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1. Faster Sensitivity Loss around Dense Scotomas than for Overall Macular Sensitivity in Stargardt Disease: ProgStar Report No. 14

Author

Etienne M. Schönbach, Rupert W. Strauss, Mohamed A. Ibrahim, Jessica L. Janes, David G. Birch, Artur V. Cideciyan, Janet S. Sunness, Beatriz Muñoz, Michael S. Ip, SriniVas R. Sadda, Hendrik P.N. Scholl, Yulia Wolfson, Millena Bittencourt, Syed Mahmood Shah, Mohamed Ahmed, Etienne Schönbach, Kaoru Fujinami, Elias Traboulsi, Justis Ehlers, Meghan Marino, Susan Crowe, Rachael Briggs, Angela Borer, Anne Pinter, Tami Fecko, Nikki Burgnoni, Carol Applegate, Leslie Russell, Michel Michaelides, Simona Degli Esposti, Anthony Moore, Andrew Webster, Sophie Connor, Jade Barnfield, Zaid Salchi, Clara Alfageme, Victoria McCudden, Maria Pefkianaki, Jonathan Aboshiha, Gerald Liew, Graham Holder, Anthony Robson, Alexa King, Daniela Ivanova Cajas Narvaez, Katy Barnard, Catherine Grigg, Hannah Dunbar, Yetunde Obadeyi, Karine Girard-Claudon, Hilary Swann, Avani Rughani, Charles Amoah, Dominic Carrington, Kanom Bibi, Emerson Ting, Mohamed Nafaz Illiyas, Hamida Begum, Andrew Carter, Anne Georgiou, Selma Lewism, Saddaf Shaheen, Harpreet Shinmar, Linda Burton, Paul Bernstein, Kimberley Wegner, Briana Lauren Sawyer, Bonnie Carlstrom, Kellian Farnsworth, Cyrie Fry, Melissa Chandler, Glen Jenkins, Donnel Creel, David Birch, Yi-Zhong Wang, Luis Rodriguez, Kirsten Locke, Martin Klein, Paulina Mejia, Samuel G. Jacobson, Sharon B. Schwartz, Rodrigo Matsui, Michaela Gruzensky, Jason Charng, Alejandro J. Roman, Eberhart Zrenner, Fadi Nasser, Gesa Astrid Hahn, Barbara Wilhelm, Tobias Peters, Benjamin Beier, Tilman Koenig, Susanne Kramer, José-Alain Sahel, Saddek Mohand-Said, Isabelle Audo, Caroline Laurent-Coriat, Ieva Sliesoraityte, Christina Zeitz, Fiona Boyard, Minh Ha Tran, Mathias Chapon, Céline Chaumette, Juliette Amaudruz, Victoria Ganem, Serge Sancho, Aurore Girmens, Robert Wojciechowski, Shazia Khan, David G. Emmert, Dennis Cain, Mark Herring, Jennifer Bassinger, Lisa Liberto, Sheila West, Ann-Margret Ervin, Beatriz Munoz, Xiangrong Kong, Kurt Dreger, Jennifer Jones, Srinivas Sadda, Anamika Jha, Alex Ho, Brendan Kramer, Ngoc Lam, Rita Tawdros, Yong Dong Zhou, Johana Carmona, Akihito Uji, Amirhossein Hariri, Amy Lock, Anthony Elshafei, Anushika Ganegoda, Christine Petrossian, Dennis Jenkins, Edward Strnad, Elmira Baghdasaryan, Eric Ito, Feliz Samson, Gloria Blanquel, Handan Akil, Jhanisus Melendez, Jianqin Lei, Jianyan Huang, Jonathan Chau, Khalil G. Falavarjani, Kristina Espino, Manfred Li, Maria Mendoza, Muneeswar Gupta Nittala, Netali Roded, Nizar Saleh, Ping Huang, Sean Pitetta, Siva Balasubramanian, Sophie Leahy, Sowmya J. Srinivas, Swetha B. Velaga, Teresa Margaryan, Tudor Tepelus, Tyler Brown, Wenying Fan, Yamileth Murillo, Yue Shi, Katherine Aguilar, Cynthia Chan, Lisa Santos, Brian Seo, Christopher Sison, Silvia Perez, Stephanie Chao, Kelly Miyasato, Julia Higgins, Zoila Luna, Anita Menchaca, Norma Gonzalez, Vicky Robledo, Karen Carig, Kirstie Baker, David Ellenbogen, Daniel Bluemel, Theo Sanford, Daisy Linares, Mei Tran, Lorane Nava, Michelle Oberoi, Mark Romero, Vivian Chiguil, Grantley Bynum-Bain, Monica Kim, Carolina Mendiguren, Xiwen Huang, Monika Smith, and Natalie Sarreal

Subjects
Adult, Male, medicine.medical_specialty, Visual acuity, Adolescent, genetic structures, Visual Acuity, ABCA4, Sensitivity and Specificity, Retina, Young Adult, Ophthalmology, Humans, Stargardt Disease, Medicine, Prospective Studies, Fluorescein Angiography, Scotoma, Prospective cohort study, medicine.diagnostic_test, biology, business.industry, Blind spot, Middle Aged, Fluorescein angiography, medicine.disease, eye diseases, Clinical trial, Stargardt disease, biology.protein, Visual Field Tests, ATP-Binding Cassette Transporters, Female, Visual Fields, medicine.symptom, business, Microperimetry, Tomography, Optical Coherence
Abstract

Mean sensitivity (MS) derived from a standard test grid using microperimetry is a sensitive outcome measure in clinical trials investigating new treatments for degenerative retinal diseases. This study hypothesizes that the functional decline is faster at the edge of the dense scotoma (eMS) than by using the overall MS.Multicenter, international, prospective cohort study: ProgStar Study.Stargardt disease type 1 patients (carrying at least 1 mutation in the ABCA4 gene) were followed over 12 months using microperimetry with a Humphrey 10-2 test grid. Customized software was developed to automatically define and selectively follow the test points directly adjacent to the dense scotoma points and to calculate their mean sensitivity (eMS).Among 361 eyes (185 patients), the mean age was 32.9 ± 15.1 years old. At baseline, MS was 10.4 ± 5.2 dB (n = 361), and the eMS was 9.3 ± 3.3 dB (n = 335). The yearly progression rate of MS (1.5 ± 2.1 dB/year) was significantly lower (β = -1.33; P.001) than that for eMS (2.9 ± 2.9 dB/year). There were no differences between progression rates using automated grading and those using manual grading (β = .09; P = .461).In Stargardt disease type 1, macular sensitivity declines significantly faster at the edge of the dense scotoma than in the overall test grid. An automated, time-efficient approach for extracting and grading eMS is possible and appears valid. Thus, eMS offers a valuable tool and sensitive outcome parameter with which to follow Stargardt patients in clinical trials, allowing clinical trial designs with shorter duration and/or smaller cohorts.

Published
2020

2. Longitudinal Changes of Fixation Location and Stability Within 12 Months in Stargardt Disease: ProgStar Report No. 12

Author

Etienne M. Schönbach, Rupert W. Strauss, Xiangrong Kong, Beatriz Muñoz, Mohamed A. Ibrahim, Janet S. Sunness, David G. Birch, Gesa-Astrid Hahn, Fadi Nasser, Eberhart Zrenner, SriniVas R. Sadda, Sheila K. West, Hendrik P.N. Scholl, Yulia Wolfson, Millena Bittencourt, Syed Mahmood Shah, Mohamed Ahmed, Etienne Schönbach, Kaoru Fujinami, Elias Traboulsi, Justis Ehlers, Meghan Marino, Susan Crowe, Rachael Briggs, Angela Borer, Anne Pinter, Tami Fecko, Nikki Burgnoni, Carol Applegate, Leslie Russell, Michel Michaelides, Simona Degli Esposti, Anthony Moore, Andrew Webster, Sophie Connor, Jade Barnfield, Zaid Salchi, Clara Alfageme, Victoria McCudden, Maria Pefkianaki, Jonathan Aboshiha, Gerald Liew, Graham Holder, Anthony Robson, Alexa King, Daniela Ivanova Cajas Narvaez, Katy Barnard, Catherine Grigg, Hannah Dunbar, Yetunde Obadeyi, Karine Girard-Claudon, Hilary Swann, Avani Rughani, Charles Amoah, Dominic Carrington, Kanom Bibi, Emerson Ting Co, Mohamed Nafaz Illiyas, Hamida Begum, Andrew Carter, Anne Georgiou, Selma Lewism, Saddaf Shaheen, Harpreet Shinmar, Linda Burton, Paul Bernstein, Kimberley Wegner, Briana Lauren Sawyer, Bonnie Carlstrom, Kellian Farnsworth, Cyrie Fry, Melissa Chandler, Glen Jenkins, Donnel Creel, David Birch, Yi-Zhong Wang, Luis Rodriguez, Kirsten Locke, Martin Klein, Paulina Mejia, Artur V. Cideciyan, Samuel G. Jacobson, Sharon B. Schwartz, Rodrigo Matsui, Michaela Gruzensky, Jason Charng, Alejandro J. Roman, Gesa Astrid Hahn, Barbara Wilhelm, Tobias Peters, Benjamin Beier, Tilman Koenig, Susanne Kramer, José-Alain Sahel, Saddek Mohand-Said, Isabelle Audo, Caroline Laurent-Coriat, Ieva Sliesoraityte, Christina Zeitz, Fiona Boyard, Minh Ha Tran, Mathias Chapon, Céline Chaumette, Juliette Amaudruz, Victoria Ganem, Serge Sancho, Aurore Girmens, Robert Wojciechowski, Shazia Khan, David G. Emmert, Dennis Cain, Mark Herring, Jennifer Bassinger, Lisa Liberto, Sheila West, Ann-Margret Ervin, Beatriz Munoz, Kurt Dreger, Jennifer Jones, Srinivas Sadda, Michael S. Ip, Anamika Jha, Alex Ho, Brendan Kramer, Ngoc Lam, Rita Tawdros, Yong Dong Zhou, Johana Carmona, Akihito Uji, Amirhossein Hariri, Amy Lock, Anthony Elshafei, Anushika Ganegoda, Christine Petrossian, Dennis Jenkins, Edward Strnad, Elmira Baghdasaryan, Eric Ito, Feliz Samson, Gloria Blanquel, Handan Akil, Jhanisus Melendez, Jianqin Lei, Jianyan Huang, Jonathan Chau, Khalil G. Falavarjani, Kristina Espino, Manfred Li, Maria Mendoza, Muneeswar Gupta Nittala, Netali Roded, Nizar Saleh, Ping Huang, Sean Pitetta, Siva Balasubramanian, Sophie Leahy, Sowmya J. Srinivas, Swetha B. Velaga, Teresa Margaryan, Tudor Tepelus, Tyler Brown, Wenying Fan, Yamileth Murillo, Yue Shi, Katherine Aguilar, Cynthia Chan, Lisa Santos, Brian Seo, Christopher Sison, Silvia Perez, Stephanie Chao, Kelly Miyasato, Julia Higgins, Zoila Luna, Anita Menchaca, Norma Gonzalez, Vicky Robledo, Karen Carig, Kirstie Baker, David Ellenbogen, Daniel Bluemel, Theo Sanford, Daisy Linares, Mei Tran, Lorane Nava, Michelle Oberoi, Mark Romero, Vivian Chiguil, Grantley Bynum-Bain, Monica Kim, Carolina Mendiguren, Xiwen Huang, Monika Smith, and Natalie Sarreal

Subjects
Adult, Male, Fixation stability, medicine.medical_specialty, Adolescent, Visual Acuity, Fixation, Ocular, Retina, Article, Standard deviation, Macular Degeneration, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Ophthalmology, medicine, Humans, Stargardt Disease, Prospective Studies, Young adult, Child, Prospective cohort study, Aged, business.industry, Middle Aged, medicine.disease, Confidence interval, Stargardt disease, Fixation (visual), 030221 ophthalmology & optometry, Visual Field Tests, Female, Visual Fields, business, 030217 neurology & neurosurgery, Natural history study, Follow-Up Studies
Abstract

Purpose To investigate the natural history of Stargardt disease (STGD1) using fixation location and fixation stability. Design Multicenter, international, prospective cohort study. Methods Fixation testing was performed using the Nidek MP-1 microperimeter as part of the prospective, multicenter, natural history study on the Prog ression of Star gardt disease (ProgStar). A total of 238 patients with ABCA4-related STGD1 were enrolled at baseline (bilateral enrollment in 86.6%) and underwent repeat testing at months 6 and 12. Results Outcome measures included the distance of the preferred retinal locus from the fovea (PRL) and the bivariate contour ellipse area (BCEA). After 12 months of follow-up, the change in the eccentricity of the PRL from the anatomic fovea was −0.0014 degrees (95% confidence interval [CI], −0.27 degrees, 0.27 degrees; P = .99). The deterioration in the stability of fixation as expressed by a larger BCEA encompassing 1 standard deviation of all fixation points was 1.21 degrees squared (deg2) (95% CI, −1.23 deg2, 3.65 deg2; P = .33). Eyes with increases and decreases in PRL eccentricity and/or BCEA values were observed. Conclusions Our observations point to the complexity of fixation parameters. The association of increasingly eccentric and unstable fixation with longer disease duration that is typically found in cross-sectional studies may be countered within individual patients by poorly understood processes like neuronal adaptation. Nevertheless, fixation parameters may serve as useful secondary outcome parameters in selected cases and for counseling patients to explain changes to their visual functionality.

Published
2018

3. Interrogation of Patient Smartphone Activity Tracker to Assist Arrhythmia Management

Author

Alfred Sacchetti, Vivek Sailam, Monika Smith, Joshua Rudner, and Carol McDougall

Subjects
Adult, Male, Time Factors, 020205 medical informatics, MEDLINE, 02 engineering and technology, Newly diagnosed, Fitness Trackers, Smartphone application, 03 medical and health sciences, 0302 clinical medicine, Atrial Fibrillation, 0202 electrical engineering, electronic engineering, information engineering, medicine, Humans, 030212 general & internal medicine, Interrogation, business.industry, Activity tracker, Atrial fibrillation, Emergency department, medicine.disease, Mobile Applications, Emergency Medicine, Medical emergency, Smartphone, business, Emergency Service, Hospital
Abstract

A 42-year-old man presented to the emergency department (ED) with newly diagnosed atrial fibrillation of unknown duration. Interrogation of the patient's wrist-worn activity tracker and smartphone application identified the onset of the arrhythmia as within the previous 3 hours, permitting electrocardioversion and discharge of the patient from the ED.

Published
2016

4. Reformulation and the Learning of French Pronominal Verbs in a Canadian French Immersion Context

Author

Merrill Swain, Monika Smith, and Sharon Lapkin

Subjects
Trace (semiology), Linguistics and Language, AP French Language, Context (language use), Second language instruction, Psychology, French immersion, Language and Linguistics, Linguistics, Task (project management)
Abstract

In this article, we examine a subset of collaborative dialogues that occurred in a multistage task where 8 Canadian grade 7 French immersion students worked together in pairs. Each pair wrote a story, noticed differences between their text and a reformulator's revision of that text, and reflected on their noticing. We analyze the transcripts of their talk in these 3 task sessions, identifying and describing the language-related episodes. We trace the development of the target language (French), with specific reference to pronominal verbs, from the learners' first collaborative draft to their posttests. The study explores task differences and differences between lower- and higher-proficiency dyads. The language-related episodes selected for close analysis, along with the posttest data, provide evidence that in most cases, learners have progressed in their correct use of pronominal verbs in French.

Published
2002

9. Business Purpose Design - English Version 2019

Author

Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, Monika Smith, Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, and Monika Smith

Abstract

Business Purpose Design is an essential guide for a human-centric and holistic purpose for businesses.Discontinuity, uncertainty, complexity, and ambiguity are driving forces of our world. Entire markets, industries, departments, and specialist areas interact and correlate with each other - unplanned and open-ended. In such a world, orientation is key to navigate, to distinguish relevant information from irrelevant, to take decisions and lead companies to create a positive future. Together with 32 outstanding personalities, from thought leaders, executives, founders, designers and scientists, Monika looks at the 30 most relevant topics für purpose entrepreneurship. Many examples, trend outlooks, and conceptional images inspire new thoughts and ideas - and reassure existing developments. Furthermore, takeaways for every topic offer a hands-on guide to act right away. With the Business Purpose Design model, organizations of any size can design, build and grow their business towards becoming impact driven. Provides deep insights into 30 most relevant topics, a toolkit and over 90 practical tips to design or and implement purpose within an organization. Co-created by over 32 practitioners from 30 disciplines. Illustrated with a critical eye by one of Europe's most sophisticated graphic-recording duo. Designed for executives, consultants, entrepreneurs, coaches, managers, designers and leaders of all types of organizations. Co-Autoren Amrei Andrasch, Jean-Philipp Almstedt, Victoria Balk, Leonid Berov, Jannis Born, Raquel Dischinger, Tina Dreisicke, Dominik Frisch, Jukka Hilmola, Nimrod Lehavi, Marcus Prosch Long Qu, Mats Richter, Friederike Rohde, Stefan Pfeifer, Romas Stukenberg, Maximilian Wächter, Maximilian Weldert

10. Business Purpose Design - English Version 2019

Author

Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, Monika Smith, Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, and Monika Smith

Abstract

Business Purpose Design is an essential guide for a human-centric and holistic purpose for businesses.Discontinuity, uncertainty, complexity, and ambiguity are driving forces of our world. Entire markets, industries, departments, and specialist areas interact and correlate with each other - unplanned and open-ended. In such a world, orientation is key to navigate, to distinguish relevant information from irrelevant, to take decisions and lead companies to create a positive future. Together with 32 outstanding personalities, from thought leaders, executives, founders, designers and scientists, Monika looks at the 30 most relevant topics für purpose entrepreneurship. Many examples, trend outlooks, and conceptional images inspire new thoughts and ideas - and reassure existing developments. Furthermore, takeaways for every topic offer a hands-on guide to act right away. With the Business Purpose Design model, organizations of any size can design, build and grow their business towards becoming impact driven. Provides deep insights into 30 most relevant topics, a toolkit and over 90 practical tips to design or and implement purpose within an organization. Co-created by over 32 practitioners from 30 disciplines. Illustrated with a critical eye by one of Europe's most sophisticated graphic-recording duo. Designed for executives, consultants, entrepreneurs, coaches, managers, designers and leaders of all types of organizations. Co-Autoren Amrei Andrasch, Jean-Philipp Almstedt, Victoria Balk, Leonid Berov, Jannis Born, Raquel Dischinger, Tina Dreisicke, Dominik Frisch, Jukka Hilmola, Nimrod Lehavi, Marcus Prosch Long Qu, Mats Richter, Friederike Rohde, Stefan Pfeifer, Romas Stukenberg, Maximilian Wächter, Maximilian Weldert

11. Business Purpose Design - English Version 2019

Author

Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, Monika Smith, Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, and Monika Smith

Abstract

Business Purpose Design is an essential guide for a human-centric and holistic purpose for businesses.Discontinuity, uncertainty, complexity, and ambiguity are driving forces of our world. Entire markets, industries, departments, and specialist areas interact and correlate with each other - unplanned and open-ended. In such a world, orientation is key to navigate, to distinguish relevant information from irrelevant, to take decisions and lead companies to create a positive future. Together with 32 outstanding personalities, from thought leaders, executives, founders, designers and scientists, Monika looks at the 30 most relevant topics für purpose entrepreneurship. Many examples, trend outlooks, and conceptional images inspire new thoughts and ideas - and reassure existing developments. Furthermore, takeaways for every topic offer a hands-on guide to act right away. With the Business Purpose Design model, organizations of any size can design, build and grow their business towards becoming impact driven. Provides deep insights into 30 most relevant topics, a toolkit and over 90 practical tips to design or and implement purpose within an organization. Co-created by over 32 practitioners from 30 disciplines. Illustrated with a critical eye by one of Europe's most sophisticated graphic-recording duo. Designed for executives, consultants, entrepreneurs, coaches, managers, designers and leaders of all types of organizations. Co-Autoren Amrei Andrasch, Jean-Philipp Almstedt, Victoria Balk, Leonid Berov, Jannis Born, Raquel Dischinger, Tina Dreisicke, Dominik Frisch, Jukka Hilmola, Nimrod Lehavi, Marcus Prosch Long Qu, Mats Richter, Friederike Rohde, Stefan Pfeifer, Romas Stukenberg, Maximilian Wächter, Maximilian Weldert

12. Business Purpose Design - English Version 2019

Author

Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, Monika Smith, Monika Smith, Shermin Voshmgir Maren Beverung, Katharina Zwielich Scot Carlson , Don Spampinato Steffan Heuer Curt Simon Harlinghausen Daniel Heltzel Philip Siefer, Friedericke Rode Martin Sinner Christian Solmecke, and Monika Smith

Abstract

Business Purpose Design is an essential guide for a human-centric and holistic purpose for businesses.Discontinuity, uncertainty, complexity, and ambiguity are driving forces of our world. Entire markets, industries, departments, and specialist areas interact and correlate with each other - unplanned and open-ended. In such a world, orientation is key to navigate, to distinguish relevant information from irrelevant, to take decisions and lead companies to create a positive future. Together with 32 outstanding personalities, from thought leaders, executives, founders, designers and scientists, Monika looks at the 30 most relevant topics für purpose entrepreneurship. Many examples, trend outlooks, and conceptional images inspire new thoughts and ideas - and reassure existing developments. Furthermore, takeaways for every topic offer a hands-on guide to act right away. With the Business Purpose Design model, organizations of any size can design, build and grow their business towards becoming impact driven. Provides deep insights into 30 most relevant topics, a toolkit and over 90 practical tips to design or and implement purpose within an organization. Co-created by over 32 practitioners from 30 disciplines. Illustrated with a critical eye by one of Europe's most sophisticated graphic-recording duo. Designed for executives, consultants, entrepreneurs, coaches, managers, designers and leaders of all types of organizations. Co-Autoren Amrei Andrasch, Jean-Philipp Almstedt, Victoria Balk, Leonid Berov, Jannis Born, Raquel Dischinger, Tina Dreisicke, Dominik Frisch, Jukka Hilmola, Nimrod Lehavi, Marcus Prosch Long Qu, Mats Richter, Friederike Rohde, Stefan Pfeifer, Romas Stukenberg, Maximilian Wächter, Maximilian Weldert

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