Results 91 to 100 of about 228,717 (299)

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

HIF1A reduces acute lung injury by optimizing carbohydrate metabolism in the alveolar epithelium

, 2013
Background: While acute lung injury (ALI) contributes significantly to critical illness, it resolves spontaneously in many instances. The majority of patients experiencing ALI require mechanical ventilation.
Christoph H Borchers, Michel Mittelbronn, Thomas Packard, Kominsky, Douglas J., Bonney, Megan, Packard, Thomas, Michel Mittelbronn (307400), Eltzschig, Holger K., Holger K Eltzschig, Kelley Brodsky (448211), Mariani, Thomas J., Borchers, Christoph H., Brodsky, Kelley, Christoph H. Borchers (159697), Thomas J. Mariani (11667), Han, Jun, Eckle, Tobias, Holger K. Eltzschig (37610), Mittelbronn, Michel Guy André, Megan Bonney (462424), Thomas J Mariani, Jun Han, Douglas J Kominsky, Douglas J. Kominsky (462426), Tobias Eckle, Jun Han (231645), Kelley Brodsky, Megan Bonney, Tobias Eckle (65757), Thomas Packard (462425)   +29 more
core   +1 more source

Pioneers in Mechanical Ventilation: Björn Jonson

, 2022
In this article, we highlight one of the pioneers of mechanical ventilation. Dr Björn Jonson is a physiologist, physician and currently a Professor Emeritus at Lund University in Sweden. He has spent the last sixty years of his life dedicated to research
Daoud, Ehab, Ehab G. Daoud
core   +1 more source

The Diagnosis That Arrived Decades Late: Living Without and Then With Myhre Syndrome

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre syndrome (MIM #139210) is a rare multisystem disorder first described in 1981, characterized by short stature, neurodevelopmental delay, joint contractures, and cardiopulmonary complications. Its molecular basis, recurrent pathogenic variants in SMAD4, was not discovered until 2011. This narrative is based on a review of medical records,
Abdallah F. Elias
wiley   +1 more source

Alternating one lung ventilation using a double lumen endobronchial tube and providing CPAP to the non-ventilated lung in a dog

, 2011
INTRODUCTION: This case report describes the anaesthetic management of exploratory thoracoscopy and alternating one lung ventilation (OLV) in a dog with a pulmonary bulla, and the application of continuous positive airway pressure (CPAP) to the non ...
Ulrich Rytz, Axiak, S., Chiara Adami, Adami, C., Rytz, U., Claudia Spadavecchia, Shannon Axiak, Spadavecchia, C.   +7 more
core   +1 more source

Quantifying the performance of a top-down natural ventilation Windcatcher™ [PDF]

, 2009
Estimating the performance of a natural ventilation system is very important if one is to correctly size the system for a particular application. Estimating the performance of a Windcatcher™ is complicated by the complex flow patterns that occur during ...
Kirby, R, Jones, BM
core   +1 more source

Animal models of chronic thromboembolic pulmonary hypertension

Animal Models and Experimental Medicine, EarlyView.
Current animal models of CTEPH. Created using BioRender.com. Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, yet life‐threatening disorder characterized by persistent pulmonary vascular obstruction and elevated pulmonary artery pressure, with progressive remodeling and subsequent right heart failure.
Yong‐Jian Zhu, Lin‐Chao Zhou, Xiao‐Xue Zhang, Xiao‐Yang Ren, Yi Yan, Shao‐Fei Liu   +5 more
wiley   +1 more source

Lung maneuvers of large amplitudes for probing physiological alterations in mouse models of asthma

Animal Models and Experimental Medicine, EarlyView.
Lung maneuvers of small amplitudes, such as the ones performed in oscillometry, are the current gold standard to probe lung mechanics in animals, but they are insensitive to detect baseline physiological alterations in mouse models of asthma. Herein, lung maneuvers of different amplitudes were compared in two mouse models of asthma.
Magali Boucher, Cyndi Henry, Marie‐Josée Beaulieu, Andrés Rojas‐Ruiz, Ynuk Bossé   +4 more
wiley   +1 more source

Equine models in translational medicine: A comparative approach to human health

Animal Models and Experimental Medicine, EarlyView.
This diagram summarizes and contrasts rodent and equine models, outlining their strengths, limitations, and applications. Horses offer naturally occurring diseases, genetic and physiological similarities to humans, and suitability for longitudinal and clinical‐scale studies.
Shayan Boozarjomehri Amnieh, Katarzyna Ropka‐Molik   +1 more
wiley   +1 more source

Porcine kidney xenotransplantation: From primate models to clinical reality

Animal Models and Experimental Medicine, EarlyView.
In the face of a critical shortage of human donor kidneys for end‐stage renal disease patients, porcine kidney xenotransplantation has emerged as a viable solution. This field has navigated major hurdles, including immune rejection, physiological incompatibilities, potential biomechanical differences and the risk of cross‐species infection. To overcome
Zihang Guo, Ling Zhang, Shoulong Deng, Chuan Qin   +3 more
wiley   +1 more source