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Sample records for supraglottic dysgenesis presenting

  1. An Unusual Presentation of 46,XY Pure Gonadal Dysgenesis: Spontaneous Breast Development and Menstruation.

    Science.gov (United States)

    Çatlı, Gönül; Alparslan, Caner; Can, P Şule; Akbay, Sinem; Kelekçi, Sefa; Atik, Tahir; Özyılmaz, Berk; Dündar, Bumin N

    2015-06-01

    46,XY pure gonadal dysgenesis (Swyer syndrome) is characterized by normal female genitalia at birth. It usually first becomes apparent in adolescence with delayed puberty and amenorrhea. Rarely, patients can present with spontaneous breast development and/or menstruation. A fifteen-year-old girl presented to our clinic with the complaint of primary amenorrhea. On physical examination, her external genitals were completely female. Breast development and pubic hair were compatible with Tanner stage V. Hormonal evaluation revealed a hypergonadotropic state despite a normal estrogen level. Chromosome analysis revealed a 46,XY karyotype. Pelvic ultrasonography showed small gonads and a normal sized uterus for age. SRY gene expression was confirmed by multiplex polymerase chain reaction. Direct sequencing on genomic DNA did not reveal a mutation in the SRY, SF1 and WT1 genes. After the diagnosis of Swyer syndrome was made, the patient started to have spontaneous menstrual cycles and therefore failed to attend her follow-up visits. After nine months, the patient underwent diagnostic laparoscopy. Frozen examination of multiple biopsies from gonad tissues revealed gonadoblastoma. With this report, we emphasize the importance of performing karyotype analysis, which is diagnostic for Swyer syndrome, in all cases with primary or secondary amenorrhea even in the presence of normal breast development. We also suggest that normal pubertal development in patients with Swyer syndrome may be associated with the presence of a hormonally active tumor.

  2. The evaluation and presentation of a rare case of gonadal dysgenesis

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    Ghafarnejad M

    1996-07-01

    Full Text Available A seventeen-years old woman was presented with infertility and primary amenorrhea. She had normal stature, femate phenotype and normal development of breasts, external genitalia, vagina and cervix. Pelvic examination showed a large lobulated mass. On sonography there was a mass with probable origin of ovary. Paraclinic tests were carried out. Gonadotropins were in postmeno pausal limits. Alpha Fetoprotein, Beta Subunit (B-HCG assay were normal Laparotomy revealed a gonadat mass on right side, normal uterus and left streak gonad. Pathologic report of tumor was dysgerminoma and teratoma. Due to pathology of tumor and Y chromosome, we advised the patient to remove the other streak gonad and have irradiatio

  3. Glottic ansd supraglottic carcinoma

    International Nuclear Information System (INIS)

    Acht, M.J.J. van; Dolsma, W.V.; Hulshof, J.H.; Leer, J.W.H.; Hermans, J.

    1989-01-01

    From 1971 through 1982, 442 patients with laryngeal carcinoma were seen at the Leiden University Hospital. They were treated either with radiotherapy alone, sandwich therapy (pre- and postoperative radiotherapy) or by surgery followed by postoperative irradiation. Three hundred and sixty-six patients with glottic or supraglottic tumours could be analysed with respect to two different treatments, complications of treatment and some prognostic factors. Two endpoints of analysis were used: disease-free interval and survival to cfancer death. In patients with glottic or supraglottic carcinoma, the survival of patients with advanced disease, treated with radiotherapy only, was worse as compared to the survival of the same category of patients who were treated with sandwich therapy (p<0.005). In small supraglottic tumours, the survival with both therapy policies was equal. There was no influence on prognosis of histological differentiation of the tumour. It appeared that interruption of radiotherapy for more tah two days had an adverse effect on survival in patients with glottic carcinoma (p=0.0001). (author). 16 refs.; 4 figs.;

  4. Spinal segmental dysgenesis

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    N Mahomed

    2009-06-01

    Full Text Available Spinal segmental dysgenesis is a rare congenital spinal abnormality , seen in neonates and infants in which a segment of the spine and spinal cord fails to develop normally . The condition is segmental with normal vertebrae above and below the malformation. This condition is commonly associated with various abnormalities that affect the heart, genitourinary, gastrointestinal tract and skeletal system. We report two cases of spinal segmental dysgenesis and the associated abnormalities.

  5. Testicular dysgenesis syndrome

    DEFF Research Database (Denmark)

    Skakkebaek, N E; Rajpert-De Meyts, E; Main, K M

    2001-01-01

    Numerous reports have recently focused on various aspects of adverse trends in male reproductive health, such as the rising incidence of testicular cancer; low and probably declining semen quality; high and possibly increasing frequencies of undescended testis and hypospadias; and an apparently...... summarizes existing evidence supporting a new concept that poor semen quality, testis cancer, undescended testis and hypospadias are symptoms of one underlying entity, the testicular dysgenesis syndrome (TDS), which may be increasingly common due to adverse environmental influences. Experimental...

  6. Airway management of a rare huge-size supraglottic mass

    International Nuclear Information System (INIS)

    Abou-Zeid, Haitham A.; Al-Ghamdi, Abdel Mohsin A.; Al-Qurain, Abdel-Aziz A.; Mokhazy, Khalid M.

    2006-01-01

    Laser excision of a huge-sized supraglottic mass nearly obstructing the airway passage is a real challenge to anesthesiologists. Upper airway obstruction due to neoplasm in supraglottic region, is traditionally managed by preoperative tracheostomy, however, such a common procedure can potentially have an impact on long-term outcome. A 26-year-old patient presented with dysphagia caused by left cystic vallecular synovial sarcoma. The airway was successfully secured via fiberoptic bronchoscopy, followed by excision of the supraglottic tumor with CO2 laser surgery. Tracheostomy was not required. The patient was discharged from the hospital on the 4th day of surgery. This case, highlights the possibility to secure the airway passage without performing preoperative tracheostomy resulting in good outcome and short hospital stay. (author)

  7. Curative radiotherapy of supraglottic cancer

    International Nuclear Information System (INIS)

    Kim, Yong Ho; Chai, Gyu Young

    1998-01-01

    The purpose of this study was to evaluate the efficacy of curative radiotherapy in the management of supraglottic cancer. Twenty-one patients with squamous cell carcinoma of the supraglottis were treated with radiotherapy at Gyeongsang National University Hospital between 1990 and 1994. Median follow-up period was 36 months and 95% were observed for at least 2 years. Actuarial survival rate at 5 years was 39.3% for 21 patients. The 5-year actuarial survival rate was 75.0% in Stage I, 42.9% in Stage II, 33.3% in Stage III, and 28.6% in Stage IV(p=0.54). The 5-year local control rate was 52.0% for 21 patients. The 5-year local control rate was 75.0% in Stage I, 57.1% in Stage II, 66.7% in Stage III, and 28.6% in Stage IV(p=0.33). Double primary cancer was developed in 3 patients and those were all esophageal cancers. In early stage(Stage I and II) supraglottic cancer, curative radiotherapy would be a treatment of choice and surgery would be better to be reserved for salvage of radiotherapy failure. In advanced stage(Stage III and IV), radiotherapy alone is inadequate for curative therapy and combination with surgery should be done in operable patients. This report emphasizes the importance of esophagoscopy and esophagogram at the follow-up of patients with supraglottic cancer

  8. CT manifestations of ileal dysgenesis

    Energy Technology Data Exchange (ETDEWEB)

    Oberhelman, Amy P.; Herman, Thomas E.; McAlister, William H. [St. Louis Children' s Hospital, Mallinckrodt Institute of Radiology, Washington University School of Medicine, Department of Radiology, St. Louis, MO (United States); Keating, James P. [St. Louis Children' s Hospital, Mallinckrodt Department of Pediatrics, Washington University School of Medicine, Division of Gastroenterology, St. Louis, MO (United States); Rollins, Michael D.; Dillon, Patrick A. [St. Louis Children' s Hospital, Department of Surgery, Washington University School of Medicine, St. Louis, MO (United States)

    2007-02-15

    Ileal dysgenesis is an uncommon condition of unknown etiology occurring in the distal ileum in the region of the vitelline duct. The CT appearance of this lesion, although not previously described to our knowledge, is characteristic. We report a patient with ileal dysgenesis who had an abdominal CT scan to evaluate chronic iron deficiency anemia and protein-losing enteropathy. Recognition of this lesion by pediatric radiologists is important; so that surgical treatment, which is simple and effective, can be initiated quickly. (orig.)

  9. CT manifestations of ileal dysgenesis

    International Nuclear Information System (INIS)

    Oberhelman, Amy P.; Herman, Thomas E.; McAlister, William H.; Keating, James P.; Rollins, Michael D.; Dillon, Patrick A.

    2007-01-01

    Ileal dysgenesis is an uncommon condition of unknown etiology occurring in the distal ileum in the region of the vitelline duct. The CT appearance of this lesion, although not previously described to our knowledge, is characteristic. We report a patient with ileal dysgenesis who had an abdominal CT scan to evaluate chronic iron deficiency anemia and protein-losing enteropathy. Recognition of this lesion by pediatric radiologists is important; so that surgical treatment, which is simple and effective, can be initiated quickly. (orig.)

  10. Treatment Results of Supraglottic Carcinoma

    International Nuclear Information System (INIS)

    Park, Charn Il; Kim, Kwang Hyun

    1984-01-01

    From March of 1979 through December of 1982, 84 patients with supraglottic carcinoma were seen and evaluated in the Department of Therapeutic Radiology, Seoul National University Hospital. Of these, 68 patients were treated with a curative intent by employing either radiotherapy alone or a combined approach of radiotherapy plus surgery. Sixteen patients refused to complete the treatment program. Seventy-three per cent of patients had T3 and T4 lesions and 63% of patients had lymph node metastasis at the time of diagnosis. Actuarial recurrence-free survival at 3 year was: Stage I-III 62%, Stage III 54%, Stage IV 18%. For T1-2, T3 and T4 lesions, the actuarial recurrence free survival at 3 years were 50%, 34% and 9% respectively. Of 39 patients with treatment failure, 29 patients (78%) had uncontrol or ultimate recurrence at the primary site while 9 patients having lymph node recurrence, 14 patients(21%) had lymph node recurrence, and 5 patients had distant metastasis. It is suggested that planned combined radiotherapy is indicated for advanced but respectable supraglottic carcinoma

  11. Testicular dysgenesis syndrome and Leydig cell function

    DEFF Research Database (Denmark)

    Joensen, U.N.; Jorgensen, N.; Rajpert-De, Meyts E.

    2008-01-01

    Fertility among human beings appear to be on the decline in many Western countries, and part of the explanation may be decreasing male fecundity. A hypothesis has been put forward that decreasing semen quality may be associated with a testicular dysgenesis syndrome (TDS), a spectrum of disorders...... originating in early foetal life. TDS comprises various aspects of impaired gonadal development and function, including testicular cancer. A growing body of evidence, including animal models and research in human beings, points to lifestyle factors and endocrine disrupters as risk factors for TDS. We present...

  12. Premature osteoarthritis of the knee associated with cartilage hypertrophy and phalangeal dysgenesis

    International Nuclear Information System (INIS)

    Vital, E.M.J.; Hutton, C.W.; Hughes, P.M.

    2005-01-01

    A woman presented with premature knee osteoarthritis associated with marked femoral cartilage hypertrophy. She also exhibited phalangeal dysgenesis, suggesting this may be an unrecognised syndrome that may predispose to knee osteoarthritis. (orig.)

  13. Computed tomography contribution to the staging of supraglottic squamous cell carcinoma

    International Nuclear Information System (INIS)

    Monte Aragao Junior, Antonio Gilson; Rapoport, Abrao; Souza, Ricardo Pires de

    2007-01-01

    Objective: The present study was aimed at evaluating the role of computed tomography in the local clinical staging of supraglottic tumors according to the TNM classification, as well as the interobserver agreement on the detection of the tumor extent. Materials and methods: Thirty-nine dossiers of inpatients of Hospital Heliopolis with supraglottic squamous cell carcinoma in the period between 1988 and 1998 were retrospectively evaluated. CT studies were individually analyzed by two radiologists. The kappa test was utilized for evaluating the interobserver agreement. Results: Computed tomography has played a decisive role in the upstaging of 38.5% of cases, as a result of a deep tumor extent undetected at clinical examination. Conclusion: Interobserver agreement was considered as excellent for vocal folds and subglottis; good for supraglottic, paraglottic and preepiglottic spaces, thyroid and cricoid cartilages and for extralaryngeal tumor extension; and regular for the base of the tongue. (author)

  14. State of the art: Rescue intubation through supraglottic airways

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    R. Hofmeyr*

    2013-12-01

    Full Text Available The use of supraglottic airways as rescue devices in failed intubation and resuscitation has become well accepted in emergency practice. Many offer or advertise the possibility of intubation through the device, but techniques and success rates vary greatly. Intubation can be achieved blindly, with the use a bougie or introducer, or with fiberoptic guidance. In this review, I examine the evidence behind different devices with various techniques, present the data from our on-going research, suggest further research directions and propose practical guidelines for clinical use in emergencies.

  15. Successful anesthetic management of a large supraglottic cyst

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    Harshal D Wagh

    2014-01-01

    Full Text Available Excision of a huge-sized supraglottic mass nearly obstructing the airway passage is a real challenge to anesthesiologists. Upper airway obstruction due to neoplasm in supraglottic region is traditionally managed by preoperative tracheostomy. However, such a common procedure can potentially have an impact on long-term outcome.

  16. Repair of damaged supraglottic airway devices: A novel method

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    Kapoor Dheeraj

    2010-06-01

    Full Text Available Abstract Damage of laryngeal mask airway and other supraglottic airway devices has always been a matter of concern. Although manufacturer recommends maximum 40 uses of LMA (and its congeners but damage before 40 uses needs to be evaluated. We hereby, describe a novel method of repair of supraglottic devices when damage occurs at mask inflation line or pilot balloon valve assembly.

  17. Leptomeningeal carcinomatosis from squamous cell carcinoma of the supraglottic larynx

    International Nuclear Information System (INIS)

    Thompson, S.R.; Veness, M.J.; Morgan, G.J.; Shannon, J.; Kench, J.G.

    2003-01-01

    Leptomeningeal carcinomatosis is an uncommon but devastating form of metastatic spread. To our knowledge, only 16 cases originating from a head and neck cancer have been reported. We describe the first case of a patient with leptomeningeal carcinomatosis arising from a laryngeal squamous cell carcinoma. Shortly after completing treatment for an advanced supraglottic laryngeal cancer, this 63-year-old man presented with lower limb neurological symptoms and signs. Radiological and cytological evidence of leptomeningeal carcinomatosis of the distal spinal canal was identified. He was treated with intrathecal methotrexate and palliative radiotherapy. Although his pain improved, his lower limb weakness worsened. He died 3 weeks after completing radiotherapy. Presumed mode of spread was via the haematogenous route. The natural history and management of leptomeningeal carcinomatosis are discussed. Clinicians should be aware of the uncommon possibility of leptomeningeal carcinomatosis in a patient presenting with an appropriate constellation of symptoms and signs, and a past history of cancer. Copyright (2003) Blackwell Science Pty Ltd

  18. Unilateral dysgenesis of the internal carotid artery: spectrum of imaging findings

    International Nuclear Information System (INIS)

    De Mendonca, J.L.F.; Viana, S.L.; Freitas, F.M.O.; Matos, V.L.; Viana, M.A.C.B.; Silva, R.F.; Quaglia, L.A.N.; Guerra, J.G.

    2008-01-01

    Dysgenesis of the internal carotid artery (ICA) is a broad term comprising hypoplasia, aplasia and agenesis of the vessel. It is a rare anomaly, often clinically silent, that can be confidently diagnosed by means of noninvasive imaging methods. After a review of teaching files, 7 patients with unilateral carotid dysgenesis were found, 2 with agenesis of the ICA, 3 with carotid aplasia, and 2 with hypoplasia of the vessel. Computed tomography (CT) and magnetic resonance angiography (MRA) of the brain were performed in all patients, with a complete magnetic resonance of the brain in 3 of them, a CT angiography of the brain in one, and MRA of the cervical arteries in 3. The fetal pattern of arterial circulation was found in 3 patients with agenesis/aplasia of the ICA and the adult pattern was found in 2 patients, being the brain circulation of normal pattern in the patients with hypoplasia of the ICA. Two patients presented signs of reduced flow to the brain hemisphere ipsilateral to the carotid dysgenesis; one of them with an old homolateral brain infarction. Far from being just an anatomic curiosity, the dysgenesis of the ICA may have serious consequences if not recognized prior to endarterectomies, carotid ligation or transsphenoidal surgery. As much as one-third of these patients will have intracranial aneurysms as well. The imaging methods, instead being mutually exclusive, are complementary in the evaluation of carotid dysgenesis. (author)

  19. Histological evidence of testicular dysgenesis in contralateral biopsies from 218 patients with testicular germ cell cancer

    DEFF Research Database (Denmark)

    Hoei-Hansen, Christina E; Holm, Mette; Rajpert-De Meyts, Ewa

    2003-01-01

    This study was prompted by a hypothesis that testicular germ cell cancer may be aetiologically linked to other male reproductive abnormalities as a part of the so-called 'testicular dysgenesis syndrome' (TDS). To corroborate the hypothesis of a common association of germ cell cancer with testicular...... dysgenesis, microscopic dysgenetic features were quantified in contralateral testicular biopsies in patients with a testicular germ cell tumour. Two hundred and eighty consecutive contralateral testicular biopsies from Danish patients with testicular cancer diagnosed in 1998-2001 were evaluated...... presenting with testicular germ cell neoplasms of the adolescent and young type. The findings therefore support the hypothesis that this cancer is part of a testicular dysgenesis syndrome. The presence of contralateral carcinoma in situ was higher in the present study than previously reported....

  20. Cytogenetic and autoradiographic investigations in gonadal dysgenesis

    International Nuclear Information System (INIS)

    Baron, J.; Warenik-Szymankiewicz, A.; Medical Academy, Poznan

    1977-01-01

    Cytogenetic analysis in 23 patients with Turner's syndrome and in 33 women with pure gonadal dysgenesis consisted of sex chromatin determination and karyotype studies employing autoradiography in questionable cases. Here autoradiography is used as an indispensable complement to cytogenetic techniques. The labelling behaviour of aberrant chromosomes is described. After treatment of the autoradiographic films for more differentiation in results is employed

  1. Analysis of Measured and Simulated Supraglottal Acoustic Waves.

    Science.gov (United States)

    Fraile, Rubén; Evdokimova, Vera V; Evgrafova, Karina V; Godino-Llorente, Juan I; Skrelin, Pavel A

    2016-09-01

    To date, although much attention has been paid to the estimation and modeling of the voice source (ie, the glottal airflow volume velocity), the measurement and characterization of the supraglottal pressure wave have been much less studied. Some previous results have unveiled that the supraglottal pressure wave has some spectral resonances similar to those of the voice pressure wave. This makes the supraglottal wave partially intelligible. Although the explanation for such effect seems to be clearly related to the reflected pressure wave traveling upstream along the vocal tract, the influence that nonlinear source-filter interaction has on it is not as clear. This article provides an insight into this issue by comparing the acoustic analyses of measured and simulated supraglottal and voice waves. Simulations have been performed using a high-dimensional discrete vocal fold model. Results of such comparative analysis indicate that spectral resonances in the supraglottal wave are mainly caused by the regressive pressure wave that travels upstream along the vocal tract and not by source-tract interaction. On the contrary and according to simulation results, source-tract interaction has a role in the loss of intelligibility that happens in the supraglottal wave with respect to the voice wave. This loss of intelligibility mainly corresponds to spectral differences for frequencies above 1500 Hz. Copyright © 2016 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  2. Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome with a mutation

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    Yong Suk Shim

    2015-03-01

    Full Text Available Hypoparathyroidism, sensorineural deafness, and renal dysgenesis syndrome is an autosomal dominant disease caused by mutations in the GATA3 gene on chromosome 10p15. We identified a patient diagnosed with hypoparathyroidism who also had a family history of hypoparathyroidism and sensorineural deafness, present in the father. The patient was subsequently diagnosed and found to be a heterozygote for an insertion mutation c.255_256ins4 (GTGC in exon 2 of GATA3. His father was also confirmed to have the same mutation in GATA3.

  3. The role of postradiotherapy neck dissection in supraglottic carcinoma

    International Nuclear Information System (INIS)

    Chan, Annie W.; Ancukiewicz, Marek; Carballo, Natalia; Montgomery, William; Wang, C.C.

    2001-01-01

    Purpose: To evaluate our policy of performing neck dissection based on regional response after definitive radiotherapy in patients with supraglottic carcinoma and to identify the prognostic factors in this group of patients. Methods and Materials: Between 1970 and 1995, 121 patients with node-positive squamous cell carcinoma of the supraglottic larynx were treated with definitive radiotherapy. Sixty-nine percent of patients presented with 1997 AJCC Stage IV disease. The N-stage distribution was N1, 49; N2, 62; and N3, 10. The median size of the lymph nodes was 3 cm (range, 0.5-8 cm). Forty-five patients received once-a-day treatment with a median total dose of 65 Gy (range, 58.0-70.8 Gy) in 1.8-2.0 Gy per fraction over 48 days, and 76 patients received split-course accelerated hyperfractionation with a median total dose of 67.2 Gy (range, 63.2-73.6 Gy) in 1.6 Gy twice a day over 43 days. Patients whose lymph nodes were not clinically detectable at 4-6 weeks after the completion of radiotherapy (complete response) were followed without any neck dissection. Patients with persistent neck adenopathy (partial response) underwent neck dissection whenever possible. Mean follow-up of the living patients was 6.5 years. Results: Regional response was related to the size of lymph nodes at presentation. Eighty-seven percent of patients with nodal size of 3 cm or less had a complete response, whereas 43% of patients with nodal size greater than 3 cm had a partial response. The rate of regional control at 3 years for all patients in the study was 66%. The 3-year ultimate regional control rate after salvage neck dissection was 75%. A relapse in both the primary and regional sites was the most common pattern of relapse, accounting for 39% of all the failures. Local failure was associated with subsequent regional relapse with a relative risk of 4.3. For patients with completeresponse in whom postradiotherapy neck dissection was withheld, the regional control rates were 75% and 86

  4. Comparative Assessment of Three Approaches of Teaching Nonmedically Trained Persons in the Handling of Supraglottic Airways: A Randomized Controlled Trial.

    Science.gov (United States)

    Hensel, Mario; Schmidbauer, Willi; Benker, Michael; Schmieder, Paula; Kerner, Thoralf

    2017-03-01

    The use of supraglottic airways has been recommended in combat trauma airway management. To ensure an adequate airway management on the battlefield, suitable training concepts are sought to efficiently teach as many soldiers as possible. Our aim was to compare three approaches of teaching laypersons in the handling of supraglottic airways in a mannequin model. In this prospective randomized blinded study, 285 military service men without any medical background were divided into three groups and trained in the use of the Laryngeal Mask Airway Supreme (LMA) and the Laryngeal Tube Disposable (LT-D). The first group received a theoretical lecture, the second group was shown an instruction video, and the third group underwent a practical training. Immediately after instruction participants were asked to place the supraglottic airway and ventilate the mannequin within 60 seconds. The entire test was repeated 3 months later. Test results were evaluated with regard to success rate, insertion time, ability to judge the correct placement, and degree of difficulty. Practical training showed the highest success rate when placing supraglottic airways immediately after the instruction (lecture: 68%, video: 74%, training: 94%); (training vs. lecture and training vs. video, p training: 78%); (training vs. lecture, p = 0.019 and training vs. video, p = 0.025). Immediately after the instruction practical training was also superior in terms of insertion time, ability to judge the correct placement, and the self-rated degree of difficulty (p place a supraglottic airway immediately following minimal instruction and after 3 months as well. Study participants achieved the best results after practical training followed by video presentation and finally lecture regardless of the airway device used. There are two possible reasons why practical training is the superior method. Firstly, the success is tied to more time spent with the learners. Secondly, practical training seems to be the best

  5. MRI Findings of Coexistence of Ectopic Neurohypophysis, Corpus Callosum Dysgenesis, and Periventricular Neuronal Heterotopia

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    Harun Arslan

    2014-01-01

    Full Text Available Ectopic neurohypophysis is a pituitary gland abnormality, which can accompany growth hormone deficiency associated with dwarfism. Here we present magnetic resonance imaging (MRI findings of a rare case of ectopic neurohypophysis, corpus callosum dysgenesis, and periventricular neuronal heterotopia coexisting, with a review of the literature.

  6. MAP3K1-related gonadal dysgenesis: Six new cases and review of the literature.

    Science.gov (United States)

    Granados, Andrea; Alaniz, Veronica I; Mohnach, Lauren; Barseghyan, Hayk; Vilain, Eric; Ostrer, Harry; Quint, Elisabeth H; Chen, Ming; Keegan, Catherine E

    2017-06-01

    Investigation of disorders of sex development (DSD) has resulted in the discovery of multiple sex-determining genes. MAP3K1 encodes a signal transduction regulator in the sex determination pathway and is emerging as one of the more common genes responsible for 46,XY DSD presenting as complete or partial gonadal dysgenesis. Clinical assessment, endocrine evaluation, and genetic analysis were performed in six individuals from four unrelated families with 46,XY DSD. All six individuals were found to have likely pathogenic MAP3K1 variants. Three of these individuals presented with complete gonadal dysgenesis, characterized by bilateral streak gonads with typical internal and external female genitalia, while the other three presented with partial gonadal dysgenesis, characterized by incomplete testicular development, resulting in clitoral hypertrophy with otherwise typical female external genitalia. Testing for MAP3K1 variants should be considered in patients with 46,XY complete or partial gonadal dysgenesis, particularly in families with multiple members affected with 46,XY DSD. Identification of a MAP3K1 variant should prompt an evaluation for DSD in female siblings of the proband. © 2017 Wiley Periodicals, Inc.

  7. [Relationship between phthalates and testicular dysgenesis syndrome].

    Science.gov (United States)

    Chen, Guo-Rong; Dong, Lei; Ge, Ren-Shan; Hardy, Matthew P

    2007-03-01

    Recent epidemiological evidence demonstrates that boys born to women exposed to phthalates during pregnancy have an increased incidence of cryptorchidism, hypospadias, testicular cancer and spermatogenic dysfunction, which are collectively referred to as testicular dysgenesis syndrome (TDS). TDS may be attributed to the dysfunction of Leydig cells and Sertoli cells during their differentiation after exposure to phthalates in utero. Fox example, Leydig cell functions are significantly affected by phthalates, leading to the decrease of two Leydig cell products--insulin-like growth factor 3 (INSL3) and testosterone, which are critical factors for testis descent. The disorientation of Leydig cells and Sertoli cells in the adult testis may be the cause of spermatogenic dysfunction.

  8. Ovarian dysgenesis in an alpaca with a minute chromosome 36.

    Science.gov (United States)

    Fellows, Elizabeth; Kutzler, Michelle; Avila, Felipe; Das, Pranab J; Raudsepp, Terje

    2014-01-01

    A 4-year-old female alpaca (Lama pacos [LPA]) was presented to the Oregon State Veterinary Teaching Hospital for failure to display receptive behavior to males. Although no abnormalities were found on physical examination, transrectal ultrasonographic examination of the reproductive tract revealed uterine hypoplasia and ovarian dysgenesis. Cytogenetic analysis demonstrated a normal female 74,XX karyotype with 1 exceptionally small (minute) homologue of autosome LPA36. Chromosome analysis by Giemsa staining and DAPI- and C-banding revealed that the minute LPA36 was submetacentric, AT-rich, and largely heterochromatic. Because of the small size and lack of molecular markers, it was not possible to identify the origin of the minute. There is a need to improve molecular cytogenetic tools to further study the phenomenon of this minute chromosome and its relation to female reproduction in alpacas and llamas. © The American Genetic Association. 2012. All rights reserved. For permissions, please email: journals.permissions@oup.com.

  9. Multigeneration Inheritance through Fertile XX Carriers of an NR0B1 (DAX1 Locus Duplication in a Kindred of Females with Isolated XY Gonadal Dysgenesis

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    Michela Barbaro

    2012-01-01

    Full Text Available A 160 kb minimal common region in Xp21 has been determined as the cause of XY gonadal dysgenesis, if duplicated. The region contains the MAGEB genes and the NR0B1 gene; this is the candidate for gonadal dysgenesis if overexpressed. Most patients present gonadal dysgenesis within a more complex phenotype. However, few independent cases have recently been described presenting with isolated XY gonadal dysgenesis caused by relatively small NR0B1 locus duplications. We have identified another NR0B1 duplication in two sisters with isolated XY gonadal dysgenesis with an X-linked inheritance pattern. We performed X-inactivation studies in three fertile female carriers of three different small NR0B1 locus duplications identified by our group. The carrier mothers did not show obvious skewing of X-chromosome inactivation, suggesting that NR0B1 overexpression does not impair ovarian function. We furthermore emphasize the importance to investigate the NR0B1 locus also in patients with isolated XY gonadal dysgenesis.

  10. Endoscopic management of posttraumatic supraglottic stenosis in the pediatric population.

    LENUS (Irish Health Repository)

    Oosthuizen, Johannes Christiaan

    2012-02-01

    OBJECTIVES: Pediatric blunt laryngeal trauma is a rare and potentially life-threatening entity. External injuries can be misleading, and a high index of suspicion, as well as early intervention, is essential to achieve the best possible outcome. The authors of this report review the management of blunt laryngeal trauma in the pediatric population and describe the endoscopic management of posttraumatic supraglottic stenosis. METHODS: Methods used were case report from a tertiary referral institution and review of the literature. RESULTS: We describe the case of a 13-year-old girl whom developed supraglottic stenosis following blunt laryngeal trauma. Innovative endoscopic techniques were used in the successful management of this exceedingly rare entity. CONCLUSION: Early recognition and intervention are of paramount importance if successful endoscopic management of blunt laryngeal trauma is to be considered.

  11. Premature sternal fusion in gonadal dysgenesis with coarctation

    International Nuclear Information System (INIS)

    Herman, T.E.; Kushner, D.C.; Cleveland, R.H.

    1985-01-01

    In reviewing lateral chest roentgenograms in three infants with Turner's syndrome and aortic coarctation, and in infant with mixed gonadal dysgenesis and aortic coarctation, we have found obvious sternal fusion abnormalities which are reported here. (orig./MG)

  12. Radiation recall supraglottitis. A hazard in head and neck chemotherapy

    International Nuclear Information System (INIS)

    Wallenborn, P.A.; Postma, D.S.

    1984-01-01

    The enhanced effects of chemotherapy on previously irradiated tissue have been well demonstrated. When chemotherapy is given some time after irradiation and elicits a tissue reaction in the radiation field, the reaction is termed radiation recall. We review known interactions between chemotherapy and radiotherapy and report, to our knowledge, the first case of a supraglottitis radiation recall reaction. Familiarity with this phenomenon and potential complications of chemotherapy following head and neck irradiation may expedite early diagnosis and appropriate lifesaving treatment

  13. Demonstration of transoral robotic supraglottic laryngectomy and total laryngectomy in cadaveric specimens using the Medrobotics Flex System.

    Science.gov (United States)

    Funk, Emily; Goldenberg, David; Goyal, Neerav

    2017-06-01

    Current management of laryngeal malignancies is associated with significant morbidity. Application of minimally invasive transoral techniques may reduce the morbidity associated with traditional procedures. The purpose of this study was to present our investigation of the utility of a novel flexible robotic system for transoral supraglottic laryngectomy and total laryngectomy. Transoral total laryngectomy and transoral supraglottic laryngectomy were performed in cadaveric specimens using the Flex Robotic System (Medrobotics, Raynham, MA). All procedures were completed successfully in the cadaveric models. The articulated endoscope allowed for access to the desired surgical site. Flexible instruments enabled an atraumatic approach and allowed for precise surgical technique. Access to deep anatomic structures remains problematic using current minimally invasive robotic approaches. Improvements in visualization and access to the laryngopharyngeal complex offered by this system may improve surgical applications to the larynx. This study demonstrates the technical feasibility using the Flex Robotic System for transoral robotic supraglottic laryngectomy and total laryngectomy. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1218-1225, 2017. © 2017 Wiley Periodicals, Inc.

  14. An Uncommon Complication with a Supraglottic Airway: The King LT.

    Science.gov (United States)

    Brown, Sara; Cherian, Verghese T; Greco, Katherine; Mets, Elbert; Budde, Arne

    2016-02-15

    General anesthesia was administered in an 18-year-old man for removal of hardware from his right knee using a King Laryngeal Tube supraglottic airway. An hour after extubation, he reported inability to swallow with no respiratory distress. Examination showed an edematous uvula, which took 3 days to subside with anti-inflammatory medication. During the positioning of the King Laryngeal Tube, it was pulled back to ensure adequate ventilation. The inflated cuff could have dragged the uvula and folded it on itself, leading to venous congestion and edema.

  15. Testicular dysgenesis syndrome: foetal origin of adult reproductive problems

    DEFF Research Database (Denmark)

    Wohlfahrt-Veje, Christine; Main, Katharina M; Skakkebaek, Niels Erik

    2009-01-01

    , the risk of testis cancer is significantly increased in men with cryptorchidism and/or infertility. Several recent studies point towards early dysgenesis of the foetal testis as the biological link between these disorders. Dysgenesis has been demonstrated in biopsies of the contralateral testis of men...... with testis cancer and in infertile men. The histological evidence includes immature seminiferous tubules with undifferentiated Sertoli cells, microliths and Sertoli-cell only tubules. Dysgenetic testes often have an irregular ultrasound pattern, where microliths may also be visible. Our current hypothesis...

  16. Laryngeal sarcoidosis: a case report presenting transglottic involvement

    NARCIS (Netherlands)

    Broek, E.M.J.M. van den; Heijnen, B.J.; Verbist, B.M.; Sjögren, E.V.

    2013-01-01

    Isolated laryngeal sarcoidosis is a very rare disease. In most cases, it will present as a supraglottic pale edematous swelling. In our case, the patient presented with hoarseness and dyspnea during exertion. Laryngeal examination did show not only supraglottic edema but also prominent subglottic

  17. Spinal segmental dysgenesis | Mahomed | SA Journal of Radiology

    African Journals Online (AJOL)

    Spinal segmental dysgenesis is a rare congenital spinal abnormality seen in neonates and infants, in which a segment of the spine and spinal cord fails to develop normally. The condition is segmental in nature, with vertebrae above and below the malformation. It is commonly associated with various abnormalities that ...

  18. Testicular dysgenesis syndrome: mechanistic insights and potential new downstream effects

    DEFF Research Database (Denmark)

    Sharpe, R.M.; Skakkebæk, Niels Erik

    2008-01-01

    Reproductive disorders of newborn (cryptorchidism, hypospadias) and young adult males (low sperm counts, testicular germ cell cancer) are common and/or increasing in incidence. It has been hypothesized that these disorders may comprise a testicular dysgenesis syndrome (TDS) with a common origin...

  19. Transoral videolaryngoscopic surgery (TOVS) for hypopharyngeal and supraglottic cancer

    International Nuclear Information System (INIS)

    Tomifuji, Masayuki; Araki, Koji; Yamashita, Taku; Shiotani, Akihiro

    2011-01-01

    We proposed transoral videolaryngoscopic surgery (TOVS) as a laryngeal preservation strategy for laryngopharyngeal cancer. By using a distending laryngoscope and rigid laryngeal endoscope, a large field of view and working space could be obtained which enabled us to resect tumors in en-bloc fashion. The indications for this surgery are oro-hypopharyngeal and supraglottic cancer in superficial, T1, T2 and selected T3 categories. TOVS can also be used for selected cases with recurrent tumor after radiation therapy. For resectable nodal metastasis, neck dissection can be performed simultaneously or 1-2 weeks later. In cases with more than a 1-year observation period (n=42), 5-year crude survival, disease-specific survival, and laryngeal preservation rate were 74%, 85% and 89%, respectively. A second advantage of TOVS is thorough evaluation of primary cancer lesion. Evaluation of tumor invasion depth is a promising way for optimizing the indication for neck dissection for clinically node negative cases. (author)

  20. Synchronous Supraglottic and Esophageal Squamous Cell Carcinomas Treated with a Monoisocentric Hybrid Intensity-Modulated Radiation Technique

    Directory of Open Access Journals (Sweden)

    Christian L. Barney

    2018-01-01

    Full Text Available Risk factors for squamous cell carcinomas (SCCs of the head and neck (HN and esophagus are similar. As such, synchronous primary tumors in these areas are not entirely uncommon. Definitive chemoradiation (CRT is standard care for locally advanced HNSCC and is a preferred option for inoperable esophageal SCC. Simultaneous treatment of both primaries with CRT can present technical challenges. We report a case of synchronous supraglottic and esophageal SCC primary tumors, highlighting treatment with a monoisocentric hybrid radiation technique and normal tissue toxicity considerations.

  1. Supraglottic devices as alternative equipment to airway management in state of sudden cardiac arrest

    Directory of Open Access Journals (Sweden)

    Rafał Czyż

    2017-08-01

    All of supraglottic devices are characterized by easiness in applying without experience in use them. Additionally time need to airway management in use of them is many times shorter than with traditional endotracheal intubation. Fundamental defect in these devices is a fact that they don’t provide total safety before aspiration for chime. Current literature shows us that supraglottic airway devices are perfect alternative to endotracheal intubation.

  2. Testicular dysgenesis syndrome and the development and occurrence of male reproductive disorders

    International Nuclear Information System (INIS)

    Virtanen, H.E.; Rajpert-De Meyts, E.; Main, K.M.; Skakkebaek, N.E.; Toppari, J.

    2005-01-01

    Patients with 45,X0/46XY karyotype often present with intersex phenotype and testicular dysgenesis. These patients may also have undescended testes (cryptorchidism), hypospadias and their spermatogenesis is severely disrupted. They have a high risk for testicular cancer. These patients have the most severe form of testicular dysgenesis syndrome (TDS). We have hypothesized that testicular cancer, cryptorchidism, hypospadias and poor spermatogenesis are all signs of a developmental disturbance that was named as testicular dysgenesis syndrome. The hypothesis is based on clinical and epidemiological findings and on biological and experimental evidence. Signs of TDS share several risk factors, such as small birth weight (particularly being small for gestational age), and they are risk factors for each other. All of them have background in fetal development. They show strong epidemiological links so that countries with high incidence of testicular cancer, such as Denmark, tend to also have high prevalence rates of cryptorchidism and hypospadias and poor semen quality. Vice versa, in countries with good male reproductive health, e.g., in Finland, all these aspects are better than in Denmark. Although genetic abnormalities can cause these disorders, in the majority of cases, the reasons remain unclear. Adverse trends in the incidence of male reproductive disorders suggest that environmental and life style factors contribute to the problem. Endocrine disrupters are considered as prime candidates for environmental influence. Fetal exposure to high doses of dibutyl phthalate was shown to cause a TDS-like phenotype in the rats. Studies are underway to assess whether there is any exposure-outcome relation with selected chemicals (persistent organic pollutants, pesticides, phthalates) and cryptorchidism

  3. A rare case report of 46XY mixed gonadal dysgenesis

    Directory of Open Access Journals (Sweden)

    Rakesh Arora

    2013-01-01

    Full Text Available A 16-year-old person, reared as female presented with complaints of genital ambiguity and primary amenorrhoea along with lack of secondary sexual characters, but without short stature and Turner′s stigmata. She was taking steroids after being misdiagnosed as congenital adrenal hyperplasia (CAH. Karyotype analysis revealed 46XY karyotype. There was no evidence of hypocortisolemia (cortisol 9.08 μg/dl, adrenocorticotropic hormone [ACTH] 82.5 pg/ml or elevated level of 17-OH-progesterone (0.16 ng/ml. Pooled luteinizing hormone (LH was 11.79 mIU/ml and follicle-stimulating hormone (FSH was 66.37 mIU/ml. Serum estradiol level was 25 pg/ml (21-251. Basal and 72 h post beta-human chorionic gonadotropin (hCG levels of androstenedione and testosterone levels were done (basal testosterone of 652 ng/dl and basal androstenedione of 1.17 ng/ml; 72 h post hCG testosterone of 896 ng/dl and androstenedione of 1.34 ng/ml. Magnetic resonance imaging (MRI pelvis (with ultrasonogrphy [USG] correlation revealed uterus didelphys with obstructed right moiety and bilateral ovarian-like structures. Right sided gonads and adjacent tubal structures were visualized laparoscopically and removed. Left sided gonads were not visualized and Mullerian remnants were adhered to sigmoid colon. Histopathological examination revealed presence of testicular tissue showing atrophic seminiferous tubules with hyperplasia of Leydig cells. No ovarian tissue was seen. Based on these results a diagnosis of 46XY mixed gonadal dysgenesis (MGD was made, which is rare and is difficult to distinguish from 46XY ovotesticular disorder of sexual differentiation (OT-DSD. The patient was managed with a multidisciplinary approach and fertility issues discussed with the patient′s caregivers.

  4. SUPRAGLOTTIC JET VENTILATION VERSUS CONVENTIONAL ENDOTRACHEAL VENTILATION IN MINOR LARYNGEAL SURGERIES

    Directory of Open Access Journals (Sweden)

    Illendual Upendranath

    2016-08-01

    Full Text Available Any attempt at intubation will cause many cardiovascular responses and the major concern during this time is to attenuate the same. Similar response is seen during procedures on Larynx in microlaryngeal surgery which produces an intense cardiovascular stimulation during suspension laryngoscopy and intubation. AIM OF STUDY Supraglottic jet ventilation versus conventional endotracheal ventilation in minor laryngeal surgeries. To evaluate the haemodynamic response in supraglottic jet ventilation and conventional intubation in minor laryngeal surgeries. METHODS Patients were randomised to 2 Groups: 30 patients in each group; Group A - in whom supraglottic jet ventilation was planned and Group B - in whom endotracheal intubation was planned. RESULT The haemodynamic response in terms of increase in MAP and HR is significantly more with endotracheal intubation than with supraglottic jet ventilation. CONCLUSION Our study showed that supraglottic jet ventilation showed a better haemodynamic stability when compared to conventional endotracheal intubation in patients undergoing minor laryngeal surgeries. Statistical scores were also in favour of the patients treated with supraglottic jet ventilation based on the p values.

  5. Microscopic dysgerminoma associated with anti-Ma2 paraneoplastic encephalitis in a patient with gonadal dysgenesis.

    Science.gov (United States)

    Abdulkader, Marwah M; Yousef, Mohammad M; Abdelhadi, Mohamad K; Amr, Samir S; Alabsi, Eyad S; Al-Abbadi, Mousa A

    2013-05-01

    We present a 27-yr-old female with gonadal dysgenesis (46, XY), who presented to our hospital with poor consciousness, aphasia, restlessness, and visual hallucination. Physical examination revealed normal breast development and normal external female genetalia. Computed tomography scan of the head and neck revealed the presence of brain edema, hydrocephalous, and a localized hypodense lesion in the hypothalamus. Her serum was positive for the anti-Ma2, which is associated with paraneoplastic encephalitis syndrome. Computed tomography of the abdomen revealed the presence of a 7.5×5.3×3.0 cm solid pelvic mass. Interestingly, a single microscopic focus of dysgerminoma was identified in a background of stromal fibrosis and focal dystrophic calcifications. No ovarian stroma or testicular tissue was identified. To our knowledge, this is the first case of gonadal dysgenesis presenting with anti-Ma2 paraneoplastic encephalitis with dysgerminoma. A discussion about paraneoplastic encephalitis with a microscopic dysgerminoma associated with anti-Ma2 antibody is presented.

  6. Corpus callosum dysgenesis and lipoma: embryologic and magnetic resonance imaging aspects

    International Nuclear Information System (INIS)

    Abreu Junior, Luiz de; Borri, Maria Lucia; Wolosker, Angela Maria Borri; Hartmann, Luiz Guilherme de Carvalho; Galvao Filho, Mario de Melo; D'Ippolito, Giuseppe

    2005-01-01

    The corpus callosum is the major system of association fibers that permits communication of both cerebral hemispheres. Magnetic resonance imaging has improved the study of brain malformations, including the corpus callosum dysgenesis. Lipoma is a common finding in the spectrum of corpus callosum dysgenesis. The purpose of these study was to review the embryologic events and the magnetic resonance imaging aspects related to the corpus callosum dysgenesis and to the formation of the related lipoma. (author)

  7. Electrophysiological Correlates of Morphological Neuroplasticity in Human Callosal Dysgenesis.

    Directory of Open Access Journals (Sweden)

    Vladimir V Lazarev

    Full Text Available In search for the functional counterpart of the alternative Probst and sigmoid bundles, considered as morphological evidence of neuroplasticity in callosal dysgenesis, electroencephalographic (EEG coherence analysis was combined with high resolution and diffusion tensor magnetic resonance imaging. Data of two patients with callosal agenesis, plus two with typical partial dysgenesis with a remnant genu, and one atypical patient with a substantially reduced genu were compared to those of fifteen neurotypic controls. The interhemispheric EEG coherence between homologous nontemporal brain regions corresponded to absence or partial presence of callosal connections. A generalized coherence reduction was observed in complete acallosal patients, as well as coherence preservation in the anterior areas of the two patients with a remnant genu. jThe sigmoid bundles found in three patients with partial dysgenesis correlated with augmented EEG coherence between anterior regions of one hemisphere and posterior regions of the other. These heterologous (crossed interhemispheric connections were asymmetric in both imaging and EEG patterns, with predominance of the right-anterior-to-left-posterior connections over the mirror ones. The Probst bundles correlated with higher intrahemispheric long-distance coherence in all patients. The significant correlations observed for the delta, theta and alpha bands indicate that these alternative pathways are functional, although the neuropsychological nature of this function is still unknown.

  8. Phenotypic and molecular assessment of seven patients with 6p25 deletion syndrome: Relevance to ocular dysgenesis and hearing impairment

    Directory of Open Access Journals (Sweden)

    Ritch Robert

    2004-06-01

    Full Text Available Abstract Background Thirty-nine patients have been described with deletions involving chromosome 6p25. However, relatively few of these deletions have had molecular characterization. Common phenotypes of 6p25 deletion syndrome patients include hydrocephalus, hearing loss, and ocular, craniofacial, skeletal, cardiac, and renal malformations. Molecular characterization of deletions can identify genes that are responsible for these phenotypes. Methods We report the clinical phenotype of seven patients with terminal deletions of chromosome 6p25 and compare them to previously reported patients. Molecular characterization of the deletions was performed using polymorphic marker analysis to determine the extents of the deletions in these seven 6p25 deletion syndrome patients. Results Our results, and previous data, show that ocular dysgenesis and hearing impairment are the two most highly penetrant phenotypes of the 6p25 deletion syndrome. While deletion of the forkhead box C1 gene (FOXC1 probably underlies the ocular dysgenesis, no gene in this region is known to be involved in hearing impairment. Conclusions Ocular dysgenesis and hearing impairment are the two most common phenotypes of 6p25 deletion syndrome. We conclude that a locus for dominant hearing loss is present at 6p25 and that this locus is restricted to a region distal to D6S1617. Molecular characterization of more 6p25 deletion patients will aid in refinement of this locus and the identification of a gene involved in dominant hearing loss.

  9. Experimentally induced testicular dysgenesis syndrome originates in the masculinization programming window

    DEFF Research Database (Denmark)

    van den Driesche, Sander; Kilcoyne, Karen R.; Wagner, Ida Wagner

    2017-01-01

    and after the MPW, but only DBP exposure in the MPW causes reduced AGD, focal testicular dysgenesis, and TDS disorders (cryptorchidism, hypospadias, reduced adult testis size, and compensated adult Leydig cell failure). Focal testicular dysgenesis, reduced size of adult male reproductive organs, and TDS...

  10. Testicular dysgenesis syndrome: possible role of endocrine disrupters

    DEFF Research Database (Denmark)

    Bay, Katrine; Asklund, Camilla; Skakkebaek, Niels E

    2006-01-01

    . Clinically, the most common manifestation of TDS is probably a reduced sperm concentration, whereas the more severe form may include a high risk of testis cancer. Clinicians should be aware of the interconnection between the different features of TDS, and inclusion of a programme for early detection......The testicular dysgenesis syndrome (TDS) hypothesis proposes that the four conditions cryptorchidism, hypospadias, impaired spermatogenesis and testis cancer may all be manifestations of disturbed prenatal testicular development. The TDS hypothesis is based on epidemiological, clinical...... of testis cancer in the management of infertile men with poor semen quality is recommended....

  11. Environment, testicular dysgenesis and carcinoma in situ testis

    DEFF Research Database (Denmark)

    Olesen, Inge A; Sonne, Si Brask; Hoei-Hansen, Christina E

    2007-01-01

    The testicular dysgenesis syndrome (TDS) hypothesis proposes that a proportion of the male reproductive disorders-cryptorchidism, hypospadias, infertility and testicular cancer-may be symptoms of one underlying developmental disease, TDS, which is most likely a result of disturbed gonadal...... range of phenotypes: from the mildest and most common form, in which impaired spermatogenesis is the only symptom, to the most severe cases, in which the patient may develop testicular cancer. It is of great importance that clinicians in different specialties treating patients with TDS are aware...

  12. Testicular dysgenesis syndrome: possible role of endocrine disrupters

    DEFF Research Database (Denmark)

    Bay, Katrine; Asklund, Camilla; Skakkebaek, Niels E

    2006-01-01

    The testicular dysgenesis syndrome (TDS) hypothesis proposes that the four conditions cryptorchidism, hypospadias, impaired spermatogenesis and testis cancer may all be manifestations of disturbed prenatal testicular development. The TDS hypothesis is based on epidemiological, clinical....... Clinically, the most common manifestation of TDS is probably a reduced sperm concentration, whereas the more severe form may include a high risk of testis cancer. Clinicians should be aware of the interconnection between the different features of TDS, and inclusion of a programme for early detection...... of testis cancer in the management of infertile men with poor semen quality is recommended....

  13. A retropharyngeal-mediastinal hematoma with supraglottic and tracheal obstruction: The role of multidisciplinary airway management

    Directory of Open Access Journals (Sweden)

    Birkholz Torsten

    2010-01-01

    Full Text Available A 77-year-old man suffered hypoxemic cardiac arrest by supraglottic and tracheal airway obstruction in the emergency department. A previously unknown cervical fracture had caused a traumatic retropharyngeal-mediastinal hematoma. A lifesaving surgical emergency tracheostomy succeeded. Supraglottic and tracheal obstruction by a retropharyngeal-mediastinal hematoma with successful resuscitation via emergency tracheostomy after hypoxemic cardiac arrest has never been reported in a context of trauma. This clinically demanding case outlines the need for multidisciplinary airway management systems with continuous training and well-implemented guidelines. Only multidisciplinary staff preparedness and readily available equipments for the unanticipated difficult airway solved the catastrophic clinical situation.

  14. Prognostic value of hemoglobin concentration in radiotherapy for cancer of supraglottic larynx

    International Nuclear Information System (INIS)

    Tarnawski, Rafal; Skladowski, Krzysztof; Maciejewski, Boguslaw

    1997-01-01

    Purpose: The aim of this work is the estimation of correlations between hemoglobin concentration either before or after radiotherapy and local tumor control probability for laryngeal cancer. Methods and Materials: Retrospective analysis of 847 cases of laryngeal supraglottic squamous cell carcinoma treated with radiation alone was performed using maximum likelihood estimations, and step-wise logistic regression. All patients were in good initial performance status (Karnofsky index >70). The minimum follow-up time was 3 years. Results: Logistic regression showed that the hemoglobin concentration after radiotherapy is an important prognostic factor. There was a very strong correlation between hemoglobin concentration and tumor local control probability. Hemoglobin concentration at the beginning of radiotherapy does not correlate with treatment outcome, but any decrease of hemoglobin during therapy is a strong prognostic factor for treatment failure. Conclusions: Although regression models with many variables may be instable, the present results suggest that hemoglobin concentration after treatment is at least as important as overall treatment time. It was not possible to find out whether the low concentration of hemoglobin is an independent cause of low TCP or whether it reflects other mechanisms that may influence both hemoglobin level and the TCP

  15. Caudal dysgenesis, sirenomelia, and situs inversus totalis: a primitive defect in blastogenesis.

    Science.gov (United States)

    Rougemont, Anne-Laure; Bouron-Dal Soglio, Dorothée; Désilets, Valérie; Jovanovic, Mubina; Perreault, Gilles; Laurier Oligny, Luc; Fournet, Jean-Christophe

    2008-06-01

    Caudal dysgenesis (CD) constitutes a heterogeneous spectrum of congenital caudal anomalies, including varying degrees of agenesis of the vertebral column, as well as anorectal and genitourinary anomalies. Sirenomelia, characterized by a fusion of the lower limbs, could represent the most severe end of this spectrum. The two main debated pathogenic hypotheses are an aberrant vascular supply versus a primary axial mesoderm defect. We present the autopsy findings of two fetuses of non-diabetic mothers, with normal karyotype. Both fetuses presented situs inversus associated with a CD, in one case consisting of sirenomelia, establishing a very rare association profile that might be random. This association also suggests the occurrence of a common pathogenic mechanism, in accordance to recent genetic data, such as displayed in the Kif3A murine mutation phenotype. Some cases of sirenomelia and CD could represent developmental field defects of blastogenesis involving the caudal mesoderm, rather than being related to vascular insufficiency.

  16. Improvement of electrolaryngeal speech quality using a supraglottal voice source with compensation of vocal tract characteristics.

    Science.gov (United States)

    Wu, Liang; Wan, Congying; Wang, Supin; Wan, Mingxi

    2013-07-01

    Electrolarynx (EL) is a medical speech-recovery device designed for patients who have lost their original voice box due to laryngeal cancer. As a substitute for human larynx, the current commercial EL voice source cannot reconstruct natural EL speech under laryngectomy conditions. To eliminate the abnormal acoustic properties of EL speech, a supraglottal voice source with compensation of vocal tract characteristics was proposed and provided through an experimental EL(SGVS-EL) system. The acoustic analyses of simulated EL speech and reconstructed EL speech produced with different voice sources were performed in the normal subject and laryngectomee. The results indicated that the supraglottal voice source was successful in improving the acoustic properties of EL speech by enhancing low- frequency energy, correcting the shifted formants to normal range, and eliminating the visible spectral zeros. Both normal subject and laryngectomee also produced more natural vowels using SGVS-EL than commercial EL, even if the vocal tract parameter was substituted and the supraglottal voice source was biased to a certain degree. Therefore, supraglottal voice source is a feasible and effective approach to improving the acoustic quality of EL speech.

  17. Evaluation of the Supraglottic and Subglottic Activities Including Acoustic Assessment of the Opera-Chant Singers.

    Science.gov (United States)

    Petekkaya, Emine; Yücel, Ahmet Hilmi; Sürmelioğlu, Özgür

    2017-12-28

    Opera and chant singers learn to effectively use aerodynamic components by breathing exercises during their education. Aerodynamic components, including subglottic air pressure and airflow, deteriorate in voice disorders. This study aimed to evaluate the changes in aerodynamic parameters and supraglottic structures of men and women with different vocal registers who are in an opera and chant education program. Vocal acoustic characteristics, aerodynamic components, and supraglottic structures were evaluated in 40 opera and chant art branch students. The majority of female students were sopranos, and the male students were baritone or tenor vocalists. The acoustic analyses revealed that the mean fundamental frequency was 152.33 Hz in the males and 218.77 Hz in the females. The estimated mean subglottal pressures were similar in females (14.99 cmH 2 O) and in males (14.48 cmH 2 O). Estimated mean airflow rates were also similar in both groups. The supraglottic structure compression analyses revealed partial anterior-posterior compressions in 2 tenors and 2 sopranos, and false vocal fold compression in 2 sopranos. Opera music is sung in high-pitched sounds. Attempts to sing high-pitched notes and frequently using register transitions overstrain the vocal structures. This intense muscular effort eventually traumatizes the vocal structures and causes supraglottic activity. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  18. Phthalate-induced testicular dysgenesis syndrome: Leydig cell influence.

    Science.gov (United States)

    Hu, Guo-Xin; Lian, Qing-Quan; Ge, Ren-Shan; Hardy, Dianne O; Li, Xiao-Kun

    2009-04-01

    Phthalates, the most abundantly produced plasticizers, leach out from polyvinyl chloride plastics and disrupt androgen action. Male rats that are exposed to phthalates in utero develop symptoms characteristic of the human condition referred to as testicular dysgenesis syndrome (TDS). Environmental influences have been suspected to contribute to the increasing incidence of TDS in humans (i.e. cryptorchidism and hypospadias in newborn boys and testicular cancer and reduced sperm quality in adult males). In this review, we discuss the recent findings that prenatal exposure to phthalates affects Leydig cell function in the postnatal testis. This review also focuses on the recent progress in our understanding of how Leydig cell factors contribute to phthalate-mediated TDS.

  19. Association of p53 protein expression with clinical outcome in advanced supraglottic cancer

    International Nuclear Information System (INIS)

    Kang, Jin Oh; Hong, Seong Eon

    1998-01-01

    To determine the incidence and prognostic effect of p53 expression in patients with advanced supraglottic cancer. Twenty-one cases of total 48 advanced supraglottic cancer patients who received postoperative adjuvant radiation therapy were evaluated by immunohistochemical staining employing p53 monoclonal antibody. Three out of six stage III patients and four out of fifteen stage IV patients showed p53 expression without statistically significant difference (p=0.608). Five year survival rates are 93% in p53 negative, 86% in p53 positive patients and there was no significant difference(p=0.776). p53 expression does not show statistically significant correlation with primary tumor status(p=0.877), lymph node status(p=0.874) and age(p=0.64). There was no statistically significant correlation between traditionally known risk factors and p53 expression

  20. Unilateral congenital corneal keloid with anterior segment mesenchymal dysgenesis and subluxated lens: case report and review of literature.

    Science.gov (United States)

    Vanathi, M; Sen, Seema; Panda, Anita; Dada, Tanuj; Behera, Geeta; Khokhar, Sudharshan

    2007-01-01

    To report the unusual association of unilateral congenital corneal keloid with anterior-segment mesenchymal dysgenesis and bilateral subluxated lens. A 20-year old man presented with a mass lesion involving the left cornea. The corneal lesion had been present since birth. On biomicroscopic examination, a well-defined vascularized, grayish-white mass occupying the whole of the left cornea was seen. The right eye showed multiple peripheral corneal opacities with iridocorneal adhesions, a poorly defined supranasal limbus, and a subluxated lens. Excision biopsy of the mass was done for histopathologic examination. Histopathologic examination of the excised corneal mass showed features consistent with that of a corneal keloid: thickened keratinized epithelium, absent Bowman membrane layer, and fibrovascular hyperplasia composed of hyalinized collagen fibers with irregular orientation of the collagen lamellae. During penetrating keratoplasty of the left eye, an anomalous iris pattern with poorly defined angle and a supranasal subluxated lens was also observed. Extraction of the subluxated lens was also done. The graft failed subsequent to a nonhealing persistent epithelial defect. Our case report highlights the rare association of a unilateral congenital corneal keloid with anterior-segment mesenchymal dysgenesis and bilateral subluxated lens.

  1. Enlargement of the supraglottal cavity and its relation to stop consonant voicing.

    Science.gov (United States)

    Westbury, J R

    1983-04-01

    Measurements were made of saggital plane movements of the larynx, soft palate, and portions of the tongue, from a high-speed cinefluorographic film of utterances produced by one adult male speaker of American English. These measures were then used to approximate the temporal variations in supraglottal cavity volume during the closures of voiced and voiceless stop consonants. All data were subsequently related to a synchronous acoustic recording of the utterances. Instances of /p,t,k/ were always accompanied by silent closures, and sometimes accompanied by decreases in supraglottal volume. In contrast, instances of /b,d,g/ were always accompanied both by significant intervals of vocal fold vibration during closure, and relatively large increases in supraglottal volume. However, the magnitudes of volume increments during the voiced stops, and the means by which those increments were achieved, differed considerably across place of articulation and phonetic environment. These results are discussed in the context of a well-known model of the breath-stream control mechanism, and their relevance for a general theory of speech motor control is considered.

  2. Idiopathic Supraglottic Stenosis Refractory to Multiple Interventions Improved With Serial Office-based Steroid Injections.

    Science.gov (United States)

    Hoffman, Matthew R; Mai, Johnny P; Dailey, Seth H

    2017-10-30

    The objective of this study was to describe a patient with idiopathic supraglottic stenosis who experienced persistent disease despite multiple office-based and operative interventions, whose disease is now better controlled with scheduled serial office-based steroid injections. This is a case report and literature review. A 42-year-old female was referred for worsening supraglottic stenosis despite systemic steroids. She underwent awake tracheotomy. A thorough historical, histologic, and laboratory workup did not reveal an etiology to her stenosis. She later underwent endoscopic partial laryngectomy and was able to be decannulated. She underwent a second endoscopic partial laryngectomy two years later for worsening disease and then was managed over the next seven years with intermittent systemic steroids. Over the last year, she has undergone eight office-based steroid injections with improvement in her degree of stenosis and symptom burden. There are only four prior reported cases of idiopathic supraglottic stenosis, none of which has been managed with serial office-based steroid injections. This case report adds to the small body of literature on the management of this rare disease and proposes a new office-based treatment pathway that may help induce regression of stenosis. Copyright © 2017 The Voice Foundation. Published by Elsevier Inc. All rights reserved.

  3. Anterior segment dysgenesis associated with Williams-Beuren syndrome: a case report and review of the literature.

    Science.gov (United States)

    Todorova, Margarita G; Grieshaber, Matthias C; Cámara, Rafael J A; Miny, Peter; Palmowski-Wolfe, Anja M

    2014-05-21

    Williams-Beuren syndrome is characterized by mild mental retardation, specific neurocognitive profile, hypercalcemia during infancy, distinctive facial features and cardiovascular diseases. We report on complete ophthalmologic, sonographic and genetic evaluation of a girl with a clinical phenotype of Williams-Beuren syndrome, associated with unilateral anterior segment dysgenesis and bilateral cleft of the soft and hard palate. These phenotypic features have not been linked to the haploinsufficiency of genes involved in the microdeletion. A term born girl presented at the initial examination with clouding of the right cornea. On ultrasound biomicroscopy the anterior chamber structures were difficult to differentiate, showing severe adhesions from the opacified cornea to the iris with a kerato-irido-lenticular contact to the remnant lens, a finding consistent with Peters' anomaly. Genetic analyses including FISH confirmed a loss of the critical region 7q11.23, usually associated with the typical Williams-Beuren syndrome. Microsatellite analysis showed a loss of about 2.36 Mb. A diagnosis of Williams-Beuren syndrome was made based on the microdeletion of 7q11.23. The unique features, including unilateral microphthalmia and anterior segment dysgenesis, were unlikely to be caused by the microdeletion. Arguments in favor of the latter are unilateral manifestation, as well as the fact that numerous patients with deletions of comparable or microscopically visible size have not shown similar manifestations.

  4. Laypersons can successfully place supraglottic airways with 3 minutes of training. A comparison of four different devices in the manikin

    Directory of Open Access Journals (Sweden)

    Schälte Gereon

    2011-10-01

    Full Text Available Abstract Introduction Supraglottic airway devices have frequently been shown to facilitate airway management and are implemented in the ILCOR resuscitation algorithm. Limited data exists concerning laypersons without any medical or paramedical background. We hypothesized that even laymen would be able to operate supraglottic airway devices after a brief training session. Methods Four different supraglottic airway devices: Laryngeal Mask Classic (LMA, Laryngeal Tube (LT, Intubating Laryngeal Mask (FT and CobraPLA (Cobra were tested in 141 volunteers recruited in a technical university cafeteria and in a shopping mall. All volunteers received a brief standardized training session. Primary endpoint was the time required to definitive insertion. In a short questionnaire applicants were asked to assess the devices and to answer some general questions about BLS. Results The longest time to insertion was observed for Cobra (31.9 ± 27.9 s, range: 9-120, p 0.05, the LT (1.36 ± 0.61, p Conclusion Laypersons are able to operate supraglottic airway devices in manikin with minimal instruction. Ventilation was achieved with all devices tested after a reasonable time and with a high success rate of > 95%. The use of supraglottic airway devices in first aid and BLS algorithms should be considered.

  5. Involvement of epigenetic modifiers in the pathogenesis of testicular dysgenesis and germ cell cancer

    DEFF Research Database (Denmark)

    Lawaetz, Andreas C.; Almstrup, Kristian

    2015-01-01

    Testicular germ cell cancer manifests mainly in young adults as a seminoma or non-seminoma. The solid tumors are preceded by the presence of a non-invasive precursor cell, the carcinoma in situ cell (CIS), which shows great similarity to fetal germ cells. It is therefore hypothesized that the CIS...... of epigenetic modifiers with a focus on jumonji C enzymes in the development of testicular dysgenesis and germ cell cancer in men....... cell is a fetal germ cell that has been arrested during development due to testicular dysgenesis. CIS cells retain a fetal and open chromatin structure, and recently several epigenetic modifiers have been suggested to be involved in testicular dysgenesis in mice. We here review the possible involvement...

  6. Ascus dysgenesis in hybrid crosses of Neurospora and Sordaria (Sordariaceae).

    Science.gov (United States)

    Kasbekar, Durgadas P

    2017-07-01

    When two lineages derived from a common ancestor become reproductively isolated (e.g. Neurospora crassa and N. tetrasperma), genes that have undergone mutation and adaptive evolution in one lineage can potentially become dysfunctional when transferred into the other, since other genes have undergone mutation and evolution in the second lineage, and the derived alleles were never 'tested' together before hybrid formation. Bateson (1909), Dobzhansky (1936), and Muller (1942) recognized that incompatibility between the derived alleles could potentially make the hybrid lethal, sterile, or display some other detriment. Alternatively, the detrimental effects seen in crosses with the hybrids may result from the silencing of ascus-development genes by meiotic silencing by unpaired DNA (MSUD). Aberrant transcripts from genes improperly paired in meiosis are processed into single-stranded MSUD-associated small interfering RNA (masiRNA), which is used to degrade complementary mRNA. Recently, backcrosses of N. crassa / N. tetrasperma hybrid translocation strains with wild-type N. tetrasperma were found to elicit novel ascus dysgenesis phenotypes. One was a transmission ratio distortion that apparently disfavoured the homokaryotic ascospores formed following alternate segregation. Another was the production of heterokaryotic ascospores in eight-spored asci. Lewis (1969) also had reported sighting rare eight-spored asci with heterokaryotic ascospores in interspecific crosses in Sordaria, a related genus. Ordinarily, in both Neurospora and Sordaria, the ascospores are partitioned at the eight-nucleus stage, and ascospores in eight-spored asci are initially uninucleate. Evidently, in hybrid crosses of the family Sordariaceae, ascospore partitioning can be delayed until after one or more mitoses following the postmeiotic mitosis.

  7. A mutation in the nucleoporin-107 gene causes XX gonadal dysgenesis.

    Science.gov (United States)

    Weinberg-Shukron, Ariella; Renbaum, Paul; Kalifa, Rachel; Zeligson, Sharon; Ben-Neriah, Ziva; Dreifuss, Amatzia; Abu-Rayyan, Amal; Maatuk, Noa; Fardian, Nilly; Rekler, Dina; Kanaan, Moien; Samson, Abraham O; Levy-Lahad, Ephrat; Gerlitz, Offer; Zangen, David

    2015-11-02

    Ovarian development and maintenance are poorly understood; however, diseases that affect these processes can offer insights into the underlying mechanisms. XX female gonadal dysgenesis (XX-GD) is a rare, genetically heterogeneous disorder that is characterized by underdeveloped, dysfunctional ovaries, with subsequent lack of spontaneous pubertal development, primary amenorrhea, uterine hypoplasia, and hypergonadotropic hypogonadism. Here, we report an extended consanguineous family of Palestinian origin, in which 4 females exhibited XX-GD. Using homozygosity mapping and whole-exome sequencing, we identified a recessive missense mutation in nucleoporin-107 (NUP107, c.1339G>A, p.D447N). This mutation segregated with the XX-GD phenotype and was not present in available databases or in 150 healthy ethnically matched controls. NUP107 is a component of the nuclear pore complex, and the NUP107-associated protein SEH1 is required for oogenesis in Drosophila. In Drosophila, Nup107 knockdown in somatic gonadal cells resulted in female sterility, whereas males were fully fertile. Transgenic rescue of Drosophila females bearing the Nup107D364N mutation, which corresponds to the human NUP107 (p.D447N), resulted in almost complete sterility, with a marked reduction in progeny, morphologically aberrant eggshells, and disintegrating egg chambers, indicating defective oogenesis. These results indicate a pivotal role for NUP107 in ovarian development and suggest that nucleoporin defects may play a role in milder and more common conditions such as premature ovarian failure.

  8. Is testicular dysgenesis syndrome a genetic, endocrine, or environmental disease, or an unexplained reproductive disorder?

    Science.gov (United States)

    Xing, Jian-Sheng; Bai, Zhi-Ming

    2018-02-01

    Progressive increases in the incidence of male reproductive disorders inclusive of hypospadias, cryptorchidism, poor semen quality, and testicular germ cell cancer (TGCC) have been observed in recent times. The central hypothesis of this study asserted that these disorders may all collectively signify testicular dysgenesis syndrome (TDS). This review aimed to provide evidence verifying the reality of TDS based on four key aspects: environmental endocrine-disrupting chemicals (EDCs), genetic factors, intrauterine growth disorders and lifestyle factors. Although TDS might result from genetic polymorphisms or aberration, recent evidence has highlighted links indicating the conditions associations to both environmental and lifestyle factors due to the rapid temporal changes in the clinical symptoms observed over recent decades. Based on our review of genetic and environmental factors, a key observation of our study suggested that there is an urgent need to prioritize research in reproductive physiology and pathophysiology, particularly in highly industrialized countries facing decreasing populations. At present, current research has yet to elucidate the mechanisms of TDS, in addition to the lack of genuine consideration of a variety of potentially key factors and TDS mechanisms. In conclusion, our study revealed that environmental exposures owing to modern lifestyles are primary factors involved in the associated trends of the syndrome, which are capable of affecting the adult endocrine system via direct means or through epigenetic mechanisms. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Dysgerminoma in a case of 46, XY pure gonadal dysgenesis (swyer syndrome: a case report

    Directory of Open Access Journals (Sweden)

    He Anguang

    2011-09-01

    Full Text Available Abstract Simple 46, XY gonadal dysgenesis syndrome, also called Swyer syndrome, is known as pure gonadal dysgenesis. Individuals with the syndrome are characterized by 46, XY karyotype and phenotypically female with female genital appearance, normal Müllerian structures and absent testicular tissue. The condition usually first becomes apparent in adolescence with delayed puberty and primary amenorrhea due to the gonads have no hormonal or reproductive potential. Herein, we report a case of dysgerminoma diagnosed in a dysgenetic gonad of a 21-year-old patient with Swyer syndrome.

  10. Concurrent chemoradiotherapy for laryngeal preservation in T1/T2 supraglottic squamous cell carcinoma

    International Nuclear Information System (INIS)

    Rikimaru, Fumihide; Matsuo, Mioko; Taura, Masahiko; Higaki, Yuichiro; Tomita, Kichinobu

    2012-01-01

    We treated supraglottic squamous cell carcinoma (T1/2) with chemoradiation for laryngeal preservation and evaluated the effects of the chemoradiation at the dose of 40 Gy as an intermediate evaluation. To investigate the need for this intermediate evaluation, we retrospectively analyzed 46 patients, 43 men and 3 women aged 49 to 86 years, with supraglottic squamous cell carcinoma (T1/2) treated at our institution from January 1997 to May 2008. Overall and cause-specific three-year survival rates were 65% and 77% in all cases, 67% and 75% in T1, and 65% and 77% in T2. The three-year preservation rate of the larynx was 41% in all cases, 51% in T1, and 35% in T2. In the intermediate evaluation, the complete response rate was 58% in all cases, 77% in T1, and 48% in T2. In the cases of larynx preservation, the recurrence rate of the primary site was not significantly different between those cases who did not achieve complete response in the intermediate evaluation and those who did achieve complete response. (author)

  11. Dysgenesie spenique et syndrome d'ivemark | Balaka | Journal de la ...

    African Journals Online (AJOL)

    The authors report a Togolese case of splenic dysgenesis associating in a boy aged of 6 years: a splenic hypoplasia, a median and hypertrophic liver, an abdominal aorta and an inferior vena cava all both situated at the right side of the backbone, a dextrocardia by dextroversion, a single auricle and a complete type of ...

  12. The role of primary radiotherapy for squamous cell carcinoma of the supraglottic larynx

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Won Taek; Kim, Dong Won; Kwon, Byung Hyun; Nam, Ji Ho [College of Medicine, Pusan National Univ, Pusan (Korea, Republic of); Hur, Won Joo [College of Medicine, Donga Univ, Pusan (Korea, Republic of)

    2000-12-01

    First of all, this study was performed to assess the result of curative radiotherapy and to evaluate different possible prognostic factors for squamous cell carcinoma of the supraglottic larynx treated at the Pusan National University Hospital. The second goal of this study was by comparing our data with those of other study groups, to determine the better treatment policy of supraglottic cancer in future. Thirty-two patients with squamous cell carcinoma of the supraglottic larynx were treated with radiotherapy at Pusan National University Hospital, from August 1985 to December 1996. Minimum follow-up period was 29 months. Twenty-seven patients (84.4%.) were followed up over 5 years. Radiotherapy was delivered with 6 MV photons to the primary laryngeal tumor and regional lymphatics with shrinking field technique. All patients received radiotherapy under conventional fractionated schedule (once a. day). Median total tumor dose was 70.2 Gy (range, 55.8 to 75.6 Gy) on primary or gross tumor lesion. Thirteen patients had induction chemotherapy with cisplatin and 5-fluorouracil (1-3 cycles). Patient distribution, according to the different stages, were as follows: stage I, 5/32 (15.6%); stage II, 10/32 (31.3%); stage III, 8/32 (25%); stage IV, 9/32 (28. 1 %). The 5-year overall survival rate of the whole series (32 patients) was 51.7%. The overall survival rate at 5-years was 80% in stage I, 66.7% in stage II, 42.9% in stage III, 25% in stage IV.(p= 0,0958). The 5-year local control rates after radiotherapy were as follows: stage I, 100%; stage II, 60%; stage III, 62.5%; stage lV, 44.4% (p=0.233). Overall vocal preservation rates was 65.6%,100% in stage I, 70% in stage II, 62.5% in stage III, 44.4% in stage IV (p=0.210). There was no statistical significance in survival and local control rate between neoadjuvant chemotherapy followed by radiotherapy group and radiotherapy alone group. Severe laryngeal edema was found in 2 cases after radiotherapy. emergent tracheostomy

  13. The role of primary radiotherapy for squamous cell carcinoma of the supraglottic larynx

    International Nuclear Information System (INIS)

    Kim, Won Taek; Kim, Dong Won; Kwon, Byung Hyun; Nam, Ji Ho; Hur, Won Joo

    2000-01-01

    First of all, this study was performed to assess the result of curative radiotherapy and to evaluate different possible prognostic factors for squamous cell carcinoma of the supraglottic larynx treated at the Pusan National University Hospital. The second goal of this study was by comparing our data with those of other study groups, to determine the better treatment policy of supraglottic cancer in future. Thirty-two patients with squamous cell carcinoma of the supraglottic larynx were treated with radiotherapy at Pusan National University Hospital, from August 1985 to December 1996. Minimum follow-up period was 29 months. Twenty-seven patients (84.4%.) were followed up over 5 years. Radiotherapy was delivered with 6 MV photons to the primary laryngeal tumor and regional lymphatics with shrinking field technique. All patients received radiotherapy under conventional fractionated schedule (once a. day). Median total tumor dose was 70.2 Gy (range, 55.8 to 75.6 Gy) on primary or gross tumor lesion. Thirteen patients had induction chemotherapy with cisplatin and 5-fluorouracil (1-3 cycles). Patient distribution, according to the different stages, were as follows: stage I, 5/32 (15.6%); stage II, 10/32 (31.3%); stage III, 8/32 (25%); stage IV, 9/32 (28. 1 %). The 5-year overall survival rate of the whole series (32 patients) was 51.7%. The overall survival rate at 5-years was 80% in stage I, 66.7% in stage II, 42.9% in stage III, 25% in stage IV.(p= 0,0958). The 5-year local control rates after radiotherapy were as follows: stage I, 100%; stage II, 60%; stage III, 62.5%; stage lV, 44.4% (p=0.233). Overall vocal preservation rates was 65.6%,100% in stage I, 70% in stage II, 62.5% in stage III, 44.4% in stage IV (p=0.210). There was no statistical significance in survival and local control rate between neoadjuvant chemotherapy followed by radiotherapy group and radiotherapy alone group. Severe laryngeal edema was found in 2 cases after radiotherapy. emergent tracheostomy

  14. A Biallelic Mutation in the Homologous Recombination Repair Gene SPIDR Is Associated With Human Gonadal Dysgenesis.

    Science.gov (United States)

    Smirin-Yosef, Pola; Zuckerman-Levin, Nehama; Tzur, Shay; Granot, Yaron; Cohen, Lior; Sachsenweger, Juliane; Borck, Guntram; Lagovsky, Irina; Salmon-Divon, Mali; Wiesmüller, Lisa; Basel-Vanagaite, Lina

    2017-02-01

    Primary ovarian insufficiency (POI) is caused by ovarian follicle depletion or follicle dysfunction, characterized by amenorrhea with elevated gonadotropin levels. The disorder presents as absence of normal progression of puberty. To elucidate the cause of ovarian dysfunction in a family with POI. We performed whole-exome sequencing in 2 affected individuals. To evaluate whether DNA double-strand break (DSB) repair activities are altered in biallelic mutation carriers, we applied an enhanced green fluorescent protein-based assay for the detection of specific DSB repair pathways in blood-derived cells. Diagnoses were made at the Pediatric Endocrine Clinic, Clalit Health Services, Sharon-Shomron District, Israel. Genetic counseling and sample collection were performed at the Pediatric Genetics Unit, Schneider Children's Medical Center Israel, Petah Tikva, Israel. Two sisters born to consanguineous parents of Israeli Muslim Arab ancestry presented with a lack of normal progression of puberty, high gonadotropin levels, and hypoplastic or absent ovaries on ultrasound. Blood samples for DNA extraction were obtained from all family members. Exome analysis to elucidate the cause of POI in 2 affected sisters. Analysis revealed a stop-gain homozygous mutation in the SPIDR gene (KIAA0146) c.839G>A, p.W280*. This mutation altered SPIDR activity in homologous recombination, resulting in the accumulation of 53BP1-labeled DSBs postionizing radiation and γH2AX-labeled damage during unperturbed growth. SPIDR is important for ovarian function in humans. A biallelic mutation in this gene may be associated with ovarian dysgenesis in cases of autosomal recessive inheritance. Copyright © 2017 by the Endocrine Society

  15. Radiotherapy in supraglottic carcinoma -with respect to locoregional control and survival-

    International Nuclear Information System (INIS)

    Nam, Taek Keun; Oh, Yoon Kyeong; Chung, Woong Ki; Cho, Jae Shik; Ahn, Sung Ja; Nah, Byung Sik

    2002-01-01

    A retrospective study was undertaken to determine the role of conventional radiotherapy with or without surgery for treating a supraglottic carcinoma in terms of the local control and survival. From Jan. 1986 to Oct. 1996, a total of 134 patients were treated for a supraglottic carcinoma by radiotherapy with or without surgery. Of them, 117 patients who had completed the radiotherapy formed the base of this study. The patients were redistributed according to the revised AJCC staging system (1997). The number of patients of stage I, II, III, IVA, IVB were 6 (5%), 16 (14%), 53 (45%), 32 (27%), 10 (9%), respectively. Eighty patients were treated by radical radiotherapy in the range of 61.2 ∼ 79.2 Gy (mean:69.2 Gy) to the primary tumor and 45.0 ∼ 93.6 Gy (mean:54.0 Gy) to regional lymphatics. All patients with stage I and IVB were treated by radiotherapy alone. Thirty-seven patients underwent surgery plus postoperative radiotherapy in the range of 45.0 ∼ 68.4 Gy (mean:56.1 Gy) to the primary tumor bed and 45.0 ∼ 59.4 Gy (mean:47.2 Gy) to the regional lymphatics. Of them, 33 patients received a total laryngectomy (± lymph node dissection), three had a supraglottic horizontal laryngectomy (± lymph node dissection), and one had a primary excision alone. The 5-year survival rate (5YSR) of all patients was 43%. The 5YSR of the patients with stage I + II, III + IV were 49.9%, 41.2%, respectively (ρ = 0.27). However, the disease-specific survival rate of the patients with stage I (n=6) was 100%. The 5YSRs of patients who underwent surgery plus radiotherapy (S + RT) vs radiotherapy alone (RT) in stage II, III, IVA were 100% vs 43% (ρ = 0.17), 62% vs 52% (ρ =0.32), 58% vs 6% (ρ < 0.001), respectively. The 5-year actuarial locoregional control rate (5YLCR) of all the patients was 57%. The 5YLCR of the patients with stage I, II, III, IVA, IVB was 100%, 74%, 60%, 44%, 30%, respectively (ρ = 0.008). The 5YLCR of the patients with S + RT vs RT in stage II, III, IVA

  16. Laryngeal sarcoidosis: a case report presenting transglottic involvement.

    Science.gov (United States)

    van den Broek, Emke M J M; Heijnen, Bas J; Verbist, Berit M; Sjögren, Elisabeth V

    2013-09-01

    Isolated laryngeal sarcoidosis is a very rare disease. In most cases, it will present as a supraglottic pale edematous swelling. In our case, the patient presented with hoarseness and dyspnea during exertion. Laryngeal examination did show not only supraglottic edema but also prominent subglottic swelling and edematous true vocal folds. Histology showed noncaseating granulomas. After excluding other causes and localizations, the patient was diagnosed with laryngeal sarcoidosis and treated with systemic corticosteroid with good result. We describe our case of isolated transglottic sarcoidosis and discuss the disease, its presentation, diagnosis, and therapeutic options. Copyright © 2013 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  17. Salvage surgery following radiation failure in squamous cell carcinoma of the supraglottic larynx

    International Nuclear Information System (INIS)

    Parsons, James T.; Mendenhall, William M.; Stringer, Scott P.; Cassisi, Nicholas J.; Million, Rodney R.

    1995-01-01

    Purpose: We analyzed the clinical course of patients who developed local (primary) recurrence following high-dose irradiation of squamous cell carcinoma of the supraglottic larynx. Methods and Materials: Between October 1964 and July 1991, 206 patients with previously untreated squamous cell carcinoma of the supraglottic larynx underwent radiotherapy with curative intent. Local failure occurred in 46 (22%) patients. Successful surgical salvage was defined as no evidence of recurrent cancer for at least 2 years after salvage surgery and continuously thereafter. Results: Sixteen patients did not undergo salvage surgery because of refusal (7 patients), severe medical illness (2 patients), concurrent distant metastasis (5 patients), or unresectable neck disease (2 patients). Twenty-six patients underwent total laryngectomy, and 4 patients had a voice-sparing procedure. Successful salvage was achieved in 50% of patients who underwent surgery. The rate of successful salvage did not correlate with preirradiation T-stage or time to failure after irradiation. Most of the failures after surgery were because of failure to control the primary cancer. The overall rate of postsurgical complications was 37%. No operative or perioperative deaths occurred. The 5-year survival rate for all 46 patients, calculated from the date of irradiation failure, was 20%, while the 5-year survival rate after salvage surgery for the 30 patients who underwent the procedure was 29%. Conclusion: There are few data in the literature regarding the clinical outcome in patients whose tumors are not controlled by initial radiotherapy. In the current and previous series, one-half to two-thirds of patients who developed primary failure underwent salvage surgery, which was successful in approximately half of the operated patients, leading to a 25-30% rate of long-term disease-free survival among the entire group of patients who developed failure

  18. Dysgenesis of the corpus callosum and associated malformaaation{sup :} computed tomography and magnetic resonance imaging findings; Disgenesia do corpo caloso e mas-formacoes associadas: achados de tomografia computadorizada e ressonancia magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Montandon, Cristiano; Montandon Junior, Marcelo Eustaquio [Colegio Brasileiro de Radiologia e Diagnostico por Imagem (CBR), Sao Paulo, SP (Brazil); Ribeiro, Flavia Aparecida de Sousa; Lobo, Leonardo Valadares Barbosa; Teixeira, Kim-Ir-Sen Santos [Goias Univ., Goiania (Brazil). Hospital de Clinicas. Dept. de Diagnostico por Imagem e Anatomia Patologica]. E-mail: cabeca2@terra.com.br

    2003-10-01

    Callosal dysgenesis is a malformation of the corpus callosum with origins in the embryogenesis of the telencephalon. We reviewed the computed tomography and magnetic resonance imaging findings of 11 patients with callosal dysgenesis and one patient with normal corpus callosum associated with a lipoma. The patients were divided into three distinct groups: total agenesis (three patients), partial agenesis (six patients) and hypoplasia (two patients). Associated abnormalities were observed in nine patients, including Chiari II malformation (one patient), schizencephaly (one patient), interhemispheric cyst (two patients), Dandy-Walker cyst (one patient), nodular heterotopy (one patient) and lipoma of the corpus callosum (four patients). This paper presents a review that may contribute to the diagnosis of these disorders. (author)

  19. Testicular dysgenesis syndrome and the development and occurrence of male reproductive disorders

    DEFF Research Database (Denmark)

    Virtanen, H E; Rajpert-De Meyts, E; Main, K M

    2005-01-01

    links so that countries with high incidence of testicular cancer, such as Denmark, tend to also have high prevalence rates of cryptorchidism and hypospadias and poor semen quality. Vice versa, in countries with good male reproductive health, e.g., in Finland, all these aspects are better than in Denmark...... the most severe form of testicular dysgenesis syndrome (TDS). We have hypothesized that testicular cancer, cryptorchidism, hypospadias and poor spermatogenesis are all signs of a developmental disturbance that was named as testicular dysgenesis syndrome. The hypothesis is based on clinical...... and epidemiological findings and on biological and experimental evidence. Signs of TDS share several risk factors, such as small birth weight (particularly being small for gestational age), and they are risk factors for each other. All of them have background in fetal development. They show strong epidemiological...

  20. A rare case of 46,XX gonadal dysgenesis and Mayer-Rokitansky-Kuster-Hauser syndrome.

    Science.gov (United States)

    Manne, Sriharibabu; Veeraabhinav, C H; Jetti, Mounica; Himabindu, Yalamanchali; Donthu, Kiranmai; Badireddy, Mutyalarayudu

    2016-01-01

    46,XX gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46,XX karyotype. The phenotypic manifestations of MRKH syndrome may sometimes overlap with various other syndromes and require accurate delineation. The coexistence of both these disorders is extremely rare. Here, we report a case of 46,XX gonadal dysgenesis and MRKH syndrome with anatomically dispersed congenital anomalies unique among reported cases.

  1. Histological evidence of testicular dysgenesis in contralateral biopsies from 218 patients with testicular germ cell cancer

    DEFF Research Database (Denmark)

    Hoei-Hansen, Christina E; Holm, Mette; Rajpert-De Meyts, Ewa

    2003-01-01

    dysgenesis, microscopic dysgenetic features were quantified in contralateral testicular biopsies in patients with a testicular germ cell tumour. Two hundred and eighty consecutive contralateral testicular biopsies from Danish patients with testicular cancer diagnosed in 1998-2001 were evaluated...... retrospectively. Two hundred and eighteen specimens were subsequently included in this study, after 63 patients who did not meet inclusion criteria had to be excluded. The presence of carcinoma in situ (which is believed to originate from transformed gonocytes) was detected in 8.7% of biopsies. The incidence...... patients, areas with immature and morphologically distorted tubules were also noted. Spermatogenesis was qualitatively normal in 51.4%, whereas 11.5% had very poor or absent spermatogenesis. It is concluded that microscopic testicular dysgenesis is a frequent feature in contralateral biopsies from patients...

  2. Corpus callosum dysgenesis and lipoma: embryologic and magnetic resonance imaging aspects; Disgenesia do corpo caloso e lipoma: consideracoes embriologicas basicas e aspectos de imagem a ressonancia magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Abreu Junior, Luiz de; Borri, Maria Lucia; Wolosker, Angela Maria Borri; Hartmann, Luiz Guilherme de Carvalho; Galvao Filho, Mario de Melo [Hospital e Maternidade Sao Luiz, Sao Paulo, SP (Brazil). Setor de Diagnostico por Imagem]. E-mail: abreujr@directnet.com.br; D' Ippolito, Giuseppe [Hospital e Maternidade Sao Luiz, Sao Paulo, SP (Brazil). Setor de US/TC/RMN

    2005-07-15

    The corpus callosum is the major system of association fibers that permits communication of both cerebral hemispheres. Magnetic resonance imaging has improved the study of brain malformations, including the corpus callosum dysgenesis. Lipoma is a common finding in the spectrum of corpus callosum dysgenesis. The purpose of these study was to review the embryologic events and the magnetic resonance imaging aspects related to the corpus callosum dysgenesis and to the formation of the related lipoma. (author)

  3. Videolaryngoscopy versus Fiber-optic Intubation through a Supraglottic Airway in Children with a Difficult Airway: An Analysis from the Multicenter Pediatric Difficult Intubation Registry.

    Science.gov (United States)

    Burjek, Nicholas E; Nishisaki, Akira; Fiadjoe, John E; Adams, H Daniel; Peeples, Kenneth N; Raman, Vidya T; Olomu, Patrick N; Kovatsis, Pete G; Jagannathan, Narasimhan; Hunyady, Agnes; Bosenberg, Adrian; Tham, See; Low, Daniel; Hopkins, Paul; Glover, Chris; Olutoye, Olutoyin; Szmuk, Peter; McCloskey, John; Dalesio, Nicholas; Koka, Rahul; Greenberg, Robert; Watkins, Scott; Patel, Vikram; Reynolds, Paul; Matuszczak, Maria; Jain, Ranu; Khalil, Samia; Polaner, David; Zieg, Jennifer; Szolnoki, Judit; Sathyamoorthy, Kumar; Taicher, Brad; Riveros Perez, N Ricardo; Bhattacharya, Solmaletha; Bhalla, Tarun; Stricker, Paul; Lockman, Justin; Galvez, Jorge; Rehman, Mohamed; Von Ungern-Sternberg, Britta; Sommerfield, David; Soneru, Codruta; Chiao, Franklin; Richtsfeld, Martina; Belani, Kumar; Sarmiento, Lina; Mireles, Sam; Bilen Rosas, Guelay; Park, Raymond; Peyton, James

    2017-09-01

    The success rates and related complications of various techniques for intubation in children with difficult airways remain unknown. The primary aim of this study is to compare the success rates of fiber-optic intubation via supraglottic airway to videolaryngoscopy in children with difficult airways. Our secondary aim is to compare the complication rates of these techniques. Observational data were collected from 14 sites after management of difficult pediatric airways. Patient age, intubation technique, success per attempt, use of continuous ventilation, and complications were recorded for each case. First-attempt success and complications were compared in subjects managed with fiber-optic intubation via supraglottic airway and videolaryngoscopy. Fiber-optic intubation via supraglottic airway and videolaryngoscopy had similar first-attempt success rates (67 of 114, 59% vs. 404 of 786, 51%; odds ratio 1.35; 95% CI, 0.91 to 2.00; P = 0.16). In subjects less than 1 yr old, fiber-optic intubation via supraglottic airway was more successful on the first attempt than videolaryngoscopy (19 of 35, 54% vs. 79 of 220, 36%; odds ratio, 2.12; 95% CI, 1.04 to 4.31; P = 0.042). Complication rates were similar in the two groups (20 vs. 13%; P = 0.096). The incidence of hypoxemia was lower when continuous ventilation through the supraglottic airway was used throughout the fiber-optic intubation attempt. In this nonrandomized study, first-attempt success rates were similar for fiber-optic intubation via supraglottic airway and videolaryngoscopy. Fiber-optic intubation via supraglottic airway is associated with higher first-attempt success than videolaryngoscopy in infants with difficult airways. Continuous ventilation through the supraglottic airway during fiber-optic intubation attempts may lower the incidence of hypoxemia.

  4. Hybrid Dysgenesis in DROSOPHILA MELANOGASTER: Nature and Inheritance of P Element Regulation

    OpenAIRE

    Kidwell, Margaret G.

    1985-01-01

    The genetic determination of the control of resistance or susceptibility to germ line changes mediated by P elements was studied in two strains and in derivatives of crosses between them. One strain, characterized as true M, completely lacked P elements. The second strain, pseudo- M (M'), carried a number of P elements, but these did not have the potential to induce the gonadal sterility that is associated with P-M hybrid dysgenesis. Individuals from the true M strain were invariably unable ...

  5. Cryptorchidism as part of the testicular dysgenesis syndrome: the environmental connection

    DEFF Research Database (Denmark)

    Main, K M; Skakkebaek, N E; Toppari, J

    2009-01-01

    Cryptorchidism is part of the testicular dysgenesis syndrome (TDS), which includes other male reproductive disorders such as hypospadias, testis cancer and reduced semen quality. These diseases appear to be linked by common pathogenic mechanisms, interfering with normal fetal testis development. ...... and maternal smoking during pregnancy also appeared to be a risk factor for cryptorchidism. Thus, current evidence suggests that the development of the male reproductive tract may be susceptible to adverse effects of environmental hormone disrupters....

  6. Value of computed tomography as outcome predictor of supraglottic squamous cell carcinoma treated by definitive radiation therapy

    International Nuclear Information System (INIS)

    Hermans, Robert; Bogaert, Walter van den; Rijnders, Alexis; Baert, Albert L.

    1999-01-01

    Purpose: To investigate the value of several CT-derived tumor parameters as predictors of local outcome of supraglottic squamous cell carcinoma treated by definitive radiation therapy. Methods and Materials: The pretreatment CT studies of 103 patients with supraglottic squamous cell carcinoma were reviewed for tumoral involvement of specific laryngeal anatomic subsites and extralaryngeal tumor spread. After redigitizing the films, tumor volume was calculated with the summation-of-areas technique. Mean follow-up time was 3.4 years. Actuarial statistical analysis of local and locoregional outcome was done for each of the covariates; multivariate analysis was performed using Cox's proportional hazards model. Results: In the actuarial analysis CT-determined primary tumor volume was significantly correlated with local recurrence rate (p < 0.001). Degree of involvement of the paraglottic space at the level of the true vocal cord (p < 0.05) and subglottic extension (p < 0.001) were also significantly correlated with local recurrence rate. In the multivariate analysis, only degree of involvement of the preepiglottic space (p < 0.01) and subglottic extension (p < 0.01) were found to be independent predictors of local recurrence. Total tumor volume was the strongest independent predictor of locoregional failure (p < 0.01). Conclusions: CT-determined tumor parameters are strong predictors of local and locoregional outcome of supraglottic carcinoma treated by definitive irradiation

  7. Pediatric supraglottic airway devices in clinical practice: A prospective observational study.

    Science.gov (United States)

    Kleine-Brueggeney, Maren; Gottfried, Anne; Nabecker, Sabine; Greif, Robert; Book, Malte; Theiler, Lorenz

    2017-09-02

    Supraglottic airway devices (SGA) are commonly used in pediatric anesthesia and serve as primary or back-up devices for difficult airway management. Most SGA are marketed without proper clinical evaluation. The purpose of this study was to evaluate the performance of the pediatric LMA Supreme™, Air-Q® and Ambu® Aura-i™. This prospective observational study was performed at Bern University Hospital, Switzerland. With ethics committee approval and a waiver for written informed consent 240 children undergoing elective surgery with an ASA class I-III and a weight of 5-30 kg were included. Three different pediatric supraglottic airway devices were assessed: The LMA Supreme™, Air-Q® and Ambu® Aura-i™. Primary outcome parameter was airway leak pressure. Secondary outcome parameters included first attempt and overall success rate, insertion time, fiberoptic view through the SGA, and adverse events. The primary hypothesis was that the mean airway leak pressure of each tested SGA was 20 cmH 2 O ± 10%. None of the SGA showed a mean airway leak pressure of 20 cmH 2 O ± 10%, but mean airway leak pressures differed significantly between devices [LMA Supreme™ 18.0 (3.4) cmH 2 O, Air-Q® 15.9 (3.2) cmH 2 O, Ambu® Aura-i™ 17.3 (3.7) cmH 2 O, p < 0.001]. First attempt success rates (LMA Supreme™ 100%, Air-Q® 90%, Ambu® Aura-i™ 91%, p = 0.02) and overall success rates (LMA Supreme™ 100%, Air-Q® 91%, Ambu® Aura-i™ 95%, p = 0.02) also differed significantly. Insertion times ranged from 20 (7) seconds (Air-Q®) to 24 (6) seconds (LMA Supreme™,

  8. Are new supraglottic airway devices, tracheal tubes and airway viewing devices cost-effective?

    Science.gov (United States)

    Slinn, Simon J; Froom, Stephen R; Stacey, Mark R W; Gildersleve, Christopher D

    2015-01-01

    Over the past two decades, a plethora of new airway devices has become available to the pediatric anesthetist. While all have the laudable intention of improving patient care and some have proven clinical benefits, these devices are often costly and at times claims of an advantage over current equipment and techniques are marginal. Supraglottic airway devices are used in the majority of pediatric anesthetics delivered in the U.K., and airway-viewing devices provide an alternative for routine intubation as well as an option in the management of the difficult airway. Yet hidden beneath the convenience of the former and the technology of the latter, the impact on basic airway skills with a facemask and the lack of opportunities to fine-tune the core skill of intubation represent an unrecognised and unquantifiable cost. A judgement on this value must be factored into the absolute purchase cost and any potential benefits to the quality of patient care, thus blurring any judgement on cost-effectiveness that we might have. An overall value on cost-effectiveness though not in strict monetary terms can then be ascribed. In this review, we evaluate the role of these devices in the care of the pediatric patient and attempt to balance the advantages they offer against the cost they incur, both financial and environmental, and in any quality improvement they might offer in clinical care. © 2014 John Wiley & Sons Ltd.

  9. Testicular dysgenesis syndrome and the origin of carcinoma in situ testis.

    Science.gov (United States)

    Sonne, Si Brask; Kristensen, David Møbjerg; Novotny, Guy W; Olesen, Inge Ahlmann; Nielsen, John E; Skakkebaek, Niels E; Rajpert-De Meyts, Ewa; Leffers, Henrik

    2008-04-01

    Recent increases in male reproductive disorders have been linked to exposure to environmental factors leading to the testicular dysgenesis syndrome (TDS). Testicular cancer is the most severe condition in TDS and studies have shown a clear correlation between risk of testicular cancer and other components of TDS and that the geographical location of the mother during pregnancy can be a risk factor. This suggests that the dysgenesis has its origin in utero and that TDS is initiated by environmental factors, including possibly hormone-disrupting compounds that act on the mother and the developing foetus, but the genetic background may also play a role. The morphological similarity of carcinoma in situ (CIS) cells (the precursor of the majority of invasive testicular cancers) with primordial germ cells and gonocytes, and overlap in expression of protein markers suggests an origin of CIS from primordial germ cells or gonocytes. CIS cells and germ cell-derived cancers of the human type have so far not been described in any animal model of TDS, which could be caused by species differences in the development of the male gonad. Regardless of this, it is plausible that the dysgenesis, and hence the development of CIS cells, is a result of disturbed signalling between nurse cells and germ cells that allow embryonic germ cells to survive in the pre-pubertal and adult testis. The post-pubertal proliferation of CIS cells combined with aberrant signalling then leads to an accumulation of genetic changes in the CIS cells, which eventually results in the development of invasive testicular cancer in the adult.

  10. Mutations of the SRY-responsive enhancer of SOX9 are uncommon in XY gonadal dysgenesis.

    Science.gov (United States)

    Georg, I; Bagheri-Fam, S; Knower, K C; Wieacker, P; Scherer, Gerd; Harley, V R

    2010-01-01

    During mouse sex determination, SRY upregulates the core testis-specific enhancer of Sox9, TESCO. Mutations in human SRY are found in one third of cases with XY pure gonadal dysgenesis (XY GD; Swyer syndrome), while two thirds remain unexplained. Heterozygous SOX9 mutations can cause XY GD in association with the skeletal malformation syndrome campomelic dysplasia. We hypothesized that human TESCO mutations could cause isolated XY GD. Sixty-six XY GD cases with an intact SRY were analyzed for TESCO point mutations or deletions. No mutations were identified. We conclude that TESCO mutations are not a common cause of XY GD. Copyright © 2010 S. Karger AG, Basel.

  11. 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

  12. A rare case of mixed gonadal dysgenesis: 45, X0/ 46, X, +mar 1/ 46, X, +mar 2

    Directory of Open Access Journals (Sweden)

    Carolina Talini

    2016-07-01

    Full Text Available This study aims to report a rare case of mixed gonadal dysgenesis with mosaicism pattern 45 X0 / 46, X, +mar 1/ 46, X, +mar 2. Patient referred to the pediatric urology service with 2 months of age. Right after the baby was born it was not possible do determine the baby's gender. The karyotype was inconclusive: 45, X0. Contrast genitography and uretrocystogram showed a single perineal orifice below the micropenis and between the bifid scrotum, opacification of the bladder with male aspect urethra during urination and short penile urethra. There was also opacification of the posterior vaginal cavity with low junction to the posterior urethra and a linear opacity image suggesting uterine canal. Laparoscopy revealed bilateral gonadal bands and spermatic elements entering the inguinal canal. Müllerian derivatives were absent. Gonadal biopsy was performed and concluded that these were abnormal infantile testes. Microarray testing took a few months and resulted in: 45 X0 / 46, X, +mar 1/ 46, X, +mar 2. Patient was kept on topic androstenedione for 6 months and then underwent penoscrotal hypospadias correction. Mosaic chromosomal pattern is a rare condition that can present with a wide variety of phenotypes. Early recognition has important implications on these patients’ social and psychological life and also for their proper management.

  13. Do environmental factors play a role in the aetiology of carcinoma in situ testis and the testicular dysgenesis syndrome?

    Science.gov (United States)

    Sonne, S B; Hoei-Hansen, C E; Fisher, J S; Leffers, H; Rajpert-de Meyts, E; Skakkebaek, N E

    2004-01-01

    The hypothesis of the Testicular Dysgenesis Syndrome (TDS), first suggested in 2001, propose that several disorders of the male reproductive system such as infertility, hypospadias, cryptorchidism and testicular cancer are all symptoms of TDS, which is most likely initiated during early foetal development, and may be provoked by external factors such as endocrine disruptors in addition to genetic predisposition. Testicular germ cell tumours (TGCTs), considered the most severe symptom of TDS, have increased in incidence during the last 60 years, to become the most common malignancy in young Caucasian men aged 17-45 years. TGCTs of young men originate from carcinoma in situ (CIS) cells. In the last few years, progress has been made identifying candidate genes involved in the neoplastic development of CIS, which may elucidate the timing of the initiation of CIS, currently thought to originate in foetal life from primordial germ cells or early gonocytes. Histological dysgenetic features are frequently seen in testes affected with the TDS components testis cancer or cryptorchidism. A TDS-like phenotype can be induced in male rats by in utero exposure to high concentrations of dibutyl phthalate (DBP) suggesting that ubiquitously present environmental endocrine disruptors may play a role in the aetiology of human TDS. So far, no animal model has been able to mimick all the symptoms of TDS including TGCTs although CIS-like cells have been found in a spontaneous testicular neoplasm in a rabbit.

  14. 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

  15. 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

  16. XX ovarian dysgenesis is caused by a PSMC3IP/HOP2 mutation that abolishes coactivation of estrogen-driven transcription.

    Science.gov (United States)

    Zangen, David; Kaufman, Yotam; Zeligson, Sharon; Perlberg, Shira; Fridman, Hila; Kanaan, Moein; Abdulhadi-Atwan, Maha; Abu Libdeh, Abdulsalam; Gussow, Ayal; Kisslov, Irit; Carmel, Liran; Renbaum, Paul; Levy-Lahad, Ephrat

    2011-10-07

    XX female gonadal dysgenesis (XX-GD) is a rare, genetically heterogeneous disorder characterized by lack of spontaneous pubertal development, primary amenorrhea, uterine hypoplasia, and hypergonadotropic hypogonadism as a result of streak gonads. Most cases are unexplained but thought to be autosomal recessive. We elucidated the genetic basis of XX-GD in a highly consanguineous Palestinian family by using homozygosity mapping and candidate-gene and whole-exome sequencing. Affected females were homozygous for a 3 bp deletion (NM_016556.2, c.600_602del) in the PSMC3IP gene, leading to deletion of a glutamic acid residue (p.Glu201del) in the highly conserved C-terminal acidic domain. Proteasome 26S subunit, ATPase, 3-Interacting Protein (PSMC3IP)/Tat Binding Protein Interacting Protein (TBPIP) is a nuclear, tissue-specific protein with multiple functions. It is critical for meiotic recombination as indicated by the known role of its yeast ortholog, Hop2. Through the C terminus (not present in yeast), PSMC3IP also coactivates ligand-driven transcription mediated by estrogen, androgen, glucocorticoid, progesterone, and thyroid nuclear receptors. In cell lines, the p.Glu201del mutation abolished PSMC3IP activation of estrogen-driven transcription. Impaired estrogenic signaling can lead to ovarian dysgenesis both by affecting the size of the follicular pool created during fetal development and by failing to counteract follicular atresia during puberty. PSMC3IP joins previous genes known to be mutated in XX-GD, the FSH receptor, and BMP15, highlighting the importance of hormonal signaling in ovarian development and maintenance and suggesting a common pathway perturbed in isolated XX-GD. By analogy to other XX-GD genes, PSMC3IP is also a candidate gene for premature ovarian failure, and its role in folliculogenesis should be further investigated. Copyright © 2011 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  17. A new laryngeal mask supraglottic airway device with integrated balloon line: a descriptive and comparative bench study

    Directory of Open Access Journals (Sweden)

    Zhou YH

    2016-11-01

    Full Text Available YingHai Zhou,1 Korinne Jew2 1Research & Development, Patient Monitoring & Recovery, Medtronic Technology Center, Shanghai, People’s Republic of China; 2Medical Affairs, Minimally Invasive Therapies Group, Medtronic, Boulder, CO, USA Abstract: Laryngeal masks are invasive devices for airway management placed in the supraglottic position. The Shiley™ laryngeal mask (Shiley™ LM features an integrated inflation tube and airway shaft to facilitate product insertion and reduce the chance of tube occlusion when patients bite down. This study compared the Shiley LM to two other disposable laryngeal mask devices, the Ambu® AuraStraight™ and the LMA Unique™. Overall device design, tensile strength, flexibility of various structures, and sealing performance were measured. The Shiley LM is structurally stronger and its shaft is more resistant to compression than the other devices. The Shiley LM is generally less flexible than the other devices, but this relationship varies with device size. Sealing performance of the devices was similar in a bench assay. The results of this bench study demonstrate that the new Shiley LM resembles other commercially available laryngeal mask devices, though it exhibits greater tensile strength and lower flexibility. Keywords: laryngeal mask, supraglottic airway, supralaryngeal device

  18. A feasibility study of concomitant boost radiotheraphy for patients with cancer of the supraglottic larynx

    International Nuclear Information System (INIS)

    Bujko, K.; Skoczylas, J.Z.; Bentzen, S.M.; Hliniak, A.; Wasilewski, M.; Szutkowski, Z.J.; Osmolski, A.

    1993-01-01

    Between February 1988 and December 1989, 65 patients with supraglottic cancer completed a course of concomitant boost radiotherapy. Cases with N3 disease, a Karnovsky performance score less than 70, age above 70 years, or a second primary cancer were not eligible for the study. Distribution of the patients was: Stage T1-T2 30%, T3-T4 70%, N0 68%, N+32%. The total dose ranged from 60 Gy to 76 Gy (median 66 Gy); overall treatment time ranged from 36 to 56 days with a median of 42 days. The daily dose during the first 4 weeks was 1.8 Gy, and during the last 2 weeks it was 1.6 Gy b.i.d. with a 4- or (after September 1988) 6-h interval. The clinical impression was that the early mucosal reactions were acceptable but more severe than after conventional treatment, with confluent membranous mucosal reaction being observed in 54% of the patients. Also, this reaction was significantly more frequent in patients treated with a 4-h interval (68%) than with a 6-h interval (41%) between the daily fractions. To relieve severe dysphagia, narcotics were required in 22% of the patients. The follow-up time ranged from 22 to 50 months, median 34 months. Treatment-requiring late complications were observed in 8 patients, and the 3-year actuarial risk was 17% with 95% confidence limits (6%, 27%). Two of these patients had severe complications: One of them required a temporary tracheostomy due to arytenoid edema and the other developed a laryngo-cutaneous fistula which healed after pharmacological treatment. Actuarial 3-year local-regional control was 59% (46%, 71%) and 3-year actuarial crude survival 55% (42%, 67%). There was no significant difference in the incidence of late complications or tumor control between the two groups of patients treated with a 4- or 6-h interval between the daily fractions. This study shows that the concomitant boost regimen tired here is feasible, but also stresses that the interval between dose fractions should be 6 h or more. (orig.)

  19. Comparison Of The I-Gel Supraglottic And King Laryngotracheal Airways In A Simulated Tactical Environment.

    Science.gov (United States)

    March, Juan A; Tassey, Theresa E; Resurreccion, Noel B; Portela, Roberto C; Taylor, Stephen E

    2018-01-01

    When working in a tactical environment there are several different airway management options that exist. One published manuscript suggests that when compared to endotracheal intubation, the King LT laryngotracheal airway (KA) device minimizes time to successful tube placement and minimizes exposure in a tactical environment. However, comparison of two different blind insertion supraglottic airway devices in a tactical environment has not been performed. This study compared the I-Gel airway (IGA) to the KA in a simulated tactical environment, to determine if one device is superior in minimizing exposure and minimizing time to successful tube placement. This prospective randomized cross over trial was performed using the same methods and tactical environment employed in a previously published study, which compared endotracheal intubation versus the KA in a tactical environment. The tactical environment was simulated with a one-foot vertical barrier. The participants were paramedic students who wore an Advanced Combat Helmet (ACH) and a ballistic vest (IIIA) during the study. Participants were then randomized to perform tactical airway management on an airway manikin with either the KA or the IGA, and then again using the alternate device. The participants performed a low military type crawl and remained in this low position during each tube placement. We evaluated the time to successful tube placement between the IGA and KA. During attempts, participants were videotaped to monitor their height exposure above the barrier. Following completion, participants were asked which airway device they preferred. Data was analyzed using Student's t-test across the groups for time to ventilation and height of exposure. In total 19 paramedic students who were already at the basic EMT level participated. Time to successful placement for the KA was 39.7 seconds (95%CI: 32.7-46.7) versus 14.4 seconds (95%CI: 12.0-16.9) for the IGA, p tactical environment placement of the IGA for

  20. Tata Laksana Kasus Perempuan dengan Pure Gonadal Dysgenesis 46, XY (Sindrom Swyer

    Directory of Open Access Journals (Sweden)

    Kanadi Sumapradja

    2016-06-01

    Full Text Available Disorders of sex development (DSD adalah kelainan medis yang dikaitkan dengan ketidakcocokan antara kromosom, gonad dan fenotip. Sindrom Swyer adalah kelainan berupa disgenesis gonad 46,XY dan diikuti dengan tidak terjadinya pelepasan anti-mullerian hormone (AMH dari gonad yang mengalami disgenesis sehingga mengakibatkan berkembangnya duktus muller menjadi uterus. Keadaan tersebut mengakibatkan pasien mengeluh amenorea primer dan organ seks sekunder tidak berkembang. Orientasi gender umumnya adalah sebagai perempuan, karena kurangnya paparan hormon androgen terhadap otak. Tatalaksana kasus Disorders of sex development (DSD sangat mementingkan pasien (patient-centered sehingga tatalaksana medikamentosa maupun pembedahan harus berdasarkan pemahaman pasien terhadap orientasi gendernya. Pada makalah ini dilaporkan kasus sindrom swyer pada seorang perempuan usia 29 tahun dengan keluhan amenorea primer. Hasil analisis kromosom 46,XY (20 metafase dengan hormon follicle stimulating hormone (FSH 31.5miu/ml, luteinizing hormone (LH 10.8miu/ml, estradiol (E2 <5pg/ml, testosteron total (T <0.0025ng/ml. Tatalaksana medikamentosa adalah memberikan terapi hormon estrogen untuk membantu proses feminisasi. Karena risiko tinggi terjadinya tumor pada kasus disgenesis gonad intra-abdomen dengan kromosom Y, maka direncanakan tindakan gonadektomi. Kata kunci: Tatalaksana, Sindrom Swyer, pure gonadal dysgenesis Case Management of Pure Gonadal Dysgenesis 46, XY (Sindrom Swyer Abstract Disorders of sex development (DSD are medical conditions in which the development of chromosomal, gonadal or anatomic sex varies from normal and may be incongruent with each other. Swyer syndrome is a condition caused by pure gonadal dysgenesis 46,XY, which followed by inadequate anti-mullerian (AMH production results in maintenance and further development of mullerian duct into uterus. Therefore, many patients who suffer from this condition initially come with chief complaint of primary

  1. What is new in cryptorchidism and hypospadias--a critical review on the testicular dysgenesis hypothesis

    DEFF Research Database (Denmark)

    Thorup, Jørgen Mogens; McLachlan, Robert; Cortes, Dina

    2010-01-01

    the past 40 years, hypospadias rates have not changed with certainty over the same period. Also, recent studies demonstrate that sperm output may have declined in certain areas of Europe but is probably not declining across the globe as indicated by American studies. However, at the same time......It has been hypothesized that poor semen quality, testis cancer, undescended testis, and hypospadias are symptoms of one underlying entity--the testicular dysgenesis syndrome--leading to increasing male fertility impairment. Though testicular cancer has increased in many Western countries during......, there is increasing recognition of male infertility related to obesity and smoking. There is no certain evidence that the rates of undescended testes have been increasing with time during the last 50 years. In more than 95% of the cases, hypospadias is not associated with cryptorchidism, suggesting major differences...

  2. Testicular dysgenesis syndrome comprises some but not all cases of hypospadias and impaired spermatogenesis

    DEFF Research Database (Denmark)

    Jørgensen, N; Rajpert-De Meyts, Ewa; Main, K M

    2010-01-01

    In 2001, when the testicular dysgenesis syndrome (TDS) concept was proposed, it suggested that impaired development of foetal testes could lead to increased risks of cryptorchidism, hypospadias, decreased spermatogenesis or testis cancer. The TDS concept links the pathogenesis of the four disorders...... with cryptorchidism or TGCC. By contrast, recent studies demonstrated that among men with isolated hypospadias, only a fraction of cases are linked to TDS. There is no doubt that TDS contributes to impaired semen quality. This is most obvious for cases with visible dysgenetic features in testis histology......, but in the majority of men with impaired semen quality as the only symptom, an association with TDS is less clear. Such cases have a very heterogeneous aetiology and may be caused by a host of other - often post-natal-factors. In conclusion, the TDS as a holistic concept has inspired new research activities and led...

  3. A comparison between the v-gel supraglottic airway device and the cuffed endotracheal tube for airway management in spontaneously breathing cats during isoflurane anaesthesia

    NARCIS (Netherlands)

    van Oostrom, H.; Krauss, M.W.; Sap, R.

    2013-01-01

    Abstract OBJECTIVE: To compare airway management using the v-gel supraglottic airway device (v-gel SGAD) to that using an endotracheal tube (ETT), with respect to practicability, leakage of volatile anaesthetics and upper airway discomfort in cats. STUDY DESIGN: Prospective, randomized clinical

  4. Haploinsufficient Bmp4 ocular phenotypes include anterior segment dysgenesis with elevated intraocular pressure

    Directory of Open Access Journals (Sweden)

    Nusinowitz Steven

    2001-11-01

    Full Text Available Abstract Background Glaucoma is a blinding disease usually associated with high intraocular pressure (IOP. In some families, abnormal anterior segment development contributes to glaucoma. The genes causing anterior segment dysgenesis and glaucoma in most of these families are not identified and the affected developmental processes are poorly understood. Bone morphogenetic proteins (BMPs participate in various developmental processes. We tested the importance of Bmp4 gene dosage for ocular development and developmental glaucoma. Results Bmp4+/- mice have anterior segment abnormalities including malformed, absent or blocked trabecular meshwork and Schlemm's canal drainage structures. Mice with severe drainage structure abnormalities, over 80% or more of their angle's extent, have elevated IOP. The penetrance and severity of abnormalities is strongly influenced by genetic background, being most severe on the C57BL/6J background and absent on some other backgrounds. On the C57BL/6J background there is also persistence of the hyaloid vasculature, diminished numbers of inner retinal cells, and absence of the optic nerve. Conclusions We demonstrate that heterozygous deficiency of BMP4 results in anterior segment dysgenesis and elevated IOP. The abnormalities are similar to those in human patients with developmental glaucoma. Thus, BMP4 is a strong candidate to contribute to Axenfeld-Rieger anomaly and other developmental conditions associated with human glaucoma. BMP4 also participates in posterior segment development and wild-type levels are usually critical for optic nerve development on the C57BL/6J background. Bmp4+/- mice are useful for studying various components of ocular development, and may allow identification of strain specific modifiers affecting a variety of ocular phenotypes.

  5. Stereological estimation of nuclear volume and other quantitative histopathological parameters in the prognostic evaluation of supraglottic laryngeal squamous cell carcinoma

    DEFF Research Database (Denmark)

    Sørensen, Flemming Brandt; Bennedbaek, O; Pilgaard, J

    1989-01-01

    The aim of this study was to investigate various approaches to the grading of malignancy in pre-treatment biopsies from patients with supraglottic laryngeal squamous cell carcinoma. The prospects of objective malignancy grading based on stereological estimation of the volume-weighted mean nuclear...... observers of the latter was poor in the material which consisted of 35 biopsy specimens. Unbiased estimates of nuclear Vv were on the average 385 microns3 (CV = 0.44), with more than 90% of the associated variance attributable to differences in nuclear Vv among individual lesions. Nuclear Vv was positively....... None of the investigated categorical and quantitative parameters (cutoff points = means) reached the level of significance with respect to prognostic value. However, nuclear Vv showed the best information concerning survival (2p = 0.08), and this estimator offers optimal features for objective...

  6. Stereological estimation of nuclear volume and other quantitative histopathological parameters in the prognostic evaluation of supraglottic laryngeal squamous cell carcinoma

    DEFF Research Database (Denmark)

    Sørensen, Flemming Brandt; Bennedbaek, O; Pilgaard, J

    1989-01-01

    The aim of this study was to investigate various approaches to the grading of malignancy in pre-treatment biopsies from patients with supraglottic laryngeal squamous cell carcinoma. The prospects of objective malignancy grading based on stereological estimation of the volume-weighted mean nuclear...... volume, nuclear Vv, and nuclear volume fraction, Vv(nuc/tis), along with morphometrical 2-dimensional estimation of nuclear density index, NI, and mitotic activity index, MI, were investigated and compared with the current morphological, multifactorial grading system. The reproducibility among two...... observers of the latter was poor in the material which consisted of 35 biopsy specimens. Unbiased estimates of nuclear Vv were on the average 385 microns3 (CV = 0.44), with more than 90% of the associated variance attributable to differences in nuclear Vv among individual lesions. Nuclear Vv was positively...

  7. A randomized double-blind phase III study of nimorazole as a hypoxic radiosensitizer of primary radiotherapy in supraglottic larynx and pharynx carcinoma. Results of the Danish Head and Neck Cancer Study (DAHANCA) Protocol 5-85

    DEFF Research Database (Denmark)

    Overgaard, J; Hansen, Hanne Sand; Overgaard, Marie

    1998-01-01

    A multicenter randomized and balanced double-blind trial with the objective of assessing the efficacy and tolerance of nimorazole given as a hypoxic radiosensitizer in conjunction with primary radiotherapy of invasive carcinoma of the supraglottic larynx and pharynx....

  8. Incidence, Prevalence, Diagnostic Delay, and Clinical Presentation of Female 46,XY Disorders of Sex Development.

    Science.gov (United States)

    Berglund, Agnethe; Johannsen, Trine H; Stochholm, Kirstine; Viuff, Mette H; Fedder, Jens; Main, Katharina M; Gravholt, Claus H

    2016-12-01

    The prevalence of phenotypic females with a 46,XY karyotype is low, thus current knowledge about age and clinical presentation at diagnosis is sparse even for the most frequent conditions, androgen insensitivity syndrome (AIS), and gonadal dysgenesis. To estimate incidence, prevalence, age at diagnosis, and clinical presentation at diagnosis in 46,XY females. A nationwide study covering all known females with a 46,XY karyotype in Denmark since 1960. The diagnosis of 46,XY disorder of sex development (DSD) was determined by medical record evaluation, data from the Danish National Patient Registry, and genetic testing, if available. A total of 166 females registered as 46,XY females in the Danish Cytogenetic Central Registry were identified. A total of 124 females were classified as having 46,XY DSD, 78 with AIS and 25 with gonadal dysgenesis, whereas the remaining subjects had a variety of different diagnoses. The prevalence of 46,XY females was 6.4 per 100 000 live born females, and for AIS and gonadal dysgenesis, it was 4.1 and 1.5 per 100 000, respectively. Median age at diagnosis was 7.5 years (95% confidence interval, 4.0-13.5; range, 0-34 y) in AIS and 17.0 years (95% confidence interval, 15.5-19.0; range, 0-28 y) in gonadal dysgenesis (P = .001). Clinical presentation was dependent on cause of DSD. The first estimate on prevalence of 46,XY females is 6.4 per 100 000 live born females. The presentation of AIS and gonadal dysgenesis is distinctly different, with AIS being diagnosed during childhood and gonadal dysgenesis during pubertal years. The presenting phenotype is dependent on the cause of 46,XY DSD.

  9. Caudal dysgenesis and sirenomelia-single centre experience suggests common pathogenic basis.

    Science.gov (United States)

    Thottungal, Anjana D; Charles, Adrian K; Dickinson, Jan E; Bower, Carol

    2010-10-01

    Abnormally formed lower limbs with varying degrees of fusion are the major feature of sirenomelia whereas maldeveloped lower limbs without fusion are found in association with caudal dysgenesis (CD). The relationship between these two entities has been a topic of debate for many years. The presence of a single umbilical artery originating from the abdominal aorta was considered a major feature distinguishing sirenomelia from CD. Based on this finding, the vascular steal theory was put forward as the causative mechanism of sirenomelia. CD and sirenomelia were considered to be two entirely different entities with distinct pathogenic mechanisms. However, it is now clear that a single umbilical artery can be found in some patients of CD and normal umbilical arteries in some patients of sirenomelia. The hypothesis of primary deficiency of caudal mesoderm caused by early developmental disruption suggests that sirenomelia and CD are two ends of a spectrum of maldevelopment of caudal mesoderm. In this paper we report on the clinical and pathological features of 16 patients of CD and 9 patients of sirenomelia from our institution and review the literature. This series of cases is notable for the significant association with neural tube defects, refining the renal and urogenital pathology associated with these conditions, and supporting the concept of a continuum of the disease spectrum. Copyright © 2010 Wiley-Liss, Inc.

  10. Superselective intra-arterial chemoradiotherapy for laryngeal cancer. Is it reasonable to treat glottic cancer in a similar way to supraglottic cancer?

    International Nuclear Information System (INIS)

    Yoshizaki, Tomokazu; Murono, Shigeyuki; Wakisaka, Naohiro; Kondo, Satoru; Furukawa, Mitsuru

    2006-01-01

    The standard treatment for advanced laryngeal cancer has been shifting from total laryngectomy to various organ preservation therapies such as subtotal laryngectomy and chemoradiotherapy. Robbins showed remarkable results with RADPLAT, the superselective intra-arterial infusion of supradose cisplatin (150 mg/m 2 ), against advanced head and neck cancer. However, the volume of laryngeal cancer is smaller than those of the other sites of head and neck cancers, and so a swaller less dose of cisplatin could save advanced laryngeal cancer patients. It may be reasonable to treat these subtypes of laryngeal cancer with a different modality. Thirty-five patients with laryngeal cancer were treated with tri-weekly intra-arterial infusion of cisplatin (100 mg/body). A 200 times molar excessive amount of sodium thiosulfate was intravenously infused to reduce the toxicity of cisplatin. Ten of 16 patients with glottic cancer and 10 of 19 patients with supraglottic cancer were followed for more than 2 years. Larynx preservation rate of glottic and supraglottic cancer was 80% and 70%, and progression-free survival rate was 80% and 50%, respectively. Grade III and IV toxic events were less frequent than with RADPLAT or systemic administration of a similar dose of cisplatin. Glottic and supraglottic cancers show different clinical behaviors. Our protocol with less cisplatin than RADPLAT is especially effective for glottic cancer. (author)

  11. Testicular dysgenesis syndrome and the estrogen hypothesis: a quantitative meta-analysis.

    Science.gov (United States)

    Martin, Olwenn V; Shialis, Tassos; Lester, John N; Scrimshaw, Mark D; Boobis, Alan R; Voulvoulis, Nikolaos

    2008-02-01

    Male reproductive tract abnormalities such as hypospadias and cryptorchidism, and testicular cancer have been proposed to comprise a common syndrome together with impaired spermatogenesis with a common etiology resulting from the disruption of gonadal development during fetal life, the testicular dysgenesis syndrome (TDS). The hypothesis that in utero exposure to estrogenic agents could induce these disorders was first proposed in 1993. The only quantitative summary estimate of the association between prenatal exposure to estrogenic agents and testicular cancer was published over 10 years ago, and other systematic reviews of the association between estrogenic compounds, other than the potent pharmaceutical estrogen diethylstilbestrol (DES), and TDS end points have remained inconclusive. We conducted a quantitative meta-analysis of the association between the end points related to TDS and prenatal exposure to estrogenic agents. Inclusion in this analysis was based on mechanistic criteria, and the plausibility of an estrogen receptor (ER)-alpha-mediated mode of action was specifically explored. We included in this meta-analysis eight studies investigating the etiology of hypospadias and/or cryptorchidism that had not been identified in previous systematic reviews. Four additional studies of pharmaceutical estrogens yielded a statistically significant updated summary estimate for testicular cancer. The doubling of the risk ratios for all three end points investigated after DES exposure is consistent with a shared etiology and the TDS hypothesis but does not constitute evidence of an estrogenic mode of action. Results of the subset analyses point to the existence of unidentified sources of heterogeneity between studies or within the study population.

  12. Role of brachytherapy in treatment of epidermoid carcinomas of the vallecula after conservative supraglottic laryngectomy followed by irradiation

    International Nuclear Information System (INIS)

    Clippe, Sebastien; Pommier, Pascal; Poupart, Marc; Ceruse, Philippe; Rosenbusch, Thomas; Ramade, Antoine; Montbarbon, Xavier; Gerard, Jean-Pierre; Carrie, Christian; Ardiet, Jean-Michel

    2002-01-01

    Purpose:To evaluate survival and functional results of the treatment of carcinomas of the vallecula using surgery, irradiation, and interstitial brachytherapy. Methods and Materials: Between 1990 and 1998, 36 patients with squamous cell carcinoma of the vallecula were treated with horizontal supraglottic functional laryngectomy, external beam radiotherapy (median dose 54 Gy), and additional interstitial brachytherapy (median dose 16 Gy). Results were compared with a previous series of 22 patients treated without brachytherapy. Results: The median follow-up was 44 months. The 5-year actuarial overall survival rate was 61.3%. The 5-year specific survival rate was 86%, with 2 local failures (local control rate 94.4%) and 4 isolated distant metastases. Ten patients developed a second primary. The overall survival was 34% for 22 patients previously treated without brachytherapy. Severe toxicities occurred in 9 patients: death (related to larynx edema or inhalation, n=1), soft tissue necrosis (n=1), aspiration pneumonia (n=1), mandibular necrosis (n=2), pharyngocutaneous fistula (n=2), and laryngeal edema (n=2). All the patients fed orally with no definitive gastrostomy or tracheotomy. Conclusion: Additional brachytherapy for vallecula carcinoma seems to improve locoregional control and overall survival dramatically. Functional results were also excellent. To our knowledge, this original therapeutic schedule has never been previously described

  13. Adjuvant intravenous methotrexate or definitive radiotherapy alone for advanced squamous cancers of the oral cavity, oropharynx, supraglottic larynx or hypopharynx

    International Nuclear Information System (INIS)

    Fazekas, J.T.; Sommer, C.; Kramer, S.

    1980-01-01

    Three hundred twenty-six patients with advanced head and neck cancers were randomized to receive definitive radiotherapy alone while 312 similar patients first received intravenous Methotrexate. No significant bias was demonstrated between the two patient populations. The number of annual deaths among the two randomized categories was essentially equal during the first 5 years. Nearly one-half occurred in the first year (146 for radiation alone and 143 in the chemotherapy plus irradiation groups). Median metastasis-free survival was between 12 to 13 months in both categories. The unadjusted 5 year survivals were in the 11 to 22% range for oral cavity, oropharynx, and supraglottic larynx and 3 to 9% for hypopharynx primaries. Although several variables did exert an impact upon survival, primary (T) and lymph node (N) stage seem to be of paramount importance and Methotrexate of minor consideration. Median and 5-year survivals within the various anatomic regions were consistently better when Methotrexate was given. However, these improvements were minimal and depended upon whether comparisons were performed on adjusted or unadjusted survival figures. In view of the modest benefits attained by using this Methotrexate regimen the authors suggest that other adjuvant programs be investigated and that this schedule not be adopted for routine clinical usage

  14. 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

  15. 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.

  16. Mixed gonadal dysgenesis with Turner`s phenotype and mosaic karyotype

    Energy Technology Data Exchange (ETDEWEB)

    Tarim, O.; Lieber, E. [Maimonides Medical Center, Brooklyn, NY (United States)]|[Interfaith Medical Center, Brooklyn, NY (United States)

    1994-09-01

    A 14 8/12-year-old white female patient was evaluated for short stature and amenorrhea. The past and family history were unremarkable. The physical examination revealed a short girl (131.4 cm; height age: 9) with a weight of 39.5kg (weight age: 11-6/12). The blood pressure was in the normal range in all four extremities and the peripheral pulses were positive. She had stigmata of Turner`s syndrome including short neck and slight webbing, cubitus valgus, and shield chest. There was no heart murmur. The only pubertal sign was pubic hair of Tanner stage II. The chromosome study showed a mosaic pattern. A total of 67 cultured lymphocytes from peripheral blood were analyzed which revealed 13 cells with 45,XO; 14 with 46,XY,r(Y); 39 with 46,XY. The patient had a normal vagina and hypoplastic uterus by sonogram. The diagnosis of mixed gonadal dysgenesis was confirmed by exploratory laparotomy and bilateral gonadectomy. The histologic examination of the gonads showed a testicle on the left and a streak ovary on right. The karyotype of the testicular tissue revealed 45,XO in 32 out of 40 and 46,XY in the remaining 8 cells. Pre-operative hormonal evaluation showed elevated gonadotropin levels of FSH 73.5 and LH 12.5 mIU/ml, low estradiol level of 5 pg/ml, normal testosterone level of 18 and DHEA-S of 181 mcg/dl, and normal thyroid function test with T4 of 6 mcg/dl and TSH of 4.2 mIU/ml. Her bone age was 12 years. The patient was also found to have subnormal growth hormone (GH) secretion by overnight GH study (1.55 ng/ml), clonidine stimulation test (7.3ng/ml), and insulin stimulation test (9.2 ng/ml). She responded well to human synthetic GH treatment with a growth velocity of 11.5 cm in two years. Replacement of sex hormones will be initiated after the completion of growth.

  17. Multiple non-cell-autonomous defects underlie neocortical callosal dysgenesis in Nfib-deficient mice

    Directory of Open Access Journals (Sweden)

    Sunn Nana

    2009-12-01

    results indicate that in addition to regulating the development of midline glial populations, Nfib also regulates the expression of neuropilin 1 within the cingulate cortex. Collectively, these data indicate that defects in midline glia and cingulate cortex neurons are associated with the callosal dysgenesis seen in Nfib-deficient mice, and provide insight into how the development of these cellular populations is controlled at a molecular level.

  18. Supraglottic carcinoma: Impact of radiation therapy on outcome of patients with positive margins and extracapsular nodal disease

    International Nuclear Information System (INIS)

    Devineni, V.R.; Simpson, J.R.; Sessions, D.; Spector, J.G.; Hayden, R.; Fredrickson, J.; Fineberg, B.

    1991-01-01

    Seventy-nine patients with supraglottic carcinoma treated between 1966 and 1985 are reviewed. All patients were treated with surgery and postoperative radiation therapy. Thirty-five percent of the patients had positive margins at the site of resection of the primary tumor. Of the 25 patients who had positive nodal disease, 13 patients (52%) had either extracapsular extension or soft-tissue or adjacent organ invasion, referred to in composite as grave signs. The median follow-up of the patients was 4.9 years and all patients were followed for a minimum of 3 years. The disease-free survival for all patients was 76% at 2 years and 71% at 3 years. The locoregional control rate for all patients was 70%. This study demonstrates that there is no difference in local recurrence or disease-free survival, or time to recurrence relative to the status of the surgical margins, which may be a benefit of the postoperative radiation therapy. This study also demonstrates that there is an increase in the number of patients with grave signs with increasing nodal stage. The rate of neck recurrence in patients with grave signs was substantially higher (54%) than in patients without grave signs (8%), even though these patients also had positive lymph nodes. Interestingly, there was also a higher rate of local recurrence among patients who had grave signs. Patients receiving doses higher than 6000 cGy to the primary site had fewer local failures, although within each group of patients with positive or negative surgical margins the differences in survival were minimal

  19. Results of the radiotheraphy of the supraglottic larynx carcinomas observed at the Radiotherapy Department of the District Hospital of Varese (Italy)

    International Nuclear Information System (INIS)

    Cosentino, D.; Scandolaro, L.; Cazzaniga, F.

    1987-01-01

    The authors review the supraglottic laryngeal carcinomas examined in the Radiotherapy Department of the Ospedale Regionale of Varese, Italy, from 1979 to 1984. 255 patients have been monitored: 80 considered for radiotherapy alone and 77 for postoperatory radiotherapy treatment. The cumulative actuarial survival (pcs) after 60 months, for patients treated only with radiotherapy, is 0.66 +- e.s. 0.07; for patients treated with post-operative radiotherapy the cumulative actuarial survival (pcs) after 60 months is 0.49 +- e.s. 0.11. The irradiation techniques are described and the results achived fully discussed

  20. A genome-wide association study of men with symptoms of testicular dysgenesis syndrome and its network biology interpretation

    DEFF Research Database (Denmark)

    Dalgaard, Marlene D; Weinhold, Nils; Edsgard, Stefan Daniel

    2012-01-01

    controls with excellent reproductive health. Furthermore, they developed a novel integrative method that combines GWAS data with other TDS-relevant data types and identified additional TDS markers. The most significant findings were replicated in an independent cohort of 671 Nordic men. Results Markers......Background Testicular dysgenesis syndrome (TDS) is a common disease that links testicular germ cell cancer, cryptorchidism and some cases of hypospadias and male infertility with impaired development of the testis. The incidence of these disorders has increased over the last few decades...... located in the region of TGFBR3 and BMP7 showed association with all TDS phenotypes in both the discovery and replication cohorts. An immunohistochemistry investigation confirmed the presence of transforming growth factor β receptor type III (TGFBR3) in peritubular and Leydig cells, in both fetal...

  1. Bilateral congenital corneal keloids and anterior segment mesenchymal dysgenesis in a case of Rubinstein-Taybi syndrome.

    Science.gov (United States)

    Rao, Srinivas K; Fan, Dorothy S P; Pang, C P; Li, Winnie W Y; Ng, Joan S K; Good, William V; Lam, Dennis S C

    2002-01-01

    To report the unusual association of bilateral corneal keloids and anterior segment mesenchymal dysgenesis in a child with Rubinstein-Taybi syndrome. Case report of a 2-year-old boy. Excision of the epicorneal mass in the right eye was followed by recurrence of the lesion. Multiple penetrating keratoplasties were unsuccessful in reconstructing the anterior segment because of recurrent corneal epithelial breakdown, suggesting limbal stem cell insufficiency. Histopathology and electron microscopy of the excised mass lesion showed features typical of a corneal keloid: thickened keratinized epithelium, absent Bowman's layer, and fibrovascular hyperplasia, with haphazard orientation of the collagen lamellae. Ultrasound biomicroscopy and intraoperative findings suggested a diagnosis of Peter anomaly, but genetic analysis did not show a PAX6 mutation. The findings in our patient add to the spectrum of ocular changes described in Rubinstein-Taybi syndrome and confirm earlier reports of poor ocular prognosis in corneal keloids and Rubinstein-Taybi syndrome.

  2. Birth Defects Associated with Perturbations in Pre-implantation, Gastrulation & Axis Extension: from Conjoined Twinning to Caudal Dysgenesis

    Science.gov (United States)

    Ferrer-Vaquer, Anna; Hadjantonakis, Anna-Katerina

    2012-01-01

    Congenital malformations represent approximately 3 in 100 live births within the human population. Understanding their pathogenesis and ultimately formulating effective treatments is underpinned by knowledge of the events and factors that regulate normal embryonic development. Studies in model organisms, primarily in the mouse, the most prominent genetically tractable mammalian model, have equipped us with a rudimentary understanding of mammalian development from early lineage commitment to morphogenetic processes. In this way information provided by studies in the mouse can, in some cases, be used to draw parallels with other mammals, including human. Here we provide an overview of our current understanding of the general sequence of developmental events from early cell cleavages to gastrulation and axis extension occurring in human embryos. We will also review some of the rare birth defects occurring at these stages, in particular those resulting in conjoined twinning or caudal dysgenesis. PMID:24014416

  3. i-gel: a new supraglottic device for effective resuscitation of a very low birthweight infant with Cornelia de Lange syndrome

    Science.gov (United States)

    Galderisi, Alfonso; De Bernardo, Giuseppe; Lorenzon, Eleonora; Trevisanuto, Daniele

    2015-01-01

    Laryngeal Mask Airway (LMA) has been indicated as an effective device for airway management when face-mask ventilation and intubation have both failed in infants weighing >2000 g or delivered ≥34 weeks of gestation. All previous studies used a classic LMA. The current report describes the first case of a very low birthweight infant (1470 g, <3rd centile; 36+3gestational weeks) with micrognathia and palate cleft with Cornelia De Lange syndrome, resuscitated at birth with a new supraglottic airway device, i-gel size-1, positioned by a trainee paediatrician at first attempt. The procedure allowed reaching prompt effective ventilation and oxygenation of the patient, who was stabilised and intubated through i-gel. PMID:25809435

  4. High frequency jet ventilation through a supraglottic airway device: a case series of patients undergoing extra-corporeal shock wave lithotripsy.

    Science.gov (United States)

    Canty, D J; Dhara, S S

    2009-12-01

    High frequency jet ventilation has been shown to be beneficial during extra-corporeal shock wave lithotripsy as it reduces urinary calculus movement which increases lithotripsy efficiency with better utilisation of shockwave energy and less patient exposure to tissue trauma. In all reports, sub-glottic high frequency jet ventilation was delivered through a tracheal tube or a jet catheter requiring paralysis and direct laryngoscopy. In this study, a simple method using supraglottic jet ventilation through a laryngeal mask attached to a circle absorber anaesthetic breathing system is described. The technique avoids the need for dense neuromuscular blockade for laryngoscopy and the potential complications associated with sub-glottic instrumentation and sub-glottic jet ventilation. The technique was successfully employed in a series of patients undergoing lithotripsy under general anaesthesia as an outpatient procedure.

  5. 331 cases of clinically node-negative supraglottic carcinoma of the larynx: a study of a modest size fixed field radiotherapy approach

    International Nuclear Information System (INIS)

    Sykes, Andrew J.; Slevin, Nicholas J.; Gupta, Nirmal K.; Brewster, Allison E.

    2000-01-01

    Purpose: For node-negative supraglottic carcinoma of the larynx, radiotherapy with surgery in reserve commonly provides very good results in terms of both local control and survival, while preserving function. However uncertainty exists over the treatment of the node-negative neck. Elective whole neck radiotherapy, while effective, may be associated with significant morbidity. The purpose of this study was to examine our practice of treating a modest size, fixed field to a high biologically effective dose and compare it with the patterns of recurrence from other centers that use different dose/volume approaches. Methods and Materials: Over a 10-year period 331 patients with node-negative supraglottic carcinoma of the larynx were treated with radiotherapy at the Christie Hospital Manchester. Patients were treated with doses of 50-55 Gy in 16 fractions over 3 weeks. Data were collected retrospectively for local and regional control, survival, and morbidity. Results: Overall local control, after surgical salvage in 17 cases, was 79% (T1-92%, T2-81%, T3-67%, T4-73%). Overall regional lymph node control, after surgical salvage in 13 cases, was 84% (T1-91%, T2-88%, T3-81%, T4-72%). Five-year crude survival was 50%, but after correcting for intercurrent deaths was 70% (T1-83%, T2-78%, T3-53%, T4-61%). Serious morbidity requiring surgery was seen in 7 cases (2.1%) and was related to prescribed dose (50 Gy-0%, 52.5 Gy-1.3%, 55 Gy-3.4%). Discussion: Our results confirm that treating a modest size, fixed field to a high biologically effective dose is highly effective. It enables preservation of the larynx in most cases, with acceptable regional control and no loss of survival compared to whole neck radiotherapy regimes

  6. Incidence, prevalence, diagnostic delay, and clinical presentation of female 46,XY disorders of sex development

    DEFF Research Database (Denmark)

    Berglund, Agnethe; Johannsen, Trine H; Krag, Kirstine Stochholm

    2016-01-01

    CONTEXT: The prevalence of phenotypic females with a 46,XY karyotype is low, thus current knowledge about age and clinical presentation at diagnosis is sparse even for the most frequent conditions, androgen insensitivity syndrome (AIS), and gonadal dysgenesis. OBJECTIVE: To estimate incidence......, prevalence, age at diagnosis, and clinical presentation at diagnosis in 46,XY females. DESIGN AND SETTING: A nationwide study covering all known females with a 46,XY karyotype in Denmark since 1960. The diagnosis of 46,XY disorder of sex development (DSD) was determined by medical record evaluation, data.......0-13.5; range, 0-34 y) in AIS and 17.0 years (95% confidence interval, 15.5-19.0; range, 0-28 y) in gonadal dysgenesis (P = .001). Clinical presentation was dependent on cause of DSD. CONCLUSIONS: The first estimate on prevalence of 46,XY females is 6.4 per 100 000 live born females. The presentation of AIS...

  7. The Role of the Y-Chromosome in the Establishment of Murine Hybrid Dysgenesis and in the Analysis of the Nucleotide Sequence Organization, Genetic Transmission and Evolution of Repeated Sequences.

    Science.gov (United States)

    Nallaseth, Ferez Soli

    The Y-chromosome presents a unique cytogenetic framework for the evolution of nucleotide sequences. Alignment of nine Y-chromosomal fragments in their increasing Y-specific/non Y-specific (male/female) sequence divergence ratios was directly and inversely related to their interspersion on these two respective genomic fractions. Sequence analysis confirmed a direct relationship between divergence ratios and the Alu, LINE-1, Satellite and their derivative oligonucleotide contents. Thus their relocation on the Y-chromosome is followed by sequence divergence rather than the well documented concerted evolution of these non-coding progenitor repeated sequences. Five of the nine Y-chromosomal fragments are non-pseudoautosomal and transcribed into heterogeneous PolyA^+ RNA and thus can be retrotransposed. Evolutionary and computer analysis identified homologous oligonucleotide tracts in several human loci suggesting common and random mechanistic origins. Dysgenic genomes represent the accelerated evolution driving sequence divergence (McClintock, 1984). Sex reversal and sterility characterizing dysgenesis occurs in C57BL/6JY ^{rm Pos} but not in 129/SvY^{rm Pos} derivative strains. High frequency, random, multi-locus deletion products of the feral Y^{ rm Pos}-chromosome are generated in the germlines of F1(C57BL/6J X 129/SvY^{ rm Pos})(male) and C57BL/6JY ^{rm Pos}(male) but not in 129/SvY^{rm Pos}(male). Equal, 10^{-1}, 10^ {-2}, and 0 copies (relative to males) of Y^{rm Pos}-specific deletion products respectively characterize C57BL/6JY ^{rm Pos} (HC), (LC), (T) and (F) females. The testes determining loci of inactive Y^{rm Pos}-chromosomes in C57BL/6JY^{rm Pos} HC females are the preferentially deleted/rearranged Y ^{rm Pos}-sequences. Disruption of regulation of plasma testosterone and hepatic MUP-A mRNA levels, TRD of a 4.7 Kbp EcoR1 fragment suggest disruption of autosomal/X-chromosomal sequences. These data and the highly repeated progenitor (Alu, GATA, LINE-1

  8. A Recurrent De Novo PACS2 Heterozygous Missense Variant Causes Neonatal-Onset Developmental Epileptic Encephalopathy, Facial Dysmorphism, and Cerebellar Dysgenesis.

    Science.gov (United States)

    Olson, Heather E; Jean-Marçais, Nolwenn; Yang, Edward; Heron, Delphine; Tatton-Brown, Katrina; van der Zwaag, Paul A; Bijlsma, Emilia K; Krock, Bryan L; Backer, E; Kamsteeg, Erik-Jan; Sinnema, Margje; Reijnders, Margot R F; Bearden, David; Begtrup, Amber; Telegrafi, Aida; Lunsing, Roelineke J; Burglen, Lydie; Lesca, Gaetan; Cho, Megan T; Smith, Lacey A; Sheidley, Beth R; Moufawad El Achkar, Christelle; Pearl, Phillip L; Poduri, Annapurna; Skraban, Cara M; Tarpinian, Jennifer; Nesbitt, Addie I; Fransen van de Putte, Dietje E; Ruivenkamp, Claudia A L; Rump, Patrick; Chatron, Nicolas; Sabatier, Isabelle; De Bellescize, Julitta; Guibaud, Laurent; Sweetser, David A; Waxler, Jessica L; Wierenga, Klaas J; Donadieu, Jean; Narayanan, Vinodh; Ramsey, Keri M; Nava, Caroline; Rivière, Jean-Baptiste; Vitobello, Antonio; Tran Mau-Them, Frédéric; Philippe, Christophe; Bruel, Ange-Line; Duffourd, Yannis; Thomas, Laurel; Lelieveld, Stefan H; Schuurs-Hoeijmakers, Janneke; Brunner, Han G; Keren, Boris; Thevenon, Julien; Faivre, Laurence; Thomas, Gary; Thauvin-Robinet, Christel

    2018-05-03

    Developmental and epileptic encephalopathies (DEEs) represent a large clinical and genetic heterogeneous group of neurodevelopmental diseases. The identification of pathogenic genetic variants in DEEs remains crucial for deciphering this complex group and for accurately caring for affected individuals (clinical diagnosis, genetic counseling, impacting medical, precision therapy, clinical trials, etc.). Whole-exome sequencing and intensive data sharing identified a recurrent de novo PACS2 heterozygous missense variant in 14 unrelated individuals. Their phenotype was characterized by epilepsy, global developmental delay with or without autism, common cerebellar dysgenesis, and facial dysmorphism. Mixed focal and generalized epilepsy occurred in the neonatal period, controlled with difficulty in the first year, but many improved in early childhood. PACS2 is an important PACS1 paralog and encodes a multifunctional sorting protein involved in nuclear gene expression and pathway traffic regulation. Both proteins harbor cargo(furin)-binding regions (FBRs) that bind cargo proteins, sorting adaptors, and cellular kinase. Compared to the defined PACS1 recurrent variant series, individuals with PACS2 variant have more consistently neonatal/early-infantile-onset epilepsy that can be challenging to control. Cerebellar abnormalities may be similar but PACS2 individuals exhibit a pattern of clear dysgenesis ranging from mild to severe. Functional studies demonstrated that the PACS2 recurrent variant reduces the ability of the predicted autoregulatory domain to modulate the interaction between the PACS2 FBR and client proteins, which may disturb cellular function. These findings support the causality of this recurrent de novo PACS2 heterozygous missense in DEEs with facial dysmorphim and cerebellar dysgenesis. Copyright © 2018 American Society of Human Genetics. All rights reserved.

  9. Malformations of the midline commissures: MRI findings in different forms of callosal dysgenesis

    International Nuclear Information System (INIS)

    Kueker, W.; Mader, I.; Naegele, T.; Mayrhofer, H.; Kraegeloh-Mann, I.

    2003-01-01

    Malformations of the corpus callosum (CC) may occur in many different syndromes. Various forms have been observed. We report seven cases of malformation of the CC. Special attention is directed towards the development of the fornix and hippocampus as a hippocampal commissure is a prerequisite of normal hippocampal development. The clinical disability of the patients presented here differed significantly, which may in part be due to the different extent of this cerebral malformation. The relevance of the concomitant aplasia of the limbic system has not been addressed in detail previously in the literature. (orig.)

  10. Disgenesia do corpo caloso e más-formações associadas: achados de tomografia computadorizada e ressonância magnética Dysgenesis of the corpus callosum and associated malformations: computed tomography and magnetic resonance imaging findings

    Directory of Open Access Journals (Sweden)

    Cristiano Montandon

    2003-10-01

    Full Text Available O termo disgenesia do corpo caloso refere-se a uma má-formação deste com origem na embriogênese do telencéfalo. O relato analisa os achados de tomografia computadorizada e ressonância magnética em 11 pacientes com disgenesia calosa e em um caso de corpo caloso normal com lipoma associado. Esta pode ser distinguida em três grupos: agenesia total (três casos, agenesia parcial (seis casos e hipoplasia (dois casos. Anomalias associadas foram observadas em nove casos, incluindo má-formação de Chiari tipo II (um caso, esquizencefalia (um caso, cisto inter-hemisférico (dois casos, heterotopia nodular (um caso, cisto de Dandy-Walker (um caso e lipoma do corpo caloso (quatro casos. Este artigo demonstra um espectro destes distúrbios, auxiliando na sua interpretação diagnóstica.Callosal dysgenesis is a malformation of the corpus callosum with origins in the embryogenesis of the telencephalon. We reviewed the computed tomography and magnetic resonance imaging findings of 11 patients with callosal dysgenesis and one patient with normal corpus callosum associated with a lipoma. The patients were divided into three distinct groups: total agenesis (three patients, partial agenesis (six patients and hypoplasia (two patients. Associated abnormalities were observed in nine patients, including Chiari II malformation (one patient, schizencephaly (one patient, interhemispheric cyst (two patients, Dandy-Walker cyst (one patient, nodular heterotopy (one patient and lipoma of the corpus callosum (four patients. This paper presents a review that may contribute to the diagnosis of these disorders.

  11. Current UK practice of pediatric supraglottic airway devices - a survey of members of the Association of Paediatric Anaesthetists of Great Britain and Ireland.

    Science.gov (United States)

    Bradley, Anthony E D; White, Michelle C; Engelhardt, Thomas; Bayley, Guy; Beringer, Richard M

    2013-11-01

    Over half of general anesthetics in the UK involve supraglottic airway devices (SADs). The National Audit Project 4 undertaken by the Royal College of Anaesthetists demonstrated that aspiration was the most frequent complication relating to SAD use. SADs designed to reduce this risk (second-generation devices) are increasingly recommended in both adults and children. As well as routine use, SADs are recommended for use in cases of 'difficult airway'. This survey assessed current usage of SADs in routine practice and difficult airways. Sixteen questions, approved by the Association of Paediatric Anaesthetists of Great Britain and Ireland (APAGBI) survey committee, were distributed to all its members. Two hundred and forty-four members responded. Eighty-eight percent preferentially use first-generation rather than second-generation devices. The most important design feature was the availability of a complete range of sizes (84%). Seventy-seven percent felt that randomized controlled trials assessing SAD safety in children are needed. In cases of failed intubation, classically shaped SADs are preferred (79%). Three percent of responders intubate via an SAD routinely. Eighteen percent have employed this technique in an emergency. Thirty-six percent of responders have found an SAD to function poorly. Pediatric anesthesiologists appear slow to embrace second-generation SADs. The role of SADs in the management of difficult airways is widely accepted. Research currently has little influence over the choice of which SAD to use, which is more likely determined by personal choice and departmental preference. There is a risk that some SADs are unsafe. © 2013 John Wiley & Sons Ltd.

  12. Conditional deletion of AP-2β in mouse cranial neural crest results in anterior segment dysgenesis and early-onset glaucoma

    Directory of Open Access Journals (Sweden)

    Vanessa B. Martino

    2016-08-01

    Full Text Available Anterior segment dysgenesis (ASD encompasses a group of developmental disorders in which a closed angle phenotype in the anterior chamber of the eye can occur and 50% of patients develop glaucoma. Many ASDs are thought to involve an inappropriate patterning and migration of the periocular mesenchyme (POM, which is derived from cranial neural crest cells (NCCs and mesoderm. Although, the mechanism of this disruption is not well understood, a number of transcriptional regulatory molecules have previously been implicated in ASDs. Here, we investigate the function of the transcription factor AP-2β, encoded by Tfap2b, which is expressed in NCCs and their derivatives. Wnt1-Cre-mediated conditional deletion of Tfap2b in NCCs resulted in post-natal ocular defects typified by opacity. Histological data revealed that the conditional AP-2β NCC knockout (KO mutants exhibited dysgenesis of multiple structures in the anterior segment of the eye including defects in the corneal endothelium, corneal stroma, ciliary body and disruption in the iridocorneal angle with adherence of the iris to the cornea. We further show that this phenotype leads to a significant increase in intraocular pressure and a subsequent loss of retinal ganglion cells and optic nerve degeneration, features indicative of glaucoma. Overall, our findings demonstrate that AP-2β is required in the POM for normal development of the anterior segment of the eye and that the AP-2β NCC KO mice might serve as a new and exciting model of ASD and glaucoma that is fully penetrant and with early post-natal onset.

  13. Laryngeal sarcoidosis: presentation and management in the pediatric population.

    Science.gov (United States)

    Strychowsky, Julie E; Vargas, Sara O; Cohen, Ezra; Vielman, Rene; Son, Mary Beth; Rahbar, Reza

    2015-09-01

    Sarcoidosis is a disease characterized by systemic non-necrotizing granulomas of unknown etiology. Laryngeal sarcoidosis is extremely uncommon, especially among pediatric patients. The clinical presentation and management of this entity in the pediatric population are poorly understood. A comprehensive search in PubMed was conducted to identify all cases in the published literature. We also present a case of isolated pediatric laryngeal sarcoidosis and outline the multidisciplinary approach to evaluation and management. A previously healthy 13-year-old female presented with a five-month history of mild dysphonia, dyspnea on exertion, and diffuse supraglottic edema. Biopsy showed non-necrotizing granulomas. Treatment with methotrexate led to marked improvement. The literature search identified seven previously published cases of pediatric laryngeal sarcoidosis, four in which disease was isolated to the larynx. All patients presented with a symptomatic and diffusely edematous supraglottis. Diagnoses were based on supraglottic biopsies showing non-necrotizing granulomas; all other possible etiopathologies were excluded. Three patients responded to corticosteroid therapy alone, one patient to tumor necrosis factor (TNF) inhibitor and methotrexate, and the remainder to a combination of corticosteroid therapy and surgical debulking. Laryngeal sarcoidosis in the pediatric population is challenging to diagnose and manage. When epithelioid granulomas are encountered histologically, other causes of granulomatous inflammation must be ruled out before a diagnosis of sarcoidosis can be made. Corticosteroid therapy alone may be ineffective. Medical therapy with methotrexate alone or in combination with TNF inhibitors versus surgical debulking alone or as part of multimodality treatment should be considered. A multidisciplinary approach with involvement of an otolaryngologist, pathologist, and rheumatologist is suggested. Copyright © 2015 Elsevier Ireland Ltd. All rights

  14. A randomized double-blind phase III study of nimorazole as a hypoxic radiosensitizer of primary radiotherapy in supraglottic larynx and pharynx carcinoma. Results of the Danish Head and Neck Cancer Study (DAHANCA) Protocol 5-85

    DEFF Research Database (Denmark)

    Overgaard, J.; Hansen, H.S.; Overgaard, M.

    1998-01-01

    PURPOSE: A multicenter randomized and balanced double-blind trial with the objective of assessing the efficacy and tolerance of nimorazole given as a hypoxic radiosensitizer in conjunction with primary radiotherapy of invasive carcinoma of the supraglottic larynx and pharynx. PATIENTS AND TREATMENT...... for the end-points of final loco-regional control (including surgical salvage) and cancer-related deaths (52 versus 41%, P = 0.002). This trend was also found in the overall survival but to a lesser, non-significant extent (26 versus 16%, 10-year actuarial values, P = 0.32). Cox multivariate regression...... of dying from cancer. The compliance to radiotherapy was good and 98% of the patients received the planned dose. Late radiation-related morbidity was observed in 10% of the patients, irrespective of nimorazole treatment. Drug-related side-effects were minor and tolerable with transient nausea and vomiting...

  15. Novel Familial Variant of the Desert Hedgehog Gene: Clinical Findings in Two Sisters with 46,XY Gonadal Dysgenesis or 46,XX Karyotype and Literature Review.

    Science.gov (United States)

    Baldinotti, Fulvia; Cavallaro, Tiziana; Dati, Eleonora; Baroncelli, Giampiero I; Bertini, Veronica; Valetto, Angelo; Massart, Francesco; Fabrizi, Gian Maria; Zanette, Giampietro; Peroni, Diego; Bertelloni, Silvano

    2018-01-01

    In humans, Desert Hedgehog (DHH) gene mutations are a very rare cause of 46,XY gonadal dysgenesis (GD), eventually associated with peripheral neuropathy. Clinical records of 12 patients with 46,XY GD and unknown genetic background were reviewed and a 46,XY woman with peripheral neuropathy was individuated. Her 46,XX sister affected by similar neuropathy was also investigated. Genomic DNA was extracted and DHH exons sequenced and analyzed. A comparative genomic hybridization array was also performed. In both the 46,XY and 46,XX sisters, a homozygous c.554C>A mutation in exon 2 of the DHH gene was found, determining a premature termination codon (p.Ser 185*). Heterozygous consanguineous carrier parents showed neither reproductive problems nor peripheral neuropathy. In the proband and her sister, a 499-kb duplication in 9p22.1 was also found. A 46,XY European woman with 46,XY GD and a novel homozygous DHH pathogenic variant is reported, confirming that this gene plays a key role in male gonadal development. Her 46,XX sister, harboring the same mutation, showed normal internal and external female phenotype. Thus, DHH seems not to be involved in the ovarian development pathway or its postpubertal function. Homozygous DHH mutations cause a specific peripheral neuropathy in humans with both 46,XY and 46,XX karyotypes. © 2018 S. Karger AG, Basel.

  16. Trisomy 10p and translocation of 10q to 4p associated with selective dysgenesis of IgA-producing cells in lymphoid tissue.

    Science.gov (United States)

    Saiga, Tatsuyoshi; Hashimoto, Kazuhiro; Kimura, Nobusuke; Ono, Hisako; Hiai, Hiroshi

    2007-01-01

    A combined chromosomal abberation trisomy of the short arm of chromosome 10 associated with translocation of 10q to chromosome 4p was found in a 14-month-old boy, who died after repeated bouts of pneumonia. The translocation involved the target region 4p16.3 of Wolf-Hirschhorn syndrome and/or Pitt-Rogers-Danks syndrome. The karyotype was 46,XY,der(4)t(4;10)(p16;q11.2),i(10)(p10),ish der(4)t(4;10)(p16.3;q11.2) (D4S96+,D4Z1+),i(10) (pter ++). In addition to growth retardation and external as well as internal dysmorphism, the patient had abnormalities of the immune system, such as thymic involution, generalized lymph node enlargement, unusual distribution of T cells in lymphoid follicles, and selective IgA deficiency. The IgA-producing cells were rarely found in lymph nodes but normally in intestinal mucosa. In contrast, in the lymph nodes, the paracortical T-lymphocytes were hyperplastic, but they rarely entered the primary follicles. It is assumed that the chromosomal abnormality may lead to the dysfunction of T lymphocytes and, further, to the dysgenesis of IgA-producing cells in lymph nodes but not in intestinal mucosa. This suggests that the thymus may differentially control the subsets of IgA-producing cells in lymph nodes and intestinal mucosa.

  17. Disgenesia gonadal mixta con fórmula cromosómica 45,X/46,X, (mar. Presentación de una paciente Mixed gonodal dysgenesis with chromosomic formula 45,X/46,X (mar. A case report

    Directory of Open Access Journals (Sweden)

    Pedro González Fernández

    2002-12-01

    Full Text Available Se presentó una paciente con estigmas turnerianos y cariotipo 45,X/46,XX con diagnóstico inicial de síndrome de Turner a la que se le realizó clitoridectomía por hipertrofia del clítoris a los 8 meses de edad. Se reevaluó a los 6 años de edad y se le realizó cariotipo con técnicas de bandas G (GTG con fórmula cromosómica de 45,X/46,X, (cromosoma marcador -mar; dicho marcador dio la impresión de una deleción del cromosoma X desde Xq13 ®Xq ter y Xp22 ®Xp ter. Se completó dicho estudio con técnica molecular de reacción en cadena de la polimerasa (PCR y se identificó el gen SRY en el cromosoma marcador. Se realizó intervención quirúrgica por mínimo acceso y se comprobó ausencia de útero así como trompa en el lado derecho con ausencia de gónada y en el lado opuesto, testículo rudimentario; se planteó el diagnóstico de disgenesia gonadal mixta.A patient with Turner stigmas and karyotype 45,X/46,XX with initial diagnosis of Turner's syndrome is presented. Clitoridectomy was performed due to hypertrophy of the clitoris when she was 8. She was reevaluated at 6 and karyotype was made by using G bands techniques (GTG with chromosomic formula of 45,X/46,X, (marker chromosome -mar; such marker gave the impression of a deletion of chromosome X from Xq13 ®Xq ter and Xp22 ®Xp ter. This study was completed with the molecular technique of polymerase chain reaction (PCR and the SRY gene was identified in the marker chromosome. The patient underwent minimum access surgery and it was proved the absence of uterus and tube on the right side with no gonad and a rudimentary testis on the opposite side. Mixed gonodal dysgenesis was diagnosed.

  18. Characterization of an isodicentric Y-chromosome for the long arm in a newborn with mixed gonadal dysgenesis.

    Science.gov (United States)

    Kohn, B; Kleyman, S M; Conte, R A; Macera, M J; Glassberg, K; Verma, R S

    1997-01-01

    A newborn infant was referred for evaluation because of ambiguous genitalia. Examination of the genitalia revealed a hypospadiac phallus measuring 1.5 cm in length with chordee. Subtle phenotypic features consistent with Turner syndrome were present including hypertelorism, anti-mongoloid slant to the eyes, mild widening of the neck, but no definitive webbing, shield like chest and positive cubitus valgus. A pelvic and renal sonogram confirmed the presence of a uterus and normal-appearing kidneys. There was incomplete fusion of the scrotum. No gonads were palpable within the scrotal sac. The patient was assigned a female gender on the basis of the presence of a uterus, the phenotypic appearance of the genitalia and the malignant potential of the gonads. The cytogenetic findings with QFQ-banding revealed an abnormal karyotype, i.e., mos 46,X,idic(Y) (p11.2)[77]/45,X[29]/46,X,idic(Y) (p11?) [2]/ 47,XY,idic(Y)(p11.2)[2]/47,X,idic(Y)(p11.2), + idic(Y)(p11.2)[1]/46,XY[1]. The presence of an abnormal isodicentric Y-chromosome was evaluated by FISH-technique to ensure a finer characterization than routine methods. The genotype-phenotype correlation could not be established since mosaicisms of highly variable nature can exhibit an unpredictable outcome.

  19. Oral-facial-digital syndrome with mesoaxial polysyndactyly, common AV canal, hirschsprung disease and sacral dysgenesis: Probably a transitional type between II, VI, variant of type VI or a new type

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2014-07-01

    Full Text Available We report a 4 month old male infant, the first in order of birth of healthy first cousin consanguineous parents who has many typical features of oral-facial-digital syndrome type VI (OFDS VI including hypertelorism, bilateral convergent squint, depressed nasal bridge, and wide upturned nares, low set posteriorly rotated ears, long philtrum, gum hyperplasia with notches of the alveolar borders, high arched palate, and hyperplastic oral frenula. He has mesoaxial and postaxial, polysyndactyly which is the specific feature of OFDS VI, however the cerebellum is normal on MRI brain. He has also some rare congenital anomalies including common atrioventricular canal, hirschsprung disease, and sacral dysgenesis. This patient may have a transitional type between II and VI, a variant of type VI or a new type.

  20. 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 ...

  1. 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

  2. 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...

  3. 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

  4. 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

  5. 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 ...

  6. 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.

  7. 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.

  8. 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

  9. 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.)

  10. 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...

  11. 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

  12. Acromegaly presented as a cause of laryngeal dyspnea.

    Science.gov (United States)

    Saussez, S; Mahillon, V; Chantrain, G; Thill, M P; Lequeux, T

    2007-12-01

    Acromegalic patients can develop obstructive sleep apnea syndrome or upper airflow obstruction. The development of dyspnea is unusual and the fixation of both vocal cords is exceptional. We report the case of a patient with bilateral vocal cord paralysis. Fiberoptic laryngoscopy and computed tomography (CT) of the neck showed a supra-glottic stenosis due to a swelling of the soft tissue. A tracheostomy was first performed. Thereafter, micro-laryngoscopy using laser vaporisation of the supra-glottic soft tissue was attempted but failed to remove the tracheostomy canula. Finally, blood tests and cerebral MRI revealed an acromegaly. The patient underwent a trans-sphenoidal resection of the pituitary adenoma. Fifteen months later, fiberoptic laryngoscopy showed bilateral restoration of vocal cord mobility and the tracheostomy canula was successfully removed after 18 months. Vocal cord fixation is probably due to hypopharyngeal and laryngeal soft tissue swelling and can be reversible after successful treatment of the adenoma.

  13. Bilateral ocular abnormalities in a wild stranded harp seal (Phoca groenlandica) suggestive of anterior segment dysgenesis and persistent hyperplastic primary vitreous.

    Science.gov (United States)

    Erlacher-Reid, Claire; Colitz, Carmen M H; Abrams, Ken; Smith, Ainsley; Tuttle, Allison D

    2011-06-01

    A male yearling harp seal (Phoca groenlandica) stranded and was brought to Mystic Aquarium & Institute for Exploration's Seal Rescue and Rehabilitation Center. The seal presented with a bilateral pendular vertical nystagmus, negative menace response, and a positive palpebral response. Ophthalmological examination by slit lamp biomicroscopy revealed perilimbal corneal edema, excessive iridal surface structures, pupils that appeared to be shaped improperly (dyscoria), and suspected cataracts. Attempts to dilate the pupils with both dark-lighted conditions and repeated dosages of 10% phenylephrine and 1% atropine ophthalmic solution in each eye (OU) were unsuccessful. Ocular ultrasonography findings suggested bilateral cataracts with flattened anterior-posterior (A-P) diameter and possible persistent hyperplastic primary vitreous. It is possible that these structural congenital abnormalities could produce further ocular complications for this seal including uveitis, secondary glaucoma, retinal detachment, and/or vitreal hemorrhage in the future. This case demonstrates the importance of a thorough ophthalmological examination in stranded wild animals, especially if their symptoms appear neurological.

  14. Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

    International Nuclear Information System (INIS)

    Matushita Junior, Joao Paulo K.; Tiel, Chan; Py, Marco; Batista, Raquel Ribeiro; Gasparetto, Emerson L.

    2010-01-01

    Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SOD-plus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe. (author)

  15. Septo-optic dysplasia plus: clinical presentation and magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Matushita Junior, Joao Paulo K. [Instituto de Pos-Graduacao Medica Carlos Chagas (IPGMCC), Rio de Janeiro, RJ (Brazil); Tiel, Chan; Py, Marco [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Inst. de Neurologia Deolindo Couto; Batista, Raquel Ribeiro [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho; Gasparetto, Emerson L., E-mail: egasparetto@gmail.co [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Dept. de Radiologia

    2010-04-15

    Septo-optic dysplasia (SOD) is a heterogeneous developmental malformation characterized by optic nerve hypoplasia associated with dysgenesis of the septum pellucidum and other cerebral malformations. The clinical manifestations include psychomotor retardation, visual impairment, thermoregulatory disturbances, conjugated hyperbilirubinemia and seizures. In 2000, Miller et al. first named the association of SOD and cortical dysplasia as SOD-plus. In this report, all the cases had severe clinical impairment, presenting global developmental delay and spastic motor deficits. Subsequent reports of SOD-plus also stressed the psychomotor development delay, spastic motor deficits and seizures seen in these patients, emphasizing the severity of the brain involvement. Recently, Kwak et al. reported a case of SOD-plus presenting with cortical dysplasia involving the insular cortex bilaterally. However, differently from all the previous reports, the patient had no signs or symptoms of cortical dysfunction, except for one episode of seizure. We report an additional case of SOD-plus, which presented extensive cortical malformation, with no signs or symptoms of cortical dysfunction. We suggest that similar to the classical form of SOD, the clinical presentation in patients with SOD-plus can also range from mild to extremely severe. (author)

  16. Genetics Home Reference: renal tubular dysgenesis

    Science.gov (United States)

    ... next step, angiotensin-converting enzyme (produced from the ACE gene) converts angiotensin I to angiotensin II. Angiotensin II ... proximal tubules) and other tissues. Mutations in the ACE , AGT , AGTR1 , or REN gene impair the production or function of angiotensin II, ...

  17. Testicular dysgenesis syndrome and the estrogen hypothesis: a quantitative meta-analysis A síndrome da disgenesia testicular e a hipótese do estrogênio: uma meta-análise quantitativa

    Directory of Open Access Journals (Sweden)

    Olwenn Martin

    2008-10-01

    Full Text Available Male reproductive tract abnormalities such as hypospadias and cryptorchidism, and testicular cancer have been proposed to comprise a common syndrome together with impaired spermatogenesis with a common etiology resulting from the disruption of gonadal development during fetal life, the testicular dysgenesis syndrome (TDS. The only quantitative summary estimate of the link between prenatal exposure to estrogenic agents and testicular cancer was published over 10 years ago; other reviews of the link between estrogenic compounds, other than the potent pharmaceutical estrogen diethylstilbestrol (DES, and TDS end points have remained inconclusive. We conducted a quantitative meta-analysis of the association between the end points related to TDS and prenatal exposure to estrogenic agents. Inclusion in this analysis was based on mechanistic criteria, and the plausibility of an estrogen receptor (ER-α-mediated mode of action was specifically explored. Eight studies were included, investigating the etiology of hypospadias and/or cryptorchidism that had not been identified in previous systematic reviews. Four additional studies of pharmaceutical estrogens yielded a statistically significant updated summary estimate for testicular cancer. Results of the subset analyses point to the existence of unidentified sources of heterogeneity between studies or within the study population.Sugeriu-se que anomalias do trato reprodutivo masculino como hipospádia e criptorquidismo, assim como o câncer de testículo, componham uma síndrome comum com diminuição da espermatogênese, e de etiologia comum, a interrupção do desenvolvimento gonadal na fase fetal, a síndrome de disgenesia testicular (SDT. O único levantamento quantitativo da relação entre exposição pré-natal a agentes estrogênicos e câncer de testículo data de mais de dez anos; outras revisões da relação entre compostos estrogênicos diferentes do potente estrogênio sint

  18. 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

  19. 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.

  20. 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."

  1. Heredity of supraglottic exercise-induced laryngeal obstruction

    DEFF Research Database (Denmark)

    Walsted, Emil Schwarz; Hvedstrup, Jeppe; Eiberg, Hans

    2017-01-01

    Respiratory symptoms on exertion, such as shortness of breath and wheezing, are commonly associated with asthma, but might also arise from the larynx [1–3]. In recent years, the emergence of exercise laryngoscopy [4] has led to a better understanding of laryngeal movement during exercise, and ins...

  2. 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.

  3. 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.

  4. 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.

  5. 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…

  6. 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…

  7. 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

  8. 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.)

  9. 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.

  10. 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)

  11. 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.

  12. 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.

  13. 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

  14. 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.

  15. 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)

  16. 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.

  17. 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.

  18. 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.

  19. 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

  20. 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.)

  1. 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.

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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

  7. 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

  8. 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.)

  9. 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.

  10. 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...

  11. 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

  12. 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...

  13. 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...

  14. 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

  15. 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.

  16. 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

  17. 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...

  18. 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.)

  19. 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

  20. [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.

  1. 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.

  2. 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.

  3. 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 [...

  4. EPMA present and future

    International Nuclear Information System (INIS)

    Love, G.

    2002-01-01

    In electron probe microanalysis the objective has always been to gain the maximum information from a sample. To this end efforts have been directed at increasing detection efficiency, reducing the size of the excitation volume, analysing more difficult specimens and improving the accuracy of quantitative measurements whilst, at the same time, attempting to minimize the complexity of the analysis. The present paper comments on the progress that has been made in this areas, focuses on the latest hardware and software developments taking place and looks forward to how these may impact on the technique of EPMA. (author)

  5. 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.

  6. 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)

  7. 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

  8. Presentation to the symposium

    International Nuclear Information System (INIS)

    Busby, C.

    1997-01-01

    This paper proposes that there is a major error in the present perception of cancer risk from exposure to low level radiation from internal man-made fission products like strontium-90. The author's Second Event theory is discussed which advances the basis that fission products are different in radiation risk terms. Since the scientific community has been so ready to dismiss this idea out of hand, the author also explores scientific culture and scientific revolutions. The evidence that the idea is broadly right is surveyed and finally some studies that could be undertaken to prove or disprove the author's contention are outlined. (UK)

  9. 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)

  10. 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.'

  11. 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.

  12. 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...

  13. Film Presentation: Die Urknallmaschine

    CERN Multimedia

    Carolyn Lee

    2010-01-01

    Die Urknallmaschine, an Austrian film by Gerd Baldauf, narrated by Norbert Frischauf (Alpha Österreich - ORF, 2009).  In CERN’s gigantic complex particles are accelerated to almost the speed of light, brought to collision and made to divide into even smaller particles. Public opinion of CERN’s research is also divided. Sceptics fear that black holes may be created. Might the goal to study the origin of the world lead to its destruction? The Austrian researcher Norbert Frischauf worked at CERN for many years. With his guidance it is possible to explore the world’s largest research centre, get a glimpse of the fascinating work the scientists do there and take a crash course in particle physics. Die Urknallmaschine will be presented on Friday, 25 June from 13:00 to 14:00 in the Main Auditorium. Language: German

  14. Abdominal pregnancy - Case presentation.

    Science.gov (United States)

    Bohiltea, R; Radoi, V; Tufan, C; Horhoianu, I A; Bohiltea, C

    2015-01-01

    Abdominal pregnancy, a rare diagnosis, belongs to the ectopic pregnancy group, the leading cause of pregnancy related exitus. The positive diagnosis is very difficult to establish most often in an acute setting, leading to a staggering percent of feto-maternal morbidity and mortality. We present the case of 26-weeks-old abdominal pregnancy with partial feto-placental detachment in a patient, after hysteroscopy and in vitro fertilization, which until the acute symptoms that led to emergency laparotomy went unrecognized. The patient recovered completely and satisfactorily after surgery and, due to the high risk of uterine rupture with regard to a second pregnancy, opted for a surrogate mother. Abdominal pregnancy can be regarded as a difficult to establish diagnosis, with a greater chance in case of increased awareness. It is compulsory to be well informed in order not to be surprised by the diagnosis and to apply the correct treatment immediately as the morbidity and mortality rate is elevated.

  15. 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.

  16. Imagined futures, present lives

    DEFF Research Database (Denmark)

    Dalsgaard, Anne Line; Wildermuth, Norbert

    2006-01-01

    The article is focused thematically on the uses and meanings of media for (some) young people in Recife, a million-inhabitant city in the northeast of Brazil. The article brings together the perspective of Anne Line Dalsgaard, a long-term anthropological field researcher who is familiar with the ......The article is focused thematically on the uses and meanings of media for (some) young people in Recife, a million-inhabitant city in the northeast of Brazil. The article brings together the perspective of Anne Line Dalsgaard, a long-term anthropological field researcher who is familiar...... 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...

  17. Presenting practice financial information.

    Science.gov (United States)

    Webster, Lee Ann H

    2007-01-01

    Medical practice leadership teams, often consisting primarily of physicians with limited financial backgrounds, must make important business decisions and continuously monitor practice operations. In order to competently perform this duty, they need financial reports that are relevant and easy to understand. This article explores financial reporting and decision-making in a physician practice. It discusses reports and tools, such as ratios, graphs, and comparisons, that practices typically include in their reports. Because profitability and cash flow are often the most important financial considerations for physician practices, reports should generally focus on the impact of various activities and potential decisions upon these concerns. This article also provides communication tips for both those presenting practice financial information and those making the decisions. By communicating effectively, these leaders can best use financial information to improve decision-making and maximize financial performance.

  18. Presentation of Canada

    International Nuclear Information System (INIS)

    Hedley, Dianne E.

    1997-01-01

    In contingency of a nuclear emergency event, requiring application of intervention measures on a federal scale, Canada has of a plan ensuring the compatibility of the plans of different provinces and serving as interface between federal and provincial authorities. Exclusive of a nuclear attack against North America, by nuclear emergency it is understood an accident resulting in radionuclide release. This is called the Plan of federal intervention in case of nuclear emergency. 'Sante Canada' is the federal authority responsible for intervention in case of nuclear emergency and it has the task of preparing and coordinating the actions on a federal scale.Should the plan be set in action and if the emergency has repercussions upon the agricultural sector, the sustaining organism will be 'Agriculture and agroalimentaire Canada' which in case of emergency acts through the channels of the National System of intervention in the agro-alimentary sector (SNIUA). The paper presents the objectives, the principles of organization and operation, the responsibilities and the plans which SNIUA has in its charge to implement in case of emergency

  19. STS-69 postflight presentation

    Science.gov (United States)

    1995-10-01

    A postflight conference of the STS-69 mission is presented. The flightcrew ('The Dog Team') consisted of Cmdr. David Walker, Pilot Kenneth Cockrell, Payload Cmdr. James Voss, and Mission Specialists James Newman and Michael Gernhardt. The mission's primary objective was the deployment and retrieval of the SPARTAN-201 satellite, which investigated the interaction between the Sun and it's solar wind. Other secondary experiments and shuttle payloads included the Wake Shield Facility (WSF), which grew several layers of semiconductor films, the International Extreme Ultraviolet Hitchhiker (IEH-1), the Capillary Pumped Loop-2/Gas Bridge Assembly (CAPL-2/GBA), several Get Away Specials (GAS) experiments, the Electrolysis Performance Improvement Concept Study (EPICS), the Thermal Energy Storage (TES-2) experiment, the Commercial Generic Bioprocessing Apparatus-7 (CGBA-7), the National Institutes of Health-Cells 4 (NIH-C4) experiment, and the Biological Research in Canister-6 (BRIC-6) experiment. Earth views consisted of Saudi Arabia water wells, uncommon vortices over Oman, the Amazon River, the Bahamas, Somalia, a sunset over the Earth's horizon, and two hurricanes, Luis and Marilyn.

  20. Present energy status

    Energy Technology Data Exchange (ETDEWEB)

    Desprairies, P

    1988-04-01

    This paper reviews the past and present situation of the World Oil Market and assumes future trends and possible future evolution. Low oil prices between 1950 and 1973 have contributed to an exceptional growth of oil demand during more than 20 years which was more and more supplied by OPEC. The oil shocks of 1973 and 1979 have dramatically changed this situation. Some of the most important changes have been the emergence of non-OPEC new oil producers, the growth of substitute energies, especially natural gas and nuclear power, on the supply side; and substantial decrease of energy consumption in many industrialized countries which has greatly reduced oil demand to OPEC on the demand side. Today this situation seems somewhat stabilized (after the reversal of world oil prices in 1986) and OPEC is producing around 18 million b/day as against a producing capacity of about 30 million b/day. It is generally considered that in coming years oil prices will remain relatively low (or occasionally even become lower than today) due to excess oil production capacity and relative abundance of other types of energy. However, many experts think that these trends can be reversed when oil supply will more and more concentrate again in the Middle East; but it is hard to say if this will begin in 1995, 2000, 2010.

  1. The laryngocele; case presentation

    Directory of Open Access Journals (Sweden)

    Gabriel V. Berteșteanu

    2016-05-01

    Full Text Available Laryngocele is a rare pathology, but because of their clinical evolution and the symptoms they generate, they should always be considered as a differential diagnosis when investigating neck masses. A laryngocele is basically a herniation of the mucosa of the laryngeal ventricle (Morgagni's ventricle arising usually from the saccular region. This herniation may remain confined to the larynx - in which case the laryngocele is internal- or expand through the thyro-hyoid membrane into the structures of the neck - thus being called an external laryngocele. Usually the laryngocele has both an internal and external component thus being a mixed laryngocele. Diagnosis of laryngoceles still relies heavily on clinical signs such as tympanism, easily depressible neck mass, indirect laryngoscopy, but is now simplified by imagistic investigations (ultrasound, CT and MRI. However, the treatment of this condition is exclusively surgical and consists of total excision of the laryngocele, as well as proper identification of the point of origin from the saccule and also the final suture of the breach in order to prevent recurrence. Investigation of possible causes of obstruction of the laryngeal ventricle should always be performed (because of the possibility of an underlying malignancy as well as a follow-up protocol of the patient, given the risk of relapse. We present a recently diagnosed case of a 32 year old man with mixed laryngocele, which we have operated in our clinic.

  2. [Antibiotics: present and future].

    Science.gov (United States)

    Bérdy, János

    2013-04-14

    The author discuss the up to date interpretation of the concept of antibiotics and antibiotic research, as well as the present role of various natural, semisynthetic and synthetic antibiotic compounds in various areas of the human therapy. The origin and the total number of all antibiotics and applied antibiotics in the practice, as well as the bioactive microbial metabolites (antibiotics) in other therapeutical, non-antibiotic fields (including agriculture) are also reviewed. The author discusses main problems, such as increasing (poly)resistance, virulence of pathogens and the non-scientific factors (such as a decline of research efforts and their sociological, economic, financial and regulatory reasons). A short summary of the history of Hungarian antibiotic research is also provided. The author briefly discusses the prospects in the future and the general advantages of the natural products over synthetic compounds. It is concluded that new approaches for the investigation of the unlimited possibilities of the living world are necessary. The discovery of new types or simply neglected (micro)organisms and their biosynthetic capabilities, the introduction of new biotechnological and genetic methods (genomics, metagenom, genome mining) are absolutely required in the future.

  3. IKONOS: future and present

    Science.gov (United States)

    Schaap, Niek

    2003-04-01

    The IKONOS satellite has been operational since January 2000 and was the first commercial satellite collecting imagery with 1 meter resolution. The current life expectancy of the satellite is 10 years. Since the launch, Space Imaging Inc. (the owner of the satellite) supplied IKONOS imagery to users in many vertical markets, such as: agriculture, defense, oil & gas and telecommunications. This oral presentation will give comprehensive information about IKONOS and the future: * Block II, the successor of IKONOS. Space Imaging expects to launch in 2004 a new high-resolution satellite, ensuring both continuity and (for some years) a tandem operation with IKONOS, greatly improving the availability of imagery. * Space Imaging affiliates. IKONOS imagery collected, processed and sold by regional affiliates. These regional affiliates are strategically located around the world, like Japan Space Imaging (Tokyo), Space Imaging Middle East (Dubai) and Space Imaging Eurasia (Ankara, Turkey). * Technical briefing IKONOS. IKONOS (compared to other commercial high-resolution satellites) has superior collection capabilities. Due to, the higher orbit altitude, local reception of the imagery, bi-directional scanning and the high agility of the satellite, is the IKONOS satellite capable to collect the imagery relative quickly.

  4. 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.

  5. THE UPSIDE OF PRESENTISM

    Directory of Open Access Journals (Sweden)

    Lynn Fendler

    2009-01-01

    Full Text Available El presentismo es visto generalmente como un mal necesario en historiografía. Este artículo explora lo positivo de esta inevitabilidad. Aplicando un enfoque filosófico al análisis discursivo en la tradición de la nueva historia cultural, se distingue -de una parte-¿ entre un uso estratégico del presentismo, y -de otra- un enfoque racionalista en historia. El artículo concluye considerando algunas implicaciones políticas en historiografía de las explicaciones racionalistas y de las explicaciones estratégicamente presentistas. Los modos de comprensión racionalistas inscriben las expectativas del pasado en las visiones del futuro; asignando al historiador el papel de profeta; y perpetuando nociones de agentes ahistóricos. En contraste, las historias estratégicamente presentistas incorporan una orientación que deliberadamente emplea las lentes y perspectivas del presente en orden a tener en cuenta supuestos y perspectivas actuales. Cuando las asunciones son examinadas en relación a las perspectivas presentistas, dichos presupuestos sueltan su rienda en el pensamiento. Dado que el presentismo es inevitable, no debe ser desestimado por completo, sino que debe ser una cuestión sondeada y examinada críticamente. Con dicho foco, el presentismo estratégico en la historiografía debe posibilitar una reflexión sobre los límites de lo que es posible pensar.

  6. Texts of presentation

    Energy Technology Data Exchange (ETDEWEB)

    Magnin, G.; Vidolov, K.; Dufour-Fallot, B.; Dewarrat, Th.; Rose, T.; Favatier, A.; Gazeley, D.; Pujol, T.; Worner, D.; Van de Wel, E.; Revaz, J.M.; Clerfayt, G.; Creedy, A.; Moisan, F.; Geissler, M.; Isbell, P.; Macaluso, M.; Litzka, V.; Gillis, W.; Jarvis, I.; Gorg, M.; Bebie, B.

    2004-07-01

    Implementing a sustainable local energy policy involves a long term reflection on the general interest, energy efficiency, distributed generation and environmental protection. Providing services on a market involves looking for activities that are profitable, if possible in the 'short-term'. The aim of this conference is to analyse the possibility of reconciling these apparently contradictory requirements and how this can be achieved. This conference brings together the best specialists from European municipalities as well as important partners for local authorities (energy agencies, service companies, institutions, etc.) in order to discuss the public-private partnerships concerning the various functions that municipalities may perform in the energy field as consumers and customers, planners and organizers of urban space and rousers as regards inhabitants and economic players of their areas. This document contains the summaries of the following presentations: 1 - Performance contracting: Bulgarian municipalities use private capital for energy efficiency improvement (K. VIDOLOV, Varna (BG)), Contracting experiences in Swiss municipalities: consistent energy policy thanks to the Energy-city label (B. DUFOUR-FALLOT and T. DEWARRAT (CH)), Experience of contracting in the domestic sector (T. ROSE (GB)); 2 - Public procurement: Multicolor electricity (A. FAVATIER (CH)), Tendering for new green electricity capacity (D. GAZELEY (GB)), The Barcelona solar thermal ordinance (T. PUJOL (ES)); 3 - Urban planning and schemes: Influencing energy issues through urban planning (D. WOERNER (DE)), Tendering for the supply of energy infrastructure (E. VAN DE WEL (NL)), Concessions and public utility warranty (J.M. REVAZ (CH)); 4 - Certificate schemes: the market of green certificates in Wallonia region in a liberalized power market (G. CLERFAYT (BE)), The Carbon Neutral{sup R} project: a voluntary certification scheme with opportunity for implementation in other European

  7. Crystallography: past and present

    Science.gov (United States)

    Hodeau, J.-L.; Guinebretiere, R.

    2007-12-01

    structure (chemical order, anisotropy, charge transfer, magnetic order) versus an external parameter like temperature, pressure, magnetic or electric field. Modern crystallography is also extended to the study of very small crystals, powders, ill-ordered or non-crystallized materials. Thus presently, crystallography is concerned with any solid that “scatters” an incident beam. Nevertheless, as quoted by A. Guinier, “the problems facing crystallographers have only changed, ... new ones have appeared which require reflection and imagination, ... and which in turn may still bring much joy to all those who like crystallography” [4]. Such developments open up crystallography to modern materials like artificial ones and nanostructures with low- and/or multi-scaled-periodicities and/or extremely small “crystal size” and to materials of the “real world”, with mixtures of phases and/or amorphous contribution and/or defects, a common characteristic of ancient materials analysed in patrimonial research. In our contribution we will show by selected examples that these improvements were allowed (i) by the use of powerful sources, apparatus and detectors which allow micro-diffraction, in-situ diffraction, spectroscopy, resonant scattering, inelastic scattering, coherent scattering, (ii) by the development of methods like diffraction anomalous fine structure (DAFS), pair distribution function (PDF), simulated annealing, single object reconstruction, (iii) by combination of scattering and spectroscopy and by combination of scattering and microscopy. Such combination of different approaches is very efficient and, as said by H. Curien at the IUCr Bordeaux Congress in 1990, “in crystallography, there is a constant alternation between the crystal space and its associated reciprocal space, ... the alternation between experiment and model building is another feature of crystallography activity ..., the crystallographer relies both on his computer and on his diffractometer

  8. present

    African Journals Online (AJOL)

    as medium of instruction enhances concept formation, especially in science subjects as ... The study, on which this paper is based, was conducted to evaluate Malawi's ... corrected in the wake of information flowing from the evaluation phase (Kaplan .... concerning the background information and suggested activities (Thodi,.

  9. PRESENTATION

    OpenAIRE

    Editors, The

    2017-01-01

    To plan and to implement academic projects remain a systematic challenge for the team and the collaborators of the Electronic Journal. Each study published is an opportunity to strengthen the proposed goals and to add more value to the community involved and interested.

  10. Presentations

    International Nuclear Information System (INIS)

    2007-01-01

    The aim of the Exploratory Workshop is to encourage informal discussion and the exchange of expertise between scientists using random matrix theory in various areas of research ranging from fundamental physics: string theory, gravity, quantum chaos, information theory, complexity theory, combinatorics to applied research: biophysics, econophysics, quantitative finance and telecommunication. Such a discussion would be a first step towards creating a common European environment for researchers using this powerful theory

  11. Flowers for Algernon: steroid dysgenesis, epigenetics and brain disorders.

    Science.gov (United States)

    Sanders, Bryan K

    2012-01-01

    While a recent study has reported that early citalopram exposure alters cortical network function and produces autistic-like behaviors in male rats, when evaluating antidepressant animal models of autism spectrum disorder (ASD) it is important to note that some selective serotonin (5-HT) reuptake inhibitors alter 3α-hydroxysteroid dehydrogenase activity, and thus steroidogenesis. At least one study has examined the effect of repeated citalopram administration on the serum and brain concentration of testosterone (T) and its metabolites and shown that citalopram increases serum T. Several in vitro studies also suggest that sex steroid can alter 5-HT homeostasis. While research efforts have demonstrated that transgenic mice expressing the most common of multiple gain-of-function 5-HT reuptake transporter (SERT) coding variants, SERT Ala56, previously identified in children with ASD, exhibit autistic-like behaviors, elevated p38 MAPK-dependent transporter phosphorylation, enhanced 5-HT clearance rates and hyperserotonemia, a few studies provide some evidence that 5-HT may alter gonadal steroidogenesis. T, 17β-estradiol and synthetic estrogens are known inhibitors of AKR1C21 (BRENDA, E.C. 1.1.1.209), the epitestosterone (epiT) producing enzyme in rodents. EpiT is a naturally occurring steroid in mammals, including man. An analysis of the literature suggests that epiT may be the central mediator in the epigenetic regulation of gene expression. Over thirty years ago, it was shown that rat brain epiT production is higher in females than in males. A similar finding in humans could explain the sex differences in the incidence of autism and other brain disorders. Despite this, the role of epiT in brain development remains a long neglected area of research.

  12. 28 gonadal dysgenesis, turner's syndrome and phenotype in the ...

    African Journals Online (AJOL)

    1971-01-09

    Jan 9, 1971 ... Heart sounds were normal and the chest was clear. When aged 10 months the ... was no breast tissue; the nipples were small, hypoplastic and widely ...... for his help and advice in preparing the manuscript. I should also like ...

  13. Ascus dysgenesis in hybrid crosses of Neurospora and Sordaria ...

    Indian Academy of Sciences (India)

    DURGADAS P. Kasbekar

    2017-07-04

    Jul 4, 2017 ... Another was the production of heterokaryotic ascospores in eight-spored asci. Lewis (1969) ... 0.75 in each cross with strain C4T4 a, and by a factor of 0.5 in .... 25288 T(VR>VII) EB4 a; FGSC 25289 T(VIL>IR) IBj5 a; FGSC ...

  14. 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…

  15. 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.

  16. 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…

  17. 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

  18. 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.

  19. 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

  20. 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.…

  1. 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.…

  2. 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.

  3. 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

  4. 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

  5. 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 ...

  6. 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)

  7. 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.

  8. 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)

  9. 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.

  10. 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

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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

  16. 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.

  17. 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.

  18. 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

  19. 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.

  20. 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.

  1. 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.

  2. 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...

  3. 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.)

  4. 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.

  5. 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

  6. 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

  7. 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

  8. 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.

  9. 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

  10. 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.

  11. 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.

  12. 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.)

  13. 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, ...

  14. 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.

  15. Press book presentation progress report

    International Nuclear Information System (INIS)

    2001-07-01

    This document presents four french sites of the ANDRA concerning the radioactive waste disposal. The concerned site are the underground research laboratory of Meuse/Haute-Marne, the Aube Center, the TFA Center and the Manche Center. For each sites, data and information on the geophysics, the capacity, the management or the research programs are analysed. (A.L.B.)

  16. 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

  17. Understanding Algorithms in Different Presentations

    Science.gov (United States)

    Csernoch, Mária; Biró, Piroska; Abari, Kálmán; Máth, János

    2015-01-01

    Within the framework of the Testing Algorithmic and Application Skills project we tested first year students of Informatics at the beginning of their tertiary education. We were focusing on the students' level of understanding in different programming environments. In the present paper we provide the results from the University of Debrecen, the…

  18. 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

  19. 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)

  20. 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

  1. Unusual Presentation of Maydl's Hernia

    African Journals Online (AJOL)

    Nikhil NBA, Natarajan K, Mohanty A, et al. An. Unusual Case of Maydl's Hernia. Int J Cur Res Rev. 2013;5(6):22-5. 11. Ganesaratnam M. Maydl's hernia: Report of a Series of Seven Cases and Review of Literature. Brit J Surg. 1985;72:737-8. 12. Weledji EP, Mokake M, Ngowe MN. A Rare. Presentation of Maydl's Hernia.

  2. Secondary syphilis presenting as vertigo

    International Nuclear Information System (INIS)

    Bari, A.U.; Mehmood, T.; Khan, B.; Malik, N.; Malik, K.Z.; Sukhera, A.M.

    2006-01-01

    Syphilis is a chronic, systemic and sexually transmitted infectious disease affecting most of the organs in the body. A young African man presented with vertigo, unsteadiness of gait and a skin rash suggestive of secondary syphilis. Diagnosis was confirmed on serology and was treated with two shots of long-acting penicillin, following which his symptoms settled. (author)

  3. Energy consumption: Past, present, future

    Science.gov (United States)

    1973-01-01

    The energy consumption history of the United States and the changes which could occur in consumption characteristics in the next 50 years are presented. The various sources of energy are analyzed to show the limitations involved in development and utilization as a function of time available. Several scenarios were prepared to show the consumption and supply of energy under varying conditions.

  4. Learning through Dramatic Story Presentation

    Science.gov (United States)

    Tindall, Evie

    2012-01-01

    The use of story with dramatic presentation approaches produces an engaging and powerful instructional choice for today's adult ESL educators. Two engaging and timed-tested approaches are Reader's Theater and Tableau Vivant. Both provide English language learners with content tailored to their abilities in addition to numerable opportunities to…

  5. 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

  6. Viewfoils for environmental capabilities presentation

    International Nuclear Information System (INIS)

    1992-01-01

    This document contains information about the environmental applications of aerial surveys. It discusses the accuracy, dependability, history, advantages, and sensitivity of aerial surveys. Also included, are a brief overview of in-situ gamma spectroscopy and samples of aerial surveys. This document contains entirely of an outline and viewfoils for the presentation

  7. Present Day Philosophies of Education

    Science.gov (United States)

    Ediger, Marlow

    2006-01-01

    Presently, there are competing philosophies of education which need comparison. Two philosophies will be compared which are at opposite ends of the continuum. They are distinctly different. And yet, both schools of thought have their disciples. Each of the two will be discussed in terms of its essential features and then there will be selected…

  8. MAMMARY TUBERCULOSIS PRESENTING AS CARCINOMA

    African Journals Online (AJOL)

    She was otherwise well wizh no symptoms referable to other systems. On examination .... cardiac failure. The other ... but chronic oedema of the right arm of moderate degree was present. .... a tuberculous ulcer on the dorsum of the hand, followed ... venous system may also lead to haematogenous dissemination, producing ...

  9. 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)

  10. 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...

  11. 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)

  12. Slide Composition for Electronic Presentations

    Science.gov (United States)

    Larson, Ronald B.

    2004-01-01

    Instructors who use computer-generated graphics in their lectures have many options to consider when developing their presentations. Experts give different advice on which typefaces, background and letter colors, and background imagery improve communications. This study attempted to resolve these controversies by examining how short-term recall of…

  13. 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.

  14. 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.

  15. Neutrino astronomy: Present and future

    Indian Academy of Sciences (India)

    Abstract. I briefly review the present and future status of the burgeoning field of neu- ... near future build upon previous results. ... novel radio and acoustic techniques utilized by, for example, experiments such as. GLUE ... Left: Schematic of νµ-induced muon track in detector. Right: .... an eye toward the galactic center).

  16. 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...

  17. 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

  18. 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

  19. 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.

  20. 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.

  1. 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

  2. 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.

  3. 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

  4. 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)

  5. Amebiasis Presenting as Pleuropulmonary Disease

    OpenAIRE

    Kubitschek, Kenneth R.; Peters, Jay; Nickeson, David; Musher, Daniel M.

    1985-01-01

    Seven patients with amebic liver abscess presenting as pleuropulmonary disease were admitted to hospital initially because of pulmonary symptoms and were found to have amebic liver disease. Three categories of pleuropulmonary involvement included reactive inflammation of the pleura or lung, rupture of a hepatic abscess into the pleural space and rupture of a hepatic abscess into the bronchial airways. The preferred medical treatment is with metronidazole, but rupture of hepatic amebic abscess...

  6. Computer training, present and future.

    Science.gov (United States)

    Smith, G. A.

    1972-01-01

    The products of educational firms today lead toward a multimedia approach to the education and training of commercial programmers and systems analysts. Computer-assisted instruction or CAI is a relatively new medium to augment the other media. The government use of computers is discussed together with the importance of computer pretests. Pretests can aid in determining a person's ability to absorb a particular instructional level. The material presented in a number of computer courses is listed.

  7. Sarcoidosis presenting as penile mass

    Directory of Open Access Journals (Sweden)

    Hüseyin Semiz

    2017-01-01

    Full Text Available Sarcoidosis is an inflammatory disease with unknown cause characterized by noncaseating granuloma formations. It may present with bilateral hilar lymphadenopathy, skin lesions, eye, and musculoskeletal system involvement. Rare involvement of the genital organs (prostate, testis, epididymis has also been reported. However, penile involvement is observed quite rare. In this paper, we report a patient with penile mass who was diagnosed with sarcoidosis on the basis of the laboratory, radiological, and pathological investigations.

  8. Carcinoma caecum - a rare presentation

    International Nuclear Information System (INIS)

    Ali, T.; Mudasser, S.; Mudsser, S.; Sarwar, M.

    2010-01-01

    Caecal and right sided colonic cancers mostly present with fatigue, weakness and iron deficiency anemia. Such tumours rarely perforate posteriorly and involve the retroperitoneum. We report a case of an old Omani lady who came with insidious sign and symptoms of perforated caecum leading to retroperitoneal collection and necrotizing fasciitis of abdominal wall due to carcinoma of caecum. She underwent surgery but despite active intervention, she died because of septicemic shock. (author)

  9. Water plants past and present

    DEFF Research Database (Denmark)

    Riis, Tenna

    2006-01-01

    How has the condition of the environment developed during the last 100 years? This is an important question because the conditions of the past infl uence those of the present, and knowledge about this development makes it possible to evaluate the causes of changes in species richness, species com...... composition and environmental quality. Here we compare old and contemporary studies of the Danish stream fl ora....

  10. Burning mouth syndrome: Present perspective

    OpenAIRE

    Ramesh Parajuli

    2015-01-01

    Introduction: Burning mouth syndrome is characterized by chronic oral pain or burning sensation affecting the oral mucosa in the absence of obvious visible mucosal lesions. Patient presenting with the burning mouth sensation or pain is frequently encountered in clinical practice which poses a challenge to the treating clinician. Its exact etiology remains unknown which probably has multifactorial origin. It often affects middle or old age women and it may be accompanied by xerostomia and alte...

  11. Present status of CP violation

    International Nuclear Information System (INIS)

    Ng, J.N.

    1989-06-01

    A review of the status of CP violation in kaons is given. Status of our knowledge of quark mixing angles in the standard six quark model is presented. The role Β d o - Βd o transition plays in this study is examined. A comparison of the estimates of CP violation effects from models beyond the standard one is given. Other experiments that have the capability of testing different CP violation models are also discussed. (Author) 35 refs., 6 figs., tab

  12. 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.

  13. 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

  14. 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.

  15. 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.

  16. Diabetic Myonecrosis: An Atypical Presentation

    Directory of Open Access Journals (Sweden)

    José Hernán Martínez

    2013-01-01

    Full Text Available Diabetic myonecrosis is a frequently unrecognized complication of longstanding and poorly controlled diabetes mellitus. The clinical presentation is swelling, pain, and tenderness of the involved muscle, most commonly the thigh muscles. Management consists of conservative measures including analgesia and rest. Short-term prognosis is good, but long-term prognosis is poor with most patients dying within 5 years. Failure to properly identify this condition will expose the patient to aggressive measures that could result in increased morbidity. To our knowledge this is the first case reported in which there was involvement of multiple muscle groups including upper and lower limbs.

  17. Variable retinal presentations in nanophthalmos

    International Nuclear Information System (INIS)

    Khan, A.; Zafar, S.N.

    2009-01-01

    Nanophthalmos is an uncommon developmental ocular disorder characterized by a small eye with short axial length, high hyperopia and high lens/eye volume ratio due to arrested development of the globe in all directions. Different types of fundus changes can rarely occur with nanophthalmos. We describe five cases of nanophthalmos, each of them presenting with a different fundus appearance. Our case series highlights variability of pigmentary changes from retinal flecks to bone spicules and bull's eye maculopathy, which are rare in the combinations described here. (author)

  18. Present art of reactivity determination

    International Nuclear Information System (INIS)

    Kaneko, Yoshihiko; Nakano, Masafumi; Matsuura, Shojiro

    1977-01-01

    Experimental techniques for reactivity determination of a reactor have been one of the long standing subjects in reactor physics. Recently, such a requirement was proposed by the reactor designers and operators that the values of reactivity should be measured more accurately. This is because importance is emphasized for the role of reactivity to the performance of reactor safety, economics and operability. Motivated by the requirement, some remarkable progresses are being made for the improvement of the experimental techniques. Then, the present review summarizes the research activities on this subject, identifies several reactor physics problems to be overcome, and makes mention of the future targets. (auth.)

  19. Addison's disease presenting with perimyocarditis.

    Science.gov (United States)

    Baranski Lamback, Elisa; Morandi, Grazia; Rapti, Eleni; Christov, Georgi; Brogan, Paul A; Hindmarsh, Peter

    2018-01-26

    Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association. A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA. The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process. Autoimmune adrenal insufficiency should be considered in all cases of pericarditis and perimyocarditis, especially when the severity of clinical manifestations exceeds the expected for the severity of the cardiac findings, as timely identification and prompt treatment may be life-saving.

  20. Metastatic liposarcoma present in mediastinum

    International Nuclear Information System (INIS)

    Vazquez Gonzalez, Jose Manuel; Danta Fundora, Debora; Collado Otero, Juan Carlos; Paredes Lopez, Dagmar I

    2009-01-01

    Authors present the case of a woman aged 59 operated on in three occasions due to the presence of neck nodules. Biopsy from two of them informs that be about a pleomorphous liposarcoma. After 6 years she came to consultation by a picture of high retrosternal pain and slight dyspnea. Thorax tomography shows a solid mass in anterior mediastinum. We made exeresis of a 10 cm diameter soft solid mass. Pathological Anatomy Service reported on relapse of a pleomorphous liposarcoma. In general, the postoperative course was satisfactory. Literature reviewed shows that the surgical treatment is the more used procedure in these cases. (author)

  1. Presentation at Innoventure Annual Competition

    Energy Technology Data Exchange (ETDEWEB)

    None

    2010-03-31

    This report documents the components of the workshop presented at the recent annual competition for the Innoventure program. The goal of the workshop was to focus on the delivery of science, technology, engineering, and math (STEM) concepts in a hands-on experiential learning format to increase interest in national security careers at NNSA, most of which are in the STEM fields. This work is a part of the National Security Preparedness Project (NSPP), being performed under a Department of Energy (DOE)/National Nuclear Security Administration (NNSA) grant.

  2. Vitality of optical vortices (Presentation)

    CSIR Research Space (South Africa)

    Roux, FS

    2014-02-01

    Full Text Available stream_source_info Roux3_2014.pdf.txt stream_content_type text/plain stream_size 3018 Content-Encoding UTF-8 stream_name Roux3_2014.pdf.txt Content-Type text/plain; charset=UTF-8 Title Vitality of optical vortices F Stef... Roux Presented at Complex Light and Optical Force VIII SPIE Photonics West 2014 Moscone Center, San Francisco, California USA 5 February 2014 CSIR National Laser Centre, Pretoria, South Africa – p. 1/11 Speckle Amplitude Phase – p. 2/11 Vortex...

  3. Cartography past, present and future

    CERN Document Server

    Rhind, DW

    2013-01-01

    Making maps dates back at least four thousand years and it is widely recognised that many maps are of great historical value and present a skilled method of summarising the real world on a sheet of paper. Less well known is the judgement involved in the selection and simplification of features, the complex transformation of space and the exacting standards which are needed in cartography. This book is primarily a tribute to Professor F.J. Ormeling, former President and Secretary/Treasurer of the ICA and gives a wide ranging review of the current status of cartography, how this status was atta

  4. STS-95 Post Flight Presentation

    Science.gov (United States)

    1998-01-01

    The STS-95 flight crew, Cmdr. Curtis L. Brown, Pilot Steven W. Lindsey, Mission Specialists Scott E. Parazynski, Stephen K. Robinson, and Pedro Duque, and Payload Specialists Chiaki Mukai and John H. Glenn present a video mission over-view of their space flight. Images include prelaunch activities such as eating the traditional breakfast, crew suit-up, and the ride out to the launch pad. Also, included are various panoramic views of the shuttle on the pad. The crew can be seen being readied in the "whiteroom" for their mission. After the closing of the hatch and arm retraction, launch activities are shown including countdown, engine ignition, launch, and the separation of the Solid Rocket Boosters. The primary objectives, which include the conducting of a variety of science experiments in the pressurized SPACEHAB module, the deployment and retrieval of the Spartan free-flyer payload, and operations with the HST Orbiting Systems Test (HOST) and the International Extreme Ultraviolet Hitchhiker (IEH) payloads are discussed in both the video and still photo presentation.

  5. OROPHARYNGEAL TUBERCULOSIS: AN UNUSUAL PRESENTATION

    Directory of Open Access Journals (Sweden)

    M H Dadgarnia

    2008-12-01

    Full Text Available "nTuberculosis (TB still represents a major public health problem worldwide. The primary form of disease is most often localized to the lung. In a minority of patients, progressive pulmonary disease spreads to other organ systems through self inoculation via infected sputum, blood and lymphatic system, establishing the secondary form of tuberculosis. We present a patient that was referred to us with complaint of ulcerative mouth lesions from 3 months ago. In physical examination multiple erythematous and irregularly ulcerative lesions affecting soft palate area, uvula and anterior tonsillar pillar was noted bilaterally. Punch biopsy was done from several points that revealed chronic granulomatous inflammation. Ziehl-Nielsen staining of cultured specimen demonstrated acid-fast bacilli. Chest X-ray showed apical pulmonary involvement, suggesting tuberculosis infection. Patient was treated with anti-tuberculosis 4 drugs regimen. In the one year follow-up period after complete treatment; patient didn't have any evidence of disease. Oral and oropharyngeal TB lesions are uncommon, it is estimated that only 0.05-5% of total TB cases may present with oral manifestations, but should be an important consideration in the differential diagnosis of lesions that appear in the oral cavity and oropharynx. The secondary form is more frequent and involves mainly the tongue but involvement of pharynx is quite rare condition. Although tuberculosis of oropharynx is relatively rare, with the increasing incidence of tuberculosis, it must be considered in the differential diagnosis of atypical ulcerative lesions of the mouth and oropharynx.

  6. Early presentation of primary glioblastoma.

    Science.gov (United States)

    Faguer, R; Tanguy, J-Y; Rousseau, A; Clavreul, A; Menei, P

    2014-08-01

    Clinical and neuroimaging findings of glioblastomas (GBM) at an early stage have rarely been described and those tumors are most probably under-diagnosed. Furthermore, their genetic alterations, to our knowledge, have never been previously reported. We report the clinical as well as neuroimaging findings of four early cases of patients with GBM. In our series, early stage GBM occurred at a mean age of 57 years. All patients had seizures as their first symptom. In all early stages, MRI showed a hyperintense signal on T2-weighted sequences and an enhancement on GdE-T1WI sequences. A hyperintense signal on diffusion sequences with a low ADC value was also found. These early observed occurrences of GBM developed rapidly and presented the MRI characteristics of classic GBM within a few weeks. The GBM size was multiplied by 32 in one month. Immunohistochemical analysis indicated the de novo nature of these tumors, i.e. absence of mutant IDH1 R132H protein expression, which is a diagnostic marker of low-grade diffuse glioma and secondary GBM. A better knowledge of early GBM presentation would allow a more suitable management of the patients and may improve their prognosis. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  7. Teratology - past, present and future.

    Science.gov (United States)

    Ujházy, Eduard; Mach, Mojmír; Navarová, Jana; Brucknerová, Ingrid; Dubovický, Michal

    2012-12-01

    Teratology is the science that studies the causes, mechanisms, and patterns of abnormal development. The authors present an updated overview of the most important milestones and stages of the development of modern teratology. Development of knowledge and society led to the recognition that causes of congenital developmental disorders (CDDs) might be caused by various mechanical effects, foetal diseases, and retarded or arrested development of the embryo and foetus. Based on the analysis of the historical development of hypotheses and theories representing a decisive contribution to this field, we present a survey of the six Wilson's fundamental principles of teratology. The aim of observing these principles is to get insight into developmental relations and to understand mechanisms of action on the level of cell populations (elementary morphogenetic processes), tissues and organs. It is important to realise that any negative intervention into the normal course of these processes, either on genetic or non-genetic basis, inevitably leads to a sequence of subsequent changes resulting in CDDs. Moreover, the classical toxicologic monotonic dose-response paradigm recently has been challenged by the so-called "low dose-hypothesis", particularly in the case of endocrine active substances. These include some pesticides, dioxins, polychlorobiphenyls (PCBs), and bisphenol A. Despite modern approaches of molecular biology and genetics, along with top diagnostic techniques, we are still not able to identify the actual cause in more than 65 to 70% of all congenital defects classified as having an unknown etiology. Today CDDs include any birth defect, either morphological, biochemical, or behavioural.

  8. Cutaneous sporotrichosis: Unusual clinical presentations

    Directory of Open Access Journals (Sweden)

    Mahajan Vikram

    2010-01-01

    Full Text Available Three unusual clinical forms of sporotrichosis described in this paper will be a primer for the clinicians for an early diagnosis and treatment, especially in its unusual presentations. Case 1, a 52-year-old man, developed sporotrichosis over pre-existing facial nodulo-ulcerative basal cell carcinoma of seven-year duration, due to its contamination perhaps from topical herbal pastes and lymphocutaneous sporotrichosis over right hand/forearm from facial lesion/herbal paste. Case 2, a 25-year-old woman, presented with disseminated systemic-cutaneous, osteoarticular and possibly pleural (effusion sporotrichosis. There was no laboratory evidence of tuberculosis and treatment with anti-tuberculosis drugs (ATT did not benefit. Both these cases were diagnosed by histopathology/culture of S. schenckii from tissue specimens. Case 3, a 20-year-old girl, had multiple intensely pruritic, nodular lesions over/around left knee of two-year duration. She was diagnosed clinically as a case of prurigo nodularis and histologically as cutaneous tuberculosis, albeit, other laboratory investigations and treatment with ATT did not support the diagnosis. All the three patients responded well to saturated solution of potassium iodide (SSKI therapy. A high clinical suspicion is important in early diagnosis and treatment to prevent chronicity and morbidity in these patients. SSKI is fairly safe and effective when itraconazole is not affordable/ available.

  9. Presentation Extensions of the SOAP

    Science.gov (United States)

    Carnright, Robert; Stodden, David; Coggi, John

    2009-01-01

    A set of extensions of the Satellite Orbit Analysis Program (SOAP) enables simultaneous and/or sequential presentation of information from multiple sources. SOAP is used in the aerospace community as a means of collaborative visualization and analysis of data on planned spacecraft missions. The following definitions of terms also describe the display modalities of SOAP as now extended: In SOAP terminology, View signifies an animated three-dimensional (3D) scene, two-dimensional still image, plot of numerical data, or any other visible display derived from a computational simulation or other data source; a) "Viewport" signifies a rectangular portion of a computer-display window containing a view; b) "Palette" signifies a collection of one or more viewports configured for simultaneous (split-screen) display in the same window; c) "Slide" signifies a palette with a beginning and ending time and an animation time step; and d) "Presentation" signifies a prescribed sequence of slides. For example, multiple 3D views from different locations can be crafted for simultaneous display and combined with numerical plots and other representations of data for both qualitative and quantitative analysis. The resulting sets of views can be temporally sequenced to convey visual impressions of a sequence of events for a planned mission.

  10. Bilirubin present in diverse angiosperms.

    Science.gov (United States)

    Pirone, Cary; Johnson, Jodie V; Quirke, J Martin E; Priestap, Horacio A; Lee, David

    2010-01-01

    Bilirubin is an orange-yellow tetrapyrrole produced from the breakdown of heme by mammals and some other vertebrates. Plants, algae and cyanobacteria synthesize molecules similar to bilirubin, including the protein-bound bilins and phytochromobilin which harvest or sense light. Recently, we discovered bilirubin in the arils of Strelitzia nicolai, the White Bird of Paradise Tree, which was the first example of this molecule in a higher plant. Subsequently, we identified bilirubin in both the arils and the flowers of Strelitzia reginae, the Bird of Paradise Flower. In the arils of both species, bilirubin is present as the primary pigment, and thus functions to produce colour. Previously, no tetrapyrroles were known to generate display colour in plants. We were therefore interested in determining whether bilirubin is broadly distributed in the plant kingdom and whether it contributes to colour in other species. In this paper, we use HPLC/UV and HPLC/UV/electrospray ionization-tandem mass spectrometry (HPLC/UV/ESI-MS/MS) to search for bilirubin in 10 species across diverse angiosperm lineages. Bilirubin was present in eight species from the orders Zingiberales, Arecales and Myrtales, but only contributed to colour in species within the Strelitziaceae. The wide distribution of bilirubin in angiosperms indicates the need to re-assess some metabolic details of an important and universal biosynthetic pathway in plants, and further explore its evolutionary history and function. Although colour production was limited to the Strelitziaceae in this study, further sampling may indicate otherwise.

  11. Anaphylactic reactions presenting with hypertension.

    Science.gov (United States)

    Solmazgul, Emrullah; Kutlu, Ali; Dogru, Salim; Ozalper, Veysel; Cetindagli, Ibrahim; Sezer, Ogun; Salmanoglu, Musa; Kilic, Erol; Karabacak, Ercan; Ozturk, Sami

    2016-01-01

    Although a few case reports about hypertensive anaphylaxis (HA) are available in the present literature, there is no study about the prevalence of HA. In this study, we review our cases with anaphylaxis presenting with hypertension and ascertain its prevalence. The documents of the patients who had anaphylactic reactions after the procedures performed for the diagnosis and treatment of allergic diseases in GATA Haydarpasa Clinic of Allergy and Immunology between January 2010 and December 2014 were retrospectively reviewed. Within the study period, 324 patients had undergone 4332 procedures in which 62 of them had developed anaphylaxis. During the procedures, the rate of anaphylaxis was found to be 1.43 %. The rate of HA among the anaphylaxis patients was 12.9 % (8 of 62 patients). During treatments, 2 patients received adrenaline injections without any adverse reaction. HA may be seen at a considerable rate during an anaphylactic reaction. Anaphylaxis and hypertension can be recovered by adrenaline injection when required. According to the best of our knowledge, this study is the first original study about the prevalence of HA in English-language medical literature.

  12. STUDY OF THE CLINICAL PROFILE AND AETIOLOGY OF VARIOUS DISORDERS OF SEX DEVELOPMENT PRESENTING TO ENDOCRINE OPD OF A TERTIARY CARE HOSPITAL

    Directory of Open Access Journals (Sweden)

    Ipsita Mishra

    2017-10-01

    Full Text Available BACKGROUND Disorders of Sex Development (DSD, formerly described as intersex conditions, are a conglomerate of rare disorders defined as discrepancy of chromosomal, gonadal or anatomic sex. There are limited data on the incidence of DSD with an overall incidence of 1:5,500, but varies with population. Congenital adrenal hyperplasia and mixed gonadal dysgenesis are the most common causes of ambiguous genitalia constituting approximately 50% of all cases presenting with genital ambiguity at birth. The aim of the study is to study the clinical profile and aetiology, mean age of presentation of common aetiologies, initial sex of rearing based on genital ambiguity and correctness of sex of rearing since birth as compared to genetic karyotype after diagnosis of patients of various disorders of sex development presenting to endocrine OPD of a tertiary care hospital. MATERIALS AND METHODS We assessed the records of patients who were evaluated for hypogonadism and genital ambiguity between March 2014 to June 2017 in our endocrine department. The patients were classified on the basis of clinical features, hormonal investigations, imaging studies, karyotype and laparoscopy/biopsy studies as indicated. Design- Cross-sectional study. RESULTS Distribution of DSD by category were 46, XY DSD (41.6%; 46, XX DSD (27.3%; SCD DSD (30.9%. Distribution of DSD by subtypes were 46, XY DSD; -5α reductase (37.1%; IHH (20.1%; Kallmann syndrome (14.28%; bilateral anorchia (11%; PAIS (8.5%; CAIS (2.8%; CAH (2.8%; 46, XX DSD-CAH (34.1%; IHH (21.7%; 46, XX OTD (13%; ACC (8.6%; classic CAH (4.3%; SCD DSD-KFS (53.8%; TS (38.4% and MGD (7.69%. Mean age of presentation of DSD; 5α reductase (7.5 yrs., PAIS (14.33 yrs., CAH (9.3 yrs., KFS (25 yrs. and TS (17 yrs.. CONCLUSION 46 XY DSD comprises 41.6% of cases of which 5α reductase deficiency is the most common aetiology. CAH was the main subtype of 46, XX DSD. KFS was the main subtype of SCD DSD. DSD pose a serious challenge not

  13. Uncommon presentation of pulmonary aspergilloma

    Directory of Open Access Journals (Sweden)

    Baradkar V

    2009-01-01

    Full Text Available Cases of pulmonary aspergilloma without any predisposing factors are rarely reported. Clinical presentation varies from case to case. Here, we report a case of pulmonary aspergilloma in a 60-year-old male patient who was admitted to the Intensive Respiratory Care Unit with spontaneous pneumothorax. The patient had a history of dyspnea on exertion since 9 months and mild haemoptysis since the last 6 months. A computerised tomographic scan of the lungs showed a lesion in the left main bronchus along with obstructive emphysema of the right lung, moderate pneumothorax and mediastinal emphysema. Bronchoscopy was performed and the biopsy samples were processed for histopathological examination and culture on Sabouraud′s dextrose agar, which yielded growth of Aspergillus flavus. Repeat sputum samples also yielded the growth of A. flavus . The patient responded to intravenous liposomaamphotericin B and intercostal drainage.

  14. Site selection: Past and present

    International Nuclear Information System (INIS)

    Tilford, N.R.

    1994-01-01

    Site selection has been going on since the earliest times. The process has evolved through the Industrial Revolution to the present period of exploding population and environmental awareness. Now the work must be done both with increasing sophistication and greater transparency. Modern techniques for site selection have been developed during the last two decades or so, utilizing a teachable body of knowledge and a growing literature. Many firms and individuals have contributed to this growing field. The driving force has been the need for such a process in siting and licensing of critical facilities such as nuclear power plants. A list of crucial, documented steps for identifying social impacts and acceptability are provided. A recent innovation is the self-selection method developed by government. The Superconducting Supercollider serves as an example of this approach. Geological or geologically dependent factors often dominate the process. The role as engineering and environmental geoscientists is to provide responsible leadership, consultation, and communication to the effort

  15. Neurocysticercosis presenting as pseudobulbar palsy

    Directory of Open Access Journals (Sweden)

    Arinaganahalli Subbanna Praveen Kumar

    2014-01-01

    Full Text Available Neurocysticercosis (NCC is the most common helminthic infestation of the central nervous system (CNS and a leading cause of acquired epilepsy worldwide. The common manifestations of NCC are seizures and headache. The NCC as a cause of pseudobulbar palsy is very unusual and not reported yet in the literature. A pseudobulbar palsy can occur in any disorder that causes bilateral corticobulbar disease. The common etiologies of pseudobulbar palsy are vascular, demyelinative, or motor neuron disease. We report a 38-year-old female patient who presented with partial seizures and pseudobulbar palsy. The MRI brain showed multiple small cysts with scolex in both the cerebral hemispheres and a giant intraparenchymal cyst. Our patient responded well to standard treatment of neurocysticercosis and antiepileptics.

  16. Present challenges in hadrontherapy techniques

    Science.gov (United States)

    Amaldi, U.; Braccini, S.

    2011-07-01

    Hadrontherapy is a high-precision technique in cancer radiation therapy, which allows obtaining a superior conformal treatment with respect to photons used in conventional radiation therapy. To reach this ambitious goal without reducing the patient throughput needed in a hospital-based environment, the physical and radiobiological properties of charged hadrons, protons and carbon ions in particular, have to be exploited at best, making use of the most modern technologies issued from research in nuclear and particle physics. In the present days, we are assisting to a continuous technological challenge, leading to the conception and to the development of innovative methods and instruments. In this paper, the most relevant challenges in dose delivery systems, gantries, imaging, quality assurance and particle accelerators are reviewed.

  17. Ebolavirus Evolution: Past and Present.

    Science.gov (United States)

    de La Vega, Marc-Antoine; Stein, Derek; Kobinger, Gary P

    2015-01-01

    The past year has marked the most devastating Ebola outbreak the world has ever witnessed, with over 28,000 cases and over 11,000 deaths. Ebola virus (EBOV) has now been around for almost 50 years. In this review, we discuss past and present outbreaks of EBOV and how those variants evolved over time. We explore and discuss selective pressures that drive the evolution of different Ebola variants, and how they may modify the efficacy of therapeutic treatments and vaccines currently being developed. Finally, given the unprecedented size and spread of the outbreak, as well as the extended period of replication in human hosts, specific attention is given to the 2014-2015 West African outbreak variant (Makona).

  18. Ebolavirus Evolution: Past and Present.

    Directory of Open Access Journals (Sweden)

    Marc-Antoine de La Vega

    Full Text Available The past year has marked the most devastating Ebola outbreak the world has ever witnessed, with over 28,000 cases and over 11,000 deaths. Ebola virus (EBOV has now been around for almost 50 years. In this review, we discuss past and present outbreaks of EBOV and how those variants evolved over time. We explore and discuss selective pressures that drive the evolution of different Ebola variants, and how they may modify the efficacy of therapeutic treatments and vaccines currently being developed. Finally, given the unprecedented size and spread of the outbreak, as well as the extended period of replication in human hosts, specific attention is given to the 2014-2015 West African outbreak variant (Makona.

  19. Tritium practices past and present

    International Nuclear Information System (INIS)

    Gede, V.P.; Gildea, P.D.

    1980-01-01

    History of the production and use of tritium, as well as handling techniques, are reviewed. Handling techniques first used at Lawrence Livermore National Laboratory made use of glass vacuum systems and relatively crude ion chambers for monitoring airborne activity. The first use of inert atmosphere glove boxes demonstrated that uptake through the skin could be a serious personnel exposure problem. Growing environmental concerns in the early 1970's resulted in the implementation by the Atomic Energy Commission of a new criteria to limit atmospheric tritium releases to levels as low as practicable. An important result of the new criteria was the development of containment and recovery systems to capture tritium rather than vent it to the atmosphere. The Sandia National Laboratories, Livermore, Tritium Research Laboratory containment and decontamination systems are presented as a typical example of this technology. The application of computers to control systems is expected to provide the greatest potential for change in future tritium handling practices

  20. Speaker's presentations. Energy supply security

    International Nuclear Information System (INIS)

    Pierret, Ch.

    2000-01-01

    This document is a collection of most of the papers used by the speakers of the European Seminar on Energy Supply Security organised in Paris (at the French Ministry of Economy, Finance and Industry) on 24 November 2000 by the General Direction of Energy and Raw Materials, in co-operation with the European Commission and the French Planning Office. About 250 attendees were present, including a lot of high level Civil Servants from the 15 European State members, and their questions have allowed to create a rich debate. It took place five days before the publication, on 29 November 2000, by the European Commission, of the Green Paper 'Towards a European Strategy for the Security of Energy Supply'. This French initiative, which took place within the framework of the European Presidency of the European Union, during the second half-year 2000. will bring a first impetus to the brainstorming launched by the Commission. (author)

  1. Different presentations of pulmonary criptococosis

    International Nuclear Information System (INIS)

    Londono Palacio, Natalia; Ojeda Paulina; Ortiz, Guillermo; Alwers Rolf C; Munive, Abraham Ali

    1998-01-01

    We presented 4 cases studies of criptococosis, all of them with pulmonary compromise, diagnosed at Hospital Santa Clara in a period of 5 years. It's important to note that none of the patients were HIV positive. In the first case studied the cellular immune compromise has, a multifactorial origin: rheumatoid arthritis and the administration of corticoids. In the second case studied, SLE can explain its origin. In the third case studied, because of the short hospital stay, the investigation with respect to the etiology of the immune-compromise was limited. In the fourth case studied, because of a low CD4 count Elisa immunoassay for HIV was repeated without its confirmation. Spontaneous recuperation was obtained, being free of infection for a period of five years

  2. Director general presentation to personnel

    CERN Multimedia

    2016-01-01

    Dear Colleagues, Many important discussions are scheduled for the upcoming Council Week (13-17 June) on topics including the Medium-Term Plan, the Pension Fund and other matters of great relevance to us.   I would therefore like to share the main outcome of the week with you and I invite you to join me and the Directors in the Main Auditorium at 10 a.m. on Thursday 23 June. The meeting will last about one hour and a webcast will also be available. Best regards, Fabiola Gianotti DG presentation to personnel Thursday 23 June at 10 am Main Auditorium Retransmission in Council Chamber, IT Auditorium, Kjell Jonhsen Auditorium, Prevessin 864-1-C02 Webcast on cern.ch/webcast More information on the event page.

  3. Atypical presentations of celiac disease

    Directory of Open Access Journals (Sweden)

    Balasa Adriana Luminita

    2016-08-01

    Full Text Available In this study we evaluated the association of celiac disease in 81 children with autoimmune disease and genetic syndromes over a two years periods (January 2014 to July 2016 in Pediatric Clinic in Constanta. Because the extraintestinal symptoms are an atypical presentation of celiac disease we determined in these children the presence of celiac disease antibodies: Anti-tissue Transglutaminase Antibody IgA and IgA total serum level as a screening method followeds in selective cases by Anti-tissue Transglutaminase Antibody IgG, anti-endomysial antibodies, deamidated gliadin antibodies IgA and IgG and intestinal biopsia. In our study 8 patients had been diagnosed with celiac disease with extraintestinal symptoms, of which 4 with type 1 diabetes, 1 patient with ataxia, 2 patients with dermatitis herpetiformis and 1 patient with Down syndrome that associate also autoimmune thyroiditis, alopecia areata, enamel hypoplasia.

  4. Oropharyngeal styloids: An unusual presentation

    Directory of Open Access Journals (Sweden)

    Lathadevi Hassan Thotappa

    2012-01-01

    Full Text Available The close proximity of the styloid process to many of the vital neurovascular structures in the neck makes it clinically significant. The styloid process is said to be elongated if it is longer than 3.0 cm in length. Anatomical variations are very common and clinical symptoms arising from such variations have to be recognized. Elongated styloid processes may cause chronic throat pain along with foreign body sensation, dysphagia, vague facial pain, and otalgia. Surgical excision of an elongated styloid is considered as a satisfactory treatment for such cases. Here, we present a unique case of bilaterally elongated styloids that could be visualized just by depressing the tongue, when they appeared like the tusks of an elephant in the oropharyngeal region.

  5. Splenic abscess: a rare presentation

    Directory of Open Access Journals (Sweden)

    Mohit Bhatia

    2015-01-01

    Full Text Available Splenic abscess is a rare clinical entity with an incidence of 0.2-0.7% in autopsy-based studies. When untreated, splenic abscess is associated with nearly 100% mortality; in treated patients, the mortality rate is 16.6% during the first 90 days. It mostly occurs in patients with neoplasia, immunodeficiency, trauma, diabetes or splenic infarct. The incidence of splenic abscess is thought to be growing because of the increase in the number of immunocompromised patients who are particularly at risk for this disease and also because of the widespread use of diagnostic modalities. However, the optimal treatment for this remains unclear. We present a case of a 42-year-old man diagnosed with multiloculated splenic abscess and was subjected to splenectomy.

  6. Safeguards instrumentation: past, present, future

    International Nuclear Information System (INIS)

    Higinbotham, W.A.

    1982-01-01

    Instruments are essential for accounting, for surveillance and for protection of nuclear materials. The development and application of such instrumentation is reviewed, with special attention to international safeguards applications. Active and passive nondestructive assay techniques are some 25 years of age. The important advances have been in learning how to use them effectively for specific applications, accompanied by major advances in radiation detectors, electronics, and, more recently, in mini-computers. The progress in seals has been disappointingly slow. Surveillance cameras have been widely used for many applications other than safeguards. The revolution in TV technology will have important implications. More sophisticated containment/surveillance equipment is being developed but has yet to be exploited. On the basis of this history, some expectations for instrumentation in the near future are presented

  7. Cavernous hemangioma presenting marked hyperostosis

    International Nuclear Information System (INIS)

    Kobata, Hitoshi; Miyake, Hiroji; Kitamura, Junji; Kajikawa, Hiroshi; Ohta, Tomio

    1988-01-01

    The authors report here a case of hemangioma of the left parietal bone which presented headache and papilledema. This patient is a 37-year-old female who had, prior to admission, complained of increasing headache for one year and blurred vision for three months. She had no history of head injury. Local physical examinations revealed a slight bulging in her left parietal region which was insensitive to palpation and not adherent to the overlying scalp. Neurological examinations revealed bilateral papilledema and an incongruous bitemporal upper quadrant defect in the visual field. All the other neurological and laboratory data were normal. A plain skull roentogenogram showed a 9 x 9 cm osteolytic and characteristic honeycomb lesion in the parietal region. Systemic bone survey revealed a similar lesion in the right tibia which was not histologically examined. A marked accumulation of isotopes was detected on the bone scintigrams at both lesions. Selective external carotid angiograms demonstrated a tumor stain fed by the superficial temporal, occipital, and middle meningial arteries. CT scans of the brain and skull clearly showed a local thickening of and structural changes in the skull bone and also a mass effect on the brain and lateral ventricle. The lesioned bone was removed en bloc and replaced by an artificial bone. It was highly vascular, but not adherent to the overlying dura. The post-operative course was uneventful, and the headache and papilledema disappeared. Hemangioma of the skull presenting marked hyperostosis, as reported above, seems to be rare. In addition, in this case, skeletal angioma without any clinical manifestation was detected. Clinical and radiological pictures of the hemangioma of the skull and other bones were briefly discussed. (author)

  8. Amebiasis Presenting as Pleuropulmonary Disease

    Science.gov (United States)

    Kubitschek, Kenneth R.; Peters, Jay; Nickeson, David; Musher, Daniel M.

    1985-01-01

    Seven patients with amebic liver abscess presenting as pleuropulmonary disease were admitted to hospital initially because of pulmonary symptoms and were found to have amebic liver disease. Three categories of pleuropulmonary involvement included reactive inflammation of the pleura or lung, rupture of a hepatic abscess into the pleural space and rupture of a hepatic abscess into the bronchial airways. The preferred medical treatment is with metronidazole, but rupture of hepatic amebic abscess into the pleural space requires drainage in addition to medical therapy. In contrast, rupture into the bronchus may provide spontaneous drainage so that only medical therapy is needed. Recovery from amebiasis in all three categories is generally complete. Morbidity and mortality increase with failure to correctly identify amebic infection of the liver as the underlying cause. Because, in new cases, no findings specifically suggest that pleuropulmonary disease is a complication of hepatic amebic abscess, this possibility needs to be considered, especially in persons who are at risk of having been infected with amebae. ImagesFigure 1.Figure 2. PMID:4013249

  9. Surface Disinfections: Present and Future

    Directory of Open Access Journals (Sweden)

    Matteo Saccucci

    2018-01-01

    Full Text Available The propagation of antibiotic resistance increases the chances of major infections for patients during hospitalization and the spread of health related diseases. Therefore finding new and effective solutions to prevent the proliferation of pathogenic microorganisms is critical, in order to protect hospital environment, such as the surfaces of biomedical devices. Modern nanotechnology has proven to be an effective countermeasure to tackle the threat of infections. On this note, recent scientific breakthroughs have demonstrated that antimicrobial nanomaterials are effective in preventing pathogens from developing resistance. Despite the ability to destroy a great deal of bacteria and control the outbreak of infections, nanomaterials present many other advantages. Moreover, it is unlikely for nanomaterials to develop resistance due to their multiple and simultaneous bactericidal mechanisms. In recent years, science has explored more complex antimicrobial coatings and nanomaterials based on graphene have shown great potential in antibacterial treatment. The purpose of this article is to deepen the discussion on the threat of infections related to surface disinfection and to assess the state of the art and potential solutions, with specific focus on disinfection procedures using nanomaterials.

  10. Database Systems - Present and Future

    Directory of Open Access Journals (Sweden)

    2009-01-01

    Full Text Available The database systems have nowadays an increasingly important role in the knowledge-based society, in which computers have penetrated all fields of activity and the Internet tends to develop worldwide. In the current informatics context, the development of the applications with databases is the work of the specialists. Using databases, reach a database from various applications, and also some of related concepts, have become accessible to all categories of IT users. This paper aims to summarize the curricular area regarding the fundamental database systems issues, which are necessary in order to train specialists in economic informatics higher education. The database systems integrate and interfere with several informatics technologies and therefore are more difficult to understand and use. Thus, students should know already a set of minimum, mandatory concepts and their practical implementation: computer systems, programming techniques, programming languages, data structures. The article also presents the actual trends in the evolution of the database systems, in the context of economic informatics.

  11. STS-70 Post Flight Presentation

    Science.gov (United States)

    Peterson, Glen (Editor)

    1995-01-01

    In this post-flight overview, the flight crew of the STS-70 mission, Tom Henricks (Cmdr.), Kevin Kregel (Pilot), Major Nancy Currie (MS), Dr. Mary Ellen Weber (MS), and Dr. Don Thomas (MS), discuss their mission and accompanying experiments. Pre-flight, launch, and orbital footage is followed by the in-orbit deployment of the Tracking and Data Relay Satellite (TDRS) and a discussion of the following spaceborne experiments: a microgravity bioreactor experiment to grow 3D body-like tissue; pregnant rat muscular changes in microgravity; embryonic development in microgravity; Shuttle Amateur Radio Experiment (SAREX); terrain surface imagery using the HERCULES camera; and a range of other physiological tests, including an eye and vision test. Views of Earth include: tropical storm Chantal; the Nile River and Red Sea; lightning over Brazil. A three planet view (Earth, Mars, and Venus) was taken right before sunrise. The end footage shows shuttle pre-landing checkout, entry, and landing, along with a slide presentation of the flight.

  12. A cool present for LEIR

    CERN Multimedia

    2005-01-01

    LEIR (Low Energy Ion Ring), which will supply lead ions to the LHC experiments, has taken delivery of one of its key components, its electron cooling system. From left to right, Gérard Tranquille, Virginia Prieto and Roland Sautier, in charge of the electron cooling system for LEIR at CERN, and Christian Lacroix, in charge of installation for the LEIR machine. On 16 December, the day before CERN's annual closure, the LEIR teams received a rather impressive Christmas present. The "parcel" from Russia, measuring 7 metres in length and 4 metres in height, weighed no less than 20 tonnes! The component will, in fact, be one of the key elements of the future LEIR, namely its electron cooling system. LEIR is one of the links in the injector chain that will supply lead ions to the LHC experiments, in particular ALICE (see Bulletin No. 28/2004 of 5 July 2004), within the framework of the I-LHC Project. The electron cooling system is designed to reduce and standardise transverse ion velocity. This focuses the bea...

  13. Present status of Tokamak research

    International Nuclear Information System (INIS)

    Basu, Jayanta

    1991-01-01

    The scenario of thermonuclear fusion research is presented, and the tokamak which is the most promising candidate as a fusion reactor is introduced. A brief survey is given of the most noteworthy tokamaks in the global context, and fusion programmes relating to Next Step devices are outlined. Supplementary heating of tokamak plasma by different methods is briefly reviewed; the latest achievements in heating to fusion temperatures are also reported. The progress towards the high value of the fusion product necessary for ignition is described. The improvement in plasma confinement brought about especially by the H-mode, is discussed. The latest situation in pushing up Β for increasing the efficiency of a tokamak is elucidated. Mention is made of the different types of wall treatment of the tokamak vessel for impurity control, which has led to a significant improvement in tokamak performance. Different methods of current drive for steady state tokamak operation are reviewed, and the issue of current drive efficiency is addressed. A short resume is given of the various diagnostic methods which are employed on a routine basis in the major tokamak centres. A few diagnostics recently developed or proposed in the context of the advanced tokamaks as well as the Next Step devices are indicated. The important role of the interplay between theory, experiment and simulation is noted, and the areas of investigation requiring concerted effort for further progress in tokamak research are identified. (author). 17 refs

  14. Presentation of suspected pediatric uveitis.

    Science.gov (United States)

    Saeed, Muhammad Usman; Raza, Syed Hamid; Goyal, Sudeshna; Cleary, Gavin; Newman, William David; Chandna, Arvind

    2014-01-01

    Presentation of suspected pediatric uveitis: Pediatric uveitis is usually managed in specialized ophthalmic centers in the UK. Meaningful data acquisition in these clinics may be helpful in clinical governance, and healthcare planning in a specialty that is gradually changing due to changes in treatment choices. Retrospective analysis of prospectively acquired data in the Liverpool pediatric uveitis database was performed. Analysis of our data, based on 147 patients, with a mean age of 10 years, indicated a female to male ratio of 2:1. 99% of patients were Caucasian. Our data indicates 86% of all patients attending the uveitis clinic were diagnosed with juvenile idiopathic arthritis, followed by intermediate uveitis 5% and idiopathic uveitis 4%. 46% of patients required treatment. Systemic treatment included methotrexate (34%), prednisolone (14%), etanercept (6%), ciclosporin (6%), mycophenolate (3%), and infliximab (1%). Severe visual loss (defined by counting fingers or below vision) was seen in 10 eyes despite appropriately treated chronic uveitis. Our data shows uveitis-related ocular morbidity in a predominantly pediatric Caucasian population. Patients with severe and chronic uveitis may experience significant uveitis-related complications and subsequent visual loss despite aggressive treatment.

  15. UNSCEAR: present and future activities

    International Nuclear Information System (INIS)

    Holm, Lars-Erik

    2000-01-01

    United Nations Scientific Committee on the Effects of Atomic Radiation (UNSCEAR) was established in 1955. The Committee reviews the exposure of the world population to all sources of radiation under normal circumstances as well as after accidents, and is also the prime international scientific body reviewing and assessing the health risks of exposure to ionizing radiation. Its estimates have been and are still being used by major international bodies including the ICRP, IAEA, and WHO. UNSCEAR's publications form the scientific basis on which international and national agencies develop appropriate radiation protection standards for workers, patients and the general public. UNSCEAR reports yearly to the General Assembly and submits regularly comprehensive reports with detailed scientific annexes on the sources and biological effects of ionizing radiation. In 1995, UNSCEAR began a new program of review of the sources, exposure and biological effects of ionizing radiation. A comprehensive report with scientific annexes will be published in the year 2000. The report will include exposures from natural and man-made radiation sources, occupational and medical radiation exposures, and dose assessment methodologies. The biological effects will cover epidemiological evaluation of radiation-induced cancer, DNA repair and mutagenesis, hereditary effects, combined effects of radiation and other agents, as well as models, mechanisms and uncertainties of biological effects at low radiation doses. An assessment of exposures and effects of the Chernobyl accidents is also made. A brief overview is presented here of the range of subjects considered by the Committee. (author)

  16. Exoplanets: Past, Present, and Future

    Directory of Open Access Journals (Sweden)

    Chien-Hsiu Lee

    2018-04-01

    Full Text Available Our understanding of extra-solar planet systems is highly driven by advances in observations in the past decade. Thanks to high precision spectrographs, we are able to reveal unseen companions to stars with the radial velocity method. High precision photometry from the space, especially with the Kepler mission, enables us to detect planets when they transit their stars and dim the stellar light by merely one percent or smaller. Ultra wide-field, high cadence, continuous monitoring of the Galactic bulge from different sites around the southern hemisphere provides us the opportunity to observe microlensing effects caused by planetary systems from the solar neighborhood, all the way to the Milky Way center. The exquisite AO imaging from ground-based large telescopes, coupled with high-contrast coronagraph, captured the photons directly emitted by planets around other stars. In this article, I present a concise review of the extra-solar planet discoveries, discussing the strengths and weaknesses of the major planetary detection methods, providing an overview of our current understanding of planetary formation and evolution given the tremendous observations delivered by various methods, as well as on-going and planned observation endeavors to provide a clear picture of extra-solar planetary systems.

  17. Presentation of the results 97

    International Nuclear Information System (INIS)

    1998-01-01

    Gaz de France (GDF) presents in a press kit the main results achieved in 1997. The European community has formulated a project of definition of rules concerning the natural gas market. Minima values for annual consumptions have been defined and the consumption and use of natural gas in EC countries is described. The financial results of GDF in 1997 are compared with previous years. Net sales grew by nearly 1 billion french francs to reach 55 billion. Overall sales were down by 4% but the share of natural gas in french energy consumption increased and sales to industrial users went up in particular due to new cogeneration plants installed in 1996. A comparison of gas prices in Europe is given for the years 1985 to 1998. The efforts of GDF to offer increased quality of services concerning in particular safety aspects as checking programs and installation of new safeguard appliances are described. The efforts of GDF to increase the energy market share of natural gas resulted in the development of new products like cogeneration, air conditioning and fuels for automotive needs. (C.B.)

  18. Nuclear Fuels: Present and Future

    Directory of Open Access Journals (Sweden)

    Donald R. Olander

    2009-02-01

    Full Text Available The important new developments in nuclear fuels and their problems are reviewed and compared with the status of present light-water reactor fuels. The limitations of these fuels and the reactors they power are reviewed with respect to important recent concerns, namely provision of outlet coolant temperatures high enough for use in H2 production, destruction of plutonium to eliminate proliferation concerns, and burning of the minor actinides to reduce the waste repository heat load and long-term radiation hazard. In addition to current oxide-based fuel-rod designs, the hydride fuel with liquid metal thermal bonding of the fuel-cladding gap is covered. Finally, two of the most promising Generation IV reactor concepts, the Very High Temperature Reactor and the Sodium Fast Reactor, and the accompanying reprocessing technologies, aqueous-based UREX and pyrometallurgical, are summarized. In all of the topics covered, the thermodynamics involved in the material's behavior under irradiation and in the reprocessing schemes are emphasized.

  19. [ISRAEL NEONATOLOGY: PRESENT AND FUTURE].

    Science.gov (United States)

    Dollberg, Shaul

    2016-01-01

    The practice of neonatology in Israel debuted in the 1970s as local enterprises by individual hospitals that needed to provide sick and preterm newly born infants with up-to-date and effective care. Descriptions of research and advances in humane and gentle treatment during neonatal care for preterm infants and their families, as well as prevention of neonatal infections, follow-up of preterm infants and care of full-term infants are presented in this issue. The Israel National Very Low Birth Weight (VLBW) Infant database provides an excellent source of knowledge, which has led to multiple scientific publications. Recent international comparisons of the outcome of preterm VLBW infants, made possible by this unique database in Israel, has provided the neonatal community and the Ministry of Health with insights as to the differences in prognosis between Israel and other countries, especially among extremely low birth weight infants. At the border of viability, mortality in Israelis significantly higher than that reported in other countries and proactive steps undertaken to examine these differences and prompt correctional action should be pursued. The Israel Ministry of Health started positive initiatives and should ensure that their steps are implemented at the preterm infant's bedside.

  20. Source rock hydrocarbons. Present status

    International Nuclear Information System (INIS)

    Vially, R.; Maisonnier, G.; Rouaud, T.

    2013-01-01

    This report first presents the characteristics of conventional oil and gas system, and the classification of liquid and gaseous non conventional hydrocarbons, with the peculiar case of coal-bed methane. The authors then describe how source rock hydrocarbons are produced: production of shale oils and gases (horizontal drilling, hydraulic fracturing, exploitation) and of coal-bed methane and coal mine methane. In the next part, they address and discuss the environmental impact of source rock hydrocarbon production: installation footprint, water resource management, drilling fluids, fracturing fluids composition, toxicity and recycling, air pollution, induced seismicity, pollutions from other exploitation and production activities. They propose an overview of the exploitation and production of source rock gas, coal-bed gas and other non conventional gases in the world. They describe the current development and discuss their economic impacts: world oil context and trends in the USA, in Canada and other countries, impacts on the North American market, on the world oil industry, on refining industries, on the world oil balance. They analyse the economic impacts of non conventional gases: development potential, stakes for the world gas trade, consequence for gas prices, development opportunities for oil companies and for the transport sector, impact on CO 2 emissions, macro-economic impact in the case of the USA

  1. Radiation epidemiology: Past and present

    International Nuclear Information System (INIS)

    Boice, J.D. Jr.

    1997-01-01

    Major advancements in radiation epidemiology have occurred during the last several years in studies of atomic bomb survivors, patients given medical radiation, and radiation workers, including underground miners. Risks associated with the Chernobyl accident, indoor radon and childhood exposure to I-131 have yet to be elucidated. Situations in the former Soviet Union around Chelyabinsk, a nuclear installation in the southern Urals, and in the Altai, which received radioactive fallout from weapons testing at Semipalatinsk, Kazakhstan, have the potential to provide information on the effects of chronic radiation exposure. Since Roentgen's discovery of x-rays just 100 years ago, a tremendous amount of knowledge has been accumulated about human health effects following irradiation. The 1994 UNSCEAR report contains the latest compilation and synthesis of radiation epidemiology. This overview will cover epidemiology from a radiation perspective. The different types of study methodologies will be described, followed by a kaleidoscope coverage of past and present studies; ending with some remaining questions in radiation epidemiology. This should set the stage for future chapters, and stimulate thinking about implications of the new data on radiation cancer risks

  2. Extraskeletal presentation of Ewing's Sarcoma.

    Science.gov (United States)

    Mangual, Danny; Bisbal-Matos, Luis A; Jiménez-Lee, Ricardo; Vélez, Román; Noy, Miguel

    2018-03-01

    The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the patient remains in complete remission.

  3. Clinical presentation of primary hypothyroidism

    International Nuclear Information System (INIS)

    Ahmed, B.; Hussain, T.; Memon, A. R.; Solangi, G. A.

    2001-01-01

    Objective: To determine the clinical preparation of primary hypothyroidism at the time of diagnosis. Design: It was an observational and prospective study. Place and Duration of Study: Civil Hospital, Karachi from 1st January 1997 to 31st December 1997. Subjects and Methods: Forty-eight consecutive cases of primary hypothyroidism were included. A detailed history with special emphasis on symptoms and signs was conducted and the findings observed were noted in previously made proforma. Results: Primary hypothyroidism was found to be 5 times more common in female patients, with male to female ratio 1:5, majority of cases (33.33%) were between 41 to 50 years of age. The common symptoms at the time of presentation were tiredness (95.8%), weakness (91.6%), weight gain (85.4%), hoarseness of voice (83.3%), cold intolerance (77.0%) and constipation (75.0%) in patients. Physical signs observed were delayed relaxation of ankle jerk (93.7%), periorbital edema (83.3%), thick tongue (62.5%), goiter (50.0%), dry and coarse skin (47.9%) in patients. Serum, TSH, T4 and T3 were performed in all cases through immunoradiometric technique. Serum TSH was markedly elevated, with normal to reduced T4 and T3 levels in all cases. Conclusion: Primary hypothyroidism was found more common in female than male of the age group 41-50 years. Weakness, tiredness, hoarseness of voice and constipation were the common symptoms noted. The diagnosis is almost certain on clinical grounds if the patient happens to have delayed relaxation of ankle jerks and periorbital puffiness. (author)

  4. Past and present Aral Sea

    Science.gov (United States)

    Dukhovniy, Viktor; Stulina, Galina; Eshchanov, Odylbek

    2013-04-01

    The tragedy of disappearing of Aral Sea is well known to the World. Before and after collapse of Soviet Union, a huge quantity of scientific and popular editions described with grief the situation around the Aral Sea. After the NIS states became independent, World Bank, UNDP, UNEP in proper competition with each other had provided some assessment of the situation through presentation of some small and medium grants, but after 2000, the local population remained alone with own problems. Although on the eyes of the present generation a unique transformation of great water body into deserts took place, the global scientific community did not find forces and financing for real and detail investigation of the processes accompanying the Sea shrinking and land formation. We should acknowledge and give big respect to NATO, later to German Government that through GTZ (now GIZ) - German International Collaboration Agency - and GFZ (Potzdam) paid attention to this area of environment crisis and organized scientific and protective design in the so-called Priaralie - the territory around the drying Sea and delta of the two rivers - Amudarya and Syrdarya. Thank to this assistance, the local specialists in collaboration with limited a number of foreign scientists (N.Aladin, P.Zavialov, Joop de Schutter, Hans Wilps, Hedi Oberhansli) organized significant works for detail socioeconomic, ecological and hydrological assessment situation in Priaralie and on the Aral sea coast. On this base, Ministry of Agriculture and Water resources of Uzbekistan and State Committee of Water resources of Kazakhstan developed a plan of rehabilitation of Amudarya and Syrdarya deltas and started implementation of these projects. If Kazakh water authority moved ahead in wetland restoration faster, a forestation of delta and drying bed of Aral Sea got big success in Uzbek territory. 244 thousands hectares of saxsaul and tamarix were planted for protection of the Priaralie. By request of GTZ SIC, ICWC

  5. Como responder ao momento presente?

    Directory of Open Access Journals (Sweden)

    Maria Filomena Molder

    2013-12-01

    Full Text Available http://dx.doi.org/10.5007/1984-784X.2013v13n19p13 Foi com esta pergunta — já um efeito de um primeiro encontro entre Irene Pimentel e eu própria — que decidimos desafiar colegas, estudantes e funci­onários da nossa Faculdade, FCSH (Faculdade de Ciências Sociais e Huma­nas, de outras Faculdades da Universidade Nova de Lisboa, de outras Uni­versidades e todos os interessados em con­siderar e discutir em comum aquilo que se passava em Portugal e que no anúncio da Jornada de 6 de De­zembro de 2012 se descrevia como um “processo de desmantela­mento social, económico e cultural sem precedentes — pese embora tantas compara­ções, baseadas na premissa da ‘eterna repetição’ — e cujas consequências não param de exceder as previsões dos responsáveis por esse desmantelamento”. Acedendo com todo o empenho e gratidão ao convite que me foi dirigido por Humberto Brito para fazer uma resenha da Jornada a publicar no primeiro número de Forma de Vida (saúdo a revista e o título, decidi-me, no entanto, a pôr de lado a resenha, que sob a forma de “Editorial” será em breve publi­cada no blogue Responder ao Momento Presente, entre­tanto criado, conjuntamente com os textos escritos pelos nossos convidados, com as parti­cipações de pessoas que corresponderam ao nosso apelo e ainda com contri­bui­ções que se alargaram para lá da Jornada; a que se juntará uma gravação em video, também disponível no Youtube.   Texto publicado originalmente em Forma de Vida, Lisboa, n.1, fev. 2013. Agrade­cemos à autora por permitir a republicação neste número do Boletim. [N.E.

  6. A rare case of Turner′s syndrome presenting with Mullerian agenesis

    Directory of Open Access Journals (Sweden)

    Suresh Vaddadi

    2013-01-01

    Full Text Available Turner′s syndrome also called as Ullrich Turner′s syndrome, is a disease of unclear pathogenesis characterized by complete or partial absence of one sex chromosome, with or without cell line mosaicism in a phenotypic female with short stature. Various anomalies result in a constellation of features, of which the most disturbing is primary amenorrhea due to gonadal dysgenesis. Hormone therapy in these patients can often result in successful menstruation, and scope for subsequent pregnancy because of anatomically normal uterus and vagina. Coexisting Mullerian agenesis in these patients can jeopardize the chances of future pregnancy as they have associated structural abnormalities of the uterus and vagina. We report a rare case of middle-aged female with Turner′s syndrome and Mullerian agenesis having absent secondary sexual characters and missing uterus with incompletely formed vagina.

  7. Draft Workshop Summary and Presentations

    International Nuclear Information System (INIS)

    Houssin, Didier; ); Tam, Cecilia; ); Cameron, Ron; ); Paillere, Henri; ); Lisann, Beth; Ehlert, Andreas; Kaufer, Barry; Decobert, Veronique; Beutier, Didier; Crepin, Anne; Aurela, Jorma; Baritaud, Manuel; ); Rooke, Oliver; Blundell, Neil; ); Shropshire, David; Noren, Bjarne; Blundell, Neil; ); Dujardin, Thierry; )

    2014-01-01

    the electricity market reform in the UK); Session 4: Nuclear regulation and safety (Regulation for new build and new comer countries and Fukushima Safety Action Plan; WANO's contribution to strengthen nuclear safety worldwide; Strengthening Good Practice within Nuclear Safety post Fukushima-Daiichi); Session 5: Key Message for the Road-map update (Outline current status of nuclear technology development and need for additional R and D to address increased safety requirements; Provide an updated vision for nuclear energy's role in a low carbon energy system; Identify barriers and actions needed to accelerate the development and deployment of nuclear technologies to meet the Road-map vision; Identify and share lessons learned and best practices in nuclear regulation, front and back end fuel cycle practices, construction and financing. This document brings together the available presentations (slides) given at this workshop: 1 - Road-map overview and role of nuclear in IEA scenarios (Cecilia Tam, IEA); 2 - The Direction Of The 2014 Nuclear Road-map (Ron Cameron, OECD/NEA); 3 - Technology development needs for nuclear energy, 1.a: Reactor technology (Henri Paillere, OECD/NEA); 4 - IEA/NEA Nuclear Technology Road-map Update (Beth Lisann, US-DoE); 5 - Update of the Nuclear Energy Technology Road-map - Nuclear Decommissioning (Andreas Ehlert, E-On); 6 - Overcoming barriers to nuclear build - other than financing (Veronique Decobert, Westinghouse); 7 - Development of the supply chain: relying on local industrial involvement (Didier Beutier, Areva); 8 - Training and capacity development needs (Jorma Aurela, Finnish Ministry of Employment and the Economy); 9 - Key conditions for successful Nuclear new build financing (Anne Crepin, Areva); 10 - Regulation for New Build and Newcomers, IAEA Nuclear Safety Action Plan (David Shropshire, IAEA); 11 - WANO's contribution to strengthen nuclear safety worldwide (Bjarne Noren, WANO); 12 - Strengthening Good Practice within Nuclear Safety

  8. 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

  9. 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.

  10. 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.

  11. 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

  12. 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)

  13. 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

  14. 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.

  15. 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

  16. 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.

  17. 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...

  18. 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)

  19. 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)

  20. 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.)

  1. 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

  2. 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.

  3. 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

  4. 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.

  5. 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.

  6. 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.

  7. 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.

  8. 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.

  9. 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

  10. 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...

  11. 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.

  12. 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.

  13. 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

  14. 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.

  15. 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)

  16. 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.

  17. Selected Regional Judicial Officer Cases, 2005 - Present

    Data.gov (United States)

    U.S. Environmental Protection Agency — This dataset contains selected cases involving EPA's Regional Judicial Officers (RJOs) from 2005 to present. EPA's Regional Judicial Officers (RJOs) perform...

  18. 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…

  19. 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)

  20. Speechline - a method for teaching oral presentation

    DEFF Research Database (Denmark)

    Juel, Henrik

    2015-01-01

    The Speech-line method aims at improving the active rhetorical skills of students, i.e. their ability to present academic subjects in a clear and convincing manner. This is relevant not only for everyday classroom work, but also for oral exams, for presenting research projects, and for addressing...

  1. 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.

  2. 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

  3. The spanish radiotherapy park: past and present

    International Nuclear Information System (INIS)

    Tormo Ferrero, Manuel J.

    2001-01-01

    The present article has as objective to provide a general overview on the spanish radiotherapy park, presenting how was its start and evolution until the current state. Considering only the units of teletherapy and the accelerators. Actually in Spain there is 28 units of Cobalt therapy, in functioning during the last two decades, being advised a rapid substitution to accelerators

  4. 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 ...

  5. Acute intermittent porphyria presenting as progressive muscular ...

    African Journals Online (AJOL)

    Acute intermittent porphyria, the most common porphyria affecting the nervous system, typically presents with neurovisceral crises followed by a motor neuropathy. We describe a 23-year-old black South African man presenting with a progressive stuttering, lower motor neuron syndrome developing over months. He had not ...

  6. Story Presentation Effects on Children's Retell Content

    Science.gov (United States)

    Schneider, Phyllis; Dube, Rita Vis

    2005-01-01

    This study investigated the possibility that the amount of content children include in their stories is affected by how stories are presented. Simple stories were presented to kindergarten and Grade 2 children in 3 conditions: orally (oral only), pictorially (pictures only), and combined oral and pictures. The kindergarteners recalled more content…

  7. 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

  8. 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...

  9. Central nervous system tuberculomata presenting as internuclear ...

    African Journals Online (AJOL)

    Central nervous system (CNS) tuberculoma can have variable presentation depending upon the site and number of tuberculomata. We are reporting a rare case of a 15 years old girl who presented to our hospital with binocular diplopia on right gaze. Clinical examination revealed left sided internuclear ophthalmoplegia ...

  10. 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

  11. Extranodal presentation in patients with acquired immunodeficiency ...

    African Journals Online (AJOL)

    NHL is the second most common malignancy associated with HIV infection. The clinical presentation varies, and while patients may present with symptomatic lymphadenopathy, many have extranodal disease. Extranodal lymphomas arise from tissue other than lymph nodes and even sites that normally contain no lymphoid ...

  12. Changing Dynamics in the Voluntary Market (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Heeter, J.

    2014-12-01

    Voluntary green power markets are those in which consumers and institutions voluntarily purchase renewable energy to match their electricity needs. This presentation, presented at the Renewable Energy Markets Conference in December 2014, outlines the voluntary market in 2013, including community choice aggregation and community solar.

  13. 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...

  14. Genitourinary complications as initial presentation of inherited ...

    African Journals Online (AJOL)

    Epidermolysis bullosa (EB) is a rare disorder that presents with urological complications. We present a 6-year-old boy admitted with urological symptoms that revealed an inherited EB misdiagnosed. We also review the literature on this disorder and management of the common urological complications.

  15. Using the Microcomputer for Advertising Research Presentations.

    Science.gov (United States)

    Larkin, Ernest F.

    A midwestern university is testing a program that uses the Apple II computer to help students in an advertising research course develop their skills in preparing and presenting research reports using computer generated graphics for both oral and written presentations. One of the course requirements is the preparation of a final project, including…

  16. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Background: Acute Lymphoblastic Leukaemia in children commonly presents with osteo articular manifestations that may mimic Juvenile Idiopathic Arthritis. This may create considerable diagnostic difficulty and lead to delay in commencing appropriate treatment. Case: An eight year old boy who presented with multiple ...

  17. Diffuse benign gastric inflammatory hyperplastic polyps presenting ...

    African Journals Online (AJOL)

    Benign gastric inflammatory hyperplasic polyps are benign lesions that rarely occur in young age. We report a case of diffuse benign gastric inflammatory hyperplastic polyps in a 19 year old boy who presented with cough, nausea, and hematamesis. In the presented case symptoms such as nausea and vomiting are non ...

  18. 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.

  19. 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...

  20. 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.

  1. 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.

  2. 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

  3. 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.

  4. 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.

  5. An unusual presentation of galactosemia: Hemophagocytic lymphohistiocytosis

    Directory of Open Access Journals (Sweden)

    Ahmet Afşin Kundak

    2012-12-01

    Full Text Available Hemophagocytic lymphohistiocytosis (HLH is a rare life-threatening condition. Uncontrolled proliferation of activated lymphocytes secreting high amounts of inflammatory cytokines seems to be the main pathogenesis. The diagnosis of HLH can often be difficult. It may presents in many forms such as fever of unknown origin, hepatitis, acute liver failure, and sepsis-like illness. Here we present a newborn galactosemia case presented with HLH. Close monitoring of the diagnostic criteria of HLH during the course of galactosemia-associated hemophagocytosis, both before and after dietary treatment, should be performed in order to fully determine if the triggering mechanism is infection or accumulation of metabolites.

  6. Past and present trends of nuclear chemistry

    International Nuclear Information System (INIS)

    Matel, L.; Kuruc, J.

    2007-01-01

    This book represents not only the papers and lectures presented on the Seminar at the occasion of forty years of foundation of the Department of Nuclear Chemistry which took place on October 3 - 5, 2006 in Kezmarske Zlaby (High Tatras). It also contains the papers and presentations of post-graduate students and workers of the Department of Nuclear Chemistry as well as colleagues working in different field of nuclear chemistry and radioecology on various workplaces in the Slovak Republic, too. The book contains 17 papers, 15 presentations, photographs and 3 short video recording

  7. Gynaecomastia as the initial presentation of thyrotoxicosis

    Directory of Open Access Journals (Sweden)

    Triranjan Sanyal

    2012-01-01

    Full Text Available Gynaecomastia is a well-recognized manifestation of thyrotoxicosis occurring in a widely variable percentage. Some studies have reported the percentage to be high as 40% while others report it being around 10%. The variation can be attributed to the difference in the criteria used to define gynae-comaslia or racial factors. However, thyrotoxicosis presenting with gynaceomastia as an intial manifestation is very rare. Only handful case reports are found in literature. Here we present a case of 35-year-old Indian male, with thyrotoxcosis who presented initially with painful bilateral gynaceomastia.

  8. 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.

  9. Lymphogranuloma Venereum Presenting as a Rectovaginal Fistula

    Directory of Open Access Journals (Sweden)

    C. M. Lynch

    1999-01-01

    Full Text Available Lymphogranuloma venereum (LGV is a rare form of the sexually transmitted disease caused by Chlamydia trachomatis. In the United States, there are fewer than 350 cases per year. In a review of the world’s literature, there has not been a case reported in the last thirty years of a case ofLGV presenting as a rectovaginal fistula. We present a case of an otherwise healthy American woman who presented with a rectovaginal fistula. Although uncommon, LGV does occur in developed countries and may have devastating tissue destruction if not recognized and treated before the tertiary stage. Infect. Dis. Obstet. Gynecol. 7:199–201, 1999.

  10. Lymphogranuloma venereum presenting as a rectovaginal fistula.

    Science.gov (United States)

    Lynch, C M; Felder, T L; Schwandt, R A; Shashy, R G

    1999-01-01

    Lymphogranuloma venereum (LGV) is a rare form of the sexually transmitted disease caused by Chlamydia trachomatis. In the United States, there are fewer than 350 cases per year. In a review of the world's literature, there has not been a case reported in the last thirty years of a case of LGV presenting as a rectovaginal fistula. We present a case of an otherwise healthy American woman who presented with a rectovaginal fistula. Although uncommon, LGV does occur in developed countries and may have devastating tissue destruction if not recognized and treated before the tertiary stage. PMID:10449269

  11. 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.

  12. 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.

  13. 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)

  14. Moxibustion for Cephalic Version of Breech Presentation.

    Science.gov (United States)

    Schlaeger, Judith M; Stoffel, Cynthia L; Bussell, Jeanie L; Cai, Hui Yan; Takayama, Miho; Yajima, Hiroyoshi; Takakura, Nobuari

    2018-05-01

    Moxibustion, a form of traditional Chinese medicine (TCM), is the burning of the herb moxa (Folium Artemisiae argyi or mugwort) over acupuncture points. It is often used in China to facilitate cephalic version of breech presentation. This article reviews the history, philosophy, therapeutic use, possible mechanisms of action, and literature pertaining to its use for this indication. For moxibustion, moxa can be rolled into stick form, placed directly on the skin, or placed on an acupuncture needle and ignited to warm acupuncture points. Studies have demonstrated that moxibustion may promote cephalic version of breech presentation and may facilitate external cephalic version. However, there is currently a paucity of research on the effects of moxibustion on cephalic version of breech presentation, and thus there is a need for further studies. Areas needing more investigation include efficacy, safety, optimal technique, and best protocol for cephalic version of breech presentation. © 2018 by the American College of Nurse-Midwives.

  15. 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....

  16. Demographics and presenting clinical features of childhood ...

    African Journals Online (AJOL)

    annaline

    predominance in adolescence and adulthood. The true incidence of ... Conclusion. There is a diverse array of presenting features in childhood SLE. There has .... difficult by the psychological problems associated with coping with a chronic ...

  17. 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

  18. Suntech, presentation of a new giant

    International Nuclear Information System (INIS)

    Marandet, L.

    2007-01-01

    In few years this chinese Group became the third world producer of solar cells. This success is resulting of real policy of investment and expansion. the author proposes a presentation of this firm and their success. (A.L.B.)

  19. injury to presentation delays among musculoskeletal trauma ...

    African Journals Online (AJOL)

    Background: Injuries are a common cause of morbidity and mortality in the developing world with road traffic ... Methods: All musculoskeletal injury patients presenting to Mulago Hospital were prospectively .... who fell or were injured in sports.

  20. Systemic Lupus Erythematosus Presenting as Acute Adrenal ...

    African Journals Online (AJOL)

    hanumantp

    presented to us with a history of anorexia, progressive darkening of the face ... to us in an acute hypoadrenal state and was found to have Systemic lupus erythematosus with renal involvement. .... Textbook of Endocrinology. 11th ed. Saunders: ...

  1. 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

  2. The Effect of Summarizing and Presentation Strategies

    Directory of Open Access Journals (Sweden)

    Hooshang Khoshsima

    2014-07-01

    Full Text Available The present study aimed to find out the effect of summarizing and presentation strategies on Iranian intermediate EFL learners’ reading comprehension. 61 students were selected and divided into two experimental and control groups. The homogeneity of their proficiency level was established using a TOEFL proficiency test. The experimental group used the two strategies three sessions each week for twenty weeks, while the control group was not trained on the strategies. After every two-week instruction, an immediate posttest was administered. At the end of the study, a post-test was administered to both groups. Paired-sample t-test and Independent sample t-test were used for analysis. The results of the study revealed that summarizing and presentation strategies had significant effect on promoting reading comprehension of intermediate EFL learners. It also indicated that the presentation strategy was significantly more effective on students’ reading comprehension.

  3. Marine geophysical data management and presentation system

    Digital Repository Service at National Institute of Oceanography (India)

    Kunte, P.D.

    ) of the National Institute of Oceanography, Goa, India. GPDMPS is designed for the computerized storage retrieval and presentation of marine geophysical data and information. For the systematic management of geophysical data and information, GPDMPS is subdivided...

  4. Electronic Resources Management Project Presentation 2012

    KAUST Repository

    Ramli, Rindra M.

    2012-01-01

    This presentation describes the electronic resources management project undertaken by the KAUST library. The objectives of this project is to migrate information from MS Sharepoint to Millennium ERM module. One of the advantages of this migration

  5. Explorers Presentation: Explaining the Tides to Children

    OpenAIRE

    Institute, Marine

    2015-01-01

    Explaining the tides to children Presentation includes information about: Orbits of the Earth, Moon and Sun; Moon phases and the lunar cycle; Gravity; Gravity and the tide; Types of tides; The tides and me!; Tide tables; Extra insight

  6. Clinico-Pathologic Presentation and Management of ...

    African Journals Online (AJOL)

    Von Recklinghausen's) Disease among North-Eastern Nigerians: A six year review. ... presentation and to highlight the need of multi disciplinary approach in the management of this condition. Methodology: The case records of patients who ...

  7. Presentations: Adverse Outcome Pathways for Abnormal Phenotypes

    Science.gov (United States)

    Birth defects affect many infants and the etiology for most are unknown. Although environmental factors are known to influence pregnancy outcome, thousands of chemicals, present in the environment, are untested for developmental toxicity potential. Application of computational p...

  8. Colorectal cancer presenting as bone metastasis

    Directory of Open Access Journals (Sweden)

    M C Suresh Babu

    2017-01-01

    Conclusions: In this study, the patients of colorectal cancer presenting with bone metastasis were of male sex and younger age. The factors that were associated with reduced survival were extraosseous and liver involvement.

  9. 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

  10. Endodontic microsurgery, presentation of a clinical case

    International Nuclear Information System (INIS)

    Zeledon Mayorga, Rodolfo

    2009-01-01

    A literature review is conducted on endodontic surgery. The report of a clinical case is facilitated. The technique chosen according to the clinical and radiographic examination was endodontic microsurgery, the case has presented a positive evolution of four years [es

  11. 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.

  12. Nanotechnology in sustainable agriculture: Present concerns and ...

    African Journals Online (AJOL)

    Nanotechnology in sustainable agriculture: Present concerns and future aspects. ... of those living in developing countries face daily food shortages as a result of ... applications in agricultural, food, and water safety that could have significant ...

  13. Techno-Economics & Life Cycle Assessment (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Dutta, A.; Davis, R.

    2011-12-01

    This presentation provides an overview of the techno-economic analysis (TEA) and life cycle assessment (LCA) capabilities at the National Renewable Energy Laboratory (NREL) and describes the value of working with NREL on TEA and LCA.

  14. 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.

  15. 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.

  16. Present status of radiation processing in Japan

    International Nuclear Information System (INIS)

    Tabata, Y.

    1984-01-01

    Crosslinking of insulating materials including cables, tubes, sheets, pipes and polymer foam, curing of coating, surface treatment and other processes developed recently will be presented. Future prospects in this field will be discussed. (Author) [pt

  17. Cloud Based Applications and Platforms (Presentation)

    Energy Technology Data Exchange (ETDEWEB)

    Brodt-Giles, D.

    2014-05-15

    Presentation to the Cloud Computing East 2014 Conference, where we are highlighting our cloud computing strategy, describing the platforms on the cloud (including Smartgrid.gov), and defining our process for implementing cloud based applications.

  18. Abnormal Position and Presentation of the Fetus

    Science.gov (United States)

    ... Delivery Introduction to Complications of Labor and Delivery Abnormal Position and Presentation of the Fetus Amniotic Fluid Embolism Excessive Uterine Bleeding at Delivery Fetal Distress Inverted Uterus Labor That ...

  19. 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.

  20. 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.

  1. 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.

  2. NEONATAL TERATOMA PRESENTING AS HYGROMA-COLLI

    NARCIS (Netherlands)

    JAARSMA, AS; TAMMINGA, RYJ; DELANGEN, ZJ; NIKKELS, PGJ; KIMPEN, JLL

    We describe a neonate with a large tumour involving cranial, cervical and upper mediastinal regions, which presented clinically as hygroma colli. Radiological and pathological investigations showed characteristics of a mature teratoma and prominent cystic components within the tumour. These findings

  3. Present status and physics prospects of SSC

    International Nuclear Information System (INIS)

    Sugimoto, Shojiro

    1990-01-01

    Physics prospects of Superconducting Super Collider (SSC) are discussed, referring to other coming colliders. In addition, the present status of the SSC project and detector proposals for the experiments are described. (author)

  4. Subdural Empyema: Clinical Presentations and Management ...

    African Journals Online (AJOL)

    [craniotomy vs. burr hole, aspiration, and drainage. (BAAD)] depends on the ... the dura and the subarachnoid mater within the brain ambient ... presentations. Posterior fossa right-sided SDE was seen ... the left side of the brain, 15 (83.3%).

  5. Clinical Presentation of Pediatric Myocarditis in Taiwan

    Directory of Open Access Journals (Sweden)

    Hsiang-Ju Hsiao

    2011-06-01

    Conclusions: Pediatric myocarditis presents primarily with gastrointestinal symptoms in Taiwan. Careful check of heart rhythm may provide a useful objective marker of myocarditis. The predictors of a poor prognosis were gastrointestinal symptoms, hepatomegaly, and hypotension.

  6. Human-machine Interface for Presentation Robot

    Czech Academy of Sciences Publication Activity Database

    Krejsa, Jiří; Ondroušek, V.

    2012-01-01

    Roč. 6, č. 2 (2012), s. 17-21 ISSN 1897-8649 Institutional research plan: CEZ:AV0Z20760514 Keywords : human-robot interface * mobile robot * presentation robot Subject RIV: JD - Computer Applications, Robotics

  7. 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.

  8. An unusual presentation of a glomus tumour.

    LENUS (Irish Health Repository)

    Nugent, N

    2011-02-01

    Glomus tumours are benign, soft tissue tumours, usually of fingertips. Classically they present with severe pain, temperature sensitivity and localised tenderness. The diagnosis is often delayed due to sometimes non-specific symptoms and rarity of the disorder. While usually a clinical diagnosis, imaging may be necessary for diagnosis and localisation. We present a case of glomus tumour of the fingertip with an unusual history.

  9. Waardenburg syndrome presenting with constipation since birth.

    Science.gov (United States)

    Gupta, R; Sharma, S B; Mathur, P; Agrawal, L D

    2014-12-01

    Shah-Waardenburg syndrome is Waardenburg syndrome associated with Hirschsprung's disease. A 10-day-old full-term male neonate of Waardenburg syndrome presented with constipation since birth along with features of small bowel obstruction. Exploratory laparotomy revealed distended proximal jejunal and ileal loops along with microcolon; an ileostomy was performed. Postoperatively patient developed sepsis and died. Histopathology confirmed total colonic aganglionosis. Suspect familial Shah-Waardenburg syndrome in a neonate of Waardenburg syndrome presenting with constipation since birth or intestinal obstruction.

  10. Digital radiography: Present detectors and future developments

    International Nuclear Information System (INIS)

    Perez-Mendez, V.

    1990-08-01

    Present detectors for digital radiography are of two classes: real time detectors and storage (non real time) types. Present real time detectors consist of image intensifier tubes with an internal cesium iodide layer x-ray converter. Non real time detectors involve linear sweep arrays or storage detectors such as film. Future detectors discussed here can be of both types utilizing new technologies such as hydrogenated amorphous silicon photodiode arrays coupled to thin film transistor arrays. 17 refs., 10 figs

  11. International astronomical remote present observation on IRC.

    Science.gov (United States)

    Ji, Kaifan; Cao, Wenda; Song, Qian

    On March 6 - 7, 1997, an international astronomical remote present observation (RPO) was made on Internet Relay Chat (IRC) for the first time. Seven groups in four countries, China, United States, Canada and Great Britain, used the 1 meter telescope of Yunnan observatory together by the way of remote present observation. Within minutes, images were "On-line" by FTP, and every one was able to get them by anonymous ftp and discuss them on IRC from different widely separated sites.

  12. 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

  13. A Dementia Case Presenting with Psychotic Symptoms

    Directory of Open Access Journals (Sweden)

    Osman Ozdemir

    2013-06-01

    Full Text Available Dementia is a progressive clinical syndrome in which affected areas of brain function may be affected, such as memory, language, abstract thinking, problem solving and attention. Psychotic symptoms include auditory and visual hallucinations and delusions, which usually occur in the dementia. In this paper, a dementia case presenting with psychotic symptoms is presented. [Cukurova Med J 2013; 38(3.000: 482-486

  14. Invited presentations. College on soil physics 2003

    International Nuclear Information System (INIS)

    Gabriels, D.M.; Ghirardi, G.; Nielsen, D.R.; Pla Sentis, I.; Skidmore, E.L.

    2004-01-01

    The present book is a partial compilation of contributions from selected former participants of the College on Soil Physics invited to make presentations related to their achievements as a result of attending the College. It also serves as a testimony of the existing links between soil physicists throughout the world strengthened by the support and programs of the International Centre for Theoretical Physics originally envisioned by Abdus Salam to foster the growth of advanced studies and physics research in developing countries

  15. Invited presentations. College on soil physics 2003

    Energy Technology Data Exchange (ETDEWEB)

    Gabriels, D M [Univ. Ghent (Belgium); Ghirardi, G [Univ. Trieste (Italy); Nielsen, D R [Univ. California (United States); Pla Sentis, I [Univ. Lleida (Spain); Skidmore, E L [Kansas State Univ. (United States)

    2004-05-15

    The present book is a partial compilation of contributions from selected former participants of the College on Soil Physics invited to make presentations related to their achievements as a result of attending the College. It also serves as a testimony of the existing links between soil physicists throughout the world strengthened by the support and programs of the International Centre for Theoretical Physics originally envisioned by Abdus Salam to foster the growth of advanced studies and physics research in developing countries.

  16. 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. 

  17. 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\

  18. Computer Presentation Programs and Teaching Research Methodologies

    OpenAIRE

    Motamedi, Vahid

    2015-01-01

    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 pres...

  19. 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.

  20. De morseir syndrome presenting as ambiguous genitalia

    OpenAIRE

    Anubhav Thukral; S Chitra; Partho P Chakraborty; Ajitesh Roy; Soumik Goswami; Rana Bhattacharjee; Deep Dutta; Indira Maisnam; Sujoy Ghosh; Satinath Mukherjee; Subhankar Chowdhury

    2012-01-01

    Background: A 10-year-old boy presented with genital ambiguity, poor linear growth, and delayed milestones. The aim and to highlight that although rare but congenital, hypogonadotropic hypogonadism may rarely present as ambiguity. Materials and Methods: The patient was found to have bilateral cryptorchidism with proximal penile hypospadias, microphallus with a proportionate dwarfism with mildly delayed bone age, and karyotype 46XY. Euthyroid with normal steroid axis, growth hormone insufficie...

  1. 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

  2. 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)

  3. 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

  4. 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.

  5. 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.

  6. Pathogenesis of germ cell neoplasia in testicular dysgenesis and disorders of sex development

    DEFF Research Database (Denmark)

    Jørgensen, Anne; Lindhardt Johansen, Marie; Juul, Anders

    2015-01-01

    in individuals with 46,XY DSD. We summarise knowledge concerning development and sex differentiation of human gonads, with focus on sex-dimorphic steps of germ cell maturation, including meiosis. We also briefly outline the histopathology of germ cell neoplasia in situ (GCNIS) and gonadoblastoma (GDB), which......Development of human gonads is a sex-dimorphic process which evolved to produce sex-specific types of germ cells. The process of gonadal sex differentiation is directed by the action of the somatic cells and ultimately results in germ cells differentiating to become functional gametes through...

  7. Tata Laksana Kasus Perempuan dengan Pure Gonadal Dysgenesis 46, XY (Sindrom Swyer)

    OpenAIRE

    Kanadi Sumapradja; Mila Maidarti; Achmad K. Harzif; Budi Wiweko; Gita Pratama; Muharam Natadisastra; Andon Hestiantoro

    2016-01-01

    Disorders of sex development (DSD) adalah kelainan medis yang dikaitkan dengan ketidakcocokan antara kromosom, gonad dan fenotip. Sindrom Swyer adalah kelainan berupa disgenesis gonad 46,XY dan diikuti dengan tidak terjadinya pelepasan anti-mullerian hormone (AMH) dari gonad yang mengalami disgenesis sehingga mengakibatkan berkembangnya duktus muller menjadi uterus. Keadaan tersebut mengakibatkan pasien mengeluh amenorea primer dan organ seks sekunder tidak berkembang. Orientasi gender umumn...

  8. Environmental influences on ovarian dysgenesis - developmental windows sensitive to chemical exposures

    DEFF Research Database (Denmark)

    Johansson, Hanna Katarina Lilith; Svingen, Terje; Fowler, Paul A

    2017-01-01

    A woman's reproductive health and ability to have children directly affect numerous aspects of her life, from personal well-being and socioeconomic standing, to morbidity and lifespan. In turn, reproductive health depends on the development of correctly functioning ovaries, a process that starts ...

  9. Cryptorchidism and hypospadias as a sign of testicular dysgenesis syndrome (TDS): environmental connection

    DEFF Research Database (Denmark)

    Toppari, Jorma; Virtanen, Helena E; Main, Katharina M

    2010-01-01

    Cryptorchidism and hypospadias are common genital birth defects that affect 2-9% and 0.2-1% of male newborns, respectively. The incidence of both defects shows large geographic variation, and in several countries increasing trends have been reported. The conditions share many risk factors, and th...

  10. Spectrum of mutations in the renin-angiotensin system genes in autosomal recessive renal tubular dysgenesis

    DEFF Research Database (Denmark)

    Gribouval, Olivier; Morinière, Vincent; Pawtowski, Audrey

    2012-01-01

    , pulmonary hypoplasia, and refractory arterial hypotension. The disease is linked to mutations in the genes encoding several components of the renin-angiotensin system (RAS): AGT (angiotensinogen), REN (renin), ACE (angiotensin-converting enzyme), and AGTR1 (angiotensin II receptor type 1). Here, we review...... the series of 54 distinct mutations identified in 48 unrelated families. Most of them are novel and ACE mutations are the most frequent, observed in two-thirds of families (64.6%). The severity of the clinical course was similar whatever the mutated gene, which underlines the importance of a functional RAS...

  11. Cryptorchidism and hypospadias as a sign of testicular dysgenesis syndrome (TDS): environmental connection

    DEFF Research Database (Denmark)

    Toppari, Jorma; Virtanen, Helena E; Main, Katharina M

    2010-01-01

    . A monogenic reason for cryptorchidism or hypospadias has been identified only in a small proportion of all cases. Environmental effects appear to play a major role in TDS. Exposure to several persistent chemicals has been found to be associated with the risk of cryptorchidism, and exposure to anti......-androgenic phthalates has been shown to be associated with hormonal changes predisposing to male reproductive problems. Despite progress in identification of endocrine-disrupting substances, we are still far from knowing all the risk factors for these birth defects, and advice for prevention must be based......Cryptorchidism and hypospadias are common genital birth defects that affect 2-9% and 0.2-1% of male newborns, respectively. The incidence of both defects shows large geographic variation, and in several countries increasing trends have been reported. The conditions share many risk factors...

  12. Anterior segment dysgenesis (Peters' anomaly) in two snow leopard (Panthera uncia) cubs

    DEFF Research Database (Denmark)

    Hamoudi, Hassan; Rudnick, Jens-Christian; Prause, Jan Ulrik

    2013-01-01

    remnant of the hyaloid artery. The male had hydrocephalus and thus some of the features of Peters' plus syndrome (Peters' anomaly in addition to systemic malformations). The histological findings in the eyes of these snow leopard siblings are identical with those described in humans with Peters' anomaly....

  13. Gonadal Dysgenesis: Report of a Middle-Aged Patient with Un ...

    African Journals Online (AJOL)

    1971-12-11

    Dec 11, 1971 ... The extreme delay of epiphyseal maturation encountered in this case is thought to be the longest yet recorded in the literature. The finding supports the contention that delayed epiphyseal maturation is always to be found in gonadal .... The remain- ing 20% with 'female' pattern do not, as a rule, show.

  14. Experimentally induced testicular dysgenesis syndrome originates in the masculinization programming window

    DEFF Research Database (Denmark)

    van den Driesche, Sander; Kilcoyne, Karen R; Wagner, Ida

    2017-01-01

    suppresses testosterone production by the fetal testis. We evaluated if induction of TDS via testosterone suppression is restricted to the "masculinization programming window" (MPW), as indicated by reduction in anogenital distance (AGD). We show that DBP suppresses fetal testosterone equally during...

  15. Testicular dysgenesis syndrome comprises some but not all cases of hypospadias and impaired spermatogenesis

    DEFF Research Database (Denmark)

    Jørgensen, N; Rajpert-De Meyts, Ewa; Main, K M

    2010-01-01

    , but in the majority of men with impaired semen quality as the only symptom, an association with TDS is less clear. Such cases have a very heterogeneous aetiology and may be caused by a host of other - often post-natal-factors. In conclusion, the TDS as a holistic concept has inspired new research activities and led...

  16. Genetics Home Reference: sudden infant death with dysgenesis of the testes syndrome

    Science.gov (United States)

    ... 1 is involved in the development of the male reproductive system and the brain. The TSPYL1 gene mutation that ... disorder by disrupting the normal development of the male reproductive system and the brain, particularly the brainstem. Research findings ...

  17. Olfactory bulb dysgenesis, mirror neuron system dysfunction, and autonomic dysregulation as the neural basis for autism.

    Science.gov (United States)

    Brang, David; Ramachandran, V S

    2010-05-01

    Autism is a disorder characterized by social withdrawal, impoverished language and empathy, and a profound inability to adopt another's viewpoint - a failure to construct a "theory of mind" for interpreting another person's thoughts and intentions. We previously showed that these symptoms might be explained, in part, by a paucity of mirror neurons. Prompted by an MRI report of an individual with autism, we now suggest that there may be, in addition, a congenital aplasia/dysplasia of the olfactory bulbs with consequent reduction of vasopressin and oxytocin receptor binding. There may also be sub-clinical temporal lobe epilepsy affecting the recently discovered third visual system that is rich in "empathy" related mirror neurons (MNS) and projects (via the TOP junction - just below the inferior parietal lobule) to limbic structures that regulate autonomic outflow. This causes deranged autonomic feedback, resulting in additional deficiencies in MNS with loss of emotional empathy and introspection.

  18. Testicular dysgenesis syndrome and the origin of carcinoma in situ testis

    DEFF Research Database (Denmark)

    Sonne, Si Brask; Kristensen, David Møbjerg; Novotny, Guy W

    2008-01-01

    foetus, but the genetic background may also play a role. The morphological similarity of carcinoma in situ (CIS) cells (the precursor of the majority of invasive testicular cancers) with primordial germ cells and gonocytes, and overlap in expression of protein markers suggests an origin of CIS from...

  19. 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

  20. 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)