Private-Law Models for Official Immunity

A method for correcting matching errors in an ADC is presented. The method uses the unknown input data from the application and does not require any test signal. Two algorithms for implementing the method are compared. One algorithm is general and works on any type of ADC. The other algorithm utilizes the subranged architecture of a specific ADC and is very simple to implement in hardware. The signal quality is similar for both algorithms

Construction Management and Design-Build/Fast Track Construction From the Perspective of a Generla Contractor

In the accompanying paper a method for blind (i.e., no calibration needed) estimation and compensation of the time errors in a time interleaved ADC system was presented. In this paper we evaluate this method. The Cramer-Rao bound is calculated, both for additive noise and random clock jitter. Monte-Carlo simulations have also been done to compare to the CRB. Finally, the estimation method is validated on measurements from areal time interleaved ADC system with 16 ADCs

Exploring the Effect of Silicon on the High Temperature Corrosion of Lean FeCrAl Alloys in Humid Air

A new approach to reduce the chromium and aluminium concentrations in FeCrAl alloys without significantly impairing corrosion resistance is to alloy with 1-2 wt.% silicon. This paper investigates the "silicon effect" on oxidation by comparing the oxidation behavior and scale microstructure of two FeCrAl alloys, one alloyed with silicon and the other not, in dry and wet air at 600 degrees C and 800 degrees C. Both alloys formed thin protective oxide scales and the Cr-evaporation rates were small. In wet air at 800 degrees C the Si-alloyed FeCrAl formed an oxide scale containing mullite and tridymite together with alpha- and gamma-alumina. It is suggested that the reported improvement of the corrosion resistance of Al- and Cr-lean FeCrAl\u27s by silicon alloying is caused by the appearance of Si-rich phases in the scale

The Influence of Oxide-Scale Microstructure on KCl(s)-Induced Corrosion of Low-Alloyed Steel at 400 \ub0C

The high-temperature corrosion of low-alloyed steels and stainless steels in the presence of KCl(s) has been studied extensively in the last decades by several authors. The effect of KCl(s) on the initial corrosion attack has retained extra focus. However, the mechanisms behind the long-term behavior, e.g., when an oxide scale has already formed, in the presence of KCl(s) are still unclear. The aim of this study was to investigate the effect of the microstructure of a pre-formed oxide scale on low-alloyed steel (Fe–2.25Cr–1Mo) when exposed to small amounts of KCl(s). The pre-oxidation exposures were performed at different temperatures and durations in order to create oxide scales with different microstructures but with similar thicknesses. After detailed characterization, the pre-oxidized samples were exposed to 5%O2\ua0+\ua020%H2O\ua0+\ua075%N2 (+KCl(s)) at 400\ua0\ub0C for 24, 48, and 168\ua0h and analyzed with scanning electron microscopy, energy-dispersive X-ray spectroscopy, X-ray diffraction, and focused ion beam. The microstructural investigation indicated that Cl-induced corrosion is a combination of oxide thickness and microstructure, and the breakaway mechanism in the presence of KCl(s) is diffusion-controlled as porosity changes prior to breakaway oxidation were observed

Haploidentical stem cell transplantation in two children with mucopolysaccharidosis VI: clinical and biochemical outcome

Background: Mucopolysaccharidosis VI (MPS VI) is an autosomal recessive progressive multiorgan disorder due to mutation in the gene encoding the enzyme Arylsulfatase B (ARSB). Dysfunctional ARSB causes lysosomal accumulation of glycosaminoglycans (GAG). Currently, enzyme replacement therapy (ERT) is preferred to hematopoietic stem cell transplantation (SCT) due to the treatment-related risks of the latter. However, ERT constitutes an expensive life-long treatment. Increased experience and safety of SCT-procedures in recent years suggest that SCT should be further explored as a treatment option. This is the first report on haploidentical SCT in patients with MPS VI. The primary objective was to assess the treatment safety and clinical and biochemical outcome. Patients and methods: Two siblings diagnosed with MPS VI at 10 months of age and at birth with genotype p. C192R, reported as mild to intermediate phenotype, underwent unrelated umbilical cord blood transplantation pre-symptomatic. Due to graft failure, both patients were urgently re-transplantated with haploidentical SCT with the father as donor. Continuous clinical and biochemical status was monitored and concluded 3.8 and 4.6 years after the haploidentical SCT. Results: Haploidentical SCT resulted in prompt and sustained engraftment. Complete donor chimerism was achieved in both patients, apart from mixed B cells chimerism in patient 2. ARSB activity in leukocytes post transplant increased from 0.0 to 19.0 mu kat/kg protein (patient 1) and from 3.6 to 17.9 mu kat/kg protein (patient 2) (ref. 17-40). Total urinary GAG normalized in both patients, although patient 2's values slightly exceed normal range since 6 months. However, dermatan sulfaturia was substantially normalized since 16 months and 12 months post-SCT, respectively. Height was -1.85 SD and -1.27 SD at follow-up. Patient 1 had impaired visual acuity and discrete hepatomegaly. Patient 2 had elevated intraocular pressure and X-ray revealed steep acetabular angles and slightly flattened lumbar vertebrae. Conclusion: This study demonstrates that young children with MPS VI tolerate haploidentical SCT. Normalization of enzyme production and dermatan sulfaturia indicates correction of the inborn error of metabolism and coincide with no obvious symptoms of progressive MPS VI up to 4.6 years post-SCT

Fate of Antioxidative Compounds within Bark during Storage: A Case of Norway Spruce Logs

Softwood bark is an important by-product of forest industry. Currently, bark is under-utilized and mainly directed for energy production, although it can be extracted with hot water to obtain compounds for value-added use. In Norway spruce (Picea abies [L.] Karst.) bark, condensed tannins and stilbene glycosides are among the compounds that comprise majority of the antioxidative extractives. For developing feasible production chain for softwood bark extractives, knowledge on raw material quality is critical. This study examined the fate of spruce bark tannins and stilbenes during storage treatment with two seasonal replications (i.e., during winter and summer). In the experiment, mature logs were harvested and stored outside. During six-month-storage periods, samples were periodically collected for chemical analysis from both inner and outer bark layers. Additionally, bark extractives were analyzed for antioxidative activities by FRAP, ORAC, and H2O2 scavenging assays. According to the results, stilbenes rapidly degraded during storage, whereas tannins were more stable: only 5–7% of the original stilbene amount and ca. 30–50% of the original amount of condensed tannins were found after 24-week-storage. Summer conditions led to the faster modification of bark chemistry than winter conditions. Changes in antioxidative activity were less pronounced than those of analyzed chemical compounds, indicating that the derivatives of the compounds contribute to the antioxidative activity. The results of the assays showed that, on average, ca. 27% of the original antioxidative capacity remained 24 weeks after the onset of the storage treatment, while a large variation (2–95% of the original capacity remaining) was found between assays, seasons, and bark layers. Inner bark preserved its activities longer than outer bark, and intact bark attached to timber is expected to maintain its activities longer than a debarked one. Thus, to ensure prolonged quality, no debarking before storage is suggested: outer bark protects the inner bark, and debarking enhances the degradation

Fate of Antioxidative Compounds within Bark during Storage: A Case of Norway Spruce Logs

Softwood bark is an important by-product of forest industry. Currently, bark is under-utilized and mainly directed for energy production, although it can be extracted with hot water to obtain compounds for value-added use. In Norway spruce (Picea abies [L.] Karst.) bark, condensed tannins and stilbene glycosides are among the compounds that comprise majority of the antioxidative extractives. For developing feasible production chain for softwood bark extractives, knowledge on raw material quality is critical. This study examined the fate of spruce bark tannins and stilbenes during storage treatment with two seasonal replications (i.e., during winter and summer). In the experiment, mature logs were harvested and stored outside. During six-month-storage periods, samples were periodically collected for chemical analysis from both inner and outer bark layers. Additionally, bark extractives were analyzed for antioxidative activities by FRAP, ORAC, and H2O2 scavenging assays. According to the results, stilbenes rapidly degraded during storage, whereas tannins were more stable: only 5–7% of the original stilbene amount and ca. 30–50% of the original amount of condensed tannins were found after 24-week-storage. Summer conditions led to the faster modification of bark chemistry than winter conditions. Changes in antioxidative activity were less pronounced than those of analyzed chemical compounds, indicating that the derivatives of the compounds contribute to the antioxidative activity. The results of the assays showed that, on average, ca. 27% of the original antioxidative capacity remained 24 weeks after the onset of the storage treatment, while a large variation (2–95% of the original capacity remaining) was found between assays, seasons, and bark layers. Inner bark preserved its activities longer than outer bark, and intact bark attached to timber is expected to maintain its activities longer than a debarked one. Thus, to ensure prolonged quality, no debarking before storage is suggested: outer bark protects the inner bark, and debarking enhances the degradation

Efficacy of an external chromia layer in reducing nitridation of high temperature alloys

Six high temperature alloys have been exposed in N2/H2 environments at 900 \ub0C. In order to study the efficacy of a chromia barrier layer against nitrogen ingress, experiments were performed in two environments having the same N2/H2 ratio but slightly different water content, chromia formation being spontaneous in one case only. The samples were evaluated by SEM/STEM/EDX, XRD, gravimetry and GD-OES. The presence of an external chromia scale reduced nitridation of the alloy by 50–95%. Furthermore, in the presence of a continuous alumina layer no nitridation of the alloy was detected

De novo DHDDS variants cause a neurodevelopmental and neurodegenerative disorder with myoclonus

Subcellular membrane systems are highly enriched in dolichol, whose role in organelle homeostasis and endosomal-lysosomal pathway remains largely unclear besides being involved in protein glycosylation. DHDDS encodes for the catalytic subunit (DHDDS) of the enzyme cis-prenyltransferase (cis-PTase), involved in dolichol biosynthesis and dolichol-dependent protein glycosylation in the endoplasmic reticulum. An autosomal recessive form of retinitis pigmentosa (retinitis pigmentosa 59) has been associated with a recurrent DHDDS variant. Moreover, two recurring de novo substitutions were detected in a few cases presenting with neurodevelopmental disorder, epilepsy, and movement disorder. We evaluated a large cohort of patients (n=25) with de novo pathogenic variants in DHDDS and provided the first systematic description of the clinical features and long-term outcome of this new neurodevelopmental and neurodegenerative disorder. The functional impact of the identified variants was explored by yeast complementation system and enzymatic assay. Patients presented during infancy or childhood with a variable association of neurodevelopmental disorder, generalized epilepsy, action myoclonus/cortical tremor, and ataxia. Later in the disease course they experienced a slow neurological decline with the emergence of hyperkinetic and/or hypokinetic movement disorder, cognitive deterioration, and psychiatric disturbances. Storage of lipidic material and altered lysosomes were detected in myelinated fibers and fibroblasts, suggesting a dysfunction of the lysosomal enzymatic scavenger machinery. Serum glycoprotein hypoglycosylation was not detected and, in contrast to retinitis pigmentosa and other congenital disorders of glycosylation involving dolichol metabolism, the urinary dolichol D18/D19 ratio was normal. Mapping the disease-causing variants into the protein structure revealed that most of them clustered around the active site of the DHDDS subunit. Functional studies using yeast complementation assay and in vitro activity measurements confirmed that these changes affected the catalytic activity of the cis-PTase and showed growth defect in yeast complementation system as compared with the wild-type enzyme and retinitis pigmentosa-associated protein. In conclusion, we characterized a distinctive neurodegenerative disorder due to de novo DHDDS variants, which clinically belongs to the spectrum of genetic progressive encephalopathies with myoclonus. Clinical and biochemical data from this cohort depicted a condition at the intersection of congenital disorders of glycosylation and inherited storage diseases with several features akin to of progressive myoclonus epilepsy such as neuronal ceroid lipofuscinosis and other lysosomal disorders

A united statement of the global chiropractic research community against the pseudoscientific claim that chiropractic care boosts immunity.

BACKGROUND: In the midst of the coronavirus pandemic, the International Chiropractors Association (ICA) posted reports claiming that chiropractic care can impact the immune system. These claims clash with recommendations from the World Health Organization and World Federation of Chiropractic. We discuss the scientific validity of the claims made in these ICA reports. MAIN BODY: We reviewed the two reports posted by the ICA on their website on March 20 and March 28, 2020. We explored the method used to develop the claim that chiropractic adjustments impact the immune system and discuss the scientific merit of that claim. We provide a response to the ICA reports and explain why this claim lacks scientific credibility and is dangerous to the public. More than 150 researchers from 11 countries reviewed and endorsed our response. CONCLUSION: In their reports, the ICA provided no valid clinical scientific evidence that chiropractic care can impact the immune system. We call on regulatory authorities and professional leaders to take robust political and regulatory action against those claiming that chiropractic adjustments have a clinical impact on the immune system