bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neonatal screening for glutaric aciduria type I: Strategies to proceed
Authors: Lindner M. , Ho S. , Fang‐Hoffmann J. , Hoffmann G. F. , Kölker S. 
Published: June 2006

SummaryAcute encephalopathic crisis in glutaric aciduria type I results in an unfavourable disease course and poor outcome, dominated by dystonia, feeding problems, seizures and reduced life expectancy. A conditio sine qua non for the prevention of irreversible brain damage is timely diagnosis and start of therapy, i.e. before the onset of neurological disease. As there are no specific clinical signs or symptoms that allow a reliable detection of these patients before the manifestation of encephalopathic crises, neonatal screening programmes for glutaric aciduria type I have been established in some countries using analysis of glutarylcarnitine in dried blood spots by tandem mass spectrometry. This article summarizes recent strategies, pitfalls and shortcomings of mass screening for glutaric aciduria type I, focusing on the relevant risk of missing patients with a mild biochemical phenotype (i.e. low excretors). Furthermore, it evaluates a binary strategy – using glutarylcarnitine as primary variable and glutarylcarnitine/acylcarnitine ratios as secondary variable – to improve the diagnostic sensitivity and specificity of neonatal screening for glutaric aciduria type I. An optimization of diagnostic as well as therapeutic procedures must be achieved before screening for glutaric aciduria type I can be regarded as reliable and beneficial for all patients.
Clinical approach to treatable inborn metabolic diseases: An introduction
Authors: Saudubray J.‐M. , Sedel F. , Walter J. H. 
Published: June 2006

SummaryIn view of the major improvements in treatment, it has become increasingly important that in order for first‐line physicians not to miss a treatable disorder they should be able initiate a simple method of clinical screening, particularly in the emergency room. We present a simplified classification of treatable inborn errors of metabolism in three groups. Group 1 includes inborn errors of intermediary metabolism that give rise to an acute or chronic intoxication. It encompasses aminoacidopathies, organic acidurias, urea cycle disorders, sugar intolerances, metal disorders and porphyrias. Clinical expression can be acute or systemic or can involve a specific organ, and can strike in the neonatal period or later and intermittently from infancy to late adulthood. Most of these disorders are treatable and require the emergency removal of the toxin by special diets, extracorporeal procedures, cleansing drugs or vitamins. Group 2 includes inborn errors of intermediary metabolism that affect the cytoplasmic and mitochondrial energetic processes. Cytoplasmic defects encompass those affecting glycolysis, glycogenosis, gluconeogenesis, hyperinsulinisms, and creatine and pentose phosphate pathways; the latter are untreatable. Mitochondrial defects include respiratory chain disorders, and Krebs cycle and pyruvate oxidation defects, mostly untreatable, and disorders of fatty acid oxidation and ketone bodies that are treatable. Group 3 involves cellular organelles and includes lysosomal, peroxisomal, glycosylation, and cholesterol synthesis defects. Among these, some lysosomal disorders can be efficiently treated by enzyme replacement or substrate reduction therapies. Physicians can be faced with the possibility of a treatable inborn error in an emergency, either in the neonatal period or late in infancy to adulthood, or as chronic and progressive symptoms – general (failure to thrive), neurological, or specific for various organs or systems. These symptoms are summarized in four tables. In addition, an extensive list of medications used in the treatment of inborn errors is presented.
Practical type inference for arbitrary-rank types
Authors: JONES SIMON PEYTON , VYTINIOTIS DIMITRIOS , WEIRICH STEPHANIE , SHIELDS MARK 
Published: June 2006

AbstractHaskell's popularity has driven the need for ever more expressive type system features, most of which threaten the decidability and practicality of Damas-Milner type inference. One such feature is the ability to write functions with higher-rank types – that is, functions that take polymorphic functions as their arguments. Complete type inference is known to be undecidable for higher-rank (impredicative) type systems, but in practice programmers are more than willing to add type annotations to guide the type inference engine, and to document their code. However, the choice of just what annotations are required, and what changes are required in the type system and its inference algorithm, has been an ongoing topic of research. We take as our starting point a λ-calculus proposed by Odersky and Läufer. Their system supports arbitrary-rank polymorphism through the exploitation of type annotations on λ-bound arguments and arbitrary sub-terms. Though elegant, and more convenient than some other proposals, Odersky and Läufer's system requires many annotations. We show how to use local type inference (invented by Pierce and Turner) to greatly reduce the annotation burden, to the point where higher-rank types become eminently usable. Higher-rank types have a very modest impact on type inference. We substantiate this claim in a very concrete way, by presenting a complete type-inference engine, written in Haskell, for a traditional Damas-Milner type system, and then showing how to extend it for higher-rank types. We write the type-inference engine using a monadic framework: it turns out to be a particularly compelling example of monads in action. The paper is long, but is strongly tutorial in style. Although we use Haskell as our example source language, and our implementation language, much of our work is directly applicable to any ML-like functional language.
THE EFFECT OF THE REPLACEMENT OF FREE SILICA BY ALUMINA AND ZIRCONIA IN ELECTRICAL PORCELAIN1
Authors: Twells Robert , Lin C. C. 
Published: June 2006

ABSTRACTEffect on electrical porcelain of the replacement of free silica by alumina and zirconia.—Since it had been suggested that free silica may be detrimental to the mechanical and the dielectric strengths of electrical porcelain, a series of sixteen batches was prepared in which various proportions of the free silica were replaced, weight for weight, by alumina, zirconia or combinations of both, and bars and disks, after burning at cone 8 l/2 to 9 or at cone 12, were tested for shrinkage, transverse strength, impact strength, heat resistance, absorption of moisture and dye penetration. The results show that resistance to sudden temperature changes can be greatly improved by substituting zirconia,—that the danger of overburning can be greatly decreased by substituting alumina and that the mechanical strengths can be increased by substituting either or both; but in the case of dielectric strength, no improvement was obtained by substituting for the free silica.
NOTE ON THE EFFECT OF TIME ON THE DRYING SHRINKAGE OF CLAYS1
Authors: Geller R. F. 
Published: June 2006

ABSTRACTThe drying shrinkage or clays were studied to determine how quickly different types can be dried and to note whether or not the time of drying influences the amount of shrinkage. Three shales, two ball clays and one fire‐clay were tested and the following conclusions reached: (a), drying time depends largely on water content; (b), the structure of the clay is an important factor; (c), the amount of total shrinkage is proportional to the water content; (d), the amount of total shrinkage is not influenced by the rapidity of drying.
THE RELATION OF THE ARTIST TO THE MANUFACTURER1
Authors: Dressler Conrad 
Published: June 2006

ABSTRACTThe achievements of the past owed their beauty to the artist. The excellent technique of the present is due to the efforts of the manufacturer and scientist. The latter quality has now almost superseded the former; yet the esthetic needs are eternal and can not be denied. The efforts of solitary artists are unavailing in a modern world of vast undertakings. It is for the modern factory to reintroduce the artistic spirit.
MECHANISM OF PLASTICITY FROM COLLOID STANDPOINT1
Authors: Bole G. A. 
Published: June 2006

ABSTSRACT Introduction.—Brief statement of present conceptions of the colloid chemists. Theory.—Plasticity is caused by the, enveloping film of colloidal material which surrounds the clay grains, this film being of opposite polarity to the grain proper. Addition of electrolyte acting as deflocculant causes outer layer of film to be attracted to ion of same polarity as clay particle, thus reducing effective diameter and increasing polarity of plasticity inducing particles. Particles which were held by surface forces of “particles in contact” are no longer in contact and are actually repelling each other due to like and increased polarity. A flocculating ion drives action in opposite direction causing an increase in depth of colloid film thereby increasing surface in actual contact and decreasing repulsive force of plasticity inducing particles. Transport numbers as well as polarity are governing factors in ions of electrolytes. Measurements.—Touch method is proven to be unreliable. Desirability of a unit of measurement. Plea for a unit of colloidality and standard method for measurement.
NOTES ON SHIVERING OF TERRA COTTA1
Authors: Carruthers John L. 
Published: June 2006

ABSTRACTSix terra cotta clays are tested for shivering as a body trouble using a white mat glaze, at cone 3 starting. One non‐shivering clay and one badly shivering clay are experimented with, to produce and overcome shivering. Causes of Shivering.—From this work and that of others which has been checked, uses are

presence of finely divided silica in the body, from

highly siliceous clays
finely ground grog of a siliceous nature

presence of soluble salts in the clay, grog, or tempering water
longer firing periods

Methods of Eliminating Shivering.

Use of a flux such as feldspar, felsite, or similar rock to overcome action of fine silica
Use of coarse grog or sand
Use of barium carbonate to overcame effects of salts
THE ITALIAN MAJOLICA PROCESS AND PAINTING OVER TIN ENAMELS1
Authors: Rhead Frederick H. 
Published: June 2006

ABSTRACTReference is made to historical types. Practical conditions are discussed, types of glazes are given, and methods of application are outlined. There are also comments in connection with one‐fire products.The processes involved are discussions A‐3 and A‐4: “The Italian Majolica Process,”“Painting over Tin Enamels,” are so closely related that they are taken together.
TALC AS A FLUX FOR HIGH TENSION INSULATOR PORCELAINS1
Authors: Twells Robert 
Published: June 2006

ABSTRACTMgO has some possibilities as a partial or total flux in high tension insulator porcelains. To be practical for this purpose it must be introduced into the body through some cheap material which will not make a large percentage of calcine necessary.Talc seems to fulfill these requirements better than other sources of MgO more commonly used.A typical high tension insulator body of the general formula 0.21 K2O, 1.0 Al2O3, 4.45 SiO2 was used as a standard. The experimental work consisted chiefly in the partial replacement of the K2O by MgO, the RO being varied between 0.14 and 0.30 equivalents. Talc was used as the source of MgO.The bodies were fired to temperatures ranging between cones 9 and 11 and were tested in most cases only for absorption and transverse strength.The results at cone 10½ indicate (1) a small addition of talc greatly decreases the percentage of feldspar necessary for vitrification, (2) additional small amounts of talc have much less effect than the first small addition, (3) about 0.16 equivalents of Ro are needed for vitrification at this temperature when small percentages of talc are used. This corresponds to about 15 to 22.5% of feldspar and about 2.5% to 0.75% of talc, (4) in the properly vitrified bodies the addition of the MgO at the expense of the K2O, within the limits tested, tends to increase the transverse strength. Vitrified bodies of this general type show little improvement, if any, over regular triaxial porcelains. The writer recommends as the next logical step a trial of the effect of the replacement of feldspar entirely or almost entirely by talc and the use of higher temperatures to obtain vitrification.
EFFECTIVENESS OF DIFFERENT METHODS OF MAKING ABSORPTION DETERMINATIONS AS APPLIED TO HOLLOW BUILDING TILE1
Authors: Foster Harry D. 
Published: June 2006

ABSTRACT The experimental work consisted primarily in selecting specimens from hollow tile from seventeen sources and saturating them for the absorption determination by (1) immersion in water at room temperature for various periods ranging from fifteen minutes to nine days, (2) boiling for various periods ranging from 1 hour to 5 hours and then cooling in water at room temperature for at least one hour, and (3) repeated vacuum treatments. The true porosity for some of the specimens was then obtained from the specific gravity and the per cent saturation reached by the various methods was calculated. The results show that the five‐hour boiling treatment gives results 1.2 greater than 72 hours immersion in cold water for the shale tile, 1.35 greater for the fire clay tile, 1.20 greater for the surface clay tile, 1.25 greater for the mixed clay tile and 1.28 greater for an average of all tile. They also show that the cold water immersion treatment gives 73.5 per cent saturation, the boiling treatment 92.7 per cent saturation, and the vacuum treatment 97.0 per cent saturation. In general the paper shows that the most consistent and practical way of determining the absorption of hollow tile is by boiling for five hours and then cooling for at least one hour before the saturated weight is taken.
NOTE ON LADLE BRICK1
Authors: Moulton D. A. 
Published: June 2006

ABSTRACT Review of Literature on Ladle Brick.—(1) American ladle in which it is stated no chemical reactions occur; (2) English ladle in which four or five chemical reactions occur simultaneously. Fusion Point of Ladle Brick ranging from cones 17 to 30 depending on behavior of clay at temperatures below the fusion point. Process of Manufacture.—Two processes, namely dry press and stiff‐mud with preference for dry press. Modification of standard refractories is suggested.
OBSERVATIONS ON THE FACTORY CONTROL OF FISH‐SCALING1
Authors: Grainer John S. 
Published: June 2006

ABSTRACTAn analysis of fish‐scaling, its causes and prevention, based upon observations and experience of practical work. Three principal causes of fish‐scaling are: (1) Improper firing, either underfiring or overfiring, especially the latter since its effects arc usually not evident until the ware is finished and marketed; (2) The use of a clay which does not give a free running uniform coat without the use of excessive amounts of flotation agents. Clays which, when stirred up with water give a persistent suspension are recommended. (3) Improper type of furnace for the firing operation. The author prefers the open type furnace for the firing of enameled ware.
MANUFACTURE OF SINGLE COAT GRAY ENAMELED WARE1
Authors: Arnold Howard C. 
Published: June 2006

ABSTRACTThe manufacture of single coat, mottled gray enameled ware presents a diversity of problems as will be evident from a casual consideration of the following specifications.1.—The black shape has to be completely covered in one coat as the lack of a second, or cover coat makes it impossible to hide a blemish, scratch or mark.2.—This coat must have all the gloss, luster and appearance of a finish coat.3.—It must show two colors, and these colors must be produced from the same enamel and in a single dipping.4.—The two colors must be present in correct amounts and, at the same time, must be distributed in such a way as to give a pleasing effect.
COMPARISON OF HOT AND COLD MODULUS OF RUPTURE FOR SILICA BRICK
Authors: McGee E. N. 
Published: June 2006

ABSTRACT Purpose of the Investigation.—(1) To obtain relative values for the cross‐breaking strength of silica brick at temperatures encountered in coke oven practice.(2) To correlate the hot modulus of rupture test, if possible, with the cold modulus of rupture, or cold crushing test, either of which is cheaper and more easily conducted.This report gives the method of making the test, difficulties encountered and results obtained. The report shows a comparison of cold crushing, cold modulus of rupture and hot modulus of rupture on a series of silica brick made from special mixes, commercially burned. Conclusions.—The modulus of rupture of a silica brick at 1350°C is approximately one‐third the strength at atmospheric temperature. For this series it averaged from 130 to 189 lbs. per square inch.Too rapid or eccentric heating up to red heat may cause such weakening of the structure or bond that the brick will break under very low pressure.Cross‐breaking strength decreases as the temperature increases.Hot modulus of rupture test appears to give results, in most respects, comparable to the cold test, and for routine testing it would seem advisable to use the cold test since it can be made in much shorter time.
CARBORUNDUM BRICK1
Authors: Peters M. F. 
Published: June 2006

ABSTRACTA study of carborundum‐clay mixtures with reference to the relation between the proportioning and sizing of the constituents and the properties of the refractory as expressed by the tensile strength, the load test, the slagging and the spalling tests. For good working conditions the amount of clay must suffice to cover the grains of SiC. When subjected to crushing the linear compression varies as the second power of the percentage of carborundum. Oxidizing furnace conditions and the presence of iron oxide, lead oxide and lime reduce the resistance to slag action. The resistance to spalling depends upon the content of SiC, the tensile strength, the coefficient of expansion and the thermal conductivity of the clay. A formula is proposed for the estimation of the life of the carborundum refractories, and a theoretical explanation of their physical properties is offered.
THE TESTING OF SILICA BRICK
Authors: Endell K. H 
Published: June 2006

ABSTRACTThe Steger Load Test Apparatus. —Pressure was applied, in a granular‐carbon resistance furnace, to the small test cylinders by means of a hollow carborundum rod. The temperature was read with an optical pyrometer sighted through this hollow pressure red. The volume changes were indicated by a pointer and scale and recorded by means of a rotating drum. (See Fig. 1.)Estimation of Quartz and Cristobalite in the Finished Br —The areas occupied by each type of crystal on the photomicrograph were determined with the aid of a transparent cross‐section grating.Suggested Specifications for A1 Silica Brick. —Trust specific gravity not greater than 2.38; not more than 2% permanent Linear expansion after heating to 1600°C in one and one‐half hours, and holding at that temperature for one‐half hour; the amount of quartz land silicates) as determined by the grating method applied to photomicrograph should not exceed 15%.
TEMPERATURE CONTROL OF DRESSLER TUNNEL KILN1
Authors: Harrison T. R. 
Published: June 2006

ABSTRACTThe method of graded adjustment of fuel supply was adopted. Adjustments to compensate for variations in quality of fuel gas, which often amounted to as high as 30% change in the gas pressure at the burners within two or three minutes, are produced through the agency of the variation in the length of a bunsen burner flame as different kinds of gas are supplied under the same pressure. The adjustment is thus made without the temperature departing from the desired value.Further minor adjustments are made according to slight departures from normal of the combustion chamber temperatures. The pyrometer control contacts are moved away from the normal position simultaneously as the gas pressure is adjusted, the magnitude of this adjustment being proportional to the necessary motion of the contact points to bring them to the momentary position of the pyrometer pointer. The contact points are then automatically returned to their normal position at a predetermined (constant or variable) rate which is the estimated rate at which the kiln should return to its normal value when the correct adjustment has been made. A sufficient correction is thus made promptly without over‐correction during the necessary period of recovery as demanded by the kiln lag.
OPHTHALMIC GLASS—A NEW AMERICAN PRODUCT
Authors: Montgomery Robert J. 
Published: June 2006

ABSTRACTMost of the ophthalmic or spectacle glass used in this country was imported prior to 1914. At present very little is imported and the industry is firmly established in the United States. The various glasses used are listed and their defects discussed. The problem of fusing bifocal lenses is covered in considerable detail. Marked improvement has been made in goggle glasses and colored glasses are being standardized. The effect of sunlight on certain clear and colored glasses is given.
THE FLINT CLAY SITUATION IN OHIO1
Authors: Stout Wilbur 
Published: June 2006

ABSTRACTA discussion of the flint clays of Ohio with special reference to Sciotoville, Lower Kittanning, Oak Hill and Lower Freeport clays. It is shown that (1) flint clay beds of high heat duty quality in Ohio are small but well‐defined (2) there is small prospect of finding other deposits under deeper covering (3) the most easily available material is exhausted (4) the present supply will last present plants at least fifty years but will not permit much increased consumption (5) conservation of high heat duty clays should begin now before supply is gone.
A SKIAGRAPHIC STUDY OF FABRICATED GLASS ARTICLES1
Authors: Spencer C. D. , Badger A. E. 
Published: June 2006

ABSTRACTA method is described whereby defective construction may be determined in fabricated glass articles. The principle is the same as that of the immersion method of grading optical glass. The glass object is immersed in a fluid whose refractive index for some monochromatic source of illumination is approximately equal to that of the article. Under these conditions it is possible to observe defects in the structure of complicated glass objects.
THE RELATIVE MAGNITUDE OF RADIATION AND CONVECTION HEATING IN A MUFFLE KILN1
Authors: Littleton J. T. 
Published: June 2006

ABSTRACTRates of Heating by Convection and by Radiation in a Muffle Kiln, 38°×18°× 36° high, were each determined for temperatures from 350° to 800°C from measurements with a steady flow water calorimeter whose surface was first gold plated and then covered with a mixture of platinum black and lamp black. Taking the reflecting powers as 91 and 4 per cent, respectively, for the two surfaces, the radiation heating increases approximately according to the Stefan‐Boltzmann fourth power law, while the convection heating comes out proportional to the temperature difference between calorimeter and muffle; that is, C=γ(T‐t) where γ= 2.34×10−4 gm. cal./cm.2 sec. The ratio of convection to radiation decreases from about .40 at 350° to .10 at 800°C, so that for the higher temperatures the convection heating may be neglected in rough computations of the rate of heating in such a kiln.
VITREOUS ENAMELING WITH ELECTRIC HEAT1
Authors: COLLINS E. F. 
Published: June 2006

ABSTRACTThis paper is descriptive of the suitability of the metallic resistor furnace for the application of vitreous enamels. Comparisons are made with fuel fired furnaces. Uniformity and perfect fusion of enamel results from the high grade temperature control of the electric furnace. Atmospheres vitiated with products of combustion can not be present in the electric furnace to give defective coatings. Examples of operating furnaces for watch dials and for bath tubs are given and the average performance, in time and input, for bath tub furnaces. It is believed by the author that the electric heating process can not long be considered as anything but the most economical over all, and its standardization for this purpose will eventually result.