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Sample records for becker thilo pionteck

  1. Peter Becker and his Nazi past: the man behind Becker muscular dystrophy and Becker myotonia.

    Science.gov (United States)

    Zeidman, Lawrence A; Kondziella, Daniel

    2014-04-01

    Peter Becker was a German neurologist who helped classify the muscular dystrophies, and described Becker muscular dystrophy and Becker myotonia. His involvement in National Socialism began in 1933, when he was compelled by his peers to join the SA (brown shirts). He later joined the Nazi party, the Nazi Doctors Association, and the Nazi Lecturers' Association. He renewed his SA membership to maintain his position at a genetics institute. Colleagues stated postwar that he was not an active Nazi, and he was de-Nazified in 1947, able to continue his career. Later, Becker admitted to most, but not all, of his Nazi memberships in his autobiography, and wrote 2 books exploring the origins of Nazism and racial hygiene. The "neurologic court of opinion" must weigh in on how we should best remember Becker, and at the very least, we as neurologists must learn the dangers of career opportunism at any cost.

  2. Peter Becker and his Nazi past

    DEFF Research Database (Denmark)

    Zeidman, Lawrence A; Kondziella, Daniel

    2014-01-01

    Peter Becker was a German neurologist who helped classify the muscular dystrophies, and described Becker muscular dystrophy and Becker myotonia. His involvement in National Socialism began in 1933, when he was compelled by his peers to join the SA (brown shirts). He later joined the Nazi party, t...... cost....

  3. History's Purpose: Becker or Ortega?

    Science.gov (United States)

    Scudder, John R., Jr.; Gulick, Barbara

    1972-01-01

    The transition from a functionally pragmatic interpretation of history to a humanistic existential one is explored by comparing the philosophy of the eminent intellectual historian, Carl Becker, with that of Jose Ortega y Gasset, who interprets history from a humanistic point of view. (Author/SM)

  4. History's Purpose: Becker or Ortega?

    Science.gov (United States)

    Scudder, John R., Jr.; Gulick, Barbara

    1972-01-01

    The transition from a functionally pragmatic interpretation of history to a humanistic existential one is explored by comparing the philosophy of the eminent intellectual historian, Carl Becker, with that of Jose Ortega y Gasset, who interprets history from a humanistic point of view. (Author/SM)

  5. Becker-Blaschke problem of space

    Science.gov (United States)

    Bernard, Julien

    2015-11-01

    In a letter to Weyl, Becker proposed a new way to solve the problem of space in the relativistic context. This is the result of Becker's encounter with the two traditions of thinking about space: Husserlian transcendental phenomenology and Blaschke's equiaffine differential geometry. I reconstruct the mathematical content of the Becker-Blaschke solution to the problem of space and highlight the philosophical ideas that guide this construction. This permits me to underline some common properties of Riemannian and Minkowskian manifolds in terms of an unusual notion of isotropy. Finally, I will use this construction as a support to analyze several philosophical differences between Weyl's and Becker's proposals.

  6. A review of the genera Cleigastra Macquart, Gonarcticus Becker, Gonatherus Rondani, Hexamitocera Becker, Nanna Strobl, Orthacheta Becker and Spathephilus Becker (Diptera, Scathophagidae) of Russia.

    Science.gov (United States)

    Ozerov, A L; Krivosheina, M G

    2015-09-03

    Flies of the genera Cleigastra Macquart, 1835, Gonarcticus Becker, 1894, Gonatherus Rondani, 1856, Hexamitocera Becker, 1894, Nanna Strobl, 1894, Orthacheta Becker, 1894 and Spathephilus Becker, 1894 (all Scathophagidae) of the fauna of Russia are reviewed. Key to genera, generic descriptions and keys for determination of species are given, and data on geographical distribution are summarized. One species, Nanna cryophila sp. nov., is described as new to science. One new synonymy is proposed: Nanna kamtschatkense (Hendel, 1930) = Nanna tibiella (Zetterstedt, 1838). Orthacheta cornuta (Loew, 1863) is recorded from Europe for the first time. Gonarcticus arcticus (Becker, 1907) is newly recorded from the Palaearctic Region and Russia. Additionally, Nanna flavipes (Fallén, 1819) is newly recorded from China, and Spathephilus nigriventris (Loew, 1864) is newly recorded from Kazakhstan.

  7. Cardiomyopathy in becker muscular dystrophy:Overview

    Institute of Scientific and Technical Information of China (English)

    Rady Ho; My-Le Nguyen; Paul Mather

    2016-01-01

    Becker muscular dystrophy(BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

  8. Rafał Becker: psychiatrist, eugenist, Zionist.

    Science.gov (United States)

    Marcinowski, Filip; Nasierowski, Tadeusz

    2016-01-01

    In the interwar period the eugenic ideas gained the status of a scientific theory and become attractive to a wide range of physicians. Among them were doctors of Jewish origin who perceived eugenics as a tool in the fight for biological rebirth of the Jewish nation. Polish-Jewish psychiatrist Raphael Becker (1891-1939?), the author of dozens of scientific papers, was the most famous eugenist among Jewish psychiatrists, not only in Poland but also in Europe. After graduation in medicine at the University in Zurich and training in the psychiatry clinic Burghölzli under the guidance of Eugen Bleuler, Rafał Becker became interested in the question of epidemiology of mental disorders among the Jews. In the interwar period, dealing with the statistics of mental disorders among Polish Jews, and directing a psychiatric hospital "Zofiówka" in Otwock, he significantly contributed to the development of medical care for the mentally ill Jews in Poland. Becker's scientific ideas were greatly influenced by the work of Alfred Adler and Ernst Kretschmer. The article presents the life and scientific achievements of Becker, with particular emphasis on his views on eugenics.

  9. Rafał Becker: psychiatrist, eugenist, Zionist

    Directory of Open Access Journals (Sweden)

    Filip Marcinowski

    2016-02-01

    Full Text Available In the interwar period the eugenic ideas gained the status of a scientific theory and become attractive to a wide range of physicians. Among them were doctors of Jewish origin who perceived eugenics as a tool in the fight for biological rebirth of the Jewish nation. Polish-Jewish psychiatrist Raphael Becker (1891–1939?, the author of dozens of scientific papers, was the most famous eugenist among Jewish psychiatrists, not only in Poland but also in Europe. After graduation in medicine at the University in Zurich and training in the psychiatry clinic Burghölzli under the guidance of Eugen Bleuler, Rafał Becker became interested in the question of epidemiology of mental disorders among the Jews. In the interwar period, dealing with the statistics of mental disorders among Polish Jews, and directing a psychiatric hospital “Zofiówka” in Otwock, he significantly contributed to the development of medical care for the mentally ill Jews in Poland. Becker’s scientific ideas were greatly influenced by the work of Alfred Adler and Ernst Kretschmer. The article presents the life and scientific achievements of Becker, with particular emphasis on his views on eugenics.

  10. Dr. Peter Emil Becker and the Third Reich.

    Science.gov (United States)

    Hill, Frank

    2013-08-01

    In 1985 the physician after whom Becker Muscular Dystrophy is named, German neurologist Dr. Peter Emil Becker (1908-2000), published an autobiographical article in the American Journal of Medical Genetics in which he disavowed any association with the Nazi Party. A closer look at the evidence, however, suggests otherwise. Review of war records and related sources raise concern for Dr. Becker's affiliation with the Nazi Party and his contributions to its ideology.

  11. Do you know this syndrome? Becker nevus syndrome.

    Science.gov (United States)

    Steiner, Denise; Silva, Fernanda Ayres de Morais e; Pessanha, André César Antiori Freire; Bialeski, Nediana; Feola, Camila; Buzzoni, Carla Arantes Bertolucci

    2011-01-01

    Becker nevus syndrome is the association of Becker's nevus with breast hypoplasia and other ipsilateral bone or muscle changes. It is considered to be a hormone-dependent disorder caused by a disturbance in the activity of the androgen receptor that appears to be increased in Becker's nevus, which may influence the development of associated lesions. We present a relevant case of this syndrome due to the rare systematization of the lesions in addition to the exuberant extracutaneous involvement in this case.

  12. Becker and Lomnitz rheological models: A comparison

    Science.gov (United States)

    Mainardi, Francesco; Spada, Giorgio

    2012-07-01

    The viscoelastic material functions for the Becker and the Lomnitz rheological models, sometimes employed to describe the transient flow of rocks, are studied and compared. Their creep functions, which are known in a closed form, share a similar time dependence and asymptotic behavior. This is also found for the relaxation functions, obtained by solving numerically a Volterra equation of the second kind. We show that the two rheologies constitute a clear example of broadly similar creep and relaxation patterns associated with neatly distinct retardation spectra, for which analytical expressions are available.

  13. Genetics Home Reference: Duchenne and Becker muscular dystrophy

    Science.gov (United States)

    ... Research Institute National Institute of Neurological Disorders and Stroke Educational Resources (13 links) Centers for Disease Control and Prevention Centre for Genetics Education Cleveland Clinic: Muscular Dystrophy Disease InfoSearch: Becker ...

  14. Soul / Randy Becker, Bill Evans, Evelin Samuel ; interv. Sven Sapelson

    Index Scriptorium Estoniae

    Becker, Randy

    2007-01-01

    The Soulbop Bandi rajajad Randy Becker ja Bill Evans ning meie Evelin Samuel gospeli ja rhytmblues'i juurtega soulist. Lk. 72-73 Inessa Josingu ja Ande Kaalepi souliteemaline fotolavastusega ruumikujundus

  15. Becker nevus with vitiligo and lichen planus: Cocktail of dermatoses

    Directory of Open Access Journals (Sweden)

    Sanjeev Gupta

    2010-07-01

    Full Text Available Context: Becker nevus has been reported to be associated with lichen planus (LP in isolated case reports in past. The association of LP and vitiligo has been noted in few cases and has been attributed to a common autoimmune etiology. The coexistence of lichen planus, vitiligo and Becker nevus has not been reported so far. Case Report: A thirty five years old male presented with lesions of Becker nevus along with vitiligo and Lichen planus coexisting at one place on right side of the chest. Vitiligo and Becker nevus could not be treated. Lichen planus was confirmed histopathologically. We were able to treat lichen planus with topical potent steroids, tacrolimus and systemic antihistamines. The vitiligo lesion in our case was resistant to treatment. Conclusion: This case is being reported for the rare occurrence of three different well defined skin conditions in our patient and reviews the possible known etiological factors for their coexistence.

  16. Soul / Randy Becker, Bill Evans, Evelin Samuel ; interv. Sven Sapelson

    Index Scriptorium Estoniae

    Becker, Randy

    2007-01-01

    The Soulbop Bandi rajajad Randy Becker ja Bill Evans ning meie Evelin Samuel gospeli ja rhytmblues'i juurtega soulist. Lk. 72-73 Inessa Josingu ja Ande Kaalepi souliteemaline fotolavastusega ruumikujundus

  17. Parcels and Land Ownership, Published in 2008, Becker County Government.

    Data.gov (United States)

    NSGIC GIS Inventory (aka Ramona) — This Parcels and Land Ownership dataset as of 2008. The extent of these data is generally Becker County, MN. This metadata was auto-generated through the Ramona GIS...

  18. Valley sign in Becker muscular dystrophy and outliers of Duchenne and Becker muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Pradhan Sunil

    2004-04-01

    Full Text Available Valley sign has been described in patients with Duchenne muscular dystrophy (DMD. As there are genetic and clinical similarities between DMD and Becker muscular dystrophy (BMD, this clinical sign is evaluated in this study in BMD and DMD/BMD outliers. To evaluate the sign, 28 patients with Becker muscular dystrophy (BMD, 8 DMD/BMD outliers and 44 age-matched male controls with other neuromuscular diseases were studied. The sign was examined after asking patients to abduct their arms to about 90ºwith hands directed upwards; the muscle bulk over the back of the shoulders was observed. The sign was considered positive if the infraspinatus and deltoid muscles were enlarged and between these two muscles, the muscles forming the posterior axillary fold were wasted as if there were a valley between the two mounts. Twenty-five BMD patients and 7 DMD/BMD outliers had positive valley sign. However, it was less remarkable in comparison to DMD. It was absent in all the 44 controls. It was concluded that the presence of valley sign may help in differentiating BMD from other progressive neuromuscular disorders of that age group.

  19. Gary Becker and the economics of trafficking in human beings

    NARCIS (Netherlands)

    de Hert, Paul; Muraszkiewic, Julia

    2014-01-01

    Gary Becker died on 3 May 2014. He was an economist, Noble prize winner and author. He was declared by Milton Friedman as the “the greatest social scientist who has lived and worked in the last half century”. His work focused on economics and sociology, and through the lenses of these disciplines he

  20. Cardiac involvement in carriers of Duchenne and Becker muscular dystrophy

    NARCIS (Netherlands)

    Hoogerwaard, EM; van der Wouw, PA; Wilde, AAM; Bakker, E; Ippel, PF; Oosterwijk, JC; Majoor-Krakauer, DF; van Essen, AJ; Leschot, NJ; de Visser, M

    1999-01-01

    A cross-sectional study in a cohort of DNA proven carriers of Duchenne (DMD) and Becker (BMD) muscular dystrophy was undertaken with the following objectives: (1) to estimate the frequency of electrocardiographic (ECG) and echocardiographic abnormalities; (2) to establish the proportion of carriers

  1. Construct Validity of Measures of Becker's Side Bet Theory.

    Science.gov (United States)

    Shore, Lynn M.; Tetrick, Lois E.; Shore, Ted H.; Barksdale, Kevin

    2000-01-01

    Becker's side bet theory (remaining in a job because of perceived costs of leaving) was tested using data from 327 working business students. Three factors were most consistent with the theory: bureaucratic organization, nonwork-related concerns, and adjustment to social position. Attachment to the organization was significantly linked to tangible…

  2. [Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy].

    Science.gov (United States)

    Silva, Helga Cristina Almeida da; Hiray, Marcia; Vainzof, Mariz; Schmidt, Beny; Oliveira, Acary Souza Bulle; Amaral, José Luiz Gomes do

    2017-05-31

    Duchenne/Becker muscular dystrophy affects skeletal muscles and leads to progressive muscle weakness and risk of atypical anesthetic reactions following exposure to succinylcholine or halogenated agents. The aim of this report is to describe the investigation and diagnosis of a patient with Becker muscular dystrophy and review the care required in anesthesia. Male patient, 14 years old, referred for hyperCKemia (chronic increase of serum creatine kinase levels - CK), with CK values of 7,779-29,040IU.L(-1) (normal 174IU.L(-1)). He presented with a discrete delay in motor milestones acquisition (sitting at 9 months, walking at 18 months). He had a history of liver transplantation. In the neurological examination, the patient showed difficulty in walking on one's heels, myopathic sign (hands supported on the thighs to stand), high arched palate, calf hypertrophy, winged scapulae, global muscle hypotonia and arreflexia. Spirometry showed mild restrictive respiratory insufficiency (forced vital capacity: 77% of predicted). The in vitro muscle contracture test in response to halothane and caffeine was normal. Muscular dystrophy analysis by Western blot showed reduced dystrophin (20% of normal) for both antibodies (C and N-terminal), allowing the diagnosis of Becker muscular dystrophy. On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia. Copyright © 2017 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

  3. Rational choice theory and Becker's model of random behavior

    Directory of Open Access Journals (Sweden)

    Krstić Miloš

    2015-01-01

    Full Text Available According to rational choice theory, rational consumers tend to maximize utility under a given budget constraints. This will be achieved if they choose a combination of goods that can satisfy their needs and provide the maximum level of utility. Gary Becker, on the other hand, imagines irrational consumers who choose bundle on the budget line. As irrational consumers have an equal probability of choosing any bundle on the budget line, on average, we expect that they will pick the bundle lying at the midpoint of the line. The results of research in which artificial Becker's agents choose among more than two commodities, rational choice theory is small and more than two budget/price situations show that the percentage of agents whose behavior violate. Adding some factors to Becker's model of random behavior, experimenters can minimize these minor violations. Therefore, rational choice theory is unfalsifiable. The results of our research have confirmed this theory. In addition, in the paper we discussed about explanatory value of rational choice theory in specific circumstances (positive substitution effect and we concluded that the explanatory value of rational choice theory was significantly reduced in specific cases.

  4. 在路上--记Jason Becker(下)

    Institute of Scientific and Technical Information of China (English)

    孔祥龙

    2003-01-01

    @@ 在上期我们向大家介绍了Jason Becker的大致人生经历,而许多人更加关心他现在的生活情况.因此,在最近我们对他做了一次专访,我们用E-mail向他提问,然后他把要说的用他的方式来传达给他的母亲--Pat Becker.如下就是我们从他那里得知的事情:

  5. Resistance training in patients with limb-girdle and becker muscular dystrophies

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Andersen, Søren P; Ingelsrud, Lina H;

    2013-01-01

    In this study we investigated the effect of strength training in patients with limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD).......In this study we investigated the effect of strength training in patients with limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD)....

  6. The NCSS Curriculum Standards: A Response to Anna Ochoa-Becker.

    Science.gov (United States)

    Adler, Susan

    2001-01-01

    Provides a response to the April 2001 "Social Education" article, "A Critique of the NCSS Curriculum Standards," (Anna Ochoa-Becker). Focuses on the critiques offered by Ochoa-Becker that address the lack of time given to certain issues. (CMK)

  7. 77 FR 33235 - Notice of Realty Action: Modified Competitive Sale of Public Lands in Becker County, MN

    Science.gov (United States)

    2012-06-05

    ... Bureau of Land Management Notice of Realty Action: Modified Competitive Sale of Public Lands in Becker... parcels of public land totaling 1 acre in Becker County, Minnesota. The sale will be subject to the... area described contains 0.50 acres in Becker County, and is proposed for sale to the...

  8. Measuring Disease Severity in Duchenne and Becker Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Melinda F. Davis

    2010-10-01

    Full Text Available Medical investigations use a wide variety of outcome indicators that are often not comparable. It can be challenging to integrate results across multiple studies that do not share a common metric. Some conditions such as Duchenne and Becker muscular dystrophy have a predictable course of disease progression. Severity can be inferred from a patient's medical history. This paper describes the development of a disease severity measure using common markers of disease progression. Rasch modeling was used to estimate severity using dichotomous events that indicate disease progression. Caregivers of 34 young men with Duchenne or Becker muscular dystrophy completed structured interviews about their care and medical history. Interview questions included surgeries (tendon release, scoliosis, tracheostomy, respiratory equipment (assisted ventilation, cough assist devices, and the use of other medical equipment (e.g., braces, walkers, wheelchairs, transfer boards, hospital beds. The resulting measure had a reliability of .83. The correlation between the severity measure and the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS was .68. Preliminary results and item calibrations are provided for the severity measure that can be estimated from caregiver reports or administrative data. DOI: 10.2458/azu_jmmss.v1i1.76

  9. Rimmed vacuoles in Becker muscular dystrophy have similar features with inclusion myopathies.

    Directory of Open Access Journals (Sweden)

    Kazunari Momma

    Full Text Available Rimmed vacuoles in myofibers are thought to be due to the accumulation of autophagic vacuoles, and can be characteristic in certain myopathies with protein inclusions in myofibers. In this study, we performed a detailed clinical, molecular, and pathological characterization of Becker muscular dystrophy patients who have rimmed vacuoles in muscles. Among 65 Becker muscular dystrophy patients, we identified 12 patients who have rimmed vacuoles and 11 patients who have deletions in exons 45-48 in DMD gene. All patients having rimmed vacuoles showed milder clinical features compared to those without rimmed vacuoles. Interestingly, the rimmed vacuoles in Becker muscular dystrophy muscles seem to represent autophagic vacuoles and are also associated with polyubiquitinated protein aggregates. These findings support the notion that rimmed vacuoles can appear in Becker muscular dystrophy, and may be related to the chronic changes in muscle pathology induced by certain mutations in the DMD gene.

  10. Rimmed vacuoles in Becker muscular dystrophy have similar features with inclusion myopathies.

    Science.gov (United States)

    Momma, Kazunari; Noguchi, Satoru; Malicdan, May Christine V; Hayashi, Yukiko K; Minami, Narihiro; Kamakura, Keiko; Nonaka, Ikuya; Nishino, Ichizo

    2012-01-01

    Rimmed vacuoles in myofibers are thought to be due to the accumulation of autophagic vacuoles, and can be characteristic in certain myopathies with protein inclusions in myofibers. In this study, we performed a detailed clinical, molecular, and pathological characterization of Becker muscular dystrophy patients who have rimmed vacuoles in muscles. Among 65 Becker muscular dystrophy patients, we identified 12 patients who have rimmed vacuoles and 11 patients who have deletions in exons 45-48 in DMD gene. All patients having rimmed vacuoles showed milder clinical features compared to those without rimmed vacuoles. Interestingly, the rimmed vacuoles in Becker muscular dystrophy muscles seem to represent autophagic vacuoles and are also associated with polyubiquitinated protein aggregates. These findings support the notion that rimmed vacuoles can appear in Becker muscular dystrophy, and may be related to the chronic changes in muscle pathology induced by certain mutations in the DMD gene.

  11. Functional muscle ischemia in Duchenne and Becker muscular dystrophy

    Directory of Open Access Journals (Sweden)

    Gail D Thomas

    2013-12-01

    Full Text Available Duchenne and Becker muscular dystrophy (DMD/BMD comprise a spectrum of devastating X-linked muscle wasting disease for which there is no treatment. DMD/BMD is caused by mutations in the gene encoding dystrophin, a cytoskeletal protein that stabilizes the muscle membrane and also targets other proteins to the sarcolemma. Among these is the muscle-specific isoform of neuronal nitric oxide synthase (nNOSµ which binds spectrin-like repeats within dystrophin’s rod domain and the adaptor protein α-syntrophin. Dystrophin deficiency causes loss of sarcolemmal nNOSµ and reduces paracrine signaling of muscle-derived nitric oxide (NO to the microvasculature, which renders the diseased muscle fibers susceptible to functional muscle ischemia during exercise. Repeated bouts of functional ischemia superimposed on muscle fibers already weakened by dystrophin deficiency result in use-dependent focal muscle injury. Genetic and pharmacologic strategies to boost nNOSµ-NO signaling in dystrophic muscle alleviate functional muscle ischemia and show promise as novel therapeutic interventions for the treatment of DMD/BMD.

  12. Dystrophin in frameshift deletion patients with Becker Muscular Dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Gangopadhyay, S.B.; Ray, P.N.; Worton, R.G.; Sherratt, T.G.; Heckmatt, J.Z.; Dubowitz, V.; Strong, P.N.; Miller, G. (Penn State College of Medicine, Hershey, PA (United States)); Shokeir, M. (Univ. Hospital, Saskatchewan (Canada))

    1992-09-01

    In a previous study the authors identified 14 cases with Duchenne muscular dystrophy (DMD) or its milder variant, Becker muscular dystrophy (BMD), with a deletion of exons 3-7, a deletion that would be expected to shift the translational reading frame of the mRNA and give a severe phenotype. They have examined dystrophin and its mRNA from muscle biopsies of seven cases with either mild or intermediate phenotypes. In all cases they detected slightly lower-molecular-weight dystrophin in 12%-15% abundance relative to the normal. By sequencing amplified mRNA they have found that exon 2 is spliced to exon 8, a splice that produces a frameshifted mRNA, and have found no evidence for alternate splicing that might be involved in restoration of dystrophin mRNA reading frame in the patients with a mild phenotype. Other transcriptional and posttranscriptional mechanisms such as cryptic promoter, ribosomal frameshifting, and reinitiation are suggested that might play some role in restoring the reading frame. 34 refs., 5 figs. 1 tab.

  13. Cardiac involvement in Duchenne and Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    Sophie; Mavrogeni; George; Markousis-Mavrogenis; Antigoni; Papavasiliou; Genovefa; Kolovou

    2015-01-01

    Duchenne and Becker muscular dystrophy(DMD/BMD) are X-linked muscular diseases responsible for over 80% of all muscular dystrophies. Cardiac disease is a common manifestation,not necessarily related to the degree of skeletal myopathy; it may be the predominant manifestation with or without any other evidence of muscular disease. Death is usually due to ventricular dysfunction,heart block or malignant arrhythmias. Not only DMD/BMD patients,but also female carriers may present cardiac involvement. Clinically overt heart failure in dystrophinopathies may be delayed or absent,due to relative physical inactivity. The commonest electrocardiographic findings include conduction defects,arrhythmias(supraventricular or ventricular),hypertrophy and evidence of myocardial necrosis. Echocardiography can assess a marked variability of left ventricular dysfunction,independently of age of onset or mutation groups. Cardiovascular magnetic resonance(CMR) has documented a pattern of epicardial fibrosis in both dystrophinopathies’ patients and carriers that can be observed even if overt muscular disease is absent. Recently,new CMR techniques,such as postcontrast myocardial T1 mapping,have been used in Duchenne muscular dystrophy to detect diffuse myocardial fibrosis. A combined approach using clinical assessment and CMR evaluation may motivate early cardioprotective treatment in both patients and asymptomatic carriers and delay the development of serious cardiac complications.

  14. Cannabis careers revisited: applying Howard S. Becker's theory to present-day cannabis use.

    Science.gov (United States)

    Järvinen, Margaretha; Ravn, Signe

    2014-01-01

    A considerable part of today's sociological research on recreational drug use is (explicitly or implicitly) inspired by Howard Becker's classical model of deviant careers. The aim of the present paper is to directly apply Becker's theory to empirical data on present-day cannabis use and to suggest a revision of the theory. As part of this, we propose a stretch of the sociological approach represented by Becker and followers in order to include, not only recreational drug use, but also use for which young people have sought treatment. The paper is based on 30 qualitative interviews with young people in treatment for cannabis problems in Copenhagen, Denmark. We suggest a revision of Becker's career model in relation to four aspects: initiation of cannabis use, differentiation between socially integrated and individualised, disintegrated use, social control from non-users, and the users' moral stance on cannabis. A central point of the paper is that social interaction may both motivate cannabis use, as Becker proposed, and serve as a protective factor against extensive, problematic use.

  15. Cardiac and pulmonary function variability in Duchenne/Becker muscular dystrophy: an initial report.

    Science.gov (United States)

    Birnkrant, David J; Ashwath, Mahi Lakshmi; Noritz, Garey H; Merrill, Michelle C; Shah, Tushar A; Crowe, Carol A; Bahler, Robert C

    2010-09-01

    The Duchenne and Becker forms of muscular dystrophy are associated with dilated cardiomyopathy and are diseases in which pulmonary function peaks and then progressively declines. In this report, the authors quantify cardiopulmonary function variability among brothers. Brothers in 3 of 7 eligible sibships had discordant pulmonary function, with significant differences between the brothers' peak forced vital capacities and their vital capacities at last comparable age. There was no relationship between pulmonary and cardiac function among the siblings. The authors concluded that despite identical genetic mutations, cardiac and pulmonary function variability was common among brothers in their clinic with Duchenne or Becker muscular dystrophy. If confirmed by larger studies, these results have negative implications for the use of genetic testing to predict cardiopulmonary course and response to therapies in Duchenne or Becker muscular dystrophy.

  16. Becker muscular dystrophy with widespread muscle hypertrophy and a non-sense mutation of exon 2

    DEFF Research Database (Denmark)

    Witting, Nanna; Duno, M; Vissing, J

    2013-01-01

    Becker muscular dystrophy features progressive proximal weakness, wasting and often focal hypertrophy. We present a patient with pain and cramps from adolescence. Widespread muscle hypertrophy, preserved muscle strength and a 10-20-fold raised CPK were noted. Muscle biopsy was dystrophic......, and Western blot showed a 95% reduction of dystrophin levels. Genetic analyses revealed a non-sense mutation in exon 2 of the dystrophin gene. This mutation is predicted to result in a Duchenne phenotype, but resulted in a mild Becker muscular dystrophy with widespread muscle hypertrophy. We suggest...

  17. Cardiac abnormalities in a follow-up study on carriers of Duchenne and Becker muscular dystrophy

    NARCIS (Netherlands)

    van Westrum, S. M. Schade; Hoogerwaard, E. M.; Dekker, L.; Standaar, T. S.; Bakker, E.; Ippel, P. F.; Oosterwijk, J. C.; Majoor-Krakauer, D. F.; van Essen, A. J.; Leschot, N. J.; Wilde, A. A. M.; de Haan, R. J.; de Visser, M.; van der Kooi, A. J.

    2011-01-01

    Objectives: Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated cardio

  18. The clinical and molecular genetic approach to Duchenne and Becker muscular dystrophy : an updated protocol

    NARCIS (Netherlands)

    vanEssen, AJ; Kneppers, ALJ; vanderHout, AH; Scheffer, H; Ginjaar, IB; tenKate, LP; vanOmmen, GJB; Buys, CHCM; Bakker, E

    1997-01-01

    Detection of large rearrangements in the dystrophin gene in Duchenne and Becker muscular dystrophy is possible in about 65-70% of patients by Southern blotting or multiplex PCR. Subsequently, carrier detection is possible by assessing the intensity of relevant bands, but preferably by a non-quantita

  19. Progression of cardiac involvement in patients with limb-girdle type 2 and Becker muscular dystrophies

    DEFF Research Database (Denmark)

    Petri, Helle; Sveen, Marie-Louise; Thune, Jens Jakob;

    2015-01-01

    AIM: To assess the degree and progression of cardiac involvement in patients with limb-girdle type 2 (LGMD2) and Becker muscular dystrophies (BMD). METHODS: A follow-up study of 100 LGMD2 (types A-L) and 30 BMD patients assessed by electrocardiogram (ECG) and echocardiography, supplemented...

  20. 75 FR 68377 - Tamarac National Wildlife Refuge, Becker County, and Tamarac Wetland Management District, Minnesota

    Science.gov (United States)

    2010-11-05

    ... Fish and Wildlife Service Tamarac National Wildlife Refuge, Becker County, and Tamarac Wetland... the Environmental Assessment (EA) for Tamarac National Wildlife Refuge (NWR) and Tamarac Wetland... 8,577 acres of wetland easements distributed throughout five counties. The Draft CCP and EA...

  1. 77 FR 52346 - Hamden Slough National Wildlife Refuge, Becker County, MN

    Science.gov (United States)

    2012-08-29

    ... From the Federal Register Online via the Government Publishing Office DEPARTMENT OF THE INTERIOR Fish and Wildlife Service Hamden Slough National Wildlife Refuge, Becker County, MN AGENCY: Fish and..., Detroit Lakes, MN 56501. In-Person Drop Off: You may drop off comments during regular business hours at...

  2. Community-engaged approaches to explore research priorities in Duchenne and Becker muscular dystrophy

    NARCIS (Netherlands)

    Peay, Holly Landrum

    2015-01-01

    This thesis presents a series of translational research studies to explore topics of importance to a patient stakeholder community--Duchenne and Becker muscular dystrophy. The overarching objective was to inform a patient/family foundation's interventions and policy and advocacy approaches. Results

  3. Diagnosis of becker muscular dystrophy : Results of Re-analysis of DNA samples

    NARCIS (Netherlands)

    Straathof, Chiara S M; Van Heusden, Dave; Ippel, Pieternella F.; Post, Jan G.; Voermans, Nicol C.; De Visser, Marianne; Brusse, Esther; Van Den Bergen, Janneke C.; Van Der Kooi, Anneke J.; Verschuuren, Jan J G M; Ginjaar, Hendrika B.

    2015-01-01

    Introduction: The phenotype of Becker muscular dystrophy (BMD) is highly variable, and the disease may be underdiagnosed. We searched for new mutations in the DMD gene in a cohort of previously undiagnosed patients who had been referred in the period 1985-1995. Methods: All requests for DNA analysis

  4. Clinical genetic aspects of Duchenne and Becker muscular dystrophy in the Netherlands

    NARCIS (Netherlands)

    Helderman-van den Enden, Apollonia Theodora Josina Maria

    2012-01-01

    Dystrophinopathies include the well known Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). This thesis is a collection of several clinical and genetic studies on dystrophinopathies with implications for genetic counselling of patients and their families and for future therapy (

  5. Studying the role of dystrophin-associated proteins in influencing Becker muscular dystrophy disease severity.

    Science.gov (United States)

    van den Bergen, J C; Wokke, B H A; Hulsker, M A; Verschuuren, J J G M; Aartsma-Rus, A M

    2015-03-01

    Becker muscular dystrophy is characterized by a variable disease course. Many factors have been implicated to contribute to this diversity, among which the expression of several components of the dystrophin associated glycoprotein complex. Together with dystrophin, most of these proteins anchor the muscle fiber cytoskeleton to the extracellular matrix, thus protecting the muscle from contraction induced injury, while nNOS is primarily involved in inducing vasodilation during muscle contraction, enabling adequate muscle oxygenation. In the current study, we investigated the role of three components of the dystrophin associated glycoprotein complex (beta-dystroglycan, gamma-sarcoglycan and nNOS) and the dystrophin homologue utrophin on disease severity in Becker patients. Strength measurements, data about disease course and fresh muscle biopsies of the anterior tibial muscle were obtained from 24 Becker patients aged 19 to 66. The designation of Becker muscular dystrophy in this study was based on the mutation and not on the clinical severity. Contrary to previous studies, we were unable to find a relationship between expression of nNOS, beta-dystroglycan and gamma-sarcoglycan at the sarcolemma and disease severity, as measured by muscle strength in five muscle groups and age at reaching several disease milestones. Unexpectedly, we found an inverse correlation between utrophin expression at the sarcolemma and age at reaching disease milestones.

  6. 78 FR 3910 - Hamden Slough National Wildlife Refuge, Becker County, MN; Final Comprehensive Conservation Plan...

    Science.gov (United States)

    2013-01-17

    ... Refuge, which we began by publishing a notice of intent in the Federal Register (75 FR 7289) on February... (77 FR 52346) on August 29, 2012. The 30-day comment period ended on September 28, 2012. A summary of... Fish and Wildlife Service Hamden Slough National Wildlife Refuge, Becker County, MN;...

  7. Young, Becker, and Pike's "Rogerian" Rhetoric: A Twenty-Year Reassessment.

    Science.gov (United States)

    Brent, Doug

    1991-01-01

    Concludes that the Rogerian rhetoric of Richard Young, Alton Becker, and Kenneth Pike (as presented in their book "Rhetoric: Discovery and Change") shows its age by not being quite the epistemic rhetoric contemporary rhetoricians have come to require. Argues that the Rogerian insights can still provide a focus for a reformed rhetoric of…

  8. Effect of sildenafil on skeletal and cardiac muscle in Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Witting, Nanna; Kruuse, Christina; Nyhuus, Bo

    2014-01-01

    OBJECTIVE: Patients with Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy lack neuronal nitric oxide synthase (nNOS). nNOS mediates physiological sympatholysis, thus ensuring adequate blood supply to working muscle. In mice lacking dystrophin, restoration of nNOS effects by a phosp...

  9. Duchenne and Becker muscular dystrophy: a molecular and immunohistochemical approach Distrofia muscular de Duchenne e Becker: abordagem molecular e imuno-histoquímica

    OpenAIRE

    Aline Andrade Freund; Rosana Herminia Scola; Raquel Cristina Arndt; Paulo José Lorenzoni; Claudia Kamoy Kay; Lineu Cesar Werneck

    2007-01-01

    Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are caused by mutations in the dystrophin gene. We studied 106 patients with a diagnosis of probable DMD/BMD by analyzing 20 exons of the dystrophin gene in their blood and, in some of the cases, by immunohistochemical assays for dystrophin in muscle biopsies. In 71.7% of the patients, deletions were found in at least one of the exons; 68% of these deletions were in the hot-spot 3' region. Deletions were found in 81.5% of t...

  10. Harberger-Laursen-Metzler Effect with Modified Becker-Mulligan Preference by Dynamic Optimization

    Directory of Open Access Journals (Sweden)

    Deng-Shan Wang

    2016-01-01

    Full Text Available We investigate the effects of terms-of-trade shocks on the spending and current account where households with the modified Becker-Mulligan endogenous time preference maximize their utility over an infinite planning period. Our results show that, with the modified Becker-Mulligan preference, the effect of the deterioration in terms of trade on the current account depends on people’s characters. However, with the second preference we have considered, the deterioration in terms of trade will result in a current account deficit, which is the same as Obstfeld (1982, where households with Uzawa endogenous time preference are considered; deterioration in terms of trade leads to a decline in the current account. These theoretical results are consistent with the empirical evidence by numerical simulations.

  11. Becker muscular dystrophy with widespread muscle hypertrophy and a non-sense mutation of exon 2.

    Science.gov (United States)

    Witting, N; Duno, M; Vissing, J

    2013-01-01

    Becker muscular dystrophy features progressive proximal weakness, wasting and often focal hypertrophy. We present a patient with pain and cramps from adolescence. Widespread muscle hypertrophy, preserved muscle strength and a 10-20-fold raised CPK were noted. Muscle biopsy was dystrophic, and Western blot showed a 95% reduction of dystrophin levels. Genetic analyses revealed a non-sense mutation in exon 2 of the dystrophin gene. This mutation is predicted to result in a Duchenne phenotype, but resulted in a mild Becker muscular dystrophy with widespread muscle hypertrophy. We suggest that this unusual phenotype is caused by translation re-initiation downstream from the mutation site. Copyright © 2012 Elsevier B.V. All rights reserved.

  12. Cardiac and Pulmonary Function Variability in Duchenne/Becker Muscular Dystrophy: An Initial Report

    OpenAIRE

    Birnkrant, David J.; Ashwath, Mahi Lakshmi; Noritz, Garey H.; Merrill, Michelle C.; Shah, Tushar A.; Crowe, Carol A.; Bahler, Robert C.

    2010-01-01

    The Duchenne and Becker forms of muscular dystrophy are associated with dilated cardiomyopathy and are diseases in which pulmonary function peaks, then progressively declines. In this report, we quantify cardiopulmonary function variability among brothers. Brothers in 3 of 7 eligible sibships had discordant pulmonary function, with significant differences between the brothers' peak forced vital capacities and their vital capacities at last comparable age. There was no relationship between pul...

  13. Complementary and alternative medicine for Duchenne and Becker muscular dystrophies: characteristics of users and caregivers.

    Science.gov (United States)

    Zhu, Yong; Romitti, Paul A; Conway, Kristin M; Andrews, Jennifer; Liu, Ke; Meaney, F John; Street, Natalie; Puzhankara, Soman; Druschel, Charlotte M; Matthews, Dennis J

    2014-07-01

    Complementary and alternative medicine is frequently used in the management of chronic pediatric diseases, but little is known about its use by those with Duchenne or Becker muscular dystrophy. Complementary and alternative medicine use by male patients with Duchenne or Becker muscular dystrophy and associations with characteristics of male patients and their caregivers were examined through interviews with 362 primary caregivers identified from the Muscular Dystrophy Surveillance, Tracking, and Research Network. Overall, 272 of the 362 (75.1%) primary caregivers reported that they had used any complementary and alternative medicine for the oldest Muscular Dystrophy Surveillance, Tracking, and Research Network male in their family. The most commonly reported therapies were from the mind-body medicine domain (61.0%) followed by those from the biologically based practice (39.2%), manipulative and body-based practice (29.3%), and whole medical system (6.9%) domains. Aquatherapy, prayer and/or blessing, special diet, and massage were the most frequently used therapies. Compared with nonusers, male patients who used any therapy were more likely to have an early onset of symptoms and use a wheel chair; their caregivers were more likely to be non-Hispanic white. Among domains, associations were observed with caregiver education and family income (mind-body medicines [excluding prayer and/or blessing only] and whole medical systems) and Muscular Dystrophy Surveillance, Tracking, and Research Network site (biologically based practices and mind-body medicines [excluding prayer and/or blessing only]). Complementary and alternative medicine use was common in the management of Duchenne and Becker muscular dystrophies among Muscular Dystrophy Surveillance, Tracking, and Research Network males. This widespread use suggests further study to evaluate the efficacy of integrating complementary and alternative medicine into treatment regimens for Duchenne and Becker muscular

  14. Competition and the Racial Wage Gap: Testing Becker's Model of Employer Discrimination

    OpenAIRE

    Hirata, Guilherme; Soares, Rodrigo R.

    2016-01-01

    According to Becker's (1957) theory of taste-based employer discrimination, pure economic rents are necessary for discrimination to be observed in the labor market. Increased competition and reduced rents in the market for final goods should therefore lead to reduced labor market discrimination. We look at the natural experiment represented by the Brazilian trade liberalization from the early 1990s to study the effect of increased competition in the market for final goods on racial discrimina...

  15. Growth Hormone Deficiency in a Patient with Becker Muscular Dystrophy: A Pediatric Case Report

    OpenAIRE

    Valeria Calcaterra; Annachiara Malvezzi; Rossana Toglia; Angela Berardinelli; Elena Bozzola; Mauro Bozzola; Daniela Larizza

    2013-01-01

    Objective. To describe a biochemical growth hormone (GH) deficiency and to evaluate therapeutic result in a six-year-old male with Becker muscular dystrophy (BMD). Methods. GH peak was evaluated after response to arginine and insulin. Bone age was evaluated according to Greulich and Pyle method. Results. The GH-supplementary therapy was very effective in terms of growth gain. Conclusion. The possibility of a growth hormone deficiency and treatment with GH in patients with BMD cannot be exclu...

  16. Newly found sunspot observations by Peter Becker from Rostock for 1708, 1709, and 1710

    CERN Document Server

    Neuhaeuser, Ralph; Pfitzner, Elvira; Richter, Susanne

    2015-01-01

    We present a few newly found old sunspot observations from the years AD 1708, 1709, and 1710, which were obtained by Peter Becker from Rostock, Germany. For 1709, Becker gave a detailed drawing: he observed a sunspot group made up of two spots on Jan 5, 6, and 7, and just one of the two spots was observed on Jan 8 and 9. We present his drawing and his explanatory text. We can measure the latitude and longitude of these two spots and estimate their sizes for all five days. While the spots and groups in 1708 and the spot on four of the five days in January 1709 were known before from other observers (e.g. Hoyt & Schatten 1998), the location of the spots in early January 1709 were not known before, so that they can now be considered in reconstructed butterfly diagrams. The sunspots detected by Becker on 1709 Jan 5 and 1710 Sep 10 were not known before at all, as the only observer known for those two dates, La Hire, did not detect that spot (group). We estimate new group sunspot numbers for the relevant days,...

  17. Newly found sunspot observations by Peter Becker from Rostock for 1708, 1709, and 1710

    Science.gov (United States)

    Neuhäuser , R.; Arlt, R.; Pfitzner, E.; Richter, S.

    2015-09-01

    We present a few newly found old sunspot observations from the years AD 1708, 1709, and 1710, which were obtained by Peter Becker from Rostock, Germany. For 1709, Becker gave a detailed drawing: he observed a sunspot group made up of two spots on January 5, 6, and 7, and just one of the two spots was observed on January 8 and 9. We present his drawing and his explanatory text. We can measure the latitude and longitude of these two spots and estimate their sizes for all five days. While the spots and groups in 1708 and the spot on four of the five days in January 1709 were known before from other observers (e.g. Hoyt & Schatten 1998), the location of the spots in early January 1709 were not known before, so that they can now be considered in reconstructed butterfly diagrams. The sunspots detected by Becker on 1709 January 5 and 1710 September 10 were not known before at all, as the only observer known for those two dates, La Hire, did not detect that spot (group). We estimate new group sunspot numbers for the relevant days, months, and years. The time around 1708-1710 is important, because it documents the recovery of solar activity towards the end of the Maunder Grand Minimum. We also show two new spot observations from G. Kirch for 1708 September 13 and 14 as described in his letter to Wurzelbaur (dated Berlin AD 1708 December 19).

  18. Validation of the modified Becker's split-window approach for retrieving land surface temperature from AVHRR

    Science.gov (United States)

    Quan, Weijun; Chen, Hongbin; Han, Xiuzhen; Ma, Zhiqiang

    2015-10-01

    To further verify the modified Becker's split-window approach for retrieving land surface temperature (LST) from long-term Advanced Very High Resolution Radiometer (AVHRR) data, a cross-validation and a radiance-based (R-based) validation are performed and examined in this paper. In the cross-validation, 3481 LST data pairs are extracted from the AVHRR LST product retrieved with the modified Becker's approach and compared with the Moderate Resolution Imaging Spectroradiometer (MODIS) LST product (MYD11A1) for the period 2002-2008, relative to the positions of 548 weather stations in China. The results show that in most cases, the AVHRR LST values are higher than the MYD11A1. When the AVHRR LSTs are adjusted with a linear regression, the values are close to the MYD11A1, showing a good linear relationship between the two datasets ( R 2 = 0.91). In the R-based validation, comparison is made between AVHRR LST retrieved from the modified Becker's approach and the inversed LST from the Moderate Resolution Transmittance Model (MODTRAN) consolidated with observed temperature and humidity profiles at four radiosonde stations. The results show that the retrieved AVHRR LST deviates from the MODTRAN inversed LST by-1.3 (-2.5) K when the total water vapor amount is less (larger) than 20 mm. This provides useful hints for further improvement of the LST retrieval algorithms' accuracy and consistency.

  19. Antioxidant and cytoprotective properties of infusions from leaves and inflorescences of Achillea collina Becker ex Rchb.

    Science.gov (United States)

    Giorgi, Annamaria; Bombelli, Raffaella; Luini, Alessandra; Speranza, Giovanna; Cosentino, Marco; Lecchini, Sergio; Cocucci, Maurizio

    2009-04-01

    Plants are the main source of molecules with antioxidant and radical scavenging properties that aid the natural defence systems of cells and may be involved in the preservation of human health, particularly preventing all the physiopathological conditions where oxidative damage is a hallmark. Achillea collina Becker ex Rchb. is a medicinal plant of the Achillea millefolium aggregate (yarrow) traditionally used, particularly in mountain areas, as an infusion or alcohol extract for its digestive, antiinflammatory, analgesic, antipyretic and wound healing properties. The aim of this study was to investigate the antioxidant capacity and cytoprotective activity against oxidative stress of infusions obtained from the leaves and inflorescences of Achillea collina Becker ex Rchb., assessed by chemical (free radical scavenging activity by DPPH and Folin Ciocalteu assay) and biological assays (in vitro model of cytotoxicity and lipid peroxidation in PC12 cells line). Infusions of leaves had the highest antioxidant properties and cytoprotective activity. The antioxidant capacity was significantly correlated with the total phenolic content but not with the cytoprotective profile. Achillea collina Becker ex Rchb. has good antioxidant and cytoprotective properties, suggesting further investigations on its chemical composition and potential health value, particularly for traditionally prepared infusions of leaves.

  20. [Jan Kusberg, Kleine Geschichte St. Petersburgs. (Regensburg, 2009) ; Ingrid Bohn. Kleine Geschichte Stockholms. (Regensburg, 2008) ; Konrad Dittrich. Kleine Lübecker Stadtgeschichte. (Regensburg, 2007)] / Dennis Hortmuth

    Index Scriptorium Estoniae

    Hormuth, Dennis

    2011-01-01

    Arvustus: Jan Kusberg, Kleine Geschichte St. Petersburgs. (Regensburg, 2009) ; Ingrid Bohn. Kleine Geschichte Stockholms. (Regensburg, 2008) ; Konrad Dittrich. Kleine Lübecker Stadtgeschichte. (Regensburg, 2007)

  1. [Jan Kusberg, Kleine Geschichte St. Petersburgs. (Regensburg, 2009) ; Ingrid Bohn. Kleine Geschichte Stockholms. (Regensburg, 2008) ; Konrad Dittrich. Kleine Lübecker Stadtgeschichte. (Regensburg, 2007)] / Dennis Hortmuth

    Index Scriptorium Estoniae

    Hormuth, Dennis

    2011-01-01

    Arvustus: Jan Kusberg, Kleine Geschichte St. Petersburgs. (Regensburg, 2009) ; Ingrid Bohn. Kleine Geschichte Stockholms. (Regensburg, 2008) ; Konrad Dittrich. Kleine Lübecker Stadtgeschichte. (Regensburg, 2007)

  2. Muscle MRS detects elevated PDE/ATP ratios prior to fatty infiltration in Becker muscular dystrophy.

    Science.gov (United States)

    Wokke, B H; Hooijmans, M T; van den Bergen, J C; Webb, A G; Verschuuren, J J; Kan, H E

    2014-11-01

    Becker muscular dystrophy (BMD) is characterized by progressive muscle weakness. Muscles show structural changes (fatty infiltration, fibrosis) and metabolic changes, both of which can be assessed using MRI and MRS. It is unknown at what stage of the disease process metabolic changes arise and how this might vary for different metabolites. In this study we assessed metabolic changes in skeletal muscles of Becker patients, both with and without fatty infiltration, quantified via Dixon MRI and (31) P MRS. MRI and (31) P MRS scans were obtained from 25 Becker patients and 14 healthy controls using a 7 T MR scanner. Five lower-leg muscles were individually assessed for fat and muscle metabolite levels. In the peroneus, soleus and anterior tibialis muscles with non-increased fat levels, PDE/ATP ratios were higher (P < 0.02) compared with controls, whereas in all muscles with increased fat levels PDE/ATP ratios were higher compared with healthy controls (P ≤ 0.05). The Pi /ATP ratio in the peroneus muscles was higher in muscles with increased fat fractions (P = 0.005), and the PCr/ATP ratio was lower in the anterior tibialis muscles with increased fat fractions (P = 0.005). There were no other significant changes in metabolites, but an increase in tissue pH was found in all muscles of the total group of BMD patients in comparison with healthy controls (P < 0.05). These findings suggest that (31) P MRS can be used to detect early changes in individual muscles of BMD patients, which are present before the onset of fatty infiltration.

  3. Rapid carrier and prenatal diagnosis of Duchenne and Becker muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Roberts, R.G.; Cole, C.G.; Hart, K.A.; Bobrow, M.; Bentley, D.R. (Guy' s Hospital, London (England))

    1989-01-25

    Carrier and prenatal diagnosis of Duchenne and Becker muscular dystrophy (DMD and BMD) by DNA methods uses Southern blotting to detect either the informative segregation of restriction fragment length polymorphisms (RFLPs) or the absence of restriction fragments in affected males. Recently, the use of the polymerase chain reaction (PCR) for rapid detection of deletions in some affected males was reported eliminating the need for Southern blotting of 37% of all samples. This approach is not applicable, however, to non-deletion cases or for carrier diagnosis. The authors have used PCR for rapid analysis of intragenic RFLPs to permit both carrier and prenatal diagnosis in the majority of familial cases.

  4. Growth Hormone Deficiency in a Patient with Becker Muscular Dystrophy: A Pediatric Case Report

    Directory of Open Access Journals (Sweden)

    Valeria Calcaterra

    2013-01-01

    Full Text Available Objective. To describe a biochemical growth hormone (GH deficiency and to evaluate therapeutic result in a six-year-old male with Becker muscular dystrophy (BMD. Methods. GH peak was evaluated after response to arginine and insulin. Bone age was evaluated according to Greulich and Pyle method. Results. The GH-supplementary therapy was very effective in terms of growth gain. Conclusion. The possibility of a growth hormone deficiency and treatment with GH in patients with BMD cannot be excluded, especially considering the good therapeutic response.

  5. Growth hormone deficiency in a patient with becker muscular dystrophy: a pediatric case report.

    Science.gov (United States)

    Calcaterra, Valeria; Malvezzi, Annachiara; Toglia, Rossana; Berardinelli, Angela; Bozzola, Elena; Bozzola, Mauro; Larizza, Daniela

    2013-01-01

    Objective. To describe a biochemical growth hormone (GH) deficiency and to evaluate therapeutic result in a six-year-old male with Becker muscular dystrophy (BMD). Methods. GH peak was evaluated after response to arginine and insulin. Bone age was evaluated according to Greulich and Pyle method. Results. The GH-supplementary therapy was very effective in terms of growth gain. Conclusion. The possibility of a growth hormone deficiency and treatment with GH in patients with BMD cannot be excluded, especially considering the good therapeutic response.

  6. CAPABILITIES OF ONE-STAGE BREAST REPAIR WITH A BECKER EXPANDING ENDOPROSTHESIS

    Directory of Open Access Journals (Sweden)

    N. R. Fedyanina

    2009-01-01

    Full Text Available Organ-preserving surgical interventions can be performed owing to improved drug and radiation therapy methods. When radical resec- tion is contraindicated, radical mastectomy is carried out, which is a serious psychological trauma to a woman.In this connection, plastic reparative surgery for breast malignancies is growing in importance. An operation using silicone implants is technically much simpler and less traumatic to patients; therefore one-stage repair with a Becker expanding endoprosthesis both alone and that in combination with displaced flaps occupy a highly important place.

  7. Analysis of 24 Cases of Becker's Nevus%Becker痣24例分析

    Institute of Scientific and Technical Information of China (English)

    李钟洙; 黄一锦; 张召力; 廖永强; 张海萍

    2012-01-01

    目的 探讨Becker痣的临床特点、合并症和组织病理表现.方法 回顾性分析24例Becker痣临床和病理资料.结果 24例均为散发病例,发病年龄主要集中在10 ~ 20岁(83.33%),发病部位以躯干前部和肩胛部居多(45.83%),典型皮损为较大的单侧分布的多毛的色素沉着斑,组织病理主要表现是表皮不同程度角化过度和棘层肥厚,表皮突较规则地向下延伸,基底层黑素颗粒增多,真皮网状层常见较大的形态不规则的平滑肌纤维束,伴有并发症共5例(20.83%).结论 根据Becker痣典型皮损特点结合组织病理表现临床可以确诊,但是做为一种遗传相关的错构瘤可伴发其他皮肤肌肉骨骼异常,需仔细探明.%Objective To survey the Clinical features, complications and pathological manifestations of Becker's nevus. Methods The clinical and pathological materials of 24 cases of Becker' s nevus were analysed retrospectively. Results All of 24 cases occurred sporadically. The age of onset was mainly around 10 — 20 years old (83. 33% ). The lesions were located predominantly on the anterior trunk or on the scapular region (45.83% ). The typical primary lesion was large, unilateral hyperpigmented patch with hypertrichosis. The histopathology showed varying degrees of hyperkeratosis and acanthosis of the epidermis, regular elongation of the rete ridges,and basal layer hyperpigmentation ,usually larger sized and irregularly formed smooth muscle fiber scattered haphazardly in reticular dermis. Five cases were associated with different kinds of complication (20. 83% ). Conclusion Becker's nevus can be confirmed on the basis of clinical features and pathological examination. As a genetic-related hamartoma, however, Becker' s nevus may be accompanied with other cutaneous and musculoskeletal anomalies that need to be carefully proven.

  8. Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

    DEFF Research Database (Denmark)

    Løkken, Nicoline; Hedermann, Gitte; Thomsen, Carsten;

    2016-01-01

    We investigated whether a linear relationship between muscle strength and cross-sectional area (CSA) is preserved in calf muscles of patients with Becker muscular dystrophy (BMD, n = 14) and limb-girdle type 2I muscular dystrophy (LGMD2I, n = 11), before and after correcting for muscle fat...... infiltration. The Dixon magnetic resonance imaging technique was used to quantify fat and calculate a fat-free contractile CSA. Strength was assessed by dynamometry. Muscle strength/CSA relationships were significantly lower in patients versus controls. The strength/contractile-CSA relationship was still...

  9. Notiz zum Erscheinungsdatum der "Flora der Gegend um Frankfurt am Main" von Johannes Becker

    OpenAIRE

    Buttler, Karl Peter

    2012-01-01

    Der erste Band von Beckers „Flora der Gegend um Frankfurt am Main“ wurde zwischen dem 14. Oktober und dem 8. November 1827 publiziert. Dank einer Aktennotiz im Archiv der Wetterauischen Gesellschaft für die gesamte Naturkunde in Hanau kann das Erscheinungsdatum auf diesen Zeitraum eingeengt werden. The first volume of Becker’s “Flora der Gegend um Frankfurt am Main” (Flora in the Frankfurt am Main Region) was published between 14 October and 8 November 1827. The publication date can be att...

  10. RESULTS OF MEDICO-GENETIC STUDY OF PATIENTS WITH DUCHENNE/BECKER PROGRESSIVE MUSCULAR DYSTROPHIES IN UZBEKISTAN

    Directory of Open Access Journals (Sweden)

    Umida Tulkinovna Omonova

    2014-11-01

    Full Text Available The purpose of study was to analyze clinical and genetic polymorphism of Duchenne/Becker progressive muscular dystrophies among patients with neuromuscular diseases in Uzbekistan. 106 male patients with progressive pseudohypertrophic forms of muscular dystrophy were retrospectively and prospectively analyzed in the period from 2004 till 2014: 93 patients with Duchenne PMD aged from 3 years to 18 years and 13 patients with Becker PMD aged from 10 years to 25 years, who had been examined in the medico-genetic consulting department of the Republican Center “Mother and Child Screening” of Tashkent city. Comprehensive clinical, neurophysiological, biochemical and genetic study of patients as the integral part in the differential diagnosis of Duchenne/Becker progressive muscular dystrophies allows creating the national database on D/B PMD to prevent the birth of children in families burdened by this disease.

  11. Gary Becker répond à Foucault sur l’analyse économique des crimes et des peines

    OpenAIRE

    Bernard E. Harcourt

    2015-01-01

    Après un premier débat autour de la pensée néolibérale américaine de la Chicago School of Economics – traduit et publiée dans le numéro 3 de Socio (Becker, Ewald et Harcourt, 2014) – le regard de Gary Becker se retourne vers l’analyse économique de la criminalité et du droit pénal. Foucault avait consacré sa leçon du 21 mars 1979 à une analyse prémonitoire d’un article phare de Becker, « Crime and punishment: An economic approach », publié en 1968 dans le Journal of Political Economy (Foucaul...

  12. Sexo, divorcio y machismo: En torno a Gary Becker, Premio Nobel de Economía 1992

    OpenAIRE

    Fernández-Baca, Jorge

    2014-01-01

    Gary Becker ha dedicado su vida a extender el campo de aplicación de la teoría económica a todas las actividades del ser humano. En su Tratado sobre la Familia, Becker estudia los problemas de la discriminación sexual, la poligamia y la monogamia, la elección de parejas, la demanda de hijos y el divorcio, desde el punto de vista de la inversión en capital humano. Este es posiblemente el trabajo que lo ha llevado a merecer el Premio Nobel de Economía y del cual se ofrece aquí un breve comentar...

  13. Orthodontic treatment in a patient with unilateral open-bite and Becker muscular dystrophy. A 5-year follow-up

    Directory of Open Access Journals (Sweden)

    Juan Fernando Aristizabal

    2014-12-01

    Full Text Available INTRODUCTION: Becker muscular dystrophy is an X-chromosomal linked anomaly characterized by progressive muscle wear and weakness. This case report shows the orthodontic treatment of a Becker muscular dystrophy patient with unilateral open bite.METHODS: To correct patient's malocclusion, general anesthesia and orthognathic surgery were not considered as an option. Conventional orthodontic treatment with intermaxillary elastics and muscular functional therapy were employed instead.RESULTS: After 36 months, open bite was corrected. The case remains stable after a 5-year post-treatment retention period.

  14. Ways of Telling About Society. Howard S. Becker in Conversation With Reiner Keller

    Directory of Open Access Journals (Sweden)

    Howard S. Becker

    2016-04-01

    Full Text Available In the following conversation, Howard S. BECKER talks about his lifelong travel with and between sociology and jazz music, his professional training as a sociologist, the hazards of a career, and his involvement with photography and performance. He reflects on the different ways used by artists and sociologists to tell solid stories about social phenomena, and tells a compelling account in its own right about the methodology of sound sociological field work and case study research. By explaining core concepts of his sociological perspective (such as the concept of labeling and "doing things together" and referring to concrete research examples, BECKER in all modesty fully engages with what could be called today's sociological imagination, leaving narrow disciplinary constraints behind in order to explore society with curiosity, using methodologically sensible but nevertheless refreshing approaches. The audio file is accessible from http://dx.doi.org/10.5281/zenodo.49829. URN: http://nbn-resolving.de/urn:nbn:de:0114-fqs1602122

  15. TNF-α-Induced microRNAs Control Dystrophin Expression in Becker Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Alyson A. Fiorillo

    2015-09-01

    Full Text Available The amount and distribution of dystrophin protein in myofibers and muscle is highly variable in Becker muscular dystrophy and in exon-skipping trials for Duchenne muscular dystrophy. Here, we investigate a molecular basis for this variability. In muscle from Becker patients sharing the same exon 45–47 in-frame deletion, dystrophin levels negatively correlate with microRNAs predicted to target dystrophin. Seven microRNAs inhibit dystrophin expression in vitro, and three are validated in vivo (miR-146b/miR-374a/miR-31. microRNAs are expressed in dystrophic myofibers and increase with age and disease severity. In exon-skipping-treated mdx mice, microRNAs are significantly higher in muscles with low dystrophin rescue. TNF-α increases microRNA levels in vitro whereas NFκB inhibition blocks this in vitro and in vivo. Collectively, these data show that microRNAs contribute to variable dystrophin levels in muscular dystrophy. Our findings suggest a model where chronic inflammation in distinct microenvironments induces pathological microRNAs, initiating a self-sustaining feedback loop that exacerbates disease progression.

  16. DGGE based whole-gene mutation scanning of the dystrophlin gene in Duchenne and Becker muscular dystrophy patients

    NARCIS (Netherlands)

    Hofstra, RMW; Mulder, IM; Vossen, R; de Koning-Gans, PAM; Kraak, M; Ginjaar, IB; van der Hout, AH; Bakker, E; Buys, CHCM; van Essen, AJ; den Dunnen, JT

    2004-01-01

    Duchenne and Becker muscular dystrophy (DMD and BMD) are caused by mutations in the dystrophin gene. Large rearrangements in the gene are found in about two,thirds of DMD patients, with similar to60% carrying deletions and 5-10% carrying duplications. Most of the remaining 30-35% of patients are exp

  17. Five new species of Centorisoma Becker from China, with an updated key to world species (Diptera, Chloropidae).

    Science.gov (United States)

    Liu, Xiaoyan; Yang, Ding

    2014-06-19

    A review of the species of the genus Centorisoma Becker from China is provided. The following 5 species are described as new to science: C. convexum sp. nov., C. mediconvexum sp. nov., C. neimengguensis sp. nov., C. pentagonium sp. nov. and C. sexangulatum sp. nov. An updated key to the world species of genus Centorisoma is given.

  18. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in the Netherlands : a cohort study

    NARCIS (Netherlands)

    Hoogerwaard, EM; Bakker, E; Ippel, PF; Oosterwijk, JC; Majoor-Krakauer, DF; Leschot, NJ; Van Essen, AJ; Brunner, HG; van der Wouw, PA; Wilde, AAM; de Visser, M

    1999-01-01

    Background Carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) may show muscle weakness or dilated cardiomyopathy. Studies focusing on skeletal-muscle involvement were done before DNA analysis was possible. We undertook a cross-sectional study in a population of definit

  19. Signs and symptoms of Duchenne muscular dystrophy and Becker muscular dystrophy among carriers in the Netherlands : a cohort study

    NARCIS (Netherlands)

    Hoogerwaard, EM; Bakker, E; Ippel, PF; Oosterwijk, JC; Majoor-Krakauer, DF; Leschot, NJ; Van Essen, AJ; Brunner, HG; van der Wouw, PA; Wilde, AAM; de Visser, Marianne

    1999-01-01

    Background Carriers of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) may show muscle weakness or dilated cardiomyopathy. Studies focusing on skeletal-muscle involvement were done before DNA analysis was possible. We undertook a cross-sectional study in a population of

  20. Carrier detection in Becker muscular dystrophy using creatine kinase estimation and DNA analysis.

    Science.gov (United States)

    Kingston, H M; Sarfarazi, M; Newcombe, R G; Willis, N; Harper, P S

    1985-04-01

    Serum creatine kinase levels in 39 control females and 59 obligate carriers of Becker muscular dystrophy (BMD) have been used to construct likelihood ratios for carrier detection. In 24 possible carriers of BMD, analysis of DNA with X chromosome specific DNA probes linked to the dystrophy gene, has been used in conjunction with creatine kinase measurement to calculate final risk estimates of carrier status. Incorporation of information from probe genotype into the Bayesian calculation, enables a substantially lower risk to be deliniated for some possible carriers of the BMD gene. Thus, although the existing DNA probes are not sufficiently closely linked to BMD to be used in prenatal diagnosis, they can make a major contribution to genetic counseling by refining the estimated probability of carrier status.

  1. Pseudoexon activation increases phenotype severity in a Becker muscular dystrophy patient.

    Science.gov (United States)

    Greer, Kane; Mizzi, Kayla; Rice, Emily; Kuster, Lukas; Barrero, Roberto A; Bellgard, Matthew I; Lynch, Bryan J; Foley, Aileen Reghan; O Rathallaigh, Eoin; Wilton, Steve D; Fletcher, Sue

    2015-07-01

    We report a dystrophinopathy patient with an in-frame deletion of DMD exons 45-47, and therefore a genetic diagnosis of Becker muscular dystrophy, who presented with a more severe than expected phenotype. Analysis of the patient DMD mRNA revealed an 82 bp pseudoexon, derived from intron 44, that disrupts the reading frame and is expected to yield a nonfunctional dystrophin. Since the sequence of the pseudoexon and canonical splice sites does not differ from the reference sequence, we concluded that the genomic rearrangement promoted recognition of the pseudoexon, causing a severe dystrophic phenotype. We characterized the deletion breakpoints and identified motifs that might influence selection of the pseudoexon. We concluded that the donor splice site was strengthened by juxtaposition of intron 47, and loss of intron 44 silencer elements, normally located downstream of the pseudoexon donor splice site, further enhanced pseudoexon selection and inclusion in the DMD transcript in this patient.

  2. Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Sveen, Marie-Louise; Thune, Jens Jakob; Køber, Lars;

    2008-01-01

    of dystrophic changes on muscle biopsy. CONCLUSIONS: This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.......OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology...... at Rigshospitalet. Patients One hundred one patients with LGMD2A-I and BMD and 29 patients with LGMD2 and no molecular diagnosis. MAIN OUTCOME MEASURES: Clinical investigation, echocardiography, and electrocardiographic findings. RESULTS: Cardiac involvement was present in 24 of 100 patients (24%) with LGMD2A...

  3. Deletion Analysis Of The Duchenne/Becker Muscular Dystrophy Gene Using Multiplex Polymerase Chain Reaction

    Directory of Open Access Journals (Sweden)

    Dastur P

    2004-01-01

    Full Text Available The diagnosis of Duchenna Muscular Dystrophy (DMD and Becker Muscular Dystorphy (BMD is mainly based on clinical profile, serum CPK values, muscle biopsy and immunostaining for dystrophin. This was done in 100 unrelated patients using 19 exons including the promoter region in two sets of multiplex polymerase chain reaction (PCR. These primers amplify most of the exons in the deletion prone ′hot spot′ regions allowing determinations of deletion end points. Intragenic deletions were detected in 74 patients indicating that the use of PCR- based assays will allow deletion detection help in prenatal diagnosis for most of the DMD/BMD patients. The frequency of deletions observed in the present study was 74%.

  4. Duchenne and Becker Muscular Dystrophy: Contribution of a Molecular and Immunohistochemical Analysis in Diagnosis in Morocco

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    Hanane Bellayou

    2009-01-01

    Full Text Available Duchenne muscular dystrophy (DMD and Becker muscular dystrophy (BMD are X-linked recessive disorders caused by mutations of the DMD gene located at Xp21. In DMD patients, dystrophin is virtually absent; whereas BMD patients have 10% to 40% of the normal amount. Deletions in the dystrophin gene represent 65% of mutations in DMD/BMD patients. To explain the contribution of immunohistochemical and genetic analysis in the diagnosis of these dystrophies, we present 10 cases of DMD/BMD with particular features. We have analyzed the patients with immunohistochemical staining and PCR multiplex to screen for exons deletions. Determination of the quantity and distribution of dystrophin by immunohistochemical staining can confirm the presence of dystrophinopathy and allows differentiation between DMD and BMD, but dystrophin staining is not always conclusive in BMD. Therefore, only identification involved mutation by genetic analysis can establish a correct diagnosis.

  5. Relatively low proportion of dystrophin gene deletions in Israeili Duchenne and Becker muscular dystrophy patients

    Energy Technology Data Exchange (ETDEWEB)

    Shomrat, R.; Gluck, E.; Legum, C.; Shiloh, Y. [Tel Aviv Univ. (Israel)

    1994-02-15

    Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are allelic disorders caused by mutations in the X-linked dystrophin gene. The most common mutations in western populations are deletions that are spread non-randomly throughout the gene. Molecular analysis of the dystrophin gene structure by hybridization of the full length cDNA to Southern blots and by PCR in 62 unrelated Israeli male DMD/BMD patients showed deletions in 23 (37%). This proportion is significantly lower than that found in European and North American populations (55-65%). Seventy-eight percent of the deletions were confined to exons 44-52, half of these exons 44-45, and the remaining 22% to exons 1 and 19. There was no correlation between the size of the deletion and the severity of the disease. All the deletions causing frameshift resulted in the DMD phenotypes. 43 refs., 1 fig., 1 tab.

  6. Dystrophin, utrophin and {beta}-dystroglycan expression in skeletal muscle from patients with Becker muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Kawajiri, Masakazu; Mitsui, Takao; Kawai, Hisaomi [Univ. of Tokushima (Japan)] [and others

    1996-08-01

    The precise localization and semiquantitative correlation of dystrophin, utrophin and {beta}-dystroglycan expression on the sarcolemma of skeletal muscle cells obtained from patients with Becker muscular dystrophy (BMD) was studied using three types of double immunofluorescence. Staining intensity was measured using a confocal laser microscope. Each of these proteins was identified at the same locus on the sarcolemma. The staining intensities of dystrophin and utrophin were approximately reciprocal at sarcolemmal sites where dystrophin expression was obviously observed. The staining intensity of {beta}-dystroglycan was strong in areas where dystrophin staining was also strong and utrophin expression was weak. Quantitative analysis revealed that the staining intensity of {beta}-dystroglycan minus that of dystrophin approximated the staining intensity of utrophin, indicating that the sum of dystrophin and utrophin expression corresponds to that of {beta}-dystroglycan. These results suggest that utrophin may compensate for dystrophin deficiency found in BMD by binding to {beta}-dystroglycan. 35 refs., 3 figs., 1 tab.

  7. Becker and limb-girdle muscular dystrophy associated with pituitary dwarfism.

    Science.gov (United States)

    Marconi, G; Taiuti, R; Sbrilli, C; Pizzi, A

    1987-08-01

    In 1981 a report appeared of a patient with Duchenne muscular dystrophy associated with dwarfism caused by growth hormone deficiency, in whom the muscular disease was unusually benign. The authors suggested that the benign course might be related to the growth hormone deficiency and dwarfism. Other authors later supported this idea, having observed that in dystrophic mice and hamsters with congenital and experimentally induced pituitary dwarfism, respectively, pathological expressions of the dystrophy were markedly reduced. In this paper one case of Becker and one of limb-girdle dystrophy, each associated with short stature and growth hormone deficiency are described. In these cases the disease did not have a particularly benign course. It is concluded that caution is necessary, at least in certain cases, before an association between reduced muscular growth and the dystrophic process can be assumed.

  8. Howard S. Becker : un sociologue de la haute mer

    Directory of Open Access Journals (Sweden)

    Bruno Péquignot

    2010-12-01

    Full Text Available Une révolution psychique vient sûrement de se produire en ce siècle ; la raison humaine vient d’être désancrée et la connaissance a quitté les rives du réel immédiat. N’y a-t-il pas alors anachronisme à cultiver le goût du port, de la certitude, du système ?Gaston Bachelard (1936, p. 11.Terminant la lecture du dernier livre (traduit en français de Howard S. Becker, cette phrase de Gaston Bachelard, que j’ai souvent citée et qui me poursuit comme le texte d’où elle est issue depuis bien long...

  9. Extensive functional evaluations to monitor aerobic training in Becker Muscular Dystrophy: A case report

    Directory of Open Access Journals (Sweden)

    Caterina Tramonti

    2016-06-01

    Full Text Available Low-intensity aerobic training seems to have positive effects on muscle strength, endurance and fatigue in Becker Muscular Dystrophy (BMD patients. We describe the case of a 33-year old BMD man, who performed a four-week aerobic training. Extensive functional evaluations were executed to monitor the efficacy of the rehabilitative treatment. Results evidenced an increased force exertion and an improvement in muscle contraction during sustained exercise. An improvement of walk velocity, together with agility, endurance capacity and oxygen consumption during exercise was observed. Moreover, an enhanced metabolic efficiency was evidenced, as shown by reduced lactate blood levels after training. Interestingly, CK showed higher levels after the training protocol, revealing possible muscle damage. In conclusion, aerobic training may represent an effective method improving exercise performance, functional status and metabolic efficiency. Anyway, a careful functional assessment should be taken into account as a useful approach in the management of the disease’s rehabilitative treatment.

  10. Duchenne and Becker muscular dystrophies: An Indian update on genetics and rehabilitation

    Directory of Open Access Journals (Sweden)

    Nadkarni Jayshree

    2008-01-01

    Full Text Available The application of molecular diagnostic techniques has greatly improved the diagnosis, carrier detection, prenatal testing and genetic counseling for families with Duchenne and Becker muscular dystrophy (D/BMD in India. The prediction of Duchenne muscular dystrophy (DMD patients to have out-framed deletions and Becker′s muscular dystrophy (BMD patients to have in-frame deletions of dystrophin gene holds well in the vast majority of cases. Mutation detection is obviously critical for diagnosis but it may also be important for future therapeutic purposes. These factors underscore the need for earlier referral, genetic counseling and provision of support and rehabilitation services which are the main priorities for psychosocial assessment and intervention at medical and social levels.

  11. Dialogue avec Howard Becker : comment parler de la société ?

    Directory of Open Access Journals (Sweden)

    Jacques Hamel

    2010-12-01

    Full Text Available Il n’est rien de plus intéressant que de lire le résumé d’un livre écrit par son propre auteur, surtout quand ce dernier a pour nom Howard Becker, l’esprit libre et imaginatif de la sociologie américaine. En effet, la densité et la richesse de Comment parler de la société ? – comme de ses autres écrits – peuvent avoir déjoué l’attention des lecteurs et les avoir incités à négliger certaines thèses chères à l’auteur que celui-ci veut mettre en exergue en s’employant à en donner le compte rendu...

  12. Deletion Analysis Of The Duchenne/Becker Muscular Dystrophy Gene Using Multiplex Polymerase Chain Reaction

    Directory of Open Access Journals (Sweden)

    Dastur R

    2003-01-01

    Full Text Available The diagnosis of Duchenne Muscular Dystrophy (DMD and Becker Muscular Dystrophy (BMD is mainly based on clinical profile, serum CPK values, muscle biopsy and immunostaining for dystrophin. Most recent and accurate method for diagnosing DMD/BMD is by detection of mutations in the DMD gene. This was done in 100 unrelated patients using 19 exons including the promoter region in two sets of multiplex polymerase chain reaction (PCR. These primers amplify most of the exons in the deletion prone ′hotspot′ regions allowing determination of deletion end point. Intragenic deletions were detected in 74 patients indicating that the use of PCR-based assays will allow deletion detection help in prenatal diagnosis for most of the DMD/BMD patients. The frequency of deletions observed in the present study was 74%.

  13. A Becker myotonia patient with compound heterozygosity for CLCN1 mutations and Prinzmetal angina pectoris.

    Science.gov (United States)

    Zielonka, Daniel; Jurkat-Rott, Karin; Stachowiak, Paweł; Bryl, Anna; Marcinkowski, Jerzy T; Lehmann-Horn, Frank

    2012-04-01

    Becker myotonia is a recessive muscle disease with prevalence of > 1:50,000. It is caused by markedly reduced function of the chloride channel encoded by CLCN1. We describe a Polish patient with severe myotonia, transient weakness, and muscle cramps who only responds to lidocaine. In addition, the patient has Prinzmetal angina pectoris and multiple lipomatosis. He is compound heterozygeous for a novel p.W303X and a frequent p.R894X CLCN1 mutation. CLCN1 exon number variation was excluded by MLPA. His son with latent myotonia was heterozygeous for p.R894X. We discuss the potential relations of the three rare diseases and the inheritance of p.R894X.

  14. Teoria Microeconômica de Becker e Fecundidade no Brasil

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    Danielle Nascimento

    2012-12-01

    Full Text Available No estudo da dinâmica demográfica, os indicadores de natalidade são fundamentais para o entendimento do crescimento natural de uma população. Assim como a mortalidade, a natalidade é influenciada por fatores biológicos e socioeconômicos. No Brasil, segundo os dados do Censo Demográfico de 2010, a natalidade destacou-se pelo seu comportamento e demonstrou que esse evento é cada vez mais influenciado pelos aspectos socioeconômicos do país. Segundo Becker, esse processo é resultado de uma combinação ótima que os pais fazem entre filhos e outros bens de forma que a sua satisfação seja maximizada, dada a sua restrição orçamentária.

  15. Co-occurrence of vitiligo and Becker's nevus: A case report

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    Ayşegül Yalçınkaya İyidal

    2016-12-01

    Full Text Available Vitiligo is an acquired disorder with an unknown etiology in which genetic and non-genetic factors coexist. Melanocytes are destructed in the affected skin areas and clinically depigmented macules and patches appear on the skin. Becker's nevus (BN appears as hyperpigmented macule, patch or verrucous plaques with sharp and irregular margins and often unilateral occurrence and with associated hypertrichosis in various degrees. Although its pathogenesis is unknown, it is suggested to represent a hamartomatous lesion harboring androgen receptors on the lesion. In this report, we present a 19-year-old male patient who developed vitiligo lesions and then BN adjacent to the vitiligo lesion in the right upper back portion of the body ten years after the initial vitiligo lesion.

  16. Becker muscular dystrophy in Indian patients: Analysis of dystrophin gene deletion patterns

    Directory of Open Access Journals (Sweden)

    Dastur Rashna

    2008-01-01

    Full Text Available Background: Becker muscular dystrophy (BMD is caused by mutations in the dystrophin gene with variable phenotypes. Becker muscular dystrophy patients have low levels of nearly full-length dystrophin and carry in-frame mutations, which allow partial functioning of the protein. Aim: To study the deletion patterns of BMD and to correlate the same with reading frame rule and different phenotypes. Setting: A tertiary care teaching hospital. Design: This is a prospective hospital-based study. Materials and Methods: Thirty-two exons spanning different "hot spot" regions using Multiplex PCR techniques were studied in 347 patients. Two hundred and twenty-two showed deletions in one or more of the 32 exons. Out of these, 46 diagnosed as BMD patients were analyzed. Results: Forty-six BMD patients showed deletions in both regions of the dystrophin gene. Out of these 89.1% (41/46 were in-frame deletions. Deletions starting with Exon 45 were found in 76.1% (35/46 of the cases. Mutations in the majority of cases i.e. 39/46 (84.8% were seen in 3′ downstream region (Exon 45-55, distal rod domain. Few, i.e. 5/46 (10.8% showed deletions in 5′ upstream region (Exons 3-20, N-terminus and proximal rod domain of the gene, while in 2/46 (4.4% large mutations (>40 bp spanning both regions (Exons 3-55 were detected. Conclusion: This significant gene deletion analysis has been carried out for BMD patients particularly from Western India using 32 exons.

  17. Duchenne and Becker muscular dystrophy: a molecular and immunohistochemical approach Distrofia muscular de Duchenne e Becker: abordagem molecular e imuno-histoquímica

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    Aline Andrade Freund

    2007-03-01

    Full Text Available Duchenne muscular dystrophy (DMD and Becker muscular dystrophy (BMD are caused by mutations in the dystrophin gene. We studied 106 patients with a diagnosis of probable DMD/BMD by analyzing 20 exons of the dystrophin gene in their blood and, in some of the cases, by immunohistochemical assays for dystrophin in muscle biopsies. In 71.7% of the patients, deletions were found in at least one of the exons; 68% of these deletions were in the hot-spot 3' region. Deletions were found in 81.5% of the DMD cases and in all the BMD cases. The cases without deletions, which included the only woman in the study with DMD, had dystrophin deficiency. The symptomatic female carriers had no deletions but had abnormal dystrophin distribution in the sarcolemma (discontinuous immunostains. The following diagnoses were made for the remaining cases without deletions with the aid of a muscle biopsy: spinal muscular atrophy, congenital myopathy; sarcoglycan deficiency and unclassified limb-girdle muscular dystrophy. Dystrophin analysis by immunohistochemistry continues to be the most specific method for diagnosis of DMD/BMD and should be used when no exon deletions are found in the dystrophin gene in the blood.As distrofias musculares de Duchenne (DMD e de Becker (DMB são doenças causadas por mutação no gene da distrofina. Foram estudados 106 casos com a suspeita diagnóstica de DMD/BMD com a analise de 20 exons do gene da distrofina no sangue e biópsia muscular com imuno-histoquímica para distrofina em alguns casos. Em 71,7% dos casos foi encontrada deleção em pelo menos um dos exons, sendo que 68% das deleções localizam-se na região 3' hot spot. Foram encontradas deleções em 81,5% dos DMD e em todos os BMD, sendo que os sem deleção tinham deficiência de distrofina, incluindo a mulher com DMD. As portadoras sintomáticas não tinham deleções mas anormalidades na distribuição da distrofina no sarcolema. Os outros casos sem deleção, com auxilio da

  18. Notas sobre la microsociología y el cuerpo. Las microcorporalidades de Becker, Scott, Bourdieu y de Certeau

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    Eugenia Fraga

    2016-01-01

    Full Text Available En el presente trabajo nos interesa indagar en el modo en que la perspectiva microsociológica ha pensado el tema del cuerpo. Queremos ver de qué formas, autores tan variados y a la vez tan afines como Howard Becker, James Scott, Pierre Bourdieu y Michel de Certeau, herederos en distinta medida del interaccionismo simbólico de George Mead, han concebido aquella dimensión tan central –y a veces tan soslayada– de la vida social como es la corporalidad. Para ello deberemos, en primer lugar, profundizar en las reflexiones de Mead, antecedente relevante del resto de los autores aquí trabajados. En segundo lugar, deberemos realizar un rastreo de los distintos conceptos utilizados por Becker, Scott, Bourdieu y de Certeau, para hablar del cuerpo humano. Finalmente, intentaremos esbozar unas conclusiones comparativas. Abstract It is the interest of the present paper to look into how microsociological views have approached the issue of the body. We want to understand how authors as diverse and, at the same time, as close as Howard Becker, James Scott, Pierre Bourdieu and Michel de Certeau, heirs in different degrees of George Mead’s symbolic interactionism, have all considered a dimension as central –and sometimes as overlooked- of social life as corporality. For this purpose we must, in first place, dig deeper into Mead’s reflections, a relevant predecessor of all the authors referred here. Then, we must track the different concepts used by Becker, Scott, Bourdieu and de Certeau to refer to the human body. Finally, we will outline some comparative conclusions.

  19. Evidence of Insulin Resistance and Other Metabolic Alterations in Boys with Duchenne or Becker Muscular Dystrophy

    Science.gov (United States)

    Rodríguez-Cruz, Maricela; Sanchez, Raúl; Escobar, Rosa E.; Cruz-Guzmán, Oriana del Rocío; López-Alarcón, Mardia; Bernabe García, Mariela; Coral-Vázquez, Ramón; Matute, Guadalupe; Velázquez Wong, Ana Claudia

    2015-01-01

    Aim. Our aim was (1) to determine the frequency of insulin resistance (IR) in patients with Duchenne/Becker muscular dystrophy (DMD/BMD), (2) to identify deleted exons of DMD gene associated with obesity and IR, and (3) to explore some likely molecular mechanisms leading to IR. Materials and Methods. In 66 patients with DMD/BMD without corticosteroids treatment, IR, obesity, and body fat mass were evaluated. Molecules involved in glucose metabolism were analyzed in muscle biopsies. Results show that 18.3%, 22.7%, and 68% were underweight, overweight, or obese, and with high adiposity, respectively; 48.5% and 36.4% presented hyperinsulinemia and IR, respectively. Underweight patients (27.3%) exhibited hyperinsulinemia and IR. Carriers of deletions in exons 45 (OR = 9.32; 95% CI = 1.16–74.69) and 50 (OR = 8.73; 95% CI = 1.17–65.10) from DMD gene presented higher risk for IR than noncarriers. We observed a greater staining of cytoplasmic aggregates for GLUT4 in muscle biopsies than healthy muscle tissue. Conclusion. Obesity, hyperinsulinemia, and IR were observed in DMD/BMD patients and are independent of corticosteroids treatment. Carriers of deletion in exons 45 or 50 from DMD gene are at risk for developing IR. It is suggested that alteration in GLUT4 in muscle fibers from DMD patients could be involved in IR. PMID:26089900

  20. Evidence of Insulin Resistance and Other Metabolic Alterations in Boys with Duchenne or Becker Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Maricela Rodríguez-Cruz

    2015-01-01

    Full Text Available Aim. Our aim was (1 to determine the frequency of insulin resistance (IR in patients with Duchenne/Becker muscular dystrophy (DMD/BMD, (2 to identify deleted exons of DMD gene associated with obesity and IR, and (3 to explore some likely molecular mechanisms leading to IR. Materials and Methods. In 66 patients with DMD/BMD without corticosteroids treatment, IR, obesity, and body fat mass were evaluated. Molecules involved in glucose metabolism were analyzed in muscle biopsies. Results show that 18.3%, 22.7%, and 68% were underweight, overweight, or obese, and with high adiposity, respectively; 48.5% and 36.4% presented hyperinsulinemia and IR, respectively. Underweight patients (27.3% exhibited hyperinsulinemia and IR. Carriers of deletions in exons 45 (OR = 9.32; 95% CI = 1.16–74.69 and 50 (OR = 8.73; 95% CI = 1.17–65.10 from DMD gene presented higher risk for IR than noncarriers. We observed a greater staining of cytoplasmic aggregates for GLUT4 in muscle biopsies than healthy muscle tissue. Conclusion. Obesity, hyperinsulinemia, and IR were observed in DMD/BMD patients and are independent of corticosteroids treatment. Carriers of deletion in exons 45 or 50 from DMD gene are at risk for developing IR. It is suggested that alteration in GLUT4 in muscle fibers from DMD patients could be involved in IR.

  1. Becker muscular dystrophy due to an intronic splicing mutation inducing a dual dystrophin transcript.

    Science.gov (United States)

    Todeschini, Alice; Gualandi, Francesca; Trabanelli, Cecilia; Armaroli, Annarita; Ravani, Anna; Fanin, Marina; Rota, Silvia; Bello, Luca; Ferlini, Alessandra; Pegoraro, Elena; Padovani, Alessandro; Filosto, Massimiliano

    2016-10-01

    We describe a 29-year-old patient who complained of left thigh muscle weakness since he was 23 and of moderate proximal weakness of both lower limbs with difficulty in climbing stairs and running since he was 27. Mild weakness of iliopsoas and quadriceps muscles and muscle atrophy of both the distal forearm and thigh were observed upon clinical examination. He harboured a novel c.1150-3C>G substitution in the DMD gene, affecting the intron 10 acceptor splice site and causing exon 11 skipping and an out-of-frame transcript. However, protein of normal molecular weight but in reduced amounts was observed on Western Blot analysis. Reverse transcription analysis on muscle RNA showed production, via alternative splicing, of a transcript missing exon 11 as well as a low abundant full-length transcript which is enough to avoid the severe Duchenne phenotype. Our study showed that a reduced amount of full length dystrophin leads to a mild form of Becker muscular dystrophy. These results confirm earlier findings that low amounts of dystrophin can be associated with a milder phenotype, which is promising for therapies aiming at dystrophin restoration. Copyright © 2016 Elsevier B.V. All rights reserved.

  2. ["Skin reducing mastectomy" and immediate breast reconstruction with Becker 35 contour profile breast implant: our experience].

    Science.gov (United States)

    Chiummariello, S; Arleo, S; Pataia, E; Iera, M; Alfano, C

    2012-02-01

    We describe in this paper our experience with the technique of skin-reducing mastectomy in the treatment of breast cancer. Between October 2005 and April 2010 in our Center 33 patients underwent breast surgery utilizing the skin-reducing mastectomy technique and immediate breast reconstruction with expansor/definitive implant Becker 35 contour profile. Contralateral symmetrization was performed in the same operation. All selected patients satisfied inclusion critera either for nipple-sparing mastectomy or prophylactic mastectomy. We selected patients who had large ptosic breasts: areola-submammary fold >8 cm and jugular-nipple distance >25 cm. After histologic exam to confirm the lack of retroareolar tissue infiltration the nipple was preserved in a superior flap. Twenty-four out of 33 patients (72.7%) did not suffer from complications. In 9 patients (27.3%) we observed early complications. During follow-up we observed in 24 patients (72.7%) Baker I capsular contracture, in 9 patients (27.3%) Baker II capsular contracture. Esthetic results of symmetry, shape and volume were good to optimal. Patients' satisfaction was similar. During the follow up (6-33 months, average 17.7 months) no patient suffered from breast cancer recurrence. The "skin-reducing mastectomy" reduces mutilation and unsightly scar visibility. It gives a pleasant aesthetic outcome without hindering oncological safety.

  3. Duchenne or Becker muscular dystrophy: A clinical, genetic and immunohistochemical study in China

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    Qian Wang

    2011-01-01

    Full Text Available Background and Objective: Duchenne and Becker muscular dystrophies are X-linked diseases caused by mutations in the dystrophin gene, which affect approximately 1 in 3,500 and 1 in 18,000 boys, respectively. The aim of this work was to develop a method to assist the diagnosis and classification of the disease. Materials and Methods: A large data set of dystrophin mutations was detected in 167 Chinese patients by multiplex ligation-dependent probe amplification and sequencing. Muscle biopsy, immunohistochemistry and STR analysis were also carried out in the patients and carriers. Results: One hundred and three deletions, 23 duplications and two-point mutations. The deletion of one or more exons was detected in 103 (61.7% patients. The region spanning exons 44-55 was the most frequent deletion. The duplication was identified in 23 (13.8% patients, which was more common than previously reported. Most duplications were found in exons 2-18. Six out of the 45 muscle biopsies analyzed showed the presence of other muscle diseases. Conclusions: This study may be important to enable comparisons of mutation type and the most appropriate analytical approach for samples from different geographical areas and ethnicities.

  4. Serum Creatinine Level: A Supplemental Index to Distinguish Duchenne Muscular Dystrophy from Becker Muscular Dystrophy

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    Huili Zhang

    2015-01-01

    Full Text Available Background. To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn level reflects disease severity. Methods. Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy. Results. Serum Crn level had a strong inverse correlation with Vignos score by simple correlation (r=-0.793 and partial correlation analysis after adjustment for age, height, and weight (r=-0.791; both P<0.01. Serum Crn level was significantly higher in patients with in-frame than out-of-frame mutations (Z=-4.716, P<0.01 and in Becker muscular dystrophy (BMD patients than Duchenne muscular dystrophy (DMD patients at ages 4, 5, 7, and 9 yr (all P<0.0125. After adjusting for age, height, and weight, BMD patients still had a significantly higher serum Crn level than DMD patients (β=7.140, t=6.277, P<0.01. Conclusions. Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.

  5. "Molecular Analysis of Iranian Patients with Duchenne/Becker Muscular Dystrophies"

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    S Kheradmand kia

    2003-09-01

    Full Text Available Duchenne Muscular Dystrophy (DMD and the milder allelic Becker Muscular Dystrophy (BMD are X-linked disorders. Both DMD & BMD result from heterogenous mutation in the dystrophin gene and in about 65% of the cases one or more exons of the gene are deleted or duplicated. One third of cases arise from new mutation and the rest are familial. To analyze the prevalence of deletion in Iranian patients, a deletion screening was performed on group 18 exons of dystrophin gene. Deletions were detected in 56.8% of patients. Seventy four percent of deleted exons were located in the major hot spot region, whereas 26% were in the minor hot spot one. The most frequently deleted exons were exons 50, 48 & 47 16.2%, 16.2% & 12% respectively. No deletion was detected in exon 43. The intragenic RFLP analysis (pERT87-15/BamHI & pERT87-8/Taql were carried out on DNA samples obtained from 22 Iranian unrelated families (196 males & females showing DMD & BMD clinical symptoms, that 45% of them had informative patterns. The percentage of heterozygosity was 22.75% for BamHl intragenic RFLP, and 22.75% for Taql intragenic RFLP.

  6. Molecular Diagnosis of Duchenne/Becker Muscular Dystrophy: Analysis of Exons Deletion and Carrier Detection

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    Mohammad Taghi Akbari

    2010-01-01

    Full Text Available Objective: Duchenne and Becker Muscular Dystrophy (DMD and BMD are X-linked conditionsresulting from a defect in the dystrophin gene located at Xp21.2. DMD is the mostfrequent neuromuscular disease in humans (1/3500 male newborns. In approximately65% of DMD and BMD patients, deletions in the dystrophin gene have been identified asthe molecular determinant. The frequency and distribution of dystrophin gene deletions inDMD/BMD patients from different populations are different.The aim of this study was to delineate various types of deleted exons and their frequencyin affected male patients and identification of carrier females by linkage analysis.Materials and Methods: In this study 100 unrelated patients with DMD/BMD were studiedfor intragenic deletions in 28 exons and the promoter region of the dystrophin geneusing multiplex PCR. We also performed linkage analysis within the dystrophin gene utilizing8 short tandem repeat markers.Results: Fifty-two (52% patients showed intragenic deletions. A total of 81% of the deletionswere located at the distal hot spot region (44-55 exons and 19% of the deletionswere located at the proximal region (exon 2-19. The most frequent deleted exons were47(16%, 48 and 46 (11%.Most of the STR markers showed heterozygosity in the families studied. The linkageanalysis was useful for detecting carrier status.Conclusion: The present study suggests that intragenic dystrophin gene deletions occurwith the same frequency in Iranian patients compared with other ethnic groups.

  7. Fluorescent multiplex linkage analysis and carrier detection for Duchenne/Becker muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Schwartz, L.S.; Hoffman, E.P. (Univ. of Pittsburgh Schoool of Medicine, Pittsburgh, PA (United States)); Tarleton, J. (Self Memorial Hospital, Greenwood, SC (United States)); Popovich, B. (Children' s Hosptial and Health Center, San Diego, CA (United States)); Seltzer, W.K. (Univ. of Colorado Health Sciences Center, Denver, CO (United States))

    1992-10-01

    The authors have developed a fast and accurate PCR-based linkage and carrier detection protocol for families of Duchenne muscular dystrophy (DMD)/Becker muscular dystrophy (BMD) patients with or without detectable deletions of the dystrophin gene, using fluorescent PCR products analyzed on an automated sequencer. When a deletion is found in the affected male DMD/BMD patient by standard multiplex PCR, fluorescently labeled primers specific for the deleted and nondeleted exon(s) are used to amplify the DNA of at-risk female relatives by using multiplex PCR at low cycle number (20 cycles). The products are then quantitatively analyzed on an automatic sequencer to determine whether they are heterozygous for the deletion and thus are carriers. As a confirmation of the deletion data, and in cases in which a deletion is not found in the proband, fluorescent multiplex PCR linkage is done by using four previously described polymorphic dinucleotide sequences. The four (CA)[sub n] repeats are located throughout the dystrophin gene, making the analysis highly informative and accurate. The authors present the successful application of this protocol in families who proved refractory to more traditional analyses. 22 refs., 3 figs.

  8. Genetic polymorphism in muscle biopsies of Duchenne and Becker muscular dystrophy patients.

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    Anand A

    1999-07-01

    Full Text Available Duchenne muscular dystrophy (DMD, with an incidence of one in 3500 male new borns, and its milder variant, Becker muscular dystrophy (BMD, are allelic X-linked recessive disorders, caused by mutations in the gene coding for dystrophin, a 427 kD cytoskeleton protein. There are no available molecular markers to differentiate these two. The purpose of this study was to study genetic polymorphism in muscular dystrophy and explore its potential in discriminating these two allelic forms of the disease. The results revealed unambiguously the presence of three transcripts : 598bp, 849bp and 1583bp long which are selectively expressed in the muscles afflicted with muscular dystrophy as compared to the normal muscle. 1583bp gene transcript was conspicuously present in the muscle tissues of both DMD and BMD patients whereas 598bp and 849bp long transcripts were exclusively present in DMD but not in BMD patients or normal human subjects. These gene transcripts had no sequence homology with dystrophin gene and these were also present in the families belonging to DMD and BMD patients. These results point to the fact that based upon the selective expression of these three gene transcripts, one could not only differentiate between DMD and BMD diseases at the molecular level, but also between normal and dystrophic muscle. Further, these findings also reveal that apart from dystrophin gene, these gene transcripts may also be responsible for the differential progression of DMD/BMD phenotype.

  9. A case of Becker muscular dystrophy with early manifestation of cardiomyopathy

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    Ki Hyun Doo

    2012-09-01

    Full Text Available An 18-year-old boy was admitted with chest discomfort, nausea, and dyspnea at rest. At the age of 3 years, he underwent muscle biopsy and dystrophin gene analysis owing to an enlarged calf muscle and elevated serum kinase level (6,378 U/L without overt weakness; based on the results, Becker muscular dystrophy (BMD was diagnosed. The dystrophin gene showed deletion of exons 45 to 49. He remained ambulant and could step upstairs without significant difficulties. A chest roentgenogram showed cardiomegaly (cardiothoracic ratio, 54%, and his electrocardiogram (ECG showed abnormal ST-T wave, biatrial enlargement, and left ventricular hypertrophy. The 2-dimensional and M-mode ECGs showed a severely dilated left ventricular cavity with diffuse hypokinesis. The systolic indices were reduced, including fractional shortening (9% and ejection fraction (19%. Despite receiving intensive medical treatment, he died from congestive heart failure 5 months after the initial cardiac symptoms. We report a case of BMD with early-onset dilated cardiomyopathy associated with deletion of exons 45 to 49. Early cardiomyopathy can occur in BMD patients with certain genotypes; therefore, careful follow-up is required even in patients with mild phenotypes of BMD.

  10. Dystrophin and the two related genetic diseases, Duchenne and Becker muscular dystrophies

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    Elisabeth Le Rumeur

    2015-07-01

    Full Text Available Mutations of the dystrophin DMD gene, essentially deletions of one or several exons, are the cause of two devastating and to date incurable diseases, Duchenne (DMD and Becker (BMD muscular dystrophies. Depending upon the preservation or not of the reading frame, dystrophin is completely absent in DMD, or present in either a mutated or a truncated form in BMD. DMD is a severe disease which leads to a premature death of the patients. Therapy approaches are evolving with the aim to transform the severe DMD in the BMD form of the disease by restoring the expression of a mutated or truncated dystrophin. These therapies are based on the assumption that BMD is a mild disease. However, this is not completely true as BMD patients are more or less severely affected and no molecular basis of this heterogeneity of the BMD form of the disease is yet understood. The aim of this review is to report for the correlation between dystrophin structures in BMD deletions in view of this heterogeneity and to emphasize that examining BMD patients in details is highly relevant to anticipate for DMD therapy effects.

  11. Dystrophin hydrophobic regions in the pathogenesis of Duchenne and Becker muscular dystrophies

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    Yingyin Liang

    2015-05-01

    Full Text Available The aim of our study was to determine the role of dystrophin hydrophobic regions in the pathogenesis of Duchenne (DMD and Becker (BMD muscular dystrophies, by the Kyte-Doolittle scale mean hydrophobicity profile and 3D molecular models. A total of 1038 cases diagnosed with DMD or BMD with the in-frame mutation were collected in our hospital and the Leiden DMD information database in the period 2002-2013. Correlation between clinical types and genotypes were determined on the basis of these two sources. In addition, the Kyte-Doolittle scale mean hydrophobicity of dystrophin was analyzed using BioEdit software and the models of the hydrophobic domains of dystrophin were constructed. The presence of four hydrophobic regions is confirmed. They include the calponin homology CH2 domain on the actin-binding domain (ABD, spectrin-type repeat 16, hinge III and the EF Hand domain. The severe symptoms of DMD usually develop as a result of the mutational disruption in the hydrophobic regions I, II and IV of dystrophin – those that bind associated proteins of the dystrophin-glycoprotein complex (DGC. On the other hand, when the hydrophobic region III is deleted, the connection of the ordered repeat domains of the central rod domain remains intact, resulting in the less severe clinical presentation. We conclude that mutational changes in the structure of hydrophobic regions of dystrophin play an important role in the pathogenesis of DMD.

  12. A novel splice site mutation in a Becker muscular dystrophy patient.

    Science.gov (United States)

    Bartolo, C; Papp, A C; Snyder, P J; Sedra, M S; Burghes, A H; Hall, C D; Mendell, J R; Prior, T W

    1996-04-01

    A Becker muscular dystrophy patient was found to have a single base substitution at the 5' end of intron 54. This single base substitution disrupts the invariant GT dinucleotide within the 5' donor splice site and was shown to cause an out of frame deletion of exon 54 during mRNA processing. This is predicted to produce a truncated dystrophin protein which is more consistent with a DMD phenotype. However, small quantities of normal mRNA are also transcribed and these are sufficient to produce a reduced amount of normal molecular weight dystrophin and give rise to a milder BMD phenotype. This indicates that a single base substitution at an invariant dinucleotide of the splice site consensus sequence may still allow read through of the message and allow the production of some normal protein. This shows that there are a greater number of possible intronic mutations that can lead to a mild phenotype and it also underlines the importance of performing cDNA analysis when screening for small gene alterations in the BMD patient population.

  13. Duchenne's/Becker's muscular dystrophy: Analysis of genotype-feno-type correlation in 28 patients

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    Keckarević Milica

    2002-01-01

    Full Text Available Duchenne's and Becker's muscular dystrophy (DMD & BMD is a X linked disease caused by mutations in the dystrophic gene. DMD is the malign form of the disease, which significantly shortens the lifetime of the patient, while BMD has late onset with slow progression. Sixty five percent of DMD and BMD cases are caused by deletion of one or more exons in the dystrophic gene, while duplications cause these diseases in 6 to 7% of the cases. There are two hot spots for deletions and duplications. These are exons in the proximal part of the gene (3rd to 18th and exons of a distal part of the gene (45th to 52nd. The remaining 30% of DMD and BMD cases are caused by point mutations, small deletions or inversions in the dystrophic gene. The correlation between School of Medicine, University of Belgrade, Belgrade the severity of the disease and the position of deletion shows that most of the out of frame deletions cause DMD phenotype, while in frame deletions result in BMD pheno-type. We report on the results of 28 non-related DMD and BMD patients. In 57% of cases deletions were detected and all were found in the distal hot spot of the gene. These results suggest that in most of the cases, out of frame deletions produce DMD phenotype while in frame deletions result in BMD phenotype. This is in compliance with data from literature.

  14. Influence of environmental factors on composition of phenolic antioxidants of Achillea collina Becker ex Rchb.

    Science.gov (United States)

    Giorgi, Annamaria; Madeo, Moira; Speranza, Giovanna; Cocucci, Maurizio

    2010-10-01

    Effects of environmental growth conditions on the antioxidant capacity, total phenolic content and composition of Achillea collina Becker ex Rchb. were investigated. Methanol extracts and infusions obtained from leaves and inflorescences of plants cultivated in the Italian Alps at two different altitudes (600 and 1050 m a.s.l.) were evaluated. Infusions exhibited the highest antioxidant capacity (1/IC(50) values from 4.35 ± 0.72 to 8.90 ± 0.74), total phenolic content (from 31.39 ± 4.92 to 49.36 ± 5.70 mg gallic acid equivalents (GAE) g(-1) DW), chlorogenic acid (from 9.21 ± 1.52 to 31.27 ± 6.88 mg g(-1) DW), 3,5-di-O-caffeoylquinic acid (from 12.28 ± 3.25 to 25.13 ± 1.99 mg g(-1) DW) and 4,5-di-O-caffeoylquinic acid (from 7.38 ± 1.01 to 12.78 ± 2.61 mg g(-1) DW) content. Climate (as influenced by altitude) was shown to be the main environmental factor influencing yarrow composition and properties. Leaf extracts from the higher experimental site showed a 2-4-fold increase of chlorogenic acid level. Achillea collina can be considered as a very good source of bioactive phenolic compounds, and growing it at high altitude may constitute an effective way to significantly enhance its quality for both medicinal and nutritional uses.

  15. Clinical study of DMD gene point mutation causing Becker muscular dystrophy

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    Ji-qing CAO

    2015-07-01

    Full Text Available Background  DMD gene point mutation, mainly nonsense mutation, always cause the most severe Duchenne muscular dystrophy (DMD. However, we also observed some cases of Becker muscular dystrophy (BMD carrying DMD point mutation. This paper aims to explore the mechanism of DMD point mutation causing BMD, in order to enhance the understanding of mutation types of BMD.  Methods  Sequence analysis was performed in 11 cases of BMD confirmed by typical clinical manifestations and muscle biopsy. The exon of DMD gene was detected non-deletion or duplication by multiplex ligation-dependent probe amplification (MLPA.  Results  Eleven patients carried 10 mutation types without mutational hotspot. Six patients carried nonsense mutations [c.5002G>T, p.(Glu1668X; c.1615C > T, p.(Arg539X; c.7105G > T, p.(Glu2369X; c.5287C > T, p.(Arg1763X; c.9284T > G, p.(Leu3095X]. One patient carried missense mutation [c.5234G > A, p.(Arg1745His]. Two patients carried frameshift mutations (c.10231dupT, c.10491delC. Two patients carried splicing site mutations (c.4518 + 3A > T, c.649 + 2T > C.  Conclusions  DMD gene point mutation may result in BMD with mild clinical symptoms. When clinical manifestations suggest the possibility of BMD and MLPA reveals non?deletion or duplication mutation of DMD gene, BMD should be considered. Study on the mechanism of DMD point mutation causing BMD is very important for gene therapy of DMD. DOI: 10.3969/j.issn.1672-6731.2015.06.005

  16. Los mundos de Howard Becker : Becker, H. (2008). Los mundos del arte. Sociología del trabajo artístico. Buenos Aires: Universidad Nacional de Quilmes.

    OpenAIRE

    Aliano, Nicolás

    2011-01-01

    Podríamos afirmar que la publicación en español del ya clásico Art Worlds (1982) de Howard Becker (Chicago, 1928), viene a renovar la algo entumecida pregunta por la "identidad" en el ámbito de la sociología de la cultura. Digo "podríamos" porque en verdad el libro no viene a darnos una respuesta a esa pregunta, sino más bien a abrir otra serie de interrogantes en un cambio de eje: un desplazamiento, digamos, desde las "cuestiones de identidad" a las "cuestiones de organización". En este sent...

  17. In the aftermath of State v. Becker: a review of state and federal jury instructions on insanity acquittal disposition.

    Science.gov (United States)

    Piel, Jennifer

    2012-01-01

    An important topic related to the insanity defense is what jurors should be told about the disposition of a defendant found not guilty by reason of insanity (NGRI). In the federal court system, jurors are not instructed about the consequences of an NGRI verdict. State courts, however, are divided on the question. The federal precedent, Shannon v. United States, and the most recent state case to rule on NGRI juror instructions, State v. Becker, are reviewed in detail. What follows is the author's critique of the principal arguments for and against a jury instruction on NGRI disposition. The author argues in favor of a jury instruction on the consequences of an NGRI verdict.

  18. Notas sobre la microsociología y el cuerpo. Las microcorporalidades de Becker, Scott, Bourdieu y de Certeau

    OpenAIRE

    Eugenia Fraga

    2016-01-01

    En el presente trabajo nos interesa indagar en el modo en que la perspectiva microsociológica ha pensado el tema del cuerpo. Queremos ver de qué formas, autores tan variados y a la vez tan afines como Howard Becker, James Scott, Pierre Bourdieu y Michel de Certeau, herederos en distinta medida del interaccionismo simbólico de George Mead, han concebido aquella dimensión tan central –y a veces tan soslayada– de la vida social como es la corporalidad. Para ello deberemos, en primer lugar, profu...

  19. Cardiac involvement of progressive muscular dystrophy (Becker type, Limb-girdle type and Fukuyama type) evaluated by radionuclide method

    Energy Technology Data Exchange (ETDEWEB)

    Nagamachi, Shigeki; Inoue, Kenjiro; Jinnouchi, Seishi; Hoshi, Hiroaki; Ono, Seiji; Ohnishi, Takashi; Futami, Shigemi; Watanabe, Katsushi; Hayashi, Tohru (Miyazaki Medical Coll., Kiyotake (Japan))

    1994-02-01

    Tl-201 SPECT and Tc-99m-Human serum albumin (HSA) multigated radionuclide ventriculography were performed on 11 patients with progressive muscular dystrophy (Becker type 2, Fukuyama type 2, Limb-girdle type 7) to evaluate myocardial involvement. Hypoperfusion was detected in 8 patients on Tl-201 SPECT. Decreases in both systolic function (left ventricular ejection fraction; LVEF) and diastolic function (peak filling rate; PFR) were also seen in these patients. A high incidence of myocardial involvement of these kinds of progressive muscular dystrophy was suggested. (author).

  20. Becker's Happy Life in Shenyang%贝克在沈阳生活得很快乐

    Institute of Scientific and Technical Information of China (English)

    博彦

    2010-01-01

    @@ 来自德国的贝克先生(Ralf Becker)5年前就曾经来过中国的上海,给他留下深刻的印象.从那时起他心中埋下了一个心愿,"如果有机会还会再来中国的".让他没有想到的,短短两年后的2007年,贝克先生果真又重新踏上了中国这片向往的土地.

  1. Estimated ground-water use in Becker, Clay, Douglas, Grant, Otter Tail, and Wilkin Counties, Minnesota, for 2030 and 2050

    Science.gov (United States)

    Winterstein, Thomas A.

    2007-01-01

    The U.S. Department of the Interior, Bureau of Reclamation, is studying six alternatives for delivering water to the Red River of the North Valley in North Dakota and to the cities of Breckenridge, Moorhead, and East Grand Forks, Minnesota. In order to evaluate these alternatives the Bureau of Reclamation needs estimates of ground-water use for 2030 and 2050 for six counties in Minnesota: Becker, Clay, Douglas, Grant, Otter Tail, and Wilkin Counties. The U.S. Geological Survey, in cooperation with the Bureau of Reclamation, conducted a study to estimate ground-water use in these counties for 2030 and 2050.

  2. Comment on "Carbon farming in hot, dry coastal areas: an option for climate change mitigation" by Becker et al. (2013)

    OpenAIRE

    M. Heimann

    2014-01-01

    Becker et al. (2013) argue that an afforestation of 0.73 × 109 ha with Jatropha curcas plants would generate an additional terrestrial carbon sink of 4.3 PgC yr−1, enough to stabilise the atmospheric mixing ratio of carbon dioxide (CO2) at current levels. However, this is not consistent with the dynamics of the global carbon cycle. Using a well-established global carbon cycle model, the effect of adding such a hypothetical sink leads to a reduction of atmospheric CO2 levels ...

  3. Identificación de mutaciones y diagnóstico molecular de portadoras en familias mexicanas con distrofia muscular Duchenne/Becker

    OpenAIRE

    S. Canizales; Salamanca, F.; GarcÍa, N.; D. Arenas

    2008-01-01

    Introducción: La Distrofia muscular de Duchenne/Becker (DMD/BMD) es la miopatía hereditaria más frecuente en las poblaciones humanas. Se caracteriza por una debilidad muscular progresiva que ocasiona, para el tipo Duchenne, la muerte por falla cardiaca y/o respiratoria durante la segunda década de la vida. Para el tipo Becker las alteraciones musculares son menos severas y los pacientes generalmente sobreviven hasta la edad adulta. El gen DMD, responsable de la enfermedad, se localiza en el b...

  4. Atualização do tratamento fisioterapêutico das distrofias musculares de Duchenne e de Becker - doi:10.5020/18061230.2005.p41

    OpenAIRE

    Simone Rizzo Nique da Silva; Sílvia Lemos Fagundes

    2012-01-01

    As distrofias musculares de Duchenne (DMD) e de Becker (DMB) consistem em distúrbios neuromusculares genéticos de herança autossômica recessiva, ligados ao cromossomo X, incuráveis e progressivos. O papel do profissional fisioterapeuta diante desses agravos ainda se encontra muito segmentário, com opiniões e condutas bastante controversas. Este estudo consiste em uma revisão bibliográfica sobre o tratamento fisioterapêutico das distrofias de Duchenne (DMD) e de Becker (DMB) e pretende evidenc...

  5. A comprehensive database of Duchenne and Becker muscular dystrophy patients in Children's Hospital of Fudan University

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    Xi-hua LI

    2015-05-01

    Full Text Available Background China is one of the countries that have the largest number of patients suffering from Duchenne and Becker muscular dystrophy (DMD/BMD. Although the building of international DMD/BMD databases has laid a foundation for clinical drug development and clinical trials, it has not yet been carried out in China. In this study, a modified registry form of Remudy was applied to 229 DMD/BMD patients in order to establish a comprehensive database, which will lay the groundwork for international cooperation.  Methods A total of 229 DMD/BMD patients diagnosed by genetic testing or muscle biopsy admitted in Children's Hospital of Fudan University (CHFU during the period of August 2011 to December 2013 were enrolled in this study. The data included sex, age, age at diagnosis, geographic distribution of patients, DMD gene mutation types, family history, walking capability, cardiac and respiratory function, steroid treatment and rehabilitation intervention.  Results There were 194 DMD and 35 BMD male patients who were diagnosed at the age of 0-18 years, and among them, most patients were diagnosed at the age of > 3-4 (16.59%, 38/229 and > 7-8 (14.85%, 34/229 years. Exon deletion was the most frequent genetic mutations for DMD/BMD [65.46% (127/194 and 74.29% (26/35], respectively. Patients with a family history accounted for 23.14% (53/229. The rate of DMD registrants losing walking capability was 17.53% (34/194, and all the BMD registrants were able to walk. Cardiac functions were examined in 46.29% (106/229 DMD/BMD boys and respiratory functions were examined in 17.90% (41/229 DMD/BMD boys. The proportion of DMD patients receiving prednisone with dosage of 0.75 mg/(kg·d was 26.29% (51/194.  Conclusions This database describes in detail the genotype, clinical manifestation, diagnosis and treatment and rehabilitation status of 229 DMD/BMD patients in China. The database not only provides comprehensive information for DMD/BMD patient management

  6. Becker Acroma贝格罗马在亚洲市场采取的新举措

    Institute of Scientific and Technical Information of China (English)

    李旭汕

    2006-01-01

    随着Becker Acroma贝格罗马近几年在亚洲和中国市场的长足发展,准备进一步采取新举措来实现持续性的增长。其在青岛的代表处进行了扩充,搬迁了新址,并且还在青岛建立了迄今为止亚洲区的第一家工厂。经过扩充的Becker Acroma贝格罗马青岛新厂区,位于青岛株洲路高新技术开发区,厂区包括仓库、办公室、生产单元、技术实验室和其在亚洲区第一家技术中心。还将建立仓库、技术实验室、办公室、亚洲第一间技术中心等设施。

  7. Read-through strategies for suppression of nonsense mutations in Duchenne/ Becker muscular dystrophy: aminoglycosides and ataluren (PTC124).

    Science.gov (United States)

    Finkel, Richard S

    2010-09-01

    Nucleotide changes within an exon can alter the trinucleotide normally encoding a particular amino acid, such that a new ''stop'' signal is transcribed into the mRNA open reading frame. This causes the ribosome to prematurely terminate its reading of the mRNA, leading to nonsense-mediated decay of the transcript and lack of production of a normal full-length protein. Such premature termination codon mutations occur in an estimated 10% to 15% of many genetically based disorders, including Duchenne/Becker muscular dystrophy. Therapeutic strategies have been developed to induce ribosomal read-through of nonsense mutations in mRNA and allow production of a full-length functional protein. Small-molecule drugs (aminoglycosides and ataluren [PTC124]) have been developed and are in clinical testing in patients with nonsense mutations within the dystrophin gene. Use of nonsense mutation suppression in Duchenne/Becker muscular dystrophy may offer the prospect of targeting the specific mutation causing the disease and correcting the fundamental pathophysiology.

  8. Readthrough Strategies for Suppression of Nonsense Mutations in Duchenne/Becker Muscular Dystrophy: Aminoglycosides and Ataluren (PTC124)

    Science.gov (United States)

    Finkel, Richard S.

    2013-01-01

    Nucleotide changes within an exon may alter the trinucleotide normally encoding a particular amino acid, such that a new “stop” signal is transcribed into the mRNA open reading frame. This causes the ribosome to prematurely terminate its reading of the mRNA, leading to nonsense-mediated decay of the transcript and lack of production of a normal full-length protein. Such premature termination codon mutations occur in an estimated 10% to 15% of many genetically based disorders, including Duchenne/Becker muscular dystrophy. Therapeutic strategies have been developed to induce ribosomal readthrough of nonsense mutations in mRNA and allow production of a full-length functional protein. Small molecule drugs (aminoglycosides and ataluren [PTC124]) have been developed and are in clinical testing in patients with nonsense mutations within the dystrophin gene. Use of nonsense mutation suppression in Duchenne/Becker muscular dystrophy may offer the prospect of targeting the specific mutation causing the disease and correcting the fundamental pathophysiology. PMID:20519671

  9. Three new species of Rectiostoma Becker, 1982 (Lepidoptera: Gelechioidea: Depressariidae) from Area de Conservación Guanacaste, northwestern Costa Rica

    Science.gov (United States)

    We describe three new species of Rectiostoma Becker, 1982 from Costa Rica: R. annemayae Heikkilä and Metz spec. nov., R. eowilsoni Heikkilä and Metz spec. nov. and R. philipmayi Heikkilä and Metz spec. nov. We used a data set of DNA COI-barcodes accumulated for Lepidoptera collected at Area de Conse...

  10. More deletions in the 5{prime} region than in the central region of the dystrophin gene were identified among Filipino Duchenne and Becker muscular dystrophy patients

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1995-11-06

    This report describes mutations in the dystrophin gene and the frequency of these mutations in Filipino pedigrees with Duchenne and Becker muscular dystrophy (DMD/BMD). The findings suggest the presence of genetic variability among DMD/BMD patients in different populations. 13 refs., 1 tab.

  11. Deleciones en el gen de la distrofina en 62 familias colombianas: correlación genotipo-fenotipo para la distrofia muscular de Duchenne y Becker

    National Research Council Canada - National Science Library

    Claudia T. Silva; Dora Fonseca; Carlos Martín Restrepo; Nora C. Contreras; Heidi E. Mateus

    2009-01-01

    INTRODUCCIÓN: La correlación genotipo-fenotipo se estableció mediante el análisis de deleciones del gen de la distrofina en pacientes con distrofia muscular de Duchenne y Becker (DMD/DMB). OBJETIVOS...

  12. Treinamento físico na distrofia muscular de becker associada à insuficiência cardíaca

    Directory of Open Access Journals (Sweden)

    Jean Marcelo Roque

    2011-12-01

    Full Text Available A distrofia muscular de Becker (DMB integra as distrofinopatias que ocorrem devido a mutações genéticas que expressam a proteína distrofina no cromossomo X. O início dos sintomas neuromusculares normalmente precede o comprometimento da função cardíaca, podendo acontecer inversamente pela insuficiência cardíaca (IC. O treinamento físico é bem estabelecido na IC, porém, quando associada à DMB, é controverso e sem fundamento científico. Apresentamos o caso de um paciente com DMB associada à IC em fila de transplante cardíaco submetido a um programa de treinamento físico.

  13. Germinal mosaicism in a sample of families with Duchenne/Becker muscular dystrophy with partial deletions in the DMD gene.

    Science.gov (United States)

    Bermúdez-López, Cesárea; García-de Teresa, Benilde; González-del Angel, Ariadna; Alcántara-Ortigoza, Miguel Angel

    2014-02-01

    Germinal mosaicism should be considered when estimating the recurrence risk in families with Duchenne/Becker muscular dystrophy (D/BMD). Germinal mosaicism, however, has not been assessed in Mexican families with deletions in the DMD gene. To determine the distribution of deletions in the two hot spots and the proportion of de novo and transmitted deletions, we analyzed 153 individuals with D/BMD and a DMD partial deletion and 322 of their maternal female relatives. Predilection for the distal hot spot was observed in 112 families (73%), while gene dosage analysis of female relatives of D/BMD patients identified germinal mosaicism deletions in at least 11.6% of the patients' families, thought to result from de novo mutations. Recurrence risk due to germinal mosaicism justifies carrier detection in maternal female relatives and prenatal diagnosis in mothers of individuals with apparently de novo DMD deletions.

  14. Evaluation of point mutations in dystrophin gene in Iranian Duchenne and Becker muscular dystrophy patients: introducing three novel variants

    Indian Academy of Sciences (India)

    MARYAM HAGHSHENAS; MOHAMMAD TAGHI AKBARI; SHOHREH ZARE KARIZI; FARAVAREH KHORDADPOOR DEILAMANI; SHAHRIAR NAFISSI; ZIVAR SALEHI

    2016-06-01

    Duchenne and Becker muscular dystrophies (DMD and BMD) are X-linked neuromuscular diseases characterized by progres-sive muscular weakness and degeneration of skeletal muscles. Approximately two-thirds of the patients have large deletionsor duplications in the dystrophin gene and the remaining one-third have point mutations. This study was performed to eval-uate point mutations in Iranian DMD/BMD male patients. A total of 29 DNA samples from patients who did not show anylarge deletion/duplication mutations following multiplex polymerase chain reaction (PCR) and multiplex ligation-dependentprobe amplification (MLPA) screening were sequenced for detection of point mutations in exons 50–79. Also exon 44 wassequenced in one sample in which a false positive deletion was detected by MLPA method. Cycle sequencing revealed fournonsense, one frameshift and two splice site mutations as well as two missense variants

  15. Mutation analysis in Duchenne and Becker muscular dystrophy patients from Bulgaria shows a peculiar distribution of breakpoints by intron

    Energy Technology Data Exchange (ETDEWEB)

    Todorova, A.; Bronzova, J.; Kremensky, I. [Univ. Hospital of Obstetrics and Gynecology, Sofia (Bulgaria)] [and others

    1996-10-02

    For the first time in Bulgaria, a deletion/duplication screening was performed on a group of 84 unrelated Duchenne/Becker muscular dystrophy patients, and the breakpoint distribution in the dystrophin gene was analyzed. Intragenic deletions were detected in 67.8% of patients, and intragenic duplications in 2.4%. A peculiar distribution of deletion breakpoints was found. Only 13.2% of the deletion breakpoints fell in the {open_quotes}classical{close_quotes} hot spot in intron 44, whereas the majority (> 54%) were located within the segment encompassing introns 45-51, which includes intron 50, the richest in breakpoints (16%) in the Bulgarian sample. Comparison with data from Greece and Turkey points at the probable existence of a deletion hot spot within intron 50, which might be a characteristic of populations of the Balkan region. 17 refs., 2 figs.

  16. Adequate managment of patients with dystrophinopathies (muscular dystrophy Duchenne/Becker: objective scales and additional diagnostic methods

    Directory of Open Access Journals (Sweden)

    A. S. Nosko

    2014-01-01

    Full Text Available There are still no guidlines on managment of Duchenne/Becker myodystrophy in domestic medical practice. It leads to decrease of quality of life and, what is more important, lifespan of patients. In this article we have described our Western coleagues lаst decade experience, including consensus guidelines published in 2010 on mаnаgment of Duchenne myodystrophy, supplemented with our practicle experience. We have described standardized motor development scale and muscle tone score for patients with MDD/MDB, and algorithm of multidiscipline care with focus on prevention, diagnosis and treatment of main disease and steroid therapy complications: cardiovascular, orthopedics, respirator etc. These recommendations not only improve quality of live and extend lifespan of MDD/MDB patients, but allow to take part in multicentre trials on searching of pathognomonic and symptomatic treatment.

  17. Evaluation of point mutations in dystrophin gene in Iranian Duchenne and Becker muscular dystrophy patients: introducing three novel variants.

    Science.gov (United States)

    Haghshenas, Maryam; Akbari, Mohammad Taghi; Karizi, Shohreh Zare; Deilamani, Faravareh Khordadpoor; Nafissi, Shahriar; Salehi, Zivar

    2016-06-01

    Duchenne and Becker muscular dystrophies (DMD and BMD) are X-linked neuromuscular diseases characterized by progressive muscular weakness and degeneration of skeletal muscles. Approximately two-thirds of the patients have large deletions or duplications in the dystrophin gene and the remaining one-third have point mutations. This study was performed to evaluate point mutations in Iranian DMD/BMD male patients. A total of 29 DNA samples from patients who did not show any large deletion/duplication mutations following multiplex polymerase chain reaction (PCR) and multiplex ligation-dependent probe amplification (MLPA) screening were sequenced for detection of point mutations in exons 50-79. Also exon 44 was sequenced in one sample in which a false positive deletion was detected by MLPA method. Cycle sequencing revealed four nonsense, one frameshift and two splice site mutations as well as two missense variants.

  18. A new species of Leptometopa Becker, 1903 (Diptera, Milichiidae and an identification key for the neotropical species of the genus

    Directory of Open Access Journals (Sweden)

    Ramon Luciano de Mello

    2007-01-01

    Full Text Available The Milichiidae family includes species of small acalyptratae flies distributed into 19 genera, over all biogeographic regions. The genus Leptometopa Becker, 1903, positioned among the Madizinae, is distributed worldwide. The genus is composed of 19 species, of which three are recorded for the Neotropical region: L. halteralis (Coquillett, 1900, L. latipes (Meigen, 1830 and L. niveipennis (Strobl, 1898. Studying material collected in a cave of Amazonas State, Brazil, the authors found a new species of Leptometopa which is described and illustrated herein. The new species L. veracildae n. sp. represents the first record of the genus in South America. An identification key for all Neotropical species is also presented.A família Milichiidae inclui espécies de pequenas moscas acaliptradas descritas em 19 gêneros, distribuídos em todas as regiões biogeográficas. O gênero Leptometopa Becker, 1903, posicionado entre os Madizinae, é atualmente composto de 19 espécies com distribuição mundial, das quais três são registradas para a região Neotropical: L. halteralis (Coquillett, 1900, L. latipes (Meigen, 1830 e L. niveipennis (Strobl, 1898. Analisando material coletado em uma caverna do estado do Amazonas, Brasil, os autores identificaram uma nova espécie de Leptometopa que é aqui descrita e ilustrada. A nova espécie Leptometopa veracildae sp. nov. representa o primeiro registro do gênero na América do Sul. Também é apresentada uma chave de identificação das espécies neotropicais.

  19. Element storage in spores of Gigaspora margarita Becker & Hall measured by electron energy loss spectroscopy (EELS Elementos de armazenamento em esporos de Gigaspora margarita Becker & Hall medidos por espectroscopia com perda de energia em elétrons (EEES

    Directory of Open Access Journals (Sweden)

    Andre Freire Cruz

    2004-09-01

    Full Text Available The storage structures of spores of Gigaspora margarita Becker & Hall, an arbuscular mycorrhizal fungus, were analyzed by a transmission electronic microscope (TEM and electron energy loss spectroscopy (EELS. The spores were hydrated, cryofixed using high pressure freezing, submitted to freeze substitution, embedded and prepared for TEM observations. The cryotechniques revealed that the storage structures in the spores are composed of lipids, electron dense granules (EDG, protein bodies and glycogen particles. The EELS detected the presence of nitrogen (N and carbon (C in these storage structures and in Bacteria-like organisms (BLO. Some amount of P was detected in the vacuoles and in BLO. The TEM and EELS techniques indicate the presence of storage structures in the fungal spore, and some essential elements (N, P and C in these structures.As estruturas de armazenamento em esporos de Gigaspora margarita Becker & Hall, um fungo micorrízico arbuscular, foram analisadas por microscópio eletrônico de transmissão (MET e espectroscopia com perda de energia em elétrons (EEES. Os esporos foram hidratados, criofixados por pressão em baixa temperatura e submetidos a substituição em congelamento, embebidos e preparados para observações no MET. As técnicas de criofixação revelaram que as estruturas de armazenamento nos esporos são compostas de lipídios, grânulos elétron densos (GED, corpos protéicos e partículas de glicogênio. Por EEES foi detectada a presença de nitrogênio (N e carbono (C nestas estruturas de armazenamento e em organismos similares a bactérias (OSB. Uma pequena quantia de P foi detectada nos vacúolos e nos OSB. O exame por MET e EEES indicaram a presença de estruturas de armazenamento nos esporos e alguns elementos essenciais (N, P e C nessas.

  20. 贝格罗马(Becker Acroma):品质、创新、服务是发展要素

    Institute of Scientific and Technical Information of China (English)

    《国际木业》编辑部

    2008-01-01

    2008年3月12日,在上海DOMTEX Asia展会期间,贝格罗马(Becker Acroma)举行了一次木器漆方面的产品介绍会,为参会的50多名行业内的人士详细介绍了贝格罗马各类优质产品。

  1. Risk Factors Associated With Complication Rates of Becker-Type Expander Implants in Relation to Implant Survival: Review of 314 Implants in 237 Patients.

    Science.gov (United States)

    Taboada-Suarez, Antonio; Brea-García, Beatriz; Magán-Muñoz, Fernando; Couto-González, Iván; González-Álvarez, Eduardo

    2015-12-01

    Although autologous tissue reconstruction is the best option for breast reconstruction, using implants is still a reliable and simple method, offering acceptable aesthetic results. Becker-type implants are permanent implants that offer a 1-stage reconstructive option. A retrospective study was carried out in our center reviewing the clinical reports of 237 patients, in whom a total of 314 Becker-type prostheses were implanted. Overall survival was calculated using a Kaplan-Meier estimate. Cox proportional hazard models were used to calculate adjusted hazard ratios. At the end of the study, 214 expanders (68.15%) presented no complications, 40 (12.47%) developed significant capsular contracture, in 27 (8.60%) infection occurred, 24 (7.64%) suffered minor complications, and 9 (2.87%) ruptured. The mean survival time of the expanders was 120.41 months (95% CI: 109.62, 131.19). Radiotherapy, chemotherapy, high Molecular Immunology Borstel, age, mastectomy performed previously to the implant, ductal carcinoma, advanced tumoral stage, experience of the surgeon, and Becker 35-type implants were significantly related to a high number of complications in relation to the survival of the implants. Cox regression analysis revealed that the main risk factors for the survival of expander implants included radiotherapy and surgeon experience. The complication hazard ratio or relative risk caused by these 2 factors was 1.976 and 1.680, respectively. One-stage reconstruction using Becker-type expanders is an appropriate, simple, and reliable option in delayed breast reconstruction in patients who have not received radiotherapy and as long as the procedure is carried out by surgeons skilled in the technique.

  2. 贝格罗马(Becker Acroma):品质、创新、服务是发展要素

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008年3月12日,在上海DOMTEX Asia展会期间,贝格罗马(Becker Acroma)举行了一次木器漆方面的产品介绍会,为参会的50多名行业内的人士详细介绍了贝格罗马各类优质产品。

  3. Expectations and experiences of investigators and parents involved in a clinical trial for Duchenne/Becker muscular dystrophy.

    Science.gov (United States)

    Peay, Holly L; Tibben, Aad; Fisher, Tyler; Brenna, Ethan; Biesecker, Barbara B

    2014-02-01

    The social context of rare disease research is changing, with increased community engagement around drug development and clinical trials. This engagement may benefit patients and families but may also lead to heightened trial expectations and therapeutic misconception. Clinical investigators are also susceptible to harboring high expectations. Little is known about parental motivations and expectations for clinical trials for rare pediatric disorders. We describe the experience of parents and clinical investigators involved in a phase II clinical trial for Duchenne and Becker muscular dystrophy: their expectations, hopes, motivations, and reactions to the termination of the trial. This qualitative study was based on interviews with clinical investigators and parents of sons with Duchenne and Becker muscular dystrophy (DBMD) who participated in the phase IIa or IIb ataluren clinical trial in the United States. Interviews were transcribed and coded for thematic analysis. Participants were 12 parents of affected boys receiving active drug and 9 clinical investigators. High trial expectations of direct benefit were reported by parents and many clinicians. Investigators described monitoring and managing parents' expectations; several worried about their own involvement in increasing parents' expectations. Most parents were able to differentiate their expectations from their optimistic hopes for a cure. Parents' expectations arose from other parents, advocacy organizations, and the sponsor. All parents reported some degree of clinical benefit to their children. Secondary benefits were hopefulness and powerful feelings associated with active efforts to affect the disease course. Parents and clinical investigators reported strong, close relationships that were mutually important. Parents and clinicians felt valued by the sponsor for the majority of the trial. When the trial abruptly stopped, they described loss of engagement, distress, and feeling unprepared for the

  4. Extension of the analytical kinetics of micellar relaxation: Improving a relation between the Becker-Döring difference equations and their Fokker-Planck approximation

    Science.gov (United States)

    Babintsev, I. A.; Adzhemyan, L. Ts.; Shchekin, A. K.

    2017-08-01

    Relaxation of micellar systems can be described with the help of the Becker-Döring kinetic difference equations for aggregate concentrations. Passing in these equations to continual description, when the aggregation number is considered as continuous variable and the concentration difference is replaced by the concentration differential, allows one to find analytically the eigenvalues (to whom the inverse times of micellar relaxation are related) and eigenfunctions (or the modes of fast relaxation) of the linearized differential operator of the kinetic equation corresponding to the Fokker-Planck approximation. At this the spectrum of eigenvalues appears to be degenerated at some surfactant concentrations. However, as has been recently found by us, there is no such a degeneracy at numerical determination of the eigenvalues of the matrix of coefficients for the linearized difference Becker-Döring equations. It is shown in this work in the frameworks of the perturbation theory, that taking into account the corrections to the kinetic equation produced by second derivatives at transition from differences to differentials and by deviation of the aggregation work from a parabolic form in the vicinity of the work minimum, lifts the degeneracy of eigenvalues and improves markedly the agreement of concentration-dependent fast relaxation time with the results of the numerical solution of the linearized Becker-Döring difference equations.

  5. Pathogenicity of Metarrhizium anisopliae (Metsch.) Sorokin and Beauveria bassiana (Bals.) Vuillemin isolates to Scaptocoris carvalhoi Becker (Hemiptera, Cydnidae); Patogenicidade de isolados de Metarhizium anisopliae (Metsch.) Sorokin e de Beauveria bassiana (Bals.) Vuillemin a Scaptocoris carvalhoi Becker (Hemiptera, Cydnidae)

    Energy Technology Data Exchange (ETDEWEB)

    Xavier, Luciane Modenez Saldivar [Universidade Federal da Grande Dourados (UFGD), Dourados, MS (Brazil)], e-mail: luciane_modenez@ibest.com.br; Avila, Crebio Jose [EMBRAPA Agropecuaria Oeste, Dourados, MS (Brazil)], e-mail: crebio@cpao.embrapa.br

    2006-12-15

    Pathogenicity of the fungi Metarrhizium anisopliae (Metsch.) Sorokin and Beauveria bassiana (Bals.) Vuillemin to stink bug Scaptocoris carvalhoi Becker, 1967 was evaluated under laboratory and greenhouse conditions. Experiments were carried out at EMBRAPA Agropecuaria Oeste, Dourados, Mato Grosso do Sul State, Brazil, in 2003. Ten M. anisopliae and eleven B. bassiana isolates were evaluated in laboratory using a completely randomized experimental design with five replicates (10 adults and 5 nymphs/plot). The pathogenicity of M. anisopliae isolate (Ma69) was also separately evaluated against nymphs and adults in laboratory and greenhouse. The stink bug mortality levels were higher for M. anisopliae isolates (between 73.3% and 94.7% than for B. bassiana isolates (between 10.7% and 78.7%). In greenhouse, stink bug mortality due to the M. anisopliae isolate (Ma69) was 57.3%, and there was no difference of mortality for nymphs and adults of stink bug in laboratory. However, in greenhouse, mortality levels were significantly higher (p<0,05) for nymphs (38,4%) than for adults (16,2%). From these data, we conclude that M. anisopliae isolate Ma69 was efficient to control S. carvalhoi in laboratory and in greenhouse, thus being a promising choice for use as a microbial insecticide under field conditions. (author)

  6. Composition, antioxidant and antimicrobial activity of the essential oil of Achillea collina Becker growing wild in Western Romania

    Directory of Open Access Journals (Sweden)

    Jianu Călin

    2015-01-01

    Full Text Available The investigation of the chemical composition, antioxidant and antimicrobial properties of the essential oil of Achillea collina Becker growing wild in western Romania was the aim of this study. The chemical composition of the essential oil was evaluated by GC-MS. The major compounds identified were chamazulene (38.89%, germacrene D (12.90%, beta-caryophyllene (11.52% and beta-pinene (10.66%. The antimicrobial activity was assessed by the diffusimetric method against seven common food-related bacteria. No effects were observed against Clostridium perfringens and Streptococcus pyogenes. The antioxidant activity was evaluated using the DPPH test, the essential oil (IC50 = 25.03 ± 0.12 μg/ml demonstrated a stronger scavenging effect than BHA and lower than that of ascorbic acid and propyl gallate. The results reveal strong antimicrobial and antioxidant properties of the essential oil tested and contribute to future research to find new sources of natural antiseptics and antioxidants: a viable and safe alternative to reduce the use of synthetic additives.

  7. Clinicopathological Features and Immunohistochemical Alterations of Keratinocyte Proliferation, Melanocyte Density, Smooth Muscle Hyperplasia and Nerve Fiber Distribution in Becker's Nevus

    Science.gov (United States)

    Sheng, Ping; Cheng, Yun-Long; Cai, Chuan-Chuan; Guo, Wei-Jin; Zhou, Ying; Shi, Ge

    2016-01-01

    Background Although Becker's nevus (BN) is a relatively common disease, the systematic studies of clinicopathological and immunohistochemical results are poorly reported. Objective To investigate the clinicopathological features and immunohistochemical alterations of keratinocyte proliferation, melanocyte density, smooth muscle hyperplasia and nerve fiber distribution in BN. Methods Clinical and pathological data were collected in 60 newly-diagnosed BN cases. Immunohistochemical stain of Ki-67, Melan-A, keratin 15, smooth muscle actin and protein gene product 9.5 was performed in 21 cases. Results The median diagnostic and onset age was 17 and 12 years, respectively. Skin lesions usually appeared on the upper trunk and upper limbs. The pathological features included the rete ridge elongation and fusion and basal hyperpigmentation. Epidermal Ki-67, Melan-A and keratin 15 expression and dermal nerve fiber length were significantly higher in lesional and perilesional skin than in normal skin (p<0.05~0.01), while smooth muscle actin expression was upregulated only in skin lesion (p<0.05). Conclusion Although the clinical diagnosis of BN is often straightforward, histopathology is helpful to differentiate from other pigmentary disorders. The hyperproliferation of keratinocytes, melanocytes, arrector pili muscle and dermal nerve fibers could be involved in the pathogenesis of BN. PMID:27904268

  8. Comparative investigation of 11 Achillea collina Becker accessions concerning phenological, morphological, productional features and active agent content

    Directory of Open Access Journals (Sweden)

    Kindlovits, Sára

    2016-07-01

    Full Text Available Eleven Achillea collina Becker accessions of different origin were tested in open field plots during three years for their phenological, morphological, productional features and active material content in Budapest, Hungary. Among the tested plant materials European selected cultivars, Hungarian cultivated stocks and populations from wild growing habitats were investigated. Concerning flowering time, two types (early and late were distinguished. Flowering time of the less abundant late type, represented by Hungarian variety ‘Azulenka’ and ‘Gb22’, started approximately 2 weeks later than that of the early flowering type. Plant height and length of flowering horizon varied only slightly among taxa, and increased after the first year of cultivation. The proportion of useful plant organs in the drug was stable. Biomass and drug production of the investigated genotypes was variable, late flowering types providing higher yields. Essential oil, proazulene, total phenolic and flavonoid content varied on a large scale among accessions and years. Results demonstrate the high intraspecific variability of A. collina and also the role of valuable genotypes in drug production. Selected cultivars may provide stable and good yields and drug quality under particular environmental conditions, while genotypes of wild origin may be valuable sources of future breeding programs.

  9. Genetic diagnosis of Duchenne/Becker muscular dystrophy using next-generation sequencing: validation analysis of DMD mutations

    Science.gov (United States)

    Okubo, Mariko; Minami, Narihiro; Goto, Kanako; Goto, Yuichi; Noguchi, Satoru; Mitsuhashi, Satomi; Nishino, Ichizo

    2016-01-01

    Duchenne and Becker muscular dystrophies (DMD/BMD) are the most common inherited neuromuscular disease. The genetic diagnosis is not easily made because of the large size of the dystrophin gene, complex mutational spectrum and high number of tests patients undergo for diagnosis. Multiplex ligation-dependent probe amplification (MLPA) has been used as the initial diagnostic test of choice. Although MLPA can diagnose 70% of DMD/BMD patients having deletions/duplications, the remaining 30% of patients with small mutations require further analysis, such as Sanger sequencing. We applied a high-throughput method using Ion Torrent next-generation sequencing technology and diagnosed 92% of patients with DMD/BMD in a single analysis. We designed a multiplex primer pool for DMD and sequenced 67 cases having different mutations: 37 with deletions/duplications and 30 with small mutations or short insertions/deletions in DMD, using an Ion PGM sequencer. The results were compared with those from MLPA or Sanger sequencing. All deletions were detected. In contrast, 50% of duplications were correctly identified compared with the MLPA method. Small insertions in consecutive bases could not be detected. We estimated that Ion Torrent sequencing could diagnose ~92% of DMD/BMD patients according to the mutational spectrum of our cohort. Our results clearly indicate that this method is suitable for routine clinical practice providing novel insights into comprehensive genetic information for future molecular therapy. PMID:26911353

  10. Becker Muscular Dystrophy (BMD) caused by duplication of exons 3-6 of the dystrophin gene presenting as dilated cardiomyopathy

    Energy Technology Data Exchange (ETDEWEB)

    Tsai, A.C.; Allingham-Hawkins, D.J.; Becker, L. [Univ. of Toronto, Ontario (Canada)] [and others

    1994-09-01

    X-linked dilated cardiomyopathy (XLCM) is a progressive myocardial disease presenting with congestive heart failure in teenage males without clinical signs of skeletal myopathy. Tight linkage of XLCM to the DMD locus has been demonstrated; it has been suggested that, at least in some families, XLCM is a {open_quotes}dystrophinopathy.{close_quotes} We report a 14-year-old boy who presented with acute heart failure due to dilated cardiomyopathy. He had no history of muscle weakness, but physical examination revealed pseudohypertrophy of the calf muscles. He subsequently received a heart transplantation. Family history was negative. Serum CK level at the time of diagnosis was 10,416. Myocardial biopsy showed no evidence of carditis. Dystrophin staining of cardiac and skeletal muscle with anti-sera to COOH and NH{sub 2}termini showed a patchy distribution of positivity suggestive of Becker muscular dystrophy. Analysis of 18 of the 79 dystrophin exons detected a duplication that included exons 3-6. The proband`s mother has an elevated serum CK and was confirmed to be a carrier of the same duplication. A mutation in the muscle promotor region of the dystrophin gene has been implicated in the etiology of SLCM. However, Towbin et al. (1991) argued that other 5{prime} mutations in the dystrophin gene could cause selective cardiomyopathy. The findings in our patient support the latter hypothesis. This suggests that there are multiple regions in the dystrophin gene which, when disrupted, can cause isolated dilated cardiomyopathy.

  11. Characterization of genetic deletions in Becker muscular dystrophy using monoclonal antibodies against a deletion-prone region of dystrophin

    Energy Technology Data Exchange (ETDEWEB)

    Thanh, L.T.; Man, Nguyen Thi; Morris, G.E. [Wales Institute, Clwyd (United Kingdom)] [and others

    1995-08-28

    We have produced a new panel of 20 monoclonal antibodies (mAbs) against a region of the dystrophin protein corresponding to a deletion-prone region of the Duchenne muscular dystrophy gene (exons 45-50). We show that immunohistochemistry or Western blotting with these {open_quotes}exon-specific{close_quotes} mAbs can provide a valuable addition to Southern blotting or PCR methods for the accurate identification of genetic deletions in Becker muscular dystrophy patients. The antibodies were mapped to the following exons: exon 45 (2 mAbs), exon 46 (6), exon 47 (1), exons 47/48 (4), exons 48-50 (6), and exon 50 (1). PCR amplification of single exons or groups of exons was used both to produce specific dystrophin immunogens and to map the mAbs obtained. PCR-mediated mutagenesis was also used to identify regions of dystrophin important for mAb binding. Because the mAbs can be used to characterize the dystrophin produced by individual muscle fibres, they will also be useful for studying {open_quotes}revertant{close_quotes} fibres in Duchenne muscle and for monitoring the results of myoblast therapy trials in MD patients with deletions in this region of the dystrophin gene. 27 refs., 7 figs., 3 tabs.

  12. Deletion analysis of the dystrophin gene in Duchenne and Becker muscular dystrophy patients: Use in carrier diagnosis

    Directory of Open Access Journals (Sweden)

    Kumari D

    2003-04-01

    Full Text Available The dystrophin gene was analyzed in 8 Duchenne muscular dystrophy (DMD and 10 Becker muscular dystrophy (BMD unrelated families (22 subjects: 18 index cases and 4 sibs for the presence of deletions by multiplex polymerase chain reaction (mPCR; 27 exons and Southern hybridization using 8 cDMD probes. Deletions were identified in 5 DMD and 7 BMD patients (6 index cases and 1 sib. The concordance between the clinical phenotype and 'reading frame hypothesis' was observed in 11/12 patients (92%. The female relatives of DMD/BMD patients with identifiable deletions were examined by quantitative mPCR. Carriers were identified in 7 families. We also describe a variation in the HindIII pattern with cDNA probe 8 and 11-14. Molecular characterization of the dystrophin gene in this study has been helpful in advising the patients concerning the inheritance of the condition, and carrier diagnosis of female relatives, and should also prove useful for prenatal diagnosis.

  13. A different spectrum of DMD gene mutations in local Chinese patients with Duchenne/Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    Ivan Fai-man Lo; Kent Keung-san Lai; Tony Ming-for Tong; Stephen Tak-sum Lam

    2006-01-01

    Background Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are X-linked recessive, allelic disorders. This study was conducted to look into the spectrum of DMD gene mutations in Hong Kong Chinese patients with Duchenne or Becket muscular dystrophy (DMD/BMD), and to study genotype-phenotype correlation.Methods A retrospective review of 67 patients.Results Twenty-three (34.3%) patients had exon deletions; whereas 5 (7.5%) patients had exon duplications.Twenty-three (34.3%) patients had small mutations, including 17-point mutations and 6 small insertions or deletions. No correlation was found between the type of mutation and the muscle phenotype or mental retardation.Significantly fewer maternal carriers were found in patients with exon deletions, and a positive family history was more common in those with small mutations. DMD phenotype was significantly less common in patients with exon deletions/duplications at the 5' hotspot, whereas all 4 small mutations associated with mental retardation were located in the 3' end of the gene.Conclusions The percentage of DMD exon deletions in local Chinese patients was significantly lower than the commonly quoted 60%. This indicated an ethnic or regional difference in predisposition to DMD exon deletions.

  14. Delayed entry into first marriage and marital stability: Further evidence on the Becker-Landes-Michael hypothesis

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    Evelyn Lehrer

    2013-09-01

    Full Text Available BACKGROUND In their pioneering research, Becker, Landes, and Michael (1977 found that beyond age 30 there is a positive relationship between women's age at first marriage and marital instability. They interpreted this finding as a "poor-match effect" emerging when the biological clock begins to tick. OBJECTIVE Our objective was to ascertain with more recent data whether or not there is evidence of a poor match effect and if so, whether it is associated with higher marital instability. METHODS We used data on non-Hispanic white women from the 2006-2010 National Surveys of Family Growth (NSFG (N = 3,184. RESULTS We found evidence of the existence of a poor-match effect: women who delay marriage disproportionately make unconventional matches, which are generally associated with high marital instability. We also found, however, that their unions are very solid. Both of these results were consistent with earlier findings for the 1995 and 2002-2003 NSFG cycles. In attempting to explain this puzzle, we proposed and tested competing hypotheses. We found that the destabilizing effects associated with indicators of unconventional matches are also present in marriages contracted late, but are dwarfed by the stabilizing influences associated with higher levels of education and older ages. CONCLUSIONS This paper contributes to our understanding of the determinants of marital instability and the poor match effect by providing a new interpretation for the puzzle described above. COMMENTS Our findings have implications for analyses of changes over time in the extent of positive assortative mating in the marriage market, and for the extensive literature showing that heterogamy in traits that are complementary in the context of marriage is destabilizing -- heterogamous marriages contracted at a late age are likely to be stable.

  15. Duchenne/Becker muscular dystrophy: A report on clinical, biochemical, and genetic study in Gujarat population, India

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    Mandava V Rao

    2014-01-01

    Full Text Available Objective: In India, various groups have studied different regions to find out deletion pattern of dystrophin gene. We have investigated its deletion pattern among Duchenne/Becker muscular dystrophy (D/BMD patients across Gujarat. Moreover, in this study we also correlate the same with reading frame rule. However, we too consider various clinicopathological features to establish as adjunct indices when deletion detection fails. Materials and Methods: In this pilot study, a total of 88 D/BMD patients consulting at our centers in Gujarat, India were included. All patients were reviewed on basis of their clinical characteristics, tested by three primer sets of 10-plex, 9-plex, and 7-plex polymerase chain reaction (PCR for genetic analysis; whereas, biochemical indices were measured using automated biochemical analyzers. Results: The diagnosis of D/BMD was confirmed by multiplex-PCR (M-PCR in D/BMD patients. A number of 65 (73.86% out of 88 patients showed deletion in dystrophin gene. The exon 50 (58.46% was the most frequent deletion found in our study. The mean age of onset of DMD and BMD was 4.09 ΁ 0.15 and 7.14 ΁ 0.55 years, respectively. In patients, mean creatine phosphokinase (CPK, lactate dehydrogenase (LDH, and myoglobin levels were elevated significantly (P < 0.05 in comparison to controls. Addition to CPK, LDH and myoglobin are good adjunct when deletion detection failed. These data are further in accordance with world literature when correlated with frame rule. Conclusion: The analysis has been carried out for the first time for a total of 88 D/BMD patients particularly from Gujarat, India. More research is essential to elucidate specific mutation pattern in association with management and therapies of proband.

  16. Correlation of Utrophin Levels with the Dystrophin Protein Complex and Muscle Fibre Regeneration in Duchenne and Becker Muscular Dystrophy Muscle Biopsies.

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    Narinder Janghra

    Full Text Available Duchenne muscular dystrophy is a severe and currently incurable progressive neuromuscular condition, caused by mutations in the DMD gene that result in the inability to produce dystrophin. Lack of dystrophin leads to loss of muscle fibres and a reduction in muscle mass and function. There is evidence from dystrophin-deficient mouse models that increasing levels of utrophin at the muscle fibre sarcolemma by genetic or pharmacological means significantly reduces the muscular dystrophy pathology. In order to determine the efficacy of utrophin modulators in clinical trials, it is necessary to accurately measure utrophin levels and other biomarkers on a fibre by fibre basis within a biopsy section. Our aim was to develop robust and reproducible staining and imaging protocols to quantify sarcolemmal utrophin levels, sarcolemmal dystrophin complex members and numbers of regenerating fibres within a biopsy section. We quantified sarcolemmal utrophin in mature and regenerating fibres and the percentage of regenerating muscle fibres, in muscle biopsies from Duchenne, the milder Becker muscular dystrophy and controls. Fluorescent immunostaining followed by image analysis was performed to quantify utrophin intensity and β-dystrogylcan and ɣ -sarcoglycan intensity at the sarcolemma. Antibodies to fetal and developmental myosins were used to identify regenerating muscle fibres allowing the accurate calculation of percentage regeneration fibres in the biopsy. Our results indicate that muscle biopsies from Becker muscular dystrophy patients have fewer numbers of regenerating fibres and reduced utrophin intensity compared to muscle biopsies from Duchenne muscular dystrophy patients. Of particular interest, we show for the first time that the percentage of regenerating muscle fibres within the muscle biopsy correlate with the clinical severity of Becker and Duchenne muscular dystrophy patients from whom the biopsy was taken. The ongoing development of these

  17. Revision of New World Species of the Shore-fly Subgenus Allotrichoma Becker of the Genus Allotrichoma with Description of the Subgenus Neotrichoma (Diptera, Ephydridae, Hecamedini

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    Wayne Mathis

    2012-01-01

    Full Text Available The New World species of the subgenera Allotrichoma Becker and Neotrichoma (new subgenus are revised, including a phylogenetic analysis of the species groups and subgenera within the genus Allotrichoma. For phylogenetic perspective and to document the monophyly of the genus Allotrichoma and its included subgenera and species groups, we also provide a cladistic analysis of genera within the tribe Hecamedini. The ingroup included seven exemplar congeners from within Allotrichoma. Outgroup sampling included exemplars of other genera within Hecamedini and from the putative sister group, Lipochaetini, and to root the analysis, we used an exemplar of the tribe Discocerinini. Analyses with successive weighting and implied weighting recovered a monophyletic Allotrichoma and indicated clades within the genus. Eight new species are described (type locality in parenthesis: A. bifurcatum (Utah. Utah: Lake Shore (40°06.9`N, 111°41.8`W; 1370 m, A. dynatum (Oregon. Benton: Finley National Wildlife Refuge (44°24.6`N, 123°19.5`W, A. occidentale (Oregon. Lake: Lakeview (44 km E; Drake Creek; 42°11`N, 119°59.3`W, A. robustum (California. Kern: Kern River (35°16.1`N, 119°18.4`W, A. sabroskyi (New Mexico. Sandoval: La Cueva (Junction of Highways 126 and 4; 35°52`N, 106°38.4`W; 2342 m, A. wallowa (Oregon Baker: Goose Creek (35 km E Baker City; 44°49.2`N, 117°27.79`W; 825 m, A. baliops (Florida. Monroe: Key West (Willie Ward Park; 24°32.9`N, 81°47.9`W, and A. insulare (Dominica. Cabrits Swamp (15°35`N, 61°29`W. Within Allotrichoma, we recognize three subgenera of which one, Neotrichoma (type species: A. atrilabre, is newly described. All known species from the New World are described with an emphasis on structures of the male terminalia, which are fully illustrated. Detailed locality data and distribution maps for the New World species are provided. A lectotype is designated for Discocerina simplex Loew and a neotype is designated for Allotrichoma

  18. Denis P. et Becker C. (dir), L’épidémie de sida en Afrique sub-saharienne. Regards historiens

    OpenAIRE

    Desclaux, Alice

    2009-01-01

    En 2004, l’université de Louvain la Neuve organisait un colloque sur l’histoire du sida en Afrique, avec l’ambition de retracer la genèse spatio-temporelle de l’épidémie. L’ouvrage collectif que publient Philippe Denis et Charles Becker dans les suites de ce colloque traite le sida comme un fait historique, en affichant quatre axes d’analyse : la dynamique de l’épidémie, sa situation dans l’histoire des épidémies, les réponses sociales, et les débats et controverses qui jalonnent la productio...

  19. Becker y Chapman, criminólogos internacionales: el interaccionismo simbólico en criminología visto en dos de sus representantes

    OpenAIRE

    Castillo Barrantes, Enrique

    2014-01-01

    Se parte de una presentación esquemática del interaccionismo en sociología general, siguiendo los lineamientos de una presentación hecha por Arnold M. Rose, para luego exponer las manifestaciones de dicha corriente en el campo criminológico, tal y como resulta de las principales obras de Howard S. Becker y Dennis Chapman.Tras pasar revistas sobre los temas más sobresalientes enfocados por ambos autores, se ponen en evidencia los aportes del interaccionismo y las nuevas posibilidades de aplica...

  20. Becker y Chapman, criminólogos internacionales: el interaccionismo simbólico en criminología visto en dos de sus representantes

    OpenAIRE

    Castillo Barrantes, Enrique

    2014-01-01

    Se parte de una presentación esquemática del interaccionismo en sociología general, siguiendo los lineamientos de una presentación hecha por Arnold M. Rose, para luego exponer las manifestaciones de dicha corriente en el campo criminológico, tal y como resulta de las principales obras de Howard S. Becker y Dennis Chapman.Tras pasar revistas sobre los temas más sobresalientes enfocados por ambos autores, se ponen en evidencia los aportes del interaccionismo y las nuevas posibilidades de aplica...

  1. 专业化分工与浙江省产业集群——基于Becker-Murphy模型的分析

    Institute of Scientific and Technical Information of China (English)

    华洋洋

    2009-01-01

    长江三角洲中的浙江省,经过20多年的市场化发展,出现了许多产业集群区域,正是这些区域的发展推动了整个地区的经济增长.本文从浙江省产业集群的现状出发,通过对Becker-Murphy模型的分析,提出对浙江省产业集群分工的建议.

  2. Deleciones en el gen de la distrofina en 62 familias colombianas: correlación genotipo-fenotipo para la distrofia muscular de Duchenne y Becker

    OpenAIRE

    Silva, Claudia T.; Dora Fonseca; Carlos Martín Restrepo; Nora C. Contreras; Mateus, Heidi E

    2004-01-01

    INTRODUCCIÓN: La correlación genotipo-fenotipo se estableció mediante el análisis de deleciones del gen de la distrofina en pacientes con distrofia muscular de Duchenne y Becker (DMD/DMB). OBJETIVOS: Establecer la correlación entre el genotipo molecular y el fenotipo clínico de los pacientes. MATERIALES Y MÉTODOS: Se analizaron 62 afectados mediante amplificaciones por PCR múltiplex de 18 exones ubicados en los dos puntos proclives dentro del gen. RESULTADOS: En la población analizada, 19 p...

  3. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach

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    Thiene Gaetano

    2008-11-01

    Full Text Available Abstract Background Becker-Kiener muscular dystrophy (BMD represents an X-linked genetic disease associated with myocardial involvement potentially resulting in dilated cardiomyopathy (DCM. Early diagnosis of cardiac involvement may permit earlier institution of heart failure treatment and extend life span in these patients. Both echocardiography and nuclear imaging methods are capable of detecting later stages of cardiac involvement characterised by wall motion abnormalities. Cardiovascular magnetic resonance (CMR has the potential to detect cardiac involvement by depicting early scar formation that may appear before onset of wall motion abnormalities. Methods In a prospective two-center-study, 15 male patients with BMD (median age 37 years; range 11 years to 56 years underwent comprehensive neurological and cardiac evaluations including physical examination, echocardiography and CMR. A 16-segment model was applied for evaluation of regional wall motion abnormalities (rWMA. The CMR study included late gadolinium enhancement (LGE imaging with quantification of myocardial damage. Results Abnormal echocardiographic results were found in eight of 15 (53.3% patients with all of them demonstrating reduced left ventricular ejection fraction (LVEF and rWMA. CMR revealed abnormal findings in 12 of 15 (80.0% patients (p = 0.04 with 10 (66.6% having reduced LVEF (p = 0.16 and 9 (64.3% demonstrating rWMA (p = 0.38. Myocardial damage as assessed by LGE-imaging was detected in 11 of 15 (73.3% patients with a median myocardial damage extent of 13.0% (range 0 to 38.0%, an age-related increase and a typical subepicardial distribution pattern in the inferolateral wall. Ten patients (66.7% were in need of medical heart failure therapy based on CMR results. However, only 4 patients (26.7% were already taking medication based on clinical criteria (p = 0.009. Conclusion Cardiac involvement in patients with BMD is underdiagnosed by echocardiographic methods resulting

  4. Deleciones en el gen de la distrofina en 62 familias colombianas: correlación genotipo-fenotipo para la distrofia muscular de Duchenne y Becker.

    Directory of Open Access Journals (Sweden)

    Claudia T. Silva

    2009-11-01

    Full Text Available INTRODUCCIÓN: La correlación genotipo-fenotipo se estableció mediante el análisis de deleciones del gen de la distrofina en pacientes con distrofia muscular de Duchenne y Becker (DMD/DMB. OBJETIVOS: Establecer la correlación entre el genotipo molecular y el fenotipo clínico de los pacientes. MATERIALES Y MÉTODOS: Se analizaron 62 afectados mediante amplificaciones por PCR múltiplex de 18 exones ubicados en los dos puntos proclives dentro del gen. RESULTADOS: En la población analizada, 19 pacientes mostraron deleción en el gen de la distrofina con los 18 exones estudiados, esto corresponde a 31% de hombres afectados con deleción. CONCLUSIONES: Teniendo en cuenta la hipótesis del corrimiento del marco de lectura traduccional (CMLT y la mutación observada en los afectados, se pudo determinar que las mutaciones out frame, resultan en pacientes con el fenotipo severo o distrofia muscular de Duchenne y las mutaciones in frame, resultan en pacientes con el fenotipo leve o distrofia muscular de Becker. Se pudo predecir un cuadro clínico de DMD o DMB en 79% de los casos, lo cual permite utilizar este sistema diagnóstico como una herramienta importante para ayudarle a los neurólogos en la valoración clínica de los pacientes en los cuales se encuentra deleciones.

  5. Becker muscular dystrophy

    Science.gov (United States)

    ... Inactivity (such as bed rest) can make the muscle disease worse. Physical therapy may be helpful to maintain ... Philadelphia, PA: Elsevier; 2016:chap 609. Selcen D. Muscle diseases. In: Goldman L, Schafer AI, eds. Goldman's Cecil ...

  6. Willingness to pay for small solar powered bed net fans: results of a Becker-DeGroot-Marschak auction in Ghana.

    Science.gov (United States)

    Yukich, Joshua O; Briët, Olivier J T; Ahorlu, Collins K; Nardini, Peter; Keating, Joseph

    2017-08-07

    Long-lasting insecticidal nets (LLINs) are one of the main interventions recommended by the World Health Organization for malaria vector control. LLINs are ineffective if they are not being used. Subsequent to the completion of a cluster randomized cross over trial conducted in rural Greater Accra where participants were provided with the 'Bɔkɔɔ System'-a set of solar powered net fan and light consoles with a solar panel and battery-or alternative household water filters, all trial participants were invited to participate in a Becker-DeGroot-Marschak auction to determine the mean willingness to pay (WTP) for the fan and light consoles and to estimate the demand curve for the units. Results demonstraed a mean WTP of approximately 55 Cedis (~13 USD). Demand results suggested that at a price which would support full manufacturing cost recovery, a majority of households in the area would be willing to purchase at least one such unit.

  7. Early cardiac failure in a child with Becker muscular dystrophy is due to an abnormally low amount of dystrophin transcript lacking exon 13.

    Science.gov (United States)

    Ishigaki, C; Patria, S Y; Nishio, H; Yoshioka, A; Matsuo, M

    1997-12-01

    Two Japanese brothers with Becker muscular dystrophy were shown by polymerase chain reaction (PCR) and cDNA sequence analysis to produce a dystrophin gene transcript lacking a single exon: that is, number 13. Despite having the same deletion mutation, the brothers showed clearly different clinical phenotypes: the younger brother developed cardiac failure at the age of nine, while the elder brother was asymptomatic. As alternative splicing was not responsible for this clinical difference, the amount of dystrophin transcript was examined by using reverse transcription semi-nested and parallel PCR. The results showed that the amount of the dystrophin transcript in the younger brother was 20% of that of the elder brother. This finding suggested that lesser amount of dystrophin transcript in the younger brother was responsible for the early onset of cardiac failure. This would represent a novel molecular mechanism for dystrophinopathy.

  8. A case of Becker muscular dystrophy resulting from the skipping of four contiguous exons (71-74) of the dystrophin gene during mRNA maturation.

    Science.gov (United States)

    Patria, S Y; Alimsardjono, H; Nishio, H; Takeshima, Y; Nakamura, H; Matsuo, M

    1996-07-01

    The mutations in one-third of both Duchenne and Becker muscular dystrophy patients remain unknown because they do not involve gross rearrangements of the dystrophin gene. Here we report the first example of multiple exon skipping during the splicing of dystrophin mRNA precursor encoded by an apparently normal dystrophin gene. A 9-year-old Japanese boy exhibiting excessive fatigue and high serum creatine kinase activity was examined for dystrophinopathy. An immunohistochemical study of muscle tissue biopsy disclosed faint and discontinuous staining of the N-terminal and rod domains of dystrophin but no staining at all of the C-terminal domain of dystrophin. The dystrophin transcript from muscle tissue was analyzed by the reverse transcriptase polymerase chain reaction. An amplified product encompassing exons 67-79 of dystrophin cDNA was found to be smaller than that of the wild-type product. Sequence analysis of this fragment showed that the 3' end of exon 70 was directly connected to the 5' end of exon 75 and, thus, that exons 71-74 were completely absent. As a result, a truncated dystrophin protein lacking 110 amino acids from the C-terminal domain should result from translation of this truncated mRNA, and the patient was diagnosed as having Becker muscular dystrophy at the molecular level. Genomic DNA was analyzed to identify the cause of the disappearance of these exons. Every exon-encompassing region could be amplified from genomic DNA, indicating that the dystrophin gene is intact. Furthermore, sequencing of these amplified products did not disclose any particular nucleotide change that could be responsible for the multiple exon skipping observed. Considering that exons 71-74 are spliced out alternatively in some tissue-specific isoforms, to suppose that the alternative splicing machinery is present in the muscle tissue of the index case and that it is activated by an undetermined mechanism is reasonable. These results illustrate a novel genetic anomaly that

  9. Atualização do tratamento fisioterapêutico das distrofias musculares de Duchenne e de Becker - doi:10.5020/18061230.2005.p41

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    Simone Rizzo Nique da Silva

    2012-01-01

    Full Text Available As distrofias musculares de Duchenne (DMD e de Becker (DMB consistem em distúrbios neuromusculares genéticos de herança autossômica recessiva, ligados ao cromossomo X, incuráveis e progressivos. O papel do profissional fisioterapeuta diante desses agravos ainda se encontra muito segmentário, com opiniões e condutas bastante controversas. Este estudo consiste em uma revisão bibliográfica sobre o tratamento fisioterapêutico das distrofias de Duchenne (DMD e de Becker (DMB e pretende evidenciar a importância da realização de Fisioterapia nessas doenças. Portanto, foi realizada pesquisa bibliográfica dos últimos 12 anos abordando os temas: DMD e DMB, alterações cardiorrespiratórias e o tratamento fisioterapêutico. O trabalho foi realizado nas bibliotecas do Centro Universitário Feevale com busca em centro de informações, sites especializados, bancos de dados e em revistas de maior impacto internacional. As referências pesquisadas sugerem que pacientes com DMD e DMB necessitam de tratamento fisioterapêutico, que os procedimentos devem ser adaptados à faixa etária e que visam retardar a evolução clínica e prevenir complicações secundárias da doença. A evolução de novas técnicas e instrumentos utilizáveis na fisioterapia têm contribuído sobremaneira para o objetivo de melhoria de qualidade de vida e a funcionalidade desses pacientes.

  10. [Rolf Hammel-Kiesow. Der Lübecker Katasterplan des 19. Jahrhunderts als historische Quelle. Überlegungen zur Stadtentwicklung Lübecks aus archäologischer, historischer und bauhistorischen Zicht] / Dennis Hormuth

    Index Scriptorium Estoniae

    Hormuth, Dennis

    2013-01-01

    Arvustus : Rolf Hammel-Kiesow. Der LÜbecker Katasterplan des 19. Jahrhunderts als historische Quelle. Überlegungen zur Stadtentwicklung Lübecks aus archäologischer, historischer und bauhistorischen Sicht. In: Stadtgründung und Stadterweiterung. Beiträge von Archäologie und Stadtgeschichtsforschung (=Beiträge zur Geschichte der Städte Mitteleuropas XXII). Linz, 2011. S. 75-104

  11. [Rolf Hammel-Kiesow. Der Lübecker Katasterplan des 19. Jahrhunderts als historische Quelle. Überlegungen zur Stadtentwicklung Lübecks aus archäologischer, historischer und bauhistorischen Zicht] / Dennis Hormuth

    Index Scriptorium Estoniae

    Hormuth, Dennis

    2013-01-01

    Arvustus : Rolf Hammel-Kiesow. Der LÜbecker Katasterplan des 19. Jahrhunderts als historische Quelle. Überlegungen zur Stadtentwicklung Lübecks aus archäologischer, historischer und bauhistorischen Sicht. In: Stadtgründung und Stadterweiterung. Beiträge von Archäologie und Stadtgeschichtsforschung (=Beiträge zur Geschichte der Städte Mitteleuropas XXII). Linz, 2011. S. 75-104

  12. Deleciones en el gen de la distrofina en 62 familias colombianas: correlación genotipo-fenotipo para la distrofia muscular de Duchenne y Becker

    Directory of Open Access Journals (Sweden)

    Carlos M. Restrepo

    2004-12-01

    Full Text Available Introduction: A genotype-phenotype correlation was established by dystrophin gene deletion analysis in Duchenne and Becker muscular dystrophies patients (DMD/BMD. Objectives: To establish a correlation between molecular genotype and clinical phenotype in a Colombian population. Materials and methods: A PCR (Polymerase Chain Reaction amplification of 18 exons (included in the two hot spots regions was performed in 62 affected families. Results: Nineteen patients showed deletions in several exons of the dystrophin gene. This corresponds to 31% of analyzed males in the present population. Conclusions: For each DMD/DMB affected male with deletion in the dystrophin gene, a correlation between disease severity and extent of deletion was established. The data showed that most out-frame deletions cause DMD phenotype, while the in-frame deletions results in BMD phenotype. This correlation was described by Koenig in his “open reading frame hypothesis”. In the present study it was possible to establish a direct correlation between mutation state and clinical severity in 79% of patients. This may help clinical evaluation of DMD/DMB patients.

  13. Screening Duchenne and Becker muscular dystrophy patients for deletions in 30 exons of the dystrophin gene by three-multiplex PCR

    Energy Technology Data Exchange (ETDEWEB)

    Risch, N. (Yale Univ., New Haven, CT (United States))

    1992-09-01

    Deletion mutations of the dystrophin gene may cause either the severe Duchenne muscular dystrophy (DMD) or the milder, allelic Becker muscular dystrophy (BMD) and are clustered in two high-frequency-deletion regions (HFDRs) located, respectively, 500 kb and 1,200 kb downstream from the 5[prime] end of the gene. Three PCR reactions described allowed the analysis of a total of 30 exons and led, to the identification of three additional deletions involving the following exons: (a) 42 only, (b) 28-42, and (c) 16 only, none of which were detected with the two original multiplex reactions. Therefore, the three modified multiplexes detected 95 of the 96 deletions identified among the 152 patients studied so far by using Southern analysis and cDNA probes. The only deletion that remained undetected with this system involves exons 22-25 and generates the junction fragment described elsewhere. The percentage of deletion mutations among DMS/BMD patients amounts to 63%, which is in agreement with similar estimates from other laboratories. When field-inversion gel electrophoresis is coupled to Southern analysis, the detection rate of deletion and duplication mutations reaches 65%.

  14. Comparison of the clinical state and its changes in patients with Duchenne and Becker muscular dystrophy with results of in vivo {sup 31}P magnetic resonance spectroscopy

    Energy Technology Data Exchange (ETDEWEB)

    Hajek, M. [MR Unit, Inst. for Clinical and Experimental Medicine, Prague (Czech Republic); Grosmanova, A. [Dept. of Neuropediatrics, Thomayer`s Hospital, Prague (Czech Republic); Horska, A. [MR Unit, Inst. for Clinical and Experimental Medicine, Prague (Czech Republic); Urban, P. [Dept. of Analytical Chemistry, Prague Inst. of Chemical Technology (Czech Republic)

    1993-12-01

    A total of 14 boys with the Duchenne and Becker forms of muscular dystrophy (DMD, BMD) were examined using {sup 31}P magnetic resonance (MR) spectroscopy; 12 boys were examined repeatedly. The results were correlated with clinical findings (including those of genetic tests) and with data obtained from examinations of an age-matched control group. Evaluation of results using principal component analysis revealed maximum variability in the following ratios: phosphocreatine/inorganic phosphate (PCr/Pi), phosphocreatine/phosphodiesters (PCr/PDe) and phosphocreatine/phosphomonoesters (PCr/PMe). A decrease in PCr/Pi correlates with weakness of the hip girdle and of the lower part of the shoulder girdle in DMD/BMD patients. The values of all ratios in the group of patients with the DMD phenotype differ significantly from results obtained in the group with the BMD phenotype. Continuous follow-up of patients using {sup 31}P MR spectroscopy revealed a marked decrease in PCr/Pi in DMD/BMD patients at an age that could be expected in subjects with a typical clinical course of DMD/BMD. An attempt to manage a concomitant disease with prednisone and carnitene was followed by an increase in PCr/Pi in 3 cases. A rise in the PCr/Pi ratio signalled clinical improvement in the patients. A decrease in PCr/Pi was found after controlled physical training, a finding consistent with data obtained from clinical observations describing an adverse effect of physical stress on the dystrophic process. (orig.)

  15. Early-progressive dilated cardiomyopathy in a family with Becker muscular dystrophy related to a novel frameshift mutation in the dystrophin gene exon 27.

    Science.gov (United States)

    Tsuda, Takeshi; Fitzgerald, Kristi; Scavena, Mena; Gidding, Samuel; Cox, Mary O; Marks, Harold; Flanigan, Kevin M; Moore, Steven A

    2015-03-01

    We report a family in which two male siblings with Becker muscular dystrophy (BMD) developed severe dilated cardiomyopathy (DCM) and progressive heart failure (HF) at age 11 years; one died at age 14 years while awaiting heart transplant and the other underwent left ventricular assist device implantation at the same age. Genetic analysis of one sibling showed a novel frameshift mutation in exon 27 of Duchenne muscular dystrophy (DMD) gene (c.3779_3785delCTTTGGAinsGG), in which seven base pairs are deleted and two are inserted. Although this predicts an amino-acid substitution and premature termination (p.Thr1260Argfs*8), muscle biopsy dystrophin immunostaining instead indicates that the mutation is more likely to alter splicing. Despite relatively preserved skeletal muscular performance, both the siblings developed progressive HF secondary to early-onset DCM. In addition, their 7-year-old nephew with delayed gross motor development, mild proximal muscle weakness and markedly elevated serum creatine kinase level (>13 000 IU l(-1)) at 16 months was recently demonstrated to have the familial DMD mutation. Here, we report a novel genotype of BMD with early-onset DCM and progressive lethal HF during early adolescence.

  16. Clinical and molecular diagnosis of a Costa Rican family with autosomal recessive myotonia congenita (Becker disease carrying a new mutation in the CLCN1 gene

    Directory of Open Access Journals (Sweden)

    Fernando Morales

    2008-03-01

    Full Text Available Myotonia congenita is a muscular disease characterized by myotonia, hypertrophy, and stiffness. It is inherited as either autosomal dominant or recessive known as Thomsen and Becker diseases, respectively. Here we confirm the clinical diagnosis of a family diagnosed with a myotonic condition many years ago and report a new mutation in the CLCN1 gene. The clinical diagnosis was established using ocular, cardiac, neurological and electrophysiological tests and the molecular diagnosis was done by PCR, SSCP and sequencing of the CLCN1 gene. The proband and the other affected individuals exhibited proximal and distal muscle weakness but no hypertrophy or muscular pain was found. The myotatic reflexes were lessened and sensibility was normal. Electrical and clinical myotonia was found only in the sufferers. Slit lamp and electrocardiogram tests were normal. Two affected probands presented diminution of the sensitive conduction velocities and prolonged sensory distal latencies. The clinical spectrum for this family is in agreement with a clinical diagnosis of Becker myotonia. This was confirmed by molecular diagnosis where a new disease-causing mutation (Q412P was found in the family and absent in 200 unaffected chromosomes. No latent myotonia was found in this family; therefore the ability to cause this subclinical sign might be intrinsic to each mutation. Implications of the structure-function-genotype relationship for this and other mutations are discussed. Adequate clinical diagnosis of a neuromuscular disorder would allow focusing the molecular studies toward the confirmation of the initial diagnosis, leading to a proper clinical management, genetic counseling and improving in the quality of life of the patients and relatives. Rev. Biol. Trop. 56 (1: 1-11. Epub 2008 March 31.La miotonía congénita es una enfermedad muscular caracterizada por miotonía, hipertrofia y rigidez. Se presenta con dos patrones de herencia, autosómica dominante en cuyo

  17. 假肥大型肌营养不良症的产前基因诊断%Prenatal molecular diagnosis of Duchenne and Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    黎青; 李少英; 胡冬贵; 孙筱放; 陈敦金; 张成; 蒋玮莹

    2006-01-01

    Objective: Duchenne and Becker muscular dystrophy (DMD/BMD) is an X-linked lethal recessive disease caused by mutations in the dystrophy gene. There is no efficient treatment for this serious and disabling disease. We established a combination method to detect carriers and perform prenatal diagnosis. Methods: In our study, from 1994 to 2005, using a different combination of 5 methods, including SRY gene amplification, multiplex PCR, multiplex Fluorescence PCR capillary electrophoresis, multiplex ligation-dependent probe amplification (MLPA) and linkage analysis of short tandem repeats (STR), 36 prenatal diagnosis were performed for pregnancies at risk of having a DMD/BMD baby through amniocentesis. Results: Fourteen out of 21 male fetuses were found to be affected and respective pregnancies were terminated. A combined diagnostic rate of 83% was achieved for 30 cases with deletions, duplications, and non-deletion mutations after tested by more than one method. Conclusion: Using a combined method, we can diagnoses patients and carriers in DMD families, and perform prenatal diagnosis for the risk fetus. MLPA provides a simple, rapid and accurate method for deletions and duplications of all the 79 DMD exons. MLPA method for DMD diagnosis is the first report in our country.

  18. Patogenicidade, DL50 e TL50 de isolados de Metarhizium anisopliae (Metsch. Sorok. para o percevejo castanho das raízes Scaptocoris carvalhoi BECKER (Hemiptera: Cydnidae

    Directory of Open Access Journals (Sweden)

    Xavier Luciane Modenez Saldivar

    2005-01-01

    Full Text Available Objetivou-se neste trabalho avaliar a patogenicidade de isolados do fungo Metarhizium anisopliae em Scaptocoris carvalhoi BECKER, 1967, bem como determinar a Dose Letal média (DL50 e o Tempo Letal médio (TL50, em laboratório. Os experimentos foram conduzidos na Embrapa Agropecuária Oeste em Dourados, MS, durante 2003. Suspensões de quatro isolados de M. anisopliae (Ma7, Ma69, Ma283 e Ma342 foram preparadas nas concentrações 10(4, 10(5, 10(6, 10(7 e 10(8 conídios mL-1 e inoculados topicamente 5mil dessas suspensoes sobre S. carvalhoi correspondendo, respectivamente, a 50, 500, 5.000, 50.000 e 500.000 conídios percevejo-1. Após a inoculação, os insetos (10 adultos e cinco ninfas foram acondicionados em gerbox (parcela e mantidos em câmaras climatizadas reguladas para 26?1masculineC, UR 85%, sem fotofase. Na maior dose testada (500.000 conídios inseto-1, observou-se um incremento significativo de mortalidade do percevejo com o aumento da dose do fungo, para todos os isolados testados. Os menores valores da DL50 foram observados com os isolados Ma69 e Ma7 e o maior com Ma283. Os valores do TL50 variaram de 0,32 a 5,84 dias, sem diferirem significativamente, entre si. Os isolados Ma69 e Ma7 apresentam potencial para serem empregados no controle de S. carvalhoi a campo.

  19. Reliability of hand-held dynamometry for measurement of lower limb muscle strength in children with Duchenne and Becker muscular dystrophy

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    Wei SHI

    2015-05-01

    Full Text Available Objective To determine the reliability of hand-held dynamometry (HHD for lower limb isometric muscle strength measurement in children with Duchenne and Becker muscular dystrophy (DMD/BMD.  Methods A total of 21 children [20 males and one female; mean age was (7.88 ± 2.87 years, ranging between 3.96-14.09 years; mean age at diagnosis was (5.88 ± 2.88 years, ranging between 1.35-12.89 years; mean height was (120.64 ± 16.30 cm, ranging between 97-153 cm; mean body weight was (24.62 ± 9.05 kg, ranging between 14-50 kg] with DMD (19/21 and BMD (2/21 were involved from Rehabilitation Center of Children's Hospital of Fudan University. The muscle strength of hip, knee and ankle was measured by HHD under standardized test methods. The test-retest results were compared to determine the inter-test reliability, and the results among testers were compared to determine the inter-tester reliability.  Results HHD showed fine inter-tester reliability (ICC = 0.762-0.978 and inter-test reliability (ICC = 0.690-0.938 in measuring lower limb muscle strength of children with DMD/BMD. Results also showed relatively poor reliability in distal muscle groups (foot plantar flexion and dorsiflexion.  Conclusions HHD, showing fine inter-tester and inter-test reliability in measuring the lower limb muscle strength of children with DMD/BMD, can be used in monitoring muscle strength changing and assessing effects of clinical interventions. DOI: 10.3969/j.issn.1672-6731.2015.05.009

  20. The interactive effect of phosphorus and nitrogen on "in vitro" spore germination of Glomus etunicatum Becker & Gerdemann, root growth and mycorrhizal colonization

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    Bressan Wellington

    2001-01-01

    Full Text Available The effects of P and N amendment and its interactions on spore germination, root growth and colonized root length by Glomus etunicatum Becker & Gerdemann (INVAM S329 was studied "in vitro" in RiT - DNA transformed roots of Anthylis vulneraria sub sp. Sampaiana (Kidney vetch. Three N media concentrations (5, 10 and 50 mg/l at P constant level (2 mg/l and three P media concentrations (2, 10 and 20 mg/l at N constant level (5 mg/l were utilized as a treatment. Bécard & Fortin medium was used as a basal medium for root growth and colonized root length, and water/agar (0.8% media was the control for spore germination. Spore germination of G. etunicatum at low P level was reduced by N addition in relation to the control media, and at low N level addition of P stimulated spore germination. Total root length was stimulated by N addtion at low P level, but no significant difference (p£0.05 was observed between 10 and 50 mg/l of N. P addition at low N level media also stimulated total root growth, and a significant difference (p£0.05 was observed among P concentrations. Colonized root length by G. etunicatum increased significantly (p£0.05 with P additions at low N levels. Under low P level no significant differences was found between 10 and 50 mg/l of N. These results demonstrate that the interaction between P and N affect differently spore germination, root growth and colonized root lenght.

  1. Factors affecting "in vitro" plant development and root colonization of sweet potato by Glomus etunicatum Becker & Gerd Fatores que afetam o desenvolvimento da planta e a colonização radicular "in vitro", da batata doce por Glomus etunicatum Becker & Gerd.

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    Wellington Bressan

    2002-01-01

    Full Text Available Nutrients media (Murashige & Skoog, Hoagland & Arnon and White's media supplemented or not with sucrose and substrates (vermiculite, agar and natrosol were tested for their effects on plant development and root colonization of micropropagated sweet potato, cv. White Star, by Glomus etunicatum Becker & Gerdemann (isolate INVAM FL S329. Addition of sucrose (3% did not affect plant development. However, hyphal growth and root colonization were depressed. Contrasting responses to media nutrient concentration were observed for plant height, root colonization, and hyphal growth. The highest concentration of nutrients in Murashige & Skoog medium improved plant development, but this medium decreased hypha growth and inhibited root colonization. Plants growing in vermiculite substrate had higher (p£0.05 development and mycorrhizal root colonization than those growing in agar or natrosol. The results indicate that colonization of micropropagated sweet potato by arbuscular mycorrhizal fungi is affected by media composition and type of substrate.Os meios de Murashige e Skoog, Hoagland, Arnon e White, suplementados ou não com sacorose, e tendo como substratos vermiculita, ágar ou natrosol foram utilizados para avaliar seus efeitos sobre o desenvolvimento da batata doce, c.v. White Star, e sua colonização por Glomus etunicatum Becker & Gerd. (isolado INVAM FL S329. A adição de sacorose (3% ao meio não afetou o desenvolvimento da planta, porém reduziu o crescimento das hifas de G. etunicatum e a colonização das raízes. A concentração de nutrientes dos meios utilizados mostrou efeitos contrastantes entre altura da planta, crescimento das hifas e colonização das raízes por G. etunicatum. A alta concentração de nutrientes no meio Murashige e Skoog estimulou o crescimento das plantas, reduziu o desenvolvimento das hifas e inibiu a colonização das raízes pelo fungo micorrízico. Plantas desenvolvidas em vermiculita mostraram maior

  2. La tesis de título de Guillermo Ulriksen Becker (1952: Bases para la Planeación Regional del Norte Chico: Provincias de Atacama y Coquimbo. /Graduation Project (Thesis of the architect William Ulriksen Becker (1952: Basis for Region Planning of the “Norte Chico”: Atacama and Coquimbo provinces.

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    Pávez Reyes, María Isabel

    2008-06-01

    Full Text Available Se presenta la tesis de título en planeamiento regional de Guillermo Ulriksen Becker (n.1905 – m. 1979 presentada en 1953 en la, entonces, “Facultad de Arquitectura” de la Universidad de Chile: Bases para la Planeación Regional del Norte Chico: Provincias de Atacama y Coquimb. Este estudio que constituye no sólo una radiografía del estado del desarrollo del Norte Chico hace cincuenta y cinco años, sino que también un señalamiento del potencial posible de desarrollar en la perspectiva del largo plazo, y de los aspectos negativos que se van sucediendo o pudieran suceder atentando contra dicho potencial./This work presents the graduation project of Guillermo Ulriksen (1905-1979. That thesis was presented to get the title of architect in the then called "Facultad de Arquitectura" of the Universidad de Chile, and it shows a complete scan of the development of the "Norte Chico", 55 years ago, indicating its potentials in a long term basis.

  3. A novel point mutation (G[sup [minus]1] to T) in a 5[prime] splice donor site of intron 13 of the dystrophin gene results in exon skipping and is responsible for Becker Muscular Dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Hagiwara, Yoko; Nishio, Hisahide; Kitoh, Yoshihiko; Takeshima, Yasuhiro; Narita, Naoko; Wada, Hiroko; Yokoyama, Mitsuhiro; Nakamura, Hajime; Matsuo, Masafumi (Kobe Univ. School of Medicine (Japan))

    1994-01-01

    The mutations in one-third of Duchenne and Becker muscular dystrophy patients remain unknown, as they do not involve gross rearrangements of the dystrophin gene. The authors now report a defect in the splicing of precursor mRNA (pre-mRNA), resulting from a maternally inherited mutation of the dystrophin gene in a patient with Becker muscular dystrophy. This defect results from a G-to-T transversion at the terminal nucleotide of exon 13, within the 5[prime] splice site of intron 13, and causes complete skipping of exon 13 during processing of dystrophin pre-mRNA. The predicted polypeptide encoded by the aberrant mRNA is a truncated dystrophin lacking 40 amino acids from the amino-proximal end of the rod domain. This is the first report of an intraexon point mutation that completely inactivates a 5[prime] splice donor site in dystrophin pre-mRNA. Analysis of the genomic context of the G[sup [minus]1]-to-T mutation at the 5[prime] splice site supports the exon-definition model of pre-mRNA splicing and contributes to the understanding of splice-site selection. 48 refs., 5 figs.

  4. Multi-scale times and modes of fast and slow relaxation in solutions with coexisting spherical and cylindrical micelles according to the difference Becker-Döring kinetic equations

    Science.gov (United States)

    Babintsev, Ilya A.; Adzhemyan, Loran Ts.; Shchekin, Alexander K.

    2014-08-01

    The eigenvalues and eigenvectors of the matrix of coefficients of the linearized kinetic equations applied to aggregation in surfactant solution determine the full spectrum of characteristic times and specific modes of micellar relaxation. The dependence of these relaxation times and modes on the total surfactant concentration has been analyzed for concentrations in the vicinity and well above the second critical micelle concentration (cmc2) for systems with coexisting spherical and cylindrical micelles. The analysis has been done on the basis of a discrete form of the Becker-Döring kinetic equations employing the Smoluchowsky diffusion model for the attachment rates of surfactant monomers to surfactant aggregates with matching the rates for spherical aggregates and the rates for large cylindrical micelles. The equilibrium distribution of surfactant aggregates in solution has been modeled as having one maximum for monomers, another maximum for spherical micelles and wide slowly descending branch for cylindrical micelles. The results of computations have been compared with the analytical ones known in the limiting cases from solutions of the continuous Becker-Döring kinetic equation. They demonstrated a fair agreement even in the vicinity of the cmc2 where the analytical theory looses formally its applicability.

  5. Gary Becker: Model Economic Scientist

    Science.gov (United States)

    2015-01-01

    This paper presents Gary Becker’s approach to conducting creative, empirically fruitful economic research. It describes the traits and methodology that made him such a productive and influential scholar. PMID:26705367

  6. Duchenne and Becker Muscular Dystrophies

    Science.gov (United States)

    ... the Achilles tendon stretched while the child is sleeping. Standing for a few hours each day, even with minimal weight bearing, promotes ... be some- what restricted to keep weight down. Obesity puts greater stress on ... have any harmful effect on the muscles. Those on prednisone and those ...

  7. 以晕厥发病的成年Becker型肌营养不良症1例报道%One report of adult onset Becker muscular dystrophy with syncope

    Institute of Scientific and Technical Information of China (English)

    沈沸; 俞羚; 陆钦池; 朱莹; 李焰生

    2009-01-01

    @@ Becker型肌营养不良症(Becker muscular dystrophy, BMD)是由抗肌萎缩蛋白(Dystrophin蛋白)缺陷导致的缓慢进展的肌肉萎缩、无力伴假性肌肥大为特征的遗传性肌肉疾病.传统上,BMD多在5~10岁起病,至20~25岁丧失独立行走能力,存活至40岁左右[1].随着对Dystrophin蛋白基因的研究深入,已发现一些30岁以后发病或早期以心肌损害为主要表现的BMD[2,3].本文报道1例以心肌损害导致晕厥而首发的成年起病的BMD.

  8. 女性DMD/BMD携带者的临床表现与发病机制研究%Symptoms and pathogenesis of manifesting carriers of Duchenne/Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    张莉; 李西华; 王艺

    2013-01-01

    Duchenne and Becker muscular dystrophies,due to genovariation of the dystrophin gene,are X-linked recessively inherited progressive neuromuscular diseases and affect mainly male individuals.While it is estimated that 20% of female carriers may have various symptoms.The clinical picture of carriers includes muscle weakness,cardiac involvement,cognitive impairment and muscle cramps/myalgias,as well as an elevated serum creatine kinase level and dystrophin abnormalities in muscle tissue.Apart from some cases associated with chromosome aberrations,the pathogenesis in most female carriers are caused by skewed X inactivation.Given the low incidence of manifesting carriers and the wide spectrum of phenotypes,the diagnosis and treatment may be delayed.In addition,there is little experience to guide management and predict prognosis.%Duchenne和Becker肌营养不良(Duchenne/Becker muscular dystrophy,DMD/BMD)为dystrophin基因变异所致的X伴性隐性遗传病,主要影响男性患者,而20%左右的女性携带者表现出不同的临床症状,如肌无力、心脏受累、认知功能障碍、肌肉疼痛或痉挛等,伴有血清肌酸激酶的升高和肌肉病理的改变.倾斜的X染色体失活是主要的发病机制,在一些病例报道中也发现了一些特殊的核型导致疾病的进展.由于有症状的携带者发病率低、临床表现差异较大,且临床缺少规范的诊治管理和随访评估的经验,因此经常得不到及时准确的诊治.该文对女性携带者的症状、诊断及发病机制进行综述.

  9. A modified Becker's split window approach for retrieving land surface temperature from the AVHRR and VIRR data%基于AVHRR和VIRR数据的改进型Becker“分裂窗”地表温度反演算法

    Institute of Scientific and Technical Information of China (English)

    权维俊; 韩秀珍; 陈洪滨

    2012-01-01

    为了将基于NOAA-9/AVHRR数据提出的Becker和Li的“分裂窗”地表温度算法成功地应用于长序列NOAA/AVHRR和FY 3A/VIRR数据的地表温度反演,为气候变化研究提供长序列、高精度、高分辨率的地表温度数据集,从辐射传输方程出发,首先利用MODTRA 4.1模式模拟了多种地表和大气状态下的光谱辐亮度数据,并结合AVHRR和VIRR通道4、5的光谱响应函数建立了温度数据集(TS,T4,T5);然后,基于该数据集采用最小二乘法重新计算了Becker和Li算法中的各参数,提出了一个适用于NOAA/AVHRR和FY-3A/VIRR数据的改进型Becker和Li分裂窗地表温度反演算法;并利用改进型算法对2008年4月27日03时12分(世界时)观测的一景覆盖北京地区的NOAA-17/AVHRR数据进行了地表温度的反演,将反演结果与日本东京大学提供的同地区、同时相的MODIS地表温度产品进行了对比分析.结果表明,两种地表温度产品的相关系数为0.88,均方根偏差(RMSD)为2.1K;在两种地表温度差值图像的频率直方图上有69.6%的像元的值在±2K之内,37%的像元的值在±1K之内.%In order to successfully apply the Becker and Li's split window approach, which was proposed based on the NOAA-9 AVHRR data, to estimate the Land Surface Temperature (LST) from the different AVHRRs and VIRR data and further to provide a high-precision, long-time, and high-resolution LST dataset for climate change research, a modified Becker and Li's split widow approach is developed based on the radiative transfer equation in this paper. To begin with, the MODTRAN 4. 1 is used to generate the spectral radiance data under a variety of surface and atmosphere conditions. Then, the temperature dataset (TS,T4,T5) is built by convolving the spectral radiance data with the spectral response functions of channels 4 and 5 of the AVHRRs and VIRR. The parameters of the Becker and Li's split window approach are subsequently recalculated based on

  10. Identificación de portadoras de distrofia muscular de Duchenne y Becker (DMD/DMB mediante análisis de dosis génica y polimorfismos de ADN

    Directory of Open Access Journals (Sweden)

    Patricia Hernández

    2000-09-01

    Full Text Available Se identificaron 7 mujeres portadoras y 15 no portadoras de deleción en 16 familias con distrofia muscular de Duchenne o de Becker (DMDIDMB mediante PCR multiplex cuantitativa y análisis de dosis génica. Los afectados con DMDIDMB mostraron deleciones en 31.25% de los casos y el estado materno de portadora se estableció por PCR multiplex cuantitativa y análisis de dosis génica. En este estudio no se observó ninguna portadora de duplicación. Tanto las mujeres portadoras de deleción como las no portadoras de 23 familias con DMD/DMB se estudiaron con los polimorfismos intragénicos dinucleotidicos D45 y SK12, para construir haplotipos del cromosoma X; 63% de las mujeres analizadas fueron 'informativas' (heterocigotas en este estudio. Se brindó asesoramiento genético después del análisis molecular descrito, dándoles a conocer los riesgos y los métodos de prevención de la DMD/DMB.

  11. Distrofina na diferenciação das distrofias de duchenne e becker estudo imuno-histoquímico comparado com o estádio clínico, enzimas séricas e biópsia muscular

    OpenAIRE

    Lineu Cesar Werneck; Eduardo Bonilla

    1990-01-01

    Foram estudados 55 casos de distrofia muscular progressiva (34 Duchenne, 12 Duchenne com distrofina residual e 9 Becker), comparando idade, época de início e tempo de sintomas, graduação na escala de Vignos e Archibald, níveis de enzimas séricas e presença de distrofina nas biópsias musculares por imunofluorescência. A intensidade dos sintomas, gravidade do quadro clínico, proliferação de tecido ccnjuntivo endomisial e infiltração por tecido adiposo estão inversamente relacionadas à quantidad...

  12. Distrofina na diferenciação das distrofias de duchenne e becker estudo imuno-histoquímico comparado com o estádio clínico, enzimas séricas e biópsia muscular

    Directory of Open Access Journals (Sweden)

    Lineu Cesar Werneck

    1990-12-01

    Full Text Available Foram estudados 55 casos de distrofia muscular progressiva (34 Duchenne, 12 Duchenne com distrofina residual e 9 Becker, comparando idade, época de início e tempo de sintomas, graduação na escala de Vignos e Archibald, níveis de enzimas séricas e presença de distrofina nas biópsias musculares por imunofluorescência. A intensidade dos sintomas, gravidade do quadro clínico, proliferação de tecido ccnjuntivo endomisial e infiltração por tecido adiposo estão inversamente relacionadas à quantidade de distrofina presente nas biópsias e, diretamente, à presença de fibras hipertróficas e fibras angulares escuras atróficas. Nos comentários são abordados alguns aspectos sobre a diferenciação da distrofia muscular de Duchenne e Becker, a distrofina residual nos casos de Duchenne e a importância do teste para o diagnóstico adequado.

  13. Current status on diagnosis and management of Duchenne/Becker muscular dystrophy in Chongqing area%假肥大型肌营养不良的诊治与生存质量分析

    Institute of Scientific and Technical Information of China (English)

    胡君; 蒋莉; 袁召建; 朱进; 孔敏; 叶园珍

    2012-01-01

    Objective To improve the diagnosis and management of Duchenne/Becker muscular dystrophy (DMD/BMD). Methods Clinical data of 294 DMD/BMD cases were collected. Genomic DNA was extracted from the peripheral blood leukocytes using standard procedures and multiple polymerase chain reaction (mPCR) + short tandem repeat (STR) were applied to detect Dystrophin gene mutation. Gastrocnemius muscle biopsies were peroformed on 17 cases without Dystrophin gene mutations. The standard procedure of cross-culture adaptation was used to develop the Chinese version Pediatric Quality of Life Inventory? 3.0 Neuromuscular Module (PedsQLTM 3.0 NMM). The Chinese version PedsQLTM 3.0 NMM was then to performed on 39 patients and their parents. Results In total of 294 cases, ex-ons and STR deletion of Dystrophin were identified in % cases (32.65%) and exons deletions in central hot spot of recombination (exon 44-51) were identified in 82 cases (85.42%). Gastrocnemius muscle biopsies were positive in 14 cases (82.35%) of the 17 cases undergoing, biopsy. Chinese version PedsQLTM 3.0 NMM in 39 cases showed that patients with more severe conditions had lower scores of PedsQLTM 3.0 NMM (P < 0.05) , lower scores in communication and family dimensions (P > 0.05). Among the 101 DMD cases, only 18 were used prednisone with 0.75 mg/kg daily. Conclusions DMD/BMD is not uncommon in Southwest of China. Application of mPCR + STR can improve the detection accuracy of Dystrophin gene mutations but with low positive rate. Muscle biopsy is a good supplementary means for the diagnosis, illness severity is the main factor affecting quality of life (QOL) of DMD/BMD patients. DMD/BMD patients are generally lack of good communication and have a poor family economy. It is very urgent to develop the diagnosis and management of DMD/BMD guideline suitable for China's national conditions.%目的 分析西南地区假肥大型肌营养不良(Duchenne/Becker muscular dystrophy,DMD/BMD)患儿的诊治现状及生存

  14. 假肥大型进行性肌营养不良120例疑诊患者的基因诊断%Genetic Diagnosis of 120 Suspected Cases with Duchenne/Becker Muscular Dystrophy

    Institute of Scientific and Technical Information of China (English)

    贺静; 朱宝生; 唐新华; 李利; 郑淑芳; 陈红; 苏洁; 章印红; 李秀玲

    2011-01-01

    目的 对120例假肥大型进行性肌营养不良(DMD/BMD )疑诊患者进行基因诊断,探讨云南人群DMD基因的缺失分布特点.方法 应用多重PCR法对2004年1月-2010年3月在本院遗传诊断中心门诊就诊的120例疑诊患者(均为男性;就诊年龄1个月~14岁)的DMD基因常见缺失的18个外显子进行检测,按缺失位点分析DMD患者的缺失分布情况.结果 在120例疑诊患者中检出61例DMD基因存在缺失,缺失发生在DMD基因5′端者占总例数的18.97%,缺失发生在基因中央区域者占81.03%.在未检出缺失的59例疑诊患者中,32例通过临床回访,其中24例按临床表现及相关检查临床诊断为DMD,另8例不符合DMD诊断.另有27例失访或不能明确为DMD.结论 云南人群中DMD基因外显子的缺失主要集中于DMD基因中央区域.对DMD疑诊患者DMD基因缺失检测后进行临床回访的临床诊疗具有重要意义.%Objective Genetic diagnosis of 120 cases of suspected Duchenne/Becker muscular dystrophy(DMD/BMD) were carried out to investigate the distribution of DMD gene deletion within populations in Yunnan province.Methods Multiplex polymerase chain reaction (mPCR) was applied for deletion analysis of 18 exons of DMD gene with higher deletion frequency in 120 cases of suspected DMD/BMD ( male,aged from 1 month to 14 years) who visited the Genetic Diagnosis Center of the First People's Hospital of Yunnan Province from Jan.2004 to Mar.2010.Distribution of DMD gene exon deletion was summarized consequently.Results Sixty - one of 120 cases had detected DMD exon deletions.Further analysis showed that 18.97% of gene exon deletions located in 5' -flanking region,81.03% located in central region.Thirty - two cases of 59 cases who had not been detected exon deletion were subjected to follow - up.Among them ,24 cases were diagnosed as DMD according to clinical manifestation and laboratory data, DMD could be excluded for the rest 8 eases.Furthermore ,27 cases

  15. Diferenciação precoce entre distrofia muscular de duchenne e de becker estudo clínico, laboratorial, eletrofisiológico, histoquímico e imuno-histoquímico de 138 casos: clinical, laboratory, electrophysiologic, histochemical and immunohistochemical study of 138 cases

    OpenAIRE

    Lineu C.Werneck; Leila E. Ferraz; Scola,Rosana H.

    1992-01-01

    Foram estudados por análise estatística 194 parâmetros clínicos, laboratoriais, eletrofisiológicos, histoquímicos e imuno-histoquímicos de 112 casos de distrofia muscular de Duchenne (DMD) e de 26 casos de distrofia muscular de Becker (DMB). Houve diferença significante (p < 0,05) entre as duas doenças com respeito a época de início de diversos sintomas, dados do exame neurológico e alterações específicas na biópsia muscular. A biópsia muscular (BM) isolada foi diagnóstica em 52,7% dos casos ...

  16. Correlation between electroretinographic findings, clinical phenotypic and genotypic analysis in Duchenne and Becker muscular dystrophy%进行性肌营养不良患者视网膜眼电图表型与临床分型及基因型的关系

    Institute of Scientific and Technical Information of China (English)

    杨渝; 张成; 盛文利; 潘速跃; 吴德正; 江福钿

    2001-01-01

    Objective To explore the relationship between electrophysiological changes, clinical phenotype and genotype in Duchenne and Becker muscular dystrophy(DMD/BMD), to address the expression and roles of dystrophin and its isoforms on the retina, and to inquire into the molecular mechanism of the abnormal electroretinogram(ERG) on DMD/BMD patients with different genotype.Methods Gene deletions were screened by multiplex DNA amplification with eleven primers on twenty-two consecutive patients with DMD and BMD, and then, the ERG was tested according to international ERG standard.Results ERG phenotype was associated with the site of DMD gene defects rather than the severity of the phenotype. Patients with deletion in the central region of the gene had more severe changes in the scotopic ERG as compared to those with gene non-deletion.Conclusion The ERG genotype-phenotype correlation suggests that DP260 may play the most important role in the retinal neurotransmission.%目的 研究进行性肌营养不良(Duchenne/Becker muscular dystrophy, DMD/BMD)患者视网膜眼电图(electroretinogram,ERG)表型与临床分型以及基因型的关系,进一步探讨不同基因型的DMD患者抗肌营养不良蛋白(dystrophin)及其同源蛋白在视网膜上的表达及功能,揭示DMD出现ERG异常的分子机理。方法 用11对引物对22例临床确诊的DMD/BMD患者作三步多重PCR进行基因缺失分析,并行ERG检查。结果 DMD/BMD患者ERG改变与临床分型及病情严重程度无关,与DMD/BMD的基因型有关,基因中央区缺失型的ERG异常率明显高于基因非缺失型。结论 DMD/BMD的ERG改变与DMD基因突变位点有关,可能DP260转录启动子与视网膜电信号的传导关系最密切。

  17. Diferenciação precoce entre distrofia muscular de duchenne e de becker estudo clínico, laboratorial, eletrofisiológico, histoquímico e imuno-histoquímico de 138 casos: clinical, laboratory, electrophysiologic, histochemical and immunohistochemical study of 138 cases

    Directory of Open Access Journals (Sweden)

    Lineu C. Werneck

    1992-12-01

    Full Text Available Foram estudados por análise estatística 194 parâmetros clínicos, laboratoriais, eletrofisiológicos, histoquímicos e imuno-histoquímicos de 112 casos de distrofia muscular de Duchenne (DMD e de 26 casos de distrofia muscular de Becker (DMB. Houve diferença significante (p < 0,05 entre as duas doenças com respeito a época de início de diversos sintomas, dados do exame neurológico e alterações específicas na biópsia muscular. A biópsia muscular (BM isolada foi diagnóstica em 52,7% dos casos de DMD e em 69,2% dos de DMB. Por imunofluorescência (60 casos a distrofina estava ausente em 87% dos casos de DMD e apresentava falhas focais em todos os casos de DMB. A BM foi concordante com a imunofluorescência para distrofina em 82,6% dos casos de DMD e 71,4% dos de DMB. São feitos comentários a respeito dos dados encontrados e os da literatura, bem como sobre a dificuldade diagnóstica para diferenciação precoce das duas entidades.

  18. 一个假肥大型肌营养不良症伴心脏扩大家系的遗传学及临床研究%Genetic and clinical study on a family with Becker's muscular dystrophy combined with cardiac dilatation

    Institute of Scientific and Technical Information of China (English)

    刘亚欣; 邹玉宝; 蒋雄京; 张慧敏; 吴海英; 郑德裕; 惠汝太; 高凌根; 周宪梁; 宋雷; 王林平; 张琳; 田涛; 孙凯; 王继征

    2012-01-01

    Background Duchenne's or Becker's muscular dystrophy is a serious X- linked neuromuscular disorder. Mutations in the dystro-phin gene on chromosome Xp21.1. have been reported to cause BMD. It primarily involved the skeletal muscle, characterized by weakness and muscle atrophy of hereditary muscle disease following as low progressive increase of symme try. DMD is usually with serious condition and with a poor prognosis. BMD patients can usually walk and have a near normal life span, and there is a chance of them developing cardiac problems. Objective: To study the clinical characteristics and genetic analysis and management of Becker's muscular dystrophy combined with cardiac involvement, and to expand our understanding of this disorder. Methods The proband and the family members had genetic testing, and these subjects also had physical examination and received muscle biopsy and routine pathological checks and cardiac evaluation. One hundred control subjects without diagnostic features of BMD were also recruited. Genomic DNA was extracted from leukocytes of peripheral blood from the patients and the control subjects. We screened the dystrophin gene in the indexes, and also screened for the mutation in their families and 100 controls. Results The proband was diagnosed with BMD by the identification of a mutation( c.4998_5000Del GCA,p.l667del) in the exon 35 of the dystrophin gene. Three cases were diagnosed with BMD by the identification of this mutation. All genetically affected subjects had a history of weakness of the quadriceps femoris as well as increased serum creatine kinase level at rest and cardiac involvement. Conclusion Genetic analysis enables a precise diagnosis of BMD, and heart transplantation is an effective treatment for the patient with severe heart failure.%背景假肥大型进行性肌营养不良是由抗肌萎缩蛋白基因突所致的一种X连锁隐性遗传神经肌肉疾病,又称为杜氏或贝克肌营养不良(Duchenne's or Becker

  19. Errata: Measuring Disease Severity in Duchenne and Becker Muscular Dystrophy

    Directory of Open Access Journals (Sweden)

    Melinda F. Davis

    2011-05-01

    Full Text Available Reports an error in Davis et al. (2010.  The functional motor scale used in Davis et al. (2010 was the EK (Egen Klassifikation Scale, rather than the Amyotrophic Lateral Sclerosis Functional Rating Scale (Steffensen et al., 2002; Cedarbaum & Stambler, 1997.  Both scales are 10-item, disease-specific measures that assess mobility and respiratory function in individuals with progressive muscle weakness.  This error does not change the conclusions. DOI: 10.2458/azu_jmmss.v1i2.12366

  20. 在路上--记Jason Becker(上)

    Institute of Scientific and Technical Information of China (English)

    孔祥龙

    2002-01-01

      如果你是疯狂的摇滚乐迷,你一定会熟悉以下事情:Thin Lizzy的Phil Lynoff英年早逝、John Lennon祭日、Kurt Cobain的自杀身亡或是Joplin的去世.这些事件你可以从几乎所有的音乐网站或杂志甚至是与音乐没什么关系的时尚书籍上得知,这些都是摇滚舞台上众所周知的"新闻".不过有一个故事你以前可能从未从任何的媒体上看到过相关的报道,但马上你就可以看到这个可能是最悲壮的摇滚传奇---吉他演奏家、作曲家Jason Becker的故事.Becker从没有一张销量过百万的唱片,也没有当过主流音乐杂志的封面人物,甚至在音乐电视台里你也不会见到他的身影.……

  1. Duchenne and Becker muscular dystrophy prevalence in South ...

    African Journals Online (AJOL)

    1993-07-28

    Jul 28, 1993 ... County, Georgia: relationship to sodium and potassium intake and plasma renin ... urinary kallikrein concentration in childhood: relation to blood pressure, race and .... clinical course culminating in death in the teenage years.

  2. 77 FR 72387 - Jeffery J. Becker, D.D.S., and Jeffery J. Becker, D.D.S., Affordable Care Decision and Order

    Science.gov (United States)

    2012-12-05

    ... and Order On December 22, 2011, Chief Administrative Law Judge John J. Mulrooney, II, (hereinafter... word ``principal'' ordinarily means the ``most important, consequential, or influential,'' Webster's... Administrative Law Judge John J. Mulrooney, II. On July 28, 2011, the Deputy Assistant Administrator of the...

  3. Effects of Sildenafil on Cerebrovascular Reactivity in Patients with Becker Muscular Dystrophy

    DEFF Research Database (Denmark)

    Lindberg, Ulrich; Witting, Nanna; Lundgaard, Stine J.

    2017-01-01

    level-dependent (BOLD) response and cerebral blood flow (CBF) in patients with BMD. Seventeen patients (mean ± SD age 38.5 ± 10.8 years) with BMD were included in this randomized, double-blind, placebo-controlled, crossover trial. Twelve patients completed the entire study. Effects of sildenafil were...... increased the event-related sensory and visual BOLD response compared with placebo (p related...... neurovascular responses. Further studies in patients with BMD may help clarify the roles of dystrophin and nNOS in neurovascular coupling in general, and in patients with BMD in particular....

  4. Body weight-supported training in Becker and limb girdle 2I muscular dystrophy

    DEFF Research Database (Denmark)

    Jensen, Bente R; Berthelsen, Martin P; Husu, Edith;

    2016-01-01

    (walk/run, jogging in place or high knee-lift) training 3 times/week in a lower-body positive pressure environment. Closed-kinetic-chain leg muscle strength, isometric knee strength, rate of force development (RFD), and reaction time were evaluated. RESULTS: Baseline data indicated an intact neural...... activation pattern but showed compromised muscle contractile properties. Training (compliance 91%) improved functional leg muscle strength. Squat series performance increased 30%, calf raises 45%, and lunges 23%. CONCLUSIONS: Anti-gravity training improved closed-kinetic-chain leg muscle strength despite...... no changes in isometric knee extension strength and absolute RFD. The improved closed-kinetic-chain performance may relate to neural adaptation involving motor learning and/or improved muscle strength of other muscles than the weak knee extensors. Muscle Nerve 54: 239-243, 2016....

  5. Endurance training improves fitness and strength in patients with Becker muscular dystrophy

    DEFF Research Database (Denmark)

    Sveen, Marie Louise; Jeppesen, Tina D; Hauerslev, Simon

    2008-01-01

    Studies in a dystrophinopathy model (the mdx mouse) suggest that exercise training may be deleterious for muscle integrity, but exercise has never been studied in detail in humans with defects of dystrophin. We studied the effect of endurance training on conditioning in patients...... in healthy subjects (16 +/- 2% and 17 +/- 2%). CK levels did not increase with training, and number of central nuclei, necrotic fibres and fibres expressing neonatal myosin heavy chain did not change in muscle biopsies. Strength in muscles involved in cycle exercise (knee extension, and dorsi- and plantar......-flexion) increased significantly by 13-40%. Cardiac pump function, measured by echocardiography, did not change with training. All improvements and safety markers were maintained after 1 year of training. Endurance training is a safe method to increase exercise performance and daily function in patients with BMD...

  6. Carrier detection of duchenne and becker muscular dystrophy using muscle dystrophin immunohistochemistry

    Directory of Open Access Journals (Sweden)

    Acary S. Bulle Oliveira

    1992-12-01

    Full Text Available To ascertain whether dystrophin immunohistochemistry could improve DMD/ BMD carrier detection, we analyzed 14 muscle biopsies from 13 DMD and one BMD probable and possible carriers. All women were also evaluated using conventional methods, including genetic analysis, clinical and neurological evaluation, serum CK levels, KMG, and muscle biopsy. In 6 cases, there was a mosaic of dystrophin-positive and dystrophin-deficient fibers that allowed to make the diagnosis of a carrier state. Comparing dystrophin immunohistochemistry to the traditional methods, it was noted that this method is less sensitive than serum CK measuremens, but is more sensitive than EMG and muscle biopsy. The use of dystrophin immunohistochemistry in addition to CK, EMG and muscle biopsy improved the accuracy of carrier detection. This method is also helpful to distinguish manifesting DMD carriers from patients with other neuromuscular diseases like limb-girdle muscular dystrophy and spinal muscular atrophy.

  7. Das Lübecker Repetitorium "Innere Kompakt" [The Luebeck compact revision course in Internal Medicine

    Directory of Open Access Journals (Sweden)

    Kühn, Johanna

    2007-08-01

    Full Text Available [english] Introduction: The initiation of the amended second state examination in human medicine in autumn 2006 made many students feel insecure. Aim of the Luebeck compact revision course in Internal Medicine was to reassure the students by optimal preparation. Project description: In September 2006, for the first time, 75 students in their final practical year of study participated in the revision course. During eight days, the relevant topics for the second state examination in Internal Medicine were discussed – as lectures in the morning and in small working groups with case presentations in the afternoon. Results: Daily evaluation as well as a detailed evaluation at the end of the course point out the positive and negative aspects of the project. A final grade of 1.92 and a level of recommendation of 100% reflect a very positive feedback. Discussion and conclusion: To ensure a successful realisation of such a course, time and effort may not be underrated: it takes i.e. a structured time-table and motivated tutors as well as a detailed planning and administrative tasks to realise such a project. However, the students´ better preparation for their examinations and jobs compensate the additional time and effort. Therefore, we decided to establish comparable revision courses in Internal Medicine annually, and other clinical areas like Surgery will follow. [german] Einleitung: Die Einführung des neuen zweiten Staatsexamens im Herbst letzten Jahres führte bei vielen Medizinstudenten zu Verunsicherung. Diesem so genannten „Hammerexamen“ durch optimale Vorbereitung seinen Schrecken zu nehmen, war Ziel des neu eingeführten Repetitoriums der Inneren Medizin in Lübeck. Projektbeschreibung: Erstmals im September letzten Jahres nahmen 75 Studierende im Praktischen Jahr am Repetitorium „Innere kompakt“ teil. Innerhalb von acht Tagen wurden die wesentlichen prüfungsrelevanten Inhalte der Inneren Medizin behandelt – vormittags als Vorlesung, nachmittags als interaktiver Kleingruppenunterricht. Ergebnisse: Durch tägliche Evaluierung und eine ausführliche Abschlussevaluation konnten positive Ansätze ebenso wie Schwachstellen ermittelt werden. Bei einer durchschnittlichen Gesamtnote von 1,92 und einer Weiterempfehlungsrate von 100% fiel die Rückmeldung sehr positiv aus. Diskussion und Schlussfolgerung: Um ein gutes Gelingen zu gewährleisten, sollte man den Aufwand eines Repetitoriums nicht unterschätzen: ein strukturierter Stundenplan und engagierte Dozenten gehören ebenso dazu wie sorgfältige Planung und Verwaltungsaufgaben. Die mögliche Mehrbelastung wird jedoch durch gute Prüfungs- und Berufsvorbereitung der Studenten wieder aufgewogen. Dies hat uns in Lübeck dazu bewogen, das Repetitorium in der Inneren Medizin nun jährlich anzubieten und weitere Repetitorien in klinischen Fächern wie der Chirurgie folgen zu lassen.

  8. Becoming a medicinal marijuana user: applying Becker's analysis of recreational cannabis users to a medicinal framework

    OpenAIRE

    Cohen, Elysha

    2015-01-01

    This study examines the process involved in becoming a medicinal marijuana patient, drawing upon Becker’s (1953) analysis of recreational marijuana users as a guide. Semi-structured open ended qualitative interviews were conducted with a purposively chosen sample (n=22) of medical marijuana patients currently using cannabis to alleviate symptom(s) of an underlying medical condition(s). Nine participants (50%) describe a seamless transition without any period of desistance, seven participants ...

  9. On potential enemies and potential targets / Egdunas Racius ; interv. Sven Becker

    Index Scriptorium Estoniae

    Racius, Egdunas

    2006-01-01

    Araabia regiooni tundev spetsialist annab intervjuus ülevaate olukorrast Iraagis, vastates küsimustele, mis puudutavad riigis demokraatia üles ehitamist, terrorismiga toimetulemist ning Leedu sõjaväelaste viibimist Iraagis. Lisa: Egdunas Racius

  10. On potential enemies and potential targets / Egdunas Racius ; interv. Sven Becker

    Index Scriptorium Estoniae

    Racius, Egdunas

    2006-01-01

    Araabia regiooni tundev spetsialist annab intervjuus ülevaate olukorrast Iraagis, vastates küsimustele, mis puudutavad riigis demokraatia üles ehitamist, terrorismiga toimetulemist ning Leedu sõjaväelaste viibimist Iraagis. Lisa: Egdunas Racius

  11. VizieR Online Data Catalog: Hydrogen and Helium EOS in brown dwarfs (Becker+, 2014)

    Science.gov (United States)

    Becker, A.; Lorenzen, W.; Fortney, J. J.; Nettelmann, N.; Schottler, M.; Redmer, R.

    2015-02-01

    We present new equations of state (EOSs) for hydrogen and helium covering a wide range of temperatures from 60K to 107K and densities from 10-10g/cm3 to 103g/cm3. They include an extended set of ab initio EOS data for the strongly correlated quantum regime with an accurate connection to data derived from other approaches for the neighboring regions. We compare linear mixing isotherms based on our EOS tables with available real mixture data. A first important astrophysical application of this new EOS data is the calculation of interior models for Jupiter and comparison with recent results. Second, mass-radius relations are calculated for Brown Dwarfs (BDs) which we compare with predictions derived from the widely used EOS of Saumon, Chabrier, and van Horn. Furthermore, we calculate interior models for typical BDs with different masses, namely, Corot-3b, Gliese-229b, and Corot-15b, and the giant planet KOI-889b. The predictions for the central pressures and densities differ by up to 10% dependent on the EOS used. (2 data files).

  12. 如果我仍能弹——访Jason Becker

    Institute of Scientific and Technical Information of China (English)

    2004-01-01

    有一个故事你以前可能从未从任何的媒体上看到过相关的报道,但马上你就可以看到这个可能是最悲壮的摇滚传奇——吉他演奏家/作曲家Jason Becker的故事。Becker从没有一张销量过百万的唱片,也没有当过主流音乐杂志的封面人物,甚至在音乐电视台里你也不会见到他的身影……

  13. Progress on Diagnosis and Treatment in Becker Nevus%Becker痣的诊疗进展

    Institute of Scientific and Technical Information of China (English)

    许庆强; 闫小宁

    2015-01-01

    目的 回顾色素性毛表皮痣病因与发病机制、临床表现与治疗的相关文献,探讨临床治疗策略方法 对色素性毛表皮痣相关文献进行描述与总结.结果 色素性毛表皮痣目前病因及发病机制尚不清楚,是一种良性皮肤错构瘤,可伴发皮肤肌肉骨骼发育异常,称为Becker痣综合征;单纯皮肤症状可尝试采用激光治疗,合并其他发育缺陷可采用手术治疗.结论 色素性毛表皮痣病因及发病机制尚不清楚,激光是常用治疗单纯皮肤异常的方法.

  14. Russia puts Europe to a test of morality / Vytautas Landsbergis ; interv. Sven Becker

    Index Scriptorium Estoniae

    Landsbergis, Vytautas

    2006-01-01

    Euroopa Parlamendi liige vastab küsimustele, mis puudutavad Leedut Euroopa Liidu liikmesriigina, Euroopa Liidu ja Venemaa suhteid, Vene-Saksa gaasitoru ja Leedu sisepoliitikat. Lisa: Vytautas Landsbergise CV

  15. A NEW APPROACH TO GENE DIAGNOSIS OF DUCHENNE/BECKER MUSCULAR DYSTROPHY AMPLIFIED FRAGMENT LENGTH POLYMORPHISMS

    Institute of Scientific and Technical Information of China (English)

    许顺斌; 黄尚志; 罗会元

    1994-01-01

    Four (CA), repeats, located in introns,44,45,49 and 50 of the dystrophin gene,were evaluated in Chinese.These loci are highly polymorphic,with polymorphism information contents of 0.872,0.772,0.870 and 0.718,respectively.All four loci can be easily amplified and labelled using two duplex PCR reactions with α-32P-dCTP and can be detected by denaturing polyacrylamide gel electrophoresis.Using these four loci and the two polymorphic(CA)n repeats located at the 5′ and 3′ ends of the dystrophin gene,we have developed a new PCR-based procedure-Amp-FLP( amplified fragment length polymorphism)linkage analysis for the gene diagnosis of DMD/BMD.This method can detect intragenic recombination rapidly and efficiently and greatly improves the success rate of carrier deterction and prenatal diagnosis in non-deletion DMD/BMD families.All of the loci used in this procedure are intragenic.In addition ,the loci in introns 44,45,49 and 50 are located in the deletion-prone region of the dystrophin gene,making them valuable and usefui in the identification of deletion mutations.Here we report one case of deletion detection using these four loci.

  16. Russia puts Europe to a test of morality / Vytautas Landsbergis ; interv. Sven Becker

    Index Scriptorium Estoniae

    Landsbergis, Vytautas

    2006-01-01

    Euroopa Parlamendi liige vastab küsimustele, mis puudutavad Leedut Euroopa Liidu liikmesriigina, Euroopa Liidu ja Venemaa suhteid, Vene-Saksa gaasitoru ja Leedu sisepoliitikat. Lisa: Vytautas Landsbergise CV

  17. Saksa pankur teotab immigrante ja juute / Inga Höglund

    Index Scriptorium Estoniae

    Höglund, Inga

    2010-01-01

    Saksa pankuri ja poliitiku Thilo Sarrazini raamatus "Saksamaa hävitab end" käsitletakse immigratsioonipoliitika küsimusi, paljusid tema arusaamu võib pidada rassistlikuks, antisemitistlikuks või islamivastaseks

  18. Complexo distrofina-gllcoproteínas - a propósito de um doente com distrofia de Becker

    OpenAIRE

    Monteiro, Cecília; Taipa, Ricardo; Melo Pires, Manuel; Guimarães, António; Gonçalves, Ana Rita; Vieira, Emília; Santos, Rosário; Santos, Manuela

    2012-01-01

    Introdução: A distrofina faz parte do complexo distrofina-glicoproteínas (DAG - dystrophin associated glicoproteins) que inclui as sarcoglicanos α, β, y e δ, bem com as distroglicanos α e β. O estudo imunohistoquímico da biopsia muscular das distrofinopatias pode assim mostrar alterações na marcação não só da distrofina, como também das outras proteínas associadas. Caso clínico: Apresentamos um doente do sexo masculino com quadro clínico caracterizado por cãibras e dores musculares d...

  19. Detection of Duchenne/Becker Muscular Dystrophy Carriers in a Group of Iranian Families by Linkage Analysis

    Directory of Open Access Journals (Sweden)

    Fardeen Ali Malayeri

    2011-03-01

    Full Text Available This study determines the value of linkage analysis using six RFLP markers for carrier detection and prenatal diagnosis in familial DMD/BMD cases and their family members for the first time in the Iranian population. We studied the dystrophin gene in 33 unrelated patients with clinical diagnosis of DMD or BMD. Subsequently, we determined the rate of heterozygosity for six intragenic RFLP markers in the mothers of patients with dystrophin gene deletions. Finally, we studied the efficiency of linkage analysis by using RFLP markers for carrier status detection of DMD/BMD. In 63.6% of the patients we found one or more deletions. The most common heterozygous RFLP marker with 57.1% heterozygosity was pERT87.15Taq1. More than 80% of mothers in two groups of familial or non-familial cases had at least two heterozygous markers. Family linkage analysis was informative in more than 80% of the cases, allowing for accurate carrier detection. We found that linkage analysis using these six RFLP markers for carrier detection and prenatal diagnosis is a rapid, easy, reliable, and inexpensive method, suitable for most routine diagnostic services. The heterozygosity frequency of these markers is high enough in the Iranian population to allow carrier detection and prenatal diagnosis of DMD/BMD in more than 80% of familial cases in Iran.

  20. Becker muscular dystrophy patients with deletions around exon 51; a promising outlook for exon skipping therapy in Duchenne patients.

    NARCIS (Netherlands)

    Helderman-van den Enden, A.T.; Straathof, C.S.; Aartsma-Rus, A.; Dunnen, J.T. den; Verbist, B.M.; Bakker, E.; Verschuuren, J.J.; Ginjaar, H.B.

    2010-01-01

    Theoretically, 13% of patients with Duchenne muscular dystrophy may benefit from antisense-mediated skipping of exon 51 to restore the reading frame, which results in the production of a shortened dystrophin protein. We give a detailed description with longitudinal follow up of three patients with B

  1. Deletion of exon 26 of the dystrophin gene is associated with a mild Becker muscular dystrophy phenotype

    DEFF Research Database (Denmark)

    Witting, Nanna; Duno, Morten; Vissing, John

    2011-01-01

    calf hypertrophy was noted. Creatine kinase was normal or raised maximally to 500 U/l. The muscle biopsy was myopathic with increased fiber size variation and many internal nuclei, but no dystrophy. No comorbidity was found. In both cases, western blot showed a reduced dystrophin band. Genetic...... associated with an exon 26 deletion. The proband, a 23-year-old man, had slightly delayed motor milestones, walking 1 1/2 years old. He had no complaints of muscle weakness, but had muscle pain. Clinical examination revealed no muscle wasting or loss of power, but his CK was 1500-7000 U/l. Muscle biopsy...... showed dystrophic changes. He had comorbidity with dystonia, slight mental retardation, low stature and neuropathy. The brother of the proband's mother came to medical attention when he was 43 years old. He complained about muscle pain. On examination, a MRC grade 4+ hip extention palsy and a discrete...

  2. 补脑膏治疗 Becker 型进行性肌营养不良症1例

    Institute of Scientific and Technical Information of China (English)

    郑晓霞; 王英瑞; 张英菊; 李妍怡

    2015-01-01

    目的:记录及分析中医补脑膏治疗临床诊断的Becker型进行性肌营养不良症( BMD),为临床治疗提供参考。方法对该例进行性营养不良患者的病历资料进行回顾性分析。结果补脑膏治疗该病例取得良好效果。结论补脑膏治疗Becker型进行性肌营养不良症在此例中取得显著疗效,但此报道中临床病案有限,需进一步的收集、分析本病的临床资料,使补脑膏在治疗本病上取得更大的发展空间。

  3. Deletion of exon 26 of the dystrophin gene is associated with a mild Becker muscular dystrophy phenotype

    DEFF Research Database (Denmark)

    Witting, Nanna; Duno, Morten; Vissing, John

    2011-01-01

    calf hypertrophy was noted. Creatine kinase was normal or raised maximally to 500 U/l. The muscle biopsy was myopathic with increased fiber size variation and many internal nuclei, but no dystrophy. No comorbidity was found. In both cases, western blot showed a reduced dystrophin band. Genetic...... associated with an exon 26 deletion. The proband, a 23-year-old man, had slightly delayed motor milestones, walking 1 1/2 years old. He had no complaints of muscle weakness, but had muscle pain. Clinical examination revealed no muscle wasting or loss of power, but his CK was 1500-7000 U/l. Muscle biopsy...... showed dystrophic changes. He had comorbidity with dystonia, slight mental retardation, low stature and neuropathy. The brother of the proband's mother came to medical attention when he was 43 years old. He complained about muscle pain. On examination, a MRC grade 4+ hip extention palsy and a discrete...

  4. Tamizaje de deleciones en pacientes con distrofia muscular de Duchenne (DMD) o Becker-Kiener (BMD) mediante PCR multiplex en Costa Rica, 1998-2000

    OpenAIRE

    Sancho-Fernández, Vanessa M.; Saborio, Manuel; de Céspedes-Montealegre, Carlos; Azofeifa-Navas, Jorge

    2001-01-01

    Artículo científico -- Universidad de Costa Rica, Instituto de Investigaciones en Salud. 2001 Objetivo: Iniciar los estudios genético moleculares sobre las distrofinopatías en Costa Rica. Materiales y Métodos: Treinta y un pacientes varones, diagnosticados con distrofia muscular, que podrían ser distrofinopáticos fueron reevaluados clínicamente. Veintitrés mostraron un fenotipo de DMD y dos de BMD. Seis no mostraron síntomas definitivos de distrofinopatías. ADN de los pacientes fue analiza...

  5. Identificación de mutaciones y diagnóstico molecular de portadoras en familias mexicanas con distrofia muscular Duchenne/Becker

    Directory of Open Access Journals (Sweden)

    S. Canizales

    2008-01-01

    Discusión o Conclusión: El porcentaje de eliminaciones genéticas en los pacientes estudiados fue menor al reportado en otras poblaciones. Un estudio con un mayor número de individuos permitirá saber sí esta baja frecuencia es característica de nuestra población. El uso de estudios de ligamiento genético y de RT-PCR aumenta la posibilidad de definir el estado portador en mujeres pertenecientes a familias con DMD/BMD y así poder ofrecerles un adecuado consejo genético. Este es el primer trabajo realizado en México, donde se usó el RT-PCR como herramienta diagnóstica en esta distrofia, se identificó por primera vez, a nivel molecular, un paciente con Síndrome de genes continuos y se demostró un empalme alternativo en un paciente con esta miopatía para explicar su fenotipo.

  6. IDENTIFICACIÓN DE DELECIONES EN AFECTADOS DE DISTROFIA MUSCULAR DE DUCHENNE Y BECKER (DMD/DMB Y DIAGNÓSTICO DE PORTADORAS POR METODOLOGÍAS MOLECULARES

    Directory of Open Access Journals (Sweden)

    Patricia Hernández Rodríguez

    2002-06-01

    Full Text Available Se diseñó un ensayo de PCR múltiplex (6-plex que amplifica simultáneamente 6 exones del gen de la distrofina, estos exones son los que presentan mayor frecuencia de mutación. La proporción de deleciones observada en este estudio mediante el sistema 6-plex correspondió al31,25%, además el60% del total de las deleciones involucró los exones 44 al 52. Con el fin de identificar mujeres portadoras de DMD y DMB se utilizó el cálculo de dosis génica, a través de esta metodología fueron identificadas 7 mujeres como portadoras y 15 como no portadoras de deleción para los exones analizados, en este estudio no se encontró ninguna mujer como portadora de duplicación. Con la utilización de polimorfismos dinucleotídicos (CAn localizados en el interior del gen fue posible establecer inforrnación sobre el cromosoma X que posiblemente está afectado en el 63% de las mujeresanalizadas.

  7. The regulation of market communication and market behaviour: corporate social responsibility and the directives on unfair commercial practices and unfair contract terms / Anna Beckers

    Index Scriptorium Estoniae

    Beckers, Anna

    2017-01-01

    Ettevõtete sotsiaalse vastutuse poliitika õiguslikust määratlemisest ELi õiguses tarbijakaitse direktiivide 2005/29/EÜ (ebaausad kaubandustavad ettevõtjate ja tarbijate vahel) ja 1993/13/EMÜ (ebaõiglased tingimused tarbijalepingutes) taustal

  8. A HindIII polymorphism detected by cDMD 4-5a at the DMD locus in a family with Becker muscular dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Gibb, M.F.; Greenberg, C.R.; Carson, N.L. [Health Sciences Centre, Winnipeg, Manitoba (Canada)

    1994-09-01

    Deletions within the dystrophin gene can be detected by hybridizing a series of cDNA probes to HindIII-digested DNA, with the absence of one or more fragments indicating the presence of a deletion. However, incorrect interpretations can be made if the absence of a fragment is due to a polymorphism rather than a deletion. Otto and Rothbery reported that the 5.2 kb fragment detected by cM 4-5a could be resolved, with extended electrophoresis, into two fragments estimated to be 5.2 and 5.15 kb in size. They concluded that the extra fragment of this doublet appears to be polymorphic, inherited in a Mendelian dominant fashion. The mother, who is an obligate carrier of BMD, does not have the upper fragment as is the case for her normal and affected sons. The father, who clinically has no evidence of neuromuscular disease, does have the upper fragment as do all their daughters. Given a dominant pattern of inheritance, the daughters should be heterozygous. Analysis of one grandson, who was predicted to have inherited the grandpaternal dystrophin gene, showed that he did have the upper fragment, consistent with our conclusions. To date, we have been unable to analyze a grandson that has inherited the grandmaternal allele; however, presuably he would not have the upper fragment of this doublet. We conclude that there likely is a dominant HindIII polymorphism detected with the cDMD 4-5a probe at the DMD locus. Population studies will be required to determine the frequency of this polymorphism; however, it should be noted that absence of the upper fragment of this doublet in a male with BMD/DMD does not necessarily correspond to the presence of a deletion.

  9. Re cognition of sex discrimination: improvement of Becker theory%重新认识性别歧视——对Becker理论的改进

    Institute of Scientific and Technical Information of China (English)

    韩世鹏

    2007-01-01

    性别歧视是一种全球性现象,它使女性在就业和收入方面处于社会的较低层次,而且也不必要的降低了经济效率.本文通过分析男女雇员由于个性特征差别引起的工资差异的重要性,将雇主的偏好划分为主动性歧视和被动性歧视,并重新认识性别歧视及Becker关于歧视的理论.从而对解决性别歧视的对策与方法产生了重要的影响.

  10. Becker型肌营养不良心脏受累一例报道%A Case Report of Cardiac Involvement by Becker Type Muscular Dystrophy

    Institute of Scientific and Technical Information of China (English)

    张大东; 孙寅光

    2001-01-01

    目的探讨Becker型肌营养不良症心脏受累的临床特点.方法对1例Becker型肌营养不良的临床资料进行回顾性分析.结果 33岁男性患者,少年时起病,四肢肌力减退,肌肉萎缩呈进行性加重,肌酸激酶持续异常升高,肌电图示肌源性损伤,DNA检查提示DMD基因缺陷.29岁时发现心脏受累.结论 Becker型肌营养不良症进展缓慢,心电图、心肌酶等检查有助于心肌受累的早期诊断;早检出病变基因携带者可防止该病的遗传发生.

  11. Becker与Grossman健康需求模型的理论分析%A Theoretical Analysis of Becker and Grossman Health Demand Model

    Institute of Scientific and Technical Information of China (English)

    王小万; 刘丽杭

    2006-01-01

    从经济学的角度,在阐述健康需求模型(Becker模型与Grossman模型)的基础上,系统分析了健康需求的基本理论,以及健康需求与医疗服务需求之间的相互关系,并围绕影响居民健康与医疗服务需求的有关因素进行了系统而具体的分析,希望能够为我国卫生改革与政策制定在理论上的突破寻求一些基本的理论依据.

  12. D-Optimal Designs of Parameter Estimation for the Becker Homogeneous Models%Becker齐次模型参数估计的D-最优设计

    Institute of Scientific and Technical Information of China (English)

    关颖男; 薛海玲

    1998-01-01

    对于三分量混料试验的Becker模型(Ⅰ),(Ⅱ)和(Ⅲ),给出了参数估计的D-最优设计.利用偏微分方程中椭圆型方程的极值原理和对称多项式这两种数学工具解决了它们的最优设计问题.

  13. Becker型肌营养不良一例报道及病理分析%Immunohistochemical and Electric Microscope Diagnosis of Becker Muscular Dystrophy: A Case Report

    Institute of Scientific and Technical Information of China (English)

    丁肖媛; 盛德俊; 沈朝斌; 龚建宏; 戴强; 吴岚莹

    2010-01-01

    本病例报道通过免疫组化和肌肉超微结构改变等病理指标方法确诊1例幼儿为Becker型肌营养不良.本文回顾和综述了该病种在诊断方面的进展,探讨了该疾病早期诊断的可能性和免疫组化病理的重要性.

  14. Prime Contract Awards Alphabetically by Contractor, by State or Country, and Place, Fiscal Year 1987. Part 2. American Steel & Wldg. Company-Becker Sand & Gravel Company, Incorporated.

    Science.gov (United States)

    1987-01-01

    t 4 -N(-1N "O 1-It z ) 0 UI4N -" niL Cv44Cl) l * RtU )L OClCC O(w0 3 0 a) CYOa)a)0)0 C0 0 0 0 4l ’ 0 N 𔃻 (n.Jm-4N - tTC L n n(Dt (0( DC r -P ,0 00 ’-4...14 -4 :.- ISA PA c ic C-4 -4 L)I C041-4 -. -4 m4s( 0 ~ -14 0 tO x4 -4-0 -6 -4 2:4,4 NE CY U4 c-4 - L,4 -44 441.4 -4- 005(0.440 00 In In m0 40 mu 00

  15. IDENTIFICACIÓN DE DELECIONES EN AFECTADOS DE DISTROFIA MUSCULAR DE DUCHENNE Y BECKER (DMD/DMB) Y DIAGNÓSTICO DE PORTADORAS POR METODOLOGÍAS MOLECULARES

    OpenAIRE

    Patricia Hernández Rodríguez; Carlos Martín Restrepo

    2013-01-01

    Se diseñó un ensayo de PCR múltiplex (6-plex) que amplifica simultáneamente 6 exones del gen de la distrofina, estos exones son los que presentan mayor frecuencia de mutación. La proporción de deleciones observada en este estudio mediante el sistema 6-plex correspondió al31,25%, además el60% del total de las deleciones involucró los exones 44 al 52. Con el fin de identificar mujeres portadoras de DMD y DMB se utilizó el cálculo de dosis génica, a través de esta metodología fueron identificada...

  16. Juhtimine ja juhtiv kultuur / Jürgen Habermas

    Index Scriptorium Estoniae

    Habermas, Jürgen, 1929-

    2010-01-01

    Autor kirjutab Saksamaa ühiskondlikest ja poliitilistest suundumustest. Probleemidest sisserännanutega, üha suurenevast toetusest mittepoliitilistele tegelastele poliitikasfääris ja vastuseisust parteide poliitikale, kodanikualagatuslikust vastuhakust. Thilo Sarrazini raamatust "Saksamaa kaotab end ära"

  17. Kas keskpankur ei tohi sisserändajaid sarjata? / Kaivo Kopli

    Index Scriptorium Estoniae

    Kopli, Kaivo

    2010-01-01

    Saksa keskpanga juhatuse liige Thilo Sarrazin on esitanud immigrantide, muslimite jt. kohta kriitilisi seisukohti. Keskpanga juhatus on palunud Sarrazini ametist vabastamist, küsitluste andmeil ei soovi 50-95% sakslastest pankuri vallandamist ning toetab kriitikat immigrantide ja integratsiooni valdkonnas

  18. Populism või normaliseerumine? / Jeroen Bult

    Index Scriptorium Estoniae

    Bult, Jeroen

    2010-01-01

    Autor leiab, et Saksamaa kõrge riigiametniku Thilo Sarrazini raamat "Deutschland schafft sich ab. Wie wir unser Land aufs Spiel setzen", milles ta kritiseerib islamiusulisi sisserändajaid, võiks algatada rahvusliku debati: millist laadi immigrante on vananeva elanikkonnaga Saksamaale vaja

  19. Juhtimine ja juhtiv kultuur / Jürgen Habermas

    Index Scriptorium Estoniae

    Habermas, Jürgen, 1929-

    2010-01-01

    Autor kirjutab Saksamaa ühiskondlikest ja poliitilistest suundumustest. Probleemidest sisserännanutega, üha suurenevast toetusest mittepoliitilistele tegelastele poliitikasfääris ja vastuseisust parteide poliitikale, kodanikualagatuslikust vastuhakust. Thilo Sarrazini raamatust "Saksamaa kaotab end ära"

  20. Merkel kaitses tuliselt sõnavabadust / Inga Höglund

    Index Scriptorium Estoniae

    Höglund, Inga

    2010-01-01

    Saksamaal otsustati tunnustada taani karikaturisti Kurt Westergaardi 5 aastat tagasi joonistatud Muhamedi karikatuuri eest. Kurt Westergaardi ja ajakirjandusvabaduse tunnustamiseks lavale astunud Saksamaa kansleri Angela Merkeli kõnest. Angela Merkel leidis, et endise keskpanga nõukogu juhtkonda kuulunud Thilo Sarrazini islamivaenulikke sõnavõtte ei saa karikatuuriga võrrelda, kuna kõrgel riigiametnikul ei ole sobiv esineda taoliste sõnavõttudega

  1. Identificación de portadoras de distrofia muscular de Duchenne y Becker (DMD/DMB) mediante análisis de dosis génica y polimorfismos de DNA

    OpenAIRE

    Restrepo C. M.; Silva C. T.; Gómez Y.; Hernández P.

    2001-01-01

    La DMD/DMB es la distrofia muscular más común en el hombre, se hereda de manera Ligada al Sexo Recesiva y se debe, en muchos casos, a deleciones de uno o más exones del gen de la Distrofina; esta ocurre por mutaciones "de novo" (40%), aunque en los casos familiares, la madre y algunas mujeres por línea materna serán portadoras con un riesgo de 50% de hijos afectados, identificación que es difícil en ausencia de estudios de DNA. El presente estudio identifica mujeres portadoras mediante el aná...

  2. Nuevos casos de coccidiosis bovina en León. Denuncia de Eimeria bovis (Züblin,1908) Fiebiger, 1912, E. auburnensis Christensen y Porter, 1939 y E. ellipsoidalis Becker y Frye, 1929

    OpenAIRE

    Cordero del Campillo, Miguel

    1981-01-01

    P. 61-72 Se estudian nuevos focos de coccidiosis bovina en la provincia de León. En la zona montañosa del NE, se identifica, nuevamente Eimeria zürni más Eimeria auburnensis. En las cercanías de la ciudad de León se comprobó la existencia de Eimeria bovis, Eimeria auburnensis y Eimeria ellipsoidalis. La máxima frecuencia corre a cargo de Eimeria zürni y Eimeria bovis, dotadas también de mayor poder patógeno. Los datos morfológicos de Eimeria zürni concuerdan con los estudiados por el autor...

  3. Multiple PCR for detecting the deletion of DMD gene exon in patients with Duchenne/Becker muscular dystrophy%多重PCR检测DMD/BMD患者DMD基因外显子缺失的实验研究

    Institute of Scientific and Technical Information of China (English)

    郑世珍; 胡华; 李瑾; 李福祥; 邓志宽; 李真; 陈琰; 陈华萍; 戢福云

    2010-01-01

    目的 探索建立Duchenne/Becker肌营养不(DMD/BMD)的稳定高效的基因诊断方法,为该疾病的临床鉴别诊断提供参考.方法 收集2008年5月-2010年5月在第三军医大学新桥医院就诊的汉族男性DMD/BMD患者64例,年龄0.7~45(9.3±1.1)岁.抽取患者外周静脉血5ml,提取基因组DNA后,采用多重PCR(mPCR)扩增DMD/BMD患者DMD基因缺失热区内的18个外显子,确定DMD/BMD患者DMD基因外显子缺失类型.结果 64例DMD/BMD患者中,31例(48.44%)存在DMD基因缺失热区内的不同外显子缺失.其中,外显子50缺失频率最高,为35.48%(11/31);外显子47和43次之,分别为32.26%和25.81%(10/31和8/31),外显子45和49缺失率均为19.35%(6/31),外显子19和48缺失率均为16.13%(5/31).缺失的外显子主要集中在DMD基因的中央缺失热区和5'端缺失热区.结论 目前国内外采用的mPCR方法在DMD/BMD患者的临床诊断中发挥了重要作用.探索DMD基因其他缺失热点、重复突变热点或点突变热点,并以此为依据建立新的DMD/BMD基因诊断方法仍然是提高DMD/BMD基因诊断率的发展方向.

  4. Detection deleted Duchenne and Becker muscular dystrophy patients by DNA microarray%应用DNA微阵列技术检测缺失型DMD/BMD患者的研究

    Institute of Scientific and Technical Information of China (English)

    杜文津; 万琪; 陈晋文; 张改华

    2008-01-01

    目的 研究Duchenne型肌营养不良(DMD)/Becker型肌营养不良(BMD)患者基因缺失检测的可行技术.方法 应用分子克隆的方法扩增DMD基因18个常见易缺失外显子片段,以此作为探针制备出简易DNA微阵列,对30例DMD/BMD患者和5例健康对照的基因进行检测分析.部分结果与PCR方法比较结果一致性.结果 应用简易DNA微阵列检测出21例DMD/BMD患者具有不同程度的外显子缺失,10例经PCR检测得到了完全验证.结论 DNA微阵列技术检测缺失型DMD/BMD患者简便、准确、灵敏,具有临床应用价值.

  5. Identificación de portadoras de distrofia muscular de Duchenne y Becker (DMD/DMB mediante análisis de dosis génica y polimorfismos de DNA

    Directory of Open Access Journals (Sweden)

    Restrepo C. M.

    2001-06-01

    Full Text Available La DMD/DMB es la distrofia muscular más común en el hombre, se hereda de manera Ligada al Sexo Recesiva y se debe, en muchos casos, a deleciones de uno o más exones del gen de la Distrofina; esta ocurre por mutaciones "de novo" (40%, aunque en los casos familiares, la madre y algunas mujeres por línea materna serán portadoras con un riesgo de 50% de hijos afectados, identificación que es difícil en ausencia de estudios de DNA. El presente estudio identifica mujeres portadoras mediante el análisis de dosis génica y/o polimorfismos intragénicos de DNA (1-7.

  6. Duchenne/Becker肌营养不良骨骼肌病变的超微结构特征%Ultrastructural features of skeletal muscle in the patients with Duchenne/Becker muscular dystrophy.

    Institute of Scientific and Technical Information of China (English)

    蔡莉静; 李丽; 曹兴国; 朱海青; 殷国庆

    2009-01-01

    目的 探讨Duchenne/Becker营养不良(D/B MD)患儿肌细胞损伤的特征性超微结构改变.方法 2000年6月至2007年7月对东南大学医学院附属南京第二医院收治的6例D/BMD进行临床分析和肌肉组织的组织学检查.进行ATP(pH 9.4)、NADH-TR酶组织化学染色、抗肌萎缩蛋白免疫组化染色,对超微结构进行观察分析.结果 通过酶组织化学和免疫组织化学检测,此6例均明确诊断为D/BMD.超微结构检查:同一标本中可见肌纤维病变轻重不一,损伤肌纤维均有肌浆膜破坏,细胞外基质、基底膜破坏,肌细胞边缘有成束的细丝.严重病变细胞内肌丝、肌节、线粒体、核均有破坏.结论D/B MD患儿骨骼肌细胞普遍存在肌浆膜和基底膜损伤.

  7. Detection of Duchenne and Becker muscular dystrophy patients by DNA microarray%DNA微阵列技术检测Duchenne型/Becker型肌营养不良患者的临床应用研究

    Institute of Scientific and Technical Information of China (English)

    杜文津; 万琪; 吴保仁; 闫小君

    2003-01-01

    目的研究检测Duchenne 型肌营养不良(DMD)/Becker型肌营养不良(BMD)患者基因缺失的可行技术.方法应用分子克隆的方法扩增DMD基因18个常见易缺失外显子片段,以此作为探针制备出简易DNA微阵列,对30例DMD/BMD患者和5例健康对照的基因进行检测分析.部分结果与PCR的方法作了比较.结果应用简易DNA微阵列检测出21例DMD/BMD患者具有不同程度的外显子缺失,10例经PCR检测得到了完全验证.结论 DNA微阵列技术检测DMD/BMD患者简便、准确、灵敏,可在临床诊断中应用.

  8. DMD基因点突变致Becker型肌营养不良症临床研究%Clinical study of DMD gene point mutation causing Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    操基清; 杨娟; 李亚勤; 冯善伟; 陈菲; 郑卉; 梁颖茵; 赵保健; 张旭

    2015-01-01

    研究背景 DMD基因点突变,主要是无义突变,可以引起基因编码提前终止,使产生的目的蛋白不稳定而降解,导致临床症状较重的Duchenne型肌营养不良症,而在实际工作中可见临床表型为症状较轻的Becker型肌营养不良症的DMD点突变患者.本研究旨在探讨DMD基因点突变导致Becker型肌营养不良症的发病机制,以加深对Becker型肌营养不良症基因突变类型的认识.方法 共11例临床和肌肉活检明确诊断、多重连接依赖性探针扩增(MLPA)显示DMD基因外显子非缺失或重复突变的Becker型肌营养不良症患者,高通量第2代DNA测序法检测DMD基因外显子突变类型.结果11例Becker型肌营养不良症患者携带10种突变类型,无突变热点;6例携带无义突变[c.5002G>T,p.(Glu 1668X);c.1615C>T,p.(Arg539X);c.7105 G>T,p.(Glu2369X);c.5287C>T,p.(Arg 1763X);c.9284T>G,p.(Leu3095X)];1例携带错义突变[c.5234G>A,p.(Arg1745His)];2例携带框移突变(c.10231dupT,c.10491delC);2例携带剪切位点突变(c.4518+3A>T,c.649+ 2T>C).结论 DMD基因点突变可以引起临床症状较轻的Becker型肌营养不良症,当MLPA技术显示DMD基因为非缺失和重复突变时,切勿漏诊Becker型肌营养不良症,研究其发生机制对基因治疗Duchenne型肌营养不良症有重要借鉴意义.

  9. FSI 晶圆表面处理技术的专家--访FSI半导体设备有限公司市场营销副总裁Dr.Scott Becker

    Institute of Scientific and Technical Information of China (English)

    陈宏

    2006-01-01

    @@ Scott Becker现任FSI公司市场营销副总裁.Becker博士从1983年到1985年在FSI担任工艺开发工程师.1992年,他再次加入FSI并担任单晶圆产品经理,1999年兼任FSI首席技术专家.

  10. Tn7-mediated Introduction of DNA into Bacmid-cloned Pseudorabies Virus Genome for Rapid Construction of Recombinant Viruses

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    lacZα-mini-attTn7 was inserted into the intergenic region between the gG and gD genes in a PRV bacterial artificial chromosome (BAC) by homologous recombination in E. coli. The resulting recombinant BAC (pBeckerZF1) was confirmed by PCR and sequencing. Green fluorescent protein (GFP) gene was then transposed into pBeckerZF1 by transposon Tn7 to generate pBeckerZF2. Recombinant viruses vBeckerZF1 and vBeckerZF2 were generated by transfection with the corresponding BAC pBeckerZF1 or pBeckerZF2. The titers and cytopathic effect (CPE) observed for by vBeckerZF1 and vBeckerZF2 was comparable to that of the parental virus vBecker3. vBeckerZF2 was serial passaged for five rounds in cell culture, and the mini-Tn7 insertion was stably maintained in viral genome. These results show that recombinant viruses can be rapidly and reliably created by Tn7-mediated transposition. This technology should accelerate greatly the pace at which recombinant PRV can be generated and, thus, facilitate the use of recombinant viruses for detailed mutagenic studies.

  11. Chordal Loewner chains with quasiconformal extensions

    OpenAIRE

    2015-01-01

    In 1972, Becker [J. Reine Angew. Math. 255 (1972), 23-43] discovered a construction of quasiconformal extensions making use of the classical radial Loewner chains. In this paper we develop a chordal analogue of Becker's construction. As an application, we establish new sufficient conditions for quasiconformal extendibility of holomorphic functions and give a simplified proof of one well-known result by Becker and Pommerenke for functions in the half-plane [J. Reine Angew. Math. 354 (1984), 74...

  12. Comment on "Search for Muon Neutrino Oscillations with the Irvine-Michigan-Brookhaven Detector"

    CERN Document Server

    Sulak, J M LoSecco L R

    2016-01-01

    The neutrino flux used in Becker-Szendy {\\em et al.}\\cite{BZ} is now known to be in error. Becker-Szendy {\\em et al.}\\cite{BZ} depended heavily on the Lee and Koh flux\\cite{LeeKoh} which was noted as erroneous in Gaisser {\\em et al.}\\cite{Bug}. This makes the results of Becker-Szendy {\\em et al.}\\cite{BZ} unreliable.

  13. Submarine Combat Systems Engineering Project Capstone Project

    Science.gov (United States)

    2011-06-06

    Combat Systems Engineering Project Capstone Project by John Becker Denman Sweetman Shaun Cookinham Mark Wasilewski Shawn Goode Samuel D...This report was prepared by: John Becker Denman Sweetman Shaun Cookinham Mark Wasilewski Shawn Goode Samuel D. Winograd David Rhodes...ELEMENT NUMBER 6. AUTHOR(S) John Becker, Shaun Cookinham, Shawn Goode, David Rhodes 5d. PROJECT NUMBER Mark Wasilewski , Samuel Winograd 5e. TASK

  14. 77 FR 2085 - Notice of Inventory Completion: Minnesota Indian Affairs Council, Bemidji, MN

    Science.gov (United States)

    2012-01-13

    ..., Becker County, MN, by unknown person(s) and donated to the Becker County Historical Society (HR-1). In... Indian mortuary practices over a broad temporal span and cannot be associated with any single... Historical Society (HR-2). In 1997, the human remains were transferred to the Minnesota Office of the State...

  15. High School Students' Use of Paper-Based and Internet-Based Information Sources in the Engineering Design Process

    Science.gov (United States)

    Pieper, Jon; Mentzer, Nathan

    2013-01-01

    Mentzer and Becker (2011) and Becker and Mentzer (2012) demonstrated that high school students engaged in engineering design problems spent more time accessing information and spent more time designing when provided with Internet access. They studied high school students engaged in an engineering design challenge. The two studies attempted to…

  16. Changes in Smoking Behavior Following a Strict No-Smoking Policy in U.S. Navy Recruit Training

    Science.gov (United States)

    1993-08-01

    Pitchford, 1988; Biener, Abrams, Follick, & Dean, 1989; Becker, Conner, Waranch, Stillnan, Pennington, Lees, & Oski , 1989; Borland, Chapman, Owen...1-39). Heidelberg: Springer. Becker, D.M., Conner, H.F., Waranch, H.R., StiUMan, F., Pennington, L., Lees, P.S., & Oski , F. (1989). The Impact of a

  17. "Hvordan er det gået til?"

    DEFF Research Database (Denmark)

    Jourdan, Michael

    2004-01-01

    Forsimplede farmakologiske teorier kommer til kort. At lære at blive hasbruger er en kompliceret, social proces. Howard S. Beckers klassiske artikel fra 1953 om emnet introduceres.......Forsimplede farmakologiske teorier kommer til kort. At lære at blive hasbruger er en kompliceret, social proces. Howard S. Beckers klassiske artikel fra 1953 om emnet introduceres....

  18. Using Numbers in Qualitative Research

    Science.gov (United States)

    Maxwell, Joseph A.

    2010-01-01

    The use of numerical/quantitative data in qualitative research studies and reports has been controversial. Prominent qualitative researchers such as Howard Becker and Martyn Hammersley have supported the inclusion of what Becker called "quasi-statistics": simple counts of things to make statements such as "some," "usually," and "most" more…

  19. Sequential causal learning in humans and rats

    NARCIS (Netherlands)

    Lu, H.; Rojas, R.R.; Beckers, T.; Yuille, A.; Love, B.C.; McRae, K.; Sloutsky, V.M.

    2008-01-01

    Recent experiments (Beckers, De Houwer, Pineño, & Miller, 2005;Beckers, Miller, De Houwer, & Urushihara, 2006) have shown that pretraining with unrelated cues can dramatically influence the performance of humans in a causal learning paradigm and rats in a standard Pavlovian conditioning paradigm. Su

  20. A Survey of Principles Instructors: Why Lecture Prevails

    Science.gov (United States)

    Goffe, William L.; Kauper, David

    2014-01-01

    For many years, surveys have shown that lecture is the dominant method for teaching principles of economics (Watts and Schaur 2011; Watts and Becker 2008; Becker and Watts 1996, 2001a, b). The authors confirm this and augment it by asking why principles instructors teach the way they do. The respondents, 340 principles instructors at the 2012…

  1. Disease: H00986 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available 733-49 (1987) PMID:22167768 (description, gene) Vogt J, Morgan NV, Rehal P, Faivre L, Brueton LA, Becker K, ...(2012) PMID:18252226 (description, gene) Michalk A, Stricker S, Becker J, Rupps R, Pantzar T, Miertus J, Bot

  2. Using Fluorescence in situ Hybridization to Identify Duchenne/Becker Muscular Dystrophy (DMD/BMD) Deletion Carriers%荧光原位杂交技术在检测假性肥大型肌营养不良症缺失型携带者中的应用

    Institute of Scientific and Technical Information of China (English)

    肖艳萍; 蒋秀蓉; 王仁礼

    2002-01-01

    目的:应用荧光原位杂交(FISH)筛查技术检测假性肥大型肌营养不良症(DMD/BMD)缺失型携带者.方法:以外显子特异Cosmid DNA为探针(含18个外显子),采用中期和间期单色FISH技术,对9例正常男、女性及来自不同缺失型DMD/BMD家系的5例女性外周血标本、来自健康孕妇的2例羊水和2例绒毛标本进行分析.结果:72~100%外周血淋巴细胞中期相或间期核、60~70%羊水细胞间期核、95~99%绒毛细胞间期核显示预期信号.FISH检出1名、排除2名缺失型携带者.结论:充分利用FISH技术优点,结合现有其它技术,可有效筛查DMD/BMD缺失型携带者,并为女性胎儿DMD/BMD缺失型携带者产前诊断奠定基础.

  3. Structure May Be Key to Incorporating Library School Interns in Academic Library Environments. A Review of: Sargent, A. R., Becker, B. W., & Klingberg, S. (2011. Incorporating library school interns on academic library subject teams. The Journal of Academic Librarianship, 37(1, 28-33. doi: 10.1016/j.acalib.2010.10.004

    Directory of Open Access Journals (Sweden)

    Heather R. Williams

    2011-09-01

    Full Text Available Objective – To evaluate the effectiveness of the San Jose State University Library internship program.Design – Focus group; single point in time; qualitative design.Setting – Large academic library in the United States of America.Subjects – Nine former interns of the San Jose State University (SJSU Library.Methods - Nine former interns of the SJSU Library internship program participated in a single 90-minute session. No inducements for participation were offered. A moderator asked a series of 10 questions designed to gather feedback in three areas: 1 “the internship as part of the Masters program,” 2 “the internship’s role in the realization of personal objectives and professional development,” and 3 “the experience of working in team based activities.” A digital voice recorder captured the participants’ responses, allowing for detailed analysis of the responses after the session.Main Results – The interns deemed their overall experience successful, as all indicated they achieved their professional development objectives for the internship. However, the interns also indicated their experience could have been improved by the appointment of a single dedicated coordinator for recruitment and oversight, as well as more feedback on the quality of their work, especially for course-related instruction.Conclusion – The SJSU Library determined that the internship program was advantageous to both the Library and the interns. All of the interns who participated in the focus group achieved their profession development objectives for the internship. Additionally, the Library received valuable feedback for improving the program. Suggestions included appointing a dedicated internship coordinator, allowing interns more of an opportunity to choose their projects, and ensuring that interns are offered frequent feedback about the quality of their work.

  4. New Thoughts on the Study of Tourism Expenditure Theory--An Analysis on Becker's New Theory of Consumers' Behaviour%旅游消费理论研究的新思考--评析贝克尔的新消费者行为理论

    Institute of Scientific and Technical Information of China (English)

    钱林晓

    2000-01-01

    在评析贝克尔的新消费者行为理论的基础上,指出虽然贝克尔的理论还存在一定的缺陷,但是该理论对消费研究提出的新思路有极高的参考价值,是旅游消费理论研究应该借鉴与吸纳的.

  5. A comprehensive database of Duchenne and Becker muscular dystrophy patients in Children's Hospital of Fudan University%复旦大学附属儿科医院Duchenne型和Becker型肌营养不良症数据库的建立

    Institute of Scientific and Technical Information of China (English)

    李西华; 赵蕾; 胡超平; 施亿赟; 周水珍; 王艺

    2015-01-01

    研究背景 我国是Duchenne型和Becker型肌营养不良症患病率最高的国家之一,虽然国际间建立的Duchenne型和Becker型肌营养不良症数据库为临床药物研发和临床试验奠定了基础,但在我国尚未全面开展.本研究参照日本Remudy登记表的设计,建立复旦大学附属儿科医院Duchenne型和Becker型肌营养不良症数据库(CHFU),为国际间合作提供了条件.方法 纳入2011年8月-2013年12月在复旦大学附属儿科医院就诊并经基因检测或肌肉活检明确诊断的Duchenne型和Becker型肌营养不良症患儿229例,登记患儿性别、年龄、明确诊断年龄、地域分布、DMD基因突变类型、家族史、行走能力、心肺功能、激素治疗和康复干预等信息.结果 229例患儿中Duchenne型肌营养不良症194例、Becker型肌营养不良症35例;均为男性;>3~4岁(16.59%,38/229)和>7~8岁(14.85%,34/229)是明确诊断的高峰年龄.基因突变类型以缺失突变为主,在Duchenne型和Becker型肌营养不良症中分别占65.46%(127/194)和74.29%(26/35);有家族史者占23.14%(53/229);17.53%(34/194)的Duchenne型肌营养不良症患儿丧失行走能力,Becker型肌营养不良症患儿均保留行走能力;接受心脏功能和呼吸功能监测的患儿分别占46.29%(106/229)和17.90%(41/229);接受泼尼松0.75 mg/(kg·d)治疗的Duchenne型肌营养不良症患儿占26.29%(51/194).结论 CHFU数据库详细描述了Duchenne型和Becker型肌营养不良症患儿基因型、临床表现、诊断与治疗、康复情况,不仅为患者管理提供全面详细的信息,而且有助于促进我国临床试验的发展、推动前瞻性治疗性研究,以及更好地管理Duchenne型和Becker型肌营养不良症患儿及其家庭.

  6. 定量等长肌力测定在Duchenne型和Becker型肌营养不良症患儿下肢肌力测定中的信度评价%Reliability of hand-held dynamometry for measurement of lower limb muscle strength in children with Duchenne and Becker muscular dystrophy

    Institute of Scientific and Technical Information of China (English)

    史惟; 李惠; 苏怡; 陆恺; 侯芳华; 杨红

    2015-01-01

    目的 评价定量等长肌力测定在Duchenne型和Becker型肌营养不良症患儿下肢肌力测定中的信度.方法 共21例Duchenne型(19例)和Becker型(2例)肌营养不良症患儿,采用手持式肌力测定仪检测患儿髋、膝、踝等部位肌群肌力,比较不同检测者间和同一检测者两次测试结果间的相关性,以评价手持式肌力测定仪的不同检测者间信度和重测信度.结果 手持式肌力测定仪测定Duchenne型和Becker型肌营养不良症患儿下肢肌群具有良好的不同检测者间信度(ICC=0.762 ~ 0.978)和重测信度(ICC=0.690 ~ 0.938),但二者对远端肌群(足跖屈和足背屈)信度均相对较低.结论 手持式肌力测定仪在Duchenne型和Becker型肌营养不良症患儿下肢肌力测定中具有较好的不同检测者间信度和重测信度,可以用于监测肌力变化和评价临床疗效.

  7. A new species of the genus Polyodaspis Duda, 1933 (Diptera: Acalyptratae: Chloropidae) from Central Asia with a key to the Palaearctic species.

    Science.gov (United States)

    Nartshuk, E P

    2016-01-28

    A new species, Polyodaspis ferulae n. sp., from Central Asia (Uzbekistan) is described. The species was collected on inflorescences of Ferula foetida (Apiaceae). Siphonella levicola Becker in Becker and Stein, 1913 described from Iran is synonymized with Polyodaspis sulcicollis (Meigen, 1838) based on investigation of the holotype. A key to 9 species of the genus Polyodaspis Duda, 1933 is proposed with illustrations of male genitalia. Polyodaspis and Anacamptoneurum Becker, 1903 are treated as separate genera. New localities of P. sulcicollis (Meigen, 1838) from Kazakhstan and Central Asia are given.

  8. Disease: H00562 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00562 Dystrophinopathies, including: Duchenne muscular dystrophy (DMD); Becker muscular... dystrophy (BMD); X-linked dilated cardiomyopathy (XLCM) Duchenne muscular dystrophy (DMD) is character...ized by weakness of leg, pelvic and shoulder girdle muscles starting in early childhood. Becker muscular dys...s HJ, Faber CG Hereditary muscular dystrophies and the heart. Neuromuscul Disord 20:479-92 (2010) PMID:11879...K, Speer A, Braun T, Hubner C Heterozygous myogenic factor 6 mutation associated with myopathy and severe course of Becker muscular dystrophy. Neuromuscul Disord 10:572-7 (2000) ...

  9. Disastrous Dialogue

    DEFF Research Database (Denmark)

    Just, Sine Nørholm; Berg, Kristine Marie

    2016-01-01

    In 2010 the Danish artist Søren Thilo Funder was in Cairo to produce the art film Disastrous Dialogue. As Funder set to work he had a foreboding about how politically charged the piece might be. When he cut the film, however, events had exceeded his most fateful premonitions, reshaping the interp...... through the lens of the concept of plasticity. This leads us to propose a plastic understanding of agency as both formed by and formative of meaningful relationships—and able to creatively destruct and, thereby, transform configurations of meaning....... the interpretative context completely. The changes in Egyptian society, thus, altered the possible meaning–agency relations of the finished work. Through a close reading and a conceptually guided criticism of the text–context relationships of Disastrous Dialogue we explore interrelations of meaning and agency...

  10. A METHODOLOGY FOR THE EVALUATION OF PROCESS SUSTAINABILITY

    Science.gov (United States)

    The twelve principles of green chemistry (Anastas and Warner, 1998) provide a foundation and pathway which allows researchers to incorporate greenness into existing reactions or when developing new technologies. Research from our laboratory (Gonzalez and Becker, 2002) has adopted...

  11. Browse Title Index

    African Journals Online (AJOL)

    Items 1 - 50 of 317 ... Working with Groups by Lilly Becker (ed.) Abstract ... Ciara Wynne, Caoimhe Doyle, Rachel Kenny, Eileen Brosnan, John Sharry ... Karmel W. Choi, Melissa H. Watt, Donald Skinner, Seth C. Kalichman, Kathleen J. Sikkema.

  12. Evaluation of Limb-Girdle Muscular Dystrophy

    Science.gov (United States)

    2014-03-06

    Becker Muscular Dystrophy; Limb-Girdle Muscular Dystrophy, Type 2A (Calpain-3 Deficiency); Limb-Girdle Muscular Dystrophy, Type 2B (Miyoshi Myopathy, Dysferlin Deficiency); Limb-Girdle Muscular Dystrophy, Type 2I (FKRP-deficiency)

  13. Discrimination and Skill Differences in an Equilibrium Search Model

    NARCIS (Netherlands)

    Bowlus, Audra J.; Eckstein, Zvi

    1998-01-01

    In this paper we analyze an equilibrium search model with threesources for wage andunemployment differentials among workers with the same (observed) human capital but different appearance (race): unobserved productivity (skill), search intensities and discrimination (Becker 1957)due to an

  14. Cannabis careers revisited

    DEFF Research Database (Denmark)

    Järvinen, Margaretha; Ravn, Signe

    2014-01-01

    A considerable part of today's sociological research on recreational drug use is (explicitly or implicitly) inspired by Howard Becker's classical model of deviant careers. The aim of the present paper is to directly apply Becker's theory to empirical data on present-day cannabis use and to suggest...... in treatment for cannabis problems in Copenhagen, Denmark. We suggest a revision of Becker's career model in relation to four aspects: initiation of cannabis use, differentiation between socially integrated and individualised, disintegrated use, social control from non-users, and the users' moral stance...... on cannabis. A central point of the paper is that social interaction may both motivate cannabis use, as Becker proposed, and serve as a protective factor against extensive, problematic use....

  15. Nasal polyps

    Science.gov (United States)

    ... BS, Burks AW, et al, eds. Middleton's Allergy: Principles and Practice . 8th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 43. Becker SS. Surgical management of polyps in the treatment of nasal airway ...

  16. LANTHANIDE ENHANCE LUMINESCENCE (LEL) WITH ONE AND TWO PHOTON EXCITATION OF QUANTUM DYES LANTHANIDE (III) - MACROCYCLES

    Science.gov (United States)

    Title: Lanthanide Enhance Luminescence (LEL) with one and two photon excitation of Quantum Dyes? Lanthanide(III)-Macrocycles Principal Author:Robert C. Leif, Newport InstrumentsSecondary Authors:Margie C. Becker, Phoenix Flow Systems Al Bromm, Virginia Commonw...

  17. Discrimination and Skill Differences in an Equilibrium Search Model

    NARCIS (Netherlands)

    Bowlus, Audra J.; Eckstein, Zvi

    1998-01-01

    In this paper we analyze an equilibrium search model with threesources for wage andunemployment differentials among workers with the same (observed) human capital but different appearance (race): unobserved productivity (skill), search intensities and discrimination (Becker 1957)due to an appearanc

  18. Pop / Anneli Remme

    Index Scriptorium Estoniae

    Remme, Anneli, 1968-

    2004-01-01

    Heliplaatidest: Hortus Musicus "Eesti heliloojad III", Brad Mehldau Trio "Anything Goes", Vitamins For You "I'm sorry for ever and for always", The Coral "Nightfreak and the Sons of Becker", Lionel Richie "Just For You", David Byrne "Grown Backwards"

  19. Plaadid / Valner Valme

    Index Scriptorium Estoniae

    Valme, Valner, 1970-

    2004-01-01

    Uutest plaatidest The Von Bondies "Pawn Shoppe Heart", Papa M "Hole of Burning Alms", Georg Levin "Can't Hold Back", The Coral "Nightfreak And The Sons Of Becker", "In America", Lamb "Between Darkness and Wonder"

  20. Short communication

    African Journals Online (AJOL)

    abp

    2013-09-30

    Sep 30, 2013 ... We also found a decrease in patients with missing WHO clinical stage ... These findings indicate that services offered to HIV infected persons pre-ART has ... Becker J, Tsague L, Sahabo R, Twyman P. Provider Initiated.

  1. Pop / Anneli Remme

    Index Scriptorium Estoniae

    Remme, Anneli, 1968-

    2004-01-01

    Heliplaatidest: Hortus Musicus "Eesti heliloojad III", Brad Mehldau Trio "Anything Goes", Vitamins For You "I'm sorry for ever and for always", The Coral "Nightfreak and the Sons of Becker", Lionel Richie "Just For You", David Byrne "Grown Backwards"

  2. Plaadid / Valner Valme

    Index Scriptorium Estoniae

    Valme, Valner, 1970-

    2004-01-01

    Uutest plaatidest The Von Bondies "Pawn Shoppe Heart", Papa M "Hole of Burning Alms", Georg Levin "Can't Hold Back", The Coral "Nightfreak And The Sons Of Becker", "In America", Lamb "Between Darkness and Wonder"

  3. At R209

    CERN Multimedia

    1977-01-01

    Side view of R209, showing a large muon chamber in front of the Iron toroid magnet. Gunter Feilhauer is looking down from top of the magnet. Ulrich Becker (left), Lars Leistam (right) stand on the floor.

  4. Disease: H00543 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available Bergmann C, Fliegauf M, Bruchle NO, Frank V, Olbrich H, Kirschner J, Schermer B, Schmedding I, Kispert A, Kranzlin B, Nurnberg G, Bec...ker C, Grimm T, Girschick G, Lynch SA, Kelehan P, Sender

  5. Margaret Huggins and Tulse Hill Observatory

    Science.gov (United States)

    Becker, Barbara J.

    2016-04-01

    Photography, instrument design, methodology, interpretation - all skills brought to William Huggins' observatory by his persistent and careful wife Margaret. Together they developed spectroscopy into a powerful research tool. Barbara Becker tells the story.

  6. It Bears Repeating: Understanding the Work of the Teacher: Some Light from the Past.

    Science.gov (United States)

    Fortgang, Stephen J.

    1980-01-01

    Reviews the work of Howard S. Becker who, in the 1950s, analyzed teaching as a service occupation and drew parallels with other such occupations in terms of worker-client relationships and authority structures. (SJL)

  7. Disease: H00705 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available disease, Becker disease is more common, more insidious, and has initial symptoms that occur later in child...hood. Two additional forms of myotonia congenita have been described: myotonia levi

  8. LANTHANIDE ENHANCE LUMINESCENCE (LEL) WITH ONE AND TWO PHOTON EXCITATION OF QUANTUM DYES LANTHANIDE (III) - MACROCYCLES

    Science.gov (United States)

    Title: Lanthanide Enhance Luminescence (LEL) with one and two photon excitation of Quantum Dyes? Lanthanide(III)-Macrocycles Principal Author:Robert C. Leif, Newport InstrumentsSecondary Authors:Margie C. Becker, Phoenix Flow Systems Al Bromm, Virginia Commonw...

  9. Genetics Home Reference: Duane-radial ray syndrome

    Science.gov (United States)

    ... 83. Citation on PubMed Kohlhase J, Heinrich M, Schubert L, Liebers M, Kispert A, Laccone F, Turnpenny ... 23):2979-87. Citation on PubMed Kohlhase J, Schubert L, Liebers M, Rauch A, Becker K, Mohammed ...

  10. Photo- and laser therapy in pigment disorders

    NARCIS (Netherlands)

    Wind, B.S.

    2011-01-01

    Bas Wind behandelt de effecten van laserbehandelingen bij melasma (zwangerschapsmasker, een aandoening gekenmerkt door donkere vlekken in het gezicht) en de naevus van Becker (pigmentvlekken op de schouders). Ook onderzocht Wind vitiligo, een huidziekte waarbij pigmentcellen verloren gaan waardoor

  11. Saksa kunstiklassika Worpswede külakesest / Mai Levin

    Index Scriptorium Estoniae

    Levin, Mai, 1942-

    2000-01-01

    Tallinna Kunstihoone galeriis saksa maalikunsti näitus "Paula Modersohn-Becker ja teised Worpswede kunstnikud 1895-1906". Pikemalt modernistliku primitivismi esindaja Paula Modersohn-Beckeri loomingust

  12. GREEN CATALYZED OXIDATION OF HYDROCARBONS IN ALTERNATIVE SOLVENT SYSTEMS GENERATED BY PARIS II

    Science.gov (United States)

    Green Catalyzed Oxidation of Hydrocarbons in Alternative Solvent Systems Generated by PARIS IIMichael A. Gonzalez*, Thomas M. Becker, and Paul F. Harten; Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26...

  13. GREEN CATALYZED OXIDATION OF HYDROCARBONS IN ALTERNATIVE SOLVENT SYSTEMS GENERATED BY PARIS II DECHEMA; GREEN SOLVENTS FOR CATALYSIS - ENVIRONMENTALLY BENIGN REACTION MEDIA

    Science.gov (United States)

    Green catalyzed oxidation of hydrocarbons in alternative solvent systems generated by PARIS IIThomas M. Becker, Michael A. Gonzalez, Paul F. Harten; Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26 West Mar...

  14. Red River of the North Reconnaissance Report: Ottertail River Subbasin.

    Science.gov (United States)

    1980-12-01

    inmigration rate in the two counties that constitute the major portion of the subbasin (Becker and Ottertail). Between 1970 and 1977, Becker had an... inmigration rate of 8.9 percent, and Ottertail’s inmigration rate was 6.4 percent. Preliminary figures for 1978 indicate that these counties are still...experiencing inmigration . Telephone contacts with public officials indicate that the inmigration pattern results from: (1) the decline in the farm

  15. Competition among pressure groups for political influence over the determination of accounting standards

    OpenAIRE

    Klumpes, P J M

    1998-01-01

    This paper integrates prior studies of accounting policy choice and lobbying activities by testing the empirical implications of Becker''s (1983) theory of competition among pressure groups for political influence over the determination of accounting standards. The theory is applied to explain the nature and outcome of conflict among pressure groups representing financial intermediaries (suppliers) and pension fund members (users) over the development of conflicting Australian pension account...

  16. Stemcell Information: SKIP001125 [SKIP Stemcell Database[Archive

    Lifescience Database Archive (English)

    Full Text Available Becker type 300376 ... -- -- ... No No Disease specific iPS cell line derived from a patient : Muscular ...dystrophy, Becker type. HPS0233 was derived from the same patient.iPS cells were created using the Sendai vi...rus vector. Order Form (C-0042, C-0057, C-0007 or C-0007p). 疾患特異的iPS細胞株。ベッカー型筋ジストロフィー患者

  17. Escape from Evil? Notes on Capacity, Tragedy, Coding and Non-Destructive Immortality Projects

    Directory of Open Access Journals (Sweden)

    Ronnie Lippens

    2015-09-01

    Full Text Available Like James Hardie-Bick’s (2015 contribution this paper elaborates on the importance for socio-legal studies of Ernest Becker’s (1924-1974 work on the destructive consequences of human … all too human forms of death denial. With Becker, it makes an attempt to think through the question, ‘Is it possible, in the light of the human condition, to escape evil?’ Terminally ill by the early 1970s, Becker himself was left no time to complete his thoughts on this matter. Focussing on the point where Becker left the discussion when he died in 1974 we make an attempt here, still with an eye on the aforementioned question, to explore the work of a number of philosophers whose work we know Becker was familiar with: Benedictus de Spinoza, Henri Bergson, and Albert Camus. In their work, it is argued here, one could find elements towards thinking through the question of evil, and the (impossibility of escaping it. Al igual que el artículo de James Hardie-Bick (2015, este artículo profundiza en la importancia que tiene para los estudios sociojurídicos el trabajo de Ernest Becker (1924-1974 sobre las consecuencias destructivas del ser humano… y la tan humana negación de la muerte. Con Becker, se realiza un intento de reflexionar sobre esta pregunta: “Es posible, a la luz de la condición humana, escapar del mal? Enfermo terminal a comienzos de los años 70, el propio Becker no tuvo tiempo de completar sus pensamientos sobre este asunto. Centrándonos en el punto en el que Becker dejó el debate, cuando murió en 1974, y sin perder de vista esta pregunta, se intenta analizar el trabajo de una serie de filósofos, con el que estaba familiarizado Becker: Benedictus de Spinoza, Henri Bergson y Albert Camus. Se defiende que en su trabajo se pueden encontrar elementos que hacen pensar sobre la cuestión del mal, y la (imposibilidad de escapar de él.DOWNLOAD THIS PAPER FROM SSRN: http://ssrn.com/abstract=2619429

  18. 6th International Symposium on Molecular Allergology (ISMA

    Directory of Open Access Journals (Sweden)

    Christiane Hilger

    2016-10-01

    -patient quantification of allergen-specific IgE Petra Zavadakova, Aurélie Buchwalder, Fabien Rebeaud, Iwan Märki Symposium 4: Relevance of molecular diagnostics for intervention and treatment O7 Longitudinal analysis of Bet v 1-specific epitope repertoires during birch pollen immunotherapy Barbara Gepp, Nina Lengger, Christian Möbs, Wolfgang Pfützner, Christian Radauer, Barbara Bohle O8 A natural CCD-free tool: is polistes sp. venom suitable for polybia paulista diagnosis and therapy? Karine Marafigo De Amicis, Alexandra Sayuri Watanabe, Clovis Eduardo Galvao, Daniele Danella Figo, Jose Roberto Aparecido Santos-Pinto, Mario Sergio Palma, Fabio Fernandes Morato Castro, Jorge Kalil, Fatima Ferreira, Gabriele Gadermaier, Keity Souza Santos Symposium 5: The advent of molecular allergology in epidemiology O9 Peanut oleosins: from identification to diagnostic testing Christian Schwager, Skadi Kull, Frauke Schocker, Jochen Behrends, Wolf-Meinhard Becker, Uta Jappe O10 Endotypes of oral allergy syndrome in childhood: a molecular diagnostic approach Carla Mastrorilli, Salvatore Tripodi, Carlo Caffarelli, Riccardo Asero, Arianna Dondi, Giampaolo Ricci, Carlotta Povesi Dascola, Elisabetta Calamelli, Andrea Di Rienzo Businco, Annamaria Bianchi, Tullio Frediani, Carmen Verga, Iride Dello Iacono, Diego Peroni, Giuseppe Pingitore, Roberto Bernardini, Paolo Maria Matricardi Symposium 6: Molecular AIT: which approaches will make it to market? O11 Mbc4: an innovative molecule to tackle birch pollen and concomitant food allergies Heidi Hofer, Claudia Asam, Michael Hauser, Peter Briza, Martin Himly, Christof Ebner, Fatima Ferreira O12 Challenges and solutions associated with the production of recombinant Bet v 1 allergen as a therapeutic protein Emmanuel Nony, Maxime Le Mignon, Pierrick Lemoine, Karine Jain, Kathy Abiteboul, Monica Arvidsson, Sabina Rak, Philippe Moingeon Clinical Cases: Breakthroughs and headaches from CRD: interactive session CC1 Anaphylaxis caused by lipid transfer proteins: a

  19. Michel Foucault’s ‘Apology’ for Neoliberalism

    DEFF Research Database (Denmark)

    Dean, Mitchell

    2014-01-01

    Lecture delivered at the British Library on the 30th anniversary of the death of Michel Foucault, June 25, 2014. This lecture evaluates the claim made by one of his closest followers, François Ewald, that Foucault offered an apology for neoliberalism, particularly of the American school represented...... by Gary Becker. It draws on exchanges between Ewald and Becker in 2012 and 2013 at the University of Chicago shortly before the latter’s death. It places Foucault in relation to the then emergent Second Left in France, the critique of the welfare state, and, more broadly, the late-twentieth-century social...

  20. Electromagnetic fields and interactions

    CERN Document Server

    Becker, Richard

    1982-01-01

    For more than a century, ""Becker"" and its forerunner, ""Abraham-Becker,"" have served as the bible of electromagnetic theory for countless students. This definitive translation of the physics classic features both volumes of the original text.Volume I, on electromagnetic theory, includes an introduction to vector and tensor calculus, the electrostatic field, electric current and the field, and the theory of relativity. The second volume comprises a self-contained introduction to quantum theory that covers the classical principles of electron theory and quantum mechanics, problems involving

  1. El vacío institucional en el modelo de elección racional aplicado a la fecundidad

    Directory of Open Access Journals (Sweden)

    Rafael Barrera Gutiérrez

    2011-12-01

    Full Text Available Este artículo trata dos ideas básicas. La primera es que el modelo de racionalidad aplicado a la fecundidad y la interacción entre cantidad y calidad no fueron formulados inicialmente por Becker, y aunque dicha relación hoy parece clara y evidente no es obvia. La segunda es que desde el comienzo el análisis de la fecundidad ha considerado el contexto institucional. Las críticas al modelo de Becker y los desarrollos posteriores refuerzan la importancia de las instituciones para explicar las variaciones de la fecundidad.

  2. Challenge energy policy turnaround; Herausforderung Energiewende

    Energy Technology Data Exchange (ETDEWEB)

    Becker, Michael; Brandt-Schwabedissen, Annette; Graaff, Rudolf; Queitsch, Peter; Thomas, Roland [Staedte- und Gemeindebund Nordrhein-Westfalen e.V., Duesseldorf (Germany); Becker, Sven [Trianel GmbH, Aachen (Germany); Portz, Norbert; Schmitz, Johannes [Deutscher Staedte- und Gemeindebund, Berlin (Germany)

    2011-07-01

    The documentation under consideration makes suggestions to cities and communities in light of the energy policy turnaround. The documentation contains the following contributions: (1) Power generation by means of renewable energy resources (Johannes Schmitz); (2) The energy policy turnaround needs acceptance - communication as the key to success (Sven Becker); (3) Climate-conscious communal construction planning (Michael Becker); (4) Establishment of climate concepts (Peter Queitsch); (5) Energetic measures at buildings (Annette Brandt-Schwabedissen); (6) Energy political turnaround and awarding (Norbert Portz); (7) Electromobility (Roland Thomas); (8) Position paper of DStB for the energy policy turnaround.

  3. Necessary Illusions: Life, Death and the Construction of Meaning

    Directory of Open Access Journals (Sweden)

    James Hardie-Bick

    2015-09-01

    Full Text Available This paper introduces the work of the late cultural anthropologist Ernest Becker. Becker argued that the cause of human suffering is directly related to the strategies people use to cope with their mortality awareness. By concentrating on his last two books, The Denial of Death (1973 and Escape from Evil (1975, the aim of this paper is to provide an overview of Becker’s mature theory to show how his work on destructiveness is necessary for developing a socially engaged social theory. Whilst his theory on the human condition explores some of the darkest aspects of human existence, by examining why people are capable of extreme forms of cruelty Becker directly encouraged an honest dialogue concerning our existential predicament. This paper highlights the necessity of Becker’s theory of evil for opening up new possibilities for living in a more humane world. Este artículo presenta el trabajo del antropólogo cultural Ernest Becker. Becker argumentó que la causa del sufrimiento humano está directamente relacionada con las estrategias que usan las personas para hacer frente a su conciencia de mortalidad. Analizando sus dos últimos libros, La negación de la muerte (1973 y La lucha contra el mal (1975, el objetivo de este artículo es ofrecer una visión general de la teoría madura de Becker para demostrar cómo su estudio sobre la destructividad es necesario para desarrollar una teoría social socialmente comprometida. A pesar de que su teoría sobre la condición humana explora algunos de los aspectos más oscuros de la existencia humana, Becker directamente fomenta un diálogo honesto sobre nuestro predicamento existencial, examinando por qué las personas son capaces de formas extremas de crueldad. Este artículo destaca la necesidad de la teoría de Becker del mal, para facilitar nuevas posibilidades de vivir en un mundo más humano. DOWNLOAD THIS PAPER FROM SSRN: http://ssrn.com/abstract=2619408

  4. Cool Minds in Heated Debates? Migration-related Attitudes in Germany Before and After a Natural Intervention

    Directory of Open Access Journals (Sweden)

    Claudia Diehl

    2012-05-01

    Full Text Available Data from the Transatlantic Trends: Immigration survey was used to investigate whether the debate surrounding Thilo Sarrazin’s immigration-skeptical Deutschland schafft sich ab (Germany abolishes itself had any impact on migration-related attitudes in Germany. The book was published in August 2010 and fieldwork took place during the evolving debate, providing a unique opportunity to study the impact of a major media event on public attitudes. Descriptive findings on the aggregate level show no substantial change in migration-related attitudes in the months after publication. More detailed findings reveal a significant increase in skepticism only for respondents with low levels of education, whose assessment of Muslim migrants’ integration became more negative during the debate. There are two possible reasons for the lack of more substantial attitudinal change. Firstly, the debate was highly polarized and lacked the consonant national media coverage that is an important precondition for media effects on public opinion. Secondly, there were no additional “external shocks” prior to the book’s release, such as a high levels of immigration, that could have made the public more susceptible to criticism of the impact of migration.

  5. Surgical and nonsurgical treatment of total rupture of the pectoralis major muscle in athletes: update and critical appraisal

    Directory of Open Access Journals (Sweden)

    Jörn Kircher

    2010-10-01

    Full Text Available Jörn Kircher, Christoph Ziskoven, Thilo Patzer, Daniela Zaps, Bernd Bittersohl, Rüdiger KrauspeUniversity Hospital, Orthopaedic Department, Heinrich-Heine University Düsseldorf, Düsseldorf, GermanyAbstract: The complete rupture of the pectoralis major tendon is an uncommon injury but has become increasingly common among athletes in recent years. This may be due to a higher number of individuals taking part in high-impact sports and weightlifting as well as the use of anabolic substances, which can make muscles and tendons vulnerable to injury. In recent literature, there are only few recommendations to rely on conservative treatment alone, but there are a number of reports and case series recommending early surgical intervention. Comparing the results of the two treatment regimens, there is clear evidence for a superior outcome after surgical repair with better cosmesis, better functional results, regaining of muscle power, and return to sports compared with the conservative treatment. In summary, anatomic surgical repair is the treatment of choice for complete acute ruptures of the pectoralis major tendon or muscle in athletes.Keywords: pectoralis major, rupture, athlete, conservative treatment, surgical treatment, steroid, tendon, sports injury

  6. Editorial: Letter from the Editors

    Directory of Open Access Journals (Sweden)

    Wilhelm Heitmeyer

    2012-05-01

    Full Text Available This time our focus section features work from the first conference hosted by IJCV. In 2011 Donatella della Porta and Gary LaFree, both members of the journal’s advisory board, brought together in Bielefeld experts from around the world to thoroughly debate the question of radicalization and de-radicalization. In contrast to prior approaches that mostly focused on the context of terrorism, the aim of this focus section is to present work employing new theoretical and methodological approaches to generate findings that go well beyond terrorism and related phenomena and thus contribute to a more comprehensive understanding of the processes, dynamics, and mechanisms of radicalization and de-radicalization. Our heartfelt thanks go to the two guest editors – as well as the entire conference team – for realising this ambitious project and for putting together this collection of papers.The open section contains two articles of additional interest. Rafi Nets-Zehngut uses the example of the 1948 Palestinian exodus to describe the characteristics of internal and external collective memories in Israeli society. The section closes with a contribution by Claudia Diehl and Jan-Philipp Steinmann who explore the impact of the release and public discussion of Thilo Sarrazin’s book Deutschland schafft sich ab (Germany abolishes itself on public perception of immigrants in Germany.

  7. The Effects of Parenting Styles on Children's Self-Esteem: A Developmental Perspective.

    Science.gov (United States)

    Elings, Joette Renee

    The effects of parenting styles on children's self-esteem were examined by dividing the empirical literature into three age groups: the preschool child; the elementary child; and the adolescent. Major literature reviews by W. C. Becker, B. Martin, and E. E. Maccoby and J. A. Martin are discussed and inconsistencies among the reviews are examined.…

  8. Local anesthesia part 2: technical considerations.

    Science.gov (United States)

    Reed, Kenneth L; Malamed, Stanley F; Fonner, Andrea M

    2012-01-01

    An earlier paper by Becker and Reed provided an in-depth review of the pharmacology of local anesthetics. This continuing education article will discuss the importance to the safe and effective delivery of these drugs, including needle gauge, traditional and alternative injection techniques, and methods to make injections more comfortable to patients.

  9. Minority Language Researchers and Their Role in Policy Development

    Science.gov (United States)

    Gorter, Durk

    2012-01-01

    This paper deals with the role of researchers in the development of language policies for European minority languages. This question is placed in the context of a long-standing debate in sociology to which several authors have contributed; among them are Max Weber, Howard Becker and Alvin Gouldner. This article also briefly refers to the European…

  10. Derivation of Human Induced Pluripotent Stem (iPS) Cells to Heritable Cardiac Arrhythmias

    Science.gov (United States)

    2016-03-14

    Inherited Cardiac Arrythmias; Long QT Syndrome (LQTS); Brugada Syndrome (BrS); Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT); Early Repolarization Syndrome (ERS); Arrhythmogenic Cardiomyopathy (AC, ARVD/C); Hypertrophic Cardiomyopathy (HCM); Dilated Cardiomyopathy (DCM); Muscular Dystrophies (Duchenne, Becker, Myotonic Dystrophy); Normal Control Subjects

  11. HOXC9-Induced Differentiation in Neuroblastoma Development

    Science.gov (United States)

    2013-10-01

    Roesch A, Mueller AM, Stempfl T, Moehle C, Landthaler M, Vogt T: RBP2-H1/JARID1B is a transcriptional regulator with a tumor suppressive potential in...melanoma cells. Int J Cancer 2008, 122:1047-1057. 14. Roesch A, Becker B, Schneider-Brachert W, Hagen I, Landthaler M, Vogt T: Re-expression of the

  12. The Bicycle Helmet Attitudes Scale: Using the Health Belief Model to Predict Helmet Use among Undergraduates

    Science.gov (United States)

    Ross, Thomas P.; Ross, Lisa Thomson; Rahman, Annalise; Cataldo, Shayla

    2010-01-01

    Objective: This study examined bicycle helmet attitudes and practices of college undergraduates and developed the Bicycle Helmet Attitudes Scale, which was guided by the Health Belief Model (HBM; Rosenstock, 1974, in Becker MH, ed. "The Health Belief Model and Personal Health Behavior". Thorofare, NJ: Charles B. Slack; 1974:328-335) to predict…

  13. Gender Integration of a Traditionally Male Field: A Definition of the Occupation.

    Science.gov (United States)

    1982-12-01

    Images embodied in legendary figures. such as Florence Nightingale . Clarence Drrow, and Vince Lombardi, or media heroes, such as Marcus Welby, Lou Grant... Florence Nightingale : Functions of the Heroine Legend in an Occupational Sub-Culture," Human Orqanizatlon XXIII, No. 2 (1964), 123-130. Becker, Howard S

  14. Kunsten i den kulturpolitiske forskning

    DEFF Research Database (Denmark)

    Hansen, Louise Ejgod

    2011-01-01

    worlds (Amsterdam, 2009) må, efter at have gennemgået en kanonisk række af kulturteoretikere, konstatere, herunder Danto, Becker og Bourdieu, at det ikke med udgangspunkt i disse teorier er muligt at besvare spørgsmålet: Hvad gør kunsten? (van Maanen, 2009, s. 149), hvorefter han formulerer sit eget bud...

  15. Anti-Angiogenic Gene Therapy for Prostate Cancer

    Science.gov (United States)

    2004-04-01

    in immunocompetent animals (47). 16. Kisker, 0., Becker, C. M., Prox, D., Fannon, M., D’Amato. R., Flynn, E.. Fogler , Persistence and stable expression...Pluda, J., Fogler , W., Schiller, J. H., and Wilding, rAAV is the poor transduction efficiency in primary tumors as well as G. Phase I phannacokinetic

  16. Utilization and Perceived Impact of Smart Phone Apps Among Persons Pursuing Mental Health Services

    Science.gov (United States)

    2015-12-01

    impact of smart phone apps among persons pursuing mental health services Robin E. Becker, MA*, Daniel G. Cassidy, PhD, and William C. Isler, PhD...applications (apps) designed for use on phones and other ‘ smart ’ devices. The purpose of this study is to address 1) whether and by what means individuals

  17. Factors of the Earning Functions and Their Influence on the Intellectual Capital of an Organization

    Science.gov (United States)

    Ileanu, Bogdan Vasile; Tanasoiu, Ovidiu Emil

    2008-01-01

    This paper tries to consider some earning function as "start point" for the construction of indicators for intellectual capital measure. The analyze combines concepts from Mincer's and Becker theories and intellectual capital definitions currently in use. The correlation, significance and relation between elements are shown using three econometric…

  18. The Child Quantity-Quality Trade-Off During the Industrial Revolution in England

    DEFF Research Database (Denmark)

    Klemp, Marc; Weisdorf, Jacob L.

    We take Gary Becker's child quantity-quality trade-off hypothesis to the historical record, investigating the causal link from family size to the literacy status of offspring using data from Anglican parish registers, c. 1700-1830. Extraordinarily forhistorical data, the parish records enable us ...

  19. Poor Facial Affect Recognition among Boys with Duchenne Muscular Dystrophy

    Science.gov (United States)

    Hinton, V. J.; Fee, R. J.; De Vivo, D. C.; Goldstein, E.

    2007-01-01

    Children with Duchenne or Becker muscular dystrophy (MD) have delayed language and poor social skills and some meet criteria for Pervasive Developmental Disorder, yet they are identified by molecular, rather than behavioral, characteristics. To determine whether comprehension of facial affect is compromised in boys with MD, children were given a…

  20. High Altitude Atmospheric Investigations. Part II. Development of in-situ Experiments for Measurements in the D-Region of the Ionosphere and Their Results.

    Science.gov (United States)

    1981-06-03

    solar flux data related to Aeronomy (Solar Cycle 20), COSPAR Technique manual Series, Manual No. 7, (Febr. 1978). 22 Sanchez Muniosguren, L.V...Estructura de la alta Mesosfera, Publicacion A-68, Servicio Meteorologico Nacional, Madrid (1977). 23 Becker W.: Personal communication 24 Rose G., and H.U

  1. Berlinskaja stena i Rimskije kanikulõ / Jevgeni Levik

    Index Scriptorium Estoniae

    Levik, Jevgeni

    2003-01-01

    Mängufilmid "Hüvasti, Lenin!" ("Good Bye, Lenin!") : režissöör Wolfgang Becker : Saksamaa 2003 ja "Lizzie McGuire - koolitüdrukust popstaariks" ("The Lizzie McGuire Movie") : režissöör Jim Fall : Ameerika Ühendriigid 2003

  2. Methodology of Law and Economics

    NARCIS (Netherlands)

    A.M. Pacces (Alessio Maria); L.T. Visscher (Louis)

    2011-01-01

    textabstractIntroduction A chapter on the methodology of law and economics, i.e. the economic analysis of law, concerns the methodology of economics. The above quote (Becker 1976, 5) shows that economics should not be defined by its subject, but by its method (also Veljanovski 2007, 19). This method

  3. Tamborg, A. & Kiær, K. (2017): Professional practices: Planning lessons with learning platforms/ Paper, NERA-konference, marts 2017

    DEFF Research Database (Denmark)

    Kiær, Karina; Lindenskov Tamborg, Andreas

    2017-01-01

    Currently, an increased number of technologies are becoming available that support teachers in planning lessons (Johnson, Adams Becker, & Hall, 2015), and though there is evidence that technologies can influence teachers practice (Gros 2016) there is still little research on how the newly...

  4. Targeting Johnny: Analyzing the Legality, Legitimacy, and Policy Implications of an Enemy Attack on CONUS RPA Units

    Science.gov (United States)

    2013-02-14

    proffered by Saint Thomas Aquinas , it postulates it is permissible to perform an act likely to have negative consequences, if four conditions are...8 June 1977. Becker, Thomas and Lt Col Richard L. Dashiell. (Academic Directors, The JAG School). Interview, 9 November 2012. Bellamy, Alex J

  5. Meaning of Muscular Dystrophy

    Science.gov (United States)

    ... MD Living With MD en español Qué significa distrofia muscular Over Labor Day, just as you're going ... blood test if a kid has Becker or Duchenne MD. Or the doctor might take a small piece of the muscle and look at it under a microscope to ...

  6. Anti- and Pro-Immigrant Entrepreneurs Labeling Theory Revisited

    Science.gov (United States)

    Wilson, Tamar Diana

    2009-01-01

    Almost forty years ago, noted immigration scholar Jorge A. Bustamante published an article in the "American Journal of Sociology" applying Howard Becker's labeling theory to the phenomenon of deviantizing and stigmatizing the undocumented. While immigration laws and some of the players involved have changed since his article was published,…

  7. NACA Conference on Aircraft Loads, Flutter, and Structures: A compilation of Papers Presented.

    Science.gov (United States)

    1953-03-04

    BAKER, John E. NACA - Langley Laboratory BANK , Herman Jet Propulsion Laboratory - C.I.T. BECK, Edgar NACA Headquarters BECKER, John V. NACA - Langley... deMoraes , Carlos A.: Correlation of Supersonic Convective Heat-Transfer Coefficients From Measurements of the Skin Temperature of a Parabolic Body of

  8. Disease: H00196 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available ers of purine and pyrimidine metabolism. Mol Genet Metab 86:25-33 (2005) PMID:10066814 Ahmed M, Taylor W, Sm...normal human phosphoribosylpyrophosphate synthetase. J Biol Chem 274:7482-8 (1999) PMID:8702702 Becker MA, Taylor

  9. Global Education.

    Science.gov (United States)

    Longstreet, Wilma S., Ed.

    1988-01-01

    This issue contains an introduction ("The Promise and Perplexity of Globalism," by W. Longstreet) and seven articles dedicated to exploring the meaning of global education for today's schools. "Global Education: An Overview" (J. Becker) develops possible definitions, identifies objectives and skills, and addresses questions and…

  10. Achieving High Performance in Parallel Applications via Kernel-Application Interaction

    Science.gov (United States)

    1996-04-01

    empirical numbers. Tom LeBlanc’s past experience in real-time systems and insightful criticisms proved valuable, and Professor Abraham Seid- mann’s...Becker, P. Das, J. Karlsson, and C. Quiroz . Operating system support for animate vi- sion. Journal of Parallel and Distributed Computing, 15(2):103

  11. Testing the Human Capital Development Model: The Case of Apprenticeships in Turkey

    Science.gov (United States)

    Akpinar, Taner; Gün, Servet

    2016-01-01

    Human capital theory was developed to study how individual agents make rational choices or how they invest in human capital to maximize their welfare. One of the leading founders of this perspective, Becker, argues that schooling, on-the-job training, medical care, migration and searching for information about prices and incomes are different…

  12. Poor Facial Affect Recognition among Boys with Duchenne Muscular Dystrophy

    Science.gov (United States)

    Hinton, V. J.; Fee, R. J.; De Vivo, D. C.; Goldstein, E.

    2007-01-01

    Children with Duchenne or Becker muscular dystrophy (MD) have delayed language and poor social skills and some meet criteria for Pervasive Developmental Disorder, yet they are identified by molecular, rather than behavioral, characteristics. To determine whether comprehension of facial affect is compromised in boys with MD, children were given a…

  13. A Comparison of the Fit between the Organizational Climate of the Coast Guard, the Job/Career Expectations of Black College Graduates, and Their Perceptions about the Coast Guard.

    Science.gov (United States)

    1980-12-01

    Introduction To Motivation. Princeton: D. Van Nostrand Co., 1964. Becker, Howard S. and Carper , James W. "The Development of Identification with an...Guard Affirmative Action, Masters Thesis, Naval Postgraduate School, Monterey, Ca. 1979. 189 Turner, Barbara F. and McCaffery, JoAnne Hammar

  14. Body Mass Index, Nutrient Intakes, Health Behaviours and Nutrition Knowledge: A Quantile Regression Application in Taiwan

    Science.gov (United States)

    Chen, Shih-Neng; Tseng, Jauling

    2010-01-01

    Objective: To assess various marginal effects of nutrient intakes, health behaviours and nutrition knowledge on the entire distribution of body mass index (BMI) across individuals. Design: Quantitative and distributional study. Setting: Taiwan. Methods: This study applies Becker's (1965) model of health production to construct an individual's BMI…

  15. PARENTAL ORIGIN AND GERMLINE MOSAICISM OF DELETIONS AND DUPLICATIONS OF THE DYSTROPHIN GENE - A EUROPEAN STUDY

    NARCIS (Netherlands)

    VANESSEN, AJ; ABBS, S; BAIGET, M; BAKKER, E; BOILEAU, C; VANBROECKHOVEN, C; BUSHBY, K; CLARKE, A; CLAUSTRES, M; COVONE, AE; FERRARI, M; FERLINI, A; GALLUZZI, G; GRIMM, T; GRUBBEN, C; JEANPIERRE, M; KAARIAINEN, H; LIECHTIGALLATI, S; MELIS, MA; VANOMMEN, GJB; PONCIN, JE; SCHEFFER, H; SCHWARTZ, M; SPEER, A; STUHRMANN, M; VERELLENDUMOULIN, C; WILCOX, DE; TENKATE, LP

    1992-01-01

    Knowledge about the parental origin of new mutations and the occurrence of germline mosaicism is important for estimating recurrence risks in Duchenne (DMD) and Becker muscular dystrophy (BMD). However, there are problems in resolving these issues partly because not all mutations can as yet be direc

  16. Raamatuautomaadid Hamburgis / A. Bösch ; refereerinud Lea Laurberg-Heckelmann

    Index Scriptorium Estoniae

    Bösch, A.

    2012-01-01

    Refereeritud väljaandest: Bösch, A. Eine Schachtel Literatur ziehen: Bettina von Bülow betreibt in Hamburg Bücherautomaten - Das Angebot reicht vom Comics bis zum Stadtführer. - Lübecker Nachrichten, 30./31.10.2011, lk. 19

  17. A Stand-Alone Information System for Small Air Force Hospital Laboratories.

    Science.gov (United States)

    1986-01-01

    1 November 1984): 171-172. 3. Becker, Brenda L. "The Impact of DRGs on New Jersey Labs." Medical Laboratory Observer 16 (January, 1984): 30-41. 4...Inwood, Martin J.; Mellor, Leslie D.; Sergovich, F.; Spencer , Frank; and Thomson, Sam. Lynch’s Medical Laboratory Technology. 3rd ed. Philadelphia: W

  18. An Analysis of Causes of Contract Price Change for Competitive Procurements of Replenishment Spare Parts.

    Science.gov (United States)

    1984-09-01

    J. H. Purchasing and Supply Management (Fourth Edition). London: Chapman and Hall, 1978. 4. Becker, Richard A. and John M. Chambers. S A Language ... Acqusition Costs. IDA Paper P---35. Institute for Defense Analysis, Arlington VA, September 1979 (AD-A078 232). 13. Davis, FayeR., Procurement Analyst

  19. The Child Quantity-Quality Trade-Off During the Industrial Revolution in England

    DEFF Research Database (Denmark)

    Klemp, Marc; Weisdorf, Jacob L.

    We take Gary Becker's child quantity-quality trade-off hypothesis to the historical record, investigating the causal link from family size to the literacy status of offspring using data from Anglican parish registers, c. 1700-1830. Extraordinarily forhistorical data, the parish records enable us...

  20. Aligning Instruction and Assessment with Game and Simulation Design. CRESST Report 780

    Science.gov (United States)

    Wainess, Richard; Koenig, Alan; Kerr, Deirdre

    2011-01-01

    Effective design of training-related games (games for training and/or assessment) requires synergy between the mechanisms for delivering instructional content and the mechanisms for learning game play and game functionality (Becker, 2006). The learning domain must be embedded as a core game mechanic: that is, the game cannot be advanced or won…

  1. A Technology Integration Education (TIE) Model: Millennial Preservice Teachers' Motivations about Technological, Pedagogical, and Content Knowledge (TPACK) Competencies

    Science.gov (United States)

    Holland, Denise D.; Piper, Randy T.

    2014-01-01

    Nobel laureates Schultz (1971) and Becker (1964, 1993) reinvigorated the analysis of education investments. Human capital investments that improve cognitive skills for elementary and secondary students have important economic implications. An interdisciplinary, 12-construct technology integration education (TIE) model was developed. The sample…

  2. Reference: 13 [Arabidopsis Phenome Database[Archive

    Lifescience Database Archive (English)

    Full Text Available 13 http://metadb.riken.jp/db/SciNetS_ria224i/cria224u4ria224u12417702i Stacey Minviluz...idopsis. 11 2799-811 12417702 2002 Nov The Plant cell Becker Jeffrey|Koh Serry|Stacey Gary|Stacey Minviluz G

  3. Distribution of the mRNA for protein phosphatase T in rat brain

    NARCIS (Netherlands)

    Becker, W; Buttini, M; Limonta, S; Boddeke, H; Joost, HG

    1996-01-01

    We have recently cloned a novel protein serine/threonine phosphatase (PPT) from rat mRNA which is predominantly expressed in the brain (Becker et al., J. Biol. Chem., 269 (1994) 22586-22592). In the present study, the regional distribution of PPT mRNA in the brain of adult rats was characterized by

  4. Curricular Models for Culturally Disadvantaged

    Science.gov (United States)

    King, Clement T.

    1976-01-01

    Two psychological theories which seem to have had a great impact on compensatory education programs are Skinnerian reinforcement theory and the cognitive developmental theory derived in part from Jean Piaget. The Englemann-Becker program is a typical example of the Programmed Curricular kind of program; the Florida Project, an Open Framework; and…

  5. Becker′s Naevus Of The Lower Limb : An Unusual Site

    Directory of Open Access Journals (Sweden)

    Rathi Sanjay K

    2002-01-01

    Full Text Available Two males aged 18 and 20 years old presented with asymptomatic brown macules with hypertrichosis on the left thigh. Becker’s naevus less commonly involves the lower limb, a site which is reported in only 3% of the patients.

  6. Eruptive collagenoma

    Directory of Open Access Journals (Sweden)

    Mukhi Sanjay

    2002-01-01

    Full Text Available A case of eruptive collagenoma in a male is being reported. He presented with asymptomatic nodules and plaques over the trunk, upper extremity and face of 15 years duration. Family history was negative. Histopathology with H & E stain followed by Verhoeff van Gieson s staining revealed increased collagen in dermis confirming the diagnosis of eruptive collagenoma. Patient also had Beckers naevus.

  7. Blending Methodology of Linear Parameter Varying Control Synthesis of F-16 Aircraft System

    Science.gov (United States)

    2001-10-01

    During Powered Approach, in Proceedings of the American Control Conference , Albuquerque, NM, 1997, pp. 123{127. [6] G. Becker, Quadratic Stability and...Control of a Generic Missile, in Proceedings of the American Control Conference , Chicago, IL, 2000, pp. 3692{3696. Appendix A. Nonlinear Equations of F-16

  8. Old Testament exegesis: Reflections on methodology

    African Journals Online (AJOL)

    p1243322

    1914:v). 2 Becker (2005:3) states as follows: “Die alttestamentliche Texte haben ein Alter von etwa. 2800 bis 2000 ..... time factor ...”. 25 In this .... Old Testament and early Judaism. FS. A S van der Woude, 137-153. Leiden: Brill. (S.VT 73.).

  9. Dystrophin insufficiency causes selective muscle histopathology and loss of dystrophin-glycoprotein complex assembly in pig skeletal muscle

    Science.gov (United States)

    Duchenne muscular dystrophy (DMD) is caused by a dystrophin deficiency while Becker muscular dystrophy (BMD) is caused by a dystrophin insufficiency or expression of a partially functional protein product. Both of these dystrophinopathies are most commonly studied using the mdx mouse and a golden r...

  10. Berlinskaja stena i Rimskije kanikulõ / Jevgeni Levik

    Index Scriptorium Estoniae

    Levik, Jevgeni

    2003-01-01

    Mängufilmid "Hüvasti, Lenin!" ("Good Bye, Lenin!") : režissöör Wolfgang Becker : Saksamaa 2003 ja "Lizzie McGuire - koolitüdrukust popstaariks" ("The Lizzie McGuire Movie") : režissöör Jim Fall : Ameerika Ühendriigid 2003

  11. A Model of Interpersonal Persuasion.

    Science.gov (United States)

    Cegala, Donald J.

    The traditional views of audience analysis and rhetorical strategy are examined in terms of modifications necessary for application to persuasion in interpersonal communication contexts. To obtain guidance for ways in which the traditional concepts may be modified, a framework consisting of selected work by Erving Goffman and Ernest Becker is…

  12. An Explication and Partial Test of a Model of Interpersonal Persuasion.

    Science.gov (United States)

    Cegala, Donald J.

    In applying Ernest Becker's work on self-esteem to Erving Goffman's theory of interpersonal society, this paper suggests that Goffman's views about the politeness structure of face-to-face society might be viewed as largely grounded in a basic motivation for individuals to avoid anxiety due to loss of self-esteem. The paper uses this application…

  13. Rogerian Rhetoric: Pedagogy and the Ethos of Seduction.

    Science.gov (United States)

    Hawk, Byron

    In "Rhetoric, Discovery and Change" (1970), R. Young, A. Becker, and K. Pike took Carl Rogers' empathetic approach out of the context of one-on-one therapy and put it into the writing classroom. They proposed the now standard formulaic structure of argumentation which emphasizes a strong thesis up front, a detailed account of the opposing…

  14. TMFunction data: 101 [TMFunction[Archive

    Lifescience Database Archive (English)

    Full Text Available flow cytometry ... immunoprecipitation, immunoplotting ... EPOR_MOUSE (P14753) Helix ... receptor; signal transduction; binding site; flexibility ...einrich AC, Offe JK, Becker V, Cohen J, Neumann D, Klingmuller U. J Biol Chem. 2002 Jul 19;277(29):26547-52.

  15. Reply to "Comment on `Generalized exclusion processes: Transport coefficients' "

    Science.gov (United States)

    Arita, Chikashi; Krapivsky, P. L.; Mallick, Kirone

    2016-07-01

    We reply to the Comment of Becker, Nelissen, Cleuren, Partoens, and Van den Broeck [Phys. Rev. E 93, 046101 (2016), 10.1103/PhysRevE.93.046101] on our article [Arita, Krapivsky, and Mallick, Phys. Rev. E 90, 052108 (2014), 10.1103/PhysRevE.90.052108] about the transport properties of a class of generalized exclusion processes.

  16. Theoretical justification of the von Weimarn law under homogeneous condensation in the free-molecular regime

    Science.gov (United States)

    Kurasov, V. B.

    2016-08-01

    The von Weimarn (Weymarn) law establishes the power dependence for the radius of formed droplets on vapor supersaturation initially created in the system. A theoretical derivation of this law is presented based on the classical Volmer-Becker-Döring-Zel'dovich-Frenkel approach. Analytical expressions have been obtained for all coefficients in this law.

  17. Para estimular a escrita científica

    Directory of Open Access Journals (Sweden)

    Elimar Nascimento

    2016-04-01

    Full Text Available Howard S. Becker. Truques da escrita. Para começar e terminar teses, livros e artigos. São Paulo: Zahar, 2014. 253 p. Tradução de Denise Bottmann; revisão de Karina Kuschnir. ISBN 978-85-378-1394-2.

  18. La teoría del consumidor: enfoques alternativos.

    OpenAIRE

    Eloy, Ávalos

    2011-01-01

    In this paper we present four alternative approaches to consumer theory of Hicks - Allen. Be developed revealed preference approach of Samuelson, the lexicographical preferences, the preferences of the characteristics of the goods of Lancaster and finally the focus of consumption and time allocation of Becker.

  19. 'A Theory of the Allocation of Time' Turns Fifty: Its Impact on the Field of Health Economics.

    Science.gov (United States)

    Grossman, Michael

    2016-01-01

    The year 2015 is the 50th anniversary of the publication of 'A Theory of the Allocation of Time,' by Gary S. Becker in the 1965 volume of The Economic Journal. To mark that occasion, this editorial focuses on the importance of that paper in the history and evolution of the field of health economics. Copyright © 2015 John Wiley & Sons, Ltd.

  20. Effectiveness of Peer-Led Eating Disorders Prevention: A Replication Trial

    Science.gov (United States)

    Becker, Carolyn Black; Bull, Stephanie; Schaumberg, Katherine; Cauble, Adele; Franco, Amanda

    2008-01-01

    The aim of this study was to replicate and extend results of a previous trial that investigated the effectiveness of 2 peer-led eating disorders prevention interventions in reducing eating disorder risk factors in undergraduate women (C. B. Becker, L. M. Smith, & A. C. Ciao, 2006). To extend findings from the previous study by allowing for…

  1. In-situ Detection of Contaminant Plumes in Ground Water

    Science.gov (United States)

    1990-08-01

    Dao, M. Jouan, H. Feurier and H. tation, Los Angeles, in press. Saisse (1986) Simulation et optimisation des capteurs A Weyer, L.G., K.J. Becker and...H.B. Leach.(1987) fibres optiques adjacentes. Applied Optics, 25: Remote sensing fiber optic probe NIR spectroscopy 3448-3454. coupled with

  2. Accurate assessment of intragenic recombination frequency within the Duchenne muscular dystrophy gene.

    Science.gov (United States)

    Abbs, S; Roberts, R G; Mathew, C G; Bentley, D R; Bobrow, M

    1990-08-01

    Polymorphic loci that lie at the two extremities of the Duchenne/Becker muscular dystrophy (DMD/BMD) gene have been used to estimate intragenic recombination rates. Multipoint linkage analysis of the CEPH panel of families suggests a total intragenic recombination frequency of nearly 0.12 (confidence intervals 0.041-0.226) over the genomic length of approximately 2 Mb.

  3. The Effects of Parenting Styles on Children's Self-Esteem: A Developmental Perspective.

    Science.gov (United States)

    Elings, Joette Renee

    The effects of parenting styles on children's self-esteem were examined by dividing the empirical literature into three age groups: the preschool child; the elementary child; and the adolescent. Major literature reviews by W. C. Becker, B. Martin, and E. E. Maccoby and J. A. Martin are discussed and inconsistencies among the reviews are examined.…

  4. Testing the Human Capital Development Model: The Case of Apprenticeships in Turkey

    Science.gov (United States)

    Akpinar, Taner; Gün, Servet

    2016-01-01

    Human capital theory was developed to study how individual agents make rational choices or how they invest in human capital to maximize their welfare. One of the leading founders of this perspective, Becker, argues that schooling, on-the-job training, medical care, migration and searching for information about prices and incomes are different…

  5. Local Anesthesia Part 2: Technical Considerations

    Science.gov (United States)

    Reed, Kenneth L.; Malamed, Stanley F.; Fonner, Andrea M.

    2012-01-01

    An earlier paper by Becker and Reed provided an in-depth review of the pharmacology of local anesthetics. This continuing education article will discuss the importance to the safe and effective delivery of these drugs, including needle gauge, traditional and alternative injection techniques, and methods to make injections more comfortable to patients. PMID:23050753

  6. Optimal taxation of married couples with household production

    DEFF Research Database (Denmark)

    Kreiner, Claus Thustrup; Kleven, Henrik Jacobsen

    2007-01-01

    The literature suggests that the concern for economic efficiency calls for individual-based taxation of married couples with a higher rate on the primary earner. This paper reconsiders the choice of tax unit in the Becker model of household production. In the absence of restrictions on the use of...

  7. The Bicycle Helmet Attitudes Scale: Using the Health Belief Model to Predict Helmet Use among Undergraduates

    Science.gov (United States)

    Ross, Thomas P.; Ross, Lisa Thomson; Rahman, Annalise; Cataldo, Shayla

    2010-01-01

    Objective: This study examined bicycle helmet attitudes and practices of college undergraduates and developed the Bicycle Helmet Attitudes Scale, which was guided by the Health Belief Model (HBM; Rosenstock, 1974, in Becker MH, ed. "The Health Belief Model and Personal Health Behavior". Thorofare, NJ: Charles B. Slack; 1974:328-335) to predict…

  8. SSP推进器——一项独特的设计

    Institute of Scientific and Technical Information of China (English)

    梁华生

    2000-01-01

    The CONSORTIUM PROPULSOR, a joint venture of SCHOTTEL-Werft Josef Becker GmbH & Co. KG and Siemens AG, Marine Engineering, has developed a new podded azimuthing diesel-electric propulsion system for power outputs in the range of 5 to 30 MW per unit.

  9. Why do successful restaurants not raise their prices? : Market forces of demand and the all-or-nothing demand curve

    NARCIS (Netherlands)

    Folmer, Henk; Leen, Auke

    Becker (J Political Econ 99:1109-1116, 1991) addresses the problem of persistent queues at popular restaurants. He poses the question why such restaurants do not raise their prices, thus reducing the queues while expanding profits. He presents a solution based on the assumption that demand by a

  10. Object and Method in Rhetorical Criticism: From Wichelns to Leff and McGee.

    Science.gov (United States)

    Gaonkar, Dilip Parameshwar

    1990-01-01

    Examines the fluctuating dialectic between object and method in three parts: (1) detailed reading of Herbert Wichelns' founding essay; (2) discussion of three influential responses to this dialectic by Ernest Wrage, Samuel Becker, and Edwin Black; and (3) analysis of Michael Leff's and Michael McGee's attempt to reconnect object and method. (KEH)

  11. Special Section: A Debate on Research Techniques in Economic Education

    Science.gov (United States)

    Dawson, George G.; And Others

    1976-01-01

    Dawson introduces three articles which debate merits of research techniques in undergraduate economic education. William E. Becker criticizes John C. Soper's models, multicollinearity argument, and student incentives in a research project; Soper replies; Robert Highsmith critically analyzes strengths and weaknesses of each argument. (AV)

  12. Distribution of the mRNA for protein phosphatase T in rat brain

    NARCIS (Netherlands)

    Becker, W; Buttini, M; Limonta, S; Boddeke, H; Joost, HG

    1996-01-01

    We have recently cloned a novel protein serine/threonine phosphatase (PPT) from rat mRNA which is predominantly expressed in the brain (Becker et al., J. Biol. Chem., 269 (1994) 22586-22592). In the present study, the regional distribution of PPT mRNA in the brain of adult rats was characterized by

  13. Strategic Performance Options in Professional Service Organizations

    NARCIS (Netherlands)

    Doorewaard, J.A.C.M.; Meihuizen, H.E.

    2000-01-01

    In the current debate on HRM and organisational performance (Mahoney and Pandian, 1992; Storey, 1992; Paauwe, 1994; Wright et al, 1994; Flood et al, 1995; Becker and Gerhardt, 1996; Boxall, 1996; Mueller, 1996; Beatty and Schneider, 1997; Coff, 1997; Guest, 1997; Paauwe and Richardson, 1997; Boxall

  14. Screencasts and Standards: Connecting an Introductory Journalism Research Course with Information Literacy

    Science.gov (United States)

    Kuban, Adam J.; Mulligan, Laura MacLeod

    2014-01-01

    Academic literature suggests that today's college students are not as information literate as educators might expect, despite their generation being frequently described as "digital natives" or "Millennials" (Becker, 2009). A faculty survey at Pima Community College in Tucson, AZ, revealed that students' actual…

  15. Strategic Performance Options in Professional Service Organizations

    NARCIS (Netherlands)

    Doorewaard, J.A.C.M.; Meihuizen, H.E.

    2000-01-01

    In the current debate on HRM and organisational performance (Mahoney and Pandian, 1992; Storey, 1992; Paauwe, 1994; Wright et al, 1994; Flood et al, 1995; Becker and Gerhardt, 1996; Boxall, 1996; Mueller, 1996; Beatty and Schneider, 1997; Coff, 1997; Guest, 1997; Paauwe and Richardson, 1997;

  16. Meaning of Muscular Dystrophy

    Science.gov (United States)

    ... MD Living With MD en español Qué significa distrofia muscular Over Labor Day, just as you're ... grown-up. This article talks about two types: Duchenne and Becker MD. Generally, only boys get Duchenne ...

  17. Raamatuautomaadid Hamburgis / A. Bösch ; refereerinud Lea Laurberg-Heckelmann

    Index Scriptorium Estoniae

    Bösch, A.

    2012-01-01

    Refereeritud väljaandest: Bösch, A. Eine Schachtel Literatur ziehen: Bettina von Bülow betreibt in Hamburg Bücherautomaten - Das Angebot reicht vom Comics bis zum Stadtführer. - Lübecker Nachrichten, 30./31.10.2011, lk. 19

  18. Use of Agent Based Modelling to Investigate the Dynamics of Slum ...

    African Journals Online (AJOL)

    pnyamhute

    It is a discrete space in a virtual world in which ..... horizontal sprawl year on year, though in reality vertical sprawl is now also in progress. .... Augustijn-Beckers EW 2008 in Cross - fertilization between three different teaching modes for geo -.

  19. Department of Clinical Investigation, Madigan Army Medical Center, Tacoma, Washington. Annual Research Progress Report, Fiscal Year 1993

    Science.gov (United States)

    1993-09-30

    Pathologic Correlation: Hemangioblastoma. Amer Journal Murphy FM, Rushing EJ of Neuroradiology 13: 1343-1352, 1993. Koo B, Becker LR, Mitochondrial ...may include any of the following: local cellulitis , bacteremia/sepsis, osteomyelitis, streptococcal toxic shock-like syndrome, arthritis, pneumonia

  20. Minority Language Researchers and Their Role in Policy Development

    Science.gov (United States)

    Gorter, Durk

    2012-01-01

    This paper deals with the role of researchers in the development of language policies for European minority languages. This question is placed in the context of a long-standing debate in sociology to which several authors have contributed; among them are Max Weber, Howard Becker and Alvin Gouldner. This article also briefly refers to the European…

  1. I snova pro ljubov... k paukam i politseiskim / Jevgeni Levik

    Index Scriptorium Estoniae

    Levik, Jevgeni

    2002-01-01

    Prantsuse mängufilm "Armastab, ei armasta" ("A la folie...pas du tout") : režissöör Laetitia Colombani ja kaks ameerika mängufilmi - "Super Troopers" : režissöör Jay Chandrasekhar ning "Van Wilder : Party Liaison" : režissöör Walt Becker

  2. Hoone kui bürokraatia tipp / Piret Tali

    Index Scriptorium Estoniae

    Tali, Piret, 1972-

    2004-01-01

    Tutvustatakse vastrenoveeritud Euroopa Komisjoni peahoonet Berlaymont Brüsselis. Arhitekt Lucien de Vestel koos kaastöötajatega. Hoone ümberehituse arhitektid olid Pierre Lallemand, Steven Beckers ja Wilfred van Campenhout. Hoone renoveeriti ehitamisel kasutatud asbesti tõttu

  3. Cardiovascular events in acute coronary syndrome patients with peripheral arterial disease treated with ticagrelor compared to clopidogrel: Data from the PLATO trials

    DEFF Research Database (Denmark)

    Patel, Manesh R.; Becker, Richard C.; Wojdyla, Daniel M.;

    Abstract 14299: Cardiovascular Events in Acute Coronary Syndrome Patients With Peripheral Arterial Disease Treated With Ticagrelor Compared to Clopidogrel: Data From the PLATO Trial Manesh R Patel1; Richard C Becker1; Daniel M Wojdyla2; Håkan Emanuelsson3; William Hiatt4; Jay Horrow5; Steen Husted6...

  4. Reference Sources: Little Known to Basic

    Science.gov (United States)

    RQ, 1970

    1970-01-01

    This article lists seventy-eight reference sources, and is divided into the following four sections: (1) Useful But Little-Known Reference Tools by John Fetros, (2) The Statesman's Year-Book by James Becker, (3) Single Volume Scientific Encyclopedias by Jack Clarke and (4) Ten Basic Sources of Business and Economic Statistics by James Woy. (MF)

  5. Beyond Sarrazin? Zur Darstellung von Migration in deutschen Medien am Beispiel der Berichterstattung in SPIEGEL und BILD

    Directory of Open Access Journals (Sweden)

    Ulrike Irrgang

    2011-12-01

    Full Text Available This paper examines how the German news magazine DER SPIEGEL and the daily BILD cover the issue of migration in Germany. As other studies in the early 1990s have shown, the debate in both print media was dominated by negative and polemic articles. The analysis conducted in this paper clarifies to what extent this also applies to the more recent news coverage. By using quantitative and qualitative content analysis, the paper tries to answer the following questions: What kind of actors and institutional players are involved in the covered debate? How is the phenomenon of migration framed politically, economically or culturally? In which way do both news media evaluate migration? The case study includes 30 articles of DER SPIEGEL and 54 articles of BILD; all of them were published in August and October 2010. At that time, Thilo Sarrazin released his book “Deutschland schafft sich ab” which focuses on Muslim immigration and revived the public debate on immigration and integration in Germany. The paper also examines, if this event has affected the news coverage. The results show that the daily BILD still covers migration in a very unbalanced manner. Muslim immigrants are predominantly represented as a threat to the “German culture”. DER SPIEGEL covers the debate in a more differentiated way with articles written by migrants showing Muslims and migrants as part of the German society. However, there are also statements made by politicians as well as entire articles that only emphasise the economic benefit of migration and thereby objectify people with a migrant background. In both print media, the book release had an apparent effect on the coverage. This can be recognized by an increase of articles on migration in October 2010.

  6. The Humorous French: Effroyables Jardins%残酷中饱含幽默亮色的人性:《恐怖花园》

    Institute of Scientific and Technical Information of China (English)

    刘宇

    2005-01-01

    片名:《恐怖花园》(Effroyables Jardinsl;片长:95分钟年代:2003年;出品国:法国;编剧:让·贝克(Jean Becker);导演:让·贝克(JeanBecker) 让·葛斯莫(Jean Cosmos) 主演:雅克·维勒埃(Jacques Viiieret)安德赫·杜合利叶(Andr e Dussollier)蒂埃里·莱赫米特(Thierry Lhermitte)伯努瓦·玛伊麦尔(Benot Maximel) ;雅克是小镇上一名教师。平时严肃认真、受人尊敬的他每到星期天和节日都会到镇上演出,扮演小丑,为大家带去欢乐。

  7. Awareness Programs and Change in Taste-based Caste Prejudice

    DEFF Research Database (Denmark)

    Banerjee, Ritwik; Gupta, Nabanita Datta

    2015-01-01

    Becker's theory of taste-based discrimination predicts that relative employment of the discriminated social group will improve if there is a decrease in the level of prejudice for the marginally discriminating employer. In this paper we experimentally test this prediction offered by Garry Becker...... in his seminal work on taste based discrimination, in the context of caste in India, with management students (potential employers in the near future) as subjects. First, we measure caste prejudice and show that awareness through a TV social program reduces implicit prejudice against the lower caste...... and the reduction is sustained over time. Second, we find that the treatment reduces the prejudice levels of those in the left tail of the prejudice distribution - the group which can potentially affect real outcomes as predicted by the theory. And finally, a larger share of the treatment group subjects exhibit...

  8. Awareness programs and change in taste-based caste prejudice.

    Science.gov (United States)

    Banerjee, Ritwik; Datta Gupta, Nabanita

    2015-01-01

    Becker's theory of taste-based discrimination predicts that relative employment of the discriminated social group will improve if there is a decrease in the level of prejudice for the marginally discriminating employer. In this paper we experimentally test this prediction offered by Garry Becker in his seminal work on taste based discrimination, in the context of caste in India, with management students (potential employers in the near future) as subjects. First, we measure caste prejudice and show that awareness through a TV social program reduces implicit prejudice against the lower caste and the reduction is sustained over time. Second, we find that the treatment reduces the prejudice levels of those in the left tail of the prejudice distribution--the group which can potentially affect real outcomes as predicted by the theory. And finally, a larger share of the treatment group subjects exhibit favorable opinion about reservation in jobs for the lower caste.

  9. Eliciting and Measuring Betrayal Aversion using the BDM Mechanism*

    Science.gov (United States)

    Quercia, Simone

    2016-01-01

    Betrayal aversion has been operationalized as the evidence that subjects demand a higher risk premium to take social risks compared to natural risks. This evidence has been first shown by Bohnet and Zeckhauser (2004) using an adaptation of the Becker – DeGroot – Marschak mechanism (BDM, Becker et al. (1964)). We compare their implementation of the BDM mechanism with a new version designed to facilitate subjects’ comprehension. We find that, although the two versions produce different distributions of values, the size of betrayal aversion, measured as an average treatment difference between social and natural risk settings, is not different across the two versions. We further show that our implementation is preferable to use in practice as it reduces substantially subjects’ mistakes and the likelihood of noisy valuations. PMID:27366658

  10. Union formation in later life: economic determinants of cohabitation and remarriage among older adults.

    Science.gov (United States)

    Vespa, Jonathan

    2012-08-01

    This study builds on Becker's and Oppenheimer's theories of union formation to examine the economic determinants of marriage and cohabitation during older adulthood. Based on the 1998-2006 Health and Retirement Study and a sample of previously married Americans who are at least 50 years old, results show that wealthier older adults, regardless of gender, are more likely to repartner than stay single. Wealth has no discernable effect on the likelihood of remarrying versus cohabiting. Among the oldest men, the positive associations between wealth and repartnering are entirely due to housing assets. Results suggest that Oppenheimer's theory of marriage timing may be more applicable to later-life union formation than Becker's independence hypothesis. Further, economic disadvantage does not appear to characterize later-life cohabitation, unlike cohabitation during young adulthood. These findings help illuminate the union formation process during older adulthood and are timely considering demographic changes reshaping the American population.

  11. On the predictions and limitations of the Becker–Döring model for reaction kinetics in micellar surfactant solutions

    KAUST Repository

    Griffiths, I.M.

    2011-08-01

    We investigate the breakdown of a system of micellar aggregates in a surfactant solution following an order-one dilution. We derive a mathematical model based on the Becker-Döring system of equations, using realistic expressions for the reaction constants fit to results from Molecular Dynamics simulations. We exploit the largeness of typical aggregation numbers to derive a continuum model, substituting a large system of ordinary differential equations for a partial differential equation in two independent variables: time and aggregate size. Numerical solutions demonstrate that re-equilibration occurs in two distinct stages over well-separated timescales, in agreement with experiment and with previous theories. We conclude by exposing a limitation in the Becker-Döring theory for re-equilibration of surfactant solutions. © 2011 Elsevier Inc.

  12. Effect of a vocationally-focused brief cognitive behavioural intervention on employment-related outcomes for individuals with mood and anxiety disorders.

    Science.gov (United States)

    Kidd, Sean A; Boyd, Geoffrey M; Bieling, Peter; Pike, Shannon; Kazarian-Keith, Dawnna

    2008-01-01

    Despite an increasing emphasis on the importance of vocational success to the quality of life of individuals with mental illness (Bond, Drake, & Becker, 2008), minimal work has examined the impact of cognitive behavioural interventions that focus on vocational stressors. Vocational stressors commonly faced by persons with mental illness include difficulties with work task completion, obtaining employment, and coping with interpersonal stressors (Becker et al., 1998). The purpose of this pilot study was to examine the effectiveness of a brief cognitive behavioural therapy group intervention that targets vocational stressors for individuals whose vocational functioning had been significantly impacted by mental illness. Participants included 16 individuals with mood and anxiety disorder diagnoses. After this intervention, it was found that employed persons reported an improved sense of mastery in the completion of work tasks, improved satisfaction with work supervision, and decreased satisfaction with advancement and job security. Unemployed participants reported improved expectancy for employment success.

  13. Electrical Enhancement of Healing in Combat Injuries to Hard and Soft Tissues.

    Science.gov (United States)

    1979-04-01

    correlation in a given bone or portion thereof between the amount of calcified tissue (as determined by bone ash values) and the compression or...regulating bone architecture. In: Fleisch, H. Blackwood and M. Owen, editors, Proceedings of the Third European Symposium on Calcified Tissue...1970). The effect of electric current on rat tail tendon collagen in solution. Calc. Tis. Res. 4:330. 12. Norton, L.A. and Becker, S.J. (1972). Altered

  14. The empire of economics El imperio de la economía

    Directory of Open Access Journals (Sweden)

    Ovejero Lucas Félix

    1997-12-01

    Full Text Available This article comments on the use of microeconomic instruments in fields that once were the exclusive preserve of the other social sciences, and its consequences for current theoretical activities. It looks particularly at the Theory of the Family by Becker, who has been accused of being the person most responsible for 'economic imperialism', that is, for applying the view of the calculating and selfish individual --homo economicus-- to the study of social processes that had usually been explained by means of history or institutions. In spite of strong criticisms of Becker, the author considers his work to be meritorious insofar as it contributes to showing, in a passive form, the excessive 'padding' that is hidden behind the trivialities or the darkness --which is not profundity but rather vacuity-- of certain social literature.En este artículo, se comenta el uso del instrumental microeconómico en campos que antes era corto exclusivo de las demás ciencias sociales y sus concecuencias para el quehacer teórico en la actualidad. Se detiene especialmente en la Teoría de la Familia Becker, quien ha sido acusado de ser el principal responsable del 'imperialismo económico', es decir, de aplicar la óptica del individuo calculador y egosita --homo economicus-- al estudio de procesos sociales que solían explicar mediante la historia o las instituciones. A pesar de sus fuertes críticas al trabajo de Becker, el autor considera que su trabajo es meritorio en tanto contribuye a mostrar, en forma pasiva, el exceso de 'paja' que se oculta detrás de las trivialidades o de la oscuridad --que no es profunda sino vacuidad-- de cierta literatura social.

  15. Awarding a Prize

    DEFF Research Database (Denmark)

    Moeran, Brian

    2013-01-01

    This article describes and analyses the selection and prize awarding processes for a biennial ceramics exhibition in Japan. Based on long-term fieldwork in the “art world” (Becker 1982) of contemporary Japanese ceramics, as well as on participant observation of the processes concerned, the article...... addresses and draws upon two sets of sociological writings: one concerned with prizes and awards; the other with evaluative practices....

  16. Diagnostic clues and manifesting carriers in fukutin-related protein (FKRP) limb-girdle muscular dystrophy.

    Science.gov (United States)

    Schottlaender, Lucia V; Petzold, Axel; Wood, Nicholas; Houlden, Henry

    2015-01-15

    Mutations in the fukutin-related protein (FKRP) gene are a known cause of autosomal recessive limb-girdle muscular dystrophy. Clinically, patients resemble Becker's muscular dystrophy and generally present in the first two decades of life with a mild, progressive phenotype. Cardiac involvement is variable. Heterozygous carriers are usually clinically unaffected. We report a patient presenting later in life with life-threatening cardiac failure and we describe for the first time clinically manifesting carriers in the family.

  17. Self-Stabilizing and Efficient Robust Uncertainty Management

    Science.gov (United States)

    2011-10-01

    Orientation of Bees. Cambridge, Mass.: The Belknap Press of Harvard University Press. 24 Aristotle, Historia animalium, IX, 40, Becker 624b; modified... University Department of Communication Systems Engineering Ben-Gurion Boulevard Beer-Sheva, Israel 84751 October 2011 Final Report for 15...Segal 5d. PROJECT NUMBER 5d. TASK NUMBER 5e. WORK UNIT NUMBER 7. PERFORMING ORGANIZATION NAME(S) AND ADDRESS(ES) Ben Gurion University

  18. Proceedings of the Department of Defense Natural Resources Leadership Conference Held in Colorado Springs, CO on 12-16 August 1991

    Science.gov (United States)

    1991-11-22

    48753; (A/V) 623-5180 Becker, Charles SMOPB-EMB; Pine Bluff Arsenal, Pine Bluff, Arkansas 71602-9500; (A/V) 966-2834 Belew, Gary HQ Ft. Carson, 4th...31314- 5300; (912) 767-2584 Buck, Slader Office of Environment & Safety; MCB Camp Pendleton, CA 92055 Bukowski , Grace RAMA/Citizen Alert; PO Box 5339...Freeland, Albert W. DEH, Environmental Management, Fort Knox, KY 40121 Freeman, Charles ESO/DEB Hanscom AFB, MA 01371 Freese, Elizabeth COMNAVSECGRO

  19. The Economics of Crime: Investigating the Drugs-Crime Channel

    OpenAIRE

    Entorf, Horst; Winker, Peter

    2002-01-01

    The rising trends both in drug addiction and crime rates are of major public concern in Germany. Surprisingly, the economic theory of crime seems to ignore the drugs-crime nexus, whereas the criminological literature considers illicit drug use a main reason of criminal activities. This paper provides an econometric assessment of the drugs-crime channel within a Becker-Ehrlich model of crime supply. We analyse three different channels from drug abuse to crime: system-related, economic-related ...

  20. La teoría económica, el afecto y la familia

    Directory of Open Access Journals (Sweden)

    Cuevas , Homero

    2000-06-01

    Full Text Available Este ensayo es un inventario bibliográfico, comentado, de las referencias en el pensamiento económico sobre el tema del afecto y la familia, desde Adam Smith hasta los autores que recientemente han formalizado modelos sobre el tema. Dentro de los autores comentados se encuentran: Smith, Malthus, Sade, Fourier, J.S. Mill, Masoch, Engels, Veblen, Boulding, Tullock, Posner, Becker, Pollack y Bergstrom.

  1. China’s Military Support Facility in Djibouti: The Economic and Security Dimensions of China’s First Overseas Base

    Science.gov (United States)

    2017-07-01

    First Overseas Base Erica Downs, Jeffrey Becker and Patrick deGategno July 2017 This work was performed under Federal Government Contract...existing data sources, gathering and maintaining the data needed, and completing and reviewing this collection of information. Send comments regarding...metric tons of annual service capacity to Djibouti’s port infrastructure, more than doubling the amount of cargo it can handle. Phase 1 opened in

  2. Survey on Classifying Human Actions Through Visual Sensors

    Science.gov (United States)

    2011-05-04

    HMMs for gesture recognition . A threshold model is built to provide dynamic threshold values to distinguish between meaningful and meaningless...Becker, D., Azarbayejani, A., “Invariant Features for 3-D Jester Recognition,” Proceedings from IEEE Automatic Face and Gesture Recognition (AFGR), 1996...Hidden Markov Models for Gesture Recognition ,” IEEE Transaction on Pattern Analysis and Machine Intelligence (PAMI), Vol. 21, 1999, pp. 884-899. [7

  3. Rethinking Food: How United States Agriculture Production Affects Security Policy and Global Markets

    Science.gov (United States)

    2014-06-13

    businesses. In 1978, USAID introduced Policy Directive 71, which affected palm oil , citrus and sugar projects, and related products. This internal...Economic Coordination and Development PD Policy Directive PL-480 Public Law 480 SALT Strategic Arms Limitation Talks RTAA Reciprocal Trade Agreement...field agents that assist in implementing broad US policy statements or legislation (Becker 2014). PL-480– Public Law 480, or Food for Peace. This law

  4. Three-point disc amplitudes in the RNS formalism

    Science.gov (United States)

    Becker, Katrin; Becker, Melanie; Robbins, Daniel; Su, Ning

    2016-06-01

    We calculate all tree level string theory vacuum to Dp-brane disc amplitudes involving an arbitrary RR-state and two NS-NS vertex operators. This computation was earlier performed by K. Becker, Guo, and Robbins for the simplest case of a RR-state of type C (p - 3). Here we use the aid of a computer to calculate all possible three-point amplitudes involving a RR-vertex operator of type C (p + 1 + 2 k).

  5. Evidence of Rational Addiction to Carbonated Soft Drinks?

    OpenAIRE

    Rigoberto A. Lopez; Xiaoou Liu

    2012-01-01

    The purpose of this paper is to apply the Becker-Murphy theory of rational addiction to the case of carbonated soft drinks. The research aims to reveal the rational addiction evidence of carbonated soft drinks and derive policy implications under this evidence. Consumers' rational addictive evidence for carbonated soft drinks provides a warning for the Chinese government to regulate the industry, due to its bad health consequences. – The authors empirically apply a time-varying parameter mode...

  6. International Conference on the Physics of Electronic and Atomic Collisions (16th), Held in New York, NY on 26 July-1 August 1989. Third Conference Program

    Science.gov (United States)

    1989-08-01

    Logic Memory and Packaging Department) Le Croy Instruments The University of Massachusetts MKS Instruments, Inc. Newport Corp. Questek, Inc...Banquet: El]Vegotanian H- Kosher Fi1 Othter Mk -’thod of Payment. H-- Chock (Please make chock payable to XVI ICPF AC., U S. Funds Only) L IMa-ster Card...In The Measurement Of Coherence Parameters 168 Tue 45 In Electron - Heavy Noble Gas Collisions A. ShukLa , K.E. Martus and K. Becker Measurement Of The

  7. The Supply of Young Craftsmen to an Industry.

    Science.gov (United States)

    1980-06-12

    34Recruiting and Retaining Participants in a Manpower Program." Industrial and Labor Relations Review 26 (January 1973): 842-50. TAUSSIG , MICHAEL K. "An...occupation. iSee Michael J. Boskin, "A Conditional Logit Model of Occupational Choice," Journal of Political Economy 82 (March- April 1974): 389-98...OCCUPATIONAL CHOICE Books See general references, esp. Becker and Cottell. Articles BOSKIN, MICHAEL J. "A Conditional Logit Model of Occupational Choice

  8. Women, Fertility and Economics: Fifty Years of Research

    OpenAIRE

    Felipe Del Río; Nelson Alvis; Martha Yánez; Raúl Quejada; Karina Acevedo

    2010-01-01

    This paper provides a systematic review of the scientific literature on fertility and its relationship with economics. We explore theoretical and empirical frameworks developed in the last fifty years, emphasizing on the classical (Becker, Easterling) and unorthodox approaches (Bongaarts, Iannaccone, Lehrer). This literature review focused on journals of economics, sociology and demography indexed in the Journal Storage database (JSTOR), Elsevier’s research database (ScienceDirect), Elton B. ...

  9. Sensitivity Simulation of Compressed Sensing Based Electronic Warfare Receiver Using Orthogonal Matching Pursuit Algorithm

    Science.gov (United States)

    2016-02-01

    with equal probability. The scheme was proposed [2] for image processing using single pixel camera, where the field of view was masked by a grid...distribution unlimited. 3.0 SUMMARY AND FUTURE WORK Compressed sensing technology has generated interest in image and signal processing . It is of great...Reconstruction Algorithm, Master Thesis , Feb, 2009. [5] S. R. Becker, “Practical Compressed Sensing: Modern Data Acquistion and Signal Processing

  10. Discrimination and Skill Differences in an Equilibrium Search Model

    OpenAIRE

    Bowlus, Audra J.; Eckstein, Zvi

    1998-01-01

    In this paper, we analyse an equilibrium search model with three sources for wage and unemployment differentials among workers with the same (observed) human capital but different appearance (race): unobserved productivity (skill), search intensities and discrimination (Becker 1957) due to an appearance-based employer disutility factor. Because these sources affect the earnings distributions differently, empirical identification of these potential sources for the explanation of wage and unemp...

  11. Testing the Children Quantity-Quality Trade-Off in India

    OpenAIRE

    Kumar, Santosh; Kugler, Adriana

    2011-01-01

    Using data from a representative sample from India, we test the empirical validity of Quantity-Quality tarde-off model of Becker and Lewis (1973). To address the endogeneity arising from the joint determination of quantity and quality of children by parents, we instrument the family size by sex of the first child. We find a negative relationship between family size and children's educational attainment, even after controlling for parent's characteristics and birth order of children. ...

  12. Erratum to: Six years ahead: a longitudinal analysis regarding course and predictive value of the Strengths and Difficulties Questionnaire (SDQ) in children and adolescents.

    Science.gov (United States)

    Becker, Andreas; Rothenberger, Aribert; Sohn, Alexander; Ravens-Sieberer, Ulrike; Klasen, Fionna

    2015-06-01

    Erratum to: Eur Child Adolesc PsychiatryDOI 10.1007/s00787‑014‑0640‑x Unfortunately, the names of two authors, Ulrike Ravens- Sieberer and Fionna Klasen, were omitted in the original publication of the article. Please find the correct author list below:Andreas Becker · Aribert Rothenberger · Alexander Sohn · Ulrike Ravens-Sieberer · Fionna Klasen · The BELLA study group

  13. Disgust and Shame Based Safe Water and Handwashing Promotion

    Science.gov (United States)

    2015-06-11

    Develop a New Group Version of the Becker-DeGroot-Marsckek (BDM) Auction to Measure Willingness to Pay of Compound Members for Shared Hardware.; Develop a New Survey Instrument to Measure Behavioural Determinants of Hand Washing and Water Treatment Like Disgust and Shame or Social Pressure.; Identify New Methods for Measuring Hand Washing and Water Treatment Behaviour.; Compare the Effectiveness of the Disgust and Shame Based Interventions With Standard Public Health Interventions.

  14. 爱的孤独

    Institute of Scientific and Technical Information of China (English)

    刘皓明

    2000-01-01

    一九0二年二月十二日;同不来梅的女雕塑家克拉拉·威士特霍夫(Clara Westhoff)结婚不到一年的诗人里尔克(Rainer Maria Rilke)在给妻子的女友,艺术家保拉·贝克(Paula Becker)的信中这样写道:

  15. The Military Family: A Selective Bibliography.

    Science.gov (United States)

    1981-09-01

    Program Management ." Commanh r.; Digest 18: entire issue, September 11, 1975 "CHAMPUS Complexitis Explained by ROA Sui-gt.n." CO),. Ialrrv G. Becker...8217’Europe Clasrooms (Delcinse D~ept, ’schools for’ >1 I itarv I~jndn 1i 1 GAO. "’ A ir Forc e 𔃻’imts 30:5 , Sept cmoh r 24 , 1 969 ’’Expanded

  16. Towards Distributed Intelligence: A High Level Definition

    Science.gov (United States)

    2004-12-01

    IEEE Transactions on Robotics and Automation, 8(4), June 1991. 5. Ronald C. Arkin and Johnathan...Arkin. Behavior-based formation control for multi-robot teams. IEEE Transactions on Robotics and Automation, 1999. 9. R. Beckers, O.E. Holland, and J.L...for off road navigation. IEEE Transactions on Robotics and Automation, 10(6):776–782, December 1994. 59. O. Khatib. Real time obstacle avoidance

  17. Preservation of Muscle Force in Mdx3cv Mice Correlates with Low-Level Expression of a Near Full-Length Dystrophin Protein

    OpenAIRE

    2008-01-01

    The complete absence of dystrophin causes Duchenne muscular dystrophy. Its restoration by greater than 20% is needed to reduce muscle pathology and improve muscle force. Dystrophin levels lower than 20% are considered therapeutically irrelevant but are associated with a less severe phenotype in certain Becker muscular dystrophy patients. To understand the role of low-level dystrophin expression, we compared muscle force and pathology in mdx3cv and mdx4cv mice. Dystrophin was eliminated in mdx...

  18. Unregulated high seas fisheries: the interlopers issue

    OpenAIRE

    Coelho, M. P.; Filipe, J.; Manuel Alberto M. Ferreira

    2015-01-01

    Illegal behaviour and public enforcement of law are important theoretical and empirical subjects for Economics. They were dormant in economic scholarship, until the article of Becker, 1968, “Crime and Punishment: An Economic Approach”. In the context of Fisheries Economics, the problem can be seen as an externality arising when exclusive property rights are absent. That absence depends on the costs of defining and enforcing exclusivity and the problem becomes more complex when fisheries are t...

  19. 宣伟公司成功收购北欧木器涂料巨头贝格罗马

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    2010年9月1日,总部位于美国克利夫兰的全球第三大涂料生产商宣伟公司(Sherwin.Williams)正式宣布,已经成功完成对瑞典贝格集团(AB Wilh Becker)旗下木器涂料品牌贝格罗马(Becker Acroma)的收购。

  20. Catalog of Completed Studies, US Army Health Care Studies and Clinical Investigation Activity.

    Science.gov (United States)

    1984-12-10

    Environment Substudy: AD A139001 Effects of Chemical Protective Clothing on the Performance of Basic Medical Tasks (HCSD Report #83-001) 8 DATE TITLE...Contact urticaria to parabens , Arch. Dermatol, 1979, 115:1231-1232. *13 Smith, E. B., Padilla, R. S., McCabe, J. M., Becker, L. E., Benzoyl...Chemical Environment . --3-Report #83-001. Ft. Sam Houston, TX: Health Care Studies and Clinical Investigation Activity, December 1982. 19 Penetar, D. M

  1. Politique de formation professionnelle et d'emploi en Haïti : le cas du secteur du tourisme (1980-2010)

    OpenAIRE

    Doré, Guichard

    2010-01-01

    The study on vocational training policy and employment in Haiti focuses on the tourism industry taking into account the strengths and geographic position. Join in the tradition of human capital theory of Becker G. and linking the issues of training and low level of development, the work explores the main brake material and immaterial acts negatively on the tourism sector and subsequently the country's socioeconomic development. Adopting an approach based on systems analysis of Mélèze J. suppl...

  2. Porcine Zygote Injection with Cas9/sgRNA Results in DMD-Modified Pig with Muscle Dystrophy

    OpenAIRE

    Hong-Hao Yu; Heng Zhao; Yu-Bo Qing; Wei-Rong Pan; Bao-Yu Jia; Hong-Ye Zhao; Xing-Xu Huang; Hong-Jiang Wei

    2016-01-01

    Dystrophinopathy, including Duchenne muscle dystrophy (DMD) and Becker muscle dystrophy (BMD) is an incurable X-linked hereditary muscle dystrophy caused by a mutation in the DMD gene in coding dystrophin. Advances in further understanding DMD/BMD for therapy are expected. Studies on mdx mice and dogs with muscle dystrophy provide limited insight into DMD disease mechanisms and therapeutic testing because of the different pathological manifestations. Miniature pigs share similar physiology an...

  3. Market-Based Instruments for the Conservation of Underutilized Crops: In-Store Experimental Auction of Native Chili Products in Bolivia

    OpenAIRE

    2014-01-01

    Native chilies ( Capsicum spp. ) are currently underutilized in Bolivia, one of this crop’s centers of diversity. Fewer local farmers cultivate native chilies annually due to low market demand. Increasing its private use value can lead to the in-situ conservation of this crop. The objective of the paper is to evaluate the market acceptability of three native chili products: (a) chili marmalade; (b) chili cooking paste; and (c) pickled chilies. Multi-product Becker-DeGroot-Marschak experimenta...

  4. Papers from the Fifth Regional Meeting of the Chicago Linguistic Society, April 18-19, 1969.

    Science.gov (United States)

    Binnick, Robert I., Ed.; And Others

    The topics covered in this volume range over the entire field of linguistics. The authors are: A.L. Becker and D.G. Arms, W.R. Cantrall, G. Cohen, D.J. Darden, A. Davison, D. Elliott and others, J.E. Emonds, B. Fraser, G.M. Green, J.T. Heringer, L.R. Horn, L. Kartunnen, G. Lakoff, R. Lakoff, J.M. Lindholm, V.B. Makkai, J.L. Morgan, Y.C. Morin and…

  5. Station Calibration and Regionalization in China and Surrounding Areas

    Science.gov (United States)

    2005-01-01

    McQuarrie and Tsai, 1998). It also has several cousins such as the corrected-AIC (AICc), Bayesian Information Criteria, Mallows Cp- criteria, and the Swartz...the squared residuals of the inversion, n is the total number of data, and k is the effective number of parameters of the inversion ( McQuarrie and Tsai...UNCLASSIFIED) McQuarrie , A., and C-L Tsai, Regression and Time Series Model Selection, World Scientific, 1998. (UNCLASSIFIED) Michel, G., M. Becker

  6. Diagnosi molecolare della Distrofia Muscolare di Duchenne e della Neurofibromatosi tipo 1 con la tecnologia di sequenziamento Next Generation

    OpenAIRE

    Chessa, Roberta

    2014-01-01

    Duchenne/Becker muscular dystrophies (DMD/BMD) are the most frequent inherited neuromuscular diseases caused by mutations in the dystrophin gene (DMD). Neurofibromatosis type 1 (NF1), caused by mutations in the neurofibromin gene (NF1), is a multisystem genetic disorder that commonly is associated with cutaneous, neurologic, and orthopedic manifestations. Full characterisation of the mutational spectrum is necessary for genetic counselling, prenatal diagnosis and selecting the patients eligib...

  7. Individual Decision-Making to Commit a Crime: A Survey of Early Models

    OpenAIRE

    Roman Horváth; Eva Kolomazníková

    2003-01-01

    The authors provide a survey of the most important findings of the early models of the economics of crime, namely the models of Becker, Ehrlich and Heineke. These models study rational individual decision-making about entering into illegal activities. Probability and size of punishment, attitudes towards risk, gains from crime and income are the main variables influencing the results of individual behavior. The authors also discuss weaknesses of these models such as their static nature or the...

  8. Frege - The Unintentional Linguist. On Frege's Views of Language in the Context of 19th Century German Linguistics

    DEFF Research Database (Denmark)

    Nielsen, Karsten Hvidtfelt

    2009-01-01

    Igennem hele sin professionelle kariere betragtede Frege sproget som en farlig trussel mod den videnskabelige erkendelse. Jeg viser først hvorledes Frege udviklede sin matematiske sproglogik i ofte uintenderet overensstemmelse med samtidige tendenser både inden for tysk lingvistik (Becker, Steint...... sprogskepticisme (Freges såkaldte paradoks). Artiklen slutter med en evaluering af Freges relevans for moderne lingvistik...

  9. Identification and Localization of Minimal MHC-restricted CD8+ T Cell Epitopes within the Plasmodium falciparum AMA1 Protein

    Science.gov (United States)

    2010-08-24

    Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the...A, Muratova O, Awkal M, et al: Phase 1 clinical trial of apical membrane antigen 1: an asexual blood-stage vaccine for Plasmodium falciparum malaria...PfCP-2.9, an asexual blood-stage vaccine candidate of Plasmodium falciparum. Malar J 2010, 9(1):94. 40. Senger T, Becker MR, Schadlich L, Waterboer T

  10. The Economics of Crime: Investigating the Drugs-Crime Channel

    OpenAIRE

    Entorf, Horst; Winker, Peter

    2002-01-01

    The rising trends both in drug addiction and crime rates are of major public concern in Germany. Surprisingly, the economic theory of crime seems to ignore the drugs-crime nexus, whereas the criminological literature considers illicit drug use a main reason of criminal activities. This paper provides an econometric assessment of the drugs-crime channel within a Becker-Ehrlich model of crime supply. We analyse three different channels from drug abuse to crime: system-related, economic-related ...

  11. Plasmonic Aptamer-Gold Nanoparticle Sensors for Small Molecule Fingerprint Identification

    Science.gov (United States)

    2014-08-01

    Acknowledgements This work was partially funded by the Air Force Office of Scientific Research and the Defense Forensic Science Center...Pristinski, M. L. Becker, A. Karim, J. F. Douglas, ACS Nano, 2010, 4(1), 365-379 41. C. H. Lin, D. J. Patel, Chemistry & Biology, 1997, 4(11), 817...Cincinnati, 812 Rhodes Hall, Cincinnati, OH 45221, United States c Current address: College of Arts and Sciences, Department of Chemistry and

  12. Creech Air Force Base Resort Property in Indian Springs, Nevada. Environmental Baseline Survey Report

    Science.gov (United States)

    2011-04-01

    from the irrigation well system reacted vigorously with the hydrochloric acid (HCl) used to preserve the samples. This reaction to HCl was likely due...United States Post Office and a pizza place/bar. The casino was constru cted in 1984, the motel in 1988, and the Chevron station in 1989. Mr. Becker was...D Fed. Land Use: Wilde mess Areas, Wildlife Preserves .................... . Fed

  13. A Long-Term Comparison of GPS Carrierphase Frequency Transfer and Two-Way Satellite Time/Frequency Transfer

    Science.gov (United States)

    2007-01-01

    Metrologia , 42, 411-422. [2] C. Hackman, J. Levine, T. E. Parker, D. Piester and J. Becker, 2006, “A Straightforward Frequency- Estimation Technique for... Metrologia , 43, 109-120. [6] http://igscb.jpl.nasa.gov [7] G. Blewitt, 1989, “Carrier-Phase Ambiguity Resolution for the Global Positioning System Applied... Metrologia , 40, S270-S288. [14] The Institute of Electrical and Electronics Engineers, 1999, IEEE Standard Definitions of Physical Quantities for Fundamental

  14. Rational Addiction Evidence From Carbonated Soft Drinks

    OpenAIRE

    Xiaoou, Liu

    2009-01-01

    This paper applies the Becker-Murphy (1988) theory of rational addiction to the case of carbonated soft drinks, using a time-varying parameter model and scanner data from 46 U.S. cities. Empirical results provide strong evidence that carbonated soft drinks are rationally addictive, thus opening the door to taxation and regulation. Taking rational addition into account, estimated demand elasticities are much lower than previous estimates using scanner data.

  15. US Army Institute of Surgical Research Annual Research Progress Report for Fiscal Year 1991

    Science.gov (United States)

    1991-10-01

    Aesthetic Plast Surg 12:95-9, 1988. 5. Quinn KJ, Evans JM. Courtney JM, et al: Non-pressure treatment of hypertrophic scars. Burns 12:102-8, 1985...Trauma 8:1049-51, 1968. 16. Wolcott LE, Wheeler PC, Hardwicke HM, Rowley BA: Accelerated healing of skin ulcers by electrotherapy : Preliminary clinical... Electrotherapy for acceleration of wound healing: Low intensity direct current. Arch Phys Med Rehabil 66:443-6, 1985. 1 22. Becker RO, Spadaro JA

  16. Industriarbejdere mellem tradition og forandring

    DEFF Research Database (Denmark)

    Caraker, Emmett

    Eksisterer det klassiske arbejderkollektiv, på hvilke betingelser og hvorfor? Det er denne afhand-lings problemstilling. Undersøgelsen foregår i 2 industrivirksomheder, og i alt 80 industriarbejdere er interviewet. I den ene case møder industriarbejderne efter en virksomhedsoverdragelse ledelsens...... Lysgaard, Mancur Olson, E. P. Thompson, Michael Vester, E.O. Wright, Claus Offe, Thomas Leithäuser, Regina Becker-Schmidt, Oscar Negt....

  17. Chemical Equilibrium Mixture Computations for Energetic Material Combustion in Closed Vessels

    Science.gov (United States)

    2004-12-01

    are used with well-known equations of state stemming from Redlich , Kwong and Soave (RKS); Benedict, Webb, Rubin, Starling and Han (BWRSH); Becker...mixture of numerous imperfect gases and compressible liquid and solid species with phase transitions for closed-vessel applications . This code minimizes...solid species. The benefits of the computational methods in providing accurate pre- dictions for the applications mentioned previously de- pend

  18. 内生偏好、公共财政运作效率与哈伯格-劳尔森-梅茨勒效应%Endogenous Preference, Operational Efficiency of Public Finance and H-L-M Effect

    Institute of Scientific and Technical Information of China (English)

    朱军

    2013-01-01

    经典的“H-L-M”效应(“哈伯格-劳尔森-梅茨勒”效应)已经被各国经济学者探究了半个多世纪,但截止到目前,还没有文献基于Becker-Mulligan (1997)的贴现偏好进行理论分析,并且目前所有的研究均是考虑政府使用资源的确定性状态.对此,本文基于Becker-Mulligan (1997)的贴现因子讨论是否存在“H-L-M”效应,并比较了政府不同财政运转效率的结果.研究发现:在Becker-Mulligan (1997)贴现偏好下,贸易条件恶化将会导致经常账户出现盈余;而贸易条件恶化时,政府财政运转效率对于经常账户的重新平衡具有“加速”作用.

  19. DMD transcript imbalance determines dystrophin levels.

    Science.gov (United States)

    Spitali, Pietro; van den Bergen, Janneke C; Verhaart, Ingrid E C; Wokke, Beatrijs; Janson, Anneke A M; van den Eijnde, Rani; den Dunnen, Johan T; Laros, Jeroen F J; Verschuuren, Jan J G M; 't Hoen, Peter A C; Aartsma-Rus, Annemieke

    2013-12-01

    Duchenne and Becker muscular dystrophies are caused by out-of-frame and in-frame mutations, respectively, in the dystrophin encoding DMD gene. Molecular therapies targeting the precursor-mRNA are in clinical trials and show promising results. These approaches will depend on the stability and expression levels of dystrophin mRNA in skeletal muscles and heart. We report that the DMD gene is more highly expressed in heart than in skeletal muscles, in mice and humans. The transcript mutated in the mdx mouse model shows a 5' to 3' imbalance compared with that of its wild-type counterpart and reading frame restoration via antisense-mediated exon skipping does not correct this event. We also report significant transcript instability in 22 patients with Becker dystrophy, clarifying the fact that transcript imbalance is not caused by premature nonsense mutations. Finally, we demonstrate that transcript stability, rather than transcriptional rate, is an important determinant of dystrophin protein levels in patients with Becker dystrophy. We suggest that the availability of the complete transcript is a key factor to determine protein abundance and thus will influence the outcome of mRNA-targeting therapies.

  20. Recommendations for competency in allergy training for undergraduates qualifying as medical practitioners: a position paper of the world allergy organization.

    Science.gov (United States)

    Potter, Paul C; Warner, John O; Pawankar, Ruby; Kaliner, Michael A; Del Giacco, Sergio; Rosenwasser, Lanny

    2009-08-01

    The Council acknowledges specific comments from: The American Academy of Allergy, Asthma and Immunology (AAAAI) (Amal H Assa'ad); The American College of Allergy, Asthma and Immunology (ACAAI) (Mark Dykewicz, D. Betty Lew, Bryan L. Martin); The Argentine Association of Allergy and Immunology (Ledit RF Ardusso); The Argentine Society of Allergy and Immunopathology (Estrella Asayag); The Australasian Society of Clinical Immunology and Allergy (ASCIA) (Jill Smith); The British Society for Allergy and Clinical Immunology (Stephen Durham); The Brazilian Society of Allergy and Immunopathology (Nelson Rosario); The Bulgarian Society of Allergology (Vasil Dimitrov); The Canadian Society of Allergy and Clinical Immunology (CSACI) (Richard Warrington); The Chilean Society of Allergy and Immunology (Jessica Salinas); The Chinese Society of Allergology (Zhang Hongyu, Yin Jia); The Czech Society of Allergology and Clinical Immunology (Jiri Litzman); The Danish Society of Allergology (Lone Winther, Peter Plaschke); The Egyptian Society of Allergy and Clinical Immunology (Kamal Maurice Hanna); The Egyptian Society of Pediatric Allergy and Immunology (Yehia El-Gamal); The German Society for Allergy and Clinical Immunology (Thilo Jakob, Claus Bachert, Bernhard Przybilla); The Hungarian Society of Allergology and Clinical Immunology (Kristof Nekam); The Icelandic Society of Allergy and Clinical Immunology (Björn R. Lúđvíksson); The Italian Association of Territorial and Hospital Allergists (Riccardo Asero); The Italian Society of Allergy and Clinical Immunology (Luigi Fontana); The Japanese Society of Allergology (Sankei Nishima); The Korean Academy of Asthma Allergy and Clinical Immunology (Joon Sung Lee, Hae-Sim Park); The Latvian Association of Allergists (Ieva Cirule); The Lebanese Society of Allergy & Immunology (Fares Zaitoun); The Mongolian Society of Allergology (S. Munkhbayarlakh); The Allergy and Clinical Immunology Society (Singapore) (Chng Hiok Hee); The Allergy

  1. Lurasidone for the treatment of bipolar depression: an evidence-based review

    Directory of Open Access Journals (Sweden)

    Franklin R

    2015-08-01

    Full Text Available Rachel Franklin,1 Sam Zorowitz,1 Andrew K Corse,1 Alik S Widge,2 Thilo Deckersbach1 1Division of Neurotherapeutics, Department of Psychiatry, Massachusetts General Hospital, Harvard Medical School, Charlestown, 2Picower Institute for Learning and Memory, Massachusetts Institute of Technology, Cambridge, MA, USA Abstract: Bipolar disorder (BD is a debilitating and difficult-to-treat psychiatric disease that presents a serious burden to patients’ lives as well as health care systems around the world. The essential diagnostic criterion for BD is episodes of mania or hypomania; however, the patients report that the majority of their time is spent in a depressive phase. Current treatment options for this component of BD have yet to achieve satisfactory remission rates. Lurasidone is a drug in the benzisothiazole class approved by the US Food and Drug Administration in June 2013 for the acute treatment of bipolar depression. Its pharmacological profile features high-affinity antagonism at D2, 5-HT2A, and 5-HT7 receptors; moderate-affinity antagonism at α2C-adrenergic receptors; low- to very low-affinity antagonism at α1A-adrenergic, α2A-adrenergic, H1, M1, and 5-HT2C receptors; and high-affinity partial agonism at 5-HT1A. Preliminary findings from two recent double-blinded clinical trials suggest that lurasidone is efficacious in treating bipolar I depression, with clinical effects manifesting as early as the first 2–3 weeks of treatment (as measured by the Montgomery–Åsberg Depression Rating Scale and Clinical Global Impressions Scale for use in bipolar illness. Its therapeutic benefit appears to be comparable to the current US Food and Drug Administration-indicated treatments: quetiapine and olanzapine–fluoxetine, according to a measure of effect size known as number needed to treat. These studies reported relatively limited extrapyramidal and metabolic side effects as a result of treatment with lurasidone, with the most common side

  2. Assessment of Mono- and Split-Window Approaches for Time Series Processing of LST from AVHRR—A TIMELINE Round Robin

    Directory of Open Access Journals (Sweden)

    Corinne Myrtha Frey

    2017-01-01

    Full Text Available Processing of land surface temperature from long time series of AVHRR (Advanced Very High Resolution Radiometer requires stable algorithms, which are well characterized in terms of accuracy, precision and sensitivity. This assessment presents a comparison of four mono-window (Price 1983, Qin et al., 2001, Jiménez-Muñoz and Sobrino 2003, linear approach and six split-window algorithms (Price 1984, Becker and Li 1990, Ulivieri et al., 1994, Wan and Dozier 1996, Yu 2008, Jiménez-Muñoz and Sobrino 2008 to estimate LST from top of atmosphere brightness temperatures, emissivity and columnar water vapour. Where possible, new coefficients were estimated matching the spectral response curves of the different AVHRR sensors of the past and present. The consideration of unique spectral response curves is necessary to avoid artificial anomalies and wrong trends when processing time series data. Using simulated data on the base of a large atmospheric profile database covering many different states of the atmosphere, biomes and geographical regions, it was assessed (a to what accuracy and precision LST can be estimated using before mentioned algorithms and (b how sensitive the algorithms are to errors in their input variables. It was found, that the split-window algorithms performed almost equally well, differences were found mainly in their sensitivity to input bands, resulting in the Becker and Li 1990 and Price 1984 split-window algorithm to perform best. Amongst the mono-window algorithms, larger deviations occurred in terms of accuracy, precision and sensitivity. The Qin et al., 2001 algorithm was found to be the best performing mono-window algorithm. A short comparison of the application of the Becker and Li 1990 coefficients to AVHRR with the MODIS LST product confirmed the approach to be physically sound.

  3. Uydu Verileri Kullanılarak İzmir Şehir Merkezinin Yer Yüzey Sıcaklığının Tahmini

    Directory of Open Access Journals (Sweden)

    Mehmet ŞAHİN

    2014-07-01

    Full Text Available Yer Yüzey Sıcaklığı (YYS, dünya yüzeyi ve atmosfer arasında enerji değişimini kontrol ettiği için önemli bir parametredir. Buna ek olarak; iklimsel değişim, sayısal hava tahmini, evrensel su döngüsü, kuraklık indeksi, don gibi çoğu çevresel niceliklerin modellemelerinin geliştirilmesinde kullanılmaktadır. Bu çalışmada, YYS verilerini elde etmek için NOAA (Ulusal Okyanus ve Atmosfer Yönetimi/AVHRR (Yüksek Çözünürlüklü Radyometre verilerinin termal kanalları (kanal 4 ve 5 ve YYS algoritmaları (Price–1984, Becker ve Li–1990, Ulivieri vd.-1994 birlikte kullanılmıştır. İzmir şehri için yapılan istatistiksel çalışmaların sonunda Price–1984, Becker ve Li–1990, Ulivieri vd.-1994 algoritmalarının OKKH (Ortalama Kare Kök Hatası değerleri sırasıyla 3,6150 °K; 2,2430 °K; 2,3905 °K bulunmuştur. Araştırma bulguları, İzmir şehri için Becker ve Li–1990, Ulivieri vd.-1994 algoritmalarının YYS tahmininde kullanılabileceğini ortaya koymaktadır.

  4. Deletion of Dystrophin In-Frame Exon 5 Leads to a Severe Phenotype: Guidance for Exon Skipping Strategies.

    Directory of Open Access Journals (Sweden)

    Zhi Yon Charles Toh

    Full Text Available Duchenne and Becker muscular dystrophy severity depends upon the nature and location of the DMD gene lesion and generally correlates with the dystrophin open reading frame. However, there are striking exceptions where an in-frame genomic deletion leads to severe pathology or protein-truncating mutations (nonsense or frame-shifting indels manifest as mild disease. Exceptions to the dystrophin reading frame rule are usually resolved after molecular diagnosis on muscle RNA. We report a moderate/severe Becker muscular dystrophy patient with an in-frame genomic deletion of DMD exon 5. This mutation has been reported by others as resulting in Duchenne or Intermediate muscular dystrophy, and the loss of this in-frame exon in one patient led to multiple splicing events, including omission of exon 6, that disrupts the open reading frame and is consistent with a severe phenotype. The patient described has a deletion of dystrophin exon 5 that does not compromise recognition of exon 6, and although the deletion does not disrupt the reading frame, his clinical presentation is more severe than would be expected for classical Becker muscular dystrophy. We suggest that the dystrophin isoform lacking the actin-binding sequence encoded by exon 5 is compromised, reflected by the phenotype resulting from induction of this dystrophin isoform in mouse muscle in vivo. Hence, exon skipping to address DMD-causing mutations within DMD exon 5 may not yield an isoform that confers marked clinical benefit. Additional studies will be required to determine whether multi-exon skipping strategies could yield more functional dystrophin isoforms, since some BMD patients with larger in-frame deletions in this region have been reported with mild phenotypes.

  5. The People Paradox: Self-Esteem Striving, Immortality Ideologies, and Human Response to Climate Change

    Directory of Open Access Journals (Sweden)

    Janis L. Dickinson

    2009-06-01

    Full Text Available In 1973, Ernest Becker, a cultural anthropologist cross-trained in philosophy, sociology, and psychiatry, invoked consciousness of self and the inevitability of death as the primary sources of human anxiety and repression. He proposed that the psychological basis of cooperation, competition, and emotional and mental health is a tendency to hold tightly to anxiety-buffering cultural world views or "immortality projects" that serve as the basis for self-esteem and meaning. Although he focused mainly on social and political outcomes like war, torture, and genocide, he was increasingly aware that materialism, denial of nature, and immortality-striving efforts to control, rather than sanctify, the natural world were problems whose severity was increasing. In this paper I review Becker's ideas and suggest ways in which they illuminate human response to global climate change. Because immortality projects range from belief in technology and materialism to reverence for nature or belief in a celestial god, they act both as barriers to and facilitators of sustainable practices. I propose that Becker's cross-disciplinary "science of man," and the predictions it generates for proximate-level determinants of social behavior, add significantly to our understanding of and potential for managing the people paradox, i.e., that the very things that bring us symbolic immortality often conflict with our prospects for survival. Analysis of immortality projects as one of the proximate barriers to addressing climate change is both cautionary and hopeful, providing insights that should be included in the cross-disciplinary quest to uncover new pathways toward rational, social change.

  6. On pitfalls in the construction of family-based models of population growth: a note.

    Science.gov (United States)

    Kondo, H

    1986-04-01

    Recently, several attempts have been made to construct an economic theory of population based on a formal theory of the family of the type developed by Becker in 1981, but there are serious limitations in all such efforts. The typical family's problem may have no solution, even with a well-behaved concave utility function. Moreover, even when the family's maximum problem has a unique solution, the phase diagram for the stock of capital may contain no steady state other than the origin. Finally, even when there exists a nontrivial steady state for the stock of capital, the community nevertheless may be destined for extinction. The first of these pitfalls concerns the internal consistency of the models, while the second and third concern the compatibility of the models with some gross facts of life. The pitfalls can be avoided, within the Becker framework by suitably restricting the family's utility and production functions, but the restrictions required are severe. This paper shows that, alternatively, the pitfalls sometimes can be avoided by going slightly outside the Becker framework, specifically, by modifying the typical family's budget constraint to allow explicitly for the cost of raising children. In particular, it is shown that, by this means, the pitfalls can be avoided even when the famil's utility function is log-linear, the example adduced by Kemp et al. In 1984 to demonstrate the existence of pitfalls. More precisely, it is shown that the family's maximum problem has a unique solution; that nontrival steady state exists; that, even if the steady state is locally unstable, the optimal trajectory tends neither to zero nor to infinity but to a 2-period limit cycle; and that survival is possible with quite general production functions. Thus, the end product is a logically consistent and reasonable model of economic development, with both population growth and capital accumulation firmly rooted in life-cycle family planning.

  7. Current concepts in dystrophinopathies.

    Science.gov (United States)

    Viswanathan, Venkataraman

    2015-02-01

    Dystrophinopathies comprise a group of hereditary muscle disorders characterized by progressive wasting and weakness of skeletal muscle, as a result of degeneration of muscle fibers, and can be distinguished by the mode of transmission, age at onset and pattern of muscle weakness. The range of phenotypes associated with the region Xp21 has been expanding since identification of the gene in 1987. The mild end of the spectrum includes the phenotype of the muscle cramps with myoglobinuria and isolated quadriceps myopathy, while at the severe end, there are progressive muscle diseases that are classified as Duchenne / Becker muscular dystrophy (DMD/BMD).

  8. Empatía y distorsiones cognitivas en personas no reclusas y en condenados por delitos sexuales y delitos violentos en Boyacá

    OpenAIRE

    Cepeda Rodríguez , Zulma Yadira

    2012-01-01

    El fin de esta investigación consistió en explorar, describir y comparar las variables empatía y distorsiones cognitivas en tres grupos: agresores sexuales de menores de edad, delincuentes violentos no sexuales y personas sin historia de reclusión en establecimientos carcelarios por medio de una investigación transeccional de tipo descriptivo-comparativo. Para evaluar las variables de estudio se tradujo y adaptó al castellano tanto la Escala de Cognición de Becker y cols (1989), como la Medid...

  9. The embedded feature model for the interpretation of chromospheric contrast profiles

    Science.gov (United States)

    Steinitz, R.; Gebbie, K. B.; Bar, V.

    1977-01-01

    Contrast profiles obtained from chromospheric filtergrams and spectra of bright and dark mottles have to date been interpreted almost exclusively in terms of Becker's cloud model. Here we demonstrate the failure of this model to account in a physically consistent way for the observed contrasts. As an alternative, we introduce an embedded-feature model, restricting our discussion in this paper to stationary features. Our model is then characterized by three independent parameters: the density of absorbing atoms, the geometrical depth, and the profile of the absorption coefficient. An analytic approximation to the contrast resulting from such a model reproduces well the observed behavior of all types of contrast profiles.

  10. The effect of tourism on crime in Italy: A dynamic panel approach

    OpenAIRE

    Biagi, Bianca; Brandano, Maria Giovanna; Detotto, Claudio

    2012-01-01

    The purpose of this paper is to demonstrate that, for the case of Italy, ceteris paribus, tourist areas tend to have a greater amount of crime than non-tourist ones in the short and long run. Following the literature of the economics of crime à la Becker (Crime and Punishment: An Economic Approach, 1968) and Enrlich (Participation in Illegitimate Activities: A Theoretical and Empirical Investigation, 1973) and using a System GMM approach for the time span 1985–2003, the authors empirically te...

  11. Economic analysis of same-sex marriage.

    Science.gov (United States)

    Portelli, Christopher J

    2004-01-01

    This article applies the neoclassical microeconomic analysis of marriage as developed by Nobel laureate economist Gary Becker to same-sex marriage. The objective is to demonstrate that the economic analysis of marriage supports allowing same-sex marriage, and that same-sex marriages would strengthen the incentive to marry, increase the efficiency of marriage markets, provide for more children to be raised in two-parent optimum environments, and benefit states economically overall. The article concludes with an overview of the economic impact of same-sex marriages on states based on the analysis, data and fiscal information currently available from researchers and economists in the field.

  12. Empatía y distorsiones cognitivas en personas no reclusas y en condenados por delitos sexuales y delitos violentos en Boyacá

    OpenAIRE

    Cepeda Rodríguez, Zulma Yadira

    2012-01-01

    El fin de esta investigación consistió en explorar, describir y comparar las variables empatía y distorsiones cognitivas en tres grupos: agresores sexuales de menores de edad, delincuentes violentos no sexuales y personas sin historia de reclusión en establecimientos carcelarios por medio de una investigación transeccional de tipo descriptivo-comparativo. Para evaluar las variables de estudio se tradujo y adaptó al castellano tanto la Escala de Cognición de Becker y cols (1989), como la Medid...

  13. The Interactionist Imagination – Studying Meaning, Situation and Micro-Social Order

    DEFF Research Database (Denmark)

    The study of human interaction is integral to sociology and its attempt to shed light on and understand the social world. The tradition of interactionism is therefore a longstanding and time-honoured perspective within sociology, and although it has always been regarded as an alternative perspect...... to the works of: Georg Simmel, Robert E. Park, George Herbert Mead, Everett C. Hughes, Herbert Blumer, Manford H. Kuhn, Erving Goffman, Harold Garfinkel, Anselm L. Strauss, Jack D. Douglas, Howard S. Becker, Stanford M. Lyman, Arlie R. Hochschild and Gary Alan Fine....

  14. African maize porridge: a food with slow in vitro starch digestibility

    CSIR Research Space (South Africa)

    Van der Merwe, B

    2001-02-15

    Full Text Available intestine. American Journal of Clinical Nutrition, 61, 82?89. Nelles, E. M., Dewar, J., & Taylor, J. R. N. (1999). EC128ect of the par- ticle size of maize grits on extract in sorghum beer brewing. Poster presented at the 5th scienti?c and technical... convention of the Insti- tute of Brewing, Africa Section, Nairobi, Kenya. Omar, M. A. K., Seedat, M. A., Motala, A. A., Dyer, R. B., & Becker, P. (1992). The prevalence of diabetes mellitus and impaired glucose tolerance in a group of urban South African...

  15. The Long-Run Impact on Population and Income of Open Access to Land in a Model with Parental Altruism

    OpenAIRE

    2000-01-01

    Steady state levels of population and per capita income are examined using a Becker-Barro (1988) style of model of an economy with identical altruistic parents bearing costly children who receive bequests of capital and land. Inspired by the work of North (1981) and others, the problem of open access land with ancillary negative effects on private (but not public) productivity of capital is examined. It is seen that open access to land can lead to overpopulation in a ceteris paribus sense, an...

  16. IceCube expectations for two high-energy neutrino production models at active galactic nuclei

    CERN Document Server

    Argüelles, C A; Gago, A M

    2010-01-01

    We have determined the current and near-future allowed regions of the parameter spaces of two representative models of diffuse neutrino flux from AGN: one by Koers & Tinyakov (KT) and another by Becker & Biermann (BB). Our observable has been the predicted number of down- and upgoing muon-neutrinos at IceCube, after 5 years of exposure, in the range 10^510 is not allowed. Finally, we have analysed the capacity of IceCube to discriminate between the models within the parameter regions where they are simultaneously allowed. We have found that their predictions are indistinguishable only inside a small region where \\Gamma_\

  17. Calibration of Vector Magnetogram with the Nonlinear Least-squares Fitting Technique

    Institute of Scientific and Technical Information of China (English)

    Jiang-Tao Su; Hong-Qi Zhang

    2004-01-01

    To acquire Stokes profiles from observations of a simple sunspot with the Video Vector Magnetograph at Huairou Solar Observing Station(HSOS),we scanned the FeIλ5324.19 A line over the wavelength interval from 150mA redward of the line center to 150mA blueward,in steps of 10mA.With the technique of analytic inversion of Stokes profiles via nonlinear least-squares,we present the calibration coefficients for the HSOS vector magnetic magnetogram.We obtained the theoretical calibration error with linear expressions derived from the Unno-Becker equation under weak-field approximation.

  18. Secreted Protein Acidic and Rich in Cysteine (SPARC) in Human Skeletal Muscle

    DEFF Research Database (Denmark)

    Jørgensen, Louise H; Petersson, Stine J; Sellathurai, Jeeva

    2009-01-01

    indicated a function of SPARC in skeletal muscle. We therefore found it of interest to study SPARC expression in human skeletal muscle during development and in biopsies from Duchenne and Becker muscular dystrophy and congenital muscular dystrophy, congenital myopathy, inclusion body myositis...... in the majority of analyzed muscle biopsies (23 of 24), mainly in mononuclear cells of which few were pax7 positive. Myotubes and regenerating myofibers also expressed SPARC. The expression-degree seemed to reflect the severity of the lesion. In accordance with these in vivo findings, primary human...

  19. Antenatal Diagnosis and Carrier Detection of Pseudohypertrophic Muscular Dystrophy%假肥大型肌营养不良症产前诊断与遗传咨询

    Institute of Scientific and Technical Information of China (English)

    尹耕心

    2004-01-01

    假肥大型肌营养不良症(pseudohypertophic muscular dvstrophy)是由于基因缺陷引起的疾病,以进行性肌肉萎缩与肌无力为主要特征,分Duchenne型(Duchenne muscular dystrophy,DMD)和Becker型(Becker muscular dystrophy,BMD)。DMD是假肥大型肌营养不良症中最常见和最严重的类型,发病率为活产男婴的1/3500,多数于12~13岁前丧失行走能

  20. Análise discursiva da relação entre atividade turística, apropriação do território e patrimônio: contribuições para o planejamento sustentável do turismo na Bahia-Brasil

    Directory of Open Access Journals (Sweden)

    Natanael Reis Bomfim

    2009-01-01

    ownership of the territory by the tourist activity and its impacts on culture and the environment, calling attention to the need for sustainable planning in the Bahia-Brazil. To this end, we shall analyze the speech of several authors who deal with interdisciplinary way, the theme of Planning sustainable tourism and its relationship with the regional development (Bomfim, 2008, 2006; Coriolano, 2005; Days, 2003 Becker, 2002 Yazigi, 2002, 2003, Carlos, 2002; Petrocchi, 2001; Santos, 1994, 1997; Soja, 1993; Chadefaud, 1987; Thus, this proposal highlights the need to enhance communities placed outside the economic system.

  1. Three-Point Disc Amplitudes in the RNS Formalism

    CERN Document Server

    Becker, Katrin; Robbins, Daniel; Su, Ning

    2016-01-01

    We calculate all tree level string theory vacuum to Dp-brane disc amplitudes involving an arbitrary RR-state and two NS-NS vertex operators. This computation was earlier performed by K. Becker, Guo, and Robbins for the simplest case of a RR-state of type C_{p-3}. Here we use the aid of a computer to calculate all possible three-point amplitudes involving a RR-vertex operator of type C_{p+1+2k}.

  2. Necesidades de gasto en educación: un modelo orientado a garantizar la igualdad de oportunidades

    OpenAIRE

    Mongan, Juan Carlos

    2009-01-01

    Tanto desde la teoría como desde la evidencia empírica se suele identificar al gasto en educación como uno de los instrumentos más poderosos de los que dispone el Estado para combatir los problemas de pobreza estructural, desempleo, inequidad distributiva y falta de crecimiento económico. Tal afirmación se apoya en gran medida sobre la teoría del capital humano (Becker, 1964). Según ésta, la capacidad de los individuos de insertarse en el mercado laboral de manera estable y bajo condiciones d...

  3. Characterization of DLK1+ cells emerging during skeletal muscle remodeling in response to myositis, myopathies, and acute injury

    DEFF Research Database (Denmark)

    Andersen, Ditte C; Petersson, Stine J; Jørgensen, Louise H

    2009-01-01

    , DLK1 was upregulated in all human myopathies analyzed, including Duchenne- and Becker muscular dystrophies. Substantial numbers of DLK1(+) satellite cells were observed in normal neonatal and Duchenne muscle, and furthermore, myogenic DLK1(+) cells were identified during muscle regeneration in animal...... nonmyogenic DLK1(+) cells and small spindle-shaped cells coexpressing DLK1 and muscle-specific markers were observed. Myogenic differentiation was achieved when sorted DLK1(+) cells were cocultured together with primary myoblasts revealing a myogenic potential that was 10% of the DLK1(-) population...

  4. Screening the dystrophin gene suggests a high rate of polymorphism in general but no exonic deletions in schizophrenics

    Energy Technology Data Exchange (ETDEWEB)

    Lindor, N.M.; Sobell, J.L.; Thibodeau, S.N. [Mayo Clinic/Foundation, Rochester, MN (United States)] [and others

    1994-03-15

    The dystrophin gene, located at chromosome Xp21, was evaluated as a candidate gene in chronic schizophrenia in response to the report of a large family in which schizophrenia cosegregated with Becker muscular dystrophy. Genomic DNA from 94 men with chronic schizophrenia was evaluated by Southern blot analysis using cDNA probes that span exons 1-59. No exonic deletions were identified. An unexpectedly high rate of polymorphism was calculated in this study and two novel polymorphisms were found, demonstrating the usefulness of the candidate gene approach even when results of the original study are negative. 41 refs., 1 fig., 4 tabs.

  5. An Asymptotic Theory for the Re-Equilibration of a Micellar Surfactant Solution

    KAUST Repository

    Griffiths, I. M.

    2012-01-01

    Micellar surfactant solutions are characterized by a distribution of aggregates made up predominantly of premicellar aggregates (monomers, dimers, trimers, etc.) and a region of proper micelles close to the peak aggregation number, connected by an intermediate region containing a very low concentration of aggregates. Such a distribution gives rise to a distinct two-timescale reequilibration following a system dilution, known as the t1 and t2 processes, whose dynamics may be described by the Becker-Döring equations. We use a continuum version of these equations to develop a reduced asymptotic description that elucidates the behavior during each of these processes.© 2012 Society for Industrial and Applied Mathematics.

  6. Behavior of Metal Matrix Composite Materials at Cryogenic Temperatures

    Science.gov (United States)

    1990-08-18

    au: p 0 In q V~ Lnm mam -W ama q e vam qma mam mama W to inlin La. Lu cr 0 CV)) caWn (at mam -e ON q)0 W) et t) z .’ w 0 04 cm c 0ClC) w N 0 0 LU LU...90402 10 K20 (W. Messick) 1 K22 (E. Becker) 1 Sparta, Inc. C72W (R. Johnson) 1 Attn: H. Rediess 1 23041 de la Carlota , Suite 210 Laguna Hills, CA

  7. Calculation of the self-formation driving force for composite microstructure in liquid immiscible alloy system

    Institute of Scientific and Technical Information of China (English)

    LIU Xingjun; WANG Cuiping; Ikuo OHNUMA; Ryosuke KAINUMA; Kiyohito ISHIDA; CHEN Xiaohu

    2005-01-01

    Using Becker's method, we calculate the interfacial energy between two liquid phases in an immiscible system. Based on the Gibbs-Thomson equation, the force acting on the droplet towards the thermal center can be obtained by integrating the interfacial energy between the droplet and matrix liquid phase, which is related to both the radius of a droplet and the temperature gradient. In addition, the forces of gravitation and buoyancy also act on the droplet. The calculated results indicate that the resultant for these forces together mainly decides the microstructure morphology of the solidified alloy. The calculated results are in good agreement with the corresponding experimental results.

  8. Generation of Bright Phase-matched Circularly-polarized Extreme Ultraviolet High Harmonics

    Science.gov (United States)

    2014-12-08

    relation to local moments. Phys. Scr . 1993, 302 (1993). 7. Stöhr, J. et al. Element-specific magnetic microscopy with circularly polarized X-rays...Becker, W. & Kopold, R. Generation of circularly polarized high-order harmonics by two-color coplanar field mixing . Phys. Rev. A 61, 063403 (2000). 38...1995). 42. Eichmann, H. et al. Polarization-dependent high-order two-color mixing . Phys. Rev. A 51, R3414–R3417 (1995). 43. Fleischer, A., Kfir, O

  9. Macário e La cruz del diablo: a caracterização da figura do diabo na literatura fantástica

    OpenAIRE

    Bergantini, Nathália Hernandes [UNESP

    2015-01-01

    This Masters dissertation called Macário e La cruz del diablo: the figure of the devil's characterization in fantastic literature, discourses about the way as the figure of the devil sets in two Álvares de Azevedo and Gustavo Adolfo Becker 's literary works, respectively. To study these two literay works and find similarities and differences between them, it will be study the figure of the devil, a creature widely used in literary works, especially in fantastic literay works; fantastic litera...

  10. Impacto del Capital Humano en la Distribución del Ingreso

    OpenAIRE

    2005-01-01

    El siguiente trabajo investigó el impacto del capital humano en la distribución del ingreso. El capital humano se mide a partir de su distribución y de su desviación estándar, es decir, a partir de su medida relativa y absoluta. Partiendo del modelo teórico del capital humano desarrollado por Gary Becker (1966), a medida que varía la desigualdad de la educación y su distribución, su impacto en la distribución del ingreso no será necesariamente un impacto negativo. Se enco...

  11. Creating Identity in Interaction with Business Media

    DEFF Research Database (Denmark)

    Strandgaard Pedersen, Jesper; Boutaiba, Sami

    2004-01-01

    understood. Caves (2000) notes that, `economists have studied a number of industrial sectors for their special and distinctive features', but have largely missed `the creative industries supplying goods and services that we broadly associate with cultural, artistic, or simply entertainment value' (Caves...... of the Danish film industry, as important characters in the story of Zentropa have narrated themselves as a `Maverick' (Becker, 1982) within the high-framework filmmaking and is generally recognized as a remarkable example of innovativeness in Denmark (Kultur- og Erhvervsministeriet, 2000). This paper focuses...

  12. Monitoring Species of Concern Using Noninvasive Genetic Sampling and Capture-Recapture Methods

    Science.gov (United States)

    2016-11-01

    Bennett, and A.J. Kozlowski. 2003. Characteristics of current and historic kit fox (Vulpes macrotis) dens in the Great Basin Desert. Canadian Journal of...analyses. Canadian Journal of Zoology 69:82–90. Smith, A.D. 1964. Defecation rates of mule deer. Journal of Wildlife Management 28:435–444. Stein...translocated brown bear population. Journal of Applied Ecology 47:172–181. DeMay, S.M., P.A. Becker, C.A. Eidson, J.L. Rachlow, T.R. Johnson, and L.P

  13. Determinantes de la Matrícula Universitaria: Una Aplicación de la Teoría del Capital Humano al Caso Argentino.

    OpenAIRE

    Albano, Julieta

    2005-01-01

    En este trabajo se investigan los determinantes de la tasa de matriculación universitaria utilizando como marco teórico la teoría del capital humano desarrollada por Becker y extensiones de la misma. El modelo original establece para la demanda de capital humano una relación positiva con el diferencial de salarios. Se indaga si la inserción educativa actúa como compensadora de la no inclusión laboral. Para estudiar la relación entre la tasa de matriculación universitaria y los factores determ...

  14. New records of natural enemies of Plutella xylostella (L.) (Lepidoptera: Plutellidae) in Pernambuco, Brazil.

    Science.gov (United States)

    Silva-Torres, Christian S A; Pontes, Itillio V A F; Torres, Jorge B; Barros, Reginaldo

    2010-01-01

    We report the occurrence of natural enemies of Plutella xylostela (L.) in organically farmed kale in Pernambuco, Brazil. Seven natural enemies were observed parasitizing or preying on larvae and pupae of P. xylostella--three parasitoids: Cotesia plutellae Kurdjumov (Hym.: Braconidae), Conura pseudofulvovariegata (Becker) (Hym.: Chalcididae) and Tetrastichus howardi (Olliff) (Hym.: Eulophidae), and four predators: Cheiracanthium inclusum (Hentz) (Araneae: Miturgidae), Pheidole sp.Westwood (Hym.: Formicidae), nymphs and adults of Podisus nigrispinus (Dallas) (Hem.: Pentatomidae), and one unidentified species of solitary wasp. Beyond recording these natural enemies, data on predation of P. xylostella larvae in the field and laboratory by C. inclusum are presented.

  15. Reconciliation of geometry and perception in radiation physics

    CERN Document Server

    Beckers, Benoit

    2014-01-01

    Reconciliation of Geometry and Perception in Radiation Physics approaches the topic of projective geometry as it applies to radiation physics and attempts to negate its negative reputation. With an original outlook and transversal approach, the book emphasizes common geometric properties and their potential transposition between domains. After defining both radiation and geometric properties, authors Benoit and Pierre Beckers explain the necessity of reconciling geometry and perception in fields like architectural and urban physics, which are notable for the regularity of their forms an

  16. Sensitivity and Frequencies of Dystrophin Gene Mutations in Thai DMD/BMD Patients As Detected by Multiplex PCR

    Directory of Open Access Journals (Sweden)

    Thanyachai Sura

    2008-01-01

    Full Text Available Background: Duchenne muscular dystrophy (DMD, a lethal X-linked disease affecting 1 in 3500 male births, and its more benign variant, Becker muscular dystrophy (BMD, are caused by mutations in the dystrophin gene. Because of its large size, analysing the whole gene is impractical. Methods have been developed to detect the commonest mutations i.e. the deletions of the exons. Although these tests are highly specific, their sensitivity is inherently limited by the prevalence of deletions, which differs among different populations.

  17. Human muscle proteins: analysis by two-dimensional electrophoresis

    Energy Technology Data Exchange (ETDEWEB)

    Giometti, C.S.; Danon, M.J.; Anderson, N.G.

    1983-09-01

    Proteins from single frozen sections of human muscle were separated by two-dimensional gel electrophoresis and detected by fluorography or Coomassie Blue staining. The major proteins were identical in different normal muscles obtained from either sex at different ages, and in Duchenne and myotonic dystrophy samples. Congenital myopathy denervation atrophy, polymyositis, and Becker's muscular dystrophy samples, however, showed abnormal myosin light chain compositions, some with a decrease of fast-fiber myosin light chains and others with a decrease of slow-fiber light chains. These protein alterations did not correlate with any specific disease, and may be cause by generalized muscle-fiber damage.

  18. DMD and BMD in the same family due to two distinct mutations

    Energy Technology Data Exchange (ETDEWEB)

    Morandi, L.; Mora, M.; Di Blasi, C.; Brugnoni, R. [National Inst. C. Besta, Milan (Italy)] [and others

    1995-12-04

    We report on a family with a boy affected by Duchenne muscular dystrophy (DMD) and an asymptomatic cousin with a Becker-type dystrophin abnormality, diagnosed by chance. Dystrophin gene analysis showed that these conditions were caused by two distinct deletions with breakpoints in different exons. In Xp21 families, DNA analysis and dystrophin testing of asymptomatic males with high CK plasma levels might detect different dystrophin mutations in separate haplotypes as in our family, although we stress there should be clear clinical or familial indications for such testing. 24 refs., 5 figs.

  19. Breast Cancer Gene Therapy: Development of Novel Non-Invasive Magnetic Resonance Assay to Optimize Efficacy

    Science.gov (United States)

    2005-05-01

    by depth of light penetration. Fluorescent stntai for bl ica n hogical analyi [8imainghasbee prs ned n atomgrahicforatbutto Representative agents...1653. Med. 1998, 40, 1. [316] Hull, W.E.; Port, R.E.; Herrmann , R.; Britsch, B.; Kunz, W. [353] Daugherty, A.; Becker, N.N.; Scherrer, L.A.; Sobel...Kodibagkar, V.; Cui, W.; Mason, R.P. Curr. Med. Chem. 2005,12,818 16 Sinnott, M.L. Chem. Rev 1990,90,1171 17 Guder, H.J.; Herrmann , R.Z.; Zdunek, D., in

  20. Pavlovian Processes in Consumer Choice: The Physical Presence of a Good Increases Willingness-to-Pay

    OpenAIRE

    2010-01-01

    This paper describes a series of laboratory experiments studying whether the form in which items are displayed at the time of decision affects the dollar value that subjects place on them. Using a Becker-DeGroot auction under three different conditions — (i) text displays, (ii) image displays, and (iii) displays of the actual items — we find that subjects' willingness-to-pay is 40-61 percent larger in the real than in the image and text displays. Furthermore, follow-up experiments suggest the...

  1. Solid State Research: 1982-1.

    Science.gov (United States)

    1982-02-15

    Digital Converter 7 C. A Novel Technique for GaInAsP/InP Buried Heterostructure Laser Fabrication 11 II. QUANTUM ELECTRONICS 19 A. Co:MgF2 Laser: High...for GaInAsP/ Z.L. Lieu Accepted by Appi. Phys. InP Buried Heterostructure I.N. Walpole Lett. Laser Fabrication 5320 Observation of Linewidth D. Welford...F.J. Leonberger C.E. Woodward R.A. Becker 10 C. A NOVEL TECHNIQUE FOR GaInAsP/InP BURIED HETEROSTRUCTURE LASER FABRICATION A novel technique for the

  2. Patologização e penalização do uso de drogas: uma análise socioantropológica de proposições legislativas (2007-2010)

    OpenAIRE

    Silva,Martinho Braga e; Maria Célia DELDUQUE

    2015-01-01

    Com base nas contribuições de N. Elias e H. Becker, buscamos identificar os processos de patologização e penalização do uso de drogas presentes em proposições legislativas apresentadas ao Congresso Nacional durante a 53ª Legislatura. As 147 proposições reunidas através do descritor "álcool, tabaco e outras drogas" no banco de dados do Observatório da Saúde no Legislativo também dizem respeito a diferentes temas, como "alimentação e nutrição" e "propaganda". No inteiro teor de alguns projetos ...

  3. La educación y su efecto en la formación de capiltal humano y en el desarrollo económico de los países

    OpenAIRE

    Andrea Briceño Mosquera

    2011-01-01

    El efecto de la educación en la formación de capital humano y en el desarrollo económico de los países, ha sido analizado en diversos estudios teóricos y empíricos. Los enfoques clásico de Smith y neoclásico de Solow, la teoría de capital humano planteada por Danison, Schultz y Becker, y el enfoque de desarrollo y libertadde Sen entre otros, hananalizado dicho efecto, evidenciando que la educación es uno de los factores determinantes de una mayor productividad, la cual se ve reflejada en la g...

  4. Temporal evolution of mottles observed in H_alpha

    CERN Document Server

    Bostanci, Z F; Erdogan, Nurol Al

    2007-01-01

    In April 2002, H_alpha observations of the solar chromosphere with high spatial and spectral resolution were obtained with the Gottingen Fabry-Perot Spectrometer mounted in the Vacuum Tower Telescope (VTT) at the Observatorio del Teide/Tenerife. In this work, we analyze a short time sequence of a quiet region with chains of mottles. Some physical parameters of dark mottles are determined by using Beckers' cloud model which takes the source function, the Doppler width, and the velocity to be constant within the cloud along the line of sight. Here, we present the results of our study.

  5. The paradox of muscle hypertrophy in muscular dystrophy.

    Science.gov (United States)

    Kornegay, Joe N; Childers, Martin K; Bogan, Daniel J; Bogan, Janet R; Nghiem, Peter; Wang, Jiahui; Fan, Zheng; Howard, James F; Schatzberg, Scott J; Dow, Jennifer L; Grange, Robert W; Styner, Martin A; Hoffman, Eric P; Wagner, Kathryn R

    2012-02-01

    Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophy in humans and syndromes in mice, dogs, and cats. Affected humans and dogs have progressive disease that leads primarily to muscle atrophy. Mdx mice progress through an initial phase of muscle hypertrophy followed by atrophy. Cats have persistent muscle hypertrophy. Hypertrophy in humans has been attributed to deposition of fat and connective tissue (pseudohypertrophy). Increased muscle mass (true hypertrophy) has been documented in animal models. Muscle hypertrophy can exaggerate postural instability and joint contractures. Deleterious consequences of muscle hypertrophy should be considered when developing treatments for muscular dystrophy.

  6. Stochastic analysis of nucleation rates

    Science.gov (United States)

    Johansson, Jonas

    2016-02-01

    We show that approximating the Becker-Döring equations with a Langevin equation results in multiplicative noise, which in turn leads to a family of possible Fokker-Planck equations according to the Ito-Stratonovich dilemma. Using a simple and general model for the attachment and detachment rates, we find that the Ito choice approximates the nucleation rate best and also coincides with the Fokker-Planck equation resulting from the common way to Taylor expand the original set of rate equations.

  7. 以运动后肌痛为主要表现的Becker型肌营养不良症2例报告

    Institute of Scientific and Technical Information of China (English)

    王锁彬; 沈定国; 魏东宁

    2004-01-01

    假肥大型肌营养不良症主要表现为进行性加重的肌肉萎缩与无力。根据临床症状严重程度及疾病进展速度可分为Becker型(Becker muscular dystrophy,BMD)和Duchenne型(Duchenne muscular dystrophy,DMD)。肌肉无力是BMD最显著的临床特征,以活动后肌痛为主要症状者罕见。现报告2例如下。

  8. Unified description of low-order above-threshold ionization on and off axis

    Science.gov (United States)

    Becker, W.; Milošević, D. B.

    2016-02-01

    A recently developed unified description of low-order above-threshold ionization (Becker et al 2014 J. Phys. B: At. Mol. Opt. Phys. 47 204022; 2015 J. Phys. B: At. Mol. Opt. Phys. 48 151001) is revisited and extended. By considering the rescattering electron energies and angles at the classical cutoffs and the contributions of particular quantum-orbit solutions, it is shown that summing both the backward- and the forward-scattering contributions, within the low-frequency approximation, it is possible to reproduce the observed features of the ATI spectra both for low and high energies and both on and off the laser-polarization axis in the momentum plane.

  9. A case of Becker’ s nevus syndrome%Becker痣综合征1例

    Institute of Scientific and Technical Information of China (English)

    张倩; 朱文元; 骆丹

    2014-01-01

    患者,女,22岁。右肩胛及胸部淡褐色不规则色素沉着斑伴右侧乳房发育不全10余年。胸部X线结果示:脊柱下胸段稍侧弯。诊断:Becker痣综合征。%A 22 -year-old female presented with irregular pigmentation spot on the right shoulder and chest with hypoplasia of the right breast for 10 years. Chest X-ray showed the lower thoracic spine appears scoliosis slightly. The diagnosis of Becker's nevus syndrome was made.

  10. M-theory and G2 manifolds

    Science.gov (United States)

    Becker, Katrin; Becker, Melanie; Robbins, Daniel

    2015-11-01

    In this talk we report on recent progress in describing compactifications of string theory and M-theory on G2 and Spin(7) manifolds. We include the infinite set of α’-corrections and describe the entire tower of massless and massive Kaluza-Klein modes resulting from such compactifications. Contribution to the ‘Focus Issue on Gravity, Supergravity and Fundamental Physics: the Richard Arnowitt Symposium’, to be published in Physica Scripta. Based on a talk delivered by Becker at the workshop ‘Superstring Perturbation Theory’ at the Perimeter Institute, 22-24 April 2015.

  11. THE DAY ON WHICH THE PARLIAMENT SANG: AESTHETIC EXPERIENCE POSSIBILITIES

    Directory of Open Access Journals (Sweden)

    Otacílio Vaz

    2013-11-01

    Full Text Available On August 23 2009, there was a public hearing to address issues related to Carioca funk at the Legislative Assembly of Rio de Janeiro. Composers, musicians, dancers and other professionals in the style, mostly people from the outskirts of the city, were present there to fight for better working conditions and better recognition of this cultural movement. This paper analyzes from the point of view of the theorist Hans Ulrich Gumbrecht on the aesthetic experiences lived on that day. To assist this research, authors such as Howard Becker, Michael Herschmann, Norbert Elias, Adriana Carvalho Lopes and others, have been summoned here.

  12. Tratamiento con oligonucleótidos antisentido en la enfermedad de Duchenne

    OpenAIRE

    Pascual Pascual, Samuel Ignacio

    2012-01-01

    Se revisa el estado actual de los tratamientos de modificación del ARN de distrofina, que persiguen convertir la forma grave de la distrofia muscular de Duchenne a una forma leve (Becker), especialmente con la omisión del exón 51 mediante oligonucleótidos en antisentido y la terapia de restauración de la lectura de la distrofina, suprimiendo un codón de terminación prematura mediante PTC124. Constituyen un avance notable en el tratamiento. Consiguen una producción significativa de...

  13. Self consistent modeling of accretion columns in accretion powered pulsars

    Science.gov (United States)

    Falkner, Sebastian; Schwarm, Fritz-Walter; Wolff, Michael Thomas; Becker, Peter A.; Wilms, Joern

    2016-04-01

    We combine three physical models to self-consistently derive the observed flux and pulse profiles of neutron stars' accretion columns. From the thermal and bulk Comptonization model by Becker & Wolff (2006) we obtain seed photon continua produced in the dense inner regions of the accretion column. In a thin outer layer these seed continua are imprinted with cyclotron resonant scattering features calculated using Monte Carlo simulations. The observed phase and energy dependent flux corresponding to these emission profiles is then calculated, taking relativistic light bending into account. We present simulated pulse profiles and the predicted dependency of the observable X-ray spectrum as a function of pulse phase.

  14. E RENDIMENTOS DO TRABALHO NO BRASIL: DESAFIOS DE UM CENÂRIO DE TRANSFORMAÇOES

    OpenAIRE

    Saldanha Pereira, Rosangela; Zavala Zavala,Arturo

    2012-01-01

    Este artículo aborda las principales contribuciones teóricas de Schultz, Becker y Mincer referentes a los efectos de la educación en los salarios y la discriminación y segmentación en el mercado del trabajo brasileño, en el período comprendido entre 1990 y 2009. Para cuantificar el retorno de la educación utilizamos la ecuación de Mincer adaptada para captar factores de la discriminación y segmentación. El método utilizado es el de Mínimos Cuadrados Ordinarios (MQO). Las estimacionesse hicier...

  15. Estudos da associação micorrízica arbuscular em mudas de mangabeira (Hancornia speciosa Gomes)

    OpenAIRE

    Maria Carneiro Costa, Cynthia

    2004-01-01

    A associação micorrízica em mangabeira (Hancornia speciosa Gomes) foi estudada em cinco experimentos utilizando isolados de Gigaspora albida Schenck & Smith e Glomus etunicatum. Becker & Gerd. Em casa de vegetação foram avaliados: dependência micorrízica, densidade de inóculo necessária para promover o crescimento de mudas e influência do estresse hídrico nas trocas gasosas, na concentração de solutos e na atividade microbiana do solo; em campo foram determinados: espécies, número...

  16. Boys in white: a classic of qualitative research turns 50.

    Science.gov (United States)

    Nunes, Everardo Duarte; de Barros, Nelson Filice

    2014-01-01

    This article analyzes Boys in white: student culture in medical schoolby Howard S. Becker, Blanche Geer, Everett C. Hughes and Anselm Strauss, considered a model of qualitative research in sociology. The analysis investigates the trajectories of the authors, the book, qualitative analysis, and the medical students, emphasizing their importance in the origins of medical sociology and the sociology of medical education. In the trajectory of the authors, bibliographical information is given. The trajectory of qualitative research focuses on how this methodology influences the construction of the field. The investigation of the students' trajectory shows how they progress through their first years at medical school to build their own student culture.

  17. Emile Zola, La Fabrique des Rougon-Macquart

    OpenAIRE

    Raffi, Maria Emanuela

    2016-01-01

    Colette Becker continua l’impegnativa pubblicazione dei «Dossiers préparatoires» di Zola con questo V volume dedicato interamente a Germinal (NAF 10307 e 10308). Nel saggio introduttivo De la note à la fiction, l’A. prende avvio da una riflessione sull’enorme ampiezza del progetto zoliano, concepito in origine come «une étude exhaustive du réel», che implica una concezione ‘documentaristica’ del romanzo, fondato, nel caso di Zola, su una mole impressionante di note, cui si aggiungono letture,...

  18. Influencia multifactorial en el desarrollo de contractura capsular, rotura protésica y complicaciones menores de las prótesis-expansoras en la reconstrucción de mama

    OpenAIRE

    2013-01-01

    Estudiamos de forma retrospectiva la evolución de 303 implantes mamarios tipo Becker en 231 pacientes reconstruídas en el Servicio de Cirugía Plástica del C.H.U.S. Se estudiaron posibles variables que pudieran influir en la presencia de complicaciones (contractura capsular, rotura protésica y complicaciones menores) y también en la supervivencia de estos implantes a largo plazo. De todas las variables de estudio, la radioterapia actuó como variable independiente aumentando el índice de com...

  19. Diagnóstico da cardiomiopatia na distrofia muscular progressiva por ressonância magnética cardiovascular - correlação com tratamento, prognóstico e preditores genéticos

    OpenAIRE

    Marly Conceição Silva

    2013-01-01

    Introdução: Distrofia muscular progressiva nas formas de Duchenne (DMD) e Becker (DMB) são doenças caracterizadas por progressiva degeneração musculoesquelética e substituição por tecido fibrogorduroso. O envolvimento cardíaco está presente em 80% dos pacientes, apresenta curso clínico silencioso e é diagnosticado tardiamente pelos métodos tradicionais. Objetivos: 1. Investigar a progressão da fibrose miocárdica pela ressonância magnética cardíaca (RMC), em ensaio clínico randomizado para tra...

  20. An assessment of the role of the k-e vertical mixing scheme in the simulation of Southern Ocean upper dynamics

    CSIR Research Space (South Africa)

    Boodhraj, K

    2016-11-01

    Full Text Available (ECMWF) ERA-interim 89 reanalysis (Dee et al., 2011). 90 2 Reffray et al. (2015) calculated the bias for the 91 temperature by subtracting the calculated data 92 from the observed data. All plots, for the North 93 Pacific and SO, were plotted... modelisation de l'Institut Pierre-Simon 413 Laplace No 27. 414 Cushman-Rosin, B. and Beckers, J.-M. 415 (2009). Introduction to Geophysical Fluid 416 Dynamics: Physical and Numerical 417 Aspects, Academic Press. 418 Versteeg, H. and Malalasekra, W...