Trending Articles
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Orphanet J Rare Dis · May 2020
ReviewPreclinical model systems of ryanodine receptor 1-related myopathies and malignant hyperthermia: a comprehensive scoping review of works published 1990-2019.
Pathogenic variations in the gene encoding the skeletal muscle ryanodine receptor (RyR1) are associated with malignant hyperthermia (MH) susceptibility, a life-threatening hypermetabolic condition and RYR1-related myopathies (RYR1-RM), a spectrum of rare neuromuscular disorders. In RYR1-RM, intracellular calcium dysregulation, post-translational modifications, and decreased protein expression lead to a heterogenous clinical presentation including proximal muscle weakness, contractures, scoliosis, respiratory insufficiency, and ophthalmoplegia. Preclinical model systems of RYR1-RM and MH have been developed to better understand underlying pathomechanisms and test potential therapeutics. ⋯ Over the past 30 years, there were 262 publications on MH and RYR1-RM preclinical model systems featuring more than 200 unique RYR1 variations tested in a broad range of species. Findings from these studies have set the foundation for therapeutic development for MH and RYR1-RM.
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Clinics in chest medicine · Sep 2013
ReviewBedside ultrasound for the interventional pulmonologist.
This article summarizes the current literature regarding thoracic ultrasonography and specifically guides the interventional pulmonologist in use of thoracic ultrasound for practical applications.
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Antiviral treatment of herpesvirus infections is rapidly changing since the advent of new drugs with improved oral availability. The efficacy of valaciclovir, the prodrug of aciclovir, and famciclovir, the prodrug of penciclovir, in the treatment of herpes genitalis and acute herpes zoster has been well documented in large clinical trials. Both drugs are effective on zoster-associated pain. ⋯ The development of resistance against aciclovir and the other nucleoside analogues has not been a problem to date in the treatment of immunocompetent individuals. However, in immunocompromised patients, aciclovir-resistant HSV strains often emerge. In such cases, intravenous foscarnet is the current treatment of choice.
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Inadequate healthcare workforce during a disaster affects the survival and health outcome of victims. During disaster strikes, nurses may face a dilemma regarding whether or not to report for duty, facing professional duty and their personal and/or family safety that may be at stake. This is a cross-sectional descriptive study. ⋯ It is concluded that about one-third of nurses indicated that they would not report to work when a disaster strikes. This raises a warning signal for healthcare managers that they need to plan ahead to maintain an adequate workforce when disasters strike. Managers are urged to do more to understand the factors leading to nurses' unwillingness to report to work and to undertake realistic staffing planning to cope with the extra demand.