Results 41 to 50 of about 8,378 (218)

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

High level of initial Aspergillus fumigatus-specific IgE links increased risk of exacerbation in allergic bronchopulmonary aspergillosis patients

Respiratory Research
Background Elevated Aspergillus fumigatus (A. fumigatus)-specific Immunoglobulin E (IgE) is recognized as an essential diagnostic criterion for allergic bronchopulmonary aspergillosis (ABPA). However, it remains unknown whether initial A.
Hao Qian, Jia-Yan Xu, Rui Fan, Jing Shi, Hai-Wen Lu, Ling Ye, Jia-Wei Yang, Rui Jiang, Li-Sha Zhang, Yi-Fan Wu, Mei-Ling Jin, Jin-Fu Xu   +11 more
doaj   +1 more source

Emergency pharmacotherapy of the medicine disease. Respiratory system. Pulmonary thromboembolism.

Nihon Naika Gakkai Zasshi, 1995
肺血栓塞栓症は近年増加傾向にある疾患のひとつである.しかも,突然の発症より急速に重篤になる症例もあり,迅速な診断と治療がその後を左右する.臨床状況から本症を想起し疑った時点でただちに抗血栓療法を開始すべきである.治療はヘパリン,ウロキナーゼに加え,最近tissue-type plasminogen activatorや遺伝子工学による新しい血栓溶解薬も開発され今後の臨床的評価が待たれる.
openaire   +2 more sources

Comparative Effectiveness and Safety of Inebilizumab Versus Rituximab in AQP4‐IgG‐Positive NMOSD

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Rituximab (anti‐CD20, RTX) and inebilizumab (anti‐CD19, INE) represent B‐cell‐depleting therapies used for aquaporin‐4 antibody‐positive (AQP4‐IgG+) neuromyelitis optica spectrum disorder (NMOSD); however, direct comparative evidence remains limited.
Jie Lin, Binbin Xue, Dewei Xie, Jia Li, Juyuan Pan, Lanbing Zhu, Qiaowen Tong, Jing Lin, Ruofan Zhu, Xu Zhang, Junhui Xia, Xiang Li   +11 more
wiley   +1 more source

Association of asthma and bronchiectasis: Mendelian randomization analyses and observational study

Respiratory Research
Background Previous studies have demonstrated that asthma is closely associated with bronchiectasis, however, the causal relationship between asthma and bronchiectasis has not been investigated in depth.
Rui Fan, Hao Qian, Jia-Yan Xu, Jia-Yi Wang, Yue Su, Jia-Wei Yang, Fang Jiang, Wei-Jun Cao, Jin-Fu Xu   +8 more
doaj   +1 more source

Long‐Term Efficacy of Immunotherapy in Autoimmune Autonomic Ganglionopathy—A 10‐Year Follow Up Study

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Autoimmune autonomic ganglionopathy (AAG) is a rare but potentially treatable cause of severe autonomic failure. Evidence guiding long‐term immunotherapy, treatment sequencing, and residual autonomic impairment is limited. We evaluated long‐term treatment response, residual autonomic dysfunction, and relapse patterns in patients with
Giacomo Chiaro, Shiwen Koay, Gordon T. Ingle, Patricia McNamara, Laura Watson, Fion Bremner, Valeria Iodice   +6 more
wiley   +1 more source

Evaluation of Digital Technologies for Home‐Based Assessment in People With Amyotrophic Lateral Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Digital technologies hold promise for transforming healthcare by enhancing personalized treatments and offer valuable opportunities to improve patient care. Here, we evaluated several novel, self‐administered, home‐based, digital endpoints for their association with corresponding conventional standard clinical measures (primary) in ...
Arne Mueller, Vanessa Vallejo, Mónica Povedano Panadés, Orla Hardiman, Véronique Danel‐Brunaud, Senda Ajroud‐Driss, Philippe Couratier, Jeremy Shefner, Anna Gokey, Mike DiCesare, Erin Lennox, Jens Praestgaard, Lucie Brujin, Peggy Allred, Ram Miller   +14 more
wiley   +1 more source

Extracellular matrix remodelling in COPD

European Respiratory Review, 2020
The extracellular matrix (ECM) of the lung plays several important roles in lung function, as it offers a low resistant pathway that allows the exchange of gases, provides compressive strength and elasticity that supports the fragile alveolar–capillary ...
Meropi Karakioulaki, Eleni Papakonstantinou, Daiana Stolz   +2 more
doaj   +1 more source

Peripheral Neutrophil Activation and Extracellular Trap Formation in Amyotrophic Lateral Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
Markers of neutrophil activation are increased in plasma during ALS, and markers of NET formation associate with ALS survival. ABSTRACT Objectives Peripheral neutrophil levels in amyotrophic lateral sclerosis (ALS) inversely correlate with survival, suggesting a role for neutrophils in disease progression.
Lillia A. Baird, Haley McQuown, Jihyun Park, Samuel J. Teener, Ian F. Webber‐Davis, Andrew D. Carter, Dae‐Gyu Jang, Eva L. Feldman, Stephen A. Goutman, Benjamin J. Murdock   +9 more
wiley   +1 more source

Evidence for Interleukin-17C governing interleukin-17A pathogenicity and promoting asthma endotype switching in bronchiectasis

Nature Communications
The management of bronchiectasis-asthma overlap (BAO) is an important clinical issue to be addressed. Little is known regarding the endotype of BAO. Here we recruit patients with a primary diagnosis of bronchiectasis and co-existing asthma. The levels of
Yu-Wei Zhang, Yu-Hua Wen, Ling Yang, Ying-Zhou Xie, Dong Weng, Xi-Yue Shen, Jing-Ge Ma, Hai-Wen Lu, Xiao-Li Sun, Shu-Yi Gu, Bei Mao, Ai Ge, Yue Su, Fang Jiang, You-Cun Qian, Jin-Fu Xu   +15 more
doaj   +1 more source