by skin inflammation. However, whether BP180 is involved in skin inflammation remains unknown. To address this question, we generated a BP180-dysfunctional mouse strain and found that mice lacking functional BP180 (termed Δ NC16A ) developed spontaneous skin inflammatory disease, characterized by severe itch, defective skin barrier, infiltrating immune cells, elevated serum IgE levels, and increased expression of thymic stromal lymphopoietin (TSLP). Severe itch is independent of adaptive immunity and histamine, but dependent on increased expression of TSLP by keratinocytes. In addition, a high TSLP expression is detected in BP patients. Our data provide direct evidence showing that BP180 regulates skin inflammation independently of adaptive immunity, and BP180 dysfunction leads to a TSLP-mediated itch. The newly developed mouse strain could be a model for elucidation of disease mechanisms and development of novel therapeutic strategies for skin inflammation and BP180-related skin conditions.

Functional testing of keratin 14 mutant proteins associated with the three major subtypes of epidermolysis bullosa simplex

DEFF Research Database (Denmark)

Sørensen, Charlotte B; Andresen, Brage S; Jensen, Uffe B

2003-01-01

vectors were transiently transfected into normal human primary keratinocytes (NHK), HaCaT or HeLa cells in order to analyze the ability of the mutant K14 proteins to integrate into the existing endogenous keratin filament network (KFN). No effect on the keratin cytoskeleton was observed upon transfection...... of NHK with the various K14 constructs neither with nor without a subsequently induced heat-stress. In contrast, all constructs, including wild-type K14, caused collapse of the endogenous KFN in a small fraction of the transfected HeLa and HaCaT cells. However, overexpression of the mutation associated...... with the most severe form of the disease, EBS Dowling-Meara, resulted in a higher number of transfected HaCaT cells with KFN collapse (P

A model of user engagement in medical device development.

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Grocott, Patricia; Weir, Heather; Ram, Mala Bridgelal

2007-01-01

The purpose of this paper is to address three topical themes: user involvement in health services research; determining the value of new medical technologies in patient care pathways, furthering knowledge related to quality in health and social care; and knowledge exchange between manufacturers, health service supply chain networks and device users. The model is being validated in a case study in progress. The latter is a "proving ground" study for a translational research company. Medical devices play a pivotal role in the management of chronic diseases, across all care settings. Failure to engage users in device development inevitably affects the quality of clinical outcomes. A model of user engagement is presented, turning unmet needs for medical devices into viable commercial propositions. A case study investigating the perceptions of individuals with Epidermolysis Bullosa (EB), their lay and professional carers into unmet needs. EB is an inherited condition affecting the skin and mucosal linings that leads to blistering and wounds. Qualitative data are being collected to generate understanding of unmet needs and wound care products. These needs are being translated into new design concepts and prototypes. Prototypes will be evaluated in an n = 1 experimental design, generating quantitative outcomes data. There are generalisations from the case study, and the model outlined. New products for managing EB wounds can logically benefit other groups. The model is transferable to other clinical problems, which can benefit from research and technological advances that are integral to clinical needs and care.

Novel Tissue Models of Junctional Epidermolysis Bullosa to Characterize Functional Mechanisms of Sulfur Mustard Injury to Human Skin

National Research Council Canada - National Science Library

Garlick, Joanthan

2003-01-01

In the second year of our research, our laboratory has extensively studied skin pathophysiology in response to SM by adapting in vivo, human skin/nude mouse chimera to further understand mechanisms...

Manufacturing of Human Extracellular Vesicle-Based Therapeutics for Clinical Use

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Mario Gimona

2017-06-01

Full Text Available Extracellular vesicles (EVs derived from stem and progenitor cells may have therapeutic effects comparable to their parental cells and are considered promising agents for the treatment of a variety of diseases. To this end, strategies must be designed to successfully translate EV research and to develop safe and efficacious therapies, whilst taking into account the applicable regulations. Here, we discuss the requirements for manufacturing, safety, and efficacy testing of EVs along their path from the laboratory to the patient. Development of EV-therapeutics is influenced by the source cell types and the target diseases. In this article, we express our view based on our experience in manufacturing biological therapeutics for routine use or clinical testing, and focus on strategies for advancing mesenchymal stromal cell (MSC-derived EV-based therapies. We also discuss the rationale for testing MSC-EVs in selected diseases with an unmet clinical need such as critical size bone defects, epidermolysis bullosa and spinal cord injury. While the scientific community, pharmaceutical companies and clinicians are at the point of entering into clinical trials for testing the therapeutic potential of various EV-based products, the identification of the mode of action underlying the suggested potency in each therapeutic approach remains a major challenge to the translational path.

Epidermólisis bulosa en el niño y anestesia

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Joaquín L. de la Lastra Rodríguez

1999-06-01

Full Text Available La epidermólisis bulosa comprende un grupo heterogéneo de enfermedades ampollosas de la piel y mucosas de origen congénito y hereditario. El tratamiento es insatisfactorio en gran parte, por lo cual son necesarias ciertas medidas para proteger al paciente y evitar la aparición de dichas lesiones y sus complicaciones. Se describe el manejo anestesiológico en un niño de 3 años de edad, al que se le administró anestesia general intravenosa con clorhidrato de ketamina y diazepán, y se describen todos los cuidados y recomendaciones que hay que tener para evitar especialmente los roces, las presiones y las temperaturas altas.Epidermolysis bullosa is a heterogenous group of ampullary diseases of the skin and mucosas of congenital and hereditary origin. The treatment is largely unsatisfactory, and that´s why it is necessary to take some measures to protect the patient and to prevent the appareance of such lesions and their complications. It is described the anesthetic management of a 3-year-old child that was administered intravenous general anesthesia with ketamine hydrochlorate and diazepam. The care and recommendations that should be taken into account to avoid touches, pressures and high temperatures, are described.

Botanicals With Dermatologic Properties Derived From First Nations Healing: Part 1-Trees.

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Colantonio, Sophia; Rivers, Jason K

First Nations people have a long history of working with medicinal plants used to treat skin diseases. The purpose was to assess the dermatologic therapeutic potential of western red cedar, white spruce, birch, balsam poplar, and black spruce. Based on expert recommendations, 5 trees were selected that were used in First Nations medicine for cutaneous healing and have potential and/or current application to dermatology today. We searched several databases up to June 12, 2014. Western red cedar's known active principal compound, β-thujaplicin, has been studied in atopic dermatitis. White spruce's known active principal compound, 7-hydroxymatairesinol, has anti-inflammatory activity, while phase II clinical trials have been completed on a birch bark emulsion for the treatment of actinic keratoses, epidermolysis bullosa, and the healing of split thickness graft donor sites. Balsam poplar has been used clinically as an anti-aging remedy. Black spruce bark contains higher amounts of the anti-oxidant trans-resveratrol than red wine. North American traditional medicine has identified important botanical agents that are potentially relevant to both cosmetic and medical dermatology. This study is limited by the lack of good quality evidence contributing to the review. The article is limited to 5 trees, a fraction of those used by First Nations with dermatological properties.

The Effect of Autologous Serum Eye Drop Application on Epithelization in the Treatment of Various Ocular Surface Disorders and its Safety

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Fatma Selin Kaya

2012-10-01

Full Text Available Pur po se: To evaluate the effect of autologous serum application on epithelization in the treatment of ocular surface disorders in hard cases and its safety. Ma te ri al and Met hod: Patients with serious ocular surface disorders, unresponsive to conventional treatment were recruited. Clinical features of retrospective cohort of patients who were prescribed serum drops are presented. From July 2007 to January 2010, 31 eyes of 21 patients, who were given autologous serum eye drops, were included into the study. Clinical examination included epithelial changes, rose bengal/lissamine green staining, fluorescein staining, and tear film break-up time. A history of systemic disease was recorded together with systemic medications used. A complete ocular history was also obtained. Re sults: Autologous serum was used in 7 patients with delayed epithelization after penetrating keratoplasty, in 4 patients with epithelial disturbances secondary to keratitis, in 2 patients with alkali burns, in 3 patients with Stevens-Johnson syndrome, in 1 patient with ligneous conjunctivitis, in 1 patient with epidermolysis bullosa, in 1 patient with corneal burn with hot water, and in 2 patients with Sjogren syndrome. The female:male ratio was 13:8. The mean age was 36.23±24.80 standard deviation (range: 7 months-87 years. No significant sight-threatening complication has been observed with the use of serum drops. Dis cus si on: Autologous serum application is safe and efficient additional therapy in the treatment of serious ocular surface problems in difficult cases. (Turk J Ophthalmol 2012; 42: 336-41

HLA-DPDQDR is expressed in all lesional skin from patients with autoimmune skin diseases

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Ana Maria Abreu Velez

2014-04-01

Full Text Available Introduction: Human genes responsible for human antigen presentation and transplant rejection functions are located on the short arm of Chromosome 6 and are called the Major Histocompatibility Complex (MHC. Moreover, the primary physiologic function of MHC molecules is to present peptides to T lymphocytes. MHC molecules are integral components of the ligands that most T cells recognize, since the T cell receptor (TCR has specificity for complexes of foreign antigenic peptides, as well as self-MHC molecules. Aim: Our investigation attempts to investigate the presence of HLA-DPDQDR within lesional skin biopsies from patients affected by autoimmune skin blistering diseases (ABDs. Materials and Methods: We utilized immunohistochemistry (IHC to evaluate the presence of HLA-DPDQDR in lesional skin biopsies of patients affected by ABDs. We tested 30 patients with endemic pemphigus foliaceus (EPF, 15 controls from the EPF endemic area, and 15 biopsies from healthy controls from the USA. We also tested archival biopsies from patients with selected ABDs, including 30 patients with bullous pemphigoid (BP, 20 with pemphigus vulgaris (PV, 8 with pemphigus foliaceus (PF, 14 with dermatitis herpetiformis (DH and 2 with epidermolysis bullosa acquisita (EBA. Results: Most ABD biopsies stained positive for HLA-DPDQDR in the lesional blisters and/or inflamed neurovascular plexus in the superficial dermis, and also at mesenchymal-endothelial like-cell junctions in the dermis. In BP, EBA and EPF, the HLA-DPDQDR staining was also seen in the dermal eccrine sweat gland coils and and ducts. Conclusion: Here, we document that HLA-DPDQDR is expressed in several anatomic areas of lesional skin in patients with ABDs. Notably, HLA-DPDQDR positivity was also consistently present in areas of the classic immune response in pemphigus epidermal keratinocytic intercellular junctions, and at basement membrane sites in bullous pemphigoid and other subepidermal blistering diseases.

Purpura fulminans mimicking toxic epidermal necrolysis - additional value of 16S rRNA sequencing and skin biopsy.

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Dautzenberg, K H W; Polderman, F N; van Suylen, R J; Moviat, M A M

2017-05-01

Both purpura fulminans and toxic epidermal necrolysis (TEN) are rare and life-threatening disorders with a high mortality. We present a case of suspected rapidly progressive, severe pneumococcal sepsis-induced purpura fulminans complicated by multiple organ failure, severe epidermolysis and cutaneous necrosis. We show the diagnostic challenge to differentiate between purpura fulminans and TEN, as the extensive epidermolysis in purpura fulminans may mimic TEN and we highlight the additional value of repeated skin biopsies and 16S rRNA gene sequencing.

Management strategy in pregnancies with elevated second-trimester maternal serum alpha-fetoprotein based on a second assay.

Science.gov (United States)

Spaggiari, Emmanuel; Ruas, Marie; Dreux, Sophie; Valat, Anne-Sylvie; Czerkiewicz, Isabelle; Guimiot, Fabien; Schmitz, Thomas; Delezoide, Anne-Lise; Muller, Françoise

2013-04-01

To assess maternal-fetal outcomes in pregnancies associated with persistently elevated second-trimester maternal serum alpha-fetoprotein. A retrospective cohort study in 658 patients with maternal serum alpha-fetoprotein ≥2.5 multiple of median, performed at routine Down syndrome screening. Maternal serum alpha-fetoprotein was assayed a second time in 341 of them. Outcomes were recorded in all cases. The group with unexplained maternal serum alpha-fetoprotein persistently ≥2.5 multiple of median was associated with more pregnancy complications 37 of 92 (40.2%) as fetal death, preeclampsia, intrauterine growth restriction, and congenital nephrotic syndrome, compared with the group with maternal serum alpha-fetoprotein that returned to a normal level 37 of 226 (16.4%) (P alpha-fetoprotein returns to a normal level on a second assay, the risk of adverse outcome significantly decreases, but these pregnancies are still at risk of complications and therefore need close surveillance. Repeat maternal serum alpha-fetoprotein assay allows identification of patients who should be offered amniocentesis to evaluate the risk of nephrotic syndrome and epidermolysis bullosa. Alpha-fetoprotein should be monitored in pregnancies associated with unexplained high maternal serum alpha-fetoprotein. A management strategy based on ultrasound examination, second maternal serum alpha-fetoprotein assay and amniocentesis is proposed to improve prenatal counseling and management of such pregnancies. However, a prospective study remains necessary to evaluate it. Copyright © 2013 Mosby, Inc. All rights reserved.

Aberrant expression and secretion of heat shock protein 90 in patients with bullous pemphigoid.

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Stefan Tukaj

Full Text Available The cell stress chaperone heat shock protein 90 (Hsp90 has been implicated in inflammatory responses and its inhibition has proven successful in different mouse models of autoimmune diseases, including epidermolysis bullosa acquisita. Here, we investigated expression levels and secretory responses of Hsp90 in patients with bullous pemphigoid (BP, the most common subepidermal autoimmune blistering skin disease. In comparison to healthy controls, the following observations were made: (i Hsp90 was highly expressed in the skin of BP patients, whereas its serum levels were decreased and inversely associated with IgG autoantibody levels against the NC16A immunodominant region of the BP180 autoantigen, (ii in contrast, neither aberrant levels of circulating Hsp90 nor any correlation of this protein with serum autoantibodies was found in a control cohort of autoimmune bullous disease patients with pemphigus vulgaris, (iii Hsp90 was highly expressed in and restrictedly released from peripheral blood mononuclear cells of BP patients, and (iv Hsp90 was potently induced in and restrictedly secreted from human keratinocyte (HaCaT cells by BP serum and isolated anti-BP180 NC16A IgG autoantibodies, respectively. Our results reveal an upregulated Hsp90 expression at the site of inflammation and an autoantibody-mediated dysregulation of the intracellular and extracellular distribution of this chaperone in BP patients. These findings suggest that Hsp90 may play a pathophysiological role and represent a novel potential treatment target in BP.

Dental implants in patients with oral mucosal diseases - a systematic review.

Science.gov (United States)

Reichart, P A; Schmidt-Westhausen, A M; Khongkhunthian, P; Strietzel, F P

2016-05-01

To reveal dental implants survival rates in patients with oral mucosal diseases: oral lichen planus (OLP), Sjögren's syndrome (SjS), epidermolysis bullosa (EB) and systemic sclerosis (SSc). A systematic literature search using PubMed/Medline and Embase databases, utilising MeSH and search term combinations identified publications on clinical use implant-prosthetic rehabilitation in patients with OLP, SjS, EB, SSc reporting on study design, number, gender and age of patients, follow-up period exceeding 12 months, implant survival rate, published in English between 1980 and May 2015. After a mean observation period (mOP) of 53·9 months (standard deviation [SD] ±18·3), 191 implants in 57 patients with OLP showed a survival rate (SR) of 95·3% (SD ±21·2). For 17 patients with SjS (121 implants, mOP 48·6 ± 28·7 months), 28 patients with EB (165 implants, mOP 38·3 ± 16·9 months) and five patients with SSc (38 implants, mOP 38·3 ± 16·9 months), the respective SR was 91·7 ± 5·97% (SjS), 98·5 ± 2·7% (EB) and 97·4 ± 4·8% (SSc). Heterogeneity of data structure and quality of reporting outcomes did not allow for further comparative data analysis. For implant-prosthetic rehabilitation of patients suffering from OLP, SjS, EB and SSc, no evidence-based treatment guidelines are presently available. However, no strict contraindication for the placement of implants seems to be justified in patients with OLP, SjS, EB nor SSc. Implant survival rates are comparable to those of patients without oral mucosal diseases. Treatment guidelines as for dental implantation in patients with healthy oral mucosa should be followed. © 2015 John Wiley & Sons Ltd.

STUDY ON NONINFECTIOUS DERMATOSES IN PAEDIATRIC AGE

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Ananthi Mahalingam

2017-09-01

Full Text Available BACKGROUND Paediatric dermatology is a unique subspecialty in that child is not a miniature adult. Paediatric dermatoses differ from that of the adults in clinical presentation, treatment and prognosis. Various studies from India have shown infections and infestations to be the most common paediatric dermatoses. This study was planned to determine the epidemiological pattern of common noninfectious dermatoses in our paediatric patients as no such data are available from this part of the country. A cross-sectional study was undertaken to study the prevalence of the noninfectious dermatoses in all the new paediatric patients attending the Skin Outpatient Department (OPD at Villupuram Medical College over a period of three years. MATERIALS AND METHODS A total number of 550 children in the age group ranging from newborn to 12 years with noninfectious dermatoses attending the OPD for the first time were enrolled in the study. RESULTS Physiological changes of skin was the most common dermatoses in the newborn age group, while eczema was the most common dermatoses in infants, preschool and school going children. In the infants, eczema was followed by pigmentary disorders, mongolian spots, vascular nevi, ichthyosis, epidermolysis bullosa, alopecia areata and papular urticaria in the order of prevalence. Among preschool going children, eczema was followed by papular urticaria, papulosquamous disorders, pigmentary disorders, hair disorders, nevi, drug reactions, keratinisation disorders, urticaria, etc. In the school going age group, eczema was followed by papulosquamous disorders, pigmentary disorders, papular urticaria, nutritional disorders, ichthyosis, nevi, miliaria, drug reaction, hair disorders, photodermatoses, urticaria, collagen vascular disease and vascular nevi in the order of prevalence. CONCLUSION Eczema, papulosquamous disorders, papular urticaria, pigmentary disorders seem to be the most common noninfectious dermatoses in children. However

Longitudinal erythronychia: individual or multiple linear red bands of the nail plate: a review of clinical features and associated conditions.

Science.gov (United States)

Cohen, Philip R

2011-08-01

Longitudinal erythronychia is a linear red band on the nail plate that originates at the proximal nail fold, traverses the lunula, and extends to the free edge of the nail plate. Longitudinal erythronychia is classified based upon the number of nails affected and the number of red streaks present on each nail as follows: type Ia (monodactylous - single band), type Ib (monodactylous - bifid bands), type IIa (polydactylous - single band), and type IIb (polydactylous - multiple bands). Associated morphologic findings that can be present at the distal tip of the nail with longitudinal erythronychia include fragility, onycholysis, splinter hemorrhage, splitting, subungual keratosis, thinning, and V-shaped nick. Some patients with longitudinal erythronychia seek medical evaluation because of pain in the associated distal digit; however, the linear red nail plate dyschromia is often asymptomatic and the individual is concerned about the cosmetic appearance or distal nail fragility. Longitudinal erythronychia can be a clinical manifestation of an underlying local or systemic condition. Benign tumors (glomus tumor, onychopapilloma, and warty dyskeratoma), malignant neoplasms (malignant melanoma and squamous cell carcinoma), and other conditions (hemiplegia and postsurgical scar) can be associated with monodactylous longitudinal erythronychia or it may be idiopathic or the initial stage of polydactylous longitudinal erythronychia-associated systemic conditions. Polydactylous longitudinal erythronychia is most commonly reported in patients with Darier disease (keratosis follicularis); other associated conditions include acantholytic dyskeratotic epidermal nevus, acantholytic epidermolysis bullosa, acrokeratosis verruciformis of Hopf, amyloidosis, graft-versus-host disease, lichen planus, and pseudobulbar syndrome. Polydactylous longitudinal erythronychia has also been observed as an idiopathic finding. Biopsy of the nail matrix and nail bed may be necessary to establish the

Evaluation of the effect and mechanism of action of local phenytoin in treatment of vitiligo.

Science.gov (United States)

Abdou, Asmaa Gaber; Abdelwahed Gaber, Mohammed; Elnaidany, Nada Farag; Elnagar, Ayat

2017-01-01

There are many theories explaining vitiligo such as genetic, autoimmune, neural, free radicals, biochemical, intrinsic defect, melanocytorrhagy, and convergent theories. Phenytoin is a widely used anticonvulsant, which is used in cutaneous medicine for treatment of ulcers and epidermolysis bullosa. The aim of this study is to assess the effectiveness of topical phenytoin gel in the treatment of vitiligo patients and explaining the underlying mechanism using immunohistochemistry for evaluation of HMB45, CD4, and CD8. Only 9 patients out of 28 experienced response to phenytoin in the form of dull, white color change and light brown color. Post-phenytoin treatment biopsies showed decreased density of inflammation, increased melanin and increased HMB45 positive cells together with an increased number of CD4 positive lymphocytes and decreased number of CD8 positive lymphocytes. These observations did not reach significant level (P > 0.05). A high percentage of CD4 positive lymphocytes was significantly associated with a long duration of vitiligo (p = 0.03) and segmental vitiligo type (p = 0.02). The current study applied phenytoin as 2% concentrated gel for 3 months, which is a relatively short duration without observed side effects throughout the period. These results indicate that topical phenytoin of low concentrations may have beneficial effects through immunomodulatory activity by affecting CD4 and CD8 counts and subsequently the ratio between them. Further studies are recommended to combine phenytoin with other antivitiligo agents as local corticosteroids or phototherapy to clarify if it could potentiate their effects.

Under-recognition of acral peeling skin syndrome: 59 new cases with 15 novel mutations.

Science.gov (United States)

Szczecinska, W; Nesteruk, D; Wertheim-Tysarowska, K; Greenblatt, D T; Baty, D; Browne, F; Liu, L; Ozoemena, L; Terron-Kwiatkowski, A; McGrath, J A; Mellerio, J E; Morton, J; Woźniak, K; Kowalewski, C; Has, C; Moss, C

2014-11-01

Acral peeling skin syndrome (APSS) is a rare skin fragility disorder usually caused by mutations in the transglutaminase 5 gene (TGM5). We investigated the mutation spectrum of APSS in the U.K., Germany and Poland. We identified 59 children with APSS from 52 families. The phenotype was readily recognizable, with some variation in severity both within and between families. Most cases had been misdiagnosed as the localized form of epidermolysis bullosa simplex (EBS-loc). Eighteen different TGM5 mutations were identified, 15 of which were novel. Eight mutations were unique to a single family, nine each occurred in two families, while the common p.Gly113Cys mutation linked to a second missense variant p.Thr109Met occurred in 47 of the 52 families and was homozygous in 28. Most patients were of nonconsanguineous white European origin. We propose that APSS is under-reported and widely misdiagnosed as EBS-loc, with significant counselling implications as APSS is autosomal recessive while EBS-loc is dominant. We recommend screening for TGM5 mutations when EBS-loc is suspected but not confirmed by mutations in KRT5 or KRT14. Our report trebles the number of known TGM5 mutations. It provides further evidence that p.Gly113Cys is a founder mutation in the European population. This is consistent with the striking ethnic distribution of APSS in U.K., where the majority of patients are of nonconsanguineous white European origin, in contrast to the pattern of other recessive skin disorders. © 2014 British Association of Dermatologists.

Novel ENAM and LAMB3 mutations in Chinese families with hypoplastic amelogenesis imperfecta.

Science.gov (United States)

Wang, Xin; Zhao, Yuming; Yang, Yuan; Qin, Man

2015-01-01

Amelogenesis imperfecta is a group of inherited diseases affecting the quality and quantity of dental enamel. To date, mutations in more than ten genes have been associated with non-syndromic amelogenesis imperfecta (AI). Among these, ENAM and LAMB3 mutations are known to be parts of the etiology of hypoplastic AI in human cases. When both alleles of LAMB3 are defective, it could cause junctional epidermolysis bullosa (JEB), while with only one mutant allele in the C-terminus of LAMB3, it could result in severe hypoplastic AI without skin fragility. We enrolled three Chinese families with hypoplastic autosomal-dominant AI. Despite the diagnosis falling into the same type, the characteristics of their enamel hypoplasia were different. Screening of ENAM and LAMB3 genes was performed by direct sequencing of genomic DNA from blood samples. Disease-causing mutations were identified and perfectly segregated with the enamel defects in three families: a 19-bp insertion mutation in the exon 7 of ENAM (c.406_407insTCAAAAAAGCCGACCACAA, p.K136Ifs*16) in Family 1, a single-base deletion mutation in the exon 5 of ENAM (c. 139delA, p. M47Cfs*11) in Family 2, and a LAMB3 nonsense mutation in the last exon (c.3466C>T, p.Q1156X) in Family 3. Our results suggest that heterozygous mutations in ENAM and LAMB3 genes can cause hypoplastic AI with markedly different phenotypes in Chinese patients. And these findings extend the mutation spectrum of both genes and can be used for mutation screening of AI in the Chinese population.

Novel ENAM and LAMB3 mutations in Chinese families with hypoplastic amelogenesis imperfecta.

Directory of Open Access Journals (Sweden)

Xin Wang

Full Text Available Amelogenesis imperfecta is a group of inherited diseases affecting the quality and quantity of dental enamel. To date, mutations in more than ten genes have been associated with non-syndromic amelogenesis imperfecta (AI. Among these, ENAM and LAMB3 mutations are known to be parts of the etiology of hypoplastic AI in human cases. When both alleles of LAMB3 are defective, it could cause junctional epidermolysis bullosa (JEB, while with only one mutant allele in the C-terminus of LAMB3, it could result in severe hypoplastic AI without skin fragility. We enrolled three Chinese families with hypoplastic autosomal-dominant AI. Despite the diagnosis falling into the same type, the characteristics of their enamel hypoplasia were different. Screening of ENAM and LAMB3 genes was performed by direct sequencing of genomic DNA from blood samples. Disease-causing mutations were identified and perfectly segregated with the enamel defects in three families: a 19-bp insertion mutation in the exon 7 of ENAM (c.406_407insTCAAAAAAGCCGACCACAA, p.K136Ifs*16 in Family 1, a single-base deletion mutation in the exon 5 of ENAM (c. 139delA, p. M47Cfs*11 in Family 2, and a LAMB3 nonsense mutation in the last exon (c.3466C>T, p.Q1156X in Family 3. Our results suggest that heterozygous mutations in ENAM and LAMB3 genes can cause hypoplastic AI with markedly different phenotypes in Chinese patients. And these findings extend the mutation spectrum of both genes and can be used for mutation screening of AI in the Chinese population.

Impetigo asacause of sepsis ininfants

Directory of Open Access Journals (Sweden)

Alicja Moes-Wójtowicz

2015-06-01

Full Text Available Impetigo is a fairly common disease of the childhood. This infection exists in two distinct forms: bullous and non-bullous impetigo. In non-bullous impetigo, a tiny vesicle or pustule shows initially and after 4–6 days develops into a honey-coloured crusted plaque. Bullous impetigo is always caused by Staphylococcus aureus, a bacterium that produces exfoliative toxins (ETA and ETB. Infection is limited to the superficial layers of the skin, and skin changes typically occur on the face and limbs. The disease is most prevalent in children aged 2 to 5, but it can occur at any age. The treatment involves topical or systemic antibiotics depending on the severity of symptoms and the patient’s condition. The differential diagnosis of impetigo includes: staphylococcal scalded-skin syndrome, Herpes simplex infection, eosinophilic pustular folliculitis, epidermolysis bullosa, pemphigus and pemphigoid. The differentiation between impetigo and staphylococcal scalded-skin syndrome can be challenging. Both diseases are caused by the same strain of Staphylococcus aureus that produces exfoliative toxins. However, in impetigo toxins are produced only within skin lesions whereas in staphylococcal scalded-skin syndrome, toxins are produced in organs colonised by Staphylococcus aureus and then released systemically. In the course of staphylococcal scalded-skin syndrome severe tenderness of the skin and a positive Nikolsky’s sign are observed. The paper presents two cases of impetigo in infants. In both cases, the correct diagnosis was not established immediately, which delayed appropriate treatment. In one of the cases, due to the imprecise initial diagnosis and the lack of the holistic approach to the patient, a correct diagnosis was established so late, that a systemic infection developed.

Gene and cell therapy for children--new medicines, new challenges?

Science.gov (United States)

Buckland, Karen F; Bobby Gaspar, H

2014-06-01

The range of possible gene and cell therapy applications is expanding at an extremely rapid rate and advanced therapy medicinal products (ATMPs) are currently the hottest topic in novel medicines, particularly for inherited diseases. Paediatric patients stand to gain enormously from these novel therapies as it now seems plausible to develop a gene or cell therapy for a vast number of inherited diseases. There are a wide variety of potential gene and cell therapies in various stages of development. Patients who received first gene therapy treatments for primary immune deficiencies (PIDs) are reaching 10 and 15 years post-treatment, with robust and sustained immune recovery. Cell therapy clinical trials are underway for a variety of tissues including corneal, retinal and muscle repair and islet cell transplantation. Various cell therapy approaches are also being trialled to enhance the safety of bone marrow transplants, which should improve survival rates in childhood cancers and PIDs. Progress in genetic engineering of lymphocyte populations to target and kill cancerous cells is also described. If successful these ATMPs may enhance or replace the existing chemo-ablative therapy for several paediatric cancers. Emerging applications of gene therapy now include skin and neurological disorders such as epidermolysis bullosa, epilepsy and leukodystrophy. Gene therapy trials for haemophilia, muscular dystrophy and a range of metabolic disorders are underway. There is a vast array of potential advanced therapy medicinal products (ATMPs), and these are likely to be more cost effective than existing medicines. However, the first clinical trials have not been without setbacks and some of the key adverse events are discussed. Furthermore, the arrival of this novel class of therapies brings many new challenges for the healthcare industry. We present a summary of the key non-clinical factors required for successful delivery of these potential treatments. Technological advances

Gene and cell therapy for children — New medicines, new challenges?☆

Science.gov (United States)

Buckland, Karen F.; Bobby Gaspar, H.

2014-01-01

The range of possible gene and cell therapy applications is expanding at an extremely rapid rate and advanced therapy medicinal products (ATMPs) are currently the hottest topic in novel medicines, particularly for inherited diseases. Paediatric patients stand to gain enormously from these novel therapies as it now seems plausible to develop a gene or cell therapy for a vast number of inherited diseases. There are a wide variety of potential gene and cell therapies in various stages of development. Patients who received first gene therapy treatments for primary immune deficiencies (PIDs) are reaching 10 and 15 years post-treatment, with robust and sustained immune recovery. Cell therapy clinical trials are underway for a variety of tissues including corneal, retinal and muscle repair and islet cell transplantation. Various cell therapy approaches are also being trialled to enhance the safety of bone marrow transplants, which should improve survival rates in childhood cancers and PIDs. Progress in genetic engineering of lymphocyte populations to target and kill cancerous cells is also described. If successful these ATMPs may enhance or replace the existing chemo-ablative therapy for several paediatric cancers. Emerging applications of gene therapy now include skin and neurological disorders such as epidermolysis bullosa, epilepsy and leukodystrophy. Gene therapy trials for haemophilia, muscular dystrophy and a range of metabolic disorders are underway. There is a vast array of potential advanced therapy medicinal products (ATMPs), and these are likely to be more cost effective than existing medicines. However, the first clinical trials have not been without setbacks and some of the key adverse events are discussed. Furthermore, the arrival of this novel class of therapies brings many new challenges for the healthcare industry. We present a summary of the key non-clinical factors required for successful delivery of these potential treatments. Technological advances

Finding patients using similarity measures in a rare diseases-oriented clinical data warehouse: Dr. Warehouse and the needle in the needle stack.

Science.gov (United States)

Garcelon, Nicolas; Neuraz, Antoine; Benoit, Vincent; Salomon, Rémi; Kracker, Sven; Suarez, Felipe; Bahi-Buisson, Nadia; Hadj-Rabia, Smail; Fischer, Alain; Munnich, Arnold; Burgun, Anita

2017-09-01

In the context of rare diseases, it may be helpful to detect patients with similar medical histories, diagnoses and outcomes from a large number of cases with automated methods. To reduce the time to find new cases, we developed a method to find similar patients given an index case leveraging data from the electronic health records. We used the clinical data warehouse of a children academic hospital in Paris, France (Necker-Enfants Malades), containing about 400,000 patients. Our model was based on a vector space model (VSM) to compute the similarity distance between an index patient and all the patients of the data warehouse. The dimensions of the VSM were built upon Unified Medical Language System concepts extracted from clinical narratives stored in the clinical data warehouse. The VSM was enhanced using three parameters: a pertinence score (TF-IDF of the concepts), the polarity of the concept (negated/not negated) and the minimum number of concepts in common. We evaluated this model by displaying the most similar patients for five different rare diseases: Lowe Syndrome (LOWE), Dystrophic Epidermolysis Bullosa (DEB), Activated PI3K delta Syndrome (APDS), Rett Syndrome (RETT) and Dowling Meara (EBS-DM), from the clinical data warehouse representing 18, 103, 21, 84 and 7 patients respectively. The percentages of index patients returning at least one true positive similar patient in the Top30 similar patients were 94% for LOWE, 97% for DEB, 86% for APDS, 71% for EBS-DM and 99% for RETT. The mean number of patients with the exact same genetic diseases among the 30 returned patients was 51%. This tool offers new perspectives in a translational context to identify patients for genetic research. Moreover, when new molecular bases are discovered, our strategy will help to identify additional eligible patients for genetic screening. Copyright © 2017. Published by Elsevier Inc.

19-DEJ-1, a hemidesmosome-anchoring filament complex-associated monoclonal antibody. Definition of a new skin basement membrane antigenic defect in junctional and dystrophic epidermolysis bullosa

DEFF Research Database (Denmark)

Fine, J D; Horiguchi, Y; Couchman, J R

1989-01-01

A murine monoclonal antibody (19-DEJ-1) was recently produced that recognizes a unique antigenic epitope of human skin basement membrane localized to the midlamina lucida exclusively in those areas bordered by overlying hemidesmosomes. To determine whether the antigen defined by 19-DEJ-1 is norma...

Balloon dilatation in children for oesophageal strictures other than those due to primary repair of oesophageal atresia, interposition or restrictive fundoplication

International Nuclear Information System (INIS)

Fasulakis, Stephen; Andronikou, Savvas

2003-01-01

Balloon dilatation of the oesophagus in children has been performed predominantly for treating strictures, which are the result of primary repair of oesophageal atresia, interposition surgery or restrictive Nissen's fundoplication. Reports of the use of this technique for alternative causes of stricture are few. To report our experience and success with balloon dilatation of strictures due to caustic ingestion, achalasia, oesophagitis, congenital stenosis, and epidermolysis bullosa (EB) and to make comparisons with our treatment of patients with primary repair of oesophageal atresia (OA), as well as with reports in the English language literature. Retrospective review of fluoroscopically guided balloon oesophageal dilatation procedures in 19 patients over a 5-year period, and comparison of those performed for OA repair complications with those due to other diseases. The average radiation dose, per procedure, was calculated by a medical physicist. Ten patients had strictures as a result of primary repair of OA. Three patients had stricture as a result of EB, two from achalasia, two from caustic injury, one due to an oesophageal web and one from reflux oesophagitis. Our results show that the technique can also be curative for the last group and that it may be used intermittently to alleviate symptoms in ongoing diseases. We have not experienced any complications and have also calculated that, even with prolonged use of multiple procedures, the radiation exposure is comparable to other radiological techniques. Patients with alternative causes for oesophageal stricture may be treated to resolution within 2 years using balloon dilatation. Ongoing diseases such as EB require ongoing dilatation, but balloon dilatation of strictures has been successful in alleviating swallowing difficulty. Patients with stricture from OA repair sometimes need ongoing dilatation. Radiation exposure for multiple procedures, over an extended period, is comparable to that from a single

Balloon dilatation in children for oesophageal strictures other than those due to primary repair of oesophageal atresia, interposition or restrictive fundoplication

Energy Technology Data Exchange (ETDEWEB)

Fasulakis, Stephen [Royal Children' s Hospital, Melbourne (Australia); Andronikou, Savvas [Royal Children' s Hospital, Melbourne (Australia); Department of Paediatric Radiology, Red Cross Children' s Hospital, Klipfontein Road, Rondebosch, 7700, Cape Town (South Africa)

2003-10-01

Balloon dilatation of the oesophagus in children has been performed predominantly for treating strictures, which are the result of primary repair of oesophageal atresia, interposition surgery or restrictive Nissen's fundoplication. Reports of the use of this technique for alternative causes of stricture are few. To report our experience and success with balloon dilatation of strictures due to caustic ingestion, achalasia, oesophagitis, congenital stenosis, and epidermolysis bullosa (EB) and to make comparisons with our treatment of patients with primary repair of oesophageal atresia (OA), as well as with reports in the English language literature. Retrospective review of fluoroscopically guided balloon oesophageal dilatation procedures in 19 patients over a 5-year period, and comparison of those performed for OA repair complications with those due to other diseases. The average radiation dose, per procedure, was calculated by a medical physicist. Ten patients had strictures as a result of primary repair of OA. Three patients had stricture as a result of EB, two from achalasia, two from caustic injury, one due to an oesophageal web and one from reflux oesophagitis. Our results show that the technique can also be curative for the last group and that it may be used intermittently to alleviate symptoms in ongoing diseases. We have not experienced any complications and have also calculated that, even with prolonged use of multiple procedures, the radiation exposure is comparable to other radiological techniques. Patients with alternative causes for oesophageal stricture may be treated to resolution within 2 years using balloon dilatation. Ongoing diseases such as EB require ongoing dilatation, but balloon dilatation of strictures has been successful in alleviating swallowing difficulty. Patients with stricture from OA repair sometimes need ongoing dilatation. Radiation exposure for multiple procedures, over an extended period, is comparable to that from a single

Two cases with unusual mycetoma localizations in upper respiratory system.

Science.gov (United States)

Bektaş, Devrim; Ural, Ahmet; Caylan, Rahmet; Bahadır, Osman; Kul, Neslihan; Caylan, Refik

2011-01-01

Fungus balls or mycetomas are extramucosal and noninvasive accumulations of degenerating fungal hyphae. In head and neck they may localized most frequently in the paranasal sinuses, especially in the maxillary sinus. These indolent infections are mostly caused by Aspergillus spp. In this article, we present two rare fungus ball cases: one occurring in an automastoidectomy cavity of a temporal bone and the other in a concha bullosa. Typically, both patients admitted with vague symptoms consistent with chronic infection resistant to conventional antibacterial medication.

Diseases and disorders of muscle.

Science.gov (United States)

Pearson, A M; Young, R B

1993-01-01

periodic paralysis, and malignant hyperexia). Diseases of the connective tissues discussed include those of nutritional origin (scurvy, lathyrism, starvation, and protein deficiency), the genetic diseases (dermatosparaxis, Ehlers-Danlos syndrome, osteogenesis imperfecta, Marfan syndrome, homocystinuria, alcaptonuria, epidermolysis bullosa, rheumatoid arthritis in humans, polyarthritis in swine, Aleutian disease of mink, and the several types of systemic lupus erythematosus) and the acquired diseases of connective tissues (abnormal calcification, systemic sclerosis, interstitial lung disease, hepatic fibrosis, and carcinomas of the connective tissues). Several of the diseases of connective tissues may prove to be useful models for determining the relationship of collagen to meat tenderness and its other physical properties. Several other promising models for studying the nutrition-related disorders and the quality-related characteristics of meat are also reviewed.

What Is Alopecia Areata?

Science.gov (United States)

... Bullosa Lichen Sclerosus Pachyonychia Congenita Pemphigus Psoriasis Raynaud’s Phenomenon Rosacea Vitiligo All Diseases and Conditions Kids Pages ... a role. This does not mean that your child will inherit the disease. In fact, this probably ...

Pachyonychia Congenita

Science.gov (United States)

... Bullosa Lichen Sclerosus Pachyonychia Congenita Pemphigus Psoriasis Raynaud’s Phenomenon Rosacea Vitiligo All Diseases and Conditions Kids Pages ... on the soles of the feet when a child begins to walk. In some cases, blisters and ...

Pemphigus

Science.gov (United States)

... Bullosa Lichen Sclerosus Pachyonychia Congenita Pemphigus Psoriasis Raynaud’s Phenomenon Rosacea Vitiligo All Diseases and Conditions Kids Pages ... not appear to be passed from parent to child. But some people’s genes put them more at ...

Abordaje de lesiones por pénfigo ampolloso en prolapso genital

Directory of Open Access Journals (Sweden)

Olga Barredo González

Full Text Available Las enfermedades bullosas o ampollares comprenden un grupo heterogéneo de cuadros cuya lesión elemental común es la ampolla. Los pénfigos y los penfigoides pertenecen al grupo de las enfermedades bullosas de origen autoinmunitario. El prolapso genital es el descenso o desplazamiento de los órganos pélvicos, a través de la vagina y en dirección a la vulva, que se produce como consecuencia del fallo de sus elementos de suspensión y sustentación así como la inervación y musculatura del sistema urinario e intestinal. En este caso presentamos nuestra experiencia en el manejo y tratamiento tópico de lesiones por pénfigo ampolloso en prolapso visceropélvico total que dificultaban la cirugía.

The impact of the International Atomic Energy Agency (IAEA) program on radiation and tissue banking in Mexico.

Science.gov (United States)

Martínez-Pardo, María Esther; Morales Pedraza, Jorge; Sánchez Ramírez, Omar

2009-05-01

Tissue banking started in Mexico in 1948-1949, when two bone banks were established, one at the Infantile Hospital of Mexico and other at the Central Military Hospital. Mexico has benefited for the implementation of the IAEA program since through it has been able to settle down and to consolidate the Tissue Bank at the Instituto Nacional de Investigaciones Nucleares ININ (National Institute for Nuclear Research). This is the only bank in Latin America that has a Quality Management System in force, certified under ISO 9001:2000 since August 1, 2003. The first tissue processed was amnion. The main products of the BTR are amnion and pig skin. Both are biological tissues which their main use is as a wound dressing in patients with burns, scars, diabetic ulcers, epidermolysis bullosa, damaged ocular surface, etc. The General Health Law, published in 1984 and reformed in June 19, 2007, describes the procedure for the disposal of organs, tissues and human cadavers in its fourteenth title and in the Regulation for Sanitary Control. During the period 2001-2005, the ININ Tissue Bank produced 292 sterilised tissues (amnion, 86,668 cm(2), and frozen pig skin, 164,220 cm(2), at an estimated cost of 1,012,668 Mexican pesos. Until 2006, one hundred eighty five (185) patients have been treated with the use of sterilised tissues produced by the ININ Tissue Bank. The radiation source used for sterilisation of tissues is an industrial Cobalt-60 irradiator model JS-6500 AECL, which belongs to ININ. This equipment is located in other building, close to the BTR, in the Centro Nuclear de México "Dr. Nabor Carrillo Flores" (Nuclear Center of Mexico). Until 2006, six hospitals use in a routine way the sterilised tissues produced by the ININ Tissue Bank, for the treatment of burns originated by diverse agents like flame, electricity, liquids in boil, chemical reagents, as well as for the reconstruction of the ocular surface. Two of these hospitals treat patients of very low economic

A review of scoring systems for ocular involvement in chronic cutaneous bullous diseases.

Science.gov (United States)

Lee, Brendon W H; Tan, Jeremy C K; Radjenovic, Melissa; Coroneo, Minas T; Murrell, Dedee F

2018-05-22

Epidermolysis bullosa (EB) and autoimmune blistering diseases (AIBD) describe a group of rare chronic dermatoses characterized by cutaneous fragility and blistering. Although uncommon, significant ocular surface disease (OSD) may occur in both and require ophthalmological assessment. Disease scoring systems have a critical role in providing objective and accurate assessment of disease severity. The objectives of this report were, firstly, to document the prevalence and severity of ocular involvement in EB/AIBD. Secondly, to review and evaluate existing ocular and systemic scoring systems for EB/AIBD. Finally, to identify areas where further development of ocular specific tools in EB/AIBD could be pursued. A literature search was performed in October 2017 utilising Medline, Embase, and Scopus databases. The results were restricted by date of publication, between 01.01.1950 and 31.10.2017. The reference lists of these articles were then reviewed for additional relevant publications. Articles of all languages were included if an English translation was available. Articles were excluded if they were duplicates, had no reference to ocular involvement in EB/AIBD or described ocular involvement in other diseases. Descriptions of ocular involvement in EB/AIBD were identified in 88 peer-reviewed journal articles. Findings reported include but are not limited to: cicatrising conjunctivitis, meibomian gland dysfunction, dry eye disease, trichiasis, symblepharon, fornix fibrosis, keratopathy, ectropion/entropion, ankyloblepharon, corneal ulceration, visual impairment and blindness. Although scoring systems exist for assessment of OSD in mucous membrane pemphigoid, no such tools exist for the other AIBD subtypes or for EB. Several systemic scoring systems exist in the dermatological literature that are efficacious in grading overall EB/AIBD severity, but have limited inclusion of ocular features. To the best of our knowledge, there is no recognised or validated scoring systems

The Role of the Nasal and Paranasal Sinus Pathologies on the ...

African Journals Online (AJOL)

2017-10-26

Oct 26, 2017 ... Development of Chronic Otitis Media and its Subtypes: A Computed. Tomography Study ... factors, anatomical and functional characteristics of. Eustachian .... perpendicular to the mucosal surface at the right and left sides ... image shows a 44-year-old woman who has left concha bullosa, the measurement ...

Next generation human skin constructs as advanced tools for drug development.

Science.gov (United States)

Abaci, H E; Guo, Zongyou; Doucet, Yanne; Jacków, Joanna; Christiano, Angela

2017-11-01

Many diseases, as well as side effects of drugs, manifest themselves through skin symptoms. Skin is a complex tissue that hosts various specialized cell types and performs many roles including physical barrier, immune and sensory functions. Therefore, modeling skin in vitro presents technical challenges for tissue engineering. Since the first attempts at engineering human epidermis in 1970s, there has been a growing interest in generating full-thickness skin constructs mimicking physiological functions by incorporating various skin components, such as vasculature and melanocytes for pigmentation. Development of biomimetic in vitro human skin models with these physiological functions provides a new tool for drug discovery, disease modeling, regenerative medicine and basic research for skin biology. This goal, however, has long been delayed by the limited availability of different cell types, the challenges in establishing co-culture conditions, and the ability to recapitulate the 3D anatomy of the skin. Recent breakthroughs in induced pluripotent stem cell (iPSC) technology and microfabrication techniques such as 3D-printing have allowed for building more reliable and complex in vitro skin models for pharmaceutical screening. In this review, we focus on the current developments and prevailing challenges in generating skin constructs with vasculature, skin appendages such as hair follicles, pigmentation, immune response, innervation, and hypodermis. Furthermore, we discuss the promising advances that iPSC technology offers in order to generate in vitro models of genetic skin diseases, such as epidermolysis bullosa and psoriasis. We also discuss how future integration of the next generation human skin constructs onto microfluidic platforms along with other tissues could revolutionize the early stages of drug development by creating reliable evaluation of patient-specific effects of pharmaceutical agents. Impact statement Skin is a complex tissue that hosts various

Use if a soecuak sokubt ub reverse syrak artery flap to reduce venous congestion and flap necrosis

International Nuclear Information System (INIS)

Masood, T.; Ahmed, R.; Obaidullah, M.

2016-01-01

Background: Distally based sural fascio-cutaneous flap is a commonly performed plastic surgery procedure for the coverage of distal third of leg, ankle and foot defects. However congestion is the main complication of this flap which results into partial or complete loss of the flap. We devised a special splint to reduce this complication and retrospectively reviewed its effect on this complication between two groups. Methods: This retrospective study was carried out at Northwest General hospital between 1995 and 2012. Group-A included 30 patients who were managed without the splint between 1995 and 2005 and group B comprised of 35 patients were treated with the splint between 2006 and 2012. Complications like venous congestion, epidermolysis, and partial and complete flap failure were documented. Data were analyzed by SPSS.16.5 software. Chi- square test was used for data analysis. P value less than 0.05 was considered as the level of significance. Results: Total 65 patients were operated. Age of the patients ranged from 7 to 60 years. Road traffic accident and spoke wheel injury was the main cause of soft tissue loss in our patients. In group A 12 patients suffered from venous congestion. Out of 12, three patients had epidermolysis while partial flap necrosis occurred in 9 patients. Only 3 patients had venous congestion in group B. Two patients suffered from epidermolysis and one had partial flap necrosis. None of patient suffered from complete flap loss in both groups. Conclusion: Reverse sural artery flap continues to be a versatile flap for distal lower extremity reconstruction. By using a special splint to reduce pressure on the pedicle site as a modification, flap complication rate can be decreased significantly. (author)

All concha bullosa: an undefined abnormality of the lateral nasal wall.

Science.gov (United States)

Toplu, Yuksel; Bayindir, Tuba; Karatas, Erkan; Akarcay, Mustafa

2013-01-01

Pneumatization of the turbinates, are the anatomic variations of lateral nasal wall. Turbinate pneumatization, refers to the existence of air cell inside the turbinates. Pneumatization of the middle turbinate is common, whereas rare in the superior and especially inferior turbinate. In this report we presented a case who has bilaterally pneumatization of all conchas.

Reprodución de tres especies de Clidemia (Melastomataceae en Brasil

Directory of Open Access Journals (Sweden)

Gladys Flávia Melo

1999-09-01

Full Text Available Se estudió el sistema reproductivo de Clidemia capitellata (Bonpl. D. Don, C. bullosa DC. y C. hirta (L. D. Don. Las tres especies se distribuyen en las márgenes de la selva de Dois Irmos, Recife, Pernambuco, Brasil donde forman pequeñas poblaciones y se pueden calificar como especies simpátricas. El periodo de floración se extiende todo el año. Hay visitas esporádicas de abejas Halictidae (Augochloropsis sp. que recogen polen por vibración ("buzz pollination". Las tres especies son agamospérmicas y muestran un elevado índice de esterilidad masculina, medida por la escasa viabilidad polínica.We compared the reproductive systems in populations of Clidemia capitellata (Bonpl. D. Don, C. bullosa DC. and C. hirta (L. D. Don. (Melastomataceae. The three species occur in small populations in forest margins at Mata de Dois Irmos, Recife, Pernambuco, Brasil and are sympatric. They flower throughout the year, and are occasionaly visited by Halictidae bees, Augochloropsis sp.; the bees collect pollen by vibration ("buzz pollination". The three species are agamospermous and exhibit a high level of male sterility, as measured by their low pollen viability.

Virtual Presenters: Towards Interactive Virtual Presentations

NARCIS (Netherlands)

Nijholt, Antinus; Cappellini, V.; Hemsley, J.

2005-01-01

We discuss having virtual presenters in virtual environments that present information to visitors of these environments. Some current research is surveyed and we will look in particular to our research in the context of a virtual meeting room where a virtual presenter uses speech, gestures, pointing

Protocolo de atención odontológica en niños con Epidermólisis Ampollar

OpenAIRE

Gabriela Scagnet

2013-01-01

La atención odontológica de los niños con Epidermólisis ampollar (EA) o bullosa (EB) son un inmenso desafío para la práctica odontológica ya que se trata de un grupo de trastornos hereditarios que manifiestan aparición de ampollas en piel y mucosas al roce o suave trauma. Presentan múltiples manifestaciones orofaciales que requieren de un protocolo de atención interdisciplinaria específico que comprenda un alto componente preventivo. Se describirán los principales ítems del mismo y las posibi...

Making your presentation fun: creative presentation techniques

Energy Technology Data Exchange (ETDEWEB)

KEENEN,MARTHA JANE

2000-05-18

What possesses someone to volunteer and go through hoops and red tape to make a presentation at a conference? For that matter, why does anyone ever present anything to anyone? Actually, presentations are a fact of life and there are many reasons for doing a presentation and doing it well. New and existing staff need training and orientation to the way things are done here. Handing all of them a manual and hoping they read it is pretty much a waste of paper. On the other hand, an effective, entertaining and upbeat presentation on the relevant topics is more likely to stick with those people. They will even have a name and face to remember and seek out when they have an issue on or with that topic. This can be a very effective beginning for networking with new peers. The presenter is seen as knowledgeable, as a source of information on company topics and possibly evaluated as a potential mentor or future manager. Project staff and/or peers benefit from clear, concise, presentations of topical knowledge. This is one way that a group working on various aspects of the same project or program can stay in touch and in step with each other. Most importantly, presentations may be the best or only door into the minds (and budgets) of management and customers. These presentations are a wonderful opportunity to address legal and compliance issues, budget, staffing, and services. Here is a chance, maybe the only one, to demonstrate and explain the wonderfulness of a program and the benefit they get by using the services offered most effectively. An interactive presentation on legal and compliance issues can be an effective tool in helping customers and/or management make good risk management decisions.

Protocolo de atención odontológica en niños con Epidermólisis Ampollar

Directory of Open Access Journals (Sweden)

Gabriela Scagnet

2013-01-01

Full Text Available La atención odontológica de los niños con Epidermólisis ampollar (EA o bullosa (EB son un inmenso desafío para la práctica odontológica ya que se trata de un grupo de trastornos hereditarios que manifiestan aparición de ampollas en piel y mucosas al roce o suave trauma. Presentan múltiples manifestaciones orofaciales que requieren de un protocolo de atención interdisciplinaria específico que comprenda un alto componente preventivo. Se describirán los principales ítems del mismo y las posibilidades de rehabilitación bucal integral personalizada.

AUTOIMMUNE EPIDERMAL BLISTERING DISEASES

Directory of Open Access Journals (Sweden)

Ana Maria Abreu Velez

2013-11-01

Full Text Available Autoimmune bullous skin diseases (ABDs are uncommon, potentially fatal diseases of skin and mucous membranes which are associated with deposits of autoantibodies and complement against distinct molecules of the epidermis and dermal/epidermal basement membrane zone (BMZ. These autoantibodies lead to a loss in skin molecular integrity, which manifests clinically as formation of blisters or erosions. In pemphigus vulgaris, loss of adhesion occurs within the epidermis. The pioneering work of Ernst H. Beutner, Ph.D. and Robert E. Jordon, M.D. confirmed the autoimmune nature of these diseases. Walter F. Lever, M.D. contributed significantly to our understanding of the histopathologic features of these diseases. Walter Lever, M.D. and Ken Hashimoto, M.D. contributed electron microscopic studies of these diseases, especially in pemphigus vulgaris and bullous pemphigoid. In bullous pemphigoid (BP, linear IgA bullous dermatosis, epidermolysis bullosa acquisita (EBA and dermatitis herpetiformis (DH, loss of adhesion takes place within or underneath the BMZ. Classic EBA demonstrates extensive skin fragility; DH is commonly associated with gluten-sensitive enteropathy, and manifests clinically with pruritic papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The clinical spectrum of bullous pemphigoid includes tense blisters, urticarial plaques, and prurigo-like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy, and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a „cluster of jewels”-like pattern in childhood (chronic bullous disease of childhood and is more clinically heterogeneous in adulthood. Many of the autoantigens in these disorders are known and have been well characterized. ABDs may be influenced by both genetic and exogenous factors. The diagnoses of

Presentations

International Nuclear Information System (INIS)

2007-01-01

The presented materials consist of presentations of international workshop which held in Warsaw from 4 to 5 October 2007. Main subject of the meeting was progress in manufacturing as well as research program development for neutron detector which is planned to be placed at GANIL laboratory and will be used in nuclear spectroscopy research

Short and long term follow up results of (the versatile) reverse sural artery flap

International Nuclear Information System (INIS)

Ali, G.; Hameed, S.; Hassan, R.

2015-01-01

This study was designed to see the long and short term outcome of the reverse sural artery flap. Study Design: Case series. Patients and Methods: From January 2009 to December 2011, data was collected and analyzed for this study.Eighty nine patients with wounds on the ankle, heel, sole, distal leg, and foot were included in the study. They were followed up at 01 week, 02 weeks, and then 4 weekly for 06 months and at one year time from operation. They were examined for necrosis, congestion, surgical site infection, dehiscence of suture line, epidermolysis, donor site infection and functional outcome. Results: Most of the flaps healed nicely but two (2.25%) failed completely. Six flaps were delayed. However early follow up (within 04 weeks) revealed that there was partial loss of the distal 1-1.5 cm of flap in 04 patients (4.50%). Two patients (2.25%) developed superficial surgical site infection. Six patients (6.74%) developed venous congestion of the flaps which recovered within two weeks. Other minor complications included dehiscence of suture line in 3 patients (3.37%), and superficial Epidermolysis in four (4.50%) (Table-2). Twenty two patients (24.72%) returned to their work in 12-16 weeks, 31 (34.83%) in 16-20 weeks and 36 (40.45%) in 20-24 weeks. Long term follow-up to 06 months revealed hypertrophic scars at the donor site in three patients (4.91%) and recurrence of ulcer in 2 patients (3.27%). Conclusion: The sural fasciocutaneous flap provides reliable supple and durable most single-stage coverage of wounds of the distal third of the leg, heel, and foot with the results comparable to free-tissue transfer. (author)

Doenças da pele em caprinos e ovinos no semi-árido brasileiro Diseases of the skin in sheep and goats from the Brazilian semiarid

Directory of Open Access Journals (Sweden)

Juliana T.S.A. Macêdo

2008-12-01

from 31 goats and 45 sheep were studied histologically. Skin diseases were diagnosed in 35 goats (5.33% of all cases in this species and 45 sheep (13.88% of the cases in this species. The most frequent skin disease was myiasis (10 cases in goats and 7 in sheep, followed by contagious echtyma (8 cases in goats and 2 in sheep, squamous cell carcinoma (4 cases in goats and 5 in sheep, dermatophylosis (8 cases in sheep, and allergic dermatitis (1 case in goats and 1 in sheep. Two cases of pythiosis in sheep, two cases of epidermolysis bullosa in goats, one case of poisoning by Brachiaria brizantha (photosensitization and another by Leucaena leucocephala (alopecia in sheep, and one case of papillomatosis, one of pemphigus foliaceous, one of protothecosis and one of rhabdomyosarcoma in goats were also diagnosed. In 4 cases in sheep and 19 in goats the etiologic diagnosis was not obtained. With the information of the occurrence and epidemiology of the skin diseases is possible to determine efficient control measures.

Delivery presentations

Science.gov (United States)

Pregnancy - delivery presentation; Labor - delivery presentation; Occiput posterior; Occiput anterior; Brow presentation ... The mother can walk, rock, and try different delivery positions during labor to help encourage the baby ...

Workshop presentations

International Nuclear Information System (INIS)

Sanden, Per-Olof; Edland, Anne; Reiersen, Craig; Mullins, Peter; Ingemarsson, Karl-Fredrik; Bouchard, Andre; Watts, Germaine; Johnstone, John; Hollnagel, Erik; Ramberg, Patric; Reiman, Teemu

2009-01-01

An important part of the workshop was a series of invited presentations. The presentations were intended to both provide the participants with an understanding of various organisational approaches and activities as well as to stimulate the exchange of ideas during the small group discussion sessions. The presentation subjects ranged from current organisational regulations and licensee activities to new organisational research and the benefits of viewing organisations from a different perspective. There were more than a dozen invited presentations. The initial set of presentations gave the participants an overview of the background, structure, and aims of the workshop. This included a short presentation on the results from the regulatory responses to the pre-workshop survey. Representatives from four countries (Sweden, Canada, Finland, and the United Kingdom) expanded upon their survey responses with detailed presentations on both regulatory and licensee safety-related organisational activities in their countries. There were also presentations on new research concerning how to evaluate safety critical organisations and on a resilience engineering perspective to safety critical organisations. Below is the list of the presentations, the slides of which being available in Appendix 2: 1 - Workshop Welcome (Per-Olof Sanden); 2 - CSNI Working Group on Human and Organisational Factors (Craig Reiersen); 3 - Regulatory expectations on justification of suitability of licensee organisational structures, resources and competencies (Anne Edland); 4 - Justifying the suitability of licensee organisational structures, resources and competencies (Karl-Fredrik Ingemarsson); 5 - Nuclear Organisational Suitability in Canada (Andre Bouchard); 6 - Designing and Resourcing for Safety and Effectiveness (Germaine Watts); 7 - Organisational Suitability - What do you need and how do you know that you've got it? (Craig Reiersen); 8 - Suitability of Organisations - UK Regulator's View (Peter

Put Power into Your Presentations: Using Presentation Software Effectively

Science.gov (United States)

Safransky, Robert J.; Burmeister, Marsha L.

2009-01-01

Microsoft PowerPoint, Apple Keynote, and OpenOffice Impress are relatively common tools in the classroom and in the boardroom these days. What makes presentation software so popular? As the Chinese proverb declares, a picture is worth a thousand words. People like visual presentations. Presentation software can make even a dull subject come to…

RESUME La pneumatisation, ou concha bullosa du cornet inférieur ...

African Journals Online (AJOL)

1- Aydin O. , Ustundag E. , Ciftc E. , Keskin I.G. Pneumatization of the infe- rior turbinate Auris Nasus Larynx 2001 ; 28 :361,363. 2- Ariyurek. OM, Balkanci. F, Aydingoz. U, Onerci. M Pneumatized superior turbinate: A commun anatomic variation ? Surg Radiol Anat 1996; 18: 137-. 139. 3- Bolger. WE, Butzin. CA, Parsons. DS.

CATCHY PRESENTATIONS

DEFF Research Database (Denmark)

Eriksen, Kaare; Tollestrup, Christian; Ovesen, Nis

2011-01-01

An important competence for designers is the ability to communicate and present ideas and proposals for customers, partners, investors and colleagues. The Pecha Kucha principle, developed by Astrid Klein and Mark Dytham, has become a widely used and easy format for the presentation of new concepts...

Voting Present

Directory of Open Access Journals (Sweden)

James Lo

2013-12-01

Full Text Available During his time as a state senator in Illinois, Barack Obama voted “Present” 129 times, a deliberate act of nonvoting that subsequently became an important campaign issue during the 2008 presidential elections. In this article, I examine the use of Present votes in the Illinois state senate. I find evidence that Present votes can largely be characterized as protest votes used as a legislative tool by the minority party. Incorporating information from Present votes into a Bayesian polytomous item-response model, I find that this information increases the efficiency of ideal point estimates by approximately 35%. There is little evidence of significant moderation by Obama when Present votes are accounted for, though my results suggest that Obama’s voting record may have moderated significantly before his subsequent election to the U.S. Senate. My results also suggest that because legislative nonvoting may occur for a variety of reasons, naive inclusion of nonvoting behavior into vote choice models may lead to biased results.

Lecture Presentations

International Nuclear Information System (INIS)

2007-01-01

The Heavy-Ion Collisions in the LHC workshop held in Cracow from 18 to 18 May 2007. The main subject of the workshop was to present the newest results of research provided at CERN LHC collider. Additionally some theoretical models and methods used for presented data analysis were discussed

Generalized and Naevoid Epidermolytic Ichthyosis in Denmark

DEFF Research Database (Denmark)

Bygum, Anette; Virtanen, Marie; Brandrup, Flemming

2012-01-01

A Danish-Swedish collaboration was established to identify and classify a Danish cohort of patients with epidermolytic ichthyosis, also known as epidermolytic hyperkeratosis. Patients were recruited from 5 dermatology departments in Denmark, and data were obtained using a structured questionnaire...... and a systematic examination together with photographs, histopathological descriptions and blood samples for mutational analysis. Sixteen patients from 12 families with generalized or naevoid epidermolytic ichthyosis and ichthyosis bullosa of Siemens were identified. Five families had mutations in K1 and 6...... families had mutations in K10. Nine patients had been treated with systemic retinoids (etretinate, acitretin, isotretinoin or alitretinoin), but only 3 patients had acceptable treatment responses and chose to continue therapy. In conclusion epidermolytic ichthyosis is a rare disease with a prevalence...

Isolated Torticollis May Present as an Atypical Presentation of Meningitis

Directory of Open Access Journals (Sweden)

Roger Chirurgi

2012-01-01

Full Text Available Background. Bacterial meningitis is a life-threatening medical emergency that requires urgent diagnosis and treatment. Diagnosis is infrequently missed if the patient presents with the classic symptoms of fever, headache, rash, nuchal rigidity, or Kernig or Brudzinski sign. However, it may be less obvious in neonates, elderly, or immunocompromised patients. Meningitis which presents as isolated torticollis, without any other signs or symptoms, is exceedingly rare. Objective. To identify an abnormal presentation of meningitis in an adult immunocompromised patient. Case Report. We present a case of an adult diabetic male who presented multiple times to the ED with complaint of isolated torticollis, who ultimately was diagnosed with bacterial meningitis. Conclusion. We propose that in the absence of sufficient explanation for acute painful torticollis in an immunocompromised adult patient, further evaluation, possibly including a lumbar puncture may be warranted.

Information Presentation

Science.gov (United States)

Holden, K.L.; Boyer, J.L.; Sandor, A.; Thompson, S.G.; McCann, R.S.; Begault, D.R.; Adelstein, B.D.; Beutter, B.R.; Stone, L.S.

2009-01-01

The goal of the Information Presentation Directed Research Project (DRP) is to address design questions related to the presentation of information to the crew. The major areas of work, or subtasks, within this DRP are: 1) Displays, 2) Controls, 3) Electronic Procedures and Fault Management, and 4) Human Performance Modeling. This DRP is a collaborative effort between researchers at Johnson Space Center and Ames Research Center.

Phaeochromocytoma presenting with polyuria: an uncommon presentation of a rare tumour

OpenAIRE

Atapattu, N; Imalke, K A C P; Madarasinghe, M; Lamahewage, A; de Silva, K S H

2014-01-01

Summary Children rarely present with phaeochromocytoma. Their presentation differs from that of adults. The classic triad of sweating, headache and palpitation may not always present in children with phaeochromocytoma. In this study, we present a 6-year-old girl who came to us with polyuria and polydipsia for evaluation of suspected diabetes insipidus. She gave a clear history of increased sweating in the recent past. On clinical examination, she was noted to have high blood pressure. Subsequ...

Rotating Poster Presentations

Science.gov (United States)

Lagares, Manuel; Reisenleutner, Sandra

2017-01-01

Oral presentations are a common practice in foreign language classes, often used to assess students' speaking skills. Usually, the presentations are delivered by students in front of the class, often with PowerPoint slides or Prezi as support. However, frequently the audience does not engage with the presentation and thus, the benefits of this…

Presentation Technique

International Nuclear Information System (INIS)

Froejmark, M.

1992-10-01

The report presents a wide, easily understandable description of presentation technique and man-machine communication. General fundamentals for the man-machine interface are illustrated, and the factors that affect the interface are described. A model is presented for describing the operators work situation, based on three different levels in the operators behaviour. The operator reacts routinely in the face of simple, known problems, and reacts in accordance with predetermined plans in the face of more complex, recognizable problems. Deep fundamental knowledge is necessary for truly complex questions. Today's technical status and future development have been studied. In the future, the operator interface will be based on standard software. Functions such as zooming, integration of video pictures, and sound reproduction will become common. Video walls may be expected to come into use in situations in which several persons simultaneously need access to the same information. A summary of the fundamental rules for the design of good picture ergonomics and design requirements for control rooms are included in the report. In conclusion, the report describes a presentation technique within the Distribution Automation and Demand Side Management area and analyses the know-how requirements within Vattenfall. If different systems are integrated, such as geographical information systems and operation monitoring systems, strict demands are made on the expertise of the users for achieving a user-friendly technique which is matched to the needs of the human being. (3 figs.)

Stellar Presentations (Abstract)

Science.gov (United States)

Young, D.

2015-12-01

(Abstract only) The AAVSO is in the process of expanding its education, outreach and speakers bureau program. powerpoint presentations prepared for specific target audiences such as AAVSO members, educators, students, the general public, and Science Olympiad teams, coaches, event supervisors, and state directors will be available online for members to use. The presentations range from specific and general content relating to stellar evolution and variable stars to specific activities for a workshop environment. A presentation—even with a general topic—that works for high school students will not work for educators, Science Olympiad teams, or the general public. Each audience is unique and requires a different approach. The current environment necessitates presentations that are captivating for a younger generation that is embedded in a highly visual and sound-bite world of social media, twitter and U-Tube, and mobile devices. For educators, presentations and workshops for themselves and their students must support the Next Generation Science Standards (NGSS), the Common Core Content Standards, and the Science Technology, Engineering and Mathematics (STEM) initiative. Current best practices for developing relevant and engaging powerpoint presentations to deliver information to a variety of targeted audiences will be presented along with several examples.

Behavioural present value

OpenAIRE

Krzysztof Piasecki

2013-01-01

Impact of chosen behavioural factors on imprecision of present value is discussed here. The formal model of behavioural present value is offered as a result of this discussion. Behavioural present value is described here by fuzzy set. These considerations were illustrated by means of extensive numerical case study. Finally there are shown that in proposed model the return rate is given, as a fuzzy probabilistic set.

The Effects of Poster Presentations and Class Presentations on Low-Proficiency Learners

Science.gov (United States)

Prichard, Caleb; Ferreira, Dan

2014-01-01

Presentation assignments for second language speakers can take several forms, such as a traditional class presentation or a poster presentation. Poster presentations, which are given repeatedly to small groups, seem to have several advantages, including increased speaking opportunities, more interaction between the speaker and the audience, and…

Does a presentation's medium affect its message? PowerPoint, Prezi, and oral presentations.

Science.gov (United States)

Moulton, Samuel T; Türkay, Selen; Kosslyn, Stephen M

2017-01-01

Despite the prevalence of PowerPoint in professional and educational presentations, surprisingly little is known about how effective such presentations are. All else being equal, are PowerPoint presentations better than purely oral presentations or those that use alternative software tools? To address this question we recreated a real-world business scenario in which individuals presented to a corporate board. Participants (playing the role of the presenter) were randomly assigned to create PowerPoint, Prezi, or oral presentations, and then actually delivered the presentation live to other participants (playing the role of corporate executives). Across two experiments and on a variety of dimensions, participants evaluated PowerPoint presentations comparably to oral presentations, but evaluated Prezi presentations more favorably than both PowerPoint and oral presentations. There was some evidence that participants who viewed different types of presentations came to different conclusions about the business scenario, but no evidence that they remembered or comprehended the scenario differently. We conclude that the observed effects of presentation format are not merely the result of novelty, bias, experimenter-, or software-specific characteristics, but instead reveal a communication preference for using the panning-and-zooming animations that characterize Prezi presentations.

Dressed to Present: Ratings of Classroom Presentations Vary with Attire

Science.gov (United States)

Gurung, Regan A. R.; Kempen, Laura; Klemm, Kayla; Senn, Rebecca; Wysocki, Rosie

2014-01-01

This study investigates the effects of formality of dress on ratings of classroom presentations. Participants (N = 65, 66% women) from a Midwestern university in the United States rated three female students giving a presentation designed for a health psychology class in one of four outfits: casual, party, business casual, or business formal.…

Develop your presentation skills

CERN Document Server

Theobald, Theo

2016-01-01

Going beyond handling nerves and presenting PowerPoint slides, the third edition of "Develop Your Presentation Skills "offers practical advice on developing a captivating presentation, constructing compelling content, and boosting self-confidence. The book includes three new chapters on delivering a "stripped down"presentation, using new media to engage with the audience, and handling being asked to present on short notice."

Microscopia confocal en operados de queratoplastia perforante Confocal microscopy in patients operated from penetrating keratoplasty

Directory of Open Access Journals (Sweden)

Zulema Gómez Castillo

2009-06-01

Full Text Available La microscopia confocal es un examen exploratorio, práctico y poco invasivo que permite conocer las características microscópicas del tejido corneal después del trasplante, por lo que constituye una herramienta muy útil en el manejo de los pacientes operados de queratoplastia. El presente trabajo tiene como finalidad describir las características del tejido corneal en pacientes operados de este tipo de trasplante, mediante la microscopia confocal in vivo. MÉTODOS: Se realizó un estudio descriptivo, de corte transversal, en 40 ojos de 40 pacientes operados de queratoplastia perforante, en el Servicio de Córnea del Instituto Cubano de Oftalmología "Ramón Pando Ferrer", de marzo de 2006 a marzo de 2007. Se confeccionó una historia clínica oftalmológica y se les realizó a todos el examen de microscopia confocal en el injerto corneal con el microscopio confocal CONFOSCAN 4. RESULTADOS: La queratopatía bullosa pseudofáquica fue la afección más frecuente previa a la cirugía y estuvo presente en el 77,5 % de los pacientes. En el 72,5 % de los intervenidos se encontró una disminución del grosor corneal. El epitelio presentó alteraciones en el 62,5 % de los pacientes. Todos presentaron afectación de la forma y el tamaño celular endotelial. En el 82,5 % de los pacientes se observó ausencia de plexos nerviosos. CONCLUSIONES: La microscopia confocal como nueva ciencia en el campo de la oftalmología, favorece el seguimiento evolutivo de las queratoplastias perforantes y con esto no solo a prevenir la aparición de posibles complicaciones, sino además de garantizar el éxito de la cirugía y la función refractiva de la córnea.Confocal microscopy is a practical, exploratory and less invassive examination that allows finding out the microscopic characteristics of the corneal tissue after transplantation, so it is a very useful tool for the management of patients operated from keratoplasty. The present paper was aimed at describing

Scientific Presenting: Using Evidence-Based Classroom Practices to Deliver Effective Conference Presentations

Science.gov (United States)

Corwin, Lisa A.; Prunuske, Amy; Seidel, Shannon B.

2018-01-01

Scientists and educators travel great distances, spend significant time, and dedicate substantial financial resources to present at conferences. This highlights the value placed on conference interactions. Despite the importance of conferences, very little has been studied about what is learned from the presentations and how presenters can…

Presentations for librarians a complete guide to creating effective, learner-centred presentations

CERN Document Server

Hilyer, Lee Andrew

2008-01-01

Recent research on learning from multimedia presentations has indicated that the current way many people prepare their slide presentations may actually hinder learning. Considering the ubiquity of the PowerPoint presentation in business and in education, presenters should be concerned whether or not their audience members are effectively receiving the information they wish to impart. This issue is of special import for librarians who teach, as they often must convey complex information in a very limited amount of time. Combining the best evidence on multimedia learning with real-world practica

Delivery presentations

Science.gov (United States)

... has entered the pelvis. If the presenting part lies above the ischial spines, the station is reported ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

Innovative presentations for dummies

CERN Document Server

Anthony, Ray

2014-01-01

Be the speaker they follow with breakthrough innovative presentations Innovative Presentations For Dummies is a practical guide to engaging your audience with superior, creative, and ultra-compelling presentations. Using clear language and a concise style, this book goes way beyond PowerPoint to enable you to reimagine, reinvent, and remake your presentations. Learn how to stimulate, capture, and hold your audience in the palm of your hand with sound, sight, and touch, and get up to speed on the latest presentation design methods that make you a speaker who gets audiences committed and acting

Subpart AA Training Presentations

Science.gov (United States)

EPA has produced the following training presentation(s) for reporters subject to this subpart. Generally, these presentations explain how to use the reporting system e-GGRT to submit annual GHG reports to EPA.

CERN presentations

CERN Multimedia

CERN. Geneva

2011-01-01

Presentation by CERN (10 minutes each) Rolf Landua - Education and Outreach Salvatore Mele - Open Access Jean-Yves Le Meur - Digital Library in Africa Francois Fluckiger - Open Source/Standards (tbc) Tim Smith - Open Data for Science Tullio Basiglia - tbc

Conjugando el presente

Directory of Open Access Journals (Sweden)

Héctor Rojas Herazo

1968-06-01

Full Text Available La añoranza suele ser, casi siempre, la línea critica de menor resistencia. El lema de que "todo tiempo pasado fue mejor" tiene un poco la culpa. En cualquier orden del conocimiento el pasado sigue el verdadero presente. Ese presente en que nos gusta respirar y vivir. Nada de compromiso circundante. Nada de inmediatez.

Subpart DD Training Presentations

Science.gov (United States)

EPA has produced the following training presentation(s) for reporters subject to this subpart. Generally, these presentations explain the rule or show how to use the reporting system e-GGRT to submit annual GHG reports to EPA.

Subpart W Training Presentations

Science.gov (United States)

EPA has produced the following training presentation(s) for reporters subject to this subpart. Generally, these presentations explain the rule or show how to use the reporting system e-GGRT to submit annual GHG reports to EPA.

Alarm filtering and presentation

International Nuclear Information System (INIS)

Bray, M.A.

1989-01-01

This paper discusses alarm filtering and presentation in the control room of nuclear and other process control plants. Alarm generation and presentation is widely recognized as a general process control problem. Alarm systems often fail to provide meaningful alarms to operators. Alarm generation and presentation is an area in which computer aiding is feasible and provides clear benefits. Therefore, researchers have developed several computerized alarm filtering and presentation approaches. This paper discusses problems associated with alarm generation and presentation. Approaches to improving the alarm situation and installation issues of alarm system improvements are discussed. The impact of artificial intelligence (AI) technology on alarm system improvements is assessed. (orig.)

Technical presentation

CERN Document Server

FI Department

2008-01-01

RADIOSPARES, the leading catalogue distributor of components (electronic, electrical, automation, etc.) and industrial supplies will be at CERN on Friday 3 October 2008 (Main Building, Room B, from 9.00 a.m. to 3.00 p.m.) to introduce its new 2008/2009 catalogue. This will be the opportunity for us to present our complete range of products in more detail: 400 000 part numbers available on our web site (Radiospares France, RS International, extended range of components from other manufacturers); our new services: quotations, search for products not included in the catalogue, SBP products (Small Batch Production: packaging in quantities adapted to customers’ requirements); partnership with our focus manufacturers; demonstration of the on-line purchasing tool implemented on our web site in conjunction with CERN. RADIOSPARES will be accompanied by representatives of FLUKE and TYCO ELECTRONICS, who will make presentations, demonstrate materials and answer any technical questio...

Presentations

International Nuclear Information System (INIS)

2007-01-01

The PARIS meeting held in Cracow, Poland from 14 to 15 May 2007. The main subjects discussed during this meeting were the status of international project dedicated to gamma spectroscopy research. The scientific research program includes investigations of giant dipole resonance, probe of hot nuclei induced in heavy reactions, Jacobi shape transitions, isospin mixing and nuclear multifragmentation. The mentioned programme needs Rand D development such as new scintillations materials as lanthanum chlorides and bromides as well as new photo detection sensors as avalanche photodiodes - such subjects are also subjects of discussion. Additionally results of computerized simulations of scintillation detectors properties by means of GEANT- 4 code are presented

Presentation skills for nurses.

Science.gov (United States)

Foulkes, Mark

2015-02-20

This article emphasises the importance of effective presentation skills. Such skills allow nurses to share knowledge and expertise and to communicate clearly in a range of workplace scenarios. Nurses are increasingly being asked to present in formal and informal situations, such as conferences, poster presentations, job interviews, case reports and ward-based teaching. This article explores the principles underpinning the development of these skills, discusses the situations in which they could be applied and demonstrates how nurses might improve and develop as presenters.

Presentation of the ASTRAL software; Presentation du logiciel ASTRAL

Energy Technology Data Exchange (ETDEWEB)

NONE

2011-07-01

This report presents the ASTRAL software (ASTRAL means technical assistance in post-accidental radioprotection) which is aimed to be used as an aid-to-decision tool in the case of an important release of radionuclides in the environment by assessing radionuclide concentration in different environments and food products, determining the potential exposure to irradiation of concerned populations, foreseeing the evolution of the situation, and proposing different scenarios for the management of contaminated areas. The report describes the software general operation, presents the calculation module (main functionalities, concentration index calculation, dose calculation or radiological impact calculation, how countermeasures are taken into account), the data bases (contextual data, data for radio-ecological calculations and for radiological calculations), the software ergonomics (general principles, result selection and display, result printing and input data). Its briefly evokes the development quality assurance, and describes the software implementation architecture

Erythema multiforme and Stevens-Johnson syndrome in patients receiving cranial irradiation and phenytoin

International Nuclear Information System (INIS)

Delattre, J.Y.; Safai, B.; Posner, J.B.

1988-01-01

In 15 months we encountered eight patients with intracranial tumors who developed erythema multiforme (EM) or erythema multiforme bullosa (Stevens-Johnson syndrome). All occurred shortly after use of phenytoin (DPH) and brain radiation therapy (WBRT). The clinical picture differed from the classic form of EM in that the erythema began on the scalp and spread to the extremities, progressing in three cases to extensive bullous formation. There were no cases of EM among patients who received either DPH or radiotherapy alone. The combination of DPH, WBRT, and tapering of steroids seems to predispose to EM. The pathogenesis of the disorder is probably immunologic. In the absence of seizures, anticonvulsants should not be given routinely to patients with brain tumors. When anticonvulsants are necessary in patients scheduled for WBRT, DPH may not be the drug of choice

Presenting Food Science Effectively

Science.gov (United States)

Winter, Carl K.

2016-01-01

While the need to present food science information effectively is viewed as a critical competency for food scientists by the Institute of Food Technologists, most food scientists may not receive adequate training in this area. Effective presentations combine both scientific content and delivery mechanisms that demonstrate presenter enthusiasm for…

European Society of Pediatric Radiology. Abstracts of oral presentations and poster presentations

International Nuclear Information System (INIS)

Small, P.G.

1994-01-01

The 31st Congress of the European Society of Pediatric Radiology covered all aspects of diagnostic radiology. The journal issue presents the abstracts of 77 full papers and 62 poster presentations. (MG) [de

Presenting New Grammar

Institute of Scientific and Technical Information of China (English)

WU Cai-ling; WANG Xi

2015-01-01

More and more researchers have now agreed upon the necessity of teaching grammar, but it still remains controversial as how to teach the forms, with the central consideration of not to harm the meaning-focused communicative teaching method. In this essay, one of the issues in grammar teaching will be discussed as how to present new grammar to learners, through evaluating and modifying a particular presentation activity in a grammar-teaching textbook.

A retrospective analysis of 538 sinonasal fungus ball cases treated at a single tertiary medical center in Korea (1996-2015).

Science.gov (United States)

Yoon, Young Hoon; Xu, Jun; Park, Soo Kyoung; Heo, Jae Hyung; Kim, Yong Min; Rha, Ki-Sang

2017-11-01

Sinonasal fungus ball (FB) is a type of noninvasive fungal rhinosinusitis affecting immunocompetent hosts. FB, previously considered rare, has been reported with increasing frequency. We reviewed our experience of 538 cases over the past 20 years. We retrospectively examined clinical records including clinical presentations, radiological findings, management, and outcomes of FB patients who have undergone surgery for treatment. The number of FB patients who underwent endoscopic sinus surgery (ESS) was calculated annually. Causal relationships between structural variations and FB were also investigated. The number of FB patients who underwent sinus surgery has increased. The mean age was 58.3 years, and the gender ratio was approximately 2 (female): 1 (male). While the most common presenting symptoms of maxillary sinus FB patients were nasal symptoms, such as postnasal drip and nasal obstruction, sphenoid sinus FB patients presented with headache mostly. On computed tomography (CT) scans, the most common finding was intralesional hyperdensity (77.3%). There was no significant correlation between the presence of FB and structural variations (nasal septal deviation, concha bullosa, Haller cell). Median follow-up period of the patients was 11 months. Recurrence or residual disease occurred in only 6 (1.1%) cases. The number of FB patients who underwent surgery has increased steadily over the past 20 years. FB should be considered in patients with unilateral nasal symptoms and unexplained headaches. A preoperative CT scan is an essential tool in making diagnosis easier and faster. Endoscopic surgery is the treatment of choice, with a low morbidity and recurrence rate. © 2017 ARS-AAOA, LLC.

Lecture Presentations

International Nuclear Information System (INIS)

2008-01-01

The Symposium on Physics of Elementary Interactions in the LHC Era held in Warsaw from 21 to 22 April 2008. The main subject of the workshop was to present the progress in CERN LHC collider project. Additionally some satellite activities in field of education, knowledge and technology transfer in the frame of CERN - Poland cooperation were shown

Conns' syndrome - atypical presentations

International Nuclear Information System (INIS)

Kumar, K V S Hari; Modi, K D; Jha, Sangeeta; Jha, Ratan

2009-01-01

Primary hyperaldosteronism (Conns' syndrome) commonly presents with a combination of clinical features of hypokalemia and hypertension. Atypical presentations like normotension, normokalemia and neurological ailments are described in few cases. We encountered two such cases, the first presenting with acute neurological complaint and second case having insignificant hypertension. Both the patients had a characteristic biochemical and imaging profile consistent with primary hyperaldosteronism and responded to surgical resection of adrenal adenoma. (author)

Tips for Good Electronic Presentations.

Science.gov (United States)

Strasser, Dennis

1996-01-01

Describes library uses of presentation graphics software and offers tips for creating electronic presentations. Tips include: audience retention; visual aid options; software package options; presentation planning; presentation showing; and use of text, colors, and graphics. Sidebars note common presentation errors and popular presentation…

CERN Electronics Pool presentations

CERN Multimedia

2011-01-01

The CERN Electronics Pool has organised a series of presentations in collaboration with oscilloscope manufacturers. The last one will take place according to the schedule below.   Time will be available at the end of the presentation to discuss your personal needs. The Agilent presentation had to be postponed and will be organised later. -     Lecroy: Thursday, 24 November 2011, in 530-R-030, 14:00 to 16:30.

Presenting in Virtual Worlds: Towards an Architecture for a 3D Presenter explaining 2D-Presented Information

NARCIS (Netherlands)

van Welbergen, H.; Hendler, J.; Goren-Bar, D.; Nijholt, Antinus; Reidsma, Dennis; Mayora-Ibarra, O.; Zwiers, Jakob

Meeting and lecture room technology is a burgeoning field. Such technology can provide real-time support for physically present participants, for online remote participation, or for offline access to meetings or lectures. Capturing relevant information from meetings or lectures is necessary to

Punch up your podium presentations.

Science.gov (United States)

Wood, Tim J; Hollier, Amelie

2017-08-01

The goal of most presentations is to engage audience participants. Effective presenters go beyond content expertise to connect with audiences through various techniques, formats, and presentation styles. Mayer's theory of multimedia learning, neurocognitive factors, and the assertion-evidence approach are essential elements that presenters can use if they have not had formal training in creating presentations. Tips are offered on how to connect with the audience and how to avoid known pitfalls. ©2017 American Association of Nurse Practitioners.

Creating meaningful multimedia presentations

NARCIS (Netherlands)

Hardman, H.L.; Ossenbruggen, van J.R.

2006-01-01

Finding relevant information is one step in the chain of understanding information. Presenting material to a user in a suitable way is a further step. Our research focuses on using semantic annotations of multimedia elements to increase the "presentability" of retrieved information. We investigate

Spotlight on the presenter : a study into presentations of conference papers with PowerPoint

NARCIS (Netherlands)

Hertz, B.

2015-01-01

Abstract

1. Introduction

PowerPoint is the most widely used presentation software tool. As of 2012, PowerPoint had more than 200 million presenters worldwide. Presenters all over the world use the program. Some use it for university teaching, others

Why do children present to emergency departments? Exploring motivators and measures of presentation appropriateness for children presenting to a paediatric emergency department.

Science.gov (United States)

Cheek, John A; Braitberg, George; Craig, Simon; West, Adam

2017-05-01

To compare the parental motivators and referring general practitioner's (GP's) reasons for advising emergency department (ED) attendance with the assessment of ED medical staff. To compare ED clinician opinion with other published methods that have attempted to define 'primary care suitable' presentations to the ED. A prospective observational study and series of surveys regarding the attendance of children presenting to a single tertiary paediatric ED. Surveys were distributed to the treating ED clinician, the child's parent/guardian, and the referring GP. Results between the three groups were analysed and compared. There were a total of 1069 presentations during the study period. Six hundred (58.4%, 95% CI 55.3-61.4%) presentations were judged as 'ED appropriate' by the treating ED clinician. When compared with methods used to retrospectively judge whether ED patients are considered 'primary care suitable', ED clinicians disagree between 22.4 and 38.8% of the time. For patients who presented directly to ED, 85.6% did so for a medical reason, whilst 32.1% did so for a GP access reason. Being referred by a GP improved the ED clinicians' opinion of the appropriateness of the presentation (49.2 vs. 73.9%, P motivators that drive this behaviour is needed. We believe the solution to increasing utilisation of EDs by children must be a balanced approach that addresses community expectations and appropriately resources EDs. © 2017 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).

Unusual presentation of sunburn.

Science.gov (United States)

Verma, Gopalkrishna G; Dave, Dhaval; Byrne, Eileen

2008-10-01

We present three cases of sunburn to the head, presenting with oedema of the face in children aged 6, 9 and 13 years. Oedema was predominantly on the forehead and temporal region; a direct effect of gravity was associated with erythema of the scalp. Sunburn healed without any complications.

An Atypical Presentation on Insulinoma

Science.gov (United States)

2017-06-16

PUBLICATIONS/ PRESENTATIONS 1. TO: CLINICAL RESEARCH 2. FROM: (Author’s Name, Rank, Grade, Office Symbol) 3. GME/GHSE STUDENT: 4. PROTOCOL NUMBER: Kluesner...PROCESSING OF PROFESSIONAL MEDICAL RESEARCH/TECHNICAL PUBLICATIONS/ PRESENTATIONS 1st ENDORSEMENT (59 MDW/SGVU Use Only) TO: Clinical Research Division 24...CAPT JOSEPH KLUESNER FROM: 59 MDW/SGYU SUBJECT: Professional Presentation Approval 1. Your paper, entitled An Atypical Presentation of Insulinoma

Presentation to the symposium

International Nuclear Information System (INIS)

Goodhead, D.

1997-01-01

A novel theory, the Second Event theory, advanced by Chris Busby, suggested that certain man-made radioisotopes could bypass cellular genetic repair mechanisms and cause cancer at very low dose. The present paper, after some introductory comments on the effects of particle tracks on cells, goes on to present the author's own perception of this Second Event theory. (UK)

Creating an effective poster presentation.

Science.gov (United States)

Taggart, H M; Arslanian, C

2000-01-01

One way to build knowledge in nursing is to share research findings or clinical program outcomes. The dissemination of these findings is often a difficult final step in a project that has taken months or years to complete. One method of sharing findings in a relaxed and informal setting is a poster presentation. This method is an effective form for presenting findings using an interactive approach. The milieu of a poster presentation enables the presenters to interact and dialogue with colleagues. Guidelines for size and format require that the poster is clear and informative. Application of design helps to create visually appealing posters. This article summarizes elements of designing and conducting a poster presentation.

An unusual presentation of presentation of a branchial cleft cyst.

Science.gov (United States)

Vemula, Rahul; Greco, Gregory

2012-05-01

Branchial cleft cysts are congenital anomalies that arise from the aberrant embryological development of the branchial apparatus. The location of a branchial cleft cyst is determined by which branchial cleft failed to obliterate during embryological development, with the second branchial cleft cyst being the most commonly recognized lesion. Although the most common location for branchial cleft cysts is between the external auditory canal and the level of the clavicle, the literature does describe unusual locations. We present a case a 15-year-old boy who had an enlarging lesion on his back that had been present since birth. A presumptive radiologic diagnosis of lymphangioma circumscriptum was made. Upon excision of the lesion and pathologic examination, it was determined to be a branchial cleft cyst. The patient had an uneventful postoperative course, and no recurrence was noted after a 2-year follow-up. Our clinical report demonstrates a lesion on the posterior thorax that proved to be a branchial cleft cyst and should always be part of the differential diagnosis for soft tissue masses of the thorax.

Turning Scientific Presentations into Stories

Science.gov (United States)

Aruffo, Christopher

2015-01-01

To increase students' confidence in giving scientific presentations, students were shown how to present scientific findings as a narrative story. Students who were preparing to give a scientific talk attended a workshop in which they were encouraged to experience the similarities between telling a personal anecdote and presenting scientific data.…

Toxoplasmosis presented as a submental mass: a common disease, uncommon presentation.

Science.gov (United States)

Li, Bo; Zou, Jian; Wang, Wei-Ya; Liu, Shi-Xi

2015-01-01

Submental mass secondary to toxoplasmosis is not common in clinical work. A diagnosis of toxoplasmosis is rarely considered by physicians. Here we describe a 50-year-old woman presented with a progressive, painful, submental and left neck swelling for 1 month. After having obtained an insufficient evidence from the fine-needle biopsy, the patient finally received an excisional biopsy which highly indicated the possibility of lymphadenopathy consistent with toxoplasmosis. Diagnosis of toxoplasmosis was finally established by a combination of the pathological criteria, together with the positive serological finding. According to review the clinical presentations, pathological characteristics, diagnostic standard and treatment of this disease, the article aims to remind otolaryngologists who are evaluating a neck mass should be aware of the infectious cause of lymphadenopathy and the possibility of toxoplasmosis.

Presentation of CEA

International Nuclear Information System (INIS)

Graf, J.J.

1976-01-01

The organization of the CEA, its missions and means are presented. Its activities in the field of light water reactors, fast neutron reactors and PWR reactors of small and medium power for electricity and/or heat generation are emphasized [fr

Intracellular events regulating cross-presentation

Directory of Open Access Journals (Sweden)

Peter eCresswell

2012-06-01

Full Text Available Cross-presentation plays a fundamental role in the induction of CD8-T cell immunity. However, although more than three decades have passed since its discovery, surprisingly little is known about the exact mechanisms involved. Here we give an overview of the components involved at different stages of this process. First, antigens must be internalized into the cross-presenting cell. The involvement of different receptors, method of antigen uptake, and nature of the antigen can influence intracellular trafficking and access to the cross-presentation pathway. Once antigens access the endocytic system, different requirements for endosomal/phagosomal processing arise, such as proteolysis and reduction of disulfide bonds. The majority of cross-presented peptides are generated by proteasomal degradation. Therefore, antigens must cross a membrane barrier in a manner analogous to the fate of misfolded proteins in the endoplasmic reticulum (ER that are retrotranslocated into the cytosol for degradation. Indeed, some components of the ER-associated degradation (ERAD machinery have been implicated in cross-presentation. Further complicating the matter, endosomal and phagosomal compartments have been suggested as alternative sites to the ER for loading of peptides on MHC class I molecules. Finally, the antigen presenting cells involved, particularly dendritic cell subsets and their state of maturation, influence the efficiency of cross-presentation.

Present status of dibaryons

International Nuclear Information System (INIS)

Locher, M.P.

1981-01-01

In the present notes the author tries to assess the situation regarding dibaryons in the various channels with baryon number two, emphasizing the problems and discussing in greater detail the most recent developments since the Versailles Conference. One of the motivations for the present search of B=2 states is the possibility of colored subclusters for systems with six quarks which cannot occur for ordinary baryons or mesons. (Auth.)

The dilatations with the balloon catheter in treatment of the patients with the benign oesophageal stenoses

International Nuclear Information System (INIS)

Valek, V.; Benda, K.; Hrobar, P.; Mrazova, J.; Prasek, J.; Bilicky, J.; Vomela, J.; Hep, A.; Muenzova, H.

1996-01-01

Between January 1989 and June 1995 the authors dilated in the Department of Radiology in University Hospital Bohunice the benign stenosis of the oesophagus with balloon dilatation catheter in 76 of other patients. The aetiology of the stenosis was long-term reflux oesophagitis in the 39 patients (51%), achalasia 14 patients, 18%) postoperative benign stenosis in anastomosis (7 patients, 9%), corrosive stricture (5 patients, 6,5%), post-radiation stricture (5 patients, 6,5%) and epidermolysis bulosa oesophagi (2 patients, 3%). In four patients (5%) the aetiology of the stenosis was unknown. The standing of the dilatation was from 15 second to 6 minutes and dilatation was technically successful in all cases. First the authors used Owman Oesophageal Dilatation Kit (William COOK), before three years they started to used Rigiflex ORW Dilators Kit (Microvasive). In the patients with achalasia the authors currently used Rigiflex ABD Achalasia Balloon Dilator (Microvasive). (authors). 2 tabs., 3 figs., 14 refs

Summary of the presentations

International Nuclear Information System (INIS)

2002-01-01

This part summarizes the content of the six following presentations: 1. IAEA requirements/guidance for the decommissioning safety case (Mike Bell, IAEA); 2. Safety case in an EIA context (Patrick O'SULLIVAN, The Netherlands); 3. Plant configuration and the D and D licensing process (Eugenio GIL LOPEZ and Pedro CARBONERAS, Spain); 4. Accident assessment (Ivo TRIPPUTI, Italy; Ingmar LUND, Sweden); 5. Balancing radiological and industrial risk in the safety case (Pascal DEBOODT, Belgium); 6. The safety case for safe store and dormancy periods (Paul WOOLLAM, UK). Following the six presentations, the WPDD members discussed the safety case in an open discussion led by the Topical Session Chair, Frances Taylor. The discussion was wide-ranging, and topics varied from the standards that apply to releases to the environment during safe store, to decommissioning parallels with other major processing industries and facilities (such as oil rigs). Frances Taylor steered the discussion toward addressing the questions that the WPDD Core Group had proposed for the topical session. The Chair, the Rapporteur, and some members of the group sensed that the presentations had covered such an wide variety of issues, and that the content of the presentations themselves differed so greatly, that it would be difficult to distill a position paper from the information gathered, as had been the mandate from the Core Group. In addition, the meaning of the term 'Safety Case' differed greatly among participants in the topical session. Some took the term to mean the analysis and document that the implementor provides to the regulator, describing how operations will be performed safely. Others considered the term to mean the overall safety and risk conditions inherent in a nuclear facility during operations or decommissioning. With the differences in presentations and with the meaning of the terminology remaining unclear, the WPDD participants did not reach consensus conclusions on December 5, 2001

Present State Examination

DEFF Research Database (Denmark)

Pedersen, Kamilla; Bertelsen, Aksel

Present State Examination (PSE) 5. udgave er en revideret version af speciallæge i psykiatri Aksel Bertelsens oprindelige materiale. Aksel Bertelsen er ophavsmand til PSE og til den danske udgave af ICD-10. Revisionen omfatter et forbedret layout, en modernisering af sproget, tilføjelse af...

Bad (Re)Presentations

CERN Multimedia

Computer Security Team

2013-01-01

This time, we would like to address copyright violations (again) - but with a twist. While previous articles focussed on “Music, videos and the risk for CERN” or on software licences, the focus is now on using photos, music and images “found” on the Web for presentations, publications and web pages…   Just recently, a video produced for CERN and published on YouTube and Facebook used background music from a contemporary popular artist. However, the people responsible failed to obtain the appropriate permission to actually use that music. They thought that having bought it on iTunes was sufficient. But it was not: “buying” means you have the right to listen, but not the right for further distribution or re-publication. As a result, the videos were pulled from YouTube and Facebook. Similarly, how many of us integrate graphics and images “found” on the Web into our presentations?  How many of us...

Present and Future of Nuclear Robotics; Presente y futuro de la robotica nuclear

Energy Technology Data Exchange (ETDEWEB)

Bielza Ciaz-Caneja, M [ENDESA, (Spain); Carmena Servet, P [AMYS, (Spain); Gomez Santamaria, J [IBERDROLA, (Spain); Gonzalez Fernandez, J [NUCLENOR, (Spain); Izquierdo Mendoza, J A [C.N. COFRENTES, (Spain); Linares Pintos, F [ENSA, (Spain); Gonzalez, Martinez [CASA, (Spain); Muntion Ruesgas, A [C.N. STA Maria de Garona, (Spain); Serna Oliveira, M A [CEIT, (Spain)

1997-10-01

New technologies have increased the use of robotic systems in fields other than Industry. As a result, research and developers are focusing their interest in concepts like Intelligent Robotics and Robotics in Services. This paper describes the use of Robotics in Nuclear facilities, where robots can be used to protect workers in high radiation areas, to reduce total worker exposure and to minimise downtime. First, the structure of robot systems is introduced and the benefits of nuclear robots is presented. Next, the paper describes some specific nuclear applications and the families of nuclear robots present in the market. After that, a section is devoted to Nuclear Robotics in Spain, with emphasis in some of the developments being carried out at present. Finally, some reflections about the future of robots in Nuclear Industry are offered. (Author) 18 refs.

Atypical presentations of Wolframs syndrome

Directory of Open Access Journals (Sweden)

S Saran

2012-01-01

Full Text Available Background: Wolfram syndrome is a rare hereditary or sporadic neurodegenerative disorder also known as DIDMOAD. The classically described presentation is of insulin-dependent diabetes, followed by optic atrophy, central diabetes insipidus, and sensory neural deafness. Also included are less well-described presentations of Wolframs syndrome. We here present three cases of atypical presentation of this syndrome. Case 1: A 15-year-old boy with insulin-dependent diabetes was presented for evaluation of depressive symptoms associated with suicidal tendency. Neuropsychiatric manifestations are described with Wolframs syndrome, and wolframin gene, in recessive inheritance, is associated with psychiatric illnesses without other manifestations of Wolframs syndrome. Case 2: A 17-year-old diabetic boy on insulin with good control of blood sugar presented for evaluation of delayed puberty. Central hypogonadism and other anterior pituitary hormone dysfunctions are the less publicized hormone dysfunctions in Wolframs syndrome. Case 3: A 23-year-old female who was on insulin for diabetes for the past 14 years, got admitted for evaluation of sudden loss of vision. This patient had developed a vitreous hemorrhage and, on evaluation, was found to have optic atrophy, sensory neural hearing loss, and diabetes insipidus, and presented differently from the gradual loss of vision described in Wolframs syndrome. Conclusion: Wolframs syndrome being a multisystem degenerative disorder can have myriad other manifestations than the classically described features. Neuropsychiatric manifestations, depression with suicidal risk, central hypogonadism, and secondary adrenal insufficiency are among the less well-described manifestations of this syndrome.

Pseudohypertension-Like Presentation: An Exceptionally Rare Presentation in an Athletic Female Patient with Morphea

Directory of Open Access Journals (Sweden)

Ahmed Al-Imam

2016-01-01

Full Text Available Introduction. Pseudohypertension is a condition which mainly occurs due to thickening-calcification of tunica intima of the arterial wall, leading to a faulty measurement of the intra-arterial blood pressure. To the best of our knowledge, this is the first case report in literature, of a pseudohypertension-like presentation in association with Morphea en plaque. Case Presentation. This is a rare presentation of a young athletic female and a professional tennis player, with pseudohypertension-like presentation. The patient had a traumatic injury to the right elbow joint; the injury occurred during a professional tennis match. The injury was managed by immobilization, physiotherapy, and Low-Level Laser Therapy. Soon after that, the patient had a circumscribed sclerotic ivory plaque affecting the skin of the right cubital fossa. The histopathology analysis, together with the serological-hematological tests and the clinical picture, along with positive Osler’s signs, leads to the conclusive diagnosis of Morphea en plaque. The peculiar anatomic localization of the plaque anterior to the brachial artery leads to faulty blood pressure measurement as recorded by mercurial sphygmomanometer. Conclusion. This unique presentation of Morphea en plaque carries an important message in relation to the basic medical practice and in relation to the accurate measurement of the vital signs.

A centralized audio presentation manager

Energy Technology Data Exchange (ETDEWEB)

Papp, A.L. III; Blattner, M.M.

1994-05-16

The centralized audio presentation manager addresses the problems which occur when multiple programs running simultaneously attempt to use the audio output of a computer system. Time dependence of sound means that certain auditory messages must be scheduled simultaneously, which can lead to perceptual problems due to psychoacoustic phenomena. Furthermore, the combination of speech and nonspeech audio is examined; each presents its own problems of perceptibility in an acoustic environment composed of multiple auditory streams. The centralized audio presentation manager receives abstract parameterized message requests from the currently running programs, and attempts to create and present a sonic representation in the most perceptible manner through the use of a theoretically and empirically designed rule set.

Thyrotoxic crisis presenting with jaundice.

Science.gov (United States)

Wickramasinghe, R D S S; Luke, W A N V; Sebastiampillai, B S; Gunathilake, M P M L; Premaratna, R

2016-06-23

Thyrotoxic crisis is a medical emergency requiring early diagnosis and urgent management, which can be challenging due to its diverse clinical presentations. While common presentations include fever, sweating, palpitations, tremors and confusion, presence of jaundice is rare. We report a 35-year-old male who presented with jaundice due to cholestasis along with other features of thyrotoxic crisis due to Graves' disease. He had a good clinical recovery with resolution of cholestasis following treatment for thyrotoxic crisis. Jaundice can be a rare manifestation of thyrotoxic crisis, and should be considered in the differential diagnosis when other clinical features of thyrotoxic crisis are present. However secondary causes of jaundice should be looked into and excluded.

Renewable Electricity Futures (Presentation)

Energy Technology Data Exchange (ETDEWEB)

Mai, T.

2012-08-01

This presentation library summarizes findings of NREL's Renewable Electricity Futures study, published in June 2012. RE Futures investigated the challenges and impacts of achieving very high renewable electricity generation levels in the contiguous United States by 2050. It was presented at the 2012 RE AMP Annual Meeting. RE-AMP is an active network of 144 nonprofits and foundations across eight Midwestern states working on climate change and energy policy with the goal of reducing global warming pollution economy-wide 80% by 2050.

Book Presentation | 27 February

CERN Multimedia

The CERN Library

2013-01-01

"A caccia del bosone di Higgs. Magneti, governi, scienziati e particelle nell'impresa scientifica del secolo" by Luciano Maiani and Romeo Bassoli, published by Mondadori. On Wednesday 27 February Luciano Maiani will present the book "A caccia del bosone di Higgs. Magneti, governi, scienziati e particelle nell'impresa scientifica del secolo". More information here. The presentation will take place in the Council Chamber at 16.00 and will be followed by a debate and book signing. The book will be on sale. You are cordially invited.

Milk: Past and Present

Science.gov (United States)

Bulajić, S.; Đorđević, J.; Ledina, T.; Šarčević, D.; Baltić, M. Ž.

2017-09-01

Although milk/dairy consumption is part of many cultures and is recommended in most dietary guidelines around the world, its contribution to overall diet quality remains a matter of controversy, leading to a highly polarized debate within the scientific community, media and public sector. The present article, at first, describes the evolutionary roots of milk consumption, then reviews the milk-derived bioactive peptides as health-promoting components. The third part of the article, in general, presents the associations between milk nutrients, disease prevention, and health promotion.

Presentation

Directory of Open Access Journals (Sweden)

Eduardo Vicente

2013-06-01

Full Text Available In the present edition of Significação – Scientific Journal for Audiovisual Culture and in the others to follow something new is brought: the presence of thematic dossiers which are to be organized by invited scholars. The appointed subject for the very first one of them was Radio and the invited scholar, Eduardo Vicente, professor at the Graduate Course in Audiovisual and at the Postgraduate Program in Audiovisual Media and Processes of the School of Communication and Arts of the University of São Paulo (ECA-USP. Entitled Radio Beyond Borders the dossier gathers six articles and the intention of reuniting works on the perspectives of usage of such media as much as on the new possibilities of aesthetical experimenting being build up for it, especially considering the new digital technologies and technological convergences. It also intends to present works with original theoretical approach and original reflections able to reset the way we look at what is today already a centennial media. Having broadened the meaning of “beyond borders”, four foreign authors were invited to join the dossier. This is the first time they are being published in this country and so, in all cases, the articles where either written or translated into Portuguese.The dossier begins with “Radio is dead…Long live to the sound”, which is the transcription of a thought provoking lecture given by Armand Balsebre (Autonomous University of Barcelona – one of the most influential authors in the world on the Radio study field. It addresses the challenges such media is to face so that it can become “a new sound media, in the context of a new soundscape or sound-sphere, for the new listeners”. Andrew Dubber (Birmingham City University regarding the challenges posed by a Digital Era argues for a theoretical approach in radio studies which can consider a Media Ecology. The author understands the form and discourse of radio as a negotiation of affordances and

Discussion paper (presented during meeting)

International Nuclear Information System (INIS)

Allemand, R.

1975-01-01

A method of particle localization for gas chambers (backgammon game method) is presented. The cathode is geometrically cut in such a way that it is possible to establish a simple relation between the signal amplitude on the different parts of the cathode and the position of the nuclear event. The results obtained in the case of x-ray detectors are presented. The application of this method to the localization of neutrons is presently studied [fr

Climatic Change--Past, Present & Future

Science.gov (United States)

Lindholm, Roy C.

1976-01-01

Presented is a review of studies investigating factors affecting climatic changes in the Earth's atmosphere--past, present, and future. Dating methods, particularly the Oxygen 18/16 method, are discussed. (SL)

Technical presentation

CERN Document Server

FP Department

2009-01-01

07 April 2009 Technical presentation by Leuze Electronics: 14.00 – 15.00, Main Building, Room 61-1-017 (Room A) Photoelectric sensors, data identification and transmission systems, image processing systems. We at Leuze Electronics are "the sensor people": we have been specialising in optoelectronic sensors and safety technology for accident prevention for over 40 years. Our dedicated staff are all highly customer oriented. Customers of Leuze Electronics can always rely on one thing – on us! •\tFounded in 1963 •\t740 employees •\t115 MEUR turnover •\t20 subsidiaries •\t3 production facilities in southern Germany Product groups: •\tPhotoelectric sensors •\tIdentification and measurements •\tSafety devices

Unusual presentation of Klinefelter syndrome

Science.gov (United States)

Das, Chanchal; Sahana, Pranab Kumar; Sengupta, Nilanjan; Roy, Mukut; Dasgupta, Ranen

2013-01-01

Introduction: Klinefelter syndrome usually presents in the puberty and adulthood with its characteristic features. We report a boy who had Klinefelter syndrome with hypospadias and hydrocele. Case Note: Six and half year old boy had complaints of genitourinary problem in the form of hypospadias, small phallus and hydrocele. Karyotyping showed 47,XXY. Conclusion: This case illustrates that Klinefelter syndrome was presented in the infancy with hypospadias and hydrocele which are very uncommon presentation of the disease PMID:24910838

Computerized Tomographic Study on the Anatomic Variation of the Paranasal Sinus

Energy Technology Data Exchange (ETDEWEB)

Choi, Sun Kyung; Lim, Sug Young; Koh, Kwang Joon [Dept. of Oral and Maxillofacial Radiology and Institute of Oral Bio Science, College of Dentistry, Chonbuk National University, Chonju (Korea, Republic of); Park, Mi Ju [Dept. of Prosthodontics, School of Denstistry, Chonbuk National Universty, Chonju (Korea, Republic of)

1999-08-15

To evaluate the anatomic variations of the paranasal sinuses on computed tomographs. The author examined the CT images of the paranasal sinuses retrospectively in 500 patients who visited Chonbuk National University Hospital between January 1996 and December 1997. The highest incidence of anatomic variation of the paranasal sinuses in bilateral structures was agger nasi cell (73.2%), followed by concha bullosa (31.1%), Onodi cell (24.0%), Haller cell (19.8%), maxillary sinus septum (13.0%), paradoxical middle turbinate (2.5%), pneumatized uncinate process (2.0%), and bent uncinate process. The highest incidence of anatomic variation in midline structures was nasal septum deviation(53.2%), followed by nasal septumaerated (29.4%), bulla galli (24.7%) asymmetric intersphenoid septum (22.3%), and nasal septum spur (13.8%). The correlation between anatomic variation and paranasal sinusitis was not found. The results of this study will aid in the diagnosis and treatment of paranasal sinus diseases, especially in the treatment planning before functional endoscopic surgery.

Computerized Tomographic Study on the Anatomic Variation of the Paranasal Sinus

International Nuclear Information System (INIS)

Choi, Sun Kyung; Lim, Sug Young; Koh, Kwang Joon; Park, Mi Ju

1999-01-01

To evaluate the anatomic variations of the paranasal sinuses on computed tomographs. The author examined the CT images of the paranasal sinuses retrospectively in 500 patients who visited Chonbuk National University Hospital between January 1996 and December 1997. The highest incidence of anatomic variation of the paranasal sinuses in bilateral structures was agger nasi cell (73.2%), followed by concha bullosa (31.1%), Onodi cell (24.0%), Haller cell (19.8%), maxillary sinus septum (13.0%), paradoxical middle turbinate (2.5%), pneumatized uncinate process (2.0%), and bent uncinate process. The highest incidence of anatomic variation in midline structures was nasal septum deviation(53.2%), followed by nasal septumaerated (29.4%), bulla galli (24.7%) asymmetric intersphenoid septum (22.3%), and nasal septum spur (13.8%). The correlation between anatomic variation and paranasal sinusitis was not found. The results of this study will aid in the diagnosis and treatment of paranasal sinus diseases, especially in the treatment planning before functional endoscopic surgery.

Pulmonary hyalinizing granuloma presenting with dysphagia: a rare presentation.

Science.gov (United States)

Khan, Fazal; Hamid, Arsalan; Fatima, Benish; Hashmi, Shiraz; Fatimi, Saulat

2017-01-01

A 25-year-old man presented with a 2-month history of dysphagia and past history of pulmonary and intestinal tuberculosis. A barium swallow showed a point of constriction 42 mm above the gastroesophageal junction. Computed tomography revealed large opacities in bilateral lung fields, encroaching more on the esophagus. The lesion progressively compressed the esophagus as it moved inferiorly. A right posterolateral thoracotomy was performed for sub-anatomical resection of the mass. A biopsy revealed homogenous whirling hyalinized collagen fibers, highly suggestive of pulmonary hyalinizing granuloma, with no evidence of malignancy. Pulmonary hyalinizing granuloma should be considered in the differential diagnosis of longstanding dysphagia.

Agriculture Oral Presentations

International Nuclear Information System (INIS)

1997-01-01

This publication contains 23 papers related to the use of nuclear techniques in plant breeding in Turkey, effect of gamma irradiations on growing various plants, mutations and soil chemistry, etc., presented at 4. International Congress of Nuclear Agriculture and Animal Science in Bursa, Turkey, 25-27 Sep 1996. A separate abstract was prepared for each paper

An unusual presentation of sporotrichosis

Directory of Open Access Journals (Sweden)

S S Vaishampayan

2013-01-01

Full Text Available Sporotrichosis can mimic a few diseases. Sporotrichosis presenting as a hypoaesthetic plaque or plaque suggestive of leprosy has not been mentioned in literature. Herein we present a case of a 26 years old man who presented with hypoesthetic plaque on left ankle and was clinically diagnosed to have borderline tuberculoid leprosy who later developed few nodules in linear fashion on left leg, which ruptured to form non healing ulcers. Culture from ulcer grew Sporothrix schenckii. Patient was successfully treated with oral potassium iodide.

Ten steps to successful poster presentation.

Science.gov (United States)

Hardicre, Jayne; Devitt, Patric; Coad, Jane

Receiving a letter confirming acceptance for you to present a poster at a conference can evoke mixed emotions. Joy, panic, fear and dread are among the many possible emotions and this is not exclusive to first time presenters. Developing an effective poster presentation is a skill that you can learn and can provide a rewarding way to present your work in a manner less intimidating than oral presentation (Shelledy, 2004). The key to successful poster presentation is meticulous, timely, well informed preparation. This article outlines ten steps to help guide you through the process to maximize your success.

Antigen Cross-Presentation of Immune Complexes

Science.gov (United States)

Platzer, Barbara; Stout, Madeleine; Fiebiger, Edda

2014-01-01

The ability of dendritic cells (DCs) to cross-present tumor antigens has long been a focus of interest to physicians, as well as basic scientists, that aim to establish efficient cell-based cancer immune therapy. A prerequisite for exploiting this pathway for therapeutic purposes is a better understanding of the mechanisms that underlie the induction of tumor-specific cytotoxic T-lymphocyte (CTL) responses when initiated by DCs via cross-presentation. The ability of humans DC to perform cross-presentation is of utmost interest, as this cell type is a main target for cell-based immunotherapy in humans. The outcome of a cross-presentation event is guided by the nature of the antigen, the form of antigen uptake, and the subpopulation of DCs that performs presentation. Generally, CD8α+ DCs are considered to be the most potent cross-presenting DCs. This paradigm, however, only applies to soluble antigens. During adaptive immune responses, immune complexes form when antibodies interact with their specific epitopes on soluble antigens. Immunoglobulin G (IgG) immune complexes target Fc-gamma receptors on DCs to shuttle exogenous antigens efficiently into the cross-presentation pathway. This receptor-mediated cross-presentation pathway is a well-described route for the induction of strong CD8+ T cell responses. IgG-mediated cross-presentation is intriguing because it permits the CD8− DCs, which are commonly considered to be weak cross-presenters, to efficiently cross-present. Engaging multiple DC subtypes for cross-presentation might be a superior strategy to boost CTL responses in vivo. We here summarize our current understanding of how DCs use IgG-complexed antigens for the efficient induction of CTL responses. Because of its importance for human cell therapy, we also review the recent advances in the characterization of cross-presentation properties of human DC subsets. PMID:24744762

Typhoid Fever Presenting With Acute Renal Failure And Hepatitis Simultaneously - A Rare Presentation

Directory of Open Access Journals (Sweden)

Rajput R.

2016-05-01

Full Text Available Typhoid fever is an important health problem worldwide but its incidence is more in developing countries. Hepatic involvement is common, but both hepatic and renal involvement is rare in typhoid fever. We report a case of typhoid fever presenting with hepatitis and acute renal failure. A 17 year old male presenting with fever and pain abdomen was found to have raised blood urea, creatinine, liver enzymes and bilirubin. Widal and typhidot (IgM,IgG test were positive. His symptoms subsided and deranged parameters resolved with treatment of typhoid fever.

Integral Presentations of Catalan Numbers

Science.gov (United States)

Dana-Picard, Thierry

2010-01-01

We compute in three different ways the same definite parametric integral. By-products are the derivation of a combinatorial identity and two integral presentations of Catalan numbers. One of them leads to a presentation using the [gamma] function.

Presentation = Speech + Slides

Directory of Open Access Journals (Sweden)

Derik Badman

2008-12-01

Full Text Available Back in October, Aaron Schmidt posted “HOWTO give a good presentation” to his blog walking paper. His second bullet point of “thoughts” on good presentations is: Please don’t fill your slides with words. Find some relevant and pretty pictures to support what you’re saying. You can use the pictures to remind yourself what you’re going [...

Present market for nuclear energy

International Nuclear Information System (INIS)

Marzo, M.A.S.

1987-01-01

The present market for nuclear energy is present since nuclear production and electric power generation to the utilization of radioisotopes in medicine and biology. Some data about the main world suppliers to this market are shown. (E.G.) [pt

Multiphasic presentation of Rasmussen's encephalitis

NARCIS (Netherlands)

Avbersek, A.; Miserocchi, A.; McEvoy, A.W.; Patel, A.V.; Aronica, E.; Blumcke, I.; Jacques, T.S.; Acheson, J.; Thom, M.; Sisodiya, S.M.

2015-01-01

Rasmussen's encephalitis is a rare, chronic inflammatory disorder of unknown cause, characterised by drug-resistant focal epilepsy that may rarely present in adolescence or adulthood. We present a case of Rasmussen's encephalitis with prominent recurrent fluctuation in symptoms and well-documented

Clinical presentation of familial exudative vitreoretinopathy.

Science.gov (United States)

Ranchod, Tushar M; Ho, Lawrence Y; Drenser, Kimberly A; Capone, Antonio; Trese, Michael T

2011-10-01

To describe the clinical characteristics, staging and presentation of patients with familial exudative vitreoretinopathy (FEVR) in our clinical practice over the last 25 years. Case series, retrospective review. We included 273 eyes of 145 patients. Data collected from charts included gender, gestational age at birth, birthweight, age at presentation, referring diagnosis, family history, prior ocular surgery, and clinical presentation in each eye. Eyes with invasive posterior segment procedures before initial presentation were excluded. Demographics on presentation and clinical staging. Patients were slightly male predominant (57%) with a mean birthweight of 2.80 kg (range, 740 g-4.76 kg), mean gestational age of 37.8 weeks (range, 25-42), and mean age at presentation of almost 6 years (range, presentation, gestational age, and birthweight. Although a positive family history on presentation may support the diagnosis of FEVR, a negative family history is of little help. The majority of retinal folds extended radially in the temporal quadrants, but radial folds were seen in almost all quadrants. Fellow eyes demonstrated a wide variation in symmetry. The presentation of FEVR may mimic the presentation of other pediatric and adult vitreoretinal disorders, and careful examination is often crucial in making the diagnosis of FEVR. The authors have no proprietary or commercial interest in any of the materials discussed in this article. Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

Presentation Skills Workshops for Nurses.

Science.gov (United States)

Kinn, S.; Kenyon, M.

2002-01-01

Workshops were held to prepare nurses (n=87) to present results of professional activities. One year after the course, 20 had made oral and 30 written presentations. The workshops increased their confidence and were considered practical, informal, and nonthreatening. (Contains 31 references.) (SK)

Suprasellar ganglioglioma presenting with diabetes insipidus in a young boy: a rare clinical presentation.

Science.gov (United States)

Gupta, Ruchika; Suri, Vaishali; Arora, Raman; Sharma, Mehar C; Mishra, Shashwat; Singh, Manmohan; Sarkar, Chitra

2010-02-01

Gangliogliomas are rare tumors composed of an admixture of glial and neuronal components. These usually occur in young patients, who present with therapy-resistant seizures. Clinical presentation of ganglioglioma with diabetes insipidus is extremely rare with only one case reported earlier in the available literature. Due to this rarity, ganglioglioma is not considered in the differential diagnosis in a patient with diabetes insipidus. A 7-year boy presented with polyuria, polydipsia, and progressive visual loss for 18 months. Investigations revealed diabetes insipidus. Radiographic studies of the brain showed a solid and cystic mass in the suprasellar region effacing the third ventricle. Intraoperatively, diffuse thickening of bilateral optic nerves and optic chiasma was noted and a diagnosis of optic glioma was considered. A biopsy of the mass was taken, which on histopathological examination showed features of ganglioglioma. The patient was referred for further radiotherapy but was lost to follow-up. Diabetes insipidus as a presenting symptom of ganglioglioma is extremely rare. This benign tumor should be kept in mind in patients with central diabetes insipidus and a suprasellar mass lesion. This report describes the second such case in the literature.

Hyperthyroidism: an unusual case presentation.

Science.gov (United States)

Scripture, D L

1998-02-01

Hyperthyroidism is the most common disorder of the thyroid. Patients typically present with complaints consistent with a hypermetabolic state, including nervousness, weight loss, heat intolerance, palpitations, irritability, and tremor. This case report reviews a 34-year-old woman who presented with unilateral upper extremity weakness, weight gain, and an episode of atrial fibrillation, the latter coinciding with a 36-hour lack of sleep and excess alcohol and caffeine intake. Although an extensive neurologic evaluation failed to identify any abnormality, the patient's laboratory analysis revealed elevations in thyroxine (T4) and triiodothyronine (T3) levels with unsuppressed thyroid-stimulating hormone levels. Subsequent treatment with the antithyroid drug methimazole (Tapazole) provided complete relief of symptoms. This case report illustrates how health care providers can be diverted to pursue a neurologic etiology when muscle weakness presents as a unilateral symptom. Plausible alternative causes for muscle weakness and other symptoms are presented.

[Metachromatic Leukodystrophy. Case Presentation].

Science.gov (United States)

Espejo, Lina María; de la Espriella, Ricardo; Hernández, José Fernando

Metachromatic leukodystrophy (MLD) is a rare demyelinating disease (prevalence 1:40 000), also called arylsulfatase A deficiency (ARS-A), which may present with neurological and psychiatric symptoms. Clinical assessment may be difficult, due to unspecific signs and symptoms. A case is presented of a 16 year-old female patient seen in psychiatry due to behavioural changes, psychosis, and with impaired overall performance. She was initially diagnosed with schizophrenia, but the Nuclear Magnetic Resonance (NMR) scan and laboratory tests lead to the diagnosis of MLD. Copyright © 2016 Asociación Colombiana de Psiquiatría. Publicado por Elsevier España. All rights reserved.

Uncommon presentation of neuro- cysticercosis

African Journals Online (AJOL)

Uncommon presentation of neuro- cysticercosis. Neurocysticercosis (NCC) is a common parasitic infection of the central nervous system caused by the pork tapeworm Taenia solium. The clinical presentation of NCC in children includes generalised and partial seizures with or without features of raised intracranial pressure.

Rhabdomyosarcoma presenting as acute leukemia.

Science.gov (United States)

Morandi, S; Manna, A; Sabattini, E; Porcellini, A

1996-08-01

We describe a case of a very unusual presentation of rhabdomyosarcoma. An 18-year-old woman presented with symptoms and signs compatible with acute leukemia. The bone marrow picture showed diffuse involvement sustained by undifferentiated blasts that turned out to be of striated muscle origin by immunochemistry. While it is well known that rhabdomyosarcoma may metastasize to the bone marrow, extensive marrow involvement with leukemic spread as a unique clinical manifestation is extremely rare. Our observation further confirms the need to consider rhabdomyosarcoma among the possible differential diagnoses in patients who present with a leukemic picture and atypical blasts lacking all hematopoietic markers.

2014 Water Power Program Peer Review: Hydropower Technologies, Compiled Presentations (Presentation)

Energy Technology Data Exchange (ETDEWEB)

2014-02-01

This document represents a collection of all presentations given during the EERE Wind and Water Power Program's 2014 Hydropower Peer Review. The purpose of the meeting was to evaluate DOE-funded hydropower and marine and hydrokinetic R&D projects for their contribution to the mission and goals of the Water Power Program and to assess progress made against stated objectives.

Retinoblastoma: a most unusual presentation

African Journals Online (AJOL)

Dr. C. Pedro-Egbe

Although leukocoria and strabismus are the most common presenting signs of retinoblastoma, a retrospective study by Abramson et al. identified 32 distinct presenting signs of retinoblastoma.6-8. A few other studies have also noted some distinct characteristics exhibited by children whose conditions were diagnosed in the ...

A Wedge of Time: Futures in the Present and Presents Without Futures in Maputo, Mozambique

DEFF Research Database (Denmark)

Nielsen, Morten

2014-01-01

its own inevitable collapse, thus making it crucial to maintain appropriate distance between the two. Whereas the imagined perspective from the future suggests an immediate readability of the present, the inverse temporal gaze (i.e. from the present) reflects the radical uncanniness of the future...

Unexpected effects of computer presented procedures

International Nuclear Information System (INIS)

Blackman, H.S.; Nelson, W.R.

1988-01-01

Results from experiments conducted at the Idaho National Engineering Laboratory have been presented regarding the computer presentation of procedural information. The results come from the experimental evaluation of an expert system which presented procedural instructions to be performed by a nuclear power plant operator. Lessons learned and implications from the study are discussed as well as design issues that should be considered to avoid some of the pitfalls in computer presented or selected procedures

Dexmedetomidine overdosage: An unusual presentation

Directory of Open Access Journals (Sweden)

Soumya S Nath

2013-01-01

Full Text Available We present a case of dexmedetomidine toxicity in a 3-year-old child. The case report describes the features and outlines the treatment strategy adopted. The child presented with bradypnoea, bradycardia, hypotension, deep hypnosis and miosis. He was successfully managed with oxygen, saline boluses and adrenaline infusion. He became haemodynamically stable with adrenaline infusion. He started responding to painful stimuli in 3 h and became oriented in 7 h. Dexmedetomidine, a selective α2 adrenoceptor agonist, is claimed to have a wide safety margin. This case report highlights the fact that dexmedetomidine administered in a toxic dose may be life-threatening may present with miosis and adrenaline infusion may be a useful supportive treatment.

The Design Space of Information Presentation

DEFF Research Database (Denmark)

May, Michael; Petersen, Johannes

2007-01-01

A semiotic approach to the design space of information presentation is presented in which Formal Concept Analysis (FCA) is used to represent and explore attributes of abstract sign types and the media (graphical, haptic, acoustic, gestic) through which they are presented as specific...

Automatic presentation generation for scholarly hypermedia

NARCIS (Netherlands)

S. Bocconi

2003-01-01

textabstractAutomatic hypermedia presentation generation uses an information source semantic network first to select the content and then to compose it in the presentation so that the semantic relations between the information items are conveyed to the user. A hypermedia presentation can be

Unexpected effects of computer presented procedures

International Nuclear Information System (INIS)

Blackman, H.S.; Nelson, W.R.

1988-01-01

Results from experiments conducted at the Idaho National Engineering Laboratory will be presented regarding the computer presentation of procedural information. The results come from the experimental evaluation of an expert system which presented procedural instructions to be performed by a nuclear power plant operator. Lessons learned and implications from the study will be discussed as well as design issues that should be considered to avoid some of the pitfalls in computer presented or selected procedures. 1 ref., 1 fig

Present status of processing method

Energy Technology Data Exchange (ETDEWEB)

Kosako, Kazuaki [Sumitomo Atomic Energy Industries Ltd., Tokyo (Japan)

1998-11-01

Present status of processing method for a high-energy nuclear data file was examined. The NJOY94 code is the only one available to the processing. In Japan, present processing used NJOY94 is orienting toward the production of traditional cross section library, because a high-energy transport code using a high-energy cross section library is indistinct. (author)

Castleman's disease, an unusual presentation

Directory of Open Access Journals (Sweden)

Shital Munde

2015-01-01

The present report describes a 30-year-old serving male who presented with pain abdomen and on and off episodes ofmelena, found to have peripancreatic lymphadenopathy on CECT abdomen. Ultrasound guided FN AC was done in private hospital revealed reactive lymphadenopathy. Excision biopsy was done in our institute and a diagnosis of Castleman's disease was made.

Atypical Presentations of Tularemia.

Science.gov (United States)

Odegaard, Karah; Boersma, Beth; Keegan, James

2017-05-01

Francisella tularensis is a gram-negative coccobacillus that causes a condition commonly referred to as tularemia. There has been a dramatic increase in tularemia cases reported in South Dakota, many of which were challenging to diagnose due to atypical clinical manifestations. We describe an interesting case of pneumonic tularemia and summarize six similar cases, several of which presented with lung nodules suggestive of malignancy. According to the literature, this is only the third outbreak of pneumonic tularemia reported in the U.S. We believe it is important for clinicians to be aware of the increased incidence of tularemia in the area and to be vigilant in the diagnosis and management of these atypically presenting cases. Copyright© South Dakota State Medical Association.

RTNS-II: present status

International Nuclear Information System (INIS)

Heikkinen, D.W.; Logan, C.M.

1980-10-01

The present status of the RTNS-II facility is described and typical operating parameters are given. A brief discussion is given of the methods used in production of the TiT 2 targets as well as their performance and tritium handling at RTNS-II. The various types of non-interactive beam diagnostics presently in use at the neutron sources are outlined. The on-line computer system which provides a time history of an irradiation and records target performance is described. Examples are listed of several representative experimental programs which have been carried out thus far at RTNS-II. These include both active and passive experiments. Finally, several of the major improvements to the facility made since the beginning of the experimental program are given

Selected Papers and Presentations Presented at MODSIM World 2010 Conference Expo. Part 2

Science.gov (United States)

Pinelli, Thomas E. (Editor)

2011-01-01

MODSIM World 2010 was held in Hampton, Virginia, October 13-15, 2010. The theme of the 2010 conference & expo was "21st Century Decision-Making: The Art of Modeling& Simulation". The conference program consisted of seven technical tracks - Defense, Engineering and Science, Health & Medicine, Homeland Security & First Responders, The Human Dimension, K-20 STEM Education, and Serious Games & Virtual Worlds. Selected papers and presentations from MODSIM World 2010 Conference & Expo are contained in this NASA Conference Publication (CP). Section 8.0 of this CP contains papers from MODSIM World 2009 Conference & Expo that were unavailable at the time of publication of NASA/CP-2010-216205 Selected Papers Presented at MODSIM World 2009 Conference and Expo, March 2010.

Reflections: Improving Medical Students' Presentation Skills.

Science.gov (United States)

Tarkowski, Radoslaw

2017-12-01

Both good communication and presentation skills on the part of an academic teacher are crucial when trying to generate students' interest in the subject of a lecture. More generally, our task is to share knowledge in the most effective way possible. It is also worth teaching students presentation skills, as today's students are tomorrow's teachers. An engaging presentation is a powerful tool. There are some rules for presenting which I consider worthy of being discussed and taught at a medical university.

Emergency Department Referrals for Adolescent Urgent Psychiatric Consultation: Comparison of Clinical Characteristics of Repeat-presentations and Single-presentation.

Science.gov (United States)

Roberts, Nasreen; Nesdole, Robert; Hu, Tina

2018-01-01

a) to examine the demographic and clinical characteristics of repeat-presentations to an adolescent urgent psychiatric clinic, and b) to compare them with single-time presentation. This 18-month retrospective study compared repeat-presenters to age and gender matched single-time presenters. Demographic variables included age gender and ethnicity. Clinical variables included reason for referral, family history, diagnosis, recommendations and compliance. Data were analyzed using descriptive statistics, McNemar's Chi-square tests for matched pairs, and conditional logistic regression. Of 624 assessments 24% (N=151) were repeat-presentations. Compared with single-presentation, repeat-presentation group had a higher proportion of Aboriginal youth (X2 (1) = 108.28 p presentation group had higher odds of past hospital admission (OR: 3.50, p presentations for urgent psychiatric consultation constitute a quarter of referrals to the urgent psychiatric clinic. Identifying and addressing factors that contribute to repeat-presentations may, assist in improving treatment compliance by ensuring focused interventions and service delivery for these youth. In turn, this will improve access to the limited urgent services for other youth.

Clinical presentation of adult coeliac disease.

LENUS (Irish Health Repository)

Tajuddin, T

2012-02-01

The mode of presentation of coeliac disease has been changing to more atypical or silent disease. Few studies described the clinical presentation of adult coeliac disease in Ireland in recent years. We retrospectively collected the clinical data for all patients who had a diagnosis of coeliac disease made in our centre between January 07 and December 08. Forty seven adults, predominantly females (n = 30), had a confirmed diagnosis of coeliac disease made during the study period. In our patient cohort, the presenting symptom was diarrhoea in 19 (40%) patients, while 16 patients (34%) did not have any G.I. symptoms, 10 (21%) presented with anaemia. Females presented at a significantly younger age compared to males, with median ages at diagnosis of 44.5 and 57 years, respectively (p = 0.04). Females also presented more commonly with non G.I. symptoms (p = 0.07). The reasons behind this gender difference need further study.

Sinonasal anatomical variations: their relationship with chronic rhinosinusitis and effect on the severity of disease-a computerized tomography assisted anatomical and clinical study.

Science.gov (United States)

Kaygusuz, Ahmet; Haksever, Mehmet; Akduman, Davut; Aslan, Sündüs; Sayar, Zeynep

2014-09-01

The anatomy of the sinonasal area has a very wide rage of anatomical variations. The significance of these anatomical variations in pathogenesis of rhinosinusitis, which is the commonest disease in the region, is still unclear. The aims of the study were to compare the rate of sinonasal anatomical variations with development and severity of chronic rhinosinusitis patients. CT scan of paranasal sinuses images of 99 individuals were retrospectively reviewed. 65 cases of chronic rhinosinusitis (study group) who had undergone endoscopic sinus surgery were compared with 34 cases without chronic rhinosinusitis (control group). Also in study group Lund-Mackay score of the sinus disease were calculated and compared to the rate of related anatomical variations. There were 74 (74.7 %) males and 25 (25.2 %) females with ages ranging from 13 to 70 years (mean 32.2 years). The anatomical variations recorded were: Septal deviation 47 (72.3) in study and 25 (73.5 %) in control group, concha bullosa 27 (41.5 %) in study and 18 (52.9 %) in control group, overpneumatized ethmoid bulla 17 (26.1 %) in study and 14 (41.1 %) in control group, pneumatized uncinate 3 (4.6 %) in study and 3 (8.8 %) in control group, agger nasi 42 (64.6 %) in study and 19 (55.8 %) in control group, paradoxical middle turbinates 9 (13.8 %) in study and 4 (11.7 %) in control group, Onodi cell 6 (9.2 %) in study and 2 (5.8 %) in control group, Haller's cells (infraorbital ethmoid cell) 9 (13.8 %) in study and 7 (20.5 %) in control group. None of these results were statistically significant between study and control group (p > 0.05). Lund-Mackay score (which was assumed to show the severity of the disease) of the maxillary, ethmoid and frontal sinus were calculated and compared to rate of septal deviation, concha bullosa, agger nasi cells. No significant correlation was conducted (p > 0.05). The results of study showed no statistically significant correlation between sinonasal anatomical

Quantum technology past, present, future: quantum energetics (Conference Presentation)

Science.gov (United States)

Choi, Sang H.

2017-04-01

Since the development of quantum physics in the early part of the 1900s, this field of study has made remarkable contributions to our civilization. Some of these advances include lasers, light-emitting diodes (LED), sensors, spectroscopy, quantum dots, quantum gravity and quantum entanglements. In 1998, the NASA Langley Research Center established a quantum technology committee to monitor the progress in this area and initiated research to determine the potential of quantum technology for future NASA missions. The areas of interest in quantum technology at NASA included fundamental quantum-optics materials associated with quantum dots and quantum wells, device-oriented photonic crystals, smart optics, quantum conductors, quantum information and computing, teleportation theorem, and quantum energetics. A brief review of the work performed, the progress made in advancing these technologies, and the potential NASA applications of quantum technology will be presented.

Twelve Tips for Effective Electronic Presentation.

Science.gov (United States)

Crosby, Joy

1994-01-01

Offers 12 tips for effective electronic presentation. This article is intended for readers who may be considering using electronic presentation for the first time. Offers reasons for its popularity and occasions when it may be used. The tips offer assistance in the design and presentation of electronic material. (LZ)

Visual communication in presentation on physics

Science.gov (United States)

Grebenyuk, Konstantin A.

2005-06-01

It is essential that our audience be attentive during lecture, report or another presentation on physics. Therefore we have to take care of both speech and visual communication with audience. Three important aspects of successful visual aids use are singled out in this paper. The main idea is that physicists could appreciably increase efficiency of their presentations by use of these simple principles of presentation art. Recommendations offered are results of special literature research, author' s observations and experience of communication with skilled masters of presentations.

The breech presentation and the vertex presentation following an external version represent risk factors for neonatal hip instability.

Science.gov (United States)

Andersson, J E; Odén, A

2001-08-01

The aim of this study was to evaluate the frequency and type of hip-joint instability and the frequency of hip dislocation requiring treatment in neonates who had been lying in the breech presentation and were delivered vaginally after an external version or by caesarean section, and to compare them with neonates who were naturally in the vertex presentation. Breech presentations without ongoing labour were subjected to an attempted external version and, in cases where this proved unsuccessful or where labour had started, to deliver by caesarean section. None of the breech presentations was vaginally delivered. The anterior-dynamic ultrasound method was used to assess the hip-joint status of the neonates. Out of 6,571 foetuses, 257 were in breech presentation after 36 wk of pregnancy. Sixty-two were vaginally delivered following an external version to vertex presentation and 195 were delivered by caesarean section, 75 of these following unsuccessful attempts to perform a version. Treatment for congenital hip-joint dislocation was performed on 0.2%. Out of the breech presentations, 1.0% of those delivered by caesarean section were treated, while in those with vaginal delivery following an external version the treatment frequency was 3.2%. No case of late diagnosed hip dislocation was recorded. Significant differences in frequency of hip-joint instability and treatment were found between (i) neonates delivered in breech presentation and those delivered with vertex presentation, (ii) infants delivered in vertex presentation, naturally or after successful version, and (iii) those delivered by caesarean section with or without attempted external version and those delivered with vortex presentation. Breech presentation predisposes to increased hip instability. The instability is present prior to delivery and is certainly not a primary result of delivery forces. Both breech and vertex presentations following an external or spontaneous version should be considered as risk

Peripartum Cardiomyopathy Presenting as Bradycardia

OpenAIRE

Codsi, Elisabeth; Rose, Carl H.; Tweet, Marysia S.; Hayes, Sharonne N.; Best, Patricia J. M.; Blauwet, Lori A.

2017-01-01

Peripartum cardiomyopathy (PPCM) is a disease that typically affects young otherwise healthy women. As PPCM is associated with significant mortality, timely diagnosis is necessary to ensure appropriate care. To our knowledge, this represents the first reported case of PPCM presenting as symptomatic bradycardia. We describe the patient’s clinical presentation and relevant findings and review the potential etiology and ramifications of bradycardia in patients with PPCM.

PRESENT STATUS IN INDIA

Indian Academy of Sciences (India)

First page Back Continue Last page Overview Graphics. PRESENT STATUS IN INDIA. FIXED LINES – 36 MILLION. MOBILE CONNECTIONS – 14 MILLION. TELEDENSITY APPROXIMATELY 5. INTERNET CONNECTIONS – 5 MILLION. INTERNET USERS NEARLY – 25 MILLION.

Intuitionistic Assessment Of Behavioural Present Value*

OpenAIRE

Piasecki Krzysztof

2014-01-01

The article discussesd the impact of chosen behavioural factors on the imprecision of present value assessment. The formal model of behavioural present value is offered as a result of this discussion. The behavioural present value is described here as an intuitionistic fuzzy set. The significance of the replacement of a fuzzy set by an intuitionistic fuzzy set is proved.

Intracranial Castleman's disease presenting as hypopituitarism

International Nuclear Information System (INIS)

Ribeiro, L.T.; Simao, G.N.; Matos, A.L.M.; Santos, Antonio Carlos; Carlotti, C.G. Jr.; Colli, B.O.; Neder, L.; Ribeiro-Silva, A.; Castro, M. de; Rego, E.

2004-01-01

Castleman's disease is an atypical lymphoproliferative disorder that may present as a localized or multicentric form. The involvement of the central nervous system is rare. We describe here a case of Castleman's disease with involvement of the hypothalamus and meninges, presenting as hypopituitarism. Radiological and clinical pathological features are emphasized and a review of the literature is presented. (orig.)

A rare presentation of hydatid cyst

International Nuclear Information System (INIS)

Sabir, N.; Yildirim, B.; Alatas, E.; Cetin, B.

2005-01-01

Hydatid disease, although known to occur in most body areas, is extremely rare in the female reproductive system. There are different modes of presentation for the disease; however, we report and discuss a case presented with cystic vesicles passing through the vagina, which is considered as a rare presentation for secondary involvement of the uterus and both ovaries. We confirmed diagnosis with radiological examinations and serological tests. We operated on the patient, and studied the excised cysts microscopically. The gynecologist should be aware of hydatid cyst when vaginally passing a grape like vesicle is presented by the patient. (author)

AN UNUSUAL PRESENTATION OF DIC

Directory of Open Access Journals (Sweden)

Manish

2015-10-01

Full Text Available Placenta Praevia is a common obstetric problem which presents with antepartum H aemorrhage and is treated by the obstetrician. It is very rare to see a patient with placenta praevia treated in medical ward for post - partum H aemorrhage due to disseminated intravascular coagulation defect with the added complication of acute kidney injury, P ost - partum H ypertension and PRES (posterior reversible encephalopathy syndrome, hence the presentation

The presentation and treatment

African Journals Online (AJOL)

user

tion of single collocations proposals are made for an implicit presentation of complex ... function lexicographers often include items to illustrate the typical use of the ... they have no items explicitly addressed at them to elevate them to treatment.

Fostering oral presentation performance

NARCIS (Netherlands)

Ginkel, van Stan; Gulikers, Judith; Biemans, Harm; Mulder, Martin

2017-01-01

Previous research revealed significant differences in the effectiveness of various feedback sources for encouraging students’ oral presentation performance. While former studies emphasised the superiority of teacher feedback, it remains unclear whether the quality of feedback actually differs

Fibrolamellar Hepatocellular Carcinoma Presenting as Obstructive Jaundice: Uncommon Presentation of a Rare Entity

International Nuclear Information System (INIS)

Arora, Richa

2015-01-01

Fibrolamellar hepatocellular carcinoma is a rare primary malignant liver tumor, significantly different from generic hepatocellular carcinoma with distinct demographics, risk factors, imaging features, histopathology and prognosis. Unlike conventional hepatocellular carcinoma, it presents in young individuals with no preexisting hepatitis or cirrhosis and does not cause elevation of serum alpha feto proteins in most cases. This paper presents a case report of this rare tumor in a young female with an unusual clinical manifestation of obstructive jaundice (which has not been reported so far) along with a review of its imaging and pathological features, with treatment options. Fibrolamellar HCC is a rare variant of classic HCC with different epidemiology, risk factors, clinical manifestations, radiological, pathological and prognostic features. Therefore, it is important to be familiar with the entity for its early diagnosis and management

Going beyond methodological presentism

DEFF Research Database (Denmark)

Schmidt, Garbi

2017-01-01

Denmark is an example of a country where the idea of historical ethnic homogeneity stands strong. This article challenges this historical presentism: the scholarly and societal tendency to understand social phenomena within a limited contemporary framework, neglecting possible effects and similar...

Present and future superconducting cyclotrons

International Nuclear Information System (INIS)

Nolen, J.A. Jr.

1987-01-01

This paper begins with a brief review of the status of present superconducting (SC) cyclotron projects, including the two which are currently operating and the six which are under construction. The next section summarizes the main features shared by five of these machines, while the third section presents recent developments and new concepts introduced in the other three ''second generation'' SC cyclotrons. Projects in early stages of development are discussed in the fourth section

Squamous cell carcinoma presenting with trigeminal anesthesia: An uncommon presentation of head & neck cancer with unknown primary.

Science.gov (United States)

Shah, Ameer T; Dagher, Walid I; O'Leary, Miriam A; Wein, Richard O

The differential diagnosis of facial anesthesia is vast. This may be secondary to trauma, neoplasm, both intracranial and extracranial, infection, and neurologic disease. When evaluating a patient with isolated facial anesthesia, the head and neck surgeon often thinks of adenoid cystic carcinoma, which has a propensity for perineural invasion and spread. When one thinks of head and neck squamous cell carcinoma with or without unknown primary, the typical presentation involves dysphagia, odynophagia, weight loss, hoarseness, or more commonly, a neck mass. Squamous cell carcinoma presenting as facial anesthesia and perineural spread, with no primary site is quite rare. Case presentations and review of the literature. Trigeminal anesthesia is an uncommon presentation of head and neck squamous cell carcinoma with unknown primary. We present two interesting cases of invasive squamous cell carcinoma of the trigeminal nerve, with no primary site identified. We will also review the literature of head and neck malignancies with perineural spread and the management techniques for the two different cases presented. Copyright © 2016 Elsevier Inc. All rights reserved.

Pulmonary strongyloidiasis. Presentation of two cases

International Nuclear Information System (INIS)

Munive, Abraham Ali; Torres D, Carlos A Lasso A Javier J; Ojeda Leon, Paulina; Acosta R, Nohora

2002-01-01

We describe two case reports of immune-suppressed patients receiving oral steroids and who presented lung Strongyloidiasis; later they evolved toward respiratory failure, with different clinical courses. One developed severe hypoxaemia, hemodynamic instability and death. The worst prognosis in this patient was determined by diffuse infiltrates and the resulting lung injury. The other one presented a stable clinical course and evolved to full recovery; this case presented a cavern in the chest X-ray that could represent a preliminary phase for lung extension of the infection. The difference in the evolution of these two patients is determined by the different presentation of the lung damage

The efficient presentation - obstacles, strategies, success

Directory of Open Access Journals (Sweden)

Daniela-Elena Radu

2007-12-01

Full Text Available The making of presentations has become an omnipresent act of communication in the companies practice, but also in the didactic one. The notoriety and the increase of the importance of an efficient presentation lie in the transformations felt by a society of knowledge, communication being the answer in front of a constant information wave.Business people look in fear at the preparation of a presentation, the interaction with the audience andtheir feedback, all these leading to a low efficiency in transmitting the message.The speaker must overcome a group of obstacles, the most important being the reduced human capacityto capture the information transmitted orally; in this respect, he has available a group of visual instruments and strategies to capture the attention. In order to achieve efficiency one needs mental and emotional preparation, knowing the audience and the presentation’s objective and eliminating the old listing format in PowerPoint, for focusing the communication of the audience’s necessities. The present essay aims to analyze the verbal, non-verbal and para-verbal communication act that constitutes the base of any presentation, the reasons that lead to failure and the ways to increase efficiency, in order to succeed in transmitting a well structured and easy to remember message.Many researchers and practice workers within the communication, economic but also IT fields, have tried to find the solution for organizing and transmitting the message during a presentation, in accordance with the new technologies. In elaborating the present paper, we have used our personal experience and data obtained through 30 in-depth interviews,with managers in companies such Bostina Asociatii Skoda, Petrom etc.; we have used in our research articles of honored professors in social sciences from the Harvard and Stanford Universities , researches of the Minnesota and Ulster Universities, as well as works of specialists in marketing and information

Creating Meaningful Multimedia Presentations

NARCIS (Netherlands)

L. Hardman (Lynda); J.R. van Ossenbruggen (Jacco)

2006-01-01

textabstractFinding relevant information is one step in the chain of understanding information. Presenting material to a user in a suitable way is a further step. Our research focuses on using semantic annotations of multimedia elements to increase the ”presentability” of

Sirenomelia: A Rare Presentation

Science.gov (United States)

Srinivas, S; Kumar, Shiva; Reddy, Surweshwar; Prasad, Hari; Irfan, G M

2012-01-01

We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed. PMID:26023366

Sirenomelia: a rare presentation.

Science.gov (United States)

Reddy, K Ramesh; Srinivas, S; Kumar, Shiva; Reddy, Surweshwar; Prasad, Hari; Irfan, G M

2012-01-01

We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed.

Organising and presenting information.

Science.gov (United States)

Kankanady, Raghavendra; Wells, Marilyn

2013-01-01

Information management can be a daunting process for clinicians, health care providers and policy makers within the health care industry. This chapter discusses the importance of information classification and information architecture in the information economy and specific challenges faced within the health care industry. The healthcare sector has industry specific requirements for information management, standards and specifications for information presentation. Classification of information based on information criticality and the value in the health care industry is discussed in this paper. Presentation of information with reference to eHealth standards and specifications for healthcare information systems and their key requirements are also discussed, as are information architecture for eHealth implementation in Australia. This chapter also touches on information management and clinical governance since the importance of information governance is discussed by various researchers and how this is becoming of value to healthcare information management.

ECRIS 2010 - Proceedings and Presentations

International Nuclear Information System (INIS)

2012-01-01

The workshop focussed on the latest developments in performance, modeling and applications of ECR ion sources along with the associated physics and technologies, including plasma physics, beam optics, beam diagnostics, magnet technology.... The presentations were organized around 8 topics: 1) status of ECRIS operation, 2) new developments, 3) ion beams and low energy beam transport (LEBT), 4) pulsed operation, 5) applications, and 6) plasma and beam investigations. Most papers are accompanied with the slides of their presentation

Unusual presentation of cutaneous leiomyoma

Directory of Open Access Journals (Sweden)

Sapnashree Bhaskar

2014-01-01

Full Text Available Herein, we report a case of leiomyoma cutis because of its rarity and unusual presentation. The case presented with a solitary leiomyoma lesion which was painless. However, the adjacent normal appearing area was tender. A biopsy of the lesion as well as of a portion of the adjacent normal appearing area was taken, which confirmed the diagnosis of cutaneous leiomyoma. This may suggest the dormant nature of the disease which has not yet become apparent.

Processing of visually presented clock times.

Science.gov (United States)

Goolkasian, P; Park, D C

1980-11-01

The encoding and representation of visually presented clock times was investigated in three experiments utilizing a comparative judgment task. Experiment 1 explored the effects of comparing times presented in different formats (clock face, digit, or word), and Experiment 2 examined angular distance effects created by varying positions of the hands on clock faces. In Experiment 3, encoding and processing differences between clock faces and digitally presented times were directly measured. Same/different reactions to digitally presented times were faster than to times presented on a clock face, and this format effect was found to be a result of differences in processing that occurred after encoding. Angular separation also had a limited effect on processing. The findings are interpreted within the framework of theories that refer to the importance of representational codes. The applicability to the data of Bank's semantic-coding theory, Paivio's dual-coding theory, and the levels-of-processing view of memory are discussed.

Presentation of the comparison Negawatt - Negatep; Presentation de la comparaison Negawatt - Negatep

Energy Technology Data Exchange (ETDEWEB)

Acket, C.; Bacher, P

2007-02-15

France aims to divide by four the CO{sub 2} emissions for 2050. Many scenario have been proposed to reach this objective. Among them, the scenario Negatep proposed by the SLC and the scenario Negawatt proposed the association Negawatt. This document is a presentation and a comparison of these scenario with a sectoral analysis of the energy consumption. (A.L.B.)

Hera presentation generator (Poster)

NARCIS (Netherlands)

Frasincar, F.; Houben, G.J.P.M.; Barna, P.; Ellis, A.; Hagino, T.

2005-01-01

Semantic Web Information Systems (SWIS) are Web Information Systems that use Semantic Web technologies. Hera is a model-driven design methodology for SWIS. In Hera, models are represented in RDFS and model instances in RDF. The Hera Presentation Generator (HPG) is an integrated development

Analyzing in the present

DEFF Research Database (Denmark)

Revsbæk, Line; Tanggaard, Lene

2015-01-01

The article presents a notion of “analyzing in the present” as a source of inspiration in analyzing qualitative research materials. The term emerged from extensive listening to interview recordings during everyday commuting to university campus. Paying attention to the way different parts of vari...

Clinical Presentations of Acute Leukemia

International Nuclear Information System (INIS)

Shahab, F.; Raziq, F.

2014-01-01

Objective: To document the clinical presentation and epidemiology of various types of acute leukemia with their respective referral source at a tertiary level centre in Peshawar. Study Design: An observational study. Place and Duration of Study: Department of Pathology, Hayatabad Medical Complex (HMC), Peshawar, from January 2011 to May 2012. Methodology: A total of 618 bone marrow biopsy reports were reviewed. All biopsy reports labeled as acute leukemia were reviewed for age, gender, address, referring unit, diagnosis on bone marrow examination, presenting complaints, duration of illness and findings of clinical examination. Results: Ninety-two patients were diagnosed as suffering from acute leukemias (15%). ALL was most prevalent (46%), followed by AML (38%) and undifferentiated acute leukemia (16%). Males were affected more compared to females (60% vs. 40%). ALL and AML were predominant in pediatric (64%) and adults (77%) patients respectively. Patients from Afghanistan accounted for 33% of all cases followed by Peshawar (14%). Fever (77%), pallor (33%) and bleeding disorders (23%) were the main presenting complaints. Enlargement of liver, spleen and lymph nodes together was associated with ALL compared with AML (p = 0.004). Conclusion: ALL-L1 and AML-M4 were the most common sub-types. Fever, pallor and bleeding disorders were the main presenting complaints. Enlargement of liver, spleen and lymph nodes was more frequently associated with ALL compared to AML. (author)

Idiopathic intracranial hypertension: A typical presentation

International Nuclear Information System (INIS)

Algahtani, Hussein A.; Obeid, Tahir H.; Abuzinadah, Ahmad R.; Baeesa, Saleh S.

2007-01-01

Objective was to describe the clinical features of 5 patients with rare atypical presentation of idiopathic intracranial hypertension (IIH), and propose the possible mechanism of this atypical presentation. We carried out a retrospective study of 5 patients, admitted at King Khalid National Guard Hospital, Jeddah, Kingdom of Saudi Arabia with IIH during the period from January 2001 to December 2005. All were females with their age ranges from 24 to 40 years. The clinical presentations, the laboratory and imaging studies were analyzed. The opening pressures of the lumbar puncture tests were documented. All patients were presented with headache. One had typical pain of trigeminal neuralgia and one with neck pain and radiculopathy. Facial diplegia was present in one patient and two patients had bilateral 6th cranial neuropathy. Papilledema was present in all patients except in one patient. Imaging study was normal in all patients, and they had a very high opening pressure during lumbar puncture, except in one patient. All patients achieved full recovery with medical therapy in 6 to 12 weeks with no relapse during the mean follow up of 2 years. Atypical finding in IIH are rare and require a high index of suspicion for early diagnosis. (author)

Subacute sclerosing panencephalitis presenting as mania

Directory of Open Access Journals (Sweden)

Aggarwal Ashish

2011-01-01

Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

Presenting Newtonian gravitation

International Nuclear Information System (INIS)

Counihan, Martin

2007-01-01

The basic principles of the Newtonian theory of gravitation are presented in a way which students may find more logically coherent, mathematically accessible and physically interesting than other approaches. After giving relatively simple derivations of the circular hodograph and the elliptical orbit from the inverse-square law, the concept of gravitational energy is developed from vector calculus. It is argued that the energy density of a gravitational field may reasonably be regarded as -g 2 /8πG, and that the inverse-square law may be replaced by a Schwarzschild-like force law without the need to invoke non-Euclidean geometry

Keynote presentation : SAR systems

NARCIS (Netherlands)

Halsema, D. van; Otten, M.P.G.; Maas, A.P.M.; Bolt, R.J.; Anitori, L.

2011-01-01

Synthetic Aperture Radar (SAR) systems are becoming increasingly important sensors in as well the military environment as in the civilian market. In this keynote presentation an overview will be given over more than 2 decades of SAR system∼ and SAR application development at TNO in the Netherlands.

Neonatal hemophilia: a rare presentation

Directory of Open Access Journals (Sweden)

Nuno Ferreira

2015-12-01

Full Text Available Hemophilia A is a X-linked hereditary condition that lead to decreased factor VIII activity, occurs mainly in males. Decreased factor VIII activity leads to increased risk of bleeding events. During neonatal period, diagnosis is made after post-partum bleeding complication or unexpected bleeding after medical procedures. Subgaleal hemorrhage during neonatal period is a rare, severe extracranial bleeding with high mortality and usually related to traumatic labor or coagulation disorders. Subgaleal hemorrhage complications result from massive bleeding. We present a neonate with unremarkable family history and uneventful pregnancy with a vaginal delivery with no instrumentation, presenting with severe subgaleal bleeding at 52 hours of life. Aggressive support measures were implemented and bleeding managed. The unexpected bleeding lead to a coagulation study and the diagnosis of severe hemophilia A. There were no known sequelae. This case shows a rare hemophilia presentation reflecting the importance of coagulation studies when faced with unexplained severe bleeding.

XPL the Extensible Presentation Language

Directory of Open Access Journals (Sweden)

A. Santangelo

2009-01-01

Full Text Available The last decade has witnessed a growing interest in the development of web interfaces enabling both multiple ways to access contents and, at the same time, fruition by multiple modalities of interaction (point-and-click, contents reading, voice commands, gestures, etc.. In this paper we describe a framework aimed at streamlining the design process of multi-channel, multimodal interfaces enabling full reuse of software components. This framework is called the eXtensible Presentation architecture and Language (XPL, a presentation language based on design pattern paradigm that keeps separated the presentation layer from the underlying programming logic. The language supplies a methodology to expedite multimodal interface development and to reduce the effort to implement interfaces for multiple access devices, by means of using the same code. This paper describes a methodology approach based on Visual Design Pattern (ViDP and Verbal Design Pattern (VeDP, offering examples of multimodal and multichannel interfaces created with the XPL Editor.

Presentations and complications of diabetes patients presenting to diabetic clinic of Eastern Nepal

Directory of Open Access Journals (Sweden)

Nabin Kumar Singh

2014-04-01

Full Text Available Objective Diabetes mellitus leads to damage, dysfunction and failure of various organs especially eyes, kidneys, nerves and heart. The latency of occurence of hyperglycemia and diagnosis may be of long duration. This study was aimed to find out the mode of presetation of diabetes mellitus in diabetic patients attending out patients clinic of B P Koirala Institute of Health Sciences. Associated complications and comorbid condition present at the time of presenation were also studied. Methods The diabetic patients attending the Diabetic Out Patient Clinic of B P Koirala Institute of Health Sciences during June 2006 to June 2007 were included in this study.The patients details were collected from the predefined Proforma for diabetes patient from the database. This included demographic data, biochemical parameters and diabetic complications. For the purpose of study a total of 775 patients were randomly selected.The Data collected were entered and analysed using excel and SPSS(version 11.5 Results Out of 775 cases 436 (56.3% were male and 339(43.7% were female. Majority of patients 81.55% (n=632 had osmotic sympmtoms or symptoms related to complication of diabetes at the time of presentation to the clinic. Asymptomatic patient constituted 18.45% (n=143. The most common presenting complaints were polyuria (44.58%, followed by polydypsia (39.62% and polyphagia (24.88%. About 54.97% (n=426 had symptoms of complications related to diabetes. Among them most common complication was neurological (39.67%, followed by renal (10.8% metabolic (4.93%, cardiac(4.46%, autonomic neuropathy (4.93% and peripheral vascular disease (3.99%. Conclusion Majority of the patients presenting in our OPD had osmotic symptoms or symptoms related to complication of diabetes. Access to diabetes care and lack of awareness of the disease and its complication might had contributed to this. Community awareness, program for early detection and managemnet may help proper diabetes care

Disseminated gonococcal infection: an unusual presentation

Directory of Open Access Journals (Sweden)

Saroj Lohani

2016-07-01

Full Text Available Gonococcus typically affects the mucosal surfaces but in rare cases can spread to bloodstream causing disseminated gonococcal infection (DGI. The usual presentation of DGI is rash, polyarthralgia, and tenosynovitis. We present the case of a 23-year-old female who presented to our hospital with pustular rash and tenosynovitis of hand and was sent home on Augmentin. Her symptoms worsened despite treatment and she presented back to the ED. On investigation, she was found to have DGI and was appropriately treated. DGI should be kept in mind in sexually active youngsters who have only one or two features of the classic triad of rash, tenosynovitis, and arthritis. Timely management and appropriate treatment of DGI is very important to avoid complications and morbidity.

Atypical presentations of retroperitoneal giant schwannomas

Directory of Open Access Journals (Sweden)

Sait Ozbir

2011-06-01

Full Text Available Schwannomas are usually benign rare tumors that originating from Schwann cells of peripheral nerve sheaths. Presentation is generally varied and changed in a non-specific range from abdominal mass, flank pain to incidental findings. Herein we report 2 cases of retroperitoneal giant schwannomas with different clinical presentations, of whom one presented with vague abdominal pain, palpable abdominal mass for 4 years, swelling and bilateral hydronephrosis that caused by giant abdominal mass; the other one presented with right flank pain, rectal hemorrhage and lower extremities edema. Two patients were treated by complete surgical excision of masses. The histological and immunohistochemical diagnosis was reported as benign schwannoma. Both of patients are doing well and had no recurrence in 9 years and 28 months follow-up, respectively.

Radiocarbon calibration - past, present and future

International Nuclear Information System (INIS)

Plicht, J. van der

2004-01-01

Calibration of the Radiocarbon timescale is traditionally based on tree-rings dated by dendrochronology. At present, the tree-ring curve dates back to about 9900 BC. Beyond this limit, marine datasets extend the present calibration curve INTCAL98 to about 15 600 years ago. Since 1998, a wealth of AMS measurements became available, covering the complete 14 C dating range. No calibration curve can presently be recommended for the older part of the dating range until discrepancies are resolved

Promoting professional development through poster presentations.

Science.gov (United States)

Durkin, Gregory

2011-01-01

Poster presentations are commonplace at regional and national nursing conferences, although the development of a poster remains an intimidating task for many staff nurses. The author describes the staff development department's role in implementing in-house poster presentation sessions. Nursing staff are provided support and assistance in presenting posters to their colleagues at yearly sessions. The result has been increased comfort and experience with poster creation, participation in professional development activities, and dissemination of nursing practice innovation.

Rare presentation of a common disease: Idiopathic hypoparathyroidism presenting with extrapyramidal symptoms and status epilepticus

Directory of Open Access Journals (Sweden)

Kaushik Ghosh

2012-01-01

Full Text Available We report of an 18-year-old male who presented with an epileptiform disorder, features of hypocalcemia, and an extrapyramidal symptom in the form of choreoathetosis. On evaluation he had idiopathic hypoparathyroidism with extensive calcifications in the extrapyramidal system of the brain; basal ganglion, as well as in the cerebral cortex and cerebellum, which is a rare entity. We report the rare presentation of a common disorder, which requires to be considered in evaluating hypoparathyroidism.

Imagined futures, present lives

DEFF Research Database (Denmark)

Dalsgaard, Anne Line; Wildermuth, Norbert

2006-01-01

is discussed centrally in this attempt to contribute to an empirically grounded understanding of the role that media play for youth in their striving to ‘find a place in life'. In the empirical context presented in the article, imaginations, expanded and circulated by a globalized media circuit...

Radiocarbon calibration - past, present and future

Energy Technology Data Exchange (ETDEWEB)

Plicht, J. van der E-mail: plicht@phys.rug.nl

2004-08-01

Calibration of the Radiocarbon timescale is traditionally based on tree-rings dated by dendrochronology. At present, the tree-ring curve dates back to about 9900 BC. Beyond this limit, marine datasets extend the present calibration curve INTCAL98 to about 15 600 years ago. Since 1998, a wealth of AMS measurements became available, covering the complete {sup 14}C dating range. No calibration curve can presently be recommended for the older part of the dating range until discrepancies are resolved.

Lichen planus pigmentosus: two atypical presentation

Directory of Open Access Journals (Sweden)

Falguni Nag

2013-01-01

Full Text Available Lichen planus pigmentosus (LPP is a chronic pigmentary disorder with variable pattern of presentation. We here by present two cases of LPP one with parallel band like pigmentation over abdomen sparing the abdominal skin creases and other with parallel band like pattern following the Blaschko’s lines over left side of the abdomen. Our cases are unique not only for its presentation but also for the pattern of distribution and LPP should be the differential diagnosis in any pigmentary disorders.

New hazards in paediatric poisoning presentations.

LENUS (Irish Health Repository)

Moore, C

2015-02-01

Accidental ingestion is an important preventable cause of childhood morbidity. All accidental ingestion presentations (n = 478) to a tertiary paediatric ED from January 2010 to December 2011 were analysed. These results were compared with a similar study in the same institution ten years previously in 2001 and showed that while accidental ingestions constituted a higher proportion of presentations (0.5% in this study v 0.45% in 2001), fewer had investigations performed (21% v 35%) and fewer were admitted (7% v 20%). Accidental ingestions account for 0.5% of presentations and are an important focus of home safety information for parents and guardians. Paracetamol (n = 67, 14%) and liquid detergent capsules (n = 44, 9.2%) were the two most common substances implicated in these presentations, and have the potential to cause severe morbidity and mortality.

Selected Papers and Presentations Presented at MODSIM World 2010 Conference and Expo. Part 1

Science.gov (United States)

Pinelli, Thomas E. (Editor)

2011-01-01

MODSIM World 2010 was held in Hampton, Virginia, October 13-15, 2010. The theme of the 2010 conference & expo was "21st Century Decision-Making: The Art of Modeling& Simulation". The conference program consisted of seven technical tracks - Defense, Engineering and Science, Health & Medicine, Homeland Security & First Responders, The Human Dimension, K-20 STEM Education, and Serious Games & Virtual Worlds. Selected papers and presentations from MODSIM World 2010 Conference & Expo are contained in this NASA Conference Publication (CP). Section 8.0 of this CP contains papers from MODSIM World 2009 Conference & Expo that were unavailable at the time of publication of NASA/CP-2010-216205 Selected Papers Presented at MODSIM World 2009 Conference and Expo, March 2010.

5 CFR 847.602 - Present value factors.

Science.gov (United States)

2010-01-01

... 5 Administrative Personnel 2 2010-01-01 2010-01-01 false Present value factors. 847.602 Section... INSTRUMENTALITIES Additional Employee Costs Under the Retroactive Provisions § 847.602 Present value factors. (a... present value factors for all CSRS annuities; (2) One table of present value factors for FERS annuities...

 Papillary Cystadenocarcinoma of Ovary Presenting as Obstructive Jaundice: A Rare Presentation

Directory of Open Access Journals (Sweden)

Sunil Kumar

2012-03-01

Full Text Available  Obstructive jaundice resulting from malignancy of gastrointestinal tract and hepatobiliary tract has been reported in various studies. Ovarian malignancy leading to obstructive jaundice due to portal lymphadenopathy is of rare occurrence. We report a case presented with obstructive jaundice and on further evaluation, found to have ovarian papillary cyst adenocarcinoma with secondaries at porta hepatis which was managed successfully by neoadjuvant chemotherapy followed by cytoreductive surgery.

Summary of presentations and discussions

International Nuclear Information System (INIS)

Takeuchi, Mitsuo

2008-01-01

In December 2007, the Forum on Stakeholder Confidence discussed its theme entitled 'Link between research, development and demonstration (RD and D) and stakeholder confidence'. It was remarked that regulators need a technical demonstration to aid in evaluating the safety case. Local stakeholders appreciate the opportunity to visualise technological arrangements. In both cases, demonstration adds to confidence in the feasibility of solutions. Some believe there is an important role for analogues in communication with stakeholders, if handled with integrity. To explore and benchmark current practices, it was decided to hold a topical session at the 9. regular meeting of the FSC on 4 June 2008 regarding the use of analogues for confidence building. The session opened with an introductory presentation by the session rapporteur. This incorporated input provided for the purpose by FSC members in cooperation with their country's representative to the NEA RWMC 'Integration Group on the Safety Case'. Three speakers then presented the various uses of analogues by implementers, regulators and scientists to build their own confidence; a fourth speaker dealt with the experience of using natural analogues in public information. The presentations addressed the use of analogues in the field of geological disposal of high-level waste (HLW) and long-lived intermediate level (ILW-LL) radioactive waste. Then the FSC participants split into two working groups for discussion. The outcome of these discussions was reported in plenary on 6 June 2008 and it was agreed to publish proceedings of the session. The present summary, prepared by the session rapporteur with input from the NEA Secretariat, captures the main points heard in the course of the event. It combines data from the formal presentations and remarks made in discussion. The latter represent viewpoints expressed by a group whose primary focus is not natural analogues but rather stakeholder interests. The summary and viewpoints

Offshore Wind Energy Market Overview (Presentation)

Energy Technology Data Exchange (ETDEWEB)

Baring-Gould, I.

2013-07-01

This presentation describes the current international market conditions regarding offshore wind, including the breakdown of installation costs, how to reduce costs, and the physical siting considerations considered when planning offshore wind construction. The presentation offers several examples of international existing and planned offshore wind farm sites and compares existing international offshore resources with U.S. resources. The presentation covers future offshore wind trends and cites some challenges that the United States must overcome before it will be able to fully develop offshore wind sites.

Power point 2002 for successful presentation

International Nuclear Information System (INIS)

Moon, Insoo

2002-01-01

This book mentions power point 2002 for successful presentation, which deals with power point and presentation, all guide, the latest gear, for presentation, basic of power point 2002 such as slide, text compile, insertion of picture, figure and application of office guide, setting up new year plan using text like insertion text with various methods, compile effective text, and 200% application of tab, and addiction of pretty shape of characters, finishing of conversion of chinese character, and elimination of typographical error with spell checker.

An unusual presentation of testicular tumor

International Nuclear Information System (INIS)

Amin, M.U.; Rahan, T.; Haq, A.U.; Aftab, P.

2006-01-01

A case of testicular choriocarcinoma is reported in which blood mixed stools and haemoptysis were the presenting manifestations as the patient never told about the testicular swelling to his parents. Orchidectomy was performed but the patient presented again with massive hematemesis due to gastric perforation secondary to gastric metastasis. The size of the testis at diagnosis was approximately 12 x 7cm. This was also unusual as testicular choriocarcinoma presents as a small mass. The patient eventually died of the complications within one month of diagnosis. (author)

7 CFR 1786.153 - Discounted present value.

Science.gov (United States)

2010-01-01

... 7 Agriculture 12 2010-01-01 2010-01-01 false Discounted present value. 1786.153 Section 1786.153... Discounted Prepayments on RUS Electric Loans § 1786.153 Discounted present value. (a) The discounted present value shall be calculated by summing the present values of all remaining payments on all Qualified Notes...

7 CFR 1786.98 - Discounted present value.

Science.gov (United States)

2010-01-01

... 7 Agriculture 12 2010-01-01 2010-01-01 false Discounted present value. 1786.98 Section 1786.98... Discounted present value. (a) The Discounted Present Value shall be calculated by RUS before prepayment is made by summing the present values of all remaining payments on all outstanding notes according to the...

Radwaste - Multimedia presentation

International Nuclear Information System (INIS)

2000-01-01

Radioactivity is a part of the Nature since the creation of the Earth. We have learned how to use radioactivity in medicine, power industry, and in other areas of life. Similarly as in other activities, waste is generated. We offer you basic information about radioactivity and radioactive waste (radwaste) in four films: Radioactivity, Source of radwaste, Management of radwaste. In the glossary are video presentations: Container (Castor) for radioactive waste; Deep underground repository; Transport vehicle; WWER type reactor. Encyclopedic part of the program contains detailed theoretical and practical information about use of radioactivity and radioactive waste. You will find the explanation in the part Glossary. (authors)

Jejunogastric intussusception presented with hematemesis: a case presentation and review of the literature

Directory of Open Access Journals (Sweden)

Papastratis George

2001-01-01

Full Text Available Abstract Background Jejunogastric intussusception (JGI is a rare but potentially very serious complication of gastrectomy or gastrojejunostomy. To avoid mortality early diagnosis and prompt surgical intervention is mandatory. Case presentation A young man presented with epigastric pain and bilous vomiting followed by hematemesis,10 years after vagotomy and gastrojejunostomy for a bleeding duodenal ulcer. Emergency endoscopy showed JGI and the CT scan of the abdomen was compatible with this diagnosis. At laparotomy a retrograde type II, JGI was confirmed and managed by reduction of JGI without intestinal resection. Postoperative recovery was uneventful. Conclusions JGI is a rare condition and less than 200 cases have been published since its first description in 1914. The clinical picture is almost diagnostic. Endoscopy performed by someone familiar with this rare entity is certainly diagnostic and CT-Scan of the abdomen could also help. There is no medical treatment for acute JGI and the correct treatment is surgical intervention as soon as possible.

Present studies on the radiosterilized drugs

International Nuclear Information System (INIS)

Marciniec, B.; Dettlaff, K.

2007-01-01

Lecture presents present status of radiosterilization of drugs and medical materials as compared to other sterilization methods. Literature review is shown on degradation of different kinds of drugs sterilized upon action of the sterilising doses of radiation

Combined approach for gynecomastia.

Science.gov (United States)

El-Sabbagh, Ahmed Hassan

2016-01-01

Gynecomastia is a deformity of male chest. Treatment of gynecomastia varied from direct surgical excision to other techniques (mainly liposuction) to a combination of both. Skin excision is done according to the grade. In this study, experience of using liposuction adjuvant to surgical excision was described. Between September 2012 and April 2015, a total of 14 patients were treated with liposuction and surgical excision through a periareolar incision. Preoperative evaluation was done in all cases to exclude any underlying cause of gynecomastia. All fourteen patients were treated bilaterally (28 breast tissues). Their ages ranged between 13 and 33 years. Two patients were classified as grade I, and four as grade IIa, IIb or III, respectively. The first 3 patients showed seroma. Partial superficial epidermolysis of areola occurred in 2 cases. Superficial infection of incision occurred in one case and was treated conservatively. All grades of gynecomastia were managed by the same approach. Skin excision was added to a patient that had severe skin excess with limited activity and bad skin complexion. No cases required another setting or asked for 2(nd) opinion.

Presenting Different Selves to Different People: Self-Presentation as a Function of Relationship Type and Contingent Self-Esteem.

Science.gov (United States)

Øverup, Camilla S; Brunson, Julie A; Acitelli, Linda K

2015-01-01

Past work has established a connection between self-esteem and self-presentation; however, research has not explored how self-esteem that is contingent on one's relationship may influence self-presentational tactics in that relationship. Across two studies, undergraduate students reported on the extent to which their self-esteem depended on their friendship and romantic relationship, as well as the extent to which they engaged in self-presentation behaviors in those relationships. The results suggest that relationship-specific contingent self-esteem predicts relationship-specific self-presentation; however, friendship-contingent self-esteem predicted self-presentation in both friendships and romantic relationships. These results suggest that individuals are keenly and differentially attuned to qualitatively different relationships, and when perceiving potential problems, they attempt to remedy those through their self-presentations. Furthermore, results indicate the possibility that self-esteem tied to a particular relationship may not be as important as self-esteem based more generally on one's relationships.

Re-Composing the Digital Present

Directory of Open Access Journals (Sweden)

Timothy Barker

2011-05-01

Full Text Available This paper investigates the temporality that is produced in some recent and historical examples of media art. In exploring works by Janet Cardiff, Dennis Del Favero, and Omer Fast, I use the philosophy of Michel Serres and Gilles Deleuze to understand the convergence of temporalities that are composed in the digital present, as one moment in time overlays another moment. Developing Serres' concept of multi-temporality and Deleuze's philosophy of time and memory into a means to understand the non-linear time presented in these works, I argue that the different compositional strategies enacted by these artists provide the aesthetic grounding to experience “temporal thickness.” From here I investigate the interactive digital artworks Frames by Grahame Weinbren and Can You See Me Now? by the artist group Blast Theory. In this investigation, I understand interaction with technology, and the way that it shapes our sensory and processual experience, as a specifically temporal and temporalizing transaction, where human movements in the present are overlayed by technological processes.

Nocardial mycetoma: Diverse clinical presentations

Directory of Open Access Journals (Sweden)

Sharma Nand

2008-01-01

Full Text Available Nocardia spp are gram-positive, aerobic, acid-fast bacteria which exist as saprophytes in nature. Invasive disseminated infections are particularly common in immunocompromised or debilitated hosts. Superficial infections with Nocardia spp occur as a result of local trauma and contamination of the wound. Clinically, it presents as acute infection (abscesses or cellulitis, mycetoma, or sporotrichoid infection. Differential diagnosis includes eumycetoma, chromomycosis, blastomycosis, coccidioidomycosis, sporotrichosis, tuberculosis, botryomycosis, syphilis, yaws, and neoplasia. Its diagnosis is confirmed by demonstrating the causative organism in exudates (as granules, tissue specimens, or cultures. Early diagnosis will obviate need for drastic surgical measures as early institution of chemotherapy is effective in most patients. However, its diagnosis is often delayed due to diverse clinical presentations and for want of clinical suspicion, particularly in non-endemic areas. This paper presents 4 clinical forms of this not so uncommon disease, emphasizing the importance of high index of clinical suspicion, especially in non-endemic regions; and the significance of repeated examination of exudates for Nocardia granules for an early diagnosis.

Transforming your presentation into a publication.

Science.gov (United States)

Schrager, Sarina

2010-04-01

Publication in scientific journals is required for academic promotion at universities. It is also the method by which most family medicine faculty members disseminate their scholarly work, establish a reputation in their field, and develop a clinical, teaching, or scholarly expertise. Publications are able to reach a broader audience than are presentations, which makes them the epitome of academic scholarship. Most academic family physicians give presentations to students, residents, or colleagues as part of their regular job duties and at regional and national meetings, but few write articles based on those presentations.

Rett syndrome: EEG presentation.

Science.gov (United States)

Robertson, R; Langill, L; Wong, P K; Ho, H H

1988-11-01

Rett syndrome, a degenerative neurological disorder of girls, has a classical presentation and typical EEG findings. The electroencephalograms (EEGs) of 7 girls whose records have been followed from the onset of symptoms to the age of 5 or more are presented. These findings are tabulated with the Clinical Staging System of Hagberg and Witt-Engerström (1986). The records show a progressive deterioration in background rhythms in waking and sleep. The abnormalities of the background activity may only become evident at 4-5 years of age or during stage 2--the Rapid Destructive Stage. The marked contrast between waking and sleep background may not occur until stage 3--the Pseudostationary Stage. In essence EEG changes appear to lag behind clinical symptomatology by 1-3 years. An unexpected, but frequent, abnormality was central spikes seen in 5 of 7 girls. They appeared to be age related and could be evoked by tactile stimulation in 2 patients. We hypothesize that the prominent 'hand washing' mannerism may be self-stimulating and related to the appearance of central spike discharges.

Technical presentation: Next Generation Oscilloscopes

CERN Multimedia

PH Department

2011-01-01

  Rohde & Schwarz "Next Generation Oscilloscopes" - Introduction and Presentation Agenda: Wednesday 23 March  -  09:30 to 11:30 (open end) Bldg. 13-2-005 Language: English 09.30 Presentation "Next Generation Oscilloscopes" from Rohde & Schwarz RTO / RTM in theory and practice Gerard Walker 10.15 Technical design details from R&D Dr. Markus Freidhof 10.45 Scope and Probe Roadmap (confidential) Guido Schulze 11.00 Open Discussion Feedback, first impression, wishes, needs and requirements from CERN All 11.30 Expert Talks, Hands on All Mr. Dr. Markus Freidhof, Head of R&D Oscilloscopes, Rohde & Schwarz, Germany; Mr. Guido Schulze, ...

Econophysics: Past and present

Science.gov (United States)

de Area Leão Pereira, Eder Johnson; da Silva, Marcus Fernandes; Pereira, H. B. B.

2017-05-01

This paper provides a brief historical review of the relationship between economics and physics, beginning with Adam Smith being influenced by Isaac Newton's ideas up to the present day including the new econophysics discipline and some of the tools applied to the economy. Thus, this work is expected to motivate new researchers who are interested in this new discipline.

Presentation of 2004 earnings

International Nuclear Information System (INIS)

2005-03-01

This presentation offers financial information on the AREVA Group earnings for the year 2004. With manufacturing facilities in over 40 countries and a sales network in over 100, Areva offers customers technological solutions for nuclear power generation and electricity transmission and distribution. The Group also provides interconnect systems to the telecommunications, computer and automotive markets. (A.L.B.)

Late presentation of posterior urethral valves.

Science.gov (United States)

Jalbani, Imran Khan; Biyabani, Syed Raziuddin

2014-05-01

Presence of posterior urethral valves (PUV) is the most common cause of urinary tract obstruction in the male neonate. Late presentation occurs in 10% of cases. We present a case of PUVs in an adult male who presented with history of obstructive lower urinary tract symptoms and hematuria. On evaluation, he was found to have raised serum creatinine level. A voiding cystourethrogram (VCUG) could not be completely performed because of narrowing in the posterior urethra. A rigid urethrocystoscopy was performed at which he was found to have type-I posterior urethral valve which were fulgurated. A repeat uroflowmetry revealed maximum flow rate of 12 ml/second. This case highlights that PUVs is not solely a disease of infancy but may also present late. VCUG is the radiological investigation of choice but the diagnosis may be missed. A urethrocystoscopy is advised if there is a high index of suspicion.

Electric power: Past, present, and future

International Nuclear Information System (INIS)

Schnetzer, H.

1994-01-01

When, at the turn of the century, public electric power supply facilities were created and in 1908, the electric power stations of the Swiss canton of Zurich (EKZ) were built, only a third of the communities in the Zurich area could boast about being the consumers of this new energy. But what did the first electrically powered devices and machines look like? This, and more, is presented in the ''electric power house'' in Burenwisen Glattfelden in the canton of Zurich. Besides a Kaplan turbine and a sample of the most interesting devices from the past and the present, the focus of the exhibition is on the presentation of the new and old sources of light. The EKZ are pleased to be able to present their ''electric power house'' to the public, providing a broad range of information on energy-related questions and the development of electric power supply. (orig.) [de

Congenic tuberculosis. Presentation of clinical case

International Nuclear Information System (INIS)

David Calabria, Milena; Ojeda Leon, Paulina

2004-01-01

We are presenting the case of less than to 2 month old and premature infant, she had hyaline membrane disease and required mechanical ventilation during 6 days, at the first month. She presented a viral rhinopharyngitis symptomatology, perioral cyanosis with apnoea, and respiratory distress, the chest x-ray showed multiple bilateral hyperlucid images, mainly at the right side afterwards the patient presented intermittent fever of long evolution and initially received treatment for cavitary bronchopneumonia and sequel of mechanical ventilation, receiving multiple antimicrobial antibiotics, the mother had intermittent fevers since the sixth month of pregnancy and was hospitalized for suspected endometritis after delivery, she presented clinical impairment which evolved to sepsis and died in the ICU, the endometrial histopathology showed granulomas suggesting tuberculosis, due to long history of fevers in the baby and the genital tuberculosis in the mother a tuberculosis study was performed in the girl which resulted positive

Poster presentations: valuing all forms of evidence.

Science.gov (United States)

Halligan, Phil

2008-01-01

This paper aims to assist readers who are considering submitting a poster presentation to a conference to appreciate their true worth and demonstrate the important contribution they can make to nursing scholarship and secondly, to provide new insights to conference reviewers who have the task of selecting appropriate contributions for conferences. The author will argue that the use of poster presentations is an ideal opportunity to disseminate research findings and clinical innovations. It offers a new, interesting, and thought-provoking perspective on poster presentations, which includes appraisal of extant literature and reflections about the significance of poster presentations to nursing scholarship. It highlights the benefits of submitting a poster presentation and concludes with some key points for conference reviewers to consider when deciding to select the suitability of abstracts for poster presentations or concurrent sessions.

Film sound in preservation and presentation

NARCIS (Netherlands)

Campanini, S.

2014-01-01

What is the nature of film sound? How does it change through time? How can film sound be conceptually defined? To address these issues, this work assumes the perspective of film preservation and presentation practices, describing the preservation of early sound systems, as well as the presentation

AECL present and future

International Nuclear Information System (INIS)

Foster, J.S.

The history and present activities of the Crown corporation AECL are described. AECL owns limited interests in nuclear power plants and a Manitoba transmission system. AECL is the world's foremost producer of heavy water, and exports CANDU type reactors and radiation processing equipment. AECL backs up the Canadian nuclear power program wherever there is no conflict with private enterprise. (E.C.B.)

Presentation on ecoenergetic assessment

International Nuclear Information System (INIS)

Fournelle, C.

1998-01-01

The development of a tool to assess the quality of indoor air for buildings in the residential and commercial sector was presented. The computer software program was designed to allow a building operator to set an optimal target to reflect the greatest economies in energy consumption. Air quality was assessed in terms of carbon dioxide, volatile organic compounds, and other common indoor pollutants

Desulfuration technologies: past, present and future; Tecnologias de desulfuracion: pasado, presente y futuro

Energy Technology Data Exchange (ETDEWEB)

Rodas G, Arturo; Blass A, Georgina; Panama T, Luz A [Instituto de Investigaciones Electricas, Cuernavaca, Morelos (Mexico)

2003-07-01

The interest for obtaining harmony between the electric power generation and the environment has derived into the design of numerous technologies for the control of the main emissions of atmospheric polluting agents, being some of them the sulfur oxides (SOx) that, at certain levels of concentration in the atmosphere, cause health damages and to the surrounding ecosystem. The present article shows an overview of the technologies for the disposal of sulfurated compounds in thermoelectric power stations and their application trends in the future systems of electric power generation. [Spanish] El interes por lograr una armonia entre la generacion de energia electrica y el medio ambiente, ha derivado en el diseno de numerosas tecnologias para el control de las principales emisiones de contaminantes atmosfericos, siendo algunos de ellos los oxidos de azufre (SOx) que, a ciertos niveles de concentracion en la atmosfera, ocasionan danos a la salud y al ecosistema circundante. El presente articulo muestra un panorama general de las tecnologias de eliminacion de compuestos sulfurados en centrales termoelectricas y sus tendencias de aplicacion en los futuros sistemas de generacion de energia electrica.

Graphical presentation of participants' results

International Nuclear Information System (INIS)

Anon.

1991-01-01

The results obtained by 19 participating dosimetry systems are presented in this section. Section 3 of the preceding report, ''Evaluation of the Results of the First IAEA Coordinated Research Program on Intercomparison for Individual Monitoring'', explains the format and conventions used for the graphical presentation. The system number appearing in the upper left hand corner of each page can be used to correlate the dosimeter design information found in Table 2 of the preceding report with the results. However, care should be used in drawing inferences from this correlation, since system performance depends on a number of factors other than detector selection and design characteristics. (orig.)

Poster Presentations: Conceptualizing, Constructing and Critiquing

Science.gov (United States)

Newbrey, Michael G.; Baltezore, Joan M.

2006-01-01

Posters are commonly used as tools for disseminating information at scientific meetings, but many students lack an understanding of "good" poster characteristics. We present a set of characteristics for use in constructing posters for scientific meetings and classroom presentations along with tips on critiquing posters to enhance their…

CIGEO public debate. Presentation of the Citizens' Conference - Press file. Presentation of the citizens panel opinion

International Nuclear Information System (INIS)

Hermitte, Marie-Angele; Bedu, Clemence; Besnus, Francois; Brom, Jean-Marie; Grambow, Bernd; Ruedinger, Andreas; Fourniau, Jean-Michel; Bobbio, Luigi; Blatrix, Cecile

2013-01-01

After a presentation of the organisation and implementation of the Citizens' Conference, a presentation of the members of the steering committee and of the assessment committee of this conference, this document presents the opinion of the citizen panel in the framework of the public debate on the Cigeo project of deep underground disposal of radioactive wastes. This opinion notably outlines the waste issue as an inter-generational issue, comments the calendar and condition of the Cigeo project, states the panel opinion on risks which are specific to the Cigeo project, discusses the issue of recoverability and reversibility, discusses the issue of site memory, evokes the possibility of exploitation of geothermal energy, outlines the importance of health and environmental monitoring, comments opportunities for local development, and discusses cost and financing assessments. An appendix presents the different training programmes proposed during the citizens' conference

Manipulating affective state using extended picture presentations.

Science.gov (United States)

Sutton, S K; Davidson, R J; Donzella, B; Irwin, W; Dottl, D A

1997-03-01

Separate, extended series of positive, negative, and neutral pictures were presented to 24 (12 men, 12 women) undergraduates. Each series was presented on a different day, with full counterbalancing of presentation orders. Affective state was measured using (a) orbicularis oculi activity in response to acoustic startle probes during picture presentation, (b) corrugator supercilii activity between and during picture presentation, and (c) changes in self-reports of positive and negative affect. Participants exhibited larger eyeblink reflex magnitudes when viewing negative than when viewing positive pictures. Corrugator activity was also greater during the negative than during the positive picture set, during both picture presentation and the period between pictures. Self-reports of negative affect increased in response to the negative picture set, and self-reports of positive affect were greatest following the positive picture set. These findings suggest that extended picture presentation is an effective method of manipulating affective state and further highlight the utility of startle probe and facial electromyographic measures in providing on-line readouts of affective state.

Applying Technology To Facilitate Poster Presentations.

Science.gov (United States)

Marek, Pam; Christopher, Andrew N.; Koenig, Cynthia S.

2002-01-01

Promotes the use of presentation software in psychology courses to teach students technological skills that prepare them for the future. Explains that many graduates in psychology are employed in other fields after graduation. Discusses the use of presentation software with a focus on poster preparation using Microsoft PowerPoint software. (CMK)

NUT carcinoma presenting in the palate

DEFF Research Database (Denmark)

Bjornstrup, Libana Raffoul; Reibel, Jesper; Kiss, Katalin

2017-01-01

NUT carcinomas (NC) are rare and aggressive tumours characterized by chromosomal rearrangements of the gene encoding for nuclear protein of the testis (NUT) located on chromosome 15q14. This article presents a case of a 60-year-old woman diagnosed with NC presenting as a fast growing primary tumo...

25 CFR 11.903 - Presenting officer.

Science.gov (United States)

2010-04-01

... OF INDIAN AFFAIRS, DEPARTMENT OF THE INTERIOR LAW AND ORDER COURTS OF INDIAN OFFENSES AND LAW AND ORDER CODE Children's Court § 11.903 Presenting officer. (a) The agency superintendent and the chief magistrate of the children's court shall jointly appoint a presenting officer to carry out the duties and...

Controlled thermonuclear fusion. Present state and prospective

International Nuclear Information System (INIS)

Consoli, T.

1976-01-01

The interest of thermonuclear fusion for energy production is underlined. The present state of the research in this field is presented, emphasis being given to Tokamak configurations. The problems concerning confinement and additional heating in these devices are presented [fr

Marketing the sun: Text of presentations

International Nuclear Information System (INIS)

1995-04-01

The title conference and the texts of the presentations give insight into the activities and the developments in the field of solar energy and of the parties involved. The increased interest in the application of solar energy is shown in the subjects dealt with, while they are aimed more at the market than at research. Three parallel sesions and one plenary session were held. In each parallel session 20 presentations were given. In session one on the subjects market development of thermal solar energy, new housing construction in series, minimal energy houses, new solar water heaters, technical and economical options in sunny countries. In session two on the subjects policy, maintenance and renovation of existing houses, solar energy and planning, grid-connected photovoltaic systems, perspectives and experiences in sunny countries. In session three on the subjects solar cells, autonomous photovoltaic systems, future developemnts of thermal solar energy, solar architecture, market for photovoltaic systems in sunny countries. In the plenary session 3 presentations were held on the subject market for solar energy in newly built houses. Apart from the text of the session presentations 43 posters are presented on the subjects thermal solar energy (8), solar cells (9), autonomous photovoltaic systems (2), grid-connected PV systems (14), policy (2), integration (6), and sunny countries (1). tabs., figs., refs

Dynamic bowel obstruction: aetiology, clinical presentation ...

African Journals Online (AJOL)

This study was conducted to describe in our region, the aetiology, clinical presentation, management and outcome of dynamic bowel obstruction. Data were analyzed using SPSS software system. A total of 342 patients were studied. Males outnumbered females by a ratio of 2.1: 1. The median age of patients at presentation ...

Becker muscular dystrophy: an unusual presentation.

OpenAIRE

Thakker, P B; Sharma, A

1993-01-01

A 15 year old boy who presented with passing painless dark urine was found to have myoglobinuria. His creatine phosphokinase was raised, and a muscle biopsy specimen showed non-specific dystrophic changes. Subsequent DNA analysis led to the diagnosis of Becker muscular dystrophy. Myoglobinuria may be a presenting symptom of Becker muscular dystrophy.

Structuring Assignments to Improve Understanding and Presentation Skills: Experiential Learning in the Capstone Strategic Management Team Presentation

Science.gov (United States)

Helms, Marilyn M.; Whitesell, Melissa

2017-01-01

In the strategic management course, students select, analyze, and present viable future alternatives based on information provided in cases or computer simulations. Rather than understanding the entire process, the student's focus is on the final presentation. Chickering's (1977) research on active learning suggests students learn more effectively…

Menkes Disease Presenting with Epilepsia Partialis Continua

Directory of Open Access Journals (Sweden)

Tamer Rizk

2014-01-01

Full Text Available Aim. We aim to describe a female patient with Menkes disease who presented with epilepsia partialis continua. Case Presentation. Seventeen-months-old Saudi infant was presented with repetitive seizures and was diagnosed to have epilepsia partialis continua. Discussion. Menkes disease (OMIM: 309400 is considered a rare, X-linked recessive neurodegenerative disorder resulting from a mutation in the gene coding for the copper transporting ATPase (ATP7A. Affected individuals usually present with kinky hair, skeletal changes, prolonged jaundice, hypothermia, developmental regression, decreased tone, spasticity, weakness, and therapy resistant seizures. Conclusion. Raising awareness of abnormal presentation of this rare disease may help in the control of seizures through subcutaneous copper supplementation.

Normothermic thyroid storm: an unusual presentation

Science.gov (United States)

Sabir, Anas Ahmad; Sada, Kabiru; Yusuf, Bashir O.; Aliyu, Idris

2016-01-01

Thyroid storm is a rare life-threatening emergency due to thyrotoxicosis. A 30-year-old female presented with restlessness, tachycardia and vomiting but with normothermia which is an unusual presentation. There is the need for clinicians to be aware of atypical clinical features that can make the diagnosis of thyroid storm difficult. PMID:27540465

Into the intracellular logistics of cross-presentation

Directory of Open Access Journals (Sweden)

Charlotte eSadaka

2012-02-01

Full Text Available The induction of cytotoxic CD8+ T cell responses requires the presentation of antigenic peptides by MHC class I molecules (MHC I. MHC I usually present peptides derived from endogenous proteins. However, some subtypes of dendritic cells (DCs have developed the ability to efficiently present peptides derived from exogenous antigens on MHC I via a process called cross-presentation. Cross-presentation is intimately linked to the induction of anti-viral, -bacterial and -tumor cytotoxic T cell (CTL responses, as well as a wide variety of CTL-mediated diseases and transplant rejections. The molecular and cellular mechanisms underlying cross-presentation have been studied intensively since its original description, yet understanding of this process is incomplete and on the forefront of immunological research. Numerous pathways and models, some of them conflicting, have been described so far. Here, we review the various pathways reported as involved in cross-presentation, highlighting the complexity of this process. We also discuss in detail the different intracellular steps required, from antigen capture and routing, to processing and finally peptide loading, emphasizing the need for a better understanding of the cell biology of this phenomenon.

Presentation of TVO's visitor's centre

International Nuclear Information System (INIS)

Aemmaelae, V.M.

1993-01-01

There are four nuclear power plant units in Finland, two of which are PWR's owned by Imatran Voima Oy. The two BWR units are located at Olkiluoto and owned by Teollisuuden Voima Oy. This presentation tells about TVO's concept of informing the visitors at Olkiluoto. At the site there are located, in addition to the two nuclear power plant units, the intermediate storage for spent fuel, the repository for low and medium-active waste as well as the training centre. At the Olkiluoto Visitor's Centre all the activities of the company are presented using varied audio-visual aids. The centre has several exhibits and there are also different installations to show how the plant works. (author)

Sitosterolemia Presenting as Pseudohomozygous Familial Hypercholesterolemia.

Science.gov (United States)

Renner, Christian; Connor, William E; Steiner, Robert D

2016-06-01

A young girl, age 8.5 years, presented with profound hypercholesterolemia and early xanthomatosis, suggesting homozygous familial (or type II) hypercholesterolemia. The patient's low density lipoprotein (LDL) receptor function and parental lipoprotein profiles were determined to be normal, prompting revision of the initial diagnosis to pseudohomozygous familial hypercholesterolemia. When she subsequently presented with giant platelets, the case was presented to colleagues on an electronic mailing list. It was recommended that plasma and sterol analysis be performed, which led to a diagnosis of sitosterolemia. The presentation of profound hypercholesterolomia in childhood that ultimately is not attributed as due to homozygous or compound heterozygous defects in the LDL receptor gene has been termed pseudohomozygous familial (or type II) hypercholesterolemia (PHT2HC). Patients diagnosed with PHT2HC subsequently confirmed to have sitosterolemia have been previously reported only rarely. The challenge of achieving accurate specific diagnosis and appropriate workup for these conditions in children is discussed in the context of this rare case and review of the historical literature concerning these conditions. © 2016 Marshfield Clinic.

Atypical Presentation of Traumatic Aortic Injury

Directory of Open Access Journals (Sweden)

Andrew Fu Wah Ho

2014-01-01

Full Text Available Background. Blunt thoracic aorta injury (BAI is second only to head injury as cause of mortality in blunt trauma. While most patients do not survive till arrival at the hospital, for the remainder, prompt diagnosis and treatment greatly improve outcomes. We report an atypical presentation of BAI, highlighting the diagnostic challenges of this condition in the emergency department. Case Presentation. A previously well 25-year-old male presented 15 hours after injury hemodynamically stable with delirium. There were no signs or symptoms suggestive of BAI. Sonography showed small bilateral pleural effusions. Chest radiograph showed a normal mediastinum. Eventually, CT demonstrated a contained distal aortic arch disruption. The patient underwent percutaneous endovascular thoracic aortic repair and recovered well. Conclusion. This catastrophic lesion may present with few reliable signs and symptoms; hence, a high index of suspicion is crucial for early diagnosis and definitive surgical management. This paper discusses the diagnostic utility of clinical features, injury mechanism, and radiographic modalities. Consideration of mechanism of injury, clinical features, and chest radiograph findings should prompt advanced chest imaging.

Early Mycoplasma pneumoniae infection presenting as multiple pulmonary masses: an unusual presentation in a child

Energy Technology Data Exchange (ETDEWEB)

Yang, Edward; Altes, Talissa; Anupindi, Sudha A. [The Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States)

2008-04-15

Mycoplasma pneumoniae is a major cause of community-acquired pneumonia. Because most children are not imaged prior to onset of clinical symptoms, the appearance of early Mycoplasma infection has not been extensively studied. We present the case of an 11-year-old boy with large pulmonary masses incidentally detected during spine MRI evaluation for scoliosis. Eight days later, the patient developed acute respiratory symptoms, and the masses seen previously had evolved into a diffuse bronchiolitis. Diagnostic testing identified Mycoplasma pneumoniae as the likely etiology. We briefly review chest CT findings of infection by Mycoplasma and compare them to this unusual presentation of Mycoplasma pneumonia with subclinical imaging findings. (orig.)

Early Mycoplasma pneumoniae infection presenting as multiple pulmonary masses: an unusual presentation in a child

International Nuclear Information System (INIS)

Yang, Edward; Altes, Talissa; Anupindi, Sudha A.

2008-01-01

Mycoplasma pneumoniae is a major cause of community-acquired pneumonia. Because most children are not imaged prior to onset of clinical symptoms, the appearance of early Mycoplasma infection has not been extensively studied. We present the case of an 11-year-old boy with large pulmonary masses incidentally detected during spine MRI evaluation for scoliosis. Eight days later, the patient developed acute respiratory symptoms, and the masses seen previously had evolved into a diffuse bronchiolitis. Diagnostic testing identified Mycoplasma pneumoniae as the likely etiology. We briefly review chest CT findings of infection by Mycoplasma and compare them to this unusual presentation of Mycoplasma pneumonia with subclinical imaging findings. (orig.)

Past Experiences, Present Beliefs, Future Practices: Using Narratives to Re(present) Leadership Educator Identity

Science.gov (United States)

Priest, Kerry L.; Seemiller, Corey

2018-01-01

In an effort to better understand leadership educator preparation, this qualitative study explores leadership educators' identity constructions, or (re)presentations of experiences, beliefs, and practices that contribute to one's professional identity. We used three narrative approaches (storytelling, symbolic interactionism, and anticipatory…

Mediated Teaching for Information Presenting and Understanding

Directory of Open Access Journals (Sweden)

Konstantinas Danaitis

2011-04-01

Full Text Available Nowadays media is readily moving into various spheres of life. The computer, projector and screen replace trivial chalk and board. Slides are used in various designated areas: promotional presentations at meetings as a form of communication between participants and also as visual presentation of lecture material content of the report. It was found that a human catches up and remembers only about 10% of what he has read, 20% of what he has heard and even 50% of what he heard and saw. Therefore, high-quality and Professional presentation of information in the slide form has a significant impact on the uptake. The authors present a solution how to present the study material qualitatively and use the media system effectively for teaching computer graphics course. This course is taught for VGTU first or second year students of almost all specialities. This presentation of study materials facilitates the teacher's work and improves the readability and solutions of study materials.

WWNPQFT-2010 - Slides of the presentations

International Nuclear Information System (INIS)

Fried, H.M.; Huber, M.Q.; Grandou, T.; Bianchi, E.; Gracey, J.; Reys, V.; Jevicki, A.; Ferrante, D.; Bouakaz, K.; Spielmann, D.; Cucchieri, A.; Culetu, H.; Gelis, F.; Zwanziger, D.; Candelpergher, B.; Bender, C.

2013-01-01

This document is made up of the slides of the presentations. The object of this workshop is to consolidate and publicize new efforts in non-perturbative field theories. The main topics are quantum chromodynamics, Yang-Mills theory, effective locality, the Gribov-Zwanziger Lagrangian, and renormalization. A presentation is dedicated to the initial stages of high energy nucleus-nucleus collisions

Selecting a Web 2.0 Presentation Tool

Science.gov (United States)

Hodges, Charles B.; Clark, Kenneth

2011-01-01

Web-based presentation tools are sometimes referred to as "next generation presentation tools" (EDUCAUSE, 2010). At the most basic level, these tools are simply online versions of traditional presentation software, such as Microsoft's PowerPoint or Apple's Keynote, but some services offer features like web-based collaboration, online presentation…

7 CFR 1786.53 - Discounted present value.

Science.gov (United States)

2010-01-01

... 7 Agriculture 12 2010-01-01 2010-01-01 false Discounted present value. 1786.53 Section 1786.53... Special Discounted Prepayments on RUS Direct/Insured Loans § 1786.53 Discounted present value. The Discounted Present Value shall be calculated five business days before prepayment is made by summing the...

Intestinal tuberculosis presenting as acute abdomen

International Nuclear Information System (INIS)

Khan, M.A.; Majeed, F.A.; Ahmed, M.; Khan, M.N.

2012-01-01

Objectives: To study the outcome of intestinal tuberculosis presenting as acute abdomen. Study design: Descriptive Study. Place and Duration: Bolan Medical Complex Hospital (BMC) Quetta and Combined Military Hospital (CMH) Quetta from Nov 2003 to Nov 2005 from Bolan Medical Complex and from Nov 2005 to Nov 2006 in CMH Quetta. Material and Method: Thirty seven patients of acute abdomen presenting with intestinal obstruction were admitted; 28 from emergency department and 9 from out patient department. Twenty seven patients were from BMC and 10 from CMH Quetta. Patients were diagnosed as having abdominal tuberculosis on the basis of operative findings and histopathological reports. Results: Out of 37 patients presenting with acute abdomen due to intestinal obstruction, 54% were male and 46% were female with M: F ratio of 1: 1.2. Age of the patient ranged from 20 to 50 years, with maximum frequency between 30 to 40 years. Abdominal pain was the commonest presenting feature in all patients followed by constipation in 81.1% patients. Peritonism was seen in 27% patients. Different operative procedures performed were adhesionolysis 65.8%, segmental resection 7.9%, right hemicolectomy 10.5%, stricturoplasty 7.9% and ileostomy 1.3%. Mesenteric lymph node biopsy 40.8%. Conclusion: Intestinal tuberculosis is still a very important surgical problem in our country presenting as acute abdomen. A suspicion must always be kept during laparotomy and adequate tissue histopathology should supplement the diagnosis. (author)

2014 Water Power Program Peer Review: Marine and Hydrokinetic Technologies, Compiled Presentations (Presentation)

Energy Technology Data Exchange (ETDEWEB)

2014-02-01

This document represents a collection of all presentations given during the EERE Wind and Water Power Program's 2014 Marine and Hydrokinetic Peer Review. The purpose of the meeting was to evaluate DOE-funded hydropower and marine and hydrokinetic R&D projects for their contribution to the mission and goals of the Water Power Program and to assess progress made against stated objectives.

Presentation at FTP: A review

International Nuclear Information System (INIS)

Sheffield, J.

1987-01-01

This series of vugraphs presents some information concerning ORNL's involvement in the fusion energy research program. Recent and future experiments are named, and a rough estimate of funding is given

[How to prepare a poster presentation].

Science.gov (United States)

Cerezal Pesquera, L

2013-06-01

Scientific and educational poster presentations in national and international meetings are one of the most effective means of scientific communication. Poster presentations are of particular importance in a mainly visual speciality like radiology, and also play a significant role in continuing medical education in our specialty. However, poster should be no more than an intermediate step on the way to publish our work in a scientific journal. The publication rates of posters prented in courses and conferences is very low in our country. Therefore, we must continue to progress in training in scientific publication, with efforts such as this supplement. This article review how to create an effective poster presentation. Copyright © 2013 SERAM. Published by Elsevier Espana. All rights reserved.

Late presentation of HIV infection: a consensus definition

DEFF Research Database (Denmark)

Antinori, A; Coenen, T; Costagiola, D

2010-01-01

clinical definition of late presentation. The objective of this article is to present a consensus definition of late presentation of HIV infection. Methods Over the past year, two initiatives have moved towards a harmonized definition. In spring 2009, they joined efforts to identify a common definition...... of what is meant by a 'late-presenting' patient. Results Two definitions were agreed upon, as follows. Late presentation: persons presenting for care with a CD4 count below 350 cells/muL or presenting with an AIDS-defining event, regardless of the CD4 cell count. Presentation with advanced HIV disease...... able to implement this definition (either on its own or alongside their own preferred definition) when reporting surveillance or research data relating to late presentation of HIV infection....

Comparison between late-presenting and isolated neonatal ...

African Journals Online (AJOL)

Results: Isolated neonatal cases presented more often with acute respiratory symptoms (n=25; P= 0.016) and failure to thrive (n= 38; P= 0.03). Late-presenting cases presented more often with chronic respiratory symptoms (n=14; P= 0.0044) or gastrointestinal symptoms (n=12; P= 0.006). Thirty-five cases with minor or ...

The Statue of Liberty Deed of Presentation.

Science.gov (United States)

Schamel, Wynell Burroughs; Blondo, Richard A.

1992-01-01

Presents a history lesson based on the deed of presentation for the Statue of Liberty. Discusses the conception and history of the statue and its presentation to the United States as a gift from the people of France. Includes a copy of the document, topics for class discussion, and research, writing, and design activities. (DK)

The clinical presentation of pulmonary embolism

International Nuclear Information System (INIS)

Otto, A.C.; Dunn, M.; Van der Merwe, B.; Katz, M.

2004-01-01

Full text: Introduction: Pulmonary embolism is often misdiagnosed by many clinicians because of confusion with pulmonary infarction. According to present literature, the latter seldomly occurs, thus pleuritic pain and hemoptysis are usually absent. The purpose of our study was to re-evaluate the clinical presentation of pulmonary embolism with specific reference to the presence of pulmonary hypertension and pulmonary infarction. Materials and methods: Ethical committee approval was obtained to study fifteen patients with abnormal lung perfusion and high probability for pulmonary embolism retrospectively. Clinical data will be presented in table format. Appropriate symptoms and signs not mentioned was considered absent. Specific investigations not available was considered not done. Results: Will be displayed in table format. The main findings can be summarized as follows: 1. Dyspnea was present in all the patients; 2. Pleuritic pain and hemoptysis was absent in all the patients; 3. None of the patients had clinical signs of pulmonary hypertension or infarction; 4. The available special investigations confirmed the above mentioned findings. Conclusion: The absence of pulmonary infarction and pulmonary hypertension with submassive pulmonary embolism (<60% of pulmonary vascular bed occluded) was reconfirmed and should be emphasized more in clinical practice. (author)

Hypertension presenting early in pregnancy

OpenAIRE

Melvin, Audrey; Kinsley, Brendan

2015-01-01

Key Clinical Message Paraganglioma in pregnancy is an exceedingly rare and potentially life?threatening diagnosis. It is important that the clinicians consider secondary causes when women present with hypertension in early pregnancy.

Industry Presentation to Regulatory Workshop

International Nuclear Information System (INIS)

Treasure, Mark

2012-01-01

Mr. Mark Treasure from EDF NGL gave a presentation on industry perspectives on safety culture oversight. Mr. Treasure is the Nuclear Inspection and Oversight Manager within the Safety and Regulation Division. The presentation started with an explanation of the role of the nuclear inspection and oversight group (internal regulator), and their current approach to internal oversight of safety culture. A key element of the current internal regulatory oversight program is Management and Leadership Assessments. These are carried out by a team including management peers from other plants to enhance credibility. Findings can be linked to safety performance, and typically identify issues in areas such as accountability arrangements and strategic focus of the leadership team. Safety indicators have also been introduced to show trends in safety management and safety performance for each EDF UK nuclear power plant. A periodic nuclear safety culture survey is also carried out to identify focus areas and progress. The presentation included discussion on views of the role of the nuclear regulator. Important aspects were identified as: - Supporting the internal regulator by seeking to understand before taking enforcement action, maintaining an open dialogue and recognising that this area is complex and that there is rarely a 'silver bullet' solution. - Communication: being visible and actively discussing safety improvement, and use of language which emphasises nuclear safety rather than legal compliance. - Positive reinforcement to recognise efforts and encourage further improvement

Clinical presentation and staging of Hodgkin lymphoma

DEFF Research Database (Denmark)

Gallamini, Andrea; Hutchings, Martin; Ramadan, Safaa

2016-01-01

, sometimes HL is a subtle disease, difficult to diagnose for the paucity of symptoms, the absence of physical findings, or for concomitant immunologic disorders: a compete overview of the common and rare patterns of HL clinical presentation will be also offered. The future perspective of PET scan use......In the present chapter the authors present a brief overview of the diagnostic methods proposed over time for Hodgkin lymphoma (HL) spread detection, moving from surgical procedures, through standard radiological and functional imaging techniques to the present state of the art for HL staging...... tomography (FDG-PET) scan in the armamentarium of staging procedures will be presented and commented, with a special emphasis on the utility of special procedures, such as bone marrow trephine biopsy, which is deemed no longer needed in the PET era. While the HL diagnosis is straightforward in most cases...

Metabolic syndrome presenting as abdominal pain

Directory of Open Access Journals (Sweden)

Mohammed Y Al-Dossary

2017-01-01

Full Text Available Metabolic syndrome represents a sum of risk factors that lead to the occurrence of cardiovascular and cerebrovascular events. The early detection of metabolic syndrome is extremely important in adults who are at risk. Although the physiopathological mechanisms of the metabolic syndrome are not yet clear, insulin resistance plays a key role that could explain the development of type 2 diabetes mellitus in untreated metabolic syndrome patients. Here, we present the case of a 26-year-old male who was diagnosed with metabolic syndrome and severe hypertriglyceridemia after presenting with abdominal pain. Although hypertriglyceridemia and hyperglycemia are the most common predictors of metabolic syndrome, clinicians need to be vigilant for unexpected presentations in patients at risk for metabolic syndrome. This case sheds light on the importance of early detection.

QCD machines - present and future

International Nuclear Information System (INIS)

Christ, N.H.

1991-01-01

The present status of the currently working and nearly working dedicated QCD machines is reviewed and proposals for future machines are discussed with particular emphasis on the QCD Teraflop Project in the US. (orig.)

PRESENTATION POTENTIAL USING IN PEDAGOGICAL INTERACTION PROCESS

Directory of Open Access Journals (Sweden)

Olga V. Ershova

2016-01-01

Full Text Available The given article is aimed at considering multimedia presentation potential and its influence on strengthening classroom teacher-student interaction. In the article the importance of using this kind of activity in the study process is pointed in connection with educational state policy on the one hand. On the other hand, gained students’ skills as a final result of work with presentations met employers’ demand for both parent and world labour-markets and bring competitive benefit to the candidates. Scientific novelty and results. Multimedia presentation is considered as a specific complex of classroom activities. The students are oriented on the self analysis and presentation assessment. It is shown that well-organized process of peer students’ assessment allows to simultaneously helping in solving the didactic and methodical problems. To this purpose the system of assessment criteria should be developed. It has to be clear for students for making assessment feasible and time-saving. The example of a possible variant of criteria system is described; quality of the presentations prepared by students can be defined based on such system criteria. The author also analyzed software products of the three main platforms (Windows, Linux, MacOs which have different tools and allow to follow users’ needs for creating presentations. In the article there is a comparative table of the two most popular software development: the program Microsoft PowerPoint and the web-service Prezi for realizing the relevance of their use in the study process. Practical significance of the present article concludes in author’s suggestions of some recommendations for presentation potential use as a tool of improving pedagogical interaction process with contemporary students. 

Multiple myeloma presenting as mandibular pain

LENUS (Irish Health Repository)

Crowley, Miriam

2016-10-01

Introduction: Multiple myeloma (MM) is a systemic malignancy of plasma cells defined by monoclonal production of circulating immunoglobulins. Bone pain is a presenting feature in the majority of cases. Treatment may involve intravenous use of bisphosphonates, chemotherapy or haematopoietic stem cell transplantation. Here, we illustrate a first presentation of MM in a patient with mandibular pain and discuss radiographic, diagnostic and treatment challenges of orofacial issues in patients with MM.\\r\

Food irradiation - past, present and future

International Nuclear Information System (INIS)

Diehl, J.F.

2002-01-01

A review is presented of historical developments, the present situation, and expected future developments in the field of food irradiation. Acceptance of the process in different parts of the world is not uniform. In the USA and in some other countries where health authorities actively encourage the use of this technology, commercial application has greatly advanced in recent years. In contrast, progress in the European Union is still slow

Insights on the poster preparation and presentation process.

Science.gov (United States)

Moore, L W; Augspurger, P; King, M O; Proffitt, C

2001-05-01

Dissemination of research findings and effective clinical innovations is key to the growth and development of the nursing profession. Several avenues exist for the dissemination of information. One forum for communication that has gained increased recognition over the past decade is the poster presentation. Poster presentations are often a significant part of regional, national, and international nursing conferences. Although posters are frequently used to disseminate information to the nursing community, little is reported about actual poster presenters' experiences with preparation and presentation of their posters. The purpose of this article is to present insights derived from information shared by poster presenters regarding the poster preparation and presentation process. Such insights derived from the personal experiences of poster presenters may assist others to efficiently and effectively prepare and present scholarly posters that disseminate information to the nursing community. Copyright 2001 by W.B. Saunders Company

Biased lineups: sequential presentation reduces the problem.

Science.gov (United States)

Lindsay, R C; Lea, J A; Nosworthy, G J; Fulford, J A; Hector, J; LeVan, V; Seabrook, C

1991-12-01

Biased lineups have been shown to increase significantly false, but not correct, identification rates (Lindsay, Wallbridge, & Drennan, 1987; Lindsay & Wells, 1980; Malpass & Devine, 1981). Lindsay and Wells (1985) found that sequential lineup presentation reduced false identification rates, presumably by reducing reliance on relative judgment processes. Five staged-crime experiments were conducted to examine the effect of lineup biases and sequential presentation on eyewitness recognition accuracy. Sequential lineup presentation significantly reduced false identification rates from fair lineups as well as from lineups biased with regard to foil similarity, instructions, or witness attire, and from lineups biased in all of these ways. The results support recommendations that police present lineups sequentially.

A Type-II First Branchial Cleft Anomaly Presenting as a Post-Auricular Salivary Fistula: A Rare Presentation.

Science.gov (United States)

Jain, S; Deshmukh, Pt; Gupta, M; Shukla, S

2014-01-01

First branchial cleft anomalies are rare with the average age of presentation as 19 years. There is an average delay of 3.5 years between initial presentation and adequate treatment due to diagnostic dilemma. A very rare variant of first branchial cleft anomaly presenting as a post-auricular salivary fistula is reported. A 12-year-old girl presented with a history of intermittent watery discharge, more so at the time of meals from a right post-auricular opening for last 3 years. Computed tomography sialography revealed a fistulous tract connecting the sub segmental duct of the parotid gland extending along the pre-tragus region in subcutaneous plane up to mastoid tip after passing inferior to external auditory canal. Superficial parotidectomy with identification of facial nerve branches was carried out for excision of the tract. Histopathology revealed sinus tract comprising of ectodermal components and acini of the parotid gland. We classified our case into work's type-2 based on anatomical location at an angle of mandible, its relationship to parotid gland and facial nerve and previous history of ear discharge. To the best of our knowledge, this is the first case of its type to be reported.

A Type-II First Branchial Cleft Anomaly Presenting as a Post-Auricular Salivary Fistula: A Rare Presentation

Science.gov (United States)

Jain, S; Deshmukh, PT; Gupta, M; Shukla, S

2014-01-01

First branchial cleft anomalies are rare with the average age of presentation as 19 years. There is an average delay of 3.5 years between initial presentation and adequate treatment due to diagnostic dilemma. A very rare variant of first branchial cleft anomaly presenting as a post-auricular salivary fistula is reported. A 12-year-old girl presented with a history of intermittent watery discharge, more so at the time of meals from a right post-auricular opening for last 3 years. Computed tomography sialography revealed a fistulous tract connecting the sub segmental duct of the parotid gland extending along the pre-tragus region in subcutaneous plane up to mastoid tip after passing inferior to external auditory canal. Superficial parotidectomy with identification of facial nerve branches was carried out for excision of the tract. Histopathology revealed sinus tract comprising of ectodermal components and acini of the parotid gland. We classified our case into work's type-2 based on anatomical location at an angle of mandible, its relationship to parotid gland and facial nerve and previous history of ear discharge. To the best of our knowledge, this is the first case of its type to be reported. PMID:24669347

Spontaneous Pneumomediastinum with a Rare Presentation

Directory of Open Access Journals (Sweden)

Ehsan Bolvardi

2014-01-01

Full Text Available Spontaneous pneumomediastinum is an unusual and benign condition in which air is present in mediastinum. A 20-year-old male patient presented to ED with complaint of hoarseness and odynophagia from the day before, after weightlifting. The patient was nonsmoker and denied history of other diseases. On physical examination he had no dyspnea with normal vital signs. Throat examination and pulmonary auscultation were normal and no crepitation was palpable. We could not find subcutaneous emphysema in neck and chest examination. In neck and chest X-ray we found that air is present around the trachea. There was no apparent pneumothorax in CXR. In cervical and chest CT free air was present around trachea and in mediastinum. Subcutaneous emphysema was also evident. But there was no pneumothorax. The patient was admitted and went under close observation, oxygen therapy, and analgesic. The pneumomediastinum and subcutaneous emphysema gradually resolved within a week by conservative therapy and he was discharged without any complication. Many different conditions could be trigged because of pneumomediastinum but it is rarely seen in intense physical exertion such as weightlifting and bodybuilding. Two most common symptoms are retrosternal chest pain and dyspnea. But the patient here complained of hoarseness and odynophagia.

Information presentation in power plant control rooms

International Nuclear Information System (INIS)

Kautto, A.

1984-11-01

The objective of this study is to support operators' work especially in the control rooms of power plant. The exemplified process is a pressurized water (nuclear) reactor (PWR). The man-process interface is an information system that covers information refining, information presentation, information system handling, and process control. THe emphasis in this study is on the organization and presentation of information and on the alert function that is part of the information system. Another goal is to design the alert function so as to radically reduce the number of alarms during plant shutdown, e.g. during the refuelling or maintenance period and during a disturbance. Further, the experimental validation of CFMS (Critical Function Monitoring System), developed by Combustion Engineering, Inc. in the U.S.A. is described briefly. The validation was made at the Loviisa training simulator in the autumn of 1982. CFMS is a safety-related functional alarm system. The functional decomposition of information has turned out to be successful and it is helpful in designing displays. Preliminary criteria for designing displays, the structure of the information presentation system and the illustration of main interactions are presented. General practical ideas on designing the alert function seem very promising. Preliminary results of the CFMS validation are presented. Further, some ideas are presented on how to carry out the analysis and how to make such validations in the future. A new idea for the evaluation of core safety is presented, based on control theory concepts

Present and Future of Nuclear Robotics

International Nuclear Information System (INIS)

Bielza Ciaz-Caneja, M.; Carmena Servet, P.; Gomez Santamaria, J.; Gonzalez Fernandez, J.; Izquierdo Mendoza, J.A.; Linares Pintos, F.; Martinez Gonzalez; Muntion Ruesgas, A.; Serna Oliveira, M.A.

1997-01-01

New technologies have increased the use of robotic systems in fields other than Industry. As a result, research and developers are focusing their interest in concepts like Intelligent Robotics and Robotics in Services. This paper describes the use of Robotics in Nuclear facilities, where robots can be used to protect workers in high radiation areas, to reduce total worker exposure and to minimise downtime. First, the structure of robot systems is introduced and the benefits of nuclear robots is presented. Next, the paper describes some specific nuclear applications and the families of nuclear robots present in the market. After that, a section is devoted to Nuclear Robotics in Spain, with emphasis in some of the developments being carried out at present. Finally, some reflections about the future of robots in Nuclear Industry are offered. (Author) 18 refs

Endometriosis presenting like a psoas abscess

International Nuclear Information System (INIS)

Bhat, S.N.; Mohanty, S.P.; Kustagi, P.

2007-01-01

Endometriosis is the presence of ectopic endometrial tissue that responds to hormonal stimulation. Although endometriosis occurs most often in the pelvis, several unusual sites have been reported. We present a case of endometriosis mimicking, a psoas abscess. A 39-year-old lady presented around the right hip, of 5 years duration. Computed tomography (CT) scan showed a multi-loculated mass in the right iliopsoas muscle. On exploration, a hemorrhagic cystic mass was seen pressing on the femoral nerve. The lesion was excised and histological examination of the tissue showed features of endometriosis. The patient was treated by cyclic hormonal suppressive therapy for 3 months. Her pain and flexion deformity of the hip resolved completely. Retroperitoneal endometriosis presenting with deformity and pain in the hip may mimic a psoas abscess. If the possibility of this diagnosis is entertained, inappropriate treatment may be avoided. (author)

Cancer presenting during pregnancy: radiological perspectives

International Nuclear Information System (INIS)

Doyle, S.; Messiou, C.; Rutherford, J.M.; Dineen, R.A.

2009-01-01

Malignancy presenting during pregnancy is rare. When it does, there are important considerations and challenges for the radiologist. The physiological changes of pregnancy may mask signs and symptoms of malignancy leading to delayed presentation. Endocrine and physiological changes during pregnancy can interact with tumour biology to alter the behaviour and patterns of growth of certain tumours. The timing and choice of imaging technique pose potential risks to the foetus, but this must be weighed against the risks to both mother and foetus of inadequate investigation or misdiagnosis. This review outlines the general principles and approach to imaging the pregnant patient with suspected malignancy, following which there is a more detailed discussion of the effects of pregnancy on tumour biology and presentation of specific tumours. Imaging strategies are discussed for the different entities, and where possible, evidence-based imaging recommendations are made.

Clinical presentation of thyroid cancer

International Nuclear Information System (INIS)

Samuel, A.M.; Shah, D.H.

1999-01-01

The clinical manifestation of thyroid cancer (TC) as seen at the Nuclear Medicine Department, where the patients investigated prior to diagnosis of disease are clinically suspected to harbor malignancy and mostly referred for scintigraphic investigations are presented

Trismus: An unusual presentation following road accident

Directory of Open Access Journals (Sweden)

Thakur Jagdeep

2007-01-01

Full Text Available Trismus due to trauma usually follows road accidents leading to massive faciomaxillary injury. In the literature there is no report of a foreign body causing trismus following a road accident, this rare case is an exception. We present a case of isolated presentation of trismus following a road accident. This case report stresses on the thorough evaluation of patients presenting with trismus following a road accident.

HYDROGEOLOGIC CASE STUDIES (DENVER PRESENTATION)

Science.gov (United States)

Hydrogeology is the foundation of subsurface site characterization for evaluations of monitored natural attenuation (MNA). Three case studies are presented. Examples of the potentially detrimental effects of drilling additives on ground-water samples from monitoring wells are d...

Hydroelectric power in Romania. Past - present - future

International Nuclear Information System (INIS)

Bogan, V.

1996-01-01

This paper presents an analysis of the past and present situation in hydroelectric power (achievements, rates of commissioning and so on) and the future strategies for the hydroelectric power resource development in the conditions of a free market economy. At present the contribution of hydroelectric power in the Romania's total power balance is about 16,500 GW h/year which represents nearly 28 %. The theoretical hydroelectric power potential of Romania is 75,000 GW h/year while the technical potential, which could actually be developed, is only 40,000 GW h/year. Finally, there are presented the main directions in the hydroelectric power development up to the year 2020. (author) 3 tabs

Summary of the presentations and discussions

International Nuclear Information System (INIS)

Massaut, Vincent

2006-01-01

The first two presentations were given by specialists in human science, and focused more on the ethical aspects of the funding for decommissioning. Some new aspects or approaches were presented, which allowed to highlight the egalitarian and utilitarian approaches of inter-generational equity. The different ethical principles led to conclude on the need of a democratic debate on the subject, and the need of ethical guidance at international level. Some other key points, like the preservation of competences, funding and resources, were also considered in the light of the inter-generational community. The session was concluded by a presentation on the application of the ethical principles for the funding. Session 2: Actual experience in funding. The various mechanisms for funding have been analyses on their principle, current practice and merits. Three main aspects were analysed: How funds are raised? How are the funds managed? How to disburse when needed? The actual experience in different countries showed different mechanisms and return of experience. Session 3: Uncertainties. In most large industrial projects (construction, civil works, aerospace...), the return of experience shows that overrun (in time and cost) is a rather general tendency. But it depends on the type of project, on the degree of innovation, etc. Some means to avoid these overrun were presented. In the case of D and D, the main uncertainties affecting funding can be found in: cost estimate, inflow of resources, management of resources, time factor: when will the costs occur? Moreover, these uncertainties sources can also be inter-linked. One of the conclusions from this analysis is that the existing uncertainties in funding are good reasons not to postpone decommissioning operations to a too distant future. Presentations on return of experience were presented by several countries. Plenary discussions: The plenary discussions allowed to tackle the different aspects presented during the day. Some

Presentation of the ASTRAL software

International Nuclear Information System (INIS)

2011-01-01

This report presents the ASTRAL software (ASTRAL means technical assistance in post-accidental radioprotection) which is aimed to be used as an aid-to-decision tool in the case of an important release of radionuclides in the environment by assessing radionuclide concentration in different environments and food products, determining the potential exposure to irradiation of concerned populations, foreseeing the evolution of the situation, and proposing different scenarios for the management of contaminated areas. The report describes the software general operation, presents the calculation module (main functionalities, concentration index calculation, dose calculation or radiological impact calculation, how countermeasures are taken into account), the data bases (contextual data, data for radio-ecological calculations and for radiological calculations), the software ergonomics (general principles, result selection and display, result printing and input data). Its briefly evokes the development quality assurance, and describes the software implementation architecture

Presentations at NuPECC meeting

Energy Technology Data Exchange (ETDEWEB)

NONE

2006-07-01

The Nuclear Physics European Collaboration Committee (NuPECC) is an associated committee of the European Science Foundation. The objective of NuPECC is to strengthen European collaboration in nuclear sciences. In pursuing this objective the committee shall first define a network of complementary facilities within Europe and encourage optimization of their usage and secondly provide a forum for the discussion of future facilities and instrumentation. The first part of this NuPECC meeting was dedicated to the presentation of the main activities of the CENBG (Center for nuclear research of Bordeaux Gradignan), while the second part was devoted to reporting the status of current European collaborations (ECT{sup *}, ECOS, FP7, EURISOL and SPIRAL-2), a survey of operating small scale facilities in Europe is given. This document gathers only the slides presented at this meeting. (A.C.)

Presentation of economic evaluation results.

Science.gov (United States)

Chaikledkaew, Usa

2014-05-01

The first HTA guidelines for Thailand included a chapter outlining a set of guidelines on how best to report the findings of health economic evaluations, based on a review of best practice and existing guidelines on the presentation of economic evaluation results from around the world. In this second edition of HTA guidelines for Thailand, the recommendations build on the first edition by using a case study to illustrate how the guidelines can be applied in a real research context. The guidelines propose that all reporting include ten key elements: defining the scope of the study, selection of comparator(s), defining the type of economic evaluation, measurement of costs, measurement of clinical effects, handling time in economic evaluation studies, handling uncertainty and sensitivity analysis, presentation of the results, discussion of the results, and disclosure of funding and authors conflict of interest.

Presentations at NuPECC meeting

International Nuclear Information System (INIS)

2006-01-01

The Nuclear Physics European Collaboration Committee (NuPECC) is an associated committee of the European Science Foundation. The objective of NuPECC is to strengthen European collaboration in nuclear sciences. In pursuing this objective the committee shall first define a network of complementary facilities within Europe and encourage optimization of their usage and secondly provide a forum for the discussion of future facilities and instrumentation. The first part of this NuPECC meeting was dedicated to the presentation of the main activities of the CENBG (Center for nuclear research of Bordeaux Gradignan), while the second part was devoted to reporting the status of current European collaborations (ECT * , ECOS, FP7, EURISOL and SPIRAL-2), a survey of operating small scale facilities in Europe is given. This document gathers only the slides presented at this meeting. (A.C.)

Extradural lipomatosis presenting with paraplegia.

Directory of Open Access Journals (Sweden)

Deogaonkar M

1995-07-01

Full Text Available An unusual case with spinal extradural lipomatosis in a non-obese and otherwise healthy man is reported. The patient presented with a history of weakness of legs which progressed to paraplegia over a 40 day period.

Presentation Trainer Prototype 1.0

NARCIS (Netherlands)

Börner, Dirk; Specht, Marcus

2014-01-01

This software sketch comprises the first prototype of the presentation trainer. The application uses the Microsoft Kinect sensor and was built using the Processing 1.5.1 development environment. Available under the GNU LGPL licence version 3 or higher.

Nasopharyngeal bursitis: from embryology to clinical presentation

OpenAIRE

El-Shazly, Amr

2010-01-01

AE El-Shazly, S Barriat, PP LefebvreDepartment of Otorhinolaryngology and Head and Neck Surgery, Liege University Hospital, Liege, BelgiumAbstract: Nasopharyngeal bursitis is a relatively rare syndrome characterized by a collection of symptoms that multidisciplinary specialists should be aware of. Here we present an audit of cases presenting to a rhinology clinic over a two-year period, as well as an overview of the relevant embryology and different clinical presentations of nasopharyngeal bu...

Pancreatic ductal adenocarcinoma presenting with acute and chronic pancreatitis as initial presentation: is prognosis better? A comparison study..

Science.gov (United States)

Thorat, Ashok; Huang, Wen-Hsuan; Yeh, Ta-Sen; Jan, Yi-Yan; Hwang, Tsann-Long

2014-10-01

Pancreatic ductal adenocarcinoma (PDAC) may present with acute and /or chronic pancreatitis due to pancreatic ductal obstruction causing diagnostic dilemma. The aim of this retrospective study was to investigate the outcome and prognosis of the patients of PDAC presenting with pancreatitis. From 1991 to 2009, 298 patients with PDAC that underwent surgical treatment were retrospectively studied and divided in two groups depending upon initial symptomatic presentation. Group A (n=254) comprised patients without pancreatitis while group B (n=44) patients presented with acute and/or chronic pancreatitis initially. All the patients in studied cohort were surgically treated. Mean age of group A was 63.1 years & for group B it was 62.9 years. Location of tumor was in head of the pancreas in 66.14% of group A patients (n=168) and 61.36% of group B patients (n=27). Although statistically insignificant, the patients in group B had overall better 5-year survival than the patients in group A (20% vs 15.9%). This retrospective study highlights the overall better survival of PDAC patients presenting with acute and/or chronic pancreatitis than those without as contrary to previous reports which stated the poor prognosis of PDAC patients if associated with underlying pancreatitis.

Storage rings: Past, present and future

International Nuclear Information System (INIS)

Krisch, A.D.

2000-01-01

This lecture will attempt to review storage rings: past, present and future. I will spend more time on the past, because the past has produced most of our data, while the present can be rather brief. There is not yet much future data, but there are some plans about what we hope for. Professor Andy Sessler of Berkeley, who recently reviewed the early history of colliders, loaned me his slides; for this I have much appreciation

Clustering semantics for hypermedia presentation

NARCIS (Netherlands)

Alberink, M.J.; Rutledge, L.W.; Hardman, H.L.; Veenstra, M.J.A.

2004-01-01

Semantic annotations of media repositories make relationships among the stored media and relevant concepts explicit. However, these relationships and the media they join are not directly presentable as hypermedia. Previous work shows how clustering over the annotations in the repositories can

Splenic flexure volvulus presenting with gangrene

International Nuclear Information System (INIS)

Machado, Norman O; Chopra, Pradeep J; Subramanian, Sureshkannan K

2009-01-01

Volvulus of the splenic flexure is very rare cause of colonic obstruction constituting 2% of cases of colonic segmental volvulus. Primary splenic flexure volvulus (SFV) is due to congenital absence or laxity of the phrenocolic, gastro colic, and splenocolic ligaments while secondary volvulus is due to other causes including some prior surgery releasing these ligaments. A preoperative diagnosis can be established based on the characteristic radiological findings on plain x-ray abdomen and CT scan. We present a case of SFV in a young man who presented with acute abdominal pain, and distension, and illustrate the usefulness of CT scan, and plain x-ray of the abdomen in making a preoperative diagnosis. Laparotomy revealed a gangrenous SFV, which was resected and primary anastomosis was carried out. Literature is reviewed with regards to predisposing factors, presentation, investigation, and management among the more than 32 cases reported so far. (author)

Prallethrin Poisoning Presenting as Status Epilepticus

Directory of Open Access Journals (Sweden)

D Vijayaraju

2014-02-01

Full Text Available Mosquito repellents contain pyrethroid derivatives, as the active ingredient. Data regarding human toxicity following oral intake is limited. This is a case report of an individual who presented with neurotoxicity following oral ingestion of prallethrin (2.4% w/w of a higher concentration present in formulations commercially available than previously. A 22 year old male presented to the emergency Room (ER in status epilepticus. He was managed in the Intensive Care Unit (ICU. No cause for his status could be determined after all laboratory investigations. A review of history with the patient revealed that he had ingested a bottle of liquid mosquito repellent (45 ml. The patient has had no further symptoms on follow up and has been off anti epileptics. Pyrethroid containing mosquito repellents act on neuronal sodium channels causing hyperexcitiability. With no known specific antidotes, symptomatic treatment is the main stay of management.

Sarcoidosis Presenting Addison's Disease.

Science.gov (United States)

Takahashi, Kentaro; Kagami, Shin-Ichiro; Kawashima, Hirotoshi; Kashiwakuma, Daisuke; Suzuki, Yoshio; Iwamoto, Itsuo

2016-01-01

We herein describe a second Japanese case of sarcoidosis presenting Addison's disease. A 52-year-old man was diagnosed with sarcoidosis based on clinical and laboratory findings, including bilateral hilar lymphadenopathy and elevated levels of serum angiotensin-converting enzyme and lysozyme, as well as the presence of noncaseating epithelioid granulomas. The patient also exhibited general fatigue, pigmentation, weight loss, hypotension and hyponatremia, suggestive of chronic adrenocortical insufficiency. An endocrine examination confirmed primary adrenocortical insufficiency. This case suggests the direct involvement of sarcoid granuloma in the adrenal glands.

Computer Presentation Programs and Teaching Research Methodologies

Directory of Open Access Journals (Sweden)

Vahid Motamedi

2015-05-01

Full Text Available Supplementing traditional chalk and board instruction with computer delivery has been viewed positively by students who have reported increased understanding and more interaction with the instructor when computer presentations are used in the classroom. Some problems contributing to student errors while taking class notes might be transcription of numbers to the board, and handwriting of the instructor can be resolved in careful construction of computer presentations. The use of computer presentation programs promises to increase the effectiveness of learning by making content more readily available, by reducing the cost and effort of producing quality content, and by allowing content to be more easily shared. This paper describes how problems can be overcome by using presentation packages for instruction.

Clinical presentation of renal cell carcinoma

International Nuclear Information System (INIS)

Rehman, R.A.; Ashraf, S.; Jamil, N.

2015-01-01

Most common malignant tumour of the kidney is Renal Cell Carcinoma (RCC) and is known for its unpredictable clinical behaviour. Aetiology and risk factors are not completely understood. Extensive workup is being done in the understanding of the disease, especially to diagnose early and to treat promptly. The objective of this study was to determine the clinical presentation and pathological pattern of RCC. Methods: After approval from ethical committee a retrospective review of records was conducted extending from January 2012 to January 2014 to identify clinical characteristics of renal cell carcinomas. The study included all renal cancer patients presented to Sheikh Zayed Hospital Lahore with in this specified period. The data was retrieved regarding, history, physical examination and necessary investigations such as ultrasonography of abdomen and pelvis and CT scan of abdomen and pelvis. Results: There were total of 50 cases. The male to female ratio was 3:2. Mean age of patients were 52.38 (18-93) years old. Most common clinical presentation was gross haematuria(66%).The mean tumour size was 8.34 (3-24) cm. Tumour histology were clear cell (84%), papillary transitional cell carcinoma (12%) and oncosytoma contributed 4%. Conclusion: We observed that large number of the patients with RCC presented with haematuria and most of them were male. Common pathological type was clear cell carcinoma. (author)

An Unusual Presentation of Myasthenia Gravis | Chijioke | West ...

African Journals Online (AJOL)

BACK GROUND: Myasthenia gravis (MG) is generally a rare disorder and may thus be easily misdiagnosed. Misdiagnosis is even more likely when the presentation is atypical. OBJECTIVE: To present and discuss an unusual presentation of myasthenia gravis METHOD: A 67-year-old man presented with progressive ...

Trichinosis - case presentation

Directory of Open Access Journals (Sweden)

Rajko Saletinger

2007-03-01

Full Text Available Background: Trichinosis is a widespread zoonosis acquired by ingestion of undercooked meat infected with the larvae of Trichinella parasites. The disease has variable clinical manifestations ranging from asymptomatic to fatal. Majority of infections is caused by Trichinella spiralis, although there are some cases caused by other species. The dramatic decline of trichinosis reflects changes in industrial practices and increased public awareness. The majority of human cases are now associated with wild game meat and noncommercial pork. The case of previously healthy, young man with trichinosis is presented. The disease was determined with serologic testing and cured with albendazole. The source of infection has not been determined.Conclusions: In patients with fever, mialgias and eosinophilia infection with tissue nematodes should always be taken in consideration. In those who have recently consumed raw or undercooked pork and wild game meat we should think about trichinosis. The disease is rare in Slovenia.

Presentation of Pydio

CERN Multimedia

CERN. Geneva

2014-01-01

**Pydio is an open-source platform for file sharing and synchronisation.** It is wildly used by enterprises and organizations worldwide, including major universities. Pydio comes with a rich web-based interface, native clients for both iOS and Android mobile devices, and lately a new sync client for desktop platforms. Pydio v6 is of great useability and introduces many new features that makes it the ultimate sharing machine. Its architecture makes it a perfect fit for either easily deploying an on-premise dropbox solution, or building more complicated solutions where the box feature is integrated as OEM. Storage-agnostic, Pydio is a simple layer that can be deployed on top of any storage backend, thus providing scalability and high-availability out-of-the-box. Charles du Jeu is the lead developer of the solution and will briefly present its feature and how it can fit for research and engineering purpose. See https://pyd.io/ for more information.

DOE Collegiate Wind Competition (Presentation)

Energy Technology Data Exchange (ETDEWEB)

Jones, J.

2014-02-01

This presentation for the January Stakeholder Engagement and Outreach webinar outlines the expanded need for workers in the wind industry and provides an overview of the DOE Wind Competition (to be held in May 2014) and the guiding principles of the competition.

Experiments in Multimodal Information Presentation

NARCIS (Netherlands)

van Hooijdonk, Charlotte; Bosma, W.E.; Krahmer, Emiel; Maes, Alfons; Theune, Mariet; van den Bosch, Antal; Bouma, Gosse

In this chapter we describe three experiments investigating multimodal information presentation in the context of a medical QA system. In Experiment 1, we wanted to know how non-experts design (multimodal) answers to medical questions, distinguishing between what questions and how questions. In

The Present State of Behaviorism

Science.gov (United States)

Mowrer, O. Hobart

1976-01-01

Assesses the present status of behaviorism by dividing this study into an evaluation of Behaviorism 1, based explicitly on Pavlov's conception of conditioned-response learning, and Behaviorism 11, single-handedly launched and almost completely dominanted by Professor B. F. Skinner. (Author/RK)

Hypervideos and interactive multimedia presentations

NARCIS (Netherlands)

B. Meixner (Britta)

2017-01-01

textabstractHypervideos and interactive multimedia presentations allow the creation of fully interactive and enriched video. It is possible to organize video scenes in a nonlinear way. Additional information can be added to the video ranging from short descriptions to images and more videos.

DOE financial assurance presentation

International Nuclear Information System (INIS)

Huck, R.

1990-01-01

The presentation topic is California's approach to license application review in meeting financial assurances for the proposed Ward Valley site. The purpose of the presentation is to provide information on specific financial assurance provisions contained in 10 CFR Part 61 and how California intends to satisfy those requirements. Also, as rate setter, California intends to demonstrate how it will assure allowable costs to the rate base though a financial prudency review. The key provisions of financial assurance are: 10 CFR Section 61.61 - This provision requires an applicant to demonstrate its ability to finance licensed activities; 10 CFR Section 61.62 - This provision requires an applicant to provide assurance that sufficient funds will be available for site closure and stabilization; and 10 CFR Section 61.63 - This provision requires an applicant to provide 'a copy of a binding arrangement, such as a lease, between the applicant and the disposal site owner, so that sufficient funds will be available to cover the costs of the institutional control period.' To assist California in its determination of financial assurance compliance to be demonstrated by the applicant for Part 61 requirements, is NUREG guidance document 1199 'Standard Format and Content of a License Application for a Low-Level Radioactive Waste (LLRW) Disposal Facility.' The detailed financial assurance provisions of NUREG 1199 are then embodied in NUREG 1200, 'Standard Review Plant for the Review of a License Application for a LLRW Disposal Facility.'

A unique presentation of retroclival chordoma.

Directory of Open Access Journals (Sweden)

Warakaulle D

2002-10-01

Full Text Available Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.

Computing nilpotent quotients in finitely presented Lie rings

Directory of Open Access Journals (Sweden)

Csaba Schneider

1997-12-01

Full Text Available A nilpotent quotient algorithm for finitely presented Lie rings over Z (and Q is described. The paper studies the graded and non-graded cases separately. The algorithm computes the so-called nilpotent presentation for a finitely presented, nilpotent Lie ring. A nilpotent presentation consists of generators for the abelian group and the products expressed as linear combinations for pairs formed by generators. Using that presentation the word problem is decidable in L. Provided that the Lie ring L is graded, it is possible to determine the canonical presentation for a lower central factor of L. Complexity is studied and it is shown that optimising the presentation is NP-hard. Computational details are provided with examples, timing and some structure theorems obtained from computations. Implementation in C and GAP interface are available.

[Umbilical blood-gas status at cesarean section for breech presentation: a comparison with vertex presentation].

Science.gov (United States)

Haruta, M; Saeki, N; Naka, Y; Funato, T; Ohtsuki, Y

1989-10-01

Umbilical blood-gas status at elective cesarean section with oxygen inhalation for breech presentation (25 cases) was compared with that for vertex presentation (25 cases), so as to confirm the security of full-term breech fetuses delivered by cesarean section under spinal anesthesia. Umbilical arterial oxygen levels were significantly lower in the breech group (Mean PO2:18.9 mmHg; SO2:37.3%; Oxygen content:7.6 ml/dl). The number of hypoxemic fetuses was significantly higher in the breech group (the breech: 7; the vertex; 0). The other umbilical blood-gas values revealed no significant differences between the breech and vertex groups, and were within normal limits in both groups. Oxygen extraction in the breech (Mean: 49.0%) was higher than that in the vertex (32.9%). Therefore decreased umbilical blood flow in the breech was suggested. The incidence of depression at 1 minute after delivery in the breech infants (24%) was significantly higher than that in the vertex infants (0%). It became obvious in the breech that as the interval between the uterine incision and delivery increased, umbilical arterial blood tended to acidosis and the 1 minute Apgar score decreased. Cesarean section for breech presentation requires sufficient and optimal incisions of the abdominal wall and uterus as well as a skillful manual delivery technique, because the fetus or neonate should be protected against asphyxia resulting from umbilical compression and prolonged delivery interval.

Radiation myelopathy (case presentation)

International Nuclear Information System (INIS)

Sangheli, M.; Lisnic, V.; Plesca, S.; Odainic, O.; Chetrari, L.

2009-01-01

The radiotherapy is one of the most widely spread and commonly used method in treating tumors of different localization. A detailed analysis of benefits and possible side effects along with evaluation of the risk factors allows preventing one of the most difficult complication, and namely the radiation myelopathy. The delayed form of such a disease is distinguished by a pronounced unfavorable prognosis. The presented case provoked the discussion of difficulties in performing differential diagnosis, as well as the importance of determining the localization of vulnerable tissues as regards the target organ exposed to radiotherapy. (authors)

Fibrolamellar carcinoma: an unusual clinico-radiological presentation

International Nuclear Information System (INIS)

Gupta, Preeti; Dhar, Sunanda; Strickland, Nicola H.

1999-01-01

We report a case of fibrolamellar carcinoma presenting in a 26-year old female. The patient had gross ascites at presentation. Imaging studies (ultrasonography and computed tomography) strongly suggested extensive intraperitoneal drop metastases

Unilateral Congenital Cataract: Clinical Profile and Presentation.

Science.gov (United States)

Khokhar, Sudarshan; Jose, Cijin P; Sihota, Ramanjit; Midha, Neha

2018-03-01

To study the clinical profile and presentation of children with unilateral cataract. In this hospital-based, observational, cross-sectional study, patients 15 years of age or younger who presented with unilateral cataract were recruited. Cases of cataract secondary to causes such as trauma or uveitis were excluded. Age at detection and presentation, distance from the treatment center, presenting complaints, cataract morphology, and biometry were noted for each case. A total of 76 patients were recruited. Most patients presented with complaints of leukocoria. Persistent fetal vasculature accounted for 27.6% of cases and was the most common identifiable cause of cataract in this study. Subsequently, patients were divided into two groups: no persistent fetal vasculature (control) and persistent fetal vasculature. A male predominance was noted in both groups. The mean age at detection was 27.58 ± 37.02 and 6.17 ± 8.42 months and the mean age at presentation was 55.613 ± 45.21 and 14.83 ± 17.75 months in the control and persistent fetal vasculature groups, respectively. In the persistent fetal vasculature group, a significant difference was noted in the axial length, keratometry, and corneal diameter between the affected and normal eyes (P = .027, .00176, and .0114, respectively). In the control group, this difference was observed only in keratometry readings (P = .0464). The mean distance traveled by patients to reach the treatment center was 211 km. Persistent fetal vasculature is an important and less identified cause of unilateral cataract. A significant delay is noted in the detection and presentation of unilateral cataract. [J Pediatr Ophthalmol Strabismus. 2018;55(2):107-112.]. Copyright 2017, SLACK Incorporated.

Extrapontine myelinolysis presenting as acute parkinsonism

Directory of Open Access Journals (Sweden)

Ditchfield A

2006-09-01

Full Text Available Abstract Background Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated co-morbidity. Case presentation A 41-year-old woman presented with acute parkinsonism like features while on a holiday. This followed slow correction of hyponatraemia after repeated vomiting. MRI changes were suggestive of Extrapontine myelinolysis(EPM. This case is at variance with four previous cases reported in the medical literature in that the patient made a full clinical recovery and the MR changes resolved with symptomatic support alone. Conclusion Extrapontine myelinolysis could make a complete recovery with symptomatic support alone. During hyponatraemia correction, rapid osmotic shifts of fluid that cause hypernatremia, causes myelinolysis rather than absolute serum sodium level. Even gradual correction of hyponatraemia can produce myelinolysis, especially with pre-existing malnourishment, alcoholism, drug misuse, Addison's disease and immuno-suppression. Pallidial sparing is typical of EPM in MRI scans.

Typical and atypical presentations of aspergilloma

International Nuclear Information System (INIS)

Villajos, M.; Darnell, A.; Gallardo, X.; Castaner, E.; Mata, J. M.; Paedavila, E.

1999-01-01

To show the different forms of radiological presentations of aspergilloma, emphasizing the importance of recognizing the atypical forms. The explorations of 11 patients with aspergilloma were examined retrospectively between 1993 and 1997. These patients were studied using conventional X-rays and computed tomography (CT): Typical and atypical radiological findings were observed. In two patients, who presented recurrent hemoptysis, a percutaneous installation of amphotericin B was carried out with tomographic control. Out of the 11 patients, two were female and nine male. In eight of the cases the radiological findings showed an intercavity injury with different evolutionary forms, while in three of the cases there was a progressive pleural swelling. In the two patients treated pertinaciously, no significant radiological changes were observed, however, neither of them showed hemoptysis again. The pleural swelling adjacent to the cavity and/or the swelling of the cavity wall are atypical radiological presentations of the aspergilloma, that can accompany or precede the appearance of this illness. (Author) 7 refs

Depressive self-presentation: beyond self-handicapping.

Science.gov (United States)

Weary, G; Williams, J P

1990-05-01

An experiment was conducted to examine the notion that depressives' responses would reflect a protective self-presentation style (Hill, Weary, & Williams, 1986), the underlying goal of which would be the avoidance of future performance demands and potential losses in self-esteem. In this study, depressed and nondepressed Ss were asked to perform a relatively simple visual-motor task. Half of the depressed and half of the nondepressed Ss were told that if they were successful at the task, they would be asked to perform a 2nd, similar task. The remaining Ss were given no such expectation of future performance. We predicted and found that depressed compared with nondepressed Ss strategically failed at the task when presented with the possibility of future performance and further losses in esteem. Moreover, this strategic failure was associated with some costs; depressed-future performance expectancy Ss experienced more discomfort or negative affect as a result of their performance. The relationship between this depressive self-presentation and self-handicapping strategies is discussed.

Guillain-Barre Syndrome Presenting as Acute Abdomen

Directory of Open Access Journals (Sweden)

Faruk incecik

2015-09-01

Full Text Available Guillain-Barr and eacute; syndrome (GBS is the most common cause of acute flaccid paralysis in childhood. Symmetric weakness, headache, respiratory symptom, neuropathic pain, muscle pain, paresthesia, and facial palsy were the most common clinical presentations. We report 13-year-old boy with GBS who presented with acute abdominal pain. This is the first report, to our knowledge, first presented of acute abdomen of a pediatric patient with GBS. [Cukurova Med J 2015; 40(3.000: 601-603

Antenatal hemorrhage of a cervical lymphatic malformation presenting as a draining neck mass: An unusual presentation.

Science.gov (United States)

Haricharan, R N; Nawaz, M; Bettolli, M; Ferretti, E

2014-01-01

Lymphatic malformations in the neck can present as large fetal neck masses causing airway obstructions with potential perinatal demise and can pose a therapeutic challenge. We present a rare case of prenatally diagnosed large fetal neck mass with features of lymphatic malformation with intralesional hemorrhage of uncertain origin. Postnatal evaluation showed a complex cystic-solid lesion eroding through the skin with an open wound that made it clinically hard to differentiate from a teratoma. Given that malignancy could not be completely ruled out, surgery was favored. Final pathology showed a complex lymphatic malformation with intralesional hemorrhage, despite having no associated capillary, venous or arterial malformations.

Reactor enclosure. BRC meeting presentation

International Nuclear Information System (INIS)

Fisch, J.W.

1975-01-01

The latest status of key components of the Reactor Enclosure System of the Clinch River Breeder Reactor Plant is described. Areas where there have been notable design changes or significant design detail maturity in the six months since the last BRC presentation are highlighted. (auth)

Petroleum - its present and future

Digital Repository Service at National Institute of Oceanography (India)

SenGupta, R.; Qasim, S.Z.

World's "published proved" reserve of oil by the end of 1984 was known to be of the order of 95,000 million tonnes. Of this, 57% lies buried in the middle eastern countries. At the present rate of growth in consumption and production of oil...

Reninoma presenting as cardiac syncope

Science.gov (United States)

Tak, Shahid I; Wani, Mohd Lateef; Khan, Khursheed A; Alai, Mohd Sultan; Shera, Altaf Hussain; Ahangar, Abdul G; Khan, Yasir Bashir; Nayeem-ul-Hassan; Irshad, Ifat

2011-01-01

Reninoma, a renin-secreting tumor of the juxta-glomerular cells of the kidney, is a rare but surgically treatable cause of secondary hypertension in children. We report a case of reninoma presenting as cardiac syncope with long QTc on electrocardiogram due to hypokalemia. PMID:21677812

Police, managerialization and presentational strategies

NARCIS (Netherlands)

Terpstra, J.; Trommel, W.A.

2009-01-01

Purpose - The managerialization of the police may be seen as an effort to restore the legitimacy of the police. This paper aims to show that the managerialist strategy presently occurring within police forces creates new pitfalls and unintended consequences. Design/methodology/approach - The paper

BVDV: past, present, and future

Science.gov (United States)

The term bovine viral diarrhea (BVD) has come to refer to a collection of diverse clinical presentations that include respiratory, enteric and reproductive diseases accompanied by immunosuppression. BVD may be caused by one of three different species of bovine pestivirus, bovine viral diarrhea viru...

2016 Annual Technology Baseline (ATB) - Webinar Presentation

Energy Technology Data Exchange (ETDEWEB)

Cole, Wesley; Kurup, Parthiv; Hand, Maureen; Feldman, David; Sigrin, Benjamin; Lantz, Eric; Stehly, Tyler; Augustine, Chad; Turchi, Craig; Porro, Gian; O' Connor, Patrick; Waldoch, Connor

2016-09-13

This deck was presented for the 2016 Annual Technology Baseline Webinar. The presentation describes the Annual Technology Baseline, which is a compilation of current and future cost and performance data for electricity generation technologies.

Marfan's syndrome presenting with abdominal aortic aneurysm: A ...

African Journals Online (AJOL)

We present the case of a 16-year old student with Marfan's syndrome and abdominal aortic aneurysm who presented with a diagnostic conundrum. He presented with a three months history of progressive painful left upper abdominal mass and back pain. It became severe in the last two weeks before presentation and was ...

An Atypical Presentation Of Pancreatic Carcinoma With Malignant ...

African Journals Online (AJOL)

Background: Pleural effusion secondary to pancreatic tumour is a relatively rare clinical presentation. Aim of study: To present a case of a patient that had pancreatic tumour who presented with features of left sided pleural effusion with a view to highlighting the clinical features of the presentation. Method of study: The case ...

Melanoacanthoma: Uncommon presentation of an uncommon condition

Directory of Open Access Journals (Sweden)

Resham J Vasani

2013-01-01

Full Text Available Melanoacanthoma is very rare variant of seborrheic keratosis presenting as a deeply pigmented benign proliferation of melanocytes and keratinocytes usually presenting over the head, neck and trunk of elderly people. A sixty-two-years-old male was presented with a solitary slow growing asymptomatic hyperpigmented verrucous outgrowth with cerebriform surface measuring 15 cm by 8 cm present over the left inguinal region extending on to the scrotum since past 8 years. There was no associated lymphadenopathy. The histopathology revealed hyperkeratosis, papillomatosis, acanthosis with presence of melanocytes at all levels of epidermis with abundant melanin giving the diagnosis of melanoacanthoma. The patient further underwent surgical excision of the lesion. The case is being reported for its rarity, unusual location, massive size and clinical resemblance to a verrucous carcinoma.

Late presentation for HIV care across Europe

DEFF Research Database (Denmark)

Mocroft, Amanda; Lundgren, Jens; Antinori, Andrea

2015-01-01

Late presentation (LP) for HIV care across Europe remains a significant issue. We provide a cross-European update from 34 countries on the prevalence and risk factors of LP for 2010-2013. People aged ≥ 16 presenting for HIV care (earliest of HIV-diagnosis, first clinic visit or cohort enrollment......) after 1 January 2010 with available CD4 count within six months of presentation were included. LP was defined as presentation with a CD4 count HIV diagnosis. Logistic regression investigated changes in LP over time. A total.......02-1.32), and a significant decline in LP in northern Europe (aOR/year later 0.89; 95% CI: 0.85-0.94). Further improvements in effective HIV testing strategies, with a focus on vulnerable groups, are required across the European continent....

Presentation and Reception of the Rumor

Directory of Open Access Journals (Sweden)

Valdas Pruskus

2011-04-01

Full Text Available The article analyses the process of how an informal information becomes a rumor. It is possible only in a certain situation. It is called rumor situation. It has two main conditions: right place where people can share informal information; and a wish to get additional information about a person, an event or a phenomenon interesting for an individual. Information presentation is conceived as a process of three stages. First stage – pre–interaction stage: a conveyor contacts the talker and clears up his preferences. Second stage – presentation of information (content of rumor. In this stage special attention is paid to: 1 rumors “quality” which influences its acceptability and  2 tactics of rumor information presentation. There are five criteria of rumor quality: Information should be presented as special communication and confidence value; Information should not contradict the objective and obvious heart of the matter; Information should be presented in understandable and popular forrm; Information should raise a wish to find out more details and particularities; Information should be characteristic. On the ground of above-mentioned criteria it is possible to valuate acceptability of rumor to information receiver who sometimes turns to be especially finicky and critical user. In order to clear these obstacles two fold tactics is used. First, criticism towards presented information should be neutralized using active and passive ways of rumor offering. Second, interest of a receiver should be stimulated proportionally and information presented considering a receiver’s psychological type. Third stage – “exit” from a rumor: rumor unlike other forms of communication has no accomplished mechanism because is permanently “renewed”. Usually rumor dies when a person ends it voluntarily. However, it is not simply to end the game of a rumor. All the participants of rumor cycle (conveyor – receiver – conveyor do not want or, in

Cephalic version by moxibustion for breech presentation.

Science.gov (United States)

Coyle, Meaghan E; Smith, Caroline A; Peat, Brian

2012-05-16

Moxibustion (a type of Chinese medicine which involves burning a herb close to the skin) to the acupuncture point Bladder 67 (BL67) (Chinese name Zhiyin), located at the tip of the fifth toe, has been proposed as a way of correcting breech presentation. To examine the effectiveness and safety of moxibustion on changing the presentation of an unborn baby in the breech position, the need for external cephalic version (ECV), mode of birth, and perinatal morbidity and mortality for breech presentation. We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (26 March 2012), MEDLINE (1966 to 1 August 2011), EMBASE (1980 to August 2011), CINAHL (1982 to 1 August 2011), MIDIRS (1982 to 1 August 2011) and AMED (1985 to 1 August 2011) and searched bibliographies of relevant papers. The inclusion criteria were published and unpublished randomised controlled trials comparing moxibustion (either alone or in combination with acupuncture or postural techniques) with a control group (no moxibustion), or other methods (e.g. external cephalic version, acupuncture, postural techniques) in women with a singleton breech presentation. Two review authors independently assessed eligibility and trial quality and extracted data. The outcome measures were baby's presentation at birth, need for external cephalic version, mode of birth, perinatal morbidity and mortality, maternal complications and maternal satisfaction, and adverse events. Six new trials have been added to this updated review. One trial has been moved to studies awaiting classification while further data are being requested. This updated review now includes a total of eight trials (involving 1346 women). Meta-analyses were undertaken (where possible) for the main and secondary outcomes. Moxibustion was not found to reduce the number of non-cephalic presentations at birth compared with no treatment (P = 0.45). Moxibustion resulted in decreased use of oxytocin before or during labour for women who had vaginal

Rose breeding: past, present, prospects

NARCIS (Netherlands)

Vries, de D.P.; Dubois, L.A.M.

1996-01-01

In this review the PAST, PRESENT and PROSPECT will be considered as three separate periods in the history of the breeding and development of rose cultivars. The recurring theme is the genetic variation. This theme was chosen because there is justified doubt as to sufficient genetic variation

Unusual presentation of gallbladder perforation

Directory of Open Access Journals (Sweden)

G. Jayasinghe

2016-01-01

Conclusions: Percutaneous abscesses arising from the gallbladder are a rare but potentially serious consequence of acute cholecystitis, and may present in a wide variety of locations. Therefore it is imperative to conduct a full body inspection in the septic patient, even when a source has been identified.

Tubo-Ovarian Presentation of Burkitt's Lymphoma: Case Report ...

African Journals Online (AJOL)

Burkitt's lymphoma rarely presents as a primary of the ovary. High index of suspicion is required to avoid delay of definitive management. There are a few case reports presented on ovarian Burkitt's lymphoma. We present a case of a 23 year old, para 1+1 HIV negative patient who presented to the Kenyatta National Hospital ...

Present studies on the radio-sterilized drugs

International Nuclear Information System (INIS)

Marciniec, B.; Dettlaff, K.

2007-01-01

Lecture presents present status of radiosterilization of drugs and medical materials as compared to other sterilization methods. Literature review is shown on degradation of different kinds of drugs sterilized upon action of the sterilising doses of radiation

Extramedullary leukemia in children presenting with proptosis

Directory of Open Access Journals (Sweden)

Naik Milind

2009-01-01

Full Text Available Abstract Background We highlight the orbital manifestations of acute myeloid leukemia and the role of peripheral blood smear in the diagnosis of these cases. A total of 12 patients who presented with proptosis and were subsequently diagnosed to have acute myeloid leukemia based on incision biopsy or peripheral blood smear were included in the study. Results A retrospective review of all cases of acute myeloid leukemia presenting to the Orbital clinic was performed. The age at presentation, gender, presenting features, duration of symptoms and fundus features were noted. In addition the temporal relationship of the orbital disease to the diagnosis of leukemia, laterality, location of the orbital mass, imaging features and the diagnostic tools used to diagnose leukemia were noted. The median age at presentation was 6 years. The male: female ratio was 0.7:1. None of these patients had been diagnosed earlier as having acute myeloid leukemia. The presenting features included proptosis in all patients, orbital mass in 5 (41.7%, visual symptoms in 2 (16.7% and subconjunctival hemorrhage in one patient (8.3%. A diagnosis of acute myeloid leukemia was established by incision biopsy in 4 patients, subsequently confirmed by peripheral blood smear testing and bone marrow biopsy in 2 patients which revealed the presence of systemic involvement. Imprint smears of the biopsy identified blasts in 2 of 4 cases. In 8 patients presenting with ocular manifestations, diagnosis was established by peripheral blood smear examination alone which revealed a diagnosis of acute myeloid leukemia. Conclusion A peripheral blood smear should be performed in all cases of sudden onset proptosis or an orbital mass in children and young adults along with an orbital biopsy. It can always be complemented with a bone marrow biopsy especially in cases of aleukemic leukemia or when the blood smear is inconclusive.

Myasthenia Gravis: Unusual Presentations and Diagnostic Pitfalls.

Science.gov (United States)

Rodolico, Carmelo; Parisi, Daniela; Portaro, Simona; Biasini, Fiammetta; Sinicropi, Stefano; Ciranni, Annamaria; Toscano, Antonio; Messina, Sonia; Musumeci, Olimpia; Vita, Giuseppe; Girlanda, Paolo

2016-08-30

Myasthenia gravis (MG) is an autoimmune disorder presenting with fluctuating, fatigable muscle weakness. Initial symptoms classically involve ocular and proximal limb muscles. Rarely, MG may onset with unusual features, so it can be misdiagnosed with other neuromuscular diseases. To describe unusual and atypical presentations of MG in a large cohort of patients, considering and discussing diagnostic difficulties and pitfalls. We report on 21 out of 508 MG patients, coming to our department in the last 27 years and presenting with atypical or unusual features. The diagnosis was achieved performing a careful clinical examination, a proper neurophysiological assessment, the neostigmine test, the AChR and MuSK antibodies assay and chest CT-scan. Patients with atypical/unusual MG onset were the 4.4% of all MG patients population. We describe seven different clinical categories: asymmetric distal upper limbs weakness, foot drop, isolated triceps brachii weakness and foot drop, post exertional axial weakness with dropped head, acute facial dyplegia, limb-girdle MG and MG with sudden lower limbs weakness and recurrent falls. Atypical and unusual presentations may increase the risk to misdiagnose or delay MG diagnosis. Isolated limb-girdle presentation is the most frequent atypical form in our series.

Immune recovery after starting ART in HIV-infected patients presenting and not presenting with tuberculosis in South Africa.

Science.gov (United States)

Schomaker, Michael; Egger, Matthias; Maskew, Mhairi; Garone, Daniela; Prozesky, Hans; Hoffmann, Christopher J; Boulle, Andrew; Fenner, Lukas

2013-05-01

We studied the immune response after starting antiretroviral treatment (ART) in 15,646 HIV-infected patients with or without tuberculosis (TB) at presentation in 3 ART programs in South Africa between 2003 and 2010. Patients presenting with TB had similar increases in CD4 cells compared with all other patients (adjusted difference 4.9 cells/µL per 6 months, 95% confidence interval: 0.2 to 9.7). Younger age, advanced clinical stage, female sex, and lower CD4 cell count at ART start were all associated with steeper CD4 slopes. In South Africa, HIV-infected patients presenting with TB experience immune recovery after starting ART that is no worse than in other patients.