Results 51 to 60 of about 39,094 (254)

Early Clinical, Imaging, and Pathological Characteristics of SRPK3/TTN‐Digenic Myopathy

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective SRPK3/TTN‐digenic myopathy was recently established as a skeletal muscle myopathy caused by digenic inheritance. This study characterizes the early clinical presentation of SRPK3/TTN‐digenic myopathy in one previously reported and seven newly identified pediatric patients.
Rotem Orbach, Sandra Donkervoort, Carola Hedberg‐Oldfors, Giovanni Baranello, Dimah Saade, Precilla D'Souza, Ruchee Patel, Eva Michael, A. Reghan Foley, Diana Bharucha‐Goebel, S. Jin Haugland, Meghan McAnally, Omer Abdul Hamid, Katherine Chao, Ellen F. Macnamara, Alan H. Beggs, Anna Sarkozy, Juliane Mueller, Steven A. Moore, Richard S. Finkel, Cynthia J. Tifft, Francesco Muntoni, Anders Oldfors, Carsten G. Bönnemann   +23 more
wiley   +1 more source

Comparison of respiratory mechanics measurements in the volume cycled ventilation (VCV) and pressure controlled ventilation (PCV)

Fisioterapia em Movimento
Introduction Monitoring respiratory mechanics may provide important information for the intensivist, assisting in the early detection of pulmonary function changes of patients hospitalized in ICU.
Kelser de Souza Kock, Beatriz Cardoso da Rosa, Natiéle Martignago, Cardine Reis, Rosemeri Maurici   +4 more
doaj   +1 more source

Plasma EV Proteomics Identifies ECM Remodeling and Inflammatory Proteins LUM and C7 as Candidate Biomarkers in FSHD

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Facioscapulohumeral muscular dystrophy (FSHD) is one of the most debilitating and common muscular dystrophies. Despite its severity, no approved therapy exists for FSHD patients. However, several therapeutic candidates are currently under development, and some have recently entered clinical trials, marking the need for reliable ...
Mustafa Bilal Bayazit, Chiranth K. Nagaraj, Jackson S. Newell, Kim Truc Nguyen, Xilal Y. Rima, Jacob Doon‐Ralls, Eduardo Reátegui, Jeffrey M. Statland, Rabi Tawil, Kevin M. Flanigan, Scott Q. Harper, Nizar Y. Saad   +11 more
wiley   +1 more source

Re‐Purposing Sapropterin (Kuvan) for ACTA2‐Related Multisystemic Smooth Muscle Dysfunction Syndrome: A Translational Mechanistic and First‐In‐Human Therapeutic Report

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Multisystemic smooth muscle dysfunction syndrome (MSMDS) is an ultra‐rare, ACTA2‐related disorder characterized by severe cerebrovascular disease, aortic aneurysms, and smooth muscle dysfunction. Using molecular dynamics simulations and in silico drug screening, we identified that sapropterin dihydrochloride (Kuvan) is a candidate capable of ...
Moran Hausman‐Kedem, Noa Bielopolski, Vijai Krishnan, Shane Wald‐Altman, Shelly I Shrian, Omer Bar‐Yosef, Livia Kapusta, Claire Shamber, Patricia L. Musolino, Miguel Weil   +9 more
wiley   +1 more source

Respiratory mechanics in infants with severe bronchiolitis on controlled mechanical ventilation

BMC Pulmonary Medicine, 2017
Background Analysis of respiratory mechanics during mechanical ventilation (MV) is able to estimate resistive, elastic and inertial components of the working pressure of the respiratory system.
Pablo Cruces, Sebastián González-Dambrauskas, Julio Quilodrán, Jorge Valenzuela, Javier Martínez, Natalia Rivero, Pablo Arias, Franco Díaz   +7 more
doaj   +1 more source

Artificial Intelligence in Systemic Sclerosis: Clinical Applications, Challenges, and Future Directions

Arthritis Care &Research, EarlyView.
Systemic sclerosis (SSc) is a rare autoimmune disease defined by immune dysregulation, vasculopathy, and progressive fibrosis of the skin and internal organs. Despite advances in care, major complications such as interstitial lung disease (ILD) and myocardial involvement remain the leading causes of morbidity and mortality.
Cristiana Sieiro Santos, José Miguel Sequí‐Sabater, Diego Benavent   +2 more
wiley   +1 more source

Features of respiratory support during laparoscopic correction of inguinal hernias in children

Videosurgery and Other Miniinvasive Techniques, 2016
Introduction : The objectives were to study the changes in the mechanics of respiration in children undergoing surgery depending on the value of intra-abdominal pressure (IAP) during laparoscopic procedures, and to compare the effects of different ...
Volodymyr Mishchuk, Orest Lerchuk, Andriy Dvorakevych, Volodymyr Khomyak   +3 more
doaj   +1 more source

Advances in Sustainable and Wearable Textile Based Soft Robotics

Advanced Functional Materials, EarlyView.
This Review examines advances in wearable textile‐based soft robotics, focusing on sustainable materials, integrated sensing, and scalable actuation. It discusses manufacturing and system integration across healthcare, assistive robotics, prosthetics, and human–machine interfaces, and highlights key challenges in circular design, including life‐cycle ...
Zahir Abbas, Marzia Dulal, Sophia K. Novoselov, Victoria K. Novoselov, Kostya S. Novoselov, Shaila Afroj, Nazmul Karim   +6 more
wiley   +1 more source

Biodegradable and Biocompatible Functional Polymers for Biomedical Applications

Advanced Functional Materials, EarlyView.
Biodegradable and biocompatible functional polymers integrate electrical, mechanical, and stimuli‐responsive functionalities while enabling programmed degradation under physiological conditions. This review introduces recent advances in conductive, shape‐memory, self‐healing, photocurable, and adhesive polymer systems, emphasizing material design ...
Won Bae Han, Shuvra Mondal, Sungkeun Han, Chan‐Hwi Eom, Venkata Ramesh Naganaboina, Suk‐Won Hwang   +5 more
wiley   +1 more source

Alterations in Respiratory Mechanics and Neural Respiratory Drive After Restoration of Spontaneous Circulation in a Porcine Model Subjected to Different Downtimes of Cardiac Arrest

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2019
Background The potential alterations of respiratory pathophysiology after cardiopulmonary resuscitation (CPR) are relatively undefined. While untreated arrest is known to affect post–cardiopulmonary resuscitation circulation, whether it affects ...
Zhengfei Yang, Houzhen Zheng, Lin Lin, Jingying Hou, Cai Wen, Yue Wang, Qin Ling, Longyuan Jiang, Wanchun Tang, Rui Chen   +9 more
doaj   +1 more source