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We report on some members of two unrelated families showing the characteristic features of Robinow syndrome. In a consanguineous Kuwaiti family, the index case with Robinow syndrome showed some unusual features including severe IUGR, laxity of ligaments, hyperextensible joints, redundant skin folds, severe normocytic anaemia, repeated infection, increased percentage of total T cells and CD4 positive population, reduced percentage of CD8 positive cells, and EMG abnormality. In a Pakistani family with a high degree of multigenerational consanguinity, a single case with the Robinow phenotype also had congenital heart disease, mainly involving the right side of the heart, with pulmonary stenosis, tricuspid atresia, ASD, VSD, double outlet right ventricle, and right atrial isomerism. This report suggests that the disease profile of Robinow syndrome may be extended to accommodate the unusual traits mentioned above. The association of the Robinow phenotype with congenital heart disease in case 2 of this report is consistent with the previously reported finding that congenital heart disease, particularly involving the right side of the heart, may be a prominent component of Robinow syndrome in a subset of patients.  相似文献   
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Telecommunication Systems - In the era of Internet-of-things (IoT), the future 5G networks are supposed to provide ubiquitous connectivity, high speed, as well as low latency and energy efficiency...  相似文献   
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Table of Contents

Table of Contents Volume 8 2001  相似文献   
4.
Dynamic unidirectional tensile ply properties were extracted from the results of burst tests on angle ply filament wound GRP and KRP tubes under internal hoop loading. The extracted longitudinal and transverse tensile strengths as well as transverse tensile moduli exhibited strain-rate sensitivities. Shear properties were derived from test results on 55° and 65° tube angles. Derived shear stress-strain curves and shear strength values are presented at different strain rates; again clearly demonstrating rate effects on these properties. Complete sets of strain rate dependent lamina tensile properties are presented for GRP and KRP covering the strain rate regime of 1 to 102 sec-1.  相似文献   
5.
An industrial‐scale dead‐end ultrafiltration system was optimized using statistically designed experiments. Given a certain level of pollutant, a two‐level full factorial design and a central composite design were used to optimize the filtrate production of a single 8‐inch industrial ultrafiltration membrane while manipulating the levels of four factors: feed pressure, backwash pressure, forward filtration time, and backwash time. Analysis of variance and residual analysis were used to validate and check the adequacy of the developed regression models. The optimal levels were later validated experimentally. The predicted filtrate production was in reasonable agreement with the experimental data.  相似文献   
6.
1. The present study was undertaken to determine whether various anaesthetic agents affect canine gastric acid secretion independently of other experimental variables. 2. Acid secretory output was determined in dogs with chronic fistulae, by administering sedating doses of anaesthetics commonly used for studying gastric secretory mechanisms in laboratory animals. 3. The anaesthetic agents inhibited gastric acid secretion. As the inhibitory effect of the mixture of anaesthetics was pronounced, an attempt was made to study the effect of each individual anaesthetic agent separately. 4. Acetopromazine was given to sedate dogs. Although it has a long duration of action, it only had a transient inhibitory action on gastric acid secretion of 15-30 min duration. Moreover the drug reduced pentagastrin-stimulated secretion, but had no effect on histamine-stimulated secretion. 5. Thiopentone sodium given with acetopromazine produced a mild inhibitory effect on histamine-stimulated secretion for 45 min, but produced a more pronounced and sustained inhibitory effect on pentagastrin-stimulated secretion. 6. Trilene significantly inhibited both histamine- and pentagastrin-stimulated secretion. The effect on the latter was more pronounced and sustained. 7. Trauma had no significant effect on histamine-stimulated secretion, but showed a slight inhibitory effect on pentagastrin-stimulated secretion. 8. Experiments to study gastric secretory mechanisms and antisecretory drugs should take account of the potential inhibitory effects of anaesthetics. Where possible, studies in conscious dogs with gastric fistulae are preferable to experiments on anaesthetized animals.  相似文献   
7.
This publication is the 1st in a series of publications by the Expert Panel of the Flavor and Extract Manufacturers Assoc. summarizing the Panel's 3rd re‐evaluation of Generally Recognized as Safe (GRAS) status referred to as the GRASr2 program. In 2011, the Panel initiated a comprehensive program to re‐evaluate the safety of more than 2700 flavor ingredients that have previously met the criteria for GRAS status under conditions of intended use as flavor ingredients. Elements that are fundamental to the safety evaluation of flavor ingredients include exposure, structural analogy, metabolism, pharmacokinetics, and toxicology. Flavor ingredients are evaluated individually and in the context of the available scientific information on the group of structurally related substances. Scientific data relevant to the safety evaluation of the use of aliphatic acyclic and alicyclic terpenoid tertiary alcohols and structurally related substances as flavoring ingredients are evaluated. The group of aliphatic acyclic and alicyclic terpenoid tertiary alcohols and structurally related substances was reaffirmed as GRAS (GRASr2) based, in part, on their rapid absorption, metabolic detoxication, and excretion in humans and other animals; their low level of flavor use; the wide margins of safety between the conservative estimates of intake and the no‐observed‐adverse effect levels determined from subchronic studies and the lack of significant genotoxic and mutagenic potential.  相似文献   
8.
This study investigates a parameter optimization approach to maximize the specific growth rate of the Chlorella vulgaris microalgae species, its biomass productivity, and CO2 capture rate. For this purpose, the Box‐Behnken experimental design technique is applied with temperature, nitrogen to phosphorus ratio, and light‐dark cycle per day, as the growth controlling parameters. For each response, a quadratic model is developed separately describing the algal specific growth rate, biomass productivity, and CO2 capture rate, respectively. The maximum specific growth rate of 0.84 d?1 is obtained at 25 °C, with a nitrogen to phosphorus ratio of 3.4:1, and light‐dark cycles of 24/0 h. Maximum biomass productivity of 147.3 mg L?1 d?1 is found at 30 °C, with a nitrogen to phosphorus ratio of 3:1, and light‐dark cycles of 12/12 h. In addition, the maximum CO2 capture rate of 159.5 mg L?1 d?1 is also obtained at 30 °C, with a nitrogen to phosphorus ratio of 4:1, and light‐dark cycles of 23/1 h. Finally, a multi‐response optimization method is applied to maximize the specific growth rate, biomass productivity, and CO2 capture rate, simultaneously. The optimal set of 30 °C, a nitrogen to phosphorus ratio 3:1, and light‐dark cycles 16/8 h, provide the maximum specific growth rate of 0.66 per day, biomass productivity of 147.6 mg L?1 d?1, and CO2 capture rate of 141.7 mg L?1 d?1.
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