Results 71 to 80 of about 26,273 (306)

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

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

Trajectories of Physical Function in Canadian Children With Juvenile Idiopathic Arthritis

Arthritis Care &Research, EarlyView.
Objective We describe trajectories of physical function in children newly diagnosed with juvenile idiopathic arthritis (JIA) and identify trajectories with persisting functional impairments and associated baseline characteristics. Methods We included patients enrolled in the Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) Registry ...
Clare Cunningham, Meghan McPherson, Lillian Lim, Roberta A. Berard, Matthew Berkowitz, Jean‐Philippe Proulx‐Gauthier, Brian M. Feldman, Nicole Johnson, Dax G. Rumsey, Heinrike Schmeling, Lori B. Tucker, Thomas Loughin, Kristin M. Houghton, Jaime Guzman, for the Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) Registry Investigators, David A. Cabral, Gaëlle Chédeville, Ciarán M. Duffy, Kerstin Gerhold, Jaime Guzman, Linda Hiraki, Adam M. Huber, Heinrike Schmeling, Natalie J. Shiff, Lori B. Tucker, Julie Barsalou, Daniah Basodan, Michelle Batthish, Roberta A. Berard, Nicholas Blanchette, Gilles Boire, Roxana Bolaria, Alessandra Bruns, David A. Cabral, Sarah Campillo, Tania Cellucci, Mercedes Chan, Gaëlle Chédeville, Amieleena Chhabra, Julie Couture, Paul Dancey, Jean‐Jacques De Bruycker, Erkan Demirkaya, Muhammed Dhalla, Ciarán M. Duffy, Talal El Tal, Tommy Gerschman, Jaime Guzman, Liane Heale, Julie Herrington, Kristin M. Houghton, Adan M. Huber, Andrea Human, Nicole Johnson, Roman Jurencak, Bianca Lang, Claire LeBlanc, Lillian Lim, Lily S. H. Lim, Nadia Luca, Jeanine McColl, Tara McGrath, Tamara McMillan, Megan McPherson, Paivi Miettunen, Kimberly Morishita, Honyan Ng, Jonathan Park, Jean‐Philippe Proulx‐Gauthier, Johannes Roth, Evelyn Rozenblyum, Dax G. Rumsey, Heinrike Schmeling, Rosie Scuccimarri, Gordon Soon, Rebeka Stevenson, Elizabeth Stringer, Herman Tam, Lori B. Tucker, Marinka Twilt, Ruud H. J. Verstegen, Karen Watanabe Duffy   +81 more
wiley   +1 more source

Strain‐Programmable Luminescent Adhesive Patch With Tartrazine‐Mediated Optical Skin Clearing for Photochemical Tissue Bonding

Advanced Functional Materials, EarlyView.
We propose a suture‐complementary approach that integrates optical skin clearing with a strain‐programmable luminescent adhesive patch. Hyaluronic acid promotes transdermal delivery of tartrazine to improve optical clearing and stabilizes its interaction with a photosensitizer. Optical clearing increases the penetration depth of visible light into skin,
Seong‐Jong Kim, Kyungjoo Jo, Kwanghyeon Park, Jun Hee Kim, Jong Hyeong Cho, Inhoo Choi, Sei Kwang Hahn   +6 more
wiley   +1 more source

Solution‐Processed Thin‐Film Transistors With Tunable Temporal Dynamics for Neuromorphic Computing

Advanced Functional Materials, EarlyView.
Solution‐processed CNT and CNT/P3HT ion‐gated transistors exhibit materials‐defined synaptic timescales: fast CNT devices for high‐frequency spiking and slow hybrid devices for temporal integration. Embedding these dynamics into coupled reservoir‐computing and spiking neural network simulations reveals that a Hybrid‐Reservoir / CNT‐SNN architecture ...
Kevin Schnittker, Haoyi Lin, Zahra Bahrami, Strahinja Marinkovic, Sung Hyun Lee, Joseph Andrews   +5 more
wiley   +1 more source

Medical treatment for the terminally ill: the 'risk of unacceptable badness'. [PDF]

, 2005
David Crippen
openalex   +1 more source

Chamber‐Specific Decellularized Extracellular Matrices Differentially Modulate Cardiomyocyte Subtypes to Drive Engineered Heart Tissue Development and Function

Advanced Healthcare Materials, EarlyView.
Chamber‐specific decellularized extracellular matrices (ECMs) were developed, preserving native proteomic profiles of ventricular and atrial myocardium. These innate biochemical cues differentially modulate cardiomyocyte subtypes to drive engineered heart tissue development and function, highlighting the importance of incorporating regional ECM cues in
Dong Gyu Hwang, Myungji Kim, Hwanyong Choi, Jinseon Park, Minji Kim, Donghwan Kim, Bo Ram Lee, Jinah Jang   +7 more
wiley   +1 more source

Determinant factors of mortality in terminally ill patients with do-not-resuscitate orders

Formosan Journal of Surgery, 2018
Background: This study aimed to profile the clinical pictures and identify the risk factors of mortality among terminally ill patients who visited the emergency department (ED) and had signed do-not-resuscitate (DNR) consents.
Chao-Hui Su, Shih-Chiang Hung, Wan-I Chen, Li-Hui Yang, Chi-Wei Lee, Ching-Hua Hsieh   +5 more
doaj   +1 more source

A Fully Human Engineered Bone Niche With Endogenous Osteoclastogenesis Reveals Osteoclast‐Dependent Osteomimicry in Prostate Cancer Cells

Advanced Healthcare Materials, EarlyView.
A human 3D bone niche integrating osteoblasts and osteoclasts enables co‐culture with prostate cancer cell lines and patient‐derived organoids. The engineered niche (i) models aspects of reciprocal phenotypic regulation between osteoblasts and cancer cells, (ii) captures osteoclast‐enhanced osteomimicry in tumor cells, and (iii) establishes a ...
Andrea Mazzoleni, Robin Dolgos, Thomas Menter, Boris Dasen, Arnaud Scherberich, Clémentine Le Magnen, Manuele G. Muraro, Ivan Martin   +7 more
wiley   +1 more source

Natural Biomaterials for Osteochondral Repair: From Source to Strategy

Advanced Healthcare Materials, EarlyView.
Biological origin‐guided overview of natural biomaterials and therapeutic strategies for osteochondral tissue engineering. The circular diagram categorizes representative materials and strategies into plant/algae‐derived, microbial‐derived, animal‐derived, and human‐derived sources, centered on an osteochondral defect repair model.
Hengyu Liu, Hanyang Zhang, Wenbo Yang, Hao Chen, Jincheng Wang, Fei Chang   +5 more
wiley   +1 more source