Results 31 to 40 of about 8,068 (200)

Unravelling inclusion body myositis using a patient‐derived fibroblast model

Journal of Cachexia, Sarcopenia and Muscle, 2023
Background Inclusion body myositis (IBM) is an inflammatory myopathy clinically characterized by proximal and distal muscle weakness, with inflammatory infiltrates, rimmed vacuoles and mitochondrial changes in muscle histopathology.
Judith Cantó‐Santos, Laura Valls‐Roca, Ester Tobías, Francesc Josep García‐García, Mariona Guitart‐Mampel, Anna Esteve‐Codina, Beatriz Martín‐Mur, Mercedes Casado, Rafael Artuch, Estel Solsona‐Vilarrasa, José Carlos Fernandez‐Checa, Carmen García‐Ruiz, Carles Rentero, Carlos Enrich, Pedro J. Moreno‐Lozano, José César Milisenda, Francesc Cardellach, Josep M. Grau‐Junyent, Glòria Garrabou   +18 more
doaj   +1 more source

Understanding the Burden of Orofacial Involvement and Patient Treatment Preferences in Systemic Sclerosis: Results From a Large International Survey

Arthritis Care &Research, EarlyView.
Objective Orofacial manifestations are significantly impactful in patients with systemic sclerosis (SSc) yet remain understudied, with no dedicated clinical guidelines to inform their management. Methods An international online survey comprised38 questions addressing orofacial manifestations of SSc, including patients’ confidence in their treating ...
Eleni Deligianni, Lee Shapiro, Deana Gibbs, Zsuzsanna H. McMahan, Michael Hughes   +4 more
wiley   +1 more source

Impact of Six‐Month Monitoring Compared to Three‐Month Monitoring of Laboratories During Methotrexate Therapy

Arthritis Care &Research, EarlyView.
Objective To evaluate whether extending the American College of Rheumatology–recommended monitoring interval for complete blood count and liver function tests in patients receiving methotrexate (MTX) affects timely detection of medication‐related toxicity.
Spencer Simko, Ray Zhang, Sohini Mukherjee, Puneet Bajaj, Bonnie L. Bermas   +4 more
wiley   +1 more source

Diagnostic Value of Muscle [11C] PIB-PET in Inclusion Body Myositis

Frontiers in Neurology, 2020
Background: The accumulation of multiple-protein aggregates within muscle fibers is a pathological hallmark of sporadic inclusion body myositis (s-IBM) with the presence of inclusion bodies. Amyloid-beta is one of the accumulated proteins in s-IBM.
Yu-ichi Noto, Masaki Kondo, Yukiko Tsuji, Shigenori Matsushima, Toshiki Mizuno, Takahiko Tokuda, Masanori Nakagawa   +6 more
doaj   +1 more source

Histidine Supplementation Stabilizes Hearing and Vision and Improves Growth in HARS1‐Related Autosomal Recessive Disorder Associated With Usher‐Like Symptoms

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Autosomal recessive HARS1‐related disorder (originally described as Usher syndrome type 3B) caused by a homozygous Y454S variant in the histidyl‐tRNA synthetase gene (HARS1) is characterized by progressive sensorineural hearing and vision loss and respiratory deterioration with risk for sudden death following febrile illnesses.
Victoria Mok Siu, Rosan Kenana, Rana Chakrabarti, Sarah D. P. Wilhelm, Joseph Andrews, Susan J. Leat, Christina Parker, Michael Miller, Leslie A. Nangle, Wendy McCaul, Ashfia Chowdhury, Natalie Hutchings, Ryan A. Adams, Lauren Guy, Mandy Rhody, Verena Juncal, Marisa I. Mendes, Desiree E. C. Smith, Gajja S. Salomons, Angelica A. Moresco, Daphne L. McCulloch, D. Holmes Morton, Ilka U. Heinemann, C. Anthony Rupar   +23 more
wiley   +1 more source

Amyloid myopathy: a diagnostic challenge

Neurology International, 2009
Amyloid myopathy (AM) is a rare manifestation of primary systemic amyloidosis (AL). Like inflammatory myopathies, it presents with proximal muscle weakness and an increased creatine kinase level.
Heli Tuomaala, Mikko Kärppä, Hannu Tuominen, Anne M. Remes   +3 more
doaj   +1 more source

Distribution of Big Tau Isoforms in the Human Central and Peripheral Nervous System

Annals of Neurology, EarlyView.
Objective Tau is widely studied in neurodegeneration, yet most work has focused on canonical brain tau isoforms. A longer isoform, “big tau,” produced by inclusion of exon 4a, is expressed in the peripheral nervous system (PNS) and central nervous system (CNS) regions.
Rama Krishna Koppisetti, Nicolas R. Barthélemy, Kanta Horie, Cindy V. Ly, Kaleigh F. Roberts, Srinivas Koutarapu, Richard J. Perrin, Erin E. Franklin, Chiara Pedicone, Joshua Orrick, Justin Melendez, Timothy M. Miller, Chihiro Sato, Nupur Ghoshal, Alison M. Goate, Celeste M. Karch, Randall J. Bateman, Soumya Mukherjee   +17 more
wiley   +1 more source

Integrated Multi-Omics Analysis for Inferring Molecular Players in Inclusion Body Myositis

Antioxidants, 2023
Inclusion body myositis (IBM) is an acquired inflammatory myopathy affecting proximal and distal muscles that leads to weakness in patients over 50.
Judith Cantó-Santos, Laura Valls-Roca, Ester Tobías, Clara Oliva, Francesc Josep García-García, Mariona Guitart-Mampel, Félix Andújar-Sánchez, Anna Esteve-Codina, Beatriz Martín-Mur, Joan Padrosa, Raquel Aránega, Pedro J. Moreno-Lozano, José César Milisenda, Rafael Artuch, Josep M. Grau-Junyent, Glòria Garrabou   +15 more
doaj   +1 more source

Treatment for inclusion body myositis

Cochrane Database of Systematic Reviews, 2015
Inclusion body myositis (IBM) is a late-onset inflammatory muscle disease (myopathy) associated with progressive proximal and distal limb muscle atrophy and weakness. Treatment options have attempted to target inflammatory and atrophic features of this condition (for example with immunosuppressive and immunomodulating drugs, anabolic steroids, and ...
Rose, Michael R., Jones, Katherine, Leong, Kevin, Walter, Maggie C., Miller, James, Dalakas, Marinos, Brassington, Ruth, Griggs, Robert   +7 more
openaire   +5 more sources

Electromyography varies by stage in inclusion body myositis

Frontiers in Neurology
IntroductionInclusion body myositis (IBM) is a chronic inflammatory muscle disease that is characterized by mixed myogenic and neurogenic electromyography (EMG) findings.
Tomoo Mano, Tomoo Mano, Naohiko Iguchi, Nobuyuki Eura, Naoki Iwasa, Nanami Yamada, Hirosei Horikawa, Kazuma Sugie   +7 more
doaj   +1 more source