Infantile spasms as the initial symptom of biotinidase deficiency

Two patients with biotinidase deficiency had diagnoses of infantile spasms made at 1 month of age. Biotinidase deficiency may be seen early in the neonatal period without the characteristic findings such as alopecia and seborrheic dermatitis. This diagnosis should be considered in patients with infantile spasms.     

Measurements of the total molecular differential attenuation coefficients of some compounds for scattering of γ-rays

The differential attenuation coefficients for total scattering have been measured using the Si(Li) detector at photon excitation energy of 59.5 keV for Fe₂O₃, Y₂O₃, Nb₂O₅, SeO₂, Sb₂O₅ and BaO compounds at seven angles ranging from 60° to 120° at intervals of 10°. The obtained results have been compared with relativistic and non-relativistic theoretical values.     

Efficiency analysis of a hybrid copper–chlorine (Cu–Cl) cycle for nuclear-based hydrogen production

The role of the Cu–Cl cycle for thermochemical water decomposition, potentially driven by heat from a nuclear power generation station, in producing hydrogen in a sustainable way is investigated by examining efficiencies. The energy efficiency of the cycle is found to be 45% and the exergy efficiency 10%. The energy and exergy efficiencies of the cycle are observed to vary with temperatures of the reactions and reference-environment, and cycle heat losses. A parametric study is carried out considering several heat losses, reaction and reference-environment temperatures, and component-efficiency scenarios.     

Placental villous stroma as a model system for myofibroblast differentiation

Different subtypes of myofibroblasts have been described according to their cytoskeletal protein patterns. It is quite likely that these different subtypes represent distinct steps of differentiation. We propose the human placental stem villi as a particularly suitable model to study this differentiation process. During the course of pregnancy, different types of placental villi develop by differentiation of the mesenchymal stroma surrounding the fetal blood vessels. In order to characterise the differentiation of placental stromal cells in the human placenta, the expression patterns of the cytoskeletal proteins vimentin, desmin, alpha- and gamma-smooth muscle actin, pan-actin, smooth muscle myosin, and the monoclonal antibody GB 42, a marker of myofibroblasts, were investigated on placental tissue of different gestational age (7th-40th week of gestation). Proliferation patterns were assessed with the proliferation markers MIB 1 and PCNA. Additionally, dipeptidyl peptidase IV distribution was studied in term placenta and the ultrastructure of placental stromal cells was assessed by electron microscopy. Different subpopulations of extravascular stromal cells were distinguished according to typical co-expression patterns of cytoskeletal proteins. Around the fetal stem vessels in term placental villi they were arranged as concentric layers with increasing stage of differentiation. A variable layer of extravascular stromal cells lying beneath the trophoblast expressed vimentin (V) or vimentin and desmin (VD). They were mitotically active. The next layer co-expressed vimentin, desmin, and alpha-smooth muscle actin (VDA). More centrally towards the fetal vessels, extravascular stromal cells co-expressed vimentin, desmin, alpha- and gamma-smooth muscle actin, and GB 42 (VDAG). Cells close to the fetal vessels additionally co-expressed smooth muscle myosin (VDAGM). Ultrastructurally, V cells resembled typical mesenchymal cells. VD cells corresponded to fibroblasts, while VDA and VDAG cells developed features of myofibroblasts. Cells of the VDAGM-type revealed a smooth muscle cell-related ultrastructure. In earlier stages of pregnancy, stromal cell types with less complex expression patterns prevailed. The media smooth muscle cells of the fetal vessels showed a mixture of different co-expression patterns. These cells were separated from extravascular stromal cells by a layer of collagen fibres. The results obtained indicate a clearly defined spatial differentiation gradient with increasing cytoskeletal complexity in human placental stromal cells from the superficial trophoblast towards the blood vessels in the centre of the stem villi. The spatial distribution of the various stages of differentiation suggests that human placental villi could be a useful model for the study of the differentiation of myofibroblasts.     

Immunohistochemistry of matrix metalloproteinases (MMP), their substrates, and their inhibitors (TIMP) during trophoblast invasion in the human placenta

The invasion of extravillous trophoblast cells into the maternal endometrium is one of the key events in human placentation. The ability of these cells to infiltrate the uterine wall and to anchor the placenta to it as well as their ability to infiltrate and to adjust utero-placental vessels to pregnancy depends, among other things, on their ability to secrete enzymes that degrade the extracellular matrix. Most of the latter enzymes belong to the family of matrix metalloproteinases. Their activity is regulated by the tissue inhibitors of matrix metalloproteinases. We have studied the distribution patterns of matrix metalloproteinases-1, -2, -3, and -9 and their inhibitors TIMP-1 and TIMP-2 as compared to the distribution of their substrates along the invasive pathway of extravillous trophoblast of 1st, 2nd, and 3rd trimester placentas by means of light microscopy on paraffin and cryostat sections as well as at the ultrastructural level (only 3rd trimester placenta). The comparison of different methods proved to be necessary, since the immunohistochemical distribution patterns of these soluble enzymes are considerably influenced by the pretreatment of tissues. All three methods revealed immunoreactivities of both, proteinases and their inhibitors, not only intracellularly in the extravillous trophoblast but also extracellularly in its surrounding matrix, the distribution patterns depending on the stage of pregnancy and on the degree of differentiation of trophoblast cells along their invasive pathway. Within the extracellular matrix, immunolocalization of matrix metalloproteinases as well as their inhibitors showed a specific relation to certain extracellular matrix molecules.     

Reduction of Chromite in Liquid Fe-Cr-C-Si Alloys

The kinetics and the mechanism of the reduction of chromite in Fe-Cr-C-Si alloys were studied in the temperature range of 1534 °C to 1702 °C under an inert argon atmosphere. The rotating cylinder technique was used. The melt consisted of 10 and 20 wt Pct chromium, the carbon content varied from 2.8 wt Pct to saturation, and the silicon content varied from 0 to 2 wt Pct. The rotational speed of the chromite cylinder ranged from 100 to 1000 rpm. The initial chromium to iron ratios of the melts varied between 0.11 and 0.26. In Fe-C melts, the effect of rotational speed on the reduction of chromite was very limited. Carbon saturation (5.4 wt Pct) of the alloy caused the reduction to increase 1.5 times over the reduction observed in the unsaturated (4.87 wt Pct) alloy at a given rotational speed. The addition of chromium to the carbon-saturated Fe-C alloy increased the reduction rate. The addition of silicon to the liquid phase increased the reduction rate drastically. The reduction of chromite in Fe-Cr-C melts is hindered because of the formation of, approximately, a 1.5-mm-thick M₇C₃-type carbide layer around the chromite cylinders. This carbide layer did not form when silicon was present in the melt. It was found that the reduction rate is controlled by the liquid-state mass transfer of oxygen. The calculated apparent activation energies for diffusion were 102.9 and 92.9 kJ/mol of oxygen in the Si-O and C-O systems, respectively.     

Experimental study of heat transfer of buried finned pipe for ground source heat pump applications

Ground heat exchangers have vital importance for ground source heat pump applications. Various configurations tried to improve heat transfer in the soil. A new kind of aluminium finned pipe buried in the soil for this aim. In order to compare effectiveness of the Al finned pipe over the traditional PPRC pipe an experimental study carried out. The experimental GSHP system was installed at Y?ld?z Technical University Davupasa Campus on 800 m² surface area with no special surface cover. Temperature data were collected using thermocouples buried in soil horizontally and vertically at various distances from the pipe center and at the inlet and the outlet of the ground heat exchanger. Experimental results were compared with results from analytical study. To compare effectiveness of the Al finned pipe and PPRC pipe a new parameter defined as transferred amount of heat per unit mass of working fluid per unit time for this aim. It is found that Al finned pipe has higher heat transfer values than the traditional PPRC pipe.     

Investigating the effect of production process of ball mill refiner on some physical quality parameters of compound chocolate: response surface methodology approach

Chocolate compound was produced using ball mill refiner, and the effect of agitator shaft speed and refining time on the physical quality parameters (particle size, colour and steady‐state rheology) of compound chocolate was determined using response surface methodology. The shaft speed and refining time range were selected between 40–60 r.p.m. and 10–30 min, respectively. Determination coefficient of the models established for particle size, Newtonian viscosity and colour parameters (brightness, chroma and hue angle) were found to be very close to unity. Increasing shaft speed and time induced a reduction in particle size and an increase in viscosity of the samples. Temperature sweep test was also performed, and the obtained data were successfully fitted to Arrhenius equation to calculate the corresponding parameters representing temperature dependency of the compounds. The results highlighted that the establishment of such models can provide essential information in terms of optimisation of production processes regarding usage purpose of the compound chocolate.     

Determination of Antioxidant Properties of Fruit Juice by Partial Least Squares and Principal Component Regression

The multivariate calibration methods—principal component regression and partial least squares—were employed for the prediction of antioxidant capacities of fruit juices. High-performance liquid chromatography and spectrophotometric approaches were used to determine the antioxidant capacities of fruit juices. The importance of calibration design was investigated by calculating the prediction and validation errors. The influences of using independent validation sets were emphasized. Calibration design is shown to have major effect on principal component regression and partial least squares errors. The models developed on the basis of the mean-centered data were able to predict the total antioxidant activity with a precision comparable to that of the reference [2,2-azino-di-(3-ethylbenzothialozine-sulfonic acid)] method. The partial least squares model seems preferable because of its predictive and describing abilities and good interpretability of the contribution of compounds to the antioxidant capacity. The contribution of individual phenolic compounds to the antioxidant capacity was identified by high-performance liquid chromatography.     

Geochemical characteristics of major and trace elements in Sahinali Coals,Aydin, Turkey

In this study, geochemical behavior of elements is investigated in 25 coal samples collected at 10 locations of ?ahinli coaliferous units at southeast of Ayd?n, Turkey. In all the samples, X-ray powder diffraction and inductively coupled plasma mass spectrometry analyses were performed and element and mineral compositions were determined. Total organic carbon (TOC) values of coals are between 14.6 and 37.7 wt% with average of 28.5 wt%. Samples are composed of quartz, calcite, feldspar, dolomite, mica, gypsum, and salt minerals which are accompanied by illite-, kaolinite-, and smectite-type clay minerals. Comparison of average element contents of world coals and studied samples yields that K > Fe > As > U > Cs > Y > Er > Al > Th > Rb > Yb > W > Tb > Dy > Lu > Ce > Sm > La > Ho > Si > Nd > Gd > Tm > Eu > Nb > Pr > Pb > Sn > Ni > Cu > Ga > Ta > Sb > Zr > Cd > V > Zn > Co > Mo > P > Hf > Ba are found to be enriched, while Mn > Be > Ca > Sr > Bi > Mg are found to be depleted. Statistically elements are correlated with TOC.