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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. Women's basketball deafeat Kent State

Author

Reporter, Brendan Kramer | Sports

Subjects
News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcon women's basketball team picked up their second consecutive conference win on Wednesday night at the Stroh Center. They were able to hang [...]

Published
2018

4. Women's Basketball defeats Akron on late three-pointer

Author

Reporter, Brendan Kramer | Sports

Subjects
News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcon women's basketball team bounced back from not only a five game losing streak, but also a seven point deficit with only two [...]

Published
2018

5. Women's basketball falls to Ball State

Author

Reporter, Brendan Kramer | Sports

Subjects
News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcon women's basketball team dropped their fourth consecutive game and sixth in total on the season to the Ball State Cardinals Saturday afternoon. [...]

Published
2018

6. Women's Basketball suffers first road loss of season

Author

Reporter, Brendan Kramer | Sports

Subjects
College basketball, News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcon women's basketball team fell for their second time of the season by a score of 76-44 by the hands of the South [...]

Published
2018

7. Visual Acuity Loss and Associated Risk Factors in the Retrospective Progression of Stargardt Disease Study (ProgStar Report No. 2)

Author

Xiangrong Kong, Rupert W. Strauss, Michel Michaelides, Artur V. Cideciyan, José-Alain Sahel, Beatriz Muñoz, Sheila West, Hendrik P.N. Scholl, Yulia Wolfson, Millena Bittencourt, Syed Mahmood Shah, Mohammed Ahmed, Etienne Schonbach, Kaoru Fujinami, Elias Traboulsi, Justis Ehlers, Meghan Marino, Susan Crowe, Rachael Briggs, Angela Borer, Anne Pinter, Tami Fecko, Nikki Brugnoni, Janet S. Sunness, Carol Applegate, Leslie Russell, Anthony Moore, Andrew Webster, Sophie Connor, 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, Andrew Carter, Anne Georgiou, Selma Lewis, 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, Alejandro J. Roman, Eberhart Zrenner, Fadi Nasser, Gesa Astrid Hahn, Barbara Wilhelm, Tobias Peters, Benjamin Beier, Tilman Koenig, Susanne Kramer, 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, Ann-Margret Ervin, Beatriz Munoz, Kurt Dreger, Jennifer Jones, Srinivas Sadda, Anamika Jha, Alex Ho, Brendan Kramer, Amirhossein Hariri, Gloria Rebecca Blanquel, Ngoc Lam, Sean Pitetta, Yue Shi, Rita Tawdros, Christine Petrossian, Dennis Jenkins, Muneeswar Gupta, Yong Dong Zhou, 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, James Russell, Daniel Bluemel, Alex Moreno, Royal Pham, Theo Sanford, Daisy Linares, and Mei Tran

Subjects
0301 basic medicine, Adult, Male, Pediatrics, medicine.medical_specialty, Visual acuity, Adolescent, Cross-sectional study, Vision Disorders, Visual Acuity, 03 medical and health sciences, Macular Degeneration, Young Adult, 0302 clinical medicine, Risk Factors, medicine, Humans, Stargardt Disease, Age of Onset, Prospective cohort study, Child, Generalized estimating equation, Aged, Retrospective Studies, business.industry, Retrospective cohort study, Middle Aged, medicine.disease, United States, Stargardt disease, Europe, Ophthalmology, 030104 developmental biology, Cross-Sectional Studies, 030221 ophthalmology & optometry, Physical therapy, Disease Progression, Linear Models, Age of onset, medicine.symptom, business, Cohort study
Abstract

To examine the association between characteristics of Stargardt disease and visual acuity (VA), to estimate the longitudinal rate of VA loss, and to identify risk factors for VA loss.Retrospective, multicenter cohort study.A total of 176 patients (332 eyes) with molecularly and clinically confirmed Stargardt disease enrolled from the United States and Europe.Standardized data report forms were used to collect retrospective data on participants' characteristics and best-corrected or presenting VA from medical charts. Linear models with generalized estimating equations were used to estimate the cross-sectional associations, and linear mixed effects models were used to estimate the longitudinal VA loss.Yearly change in VA.The median duration of observation was 3.6 years. At baseline, older age of symptom onset was associated with better VA, and a longer duration of symptoms was associated with worse VA. Longitudinal analysis estimated an average of 0.3 lines loss (P0.0001) per year overall, but the rate varied according to baseline VA: (1) eyes with baseline VA ≥20/25 (N = 53) declined at a rate of approximately 1.0 line per year; (2) eyes with VA between 20/25 and 20/70 (N = 65) declined at a rate of approximately 0.9 lines per year; (3) eyes with VA between 20/70 and 20/200 (N = 163) declined at a rate of 0.2 lines per year; and (4) eyes with VA worse than 20/200 (n = 49) improved at a rate of 0.5 lines per year. Older age of onset was associated with slower VA loss: Patients with onset age30 years showed 0.4 lines slower change of VA per year (P = 0.01) compared with patients with onset age ≤14 years.Given the overall slow rate of VA loss, VA is unlikely to be a sensitive outcome measure for treatment trials of Stargardt disease. However, given the faster decline in younger patients and those with no or mild visual impairment, VA may be a potential outcome measure for trials targeting such subgroups of patients. These observations will need to be assessed in a prospective study bearing in mind the inherent limitations of retrospective datasets.

Published
2016

8. Identification of anterior chamber angle parameters with a portable SD-OCT device compared to a non-portable SD-OCT

Author

Kenneth M. Marion, Handan Akil, Anna Dastiridou, Brendan Kramer, Vikas Chopra, Brian A. Francis, and Dennis Jenkins

Subjects
Adult, Male, Intraclass correlation, Anterior Chamber, Glaucoma, Iris, 01 natural sciences, Anterior chamber angle, 010309 optics, 03 medical and health sciences, 0302 clinical medicine, Optics, Optical coherence tomography, 0103 physical sciences, medicine, Humans, Iridocorneal angle, Observer Variation, Reproducibility, medicine.diagnostic_test, business.industry, Schwalbe's line, Reproducibility of Results, medicine.disease, Ophthalmology, 030221 ophthalmology & optometry, Cirrus, Female, business, Tomography, Optical Coherence, Biomedical engineering
Abstract

The aim of this study is to compare a portable spectral domain optical coherence tomography (SD-OCT) device with a non-portable SD-OCT for the identification of anterior chamber angle parameters based on location of Schwalbe's line (SL) and to measure their reproducibility. 99 eyes from 46 normal, healthy participants underwent imaging of the inferior iridocorneal angle with the iVue and Cirrus SD-OCT under well-controlled low-light conditions. SL-angle opening distance (SL-AOD) and SL-trabecular iris space area (SL-TISA) were measured by masked, certified graders at the Doheny Image Reading Center using customized Image J grading software. Inter- and intrainstrument, as well as inter- and intraobserver reproducibility of SL-AOD and SL-TISA measurements were evaluated with intraclass correlation coefficients (ICCs) and Bland-Altman plots with limits of agreement. The mean SL-AOD was 0.814 ± 0.315 mm with the iVue and 0.797 ± 0.294 mm with the Cirrus. The mean SL-TISA was 0.247 ± 0.112 mm2 with iVue and 0.259 ± 0.113 mm2 with Cirrus. Interinstrument correlation coefficients (r) were 0.93 (P 0.95), intragrader (ICCs > 0.93), and intergrader (ICCs > 0.96) was excellent. Excellent agreement between the two devices was also documented with Bland-Altman analysis. Both instruments provide consistent and reproducible measurements of anterior chamber angle metrics.

Published
2016

9. Women's Basketball suffers first road loss of season

Author

Reporter, Brendan Kramer | Sports

Subjects
College basketball, News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcon women's basketball team fell for their second time of the season by a score of 76-44 by the hands of the South [...]

Published
2017

10. Women's Basketball takes both games of Thanksgiving Tournament

Author

Reporter, Brendan Kramer | Sports

Subjects
College basketball, News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcons women's basketball team competed in the FAU Thanksgiving Tournament on Friday and Saturday afternoon, successfully sweeping the competition and picking up wins [...]

Published
2017

11. Women's basketball faces first loss of the early season

Author

Reporter, Brendan Kramer | Sports

Subjects
College basketball, News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter For consecutive games, the Falcon women's basketball team surrendered a first-half lead. This one just so happened to cost them their first loss of [...]

Published
2017

12. Women's basketball defeats Eastern Kentucky in home opener

Author

Reporter, Brendan Kramer | Sports

Subjects
Basketball players, News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcons women's basketball team opened up the 2017-2018 regular season with a bang by acquiring a victory over the Eastern Kentucky Colonels that [...]

Published
2017

13. Women's basketball comes out on top in close game in Detroit

Author

Reporter, Brendan Kramer | Sports

Subjects
News, opinion and commentary, Sports and fitness
Abstract

Byline: Brendan Kramer | Sports Reporter The Falcons women's basketball team earned a hard-fought 85-81 road victory over the Detroit Mercy Titans on Monday in the Motor City. The team [...]

Published
2017

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