Background
Sjögren‐Larsson syndrome is a rare inherited neurocutaneous disorder characterized by congenital ichthyosis, spasticity, intellectual disability, seizures, and ophthalmologic changes. Most ...individuals with Sjögren‐Larsson syndrome live well into adulthood and often require surgical intervention to manage their symptomatology.
Aims
The aim of this work was to review the clinical aspects of Sjögren‐Larsson syndrome, highlight the unique anesthetic considerations associated with this disease, and provide practical recommendations about anesthetic management.
Methods
A retrospective case review from February 2013 to October 2019 was performed based on subject participation in a Sjögren‐Larsson syndrome longitudinal study at the University of Nebraska Medical Center. Anesthetic and surgical records were reviewed for the following data: age, sex, relevant comorbid conditions, anesthetic induction and maintenance agents, intravenous and oral analgesics, muscle relaxants, and anesthetic‐related complications.
Results
Fourteen patients with Sjögren‐Larsson syndrome undergoing 48 anesthetic events were identified. A variety of anesthetic techniques was utilized. No serious adverse events were encountered. The most common clinical observations were related to the ichthyosis seen in Sjögren‐Larsson syndrome, which led to difficulty in adherence of electrocardiogram leads and intravenous catheter dressings.
Conclusions
We found that anesthesia can be safely administered in patients with Sjögren‐Larsson syndrome. Providers should be aware of anesthetic management issues in Sjögren‐Larsson syndrome including challenges placing and securing lines and monitors secondary to the ichthyosis.
Gabapentin (1-(aminomethyl)cyclohexaneacetic acid) is a neuroprotective agent with antiepileptic properties. The structure is small (molecular weight less than 200), is zwitterionic, and resembles an ...amino acid with the exception that it does not contain a chiral carbon and the amino group is not alpha to the carboxylate functionality. Gabapentin is not metabolized by humans, and thus, the amount of gabapentin excreted by the renal route represents the fraction of dose absorbed. Clinical trials have reported dose-dependent bioavailabilities ranging from 73.8 +/- 18.3 to 35.7 +/- 18.3% when the dose was increased from 100 to 1600 mg. The permeability of gabapentin in the rat intestinal perfusion system was consistent with carrier-mediated absorption, i.e., a 75 to 80% decrease in permeability when the drug concentration was increased from 0.01 to 50 mM (0.46 +/- 0.05 to 0.12 +/- 0.04). Excellent agreement was obtained between the actual clinical values and the predicted values from in situ results for the fraction of dose absorbed calculated using the theoretically derived correlation, Fabs = 1 - exp(-2Peff) by Amidon et al. (Pharm. Res. 5:651-654, 1988). The permeability values obtained for gabapentin correspond to 67.4 and 30.2% of the dose absorbed at the low and high concentrations, respectively. In the everted rat intestinal ring system, gabapentin shared an inhibition profile similar to that of L-phenylalanine. Characteristics of gabapentin uptake included cross-inhibition with L-Phe, sensitivity to inhibition by L-Leu, stereoselectivity as evidenced by incomplete inhibition by D-Phe, and lack of effect by Gly.
The collaboration Energy and Transmutation of Radioactive Waste uses different setups consisting of lead, uranium and graphite irradiated by relativistic protons and deuterons to study transmutation ...of radioactive materials by produced neutrons. Our group measured spatial distribution of neutrons by means of activation samples during the assembly irradiation by the JINR Nuclotron beams. We also present results of simulations using MCNPX code and their comparison with obtained experimental data. We use Au, Al, Bi, In and Ta foils as activation detectors, but unfortunately almost no experimental cross-section data for observed threshold (n,xn) reactions are available for higher neutron energies. Therefore we carried out series experiments devoted to determination of neutron cross-sections of various threshold reactions using different quasi-monoenergetic neutron sources.
This study evaluated the extent to which two systematic observation protocols which were modified for underserved communities (low income, minorities) could be utilized to reliably assess (a) use of ...walking trails and (b) physical environmental features of these trails. This study was a supplement to the Positive Action for Today’s Health (PATH) walking trial. The modified tools were shown to be reliable methods for (a) measuring trail use and (b) assessing physical features of the trail in underserved environments. Reliability data for measuring trail use were found to be high (ICC=.98, p<.01). Reliabilities for measuring features of the trail ranged from fair to highly reliable (κ=.77−1.00; ICC=.34–1.00). The observation tools that were customized for this study were shown to be reliable instruments for measuring trail use and assessing physical features of walking trails in underserved communities.
► Modified two systematic observation protocols for use in underserved communities. ► Modified protocols assessed use of a walking trail and their physical features. ► Reliability for measuring trail use was found to be high (ICC=.98, p<.01). ► Reliability for measuring trail features was κ=.77–1.00; ICC=.34−1.00. ► Modified protocols were shown to be reliable for use in underserved communities.
We examine the link between trade liberalization and aggregate productivity, with a focus on improved market selection resulting from a reduction in trade barriers and in the dispersion of these ...barriers across producers. Our analysis exploits tariff changes across sectors after the Colombian trade reform. An additional advantage of our analysis is that our TFP measure does not include demand and price effects. We find that reduced trade protection makes plant survival depend more closely on productivity. Using a dynamic simulation, we find that enhanced selection increases aggregate productivity substantially. Trade liberalization also increases productivity of incumbent plants and improves the allocation of activity. We find larger effects on allocative efficiency with our TFP measure than with a traditional measure including price effects.
We describe a strategy for regionalizing subnational administrative units in conjunction with harmonizing changes in unit boundaries over time that can be applied to provide small-area geographic ...identifiers for census microdata. The availability of small-area identifiers blends the flexibility of individual microdata with the spatial specificity of aggregate data. Regionalizing microdata by administrative units poses a number of challenges, such as the need to aggregate individual scale data in a way that ensures confidentiality and issues arising from changing spatial boundaries over time. We describe a regionalization and harmonization strategy that creates units that satisfy spatial and other constraints while maximizing the number of units in a way that supports policy and research use. We describe this regionalization strategy for three test cases of Malawi, Brazil, and the United States. We test different algorithms and develop a semi-automated strategy for regionalization that meets data restrictions, computation, and data demands from end users.
•Small-area geographic identifiers on microdata enhance their spatial utility.•We develop a harmonization-regionalization strategy for microdata geographic units.•Harmonization is conducted before regionalization to protect confidentiality.•Full CLK and Ward regionalization algorithms perform best, depending on context.
The HADES spectrometer currently operating on the beam of SIS18 accelerator in GSI will be moved to a new position in the CBM cave of the future FAIR complex. Electromagnetic calorimeter (ECAL) will ...enable the HADES@FAIR experiment to measure data on neutral meson production in heavy ion collisions at the energy range of 2-10 A GeVon the beam of the new accelerator SIS100. Calorimeter will be based on 978 massive lead glass modules read out by photomultipliers and a novel front-end electronics. Secondary gamma beam with energies ranging from 81 MeV up to 1399 MeV from MAMI-C Mainz facility was used to verify selected technical solutions. Relative energy resolution was measured using modules with three different types of photomultipliers. Two types of developed front-end electronics as well as energy leakage between neighbouring modules under parallel and declined gamma beams were studied in detail.
The cross-sections of relativistic deuteron reactions on natural copper were studied by the means of activation method. The deuteron beams produced by JINR Nuclotron (Russia) with energies from 1 GeV ...up to 8 GeV were used. Lack of such cross-sections prevents the usage of copper foils for beam integral monitoring. The copper monitors will help us to improve the beam integral determination during ADS studies. The yttrium samples are very suitable activation detectors for monitoring of neutron fields not only in the ADS studies. But experimental cross-section data for higher energy threshold neutron reactions are still missing. This situation is the reason why we have started to study neutron reactions on yttrium by the means of quasi mono-energetic neutron source based on NPI ez cyclotron (Czech Republic).
We measured neutron cross-sections of various threshold reactions usingdifferent quasi-monoenergetic sources in the range 17 - 94 MeV. Ourmotivation comes from the ``Energy plus Transmutation ...project'', in whichAl, Au, Bi, In, Ta, Co, Y foils are used to measure the flux of high energyneutrons produced in spallation reactions. Unfortunately, no experimentalcross-section data for observed threshold (n,xn) reactions are available atneutron energies above 20 MeV.We performed 11 measurements of the (n,xn) cross-sections using the neutronsource at NPI ASCR cyclotron in ??and at TSL cyclotron in Uppsala(EFNUDAT program). Quasi-monoenergetic neutrons from p+Li reactionirradiated above mentioned foils. Irradiated foils were measured on HPGedetectors and spectroscopic corrections were applied to obtain cross-sectiondata. A comparison with TALYS cross-section calculations was performed. KCI Citation Count: 4
Estimates for the U.S. suggest that at least in some sectors productivity enhancing reallocation is the dominant factor in accounting for productivity growth. An open question, particularly relevant ...for developing countries, is whether reallocation is always productivity enhancing. It may be that imperfect competition or other barriers to competitive environments imply that the reallocation process is not fully efficient in these countries. Using a unique plant-level longitudinal dataset for Colombia for the period 1982–1998, we explore these issues by examining the interaction between market allocation, and productivity and profitability. Moreover, given the important trade, labor and financial market reforms in Colombia during the early 1990s, we explore whether and how the contribution of reallocation changed over the period of study. Our data permit measurement of plant-level quantities and prices. Taking advantage of the rich structure of our price data, we propose a sequential methodology to estimate productivity and demand shocks at the plant level. First, we estimate total factor productivity (TFP) with plant-level physical output data, where we use downstream demand to instrument inputs. We then turn to estimating demand shocks and mark-ups with plant-level price data, using TFP to instrument for output in the inverse-demand equation. We examine the evolution of the distributions of TFP and demand shocks in response to the market reforms in the 1990s. We find that market reforms are associated with rising overall productivity that is largely driven by reallocation away from low- and towards high-productivity businesses. In addition, we find that the allocation of activity across businesses is less driven by demand factors after reforms. We find that the increase in aggregate productivity post-reform is entirely accounted for by the improved allocation of activity.
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