Results 51 to 60 of about 215,489 (347)

Recommendations for the management of MPS IVA: systematic evidence- and consensus-based guidance. [PDF]

, 2019
IntroductionMucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan ...
Akyol, Mehmet Umut, Alden, Tord D, Amartino, Hernan, Ashworth, Jane, Belani, Kumar, Berger, Kenneth I, Borgo, Andrea, Braunlin, Elizabeth, Eto, Yoshikatsu, Giugliani, Roberto, Gold, Jeffrey I, Harmatz, Paul, Hendriksz, Christian, Jester, Andrea, Jones, Simon A, Karsli, Cengiz, Mackenzie, William, Marinho, Diane Ruschel, McFadyen, Andrew, McGill, Jim, Mitchell, John J, MPS Consensus Programme Co-Chairs, MPS Consensus Programme Steering Committee, Muenzer, Joseph, Okuyama, Torayuki, Orchard, Paul J, Scarpa, Maurizio, Stevens, Bob, Thomas, Sophie, Walker, Robert, Wynn, Robert   +30 more
core   +2 more sources

Life‐Threatening Bradycardia in Anti‐NMDA‐Receptor Encephalitis and a Novel Use for Permanent Pacing

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Background Pediatric anti‐NMDA receptor encephalitis (pNMDARE) is an autoantibody‐mediated disorder that can cause severe autonomic dysfunction, including symptomatic bradycardia and asystole. Dysautonomia can last for years, making it very challenging to manage.
Sarah Tucker, Abhijit Das, Andres Jimenez, Areeba Basit, Yike Jiang, Emily A. Smitherman, Heather Van Mater, Taylor Howard, Alexander J. Sandweiss, Kristen S. Fisher   +9 more
wiley   +1 more source

Therapeutic alternatives with CPAP in obstructive sleep apnea [PDF]

, 2019
Obstructive Sleep Apnea (OSA), characterized by airflow cessation (apnea) or reduction (hypopnea) due to repeated pharyngeal obstructions during sleep, causes frequent disruption of sleep and hypoxic events. The condition is linked to many adverse health
Aluas, Maria, Bordea, Roxana I, Budin, Corina E, Ciumarnean, Lorena, Cozma, Angela, Gergely, Bianca D, Maierean, Anca, Man, Milena, Rajnovean, Ruxandra   +8 more
core   +3 more sources

Sarilumab in Polyarticular‐Course Juvenile Idiopathic Arthritis: Dose‐Finding and One‐Year Analysis of a Phase 2b, Open‐Label, Multicenter Study

Arthritis Care &Research, Accepted Article.
Objective This study assessed sarilumab in treating patients with polyarticular‐course juvenile idiopathic arthritis (pcJIA). Methods This phase 2b, open‐label study (NCT02776735) consisted of three sequential parts (each with a core‐treatment and extension‐phase). During part 1, three doses were assessed in two weight groups (Group A/B: ≥30–60 kg/≥10–<
Fabrizio De Benedetti, Inmaculada C. Penadés, Irina Nikishina, Ivan Foeldvari, Alberto J. Spindler, Anna Kozlova, Nadina Rubio‐Pérez, Pierre Quartier, Zbigniew Żuber, Raul Barria, Daniel Clemente, Gabriel V. Cornejo, Katherine Marzan, Nancy Liu, Christine Xu, Stephen DiMartino, Angeliki Giannelou, Fei Cao‐Ghoul, Bolanle Akinlade, Lydie Baret‐Cormel   +19 more
wiley   +1 more source

Late-onset bleb-associated endophthalmitis and continuous positive airway pressure

American Journal of Ophthalmology Case Reports, 2018
Purpose: To raise awareness of a possible association between continuous positive airway pressure (CPAP) devices and postoperative bleb-related infection.
Erich J. Berg, John B. Davies, Mark R. Buboltz, Thomas W. Samuelson   +3 more
doaj   +1 more source

GH-secreting pituitary macroadenoma (acromegaly) associated with progressive dental malocclusion and refractory CPAP treatment [PDF]

, 2017
Background: A link between progressive dental malocclusion, the use of a continuous positive airway pressure mask and GH-secreting pituitary macroadenoma (acromegaly) has not been previously reported.
Brunet i Llobet, Lluís, Dios-Miranda, David de, Giménez Rubio, Josep Anton, Lahor i Soler, Eduard, Miranda i Rius, Jaume   +4 more
core   +2 more sources

Combining Three Peripheral Blood Biomarkers to Stratify Rheumatoid Arthritis‐Associated Interstitial Lung Disease Risk

Arthritis Care &Research, Accepted Article.
Objective The purpose was to evaluate a biomarker score consisting of MUC5B rs35705950 promoter variant, plasma matrix metalloproteinase (MMP)‐7, and serum anti‐malondialdehyde‐acetaldehyde (anti‐MAA) antibody for RA‐associated interstitial lung disease risk stratification. Methods Using a multicenter cohort of US veterans with RA, we performed a cross‐
Kelsey Coziahr, Austin M. Wheeler, Brent A. Luedders, Michael Duryee, Halie Frideres, Katherine D. Wysham, Grant W. Cannon, Gary Kunkel, Dana P. Ascherman, Paul A. Monach, Gail S. Kerr, Andreas M. Reimold, Scott M. Matson, Joshua F. Baker, Geoffrey M. Thiele, Ted R. Mikuls, Bryant R. England   +16 more
wiley   +1 more source

Comparative Hemodynamic Effects of BiPAP and APRV in Patients with ARDS: A Systematic Review

Albanian Journal of Trauma and Emergency Surgery
Introduction: Positive pressure ventilation is key in the treatment of patients with acute respiratory distress syndrome. Bilevel Positive Airway Pressure and Airway Pressure Release Ventilation modalities aim to improve oxygenation and reduce lung ...
Leon Hajdari, Egzon Daku, Gramoz Bunjaku, Basri Lenjani, Dardan Lenjani   +4 more
doaj   +1 more source

Marked Long‐term Improvement in Lung Function in Melanoma Differentiation‐associated Protein 5 (MDA5) Antibody Positive Dermatomyositis Patients: Experience of a Single Center Longitudinal Cohort in North America

Arthritis Care &Research, Accepted Article.
Objective The objective of this study was to describe the longitudinal disease course and pulmonary outcomes of North American patients with melanoma differentiation‐associated gene 5 antibody (MDA5 ab) associated dermatomyositis (DM). Methods Thirty patients with MDA5 ab DM were identified in a single center longitudinal cohort of 352 patients with ...
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

Seasonal changes in positive airway pressure adherence

Frontiers in Medicine
Through their effects on sleep duration, bedroom environments, and pollen allergies, seasonal variations may impact positive airway pressure (PAP) adherence.
Arnaud Prigent, Arnaud Prigent, Clément Blanloeil, Dany Jaffuel, Dany Jaffuel, Anne Laure Serandour, Franck Barlet, Frédéric Gagnadoux, Frédéric Gagnadoux   +8 more
doaj   +1 more source