Diagnostic and Management Issues in Patients with Late-Onset Ornithine Transcarbamylase Deficiency

Ornithine transcarbamylase deficiency (OTCD) is the most common inherited disorder of the urea cycle and, in general, is transmitted as an X-linked recessive trait. Defects in the OTC gene cause an impairment in ureagenesis, resulting in hyperammonemia, which is a direct cause of brain damage and death. Patients with late-onset OTCD can develop symptoms from infancy to later childhood, adolescence or adulthood. Clinical manifestations of adults with OTCD vary in acuity. Clinical symptoms can be aggravated by metabolic stressors or the presence of a catabolic state, or due to increased demands upon the urea. A prompt diagnosis and relevant biochemical and genetic investigations allow the rapid introduction of the right treatment and prevent long-term complications and mortality. This narrative review outlines challenges in diagnosing and managing patients with late-onset OTCD

Multi-Instrument Observations of an MSTID over Arecibo Observatory

The Penn State All-Sky Imager (PSASI) at Arecibo Observatory provides planar horizontal context to the vertical ionospheric profiles obtained by the Incoherent Seatter Radar (TSR). Electric field measurements from the Communication/Navigation Outage Forecast System (C/NOFS) satellite are mapped down geomagnetic field lines to the height of the airglow layer; allowing multi-instrument studies of field-aligned irregularities with radar, imager, and satellite. A Medium-Scale Traveling Ionospheric Disturbance (MSTID) was observed during such a conjunction near the December solstice of 2009

Inhaled nitric oxide suppresses neuroinflammation in experimental ischemic stroke

Ischemic stroke is a major global health issue and characterized by acute vascular dysfunction and subsequent neuroinflammation. However, the relationship between these processes remains elusive. In the current study, we investigated whether alleviating vascular dysfunction by restoring vascular nitric oxide (NO) reduces post-stroke inflammation. Mice were subjected to experimental stroke and received inhaled NO (iNO;50 ppm) after reperfusion. iNO normalized vascular cyclic guanosine monophosphate (cGMP) levels, reduced the elevated expression of intercellular adhesion molecule-1 (ICAM-1), and returned leukocyte adhesion to baseline levels. Reduction of vascular pathology significantly reduced the inflammatory cytokines interleukin-1 beta (Il-1 beta), interleukin-6 (Il-6), and tumor necrosis factor-alpha (TNF-alpha), within the brain parenchyma. These findings suggest that vascular dysfunction is responsible for leukocyte adhesion and that these processes drive parenchymal inflammation. Reversing vascular dysfunction may therefore emerge as a novel approach to diminish neuroinflammation after ischemic stroke and possibly other ischemic disorders

New method to characterize a machining system: application in turning

Many studies simulates the machining process by using a single degree of freedom spring-mass sytem to model the tool stiffness, or the workpiece stiffness, or the unit tool-workpiece stiffness in modelings 2D. Others impose the tool action, or use more or less complex modelings of the efforts applied by the tool taking account the tool geometry. Thus, all these models remain two-dimensional or sometimes partially three-dimensional. This paper aims at developing an experimental method allowing to determine accurately the real three-dimensional behaviour of a machining system (machine tool, cutting tool, tool-holder and associated system of force metrology six-component dynamometer). In the work-space model of machining, a new experimental procedure is implemented to determine the machining system elastic behaviour. An experimental study of machining system is presented. We propose a machining system static characterization. A decomposition in two distinct blocks of the system "Workpiece-Tool-Machine" is realized. The block Tool and the block Workpiece are studied and characterized separately by matrix stiffness and displacement (three translations and three rotations). The Castigliano's theory allows us to calculate the total stiffness matrix and the total displacement matrix. A stiffness center point and a plan of tool tip static displacement are presented in agreement with the turning machining dynamic model and especially during the self induced vibration. These results are necessary to have a good three-dimensional machining system dynamic characterization

Adiponectin receptor-1 expression is associated with good prognosis in gastric cancer

<p>Abstract</p> <p>Background</p> <p>Adiponectin is inversely related to BMI, positively correlates with insulin sensitivity, and has anti-atherogenic effects. In recent years, adiponectin has been well studied in the field of oncology. Adiponectin has been shown to have antiproliferative effects on gastric cancer, and adiponectin expression is inversely correlated with clinical staging of the disease. However, no studies have reported the correlation between serum adiponectin and receptor expression with disease progression.</p> <p>Methods</p> <p>In this study, we evaluated expression levels of 2 adiponectin receptors--AdipoR1 and AdipoR2--and attempted to correlate their expression with prognosis in gastric cancer patients. AdipoR1 and AdipoR2 expression in gastric cancer cell lines (MKN45, TMK-1, NUGC3, and NUGC4) was evaluated by western blotting analysis, and the antiproliferative potential of adiponectin was examined in vitro. Serum adiponectin levels were evaluated in 100 gastric cancer patients, and the expression of AdipoR1 and AdipoR2 was assessed by immunohistochemical staining.</p> <p>Results</p> <p>MKN45 and NUGC3 expressed higher levels of AdipoR1 compared to NUGC4, even though there was no significance in AdipoR2 expression. The antiproliferative effect of adiponectin was confirmed in MKN45 and NUGC3 at 10 μg/ml. No significant associations were observed between serum adiponectin levels and clinicopathological characteristics, but lymphatic metastasis and peritoneal dissemination were significantly higher in the negative AdipoR1 immunostaining group (24/32, <it>p </it>= 0.013 and 9/32, <it>p </it>= 0.042, respectively) compared to the positive AdipoR1 group (lymphatic metastasis, 33/68; peritoneal dissemination, 8/68). On the other hand, AdipoR2 expression was only associated with histopathological type (<it>p </it>= 0.001). In survival analysis, the AdipoR1 positive staining group had significantly longer survival rates than the negative staining group (<it>p </it>= 0.01). However, multivariate analysis indicated that AdipoR1 was not an independent prognostic factor on patient's survival on gastric cancer.</p> <p>Conclusions</p> <p>In gastric cancer, adiponectin has the possibility to be involved in cell growth suppression via AdipoR1. The presence of AdipoR1 could be a novel anticancer therapeutic target in gastric cancer.</p

Does the unification of health financing affect the distribution pattern of out‐of‐pocket health expenses in Turkey?

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/149529/1/ijsw12389_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/149529/2/ijsw12389.pd