The natural history of Becker expandable breast implants: a single-center 10-year experience.

PubMed

Sindali, Katia; Davis, Marcus; Mughal, Maleeha; Orkar, Kusu S

2013-09-01

Use of Becker expandable breast implants in single-stage breast surgery is a well-established technique; however, replacement with fixed-volume implants is common. The authors sought to analyze the long-term natural history of these implants over a wide range of surgical indications. A retrospective review of 330 consecutive patients who underwent 384 Becker expander breast reconstructions over a 10-year period in a dedicated plastic surgery unit was undertaken. Implant indication, Becker type, volume and site, complications, expander lifespan, and explant reasons were assessed. Two hundred twenty-eight patients (267 implants) and 102 patients (117 implants) underwent implantation for congenital deformities and breast cancer reconstruction, respectively. One hundred eighty-seven (48 percent) were explanted at a median period of 13.0 months (range, 9.0 to 26.0 months), 149 (39 percent) for aesthetic reasons and 38 (10 percent) for complications. Complication rates were higher in breast cancer reconstruction compared with congenital patients (19.6 percent versus 7.9 percent; p = 0.002), driven by an increased rate of wound complications (13.7 percent versus 4.4 percent; p = 0.003). Cancer-related surgery and advancing age were the only predictors of complication risk. The overall Becker expander retention rate was 24.9 percent and 46.8 percent at 150 months in the cancer reconstruction and congenital groups, respectively. Forty-seven percent of Becker implants were retained long term after congenital corrective surgery; only 25 percent were retained after postmastectomy reconstruction. Poor aesthetics was driving the exchange for fixed-volume implants, indicating that after breast cancer reconstruction, Becker expanders were being used as part of a two-stage reconstructive strategy.

Genetics and emerging treatments for Duchenne and Becker muscular dystrophy.

PubMed

Wein, Nicolas; Alfano, Lindsay; Flanigan, Kevin M

2015-06-01

Mutations in the DMD gene result in Duchenne or Becker muscular dystrophy due to absent or altered expression of the dystrophin protein. The more severe Duchenne muscular dystrophy typically presents around ages 2 to 5 with gait disturbance, and historically has led to the loss of ambulation by age 12. It is important for the practicing pediatrician, however, to be aware of other presenting signs, such as delayed motor or cognitive milestones, or elevated serum transaminases. Becker muscular dystrophy is milder, often presenting after age 5, with ambulation frequently preserved past 20 years and sometimes into late decades. Copyright © 2015 Elsevier Inc. All rights reserved.

A generalization of the Becker model in linear viscoelasticity: creep, relaxation and internal friction

NASA Astrophysics Data System (ADS)

Mainardi, Francesco; Masina, Enrico; Spada, Giorgio

2018-02-01

We present a new rheological model depending on a real parameter ν \\in [0,1], which reduces to the Maxwell body for ν =0 and to the Becker body for ν =1. The corresponding creep law is expressed in an integral form in which the exponential function of the Becker model is replaced and generalized by a Mittag-Leffler function of order ν . Then the corresponding non-dimensional creep function and its rate are studied as functions of time for different values of ν in order to visualize the transition from the classical Maxwell body to the Becker body. Based on the hereditary theory of linear viscoelasticity, we also approximate the relaxation function by solving numerically a Volterra integral equation of the second kind. In turn, the relaxation function is shown versus time for different values of ν to visualize again the transition from the classical Maxwell body to the Becker body. Furthermore, we provide a full characterization of the new model by computing, in addition to the creep and relaxation functions, the so-called specific dissipation Q^{-1} as a function of frequency, which is of particular relevance for geophysical applications.

Obituary: Peter Robert Wilson, 1929-2007

NASA Astrophysics Data System (ADS)

Snodgrass, Herschel B.

2009-01-01

It is with great sadness that I report the passing of Peter Robert Wilson, a well-known and well-loved figure in the solar physics community. Peter was on the faculty of the Department of Applied Mathematics at the University of Sydney for 39 years, and Chair of the department for 24 of these years. He was the author or co-author of more than 80 scientific research papers and a book, Solar and Stellar Activity Cycles (1994), published by Cambridge University Press. He died suddenly of a heart attack, at his home in Glebe, Australia, in the early morning of 11 November 2007. Peter was an organizer of, and participant in, many international conferences and workshops. He traveled extensively, holding visiting appointments at the University of Colorado (JILA), at Cambridge University, at the College de France (Paris), and at the California Institute of Technology [CalTech]. Most of his work was in the field of solar physics, but he also did some work on the philosophy of science and on tides. Peter came from a line of mathematicians. His father, Robert Wilson, immigrated to Australia from Glasgow in 1911, and became a mathematics teacher at Scotch College, a private school in Melbourne. There his name was changed to 'Bill' because 'Bob' was already taken." Peter's enjoyment of this story as characteristic of Australian academia (as any fan of Monty Python would understand) is indicative of his infectious sense of humor. In a similar vein, he claimed ancestry traced back to the eighteenth-century Scottish mathematician Alexander Wilson, Professor of Astronomy at the University of Glasgow. That Wilson is famous in the solar physics community for his discovery, known as the "Wilson Effect," of the photospheric depressions associated with sunspots. Peter himself could not resist writing a paper on this subject, and was delighted when the bait was taken by some less-informed colleagues who chided him for "naming an effect after himself." "Bill" Wilson married Naomi

Reflections on the Gall-Peters Projection.

ERIC Educational Resources Information Center

Robinson, Arthur H.

1987-01-01

Explains the cartographic qualities of rectangular world maps and compares the merits of various projections such as the Mercator and the recently-created Gall-Peters. States that the Gall-Peters projection does not provide a reasonable base for a general world map; that no rectangular projection does. (JDH)

Whole exome sequence analysis of Peters anomaly

PubMed Central

Weh, Eric; Reis, Linda M.; Happ, Hannah C.; Levin, Alex V.; Wheeler, Patricia G.; David, Karen L.; Carney, Erin; Angle, Brad; Hauser, Natalie

2015-01-01

Peters anomaly is a rare form of anterior segment ocular dysgenesis, which can also be associated with additional systemic defects. At this time, the majority of cases of Peters anomaly lack a genetic diagnosis. We performed whole exome sequencing of 27 patients with syndromic or isolated Peters anomaly to search for pathogenic mutations in currently known ocular genes. Among the eight previously recognized Peters anomaly genes, we identified a de novo missense mutation in PAX6, c.155G>A, p.(Cys52Tyr), in one patient. Analysis of 691 additional genes currently associated with a different ocular phenotype identified a heterozygous splicing mutation c.1025+2T>A in TFAP2A, a de novo heterozygous nonsense mutation c.715C>T, p.(Gln239*) in HCCS, a hemizygous mutation c.385G>A, p.(Glu129Lys) in NDP, a hemizygous mutation c.3446C>T, p.(Pro1149Leu) in FLNA, and compound heterozygous mutations c.1422T>A, p.(Tyr474*) and c.2544G>A, p.(Met848Ile) in SLC4A11; all mutations, except for the FLNA and SLC4A11 c.2544G>A alleles, are novel. This is the frst study to use whole exome sequencing to discern the genetic etiology of a large cohort of patients with syndromic or isolated Peters anomaly. We report five new genes associated with this condition and suggest screening of TFAP2A and FLNA in patients with Peters anomaly and relevant syndromic features and HCCS, NDP and SLC4A11 in patients with isolated Peters anomaly. PMID:25182519

Genetics Home Reference: Duchenne and Becker muscular dystrophy

MedlinePlus

... Citation on PubMed Mah JK, Korngut L, Dykeman J, Day L, Pringsheim T, Jette N. A systematic review and meta-analysis on the epidemiology of Duchenne and Becker muscular dystrophy. Neuromuscul Disord. 2014 Jun;24(6):482-91. doi: 10.1016/j.nmd.2014.03.008. Epub 2014 Mar 22. ...

The Travails of Criticality: Understanding Peter McLaren's Revolutionary Vocation. An Article Review of Peter McLaren, "Pedagogy of Insurrection" (New York: Peter Lang, 2015)

ERIC Educational Resources Information Center

Baldacchino, John

2017-01-01

This is an article review of Peter McLaren's "Pedagogy of Insurrection" (New York: Peter Lang, 2015). While it seeks to position McLaren's work within the context of critical pedagogy, this paper also assesses McLaren from the wider discussion of Marxist--Hegelian discourse as it evolved within the Left. Engaging with McLaren critically,…

Theories of Power, Poverty, and Law: In Commemoration of the Contributions of Peter Bachrach--Power, Law, and Final Thoughts: The Contributions of Peter Bachrach

ERIC Educational Resources Information Center

Schneider, Elizabeth M.

2010-01-01

I am pleased to be part of this symposium to celebrate the life and work of Peter Bachrach. Although my focus is the relevance of Peter's ideas of power to law, I want to begin with some personal comments as well as raise some final thoughts, drawing on others' contributions. Like so many of Peter's other students, I adored him. Peter's joy in…

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

PubMed

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. Copyright © 2015 Elsevier B.V. All rights reserved.

Peter F. Green - Deputy Laboratory Director, Science and Technology |

Science.gov Websites

NREL Peter F. Green - Deputy Laboratory Director, Science and Technology Peter F. Green - Deputy Laboratory Director, Science and Technology A photo of Peter Green. Green came to NREL in August than 250 collaborative publications and 20 patent disclosures. Green also served as the B.F. Goodrich

Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials.

PubMed

Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S; Verschuuren, Jan J; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E; Muntoni, Francesco

2011-12-01

Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative study on both

Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials

PubMed Central

Anthony, Karen; Cirak, Sebahattin; Torelli, Silvia; Tasca, Giorgio; Feng, Lucy; Arechavala-Gomeza, Virginia; Armaroli, Annarita; Guglieri, Michela; Straathof, Chiara S.; Verschuuren, Jan J.; Aartsma-Rus, Annemieke; Helderman-van den Enden, Paula; Bushby, Katherine; Straub, Volker; Sewry, Caroline; Ferlini, Alessandra; Ricci, Enzo; Morgan, Jennifer E.

2011-01-01

Duchenne muscular dystrophy is caused by mutations in the DMD gene that disrupt the open reading frame and prevent the full translation of its protein product, dystrophin. Restoration of the open reading frame and dystrophin production can be achieved by exon skipping using antisense oligonucleotides targeted to splicing elements. This approach aims to transform the Duchenne muscular dystrophy phenotype to that of the milder disorder, Becker muscular dystrophy, typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin. There is ongoing debate regarding the functional properties of the different internally deleted dystrophins produced by exon skipping for different mutations; more insight would be valuable to improve and better predict the outcome of exon skipping clinical trials. To this end, we have characterized the clinical phenotype of 17 patients with Becker muscular dystrophy harbouring in-frame deletions relevant to on-going or planned exon skipping clinical trials for Duchenne muscular dystrophy and correlated it to the levels of dystrophin, and dystrophin-associated protein expression. The cohort of 17 patients, selected exclusively on the basis of their genotype, included 4 asymptomatic, 12 mild and 1 severe patient. All patients had dystrophin levels of >40% of control and significantly higher dystrophin (P = 0.013), β-dystroglycan (P = 0.025) and neuronal nitric oxide synthase (P = 0.034) expression was observed in asymptomatic individuals versus symptomatic patients with Becker muscular dystrophy. Furthermore, grouping the patients by deletion, patients with Becker muscular dystrophy with deletions with an end-point of exon 51 (the skipping of which could rescue the largest group of Duchenne muscular dystrophy deletions) showed significantly higher dystrophin levels (P = 0.034) than those with deletions ending with exon 53. This is the first quantitative

Usefulness of sugammadex in a patient with Becker muscular dystrophy and dilated cardiomyopathy.

PubMed

Shimauchi, Tsukasa; Yamaura, Ken; Sugibe, Sayaka; Hoka, Sumio

2014-09-01

A 54-year-old patient with Becker muscular dystrophy and dilated cardiomyopathy underwent laparoscopic cholecystectomy under total intravenous anesthesia. Muscle relaxation was induced by rocuronium (0.4 mg/kg body weight) under train-of-four (TOF) ratio monitoring. The TOF ratio was 0 at intubation, and 0.2 at the end of surgery. Residual muscle relaxant activity was successfully reversed by sugammadex (2 mg/kg body weight) without any hemodynamic adverse effects (TOF ratio 1.0 at extubation). The clinical and hemodynamic findings suggest that sugammadex can be safely used in patients with Becker muscular dystrophy and dilated cardiomyopathy. Copyright © 2014. Published by Elsevier B.V.

Pharmacological rescue of the dystrophin-glycoprotein complex in Duchenne and Becker skeletal muscle explants by proteasome inhibitor treatment.

PubMed

Assereto, Stefania; Stringara, Silvia; Sotgia, Federica; Bonuccelli, Gloria; Broccolini, Aldobrando; Pedemonte, Marina; Traverso, Monica; Biancheri, Roberta; Zara, Federico; Bruno, Claudio; Lisanti, Michael P; Minetti, Carlo

2006-02-01

In this report, we have developed a novel method to identify compounds that rescue the dystrophin-glycoprotein complex (DGC) in patients with Duchenne or Becker muscular dystrophy. Briefly, freshly isolated skeletal muscle biopsies (termed skeletal muscle explants) from patients with Duchenne or Becker muscular dystrophy were maintained under defined cell culture conditions for a 24-h period in the absence or presence of a specific candidate compound. Using this approach, we have demonstrated that treatment with a well-characterized proteasome inhibitor, MG-132, is sufficient to rescue the expression of dystrophin, beta-dystroglycan, and alpha-sarcoglycan in skeletal muscle explants from patients with Duchenne or Becker muscular dystrophy. These data are consistent with our previous findings regarding systemic treatment with MG-132 in a dystrophin-deficient mdx mouse model (Bonuccelli G, Sotgia F, Schubert W, Park D, Frank PG, Woodman SE, Insabato L, Cammer M, Minetti C, and Lisanti MP. Am J Pathol 163: 1663-1675, 2003). Our present results may have important new implications for the possible pharmacological treatment of Duchenne or Becker muscular dystrophy in humans.

Secondary Conditions Among Males With Duchenne or Becker Muscular Dystrophy.

PubMed

Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

2017-06-01

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred nine caregivers of affected males (aged 3-31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The 5 most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones were found among nonambulatory males compared to ambulatory males. Attention-deficit hyperactivity disorder (ADHD) was more common in ambulatory than nonambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis.

Secondary conditions among males with Duchenne or Becker muscular dystrophy

PubMed Central

Latimer, Rebecca; Street, Natalie; Conway, Kristin Caspers; James, Kathy; Cunniff, Christopher; Oleszek, Joyce; Fox, Deborah; Ciafaloni, Emma; Westfield, Christina; Paramsothy, Pangaja

2017-01-01

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred and nine caregivers of affected males (aged 3–31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The five most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones, were found among non-ambulatory males compared to ambulatory males. Attention deficit hyperactivity disorder was more common in ambulatory than non-ambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis. PMID:28393671

Late-onset Becker-type muscular dystrophy in a Border terrier dog.

PubMed

Jeandel, A; Garosi, L S; Davies, L; Guo, L T; Salgüero, R; Shelton, G D

2018-01-29

A 9-year-old Border terrier was presented to a referral hospital after a 1-year history of progressive stiffness and exercise intolerance. Neurological examination was consistent with a neuromuscular disorder. Serum creatine kinase activity was mildly elevated. A myopathy was suspected based on MRI findings and electrophysiological examination. Muscle histopathology was consistent with a severe non-inflammatory myopathy of a dystrophic type. Immunofluorescence and western blotting confirmed a dystrophinopathy with an 80-kDa truncated dystrophin fragment similar to Becker muscular dystrophy in people. To our knowledge, this is the first description of a late-onset Becker-type muscular dystrophy in a dog, and the first description of a dystrophinopathy in a Border terrier. Muscular dystrophy in dogs should not be ruled out based on late onset clinical signs and only mildly elevated creatine kinase. © 2018 British Small Animal Veterinary Association.

Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): case definition in surveillance for childhood-onset Duchenne/Becker muscular dystrophy.

PubMed

Mathews, Katherine D; Cunniff, Chris; Kantamneni, Jiji R; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F John; Sladky, John; Romitti, Paul A

2010-09-01

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in 1 of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by >4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either ''definite'' or ''probable'' Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982-1987) to 94% in the cohort born 2004 to 2009.

Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet): Case Definition in Surveillance for Childhood-Onset Duchenne/Becker Muscular Dystrophy

PubMed Central

Mathews, Katherine D.; Cunniff, Chris; Kantamneni, Jiji R.; Ciafaloni, Emma; Miller, Timothy; Matthews, Dennis; Cwik, Valerie; Druschel, Charlotte; Miller, Lisa; Meaney, F. John; Sladky, John; Romitti, Paul A.

2013-01-01

The Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) is a multisite collaboration to determine the prevalence of childhood-onset Duchenne/Becker muscular dystrophy and to characterize health care and health outcomes in this population. MD STARnet uses medical record abstraction to identify patients with Duchenne/Becker muscular dystrophy born January 1, 1982 or later who resided in one of the participating sites. Critical diagnostic elements of each abstracted record are reviewed independently by ≥4 clinicians and assigned to 1 of 6 case definition categories (definite, probable, possible, asymptomatic, female, not Duchenne/Becker muscular dystrophy) by consensus. As of November 2009, 815 potential cases were reviewed. Of the cases included in analysis, 674 (82%) were either “definite” or “probable” Duchenne/Becker muscular dystrophy. These data reflect a change in diagnostic testing, as case assignment based on genetic testing increased from 67% in the oldest cohort (born 1982–1987) to 94% in the cohort born 2004–2009. PMID:20817884

SIBLING CONCORDANCE FOR CLINICAL FEATURES OF DUCHENNE AND BECKER MUSCULAR DYSTROPHIES

PubMed Central

PETTYGROVE, SYDNEY; LU, ZHENQIANG; ANDREWS, JENNIFER G.; MEANEY, F. JOHN; SHEEHAN, DANIEL W.; PRICE, ELINORA T.; FOX, DEBORAH J.; PANDYA, SHREE; OUYANG, LIJING; APKON, SUSAN D.; POWIS, ZOE; CUNNIFF, CHRISTOPHER

2015-01-01

Introduction The correlation of markers of disease severity among brothers with Duchenne or Becker muscular dystrophy has implications for clinical guidance and clinical trials. Methods Sibling pairs with Duchenne or Becker muscular dystrophy (n = 60) were compared for ages when they reached clinical milestones of disease progression, including ceased ambulation, scoliosis of ≥ 20°, and development of cardiomyopathy. Results The median age at which younger brothers reached each milestone, compared with their older brothers ranged from 25 months younger for development of cardiomyopathy to 2 months older for ceased ambulation. For each additional month of ambulation by the older brother, the hazard of ceased ambulation by the younger brother decreased by 4%. Conclusions The ages when siblings reach clinical milestones of disease vary widely between siblings. However, the time to ceased ambulation for older brothers predicts the time to ceased ambulation for their younger brothers. PMID:24030636

Ritual, Imitation and Education in R. S. Peters

ERIC Educational Resources Information Center

Warnick, Bryan R.

2009-01-01

This article reconstructs R. S. Peters' underlying theory of ritual in education, highlighting his proposed link between ritual and the imitation of teachers. Rituals set the stage for the imitation of teachers and they invite students to experience practices whose value is not easily discernable from the outside. For Peters, rituals facilitate…

Dystrophin insufficiency causes a Becker muscular dystrophy-like phenotype in swine

USDA-ARS?s Scientific Manuscript database

Duchenne muscular dystrophy (DMD) is caused by a dystrophin deficiency while Becker MD is caused by a dystrophin insufficiency or expression of a partially functional dystrophin protein. Deficiencies in existing mouse and dog models necessitate the development of a novel large animal model. Our pu...

Farewell to the Chairman - Marine Gen. Peter Pace

Science.gov Websites

oath of office from Marine Gen. Peter Pace, who retired after more than 40 years active duty military wife Lynne after giving the general his retirement certificate. Defense Dept. photo by Cherie A solutions, Marine Gen. Peter Pace said here today as he retired from the Marine Corps after more than 40

Genetic diagnosis of Duchenne and Becker muscular dystrophy using multiplex ligation-dependent probe amplification in Rwandan patients.

PubMed

Uwineza, Annette; Hitayezu, Janvier; Murorunkwere, Seraphine; Ndinkabandi, Janvier; Kalala Malu, Celestin Kaputu; Caberg, Jean Hubert; Dideberg, Vinciane; Bours, Vincent; Mutesa, Leon

2014-04-01

Duchenne and Becker muscular dystrophies are the most common clinical forms of muscular dystrophies. They are genetically X-linked diseases caused by a mutation in the dystrophin (DMD) gene. A genetic diagnosis was carried out in six Rwandan patients presenting a phenotype of Duchenne and Becker muscular dystrophies and six asymptomatic female carrier relatives using multiplex ligation-dependent probe amplification (MLPA). Our results revealed deletion of the exons 48-51 in one patient, an inherited deletion of the exons 8-21 in two brothers and a de novo deletion of the exons 46-50 in the fourth patient. No copy number variation was found in two patients. Only one female carrier presented exon deletion in the DMD gene. This is the first cohort of genetic analysis in Rwandan patients affected by Duchenne and Becker muscular dystrophies. This report confirmed that MLPA assay can be easily implemented in low-income countries.

Brain natriuretic peptide is not predictive of dilated cardiomyopathy in Becker and Duchenne muscular dystrophy patients and carriers.

PubMed

Schade van Westrum, Steven; Dekker, Lukas; de Haan, Rob; Endert, Erik; Ginjaar, Ieke; de Visser, Marianne; van der Kooi, Anneke

2013-07-16

Cardiomyopathy is reported in Duchenne and Becker muscle dystrophy patients and female carriers. Brain Natriuretic peptide (BNP) is a hormone produced mainly by ventricular cardiomyocytes and its production is up regulated in reaction to increased wall stretching. N-terminal-proBNP (NT-proBNP) has been shown to be a robust laboratory parameter to diagnose and monitor cardiac failure, and it may be helpful to screen for asymptomatic left ventricular dysfunction. Therefore we tested whether NT-proBNP can distinguish patients with Duchenne or Becker muscular dystrophy patients and carriers of a dystrophin mutation with a dilated cardiomyopathy from those without. In a cohort of Duchenne and Becker muscle dystrophy patients (n = 143) and carriers (n = 219) NT-proBNP was measured, and echocardiography was performed to diagnose dilated cardiomyopathy (DCM). In total sixty-one patients (17%) fulfilled the criteria for DCM, whereas 283 patients (78%) had an elevated NT-pro BNP. The sensitivity of NT-proBNP for DCM in patients or carriers was 85%, the specificity 23%, area under the ROC-curve = 0.56. In the specified subgroups there was also no association. Measurement of NT-pro BNP in patients suffering from Duchenne or Becker muscular dystrophy and carriers does not distinguish between those with and without dilated cardiomyopathy.

Jim Peters' collapse in the 1954 Vancouver Empire Games marathon.

PubMed

Noakes, Tim; Mekler, Jackie; Pedoe, Dan Tunstall

2008-08-01

On 7 August 1954, the world 42 km marathon record holder, Jim Peters, collapsed repeatedly during the final 385 metres of the British Empire and Commonwealth Games marathon held in Vancouver, Canada. It has been assumed that Peters collapsed from heatstroke because he ran too fast and did not drink during the race, which was held in windless, cloudless conditions with a dry-bulb temperature of 28 degrees C. Hospital records made available to us indicate that Peters might not have suffered from exertional heatstroke, which classically produces a rectal temperature > 42 degrees C, cerebral effects and, usually, a fatal outcome without vigorous active cooling. Although Peters was unconscious on admission to hospital approximately 60 minutes after he was removed from the race, his rectal temperature was 39.4 degrees C and he recovered fully, even though he was managed conservatively and not actively cooled. We propose that Peters' collapse was more likely due to a combination of hyperthermia-induced fatigue which caused him to stop running; exercise-associated postural hypotension as a result of a low peripheral vascular resistance immediately he stopped running; and combined cerebral effects of hyperthermia, hypertonic hypernatraemia associated with dehydration, and perhaps undiagnosed hypoglycaemia. But none of these conditions should cause prolonged unconsciousness, raising the possibility that Peters might have suffered from a transient encephalopathy, the exact nature of which is not understood.

77 FR 26538 - Vigue, Peter A.; Notice of Filing

Federal Register 2010, 2011, 2012, 2013, 2014

2012-05-04

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ID-6627-001] Vigue, Peter A.; Notice of Filing Take notice that on April 26, 2012, Peter A. Vigue submitted for filing, a supplement to the application for authority to hold interlocking positions filed on March 6, 2012, pursuant to...

77 FR 14773 - Vigue, Peter A.; Notice of Filing

Federal Register 2010, 2011, 2012, 2013, 2014

2012-03-13

... DEPARTMENT OF ENERGY Federal Energy Regulatory Commission [Docket No. ID-6627-001] Vigue, Peter A.; Notice of Filing Take notice that on March 6, 2012, Peter A. Vigue submitted for filing, an application for authority to hold interlocking positions, pursuant to section 305(b) of the Federal Power Act, 16...

Peter Wilcox: A new purple-skin, yellow flesh fresh market potato cultivar

USDA-ARS?s Scientific Manuscript database

Peter Wilcox is a new, medium-maturing, purple-skin, yellow-flesh potato cultivar for fresh market. Peter Wilcox also produces light-colored chips, although it is being released primarily as a fresh market potato because of its skin and flesh colors. Tubers of Peter Wilcox are attractive, smooth, wi...

PEOPLE IN PHYSICS: Interview with Peter Higgs

NASA Astrophysics Data System (ADS)

Fancey, Conducted by Norman

1998-01-01

Peter Higgs, FRSE, FRS held until recently a personal chair in theoretical physics at the University of Edinburgh and is now an emeritus professor. Peter is well known for predicting the existence of a new particle, the Higgs boson - as yet unconfirmed. He has been awarded a number of prizes in recognition of his work, most recently the Paul Dirac Medal and Prize for outstanding contributions to theoretical physics from the Institute of Physics and the 1997 High Energy and Particle Physics Prize by the European Physical Society.

Peter Waterman and his scientific legacy

NASA Technical Reports Server (NTRS)

Mishchenko, Michael I.; Kahnert, Michael; Mackowski, Daniel W.; Wriedt, Thomas

2013-01-01

Peter C. Waterman, a giant figure in the theory of electromagnetic, acoustic, and elastic wave scattering, passed away on 3 June, 2012. In view of his fundamental contributions, which to a large degree have guided the progress of these disciplines over the past five decades and affected profoundly the multifaceted research published in the Journal of Quantitative Spectroscopy and Radiative Transfer (JQSRT), we felt that it would be appropriate to solicit papers for a special issue of JQSRT commemorating Peter Waterman's scientific legacy. This initiative was endorsed by the JQSRT management and has resulted in a representative collection of high-quality papers which have undergone the same peer scrutiny as any paper submitted to JQSRT.

Breast reconstruction using permanent Becker expander implants: an 18 year experience.

PubMed

Goh, S C J; Thorne, A L; Williams, G; Laws, S A M; Rainsbury, R M

2012-12-01

Single-stage reconstruction using permanent expander implants is an established technique following mastectomy. Short and long-term outcome data following breast reconstruction using Becker tissue expanders is limited. A retrospective case note review of patients undergoing expander-based procedures between 1989 and 2007 was undertaken. Data recorded included postoperative symptoms and complications, the use of radiotherapy, revisional surgery, and device failure. Three hundred and thirteen expanders were used in 276 patients with a mean age of 48.3 (17-78) years, over the 18 year study period. The mean follow up period was 64.6 (1-199) months. 256 Becker expanders were used during 175 latissimus dorsi (LD) and 52 subpectoral (SP) reconstructions, 13 contralateral augmentations and 16 implant replacements. The postoperative infection rate was 5.8%, leading to an expander loss rate of 3.8%. The use of prophylactic antibiotics was associated with an increased postoperative infection rate (p = 0.046). Six haematomas (2.5%) and 12 cases of skin envelope necrosis (5.0%) required unscheduled intervention. Symptoms of pain, distortion and hardness were experienced by 21.3% of patients, and radiotherapy was associated with a significantly higher risk of adverse symptoms (p < 0.0001). No patient developed symptomatic implant rupture or silicone granuloma but 17.9% of reconstructions underwent revisional surgery, the rate being highest following SP reconstruction (p = 0.029). Nine patients developed injection port complications (3.8%), and the overall device failure rate was 1.3%. The original expander has been retained by 74.2% of women. The Becker permanent expander is a reliable implant associated with a low complication rate and a high retention rate when used during breast reconstruction. Copyright © 2012 Elsevier Ltd. All rights reserved.

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

PubMed

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

Peter N. Ciesielski | NREL

Science.gov Websites

Biomaterials Peter.Ciesielski@nrel.gov | 303-384-7691 Orcid ID http://orcid.org/0000-0003-3360-9210 Research background and training. My research encompasses many aspects of bioenergy and biomaterials science. Biomass research aims to improve ways by which biomass can be used as a sustainable and renewable source of fuels

Peter Pindar (John Wolcot).

ERIC Educational Resources Information Center

Vales, Robert L.

This book is designed as an introduction to John Wolcot's works for the general reader, the college student, and the college teacher. Wolcot, whose pen name was Peter Pindar, wrote topical satire on public personalities of the eighteenth century, and his methods of criticism are the motif which guides each chapter and which unites all the satires…

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

PubMed

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.

Adult patient with Becker dystrophy undergoing orthopedic surgery: an anesthesia challenge.

PubMed

Parish, Masoud; Farzin, Haleh

2018-01-01

Muscular dystrophies are considered to be a series of neuromuscular diseases with genetic causes and are characterized by progressive muscle weakness and degeneration of the skeletal muscle. The case of an adult man with Becker dystrophy referred for repair of the patella tendon tearing and patella fracture is described. He underwent successful surgery using total intravenous anesthesia without any complications.

Life-threatening Arrhythmias in a Becker Muscular Dystrophy Family due to the Duplication of Exons 3-4 of the Dystrophin Gene.

PubMed

Ishizaki, Masatoshi; Fujimoto, Akiko; Ueyama, Hidetsugu; Nishida, Yasuto; Imamura, Shigehiro; Uchino, Makoto; Ando, Yukio

2015-01-01

We herein present a report of three patients with Becker muscular dystrophy in the same family who developed complete atrioventricular block or ventricular tachycardia with severe cardiomyopathy. Our cases became unable to walk in their teens, and were introduced to mechanical ventilation due to respiratory muscle weakness in their twenties and thirties. In all three cases, a medical device such as a permanent cardiac pacemaker or an implantable cardiac defibrillator was considered to be necessary. The duplication of exons 3-4 in the dystrophin gene was detected in two of the patients. In patients with Becker muscular dystrophy, complete atrioventricular block or ventricular tachycardia within a family has rarely been reported. Thus attention should be paid to the possibility of severe arrhythmias in the severe phenotype of Becker muscular dystrophy.

R. S. Peters' Normative Conception of Education and Educational Aims

ERIC Educational Resources Information Center

Katz, Michael S.

2009-01-01

This article aims to highlight why R. S. Peters' conceptual analysis of "education" was such an important contribution to the normative field of philosophy of education. In the article, I do the following: 1) explicate Peters' conception of philosophy of education as a field of philosophy and explain his approach to the philosophical analysis of…

AAMD-Becker Reading-Free Vocational Interest Inventory Manual [and Male and Female Inventories].

ERIC Educational Resources Information Center

Becker, Ralph L.

The AAMD-Becker Reading-Free Vocational Interest Inventory is a non-reading vocational preference test for use with mentally retarded persons, particularly the educable mentally retarded at the high school level. Illustrations having occupational significance are presented in forced-choice format for selections. The instrument helps to identify…

Richard Peters and Valuing Authenticity

ERIC Educational Resources Information Center

Degenhardt, M. A. B.

2009-01-01

Richard Peters has been praised for the authenticity of his philosophy, and inquiry into aspects of the development of his philosophy reveals a profound authenticity. Yet authenticity is something he seems not to favour. The apparent paradox is resolved by observing historical changes in the understanding of authenticity as an important value.…

Risk Factors for First Fractures Among Males With Duchenne or Becker Muscular Dystrophy.

PubMed

James, Katherine A; Cunniff, Christopher; Apkon, Susan D; Mathews, Katherine; Lu, Zhenqiang; Holtzer, Caleb; Pandya, Shree; Ciafaloni, Emma; Miller, Lisa

2015-09-01

Fractures are a significant concern for individuals with Duchenne/Becker muscular dystrophy with 21% to 44% of males experiencing a fracture. Factors that increase or decrease the risk for fracture have been suggested in past research, although statistical risk has not been determined. In this retrospective cohort study, we used the Muscular Dystrophy Surveillance, Tracking and Research Network cohort, a large, population-based sample to identify risk factors associated with first fractures in patients with Duchenne or Becker muscular dystrophy. Our study cohort included males with Duchenne or Becker muscular dystrophy born between 1982 and 2006 who resided in Arizona, Colorado, Georgia, Iowa, and Western New York, retrospectively identified and followed through 2010. We utilized a multivariate Cox proportional hazard model to determine hazard ratios for relevant factors associated with first fracture risk including race/ethnicity, surveillance site, ambulation status, calcium/vitamin D use and duration, bisphosphonate use and duration, and corticosteroid use and duration. Of 747 cases, 249 had at least 1 fracture (33.3%). Full-time wheelchair use increased the risk of first fracture by 75% for every 3 months of use (hazard ratio=1.75, 95% confidence interval, 1.14, 2.68), but corticosteroid use, bisphosphonate use, and calcium/vitamin D use did not significantly affect risk in the final adjusted model. In this cohort, first fractures were common and full-time wheelchair use, but not corticosteroid use, was identified as a risk factor. The impact of prevention measures should be more thoroughly assessed. Fractures are a significant concern for individuals with dystrophinopathies, but the contribution of various risk factors has not been consistently demonstrated.

Against the Corporate Culture Ideology: An Interview with Peter Mayo

ERIC Educational Resources Information Center

Suoranta, Juha

2010-01-01

This article presents an interview with Peter Mayo, author and expert in the field of sociology of adult education, on his major influences in this area, his books, and his views on the role of radical adult education and radical scholarship in the future. In the interview, Peter Mayo states that his initial view of adult education was quite a…

Peter & Jane: A Program Showcase

ERIC Educational Resources Information Center

Kalinowski, Michael

2008-01-01

This article features the early childhood programs at Peter & Jane Kindergarten located in Petaling Java, Malaysia. The primary purpose of the programs is to lay a strong foundation for a lifelong love of learning in each child. According to principal Patricia Teh, the activities are funded by parent fees and serve children two to six years of…

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

PubMed

Fiorillo, Alyson A; Heier, Christopher R; Novak, James S; Tully, Christopher B; Brown, Kristy J; Uaesoontrachoon, Kitipong; Vila, Maria C; Ngheim, Peter P; Bello, Luca; Kornegay, Joe N; Angelini, Corrado; Partridge, Terence A; Nagaraju, Kanneboyina; Hoffman, Eric P

2015-09-08

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. Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.

Peter Waterman and T-Matrix Methods

NASA Technical Reports Server (NTRS)

Mishchenko, M. I.; Martin, P.A.

2013-01-01

This paper summarizes the scientific legacy of Peter C. Waterman (1928-2012) who introduced concepts and theoretical techniques that have had a major impact on the fields of scattering by particles and particle groups, optical particletcharacterization, radiative transfer, and remote sensing. A biographical sketch is also included.

The Future of the Digital Library: An Interview with Tom Peters

ERIC Educational Resources Information Center

Morrison, James L.; Peters, Tom

2005-01-01

This article presents an interview with Tom Peters, an academic librarian and founder of TAP Information Services, a firm that provides consulting services to libraries and other organizations in the information industry. Peters also serves as a consultant to LibraryCity, an ambitious project that seeks to make thousands of e-books in easy-to-use…

Ripples from a Passing Ship: Memories; and a Legacy of Richard Peters

ERIC Educational Resources Information Center

Harris, Kevin

2013-01-01

This paper outlines aspects and dimensions of my "relationship" with Richard Peters from 1966 onward. The underlying suggestion is that, while Peters' contribution to philosophy of education was undeniably of major proportions, both that contribution and his legacy are institutional rather than substantive. (Contains 15 notes.)

Peter V.Hobbs (1936-2005)

NASA Astrophysics Data System (ADS)

Wallace, John M.

Peter Hobbs, a professor in the Department of Atmospheric Sciences at the University of Washington in Seattle, died of pancreatic cancer on 25 July 2005.Born in 1936, Hobbs experienced what he described as “a wild, adventurous youth on the streets of postwar London.” He engaged in competitive swimming and at age 13 held the title of Junior Boxing Champion of the County of Surrey.

Autonomy in R. S. Peters' Educational Theory

ERIC Educational Resources Information Center

Cuypers, Stefaan E.

2009-01-01

Autonomy is, among other things, an actual psychological condition, a capacity that can be developed, and an educational ideal. This paper contextualises, analyses, criticises and extends the theory of Richard S. Peters on these three aspects of autonomy.

Peter Koch: wizard of wood use

Treesearch

M.E. Lora

1978-01-01

Like his pioneer forefathers, Peter Koch sees opportunity where others see obstacles. And his vision is helping to reshape the wood industry. Since 1963 Koch has directed research on processing southern woods for the U.S. Forest Service's Southern Forest Experiment Station in Pineville, Louisiana. In that time, he has invented six revolutionary machines, developed...

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

PubMed

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. Copyright © 2014 John Wiley & Sons, Ltd.

Ordovician conodonts and stratigraphy of the ST. Peter sandstone and glen wood shale, central United States

USGS Publications Warehouse

Witzke, B.J.; Metzger, R.A.

2005-01-01

The age of the St. Peter Sandstone in the central and northern Midcontinent has long been considered equivocal because of the general absence of biostratigraphically useful fossils. Conodonts recovered from the St. Peter Sandstone in Iowa, Minnesota, Nebraska, and Kansas for this study help place some age constraints on this renowned formation in its northern and western extent. Faunas from the lower St. Peter include Phragmodus flexuosus, Cahabagnathus sp., and Leptochirognathus sp., and a late Whiterockian (Chazyan) correlation is indicated. Juvenile or immature elements of P. flexuosus from these collections show morphologies trending toward P. cognitus and P. inflexus, and paedomorphic derivation of these latter species is proposed. Diverse assemblages of hyaline forms also occur in the St. Peter strata (Erismodus spp., Erraticodon sp., Curtognathus sp., Coleodus sp., Archeognathus sp., Stereoconus sp., others) along with various albid elements (Plectodina sp., Eoplacognathus sp., others). The overlying Glenwood Shale contains abundant conodonts dominated by Phragmodus cognitus, Erismodus sp., and Chirognathus duodactylus, and the fauna is interpreted as an early Mohawkian (Blackriveran) association. Certain thin shale units in the St. Peter-Glenwood succession represent condensed intervals, in part reflected by their exceptionally high conodont abundances. Some organic-rich phosphatic shale units in the lower St. Peter of western Iowa have produced equivalent yields of tens of thousands of conodonts per kilogram, and many Glenwood Shale samples yield thousands of conodonts per kilogram. Previous depositional models have proposed that the St. Peter is primarily a succession of littoral and nearshore facies forming a broadly diachronous transgressive sheet sand. However, broad-scale diachroneity cannot be demonstrated with available biostratigraphic control. The recognition of condensed marine shale units, phosphorites, ironstones, and pyritic hardgrounds in the

A Cross-Sectional Study of School Experiences of Boys with Duchenne and Becker Muscular Dystrophy

ERIC Educational Resources Information Center

Soim, Aida; Lamb, Molly; Campbell, Kimberly; Pandya, Shree; Peay, Holly; Howard, James F., Jr.; Fox, Deborah

2016-01-01

The objectives of this study were to investigate types of supportive school services received and factors related to provision of these services. We conducted a cross-sectional study to describe the school experience of males with Duchenne and Becker muscular dystrophies. Study subjects were identified through the Muscular Dystrophy Surveillance,…

Lydia Becker's "School for Science": a challenge to domesticity.

PubMed

Parker, J E

2001-01-01

Lydia Becker (1827-1890) is known as a leader of the Women's Suffrage Movement but little is known about her work to include women and girls in science. Before her energy was channelled into politics, she aimed to have a scientific career. Mid-Victorian Britain was a period in which women's intellect and potential were widely debated, and in which the dominant ideology was that their primary role in life was that of wife and mother. Science was widely regarded as a "masculine" subject which women were deliberately discouraged from studying. The author concentrates on the two main areas in which important contributions were made, the British Association for the Advancement of Science, and the Manchester School Board.

Propofol-induced violent coughing in a patient with Becker's muscular dystrophy

PubMed Central

Jain, Amit

2011-01-01

Propofol anesthesia is often associated with decreased incidence of gagging, coughing or laryngospasm, and provides intense suppression on airway reflex during tracheal intubation and laryngeal mask airway insertion. Propofol pretreatment is also effective in reducing the occurrence of opioid-induced coughing. These benefits are often attributed to bronchodilator and sedative effects of propofol. However, severe coughing following sedative doses of 1% propofol has not been reported so far. We report a rare case of violent coughing following low-dose propofol infusion in a patient with Becker's muscular dystrophy. PMID:21845012

Repair of an inguinoscrotal hernia in a patient with Becker muscular dystrophy.

PubMed

Tatulli, F; Caraglia, A; Delcuratolo, A; Cassano, S; Chetta, G S

2017-01-01

Inguinal hernia repairs are routinely performed as outpatient procedures in most patients, whereas a few require admission due to clinical or social peculiarities. Muscular dystrophies are inherited disorders characterized by progressive muscle wasting and weakness. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards a 48 y. o. male patient diagnosed with Becker Muscular Dystrophy by muscle biopsy 10 years earlier. He had a left-sided sizable inguinoscrotal hernia with repeat episodes of incarceration. An elective mesh repair with suction drainage was accomplished under selective spinal anesthesia. The post-operative course was uneventful. A few inguinal hernia repairs require admission due to peculiarities such as extensive scrotal hernias requiring suction drainage. Muscular dystrophies are inherited disorders with no cure and no two dystrophy patients are exactly alike, therefore the health issues will be different for each individual. In case of surgery there is no definite recommendation for either general or regional anesthesia. This contribution regards the successful elective mesh repair with suction drainage of a large left-sided inguino-scrotal hernia in a 48 y. o. male patient affected by Becker muscular dystrophy by selective spinal anesthesia obtained by 10 milligrams of hyperbaric bupivacaine. Effective mesh repair with suction drainage of large inguinal hernias under spinal anesthesia can be achieved in patients affected by muscular dystrophy.

Peter the Great: Linking Military Strategy to National Objectives in Imperial Russia

DTIC Science & Technology

1999-04-01

provisions) and because its forces could not move fast enough to overcome the Ottoman’s delaying tactics.1 Vasili Golitsyn, Alexei’s representative to...conclusion when, in 1694, he pitted six Streltsy regiments against his two new Guards regiments (Preobrazhenskii and Semenovskii) in mock combat. The...Press, 1971. Gray, Ian. Peter the Great: Emperor of all Russia. New York: J.B.Lippincott, 1960. Klychevsky, Vasili . Peter the Great. Boston: Beacon

Team Leader: Tom Peters--TAP Information Services

ERIC Educational Resources Information Center

Library Journal, 2005

2005-01-01

Tom Peters packs 36 hours of work into the confines of a 24-hour day. Without breaking a sweat, he juggles multiple collaborative projects, which currently include an Illinois academic library shared storage facility; a multistate virtual reference and instruction service for blind and visually impaired individuals (InfoEyes); a virtual meeting…

R. S. Peters: The Reasonableness of Ethics

ERIC Educational Resources Information Center

Haynes, Felicity

2013-01-01

This article will begin by examining the extent to which R. S. Peters merited the charge of analytic philosopher. His background in social psychology allowed him to become more pragmatic and grounded in social conventions and ordinary language than the analytic philosophers associated with empiricism, and his gradual shift from requiring internal…

Professor Peter Choyce: an early pioneer of intraocular lenses and corneal/refractive surgery.

PubMed

Pandey, Suresh K; Apple, David J

2005-06-01

Professor Peter Choyce, FRCS, DOMS, MS, was one of the pioneers of intraocular lens implant surgery. He developed an interest in artificial lens implantation following cataract surgery, a procedure that was widely criticized by the ophthalmic establishment in the UK, Europe, North America and other countries. Owing to the opposition to the intraocular lenses, Peter Choyce together with Sir Harold Ridley co-founded the International Intraocular Implant Club in 1966, which was responsible for the gradual acceptance of artificial lens implantation. Peter Choyce developed several models of intraocular lens, but did not patent the majority of them. The Choyce Mark IX, manufactured by Rayner Intraocular Lenses, became the first US Food and Drug Administration-approved intraocular lens in 1981. A review of Peter Choyce's record confirms a significant number of original innovations in the field of anterior segment surgery, including many procedures taken for granted today, but not associated with his name. These include early work on both kerato- and intraocular lens-refractive procedures, keratoprosthesis, pioneering paediatric implant procedures and others. Unfortunately his tenacious adherence to anterior chamber lens technology, while in general clinically sound, caused many to question his influence and hence he remained poorly understood even until after his death. He passed away on 8 August 2001 after a long fight with colon cancer. In this article, we provide evidence and elaborate Peter Choyce's accomplishments, which places him as one of the most innovative ophthalmologist in his surgical field in the twentieth century.

Mutations at the PAX6 locus are found in heterogeneous anterior segment malformations including Peters' anomaly.

PubMed

Hanson, I M; Fletcher, J M; Jordan, T; Brown, A; Taylor, D; Adams, R J; Punnett, H H; van Heyningen, V

1994-02-01

Mutation or deletion of the PAX6 gene underlies many cases of aniridia. Three lines of evidence now converge to implicate PAX6 more widely in anterior segment malformations including Peters' anomaly. First, a child with Peters' anomaly is deleted for one copy of PAX6. Second, affected members of a family with dominantly inherited anterior segment malformations, including Peters' anomaly are heterozygous for an R26G mutation in the PAX6 paired box. Third, a proportion of Sey/+ Smalleye mice, heterozygous for a nonsense mutation in murine Pax-6, have an ocular phenotype resembling Peters' anomaly. We therefore propose that a variety of anterior segment anomalies may be associated with PAX6 mutations.

Magnetic resonance imaging phenotyping of Becker muscular dystrophy.

PubMed

Faridian-Aragh, Neda; Wagner, Kathryn R; Leung, Doris G; Carrino, John A

2014-12-01

There is little information on magnetic resonance imaging (MRI) phenotypes of Becker muscular dystrophy (BMD). This study presents the MRI phenotyping of the upper and lower extremities of a large cohort of BMD patients. In this retrospective study, MRI images of 33 BMD subjects were evaluated for severity, distribution, and symmetry of involvement. Teres major, triceps long head, biceps brachii long head, gluteus maximus, gluteus medius, vasti, adductor longus, adductor magnus, semitendinosus, semimembranosus, and biceps femoris muscles showed the highest severity and frequency of involvement. All analyzed muscles had a high frequency of symmetric involvement. There was significant variability of involvement between muscles within some muscle groups, most notably the arm abductors, posterior arm muscles, medial thigh muscles, and lateral hip rotators. This study showed a distinctive pattern of involvement of extremity muscles in BMD subjects. © 2014 Wiley Periodicals, Inc.

Reading R. S. Peters on Education Today

ERIC Educational Resources Information Center

Cuypers, Stefaan E.; Martin, Christopher

2009-01-01

This introduction to this special issue offers an overview of R. S. Peters' seminal role in the development of modern philosophy of education, acknowledging the originality and range of his work, and indicating his continuing importance to the field. It explains the structure and organisation of the collection and provides a rationale for this…

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

PubMed

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.

The Existential Concern of the Humanities R. S. Peters' Justification of Liberal Education

ERIC Educational Resources Information Center

Cuypers, Stefaan E.

2018-01-01

Richard Stanley Peters was one of the founding fathers of analytic philosophy of education in the twentieth century. After reviewing Peters' disentanglement of the ambiguities of liberal education, I reconstruct his view on the status and the existential foundations of the humanities. What emerges from my reconstruction is an original…

The "Physically Educated" Person: Physical Education in the Philosophy of Reid, Peters and Aristotle

ERIC Educational Resources Information Center

MacAllister, James

2013-01-01

This article will derive a definition and account of the physically educated person, through an examination of the philosophy of Andrew Reid, Richard Peters and Aristotle. Initially, Reid's interpretation of Peters' views about the educational significance of practical knowledge (and physical education) will be considered. While it will…

Peter Jarvis and the Understanding of Adult Learning

ERIC Educational Resources Information Center

Illeris, Knud

2017-01-01

By comparing Peter Jarvis' understanding of learning with two other approaches--which Jarvis himself has referred to as "the most comprehensive": Etienne Wenger's "social theory of learning" and my own psychologically oriented theory of "the three dimensions of learning"--it becomes evident that Jarvis' understanding…

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

PubMed

Hamoudi, Hassan; Rudnick, Jens-Christian; Prause, Jan U; Tauscher, Kerstin; Breithaupt, Angele; Teifke, Jens P; Heegaard, Steffen

2013-07-01

Two sibling snow leopards, a male and a female, with bilateral anterior segment dysgenesis (ASD), are reported. Both snow leopards also had colobomas of both upper eyelids. All eyes exhibited a central corneal opacity associated with a defect in posterior corneal stroma, endothelium and Descemet's membrane. Iris strands were present attached to the termination of Descemet's membrane and to the periphery of the posterior corneal defect. The iris was hypoplastic, and cataract was present in all four eyes. The left eye of the female was microphthalmic, with no trabecular meshwork and with persistent remnant of the hyaloid artery. The male had hydrocephalus and thus some of the features of Peters' plus syndrome (Peters' anomaly in addition to systemic malformations). The histological findings in the eyes of these snow leopard siblings are identical with those described in humans with Peters' anomaly. © 2012 American College of Veterinary Ophthalmologists.

Theories of Power, Poverty, and Law: In Commemoration of the Contributions of Peter Bachrach--The Challenge of Peter Bachrach

ERIC Educational Resources Information Center

Lukes, Steven

2010-01-01

Peter Bachrach had a remarkable impact on those who encountered him in person and on generations of readers. Judith Baer vividly captures, among other things, his inspiring, emboldening influence on his students and the sheer fun it was to be with him. My recollections are of exciting, forward moving, intense, and probing arguments, in private and…

The Statue of Liberty Peter Max Style!

ERIC Educational Resources Information Center

Cunningham, Kathy

2012-01-01

The author's school is only 30 minutes from New York City, so every year when second-graders study towns and cities, the students do a project based on New York City landmarks. This year was the Statue of Liberty. The author introduced Peter Max's famous Pop art to her students, and explained that, as the art world kept changing, artists decided…

1. DOWNRIVER VIEW OF BRIDGE, LOOKING SOUTHSOUTHWEST Peter J. Edwards, ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

1. DOWNRIVER VIEW OF BRIDGE, LOOKING SOUTH-SOUTHWEST Peter J. Edwards, photographer, August 1988 - Four Mile Bridge, Copper Creek Road, Spans Table Rock Fork, Mollala River, Molalla, Clackamas County, OR

Peter Effect in the Preparation of Reading Teachers

ERIC Educational Resources Information Center

Binks-Cantrell, Emily; Washburn, Erin K.; Joshi, R. Malatesha; Hougen, Martha

2012-01-01

The Peter Effect (Applegate & Applegate, 2004) claimed that one cannot be expected to give what one does not possess. We applied this notion to reading teacher preparation and hypothesized that teacher educators who do not possess an understanding of basic language constructs would not prepare teacher candidates with an understanding of these…

Muscular dystrophy in a dog resembling human becker muscular dystrophy.

PubMed

Baroncelli, A B; Abellonio, F; Pagano, T B; Esposito, I; Peirone, B; Papparella, S; Paciello, O

2014-05-01

A 3-year-old, male Labrador retriever dog was presented with clinical signs of progressive exercise intolerance, bilateral elbow extension, rigidity of the forelimbs, hindlimb flexion and kyphosis. Microscopical examination of muscle tissue showed marked variability in myofibre size, replacement of muscle with mature adipose tissue and degeneration/regeneration of muscle fibres, consistent with muscular dystrophy. Immunohistochemical examination for dystrophin showed markedly reduced labelling with monoclonal antibodies specific for the rod domain and the carboxy-terminal of dystrophin, while expression of β-sarcoglycan, γ-sarcoglycan and β-dystroglycan was normal. Immunoblotting revealed a truncated dystrophin protein of approximately 135 kDa. These findings supported a diagnosis of congenital canine muscular dystrophy resembling Becker muscular dystrophy in man. Copyright © 2014 Elsevier Ltd. All rights reserved.

Becker's rational addiction theory: An empirical test with price elasticities for distilled spirits in Denmark 1911-31.

PubMed

Skog, Ole-Jørgen; Melberg, Hans Olav

2006-10-01

To test an implication of Becker's rational addiction theory, namely that price changes will lead both to simultaneous consumption changes as well as lagged changes (and potentially also immediate changes if future changes in prices are anticipated). Time-series analysis, first of aggregate sales of distilled spirits and prices, controlled for gross national product (GNP), and secondly of deaths from delirium tremens. Denmark 1911-31. Price changes were very large in the period 1916-18 due to shortages during World War I, and the Danish case can be conceived as a natural experiment. No evidence for lagged price effects in the expected direction was found. On the contrary, the evidence pointed in the opposite direction. The immediate reduction in sales following rising prices are, to some degree, counteracted by an adjustment in the opposite direction the following year. The delirium tremens data confirm this pattern. Becker's theory is not confirmed. Several possible explanations are discussed. If the pattern observed in these data is representative of a more general mechanism, current price elasticity estimates may be too high, by ignoring lagged compensatory effects.

A tribute to Peter A. Rona: A Russian Perspective

NASA Astrophysics Data System (ADS)

Sagalevich, Anatoly; Lutz, Richard A.

2015-11-01

In July 1985 Peter Rona led a cruise of the National Oceanic and Atmospheric Administration (NOAA) ship Researcher as part of the NOAA Vents Program and discovered, for the first time, black smokers, massive sulfide deposits and vent biota in the Atlantic Ocean. The site of the venting phenomena was the Trans-Atlantic Geotraverse (TAG) Hydrothermal Field on the east wall of the rift valley of the Mid-Atlantic Ridge at 26°08‧N; 44°50‧W (Rona, 1985; Rona et al., 1986). In 1986, Peter and an international research team carried out multidisciplnary investigations of both active and inactive hydrothermal zones of the TAG field using the R/V Atlantis and DSV Alvin, discovering two new species of shrimp (Rimicaris exoculata and Chorocaris chacei) (Williams and Rona, 1986) and a hexagonal-shaped form (Paleodictyon nodosum) thought to be extinct (Rona et al., 2009). In 1991 a Russian crew aboard the R/V Akademik Mstislav Keldysh, with two deep-diving, human-occupied submersibles (Mir-1 and Mir-2) (Fig. 1), had the honor of having Peter Rona and a Canadian IMAX film crew from the Stephen Low Company on board to visit the TAG hydrothermal vent field. This was the first of many deep-sea interactions between Russian deep-sea scientists and their colleagues from both the U.S. and Canada. This expedition to the TAG site was part of a major Russian undersea program aimed at exploring extreme deep-sea environments; between 1988 and 2005, the Mir submersibles visited hydrothermal vents and cold seep areas in 20 deep-sea regions throughout the world's oceans (Sagalevich, 2002). Images of several of these areas (the TAG, Snake Pit, Lost City and 9°50‧N vent fields) were obtained using an IMAX camera system emplaced for the first time within the spheres of the Mir submersibles and DSV Alvin in conjunction with the filming of science documentaries (e.g., ;Volcanoes of the Deep Sea;) produced by the Stephen Low Company in conjunction with Emory Kristof of National Geographic and

R. S. Peters' "The Justification of Education" Revisited

ERIC Educational Resources Information Center

Cuypers, Stefaan E.

2012-01-01

In his 1973 paper "The Justification of Education" R. S. Peters aspired to give a non-instrumental justification of education. Ever since, his so-called "transcendental argument" has been under attack and most critics conclude that it does not work. They have, however, thrown the baby away with the bathwater, when they furthermore concluded that…

Portrait Face-Off: Gilbert Stuart vs. Peter Max

ERIC Educational Resources Information Center

Crumpecker, Cheryl

2012-01-01

When art classes are short and infrequent, it is always a challenge to meet required state and national standards. A unit comparing and contrasting Peter Max's Pop art portraits with the realistic style of Gilbert Stuart's presidential portraits provides an opportunity to address a huge number of these requirements. Focus can change with the age…

Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study.

PubMed

Nakamura, Yuko; Saito, Yoshiaki; Kubota, Norika; Matsumura, Wataru; Hosoda, Chika; Tamasaki-Kondo, Akiko; Nishimura, Yoko; Sunada, Yoshihide; Fukada, Masuyuki; Ohno, Takako; Maegaki, Yoshihiro; Matsuo, Masafumi; Tokita, Yasuko

2018-03-08

To report on sleep hypercapnia in Becker muscular dystrophy (BMD) at earlier stages than ever recognized. This retrospective study examined nocturnal hypercapnia in six young Becker muscular dystrophy (BMD) patients with deletions of one or more exons of DMD gene. Clinical information, consecutive data on forced vital capacity (FVC%), forced expiratory volume in one second (FEV1%), peak expiratory flow (PEF%), peak cough flow (PCF), average PCO 2 in all-night monitoring, and left ventricular ejection fraction (LVEF) were reviewed. In five BMD patients, including three who were still ambulant, nocturnal average PCO 2 was elevated to >45 mmHg at 12-31 years of age. Noninvasive positive pressure ventilation was initiated in four patients. Gradual declines in FVC% and PEF% were evident in one BMD patient with exon 3-7 deletion, whereas these functions did not change in the remaining BMD patients. PCF, FEV1%, and LVEF were less informative for the assessment of respiratory function in this patient series. Sleep hypercapnia was present in certain BMD patients, which was unexpected from the routine pulmonary function tests. Individualized assessment of nocturnal PCO 2 , partly based on the deletion types, should be further explored in the clinical practice of BMD patients. Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Becker muscular dystrophy severity is linked to the structure of dystrophin.

PubMed

Nicolas, Aurélie; Raguénès-Nicol, Céline; Ben Yaou, Rabah; Ameziane-Le Hir, Sarah; Chéron, Angélique; Vié, Véronique; Claustres, Mireille; Leturcq, France; Delalande, Olivier; Hubert, Jean-François; Tuffery-Giraud, Sylvie; Giudice, Emmanuel; Le Rumeur, Elisabeth

2015-03-01

In-frame exon deletions of the Duchenne muscular dystrophy (DMD) gene produce internally truncated proteins that typically lead to Becker muscular dystrophy (BMD), a milder allelic disorder of DMD. We hypothesized that differences in the structure of mutant dystrophin may be responsible for the clinical heterogeneity observed in Becker patients and we studied four prevalent in-frame exon deletions, i.e. Δ45-47, Δ45-48, Δ45-49 and Δ45-51. Molecular homology modelling revealed that the proteins corresponding to deletions Δ45-48 and Δ45-51 displayed a similar structure (hybrid repeat) than the wild-type dystrophin, whereas deletions Δ45-47 and Δ45-49 lead to proteins with an unrelated structure (fractional repeat). All four proteins in vitro expressed in a fragment encoding repeats 16-21 were folded in α-helices and remained highly stable. Refolding dynamics were slowed and molecular surface hydrophobicity were higher in fractional repeat containing Δ45-47 and Δ45-49 deletions compared with hybrid repeat containing Δ45-48 and Δ45-51 deletions. By retrospectively collecting data for a series of French BMD patients, we showed that the age of dilated cardiomyopathy (DCM) onset was delayed by 11 and 14 years in Δ45-48 and Δ45-49 compared with Δ45-47 patients, respectively. A clear trend toward earlier wheelchair dependency (minimum of 11 years) was also observed in Δ45-47 and Δ45-49 patients compared with Δ45-48 patients. Muscle dystrophin levels were moderately reduced in most patients without clear correlation with the deletion type. Disease progression in BMD patients appears to be dependent on the deletion itself and associated with a specific structure of dystrophin at the deletion site. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

My Journey with Peter: Moving Ideas that Matter

ERIC Educational Resources Information Center

Blyth, Dale A.

2012-01-01

Youth development lost a pioneer and a champion, Peter Benson, in October. Benson was a pioneer whose ideas mattered and a champion of approaches to research and its use in the community that has made a difference around the world. Benson's work, life, and spirit have helped transform people's understanding of young people, what it takes to…

Otto Peters on Distance Education: The Industrialization of Teaching and Learning. Routledge Studies in Distance Education.

ERIC Educational Resources Information Center

Keegan, Desmond, Ed.

This book contains new and previously published translations of 11 essays and articles about the industrialization of teaching and learning in distance education that were originally published in German by Otto Peters between 1965 and 1993. A "Preface" (Desmond Keegan) and an introduction placing Peters' writings in their historical…

Peter Drucker, Knowledge Work, and the Structure of Schools.

ERIC Educational Resources Information Center

Tucker, Marc S.

1988-01-01

Discusses Peter Drucker's recommendations in "The Age of Discontinuity" (1969) concerning the decline of manual work, the ascendancy of knowledge work, and the relationship between economic stability and an educated, productive work force. Applies Drucker's principles to the movement to restructure schools, which aims to make line…

Reflections on Peters' View of the Nature and Purpose of Work in Philosophy of Education

ERIC Educational Resources Information Center

Aspin, D. N.

2013-01-01

In this article I describe the analytic approach adopted by Peters, his colleagues and followers of the "London line" in the 1960s and 1970s and argue that, even in those times, other approaches to philosophy of education were being valued and practised. I show that Peters and his colleagues later became aware of the need for philosophy of…

K istorii goroskopa Petra Velikogo %t On the history of horoscope of Peter the Great

NASA Astrophysics Data System (ADS)

Bronshtehn, V. A.

In the first part of the paper the question is discussed if Simeon Polotsky (1629-1680), poet and teacher of children of the Russian tsar Alexei Mikhailovich, was also the author of the horoscope of his son, in the future - Russian emperor Peter the Great, born in 1672. The poems by Simeon Polotsky with astrological contents are analyzed. The conclusion is supported that he could be the author of Peter the Great horoscope. In the second part a recently found text of the horoscope of Peter the Great reconstructed in 1775 by Russian astronomer Andrei Lexell of the request of historian G. F. Miller is published and discussed. It is also compared with texts previously published (in 1842) by Russian historians Pogodin and Polevoi.

Cost-Effectiveness Analysis of Diagnosis of Duchenne/Becker Muscular Dystrophy in Colombia.

PubMed

Atehortúa, Sara C; Lugo, Luz H; Ceballos, Mateo; Orozco, Esteban; Castro, Paula A; Arango, Juan C; Mateus, Heidi E

2018-03-09

To determine the cost-effectiveness ratio of different courses of action for the diagnosis of Duchenne or Becker muscular dystrophy in Colombia. The cost-effectiveness analysis was performed from the Colombian health system perspective. Decision trees were constructed, and different courses of action were compared considering the following tests: immunohistochemistry (IHC), Western blot (WB), multiplex polymerase chain reaction, multiplex ligation-dependent probe amplification (MLPA), and the complete sequencing of the dystrophin gene. The time horizon matched the duration of sample extraction and analysis. Transition probabilities were obtained from a systematic review. Costs were constructed with a type-case methodology using the consensus of experts and the valuation of resources from consulting laboratories and the 2001 Social Security Institute cost manual. Deterministic sensitivity and scenario analyses were performed with one or more unavailable alternatives. Costs were converted from Colombian pesos to US dollars using the 2014 exchange rate. In the base case, WB was the dominant strategy, with a cost of US $419.07 and a sensitivity of 100%. This approach remains the dominant strategy down to a 98.2% sensitivity and while costs do not exceed US $837.38. If WB was not available, IHC had the best cost-effectiveness ratio, followed by MLPA and sequencing. WB is a cost-effective alternative for the diagnosis of patients suspected of having Duchenne or Becker muscular dystrophy in the Colombian health system. The IHC test is rated as the second-best detection method. If these tests are not available, MLPA followed by sequencing would be the most cost-effective alternative. Copyright © 2018. Published by Elsevier Inc.

Marginal Experiments: Peter Brook and Stepping out Theatre Company

ERIC Educational Resources Information Center

Harpin, Anna

2010-01-01

This article juxtaposes the recent work of Peter Brook with a Bristol-based mental health service-user collective--Stepping Out Theatre Company. Informed by field-work with the company, this chapter explores the aesthetic and political relationship between professional, experimental theatre work and community-based performance practice. Drawing…

R. S. Peters and the Concept of Education

ERIC Educational Resources Information Center

Beckett, Kelvin Stewart

2011-01-01

In this essay Kelvin Beckett argues that Richard Peters's major work on education, "Ethics and Education," belongs on a short list of important texts we can all share. He argues this not because of the place it has in the history of philosophy of education, as important as that is, but because of the contribution it can still make to the future of…

Peters' Concept of "Education as Initiation": Communitarian or Individualist?

ERIC Educational Resources Information Center

Cotter, Richard

2013-01-01

A central element of Richard Peters' philosophy of education has been his analysis of "education as initiation". Understanding initiation is internally related to concepts of community and what it may mean to be a member. The concept of initiation assumes a mutually interdependent, dynamic relationship between the individual and community that…

University of Florida potato variety trials spotlight: 'Peter Wilcox'

USDA-ARS?s Scientific Manuscript database

'Peter Wilcox’ is a fresh market potato variety selected from progeny of a cross between B0810-1 and B0918-5, and tested under the pedigree B1816-5 by K.G. Haynes. It was jointly released by United States Department of Agriculture, North Carolina Agricultural Research Service, Agricultural Experimen...

A Case of Refractory Heart Failure in Becker Muscular Dystrophy Improved With Corticosteroid Therapy.

PubMed

Nakamura, Makiko; Sunagawa, Osahiko; Hokama, Ryo; Tsuchiya, Hiroyuki; Miyara, Takafumi; Taba, Yoji; Touma, Takashi

2016-09-28

The patient was a 26 year-old man who was referred to our hospital in June 2011 because of severe heart failure. At age 24 years, he was found to have Becker muscular dystrophy. He received enalapril for cardiac dysfunction; however, he had worsening heart failure and was thus referred to our hospital. Echocardiography showed enlargement of the left ventricle, with a diastolic dimension of 77 mm and ejection fraction of 19%. His condition improved temporarily after an infusion of dobutamine and milrinone. He was then administered amiodarone for ventricular tachycardia; however, he subsequently developed hemoptysis. Amiodarone was discontinued and corticosteroid pulse therapy was administered followed by oral prednisolone (PSL). His creatinine phosphokinase (CPK) level and cardiomegaly improved after the corticosteroid therapy. The PSL dose was reduced gradually, bisoprolol was introduced, and the catecholamine infusion was tapered. A cardiac resynchronization device was implanted; however, the patient's condition gradually worsened, which necessitated dobutamine infusion for heart failure. We readministered 30 mg PSL, which decreased the CPK level and improved the cardiomegaly. The dobutamine infusion was discontinued, and the patient was discharged. He was given 7.5 mg PSL as an outpatient, and he returned to normal life without exacerbation of the heart failure. There are similar reports showing that corticosteroids are effective for skeletal muscle improvement in Duchenne muscular dystrophy; however, their effectiveness for heart failure has been rarely reported. We experienced a case of Becker muscular dystrophy in which corticosteroid therapy was effective for refractory heart failure.

Abnormal centromere-chromatid apposition (ACCA) and Peters' anomaly.

PubMed

Wertelecki, W; Dev, V G; Superneau, D W

1985-08-01

Abnormal centromere-chromatid apposition (ACCA) was noted in a patient with Peters' anomaly. Previous reports of ACCA emphasized its association with tetraphocomelia and other congenital malformations (Roberts, SC Phocomelia, Pseudothalidomide Syndromes). This report expands the array of congenital malformations associated with ACCA and emphasizes the diagnostic importance of ocular defects for the ascertainment of additional cases of ACCA and its possible relationship with abnormal cell division.

Science Matters Podcast: Questions and Answers with EPA's Dr. Peter Grevatt

EPA Pesticide Factsheets

Listen to a podcast with Dr. Peter Grevatt, the director of EPA's Office of Children's Health Protection, as he answers questions about children's health, or read some of the highlights from the conversation here.

Weighing the Evidence in Peters' Rule: Does Neuronal Morphology Predict Connectivity?

PubMed

Rees, Christopher L; Moradi, Keivan; Ascoli, Giorgio A

2017-02-01

Although the importance of network connectivity is increasingly recognized, identifying synapses remains challenging relative to the routine characterization of neuronal morphology. Thus, researchers frequently employ axon-dendrite colocations as proxies of potential connections. This putative equivalence, commonly referred to as Peters' rule, has been recently studied at multiple levels and scales, fueling passionate debates regarding its validity. Our critical literature review identifies three conceptually distinct but often confused applications: inferring neuron type circuitry, predicting synaptic contacts among individual cells, and estimating synapse numbers within neuron pairs. Paradoxically, at the originally proposed cell-type level, Peters' rule remains largely untested. Leveraging Hippocampome.org, we validate and refine the relationship between axonal-dendritic colocations and synaptic circuits, clarifying the interpretation of existing and forthcoming data. Copyright © 2016 Elsevier Ltd. All rights reserved.

75 FR 63533 - Gulf & Ohio Railways Holding Co., Inc., H. Peter Claussen and Linda C. Claussen-Continuance in...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-10-15

... DEPARTMENT OF TRANSPORTATION Surface Transportation Board [Docket No. FD 35414] Gulf & Ohio Railways Holding Co., Inc., H. Peter Claussen and Linda C. Claussen--Continuance in Control Exemption--Lancaster & Chester Railroad, LLC Gulf & Ohio Railways Holding Co., Inc. (G&O), H. Peter Claussen and Linda...

PEOPLE IN PHYSICS: Interview with Peter Barham

NASA Astrophysics Data System (ADS)

Membrey, Conducted by Jill

2000-03-01

Dr Peter Barham, Reader in Physics at the University of Bristol, is one of the first winners of an Institute of Physics Public Awareness of Physics award. These are intended for individuals or groups who have demonstrated excellence, inspiration and innovation in bringing physics to the public. In Dr Barham's case, he has been recognized for his very successful lecture demonstrations on the physics of food for a range of audiences, as well as for supporting and encouraging others to promote physics to the general public.

A comparison of swallowing dysfunction in Becker muscular dystrophy and Duchenne muscular dystrophy.

PubMed

Yamada, Yuka; Kawakami, Michiyuki; Wada, Ayako; Otsuka, Tomoyoshi; Muraoka, Kaori; Liu, Meigen

2018-06-01

Swallowing dysfunction has been reported in Duchenne muscular dystrophy (DMD), but has not been studied in Becker muscular dystrophy (BMD). The aims of this study were to report the characteristics of swallowing dysfunction in BMD compared with DMD. The study participants were 18 patients with BMD and 18 patients with DMD. All the patients were examined using videofluorography during swallowing of 5 mL of fluid. The penetration-aspiration scale (P-A scale) and the videofluorographic dysphagia scale (VDS) were used to evaluate dysphagia. Swinyard functional ability stage was not significantly different between the BMD and DMD groups. Rate of aspiration, P-A scale score, and total VDS score did not differ across groups, but the VDS item score for laryngeal elevation was lower in the BMD group than in the DMD group (median scores 4.5 and 9, respectively; p < 0.001). In the BMD group, total VDS score significantly correlated with Swinyard stage (r = 0.78, p < 0.001), but not with age or lung function. Patients with BMD have swallowing problems similar to those observed in patients with DMD when matched according to physical functional status. These patients should be evaluated and followed-up for the duration of their disease. Implications for rehabiliation Dysphagia is one of the most critical problems in patients with progressive neuromuscular disease but dysphagia in patients with Becker muscular dystrophy (BMD) was not well known. Eighteen patients with BMD and 18 patients with Duchenne muscular dystrophy were examined with videofluorography. Patients with BMD have swallowing problems similar to those observed in patients with DMD.

Romantic Resonances in the Rhetoric of Peter Elbow's "Writing Without Teachers."

ERIC Educational Resources Information Center

Yager, Kristi

1996-01-01

Seeks to uncover the historical roots within English Romanticism of Peter Elbow's thinking in order to show the depth and complexity of his assumptions about writing. Implicitly refutes charges questioning the intellectual credibility of Elbow's work. (TB)

Knowledge of Carrier Status and Barriers to Testing among Mothers of Sons with Duchenne or Becker Muscular Dystrophy

PubMed Central

Bogue, Lauren; Peay, Holly; Martin, Ann; Lucas, Ann; Ramchandren, Sindhu

2016-01-01

Our study objective was to survey female carriers for Duchenne and Becker muscular dystrophy to identify barriers to carrier testing and the impact of carrier risk knowledge on cardiac and reproductive health management. We surveyed women who have or had biological sons with Duchenne or Becker muscular dystrophy and were enrolled in the US DuchenneConnect patient registry, with questions assessing knowledge of carrier status and recurrence risk, knowledge of care standards for carriers, and barriers to testing. Of the 182 eligible respondents, 25% did not know their carrier status and 14% incorrectly classified themselves as not at risk. Cost of testing was the most commonly identified barrier to testing. Women reporting unknown carrier status were 13 times as likely to express uncertainty regarding their recurrence risk compared to women reporting positive carrier status. 37% of women at an increased risk for cardiomyopathy had never had an echocardiogram. Women who were certain of their positive carrier status were twice as likely to have had an echocardiogram in the last five years compared to women with unknown carrier status. Future research on reducing barriers to counseling and carrier testing, such as cost, may improve care standard adherence. PMID:27863875

A Functional Central Limit Theorem for the Becker-Döring Model

NASA Astrophysics Data System (ADS)

Sun, Wen

2018-04-01

We investigate the fluctuations of the stochastic Becker-Döring model of polymerization when the initial size of the system converges to infinity. A functional central limit problem is proved for the vector of the number of polymers of a given size. It is shown that the stochastic process associated to fluctuations is converging to the strong solution of an infinite dimensional stochastic differential equation (SDE) in a Hilbert space. We also prove that, at equilibrium, the solution of this SDE is a Gaussian process. The proofs are based on a specific representation of the evolution equations, the introduction of a convenient Hilbert space and several technical estimates to control the fluctuations, especially of the first coordinate which interacts with all components of the infinite dimensional vector representing the state of the process.

The Possibilities of Transformation: Critical Research and Peter McLaren

ERIC Educational Resources Information Center

Porfilio, Brad J.

2006-01-01

The purpose of this paper is to unveil how Peter McLaren's revolutionary brand of pedagogy, multiculturalism, and research colored my two-year qualitative research study, which unearthed twenty White female future teachers' experiences and perceptions in relationship to computing technology and male-centered computing culture. His ideas positioned…

50 years of comparative biochemistry: The legacy of Peter Hochachka.

PubMed

Buck, L T; Burness, G; Campbell, K L; Darveau, C-A; Driedzic, W; Guderley, H; McClelland, G B; Moon, T W; Moyes, C D; Schulte, P M

2018-02-28

Peter Hochachka was an early pioneer in the field of comparative biochemistry. He passed away in 2002 after 4 decades of research in the discipline. To celebrate his contributions and to coincide with what would have been his 80th birthday, a group of his former students organized a symposium that ran as a satellite to the 2017 Canadian Society of Zoologists annual meeting in Winnipeg, Manitoba (Canada). This Special Issue of CBP brings together manuscripts from symposium attendees and other authors who recognize the role Peter played in the evolution of the discipline. In this article, the symposium organizers and guest editors look back on his career, celebrating his many contributions to research, acknowledging his role in training of generations of graduate students and post-doctoral fellows in comparative biochemistry and physiology. Copyright © 2018. Published by Elsevier Inc.

Is mammary reconstruction with the anatomical Becker expander a simple procedure? Complications and hidden problems leading to secondary surgical procedures: a follow-up study.

PubMed

Farace, Francesco; Faenza, Mario; Bulla, Antonio; Rubino, Corrado; Campus, Gian Vittorio

2013-06-01

Debate over the role of Becker expander implants (BEIs) in breast reconstruction is still ongoing. There are no clear indications for BEI use. The main indications for BEI use are one-stage breast reconstruction procedure and congenital breast deformities correction, due to the postoperative ability to vary BEI volume. Recent studies showed that BEIs were removed 5 years after mammary reconstruction in 68% of operated patients. This entails a further surgical procedure. BEIs should not, therefore, be regarded as one-stage prostheses. We performed a case-series study of breast reconstructions with anatomically shaped Becker-35™ implants, in order to highlight complications and to flag unseen problems, which might entail a second surgical procedure. A total of 229 patients, reconstructed from 2005 to 2010, were enrolled in this study. Data relating to implant type, volume, mean operative time and complications were recorded. All the patients underwent the same surgical procedure. The minimum follow-up period was 18 months. During a 5-year follow-up, 99 patients required secondary surgery to correct their complications or sequelae; 46 of them underwent BEI removal within 2 years of implantation, 56 within 3 years, 65 within 4 years and 74 within 5 years. Our findings show that two different sorts of complications can arise with these devices, leading to premature implant removal, one common to any breast implant and one peculiar to BEIs. The Becker implant is a permanent expander. Surgeons must, therefore, be aware that, once positioned, the Becker expander cannot be adjusted at a later date, as in two-stage expander/prosthesis reconstructions for instance. Surgeons must have a clear understanding of possible BEI complications in order to be able to discuss these with their patients. Therefore, only surgeons experienced in breast reconstruction should use BEIs. Copyright © 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by

Peter Bergmann:The Education of a Physicist

NASA Astrophysics Data System (ADS)

Halpern, Paul

2005-12-01

I explore the early life and contributions of Peter Bergmann (1915 2002), focusing on his family background, education, and ideas. I examine how Bergmann’s formative years were shaped by the outspoken influence of his mother, a leading educational reformer; the distinguished reputation of his father, a renowned materials chemist; and his cherished hope of working with Albert Einstein (1879 1955), to whom he eventually became an assistant. Inspired by these and other notable thinkers, Bergmann became an exemplary organizer, educator, and mentor in the fields of general relativity and quantum gravity.

Interview with Peter McLaren, on His Work, on His Visit to Turkey and on Ongoing Popular Struggles

ERIC Educational Resources Information Center

Fassbinder, Samuel Day

2013-01-01

Peter McLaren is, as the back cover of his (2005) Capitalists & Conquerors: a critical pedagogy against empire says, "professor of urban education at the Graduate School of Education and Information Studies at the University of California, Los Angeles". Peter is also now a Distinguished Fellow in Critical Studies at Chapman…

Advocating Science for All: An Interview with Peter J. Fensham

ERIC Educational Resources Information Center

Cardellini, Liberato

2013-01-01

After providing some glimpses of his private life, Peter Fensham, a leading figure of the prestigious Faculty of Education, Monash University (and now emeritus professor at Queensland University, Brisbane, Australia), gives some suggestions about the conditions that help students to learn meaningfully. He began his career in the field of physical…

Perspective on Open-Access Publishing: An Interview with Peter Suber

ERIC Educational Resources Information Center

Cornwell, Reid; Suber, Peter

2008-01-01

In this edition of Perspectives, Reid Cornwell discusses open-access publishing with Peter Suber, senior researcher at the Scholarly Publishing and Academic Resources Coalition, senior research professor of philosophy at Earlham College, and currently visiting fellow at Yale Law School. Open access means that scholarly work is freely and openly…

Theories of Power, Poverty, and Law: In Commemoration of the Contributions of Peter Bachrach--Empowerment and Poverty

ERIC Educational Resources Information Center

Levi, Margaret

2010-01-01

My subject is the way Peter Bachrach addressed the question of poverty. But given that I was his student and research assistant and that I am a political scientist largely because of him, it is impossible to resist a few words about the experience of being in his classrooms. Peter was an inspiring teacher of politics. The substantive content of…

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

USDA-ARS?s Scientific Manuscript database

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...

Influence of depositional environment on diagenesis in St. Peter sandstone, Michigan basin

DOE Office of Scientific and Technical Information (OSTI.GOV)

Lundgren, C.E. Jr.; Barnes, D.A.

1989-03-01

The Middle Ordovician St. Peter Sandstone in the Michigan basin was deposited in marine peritidal to storm-dominated, outer shelf depositional environments that evolved in a regionally significant transgressive pattern. The formation is bounded by carbonate and shaly clastic strata of the Prairie du Chien Group below and is transitional to condensed sequence clastics and carbonates of the Glenwood Formation above. Sedimentologic and petrographic analysis of conventional core from 25 wells suggests that reservoir quality in the formation is strongly dependent on a complex diagenetic history, especially the nature and subsequent dissolution of intergranular carbonate in the sandstone. Petrographic evidence indicatesmore » that porosity in the formation formed by dissolution of precursor dolomite of various origins and, locally, the formation of pore-filling authigenic clay (chlorite-illite). Authigenic clay is the incongruent dissolution product of dolomite, detrital K-feldspar, and, possibly, muscovite and results in diminished reservoir quality where abundant in the St. Peter Sandstone. Authigenic clay is volumetrically more significant in the upper portions of the formation and is associated with higher concentrations of detrital K-feldspar. Depositional facies controlled the distribution and types of intergranular carbonate (now dolomite) and detrital K-feldspar in the St. Peter Sandstone and hence reservoir quality; both components were more significant in storm-shelf sandstone facies.« less

Bibliography of NBDL (Naval Biodynamics Laboratory) Publications,

DTIC Science & Technology

1987-01-28

of Pro- ceedings of 9th International Conference in Engineering on Medicine and Biology , Melbourne, Australia, Aug 1971, 154 pp. 3.15 Ewing, C. L...75thFAnnii1"onfer6nce on Engineerng in Medicine and Biology . Miami Beach, FL, 4 Oct 1972, 241 pp. 3.23 Becker, E. B. Measurement of Mass Distribution...Pepper, R. L. & Wiker, S. F. Perfonnance Evaluation Tests for Environmental Researh (PETER): Collected Papers. NBUL Research Report, NBUL-8URO(8

An interview with Peter H. Buschang.

PubMed

Buschang, Peter H; dos Santos-Pinto, Ary; Araújo, Eustáquio; Ribeiro, Gerson Luiz Ulema; Jacob, Helder Baldi; Gandini Júnior, Luiz Gonzaga

2014-01-01

Dr. Peter Buschang is regent professor and director of orthodontic research. He has been at Texas A&M University Baylor College of Dentistry since 1988. Dr. Buschang received his PhD in 1980 from the University of Texas at Austin; he spent 3 years as a NIDR postdoctoral fellow at the University of Connecticut, and five years as a FRSQ scholar at the University of Montreal. Every year, Dr. Buschang teaches in 16 different courses, 7 of which he directs. In addition to more than 100 lecture hours per year, he spends hundreds of hours mentoring students. For his teaching efforts, Dr. Buschang was awarded the Robert E. Gaylord Award of Excellence in Orthodontic education in 1992, 1998, 2004, and 2010. He also gives 1-2 day evidence-based CE courses throughout the world. The residents he has taught recently honored him by pledging to fund the Peter H. Buschang Endowed Professorship of Orthodontics. His research interests pertain to craniofacial growth and assessment of treatment effects. Dr. Buschang has been funded regularly over the years by the Medical Research Council of Canada, Fonds de le Recherche en Santé du Québec, the NIH, and the American Association of Orthodontics Foundation. He has mentored over 140 Master's and PhD students, and 49 dental students. Dr. Buschang has published over 250 peer-reviewed articles, 15 book chapters and 198 abstracts. He has given over 150 invited talks and lectures in 14 different countries. For his work with the American Board of Orthodontics, Dr. Buschang was awarded the Earl E. and Wilma S. Shepard Award. Dr. Buschang is the only non-orthodontist ever to have been made an honorary member of both the American Association of Orthodontics (2005) and the Edward H. Angle Society of Orthodontics (2009), the two most prestigious orthodontic groups.

Follistatin Gene Therapy Improves Ambulation in Becker Muscular Dystrophy

PubMed Central

Al-Zaidy, Samiah A.; Sahenk, Zarife; Rodino-Klapac, Louise R.; Kaspar, Brian; Mendell, Jerry R.

2015-01-01

Abstract Follistatin is a ubiquitous secretory propeptide that functions as a potent inhibitor of the myostatin pathway, resulting in an increase in skeletal muscle mass. Its ability to interact with the pituitary activin-inhibin axis and suppress the secretion of follicle-stimulating hormone (FSH) called for caution in its clinical applicability. This limitation was circumvented by the use of one of the alternatively spliced follistatin variants, FS344, undergoing post-translational modification to FS315. This follistatin isoform is serum-based, and has a 10-fold lower affinity to activin compared to FS288. Preclinical studies of intramuscular delivery of the follistatin gene demonstrated safety and efficacy in enhancing muscle mass. We herein review the evidence supporting the utility of follistatin as a genetic enhancer to improve cellular performance. In addition, we shed light on the results of the first clinical gene transfer trial using the FS344 isoform of follistatin in subjects with Becker muscular dystrophy as well as the future directions for clinical gene therapy trials using follistatin. PMID:27858738

Follistatin Gene Therapy Improves Ambulation in Becker Muscular Dystrophy.

PubMed

Al-Zaidy, Samiah A; Sahenk, Zarife; Rodino-Klapac, Louise R; Kaspar, Brian; Mendell, Jerry R

2015-09-02

Follistatin is a ubiquitous secretory propeptide that functions as a potent inhibitor of the myostatin pathway, resulting in an increase in skeletal muscle mass. Its ability to interact with the pituitary activin-inhibin axis and suppress the secretion of follicle-stimulating hormone (FSH) called for caution in its clinical applicability. This limitation was circumvented by the use of one of the alternatively spliced follistatin variants, FS344, undergoing post-translational modification to FS315. This follistatin isoform is serum-based, and has a 10-fold lower affinity to activin compared to FS288. Preclinical studies of intramuscular delivery of the follistatin gene demonstrated safety and efficacy in enhancing muscle mass. We herein review the evidence supporting the utility of follistatin as a genetic enhancer to improve cellular performance. In addition, we shed light on the results of the first clinical gene transfer trial using the FS344 isoform of follistatin in subjects with Becker muscular dystrophy as well as the future directions for clinical gene therapy trials using follistatin.

New Myositis Classification Criteria-What We Have Learned Since Bohan and Peter.

PubMed

Leclair, Valérie; Lundberg, Ingrid E

2018-03-17

Idiopathic inflammatory myopathy (IIM) classification criteria have been a subject of debate for many decades. Despite several limitations, the Bohan and Peter criteria are still widely used. The aim of this review is to discuss the evolution of IIM classification criteria. New IIM classification criteria are periodically proposed. The discovery of myositis-specific and myositis-associated autoantibodies led to the development of clinico-serological criteria, while in-depth description of IIM morphological features improved histopathology-based criteria. The long-awaited European League Against Rheumatism and American College of Rheumatology (EULAR/ACR) IIM classification criteria were recently published. The Bohan and Peter criteria are outdated and validated classification criteria are necessary to improve research in IIM. The new EULAR/ACR IIM classification criteria are thus a definite improvement and an important step forward in the field.

Health services received by individuals with duchenne/becker muscular dystrophy.

PubMed

Pandya, Shree K; Campbell, Kim A; Andrews, Jennifer G; Meaney, F John; Ciafaloni, Emma

2016-02-01

Anecdotal reports from families and care providers suggest a wide variation in services received by individuals with Duchenne/Becker muscular dystrophy (DBMD). We documented the type and frequency of health services received by individuals with DBMD using the Muscular Dystrophy Surveillance Tracking and Research Network (MD STARnet) interview data released in June 2012. Interviews with eligible caregivers from 5 sites (Arizona, Colorado, Georgia, Iowa, and western New York) were conducted from April 2007 to March 2012. Two hundred ninety-six caregivers (66% of those contactable) participated in the interview. There were significant differences among sites in the specialists seen and services received. Concurrence with cardiac recommendations was higher than that with respiratory recommendations. The results of this survey support and quantify the anecdotal reports from families and care providers regarding the disparities in services received by individuals with DBMD. It remains to be determined whether these differences affect outcomes. © 2015 Wiley Periodicals, Inc.

The Contribution of Iona and Peter Opie to Children's Literature.

ERIC Educational Resources Information Center

McKinney, Barbara J.

Iona and Peter Opie's contribution to children's literature began with a volume on the origins of nursery rhymes. A nursery rhyme tells a brief and memorable surrealist story about people, animals, or familiar activities. Most nursery rhymes were not originally composed for children but were fragments of ballads or folk songs, remnants of ancient…

Knowledge of carrier status and barriers to testing among mothers of sons with Duchenne or Becker muscular dystrophy.

PubMed

Bogue, Lauren; Peay, Holly; Martin, Ann; Lucas, Ann; Ramchandren, Sindhu

2016-12-01

Our study objective was to survey female carriers for Duchenne and Becker muscular dystrophy to identify barriers to carrier testing and the impact of carrier risk knowledge on cardiac and reproductive health management. We surveyed women who have or had biological sons with Duchenne or Becker muscular dystrophy and were enrolled in the US DuchenneConnect patient registry, with questions assessing knowledge of carrier status and recurrence risk, knowledge of care standards for carriers, and barriers to testing. Of the 182 eligible respondents, 25% did not know their carrier status and 14% incorrectly classified themselves as not at risk. Cost of testing was the most commonly identified barrier to testing. Women reporting unknown carrier status were 13 times as likely to express uncertainty regarding their recurrence risk compared to women reporting positive carrier status. 37% of women at an increased risk for cardiomyopathy had never had an echocardiogram. Women who were certain of their positive carrier status were twice as likely to have had an echocardiogram in the last five years compared to women with unknown carrier status. Future research on reducing barriers to counseling and carrier testing, such as cost, may improve care standard adherence. Copyright © 2016 Elsevier B.V. All rights reserved.

An Intimate Portrait of Evaluation Mentorship under Peter H. Rossi

ERIC Educational Resources Information Center

Chen, Huey T.

2007-01-01

The author grew up in Taiwan and in 1995 enrolled in Kent State University's PhD program in sociology. Early in his doctoral career at Kent State, he took a program evaluation course offered by Irwin Deutscher. The textbook he used in the class was Peter Rossi and Walter Williams's 1972 book, "Evaluating Social Programs: Theory, Practice, and…

Shaping the Global Civil Society: An Interview with Michael Peters

ERIC Educational Resources Information Center

Heraud, Richard; Tesar, Marek

2017-01-01

Professor Michael A. Peters has worked in an era of transformation that has taken him from a labour-intensive paper-based form of production to the computerised reproduction of thought, and the current shift in the publishing landscape from a reader-subscription to an author-pays model. Most of what he has learned in publishing and editing he has…

Performance Evaluation Tests for Environmental Research (PETER): evaluation of 114 measures

NASA Technical Reports Server (NTRS)

Bittner, A. C. Jr; Carter, R. C.; Kennedy, R. S.; Harbeson, M. M.; Krause, M.

1986-01-01

The goal of the Performance Evaluation Tests for Environmental Research (PETER) Program was to identify a set of measures of human capabilities for use in the study of environmental and other time-course effects. 114 measures studied in the PETER Program were evaluated and categorized into four groups based upon task stability and task definition. The Recommended category contained 30 measures that clearly obtained total stabilization and had an acceptable level of reliability efficiency. The Acceptable-But-Redundant category contained 15 measures. The 37 measures in the Marginal category, which included an inordinate number of slope and other derived measures, usually had desirable features which were outweighed by faults. The 32 measures in the Unacceptable category had either differential instability or weak reliability efficiency. It is our opinion that the 30 measures in the Recommended category should be given first consideration for environmental research applications. Further, it is recommended that information pertaining to preexperimental practice requirements and stabilized reliabilities should be utilized in repeated-measures environmental studies.

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

PubMed

Janghra, Narinder; Morgan, Jennifer E; Sewry, Caroline A; Wilson, Francis X; Davies, Kay E; Muntoni, Francesco; Tinsley, Jonathon

2016-01-01

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 tools to quantify

Diagenetic controls on reservoir heterogeneity in St. Peter Sandstone, deep Michigan basin

DOE Office of Scientific and Technical Information (OSTI.GOV)

Barnes, D.A.; Turmelle, T.M.; Adam, R.

1989-03-01

The St. Peter Sandstone is a highly productive gas and condensate reservoir throughout the central part of the Michigan basin. Production occurs in several intervals: a laterally continuous zone at the top of the formation typified in the Woodville, Falmouth, and Rose City fields and less continuous intervals lower in the formation typified in the Ruwe Gulf zone of the Reed City field. Porosity is not limited to hydrocarbon productive zones, however. Diagenesis has dramatically modified primary mineralogy and textures in the formation. Dominant diagenetic components are quartz, dolomite, and clay authigenic cements, extensive chemical compaction, and pervasive mineral leaching.more » Their model for sandstone diagenesis is consistent throughout the basin. Variation in the significance of these diagenetic components is strongly templated by stratigraphically predictable facies variations within the St. Peter Sandstone.« less

Globalisation in Africa: Reflecting on Peter Jarvis's Superstructure and Substructure Model

ERIC Educational Resources Information Center

Preece, Julia

2017-01-01

This paper reflects on Peter Jarvis' book "Globalisation, lifelong learning and the learning society," volume 2--in which he describes human learning within a global context and factors contributing to globalisation. He describes the relationship of power between countries manifested as the superstructure and sub structure. The paper…

Idiopathic inflammatory myopathies in adults: A comparative study of Bohan and Peter and European Neuromuscular Center 2004 criteria.

PubMed

Challa, Sundaram; Jakati, Saumya; Uppin, Megha S; Kannan, Meena A; Liza, Rajasekhar; Murthy Jagarlapudi, M K

2018-01-01

Bohan and Peter criteria are widely used for the diagnosis of idiopathic inflammatory myopathies (IIMs). Recently, European Neuromuscular Center (ENMC) formulated criteria to identify subgroups of IIMs. To compare the two diagnostic criteria in adult IIMs. This was a retrospective review of case records of histologically confirmed IIMs in adults between January 2014 and May 2015. Both the Bohan and Peter, and ENMC 2004 criteria were applied in the same group of patients to subgroup the IIMs. Muscle biopsy was evaluated in all the four domains: muscle fiber, inflammatory, connective tissue, and vascular, with the basic panel of histological stains. Sporadic inclusion body myositis (s-IBM) was diagnosed using ENMC IBM diagnostic research criteria 2011. During the study period, 69 patients fulfilled the ENMC criteria for IIMs including 16 patients with s-IBM. The subgrouping as per the ENMC criteria (53) was: dermatomyositis (DM) in 30; polymyositis (PM) in 2; immune-mediated necrotizing myopathy (IMNM) in 9; and nonspecific myositis (NM) in 12 patients, whereas subgrouping by the Bohan and Peter criteria was DM in 9 and PM with and without connective tissue disease (CTD) in 26 patients only. There was underdiagnosis of DM, as perifascicular atrophy is not recognized as a diagnostic histological feature, and overdiagnosis of PM with and without CTD due to poor characterization of histological features in PM by the Bohan and Peter criteria. Systematic evaluation of muscle biopsy according to the ENMC criteria with basic panel of histochemical stains improved the diagnostic yield of IIM significantly when compared to the Bohan and Peter criteria.

Behavioral Determinants of Drug Action: The Contributions of Peter B. Dews

ERIC Educational Resources Information Center

Barrett, James E.

2006-01-01

Peter B. Dews played a significant role in shaping the distinctive characteristics and defining the underlying principles of the discipline of behavioral pharmacology. His early and sophisticated use of schedules of reinforcement in the 1950s, incorporated from research in the experimental analysis of behavior and integrated into the discipline of…

The Peter Effect Revisited: Reading Habits and Attitudes of College Students

ERIC Educational Resources Information Center

Applegate, Anthony J.; Applegate, Mary DeKonty; Mercantini, Martha A.; McGeehan, Catherine M.; Cobb, Jeanne B.; DeBoy, Joanne R.; Modla, Virginia B.; Lewinski, Kimberly E.

2014-01-01

Certainly a primary goal of literacy education is the creation of avid, enthusiastic, and highly motivated readers. However, in this article revisiting the Peter Effect (Applegate & Applegate, 2004), researchers surveyed more than 1,000 college sophomores and found strikingly low levels of enthusiasm for reading. Only 46.6% of surveyed…

QMHC interview: Peter R. Scholtes [by Marie E. Sinioris].

PubMed

Scholtes, P R

1993-01-01

Peter R. Scholtes has a unique perspective on what it takes to build a world-class quality organization: A transformation of the relationships, environment, and dynamics within and between individuals and groups throughout an organization. He brings an organizational development perspective to quality management and, in particular, to the approach and practices advocated by W. Edwards Deming. This interview explores Mr. Scholtes' in-depth understanding and sometimes controversial views on quality improvement teams, team training, and performance appraisal.

Is R. S. Peters' Way of Mentioning Women in His Texts Detrimental to Philosophy of Education? Some Considerations and Questions

ERIC Educational Resources Information Center

Lees, Helen E.

2012-01-01

Discussion in this article considers the unfortunate way R.S. Peters made mention of women when it was pertinent to his argumentation: portraying them, directly or indirectly, as abuse-able (murderable), deficient, aberrant, clueless and inconstant. It is argued that the high profile and esteem within which Peter's texts are held within philosophy…

The Place of Philosophy in the Training of Teachers: Peters Revisited

ERIC Educational Resources Information Center

Clark, John A.

2013-01-01

In 1964, Richard Peters examined the place of philosophy in the training of teachers. He considered three things: Why should philosophy of education be included in the training of teachers; What portion of philosophy of education should be included; How should philosophy be taught to those training to be teachers. This article explores the context…

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

PubMed

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.

Instruction in the Making: Peter Ramus and the Beginnings of Modern Schooling.

ERIC Educational Resources Information Center

Hamilton, David

This paper examines educational practice between 1450-1650, highlighting 16th century educationist, Peter Ramus, whose work is remembered in the "History of Western Philosophy" as fostering a "pedagogic marvel." Ramus' work has received scant attention from English-speaking educationists, and his niche within the educational…

Remaking Critical Pedagogy: Peter McLaren's Contribution to a Collective Work

ERIC Educational Resources Information Center

Martin, Gregory

2006-01-01

This article explores the collective works of Peter McLaren and his contribution to critical pedagogy within the field of education and beyond the academy. To understand how McLaren's work took a radical turn in the 1990s, the article traces the historical development of his praxis. In particular, McLaren's engagement with the postmodern Left and…

Implications of R. S. Peters' Notion of "Cognitive Perspective" for Science Education

ERIC Educational Resources Information Center

Hadzigeorgiou, Yannis

2017-01-01

This paper discusses R.S. Peters' notion of "cognitive perspective," which, through careful reading, can be interpreted as a wider perspective resulting from an awareness of the relationships of one's knowledge and understanding to one's own life. This interpretation makes cognitive perspective a holistic notion akin to that of…

"Staying on Task": What Constitutes Classwork in Peter Medway's "Finding a Language"?

ERIC Educational Resources Information Center

Pangilinan, J. P.

2015-01-01

In "Finding a Language", Peter Medway addresses questions of central importance to English teaching--questions of curriculum and of pedagogy. How he addresses these questions provides a sharp contrast to the current orthodoxies of a rigidly prescriptive national curriculum and a closely monitored regime of objective-led, skills-based…

Rehabilitation: disability ethics versus Peter Singer.

PubMed

McPherson, Gary W; Sobsey, Dick

2003-08-01

This commentary discusses utilitarian bioethics currently articulated by Peter Singer, one of the most widely known bioethicists of the 21st century. His controversial concepts of personhood and replaceability have important implications for people with disabilities, caregivers, and rehabilitation in general. Singer suggests that people with severe disabilities should not be considered persons and therefore have no rights or status in ethical issues. In addition, he argues that, although some people with less severe disabilities qualify as persons, their potential quality of life is significantly compromised by disability and therefore it may be ethically desirable to eliminate them so that their resources can be used for someone with superior potential. We reject these ideas, and suggest that those involved in rehabilitation carefully consider these ideas because they imply that rehabilitation is an immoral act unless it results in full and typical function.

Correlates of care for young men with Duchenne and Becker muscular dystrophy.

PubMed

Andrews, Jennifer G; Davis, Melinda F; Meaney, F John

2014-01-01

In progressive conditions, such as Duchenne and Becker muscular dystrophy (DBMD), the need for care may outpace care use. We examined correlates that contribute to utilization of needed care. Structured interviews were conducted on use of care among 34 young men with DBMD who were born before 1982. Disease severity, per capita income, and presence of other relatives with DBMD predicted greater use of services. Race/ethnicity, acculturation, and level of caregiver education did not significantly predict service utilization. We identified disparities in receipt of healthcare and related services in adult men with DBMD that can affect quality of life. Despite the high disease severity identified in this population, these men utilized only half of the services available to individuals with significant progressive conditions. Providers should be aware of low service utilization and focus on awareness and assistance to ensure access to available care. Copyright © 2013 Wiley Periodicals, Inc.

Simulation of ground-water flow in the St. Peter aquifer in an area contaminated by coal-tar derivatives, St. Louis Park, Minnesota. Water Resources Investigation

DOE Office of Scientific and Technical Information (OSTI.GOV)

Lorenz, D.L.; Stark, J.R.

1990-01-01

A model constructed to simulate ground-water flow in part of the Prairie du Chien-Jordan and St. Peter aquifers, St. Louis Park, Minnesota, was used to test hypotheses about the movement of ground water contaminated with coal-tar derivatives and to simulate alternatives for reducing the downgradient movement of contamination in the St. Peter aquifer. The model, constructed for a previous study, was applied to simulate the effects of current ground-water withdrawals on the potentiometric surface of the St. Peter aquifer. Model simulations predict that the multiaquifer wells have the potential to limit downgradient migration of contaminants in the St. Peter aquifermore » caused by cones of depression created around the multiaquifer wells. Differences in vertical leakage to the St. Peter aquifer may exist in areas of bedrock valleys. Model simulations indicate that these differences are not likely to affect significantly the general patterns of ground-water flow.« less

14. Photocopy of a photographca. 1902taken by A.W. Peters showing ...

Library of Congress Historic Buildings Survey, Historic Engineering Record, Historic Landscapes Survey

14. Photocopy of a photograph--ca. 1902--taken by A.W. Peters showing Eastwood and his party surveying for the Big Creek Project. The surveying party is visible in the upper right corner. Courtesy Mr. Charles Allan Whitney. - Little Rock Creek Dam, Little Rock Creek, Littlerock, Los Angeles County, CA

The Incompatibility of Punishment and Moral Education: A Reply to Peter Hobson.

ERIC Educational Resources Information Center

Marshall, James D.

1989-01-01

Responds to Peter Hobson's assertions concerning the relationship of punishment and moral education. Draws upon the writings of Michael Foucoult in suggesting that punishment in the legal sense does not fit well with efforts to develop rational autonomy. Suggests that traditional talk of punishment obscures the reality of practice. (KO)

Embracing the Humanistic Vision: Recurrent Themes in Peter Roberts' Recent Writings

ERIC Educational Resources Information Center

Reveley, James

2018-01-01

Running like a leitmotif through Peter Roberts' recently published philosophico-educational writings there is a humanistic thread, which this article picks out. In order to ascertain the quality of this humanism, Roberts is positioned in relation to a pair of extant humanisms: radical and integral. Points of comparability and contrast are…

Guidelines for the Perianesthesia Care of the Duchenne Muscular Dystrophy/Becker Muscular Dystrophy Patient.

PubMed

Alliod, Barbara A; Ash, Rebecca A

2016-12-01

More patients suffering with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are presenting to perianesthesia settings for emergent and nonemergent treatment and care. A group of collaborative health care providers at Rush University Medical Center in Chicago developed a multidisciplinary DMD/BMD Task Force to study this disorder and create a set of guidelines to aid those engaging in the planning, execution of care, and recovery of this unique population in the perianesthesia setting. Attention to detail, well-executed preplanning, meticulous awareness of the patient, and prearranged implementation and intervention has proven to offset potential problems and complications and is the key to a successful perianesthesia period. Copyright © 2016 American Society of PeriAnesthesia Nurses. Published by Elsevier Inc. All rights reserved.

The Authority of Truth: Religion and the John Peter Zenger Case.

ERIC Educational Resources Information Center

Nord, David Paul

An appreciation of the religious milieu of the John Peter Zenger libel case of 1735 can help explain the nature of the Zenger defense as prepared by Alexander Hamilton, the meaning of the jury's verdict, and the ambiguous legacy of the trial for freedom of expression in the United States. In essence, the case was a disputation on "truth"…

Teaching Behavior Analysis and Psychology in Social Context: An Interview with Peter Harzem.

ERIC Educational Resources Information Center

Buskist, William

2000-01-01

Presents an interview with Peter Harzem, the Hudson Professor of Psychology at Auburn University (Alabama). Addresses such issues as teaching students about behavior analysis, the differences between classical and operant conditioning, reinforcement and behaviorism, and how one can become a better teacher. (CMK)

Duchenne and Becker muscular dystrophy in adolescents: current perspectives

PubMed Central

Andrews, Jennifer G; Wahl, Richard A

2018-01-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are life-limiting and progressive neuromuscular conditions with significant comorbidities, many of which manifest during adolescence. BMD is a milder presentation of the condition and much less prevalent than DMD, making it less represented in the literature, or more severely affected individuals with BMD may be subsumed into the DMD population using clinical cutoffs. Numerous consensus documents have been published on the clinical management of DMD, the most recent of which was released in 2010. The advent of these clinical management consensus papers, particularly respiratory care, has significantly increased the life span for these individuals, and the adolescent years are now a point of transition into adult lives, rather than a period of end of life. This review outlines the literature on DMD and BMD during adolescence, focusing on clinical presentation during adolescence, impact of living with a chronic illness on adolescents, and the effect that adolescents have on their chronic illness. In addition, we describe the role that palliative-care specialists could have in improving outcomes for these individuals. The increasing proportion of individuals with DMD and BMD living into adulthood underscores the need for more research into interventions and intracacies of adolescence that can improve the social aspects of their lives. PMID:29588625

Duchenne and Becker muscular dystrophy in adolescents: current perspectives.

PubMed

Andrews, Jennifer G; Wahl, Richard A

2018-01-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are life-limiting and progressive neuromuscular conditions with significant comorbidities, many of which manifest during adolescence. BMD is a milder presentation of the condition and much less prevalent than DMD, making it less represented in the literature, or more severely affected individuals with BMD may be subsumed into the DMD population using clinical cutoffs. Numerous consensus documents have been published on the clinical management of DMD, the most recent of which was released in 2010. The advent of these clinical management consensus papers, particularly respiratory care, has significantly increased the life span for these individuals, and the adolescent years are now a point of transition into adult lives, rather than a period of end of life. This review outlines the literature on DMD and BMD during adolescence, focusing on clinical presentation during adolescence, impact of living with a chronic illness on adolescents, and the effect that adolescents have on their chronic illness. In addition, we describe the role that palliative-care specialists could have in improving outcomes for these individuals. The increasing proportion of individuals with DMD and BMD living into adulthood underscores the need for more research into interventions and intracacies of adolescence that can improve the social aspects of their lives.

[Award of the Salomon-Neumann-Medal 2017 - Speech of the Laureate Prof. Bernt-Peter Robra, 5 September 2017, St. Peter´s Church Lübeck].

PubMed

Robra, Bernt-Peter

2018-02-19

The Salomon-Neumann-Medal 2017 of the German Society for Social Medicine and Prevention (DGSMP) was awarded to Bernt-Peter Robra, Institute for Social Medicine and Health Economics (ISMG) of the Otto von Guericke University Magdeburg. The person and scientific merits of Manfred Pflanz are valued and topics of the masterplan2020-process are highlighted, that offer chances for developments in medicine and public health. © Georg Thieme Verlag KG Stuttgart · New York.

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

DOE Office of Scientific and Technical Information (OSTI.GOV)

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.

STS-81 Mission Specialist Peter Wisoff suits up

NASA Technical Reports Server (NTRS)

1997-01-01

STS-81 Mission Specialist Peter J. K. 'Jeff' Wisoff prepares for the fifth Shuttle- Mir docking as he waits in the Operations and Checkout (O&C) Building for the operation to fit him into his launch/entry suit to be completed. He conducted a spacewalk on his on his first Shuttle mission, STS- 57 and holds a doctorate degree in applied physics with an emphasis on lasers and semiconductor materials. He and five crew members will shortly depart the O&C and head for Launch Pad 39B, where the Space Shuttle Atlantis will lift off during a 7-minute window that opens at 4:27 a.m. EST, January 12.

Communicating Chemistry from "Molecules" to International Efforts: An Interview with Peter Atkins

ERIC Educational Resources Information Center

Cardellini, Liberato

2008-01-01

In this interview, Peter Atkins explains the deep motivations that compel him to sit at his desk at 6 AM writing books and textbooks. He discusses the four principal elements that help to make a chemistry textbook successful, including the secret ingredient. He also discusses the importance of problem solving, the interaction of multimedia, and…

How Relevant Is R. S. Peters' Conception of Education to Science Education?

ERIC Educational Resources Information Center

Hadzigeorgiou, Yannis; Stamatis, Panagiotis

2017-01-01

This paper discusses R. S. Peters' concept of education, particularly his notion of cognitive perspective and its relevance to school science education. In light of the problems inherent in any attempt to define the notion of scientific literacy, it is argued that the development of cognitive perspective can be considered an important, if not the…

Respectability and Relevance: Reflections on Richard Peters and Analytic Philosophy of Education

ERIC Educational Resources Information Center

Snook, Ivan

2013-01-01

I argue that, after Dewey, Peters was the first modern philosopher of education to write material (in English) that was both philosophically respectable and relevant to the day-to-day concerns of teachers. Since then, some philosophers of education have remained (more or less) relevant but not really respectable while others have "taken off into…

R. S. Peters and J. H. Newman on the Aims of Education

ERIC Educational Resources Information Center

Ozolins, Janis T.

2013-01-01

R. S. Peters never explicitly talks about wisdom as being an aim of education. He does, however, in numerous places, emphasize that education is of the whole person and that, whatever else it might be about, it involves the development of knowledge and understanding. Being educated, he claims, is incompatible with being narrowly specialized.…

Travels with Chairman of the Joint Chiefs of Staff, Marine General Peter

Science.gov Websites

that time. Story Counter-drug Efforts May Aid Afghanistan BOGOTA, Colombia, Jan. 20, 2007 – The long experience in its counter-drug effort to help the Afghan government fight a similar battle. " has working the counter-drug (issue)," Marine Gen. Peter Pace said. "It's been a very

Reason and Virtues: The Paradox of R. S. Peters on Moral Education

ERIC Educational Resources Information Center

Haydon, Graham

2009-01-01

This article examines the work of R. S. Peters on moral development and moral education, as represented in his papers collected under that name, pointing out that these writings have been relatively neglected. It approaches these writings through the lens of the "familiar story" that philosophical work on this topic switched during, roughly, the…

Critically Enlightened Romantic Values and English Pedagogy: A Response to Peter Medway

ERIC Educational Resources Information Center

Stevens, David

2011-01-01

In this response to Peter Medway's paper, "English and Enlightenment' (Changing English 17:1, 2010), I take issue with little of what he so lucidly writes, except his implicit and occasionally explicit denunciation of Romanticism as the proper basis of English pedagogy. I am concerned in this paper to emphasise the positive aspects of…

Longitudinal 2-point dixon muscle magnetic resonance imaging in becker muscular dystrophy.

PubMed

Bonati, Ulrike; Schmid, Maurice; Hafner, Patricia; Haas, Tanja; Bieri, Oliver; Gloor, Monika; Fischmann, Arne; Fischer, Dirk

2015-06-01

Quantitative MRI techniques detect disease progression in myopathies more sensitively than muscle function measures or conventional MRI. To date, only conventional MRI data using visual rating scales are available for measurement of disease progression in Becker muscular dystrophy (BMD). In 3 patients with BMD (mean age 36.8 years), the mean fat fraction (MFF) of the thigh muscles was assessed by MRI at baseline and at 1-year follow-up using a 2-point Dixon approach (2PD). The motor function measurement scale (MFM) was used for clinical assessment. The mean MFF of all muscles at baseline was 61.6% (SD 7.6). It increased by 3.7% to 65.3% (SD 4.7) at follow-up. The severity of muscle involvement varied between various muscle groups. As in other myopathies, 2PD can quantify fatty muscle degeneration in BMD and can detect disease progression in a small sample size and at relatively short imaging intervals. © 2015 Wiley Periodicals, Inc.

Late-onset Becker muscular dystrophy: Refining the clinical features and electrophysiological findings.

PubMed

Beltran Papsdorf, Tania; Howard, James F; Chahin, Nizar

2015-11-01

The aim of this study was to characterize a unique distribution of muscle involvement in sporadic Becker muscle dystrophy (BMD). Retrospective chart review, clinical examination, electrophysiological studies, cardiac testing, and genetic testing were performed in 5 patients. Predominant weakness and atrophy of biceps brachii, hip adduction, and quadriceps muscles was noted along with calf and extensor forearm hypertrophy. Finger flexor muscles were severely weak in 3 of 5 patients, a feature that could lead to a misdiagnosis of inclusion body myositis. Creatinine kinase was only mildly elevated in most patients. Electromyography was abnormal in all patients. Muscle biopsy in 1 patient demonstrated normal immunostaining for dystrophin. We found a unique and uniform distribution of muscle involvement in 5 sporadic cases of BMD. Recognizing these features is important for differentiating it from other myopathies that may have similar features and avoids unnecessary invasive procedures such as muscle biopsy. © 2015 Wiley Periodicals, Inc.

Calf muscle involvement in Becker muscular dystrophy: when size does not matter.

PubMed

Monforte, Mauro; Mercuri, Eugenio; Laschena, Francesco; Ricci, Enzo; Tasca, Giorgio

2014-12-15

Calf hypertrophy is a common feature in Becker muscular dystrophy (BMD), and it is still debated to which extent fatty degeneration or true muscle hypertrophy account for it. We wanted to investigate the relative contribution of these two components using a simple image analysis approach and their possible correlation with disease severity. Twenty-nine BMD patients' MRI scans were analyzed. A semiquantitative visual score assessing fatty replacement of calf muscles (calf MRI score, CMS) was calculated and correlated with the cross sectional area (CSA) of lower leg posterior compartment muscles, digitally measured on acquired images. The correlation between CSA and CMS was not significant. CMS in contrast correlated with disease severity (p<0.001) while CSA did not (p=0.969). In BMD, a major contribution to calf hypertrophy is provided by real muscle hypertrophy rather than by fatty degeneration. CMS appears to be a potential surrogate marker of disease severity. Copyright © 2014 Elsevier B.V. All rights reserved.

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

PubMed

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

2016-01-01

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. All requests for DNA analysis of the DMD gene in probands with suspected BMD were re-evaluated. If the phenotype was compatible with BMD, and no deletions or duplications were detected, DNA samples were screened for small mutations. In 79 of 185 referrals, no mutation was found. Analysis could be performed on 31 DNA samples. Seven different mutations, including 3 novel ones, were found. Long-term clinical follow-up is described. Refining DNA analysis in previously undiagnosed cases can identify mutations in the DMD gene and provide genetic diagnosis of BMD. A delayed diagnosis can still be valuable for the proband or the relatives of BMD patients. © 2015 Wiley Periodicals, Inc.

Prevalence and Characteristics of Chinese Patients With Duchenne and Becker Muscular Dystrophy

PubMed Central

Lo, Ivan F. M.; Cherk, Sharon W. W.; Cheng, Wai Wai; Fung, Eva L. W.; Yeung, Wai Lan; Ngan, Mary; Lee, Wing Cheong; Kwong, Ling; Wong, Suet Na; Ma, Che Kwan; Tai, Shuk Mui; Ng, Grace S. F.; Wu, Shun Ping; Wong, Virginia C. N.

2015-01-01

The aim of this collaborative study on Duchenne muscular dystrophy and Becker muscular dystrophy is to determine the prevalence and to develop data on such patients as a prelude to the development of registry in Hong Kong. Information on clinical and molecular findings, and patient care, was systematically collected in 2011 and 2012 from all Pediatric Neurology Units in Hong Kong. Ninety patients with dystrophinopathy were identified, and 83% has Duchenne muscular dystrophy. The overall prevalence of dystrophinopathy in Hong Kong in 2010 is 1.03 per 10 000 males aged 0 to 24 years. Among the Duchenne group, we observed a higher percentage (40.6%) of point mutations with a lower percentage (45.3%) of exon deletions in our patients when compared with overseas studies. Although we observed similar percentage of Duchenne group received scoliosis surgery, ventilation support, and cardiac treatment when compared with other countries, the percentage (25%) of steroid use is lower. PMID:28503591

John P. Peters and the committee of 430 physicians.

PubMed Central

Lundberg, George D.

2002-01-01

John Peters and his committee had a few basic goals. One was that local, state, and federal governments needed to provide money to construct facilities, support medical research and education, and care for the poor. And they wanted experts to call the shots. Over time, Peters and the committee got what they wanted for the most part: Hill-Burton money for building the hospitals, the rise of the National Institutes of Health, Medicare, Medicaid, a Veterans Administration system, and new and expanded medical schools. The experts calling the shots included David Kessler at the Food and Drug Administration and Surgeon General C. Everett Koop. In the halcyon days of American health system reform, back in 1993, Yale's Paul Beeson wrote about the Committee of 430 Physicians and its goals in the Pharos of Alpha Omega Alpha. Beeson was optimistic and he quoted from my 1991 JAMA health system reform editorial as a sharp contrast to what Fishbein had written - although coincidentally, we both quote Lincoln. My editorial began, "'with malice toward none, with charity for all...' so spoke Abraham Lincoln in his second inaugural address recognizing that he had no political consensus regarding either the constitutionality of states seceding or the morality of slavery being abolished. Nonetheless, he knew what was right and was able, through persuasive, often inspiring rhetoric, to conclude a bloody and decisive Civil War and constitute the foundation for this great republic.... Yet access to basic medical care for all of our inhabitants is still not a reality in this country. There are many reasons for this, not the least of which is a long-standing, systematic, institutionalized racial discrimination.... An aura of inevitablitiy is upon us. It is not acceptable morally, ethically, or economically for so many of our people to be medically uninsured or seriously underinsured. We can solve this problem. We have the knowledge and the resources, the skills, the time, and the moral

John P. Peters and the committee of 430 physicians.

PubMed

Lundberg, George D

2002-01-01

John Peters and his committee had a few basic goals. One was that local, state, and federal governments needed to provide money to construct facilities, support medical research and education, and care for the poor. And they wanted experts to call the shots. Over time, Peters and the committee got what they wanted for the most part: Hill-Burton money for building the hospitals, the rise of the National Institutes of Health, Medicare, Medicaid, a Veterans Administration system, and new and expanded medical schools. The experts calling the shots included David Kessler at the Food and Drug Administration and Surgeon General C. Everett Koop. In the halcyon days of American health system reform, back in 1993, Yale's Paul Beeson wrote about the Committee of 430 Physicians and its goals in the Pharos of Alpha Omega Alpha. Beeson was optimistic and he quoted from my 1991 JAMA health system reform editorial as a sharp contrast to what Fishbein had written - although coincidentally, we both quote Lincoln. My editorial began, "'with malice toward none, with charity for all...' so spoke Abraham Lincoln in his second inaugural address recognizing that he had no political consensus regarding either the constitutionality of states seceding or the morality of slavery being abolished. Nonetheless, he knew what was right and was able, through persuasive, often inspiring rhetoric, to conclude a bloody and decisive Civil War and constitute the foundation for this great republic.... Yet access to basic medical care for all of our inhabitants is still not a reality in this country. There are many reasons for this, not the least of which is a long-standing, systematic, institutionalized racial discrimination.... An aura of inevitablitiy is upon us. It is not acceptable morally, ethically, or economically for so many of our people to be medically uninsured or seriously underinsured. We can solve this problem. We have the knowledge and the resources, the skills, the time, and the moral

Pilot Peter Hoag and HL-10

NASA Technical Reports Server (NTRS)

1969-01-01

Air Force Major Peter Hoag stands in front of the HL-10 Lifting Body. Maj. Hoag joined the HL-10 program in 1969 and made his first glide flight on June 6, 1969. He made a total of 8 flights in the HL-10. They included the fastest lifting-body flight, which reached Mach 1.861 on Feb. 18, 1970. The HL-10 was one of five heavyweight lifting-body designs flown at NASA's Flight Research Center (FRC--later Dryden Flight Research Center), Edwards, California, from July 1966 to November 1975 to study and validate the concept of safely maneuvering and landing a low lift-over-drag vehicle designed for reentry from space. Northrop Corporation built the HL-10 and M2-F2, the first two of the fleet of 'heavy' lifting bodies flown by the NASA Flight Research Center. The contract for construction of the HL-10 and the M2-F2 was $1.8 million. 'HL' stands for horizontal landing, and '10' refers to the tenth design studied by engineers at NASA's Langley Research Center, Hampton, Va. After delivery to NASA in January 1966, the HL-10 made its first flight on Dec. 22, 1966, with research pilot Bruce Peterson in the cockpit. Although an XLR-11 rocket engine was installed in the vehicle, the first 11 drop flights from the B-52 launch aircraft were powerless glide flights to assess handling qualities, stability, and control. In the end, the HL-10 was judged to be the best handling of the three original heavy-weight lifting bodies (M2-F2/F3, HL-10, X-24A). The HL-10 was flown 37 times during the lifting body research program and logged the highest altitude and fastest speed in the Lifting Body program. On Feb. 18, 1970, Air Force test pilot Peter Hoag piloted the HL-10 to Mach 1.86 (1,228 mph). Nine days later, NASA pilot Bill Dana flew the vehicle to 90,030 feet, which became the highest altitude reached in the program. Some new and different lessons were learned through the successful flight testing of the HL-10. These lessons, when combined with information from it's sister ship, the M2

Muscle MRI and functional outcome measures in Becker muscular dystrophy.

PubMed

Barp, Andrea; Bello, Luca; Caumo, Luca; Campadello, Paola; Semplicini, Claudio; Lazzarotto, Annalisa; Sorarù, Gianni; Calore, Chiara; Rampado, Alessandro; Motta, Raffaella; Stramare, Roberto; Pegoraro, Elena

2017-11-22

Becker muscular dystrophy (BMD) is a neuromuscular disorder allelic to Duchenne muscular dystrophy (DMD), caused by in-frame mutations in the dystrophin gene, and characterized by a clinical progression that is both milder and more heterogeneous than DMD. Muscle magnetic resonance imaging (MRI) has been proposed as biomarker of disease progression in dystrophinopathies. Correlation with clinically meaningful outcome measures such as North Star Ambulatory Assessment (NSAA) and 6 minute walk test (6MWT) is paramount for biomarker qualification. In this study, 51 molecularly confirmed BMD patients (aged 7-69 years) underwent muscle MRI and were evaluated with functional measures (NSAA and 6MWT) at the time of the MRI, and subsequently after one year. We confirmed a pattern of fatty substitution involving mainly the hip extensors and most thigh muscles. Severity of muscle fatty substitution was significantly correlated with specific DMD mutations: in particular, patients with an isolated deletion of exon 48, or deletions bordering exon 51, showed milder involvement. Fat infiltration scores correlated with baseline functional measures, and predicted changes after 1 year. We conclude that in BMD, skeletal muscle MRI not only strongly correlates with motor function, but also helps in predicting functional deterioration within a 12-month time frame.

Rehabilitative technology use among individuals with Duchenne/Becker muscular dystrophy.

PubMed

Pandya, Shree; Andrews, Jennifer; Campbell, Kim; Meaney, F John

2016-01-01

To document use of rehabilitative technology among individuals with Duchenne/Becker muscular dystrophy (DBMD) among sites of the Muscular Dystrophy Surveillance, Tracking, and Research network (MD STARnet). Data from 362 caregivers who participated in the MD STARnet caregiver interview between April 2006 and March 2012 (54.7% response rate) were analyzed to assess the type, frequency and duration of use of assistive technology. Caregiver reports of technology use by individuals with DBMD across five MD STARnet sites in the US demonstrated significant regional differences in the proportion of individuals who had ever used night splints (36.9%-73.0%), standers (3.1%-22.2%) and scooters (10.7%-54.5%). Among individuals who used night splints 59.7% stopped using them at a mean age of 10.3 years after a mean duration of 2.9 years in spite of the current recommendation to continue using them through the non-ambulatory phase. Results of this comprehensive survey document the frequency of assistive device use by individuals with DBMD in the USA and also provides data on differences across the sites. Further research is needed to understand the reasons for and the impact of these differences on clinical outcomes and health related quality of life of individuals with DBMD.

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)

PubMed Central

Mathis, Wayne N.; Zatwarnicki, Tadeusz

2012-01-01

Abstract 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): Allotrichoma bifurcatum (Utah. Utah: Lake Shore (40°06.9'N, 111°41.8'W; 1370 m)), Allotrichoma dynatum (Oregon. Benton: Finley National Wildlife Refuge (44°24.6'N, 123°19.5'W)), Allotrichoma occidentale (Oregon. Lake: Lakeview (44 km E; Drake Creek; 42°11'N, 119°59.3'W)), Allotrichoma robustum (California. Kern: Kern River (35°16.1'N, 119°18.4'W)), Allotrichoma sabroskyi (New Mexico. Sandoval: La Cueva (Junction of Highways 126 and 4; 35°52'N, 106°38.4'W; 2342 m)), Allotrichoma wallowa (Oregon Baker: Goose Creek (35 km E Baker City; 44°49.2'N, 117°27.79'W; 825 m)), Allotrichoma baliops (Florida. Monroe: Key West (Willie Ward Park; 24°32.9'N, 81°47.9'W)), and Allotrichoma insulare (Dominica. Cabrits Swamp (15°35'N, 61°29'W)). Within Allotrichoma, we recognize three subgenera of which one, Neotrichoma (type species: Allotrichoma 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

[Questions on the first operation with ethyl ether as anaesthetic by Dr. Peter Parker].

PubMed

Chen, Q

2017-01-28

Ethyl ether was the first accepted effective general anaesthetic. It was introduced into China by an America missionary, Dr. Peter Parker. This was one of the historical events of medical communication between China and the West. In the records of the first operation with ether, however, Dr. Parker unusually omitted the patient's medical record number and the date of the operation, while those of other operations with ether anesthetics were all available. This was very unusual for a doctor like Peter Parker who always recorded every important case in detail in the hospital reports. It seems that he deliberately rather than carelessly omitted the information for some reasons. Based on the analysis of Parker's reports, a conclusion is made that the anesthetic effect of the case was actually ineffective. Furthermore, possible answers to this are outlined and question by discussion based on the situation that Parker faced in the late Qing era.

Formulating an Anarchist Sociology: Peter Kropotkin's Reading of Herbert Spencer.

PubMed

Adams, Matthew S

2016-01-01

The work of Herbert Spencer was a crucial influence on the development of Peter Kropotkin's historical sociology. However, scholars have underestimated this relationship; either overlooking it entirely, or minimizing Kropotkin's attachment to Spencer with the aim of maintaining the utility of his political thought in the present. This article contests these interpretations by analyzing Kropotkin's reading of Spencer's epistemological, biological, and political ideas. It argues that Kropotkin was engaged in a critical dialogue with Spencer, incorporating many Spencerian principles in his own system, but also using this reading to articulate a distinctive anarchist politics.

A Critique of Peter Jarvis's Conceptualisation of the Lifelong Learner in the Contemporary Cultural Context

ERIC Educational Resources Information Center

Bagnall, Richard G.

2017-01-01

This paper examines Peter Jarvis's conceptualisation of lifelong learners, who are seen as being the individual products of their learning engagements, constrained by their individual biological potentials. They are presented as seeking existentially authentic resolution to dialectically oppositional disjunctures between their individual…

Parental attitudes toward newborn screening for Duchenne/Becker muscular dystrophy and spinal muscular atrophy.

PubMed

Wood, Molly F; Hughes, Sarah C; Hache, Lauren P; Naylor, Edwin W; Abdel-Hamid, Hoda Z; Barmada, M Michael; Dobrowolski, Steven F; Stickler, David E; Clemens, Paula R

2014-06-01

Disease inclusion in the newborn screening (NBS) panel should consider the opinions of those most affected by the outcome of screening. We assessed the level and factors that affect parent attitudes regarding NBS panel inclusion of Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), and spinal muscular atrophy (SMA). The attitudes toward NBS for DMD, BMD, and SMA were surveyed and compared for 2 categories of parents, those with children affected with DMD, BMD, or SMA and expectant parents unselected for known family medical history. The level of support for NBS for DMD, BMD, and SMA was 95.9% among parents of children with DMD, BMD, or SMA and 92.6% among expectant parents. There was strong support for NBS for DMD, BMD, and SMA in both groups of parents. Given advances in diagnostics and promising therapeutic approaches, discussion of inclusion in NBS should continue. Copyright © 2013 Wiley Periodicals, Inc.

Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy: A Randomized Clinical Trial.

PubMed

Silva, Marly Conceição; Magalhães, Tiago Augusto; Meira, Zilda Maria Alves; Rassi, Carlos Henrique Reis Esselin; Andrade, Amanda Cristina de Souza; Gutierrez, Paulo Sampaio; Azevedo, Clerio Francisco; Gurgel-Giannetti, Juliana; Vainzof, Mariz; Zatz, Mayana; Kalil-Filho, Roberto; Rochitte, Carlos Eduardo

2017-02-01

In Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), interventions reducing the progression of myocardial disease could affect survival. To assess the effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocardial fibrosis (MF) identified on cardiovascular magnetic resonance (CMR). A randomized clinical trial conducted in 2 centers included 76 male patients with DMD or BMD undergoing 2 CMR studies with a 2-year interval for ventricular function and MF assessment. In a non-intent-to-treat trial, 42 patients with MF and normal left ventricular ejection fraction (LVEF) were randomized (1:1) to receive or not receive ACE inhibitor therapy. The study was conducted from June 26, 2009, to June 30, 2012. Data analysis was performed from June 30, 2013, to October 3, 2016. Randomization (1:1) to receive or not receive ACE inhibitor therapy. Primary outcome was MF progression from baseline to the 2-year CMR study. Of the 76 male patients included in the study, 70 had DMD (92%) and 6 had BMD (8%); mean (SD) age at baseline was 13.1 (4.4) years. Myocardial fibrosis was present in 55 patients (72%) and LV systolic dysfunction was identified in 13 patients (24%). Myocardial fibrosis at baseline was an independent indicator of lower LVEF at follow-up (coefficient [SE], -0.16 [0.07]; P = .03). Among patients with MF and preserved LVEF (42 [55%]), those randomized (21 patients in each arm) to receive ACE inhibitors demonstrated slower MF progression compared with the untreated group (mean [SD] increase of 3.1% [7.4%] vs 10.0% [6.2%] as a percentage of LV mass; P = .001). In multivariate analysis, ACE inhibitor therapy was an independent indicator of decreased MF progression (coefficient [SE], -4.51 [2.11]; P = .04). Patients with MF noted on CMR had a higher probability of cardiovascular events (event rate, 10 of 55 [18.2%] vs 0 of 21 [0%]; log-rank P = .04

Interview with Peter Samis and Mimi Michaelson, Authors of "Creating the Visitor-Centered Museum"

ERIC Educational Resources Information Center

Spero, Susan

2017-01-01

"Creating the Visitor-Centered Museum" offers insight into why and how 10 case study museums have transformed to serve the needs of their public. Susan Spero interviews authors Peter Samis and Mimi Michaelson about the purpose of the book, their case study choices, the key characteristics of visitor-centered institutions and their…

Two Is the Beginning of the End: "Peter Pan" and the Doctrine of Reminiscence

ERIC Educational Resources Information Center

Hudson, Glenda A.

2006-01-01

This article considers J.M. Barrie's satirical treatment of the Platonic doctrine of reminiscence in "Peter Pan," and how Barrie's work both honors and undercuts it. It will first analyze the Platonic notion of the doctrine of reminiscence in Wordsworth's "Ode: Intimations of Immortality from Recollections of Early Childhood" (1807). It will then…

"Operating on a Basis of Student Consent": Peter Medway's Work in "Finding a Language"

ERIC Educational Resources Information Center

Burgess, Tony

2015-01-01

Written nearly 40 years ago, Peter Medway's "Finding a Language" continues to be an arresting read, which offers a powerful vision of what might be possible in education. In this brief introduction, I set the work in context, referring to ideas that Pete engaged with and recalling a little of the times.

Mothers' psychological adaptation to Duchenne/Becker muscular dystrophy

PubMed Central

Peay, Holly L; Meiser, Bettina; Kinnett, Kathleen; Furlong, Pat; Porter, Kathryn; Tibben, Aad

2016-01-01

Duchenne and Becker muscular dystrophy (DBMD) cause significant emotional and care-related burden on caregivers, but no studies have evaluated predictors of positive caregiver outcomes, including disorder-specific psychological adaptation. Using a community-engaged approach focused on supporting mothers in positive aspects of caregiving, this prospective study aims to assess (i) the association between child's baseline functional status and mothers' illness perceptions, resilience, and coping self-efficacy; and (ii) predictors of mothers' psychological adaptation to caring for a child with DBMD. Biological mothers with at least one living child with DBMD completed a baseline survey (n=205) with 1-year (n=147) and 2-year (n=144) follow-up surveys. Worse child's baseline function was associated not only with increased caregiver burden and reduced maternal resilience, but also with perception of positive disease impact on the family. At two follow-ups, increased psychological adaptation to DBMD was predicted by resilience (β=0.264, P=0.001) and perceived positive impact (β=0.310, P<0.001), controlling for mother's age (β=−0.305, P<0.001) and income (β=−0.088, P=0.245). Child's functional status and caregiver burden of DBMD did not predict DBMD-specific adaptation. Though clinicians caring for families with DBMD should anticipate increased caregiver burden as the disorder progresses, interventions focused on caregiver burden are not expected to influence mothers' psychosocial adaptation. Efforts to improve mothers' well-being should focus on fostering mothers' resilience and enhancing perceptions of positive disease impact (benefit finding). Results suggest that psychosocial interventions can highlight strengths and well-being rather than burden and deficit. PMID:26306645

[Peter of Abano, the 'Conciliator' between magic and science].

PubMed

Federici Vescovini, Graziella

2008-01-01

Peter of Abano is a personage belonging to history as well as to legend. In this analysis his historical physiognomy of scientist and physician is reconstructed on the basis of a better knowledge of his works in medicine and astronomy (now edited), a discipline, the latest one, in which he excelled in Medieval scientific context (The Lucidator dubitabilium astronomiae (astrologiae) can be read in the critical edition by G. Federici Vescovini, Padova, Esedra, 1992). He opposed extreme position in all fields, by styling himself 'Conciliator' of opposed tendencies. He often reconfirmed a rational concept of medicine and astronomy, which dispelled any demoniac and magic features, against the fanciful legend elaborated during the following centuries.

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

PubMed

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

2016-09-01

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 severely lowered in BMD, but was almost normalized in LGMD2I. Our findings suggest close to intact contractile properties in LGMD2I, which are severely disrupted in BMD. Ann Neurol 2016;80:466-471. © 2016 American Neurological Association.

Extensive Functional Evaluations to Monitor Aerobic Training in Becker Muscular Dystrophy: A Case Report.

PubMed

Tramonti, Caterina; Rossi, Bruno; Chisari, Carmelo

2016-06-13

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.

Language and Reality. Peter Mittelstaedt's Contributions to the Philosophy of Physics

NASA Astrophysics Data System (ADS)

Falkenburg, Brigitte

2010-10-01

The article investigates the way in which Peter Mittelstaedt has been contributing to the philosophy of physics for half a century. It is shown that he pursues a path between rationalism and empiricism in the sense of Erhard Scheibe’s philosophy of the physicists. Starting from Kant’s a priori he gives a rational reconstruction of the conceptual revolutions of 20th century physics. The central topic of his philosophy of physics is the quest for semantic self-consistency, which for quantum mechanics is a hard nut to crack.

[Clinical features of patients with Becker muscular dystrophy and deletions of the rod domain of dystrophin gene].

PubMed

Wang, Yanyun; Zhu, Yuling; Yang, Juan; Li, Yaqin; Sun, Jiangwen; Zhan, Yixin; Zhang, Cheng

2018-02-10

OBJECTIVE To explore the clinical features of patients carrying deletions of the rod domain of the dystrophin gene. METHODS Clinical data of 12 Chinese patients with Becker muscular dystrophy (BMD) and such deletions was reviewed. RESULTS Most patients complained of muscle weakness of lower limbs. Two patients had muscle cramps, one had increased creatine kinase (CK) level, and one had dilated cardiomyopathy. CONCLUSION Compared with DMD, the clinical features of BMD are much more variable, particularly for those carrying deletions of the rod domain of the dystrophin gene. Muscular weakness may not be the sole complaint of BMD. The diagnosis of BMD cannot be excluded by moderately elevated CK. For male patients with dilated cardiomyopathy, the possibility of BMD should be considered.

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

PubMed

Jensen, Bente R; Berthelsen, Martin P; Husu, Edith; Christensen, Sofie B; Prahm, Kira P; Vissing, John

2016-08-01

We studied the functional effects of combined strength and aerobic anti-gravity training in severely affected patients with Becker and Limb-Girdle muscular dystrophies. Eight patients performed 10-week progressive combined strength (squats, calf raises, lunges) and aerobic (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. 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%. 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. © 2016 Wiley Periodicals, Inc.

Education, Philosophy and Politics: The Selected Works of Michael A. Peters. World Library of Educationalists

ERIC Educational Resources Information Center

Peters, Michael A.

2012-01-01

In the World Library of Educationalists series, international experts themselves compile career-long collections of what they judge to be their finest pieces--extracts from books, key articles, salient research findings, major theoretical and/practical contributions--so the world can read them in a single manageable volume. Michael A. Peters has…

Wildlife and Wildlife Habitat Loss Assessment at Green Peter-Foster Project; Middle Fork Santiam River, Oregon, 1985 Final Report.

DOE Office of Scientific and Technical Information (OSTI.GOV)

Noyes, J.H.

1986-02-01

A habitat based assessment was conducted of the US Army Corps of Engineers' Green Peter-Foster Dam and Reservoir Project on the Middle Fork Santiam River, Oregon, to determine losses or gains resulting from the development and operation of the hydroelectric related components of the project. Preconstruction, postconstruction, and recent vegetation cover types at the project site were mapped based on aerial photographs from 1955, 1972, and 1979, respectively. Vegetation cover types were identified within the affected area and acreages of each type at each period were determined. Eleven wildlife target species were selected to represent a cross-section of species groupsmore » affected by the project. An interagency team evaluated the suitability of the habitat to support the target species at each time period. An evaluation procedure which accounted for both the quantity and quality of habitat was used to aid in assessing impacts resulting from the project. The Green Peter-Foster Project extensively altered or affected 7873 acres of land and river in the Santiam River drainage. Impacts to wildlife centered around the loss of 1429 acres of grass-forb vegetation, 768 acres of shrubland, and 717 acres of open conifer forest cover types. Impacts resulting from the Green Peter-Foster Project included the loss of critical winter range for black-tailed deer and Roosevelt elk, and the loss of year-round habitat for deer, upland game birds, river otter, beaver, pileated woodpecker, and many other wildlife species. Bald eagle and osprey were benefited by an increase in foraging habitat. The potential of the affected area to support wildlife was greatly altered as a result of the Green Peter-Foster Project. Losses or gains in the potential of the habitat to support wildlife will exist over the life of the project.« less

An Interview with Peter McLaren: Comments on the State of the World-2005

ERIC Educational Resources Information Center

Shaughnessy, Michael F.

2006-01-01

Some of the wide range of thoughtful topics discussed in this interview with Peter McLaren include: (1) The top five points that McLaren tried to make in his book, "Capitalists and Conquerors: A Critical Pedagogy Against Empire"; (2) McLaren's thoughts on what he thinks the George Bush legend will be; (3) What led McLaren to ask,…

1988 Horton Medal presented to Peter S. Eagleson

NASA Astrophysics Data System (ADS)

Rodriguez-Iturbe, Ignacio; Eagleson, Peter S.

It is indeed a great honor for me, and also a great pleasure, to present to you the 1988 recipient of the Robert E. Horton Medal, Peter S. Eagleson. Shortly after it became public that Pete was the winner of this year's medal, I commented to my wife that, although I had never given a citation for an AGU medalist, this was one I would really love to give. The reason is simple: there is no one in the world who better represents the standards of excellence that a hydrologist hopes to achieve and that hydrologists hope our discipline will attain than Peter Eagleson.Pete did not start his research career in hydrology. In fact, his Ph.D. thesis in 1956 and his research up to 1965 were mainly in the field of sediment sorting and transport by waves on beaches. During this period he also investigated problems related to flow-induced vibrations of plates. His research in these areas was extremely successful, producing three chapters in books and about 20 journal papers in addition to many technical reports and journal discussions. Indeed, Pete had made his mark in wave theory and sediment transport, but it was good fortune for our field that starting about 1964 his interest in hydrology overcame all his past experience and assured a reputation in the waves and sediment field, and thus he embarked on a new adventure—bringing into hydrology the scientific rigor that existed in more academically established disciplines. With his strong fluid mechanics background—he still teaches that subject to undergraduates at MIT—Pete was ideally suited to lead the field toward new problems and also toward new approaches to old problems . . . and this he did indeed! Just in 1967 he and his students published six papers, most of them in Water Resources Research, which had a strong, an almost immediate impact on the field of hydrology. Here was somebody who was tackling the modeling of overland flow with the scientific standards of fluid mechanics. At the same time, finally somebody had

Between Fiction and Reality: Maps and Cartographic Logic in the Works of Peter Sís

ERIC Educational Resources Information Center

Cantavella, Anna Juan

2017-01-01

This article examines the use of maps in the works of Czech author-illustrator Peter Sís in order to consider the role that cartography plays in the construction of four of his biographical picturebooks: Follow the Dream: "The Story of Christopher Columbus" (2003/1991), "Starry Messenger: Galileo Galilei" (1996), "The Tree…

Evolution of Capsaicinoids in Peter Pepper (Capsicum annuum var. annuum) During Fruit Ripening.

PubMed

Barbero, Gerardo F; de Aguiar, Ana C; Carrera, Ceferino; Olachea, Ángel; Ferreiro-González, Marta; Martínez, Julian; Palma, Miguel; Barroso, Carmelo G

2016-08-01

The evolution of individual and total contents of capsaicinoids present in Peter peppers (Capsicum annuum var. annuum) at different ripening stages has been studied. Plants were grown in a glasshouse and the new peppers were marked in a temporal space of ten days. The extraction of capsaicinoids was performed by ultrasound-assisted extraction with MeOH. The capsaicinoids nordihydrocapsaicin (n-DHC), capsaicin, dihydrocapsaicin, homocapsaicin, and homodihydrocapsaicin were analyzed by ultraperformance liquid chromatography (UHPLC)-fluorescence and identified by UHPLC-Q-ToF-MS. The results indicate that the total capsaicinoids increase in a linear manner from the first point of harvest at ten days (0.283 mg/g FW) up to 90 days, at which point they reach a concentration of 1.301 mg/g FW. The evolution as a percentage of the individual capsaicinoids showed the initial predominance of capsaicin, dihydrocapsaicin, and n-DHC. Dihydrocapsaicin was the major capsaicinoid up to day 50 of maturation. After 50 days, capsaicin became the major capsaicinoid as the concentration of dihydrocapsaicin fell slightly. The time of harvest of Peter pepper based on the total capsaicinoids content should be performed as late as possible. In any case, harvesting should be performed before overripening of the fruit is observed. © 2016 Wiley-VHCA AG, Zürich.

Peter Andreas Hansen and the astronomical community - a first investigation of the Hansen papers. (German Title: Peter Andreas Hansen und die astronomische Gemeinschaft - eine erste Auswertung des Hansen-Nachlasses. )

NASA Astrophysics Data System (ADS)

Schwarz, Oliver; Strumpf, Manfred

The literary assets of Peter Andreas Hansen are deposited in the Staatsarchiv Hamburg, the Forschungs- und Landesbibliothek Gotha and the Thüringer Staatsarchiv Gotha. They were never systematically investigated. We present here some results of a first evaluation. It was possible to reconstruct the historical events with regard to the maintenance of the Astronomische Nachrichten and the Altona observatory in 1854. Hansen was a successful teacher for many young astronomers. His way of stimulating the evolution of astronomy followed Zach's tradition.

76 FR 13271 - Gulf & Ohio Railways, Inc., H. Peter Claussen and Linda C. Claussen-Continuance in Control...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-03-10

... DEPARTMENT OF TRANSPORTATION Surface Transportation Board [Docket No. FD 35414] Gulf & Ohio Railways, Inc., H. Peter Claussen and Linda C. Claussen--Continuance in Control Exemption--Lancaster & Chester Railroad, LLC AGENCY: Surface Transportation Board, DOT. ACTION: Correction to notice of exemption...

Genetic diagnosis of Duchenne and Becker muscular dystrophy using next-generation sequencing technology: comprehensive mutational search in a single platform.

PubMed

Lim, Byung Chan; Lee, Seungbok; Shin, Jong-Yeon; Kim, Jong-Il; Hwang, Hee; Kim, Ki Joong; Hwang, Yong Seung; Seo, Jeong-Sun; Chae, Jong Hee

2011-11-01

Duchenne muscular dystrophy or Becker muscular dystrophy might be a suitable candidate disease for application of next-generation sequencing in the genetic diagnosis because the complex mutational spectrum and the large size of the dystrophin gene require two or more analytical methods and have a high cost. The authors tested whether large deletions/duplications or small mutations, such as point mutations or short insertions/deletions of the dystrophin gene, could be predicted accurately in a single platform using next-generation sequencing technology. A custom solution-based target enrichment kit was designed to capture whole genomic regions of the dystrophin gene and other muscular-dystrophy-related genes. A multiplexing strategy, wherein four differently bar-coded samples were captured and sequenced together in a single lane of the Illumina Genome Analyser, was applied. The study subjects were 25 16 with deficient dystrophin expression without a large deletion/duplication and 9 with a known large deletion/duplication. Nearly 100% of the exonic region of the dystrophin gene was covered by at least eight reads with a mean read depth of 107. Pathogenic small mutations were identified in 15 of the 16 patients without a large deletion/duplication. Using these 16 patients as the standard, the authors' method accurately predicted the deleted or duplicated exons in the 9 patients with known mutations. Inclusion of non-coding regions and paired-end sequence analysis enabled accurate identification by increasing the read depth and providing information about the breakpoint junction. The current method has an advantage for the genetic diagnosis of Duchenne muscular dystrophy and Becker muscular dystrophy wherein a comprehensive mutational search may be feasible using a single platform.

Local and Global in the Formation of a Learning Theorist: Peter Jarvis and Adult Education

ERIC Educational Resources Information Center

Holford, John

2017-01-01

Peter Jarvis is a towering figure in the study of adult and lifelong education and a leading and original theorist of learning. This paper sets out his intellectual and professional biography, maps the main contours of his work and introduces fourteen papers by leading scholars devoted to his work. Five broad phases in Jarvis' life are identified:…

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

PubMed

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.

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

DOE Office of Scientific and Technical Information (OSTI.GOV)

Shomrat, R.; Gluck, E.; Legum, C.

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 themore » 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.« less

Cardiac considerations in the operative management of the patient with Duchenne or Becker muscular dystrophy.

PubMed

Cripe, Linda H; Tobias, Joseph D

2013-09-01

Duchenne muscular dystrophy/Becker muscular dystrophy (DMD/BMD) is a progressive multisystem neuromuscular disorder. In addition to the skeletal muscle, the myocardium in the DMD/BMD patient is dystrophin deficient which results in a progressive cardiomyopathy. The myopathic myocardium poses significant risk of increased morbidity and mortality at the time of major surgical procedures. Careful attention must be given to the DMD/BMD patient during the intraoperative and postoperative period. Anesthesia selection is critical and anesthetics should be avoided which have been shown to be harmful in this patient population. Preanesthesia assessment should include cardiac consultation and detailed preoperative evaluation. Intraoperative management needs to insure that the weakened myocardium is not compromised by physiologic changes such as hypotension or major fluid shifts. Finally, attention to the cardiac status of the patient must continue into the postoperative period. The surgical care of the DMD/BMD patient requires a multispecialty approach to insure operative success. © 2013 John Wiley & Sons Ltd.

Solution of Peter Winkler's Pizza Problem

NASA Astrophysics Data System (ADS)

Cibulka, Josef; Kynčl, Jan; Mészáros, Viola; Stolař, Rudolf; Valtr, Pavel

Bob cuts a pizza into slices of not necessarily equal size and shares it with Alice by alternately taking turns. One slice is taken in each turn. The first turn is Alice's. She may choose any of the slices. In all other turns only those slices can be chosen that have a neighbor slice already eaten. We prove a conjecture of Peter Winkler by showing that Alice has a strategy for obtaining 4/9 of the pizza. This is best possible, that is, there is a cutting and a strategy for Bob to get 5/9 of the pizza. We also give a characterization of Alice's best possible gain depending on the number of slices. For a given cutting of the pizza, we describe a linear time algorithm that computes Alice's strategy gaining at least 4/9 of the pizza and another algorithm that computes the optimal strategy for both players in any possible position of the game in quadratic time. We distinguish two types of turns, shifts and jumps. We prove that Alice can gain 4/9, 7/16 and 1/3 of the pizza if she is allowed to make at most two jumps, at most one jump and no jump, respectively, and the three constants are the best possible.

Shallowing-upward cyclic patterns within larger-scale transgressive-regressive (T-R) sedimentary sequences, St. Peter through Decorah Formations, Ordovician, Iowa area

DOE Office of Scientific and Technical Information (OSTI.GOV)

Witzke, B.J.

1993-03-01

Four large-scale (2--8 Ma) T-R sedimentary sequences of M. Ord. age (late Chaz.-Sherm.) were delimited by Witzke Kolata (1980) in the Iowa area, each bounded by local to regional unconformity/disconformity surfaces. These encompass both siliciclastic and carbonate intervals, in ascending order: (1) St. Peter-Glenwood fms., (2) Platteville Fm., (3) Decorah Fm., (4) Dunleith/upper Decorah fms. Finer-scale resolution of depth-related depositional features has led to regional recognition of smaller-scale shallowing-upward cyclicity contained within each large-scale sequence. Such smaller-scale cyclicity encompasses stratigraphic intervals of 1--10 m thickness, with estimated durations of 0.5--1.5 Ma. The St. Peter Sandst. has long been regarded asmore » a classic transgressive sheet sand. However, four discrete shallowing-upward packages characterize the St. Peter-Glenwood interval regionally (IA, MN, NB, KS), including western facies displaying coarsening-upward sandstone packages with condensed conodont-rich brown shale and phosphatic sediments in their lower part (local oolitic ironstone), commonly above pyritic hardgrounds. Regional continuity of small-scale cyclic patterns in M. Ord. strata of the Iowa area may suggest eustatic controls; this can be tested through inter-regional comparisons.« less

Diagnostic and clinical characteristics of early-manifesting females with Duchenne or Becker muscular dystrophy.

PubMed

Imbornoni, Lauren; Price, Elinora T; Andrews, Jennifer; Meaney, F John; Ciafaloni, Emma; Cunniff, Christopher

2014-11-01

Manifestations of Duchenne and Becker muscular dystrophy (DBMD) are present in up to 40% of heterozygous females, but there are few reports of females who exhibit skeletal muscle symptoms in childhood. From the Muscular Dystrophy Surveillance Tracking and Research Network, a multi-site population-based surveillance network for dystrophinopathy, nine symptomatic female heterozygotes with onset of symptoms prior to age 9 years were identified. The median age at diagnosis was 8.3 years, and the median interval from first symptoms to diagnosis was 1.35 years. Of the nine female heterozygotes, four had a positive family history, seven had intellectual disability and five had at least one mental health disorder. Mental health concerns included attention deficit hyperactivity disorder (ADHD), autism spectrum features, bipolar disorder, and depression. The frequency of intellectual and mental health problems in this group is higher than previously reported for affected males and for symptomatic females. These findings may have implications for diagnosis of early manifesting heterozygotes and for their health supervision. © 2014 Wiley Periodicals, Inc.

Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures.

PubMed

Segura, Leal G; Lorenz, Jessica D; Weingarten, Toby N; Scavonetto, Federica; Bojanić, Katarina; Selcen, Duygu; Sprung, Juraj

2013-09-01

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. To review the perioperative outcomes of patients with DMD and BMD. We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children. © 2013 John Wiley & Sons Ltd.

A Pedagogy of Dealienation: A Case Study in the Application of Peter Berger's "The Sacred Canopy"

ERIC Educational Resources Information Center

Chance, J. Bradley

2004-01-01

This paper explores the use of Peter Berger's theory of religion and its features of alienation and dealienation to lead students to the critical awareness of the role that human beings play in the construction of social worlds, including most especially our religious worlds. After summarizing Berger's theory of the alienating and potentially…

A Genealogical Analysis of Peter Arnold's Conceptual Account of Meaning in Movement, Sport and Physical Education

ERIC Educational Resources Information Center

Stolz, Steven A.; Thorburn, Malcolm

2017-01-01

We write as critical theorists who share an interest in how conceptions of physical education are taken forward in policy and practice. In this respect, we are particularly intrigued by Peter Arnold's conceptual account of meaning in movement, sport and physical education, and the subsequent ways in which his ideas have informed national…

Age at onset of first signs or symptoms predicts age at loss of ambulation in Duchenne and Becker Muscular Dystrophy: Data from the MD STARnet.

PubMed

Ciafaloni, Emma; Kumar, Anil; Liu, Ke; Pandya, Shree; Westfield, Christina; Fox, Deborah J; Caspers Conway, Kristin M; Cunniff, Christopher; Mathews, Katherine; West, Nancy; Romitti, Paul A; McDermott, Michael P

2016-01-01

We investigated the prognostic utility of onset age at first signs and symptoms (SS) to predict onset age at loss of ambulation (LOA) for childhood-onset Duchenne and Becker Muscular Dystrophies (DBMD). Our cohort comprised male cases with DBMD ascertained by the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet). Adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using Cox proportional hazards models for associations between onset ages of first SS and LOA. Covariates controlled for were corticosteroid use, family history of DBMD, birth year, race/ethnicity, and MD STARnet site. Onset age at first SS was considered as a continuous and as a categorical variable. A one-year increase in onset age at first SS was significantly associated with a 10% reduction in annual risk of LOA (HR = 0.90, CI = 0.87-0.94). Treating onset age at first SS as a categorical variable yielded a similar association (≥ 5 years: referent; ≥ 3 to < 5 years: HR = 1.36, CI = 1.02-1.81; 18 months to < 3 years: HR = 1.72, CI = 1.31-2.26; < 18 months: HR = 1.52, CI = 1.14-2.02). Earlier onset age at first SS is associated with earlier onset age at LOA and may have clinical utility in differentiating childhood-onset Duchenne and Becker muscular dystrophies.

Peter Andreas Hansen und die astronomische Gemeinschaft - eine erste Auswertung des Hansen-Nachlasses.

NASA Astrophysics Data System (ADS)

Schwarz, O.; Strumpf, M.

The literary assets of Peter Andreas Hansen are deposited in the Staatsarchiv Hamburg, the Forschungs- und Landesbibliothek Gotha and the Thüringer Staatsarchiv Gotha. They were never systematically investigated. The authors present here some results of a first evaluation. It was possible to reconstruct the historical events with regard to the maintenance of the Astronomische Nachrichten and the Altona observatory in 1854. Hansen was a successful teacher for many young astronomers. His way of stimulating the evolution of astronomy followed Zach's tradition.

Paradoxes of a Long Life Learning: An Exploration of Peter Jarvis's Contribution to Experiential Learning Theory

ERIC Educational Resources Information Center

Dyke, Martin

2017-01-01

The paper explores the work of Peter Jarvis related to learning with particular reference to his definitions of learning and his models of the learning process. This exploration will consider different approaches to experiential learning and demonstrate the contribution Jarvis has made, noting how his writing on the subject has changed over time.…

Application of the International Classification of Functioning, Disability and Health system to symptoms of the Duchenne and Becker muscular dystrophies.

PubMed

Conway, Kristin M; Ciafaloni, Emma; Matthews, Dennis; Westfield, Chris; James, Kathy; Paramsothy, Pangaja; Romitti, Paul A

2018-07-01

Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are X-linked recessive diseases that affect dystrophin production resulting in compromised muscle function across multiple systems. The International Classification of Functioning, Disability and Health provides a systematic classification scheme from which body functions affected by a dystrophinopathy can be identified and used to examine functional health. The infrastructure of the Muscular Dystrophy Surveillance, Tracking, and Research Network was used to identify commonly affected body functions and link selected functions to clinical surveillance data collected through medical record abstraction. Seventy-one (24 second-, 41 third- and 7 fourth-level) body function categories were selected via clinician review and consensus. Of these, 15 of 24 retained second-level categories were linked to data elements from the Muscular Dystrophy Surveillance, Tracking, and Research Network surveillance database. Our findings support continued development of a core set of body functions from the International Classification of Functioning, Disability and Health system that are representative of disease progression in dystrophinopathies and the incorporation of these functions in standardized evaluations of functional health and implementation of individualized rehabilitation care plans. Implications for Rehabilitation Duchenne and Becker muscular dystrophies, collectively referred to as dystrophinopathies, are X-linked recessive disorders that affect the production of dystrophin resulting in compromised muscle function across multiple systems. The severity and progressive nature of dystrophinopathies can have considerable impact on a patient's participation in activities across multiple life domains. Our findings support continued development of an International Classification of Functioning, Disability and Health core set for childhood-onset dystrophinopathies. A standardized

Superfund Record of Decision (EPA Region 5): Reilly Tar and Chemical Corporation Site, St. Peter Aquifer, St. Louis Park, MN. (Third remedial action), September 1990

DOE Office of Scientific and Technical Information (OSTI.GOV)

Not Available

1990-09-28

The 80-acre Reilly Tar and Chemical (St. Louis Park) site is a former coal tar distillation and wood preserving plant in St. Louis Park, Minnesota. The site overlies a complex system of aquifers, including the St. Peter aquifer that provide drinking water to area residences. The St. Peter Aquifer contains one municipal well, which is used during periods of peak demand, however, the majority of the drinking water in St. Louis Park is obtained from deeper aquifers. From 1917 to 1972, wastewater containing creosote and coal tar was discharged to onsite surface water, and as a result, small wastewater spillsmore » occurred into onsite soil. The ROD addresses Operable Unit 4 (OU4), remediation of the St. Peter aquifer. A subsequent ROD will address any remaining site problems as OU3. The primary contaminants of concern affecting the ground water are organics including PAHs and phenols.« less

Utilization of the St. Peter Sandstone in the Illinois Basin for CO2 Sequestration

DOE Office of Scientific and Technical Information (OSTI.GOV)

Will, Robert; Smith, Valerie; Leetaru, Hannes

2014-09-30

This project is part of a larger project co-funded by the United States Department of Energy (US DOE) under cooperative agreement DE-FE0002068 from 12/08/2009 through 9/31/2014. The study is to evaluate the potential of formations within the Cambro-Ordovician strata above the Mt. Simon Sandstone as potential targets for carbon dioxide (CO2) sequestration in the Illinois and Michigan Basins. This report evaluates the potential injectivity of the Ordovician St. Peter Sandstone. The evaluation of this formation was accomplished using wireline data, core data, pressure data, and seismic data acquired through funding in this project as well as existing data from twomore » additional, separately funded projects: the US DOE funded Illinois Basin – Decatur Project (IBDP) being conducted by the Midwest Geological Sequestration Consortium (MGSC) in Macon County, Illinois, and the Illinois Industrial Carbon Capture and Sequestration (ICCS) Project funded through the American Recovery and Reinvestment Act (ARRA), which received a phase two award from DOE. This study addresses the question of whether or not the St. Peter Sandstone may serve as a suitable target for CO2 sequestration at locations within the Illinois Basin where it lies at greater depths (below the underground source of drinking water (USDW)) than at the IBDP site. The work performed included numerous improvements to the existing St. Peter reservoir model created in 2010. Model size and spatial resolution were increased resulting in a 3 fold increase in the number of model cells. Seismic data was utilized to inform spatial porosity distribution and an extensive core database was used to develop porosity-permeability relationships. The analysis involved a Base Model representative of the St. Peter at “in-situ” conditions, followed by the creation of two hypothetical models at in-situ + 1,000 feet (ft.) (300 m) and in-situ + 2,000 ft. (600 m) depths through systematic depthdependent adjustment of the Base

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

NASA Astrophysics Data System (ADS)

Heimann, M.

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 in the year 2030 by 25 ppm compared to a reference scenario. However, the stabilisation of the atmospheric CO2 concentration requires a much larger additional sink or corresponding reduction of anthropogenic emissions.

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

NASA Astrophysics Data System (ADS)

Heimann, M.

2013-08-01

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 in the year 2030 by 25 ppm compared to a reference scenario. However, the stabilisation of the atmospheric CO2 concentration requires a much larger additional sink or corresponding reduction of anthropogenic emissions.

The significance of stylolitization and intergranular pressure solution in the formation of pressure compartment seals in the St. Peter Sandstone, Ordovician, Michigan basin

DOE Office of Scientific and Technical Information (OSTI.GOV)

Drzewiecki, P.A.; Simo, T.; Moline, G.

1991-03-01

The Middle to Late Ordovician St. Peter Sandstone of the Michigan basin is a fine- to medium-grained quartz sandstone. Extensive stylolitization and intergranular pressure solution have been major factors in reducing the porosity of certain horizons within the St. Peter, resulting in pressure compartmentation of the reservoir. Pressure versus depth data for various Michigan basin wells indicate that the basin contains compartments that are overpressured by as much as 500 psi. Horizons bounding these compartments are often affected by intense stylolitization (or intergranular pressure solution) and quartz cementation and have been correlated with zones of low porosity and permeability ({phi}more » = 0-3%, k = <50 {mu}d). These tight zones can be correlated within single gas fields, and some may extend across the Michigan basin. The St. Peter Sandstone has been buried to depths of about 3,500 m in the central part of the basin and 1,500 m at the margins. Intensely stylolitized zones are found at all depths throughout the basin and do not appear to change in abundance or style with depths. Factors that influence the formation, morphology, and abundance of stylolites in the St. Peter include (1) clay intraclasts, (2) intergranular clay, and (3) fine-grained, feldspar-rich sand. Stylolites also occur at contacts between quartz-cemented and carbonate-cemented zones and within well-cemented sands. Intergranular pressure solution and stylolites may be responsible for the formation of a compartment seal. Understanding their genesis can allow prediction of variations in porosity in Michigan basin well cores.« less

Identification of de novo mutations of Duchénnè/Becker muscular dystrophies in southern Spain.

PubMed

Garcia, Susana; de Haro, Tomás; Zafra-Ceres, Mercedes; Poyatos, Antonio; Gomez-Capilla, Jose A; Gomez-Llorente, Carolina

2014-01-01

Duchénnè/Becker muscular dystrophies (DMD/BMD) are X-linked diseases, which are caused by a de novo gene mutation in one-third of affected males. The study objectives were to determine the incidence of DMD/BMD in Andalusia (Spain) and to establish the percentage of affected males in whom a de novo gene mutation was responsible. Multiplex ligation-dependent probe amplification (MLPA) technology was applied to determine the incidence of DMD/BMD in 84 males with suspicion of the disease and 106 female relatives. Dystrophin gene exon deletion (89.5%) or duplication (10.5%) was detected in 38 of the 84 males by MLPA technology; de novo mutations account for 4 (16.7%) of the 24 mother-son pairs studied. MLPA technology is adequate for the molecular diagnosis of DMD/BMD and establishes whether the mother carries the molecular alteration responsible for the disease, a highly relevant issue for genetic counseling.

The pornographic anatomy book? The curious tale of the Anatomical Basis of Medical Practice.

PubMed

Halperin, Edward C

2009-02-01

Studying the history of medical education helps teach us that medicine is a social activity that occurs in the context of social mores and customs. In 1971, a major new anatomy textbook aimed at first-year medical students was published. The Anatomical Basis of Medical Practice, written by Professors R. Frederick Becker, James S. W. Wilson, and John A. Gehweiler, emphasized surface anatomy, embryology, and radiographic anatomy. At multiple places in the text, the authors used sexually suggestive and "cheeky" comments about women. A small fraction of the illustrations were stylized, posed female nude photographs purchased from California photographer Peter Gowland. These photographs, of a type typically seen in Playboy centerfolds or "pin-up girl" calendars, produced a firestorm of controversy. The book was criticized in the press and in reviews in scholarly journals, and a boycott was organized by the Association of Women in Science. The publisher received negative feedback from consumers, and the book was withdrawn from the market. The book is now a minor collector's item. Professors Becker and Wilson vigorously responded. They laid blame for the debacle on the publisher and also claimed they were the victims of a witch hunt by feminists. The Anatomical Basis of Medical Practice appeared as the women's movement became part of the American popular consciousness. It was also an era in which the public began to grapple with how to define pornography. Professor Becker and his coauthors thought that they were writing a witty, engaging, and funny book. Their detractors thought the book denigrated women.

Teaching, Learning and Education in Late Modernity: The Selected Works of Peter Jarvis. World Library of Educationalists

ERIC Educational Resources Information Center

Jarvis, Peter

2011-01-01

Professor Peter Jarvis has spent over 30 years researching, thinking and writing about some of the key and enduring issues in education. He has contributed well over 30 books and 200 papers and chapters in books on learning theory, adult education and learning, continuing professional education, nurse education, primary school education, distance…

Pratt & Whitney Advanced Ducted Propulsor (ADP) Engine Test in 40x80ft w.t.: Engineers Peter

NASA Technical Reports Server (NTRS)

1993-01-01

Pratt & Whitney Advanced Ducted Propulsor (ADP) Engine Test in 40x80ft w.t.: Engineers Peter Zell (left) and Dr Clifton Horne (right) are shown preparing a laser light sheet for a flow visualization test. Shown standing in the nacelle of the ADP is John Girvin, senior test engineer for Pratt & Whitney.

Functional changes in Becker muscular dystrophy: implications for clinical trials in dystrophinopathies.

PubMed

Bello, Luca; Campadello, Paola; Barp, Andrea; Fanin, Marina; Semplicini, Claudio; Sorarù, Gianni; Caumo, Luca; Calore, Chiara; Angelini, Corrado; Pegoraro, Elena

2016-09-01

We performed a 1-year longitudinal study of Six Minute Walk Test (6MWT), North Star Ambulatory Assessment (NSAA), and timed function tests in Becker muscular dystrophy (BMD). Skeletal muscle dystrophin was quantified by immunoblot. We grouped deletions ending on exon 45 ("del 45-x", n = 28) or 51 ("del x-51", n = 10); isolated exon 48 deletion ("del 48", n = 10); and other mutations (n = 21). Only patients in the "del 45-x" or "other" groups became non-ambulatory (n = 5, log-rank p = n.s.) or unable to run (n = 22, p < 0.001). All measures correlated positively with dystrophin quantity and negatively with age, and were significantly more impaired in the "del 45-x" and "other" groups. After one year, NSAA score decreased significantly (-0.9 ± 1.6, p < 0.001); in the "del 45-x" group, both NSAA (-1.3 ± 1.7, p = 0.001) and 6MWT (-12 ± 31 m, p = 0.059) decreased. We conclude that patients with "del x-51" or "del 48" mutations have mild or asymptomatic BMD, while "del 45-x" mutations cause comparatively severe weakness, and functional deterioration in 1 year. Furthermore, exon 51 skipping could be more effective than exon 45 skipping in Duchenne muscular dystrophy.

Forest inventory: Peter T. Johnson Wildlife Mitigation Unit, Craig Mountain, Idaho. Final Report.

DOE Office of Scientific and Technical Information (OSTI.GOV)

Narolski, Steven W.

The primary objective of this report is to determine the quantity and quality of existing forest habitat types on the 59,991-acre Peter T. Johnson Wildlife Mitigation Unit (WMU). Products from this effort include a description of the ecological condition, a map of habitat types, and an inventory of forest resources on the WMU lands. The purpose of this and other resource inventories (plant and wildlife) is to assess the current resources condition of the WMU and to provide necessary information to generate a long-term management for this area.

Serum creatinine level: a supplemental index to distinguish Duchenne muscular dystrophy from Becker muscular dystrophy.

PubMed

Zhang, Huili; Zhu, Yuling; Sun, Yiming; Liang, Yingyin; Li, Yaqin; Zhang, Yu; Deng, Langhui; Wen, Xingxuan; Zhang, Cheng

2015-01-01

To improve assessment of dystrophinopathy, the aim of this study was to identify whether serum creatinine (Crn) level reflects disease severity. Biochemical, Vignos score, and genetic data were collected on 212 boys with dystrophinopathy. 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). Serum Crn level reflected disease severity and may serve as a supplemental index to distinguish DMD from BMD in clinical practice.

PEOPLE IN PHYSICS: Interview with Professor Peter Kalmus

NASA Astrophysics Data System (ADS)

Wilson, Conducted by Catherine

1998-07-01

Peter Kalmus was born in 1933. He obtained his BSc and PhD at University College London. After a further period as a Research Associate he spent some years in America. He has been at Queen Mary and Westfield College (formerly just QMC) of the University of London since 1964, becoming Professor in 1978 and Head of Department from 1992 to 1997. He is Vice President of the Institute of Physics and also of the Royal Institution. Professor Kalmus is a well-known popularizer of physics and is active in the public understanding of science. He is a former Physics President of the British Association. He is an eminent researcher and received the Rutherford Medal and Prize for his contributions to the discovery of the W and Z particles, the carriers of the weak force. During 1998-99 he will be delivering the Institute's Schools and Colleges Lecture `Particles and the Universe', which will incorporate some of the new IOP 16-19 Physics curriculum material, in many UK locations.

Writing the True Self: Case Studies of Eng 101 Students Responding to a Peter Elbow-Inspired Curriculum

ERIC Educational Resources Information Center

Stansell, Jon W.

2012-01-01

My fulfillment in life comes through helping others develop their potential, work through their troubles, and follow their dreams. I have found that my college students embrace this aspect as I teach them expressive, author-involved writing. Peter Elbow's work asserts that this type of writing fits into his theory of personal development, which is…

Pratt & Whitney Advanced Ducted Propulsor (ADP) Engine Test in 40x80ft w.t.: Engineers Peter

NASA Technical Reports Server (NTRS)

1993-01-01

Pratt & Whitney Advanced Ducted Propulsor (ADP) Engine Test in 40x80ft w.t.: Engineers Peter Zell (left) and Dr Clifton Horne (right) are shown preparing for a laser light sheet for a flow visualization test. Shown standing in the nacelle of the ADP is John Girvin, senior test engineer for Pratt & Whitney.

Prevalence and Genetic Profile of Duchene and Becker Muscular Dystrophy in Puerto Rico.

PubMed

Ramos, Edwardo; Conde, José G; Berrios, Rafael Arias; Pardo, Sherly; Gómez, Omar; Mas Rodríguez, Manuel F

2016-05-27

Duchenne and Becker Muscular Dystrophy (DMD and BMD, respectively), are common forms of inherited muscle disease. Information regarding the epidemiology of these conditions, including genotype, is still sparse. To establish the prevalence and genetic profile of DMD and BMD in Puerto Rico. We collected data from medical records in all Muscular Dystrophy Association (MDA) clinics in Puerto Rico in order to estimate the prevalence of DMD and BMD and to describe the genotypic profile of these patients. Patients selected for data analysis matched "definite", "probable" and "possible" case definitions as established by MD STARnet. A total of 141 patients matched the inclusion criteria, with 64.5% and 35.5% being categorized into DMD and BMD, respectively. DMD and BMD prevalence in Puerto Rico was estimated at 5.18 and 2.84 per 100,000 males, respectively. Deletion was the most common form of mutation (66.7%) in the dystrophin gene, with exons in segment 45 to 47 being the most frequently affected. This is the first report of the prevalence and genetic profile characteristics of DMD and BMD in Puerto Rico. Prevalence of DMD was similar to that reported worldwide, while prevalence of BMD was higher. Genetic profile was consistent with that reported in the literature.

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

PubMed

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.

A case report: Becker muscular dystrophy presenting with epilepsy and dysgnosia induced by duplication mutation of Dystrophin gene.

PubMed

Miao, Jing; Feng, Jia-Chun; Zhu, Dan; Yu, Xue-Fan

2016-12-12

Becker muscular dystrophy (BMD), a genetic disorder of X-linked recessive inheritance, typically presents with gradually progressive muscle weakness. The condition is caused by mutations of Dystrophin gene located at Xp21.2. Epilepsy is an infrequent manifestation of BMD, while cases of BMD with dysgnosia are extremely rare. We describe a 9-year-old boy with BMD, who presented with epilepsy and dysgnosia. Serum creatine kinase level was markedly elevated (3665 U/L). Wechsler intelligence tests showed a low intelligence quotient (IQ = 65). Electromyogram showed slight myogenic changes and skeletal muscle biopsy revealed muscular dystrophy. Immunohistochemical staining showed partial positivity of sarcolemma for dystrophin-N. Multiplex ligation-dependent probe amplification revealed a duplication mutation in exons 37-44 in the Dystrophin gene. The present case report helps to better understand the clinical and genetic features of BMD.

A Novel Mutation in DMD (c.10797+5G>A) Causes Becker Muscular Dystrophy Associated with Intellectual Disability.

PubMed

Banihani, Rudaina; Baskin, Berivan; Halliday, William; Kobayashi, Jeff; Kawamura, Anne; McAdam, Laura; Ray, Peter N; Yoon, Grace

2016-04-01

Severe intellectual disability has been reported in a subgroup of patients with Duchenne muscular dystrophy but is not typically associated with Becker muscular dystrophy. The authors report a 13-year-old boy, with severe intellectual disability (Wechsler Intelligence Scales for Children-IV, Full Scale IQ < 0.1 percentile), attention-deficit hyperactivity disorder, and mild muscle weakness. He had elevated serum creatine kinase and dystrophic changes on muscle biopsy. Dystrophin immunohistochemistry revealed decreased staining with the C-terminal and mid-rod antibodies and essentially absent staining of the N-terminal immunostain. Sequencing of muscle mRNA revealed aberrant splicing due to a c.10797+5G > A mutation in DMD. Dystrophinopathy may be associated with predominantly cognitive impairment and neurobehavioral disorder, and should be considered in the differential diagnosis of unexplained cognitive or psychiatric disturbance in males.

Serials Management in the Electronic Era: Papers in Honor of Peter Gellatly, Founding Editor of "The Serials Librarian."

ERIC Educational Resources Information Center

Cole, Jim, Ed.; Williams, James W., Ed.

This book assesses progress and technical changes in the field of serials management and anticipates future directions and challenges for librarians. The book consists of 18 chapters: (1) "Introduction" (Jim Cole and James W. Williams); (2) "Peter Gellatly--Editor with a Deft Touch" (Ruth C. Carter); (3) "The "Deseret…

A new species of the genus Diplocentrus Peters, 1861 (Scorpiones, Diplocentridae) from Oaxaca, Mexico

PubMed Central

Santibáñez-López, Carlos Eduardo

2014-01-01

Abstract A new species of the genus Diplocentrus Peters, 1861 is described, based on several specimens collected in the Mexican state of Oaxaca. It is characterized by a high telotarsal spiniform setae count (4-5/5:5/6:6/6:6/6-7), and the pectinal tooth counts of 12–15, mode = 13 (male) or 11–13, mode = 12 (female). With the description of this species, the diversity of the genus is increased to 51 species in Mexico. PMID:24899864

"The physician as poet" review of: Pereira, Peter Saying the World

PubMed Central

Weishaus, Joel

2006-01-01

Peter Pereira is a family physician and a poet. I weave excerpts from Dr. Pereira's poems into a brief history of medicine's mythological and historical roots, beginning with the Egyptian god Thoth, and the Greek physician Hippocrates. Along the way, I touch on the European Middle Ages and the Islamic World. Finally, I quote poet-critic T.S. Eliot, who was an early influence on Dr. Pereira's decision to become a poet, and contemporary physician-poets Rafael Campo and William Carlos Williams. I end by placing Dr. Pereira, whose practice is oriented toward immigrant families, in his indigenous Pacific Northwest, arguing that being both physician and poet helps Pereira to live in a world that is both intimately human and naturally impersonal.

Analog of the Peter-Weyl expansion for Lorentz group

NASA Astrophysics Data System (ADS)

Perlov, Leonid

2015-11-01

The expansion of a square integrable function on SL(2, C) into the sum of the principal series matrix coefficients with the specially selected representation parameters was recently used in the Loop Quantum Gravity [C. Rovelli and F. Vidotto, Covariant Loop Quantum Gravity: An Elementary Introduction to Quantum Gravity and Spinfoam Theory (Cambridge University Press, Cambridge, 2014) and C. Rovelli, Classical Quantum Gravity 28(11), 114005 (2011)]. In this paper, we prove that the sum used originally in the Loop Quantum Gravity: ∑ j = 0 ∞ ∑ |m| ≤ j ∑ |n| ≤ j Dj m , j n ( j , τ j ) ( g ) , where j, m, n ∈ Z, τ ∈ C is convergent to a function on SL(2, C); however, the limit is not a square integrable function; therefore, such sums cannot be used for the Peter-Weyl like expansion. We propose the alternative expansion and prove that for each fixed m: ∑ j = m ∞ D j m , j m ( j , τ j ) ( g ) is convergent and that the limit is a square integrable function on SL(2, C). We then prove the analog of the Peter-Weyl expansion: any ψ(g) ∈ L2(SL(2, C)) can be decomposed into the sum: ψ ( g ) = ∑ j = m ∞ j 2 ( 1 + τ 2 ) c j m m D j m , j m ( j , τ j ) ( g ) , with the Fourier coefficients c j m m = ∫ S L ( 2 , C ) ψ ( g ) Dj m , j m j , τ j ( g ) ¯ d g , g ∈ SL(2, C), τ ∈ C, τ ≠ i, - i, j, m ∈ Z, m is fixed. We also prove convergence of the sums ∑ j = |p| ∞ ∑ |m| ≤ j ∑ |n| ≤ j dp m /j 2 Dj m , j n ( j , τ j ) ( g ) , where d|p| m /j 2 = ( j + 1 ) /1 2 ∫ S U ( 2 ) ϕ ( u ) D|p| m /j 2 ( u ) ¯ d u is ϕ(u)'s Fourier transform and p, j, m, n ∈ Z, τ ∈ C, u ∈ SU(2), g ∈ SL(2, C), thus establishing the map between the square integrable functions on SU(2) and the space of the functions on SL(2, C). Such maps were first used in Rovelli [Class. Quant. Grav. 28, 11 (2011)].

Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy.

PubMed

Martin, Elizabeth A; Barresi, Rita; Byrne, Barry J; Tsimerinov, Evgeny I; Scott, Bryan L; Walker, Ashley E; Gurudevan, Swaminatha V; Anene, Francine; Elashoff, Robert M; Thomas, Gail D; Victor, Ronald G

2012-11-28

Becker muscular dystrophy (BMD) is a progressive X-linked muscle wasting disease for which there is no treatment. Like Duchenne muscular dystrophy (DMD), BMD is caused by mutations in the gene encoding dystrophin, a structural cytoskeletal protein that also targets other proteins to the muscle sarcolemma. Among these is neuronal nitric oxide synthase (nNOSμ), which requires certain spectrin-like repeats in dystrophin's rod domain and the adaptor protein α-syntrophin to be targeted to the sarcolemma. When healthy skeletal muscle is subjected to exercise, sarcolemmal nNOSμ-derived NO attenuates local α-adrenergic vasoconstriction, thereby optimizing perfusion of muscle. We found previously that this protective mechanism is defective-causing functional muscle ischemia-in dystrophin-deficient muscles of the mdx mouse (a model of DMD) and of children with DMD, in whom nNOSμ is mislocalized to the cytosol instead of the sarcolemma. We report that this protective mechanism also is defective in men with BMD in whom the most common dystrophin mutations disrupt sarcolemmal targeting of nNOSμ. In these men, the vasoconstrictor response, measured as a decrease in muscle oxygenation, to reflex sympathetic activation is not appropriately attenuated during exercise of the dystrophic muscles. In a randomized placebo-controlled crossover trial, we show that functional muscle ischemia is alleviated and normal blood flow regulation is fully restored in the muscles of men with BMD by boosting NO-cGMP (guanosine 3',5'-monophosphate) signaling with a single dose of the drug tadalafil, a phosphodiesterase 5A inhibitor. These results further support an essential role for sarcolemmal nNOSμ in the normal modulation of sympathetic vasoconstriction in exercising human skeletal muscle and implicate the NO-cGMP pathway as a putative new target for treating BMD.

Tadalafil alleviates muscle ischemia in patients with Becker muscular dystrophy

PubMed Central

Martin, Elizabeth A.; Barresi, Rita; Byrne, Barry J.; Tsimerinov, Evgeny I.; Scott, Bryan L.; Walker, Ashley E.; Gurudevan, Swaminatha V.; Anene, Francine; Elashoff, Robert M.; Thomas, Gail D.; Victor, Ronald G.

2013-01-01

Becker muscular dystrophy (BMD) is a progressive X-linked muscle wasting disease for which there is no treatment. Like Duchenne muscular dystrophy (DMD), BMD is caused by mutations in the gene encoding dystrophin, a structural cytoskeletal protein that also targets other proteins to the muscle sarcolemma. Among these is neuronal nitric oxide synthase (nNOSμ), which requires certain spectrin-like repeats in dystrophin’s rod domain and the adaptor protein α-syntrophin to be targeted to the sarcolemma. When healthy skeletal muscle is subjected to exercise, sarcolemmal nNOSμ-derived nitric oxide (NO) attenuates local α-adrenergic vasoconstriction thereby optimizing perfusion of muscle. We found previously that this protective mechanism is defective—causing functional muscle ischemia—in dystrophin-deficient muscles of the mdx mouse (a model of DMD) and of children with DMD, in whom nNOSμ is mislocalized to the cytosol instead of the sarcolemma. Here, we report that this protective mechanism also is defective in men with BMD in whom the most common dystrophin mutations disrupt sarcolemmal targeting of nNOSμ. In these men, the vasoconstrictor response, measured as a decrease in muscle oxygenation, to reflex sympathetic activation is not appropriately attenuated during exercise of the dystrophic muscles. In a randomized placebo-controlled cross-over trial, we show that functional muscle ischemia is alleviated and normal blood flow regulation fully restored in the muscles of men with BMD by boosting NO-cGMP signaling with a single dose of the drug tadalafil, a phosphodiesterase (PDE5A) inhibitor. These results further support an essential role for sarcolemmal nNOSμ in the normal modulation of sympathetic vasoconstriction in exercising human skeletal muscle and implicate the NO-cGMP pathway as a putative new target for treating BMD. PMID:23197572

Paleozoic fluid history of the Michigan Basin: Evidence from dolomite geochemistry in the Middle Ordovician St. Peter Sandstone

DOE Office of Scientific and Technical Information (OSTI.GOV)

Winter, B.L.; Johnson, C.M.; Simo, J.A.

1995-04-03

The isotope (Sr and O) and elemental (Mg, Ca, Mn, Fe, and Sr) compositions of the various dolomites in the Middle Ordovician St. Peter Sandstone in the Michigan Basin are determined and the variations are modeled in terms of fluid-rock interaction or as mixing relations. These geochemical models, combined with the paragenetic sequence of the dolomites and late anhydrite cement, suggest the existence of at least four distinct diagenetic fluids in the St. Peter Sandstone during the paleozoic. Fluid 1 has a composition consistent with a modified older (pre-Middle Ordovician) seawater origin, which indicates that the flow path for thismore » fluid had a major upward component. This fluid resulted in the first and volumetrically most important burial dolomitization event, producing dolomite in both carbonate and quartz sandstone lithofacies in the St. Peter Sandstone. Fluid 2 has a composition consistent with a modified Middle to early Late Ordovician seawater origin, suggesting a major downward component for fluid flow. Fluid 2 produced dolomite cement in the carbonate lithofacies that postdates Fluid 1 dolomite. The composition of Fluid 3 is best interpreted to reflect a heated, deep basinal brine that had previously interacted with the K-feldspar-rich rocks near the Cambrian-Precambrian unconformity in the Michigan Basin, indicating a major upward component for fluid flow. Fluid 3 produced dolomite cement in quartz sandstone lithofacies that postdates Fluid 1 dolomite. Fluid 4 resulted in precipitation of late anhydrite in fractures. The {sup 87}Sr/{sup 86}Sr ratio of the anhydrite is consistent with Fluid 4 originating as a dilute fluid that interacted extensively with Silurian gypsum in the Michigan Basin; this indicates that the flow path of Fluid 4 had a major downward component.« less

Saving Time and Money in Michigan: Peter J. Young Talks about Professional Development, Parent Communication, Funding, and Partnerships

ERIC Educational Resources Information Center

Branzburg, Jeffrey

2007-01-01

This article presents an interview with Peter J. Young, Director of Technology at Rockford Public Schools in Michigan. In the interview, Young talked about how his district has done a lot more automation to integrate its disparate systems. He also discussed how they streamline their systems, how parents and community benefit from these efforts,…

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

PubMed

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.

CO{sub 2} Injectivity, Storage Capacity, Plume Size, and Reservoir and Seal Integrity of the Ordovician St. Peter Sandstone and the Cambrian Potosi Formation in the Illnois Basin

DOE Office of Scientific and Technical Information (OSTI.GOV)

Leetaru, Hannes; Brown, Alan; Lee, Donald

2012-05-01

The Cambro-Ordovician strata of the Illinois and Michigan Basins underlie most of the states of Illinois, Indiana, Kentucky, and Michigan. This interval also extends through much of the Midwest of the United States and, for some areas, may be the only available target for geological sequestration of CO{sub 2}. We evaluated the Cambro-Ordovician strata above the basal Mt. Simon Sandstone reservoir for sequestration potential. The two targets were the Cambrian carbonate intervals in the Knox and the Ordovician St. Peter Sandstone. The evaluation of these two formations was accomplished using wireline data, core data, pressure data, and seismic data frommore » the USDOE-funded Illinois Basin Decatur Project being conducted by the Midwest Geological Sequestration Consortium in Macon County, Illinois. Interpretations were completed using log analysis software, a reservoir flow simulator, and a finite element solver that determines rock stress and strain changes resulting from the pressure increase associated with CO{sub 2} injection. Results of this research suggest that both the St. Peter Sandstone and the Potosi Dolomite (a formation of the Knox) reservoirs may be capable of storing up to 2 million tonnes of CO{sub 2} per year for a 20-year period. Reservoir simulation results for the St. Peter indicate good injectivity and a relatively small CO{sub 2} plume. While a single St. Peter well is not likely to achieve the targeted injection rate of 2 million tonnes/year, results of this study indicate that development with three or four appropriately spaced wells may be sufficient. Reservoir simulation of the Potosi suggest that much of the CO{sub 2} flows into and through relatively thin, high permeability intervals, resulting in a large plume diameter compared with the St. Peter.« less

Some single-piston closed-cycle machines and Peter Tailer's thermal lag engine

DOE Office of Scientific and Technical Information (OSTI.GOV)

West, C.D.

1993-01-01

Peter Tailer has devised, built, and operated a beautifully simple engine with a closed working gas cycle, external heating, and only a single piston. The aim of this paper is to cast some light on the possible modes of operation for his machine. The methods develops to analyze certain aspects of Stirling cycle engines, and especially the thermodynamic losses incurred in systems that are neither perfectly isothermal nor perfectly adiabatic, can be applied to Tailer's system. The results identify two idealized cycles fr such machines; relate those cycles to a single piston, ported cylinder machine proposed earlier; and offer amore » possible explanation for the success of the thermal lag engine.« less

Bilateral anterior segment dysgenesis with the presumed Peters' anomaly in a cat.

PubMed

Park, Sangwan; Kim, Kiwoong; Kim, Youngbeum; Seo, Kangmoon

2018-02-20

A seven-month-old female domestic shorthaired cat was presented for buphthalmos in the right eye and corneal cloudiness in the left eye. Full ophthalmic examinations were performed for both eyes and enucleation was done for the right nonvisual eye. Congenital glaucoma caused by anterior segment dysgenesis was confirmed for the right eye. In the left eye, slit-lamp examination revealed focal corneal edema with several iris strands from iris collarette to the affected posterior corneal surfaces. Circular posterior corneal defect was suggested to be the cause of edema. Goniodysgenesis, additionally, was identified. Taken together, the diagnosis of Peters' anomaly which is a subtype of anterior segment dysgenesis was suggested in the left eye.

A phase 1/2a follistatin gene therapy trial for becker muscular dystrophy.

PubMed

Mendell, Jerry R; Sahenk, Zarife; Malik, Vinod; Gomez, Ana M; Flanigan, Kevin M; Lowes, Linda P; Alfano, Lindsay N; Berry, Katherine; Meadows, Eric; Lewis, Sarah; Braun, Lyndsey; Shontz, Kim; Rouhana, Maria; Clark, Kelly Reed; Rosales, Xiomara Q; Al-Zaidy, Samiah; Govoni, Alessandra; Rodino-Klapac, Louise R; Hogan, Mark J; Kaspar, Brian K

2015-01-01

Becker muscular dystrophy (BMD) is a variant of dystrophin deficiency resulting from DMD gene mutations. Phenotype is variable with loss of ambulation in late teenage or late mid-life years. There is currently no treatment for this condition. In this BMD proof-of-principle clinical trial, a potent myostatin antagonist, follistatin (FS), was used to inhibit the myostatin pathway. Extensive preclinical studies, using adeno-associated virus (AAV) to deliver follistatin, demonstrated an increase in strength. For this trial, we used the alternatively spliced FS344 to avoid potential binding to off target sites. AAV1.CMV.FS344 was delivered to six BMD patients by direct bilateral intramuscular quadriceps injections. Cohort 1 included three subjects receiving 3 × 10(11) vg/kg/leg. The distance walked on the 6MWT was the primary outcome measure. Patients 01 and 02 improved 58 meters (m) and 125 m, respectively. Patient 03 showed no change. In Cohort 2, Patients 05 and 06 received 6 × 10(11) vg/kg/leg with improved 6MWT by 108 m and 29 m, whereas, Patient 04 showed no improvement. No adverse effects were encountered. Histological changes corroborated benefit showing reduced endomysial fibrosis, reduced central nucleation, more normal fiber size distribution with muscle hypertrophy, especially at high dose. The results are encouraging for treatment of dystrophin-deficient muscle diseases.

A Phase 1/2a Follistatin Gene Therapy Trial for Becker Muscular Dystrophy

PubMed Central

Mendell, Jerry R; Sahenk, Zarife; Malik, Vinod; Gomez, Ana M; Flanigan, Kevin M; Lowes, Linda P; Alfano, Lindsay N; Berry, Katherine; Meadows, Eric; Lewis, Sarah; Braun, Lyndsey; Shontz, Kim; Rouhana, Maria; Clark, Kelly Reed; Rosales, Xiomara Q; Al-Zaidy, Samiah; Govoni, Alessandra; Rodino-Klapac, Louise R; Hogan, Mark J; Kaspar, Brian K

2015-01-01

Becker muscular dystrophy (BMD) is a variant of dystrophin deficiency resulting from DMD gene mutations. Phenotype is variable with loss of ambulation in late teenage or late mid-life years. There is currently no treatment for this condition. In this BMD proof-of-principle clinical trial, a potent myostatin antagonist, follistatin (FS), was used to inhibit the myostatin pathway. Extensive preclinical studies, using adeno-associated virus (AAV) to deliver follistatin, demonstrated an increase in strength. For this trial, we used the alternatively spliced FS344 to avoid potential binding to off target sites. AAV1.CMV.FS344 was delivered to six BMD patients by direct bilateral intramuscular quadriceps injections. Cohort 1 included three subjects receiving 3 × 1011 vg/kg/leg. The distance walked on the 6MWT was the primary outcome measure. Patients 01 and 02 improved 58 meters (m) and 125 m, respectively. Patient 03 showed no change. In Cohort 2, Patients 05 and 06 received 6 × 1011 vg/kg/leg with improved 6MWT by 108 m and 29 m, whereas, Patient 04 showed no improvement. No adverse effects were encountered. Histological changes corroborated benefit showing reduced endomysial fibrosis, reduced central nucleation, more normal fiber size distribution with muscle hypertrophy, especially at high dose. The results are encouraging for treatment of dystrophin-deficient muscle diseases. PMID:25322757

Peter Fensham--Head, Heart and Hands (on) in the Service of Science Education and Social Equity and Justice

ERIC Educational Resources Information Center

Gunstone, Richard

2009-01-01

When Peter Fensham was appointed to the new Chair of Science Education at Monash University in 1967 he was the first Professor of Science Education in Australia, and, we think, may well have been the first such professor anywhere in the world outside USA. Over the subsequent 40+ years he has made/still makes remarkable and diverse contributions to…

The Global Food Crisis: Causes and Solutions. Statement of Peter McPherson before the Senate Foreign Relations Committee

ERIC Educational Resources Information Center

National Association of State Universities and Land-Grant Colleges, 2008

2008-01-01

This paper presents Peter McPherson's statement before the Senate Foreign Relations Committee. He begins by saying that people have an immediate problem that must be addressed, hopefully in a way that does not complicate long-term solutions. They also have a long-term and more complicated agricultural problem that the world has neglected over many…

Communication; A Discussion at the Nobel Conference (5th, Gustavus Adolphus College, St. Peter, Minnesota, January 8-9, 1969).

ERIC Educational Resources Information Center

Roslansky, John D., Ed.

This book consists of five lectures on communication given at the fifth Nobel Conference. Leroy G. Augenstein explores the positive and negative consequences of man's increasing capacity to manipulate and control the human mind. Peter Marler demonstrates that all the elements necessary for a communication system to qualify as a language exist…

Abnormal short-latency synaptic plasticity in the motor cortex of subjects with Becker muscular dystrophy: a rTMS study.

PubMed

Golaszewski, Stefan; Schwenker, Kerstin; Bergmann, Jürgen; Brigo, Francesco; Christova, Monica; Trinka, Eugen; Nardone, Raffaele

2016-01-01

We used repetitive transcranial magnetic stimulation (rTMS) to further investigate motor cortex excitability in 13 patients with Becker muscular dystrophy (BMD), six of them with slight mental retardation. RTMS delivered at 5Hz frequency and suprathreshold intensity progressively increases the size of motor evoked potentials (MEPs) in healthy subjects; the rTMS-induced facilitation of MEPs was significantly reduced in the BMD patients mentally retarded or classified as borderline when compared with age-matched control subjects and the BMD patients with normal intelligence. The increase in the duration of the cortical silent period was similar in both patient groups and controls. These findings suggest an altered cortical short-term synaptic plasticity in glutamate-dependent excitatory circuits within the motor cortex in BMD patients with intellectual disabilities. RTMS studies may shed new light on the physiological mechanisms of cortical involvement in dystrophinopathies. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Yes! There is an ethics of care: an answer for Peter Allmark.

PubMed Central

Bradshaw, A

1996-01-01

This paper is a response to Peter Allmark's thesis that 'there can be no "caring" ethics'. It argues that the current preoccupation in nursing to define an ethics of care is a direct result of breaking nursing tradition. Subsequent attempts to find a moral basis for care, whether from subjective experimental perspectives such as described by Noddings, or from rational and detached approaches derived from Kant, are inevitably flawed. Writers may still implicitly presuppose a concept of care drawn from the Judaeo-Christian tradition but without explicit recourse to its moral basis nursing is left rudderless and potentially without purpose. The very concept of 'care' cut off from its roots becomes a meaningless term without either normative or descriptive content. PMID:8932719

Some single-piston closed-cycle machines and Peter Tailer`s thermal lag engine

DOE Office of Scientific and Technical Information (OSTI.GOV)

West, C.D.

1993-06-01

Peter Tailer has devised, built, and operated a beautifully simple engine with a closed working gas cycle, external heating, and only a single piston. The aim of this paper is to cast some light on the possible modes of operation for his machine. The methods develops to analyze certain aspects of Stirling cycle engines, and especially the thermodynamic losses incurred in systems that are neither perfectly isothermal nor perfectly adiabatic, can be applied to Tailer`s system. The results identify two idealized cycles fr such machines; relate those cycles to a single piston, ported cylinder machine proposed earlier; and offer amore » possible explanation for the success of the thermal lag engine.« less

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

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hagiwara, Yoko; Nishio, Hisahide; Kitoh, Yoshihiko

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 dystrophinmore » 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.« less

Psychosocial Needs and Facilitators of Mothers Caring for Children with Duchenne/Becker Muscular Dystrophy.

PubMed

Peay, Holly L; Meiser, Bettina; Kinnett, Kathleen; Tibben, Aad

2018-02-01

Care guidelines for Duchenne/Becker muscular dystrophy (DBMD) include recommendations for assessment of caregivers of patients with DBMD followed by proactive psychosocial interventions. To inform clinical assessment, this study described appraisals of psychosocial needs and caregiving facilitators of mothers of individuals with DBMD. Two hundred and five mothers completed an online survey. More than 50% endorsed unmet needs for managing uncertainty about the future and managing DBMD fears. Higher levels of unmet need were associated with less disease progression/earlier stage of DBMD (rho = -0.166 p = 0.02). Twenty-one percent regularly used respite care and 57% worried about allowing others to care for their child. Highly-endorsed care facilitators included partner relationships (63%), child's approach to life (59%), and family relationships (49%). Our findings highlight the importance of psychological and social support for caregivers. Starting when children are young, clinicians should assess caregivers' unmet psychological needs, particularly uncertainty and fear. Exploring needs and facilitators may allow clinics to target and customize interventions that build upon existing strengths and supports. Our findings have implications for efforts to promote early diagnosis and newborn screening, in that increased needs in mothers of younger children should be anticipated and built into counseling. Further research can assess whether and how unmet needs change as new therapies become available.

[Specific features of Becker Muscular Dystrophy patients and female carriers of Duchenne Muscular Dystrophy].

PubMed

Magot, A; Mercier, S; Péréon, Y

2015-12-01

Becker muscular dystrophy (BMD) was first described in 1955 and linked to the DMD gene in 1987. Compared to Duchenne muscular dystrophy (DMD), clinical onset of BMD usually occurs after the age of 12 and wheelchair is required after the age of 16. BMD is characterized by generalized weakness first affecting limb girdle muscles, hypertrophy of the calves and cardiomyopathy in males. Some patients have only mild symptoms such as cramps or elevated serum creatine kinases (SCK) throughout all their lives. SCK levels are usually elevated. Muscle biopsy (immunohistochemistry or immunoblotting) shows a dystrophic pattern with abnormal dystrophin staining. Diagnosis is confirmed by DMD gene sequencing. Deletions or duplications of one or several exons are identified in the majority of cases. A multidisciplinary approach is recommended for the care management of these patients with a particular attention to the cardiomyopathy, which is typically responsible for death but can be prevented by specific treatment. X-linked dilated cardiomyopathies linked to DMD gene are a phenotypic continuum of BMD. Some female carriers of DMD mutations exhibit clinical symptoms of variable severity, often milder and beginning later than in males. The cardiomyopathy is the most frequent feature that should be especially monitored in these patients. Genetic counselling should be systematically proposed. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Dr. Peter Cavanaugh Explains the Need and Operation of the FOOT Experiment

NASA Technical Reports Server (NTRS)

2003-01-01

This video clip is an interview with Dr. Peter Cavanaugh, principal investigator for the FOOT experiment. He explains the reasoning behind the experiment and shows some video clips of the FOOT experiment being calibrated and conducted in orbit. The heart of the FOOT experiment is an instrumented suit called the Lower Extremity Monitoring Suit (LEMS). This customized garment is a pair of Lycra cycling tights incorporating 20 carefully placed sensors and the associated wiring control units, and amplifiers. LEMS enables the electrical activity of the muscles, the angular motions of the hip, knee, and ankle joints, and the force under both feet to be measured continuously. Measurements are also made on the arm muscles. Information from the sensors can be recorded up to 14 hours on a small, wearable computer.

Prenatal molecular diagnosis of inherited neuromuscular diseases: Duchenne/Becker muscular dystrophy, myotonic dystrophy type 1 and spinal muscular atrophy.

PubMed

Esposito, Gabriella; Ruggiero, Raffaella; Savarese, Maria; Savarese, Giovanni; Tremolaterra, Maria Roberta; Salvatore, Francesco; Carsana, Antonella

2013-12-01

Neuromuscular disease is a broad term that encompasses many diseases that either directly, via an intrinsic muscle disorder, or indirectly, via a nerve disorder, impairs muscle function. Here we report the experience of our group in the counselling and molecular prenatal diagnosis of three inherited neuromuscular diseases, i.e., Duchenne/Becker muscular dystrophy (DMD/BMD), myotonic dystrophy type 1 (DM1), spinal muscular atrophy (SMA). We performed a total of 83 DMD/BMD, 15 DM1 and 54 SMA prenatal diagnoses using a combination of technologies for either direct or linkage diagnosis. We identified 16, 5 and 10 affected foetuses, respectively. The improvement of analytical procedures in recent years has increased the mutation detection rate and reduced the analytical time. Due to the complexity of the experimental procedures and the high, specific professional expertise required for both laboratory activities and the related counselling, these types of analyses should be preferentially performed in reference molecular diagnostic centres.

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

PubMed

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

Making sense of inequalities: a response to Peter Townsend.

PubMed

Klein, R

1991-01-01

This article addresses the continuing controversy generated by the Black Report on Inequalities in Health, published in Britain in 1980, in response to the defense offered by Professor Peter Townsend. The author argues that Townsend's riposte to the critics of the Black Report is flawed in at least two respects. First, Townsend fails to acknowledge that the Black Report was as much an exercise in policy advocacy as in scholarly analysis, making rather large assumptions about the links in the reasoning leading to its recommendations for a massive program of income redistribution. Second, Townsend's defense of Black's use of social class as its main tool for analyzing health inequalities dismisses too easily much of the evidence; for example, the effects of social mobility and the historical dimension. Moreover, by concentrating on social class, a heterogeneous category, analysis may ignore what is most relevant for policy-making: i.e., specific factors associated with specific forms of deprivation, located within social classes or particular geographical communities. It would therefore be more constructive if scholars were to accept and research this complexity, rather than defending the Black Report as though it were a definitive (not to say sacred) text.

Split Attention as Part of a Flexible Attentional System for Complex Scenes: Comment on Jans, Peters, and De Weerd (2010)

ERIC Educational Resources Information Center

Cave, Kyle R.; Bush, William S.; Taylor, Thalia G. G.

2010-01-01

Jans, Peters, and De Weerd (2010) examined the studies demonstrating that spatial attention can be split across 2 noncontiguous target locations. They find all these studies to be flawed and conclude that spatial attention only selects a single location at any given time. They do, however, suggest that there could be exceptional circumstances that…

Healing bodies or saving souls? Reverend Dr Peter Parker (1804-1888) as medical missionary.

PubMed

Fu, Louis

2016-05-01

The important role played by medical services in the preaching of the Gospel in China was undeniable. Anglo-American missionaries entered Canton in the early 18th century and introduced modern Western medicine to China. Reverend Dr Peter Parker, founder of medical missionaries to China, was more than that, far more advanced than his predecessors including Drs Pearson, Livingstone and Colledge. He was an enthusiastic missionary of exceptional ability and vigour as witnessed his labours at the Canton Ophthalmic Hospital. His 20 years in the medical field unexpectedly paved the way for his future career as a diplomat in the American Legation. © © IMechE 2014.

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

PubMed

Lindberg, Ulrich; Witting, Nanna; Jørgensen, Stine Lundgaard; Vissing, John; Rostrup, Egill; Larsson, Henrik Bo Wiberg; Kruuse, Christina

2017-01-01

Patients suffering from Becker muscular dystrophy (BMD) have dysfunctional dystrophin proteins and are deficient in neuronal nitric oxide synthase (nNOS) in muscles. This causes functional ischemia and contributes to muscle wasting. Similar functional ischemia may be present in brains of patients with BMD, who often have mild cognitive impairment, and nNOS may be important for the regulation of the microvascular circulation in the brain. We hypothesized that treatment with sildenafil, a phosphodiesterase type 5 inhibitor that potentiates nitric oxide responses, would augment both the blood oxygen 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 assessed by 3 T magnetic resonance (MR) scanning, evoked potentials, somatosensory task-induced BOLD functional MR imaging, regional and global perfusion, and angiography before and after 4 weeks of sildenafil, 20 mg (Revatio in gelatine capsules, oral, 3 times daily), or placebo treatment. Sildenafil increased the event-related sensory and visual BOLD response compared with placebo (p < 0.01). However, sildenafil did not alter CBF, measured by MR phase contrast mapping, or the arterial diameter of the middle cerebral artery, measured by MR angiography. We conclude that nNOS may play a role in event-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.

Sir Peter Medawar: science, creativity and the popularization of Karl Popper

PubMed Central

Calver, Neil

2013-01-01

Sir Peter Medawar was respected by scientists and literati alike. It was perhaps not surprising, then, that he would choose to involve himself in the ‘two cultures’ debate of 1959 and beyond. The focus of his intervention was the philosophy of Sir Karl Popper. However, Medawar's Popper was not the guru of falsification familiar from philosophy textbooks. Medawar's distinctive interpretation of Popper treated him instead as the source of insights into the role of creativity and imagination in scientific inquiry. This paper traces the context for Medawar's adoption of Popperian philosophy, together with its application before the debate. It then examines, within the context of the debate itself, the way in which Medawar attempted to reconcile scientific inquiry with literary practice. Medawar became increasingly convinced that not only was induction epistemologically unsound, but it was also damaging to the public role of the scientist. His construction of Popperianism would, he envisaged, provide a worthy alternative for scientists’ self-image.

The fruit flies (Diptera: Tephritidae) described by Theodor Becker from Iran and Western China revisited in the collections of the Zoological Institute, Saint-Petersburg and Museum für Naturkunde, Berlin.

PubMed

Korneyev, Severyn V; Korneyev, Valery A

2017-01-31

The type specimens of fruit flies described by Dr. Theodor Becker based on material collected in China (Xinjiang and Xizang) and Iran by Russian expeditions directed by Petr Kozlov and Mykola Zarudny are listed and figured. They are deposited in the collection of the Zoological Institute of Russian Academy of Sciences, Saint Petersburg with some duplicates in the Museum für Naturkunde, Berlin. Current concepts of the species, their morphological characters (illustrated by photographs of type specimens), current condition, and nomenclature are discussed.

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

PubMed

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.

Prevalence and Characteristics of Chinese Patients With Duchenne and Becker Muscular Dystrophy: A Territory Wide Collaborative Study in Hong Kong.

PubMed

Chan, Sophelia H S; Lo, Ivan F M; Cherk, Sharon W W; Cheng, Wai Wai; Fung, Eva L W; Yeung, Wai Lan; Ngan, Mary; Lee, Wing Cheong; Kwong, Ling; Wong, Suet Na; Ma, Che Kwan; Tai, Shuk Mui; Ng, Grace S F; Wu, Shun Ping; Wong, Virginia C N

2015-01-01

The aim of this collaborative study on Duchenne muscular dystrophy and Becker muscular dystrophy is to determine the prevalence and to develop data on such patients as a prelude to the development of registry in Hong Kong. Information on clinical and molecular findings, and patient care, was systematically collected in 2011 and 2012 from all Pediatric Neurology Units in Hong Kong. Ninety patients with dystrophinopathy were identified, and 83% has Duchenne muscular dystrophy. The overall prevalence of dystrophinopathy in Hong Kong in 2010 is 1.03 per 10 000 males aged 0 to 24 years. Among the Duchenne group, we observed a higher percentage (40.6%) of point mutations with a lower percentage (45.3%) of exon deletions in our patients when compared with overseas studies. Although we observed similar percentage of Duchenne group received scoliosis surgery, ventilation support, and cardiac treatment when compared with other countries, the percentage (25%) of steroid use is lower.

Facies identification based on conventional core-log integration: Middle Ordovician St. Peter sandstone, Michigan basin

DOE Office of Scientific and Technical Information (OSTI.GOV)

Wieczorek, L.M.; Barnes, D.A.

1989-08-01

Lithofacies comprising the massive sand in the Michigan basin are very similar to the lithofacies of the St. Peter Sandstone in parts of the adjacent Midwestern basin and arches region. Lithostratigraphic and biostratigraphic correlations justify application of the name St. Peter Sandstone to the gas-productive massive sand in the Michigan basin. Four lithofacies have been determined by core-log integration. The two lowermost lithofacies (1 and 2) were deposited in marginal-marine peritidal shoreface to sand-flat environments. Lithofacies 1A comprises cross-bedded, quartz-cemented quartzarenites. Lithofacies 1B comprises dolomitic, cross-bedded, subfeldspathic arenites. Lithofacies 1A is recognized in logs by low gamma-ray response (less thanmore » 20-25 API units) and photoelectric effect (Pe) of 1.8. Lithofacies 1B log characteristics include low gamma ray response (15-30 API units) and Pe of 2-2.5. Lithofacies 2 log signatures include moderate to high gamma-ray (30-120 API units) and Pe values that range from 2.5-3.5. The uppermost two lithofacies (3 and 4) were deposited in storm-dominated lower shoreface, to offshore, to distal marine shelf environments. Lithofacies 3 consists of burrowed, massive, wavy to planar stratified quartzarenites to subfeldspathic arenites. Log signature is generally low gamma-ray response (15-30 API units) and Pe that ranges from 1.8 to 2.5. Lithofacies 4 consists of massive, burrowed and bioturbated, dominantly authigenic clay and dolomite-cemented subfeldspathic arenite. Lithofacies 4 log response is moderate to high gamma-ray response (30-105 API units) and Pe ranging from 2.25 to 3.5.« less

The search for asbestos within the Peter Mitchell Taconite iron ore mine, near Babbitt, Minnesota.

PubMed

Ross, Malcolm; Nolan, Robert P; Nord, Gordon L

2008-10-01

Asbestos crystallizes within rock formations undergoing intense deformation characterized by folding, faulting, shearing, and dilation. Some of these conditions have prevailed during formation of the taconite iron ore deposits in the eastern Mesabi Iron Range of Minnesota. This range includes the Peter Mitchell Taconite Mine at Babbitt, Minnesota. The mine pit is over 8 miles long, up to 1 mile wide. Fifty three samples were collected from 30 sites within areas of the pit where faulting, shearing and folding occur and where fibrous minerals might occur. Eight samples from seven collecting sites contain significant amounts of ferroactinolite amphibole that is partially to completely altered to fibrous ferroactinolite. Two samples from two other sites contain ferroactinolite degraded to ropy masses of fibers consisting mostly of ferrian sepiolite as defined by X-ray diffraction and TEM and SEM X-ray spectral analysis. Samples from five other sites contain unaltered amphiboles, however some of these samples also contain a very small number of fiber bundles composed of mixtures of grunerite, ferroactinolite, and ferrian sepiolite. It is proposed that the alteration of the amphiboles was caused by reaction with water-rich acidic fluids that moved through the mine faults and shear zones. The fibrous amphiboles and ferrian sepiolite collected at the Peter Mitchell Mine composes a tiny fraction of one percent of the total rock mass of this taconite deposit; an even a smaller amount of these mineral fragments enter the ambient air during mining and milling. These fibrous minerals thus do not present a significant health hazard to the miners nor to those non-occupationally exposed. No asbestos of any type was found in the mine pit.

The Learning Curve in neurofeedback of Peter Van Deusen: A review article

PubMed Central

Ribas, Valdenilson Ribeiro; Ribas, Renata de Melo Guerra; Martins, Hugo André de Lima

2016-01-01

ABSTRACT The Learning Curve (TLC) in neurofeedback concept emerged after Peter Van Deusen compiled the results of articles on the expected electrical activity of the brain. This concept was subsequently tested on patients at four clinics in Atlanta between 1994 and 2001. The aim of this paper was to report the historical aspects of TLC. Articles published on the electronic databases MEDLINE/PubMed and Web of Science were reviewed. During patient evaluation, TLC investigates categories called disconnected, hot temporal lobes, reversal of alpha and beta waves, blocking, locking, and filtering or processing. This enables neuroscientists to use their training designs and, by means of behavioral psychology, to work on neuroregulation, as self-regulation for patients. TLC shows the relationships between electrical, mental and behavioral activity in patients. It also identifies details of patterns that can assist physicians in their choice of treatment. PMID:29213440

Intense Undular Bores on the Autumn Pycnocline of Shelf Waters of the Peter the Great Bay (Sea of Japan)

NASA Astrophysics Data System (ADS)

Dolgikh, G. I.; Novotryasov, V. V.; Yaroshchuk, I. O.; Permyakov, M. S.

2018-03-01

The results of field observations of an internal undular bore that were performed in a coastal zone of constant depth in the Sea of Japan are presented. A hydrodynamic model of undular bores is discussed according to which the recorded disturbances of the water medium are an experimental prototype of strongly nonlinear (intense) internal undular bores on the pycnocline of shelf waters of Peter the Great Bay with an intensity close to the limit.

Pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy.

PubMed

Lager, Christina; Kroksmark, Anna-Karin

2015-09-01

The purpose of this study was to explore the prevalence, nature and scope of pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy and whether the pain differs between diagnostic groups or between adolescents with different ambulation status. Furthermore to study the consequences of pain and to identify pain-exacerbating and pain-relieving factors. In a national survey, fifty-five adolescents with spinal muscular atrophy and dystrophinopathy completed a questionnaire assessing pain frequency, duration, location using a body map, intensity and discomfort using visual analogue scales, pain interference using a modified version of Brief Pain Inventory and factors exacerbating and relieving pain. Sixty-nine per cent of the adolescents reported pain during the past three months and 50% reported chronic pain. The pain prevalence did not differ significantly between diagnostic groups or between ambulators and non-ambulators. The average pain intensity was graded as mild and the worst pain as moderate. The pain typically occurred weekly, most frequently in the neck/back or legs. General activity and mood were the areas that were most affected by pain. Common pain-exacerbating factors were sitting, too much movement/activity and being lifted or transferred. Pain is a frequent problem in adolescents with spinal muscular atrophy and dystrophinopathy. The assessments used enable an understanding both of the nature and scope of pain and of the impact of pain in everyday life. The study highlights the importance of assessing pain in a systematic manner and offering an individual approach to interventions designed to reduce pain in this population. Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Shallow water sea slugs (Gastropoda: Heterobranchia) from the northwestern coast of the Sea of Japan, north of Peter the Great Bay, Russia

PubMed Central

2016-01-01

The coast of northern Primorye region, north of Peter the Great Bay has been sparsely studied in regards to its molluscan fauna, with just a few works reviewing the distribution of local mollusks. This work presents a survey of the shallow water heterobranch sea slugs currently occurring around Kievka Bay to Oprichnik Bay, Russia. Thirty-nine species of sea slugs were found in this study and the new species Cadlina olgae sp. nov., described herein. Most (24) of the species occurring in the area have widespread ranges in the northern Pacific Ocean. The eight species are endemic for the Sea of Japan and adjacent part of the Sea of Okhotsk. Seven other occur also in northern Atlantic and Arctic waters. Thirteen found species are not known from Peter the Great Bay but known from adjacent northern Pacific waters. The finding of a previously undescribed species emphasizes the need of further surveys, particularly in subtidal and deeper waters, in order to improve the knowledge on this neglected fauna in Primorye. PMID:27957399

Consecutive analysis of mutation spectrum in the dystrophin gene of 507 Korean boys with Duchenne/Becker muscular dystrophy in a single center.

PubMed

Cho, Anna; Seong, Moon-Woo; Lim, Byung Chan; Lee, Hwa Jeen; Byeon, Jung Hye; Kim, Seung Soo; Kim, Soo Yeon; Choi, Sun Ah; Wong, Ai-Lynn; Lee, Jeongho; Kim, Jon Soo; Ryu, Hye Won; Lee, Jin Sook; Kim, Hunmin; Hwang, Hee; Choi, Ji Eun; Kim, Ki Joong; Hwang, Young Seung; Hong, Ki Ho; Park, Seungman; Cho, Sung Im; Lee, Seung Jun; Park, Hyunwoong; Seo, Soo Hyun; Park, Sung Sup; Chae, Jong Hee

2017-05-01

Duchenne and Becker muscular dystrophies (DMD and BMD) are allelic X-linked recessive muscle diseases caused by mutations in the large and complex dystrophin gene. We analyzed the dystrophin gene in 507 Korean DMD/BMD patients by multiple ligation-dependent probe amplification and direct sequencing. Overall, 117 different deletions, 48 duplications, and 90 pathogenic sequence variations, including 30 novel variations, were identified. Deletions and duplications accounted for 65.4% and 13.3% of Korean dystrophinopathy, respectively, suggesting that the incidence of large rearrangements in dystrophin is similar among different ethnic groups. We also detected sequence variations in >100 probands. The small variations were dispersed across the whole gene, and 12.3% were nonsense mutations. Precise genetic characterization in patients with DMD/BMD is timely and important for implementing nationwide registration systems and future molecular therapeutic trials in Korea and globally. Muscle Nerve 55: 727-734, 2017. © 2016 Wiley Periodicals, Inc.

Identification of Neoceratitis asiatica (Becker) (Diptera: Tephritidae) based on morphological characteristics and DNA barcode.

PubMed

Guo, Shaokun; He, Jia; Zhao, Zihua; Liu, Lijun; Gao, Liyuan; Wei, Shuhua; Guo, Xiaoyu; Zhang, Rong; Li, Zhihong

2017-12-12

Neoceratitis asiatica (Becker), which especially infests wolfberry (Lycium barbarum L.), could cause serious economic losses every year in China, especially to organic wolfberry production. In some important wolfberry plantings, it is difficult and time-consuming to rear the larvae or pupae to adults for morphological identification. Molecular identification based on DNA barcode is a solution to the problem. In this study, 15 samples were collected from Ningxia, China. Among them, five adults were identified according to their morphological characteristics. The utility of mitochondrial DNA (mtDNA) cytochrome c oxidase I (COI) gene sequence as DNA barcode in distinguishing N. asiatica was evaluated by analysing Kimura 2-parameter distances and phylogenetic trees. There were significant differences between intra-specific and inter-specific genetic distances according to the barcoding gap analysis. The uncertain larval and pupal samples were within the same cluster as N. asiatica adults and formed sister cluster to N. cyanescens. A combination of morphological and molecular methods enabled accurate identification of N. asiatica. This is the first study using DNA barcode to identify N. asiatica and the obtained DNA sequences will be added to the DNA barcode database.

Peter Holland: a pioneer of occupational medicine.

PubMed Central

Murray, R

1992-01-01

The earliest recorded occupational health service in this country was that established in a cotton spinning factory at Quarry Bank Mill in Cheshire. The mill was built in 1784 by Samuel Greg and his partners. They employed local labour and also some parish apprentices. Happily, Samuel Greg was a good christian and, having created a modern factory and a model village with a church and a school, he was equally concerned for the physical welfare of his employees. Accordingly, he appointed a doctor to make pre-employment examinations of the apprentices and to visit regularly to deal with the health problems of a community of some 400 people. The man he chose was Peter Holland of Sandlebridge, who had served his medical apprenticeship under Dr Charles White of Manchester. The first record of the employment of a doctor was in 1796, but from 1804 to 1845 (doubtless in response to the early factory legislation) each visit of the doctor was entered in a day book with either an indication of fitness to work or details of the treatment required. The complete record consists of two hardback foolscap notebooks that provide a fascinating insight into the medical practice of the times when the industrial revolution was just getting under way. One of the more interesting features is the preservation of medical secrecy. Dr Holland made his comments on the case in shorthand and his instructions in longhand. By a fortunate coincidence the key to the shorthand was discovered and this has now been largely transcribed. Although much of the content of the diaries is the day by day practice of medicine at the time, there are many illuminating glimpses of the early practice of occupational medicine. Images PMID:1606023

[Comment on “Review papers: Proceedings vs. journals” by S. Peter Gary] On the venue of review papers

NASA Astrophysics Data System (ADS)

Eviatar, Aharon

S. Peter Gary has called our attention (“Review Papers: Proceedings vs. Journals,” Eos, March 3, 1987, p. 125) to the lesson of the noted philosopher of science, S.W.H. Cowley, to wit: “Proceedings are simply not accessible to the same degree as journals.” From this, Gary concludes that it would be much more desirable if space physics review articles were published in refereed journals rather than in conference proceedings. I should like to comment a bit on this, if I may.

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

PubMed

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.

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

PubMed Central

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

Analyses and description of geochemical samples, Peters Mountain Wilderness Study Area, Giles County, Virginia

USGS Publications Warehouse

Rait, Norma; Lesure, Frank Gardner

1978-01-01

Semiquantitative emission spectrographic analyses for 64 elements on 43 stream sediment and 73 rock samples from Peters Mountain Wilderness Study area, Giles County, Virginia, are reported here in detail. Locations for all samples are in Universal Transverse Mercator (UTM) coordinates. Brie[ descriptions of rock samples are also included. Rocks analysed are mostly sandstone. Samples of hematitic sandstone of the Rose Hill Formation and limonite-cemented sandstone of the Rocky Gap Sandstone contain high values of iron; these rocks are submarginal iron resources. Some of the same iron-rich samples have a little more barium, copper, cobalt, lead, silver, and/or zinc then average sandstone, but they do not suggest the presence of economic deposits of these metals. No other obviously anomalous values related to mineralized rock are present in the data.

Peter Bourne's drug policy and the perils of a public health ethic, 1976-1978.

PubMed

Clark, Claire D; Dufton, Emily

2015-02-01

As President Jimmy Carter's advisor for health issues, Peter Bourne promoted a rational and comprehensive drug strategy that combined new supply-side efforts to prevent drug use with previously established demand-side addiction treatment programs. Using a public health ethic that allowed the impact of substances on overall population health to guide drug control, Bourne advocated for marijuana decriminalization as well as increased regulations for barbiturates. A hostile political climate, a series of rumors, and pressure from both drug legalizers and prohibitionists caused Bourne to resign in disgrace in 1978. We argue that Bourne's critics used his own public health framework to challenge him, describe the health critiques that contributed to Bourne's resignation, and present the story of his departure as a cautionary tale for today's drug policy reformers.

Study of dissolved oxygen content in the Eastern Bosporus Strait (Peter the Great Bay, Sea of Japan)

NASA Astrophysics Data System (ADS)

Grigoryeva, N. I.

2017-09-01

Seasonal changes in the dissolved oxygen (DO) content in water were analyzed based on long-term observations (2006-2013) in the Eastern Bosporus Strait (Peter the Great Bay, Sea of Japan). It was found that the monthly average DO concentrations at the bottom of the strait were significantly lower in summer than the average annual long-term data. The minimum DO contents were recorded during four months, from July to October. It was shown that the DO content in water depended on changes in current directions in the strait: lower DO contents resulted from hypoxic water inflow, mostly from Amur Bay.

Executive Editor Frank Shushok, Jr. Sits down with Peter Felten and Charles C. Schroeder to Discover What Matters Most for a Meaningful Undergraduate Experience

ERIC Educational Resources Information Center

Shushok, Frank, Jr.

2017-01-01

Peter Felten is executive director of the Center for Engaged Learning and assistant provost for teaching and learning at Elon University, where he also serves as a professor of history. Charles C. Schroeder is the past president of ACPA--College Student Educators International and founding coexecutive editor of "About Campus." Felten and…

Duchenne/Becker muscular dystrophy: correlation of phenotype by electroretinography with sites of dystrophin mutations.

PubMed

Pillers, D A; Fitzgerald, K M; Duncan, N M; Rash, S M; White, R A; Dwinnell, S J; Powell, B R; Schnur, R E; Ray, P N; Cibis, G W; Weleber, R G

1999-01-01

The dark-adapted electroretinogram (ERG) of patients with Duchenne and Becker muscular dystrophy (DMD/BMD) shows a marked reduction in b-wave amplitude. Genotype-phenotype studies of mouse models for DMD show position-specific effects of the mutations upon the phenotype: mice with 5' defects of dystrophin have normal ERGs, those with defects in the central region have a normal b-wave amplitude associated with prolonged implicit times for both the b-wave and oscillatory potentials, and mice with 3' defects have a phenotype similar to that seen in DMD/BMD patients. The mouse studies suggest a key role for the carboxyl terminal dystrophin isoform, Dp260, in retinal electrophysiology. We have undertaken a systematic evaluation of DMD/BMD patients through clinical examination and review of the literature in order to determine whether the position-specific effects of mutations noted in the mouse are present in man. We have found that, in man, a wider variation of DMD defects correlate with reductions in the b-wave amplitude. Individuals with normal ERGs have mutations predominantly located 5' of the transcript initiation site of Dp260. Our results suggest that the most important determinant in the ERG b-wave phenotype is the mutation position, rather than muscle disease severity. Forty-six per cent of patients with mutations 5' of the Dp260 transcript start site have abnormal ERGs, as opposed to 94% with more distal mutations. The human genotype-phenotype correlations are consistent with a role for Dp260 in normal retinal electrophysiology and may also reflect the expression of other C-terminal dystrophin isoforms and their contributions to retinal signal transmission.

Clinical characterisation of Becker muscular dystrophy patients predicts favourable outcome in exon-skipping therapy.

PubMed

van den Bergen, J C; Schade van Westrum, S M; Dekker, L; van der Kooi, A J; de Visser, M; Wokke, B H A; Straathof, C S; Hulsker, M A; Aartsma-Rus, A; Verschuuren, J J; Ginjaar, H B

2014-01-01

Duchenne and Becker muscular dystrophy (DMD/BMD) are both caused by mutations in the DMD gene. Out-of-frame mutations in DMD lead to absence of the dystrophin protein, while in-frame BMD mutations cause production of internally deleted dystrophin. Clinically, patients with DMD loose ambulance around the age of 12, need ventilatory support at their late teens and die in their third or fourth decade due to pulmonary or cardiac failure. BMD has a more variable disease course. The disease course of patients with BMD with specific mutations could be very informative to predict the outcome of the exon-skipping therapy, aiming to restore the reading-frame in patients with DMD. Patients with BMD with a mutation equalling a DMD mutation after successful exon skipping were selected from the Dutch Dystrophinopathy Database. Information about disease course was gathered through a standardised questionnaire. Cardiac data were collected from medical correspondence and a previous study on cardiac function in BMD. Forty-eight patients were included, representing 11 different mutations. Median age of patients was 43 years (range 6-67). Nine patients were wheelchair users (26-56 years). Dilated cardiomyopathy was present in 7/36 patients. Only one patient used ventilatory support. Three patients had died at the age of 45, 50 and 76 years, respectively. This study provides mutation specific data on the course of disease in patients with BMD. It shows that the disease course of patients with BMD, with a mutation equalling a 'skipped' DMD mutation is relatively mild. This finding strongly supports the potential benefit of exon skipping in patients with DMD.

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

DOE Office of Scientific and Technical Information (OSTI.GOV)

Thanh, L.T.; Man, Nguyen Thi; Morris, G.E.

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 groupsmore » 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.« less

Patients with Duchenne and Becker muscular dystrophies are not more asymmetrical than healthy controls on timed performance of upper limb tasks

PubMed Central

Artilheiro, M.C.; Sá, C.S.C.; Fávero, F.M.; Caromano, F.A.; Voos, M.C.

2017-01-01

This study aimed to investigate possible asymmetries and relationships between performance of dominant and non-dominant upper limbs (UL) in patients with Duchenne and Becker muscular dystrophies (DMD/BMD), to compare UL performance of patients and healthy subjects and to investigate the relationship between timed performance of UL and age, motor function and muscle strength in DMD/BMD patients. Sixteen patients with DMD and 3 with BMD were evaluated with Jebsen-Taylor Test (timed performance), Vignos scale and Dimension 3 of Motor Function Measure (motor function), and Medical Research Council scale (muscle strength) on a single session. ANOVA showed no asymmetry between dominant and non-dominant UL, except in the writing subtest, in patients and in healthy controls. There were relationships between dominant and non-dominant UL performances. Correlations between timed performance, motor function and muscle strength were found, but age was not correlated with these variables. These findings may reduce the assessment time, prevent fatigue and provide more accurate clinical reasoning involving UL in DMD/BMD treatment. PMID:28746422

Occurrences of whale shark (Rhincodon typus Smith, 1828) in the Saint Peter and Saint Paul archipelago, Brazil.

PubMed

Hazin, F H V; Vaske Júnior, T; Oliveira, P G; Macena, B C L; Carvalho, F

2008-05-01

The Saint Peter and Saint Paul Archipelago in the central tropical Atlantic, is an important ground of whale sharks that are commonly sighted throughout the year close to the fishing boats in the adjacencies of the islands. In sightings reported between February 2000 and November 2005, the lengths of the individuals ranged between 1.8 to 14.0 m. The causes of these concentrations in the archipelago are still unclear, once there are no upwellings and plankton concentrations for feeding, and no reproductive activities were reported. Nevertheless, they could be associated to the spawning period of the abundant flying fishes, mainly in the first semester, when sightings were more frequent.

Reflections on Peter Slezak and the 'Sociology of Scientific Knowledge`

NASA Astrophysics Data System (ADS)

Suchting, W. A.

The paper examines central parts of the first of two papers in this journal by Peter Slezak criticising sociology of scientific knowledge and also considers, independently, some of the main philosophical issues raised by the sociologists of science, in particular David Bloor. The general conclusion is that each account alludes to different and crucial aspects of the nature of knowledge without, severally or jointly, being able to theorise them adequately. The appendix contains epistemological theses central to a more adequate theory of scientific knowledge.... our Histories of six Thousand Moons make no Mention of any other, than the two great Empires of Lilliput and Blefuscu. Which mighty Powers have ... been engaged in a most obstinate War for six and thirty Moons past. It began upon the following Occasion. It is allowed on all Hands, that the primitive Way of breaking Eggs before we eat them, was upon the larger End: But ... the Emperor [of Lilliput] ... published an Edict, commanding all his Subjects, upon great Penalties, to break the smaller End of their Eggs. The People so resented this Law, that ... there have been six Rebellions raised on that Account ... These civil Commotions were constantly fomented by the Monarchs of Blefuscu ... It is computed, that eleven Thousand have, at several Times, suffered Death, rather than break Eggs at the smaller End. Many hundred large Volumes have published upon this Controversy ...

Exonization of an Intronic LINE-1 Element Causing Becker Muscular Dystrophy as a Novel Mutational Mechanism in Dystrophin Gene

PubMed Central

Gonçalves, Ana; Coelho, Teresa; Melo-Pires, Manuel; Sousa, Mário

2017-01-01

A broad mutational spectrum in the dystrophin (DMD) gene, from large deletions/duplications to point mutations, causes Duchenne/Becker muscular dystrophy (D/BMD). Comprehensive genotyping is particularly relevant considering the mutation-centered therapies for dystrophinopathies. We report the genetic characterization of a patient with disease onset at age 13 years, elevated creatine kinase levels and reduced dystrophin labeling, where multiplex-ligation probe amplification (MLPA) and genomic sequencing failed to detect pathogenic variants. Bioinformatic, transcriptomic (real time PCR, RT-PCR), and genomic approaches (Southern blot, long-range PCR, and single molecule real-time sequencing) were used to characterize the mutation. An aberrant transcript was identified, containing a 103-nucleotide insertion between exons 51 and 52, with no similarity with the DMD gene. This corresponded to the partial exonization of a long interspersed nuclear element (LINE-1), disrupting the open reading frame. Further characterization identified a complete LINE-1 (~6 kb with typical hallmarks) deeply inserted in intron 51. Haplotyping and segregation analysis demonstrated that the mutation had a de novo origin. Besides underscoring the importance of mRNA studies in genetically unsolved cases, this is the first report of a disease-causing fully intronic LINE-1 element in DMD, adding to the diversity of mutational events that give rise to D/BMD. PMID:28972564

Exonization of an Intronic LINE-1 Element Causing Becker Muscular Dystrophy as a Novel Mutational Mechanism in Dystrophin Gene.

PubMed

Gonçalves, Ana; Oliveira, Jorge; Coelho, Teresa; Taipa, Ricardo; Melo-Pires, Manuel; Sousa, Mário; Santos, Rosário

2017-10-03

A broad mutational spectrum in the dystrophin ( DMD ) gene, from large deletions/duplications to point mutations, causes Duchenne/Becker muscular dystrophy (D/BMD). Comprehensive genotyping is particularly relevant considering the mutation-centered therapies for dystrophinopathies. We report the genetic characterization of a patient with disease onset at age 13 years, elevated creatine kinase levels and reduced dystrophin labeling, where multiplex-ligation probe amplification (MLPA) and genomic sequencing failed to detect pathogenic variants. Bioinformatic, transcriptomic (real time PCR, RT-PCR), and genomic approaches (Southern blot, long-range PCR, and single molecule real-time sequencing) were used to characterize the mutation. An aberrant transcript was identified, containing a 103-nucleotide insertion between exons 51 and 52, with no similarity with the DMD gene. This corresponded to the partial exonization of a long interspersed nuclear element (LINE-1), disrupting the open reading frame. Further characterization identified a complete LINE-1 (~6 kb with typical hallmarks) deeply inserted in intron 51. Haplotyping and segregation analysis demonstrated that the mutation had a de novo origin. Besides underscoring the importance of mRNA studies in genetically unsolved cases, this is the first report of a disease-causing fully intronic LINE-1 element in DMD , adding to the diversity of mutational events that give rise to D/BMD.

The ultrasound-guided nerve blocks of abdominal wall contributed to anesthetic management of cholecystectomy in a patient with Becker muscular dystrophy without using muscle relaxants.

PubMed

Iwata, Masato; Kuzumoto, Naoya; Akasaki, Yuka; Morioka, Masayo; Nakayama, Kana; Matsuzawa, Nobuyoshi; Kimoto, Katsuhiro; Shimomura, Toshiyuki

2017-01-01

Becker muscular dystrophy (BMD) is a progressive neuromuscular disorder caused by mutations in the dystrophin gene. The sensitivity to non-depolarizing muscle relaxant in a patient with muscle dystrophy is reportedly higher than that in normal individuals, and the duration of the effect is known to be prolonged. In this report, we present the case of a 58-year-old man with BMD who underwent laparoscopic cholecystectomy for symptomatic cholelithiasis under total intravenous anesthesia without the use of muscle-relaxant drugs and supplemented with regional anesthesia. Anesthesia was induced and maintained with propofol, remifentanil, and fentanyl; ultrasound-guided bilateral rectus sheath block (RSB) and right-sided subcostal transversus abdominis plane block (TAP) were performed. The procedure required conversion to open surgery because of hard conglutination; intraoperative and postoperative periods were uneventful. Adequate analgesia was maintained after extubation because of the effect of RSB and TAP.

Effects of fouling on the Japanese scallop Mizuhopecten yessoensis (Jay) in Peter the Great Bay (Sea of Japan)

NASA Astrophysics Data System (ADS)

Gabaev, D. D.

2013-03-01

A valuable mariculture object, the Japanese scallop Mizuhopecten (= Patinopevten) yessoensis (Jay), after six hours long transportation by air and sowing on the bottom is fouled greater by epi- and endolythical organisms than the members of the native population. It is likely that the fouling negatively affects the specimens, those that were the largest before the sowing at the bottom were not found among those that reached puberty. The results of the effects of the endolythic polychaete Polydora brevipalpa and the barnacle Hesperibalanus hesperius on the growth rate of the Japanese scallop cultivated on the bottom of Peter the Great Bay (Sea of Japan) are provided in this paper.

[Catering services bases in the Russian army under military regulation of Peter the Great].

PubMed

Konyshev, I S; adamenko, A M; Koshelev, V P

2014-01-01

At Peter I the regular army was organized and the system of target state deliveries to troops of the food is created. Provisioning and fodder was normalized as portion and ration. Portion was contained the products forpeoplefood, and ration - fodder for horses food who were used by the serviceman. Portion and ration unit was identical to all categories of the military personnel. Difference in food level consisted in that, how many portions and rations serviceman received. Up to the end of existence of Russian army in 1918 in each rota there were contractor and the cook who were engaged in foodstuff and cooking under sergeant-major and one of rota officers supervision. According to the Charter it was necessary to carry with respect and attention to officers and soldiers, their needs, including in the field of supply and catering services and providing with the food: Despite the lack of scientific justification, soldiers' nutrition was sufficient to provide fighting capacity of the Russian army.

Earthquake Magnitude Relationships for the Saint Peter and Saint Paul Archipelago, Equatorial Atlantic

NASA Astrophysics Data System (ADS)

de Melo, Guilherme W. S.; do Nascimento, Aderson F.

2018-03-01

We have investigated several relationships between ML, M(NEIC) and Mw for the earthquakes locally recorded in the Saint Peter and Saint Paul Archipelago (SPSPA), Equatorial Atlantic. Because we only have one station in the area, we could not derive attenuation relations for events recorded at different distances at different stations. Our approach was then to compare our ML estimates with magnitudes reported by NEIC. This approach produced acceptable results particularly for epicentral distance smaller than 100 km. For distances greater that 100 km, there is a systematic increase in the residuals probable due to the lack of station correction and our inability to accurately estimate Q. We also investigate the Mw—M(NEIC) relationship. We find that Mw estimates using S-wave produce smaller residuals when compared with both M(NEIC). Finally, we also investigate the ML—Mw relationship and observe that given the data set we have, the 1:1 holds. We believe that the use of the present methodologies provide consistent magnitude estimates between all the magnitudes investigated that could be used to better assess seismic hazard in the region.

Earthquake Magnitude Relationships for the Saint Peter and Saint Paul Archipelago, Equatorial Atlantic

NASA Astrophysics Data System (ADS)

de Melo, Guilherme W. S.; do Nascimento, Aderson F.

2017-12-01

We have investigated several relationships between ML, M(NEIC) and Mw for the earthquakes locally recorded in the Saint Peter and Saint Paul Archipelago (SPSPA), Equatorial Atlantic. Because we only have one station in the area, we could not derive attenuation relations for events recorded at different distances at different stations. Our approach was then to compare our ML estimates with magnitudes reported by NEIC. This approach produced acceptable results particularly for epicentral distance smaller than 100 km. For distances greater that 100 km, there is a systematic increase in the residuals probable due to the lack of station correction and our inability to accurately estimate Q. We also investigate the Mw—M(NEIC) relationship. We find that Mw estimates using S-wave produce smaller residuals when compared with both M(NEIC). Finally, we also investigate the ML—Mw relationship and observe that given the data set we have, the 1:1 holds. We believe that the use of the present methodologies provide consistent magnitude estimates between all the magnitudes investigated that could be used to better assess seismic hazard in the region.

Peter Bourne’s Drug Policy and the Perils of a Public Health Ethic, 1976–1978

PubMed Central

Dufton, Emily

2015-01-01

As President Jimmy Carter’s advisor for health issues, Peter Bourne promoted a rational and comprehensive drug strategy that combined new supply-side efforts to prevent drug use with previously established demand-side addiction treatment programs. Using a public health ethic that allowed the impact of substances on overall population health to guide drug control, Bourne advocated for marijuana decriminalization as well as increased regulations for barbiturates. A hostile political climate, a series of rumors, and pressure from both drug legalizers and prohibitionists caused Bourne to resign in disgrace in 1978. We argue that Bourne’s critics used his own public health framework to challenge him, describe the health critiques that contributed to Bourne’s resignation, and present the story of his departure as a cautionary tale for today’s drug policy reformers. PMID:25521893

A rare subclinical or mild type of Becker muscular dystrophy caused by a single exon 48 deletion of the dystrophin gene.

PubMed

Zimowski, Janusz G; Pilch, Jacek; Pawelec, Magdalena; Purzycka, Joanna K; Kubalska, Jolanta; Ziora-Jakutowicz, Karolina; Dudzińska, Magdalena; Zaremba, Jacek

2017-08-01

In the material of 227 families with Becker muscular dystrophy (BMD), we found nine non-consanguineous families with 17 male individuals carrying a rare mutation-a single exon 48 deletion of the dystrophin gene-who were affected with a very mild or subclinical form of BMD. They were usually detected thanks to accidental findings of elevated serum creatine phosphokinase (sCPK). A thorough clinical analysis of the carriers, both children (12) and adults (5), revealed in some of them muscle hypotonia (10/17) and/or very mild muscle weakness (9/17), as well as decreased tendon reflexes (6/17). Adults, apart from very mild muscle weakness and calf hypertrophy in some, had no significant abnormalities on neurological assessments and had good exercise tolerance. Parents of the children carriers of the exon 48 deletion are usually unaware of their children being affected, and possibly at risk of developing life-threatening cardiomyopathy. The same concerns the adult male carriers. Therefore, the authors postulate undertaking preventive measures such as cascade screening of the relatives of the probands. Newborn screening programmes of Duchenne muscular dystrophy (DMD)/BMD based on sCPK marked increase may be considered.

Of neoliberalism and global health: human capital, market failure and sin/social taxes

PubMed Central

Reubi, David

2016-01-01

Abstract This article tells a different but equally important story about neoliberalism and global health than the narrative on structural adjustment policies usually found in the literature. Rather than focus on macroeconomic structural adjustment policies, this story draws our attention to microeconomic taxation policies on tobacco, alcohol and sugar now widely recognised as the best strategy to control the global non-communicable disease epidemic. Structural adjustment policies are the product of the shift from statist to market-based development models, which was brought about by neoliberal thinkers like Peter Blau and Deepak Lal. In contrast, taxation policies are the result of a different epistemological rupture in international development: the move from economies and physical capital to people and human capital, advocated by Gary Becker and others. This move was part of wider change, which saw Chicago School economists, under the influence of rational choice theory, redefine the object of their discipline, from the study of markets to individual choices. It was this concern with people and their choices that made it possible for Becker and others to identify the importance of price for the demand for tobacco, alcohol and sugar. The same concern also made it easier for them to recognise that there were inefficiencies in the tobacco, alcohol and sugar markets that required government intervention. This story, I suggest, shows that structural adjustment policies and pro-market ideology do not exhaust the relationship between neoliberalism and global health and should not monopolise how we, as political and social scientists, conceive it. PMID:27721572

Analysis of subsequent publication and impact of abstracts presented at the Sir Peter Freyer Surgical Symposium: Focus on the Plenary Session.

PubMed

O' Connor, D J; Lowery, A J; Kearney, D; McAnena, O J; Sweeney, K J; Kerin, M J

2015-09-01

The quality of abstracts presented at a conference reflects the academic activity and research productivity of the surgical/scientific association concerned. The abstract to publication rate (44.5 % internationally), is an important indicator of the quality of presented research. To evaluate the publication rate and impact of abstracts presented at the plenary session of the Sir Peter Freyer Surgical Symposium over a 25-year period (1989-2014), and identify factors influencing publication. Plenary abstracts were identified from abstract books of the Symposium from 1989-2014. The authors, institution, subspecialty and research subject were recorded. A Medline search with name of the first and last author, key words and content of all abstracts was conducted to identify related publications. The impact factor (IF) of the journal and the time to publication was recorded. 298 presented abstracts resulted in 168 publications (publication rate: 56 %). Basic Science research accounted for 80 % (n = 237) of the total number of presentations with the remaining 20 % (n = 61) being categorised as clinical research. Overall, cancer research accounted for 48 % of presented work. The average time to publication was 2 ± 7 years, while 11 % of all published studies achieved publication in the year of the symposium. The median impact factor for published research was 3.558 (IF range 0-39). These results indicate that the quality of papers presented at the Sir Peter Freyer Surgical Symposium compares favourably with international equivalents, making this meeting an important forum for Irish Academic Surgery.

Personality as a Social Process: where Peter Giordano Meets Boris Parygin.

PubMed

Mironenko, Irina A

2018-06-01

In this paper I comment on the "Individual personality is best understood as process, not structure: A Confucian-inspired perspective" article by Peter Giordano (Culture & Psychology, 23(4), 502-518 (2017)), which addresses the question of how to comprehend a personality which is continuously changing and varying with changes in social contexts and situations. The issue which Giordano turns up I believe to be of great importance and topicality in the quickly changing globalizing contemporary world. Giordano's paper highlights an important problem in the development of contemporary personality psychology, but much remains to be clarified concerning his process-centric model. I introduce the theory of Boris Parygin, which can make a contribution to the discussion of personality as a process. Parygin's theory addresses the same issues that Giordano focuses on: human personality exists and develops in the context of social situation. Parygin's theoretical model of personality involves two personality schemas: a "static" one and a "dynamic" one. The "structure" and the "process" -centered approaches are joint here to complement each other. Personality life-span development, formation of personality structures, is considered as a process of interaction and dialectical confrontation with the social environment, in the course of which the personality, originally engendered by social factors, builds up its autonomy and realizes its unique individual spiritual potential.

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

PubMed

Rao, Mandava V; Sindhav, Gaurang M; Mehta, Jitendra J

2014-07-01

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. 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. 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. 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.

Generation of induced pluripotent stem cells from a Becker muscular dystrophy patient carrying a deletion of exons 45-55 of the dystrophin gene (CCMi002BMD-A-9 ∆45-55).

PubMed

Gowran, Aoife; Spaltro, Gabriella; Casalnuovo, Federica; Vigorelli, Vera; Spinelli, Pietro; Castiglioni, Elisa; Rovina, Davide; Paganini, Stefania; Di Segni, Marina; Gervasini, Cristina; Nigro, Patrizia; Pompilio, Giulio

2018-04-01

Becker muscular dystrophy (BMD) is a dystrophinopathy caused by mutations in the dystrophin gene on chromosome Xp21. BMD mutations result in truncated semi-functional dystrophin isoforms. Consequently, less severe clinical symptoms become apparent later in life compared to Duchenne muscular dystrophy. Dermal fibroblasts from a BMD patient were electroporated with episomal plasmids containing reprogramming factors to create the induced pluripotent stem cell line: CCMi002BMD-A-9 that showed pluripotent markers, were karyotypically normal and capable of trilineage differentiation. MLPA analyses performed on DNA extracted from CCMi002BMD-A-9 showed an in-frame deletion of exons 45 to 55 (CCMi002BMD-A-9 Δ45-55). Copyright © 2018 The Author(s). Published by Elsevier B.V. All rights reserved.

Cancer: A Personal Journey. Notes from the Edge.The Diary of Peter J. Morgan, M.D.

PubMed

Chabner

1997-01-01

It is a mistake to think that all personal experiences with cancer are the same. For certain, all cancer patients do confront the possibility of an early death and the prospect of pain and suffering due to the tumor and its treatment. But the specific emotional issues differ with each patient and each family, and the responses to these issues take many forms. In an eloquent and moving film, "Cancer: A Personal Journey. Notes from the Edge.," we are given the privilege of accompanying a remarkable young physician, Peter J. Morgan, on his journey with cancer, a two and one-half year journey that ended with his death at age 31. At age 29, Dr. Morgan, an internist-in-training who intended to pursue a career in hematology and oncology, noted a mass on his leg. Tragically, metastasis to the lungs had already taken place at the time of diagnosis of a synovial sarcoma. There followed the all-too-familiar story of chemotherapy and experimental treatments, pain, debilitation, and ultimately demise, and in itself this experience would move us with the sorrow of a precious life lost. What makes this particular story so remarkable are the insights of this young physician and the struggle for survival of a spirit that would not succumb to the "chaos" of cancer. In the two-year period of his life as a cancer patient, Peter Morgan kept a diary that records his thoughts, his emotional turmoil, and his reflections on life and an untimely death. In particular, we are able to understand the need for his spiritual self to remain alive and to grow despite the deterioration of his physical being. And we see that spiritual triumph in his compelling relationships with his family and his colleagues, in his reflections on art and music and nature, and most of all in his writings and his appreciation of the immense possibilities for joy in life. This is not an easy journey to watch, but the intense sadness of his experience is balanced by his friendships and the great satisfaction he derived

The Quantum Logical Challenge: Peter Mittelstaedt's Contributions to Logic and Philosophy of Science

NASA Astrophysics Data System (ADS)

Beltrametti, E.; Dalla Chiara, M. L.; Giuntini, R.

2017-12-01

Peter Mittelstaedt's contributions to quantum logic and to the foundational problems of quantum theory have significantly realized the most authentic spirit of the International Quantum Structures Association: an original research about hard technical problems, which are often "entangled" with the emergence of important changes in our general world-conceptions. During a time where both the logical and the physical community often showed a skeptical attitude towards Birkhoff and von Neumann's quantum logic, Mittelstaedt brought into light the deeply innovating features of a quantum logical thinking that allows us to overcome some strong and unrealistic assumptions of classical logical arguments. Later on his intense research on the unsharp approach to quantum theory and to the measurement problem stimulated the increasing interest for unsharp forms of quantum logic, creating a fruitful interaction between the work of quantum logicians and of many-valued logicians. Mittelstaedt's general views about quantum logic and quantum theory seem to be inspired by a conjecture that is today more and more confirmed: there is something universal in the quantum theoretic formalism that goes beyond the limits of microphysics, giving rise to interesting applications to a number of different fields.

Pattern of deletions of the dystrophin gene in Mexican Duchenne/Becker muscular dystrophy patients: the use of new designed primers for the analysis of the major deletion "hot spot" region.

PubMed

Coral-Vazquez, R; Arenas, D; Cisneros, B; Peñaloza, L; Salamanca, F; Kofman, S; Mercado, R; Montañez, C

1997-06-13

We have analyzed 59 unrelated Mexican Duchenne/Becker muscular dystrophy patients (DMD/BMD) using PCR analysis of the 2 prone deletion regions in the DMD gene. Thirty one (52%) of the patients had a deletion of one or several of the exons. Most of the alterations (87%) were clustered in exons 44-52, this being the highest percentage reported until now. In order to improve the molecular diagnosis in the Mexican population, we designed a new multiplex assay to PCR amplify exons 44-52. This assay allowed for the identification of a greater number of deletions in this region compared with the 9 and 5-plex assays previously described and to determine most of the deletion end boundaries. This is a reliable alternative for the initial screening of the DMD patients in the Mexican population.

Brachymesomelic dysplasia with Peters anomaly of the eye results from disruptions of the X chromosome near the SHOX and SOX3 genes.

PubMed

Bleyl, Steven B; Byrne, Janice L B; South, Sarah T; Dries, David C; Stevenson, David A; Rope, Alan F; Vianna-Morgante, Angela M; Schoenwolf, Gary C; Kivlin, Jane D; Brothman, Arthur; Carey, John C

2007-12-01

We report on a mother and son affected with an unusual skeletal dysplasia and anterior segment eye abnormalities. Their skeletal phenotype overlaps with the SHOX-related skeletal dysplasias and is intermediate between Leri-Weill dyschondrosteosis (LWD) and Langer Mesomelic dysplasia (LMD). The mother has bilateral Peters anomaly of the eye and was reported as having a new syndrome; the son had severe bilateral sclerocornea. Chromosome analysis showed that the mother has a pericentric inversion of the X chromosome [46,X,inv(X)(p22.3q27)] and the son, a resultant recombinant X chromosome [46,Y,rec(X)dup(Xq)inv(X)(p22.3q27)]. The observed skeletal and ophthalmologic abnormalities in both patients were similar in severity. The additional features of developmental delay, growth retardation, agenesis of the corpus callosum, cryptorchidism and hypoplastic scrotum in the son are consistent with Xq28 duplication. Analysis of the son's recombinant X chromosome showed that the Xp22.33 breakpoint lies 30-68 kb 5' of the SHOX gene. This finding suggests that the skeletal dysplasia in both mother and son is allelic with LWD and LMD and results from a novel misexpression of SHOX. Analysis of the Xq27.1 breakpoint localized it to a 90 kb interval 3' of the SOX3 gene, supporting a novel role of SOX3 misexpression in the development of Peters anomaly of the eye. (c) 2007 Wiley-Liss, Inc.

AVTA Federal Fleet PEV Readiness Data Logging and Characterization Study for Department of Veterans Affairs. James J. Peters VA Medical Center, Bronx, NY

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schey, Stephen; Francfort, Jim

This report focuses on the Department of Veterans Affairs, James J. Peters VA Medical Center (VA - Bronx) fleet to identify daily operational characteristics of select vehicles and report findings on vehicle and mission characterizations to support the successful introduction of PEVs into the agencies’ fleets. Individual observations of these selected vehicles provide the basis for recommendations related to electric vehicle adoption and whether a battery electric vehicle or plug-in hybrid electric vehicle (collectively referred to as PEVs) can fulfill the mission requirements.

Earliest depiction of vitiligo in "Venus at a Mirror" (1615) by Peter Paul Rubens (1577-1640).

PubMed

Ashrafian, Hutan

2018-06-01

The 1615 painting of Venus at a Mirror by Peter Paul Rubens is considered a powerful example of the Flemish Baroque movement. Recently it has been identified that the Venus character in the image has a goitre, however on studying the image further, I note dermato-pathology in another of the painting's main characters; the dark-skinned female typically described as the Venus' maidservant who clearly demonstrates patches of skin pigment loss on her face and neck with a concurrent streak of white hair. Together these suggest the underlying diagnosis of vitiligo. There is also a goitre in this individual suggesting thyroid disease. This new finding may offer additional insight into the historical epidemiology of disease in northern Europe but also offers further understanding of the method, origin, and pathological associations of this prominent painting from a genius artist. © 2017 Wiley Periodicals, Inc.

New U.N. program promotes multisectoral approach to AIDS prevention. Q and A [with Peter Piot].

PubMed

1996-05-01

The new joint United Nations (UN) Program on HIV/AIDS (UNAIDS) coordinates the HIV/AIDS activities of its six co-sponsors: the UN Children's Fund (UNICEF), the UN Development Program (UNDP), the UN Population Fund (UNFPA), the UN Educational, Scientific, and Cultural Organization (UNESCO), the World Health Organization (WHO), and the World Bank. In this interview, UNAIDS Executive Director Peter Piot discusses the program's goals and challenges. The UNAIDS program will be more multisectoral in scope than other efforts, involving all sectors of society that can affect the course of the epidemic or are affected by it. This includes the health and education sectors; ministries of trade, finance, planning, and development; nongovernmental and community organizations; people living with HIV and AIDS; research institutions; and the business sector. In each country, the UN agencies will form a "Theme Group on HIV/AIDS" to formulate intersectoral strategies.

Interpretation of acid α-glucosidase activity in creatine kinase elevation: A case of Becker muscular dystrophy.

PubMed

Oitani, Yoshiki; Ishiyama, Akihiko; Kosuga, Motomichi; Iwasawa, Kentaro; Ogata, Ayako; Tanaka, Fumiko; Takeshita, Eri; Shimizu-Motohashi, Yuko; Komaki, Hirofumi; Nishino, Ichizo; Okuyama, Torayuki; Sasaki, Masayuki

2018-05-16

Diagnosis of Pompe disease is sometimes challenging because it exhibits clinical similarities to muscular dystrophy. We describe a case of Becker muscular dystrophy (BMD) with a remarkable reduction in activity of the acid α-glucosidase (GAA) enzyme, caused by a combination of pathogenic mutation and polymorphism variants resulting in pseudodeficiency in GAA. The three-year-old boy demonstrated asymptomatic creatine kinase elevation. Neither exon deletion nor duplication was detected on multiplex ligation-dependent probe amplification (MLPA) of DMD. GAA enzyme activity in both dried blood spots and lymphocytes was low, at 11.7% and 7.7% of normal, respectively. However, genetic analysis of GAA detected only heterozygosity for a nonsense mutation (c.118C > T, p.Arg40 ∗ ). Muscle pathology showed no glycogen deposits and no high acid phosphatase activity. Hematoxylin-eosin staining detected scattered regenerating fibers; the fibers were faint and patchy on immunochemistry staining of dystrophin. The amount of dystrophin protein was reduced to 11.8% of normal, on Western blotting analysis. Direct sequencing analysis of DMD revealed hemizygosity for a nonsense mutation (c.72G > A, p.Trp24 ∗ ). The boy was diagnosed with BMD, despite remarkable reduction in GAA activity; further, he demonstrated heterozygosity for [p.Gly576Ser; p.Glu689Lys] polymorphism variants that indicated pseudodeficiency on another allele in GAA. Pseudodeficiency alleles are detected in approximately 4% of the Asian population; these demonstrate low activity of acid α-glucosidase (GAA), similar to levels found in Pompe disease. Clinicians should be careful in their interpretations of pseudodeficiency alleles that complicate diagnosis in cases of elevated creatine kinase. Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

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

PubMed Central

Rao, Mandava V.; Sindhav, Gaurang M.; Mehta, Jitendra J.

2014-01-01

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. PMID:25221400

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

PubMed

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.

Serum Creatinine Distinguishes Duchenne Muscular Dystrophy from Becker Muscular Dystrophy in Patients Aged ≤3 Years: A Retrospective Study

PubMed Central

Wang, Liang; Chen, Menglong; He, Ruojie; Sun, Yiming; Yang, Juan; Xiao, Lulu; Cao, Jiqing; Zhang, Huili; Zhang, Cheng

2017-01-01

Here, we investigated correlations between serum creatinine (SCRN) levels and clinical phenotypes of dystrophinopathy in young patients. Sixty-eight patients with dystrophinopathy at the Neuromuscular Clinic, The First Affiliated Hospital, Sun Yat-sen University, were selected for this study. The diagnosis of dystrophinopathy was based on clinical manifestation, biochemical changes, and molecular analysis. Some patients underwent muscle biopsies; SCRN levels were tested when patients were ≤3 years old, and reading frame changes were analyzed. Each patient was followed up, and motor function and clinical phenotype were assessed when the same patients were ≥4 years old. Our findings indicated that in young patients, lower SCRN levels were associated with increased disease severity (p < 0.01) and that SCRN levels were the highest in patients exhibiting mild Becker muscular dystrophy (BMD) (p < 0.001) and the lowest in patients with Duchenne muscular dystrophy (DMD) (p < 0.01) and were significantly higher in patients carrying in-frame mutations than in patients carrying out-of-frame mutations (p < 0.001). SCRN level cutoff values for identifying mild BMD [18 µmol/L; area under the curve (AUC): 0.947; p < 0.001] and DMD (17 µmol/L; AUC: 0.837; p < 0.001) were established. These results suggest that SCRN might be a valuable biomarker for distinguishing DMD from BMD in patients aged ≤3 years and could assist in the selection of appropriate treatment strategies. PMID:28533764

MLPA based detection of mutations in the dystrophin gene of 180 Polish families with Duchenne/Becker muscular dystrophy.

PubMed

Zimowski, Janusz G; Massalska, Diana; Holding, Mariola; Jadczak, Sylwia; Fidziańska, Elżbieta; Lusakowska, Anna; Kostera-Pruszczyk, Anna; Kamińska, Anna; Zaremba, Jacek

2014-01-01

Duchenne/Becker muscular dystrophy (DMD/BMD) is a recessive, X-linked disorder caused by a mutation in the dystrophin gene. Deletions account for approximately 60-65% of mutations, duplications for 5-10%. The remaining cases are mainly point mutations. According to Monaco theory clinical form of the disease depends on maintaining or disrupting the reading frame. The purpose of the study was to determine frequency and location of deletions and duplications in the dystrophin gene, to determine the compliance between maintaining/disrupting the reading frame and clinical form of the disease and to check the effectiveness of MLPA (multiplex ligation-dependent probe amplification) in the detection of these mutations in hemizygous patients and heterozygous female carriers. The material is composed of combined results of molecular diagnosis carried out in years 2009-2012 in 180 unrelated patients referred with the diagnosis of DMD/BMD tested by use of MLPA. We identified 110 deletions, 22 duplication (in one patient two different duplications were detected) and 2 point mutations. Deletions involved mainly exons 45-54 and 3-21, whereas most duplications involved exons 3-18. The compliance with Monaco theory was 95% for deletions and 76% for duplications. Most of mutations in the dystrophin gene were localized in the hot spots - different for deletions and duplications. MLPA enabled their quick identification, exact localization and determination whether or not they maintained or disrupted the reading frame. MLPA was also effective in detection of deletions and duplications in female carriers. Copyright © 2014 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

Geological Carbon Sequestration Storage Resource Estimates for the Ordovician St. Peter Sandstone, Illinois and Michigan Basins, USA

DOE Office of Scientific and Technical Information (OSTI.GOV)

Barnes, David; Ellett, Kevin; Leetaru, Hannes

The Cambro-Ordovician strata of the Midwest of the United States is a primary target for potential geological storage of CO2 in deep saline formations. The objective of this project is to develop a comprehensive evaluation of the Cambro-Ordovician strata in the Illinois and Michigan Basins above the basal Mount Simon Sandstone since the Mount Simon is the subject of other investigations including a demonstration-scale injection at the Illinois Basin Decatur Project. The primary reservoir targets investigated in this study are the middle Ordovician St Peter Sandstone and the late Cambrian to early Ordovician Knox Group carbonates. The topic of thismore » report is a regional-scale evaluation of the geologic storage resource potential of the St Peter Sandstone in both the Illinois and Michigan Basins. Multiple deterministic-based approaches were used in conjunction with the probabilistic-based storage efficiency factors published in the DOE methodology to estimate the carbon storage resource of the formation. Extensive data sets of core analyses and wireline logs were compiled to develop the necessary inputs for volumetric calculations. Results demonstrate how the range in uncertainty of storage resource estimates varies as a function of data availability and quality, and the underlying assumptions used in the different approaches. In the simplest approach, storage resource estimates were calculated from mapping the gross thickness of the formation and applying a single estimate of the effective mean porosity of the formation. Results from this approach led to storage resource estimates ranging from 3.3 to 35.1 Gt in the Michigan Basin, and 1.0 to 11.0 Gt in the Illinois Basin at the P10 and P90 probability level, respectively. The second approach involved consideration of the diagenetic history of the formation throughout the two basins and used depth-dependent functions of porosity to derive a more realistic spatially variable model of porosity rather than

Hydrogeology for land-use planning: the Peters Creek area, Municipality of Anchorage, Alaska

USGS Publications Warehouse

Brunett, Jilann O.; Lee, Michael

1983-01-01

Wells currently provide all water supplies in the area. Most wells obtain enough water for individual household needs from unconsolidated, principally glacial and glacioalluvial deposits. In some places, however, wells must be drilled into the underlying bedrock to obtain adequate supplies. It may be possible to develop small community supplies--for individual trailer courts or subdivisions--in areas where yields of 20 gallons per minute or greater are reported for private, domestic wells. Peters Creek is a potential source of surface-water supply, but it would have to be treated to remove glacial silt during summer months. The chemical quality of both ground water and surface water in the area in generally acceptable for most uses. Foundation and excavation conditions, the potential for water pollution from onsite disposal of wastewater through septic tank systems, and the suitability of specific areas for certain types of development may be affected by the following factors: wetlands and areas of shallow ground water underlie about 30 percent of the study area; landslope exceeds 20 percent in about a third of the area; areas of fine-grained, low-permeability sediments are present locally; bedrock is within 25 feet of the land surface in about a third of the area. (USGS)

Origin of Marshall Space Flight Center (MSFC)

NASA Image and Video Library

1950-01-01

General Medaris, (left) who was a Commander of the Army Ballistic Missile Agency (ABMA) in Redstone Arsenal, Alabama, during 1955 to 1958, shakes hands with Major General Holger Toftoy (right), who consolidated U.S. missile and rocketry development.

XRF investigation on skeletal remains from King Peter III of Aragon (1239-1285 A.D.) and Queen Blanche of Anjou (1280-1310 A.D.)

NASA Astrophysics Data System (ADS)

Piga, Giampaolo; Brunetti, Antonio; Lasio, Barbara; Enzo, Stefano; Malgosa, Assumpció

2014-03-01

We conducted an X-Ray Fluorescence investigation on bone fragments belonging to King Peter III of Aragon and Queen Blanche of Anjou. The spectroscopic analysis was carried out in selected points of the bone fragments. Several transitional elements normally unexpected in the bone composition have been found at varying level of concentration. The presence of these elements was interpreted in relation to chemical treatments for mummification of bodies as well as to dietary habits, including tools used for cooking and for the consumption of food.

HL-10 on lakebed with Jerauld R. Gentry, Peter Hoag, John A. Manke, and Bill Dana

NASA Technical Reports Server (NTRS)

1969-01-01

The four principal HL-10 pilots are seen here with the lifting body aircraft. They are, left to right; Air Force Major Jerauld R. Gentry, Air Force test pilot Peter Hoag, and NASA pilots John A. Manke and Bill Dana. All are wearing the pressure suits needed for flying above 50,000 feet. The HL-10 was one of five heavyweight lifting-body designs flown at NASA's Flight Research Center (FRC--later Dryden Flight Research Center), Edwards, California, from July 1966 to November 1975 to study and validate the concept of safely maneuvering and landing a low lift-over-drag vehicle designed for reentry from space. Northrop Corporation built the HL-10 and M2-F2, the first two of the fleet of 'heavy' lifting bodies flown by the NASA Flight Research Center. The contract for construction of the HL-10 and the M2-F2 was $1.8 million. 'HL' stands for horizontal landing, and '10' refers to the tenth design studied by engineers at NASA's Langley Research Center, Hampton, Va. After delivery to NASA in January 1966, the HL-10 made its first flight on Dec. 22, 1966, with research pilot Bruce Peterson in the cockpit. Although an XLR-11 rocket engine was installed in the vehicle, the first 11 drop flights from the B-52 launch aircraft were powerless glide flights to assess handling qualities, stability, and control. In the end, the HL-10 was judged to be the best handling of the three original heavy-weight lifting bodies (M2-F2/F3, HL-10, X-24A). The HL-10 was flown 37 times during the lifting body research program and logged the highest altitude and fastest speed in the Lifting Body program. On Feb. 18, 1970, Air Force test pilot Peter Hoag piloted the HL-10 to Mach 1.86 (1,228 mph). Nine days later, NASA pilot Bill Dana flew the vehicle to 90,030 feet, which became the highest altitude reached in the program. Some new and different lessons were learned through the successful flight testing of the HL-10. These lessons, when combined with information from it's sister ship, the M2-F2/F3

Engaging patients and caregivers in prioritizing symptoms impacting quality of life for Duchenne and Becker muscular dystrophy.

PubMed

Hollin, Ilene L; Peay, Holly; Fischer, Ryan; Janssen, Ellen M; Bridges, John F P

2018-05-26

Patient preference information (PPI) have an increasing role in regulatory decision-making, especially in benefit-risk assessment. PPI can also facilitate prioritization of symptoms to treat and inform meaningful selection of clinical trial endpoints. We engaged patients and caregivers to prioritize symptoms of Duchenne and Becker muscular dystrophy (DBMD) and explored preference heterogeneity. Best-worst scaling (object case) was used to assess priorities across 11 symptoms of DBMD that impact quality of life and for which there is unmet need. Respondents selected the most and least important symptoms to treat among a subset of five. Relative importance scores were estimated for each symptom, and preference heterogeneity was identified using mixed logit and latent class analysis. Respondents included patients (n = 59) and caregivers (n = 96) affected by DBMD. Results indicated that respondents prioritized "weaker heart pumping" [score = 5.13; 95% CI (4.67, 5.59)] and pulmonary symptoms: "lung infections" [3.15; (2.80, 3.50)] and "weaker ability to cough" [2.65; (2.33, 2.97)] as the most important symptoms to treat and "poor attention span" as the least important symptom to treat [- 5.23; (- 5.93, - 4.54)]. Statistically significant preference heterogeneity existed (p value < 0.001). At least two classes existed with different priorities. Priorities of the majority latent class (80%) reflected the aggregate results, whereas the minority latent class (20%) did not distinguish among pulmonary and other symptoms. Estimates of the relative importance for symptoms of Duchenne muscular dystrophy indicated that symptoms with direct links to morbidity and mortality were prioritized above other non-skeletal muscle symptoms. Findings suggested the existence of preference heterogeneity for symptoms, which may be related to symptom experience.

Serum Creatinine Distinguishes Duchenne Muscular Dystrophy from Becker Muscular Dystrophy in Patients Aged ≤3 Years: A Retrospective Study.

PubMed

Wang, Liang; Chen, Menglong; He, Ruojie; Sun, Yiming; Yang, Juan; Xiao, Lulu; Cao, Jiqing; Zhang, Huili; Zhang, Cheng

2017-01-01

Here, we investigated correlations between serum creatinine (SCRN) levels and clinical phenotypes of dystrophinopathy in young patients. Sixty-eight patients with dystrophinopathy at the Neuromuscular Clinic, The First Affiliated Hospital, Sun Yat-sen University, were selected for this study. The diagnosis of dystrophinopathy was based on clinical manifestation, biochemical changes, and molecular analysis. Some patients underwent muscle biopsies; SCRN levels were tested when patients were ≤3 years old, and reading frame changes were analyzed. Each patient was followed up, and motor function and clinical phenotype were assessed when the same patients were ≥4 years old. Our findings indicated that in young patients, lower SCRN levels were associated with increased disease severity ( p  < 0.01) and that SCRN levels were the highest in patients exhibiting mild Becker muscular dystrophy (BMD) ( p  < 0.001) and the lowest in patients with Duchenne muscular dystrophy (DMD) ( p  < 0.01) and were significantly higher in patients carrying in-frame mutations than in patients carrying out-of-frame mutations ( p  < 0.001). SCRN level cutoff values for identifying mild BMD [18 µmol/L; area under the curve (AUC): 0.947; p  < 0.001] and DMD (17 µmol/L; AUC: 0.837; p  < 0.001) were established. These results suggest that SCRN might be a valuable biomarker for distinguishing DMD from BMD in patients aged ≤3 years and could assist in the selection of appropriate treatment strategies.

Social involvement issues in patients with Becker muscular dystrophy: A questionnaire survey of subjects from a patient registry.

PubMed

Mori-Yoshimura, Madoka; Mizuno, Yukio; Yoshida, Sumiko; Minami, Narihiro; Yonemoto, Naohiro; Takeuchi, Fumi; Nishino, Ichizo; Murata, Miho; Takeda, Shin'ichi; Takahashi, Yuji; Kimura, En

2018-04-01

Little is known about the relationship between Becker Muscular Dystrophy (BMD) and developmental problems, school life, employment, and mental problems. We aimed to clarify whether BMD is a risk factor for developmental disorders, problematic behavior, psychiatric diseases, and other social difficulties in school life and employment. Adults with genetically or immunohistochemically confirmed BMD from the Registry of Muscular Dystrophy in Japan (REMUDY) were asked to complete a questionnaire regarding patient history, school life, employment, and mental problems. In total, 125 (68.3%) of 183 participants with BMD (median age, 37.2 years) completed the questionnaire. Of these, ten had developmental disorders (mental retardation, autism, and speech disturbance). Fifty-eight (44%) experienced bullying in school, and 39 felt the reason for bullying was physical handicap. Sixteen participants experienced problematic behavior such as cutting class, domestic violence, violent incidents, suicide attempts, or self-mutilation. Employment histories were noted by 92 (73%), of whom 15 could not continue to work due to physical handicaps. Fifteen participants had psychiatric disorders, with 5, 3 and 1 having neurosis, depression, and bipolar disorder, respectively. The other 6 participants with psychiatric disorders did not specify their diagnoses. Patients carrying a Dp140 expression change had significantly more incidences of developmental disorders, but not bullying, problematic behavior, workplace difficulties, or psychiatric disorders. Patients with BMD risk bullying and workplace difficulties, as well as developing psychiatric disorders. Parents, teachers, and supporters should be mindful of the daily environment of BMD patients and provide support to help them cope with stress. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

International treatment access and research, how you can help--interview with Dr. Peter Piot of UNAIDS. Joint United Nations Programme on HIV/AIDS. Interview by John S. James.

PubMed

Piot, P

1996-11-01

Medical advances in the United States have greatly increased the lifespan of people with AIDS, but there is no access to modern medicine for the 90 percent of AIDS patients who live in other countries. Dr. Peter Piot of the United Nations Programme on HIV/AIDS (UNAIDS) addresses the efforts of six U.N. agencies in improving treatment access throughout the world and improving research on natural and traditional medicines.

Peter Mark Roget: physician, scientist, systematist; his thesaurus and his impact on 19th-century neuroscience.

PubMed

Kruger, Lawrence; Finger, Stanley

2013-01-01

Peter Mark Roget (1779-1869) is best known for his Thesaurus, a project completed late in his long life. He trained as a physician, practiced medicine, and was interested in many branches of science. Much of his life was dedicated to the systematization of knowledge and identifying relationships. Although not an experimentalist in the modern sense of the word, he contributed to "neuroscience" in journal and encyclopaedia articles, as well as in books and lectures. He wrote extensively on comparative physiology, sensory systems, phrenology, optics, and various disorders affecting the nervous system. He viewed his two-volume Bridgewater Treatise of 1834 as his most significant achievement, turning to physiology and comparative anatomy to argue that God's existence can be seen in how living forms and their components are designed. Roget was active in many scholarly organizations, most notably the Royal Society of London, where he served for more than two decades as its secretary before "retiring" to pursue his Thesaurus. © 2013 Elsevier B.V. All rights reserved.

Becker muscular dystrophy-like myopathy regarded as so-called "fatty muscular dystrophy" in a pig: a case report and its diagnostic method.

PubMed

Horiuchi, Noriyuki; Aihara, Naoyuki; Mizutani, Hiroshi; Kousaka, Shinichi; Nagafuchi, Tsuneyuki; Ochiai, Mariko; Ochiai, Kazuhiko; Kobayashi, Yoshiyasu; Furuoka, Hidefumi; Asai, Tetsuo; Oishi, Koji

2014-03-01

We describe a case of human Becker muscular dystrophy (BMD)-like myopathy that was characterized by the declined stainability of dystrophin at sarcolemma in a pig and the immunostaining for dystrophin on the formalin-fixed, paraffin-embedded (FFPE) tissue. The present case was found in a meat inspection center. The pig looked appeared healthy at the ante-mortem inspection. Muscular abnormalities were detected after carcass dressing as pale, discolored skeletal muscles with prominent fat infiltrations and considered so-called "fatty muscular dystrophy". Microscopic examination revealed following characteristics: diffused fat infiltration into the skeletal muscle and degeneration and regeneration of the remaining skeletal muscle fibers. Any lesions that were suspected of neurogenic atrophy, traumatic muscular degeneration, glycogen storage disease or other porcine muscular disorders were not observed. The immunostaining for dystrophin was conducted and confirmed to be applicable on FFPE porcine muscular tissues and revealed diminished stainability of dystrophin at the sarcolemma in the present case. Based on the histological observations and immunostaining results, the present case was diagnosed with BMD-like myopathy associated with dystrophin abnormality in a pig. Although the genetic properties were not clear, the present BMD-like myopathy implied the occurrence of dystrophinopathy in pigs. To the best of our knowledge, this is the first report of a natural case of myopathy associated with dystrophin abnormalities in a pig.

Ultrasound tissue characterization does not differentiate genotype, but indexes ejection fraction deterioration in becker muscular dystrophy.

PubMed

Giglio, Vincenzo; Puddu, Paolo Emilio; Holland, Mark R; Camastra, Giovanni; Ansalone, Gerardo; Ricci, Enzo; Mela, Julia; Sciarra, Federico; Di Gennaro, Marco

2014-12-01

The aims of the study were, first, to assess whether myocardial ultrasound tissue characterization (UTC) in Becker muscular dystrophy (BMD) can be used to differentiate between patients with deletions and those without deletions; and second, to determine whether UTC is helpful in diagnosing the evolution of left ventricular dysfunction, a precursor of dilated cardiomyopathy. Both cyclic variation of integrated backscatter and calibrated integrated backscatter (cIBS) were assessed in 87 patients with BMD and 70 controls. The average follow-up in BMD patients was 48 ± 12 mo. UTC analysis was repeated only in a subgroup of 40 BMD patients randomly selected from the larger overall group (15 with and 25 without left ventricular dysfunction). Discrimination between BMD patients with and without dystrophin gene deletion was not possible on the basis of UTC data: average cvIBS was 5.2 ± 1.2 and 5.5 ± 1.4 dB, and average cIBS was 29.9 ± 4.7 and 29.6 ± 5.8, respectively, significantly different (p < 0.001) only from controls (8.6 ± 0.5 and 24.6 ± 1.2 dB). In patients developing left ventricular dysfunction during follow-up, cIBS increased to 31.3 ± 5.4 dB, but not significantly (p = 0.08). The highest cIBS values (34.6 ± 5.3 dB, p < 0.09 vs. baseline, p < 0.01 vs BMD patients without left ventricular dysfunction) were seen in the presence of severe left ventricular dysfunction. Multivariate statistics indicated that an absolute change of 6 dB in cIBS is associated with a high probability of left ventricular dysfunction. UTC analysis does not differentiate BMD patients with or without dystrophin gene deletion, but may be useful in indexing left ventricular dysfunction during follow-up. Copyright © 2014 World Federation for Ultrasound in Medicine & Biology. Published by Elsevier Inc. All rights reserved.

Hydraulic seals and their origin: Evidence from the stable isotope geochemistry of dolomites in the Middle Ordovician St. Peter Sandstone, Michigan basin

DOE Office of Scientific and Technical Information (OSTI.GOV)

Winter, B.L.; Johnson, C.M.; Valley, J.W.

1995-01-01

Regionally extensive, overpressured natural gas pools within the St. Peter Sandstone (Middle Ordovician) of the Michigan basin are bounded by rock types that include 3-7-m-thick zones of low-permeability, low-porosity carbonates or diagenetically banded quartz sandstones. Replacive dolomite from an approximately 5-m-thick carbonate interval in the east-central portion of the Michigan basin has very low {delta}{sup 13}C values that systematically decrease from approximately -5% at the top to -10% (PDB) at the base. {delta}{sup 18}O values for the replacive dolomite also decrease systematically with depth from approximately 27 to 23% (SMOW). These data suggest an upward decrease in isotope exchange betweenmore » the replacive dolomitization fluid and the precursors rock (i.e., the system was rock dominated at the top of the carbonate interval), which implies upward, cross-formational movement of the dolomitizing fluid. Fluid-rock interaction modeling suggest that the dolomitizing fluid had a total dissolved carbon (TDC) content of approximately 4000 ppm and a {delta}{sup 13}C value of -27%, which indicates that the carbon was primarily derived from organic diagenesis. Sr isotope and major element data suggest that this dolomitizing fluid had a modified seawater origin. The {delta}{sup 13}C values of sandstone intervals from three locations in the central portion of the Michigan basin range from -9 to -4% and are relatively invariant at a particular locality; therefore, the TDC of the dolomitizing fluid in the central Michigan basin is interpreted to have contained only about 20-30% organic carbon. The fact that all dolomites analyzed in the St. Peter Sandstone have much lower {Delta}{sup 13}C values that carbonates in adjacent formations indicates that dolomitization and the formation of hydraulic seals were related to organic matter diagenesis.« less

First checklist of the fruit flies of Morocco, including new records (Diptera, Tephritidae)

PubMed Central

Harym, Younes El; Belqat, Boutaïna

2017-01-01

Abstract The first checklist of the Tephritidae of Morocco, containing 59 species, is presented here. Out of 38 species collected during the present project, three (Campiglossa martii (Becker, 1908), Tephritis divisa (Rondani, 1871), and Terellia sp. near longicauda) present new records for North Africa, and ten (Carpomya incompleta (Becker, 1903), Chaetorellia conjuncta (Becker, 1913), Chetostoma curvinerve Rondani, 1856, Dacus frontalis (Becker, 1922), D. longistylus (Wiedemann, 1830), Dioxyna sororcula (Wiedemann, 1830), Ensina sonchi (Linnaeus, 1767), Myopites inulaedyssentericae Blot, 1827, M. stylatus Fabricius, 1794, and Tephritis vespertina (Loew, 1844)) are new for Morocco. PMID:29118602

Dystrophin Hot-Spot Mutants Leading to Becker Muscular Dystrophy Insert More Deeply into Membrane Models than the Native Protein.

PubMed

Ameziane-Le Hir, Sarah; Paboeuf, Gilles; Tascon, Christophe; Hubert, Jean-François; Le Rumeur, Elisabeth; Vié, Véronique; Raguénès-Nicol, Céline

2016-07-26

Dystrophin (DYS) is a membrane skeleton protein whose mutations lead to lethal Duchenne muscular dystrophy or to the milder Becker muscular dystrophy (BMD). One third of BMD "in-frame" exon deletions are located in the region that codes for spectrin-like repeats R16 to R21. We focused on four prevalent mutated proteins deleted in this area (called RΔ45-47, RΔ45-48, RΔ45-49, and RΔ45-51 according to the deleted exon numbers), analyzing protein/membrane interactions. Two of the mutants, RΔ45-48 and RΔ45-51, led to mild pathologies and displayed a similar triple coiled-coil structure as the full-length DYS R16-21, whereas the two others, RΔ45-47 and RΔ45-49, induced more severe pathologies and showed "fractional" structures unrelated to the normal one. To explore lipid packing, small unilamellar liposomes (SUVs) and planar monolayers were used at various initial surface pressures. The dissociation constants determined by microscale thermophoresis (MST) were much higher for the full-length DYS R161-21 than for the mutants; thus the wild type protein has weaker SUV binding. Comparing surface pressures after protein adsorption and analysis of atomic force microscopy images of mixed protein/lipid monolayers revealed that the mutants insert more into the lipid monolayer than the wild type does. In fact, in both models every deletion mutant showed more interactions with membranes than the full-length protein did. This means that mutations in the R16-21 part of dystrophin disturb the protein's molecular behavior as it relates to membranes, regardless of whether the accompanying pathology is mild or severe.

Skewed X-chromosome inactivation plays a crucial role in the onset of symptoms in carriers of Becker muscular dystrophy.

PubMed

Viggiano, Emanuela; Picillo, Esther; Ergoli, Manuela; Cirillo, Alessandra; Del Gaudio, Stefania; Politano, Luisa

2017-04-01

Becker muscular dystrophy (BMD) is an X-linked recessive disorder affecting approximately 1: 18.000 male births. Female carriers are usually asymptomatic, although 2.5-18% may present muscle or heart symptoms. In the present study, the role of the X chromosome inactivation (XCI) on the onset of symptoms in BMD carriers was analysed and compared with the pattern observed in Duchenne muscular dystrophy (DMD) carriers. XCI was determined on the lymphocytes of 36 BMD carriers (both symptomatic and not symptomatic) from 11 families requiring genetic advice at the Cardiomyology and Medical Genetics of the Second University of Naples, using the AR methylation-based assay. Carriers were subdivided into two groups, according to age above or below 50 years. Seven females from the same families known as noncarriers were used as controls. A Student's t-test for nonpaired data was performed to evaluate the differences observed in the XCI values between asymptomatic and symptomatic carriers, and carriers aged above or below 50 years. A Pearson correlation test was used to evaluate the inheritance of the XCI pattern in 19 mother-daughter pairs. The results showed that symptomatic BMD carriers had a skewed XCI with a preferential inactivation of the X chromosome carrying the normal allele, whereas the asymptomatic carriers and controls showed a random XCI. No concordance concerning the XCI pattern was observed between mothers and related daughters. The data obtained in the present study suggest that the onset of symptoms in BMD carriers is related to a skewed XCI, as observed in DMD carriers. Furthermore, they showed no concordance in the XCI pattern inheritance. Copyright © 2017 John Wiley & Sons, Ltd.

Cold-Air-Pool Structure and Evolution in a Mountain Basin: Peter Sinks, Utah

DOE Office of Scientific and Technical Information (OSTI.GOV)

Clements, Craig B.; Whiteman, Charles D.; Horel, John D.

2003-06-01

The evolution of potential temperature and wind structure during the buildup of nocturnal cold-air pools was investigated during clear, dry, September nights in Utah's Peter Sinks basin, a 1-km-diameter limestone sinkhole that holds the Utah minimum temperature record of -56 C. The evolution of cold-pool characteristics depended on the strength of prevailing flows above the basin. On an undisturbed day, a 30 C diurnal temperature range and a strong nocturnal potential temperature inversion (22 K in 100 m) were observed in the basin. Initially, downslope flows formed on the basin sidewalls. As a very strong potential temperature jump (17 K)more » developed at the top of the cold pool, however, the winds died within the basin and over the sidewalls. A persistent turbulent sublayer formed below the jump. Turbulent sensible heat flux on the basin floor became negligible shortly after sunset while the basin atmosphere continued to cool. Temperatures over the slopes, except for a 1 to 2-m-deep layer, became warmer than over the basin center at the same altitude. Cooling rates for the entire basin near sunset were comparable to the 90 W m-2 rate of loss of net longwave radiation at the basin floor, but these rates decreased to only a few watts per square meter by sunrise. This paper compares the observed cold-pool buildup in basins with inversion buildup in valleys.« less

Polarization Effects in the Luminescence of Cadmium Selenide Electrodes.

DTIC Science & Technology

1983-09-29

UWIS/DC/TR-83/3 Polarization Effects in the Luminescence of Cadmium Selenide Electrodes by Holger H. Streckert, Hal Van Ryswyk, Richard N. Biagioni ...Streckert, Hal Van Ryswyk, N00014-78-C-0633 Richard N. Biagioni and Arthur B. Ellis 9PEFRIGOGNZTNNAEADADDRESS 10. PROGRAM ELEMENT. PROJECT, TASKS

Peter Heller's a Child Analysis with Anna Freud: the significance of the case for the history of child psychoanalysis.

PubMed

Midgley, Nick

2012-02-01

A Child Analysis with Anna Freud, a collection of Anna Freud's detailed case notes of her treatment of the young Peter Heller between 1929 and 1932, was first published in English in 1990. Not only does this work give us direct access to Anna Freud's ways of thinking and working at a crucial period in the early history of child analysis; it is also one of the few records of an adult reflecting in depth on the experience of being in analysis as a child. Yet to date this work has received little attention in the psychoanalytic literature. In an attempt to redress this neglect, the Heller case study is placed in the context of Anna Freud's emerging ideas about child analysis. In particular, its significance in the development of her psychoanalytic thinking is investigated in the light of her 1927 book, The Technique of Child Analysis.

Principles of plastic surgery portrayed by the professional life of Dr John Peter Mettauer.

PubMed

Avashia, Yash J; Thaller, Seth R

2011-11-01

Regarded as "America's first plastic surgeon," Dr John Peter Mettauer's professional life displays 3 fundamental keystones of plastic surgery: education, innovation, and practice. To fully appreciate the history of our plastic surgery, one must look beyond a purely factual recount of noteworthy actions performed decades ago. Fundamental principles that governed achievements of our predecessors remain applicable even today. Dr Mettauer thrived as a medical student under the influence of distinguished professors in medicine at the University of Pennsylvania. Later, he continued to propagate their basic tenets when he established his medical institute in 1837. Throughout his life, Dr Mettauer combined ingenuity with scientific inquiry to devise numerous unprecedented surgical techniques and instruments. He was a prolific writer and exquisitely documented his work in medical journals for the benefit of both contemporary and future surgeons. One of Dr Mettauer's momentous achievements in plastic surgery that displays his remarkable capabilities was his contributions to management of both simple and complicated cases of cleft palate. He was the first to describe relaxing lateral incisions for treating complete cleft palates and, incidentally, was the first to successfully treat this in America. He invariably replicated similar success in establishing techniques for treating a wide range of anatomic deformities. Cumulatively, Dr Mettauer's lifelong commitment and diligence have truly laid a foundation for the eventual progress and success in the field of plastic surgery.

Technical note: Fu-Liou-Gu and Corti-Peter model performance evaluation for radiative retrievals from cirrus clouds

NASA Astrophysics Data System (ADS)

Lolli, Simone; Campbell, James R.; Lewis, Jasper R.; Gu, Yu; Welton, Ellsworth J.

2017-06-01

We compare, for the first time, the performance of a simplified atmospheric radiative transfer algorithm package, the Corti-Peter (CP) model, versus the more complex Fu-Liou-Gu (FLG) model, for resolving top-of-the-atmosphere radiative forcing characteristics from single-layer cirrus clouds obtained from the NASA Micro-Pulse Lidar Network database in 2010 and 2011 at Singapore and in Greenbelt, Maryland, USA, in 2012. Specifically, CP simplifies calculation of both clear-sky longwave and shortwave radiation through regression analysis applied to radiative calculations, which contributes significantly to differences between the two. The results of the intercomparison show that differences in annual net top-of-the-atmosphere (TOA) cloud radiative forcing can reach 65 %. This is particularly true when land surface temperatures are warmer than 288 K, where the CP regression analysis becomes less accurate. CP proves useful for first-order estimates of TOA cirrus cloud forcing, but may not be suitable for quantitative accuracy, including the absolute sign of cirrus cloud daytime TOA forcing that can readily oscillate around zero globally.

Microseismic noise in the Saint Peter and Saint Paul Archipelago, equatorial Atlantic

NASA Astrophysics Data System (ADS)

de Queiroz, Daniel É.; do Nascimento, Aderson F.; Schimmel, Martin

2017-12-01

Microseismic noise, also known as ambient seismic noise, are continuous vibrations mostly composed of Rayleigh waves pervasively recorded in the mili Hertz to 1 Hz frequency range. Their precise source mechanisms are under investigations and related to atmospheric perturbations and ocean gravity waves. Our purpose is to show the behavior of the microseismic noise recorded in the Saint Peter and Saint Paul Archipelago (SPSPA) with respect to wind intensity and ocean waves height in this region, between the North and South Atlantic Ocean. We have recorded both primary microseisms (PM) 0.04-0.12 Hz and the secondary microseisms (SM) 0.12-0.4 Hz during almost four years (2012-2015) and we used frequency, temporal, spatial and statistical correlation analysis to do qualitative and quantitative analysis with respect to wind speed intensity and significant wave height for the same periods. The results indicate a good correlation between the PM and the SM noise in the region particularly during the winter in the Northern Hemisphere and a poor correlation during the summer. We have also shown that probably most of the PM are generated in the SPSPA itself. We note that the intensity of SM recorded in SPSPA appears to have a seasonal behavior with the summer and winter in the Northern Hemisphere, and seems to influence the correlation between the PM and the SM, suggesting that the sources of the PM and the SM are not related to the same atmospheric event and from different places. PM generation would occur near the SPSPA whilst the SM would have distant sources towards the North Atlantic.

Transmission of white spot syndrome virus (WSSV) from Dendronereis spp. (Peters) (Nereididae) to penaeid shrimp.

PubMed

Haryadi, D; Verreth, J A J; Verdegem, M C J; Vlak, J M

2015-05-01

Dendronereis spp. (Peters) (Nereididae) is a common polychaete in shrimp ponds built on intertidal land and is natural food for shrimp in traditionally managed ponds in Indonesia. White spot syndrome virus (WSSV), an important viral pathogen of the shrimp, can replicate in this polychaete (Desrina et al. 2013); therefore, it is a potential propagative vector for virus transmission. The major aim of this study was to determine whether WSSV can be transmitted from naturally infected Dendronereis spp. to specific pathogen-free (SPF) Pacific white shrimp Litopenaeus vannamei (Boone) through feeding. WSSV was detected in naturally infected Dendronereis spp. and Penaeus monodon Fabricius from a traditional shrimp pond, and the positive animals were used in the current experiment. WSSV-infected Dendronereis spp. and P. monodon in a pond had a point prevalence of 90% and 80%, respectively, as measured by PCR. WSSV was detected in the head, gills, blood and mid-body of Dendronereis spp. WSSV from naturally infected Dendronereis spp was transmitted to SPF L. vannamei and subsequently from this shrimp to new naïve-SPF L. vannamei to cause transient infection. Our findings support the contention that Dendronereis spp, upon feeding, can be a source of WSSV infection of shrimp in ponds. © 2014 John Wiley & Sons Ltd.

Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study.

PubMed

Magliano, Lorenza; D'Angelo, Maria Grazia; Vita, Giuseppe; Pane, Marika; D'Amico, Adele; Balottin, Umberto; Angelini, Corrado; Battini, Roberta; Politano, Luisa; Patalano, Melania; Sagliocchi, Alessandra; Civati, Federica; Brighina, Erika; Vita, Gian Luca; Messina, Sonia; Sframeli, Maria; Lombardo, Maria Elena; Scalise, Roberta; Colia, Giulia; Catteruccia, Maria; Berardinelli, Angela; Motta, Maria Chiara; Gaiani, Alessandra; Semplicini, Claudio; Bello, Luca; Astrea, Guja; Zaccaro, Antonella; Scutifero, Marianna

2014-12-01

This study explored the burden in parents and healthy siblings of 4-17 year-old patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies, and whether the burden varied according to clinical aspects and social resources. Data on socio-demographic characteristics, patient's clinical history, parent and healthy children burden, and on parent's social resources were collected using self-reported questionnaires administered to 336 parents of patients with DMD (246) and BMD (90). Parents of patients with DMD reported higher burden than those of patients with BMD, especially concerning feeling of loss (84.3% DMD vs. 57.4% BMD), stigma (44.2% DMD vs. 5.5% BMD) and neglect of hobbies (69.0% DMD vs. 32.5% BMD). Despite the burden, 66% DMD and 62.4% BMD parents stated the caregiving experience had a positive impact on their lives. A minority of parents believed MD has a negative influence on the psychological well-being (31.0% DMD vs. 12.8% BMD), and social life of unaffected children (25.7% vs. 18.4%). In the DMD group, burden correlated with duration of illness and parent age, and burden was higher among parents with lower social contacts and support in emergencies. In DMD, difficulties among healthy children were reported as higher by parents who were older, had higher burden and lower social contacts. In both groups, burden increased in relation to patient disability. These findings underline that the psychological support to be provided to parents of patients with MD, should take into account clinical features of the disease.

Comparative cost of illness analysis and assessment of health care burden of Duchenne and Becker muscular dystrophies in Germany.

PubMed

Schreiber-Katz, Olivia; Klug, Constanze; Thiele, Simone; Schorling, Elisabeth; Zowe, Janet; Reilich, Peter; Nagels, Klaus H; Walter, Maggie C

2014-12-18

Our study aimed to determine the burden of illness in dystrophinopathy type Duchenne (DMD) and Becker (BMD), both leading to progressive disability, reduced working capacity and high health care utilization. A micro-costing method was used to examine the direct, indirect and informal care costs measuring the economic burden of DMD in comparison to BMD on patients, relatives, payers and society in Germany and to determine the health care burden of these diseases. Standardized questionnaires were developed based on predefined structured interview guidelines to obtain data directly from patients and caregivers using the German dystrophinopathy patient registry. The health-related quality of life (HRQOL) was analyzed using PedsQL™ Measurement Model. In total, 363 patients with genetically confirmed dystrophinopathies were enrolled. Estimated annual disease burden including direct medical/non-medical, indirect and informal care costs of DMD added up to € 78,913 while total costs in BMD were € 39,060. Informal care costs, indirect costs caused by loss of productivity and absenteeism of patients and caregivers as well as medical costs of rehabilitation services and medical aids were identified as the most important cost drivers. Total costs notably increased with disease progression and were consistent with the clinical severity; however, patients' HRQOL declined with disease progression. In conclusion, early assessments of economic aspects and the disease burden are essential to gain extensive knowledge of a distinct disease and above all play an important role in funding drug development programs for rare diseases. Therefore, our results may help to accelerate payer negotiations such as the pricing and reimbursement of new therapies, and will hopefully contribute to facilitating the efficient translation of innovations from clinical research over marketing authorization to patient access to a causative treatment.

Molecular Analysis-Based Genetic Characterization of a Cohort of Patients with Duchenne and Becker Muscular Dystrophy in Eastern China.

PubMed

Zhao, Hui-Hui; Sun, Xue-Ping; Shi, Ming-Chao; Yi, Yong-Xiang; Cheng, Hong; Wang, Xing-Xia; Xu, Qing-Cheng; Ma, Hong-Ming; Wu, Hao-Quan; Jin, Qing-Wen; Niu, Qi

2018-04-05

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are common X-linked recessive neuromuscular disorders caused by mutations in dystrophin gene. Multiplex polymerase chain reaction (multiplex PCR) and multiplex ligation-dependent probe amplification (MLPA) are the most common methods for detecting dystrophin gene mutations. This study aimed to contrast the two methods and discern the genetic characterization of patients with DMD/BMD in Eastern China. We collected 121 probands, 64 mothers of probands, and 15 fetuses in our study. The dystrophin gene was detected by multiplex PCR primarily in 28 probands, and MLPA was used in multiplex PCR-negative cases subsequently. The dystrophin gene of the remaining 93 probands and 62 female potential carriers was tested by MLPA directly. In fetuses, multiplex PCR and MLPA were performed on 4 fetuses and 10 fetuses, respectively. In addition, sequencing was also performed in 4 probands with negative MLPA. We found that 61.98% of the subjects had genetic mutations including deletions (50.41%) and duplications (11.57%). There were 43.75% of mothers as carriers of the mutation. In 15 fetuses, 2 out of 7 male fetuses were found to be unhealthy and 2 out of 8 female fetuses were found to be carriers. Exons 3-26 and 45-52 have the maximum frequency in mutation regions. In the frequency of exons individually, exon 47 and exon 50 were the most common in deleted regions and exons 5, 6, and 7 were found most frequently in duplicated regions. MLPA has better productivity and sensitivity than multiplex PCR. Prenatal diagnosis should be applied in DMD high-risk fetuses to reduce the disease incidence. Furthermore, it is the responsibility of physicians to inform female carriers the importance of prenatal diagnosis.

[Comparison analysis of muscle enzymes in children with myocarditis and Duchene/Becker muscular dystrophy].

PubMed

Zhang, Yali; Wang, Hong; Yu, Xuexin; Xing, Yanlin; Wang, Ce; He, Rong

2016-09-28

To compare the changes in muscle enzyme between children with myocarditis and Duchene/Becker muscular dystrophy (DMD/BMD), and to seek the explanations for variation.
 The retrospective analysis for 83 myocarditis children (myocarditis group) and 69 DMD/BMD children (DMD/BMD group), who were collected from Department of Pediatric of Shengjing Hospital affiliated to China Medical University since January 2008 to May 2015, was carried out. At the same time, 24 healthy children from the Department of Pediatric Development served as a control group. The examination indexes included creatine kinase (CK), creatine kinase-isoenzyme MB (CK-MB), creatine kinase isoenzyme MB mass (CK-MB mass), cardiac troponin I (cTnI) and high-sensitive-cTnT (hs-cTnT).
 1) In the myocarditis group, the CK increased from 100 to 1 000 U/L, reached a peak after 5 days, which lasted for a week and then dropped to the normal; the CK-MB reached a peak after 5 to 7 days and dropped to the normal a month later; the CK-MB mass reached a peak on the first day and dropped to the normal after 3 weeks; the cTn reached to a peak after 5 days and dropped to the normal after about 17 days; hs-cTnT reached to a peak on the first day and dropped to the normal after about 19 days. 2) In the DMD/BMD group, the CK increased significantly and 27 cases had a CK value of more than 10 000 U/L. After the treatment for 1 to 2 weeks, their enzyme rose again after a slight drop. In terms of cTnI, 6 cases showed a moderate increase, 5 of them couldn't drop to the normal level until more than 3 weeks later; the hs-cTnT increased in the 45 cases, which lasted for more than 3 weeks in the 31 cases of them and showed a tendency of persisting increase.
 The cTnI and hs-cTnT rise significantly and possess wider observation window than CK and CK-MB mass in myocarditis children, with more sensitive and specific changes. The myocardial damage can occur before myasthenia and keep this trend for a long time in the DMD

Duchenne Muscular Dystrophy and Becker Muscular Dystrophy Confirmed by Multiplex Ligation-Dependent Probe Amplification: Genotype-Phenotype Correlation in a Large Cohort.

PubMed

Vengalil, Seena; Preethish-Kumar, Veeramani; Polavarapu, Kiran; Mahadevappa, Manjunath; Sekar, Deepha; Purushottam, Meera; Thomas, Priya Treesa; Nashi, Saraswathi; Nalini, Atchayaram

2017-01-01

Studies of cases of Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) confirmed by multiplex ligation-dependent probe amplification (MLPA) have determined the clinical characteristics, genotype, and relations between the reading frame and phenotype for different countries. This is the first such study from India. A retrospective genotype-phenotype analysis of 317 MLPA-confirmed patients with DMD or BMD who visited the neuromuscular clinic of a quaternary referral center in southern India. The 317 patients comprised 279 cases of DMD (88%), 32 of BMD (10.1%), and 6 of intermediate phenotype (1.9%). Deletions accounted for 91.8% of cases, with duplications causing the remaining 8.2%. There were 254 cases of DMD (91%) with deletions and 25 (9%) due to duplications, and 31 cases (96.8%) of BMD with deletions and 1 (3.2%) due to duplication. All six cases of intermediate type were due to deletions. The most-common mutation was a single-exon deletion. Deletions of six or fewer exons constituted 68.8% of cases. The deletion of exon 50 was the most common. The reading-frame rule held in 90% of DMD and 94% of BMD cases. A tendency toward a lower IQ and earlier wheelchair dependence was observed with distal exon deletions, though a significant correlation was not found. The reading-frame rule held in 90% to 94% of children, which is consistent with reports from other parts of the world. However, testing by MLPA is a limitation, and advanced sequencing methods including analysis of the structure of mutant dystrophin is needed for more-accurate assessments of the genotype-phenotype correlation.

Johann Peter Griess FRS (1829–88): Victorian brewer and synthetic dye chemist

PubMed Central

Yates, Edwin; Yates, Andrew

2016-01-01

The German organic chemist Johann Peter Griess (1829–88), who first developed the diazotization of aryl amines (the key reaction in the synthesis of the azo dyes), and a major figure in the formation of the modern dye industry, worked for more than a quarter of a century at the brewery of Samuel Allsopp and Sons in Burton upon Trent, which, owing to the presence of several notable figures and an increase in the scientific approach to brewing, became a significant centre of scientific enquiry in the 1870s and 1880s. Unlike the other Burton brewing chemists, Griess paralleled his work at the brewery with significant contributions to the chemistry of synthetic dyes, managing to keep the two activities separate—to the extent that some of his inventions in dye chemistry were filed as patents on behalf of the German dye company BASF, without the involvement of Allsopp's. This seemingly unlikely situation can be explained partly by the very different attitudes to patent protection in Britain and in Germany combined with an apparent indifference to the significant business opportunity that the presence of a leading dye chemist presented to Allsopp's. Although his work for the brewery remained largely proprietary, Griess's discoveries in dye chemistry were exploited by the German dye industry, which quickly outpaced its British counterpart. One less well-known connection between brewing and synthetic dyes, and one that may further explain Allsopp's attitude, is the use of synthetic dyes in identifying microorganisms—the perennial preoccupation of brewers seeking to maintain yield and quality. Developments of Griess's original work continue to be applied to many areas of science and technology.

Transplantation of human umbilical cord-derived mesenchymal stems cells for the treatment of Becker muscular dystrophy in affected pedigree members.

PubMed

Li, Pang; Cui, Kai; Zhang, Bo; Wang, Zhendan; Shen, Yangyang; Wang, Xiangyu; Zhang, Jianbo; Tong, Feng; Li, Sheng

2015-04-01

The regeneration of muscle tissue has been achieved using multipotent mesenchymal stem cells in mouse models of injured skeletal muscle. In the present study, the utility of multipotent human umbilical cord-derived mesenchymal stem cells (hUC-MSCs) in the treatment of Becker muscular dystrophy (BMD), a genetic disease where muscle tissue fails to regenerate, was examined in members from a pedigree affected by BMD. The disease status was evaluated in 4 affected pedigree members (II1, II2, II3 and III2; aged 50, 46, 42 and 6 years, respectively). The transplantation of the hUC‑MSCs (performed on 3 patients, I2, II3 and III2) was performed by infusion with an intravenous drip over a 30‑min period, and the patients were evaluated at 1, 3, 4 and 12 weeks following the procedure. The evaluation was based on physical characteristics, as well as on molecular testing for serum creatine kinase (CK) and lactate dehydrogenase (LDH) levels and a histological examination of muscle biopsies. The patients suffered no adverse reactions in response to the transplantation of the hUC‑MSCs. At 1 week following transplantation all 3 patients showed improvement in the muscle force of the limbs, muscle size and daily activity. The walking gait of patient III2 had improved by 1 week post-transplantation and reached a normal status by 12 weeks. Serum CK and LDH levels were decreased relative to the baseline levels. A histological examination of muscle biopsies displayed no obvious tissue regeneration. In conclusion, the treatment of patients with BMD using hUC-MSCs was safe and of therapeutic benefit that lasted for up to 12 weeks. hUC-MSCs are, therefore, a potential cell therapy-based treatment option for patients with muscular dystrophies.

Mouse models of two missense mutations in actin-binding domain 1 of dystrophin associated with Duchenne or Becker muscular dystrophy.

PubMed

McCourt, Jackie L; Talsness, Dana M; Lindsay, Angus; Arpke, Robert W; Chatterton, Paul D; Nelson, D'anna M; Chamberlain, Christopher M; Olthoff, John T; Belanto, Joseph J; McCourt, Preston M; Kyba, Michael; Lowe, Dawn A; Ervasti, James M

2018-02-01

Missense mutations in the dystrophin protein can cause Duchenne muscular dystrophy (DMD) or Becker muscular dystrophy (BMD) through an undefined pathomechanism. In vitro studies suggest that missense mutations in the N-terminal actin-binding domain (ABD1) cause protein instability, and cultured myoblast studies reveal decreased expression levels that can be restored to wild-type with proteasome inhibitors. To further elucidate the pathophysiology of missense dystrophin in vivo, we generated two transgenic mdx mouse lines expressing L54R or L172H mutant dystrophin, which correspond to missense mutations identified in human patients with DMD or BMD, respectively. Our biochemical, histologic and physiologic analysis of the L54R and L172H mice show decreased levels of dystrophin which are proportional to the phenotypic severity. Proteasome inhibitors were ineffective in both the L54R and L172H mice, yet mice homozygous for the L172H transgene were able to express even higher levels of dystrophin which caused further improvements in muscle histology and physiology. Given that missense dystrophin is likely being degraded by the proteasome but whole body proteasome inhibition was not possible, we screened for ubiquitin-conjugating enzymes involved in targeting dystrophin to the proteasome. A myoblast cell line expressing L54R mutant dystrophin was screened with an siRNA library targeting E1, E2 and E3 ligases which identified Amn1, FBXO33, Zfand5 and Trim75. Our study establishes new mouse models of dystrophinopathy and identifies candidate E3 ligases that may specifically regulate dystrophin protein turnover in vivo. © The Author(s) 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Biology of Grapsus grapsus (L innaeus, 1758) (Brachyura, Grapsidae) in the Saint Peter and Saint Paul Archipelago, Equatorial Atlantic Ocean

NASA Astrophysics Data System (ADS)

Freire, A. S.; Pinheiro, M. A. A.; Karam-Silva, H.; Teschima, M. M.

2011-09-01

Eleven expeditions were undertaken to the Saint Peter and Saint Paul Archipelago to study the reproductive biology of Grapsus grapsus, providing additional information on limb mutilation and carapace colour. MATURE software was used to estimate morphological maturity, while gonadal analyses were conducted to estimate physiological maturity. The puberty moult took place at larger size in males (51.4 mm of carapace length) than in females (33.8 mm), while physiological maturity occurred at a similar size in males (38.4 mm) and in females (33.4 mm). Above 50 mm, the proportion of red males increased in the population, indicating that functional maturity is also related to colour pattern. Small habitat and high local population density contributed to the high rate of cannibalism. The low diversity of food items, absence of predators of large crabs and high geographic isolation are the determinants of unique behavioural and biological characteristics observed in the G. grapsus population.

The impact of implanted whale carcass on nematode communities in shallow water area of Peter the Great Bay (East Sea)

NASA Astrophysics Data System (ADS)

Pavlyuk, Olga N.; Trebukhova, Yulia A.; Tarasov, Vitalyi G.

2009-09-01

In May, 2007 we sank the remains of a Minke whale ( Balaenoptera acutorostrata) in the East Sea, Peter the Great Bay, at 30 m of water near the coast of Big Pelis Island. In the present study we describe the nematode communities in sediments under the implanted whale carcass. Abundance of nematodes increased with the distance from the carcass. Dominant trophic group was non-selective deposit feeders. The highest values of indexes of a specific diversity and evenness were noted in sediments under the whale, while domination index occurred at the highest distance from the whale. The suggestion is made that the cause of low density of nematodes in sediments under the whale is an extreme increase in number of macrofaunal animals, and predation and food competition between macro- and meiofauna. The changes noted in nematode assemblages living in an implanted whale in shallow waters are similar to those in deep-sea assemblages.

[Proposal for a Nationwide Johann-Peter Frank Cooperation Model under the National Leopoldina Initiative for Public Health and Global Health].

PubMed

Teichert, U; Kaufhold, C; Rissland, J; Tinnemann, P; Wildner, M

2016-07-01

The discussion on the development of public health affairs was invigorated anew by the report on public health in Germany of Leopoldina/Acatech/Union of the German Academies of Sciences and Humanities of the year 2015. The report urges strengthening of public health and global health in Germany and addresses explicitly the Public Health Service (PHS). This indispensable inclusion of the PHS in further strategic planning offers for the first time an opportunity for a comprehensive and sustainable practice/policy transfer on the federal, state and community level, and also a chance for a sustainable network with modern academic public health institutions together with representation of medical specialization in public health at universities, that has been absent so far. A Johann-Peter Frank model for cooperation and stepwise modelling of this transition with the inclusion of the Academies for Public Health Service is presented. © Georg Thieme Verlag KG Stuttgart · New York.

A Multi-Week Behavioral Sampling Tag for Sound Effects Studies: Design Trade-Offs and Prototype Evaluation

DTIC Science & Technology

2012-09-30

Doug Gillespie, SMRU/U. St. Andrews Mark Johnson, SMRU/U. St. Andrews Holger Klink, U. Oregon Phil Lovell , SMRU/U. St. Andrews David Mann, U. South...be be sourced from an ’ AA ’ size Lithium primary (non- rechargable) cell while the tag is attached to an animal and then from a rechargeable Lithium-ion

Euthanasia and John Paul II's "silent language of profound sharing of affection:" why Christians should care about Peter Singer.

PubMed

Jeffreys, Derek S

2001-12-01

Peter Singer's recent appointment to Princeton University created considerable controversy, most of it focused on his proposal for active euthanasia of disabled infants. Singer articulates utilitarian ideas that often appear in public discussions of euthanasia. Drawing on Pope John Paul II's work on ethics and suffering, I argue that Singer's utilitarian theory of value is impoverished. After introducing the Pope's ethic based on the imago dei, I discuss love as self-gift. I show how this concept supports a theory of value in which spiritual goods are preeminent over material goods. I then describe how suffering reveals spiritual goods, discussing how participation in Christ's suffering can alter our perception of value. I also consider how communal responses to suffering provide opportunities for self-giving. Third, I consider Singer's proposal for killing infants with hemophilia, arguing that it arbitrarily ignores spiritual goods. I then discuss proposals to kill anencephalic infants, discussing how parental responses to their suffering can demonstrate an extraordinary love in seemingly hopeless circumstances. I conclude by calling for a more sustained social response to euthanasia initiatives.

Patients with Duchenne muscular dystrophy are significantly shorter than those with Becker muscular dystrophy, with the higher incidence of short stature in Dp71 mutated subgroup.

PubMed

Matsumoto, Masaaki; Awano, Hiroyuki; Lee, Tomoko; Takeshima, Yasuhiro; Matsuo, Masafumi; Iijima, Kazumoto

2017-11-01

Duchenne and Becker muscular dystrophy (DMD/BMD) are caused by mutations in the dystrophin gene and are characterized by severe and mild progressive muscle wasting, respectively. Short stature has been reported as a feature of DMD in the Western hemisphere, but not yet confirmed in Orientals. Height of young BMD has not been fully characterized. Here, height of ambulant and steroid naive Japanese 179 DMD and 42 BMD patients between 4 and 10 years of age was retrospectively examined using height standard deviation score (SDS). The mean height SDS of DMD was -1.08 SD that was significantly smaller than normal (p < 0.001), indicating short stature of Japanese DMD. Furthermore, the mean height SDS of BMD was -0.27 SD, suggesting shorter stature than normal. Remarkably, the mean height SDS of DMD was significantly smaller than that of BMD (p < 0.0001). In DMD higher incidence of short stature (height SDS < -2.5 SD) was observed in Dp71 subgroup having mutations in dystrophin exons 63-79 than others having mutations in exons 1-62 (27.8% vs. 7.5%, p = 0.017). These suggested that height is influenced by dystrophin in not only DMD but also BMD and that dystrophin Dp71 has a role in height regulation. Copyright © 2017 Elsevier B.V. All rights reserved.

Hydrocephalus, agenesis of the corpus callosum, and cleft lip/palate represent frequent associations in fetuses with Peters' plus syndrome and B3GALTL mutations. Fetal PPS phenotypes, expanded by Dandy Walker cyst and encephalocele.

PubMed

Schoner, Katharina; Kohlhase, Juergen; Müller, Annette M; Schramm, Thomas; Plassmann, Margit; Schmitz, Ralf; Neesen, Juergen; Wieacker, Peter; Rehder, Helga

2013-01-01

Fetal pathology aims to recognize syndromal patterns of anomalies for goal-directed mutation analyses, genetic counseling, and early prenatal diagnosis in consecutive pregnancies. Here, we report on five fetuses with Peters' plus syndrome (PPS) from two distinct families aborted after prenatal ultrasound diagnosis of hydrocephaly. We performed fetal autopsies and molecular analyses. Among 44 fetuses with prenatally diagnosed hydrocephaly, four fetuses of 16 to 21 gestational weeks presented with additional cleft lip/palate and/or agenesis of the corpus callosum. Other features were growth retardation, hypertelorism, anomalies of the eyes, in part consistent with Peters' anterior chamber anomalies, mild brachymelia, brachydactyly, and also internal anomalies. Suspected PPS was confirmed by detection of B3GALTL mutation in these four fetuses and in one additional sib fetus, revealing homozygosity for the common c.660 + 1G > A donor splice site mutation in intron 8. Autosomal-recessive PPS has not yet been diagnosed prenatally. We want to alert ultrasonographers to the diagnosis of this disorder in growth-retarded fetuses with (recurrent) hydrocephaly, agenesis of the corpus callosum, and cleft lip/palate and stress the more severe fetal manifestation, describing a first such case with additional Dandy-Walker cyst and occult meningoencephalocele. © 2012 John Wiley & Sons, Ltd.

Cardiac profile of asymptomatic children with Becker and Duchenne muscular dystrophy under treatment with steroids and with/without perindopril.

PubMed

Mavrogeni, Sophie; Giannakopoulou, Aikaterini; Papavasiliou, Antigoni; Markousis-Mavrogenis, George; Pons, Roser; Karanasios, Evangelos; Noutsias, Michel; Kolovou, Genovefa; Papadopoulos, George

2017-07-24

To evaluate cardiovascular function in boys with Duchenne (DMD) and Becker (BMD) muscular dystrophy, using cardiac magnetic resonance (CMR). This is a single point cross sectional study of twenty-four boys with genetically ascertained DMD, and 10 with BMD, aged 10.5 ± 1.5 years (range 9-13), were prospectively evaluated by a 1.5 T system and compared with those of age-sex matched controls. The DMD patients were divided in 2 groups. Group A (N = 12) were under treatment with both deflazacort and perindopril, while Group B (n = 12) were under treatment with deflazacort, only. BMD patients did not take any medication. Biventricular function was assessed using a standard SSFP sequence. Late gadolinium enhancement (LGE) was assessed from T1 images taken 15 min after injection of 0.2 mg/Kg gadolinium DTPA using a 3D-T1-TFE sequence. Group A and BMDs were asymptomatic with normal ECG, 24 h ECG recording and echocardiogram. Group B were asymptomatic but 6/12 had abnormal ECG and mildly impaired LVEF. Their 24 h ECG recording revealed supraventricular and ventricular extrasystoles (all at 12-13 yrs). LV indices in Group A and BMD did not differ from those of controls. However, LV indices in Group B were significantly impaired compared with controls, Group A and BMDs (p < 0.001). An epicardial LGE area = 3 ± 0.5% of LV mass was identified in the posterolateral wall of LV only in 6/12 patients of Group B, but in not in any BMD or Group A. Children with either BMD or DMD under treatment with both deflazacort and perindopril present preserved LV function and lack of LGE. However, further large scale multicenter studies are warranted to confirm these data, including further CMR mapping approaches.

The 6-minute walk test, motor function measure and quantitative thigh muscle MRI in Becker muscular dystrophy: A cross-sectional study.

PubMed

Fischer, Dirk; Hafner, Patricia; Rubino, Daniela; Schmid, Maurice; Neuhaus, Cornelia; Jung, Hans; Bieri, Oliver; Haas, Tanja; Gloor, Monika; Fischmann, Arne; Bonati, Ulrike

2016-07-01

Becker muscular dystrophy (BMD) has an incidence of 1 in 16 000 male births. This cross-sectional study investigated the relation between validated functional scores and quantitative MRI (qMRI) of thigh muscles in 20 ambulatory BMD patients, aged 18.3-60 years (mean 31.2; SD 11.1). Clinical assessments included the motor function measure (MFM) and its subscales, as well as timed function tests such as the 6-minute walk test (6MWT) and the timed 10-m run/walk test. Quantitative MRI of the thigh muscles included the mean fat fraction (MFF) using a 2-point Dixon (2-PD) technique, and transverse relaxation time (T2) measurements. The mean MFM value was 80.4%, SD 9.44 and the D1 subscore 54.5%, SD 19.9. The median 6MWT was 195m, IQR 160-330.2. The median 10-m run/walk test was 7.4 seconds, IQR 6.1-9.3. The mean fat fraction of the thigh muscles was 55.6%, SD 17.4%, mean T2 relaxation times of all muscles: 69.9 ms, SD 14.4. The flexors had the highest MFF and T2 relaxation times, followed by the extensors and the adductors. MFF and global T2 relaxation times were highly negatively correlated with the MFM total, D1-subscore and 6MWT, and positively correlated with the 10 m run/walk test time (p < 0.01). Age was not correlated with MFF, global T2 relaxation time or clinical assessments. Both MFF and T2 measures in the thigh muscle were well correlated with clinical function in BMD and may serve as a surrogate outcome measure in clinical trials. Copyright © 2016 Elsevier B.V. All rights reserved.

Genetics Home Reference: phosphoribosylpyrophosphate synthetase superactivity

MedlinePlus

... Sources for This Page Ahmed M, Taylor W, Smith PR, Becker MA. Accelerated transcription of PRPS1 in ... 11):7482-8. Citation on PubMed Becker MA, Smith PR, Taylor W, Mustafi R, Switzer RL. The ...

Summary of Synoptic Meteorological Observations (SSMO). South America and Selected Island Coastal Marine Areas, Atlantic Islands. Volume 3. Area 17 - Tierra del Fuego, Area 18 - Falkland Islands, Area 19 -South Georgia, Area 20 - Tristan da Cunha Group, Area 21 - Trindade, Area 22 - St. Helena, Area 23 - Ascension, Area 24 - St. Peter and Paul Rocks

DTIC Science & Technology

1977-06-01

0 17.5 .0 .0 .0 .0 .0 .0 .0 FACE »21 »TW* ■■’■ "■ IIUI" »E«ICD! fOVE«-«LO 1963-1970 JANUARY TABLE 18 (CONTi IKEA 0021 TOINDADE...DIRECTION VIPSuS SE4 HEIGHTS IFTl IKEA 0024 ST, PETER AND PAUL ROCKS ,6N 29.3» s iu HGT 1-3 4-10 11-21 22-33 34-47 41« PCT 1...l". - L ■■■’■■’ • ’ " wwm PEIKOl iQVER-»LLI IS’OS-l1»?» I SFPTEMBfR TABLE 16 (CDNT1 IKEA 002« ST. PETER AND PAUL RCfKS

Heparan sulfate deficiency leads to Peters anomaly in mice by disturbing neural crest TGF-β2 signaling

PubMed Central

Iwao, Keiichiro; Inatani, Masaru; Matsumoto, Yoshihiro; Ogata-Iwao, Minako; Takihara, Yuji; Irie, Fumitoshi; Yamaguchi, Yu; Okinami, Satoshi; Tanihara, Hidenobu

2009-01-01

During human embryogenesis, neural crest cells migrate to the anterior chamber of the eye and then differentiate into the inner layers of the cornea, the iridocorneal angle, and the anterior portion of the iris. When proper development does not occur, this causes iridocorneal angle dysgenesis and intraocular pressure (IOP) elevation, which ultimately results in developmental glaucoma. Here, we show that heparan sulfate (HS) deficiency in mouse neural crest cells causes anterior chamber dysgenesis, including corneal endothelium defects, corneal stroma hypoplasia, and iridocorneal angle dysgenesis. These dysfunctions are phenotypes of the human developmental glaucoma, Peters anomaly. In the neural crest cells of mice embryos, disruption of the gene encoding exostosin 1 (Ext1), which is an indispensable enzyme for HS synthesis, resulted in disturbed TGF-β2 signaling. This led to reduced phosphorylation of Smad2 and downregulated expression of forkhead box C1 (Foxc1) and paired-like homeodomain transcription factor 2 (Pitx2), transcription factors that have been identified as the causative genes for developmental glaucoma. Furthermore, impaired interactions between HS and TGF-β2 induced developmental glaucoma, which was manifested as an IOP elevation caused by iridocorneal angle dysgenesis. These findings suggest that HS is necessary for neural crest cells to form the anterior chamber via TGF-β2 signaling. Disturbances of HS synthesis might therefore contribute to the pathology of developmental glaucoma. PMID:19509472

Optically continuous silcrete quartz cements of the St. Peter Sandstone: High precision oxygen isotope analysis by ion microprobe

NASA Astrophysics Data System (ADS)

Kelly, Jacque L.; Fu, Bin; Kita, Noriko T.; Valley, John W.

2007-08-01

A detailed oxygen isotope study of detrital quartz and authigenic quartz overgrowths from shallowly buried (<1 km) quartz arenites of the St. Peter Sandstone (in SW Wisconsin) constrains temperature and fluid sources during diagenesis. Quartz overgrowths are syntaxial (optically continuous) and show complex luminescent zonation by cathodoluminescence. Detrital quartz grains were separated from 53 rocks and analyzed for oxygen isotope ratio by laser fluorination, resulting in an average δ 18O of 10.0 ± 0.2‰ (1SD, n = 109). Twelve thin sections were analyzed by CAMECA-1280 ion microprobe (6-10 μm spot size, analytical precision better than ±0.2‰, 1SD). Detrital quartz grains have an average δ 18O of 10.0 ± 1.4‰ (1SD, n = 91) identical to the data obtained by laser fluorination. The ion microprobe data reveal true variability that is otherwise lost by homogenization of powdered samples necessary for laser fluorination. Laser fluorination uses samples that are one million times larger than the ion microprobe. Whole rock (WR) samples from the 53 rocks were analyzed by laser fluorination, giving δ 18O between 9.8‰ and 16.7‰ ( n = 110). Quartz overgrowths in thin sections from 10 rocks were analyzed by ion microprobe and average δ 18O = 29.3 ± 1.0‰ (1SD, n = 161). Given the similarity, on average, of δ 18O for all detrital quartz grains and for all quartz overgrowths, samples with higher δ 18O(WR) values can be shown to have more cement. The quartz cement in the 53 rocks, calculated by mass balance, varies from <1 to 21 vol.% cement, with one outlier at 33 vol.% cement. Eolian samples have an average of 11% cement compared to marine samples, which average 4% cement. Two models for quartz cementation have been investigated: high temperature (50-110 °C) formation from ore-forming brines related to Mississippi Valley Type (MVT) mineralization and formation as silcretes at low temperature (10-30 °C). The homogeneity of δ 18O for quartz overgrowths

Natalist Policies in the United States

ERIC Educational Resources Information Center

Lopoo, Leonard M.; Raissian, Kerri M.

2012-01-01

This retrospective reviews the policies that affect the fertility of American women, both policies designed to alter fertility intentionally as well as those that change childbearing unintentionally. Becker's seminal work on the economics of fertility serves as the theoretical foundation for this literature. After describing Becker's economic…

Comparison of Serum rAAV Serotype-Specific Antibodies in Patients with Duchenne Muscular Dystrophy, Becker Muscular Dystrophy, Inclusion Body Myositis, or GNE Myopathy.

PubMed

Zygmunt, Deborah A; Crowe, Kelly E; Flanigan, Kevin M; Martin, Paul T

2017-09-01

Recombinant adeno-associated virus (rAAV) is a commonly used gene therapy vector for the delivery of therapeutic transgenes in a variety of human diseases, but pre-existing serum antibodies to viral capsid proteins can greatly inhibit rAAV transduction of tissues. Serum was assayed from patients with Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), inclusion body myositis (IBM), and GNE myopathy (GNE). These were compared to serum from otherwise normal human subjects to determine the extent of pre-existing serum antibodies to rAAVrh74, rAAV1, rAAV2, rAAV6, rAAV8, and rAAV9. In almost all cases, patients with measurable titers to one rAAV serotype showed titers to all other serotypes tested, with average titers to rAAV2 being highest in all instances. Twenty-six percent of all young normal subjects (<18 years old) had measurable rAAV titers to all serotypes tested, and this percentage increased to almost 50% in adult normal subjects (>18 years old). Fifty percent of all IBM and GNE patients also had antibody titers to all rAAV serotypes, while only 18% of DMD and 0% of BMD patients did. In addition, serum-naïve macaques treated systemically with rAAVrh74 could develop cross-reactive antibodies to all other serotypes tested at 24 weeks post treatment. These data demonstrate that most DMD and BMD patients should be amenable to vascular rAAV-mediated treatment without the concern of treatment blockage by pre-existing serum rAAV antibodies, and that serum antibodies to rAAVrh74 are no more common than those for rAAV6, rAAV8, or rAAV9.

Recreation and Natural Area Needs Assessment (GREAT III)

DTIC Science & Technology

1982-01-01

1970 The Pennsylvania State University: Research and Computer Technician for Dr. E. L. Bergman, Department of Horticulture . Education B.S. The...Publication 1974 Becker, R. H. and R. 0. Ray. "Accessibility: An Application of the New Technology." Therapeutic Recreation Journal, Vol. 8, No. 4. 1976 Becker

The business against case revolution. An interview with Nestlé's Peter Brabeck. Interview by Sue Wetlaufer.

PubMed

Brabeck, P

2001-02-01

One of the world's most enduring companies, Nestle epitomizes everything that today's high-flying, headline-grabbing companies are not. It respects technology but doesn't consider it central to strategy. It values growth but prefers it controlled. It seeks talented professionals but wants only those who are modest in word and deed. Nestle CEO Peter Brabeck is skeptical of the relentless push for radical transformation heard from every quarter. He believes, instead, in continuous improvement through slow and steady change. Big, dramatic change is fine for a crisis, Brabeck says, but not every company is in crisis all the time. Many companies are like Nestle--performing well, growing and innovating, without frenzy, without bloodshed. While he acknowledges that every company must change in order to compete in today's turbulent marketplace, Brabeck makes the focus of his work identifying and strengthening those aspects of Nestle that should stay the same. For example, Nestle eschews the noise and energy swirling around technology. Many companies make technology the focal point of strategy, Brabeck says, but Nestle is about people, products, and brands. The company uses technology to create better products but keeps it in its right place--the background. Brabeck also talks candidly about how to fight complacency in a successful company, how to institutionalize collaboration in a decentralized organization, and how to resist pressure from analysts and money managers and focus on long-term, sustainable and profitable growth--in short, how to win the war without the revolution.

Prospecting Anticancer Compounds in Actinomycetes Recovered from the Sediments of Saint Peter and Saint Paul's Archipelago, Brazil.

PubMed

Ferreira, Elthon G; Torres, Maria da Conceição M; da Silva, Alison B; Colares, Larissa L F; Pires, Karine; Lotufo, Tito M C; Silveira, Edilberto R; Pessoa, Otília D L; Costa-Lotufo, Leticia V; Jimenez, Paula C

2016-09-01

Saint Peter and Saint Paul's Archipelago is a collection of 15 islets and rocks remotely located in the equatorial Atlantic Ocean. In this particular site, the present project intended to assess the biodiversity and biotechnological potential of bacteria from the actinomycete group. This study presents the first results of this assessment. From 21 sediment samples, 268 strains were isolated and codified as BRA followed by three numbers. Of those, 94 strains were grown in liquid media and submitted to chemical extractions with AcOEt (A), BuOH (B), and MeOH (M). A total of 224 extracts were screened for their cytotoxic activity and 41 were significantly active against HCT-116 cancer cells. The obtained IC 50 values ranged from 0.04 to 31.55 μg/ml. The HR-LC/MS dereplication analysis of the active extracts showed the occurrence of several known anticancer compounds. Individual compounds, identified using HR-MS combined with analysis of the AntiMarin database, included saliniketals A and B, piericidins A and C and glucopiericidin A, staurosporine, N-methylstaurosporine, hydroxydimethyl-staurosporine and N-carbamoylstaurosporine, salinisporamycin A, and rifamycins S and B. BRA-199, identified as Streptomyces sp., was submitted to bioassay-guided fractionation, leading to isolation of the bioactive piericidins A and C, glucopiericidin, and three known diketopiperazines, cyclo(l-Phe-trans-4-OH-l-Pro), cyclo(l-Phe-l-Pro), and cyclo(l-Trp-l-Pro). © 2016 Wiley-VHCA AG, Zürich.

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

ERIC Educational Resources Information Center

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…

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

PubMed Central

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

2014-01-01

Background 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. Purpose 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. Methods This qualitative study was based on interviews with clinical investigators and parents of sons with DBMD who participated in the phase IIa or IIb ataluren clinical trial in the United States. Interviews were transcribed and coded for thematic analysis. Results Participants were twelve parents of affected boys receiving active drug and nine 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 Revolution in Military Affairs: Allied Perspectives

DTIC Science & Technology

1999-04-01

M C H A ~ IR P A I? I~ I~ ! 6O • Revolution in Military Affairs: Allied Perspectives Robbin E Laird and Holger H. Mey D Report...STUDIES E3 Director: Hans Binnendijk PUBLICATION DIRECTORATE [] Director: Robert A. Silano [] General Editor, N D U Press: William R. Bode...13 3. THE RMA A N D REGIONAL ALLIES: THE ASIAN CASE . . . . . . . . . 19 . EUROPE A N D ]HE RMA

Remote Monitoring of Dolphins and Whales in the High Naval Activity Areas in Hawaiian Waters

DTIC Science & Technology

2012-09-30

developed by Holger Klinck and David Mellinger at Oregon State University to detect beaked (Cuvier’s and Blainville’s) whale biosonar signals is... biosonar signals with durations greater than approximately 250 µs. They are also the only odontocetes that produce frequency modulated clicks which...between 250 and 400 ms, depending on the species. The biosonar signals of sperm whales are also easy to discriminate visually from other deep diving

Artificial Photosynthesis: Beyond Mimicking Nature

DOE PAGES

Dau, Holger; Fujita, Etsuko; Sun, Licheng

2017-11-13

In this Editorial, Guest Editors Holger Dau, Etsuko Fujita, and Licheng Sun introduce the Special Issue of ChemSusChem on “Artificial Photosynthesis for Sustainable Fuels”. Here, they discuss the need for non-fossil based fuels, introduce both biological and artificial photosynthesis, and outline various important concepts in artificial photosynthesis, including molecular and solid-state catalysts for water oxidation and hydrogen evolution, catalytic CO 2 reduction, and photoelectrochemical systems.

Principles of Basic Wound Evaluation and Management in the Emergency Department

DTIC Science & Technology

2007-01-01

children. Equally acceptable cosmetic results were found when absorbable suture was compared with the use of nonabsorbable suture in pediatric facial ...compared with both nonabsorbable and absorb - able sutures [71]. Octyl-cyanoacrylates may be used in other areas of the body with low skin tension, but...71] Holger JS, Wnadersee SC, Hale DB, et al. Cosmetic outcomes of facial lacerations repaired with tissue adhesive, absorbable and non- absorbable

Artificial Photosynthesis: Beyond Mimicking Nature

DOE Office of Scientific and Technical Information (OSTI.GOV)

Dau, Holger; Fujita, Etsuko; Sun, Licheng

In this Editorial, Guest Editors Holger Dau, Etsuko Fujita, and Licheng Sun introduce the Special Issue of ChemSusChem on “Artificial Photosynthesis for Sustainable Fuels”. Here, they discuss the need for non-fossil based fuels, introduce both biological and artificial photosynthesis, and outline various important concepts in artificial photosynthesis, including molecular and solid-state catalysts for water oxidation and hydrogen evolution, catalytic CO 2 reduction, and photoelectrochemical systems.

Important Evidence Highlights the Meaning of Teacher-Child Relationships for Child Development. Commentary on: "Formations of Attachment Relationships towards Teachers Lead to Conclusions for Public Child Care"

ERIC Educational Resources Information Center

Sagi-Schwartz, Abraham

2016-01-01

In this commentary, Sagi-Schwartz evaluates the article by Beckh and Becker-Stoll (2016) on attachment relationships with non-parental caregivers and how it may contribute to public child care. Beckh and Becker-Stoll first describe important background about research on early parent-child relationships, and how their nature and quality might…

Whose Side Are We on and for Whom Do We Write? Notes on Issues and Challenges Facing Those Researching and Evaluating Public Policy

ERIC Educational Resources Information Center

Warren, Jon; Garthwaite, Kayleigh

2015-01-01

Becker (1967) poses the question "Whose side are we on?," a question which has become an enduring part of discussions within social scientific methodology. This paper explores the key issues in Becker's argument and considers its relevance to researchers today, locating this within a consideration of evaluation-based research and policy.…

Travel to Learn: How, When, and Why to Apply for Overseas Professional Development

ERIC Educational Resources Information Center

Cook, Erika; Becker, Aaron

2013-01-01

In this article, Erika Cook and Aaron Becker suggest teachers travel to "unfamiliar" lands to not only breathe life into global lessons but also to gain new perspectives and experiences as educators. For teachers who dread teaching about an unfamiliar place or culture, Cook and Becker recommend considering the very real opportunities to…

Definitions and Omissions of Heroism

ERIC Educational Resources Information Center

Martens, Jeffrey W.

2005-01-01

This article presents comments on "The Heroism of Women and Men" by Selwyn W. Becker and Alice H. Eagly. Their article specifically addressed the "cultural association of heroism with men and masculinity . . . in natural settings." Becker and Eagly evidenced roughly equivalent rates of heroism by women and men in a variety of settings. However,…

Becker muscular dystrophy

MedlinePlus

The health care provider will do a nervous system (neurological) and muscle exam. A careful medical history is also important, ... the legs and pelvis, then moves to the muscles of the shoulders, ... respiratory system Tests that may be done include: CPK blood ...

Koenraad Beckers | NREL

Science.gov Websites

postdoctoral researcher working on geothermal energy and CSP projects. His interests include heat and mass geothermal energy systems modeling, reservoir simulation, and economic analysis, as well as on the design and transfer, energy conversion and storage systems, reservoir modeling, and direct-use applications of thermal

Comparing the Heroism of Women and Men

ERIC Educational Resources Information Center

Eagly, Alice H.; Becker, Selwyn W.

2005-01-01

This article presents comments on "Definitions and Omissions of Heroism" by Jeffery W. Martens which is a comment on the original article "The Heroism of Women and Men" by Selwyn W. Becker and Alice H. Eagly. Becker and Eagly welcome the opportunity to discuss the questions about defining heroism that Martens raised in his comment on their…

A Survey of Principles Instructors: Why Lecture Prevails

ERIC Educational Resources Information Center

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…

Digital data sets that describe aquifer characteristics of the Rush Springs Aquifer in western Oklahoma

USGS Publications Warehouse

Runkle, D.L.; Becker, M.F.; Rea, Alan

1997-01-01

This diskette contains digitized aquifer boundaries and maps of hydraulic conductivity, recharge, and ground-water level elevation contours for the Rush Spring aquifer in western Oklahoma. This area encompasses all or part of Blaine, Caddo, Canadian, Comanche, Custer, Dewey, Grady, Stephens, and Washita Counties. These digital data sets were developed by Mark F. Becker to use as input into a computer model that simulated ground-water flow in the Rush Springs aquifer (Mark F. Becker, U.S. Geological Survey, written commun., 1997). For the purposes of modeling the ground-water flow in the Rush Springs aquifer, Mark F. Becker (written commun., 1997) defined the Rush Springs aquifer to include the Rush Springs Formation, alluvial and terrace deposits along major streams, and parts of the Marlow Formations, particularly in the eastern part of the aquifer boundary area. The Permian-age Rush Springs Formation consists of highly cross-bedded sandstone with some interbedded dolomite and gypsum. The Rush Springs Formation is overlain by Quaternary-age alluvial and terrace deposits that consist of unconsolidated clay, silt, sand, and gravel. The Rush Springs Formation is underlain by the Permian-age Marlow Formation that consists of interbedded sandstones, siltstones, mudstones, gypsum-anhydrite, and dolomite beds (Mark F. Becker, written commun., 1997). The parts of the Marlow Formation that have high permeability and porosity are where the Marlow Formation is included as part of the Rush Springs aquifer. The Rush Springs aquifer underlies about 2,400 square miles of western Oklahoma and is an important source of water for irrigation, livestock, industrial, municipal, and domestic use. Irrigation wells are reported to have well yields greater than 1,000 gallons per minute (Mark F. Becker, written commun., 1997). Mark F. Becker created some of the aquifer boundaries, hydraulic conductivity, and recharge data sets by digitizing parts of previously published surficial geology

Use of capillary Western immunoassay (Wes) for quantification of dystrophin levels in skeletal muscle of healthy controls and individuals with Becker and Duchenne muscular dystrophy.

PubMed

Beekman, Chantal; Janson, Anneke A; Baghat, Aabed; van Deutekom, Judith C; Datson, Nicole A

2018-01-01

Duchenne muscular dystrophy (DMD) is a neuromuscular disease characterized by progressive weakness of the skeletal and cardiac muscles. This X-linked disorder is caused by open reading frame disrupting mutations in the DMD gene, resulting in strong reduction or complete absence of dystrophin protein. In order to use dystrophin as a supportive or even surrogate biomarker in clinical studies on investigational drugs aiming at correcting the primary cause of the disease, the ability to reliably quantify dystrophin expression in muscle biopsies of DMD patients pre- and post-treatment is essential. Here we demonstrate the application of the ProteinSimple capillary immunoassay (Wes) method, a gel- and blot-free method requiring less sample, antibody and time to run than conventional Western blot assay. We optimized dystrophin quantification by Wes using 2 different antibodies and found it to be highly sensitive, reproducible and quantitative over a large dynamic range. Using a healthy control muscle sample as a reference and α-actinin as a protein loading/muscle content control, a panel of skeletal muscle samples consisting of 31 healthy controls, 25 Becker Muscle dystrophy (BMD) and 17 DMD samples was subjected to Wes analysis. In healthy controls dystrophin levels varied 3 to 5-fold between the highest and lowest muscle samples, with the reference sample representing the average of all 31 samples. In BMD muscle samples dystrophin levels ranged from 10% to 90%, with an average of 33% of the healthy muscle average, while for the DMD samples the average dystrophin level was 1.3%, ranging from 0.7% to 7% of the healthy muscle average. In conclusion, Wes is a suitable, efficient and reliable method for quantification of dystrophin expression as a biomarker in DMD clinical drug development.

Characteristics of Japanese Patients with Becker Muscular Dystrophy and Intermediate Muscular Dystrophy in a Japanese National Registry of Muscular Dystrophy (Remudy): Heterogeneity and Clinical Variation.

PubMed

Mori-Yoshimura, Madoka; Mitsuhashi, Satomi; Nakamura, Harumasa; Komaki, Hirofumi; Goto, Kanako; Yonemoto, Naohiro; Takeuchi, Fumi; Hayashi, Yukiko K; Murata, Miho; Takahashi, Yuji; Nishino, Ichizo; Takeda, Shin'ichi; Kimura, En

2018-01-01

Obtaining an adequate number of patients to conduct a natural history study for rare diseases such as Becker muscular dystrophy (BMD) is difficult. The present study used data from Remudy, a national registry for neuromuscular diseases in Japan, to conduct a phenotypic analysis of BMD. We analyzed Remudy data of participants with dystrophinopathy. All participants who were aged 17 and older and were ambulant at age 13 were included in this study. Participants were divided into two groups: those with BMD who were ambulant at age 17, and those with intermediate muscular dystrophy (IMD) who lost ambulation by age 17. Frequent mutations were analyzed by age at ambulation, cardiopulmonary function, and genotype. For clinical comparisons, participants who were administered steroids were excluded. From July 2009 through September 2015, 192 participants had registered with Remudy. Mean participant age was 34.80±13.3 (range, 17-78) years, and 52.1% of participants were ambulant. Of the entire study population, 50.5% had cardiomyopathy and 35.9% had respiratory failure. Three participants required invasive ventilation and 30 required non-invasive ventilation. Nineteen of the 30 non-invasive ventilator users were part-time users. In total, 138 (71.9%) had BMD and 54 (28.1%) had IMD. The most frequent mutation was ex45_ex47del (36 participants). Among participants with frequent in-frame mutations, those with the ex45-49del mutation lost their ambulation earlier than those with the ex45_ex47del mutation. A total of 67 different exon deletions and duplications were identified in the study population. We clarified the clinical phenotypes of Japanese patients with BMD/IMD using data from Remudy. Our results suggest that not only IMD but also BMD are associated with risk of respiratory dysfunction.

Interpreting the rational addiction model.

PubMed

Ferguson, B S

2000-10-01

The rational addiction (RA) model of Becker and Murphy (Becker GS, Murphy KM. A theory of rational addiction. J Pol Econ 1988; 96(4): 675-700) has rapidly become one of the standard models in the literature on addictive behaviour. This paper reviews some theoretical issues surrounding its use, and indicates areas in which caution should be used in applying this model.

Photobacterium sanctipauli sp. nov. isolated from bleached Madracis decactis (Scleractinia) in the St Peter & St Paul Archipelago, Mid-Atlantic Ridge, Brazil

PubMed Central

Moreira, Ana Paula B.; Duytschaever, Gwen; Chimetto Tonon, Luciane A.; Fróes, Adriana M.; de Oliveira, Louisi S.; Amado-Filho, Gilberto M.; Francini-Filho, Ronaldo B.; De Vos, Paul; Swings, Jean; Thompson, Cristiane C.

2014-01-01

Five novel strains of Photobacterium (A-394T, A-373, A-379, A-397 and A-398) were isolated from bleached coral Madracis decactis (scleractinian) in the remote St Peter & St Archipelago (SPSPA), Mid-Atlantic Ridge, Brazil. Healthy M. decactis specimens were also surveyed, but no strains were related to them. The novel isolates formed a distinct lineage based on the 16S rRNA, recA, and rpoA gene sequences analysis. Their closest phylogenetic neighbours were Photobacterium rosenbergii, P. gaetbulicola, and P. lutimaris, sharing 96.6 to 95.8% 16S rRNA gene sequence similarity. The novel species can be differentiated from the closest neighbours by several phenotypic and chemotaxonomic markers. It grows at pH 11, produces tryptophane deaminase, presents the fatty acid C18:0, but lacks C16:0 iso. The whole cell protein profile, based in MALDI-TOF MS, distinguished the strains of the novel species among each other and from the closest neighbors. In addition, we are releasing the whole genome sequence of the type strain. The name Photobacterium sanctipauli sp. nov. is proposed for this taxon. The G + C content of the type strain A-394T (= LMG27910T = CAIM1892T) is 48.2 mol%. PMID:25024905

A variation on forced migration: Wilhelm Peters (Prussia via Britain to Turkey) and Muzafer Sherif (Turkey to the United States).

PubMed

Russell, Gül

2016-01-01

In 1933 the Turkish Republic formally offered university positions to 30 German-speaking academics who were dismissed with the coming to power of the National Socialist Government. That initial number went up to 56 with the inclusion of the technical assistants. By 1948 the estimated total had increased to 199. Given renewable five-year contracts with salaries substantially higher than their Turkish counterparts, the foreign émigrés were to implement the westernization program of higher education. The ten year-old secular Turkish Republic's extensive social reforms had encompassed the adoption of the Latin alphabet, and equal rights for women, removing gender bias in hiring. Such a high concentration of émigré academics in one institution, "the highest anywhere in the world," provides a unique opportunity to study a subject which has been neglected. In this article two cases in psychology will be examined: Wilhelm Peters (1880-1963), who came, via Britain, to Istanbul in 1936 from the University of Jena in Germany, and Muzafer Sherif (1906-1988) who went to the United States from Ankara University in 1945. The purpose of the comparative analysis is to identify the features that are specific to the German experience, and those that are shared and underlie translocation in science within the multifaceted complexity of the process of forced migration.

Formal Methods for Biological Systems: Languages, Algorithms, and Applications

DTIC Science & Technology

2016-09-01

Moura. The yices SMT solver. Tool paper at http://yices.csl.sri.com/tool-paper. pdf, 2:2, 2006. 1.2 [80] Volker Ellenrieder, Martin E Fernandez Zapico...Oncology, 32(3):128–131, 2010. 6 [82] Mert Erkan, Simone Hausmann, Christoph W Michalski, Alexander A Fingerle, Martin Dobritz, Jörg Kleeff, and...data. In International Conference on Computer Aided Verification, pages 544–560. Springer, 2015. 1.1 [91] Martin Fränzle, Holger Hermanns, and Tino

Elastic-Plastic Behavior of Cyclotrimethylene Trinitramine Single Crystals Under Spherical Indentation: Modeling and Simulation

DTIC Science & Technology

2012-04-01

M. Winey and Y. M. Gupta, J. Appl. Phys. 107, 103505 (2010). 13R. Becker, Int. J. Plast. 20, 1983 (2004). 14B. Olinger, B. Roof, and H. H. Cady ...f011g, f021g (010), f011g, 021ð Þ 063512-8 J. D. Clayton and R. Becker J. Appl. Phys. 111, 063512 (2012) 18H. H. Cady , J. Chem. Eng. Data 17, 369

Optically Based Rapid Screening Method for Proven Optimal Treatment Strategies Before Treatment Begins

DTIC Science & Technology

2015-08-01

lifetime ( t2 ) corresponds to protein- bound NADH (23). Conversely, protein-bound FAD corre- sponds to the short lifetime, whereas free FAD corresponds...single photon counting (TCSPC) electronics (SPC-150, Becker and Hickl). TCSPC uses a fast detector PMT to measure the time between a laser pulse and... Becker and Hickl). A binning of nine surrounding pixels was used. Then, the fluorescence lifetime components were computed for each pixel by deconvolving

A Randomized, Controlled Trial of Intranasal Oxytocin as an Adjunct to Behavioral Therapy for Autism Spectrum Disorder

DTIC Science & Technology

2015-10-01

Fellowship of the Alexander von Humboldt Foundation , MIT Name: Dina Hirshfeld- Becker , PhD Project Role: Independent Evaluator Researcher Identifier...e.g. ORCID ID): Nearest person month worked: 3 Contribution to Project: Dr. Hirshfeld- Becker has been an independent evaluator and has evaluated ...either intranasal oxytocin or placebo. Participants and evaluators will be blind to treatment condition. In year 1 of the study, we set up the study

Sea-level rise impacts on the temporal and spatial variability of extreme water levels: A case study for St. Peter-Ording, Germany

NASA Astrophysics Data System (ADS)

Santamaria-Aguilar, S.; Arns, A.; Vafeidis, A. T.

2017-04-01

Both the temporal and spatial variability of storm surge water level (WL) curves are usually not taken into account in flood risk assessments as observational data are often scarce. In addition, sea-level rise (SLR) can further affect the variability of WLs. We analyze the temporal and spatial variability of the WL curve of 75 historical storm surge events that have been numerically simulated for St. Peter-Ording at the German North Sea coast, considering the effects induced by three SLR scenarios (RCP 4.5, RCP 8.5, and a RCP 8.5 high end scenario). We assess potential impacts of these scenarios on two parameters related to flooding: overflow volumes and fullness. Our results indicate that due to both the temporal and spatial variability of those events the resulting overflow volume can be two or even three times greater. We observe a steepening of the WL curve with an increase of the tidal range under the three SLR scenarios, although SLR induced effects are relatively higher for the RCP 4.5. The steepening of the WL curve with SLR produces a reduction of the fullness, but the changes in overflow volumes also depend on the magnitude of the storm surge event.

Cato Guldberg and Peter Waage, the history of the Law of Mass Action, and its relevance to clinical pharmacology.

PubMed

Ferner, Robin E; Aronson, Jeffrey K

2016-01-01

We have traced the historical link between the Law of Mass Action and clinical pharmacology. The Law evolved from the work of the French chemist Claude Louis Berthollet, was first formulated by Cato Guldberg and Peter Waage in 1864 and later clarified by the Dutch chemist Jacobus van 't Hoff in 1877. It has profoundly influenced our qualitative and quantitative understanding of a number of physiological and pharmacological phenomena. According to the Law of Mass Action, the velocity of a chemical reaction depends on the concentrations of the reactants. At equilibrium the concentrations of the chemicals involved bear a constant relation to each other, described by the equilibrium constant, K. The Law of Mass Action is relevant to various physiological and pharmacological concepts, including concentration-effect curves, dose-response curves, and ligand-receptor binding curves, all of which are important in describing the pharmacological actions of medications, the Langmuir adsorption isotherm, which describes the binding of medications to proteins, activation curves for transmembrane ion transport, enzyme inhibition and the Henderson-Hasselbalch equation, which describes the relation between pH, as a measure of acidity and the concentrations of the contributory acids and bases. Guldberg and Waage recognized the importance of dynamic equilibrium, while others failed to do so. Their ideas, over 150 years old, are embedded in and still relevant to clinical pharmacology. Here we explain the ideas and in a subsequent paper show how they are relevant to understanding adverse drug reactions. © 2015 The British Pharmacological Society.

Year Five of Southeast Atlantic Coastal Ocean Observing System (SEACOOS) Implementation

DTIC Science & Technology

2007-12-15

137 total]. Alvera -Azcarate, A., A. Barth, J.M. Beckers, and R.H. Weisberg, 2007. Multivariate reconstruction of missing data in sea surface...temperature, chlorophyll and wind satellite fields. Jour. Geophys. Res., 112, C03008, doi: 10.1029/2006JC003660. Alvera -Azcarate, A., A. Barth, and R.H...A., J.-M. Beckers, A. Alvera -Azcarate, and R. H. Weisberg, 2007. Filtering inertia-gravity waves from the initial conditions of the linear shallow

PARK2, a Large Common Fragile Site Gene, is Part of a Stress Response Network in Normal Cells That is Disrupted During the Development of Ovarian Cancer

DTIC Science & Technology

2007-01-01

from chromosomal regions known to contain a CFS and identified a number of other large CFS genes. This includes the Duchene Muscular Dystrophy (DMD...Callahan G, Becker NA, Phillips LA, Smith DI. Characterization of FRA6E and its potential role in autosomal recessive juvenile parkinsonism and...Department of Defense Support 1) Denison SR, Wang F, Becker NA, Schule B, Kock N, Phillips LA, Klein C, Smith DI. Alterations in the common fragile site

Proceedings of the Scientific Conference on Obscuration and Aerosol Research Held in Aberdeen Maryland on 27-30 June 1989

DTIC Science & Technology

1990-08-01

corneal structure for both normal and swollen corneas. Other problems of future interest are the understanding of the structure of scarred and dystrophied ...METHOD AND RESULTS The system of equations is solved numerically on a Cray X-MP by a finite element method with 9-node Lagrange quadrilaterals ( Becker ...Appl. Math., 42, 430. Becker , E. B., G. F. Carey, and J. T. Oden, 1981. Finite Elements: An Introduction (Vol. 1), Prentice- Hall, Englewood Cliffs, New

Estrogen Receptor Mutants/Variants in Human Breast Cancer.

DTIC Science & Technology

1995-12-01

Effect of dystrophin gene deletions on mRNA levels and processing in Duchenne and Becker dystrophies . Cell 1990, 63:1239-1248 4 Patriotis C, Makris A...the most common inherited disorder of platelets, Aspartylblucosaminuria, 2 an inherited lysosomal storage disorder, Duchenne and Becker muscular... dystrophies 3 or cancer progression.4m 5 Several estrogen receptor (ER) variant mRNAs have also been identified in human breast cancer biopsies.6,7,8, 9

Virus isolations and high population density implicate Culex antennatus (Becker) (Diptera: Culicidae) as a vector of Rift Valley Fever virus during an outbreak in the Nile Delta of Egypt.

PubMed

Hanafi, Hanafi A; Fryauff, David J; Saad, Magdi D; Soliman, Atef K; Mohareb, Emad W; Medhat, Iman; Zayed, Abdel Basset; Szumlas, Daniel E; Earhart, Kenneth C

2011-08-01

In June, 2003, Egypt's hospital-based electronic disease surveillance system began to record increased cases of acute febrile illness from governorates in the Nile Delta. In response to a request for assistance from the Egyptian Ministry of Health and the World Health Organization (WHO), the U.S. Naval Medical Research Unit No. 3 (NAMRU-3) provided assistance in identifying the cause and extent of this outbreak. Testing of human clinical samples (n=375) from nine governorates in Egypt identified 29 cases of RVF viremia that spanned the period of June to October, and a particular focus of disease in Kafr el Sheikh governorate (7.7% RVF infection rate). Veterinary samples (n=101) collected during this time in Kafr el Sheikh and screened by immunoassay for RVFV-specific IgM identified probable recent infections in cattle (10.4%) and sheep (5%). Entomologic investigations that focused in rural, rice growing villages in the Sidi Salim District of Kafr el Sheikh during August-September, 2003, collected, identified, and tested host-seeking female mosquitoes for the presence of pathogenic viruses. Three isolates of RVF virus (RVFV) were obtained from 297 tested pools of female mosquitoes and all three RVFV isolates came from Cx. antennatus (Becker). While Cx. pipiens has been considered the primary vector of RVF virus in Egypt and is often the most common man-biting species found, Cx. antennatus was the dominant species captured at the 2003 outbreak location in Kafr el Sheikh governorate. This is the first time that Cx. antennatus has been found naturally infected with RVFV in Egypt. Published by Elsevier B.V.

PARK2, a Large Common Fragile Site Gene, is Part of a Stress Response Network in Normal Cells that is Disrupted During the Development of Ovarian Cancer

DTIC Science & Technology

2008-01-01

Muscular Dystrophy (DMD) gene and the immediately adjacent large 7 Il1RAPL1 gene in FRAXC, LARGE in FRA22B (which is associated with myodystrophy when...OF THIS GRANT 1) Denison SR, Wqang F, Becker NA, Schule B, Kock N, Phillips LA, Klein C, Smith DI. Alterations in the common fragile site gene...apoptotic and stress responses. Biochem Pharmacol 2003; 66: 1347-1354. 13) Denison SR, Callahan G, Becker NA, Phillips LA, Smith DI. Alterations in

Journal of Rehabilitation Research and Development. Volume 27 Number 3, Summer 1990

DTIC Science & Technology

1990-01-01

First 119. Reflex Sympathetic Dystrophy in an Amputee: Case St., Springfield, IL 62794-9265 Study. Odderson IR. Czerniecki JM, Arch Ph\\vs Med Rehabil 71...60206 Compiegne cedex. France Persons with Disabilities. Becker HA. et al.. hit J Rehabil Res 12(3):235-250. 1989. 201. Psychological Aspects of...Hearing Aid Treatment. Contact: Heather A. Becker . University of Texas at Austin. Borre S. Courtois J. hit J Rehabil Res 12(3):347-348. 1989. School of

Digenetic Changes in Macro- to Nano-Scale Porosity in the St. Peter Sandstone:L An (Ultra) Small Angle Neutron Scattering and Backscattered Electron Imagining Analysis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Anovitz, Lawrence; Cole, David; Rother, Gernot

2013-01-01

Small- and Ultra-Small Angle Neutron Scattering (SANS and USANS) provide powerful tools for quantitative analysis of porous rocks, yielding bulk statistical information over a wide range of length scales. This study utilized (U)SANS to characterize shallowly buried quartz arenites from the St. Peter Sandstone. Backscattered electron imaging was also used to extend the data to larger scales. These samples contain significant volumes of large-scale porosity, modified by quartz overgrowths, and neutron scattering results show significant sub-micron porosity. While previous scattering data from sandstones suggest scattering is dominated by surface fractal behavior over many orders of magnitude, careful analysis of ourmore » data shows both fractal and pseudo-fractal behavior. The scattering curves are composed of subtle steps, modeled as polydispersed assemblages of pores with log-normal distributions. However, in some samples an additional surface-fractal overprint is present, while in others there is no such structure, and scattering can be explained by summation of non-fractal structures. Combined with our work on other rock-types, these data suggest that microporosity is more prevalent, and may play a much more important role than previously thought in fluid/rock interactions.« less

Cardiac involvement in female Duchenne and Becker muscular dystrophy carriers in comparison to their first-degree male relatives: a comparative cardiovascular magnetic resonance study.

PubMed

Florian, Anca; Rösch, Sabine; Bietenbeck, Michael; Engelen, Markus; Stypmann, Jörg; Waltenberger, Johannes; Sechtem, Udo; Yilmaz, Ali

2016-03-01

Duchenne (DMD) and Becker (BMD) muscular dystrophies are X-linked recessive disorders associated with both skeletal myopathy and progressive cardiomyopathy in males. Female DMD/BMD carriers (DMDc/BMDc) are mostly free of skeletal muscle symptoms, but they are also prone to cardiomyopathy. The aim of the current study was to characterize the frequency, pattern, and extent of cardiomyopathy in female DMD/BMD carriers (DMDc/BMDc) in comparison to their first-degree male MD relatives. Thirty-six (age 44 ± 14 years) female MD carriers (20 DMDc and 16 BMDc) constituted the 'MD carrier group' and were prospectively enrolled. All MD carriers underwent a complete CMR study comprising cine- and late gadolinium enhancement (LGE) imaging. In 22 of these women ('female MD carrier comparison group', 7 DMD and 15 BMD), at least one first-degree male relative with a previously established diagnosis of MD underwent the same CMR protocol and was assigned to the 'male MD comparison group' (n = 24, 6 DMD and 18 BMD). In the total MD carrier group, 17 (47%) MD carriers had at least one pathological CMR finding [5 (14%) with a reduced left ventricular ejection fraction (LV-EF) and 16 (44%) with the presence of LGE]. All LGE-positive patients (n = 16) showed non-ischaemic LGE with subepicardial involvement of the LV lateral free wall being the most frequent pattern (13/16, 81%). Compared with BMDc, DMDc demonstrated more frequently a pathological CMR result (65 vs. 19%; P = 0.008)--in spite of being significantly younger (40 ± 11 vs. 50 ± 16 years, P = 0.038). In the male MD comparison group, the same LGE pattern as in female carriers was seen, but with a significantly higher prevalence of cardiac abnormalities compared with their female carrier relatives constituting the female MD comparison group (75 vs. 27%; P = 0.003). Cardiac involvement is a frequent finding in female carriers of DMD, but less frequently observed in carriers of BMD. Those DMDc and BMDc with cardiac involvement

Evaluation of guppy (Poecilia reticulata Peters) immunization against Tetrahymena sp. by enzyme-linked immunosorbent assay (ELISA).

PubMed

Sharon, Galit; Nath, Pulak R; Isakov, Noah; Zilberg, Dina

2014-09-15

Analysis of the effectiveness of guppy (Poecilia reticulata Peters) immunization based on measurements of antibody (Ab) titers suffers from a shortage of reagents that can detect guppy antibodies (Abs). To overcome this problem, we immunized mice with different preparations of guppy immunoglobulins (Igs) and used the mouse antisera to develop a quantitative enzyme-linked immunosorbent assay (ELISA). The most efficient immunogen for mouse immunization was guppy Igs adsorbed on protein A/G beads. Antisera from mice boosted with this immunoglobulin (Ig) preparation were highly specific and contained high Ab titers. They immunoreacted in a Western blot with Ig heavy and light chains from guppy serum, and Ig heavy chain from guppy whole-body homogenate. The mouse anti-guppy Ig was applied in an ELISA aimed at comparing the efficiency of different routes of guppy immunization against Tetrahymena: (i) anal intubation with sonicated Tetrahymena (40,000 Tetrahymena/fish in a total volume of 10 μL) mixed with domperidon, deoxycholic acid and free amino acids (valine, leucine, isoleucine, phenylalanine and tryptophan), or (ii) intraperitoneal (i.p.) injection of sonicated Tetrahymena in complete Freund's adjuvant (15,000 Tetrahymena/fish in total a volume of 20 μL). Negative control fish were anally intubated with the intubation mixture without Tetrahymena, or untreated. ELISA measurement of anti-Tetrahymena Ab titer revealed a significantly higher level of Abs in i.p.-immunized guppies, compared to the anally intubated and control fish. In addition, the efficiency of immunization was tested by monitoring guppy mortality following (i) i.p. challenge with Tetrahymena (900 Tetrahymena/fish) or (ii) cold stress followed by immersion in water containing 10,000 Tetrahymena/mL. Fish mortality on day 14 post-Tetrahymena infection by i.p. injection exceeded 50% in the control and anally intubated fish, compared to 31% in i.p.-immunized fish. Immunization did not protect from

Year Five of Southeast Atlantic Coastal Ocean Observing System (SEACOOS) Implementation

DTIC Science & Technology

2008-09-30

published or in press that acknowledge SEACOOS support (2003-2008) [137 total]. Alvera -Azcárate, A., A. Barth, J.M. Beckers, and R.H. Weisberg...C03008, doi:10.1029/2006JC003660. Alvera -Azcárate, A., A. Barth, and R.H. Weisberg, 2008. The surface circulation of the Caribbean Sea and the Gulf of...reef fishes on the continental shelf. Marine Technology Society Journal 39(2): 110-118. Barth, A., J.-M. Beckers, A. Alvera -Azcárate, and R. H

Three novel serum biomarkers, miR-1, miR-133a, and miR-206 for Limb-girdle muscular dystrophy, Facioscapulohumeral muscular dystrophy, and Becker muscular dystrophy.

PubMed

Matsuzaka, Yasunari; Kishi, Soichiro; Aoki, Yoshitsugu; Komaki, Hirofumi; Oya, Yasushi; Takeda, Shin-Ichi; Hashido, Kazuo

2014-11-01

Muscular dystrophies are a clinically and genetically heterogeneous group of inherited myogenic disorders. In clinical tests for these diseases, creatine kinase (CK) is generally used as diagnostic blood-based biomarker. However, because CK levels can be altered by various other factors, such as vigorous exercise, etc., false positive is observed. Therefore, three microRNAs (miRNAs), miR-1, miR-133a, and miR-206, were previously reported as alternative biomarkers for duchenne muscular dystrophy (DMD). However, no alternative biomarkers have been established for the other muscular dystrophies. We, therefore, evaluated whether these miR-1, miR-133a, and miR-206 can be used as powerful biomarkers using the serum from muscular dystrophy patients including DMD, myotonic dystrophy 1 (DM1), limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), becker muscular dystrophy (BMD), and distal myopathy with rimmed vacuoles (DMRV) by qualitative polymerase chain reaction (PCR) amplification assay. Statistical analysis indicated that all these miRNA levels in serum represented no significant differences between all muscle disorders examined in this study and controls by Bonferroni correction. However, some of these indicated significant differences without correction for testing multiple diseases (P < 0.05). The median values of miR-1 levels in the serum of patients with LGMD, FSHD, and BMD were approximately 5.5, 3.3 and 1.7 compared to that in controls, 0.68, respectively. Similarly, those of miR-133a and miR-206 levels in the serum of BMD patients were about 2.5 and 2.1 compared to those in controls, 1.03 and 1.32, respectively. Taken together, our data demonstrate that levels of miR-1, miR-133a, and miR-206 in serum of BMD and miR-1 in sera of LGMD and FSHD patients showed no significant differences compared with those of controls by Bonferroni correction. However, the results might need increase in sample sizes to evaluate these three miRNAs as

Bubble Memory Module.

DTIC Science & Technology

1980-12-01

I AD-A093 642 ROCKWELL INTERNATIONAL ANAHEIM CA AUOEISSTAE-T F/S V/2 I BU13LE MEMORY MODULE. (U) DEC 80 0 0 BOHNING. F J BECKER NASI -14174...Cde under Contract NASI -14174 Dist’m/o National Aeronautics and Space Administration Scientific and Technical Information BranchA 1980 J Approvod ior...9/ BUBBLE M MORY MODULE.(U) DEC 80 0 BHNING, F J BECKER NASI -14174 NCLASSIFIED CB-569/201 NASA-CR-3380 ML22-fllfllf ll l ff mmlmmmmm.l®fmmM EEmmEI

[The natural resources of the expedition bay as a basis for the creation of the health resort centre at the coast of the Peter the Great Bay, the Sea of Japan].

PubMed

Vasil'chenko, V F; Badalov, N G; Derkacheva, L N

2014-01-01

The present article presents characteristics of the therapeutic and health promoting potential of the Expedition Bay, (part of the Peter the Great Bay, the Sea of Japan), known to be a deposit of therapeutic sea muds. The great variety of local therapeutic natural resources, viz. sea muds, sea weeds, friendly climate, beeches, etc., can be used for the treatment and prevention of various diseases. The Expedition Bay was designated as a local area of recreational and therapeutic value that can be used as a basis for the creation and further development of the multi-field health resort centre (or medical spa) that would attract patients from other regions of Russia and the surrounding countries. The future centre would provide medical services based in the first place on the classical methods of climatic and peloid therapy, thalassotherapy (heliotherapy, aeroionotherapy, bathing, marine algae therapy, etc.) as well as the modern balneotherapeutic and spa technologies, therapeutic tourism for the purpose of rehabilitation and health promotion.

Karyotypic variation in Rhinophylla pumilio Peters, 1865 and comparative analysis with representatives of two subfamilies of Phyllostomidae (Chiroptera)

PubMed Central

Gomes, Anderson José Baia; Nagamachi, Cleusa Yoshiko; Rodrigues, Luís Reginaldo Ribeiro; Farias, Solange Gomes; Rissino, Jorge Dores; Pieczarka, Julio Cesar

2012-01-01

Abstract The family Phyllostomidae belongs to the most abundant and diverse group of bats in the Neotropics with more morphological traits variation at the family level than any other group within mammals. In this work, we present data of chromosome banding (G, C and Ag-NOR) and Fluorescence In Situ Hybridization (FISH) for representatives of Rhinophylla pumilio Peters, 1865 collected in four states of Brazil (Amazonas, Bahia, Mato Grosso and Pará). Two karyomorphs were found in this species: 2n=34, FN=64 in populations from western Pará and Mato Grosso states and 2n=34, FN=62 from Amazonas, Bahia, and northeastern Pará and Marajó Island (northern). Difference in the Fundamental Number is determined by variation in the size of the Nucleolar Organizer Region (NOR) accompanied with heterochromatin on chromosomes of pair 16 or, alternatively, a pericentric inversion. The C-banding technique detected constitutive heterochromatin in the centromeric regions of all chromosomes and on the distal part of the long arm of pair 15 of specimens from all localities. FISH with a DNA telomeric probe did not show any interstitial sequence, and an 18S rDNA probe and silver staining revealed the presence of NOR in the long arm of the pair 15, associated with heterochromatin, and in the short arm of the pair 16 for all specimens. The intra-specific analysis using chromosome banding did not show any significant difference between the samples. The comparative analyses using G-banding have shown that nearly all chromosomes of Rhinophylla pumilio were conserved in the chromosome complements of Glossophaga soricina Pallas, 1766, Phyllostomus hastatus Pallas, 1767, Phyllostomus discolor Wagner, 1843 and Mimon crenulatum Geoffroy, 1801, with a single chromosomal pair unique to Rhinophylla pumilio (pair 15). However, two chromosomes of Mimon crenulatum are polymorphic for two independent pericentric inversions. The karyotype with 2n=34, NF=62 is probably the ancestral one for the other

Who is the Scientist-Subject? A Critique of the Neo-Kantian Scientist-Subject in Lorraine Daston and Peter Galison's Objectivity.

PubMed

Shah, Esha

2017-01-01

The main focus of this essay is to closely engage with the role of scientist-subjectivity in the making of objectivity in Lorraine Daston and Peter Galison's book Objectivity, and Daston's later and earlier works On Scientific Observation and The Moral Economy of Science. I have posited four challenges to the neo-Kantian and Foucauldian constructions of the co-implication of psychology and epistemology presented in these texts. Firstly, following Jacques Lacan's work, I have argued that the subject of science constituted by the mode of modern science suffers from paranoia. It is not the fear of subjectivity interfering with objectivity but the impossibility of knowing the truth of the real that causes paranoia. Here, I have argued that it is not the ethos of objectivity that drives epistemology as Daston and Galison suggest, but the pathos of paranoia. The second challenge builds upon Kant's own denial that the perfect correspondence between the human will and the moral law is possible. Kant himself thought that an ethical human act is impossible without the component of "pathology." This questions Daston and Galison's argument that there is always ethical imperative at the core of epistemic virtue. The third challenge contests the way Daston and Galison take appearance for being in their application of the Foucauldian concept of technologies of the self in modeling the master scientist-self. The fourth challenge questions the notion of the psychological and unconscious in the making of epistemology in Daston's later and earlier work. Against this background, I aim to make a claim that understanding and disclosing "entities" in the scientific domain presupposes an understanding of "being" in general. My goal is to open up the discussion for an alternative conception of the scientist-subject and thereby an affective and existential formulation of science.

Peter Rupnowski | NREL

Science.gov Websites

Nanoscience Center, develops novel, high-speed, high-resolution, inline-compatible, nondestructive techniques high-temperature fuel cells and Li-ion batteries. The techniques include hyper-spectral and thermal conference publications. Research Interests Low- and high-temperature fuel cells Li-ion batteries Development

Peter Graf | NREL

Science.gov Websites

-device simulation tool for organic photovoltaics research. Current research focuses are (1) optimization optimization in organic photovoltaics. Dr. Graf graduated Phi Beta Kappa, with distinction, from Stanford

Peter Hacke | NREL

Science.gov Websites

photovoltaic (PV) modules, inspections for root cause of module failures in the field, and accelerated lifetime delamination. His research interests are in modeling of degradation processes of PV modules, module integrated analysis of PV degradation data. He also explores accelerated multi-stress and combined stress testing to

Health behind bars: can exploring the history of prison health systems impact future policy?

PubMed

Weston, Kathryn M; McCarthy, Louella R; Meyering, Isobelle Barrett; Hampton, Stephen; Mackinnon, Tobias

2018-02-01

The value of history is, indeed, not scientific but moral … it prepares us to live more humanely in the present, and to meet rather than to foretell, the future - Carl Becker. Becker's quote reminds us of the importance of revealing and understanding historical practices in order to influence actions in the future. There are compelling reasons for uncovering this history, in particular to better inform government policy makers and health advocates, and to address the impacts of growing community expectations to 'make the punishment fit the crime'. Copyright © 2018 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

Biochemical and pathogenic properties of the natural isolate of Shewanella algae from Peter the great bay, sea of Japan.

PubMed

Beleneva, Irina A; Magarlamov, T Yu; Eliseikina, Marina G; Zhukova, Natalia V

2009-11-01

Pathogenic properties of the natural isolate of Shewanella algae from the coelomic fluid of the sea cucumber Apostichopus japonicus (Peter the Great Bay, Sea of Japan) were investigated. The isolate had oxydative metabolism, was positive for ornithine decarboxylase, cytochrome oxidase, catalase, DNase and gelatinase, hemolytically active, did not produce acid from carbohydrates, and did not hydrolyze urea and esculin. The strain was resistant to penicillin, amoxicillin, and ampicillin and susceptible to tetracycline and carbenicillin. Among cellular fatty acids, 13:0-i, 15:0-i, 16:0, 16:1(n-7), 17:0-i, and 17:0-ai dominated. These biochemical properties made it possible to attribute the isolated bacteria to the genus Shewanella and identified as S. algae. The cells of this bacterium were introduced into the coelomic cavity of another echinoderm, the sea urchin Strongylocentrotus nudus. As a result, in about 24h the animals became slow and 3-8days after the inoculation died. Dividing bacteria were being found during the experiment in the coelomic fluid as well as in the phagosomes of amoebocytes, i.e. cells acting as phagocytes in the coelomic fluid. The studies of the invasive properties of strain 156 showed that bacterial cells entered the subcuticular space of S. nudus and A. japonicus through the cuticle and stayed there for a long time without penetrating epithelium and exerting toxic effect upon the organisms of the laboratory animals. Pathogenic effect of S. algae can be manifested only if the cutaneous epithelium is destroyed permitting it to penetrate the lower tissue layers. The toxicity of S. algae is confirmed by in vitro experiments. The inoculation of the embryonic cells of S. nudus with samples of this bacterium caused the death of 10% of cells within an hour and 100% of cells within 12h after inoculation. The results of the investigations demonstrate that S. algae could produce opportunistic infection in the sea cucumber A. japonicus and the sea

White Paper To William Becker

EPA Pesticide Factsheets

This document may be of assistance in applying the Title V air operating permit regulations. This document is part of the Title V Policy and Guidance Database available at www2.epa.gov/title-v-operating-permits/title-v-operating-permit-policy-and-guidance-document-index. Some documents in the database are a scanned or retyped version of a paper photocopy of the original. Although we have taken considerable effort to quality assure the documents, some may contain typographical errors. Contact the office that issued the document if you need a copy of the original.

Duchenne and Becker Muscular Dystrophies

MedlinePlus

... trial in boys with the disease. In another approach, MDA-supported researchers at a biotechnology company are ... respira- tory or speech therapy consultations • annual flu shots • support groups for those affected, spouses, parents or ...

The effects of burial diagenesis on multiscale porosity in the St. Peter Sandstone: An imaging, small-angle, and ultra-small-angle neutron scattering analysis

DOE PAGES

Anovitz, Lawrence M.; Freiburg, Jared T.; Wasbrough, Matthew; ...

2017-11-06

To examine the effects of burial diagenesis on heirarchical pore structures in sandstone and compare those with the effects of overgrowth formation, we obtained samples of St. Peter Sandstone from drill cores obtained in the Illinois and Michigan Basins. The multiscale pore structure of rocks in sedimentary reservoirs and the mineralogy associated with those pores are critical factors for estimating reservoir properties, including fluid mass in place, permeability, and capillary pressures, as well as geochemical interactions between the rock and the fluid. The combination of small- and ultra-small-angle neutron scattering with backscattered electron or X ray-computed tomographic imaging, or both,more » provided a means by which pore structures were quantified at scales ranging from aproximately 1 nm to 1 cm—seven orders of magnitude. Larger scale (>10 µm) porosity showed the expected logarithmic decrease in porosity with depth, although there was significant variation in each sample group. However, small- and ultra-small-angle neutron scattering data showed that the proportion of small-scale porosity increased with depth. Porosity distributions were not continuous, but consisted of a series of log normal-like distributions at several distinct scales within these rocks. Fractal dimensions at larger scales decreased (surfaces smoothed) with increasing depth, and those at smaller scales increased (surfaces roughened) and pores become more isolated (higher lacunarity). Furthermore, data suggest that changes in pore-size distributions are controlled by both physical (compaction) and chemical effects (precipitation, cementation, dissolution).« less

The effects of burial diagenesis on multiscale porosity in the St. Peter Sandstone: An imaging, small-angle, and ultra-small-angle neutron scattering analysis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Anovitz, Lawrence M.; Freiburg, Jared T.; Wasbrough, Matthew

To examine the effects of burial diagenesis on heirarchical pore structures in sandstone and compare those with the effects of overgrowth formation, we obtained samples of St. Peter Sandstone from drill cores obtained in the Illinois and Michigan Basins. The multiscale pore structure of rocks in sedimentary reservoirs and the mineralogy associated with those pores are critical factors for estimating reservoir properties, including fluid mass in place, permeability, and capillary pressures, as well as geochemical interactions between the rock and the fluid. The combination of small- and ultra-small-angle neutron scattering with backscattered electron or X ray-computed tomographic imaging, or both,more » provided a means by which pore structures were quantified at scales ranging from aproximately 1 nm to 1 cm—seven orders of magnitude. Larger scale (>10 µm) porosity showed the expected logarithmic decrease in porosity with depth, although there was significant variation in each sample group. However, small- and ultra-small-angle neutron scattering data showed that the proportion of small-scale porosity increased with depth. Porosity distributions were not continuous, but consisted of a series of log normal-like distributions at several distinct scales within these rocks. Fractal dimensions at larger scales decreased (surfaces smoothed) with increasing depth, and those at smaller scales increased (surfaces roughened) and pores become more isolated (higher lacunarity). Furthermore, data suggest that changes in pore-size distributions are controlled by both physical (compaction) and chemical effects (precipitation, cementation, dissolution).« less

The Federal Republic of Germany and Left Wing Terrorism

DTIC Science & Technology

2003-12-01

Stoll, Peter Jurgen Boock, Susan Albrecht, Rolf Clemens Wagner, and Stefan Wisniewski. 49 Merkl , p. 199. 50 Ibid, p. 192. 51 Hans-Joachim Klein...during each 2 Peter H. Merkl , “Rollerball or Neo-Nazi Violence?,” in Peter H. Merkl (ed...commitment to non-violence is hypocritical.”28 Peter Merkl described the situation best when he said, “Terrorism, of course is not the logical result of

Biotechnology

NASA Image and Video Library

2001-08-04

In August 2001, principal investigator Jeanne Becker sent human ovarian tumor cells to the International Space Station (ISS) aboard the STS-105 mission. The tumor cells were cultured in microgravity for a 14 day growth period and were analyzed for changes in the rate of cell growth and synthesis of associated proteins. In addition, they were evaluated for the expression of several proteins that are the products of oncogenes, which cause the transformation of normal cells into cancer cells. This photo, which was taken by astronaut Frank Culbertson who conducted the experiment for Dr. Becker, shows two cell culture bags containing LN1 ovarian carcinoma cell cultures.

Ovarian Tumor Cells Studied Aboard the International Space Station (ISS)

NASA Technical Reports Server (NTRS)

2001-01-01

In August 2001, principal investigator Jeanne Becker sent human ovarian tumor cells to the International Space Station (ISS) aboard the STS-105 mission. The tumor cells were cultured in microgravity for a 14 day growth period and were analyzed for changes in the rate of cell growth and synthesis of associated proteins. In addition, they were evaluated for the expression of several proteins that are the products of oncogenes, which cause the transformation of normal cells into cancer cells. This photo, which was taken by astronaut Frank Culbertson who conducted the experiment for Dr. Becker, shows two cell culture bags containing LN1 ovarian carcinoma cell cultures.

Origin of Marshall Space Flight Center (MSFC)

NASA Image and Video Library

1950-01-01

Five pioneers pose with scale models of their missiles they created in the 1950s. From left to right: Dr. Ernst Stuhlinger, a member of the original German rocket team who directed the Research Projects Office, Army Ballistic Missile Agency (ABMA); Major General Holger Toftoy, who consolidated U.S. missile and rocketry development; Professor Herman Oberth, a rocket pioneer and Dr. von Braun's mentor; Dr. Wernher von Braun, Director, Development Operation Division, ABMA; and Dr. Robert Lusser, who served as assistant director for Reliability Engineering for ABMA. This photographis was taken February 1, 1956 by Hank Walker and appeared in February 27, 1956 issue of Life magazine.

Five Pioneers with Scale Models of Their Missiles

NASA Technical Reports Server (NTRS)

1950-01-01

Five pioneers pose with scale models of their missiles they created in the 1950s. From left to right: Dr. Ernst Stuhlinger, a member of the original German rocket team who directed the Research Projects Office, Army Ballistic Missile Agency (ABMA); Major General Holger Toftoy, who consolidated U.S. missile and rocketry development; Professor Herman Oberth, a rocket pioneer and Dr. von Braun's mentor; Dr. Wernher von Braun, Director, Development Operation Division, ABMA; and Dr. Robert Lusser, who served as assistant director for Reliability Engineering for ABMA. This photographis was taken February 1, 1956 by Hank Walker and appeared in February 27, 1956 issue of Life magazine.

Norman M. Dott, master of hypothalamic craniopharyngioma surgery: the decisive mentoring of Harvey Cushing and Percival Bailey at Peter Bent Brigham Hospital.

PubMed

Prieto, Ruth; Pascual, José M

2017-10-01

Norman McOmish Dott (1897-1973) developed surgical neurology in Edinburgh, Scotland, and was a scholar of worldwide renown. One of Dott's most notable contributions to neurosurgery was his understanding of hypothalamic physiology, mostly acquired through the comprehensive study of patients with lesions involving this region of the diencephalon, particularly craniopharyngiomas (CPs). Recognition of symptoms caused by hypothalamic disturbances allowed him to predict the accurate anatomical relationships between CPs and the hypothalamus, despite the rudimentary radiological methods available during the 1930s. His sophisticated knowledge permitted Dott to perform radical removals of CPs originating within the third ventricle floor with acceptable success. Between 1934 and 1937, he operated on 4 CP cases originating in the hypothalamus, achieving a satisfactory postoperative outcome in 3 of the 4 patients. Aware of the strong attachment of hypothalamic CPs to the infundibulo-tuberal area, Dott used a double transbasal and transventricular approach to these lesions, a strategy providing an optimal view and control of the tumor boundaries. The decisive mentorship of several legendary figures of physiology and neurosurgery greatly influenced Dott's surgical evolution. The experimental pituitary gland work he performed with Sir Edward Sharpey-Schäfer at the beginning of his career stirred Dott's curiosity about the issue of hypothalamus-pituitary relationships. As a result, he decided to move to Peter Bent Brigham Hospital (Boston, Massachusetts) in 1923, to train in neurosurgery and neuropathology under the guidance of the leaders in these fields, Harvey Williams Cushing (1869-1939) and Percival Sylvester Bailey (1892-1973). They inspired the young Dott and shared with him their clinical and pathological expertise, in addition to their surgical strategies for best approaching and removing these challenging tumors. In time, Dott would come to surpass his mentors. This

78 FR 77673 - Proposed CERCLA Administrative Cost Recovery Settlement; Cadie Auto Salvage Site, Belvidere...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-12-24

... the proposed settlement may be obtained from Peter Felitti, Assoc. Regional Counsel, EPA, Office of... addressed to Peter Felitti, Assoc. Regional Counsel, EPA, Office of Regional Counsel, Region 5, 77 W. Jackson Blvd., mail code: C-14J, Chicago, Illinois 60604. FOR FURTHER INFORMATION CONTACT: Peter Felitti...

77 FR 3475 - Change in Bank Control Notices; Acquisitions of Shares of a Bank or Bank Holding Company

Federal Register 2010, 2011, 2012, 2013, 2014

2012-01-24

... Hennepin Avenue, Minneapolis, Minnesota 55480-0291: 1. Samuel B. Gault, Saint Peter, Minnesota, and Lisa R. Gault, Chaska, Minnesota, each to acquire 25 percent or more of the shares of Saint Peter Agency, Inc., Saint Peter, Minnesota, and thereby indirectly acquire control of The Nicollet County Bank of Saint...

20 CFR 416.1166 - How we deem income to you and your eligible child from your ineligible spouse.

Code of Federal Regulations, 2010 CFR

2010-04-01

.... Example 1. Mary, a blind individual, lives with her husband, John, and their disabled child, Peter. Mary and Peter have no income, but John is employed and earns $605 per month. We determine Mary's... is eligible; therefore, there is no income to be deemed to Peter. Example 2. Al, a disabled...

Genetics Home Reference: myotonia congenita

MedlinePlus

... Manual Consumer Version: Congenital Myopathies Orphanet: Thomsen and Becker disease Patient Support and Advocacy Resources (3 links) Muscular Dystrophy Association National Organization for Rare Disorders (NORD) Resource ...

Fullerenes, fulleranes and polycyclic aromatic hydrocarbons in the Allende meteorite

NASA Technical Reports Server (NTRS)

Becker, L.; Bunch, T. E.

1997-01-01

In this paper, we confirm our earlier observations of fullerenes (C60 and C70) in the Allende meteorite (Becker et al., 1994a, 1995). Fullerene C60 was also detected in two separate C-rich (approximately 0.5-1.0%) dark inclusions (Heymann et al., 1987) that were hand picked from the Allende sample. The amounts of C60 detected were approximately 5 and approximately 10 ppb, respectively, which is considerably less than what was detected in the Allende 15/21 sample (approximately 100 ppb; Becker et al., 1994a, 1995). This suggests that fullerenes are heterogeneously distributed in the meteorite. In addition, we present evidence for fulleranes, (C60Hx), detected in separate samples by laser desorption (reflectron) time-of-flight (TOF) mass spectrometry (LDMS). The LDMS spectra for the Allende extracts were remarkably similar to the spectra generated for the synthetic fullerane mixtures. Several fullerane products were synthesized using a Rh catalyst (Becker et al., 1993a) and separated using high-performance liquid chromatography (HPLC). Polycyclic aromatic hydrocarbons (PAHs) were also observed ppm levels) that included benzofluoranthene and corannulene, a cup-shaped molecule that has been proposed as a precursor molecule to the formation of fullerenes in the gas phase (Pope et al., 1993).

What You See Is Not What You Get

ERIC Educational Resources Information Center

White-McMahon, Meredith

2010-01-01

When upset, 15-year-old Peter overreacts, dumping verbal hostility on everyone, even those trying to help. Peter's attempt to see the principal--who was out of the office--led to an emotionally explosive crisis. In this life space crisis intervention (LSCI), staff calmly tried to help Peter clarify distorted reality. But patient questioning raised…

Report on Teaching: Analysis of Some of the Most Notable Improvements in Amesican Undergraduate Teaching. No. 4.

ERIC Educational Resources Information Center

Change Magazine, 1977

1977-01-01

The fourth in a series of reports on undergraduate teaching contains articles on three disciplines: (1) geography (William D. Pattison, Salvatore J. Natoli, Peter Binzen, Charles J. Sugnet, Edwin Kiester, Jr., Sally Valente Kiester, Evan Jenkins, Peter Kakela, David Lanegran, Paul W. English, Peter Gould, and Alan DeLucia); (2) music (Theodore A.…

Quantitative Voxel-to-Voxel Comparison of TriBeam and DCT Strontium Titanate Three-Dimensional Data Sets (Postprint)

DTIC Science & Technology

2015-02-09

Peter Gumbsch Karlsruhe Institute of Technology Melanie Syha European Synchrotron Radiation Facility Peter Gumbsch Fraunhofer IWM 9...REPORT DOCUMENTATION PAGE Cont’d 6. AUTHOR(S) 3) Melanie Syha - ESRF 4) Peter Gumbsch - Fraunhofer IWM 7. PERFORMING ORGANIZATION NAME(S...4) Fraunhofer IWM , Woelerstr. 11, 79108 Freiburg, Germany Standard Form 298 (Rev. 8-98) Prescribed by ANSI Std

Findings from the experience with the punch technique for auditory osseointegrated implants: A retrospective single center comparative study.

PubMed

Bonilla, Alfonso; Magri, Carlos; Juan, Eulalia

To compare the punch technique and linear incision with soft tissue reduction for the placement of auditory osseointegrated implants (AOI) and analyze results of osseointegration obtained with the punch technique as measured with the Implant Stability Quotient (ISQ). Case review of 34 patients who received auditory osseointegrated implants between January 2010 and July 2015 and were divided into two groups according to the surgical technique: 18 with the punch technique (PT) and 16 with the linear incision technique (LI). Minimum follow-up was four months (mean: 24 months; range 4-64 months). Included in the analysis were patient profiles and records of the demographic data, surgical indications, surgical technique, implant placement, surgical time, intraoperative complications, as well as postsurgical complications (Holgers classification) and implant stability quotients (ISQ). Use of larger abutments was significantly greater in the PT group (PT, 10mm; LI, 6mm, p<0.001). The PT technique resulted in a shorter procedure than the LI (PT, 20min; LI, 45min, p<0.001). Holgers classification scores identified significantly fewer skin complications one week after surgery for the PT group; however, only small differences were seen between the two groups at the one- and three-month control visits. As shown for our cohort, the punch technique for surgical placement of AOI is faster and presents fewer immediate postoperative complications when compared to the linear incision technique. The clinical application of the ISQ is a useful, easy method to demonstrate the status of osseointegration and, thus, the stability of the device. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Peter St. John | NREL

Science.gov Websites

for microbial strain design to optimize the production of value-added chemicals from lignin using Pseudomonas putida. Featured Publications "A quantitative model for the prediction of sooting tendency

Barlow, Peter (1776-1862)

NASA Astrophysics Data System (ADS)

Murdin, P.

2000-11-01

Nautical engineer, born in Norwich, Norfolk, England, professor of mathematics in the Royal Military Academy, Woolwich, where he worked on the strength of ships' timbers, tidal engineering and ships' magnetism. His best known publication is New Mathematical Tables, giving the factors, squares, cubes, square and cube roots, reciprocals and hyperbolic logarithms of all numbers from 1 to 10 000, tog...

Juno Jupiter Into the Unknown

NASA Image and Video Library

2016-06-23

A look into the JOI Mission; Narrated by Heidi Becker, Juno Radiation Monitoring Investigation Lead: Scott Bolton, Juno Principal Investigator; Steven Levin, Juno Project Scientist; Rick Nybakken, Juno Project Manager.

Myotonia Congenita

MedlinePlus

... increased. There are two forms of the disorder: Becker-type, which is the most common form; and Thomsen’s disease, which is ... Association National Institute of Arthritis and Musculoskeletal and ...

Science and Technology Community in Crisis

DTIC Science & Technology

2002-05-01

centralized, highly bureaucratic, support system that considers itself an end, a point noted in the management literature by Peter Drucker :48 “What...Review Quarterly, Spring 2000. 48 Drucker , Peter F., Management : Tasks, Responsibilities, Practices, Apr 1993. 43 regionalization of personnel...technical areas as information technology, biotechnology, nanotechnology, etc. Peter Drucker , the well-known management expert once commented: “In

Recognizing and Nurturing Hidden Creative Potential

ERIC Educational Resources Information Center

Connell, Elizabeth E.

2010-01-01

Peter, 7 years old, spends most of his time playing with his stick alone. After several unsuccessful attempts to get Peter to join his cousins, his aunt asks the boy's parents if they have considered having him evaluated by a psychologist. Peter's parents are not as concerned about the stick behavior as is his aunt. Although the attachment to his…

Eliciting and Measuring Betrayal Aversion using the BDM Mechanism.

PubMed

Quercia, Simone

2016-05-01

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.

Illitization and paleothermal regimes in the Middle Ordovician St. Peter Sandstone, Central Michigan Basin: K-Ar, Oxygen Isotope, and fluid inclusion data

DOE Office of Scientific and Technical Information (OSTI.GOV)

Girard, J.P.; Barnes, D.A.

1995-01-01

Hydrocarbon reservoirs occur in the Middle Ordovician St. Peter Sandstone in the central Michigan basin at depths of 1.5-3.5 km and are diagenetically altered. Latest diagenetic cements include saddle dolomite, pervasive microcrystalline illite and chlorite, and quartz. A K-Ar and {sup 18}O/{sup 16}O study of the fine-grained authigenic illite in 25 samples from 16 wells covering a large area within the basin yields K-Ar ages ranging from 367 to 322 Ma and {delta}{sup 18}O values between 12.7 and 16.9% SMOW. The {delta}{sup 18}O values of diagenetic quartz overgrowths range from 15.2 to 18.9%. Fluid inclusion temperatures in the quartz cementmore » range from 70 to 170{degrees}C, reflecting multiple generations of diagenetic quartz and/or precipitation over most of the diagenetic history. Reequilibrated fluid inclusions in the saddle dolomite cement yield temperatures ranging from 90 to 150{degrees}C. A regionally significant episode of illitization occurred during the Late Devonian-Mississipian. Temperatures of illite formation are indirectly estimated to be in the range of 125-170{degrees}C and most paleodepths of illitization are between 2.8 and 3.2 km. These results imply that (1) illite formed from {sup 18}O-rich fluids, and (2) elevated geothermal gradients, i.e., greater than 34% C/km, existed in the Michigan basin in the late Paleozoic. The K-Ar ages and the {delta}{sup 18}O values are not correlated to present depths of the samples or paleodepths of illitization. Illites with young ages and low {delta}{sup 18}O values tend to be geographically distributed along the north-south branch of the buried Precambrian rift. The {delta}{sup 18}O values of the diagenetic quartz follow a similar trend. The spread of illite K-Ar ages and {delta}{sup 19}O values, and their geographic distribution, are best explained as reflecting abnormally high thermal regimes in the part of the basin located above the presumably highly fractured basement along the rift.« less

Areal lithologic changes in bedrock aquifers in southeastern Minnesota as determined from natural-gamma borehole logs methods

USGS Publications Warehouse

Woodward, D.G.

1984-01-01

Interpretation of natural-gamma logs indicates that the shaley and silty sandstones that comprise the basal St. Peter confining bed, which separates the St. Peter and Prairie du Chien aquifers, are as much as 80 feet thick in the Twin City basin, but are absent in the southern part of the embayment. Differences in potentiometric head across the basal St. Peter are about 30 feet in the Twin City basin where the confining bed is present but only 5 to 10 feet to the south where the confining bed is absent and where the St. Peter aquifer directly overlies the Prairie du Chien-Jordan aquifer.

Evaluation of Limb-Girdle Muscular Dystrophy

ClinicalTrials.gov

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)

Muscular Dystrophy

MedlinePlus

... Gardner-Medwin D. Variability in clinical, genetic and protein abnormalities in manifesting carriers of Duchenne and Becker muscular dystrophy . Neuromuscul. Disord. Jan 1993;3(1):57-64. 3. Bushby KM, Appleton R, Anderson ...

Interagency Conflict Assessment Framework: A Pragmatic Tool for Army Design

DTIC Science & Technology

2010-12-02

Peter Checkland and John Poulter, Learning for Action: A Short Definitive Account of Soft Systems Methodology and its use for Practitioners, Teachers...lend itself to mechanistic analytical methods.32 Peter Checkland and John Poulter suggest that each approach is neither right nor wrong, rather...their relationships. According to Peter Checkland , the use of what he describes as “rich pictures” are excellent tools for capturing the dynamics of a

49 CFR 37.53 - Exception for New York and Philadelphia.

Code of Federal Regulations, 2010 CFR

2010-10-01

... Eastern Paralyzed Veterans Association, Inc., James J. Peters, Terrance Moakley, and Denise Figueroa... between Eastern Paralyzed Veterans Association of Pennsylvania, Inc., and James J. Peters, individually...

78 FR 11660 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-02-19

... Boulevard, Rockville, MD 20852 (Telephone Conference Call). Contact Person: Peter Zelazowski, Ph.D..., peter[email protected] . (Catalogue of Federal Domestic Assistance Program Nos. 93.864, Population...

Eliciting and Measuring Betrayal Aversion using the BDM Mechanism*

PubMed Central

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

77 FR 60447 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2012-10-03

..., 6100 Executive Boulevard, Rockville, MD 20852 (Telephone Conference Call). Contact Person: Peter..., 301-435-6902, peter[email protected] . (Catalogue of Federal Domestic Assistance Program Nos. 93.864...

Feasibility Study of a Novel Diet-Based Intervention for Prostate Cancer

DTIC Science & Technology

2011-09-01

Trial of Diet to Alter Disease Progression in Prostate Cancer Patients on Active Surveillance.” Peter Van Veldhuizen , Jr., MD will be joining the...Administrator Nathan Eriksen SWOG Prevention Committee Chairs Powel H. Brown, MD, PhD Gary E. Goodman, MD New SWOG Co-Chair Peter Van Veldhuizen , Jr...above. The Cancer and Leukemia Group B (CALGB) will be responsible for circulating all revisions of the protocol to Dr. Peter Van Veldhuizen , Jr

Identification of Genetic Markers of the Invasive Phenotype in Human Breast Cancer

DTIC Science & Technology

2001-10-01

Genetic Markers of the Invasive Phenotype in Human Breast Cancer PRINCIPAL INVESTIGATOR: Dr. Peter Watson CONTRACTING ORGANIZATION: University of...Markers of the Invasive Phenotype DAMD17-97-1-7320 in Human Breast Cancer 6. AUTHOR(S) Dr. Peter Watson 7. PERFORMING ORGANIZATION NAME(S) AND ADDRESS(ES...markers of the invasive phenotype in human breast cancer" Dr Peter H. Watson INTRODUCTION. The acquisition of the ability to invade is the single most

Comparison of seawater CO2 system in summer between the East China Sea shelf and the Peter the Great Bay of the Japan (East) Sea

NASA Astrophysics Data System (ADS)

Chuang, K. Y.; Tishchenko, P. Y.; Gong, G. C.; Chou, W. C.; Tishchenko, P. P.; Shkirnikova, E. M.

2016-02-01

Continental shelves are active sites of air-sea CO2 exchange and represent an important component of the global carbon budget. In this study, we investigated the CO2 system and pertinent hydrographic parameters in two distinct continental shelf systems in the Northwest Pacific in summer 2014: the East China Sea shelf (ECSS) and the Peter the Great Bay (PGB) of the Japan/East Sea. The results show that the average temperature, pH, chlorophyll a and nutrients in the ECSS are higher, but salinity, dissolved inorganic carbon, and fugacity of CO2 are lower than those in the PGB. Meanwhile, the ECSS acted as a sink of atmospheric CO2, but the PGB was a source. We suggest that the observed divergent behaviors in terms of CO2 absorption between the ECSS and the PGB may be associated with their difference in riverine runoff. Under the influence of the Yangtze River, the nutrient discharge into the ECSS is much higher than that into the PGB, where only a few small rivers empty into. The high nutrient discharge into the ECSS may stimulate high biological production, which may drawdown CO2 and thereby driving the ECSS to act as a CO2 sink despite high temperature in summer. On the contrary, the warming effect may dominate over the effect of biological production in the PGB due to the limited nutrient discharge, and thus turn the PGB to be a source of atmospheric CO2. The results of this study imply that riverine nutrient discharge may exert a large control on net ecosystem productivity in shelf areas, which may subsequently play a critical role on determining whether a shelf system acts as a source or a sink of atmospheric CO2.

78 FR 17419 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2013-03-21

... Person: Peter Zelazowski, Ph.D., Scientific Review Officer, Division of Scientific Review, Eunice Kennedy..., Bethesda, MD 20892, 301-435-6902, peter[email protected] . (Catalogue of Federal Domestic Assistance...

77 FR 2083 - Notice of Inventory Completion: Minnesota Indian Affairs Council, Bemidji, MN

Federal Register 2010, 2011, 2012, 2013, 2014

2012-01-13

... the north side of Height of Land Lake, site 21BK48, by Mr. William Krause during road construction and... near Ice Cracking Lake during road construction and donated to the Becker County Historical Society (HR...

Genetics Home Reference: epilepsy-aphasia spectrum

MedlinePlus

... Van Paesschen W, Caraballo R, Fejerman N, Weckhuysen S, De Jonghe P, Larsen J, Møller RS, Hjalgrim H, ... B, Kurlemann G, Kluger G, Hahn A, Haberlandt DE, Kutzer C, Sperner J, Becker F, Weber YG, ...

Immersive Environments in ADL

DTIC Science & Technology

2009-08-20

Tracking and Storing In Browser 3-D 13 Questions or Comments? Peter Smith Team Lead, Immersive Learning Technologies peter.smith.ctr@adlnet.gov +1.407.384.5572 ...Immersive Environments in ADL Mr. Peter Smith, Lead, ADL Immersive Learning Team 08/20/2009 Report Documentation Page Form ApprovedOMB No. 0704-0188...5d. PROJECT NUMBER 5e. TASK NUMBER 5f. WORK UNIT NUMBER 7. PERFORMING ORGANIZATION NAME(S) AND ADDRESS(ES) Advanced Decision Learning (ADL),1901 N

Konrad Adenauer’s Military Advisors

DTIC Science & Technology

1989-02-13

Ausgabe. Hans-Peter Schwarz and 45 Rudolf Morsey, Hg. Vol. 1, Briefe 1945-1947 hg. v. Hans Peter Mensing. Berlin: Siedler Verlag, 1983. Vol. 2, Briefe 1949...Dietrich, Rudolf Morsey and Hans-Peter Schwarz, ed. Quellen zur Geschichte des Parlarnentarismus und der politischen Partein. Bd. 3, Auftakt zur Ara...New York: Penguin, 1982. Steiner , Jirg. European Democracies. New York: Longman, 1986. Taylor, A.J.P. The Origens of the Second World War. 2d. ed. New

Set Based Program Analysis

DTIC Science & Technology

1992-10-01

Philosophy Thesis Committee: Peter Lee, Co-Chair Frank Pfenning, Co-chair Dana Scott Joxan Jaffar, IBM Neil Jones, DIKU, Copenhagen Copyright C 1992...much of this thesis represents joint work with him. I am also indebted to Peter Lee and Frank Pfenning, my advisors at CMU. Their encouragement...I am very grateful to Peter , Frank, Joxan and CMU that this has been a synergistic experience. I would like to thank Neil Jones and Dana Scott for

75 FR 36429 - Eunice Kennedy Shriver National Institute of Child Health and Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-06-25

.... Place: Legacy Hotel and Meeting Center, 1775 Rockville Pike, Rockville, MD 20852. Contact Person: Peter..., 301-435-6902, PETER[email protected] . (Catalogue of Federal Domestic Assistance Program Nos. 93.864...

77 FR 3108 - Dividend Equivalents From Sources Within the United States

Federal Register 2010, 2011, 2012, 2013, 2014

2012-01-23

.... Erwin or D. Peter Merkel at (202) 622-3870 (not a toll-free number). SUPPLEMENTARY INFORMATION... is D. Peter Merkel, the Office of Associate Chief Counsel (International). Other personnel from the...

77 FR 53141 - Dividend Equivalents From Sources Within the United States

Federal Register 2010, 2011, 2012, 2013, 2014

2012-08-31

.... Peter Merkel at (202) 622-3870 (not a toll-free number). SUPPLEMENTARY INFORMATION: Background On... Information The principal author of these regulations is D. Peter Merkel, the Office of Associate Chief...

HOMOGENEOUS CATALYTIC OXIDATION OF HYDROCARBONS IN ALTERNATIVE SOLVENTS

EPA Science Inventory

Homogeneous Catalytic Oxidations of Hydrocarbons in Alternative Solvent Systems

Michael A. Gonzalez* and Thomas M. Becker, Sustainable Technology Division, Office of Research and Development; United States Environmental Protection Agency, 26 West Martin Luther King Drive, ...

Some Historical Points of Interest in Göttingen

NASA Astrophysics Data System (ADS)

Hentschel, Klaus

The Georgia Augusta University of Göttingen, founded in 1737, was a child of the Enlightenment, and the new sciences have always played a major role here.1 Among the teachers of physics, physical chemistry, astronomy, and related subjects we find Johann Christian Polykarp Erxleben, Georg Christoph Lichtenberg, Johann Tobias Mayer, Carl Friedrich Gauss, Johann B. Listing, Wilhelm Eduard Weber, Woldemar Voigt, Friedrich Kohlrausch, Eduard Riecke, Walther Nernst and Peter Debye — the last two subsequently moved on to Berlin. In the 1920s, physics students were jestingly referred to as “Frankierte, Bornierte und Polierte” (loosely translated as stamped, limited and polished), in allusion to their teachers, the theoretical physicist Max Born and the experimentalists James Franck and Robert Wichard Pohl, the first two being important figures in the history of quantum theory, the third, one of the founding fathers of experimental solid state physics.2 The National Socialist’s rise to power had a devastating effect on this world-renowned center for physics and mathematics. Most of its high-caliber scientists either were dismissed on the basis of the racist “Law for the Restoration of the Professional Civil Service” or themselves felt compelled to emigrate: About a dozen members of the physics faculty, including Born and Franck, and ten from the mathematics faculty left Göttingen.3 After the war, Richard Becker, who in 1936 had received a compulsory order to take the chair for theoretical physics vacant since Born’s emigration, and Friedrich Hund, who was also an enthusiastic historian of science, distinguished themselves as physics teachers there but the university as a whole never recovered its international standing of before 1933 (see Figs. 1 and 2).

75 FR 4828 - Eunice Kennedy Shriver National Institute of Child Health and Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-01-29

..., (Telephone Conference Call). Contact Person: Peter Zelazowski, PhD, Scientific Review Officer, Division of... Executive Boulevard, Rm. 5B01, Bethesda, MD 20892-7510, 301-435- 6902, peter[email protected] . (Catalogue...

75 FR 39698 - Eunice Kennedy Shriver National Institute of Child Health and Human Development; Notice of Closed...

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2010-07-12

..., (Telephone Conference Call). Contact Person: Peter Zelazowski, PhD, Scientific Review Officer, Division of... Executive Boulevard, Room 5B01, Bethesda, MD 20892-7510, 301-435- 6902, peter[email protected] . This...

78 FR 38337 - Change in Bank Control Notices; Acquisitions of Shares of a Bank or Bank Holding Company

Federal Register 2010, 2011, 2012, 2013, 2014

2013-06-26

...) 90 Hennepin Avenue, Minneapolis, Minnesota 55480-0291: 1. Peter F. Lindholm, Long Lake, Minnesota, individually and as trustee of Peter F. Lindholm 2012 Irrevocable trust; to retain voting shares of Maple Banc...

75 FR 81644 - Meeting of the Judicial Conference Advisory Committee on Rules of Appellate Procedure

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

... Mason Street, San Francisco, CA. FOR FURTHER INFORMATION CONTACT: Peter G. McCabe, Secretary, Rules... (202) 502-1820. Dated: December 21, 2010. Peter G. McCabe, Rules Committee Support Office. [FR Doc...

75 FR 81644 - Meeting of the Judicial Conference Committee on Rules of Practice and Procedure

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

..., Courtroom 5, 95 Seventh Street, San Francisco, CA. FOR FURTHER INFORMATION CONTACT: Peter G. McCabe... 20544, telephone (202) 502-1820. Dated: December 21, 2010. Peter G. McCabe, Rules Committee Support...

78 FR 10186 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

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2013-02-13

..., MD 20814. Contact Person: Peter Zelazowski, Ph.D., Scientific Review Officer, Division of Scientific... Executive Blvd., Room 5B01, Bethesda, MD 20892, 301-435-6902, peter[email protected] . (Catalogue of...

76 FR 17928 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-03-31

... Conference). Contact Person: Peter Zelazowski, PhD, Scientific Review Officer, Division of Scientific Review...., Room 5B01, Bethesda, MD 20892, 301-435-6902, peter[email protected] . (Catalogue of Federal Domestic...

76 FR 32979 - Eunice Kennedy Shriver National Institute of Child Health and Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-06-07

... 20814. Contact Person: Peter Zelazowski, PhD, Scientific Review Officer, Division of Scientific Review...., Room 5B01, Bethesda, MD 20892, 301-435-6902, peter[email protected] . (Catalogue of Federal Domestic...

75 FR 81645 - Meeting of the Judicial Conference Advisory Committee on Rules of Criminal Procedure

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

..., Pioneer Courthouse, 700 SW. Sixth Avenue, Portland, OR 97204. FOR FURTHER INFORMATION CONTACT: Peter G..., Washington, DC 20544, telephone (202) 502-1820. Dated: December 21, 2010. Peter G. McCabe, Rules Committee...

75 FR 65496 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2010-10-25

... Person: Peter Zelazowski, PhD, Scientific Review Officer, Division of Scientific Review, Eunice Kennedy... 5B01, Bethesda, MD 20892-7510, 301-435- 6902, peter[email protected] . (Catalogue of Federal Domestic...

76 FR 61720 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-10-05

..., MD 20814. Contact Person: Peter Zelazowski, PhD, Scientific Review Officer, Division of Scientific... Executive Blvd., Room 5B01, Bethesda, MD 20892, 301-435-6902, peter[email protected] . (Catalogue of...

42 CFR 406.34 - Determination of months to be counted for premium increase: Reenrollment.

Code of Federal Regulations, 2010 CFR

2010-10-01

.... Peter M enrolled during his initial enrollment period, terminated his first coverage period in August... through March, 1980) were not enough to require any increase in the premium. Peter terminated his second...

77 FR 6570 - Eunice Kennedy Shriver National Institute of Child Health and Human Development; Notice of Closed...

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2012-02-08

..., MD 20852. Contact Person: Peter Zelazowski, Ph.D., Scientific Review Officer, Division of Scientific... Executive Blvd., Room 5B01, Bethesda, MD 20892, (301) 435-6902, peter[email protected] . (Catalogue of...

76 FR 5593 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Federal Register 2010, 2011, 2012, 2013, 2014

2011-02-01

... Conference Call). Contact Person: Peter Zelazowski, PhD, Scientific Review Officer, Division Of Scientific... Executive Blvd., Room 5B01, Rockville, MD, 301-435-6902, peter[email protected] . (Catalogue of Federal...

A world revision of the bee fly tribe Usiini (Diptera, Bombyliidae) Part 2: Usia sensu stricto.

PubMed

Gibbs, David

2014-05-22

This is the second part of a world revision of the genera Usia Latreille and Parageron Paramonov, of the tribe Usiini Becker, and covers the pale-haired species, the Usia sensu stricto group. Usia sensu stricto as defined here contains 24 species of which 16 species fall into two monophyletic groups, the U. lata group with 10 species and the U. florea group with six species. Eight species cannot be placed in either of these two groups, four of them form two pairs of sibling species while the remaining four species have no clear affinities. Of the 25 formerly available names that belong in Usia sensu stricto, U. putilla Becker stat. rev., previously synonymised under U. angustifrons, is reinstated as a full species. U. sicula Egger syn. nov., is synonymised under U. manca Loew, U. anus Becker syn. nov., is synonymised under U. vestita Macquart and U. claripennis Macquart syn. nov., is synonymised under U. atrata (Fabricius). Usia vicina Macquart, formerly placed as a synonym of U. atrata, is shown to be a junior synonym of U. aenea Rossi. Five new species are described, U. anatoliensis sp. nov., U. annetteae sp. nov., U. greatheadi sp. nov., U. maghrebensis sp. nov. and U. cornigera sp. nov. Both the male and female genitalia are illustrated in detail for 21 species, female only in the cases of U. calva Loew and U. notata Loew and male only for U. incognita Paramonov.

Enabling Design

DTIC Science & Technology

2009-05-21

Figure 1. Methodology in Hierarchical Context. 2 Peter Checkland , Systems Thinking, System...Joint Forces Command, 2008. Checkland , Peter. Systems Thinking, System Practice. Chichester: John Wiley & Sons, 1981. FM 6-0 Mission Command: Command

Uterine sarcoma

MedlinePlus

... Churchill Livingstone; 2014:chap 88. Crum CP, Laury AR, Hirsch MS, Quick CM, Peters WA. Undifferentiated uterine sarcoma. In: Crum CP, Quick CM, Laury AR, Peters WA, Hirsch MS, eds. Gynecologic and Obstetric ...

27 CFR 9.107 - Lodi.

Code of Federal Regulations, 2010 CFR

2010-04-01

... (Photorevised 1987); (3) Peters, CA 1952 (Photorevised 1968); (4) Stockton East, Calif. 1968 (Photorevised 1987... through the Peters, CA map, and ending on the Stockton East, Calif. map); (3) Then proceed north along...

75 FR 81644 - Meeting of the Judicial Conference Advisory Committee on Rules of Civil Procedure

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

... East Dean Keeton Street, Austin, TX 78705. FOR FURTHER INFORMATION CONTACT: Peter G. McCabe, Secretary..., Washington, DC 20544, telephone (202) 502-1820. Dated: December 21, 2010. Peter G. McCabe, Rules Committee...

75 FR 81644 - Meeting of the Judicial Conference Advisory Committee on Rules of Bankruptcy Procedure

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

..., 950 Mason Street, San Francisco, CA. FOR FURTHER INFORMATION CONTACT: Peter G. McCabe, Secretary..., telephone (202) 502-1820. Dated: December 21, 2010. Peter G. McCabe, Rules Committee Support Office. [FR Doc...

75 FR 81644 - Hearing of The Judicial Conference Committee on Criminal Rules

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

... San Francisco, CA FOR FURTHER INFORMATION CONTACT: Peter G. McCabe, Secretary, Rules Committee Support.... Dated: December 21, 2010. Peter G. McCabe, Rules Committee Support Office. [FR Doc. 2010-32415 Filed 12...

75 FR 81645 - Meeting of the Judicial Conference Advisory Committee on Rules of Evidence

Federal Register 2010, 2011, 2012, 2013, 2014

2010-12-28

..., Philadelphia, PA 19104. FOR FURTHER INFORMATION CONTACT: Peter G. McCabe, Secretary, Rules Committee Support.... Dated: December 21, 2010. Peter G. McCabe, Rules Committee Support Office. [FR Doc. 2010-32433 Filed 12...

HOMOGENEOUS CATALYSTS FOR THE PARTIAL-OXYGENATION OF SATURATED HYDROCARBONS WITH HYDROGEN PERIOXIDE

EPA Science Inventory

A Methodology for the Evaluation of Process Sustainability
Michael Gonzalez*, Raymond Smith and Thomas Becker

United States Environmental Protection Agency; Office of Research and Development; Sustainable Technologies Division; 26 West Martin Luther King Drive; Cincinna...

The impact of group counseling on depression, post-traumatic stress and function outcomes: a prospective comparison study in the Peter C. Alderman trauma clinics in northern Uganda.

PubMed

Nakimuli-Mpungu, Etheldreda; Okello, James; Kinyanda, Eugene; Alderman, Stephen; Nakku, Juliet; Alderman, Jeffrey S; Pavia, Alison; Adaku, Alex; Allden, Kathleen; Musisi, Seggane

2013-10-01

The effectiveness of group interventions for adults with mental distress in post-conflict settings is less clear in sub-Saharan Africa. To assess the impact of group counseling intervention on depression, post-traumatic stress and function outcomes among adults attending the Peter C. Alderman Foundation (PCAF) trauma clinics in northern Uganda. 631 War affected adults were enrolled into PCAF trauma clinics. Using a quasi-experimental design, assessments were conducted at baseline, at 3 and 6 months following initiation of care. Multivariate longitudinal regression models were used to determine change in depression, post-traumatic stress and function scores over time among group counseling participants and non-participants. In comparison to non-participants, participants had faster reduction in depression scores during the 6-month follow-up period [β=-1.84, 95%CI (-3.38 to -0.30), p=0.019] and faster reduction in post-traumatic stress scores during the 3-month follow-up period [β=-2.14, 95%CI (-4.21 to -0.10), p=0.042]. At 3-month follow up, participants who attended two or more sessions had faster increase in function scores [β=3.51, 95%CI (0.61-6.40), p=0.018] than participants who attended only one session. Selection bias due to the use of non-random samples. Substantial attrition rates and small sample sizes may have resulted in insufficient statistical power to determine meaningful differences. The group counseling intervention offered in the PCAF clinics may have considerable mental health benefits over time. There is need for more research to structure, standardize and test the efficacy of this intervention using a randomized controlled trial. © 2013 Elsevier B.V. All rights reserved.

The dynamic relationships between union dissolution and women's employment: a life-history analysis of 16 countries.

PubMed

van Damme, Maike; Kalmijn, Matthijs

2014-11-01

The specialization theory from Gary Becker is often used to explain the effect of women's work on the risk of divorce. The main argument is that women with little work experience have higher economic costs to exit marriage. Using the Fertility and Family Surveys, we test for 16 countries to what extent women's employment increases the risk of separation. We also more directly examine the role of economic exit costs in separation by investigating the effect of separated women's work history during the union on women's post-separation employment. The results imply that Becker was right to some extent, especially in contexts with little female employment support. However, in settings where women's employment opportunities are more ample, sociological or psychological theories have probably more explanatory power to explain the causes and consequences of union dissolution. Copyright © 2014 Elsevier Inc. All rights reserved.

ARC-2007-ACD07-0152-030

NASA Image and Video Library

2007-08-12

Perseid Meteor flight on Google's Gulfstream Aircraft. P.I. Peter Jenniskens, SETI Group Peter Jenniskens, SETI - briefing w/L-R; J Nott, P Jenniskens, M Koop, D Holman and two Apex Aviation Corp reps.

78 FR 3034 - Sunshine Act Meeting; Agenda

Federal Register 2010, 2011, 2012, 2013, 2014

2013-01-15

... by email at [email protected] . FOR MEDIA INFORMATION CONTACT: Peter Knudson (202) 314-6219 or by email at peter[email protected] . Dated: January 11, 2013. Candi R. Bing, Federal Register Liaison Officer...