Early Clinical, Imaging, and Pathological Characteristics of SRPK3/TTN‐Digenic Myopathy
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 +23 more
wiley +1 more source
Energy efficiency with natural ventilation: a case study [PDF]
Application of laboratory analogue modelling of air flow in a naturally ventilated shopping mall is reviewed in this paper. A detailed study of the ventilation was undertaken to establish the principles and to explore how natural ventilation might ...
Woods, A. W., Fitzgerald, S. D.
core +1 more source
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 +14 more
wiley +1 more source
BackgroundData on clinical outcomes of infants with birthweights less than 501 g (ELBW<501) are limited.ObjectiveTo evaluate management strategies and clinical outcomes of ELBW<501infants compared to infants weighing 501–750 g (ELBW501–750 ...
Timothy G. Elgin +6 more
doaj +1 more source
Lung Recruitment Strategies During High Frequency Oscillatory Ventilation in Preterm Lambs
Background: High frequency oscillatory ventilation (HFOV) is considered a lung protective ventilation mode in preterm infants only if lung volume is optimized.
Martijn Miedema +14 more
doaj +1 more source
High-frequency oscillatory ventilation in children: a 10-year experience
Objectives: The aim of the study was to describe the experience with high-frequency oscillatory ventilation (HFOV) in a Portuguese Pediatric Critical Care Unit, and to evaluate whether HFOV allowed improvement in oxygenation and ventilation.
Marta Moniz +6 more
doaj +3 more sources
Onasemnogene Abeparvovec in Patients With SMA: Interim Results of the RESTORE Registry in Japan
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 +8 more
wiley +1 more source
Simulating the effect of forces pit ventilation on ammonia emission from naturally ventilated cow houses with CFD [PDF]
Atmospheric NH3, mainly originates from agricultural sources, can cause serious environmental problems related to eutrophication and soil acidification. Emissions from dairy houses are 15% of total agricultural NH3 emissions.
Campen, J.B. +3 more
core
Influence of Tracheal Obstruction on the Efficacy of Superimposed High-frequency Jet Ventilation and Single-frequency Jet Ventilation [PDF]
Both superimposed high-frequency jet ventilation (SHFJV) and single-frequency (high-frequency) jet ventilation (HFJV) have been used with success for airway surgery, but SHFJV has been found to provide higher lung volumes and better gas exchange than ...
LO MAURO, MARIA ANTONELLA +13 more
core +1 more source
Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs +7 more
wiley +1 more source

