Results 31 to 40 of about 47,079 (243)

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

Model for End‐Stage Liver Disease/Pediatric End‐Stage Liver Disease exception policy and outcomes in pediatric patients with hepatopulmonary syndrome requiring liver transplantation

Liver Transplantation, EarlyView., 2022
Abstract Hepatopulmonary syndrome (HPS) is associated with increased waitlist mortality in liver transplantation (LT) candidates. Children with HPS are granted Model for End‐Stage Liver Disease (MELD)/Pediatric End‐Stage Liver Disease (PELD) exception points for waitlist prioritization in the United States based on criterion developed for adults.
Muhammad H. Raza, Yong Kwon, Pierre Kobierski, Asish C. Misra, Angelina Lim, Cameron Goldbeck, Kambiz Etesami, Rohit Kohli, Juliet Emamaullee   +8 more
wiley   +1 more source

The Case of a 40‐Year‐Old Man With New‐Onset Status Epilepticus in the Setting of HIV Infection

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT A 40‐year‐old man from Andhra Pradesh in India, while on regular antiretroviral treatment (ART) for long‐standing HIV infection presented with a 1‐day history of new‐onset status epilepticus. CT and MRI brain showed multifocal cerebral ring lesions, some of which demonstrated characteristic ‘hole‐with‐a‐dot’ sign.
Vadde Akhil, Ramachandiran Nandhagopal
wiley   +1 more source

Chronic noninvasive ventilation in COPD: towards the identification of a responder phenotype

ERJ Open Research
Purpose Chronic noninvasive ventilation (NIV) is an effective treatment for patients with chronic hypercapnia due to COPD, but the improvement in health-related quality of life (HRQoL) varies significantly among individuals.
Tim Raveling, Jorine E. Hartman, Renzo Boersma, Peter J. Wijkstra, Marieke L. Duiverman   +4 more
doaj   +1 more source

Validation of the Hungarian version of the SRI Questionnaire

BMC Pulmonary Medicine, 2020
Background Home mechanical ventilation is a reliable treatment for patients suffering from chronic respiratory failure, improving survival and quality of life.
Luca Valko, Szabolcs Baglyas, Laszlo Kunos, Attila Terray-Horvath, Andras Lorx, Janos Gal, Wolfram Windisch   +6 more
doaj   +1 more source

Tailoring NIV by dynamic laryngoscopy in a child with spinal muscular atrophy type I

Clinical Case Reports, 2021
Dynamic laryngoscopy during noninvasive (NIV) respiratory therapy is feasible and may facilitate optimal and individualized treatment in patients with chronic respiratory failure, also in children.
Maria Vollsæter, Anett Skjoldmo, Ola Røksund, Magnus Hilland, Tiina Andersen   +4 more
doaj   +1 more source

Elephant‐Skin‐Inspired Porous Cementitious Tiles with Programmable Crack Networks for Passive Cooling

Advanced Materials, EarlyView.
Elephant‐skin‐inspired crack networks are programmed in porous diatomaceous earth (DE)‐cement composites using substrate‐guided, stress‐concentration induced fracture. The resulting crack lattices act as capillary conduits that redistribute water, while the porous matrix stores moisture.
Qingya Huang, Kun‐Hao Yu, Ji Yoon Bae, Yunchan Lee, Dorit Aviv, Shu Yang   +5 more
wiley   +1 more source

A Geometrically Transient Platform for Bioelectronic Implants

Advanced Materials, EarlyView.
Minimally invasive bioelectronic implants often compromise performance for smaller sizes. To resolve this optimization dilemma, a wireless bioelectronic implant with a transient geometry is introduced (MiFi). The origami‐inspired device miniaturizes up to sixfold for syringe insertion and autonomously unfolds post‐implantation.
Selin Olenik, John D. Goodwin, Atharv Naik, Philip Coatsworth, Ioi Chit Cheung, Farbod Amirghasemi, Anies Sohi, Ehsan Abedi, Tayeb Tadlaoui, Maral P. S. Mousavi, Andriy S. Kozlov, Victoria Salem, Andrew S. Cowburn, Firat Guder   +13 more
wiley   +1 more source

Home Mechanical Ventilation Practices in Spain and Portugal

Open Respiratory Archives, 2021
Introduction: Home mechanical ventilation (HMV) practice has changed with publication of new evidence and introduction of new technologies, and varies between countries. This study investigated current HMV use and practices in Spain and Portugal. Methods:
Antonio Antón, Salvador Díaz Lobato, Maria José Guimarães, Mari Luz Alonso-Álvarez, Sara Correia, Miguel R. Gonçalves   +5 more
doaj   +1 more source

Home mechanical ventilation in Hong Kong [PDF]

European Respiratory Journal, 2003
Home mechanical ventilation (HMV) is increasingly used to treat chronic respiratory failure. This present study was aimed to examine the trend, the disease categories treated and the outcomes of HMV use in Hong Kong.In the year 2002, all adult respiratory units in Hong Kong were invited to report to a multicentre retrospective survey of HMV use.
C M, Chu, W C, Yu, C M, Tam, C W, Lam, D S C, Hui, C K W, Lai   +5 more
openaire   +2 more sources