Infantile malignant osteopetrosis (IMO) is an autosomal recessive disorder characterized by nonfunctional osteoclasts. Approximately 50% of the patients have mutations in the TCIRG1 gene, encoding for a subunit of the osteoclast proton pump. Gene therapy represents a potential alternative treatment to allogeneic stem cell transplantation for IMO. The oc/oc mouse is a model of IMO characterized by

A recently introduced technique of topology sensitivity in method of moments is extended by the possibility of adding degrees of freedom (reconstruct) into the underlying structure. The algebraic formulation is inversion-free, suitable for parallelization, and scales favorably with the number of unknowns. The reconstruction completes the nearest neighbors procedure for an evaluation of the smalles

Subarachnoid hemorrhage (SAH) is a type of hemorrhagic stroke with a high short-term mortality rate which leads to cognitive impairments that reduce the quality of life of the majority of patients. The miRNA-143/145 cluster is highly expressed in vascular smooth muscle cells (VSMC) and has been shown to be necessary for differentiation and function, as well as an important determinant for phenotyp

We evaluate the theoretical uncertainties in next-to-leading order plus parton shower predictions for top quark pair production and decay in hadronic collisions. Our work is carried out using the Herwig 7 event generator and presents an in-depth study of variations in matching schemes with two systematically different shower algorithms, the traditional angular-ordered and alternative dipole shower

BACKGROUND: The International Contact Dermatitis Research Group increased the patch test concentration of formaldehyde from 1.0% aqueous (aq) to 2.0% aq (in 2011). OBJECTIVE: This study was designed to investigate the outcome of the decision. METHODS: Consecutive dermatitis patients in 8 different clinics were patch tested with formaldehyde 1.0% aq and 2.0% aq. The test solutions were applied with

In this paper, we propose a novel framework for the analysis of task-related heart rate variability (HRV). Respiration and HRV are measured from 92 test participants while performing a chirp-breathing task consisting of breathing at a slowly increasing frequency under metronome guidance. A non-stationary stochastic model, belonging to the class of Locally Stationary Chirp Processes, is used to mod

The design of biorefineries based on farmyard manure remains unknown, because various groups have obtained disparate results. These differences have been attributed to the composition of the material—an explanation that this study intends to develop. Several biorefinery layouts were tested in the laboratory for animal beddings with various manure contents, and the same result was obtained in all c

This research aims to produce lignin‐based biodegradable polyesters with improved thermal quality. A series of aliphatic polyesters with lignin‐based aromatic side groups were synthesized by conventional melt‐polycondensation. Decent molecular weight (21–64 kg mol−1) was achieved for the polymerizations. The molecular structures and thermal and mechanical properties of the obtained polyesters were

A Kalman filter can be used to determine material parameters using uncertain experimental data. However, starting with inappropriate initial values for material parameters might cause false local attractors or even divergence. Also, inappropriate choices of covariance errors of the state and the measurements might affect the stability of the prediction. The present method suggests a simple way to

By using dislocation simulation approach, the basic equation was formulated. A novel expansion method was proposed for solving the problem. The complete solution to the problem, including the explicit formulae for the T stresses ahead of the crack tip and the stress intensity factors were presented. The stress field characteristics were analysed in detail. It is found that the normal stresses ahea

Wrought Alloy 600 and Alloy 182 weld metal were tested for stress corrosion crack initiation in a BWR test loop. For the tests a modified bolt loaded compact tension specimen was developed, in which a U-notch replaced the conventional crack notch. The stress state close to notches of various radii was analyzed. Specimens with various notch radii were loaded to the same displacement at the bolt. A

A potential drop method, as used for estimations of crack lengths during three‐point‐beind experiments, is studied. The mechanical state is calculated using a finite element method. The deformed body obtained is used for a subsequent calculation of the electrostatic state. Calculations are performed for both two‐ and three‐dimensional models. The material is assumed to be elastic, linearly hardeni

BackgroundAt least 50% of all ambulance assignments are deemed nonurgent, while 47–96% are initiated by someone other than the patient. Previous research has highlighted the importance of being taken seriously. However, additional knowledge of how significant others experience the situation when a patient is assessed as nonurgent is needed.ObjectiveThe aim of this study was to explore the person‐c

BACKGROUND: The indication, composition and timing of administration of non-resuscitation fluid in septic shock have so far received little attention and accordingly the potential to reduce this source of fluid is unknown. The objective of the study was to quantify and characterize non-resuscitation fluid administered to patients with septic shock.METHODS: This prospective observational study was

Study of British film Denial (2016), which is about the Irving-Lipstadt trial in London in 2000, where Holocaust denier David Irving sued American Judaist Deborah Lipstadt for libel, and lost.

In this essay it is argued that Bergman deviated from his highly critical depictions of bourgeois life in the films of the 1960s and 1970s, from Persona (1966) to the television series Scenes from a Marriage (1973), in Fanny and Alexander (1982), his final contribution to films made for the cinema. Bergman himself came from an upper-class bourgeois background, and by his own account he did not tak