Explanipedia

P052: Durable fracture rate reduction in patients with OI with setrusumab treatment: 14-month data from phase 2 of the Orbit study Open

Heather M. Byers, Gary S. Gottesman, Thomas Carpenter, Danita Velasco, Maegen Wallace , et al. · 2025

Evaluation of Radiolucent Lesions in Cortices of Long Bones in Osteogenesis Imperfecta Patients Open

Carolyn Cook, Lydia Turk, Alicia M. January, Teresa Cappello, Peter A. Smith · 2024

(1)Radiolucent lesions are a characteristic finding of OI and have not been yet described in literature.(2)These lesions may represent a probable failure of ossification and lack of remodeling in the area of the lesion.(3)There is no clear…

6820 Assessing The Efficacy And Safety Of Setrusumab For Osteogenesis Imperfecta: Updated Phase 2 Data From The Phase 2/3 Orbit Study Open

Gary S. Gottesman, T O Carpenter, Melissa Wallace, Peter A. Smith, Erik A. Imel , et al. · 2024

Disclosure: G.S. Gottesman: Advisory Board Member; Self; Ultragenyx, Kyowa Kirin, Inc. Consulting Fee; Self; Ultragenyx. Research Investigator; Self; Alexion Pharmaceuticals, Inc., Ultragenyx. T.O. Carpenter: Advisory Board Member; Self; U…

Psychosocial Outcomes of Pain and Pain Management in Adults with Osteogenesis Imperfecta: A Qualitative Study Open

Whitney S Shepherd, Andrew D. Wiese, Hannah E. Cho, W. Conor Rork, Mougheesa Baig , et al. · 2024

A multicenter study to evaluate pain characteristics in osteogenesis imperfecta Open

Mercedes Rodriguez Celin, Karen M. Kruger, Angela Caudill, Chaya N. Murali, Sandesh C.S. Nagamani , et al. · 2022

The objective was to describe pain characteristics and treatments used in individuals with varying severity of osteogenesis imperfecta (OI) and investigate pain‐associated variables. This work was derived from a multicenter, longitudinal, …

What are the long-term outcomes of lateral column lengthening for pes planovalgus in cerebral palsy? Open

Karen M. Kruger, Christopher S. Constantino, Adam Graf, Ann Flanagan, Peter A. Smith , et al. · 2021

Evidence Favors Ponseti Management of Clubfoot: Commentary on an article by James A.F. Recordon, MBCHB, et al.: "A Prospective, Median 15-Year Comparison of Ponseti Casting and Surgical Treatment of Clubfoot". Open

Ken N. Kuo, Peter A. Smith · 2021

The sniff test Open

Peter A. Smith · 2021

Courtroom testimony about dogs detecting dead bodies keeps sending people to prison—even without physical evidence. Critics say the science is lacking

Osteogenesis imperfecta tooth level phenotype analysis: Cross-sectional study Open

Doaa Taqi, Hanan Moussa, Timothy Schwinghamer, Maxime Ducret, Didem Dagdeviren , et al. · 2021

Correction to: Do Bisphosphonates Alleviate Pain in Children? A Systematic Review Open

Mercedes Rodriguez Celin, Jacqueline C. Simon, Joseph J. Krzak, Alissa Fial, Karen M. Kruger , et al. · 2020

Malocclusion traits and oral health–related quality of life in children with osteogenesis imperfecta Open

Mohammadamin Najirad, Sreenath Madathil, Frank Rauch, V. Reid Sutton, Brendan Lee , et al. · 2020

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta Open

Mercedes Rodriguez Celin, Karen M. Kruger, Angela Caudill, Sandesh C.S. Nagamani, Gerald F. Harris , et al. · 2020

Background: Osteogenesis imperfecta (OI), a heritable connective tissue disorder with wide clinical variability, predisposes to recurrent fractures and bone deformity. Management requires a multidisciplinary approach in which intramedullar…

Hearing loss in individuals with osteogenesis imperfecta in North America: Results from a multicenter study Open

Keren Machol, Trevor D. Hadley, Jake Schmidt, David Cuthbertson, Henri Traboulsi , et al. · 2019

Hearing loss (HL) is an extra‐skeletal manifestation of the connective tissue disorder osteogenesis imperfecta (OI). Systematic evaluation of the prevalence and characteristics of HL in COL1A1/COL1A2‐related OI will contribute to a better …

Pediatric Outcomes Data Collection Instrument is a Useful Patient-Reported Outcome Measure for Physical Function in Children with Osteogenesis Imperfecta Open

Chaya N. Murali, David Cuthbertson, Brady Slater, Dianne Nguyen, Alicia Turner , et al. · 2019

Effect of Bisphosphonates on Function and Mobility Among Children With Osteogenesis Imperfecta: A Systematic Review Open

Christopher S. Constantino, Joseph J. Krzak, Alissa Fial, Karen M. Kruger, Jacob R. Rammer , et al. · 2019

Osteogenesis imperfecta (OI) is a rare genetic connective tissue disorder that results in bone fragility and deformity. Management is multi‐disciplinary. Although pharmacologic intervention with bisphosphonates (BP) is a standard of care f…

Mobility in osteogenesis imperfecta: a multicenter North American study Open

Karen M. Kruger, Angela Caudill, Mercedes Rodriguez Celin, Sandesh C.S. Nagamani, Jay R. Shapiro , et al. · 2019

Oro-dental and cranio-facial characteristics of osteogenesis imperfecta type V Open

Jean‐Marc Retrouvey, Doaa Taqi, Faleh Tamimi, Didem Dagdeviren, Francis H. Glorieux , et al. · 2018

A Multicenter Observational Cohort Study to Evaluate the Effects of Bisphosphonate Exposure on Bone Mineral Density and Other Health Outcomes in Osteogenesis Imperfecta Open

Jaskaran Bains, Erin M. Carter, Kate Citron, Adele L. Boskey, Jay R. Shapiro , et al. · 2018

Osteogenesis imperfecta (OI) is characterized by low bone mass and bone fragility. Using data from a large cohort of individuals with OI from the Osteogenesis Imperfecta Foundation's linked clinical research centers, we examined the associ…

Dental and facial characteristics of osteogenesis imperfecta type V Open

Jean‐Marc Retrouvey, Doaa Taqi, Faleh Tamimi, Didem Dagdeviren, Francis H. Glorieux , et al. · 2018

Osteogenesis imperfecta (OI) type V is an ultrarare heritable bone disorder caused by the heterozygous c.-14C>T mutation in IFITM5 . The dental and craniofacial phenotype has not been described in detail. In the present multicenter study (…

Mobility in Osteogenesis Imperfecta: A Multicenter North American Study Open

Karen M. Kruger, Angela Caudill, Mercedes Rodriguez Celin, Sandesh C.S. Nagamani, Jay R. Shapiro , et al. · 2018

Background Osteogenesis imperfecta (OI) is a genetic connective tissue disorder characterized by increased bone fragility and recurrent fractures. The phenotypic severity of OI has a significant influence on the ability to walk but little …

Growth characteristics in individuals with osteogenesis imperfecta in North America: results from a multicenter study Open

Mahim Jain, Allison Tam, Jay R. Shapiro, Robert D. Steiner, Peter A. Smith , et al. · 2018

Macroscopic anisotropic bone material properties in children with severe osteogenesis imperfecta Open

Carolyne Albert, John R. Jameson, Sergey Tarima, Peter A. Smith, Gerald F. Harris · 2017

Limb lengthening in pediatric patients with ollier's disease Open

Haluk Altıok, Paige Goote, Jennifer J. Beck, Peter A. Smith, Jeffrey D. Ackman , et al. · 2017

Background: Ollier's disease is a non-hereditary skeletal disorder. Orthopedic management of limb length discrepancy could be complex given the nature of bone pathology, the significant amount of length discrepancy and the difficulty to pr…

Non-Lethal Type VIII Osteogenesis Imperfecta Has Elevated Bone Matrix Mineralization Open

Nadja Fratzl‐Zelman, Aileen M. Barnes, MaryAnn Weis, Erin Carter, Theresa E. Hefferan , et al. · 2016

Context: Type VIII osteogenesis imperfecta (OI; OMIM 601915) is a recessive form of lethal or severe OI caused by null mutations in P3H1, which encodes prolyl 3-hydroxylase 1. Objectives: Clinical and bone material description of non-letha…

Walker Design for Kinetic Assessment of Upper Extremity Joint Demands in Children with Osteogenesis Imperfecta Open

Katherine A. Konop, Kelly M.B. Strifling, Mei Wang, Jeffrey P. Schwab, Jeffrey D. Ackman , et al. · 2016

Role of Micro-CT in the Visualization, Measurement, and Quantification of Bone Structure in Osteogenesis Imperfecta Open

Robert C. Molthen, John R. Jameson, Carolyne Albert, Peter A. Smith, Gerald F. Harris · 2016

Finite Element Modeling and Analysis Applications in Osteogenesis Imperfecta Open

Jessica M. Fritz, Nicole M. Grosland, Peter A. Smith, Gerald F. Harris · 2016

Understanding the biomechanics of bones in persons with osteogenesis imperfecta (OI) is a key component to further understanding the disease, optimizing treatment and quality of life, as well as injury prevention. However, it is not feasib…

Material and Structural Aspects of Bone in Osteogenesis Imperfecta Open

Carolyne Albert, John R. Jameson, Peter A. Smith, Gerald F. Harris · 2016

Bone fragility is a fundamental problem in individuals with osteogenesis imperfecta (OI). The mechanisms behind this fragility, however, are not yet well understood. Multiple factors appear to contribute to the increased fracture risk in O…

Upper Extremity Inverse Dynamics Model for Loftstrand Crutch-Assisted Gait in Children with Osteogenesis Imperfecta Open

Brooke A. Slavens, Neha Bhagchandani, Mei Wang, Peter A. Smith, Gerald F. Harris · 2016

Quantitative Assessment of Children with Osteogenesis Imperfecta Open

Adam Graf, Joseph J. Krzak, Angela Caudill, Ann Flanagan, Peter A. Smith , et al. · 2016

Assessments of children with Osteogenesis Imperfecta (OI) are typically limited to a physical exam and observations from a clinician during a hospital visit. Often quantitative information such as bone mineral density and outcome questionn…

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