Plos One
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Choroideremia (CHM) is an X- linked retinal degeneration that is symptomatic in the 1(st) or 2(nd) decade of life causing nyctalopia and loss of peripheral vision. The disease progresses through mid-life, when most patients become blind. CHM is a favorable target for gene augmentation therapy, as the disease is due to loss of function of a protein necessary for retinal cell health, Rab Escort Protein 1 (REP1). ⋯ Delivery of the CHM cDNA to affected cells restores REP1 enzymatic activity and also restores proper protein trafficking. The gene transfer is efficient and the preliminary safety data are encouraging. These studies pave the way for a human clinical trial of gene therapy for CHM.
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Non-ionic radiocontrast media (RCM) is rarely associated with hypersensitivity reactions. Premedication of patients who reacted previously to RCM with systemic corticosteroids and/or antihistamines can help reduce recurrent hypersensitivity reactions. However, premedication is still not prescribed in many cases for various reasons. This study aimed to determine the effectiveness of our novel RCM hypersensitivity surveillance and automatic recommending system for premedication. ⋯ Premedication with corticosteroid and/or antihistamine, which was increased by our novel automatic prescription system, significantly reduced breakthrough reactions in patients with a history of RCM hypersensitivity.
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Intestinal barrier dysfunction occurs in many intestinal diseases, in which proinflammatory cytokines play critical roles. However, researchers are still on the way to defining the underlying mechanisms and to evaluate therapeutic strategies for restoring intestinal barrier function. Berberine, a drug that has clinically been used to treat gastroenteritis and diarrhea for thousands of years, has been shown to protect barrier function in both endothelial and epithelial cells, but the mechanisms are completely unknown. ⋯ The increase of both MLC phosphorylation and MLCK protein expression induced by IFN-γ and TNF-α was significantly inhibited by berberine treatment. Additionally, berberine suppressed the activation of HIF-1α, but not NF-κB. Taken together, it is suggested that berberine attenuates IFN-γ and TNF-α-induced intestinal epithelial barrier dysfunction by inhibiting the signaling pathway of MLCK-dependent MLC phosphorylation mediated by HIF-1α.
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To study the relevance of high-sensitive troponin measurements in the acute workup in patients admitted to the emergency department of a large university hospital due to syncope. ⋯ On basis of the current study, where no single patient took benefit from determination of high-sensitive troponin, measurement of cardiac troponins should be reserved for patients with syncope presenting with symptoms suggestive for the presence of an acute cardiac syndrome.
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The experience of pain and disgust share many similarities, given that both are aversive experiences resulting from bodily threat and leading to defensive reactions. The aim of the present study was to investigate whether facial expressions are distinct enough to encode the specific quality of pain and disgust or whether they just encode the similar negative valence and arousal level of both states. In sixty participants pain and disgust were induced by heat stimuli and pictures, respectively. ⋯ Whereas pain was mostly encoded by contraction of the muscles surrounding the eyes (by itself or in combination with contraction of the eyebrows); disgust was mainly accompanied by contraction of the eyebrows and--in contrast to pain--by raising of the upper lip as well as the combination of upper lip raise and eyebrow contraction. Our data clearly suggests that facial expressions seem to be distinct enough to encode not only the general valence and arousal associated with these two bodily aversive experiences, namely pain and disgust, but also the specific origin of the threat to the body. This implies that the differential decoding of these two states by an observer is possible without additional verbal or contextual information, which is of special interest for clinical practice, given that raising awareness in observers about these distinct differences could help to improve the detection of pain in patients who are not able to provide a self-report of pain (e.g., patients with dementia).