Treatment of adrenoleukodystrophy with bone marrow transplantation

Three children with adrenoleukodystrophy (ALD) underwent allogeneic bone marrow transplantation (BMT) between 1992 and 1993. The first boy had attention deficits, marked neuropsychological deficits and widespread demyelination in the frontal lobes on MRI before transplantation. Four years later he has mentally deteriorated and the demyelination on MRI has progressed. The second boy had no symptoms but had white matter lesions on MRI when diagnosed. He was regularly followed with MRI and neuropsychological investigations until BMT 18 months later. A progress of the lesions was noted on the initial MRI investigations, and 4 months before BMT a worsening of deficits in attention and kinaesthetic praxis could be observed. He rapidly deteriorated after the transplantation and died 18 months later. Both PCR and in situ hybridization confirmed the presence of donor cells in the brain. The third boy had no symptoms but white matter lesions on MRI when diagnosed. The neuropsychological tests remained normal but a slight progress was observed on MRI just before transplantation. This boy is still healthy 3.5 years after BMT. BMT as treatment for ALD has to be considered very early, even if a child without symptoms but signs of demyelination on MRI, if a suitable donor is available.     

Grafting of unsaturated acids and silanes to ethylene polymers: A kinetic model

A general kinetic model was developed to simulate the grafting of monomers such as unsaturated carboxylic acids and silanes to ethylene polymers. The polymers considered were ethylene-co-vinyl acetate (EVA), ethylene-co-butyl acrylate (EBA), and low-density polyethylene (LDPE). Grafting was assumed to proceed by a free-radical mechanism involving chain transfer. Organic peroxides having a strong tendency for hydrogen abstraction were selected as the source of primary radicals. The model simulated the grafting reaction as taking place in a single screw extruder. The residence time distribution in the extruder was experimentally determined by tracer analysis, according to which the extruder was a plug-flow reactor connected to two mixed reactors in parallel. The model is able to predict the extent of grafting in terms of predefined parameters. Comparison of model predictions with available experimental data showed slight deviations, the possible causes of which are discussed. However, the kinetic behavior expected on the basis of the input parameters was observed, and, as such, the model allowed study of the effect of process variables on grafting kinetics and provided insight into the reaction mechanism.     

Recurrent DNA copy number changes in 1q, 4q, 6q, 9p, 13q, 14q and 22q detected by comparative genomic hybridization in malignant mesothelioma

Comparative genomic hybridization (CGH) analyses were performed on 27 human pleural mesothelioma tumour specimens, consisting of 18 frozen tumours and nine paraffin-embedded tumours, to screen for gains and losses of DNA sequences. Copy number changes were detected in 15 of the 27 specimens with a range from one to eight per specimen. On average, more losses than gains of genetic material were observed. The loss of DNA sequences occurred most commonly in the short arm of chromosome 9 (p21-pter), in 60% of the abnormal specimens. Other losses among the abnormal specimens were frequently detected in the long arms of chromosomes 4 (q31.1-qter, 20%), 6 (q22-q24, 33%), 13 (33%),14 (q24-qter, 33%) and 22 (q13, 20%). A gain in DNA sequences was found in the long arm of chromosome 1 (cen-qter) in 33% of the abnormal specimens. Our analysis is the first genome-wide screening for gains and losses of DNA sequences using comparative genomic hybridization in malignant pleural mesothelioma tumours. The recurrent DNA sequence changes detected in this study suggest that the corresponding chromosomal areas most probably contain genes important for the initiation and progression of mesothelioma.     

Copolymers of ethylene with butene-1 and long chain α-olefins. I. Decene-1 as long chain α-olefin

The possibility was studied of using decene-1 as comonomer with ethylene in a slurry type polymerization with Ziegler–Natta catalysts. Under the reaction conditions used decene contents remained at the 2 wt% level in ethylene/decene-1 copolymers. When additionally butene-1 was present in the polymerization, decene-1 contents were significantly higher. A synergistic effect was identified in the reactivities of butene-1 and decene-1 in terpolymerization with ethylene. The comonomer reactions were determined and comonomer contents measured by ¹³C-NMR spectroscopy. Decene-1 content had an effect on the polymer density and crystallinity, but virtually no effect on melting temperature. With high comonomer contents an additional melting range was identified in DSC curves at about 100°C.     

Modeling and Simulation of High Pressure Water Mist Systems

This paper describes work done to improve and validate the capability of fire dynamics simulator (FDS) to predict the dynamics of water mist sprays. Three single orifice and five multi-orifice spray heads are modeled with FDS based on information on the flow-rate, spray angle, operating pressure and experimentally determined particle size distribution. The capability of FDS to predict the drop size, velocity, mist flux and number concentration profiles within the spray cone is assessed. The effects of turbulence modeling on the predictions of the spray dynamics is investigated. The capability of FDS to predict the air entrainment by high-speed water sprays is validated using experiments in rectangular channels with open ends.     

Will New Antenna Materials Enable Single Path Multiple Access (SPMA)?

The aim of this paper is to introduce a novel frequency reuse concept especially for macro cellular networks to substantially increase the mobile network capacity, and simultaneously to avoid the implementation of low efficient small cells. Single path multiple access (SPMA) utilizes the characteristics of independent propagation paths for particular geographical location in the coverage area of mobile network. The proposed concept is based on the assumption that new approach will be adopted by the antenna manufacturers for producing advanced antennas by utilizing materials like metamaterials including carbon based nanotechnology, and graphene. In SPMA concept, communication between base station and mobile station happens through only single independent propagation path, and frequency resources can be reused in 5 m  \(\times \)  5 m areas or even more often in 1 m  \(\times \)  1 m areas, but limited by a base station/mobile station antenna requirement. Thus, the capacity of the network will be increased dramatically, and it can be managed in centralized manner at certain macro site locations. In already deployed cellular networks, these macro sites are mostly easily available, and that would help to implement SPMA to enhance the network capacity. Simulation results provided in this paper show the applicability of SPMA technique, by limiting the radiation of signal as single path propagation between base station and mobile station.     

Light transmission model for determination of the refractive index of solid particles in suspensions: an immersion study

We report on a model to determine the light transmission of solid particles in immersion liquids. Using the model, optical and physical properties of suspensions such as the refractive index of the particles can be retrieved. The model can also be used in the case of colored suspensions, where the detection of only the maximum transmission would give an inaccurate refractive index for a particle. The validity of the method was tested with CaF(2) particles. Potential applications include scientific studies of the optical properties of micro- and nanoparticles or microorganisms in a liquid matrix. The model is also useful in industry where monitoring of suspensions and colloids is an issue.     

Comparison between infrared and Raman spectroscopic analysis of maturing rabbit cortical bone

The molecular composition of the organic and inorganic matrices of bone undergoes alterations during maturation. The aim of this study was to compare Fourier transform infrared (FT-IR) and near-infrared (NIR) Raman microspectroscopy techniques for characterization of the composition of growing and developing bone from young to skeletally mature rabbits. Moreover, the specificity and differences of the techniques for determining bone composition were clarified. The humeri of female New Zealand White rabbits, with age range from young to skeletally mature animals (four age groups, n = 7 per group), were studied. Spectral peak areas, intensities, and ratios related to organic and inorganic matrices of bone were analyzed and compared between the age groups and between FT-IR and Raman microspectroscopic techniques. Specifically, the degree of mineralization, type-B carbonate substitution, crystallinity of hydroxyapatite (HA), mineral content, and collagen maturity were examined. Significant changes during maturation were observed in various compositional parameters with one or both techniques. Overall, the compositional parameters calculated from the Raman spectra correlated with analogous parameters calculated from the IR spectra. Collagen cross-linking (XLR), as determined through peak fitting and directly from the IR spectra, were highly correlated. The mineral/matrix ratio in the Raman spectra was evaluated with multiple different peaks representing the organic matrix. The results showed high correlation with each other. After comparison with the bone mineral density (BMD) values from micro-computed tomography (micro-CT) imaging measurements and crystal size from XRD measurements, it is suggested that Raman microspectroscopy is more sensitive than FT-IR microspectroscopy for the inorganic matrix of the bone. In the literature, similar spectroscopic parameters obtained with FT-IR and NIR Raman microspectroscopic techniques are often compared. According to the present results, however, caution is required when performing this kind of comparison.     

Ion-implanted capacitively coupled silicon strip detectors with integrated polysilicon bias resistors processed on a 100 mm wafer

Double-sided silicon strip detectors with integrated coupling capacitors and polysilicon resistors have been processed on a 100 mm wafer. A detector with an active area of 19 × 19 mm² was connected to LSI readout electronics and tested. The strip pitch of the detector is 25 μm on the p-side and 50 μm on the n-side. The readout pitch is 50 μm on both sides. The number of readout strips is 774 and the total number of strips is 1161. On the p-side a signal-to-noise of 35 has been measured using a ⁹⁰Sr β-source. The n-side has been studied using a laser.     

Comparison of benign and malignant follicular thyroid tumours by comparative genomic hybridization

DNA copy number changes were compared in 29 histologically benign follicular adenomas, of which five were atypical, and 13 follicular carcinomas of the thyroid by comparative genomic hybridization. DNA copy number changes were frequent in adenomas (14 out of 29, 48%). Most changes were gains, and they always involved a gain of the entire chromosome 7 (10 out of 29, 34%); other common gains involved chromosomes 5 (28%), 9 (10%), 12 (24%), 14 (21%), 17 (17%), 18 (14%) and X (17%). Losses were found only in four (14%) adenomas. Two of the five atypical adenomas had DNA copy number losses, and none had gains. Unlike adenomas, gains were rare and losses were frequent in carcinomas. A loss of chromosome 22 or 22q was particularly common in carcinomas (6 out of 13, 46%), whereas a loss of chromosome 22 was found in only two (7%) adenomas, one of which was atypical (P = 0.002). A loss of 1p was also frequent in carcinomas (31%), but gains of chromosomes 5, 7, 12, 14 or X that were common in adenomas were not found. Loss of chromosome 22 or 22q was present in six of the eight widely invasive follicular carcinomas, but in only one of the five minimally invasive carcinomas. We conclude that large DNA copy number changes are common in thyroid adenomas. These changes are strikingly different from those found in follicular carcinomas consisting of few losses and frequent gains, especially those of chromosome 7. A loss of chromosome 22 is common in widely invasive follicular carcinoma.