Results 81 to 90 of about 880,425 (294)

Onasemnogene Abeparvovec in Patients With SMA: Interim Results of the RESTORE Registry in Japan

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective There are limited real‐world data regarding the safety and effectiveness of onasemnogene abeparvovec (OA; Zolgensma) infusion, a one‐time gene replacement therapy, for Japanese patients with spinal muscular atrophy (SMA). We aimed to improve understanding of the real‐world outcomes for OA in Japan.
Kayoko Saito, Kamal Benguerba, Ken Tsuchida, Kazushige Yazawa, Isao Tsumiyama, Hiromitsu Kayama, Sandra P. Reyna, Farid Khan, Richard S. Finkel   +8 more
wiley   +1 more source

Noninvasive Ventilation and Mechanical Insufflator-Exsufflator for Acute Respiratory Failure in Children With Neuromuscular Disorders

Frontiers in Pediatrics, 2020
Children with neuromuscular disorder (NMD) usually have pulmonary involvement characterized by weakened respiratory muscles, insufficient coughing, and inability to clear airway secretions. When suffering from community-acquired pneumonia, these patients
Tai-Heng Chen, Tai-Heng Chen, Tai-Heng Chen, Jong-Hau Hsu, Jong-Hau Hsu   +4 more
doaj   +1 more source

A cohort study to identify simple clinical tests for chronic respiratory failure in obese patients with sleep-disordered breathing

BMJ Open Respiratory Research, 2014
Background Chronic respiratory failure complicating sleep-disordered breathing in obese patients has important adverse clinical implications in terms of morbidity, mortality and healthcare utilisation. Screening strategies are essential to identify obese
S. Mandal, S. Mandal, E. Suh, E. Suh, E. Boleat, W. Asher, M. Kamalanathan, Kai K. Lee, Kai K. Lee, Abdel Douiri, Abdel Douiri, Patrick B. Murphy, Patrick B. Murphy, J. Steier, J. Steier, Nicholas Hart   +15 more
semanticscholar   +1 more source

Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs, Marcel Naumann, Annaliis Lehto, Hanna Schön, Jens Kurth, Johannes Prudlo, Andreas Hermann, Elisabeth Kasper   +7 more
wiley   +1 more source

Toxic epidermal necrolysis complicated with respiratory failure in children: A case report

Heliyon
Stevens-Johnson syndrome or toxic epidermal necrolysis (TEN) is a severe skin and mucosal reaction that develops rapidly and has a high mortality rate. Its early identification and proper treatment are crucial to lowering the risk of death.
Xiaoqian Chen, Suhua Jiang
doaj   +1 more source

Telemedicine system for the care of patients with neuromuscular disease and chronic respiratory failure

Archives of medical science : AMS, 2014
Introduction Neuromuscular diseases cause a number of limitations which may be improved by using a telemedicine system. These include functional impairment and dependence associated with muscle weakness, the insidious development of respiratory failure ...
C. Zamarrón, Emilio Morete, Francisco González   +2 more
semanticscholar   +1 more source

Claustrum Involvement in New Onset Refractory Status Epilepticus: A Systematic Review

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT The claustrum sign is a distinctive neuroimaging finding characterized by bilateral T2/FLAIR hyperintensity of the claustrum, one of the most interconnected regions of the human brain. It was first described in new‐onset refractory status epilepticus (NORSE) and febrile infection–related epilepsy syndrome (FIRES).
Margherita Burani, Lorenzo Muccioli, Giada Giovannini, Niccolò Orlandi, Francesca Bisulli, Stefano Meletti   +5 more
wiley   +1 more source

High-flow nasal cannula oxygen therapy as an emerging option for respiratory failure: the present and the future

Therapeutic Advances in Chronic Disease, 2020
Conventional oxygen therapy (COT) and noninvasive ventilation (NIV) have been considered for decades as frontline treatment for acute or chronic respiratory failure.
Lucia Spicuzza, Matteo Schisano
doaj   +1 more source

Marked Long‐Term Improvement in Lung Function in Melanoma Differentiation–Associated Protein 5 Antibody–Positive Dermatomyositis Patients: Experience of a Single‐Center Longitudinal Cohort in North America

Arthritis Care &Research, EarlyView.
Objective The objective of this study was to describe the longitudinal disease course and pulmonary outcomes of North American patients with melanoma differentiation–associated protein 5 (MDA5) antibody–associated dermatomyositis (DM). Methods Thirty patients with MDA5 antibody–associated DM were identified in a single‐center longitudinal cohort of 352
Jenice X. Cheah, Sangmee S. Bae, Tiffany De Leon, Yuna Lee, Rong Guo, David Elashoff, Jennifer Wang, Ani Shahbazian, Christina Charles‐Schoeman   +8 more
wiley   +1 more source

Outcome of Respiratory Failure Patients Managed In Medical Intensive Care Units (ICUs)

Journal of Rawalpindi Medical College, 2018
Background: To find out the outcome of patients managed with diagnosis of respiratory failure at Medical ICUs Methods: In this cross sectional observational study all respiratory failure patients managed at Medical ICUs were included.
Mahreen Zia et al.,
doaj