bluuebunny/crossref_metadata_2025 huggingface dataset:
Propylene Oxide
Authors: Trent David L.
Published: August 2004

Propylene oxide, a commercially important chemical worldwide, is very reactive, finding application in many derivative businesses. Use is primarily as an intermediate for a wide array of products, such as polyether polyols for the polyurethane industry; propylene glycol for food, cosmetics, and heat‐transfer fluids; polyglycols as lubricants, antifoam agents, and metalworking fluids; glycol ethers for protective coatings, inks, and cleaners; and isopropanolamines for adhesives, corrosion inhibitors, and agricultural products.

Propylene oxide is produced by two process chemistries. The first, and oldest, is the chlorohydrin process. Epoxidation by caustic or milk of lime produces the epoxide. The second process involves the oxidation of an organic such as isobutane or ethylbenzene to the corresponding hydroperoxide, then epoxidation of propylene using the hydroperoxide to the epoxide and an alcohol. The alcohol,tert‐butanol or 1‐phenylethanol, is further processed to provide the commercially viable coproducts, such as methyl‐tert‐butyl ether and styrene. The global capacity for propylene oxide is about half from the chlorohydrin process and a quarter each from the hydroperoxide processes producingtert‐butanol and styrene as coproducts. These and several other potentially commercial processes are discussed.
Pumps
Authors: Nelik Lev
Published: August 2004

Pumps are used in a wide range of industrial applications in the chemical processing industries. Pumping equipment varies widely in terms of pump type, size, and materials of construction. The two principal pump types, kinetic, which is mainly centrifugal, and positive displacement, are presented and basic operating parameters such as capacity, head, power, efficiency, and specific speed are defined. Fundamentals of operation are discussed, selection guidelines given, and examples of pump applications and reliability concerns provided. Pump auxiliaries such as couplings and seals, as well as effects of cavitation, recirculation, internal hydraulic forces, wear, and corrosion, are described. Cost approximation methodology is presented along with a list of manufacturers.
Antibacterial Agents, Quinolones
Authors: Reuman M. , Lesher G. Y.
Published: August 2004

Quinolone carboxylic acids are a class of totally synthetic antibacterial agents which encompass 4‐oxo‐3‐quinolinecarboxylic acids as well as the corresponding 1,8‐naphthyridines, cinnolines, and pyrido [2,3‐d]‐pyrimidines. These classes are illustrated by ciprofloxacin, nalidixic acid, cinoxacin, and piromidic acid, respectively. Established quinolone antibacterial agents are ciprofloxacin, ofloxacin, enoxacin, norfloxacin, and pefloxacin. Quinolones exert their antibacterial activity by interfering with the replication of bacterial DNA by inhibition of the enzyme DNA gyrase. The critical reaction is the negative supercoiling of bacterial DNA, a process involving the breaking and resealing of double‐stranded circular DNA. The general method by which most newer fluoro quinolones are prepared involves a ring closure reaction to form the quinolone nucleus. For the most part quinolones are well tolerated with few reports of adverse reactions, but because of a concern that they cause arthropathy in juvenile animals, quinolones are contraindicated for children and during pregnancy. A second problem is adverse side effects of the central nervous system (CNS) and adverse reactions with other drugs. Earlier quinolones have had little impact on the domestic antiinfective market as their utility was limited primarily to urinary tract infections. Newer quinolones, because of safety, seemingly low propensity toward bacterial resistance, and vastly improved therapeutic utility, enjoy a much greater market share.
Shape‐Memory Alloys
Authors: Schetky L. McDonald
Published: August 2004

Shape‐memory alloys are capable of undergoing reversible phase transitions as a result of temperature, pressure, or other stress‐related changes. These materials exhibit a mechanical type of shape memory called pseudoelasticity and, under certain conditions, linear superelasticity. The recoverable strain or shape is generally

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. Whereas the alloys are primarily nonferrous, some ferrous alloys also exhibit shape memory.

Applications of shape‐memory alloys include those as tubes and valves in piping systems for power plants, ships, and the petroleum industry; use as explosive bolts in the aerospace and construction industry; as packaging devices for electronic materials; and as dental materials, prosthetics, and biomedical devices. Shape‐memory alloys may be found in sensors in automobiles, in consumer products, and generally in smart materials and adaptive structures.
Silicon Compounds, Silicones
Authors: Rich Jonathan , Cella James , Lewis Larry , Stein Judith , Singh Navjot , Rubinsztajn Slawomir , Wengrovius Jeff
Published: August 2004

This article begins with an introduction on common shorthand nomenclature and a history of silicones. The chemistry discussion includes a description of the direct process for formation of monomers, including the methylchlorosilane process. The silicone polymerizations described are condensations, anionic and cationic ring‐opening polymerizations, emulsions, radiation‐induced polymerizations, and plasma reactions. Discussion of silicone network formation includes those formed via peroxides, hydrosilylation, condensation, high energy, and uv radiation. Methods of characterizing silicone networks are discussed, as well as model, monodisperse, and filled networks. A section on silicone properties and uses includes discussion of fluids, heat‐cured rubber, liquid‐injection‐molding rubber, hardcoats, release coatings, and resins. A discussion of resins includes sections devoted to characterization, structure, synthesis, and applications. Room‐temperature‐vulcanized (RTV) silicones are reviewed with sections on moisture‐curable silicones, basic RTV chemistry, RTV compositions, properties, and applications. Analysis and testing of silicones are described, along with chemical analysis, instrumental methods, and determination of physical properties. Important new developments are given on health and environmental aspects of silicones. Economic aspects of the silicones industry are summarized.
Silver Compounds
Authors: Cappel C. Robert
Published: August 2004

Most silver compounds are made from aqueous silver nitrate, which in turn is made from silver metal. Silver compounds are found in nature, but are neither ubiquitous nor prevalent. Silver salts are therefore expensive. Much of the silver produced annually in the United States comes from recycled metal. The primary usage of silver compounds occurs in the photographic industry. Silver compounds are also utilized in batteries, as catalysts, to form the reflective backings of mirrors, and for electroplating silver. Silver(I) compounds and complexes are the most stable, although some materials of higher silver oxidation are known. Some aqueous silver solutions serve as antiseptics; other as analytic reagents.
Carbon, Structure, Terminology, and History
Authors: Long J. C.
Published: August 2004

Elemental carbon, atomic number six in the periodic table, at wt 12.011, occurs naturally throughout the world in either its crystalline (more ordered) or amorphous (less ordered) form. Carbonaceous materials such as soot or charcoal are examples of the amorphous form, whereas graphite and diamond are crystalline. Carbon atoms bond with other carbon atoms as well as with other elements, principally hydrogen, nitrogen, oxygen, and sulfur, to form carbon compounds, which are the subject of organic chemistry. The manufactured form of carbon and graphite is discussed within this article. In their many varying manufactured forms, carbon and graphite can exhibit a wide range of electrical, thermal, and chemical properties that are controlled by the selection of raw materials and thermal processing during manufacture. There are two allotropes of carbon: diamond and graphite. The diamond, or isotropic form, has a crystal structure that is face‐centered cubic with interatomic distances of 0.154 nm. Each atom is covalently bonded to four other carbon atoms in the form of a tetrahedron. The nature of the bonding explains the differences in properties of the two allotropic forms. The hardness of diamond is derived from the regular three‐dimensional network of σ‐bonds. Graphite, or the anisotropic form, has a structure that is composed of infinite layers of carbon atoms arranged in the form of hexagons lying in planes. The electronic ground state of carbon is 1s2, 2s2, 2p2. In diamond, the 2sand 2pelectrons mix to form four equivalent covalent σ‐bonds. In graphite, three of the four electrons form strong covalent π‐bonds with the adjacent in‐plane carbon atoms. The fourth electron forms a less strong bond between the planes. A wide variety and range of bulk carbon forms are available within the industry. In general, commercial forms are loosely characterized as carbon or graphite, but they are distinctly different. The termmanufactured carbonrefers to a bonded granular carbon body whose matrix has been subjected to a temperature typically between 900 and 2400°C.Manufactured graphiterefers to a bonded granular carbon body whose matrix has been subjected to a temperature typically in excess of 2400°C. Natural graphite has been known since the Middle Ages, but carbon was first fabricated by H. Davy in his experiments on the electric arc in the early 1800s. The manufacture of artificial graphite came about only at the end of the nineteenth century, preceded by developments in the fabrication of electrodes. The electric resistance furnace enabled the reaching of approximately 3000°C, the temperature necessary for graphitization. A new application for graphite, its use by E. Fermi in the first self‐sustaining nuclear reaction, was followed by new fields of research and new markets opened by development of the aerospace industries, including the use of carbon and graphite fibers in composite materials.
Coating Processes, Survey
Authors: Cohen Edward D. , Gutoff Edgar B.
Published: August 2004

A coating replaces air at the interface of a substrate with a new material. Coatings are applied to surfaces for both functional and decorative reasons. The processes whereby the fluid that forms the coating is transferred to a substrate, which may be a stationary support or a traveling web, are reviewed. Steps in the coating process include: preparation of the coating fluid, metering the fluid to a desired coating weight, application of the coating fluid, and removal of the solvent and drying.

The relationships between such factors as the nature of the substrate, the coating fluid rheology, coating solvent, coating uniformity and speed, number of layers, and whether the coating is to be continuous or intermittent, are considered for the various coating methods. Knife, roll, blade, gravure, slot, and extrusion coaters for single‐layer coating are presented as are the slide and precision curtain methods used for multilayer coating. Dryers and drying fundamentals are also described.
Acrylic Ester Polymers, Survey
Authors: Novak Ronald W.
Published: August 2004

The nature of the R group in its formula determines the properties of each acrylic ester and the polymers it forms. Polymers of this class are distinguished by their water‐clear color and stability on aging. Acrylic monomers are extremely versatile building blocks. They readily polymerize or copolymerize with a variety of other monomers. Copolymers with methacrylates, vinyl acetate, styrene, and acrylonitrile are commercially significant. Although the acrylics have a higher cost than many other monomers their unique characteristics and efficiency offset the higher cost. Acrylic ester polymers can be made to hydrolyze to poly(acrylic acid) or an acid salt and the corresponding alcohol. Two principal processes are used for the manufacture of monomeric acrylic esters: the semicatalytic Reppe process and the propylene oxidation process, the latter of which is preferred because of economy and safety. The toxicities of acrylic monomers range from moderate to slight. The vast majority of all commercially prepared acrylic polymers are copolymers of an acrylic ester monomer with one or more different monomers. Copolymerization has led to the development of many different resins for a variety of applications. Acrylic polymers are considered to be nontoxic, and are used primarily in coatings, textiles, adhesives, and paper.
Tellurium and Tellurium Compounds
Authors: Hoffmann James E. , King Michael G. , Carapella S. C. , Oldfield J. E. , Putnam R. D.
Published: August 2004

Tellurium is the most metallic of the abundant chalcogens. In many of its chemical properties it still resembles oxygen, sulfur, and selenium.

Tellurium is produced as a by‐product at copper refineries throughout the world. The main uses for tellurium are as a free‐machining additive in both ferrous and nonferrous metallurgy, in chemicals (30%), and in electrical applications such as solar cells and thermoelectrics. Applications of tellurium are frequently limited by the small quantities available. Tellurium and tellurium compounds generally have a low environmental impact.
Chromogenic Materials, Thermochromic
Authors: Greenberg Charles B.
Published: August 2004

Thermochromism is a reversible change in spectral properties as a result of heating and cooling. Usually the response is visible. It is observed in complexed metals, in both inorganic and organic materials generally, and in all phases of matter, though predominately in solids and solutions. There are thousands of examples in the solid and liquid phases, a few examples of which are given here. These examples have been selected to indicate the scope of transition mecanisms tat contribute to this interesting chromogenic materials category. The breadth of the subject is evident from the bibliography. It includes, on the one hand, literature that has been generated in the circle of the inorganic chemist, as well as references that are from the literature of the physicist and also the organic chemist. Indeed, thermochromism is an encompassing subject.
Titanium and Titanium Alloys
Authors: Seagle Stan R.
Published: August 2004

Titanium, the ninth most abundant element, is used principally as an oxide in paint filler for its high refractory index and as a metal in aerospace and industrial applications for its high strength‐to‐density ratio and excellent corrosion resistance. This article covers the locations and reserves of principal ore bodies and their conversion by sulfate or chloride refining processes to high purity oxides for the pigment industry, and the subsequent conversion by magnesium reduction to metallic sponge for the metal industry.

The principal metal consolidation melting processes to produce ingots from sponge are reviewed, along with the metalworking processes for converting ingots to mill products. The influence of alloying elements on properties and microstructure is discussed, along with the mechanical properties and characteristics of the principal alloys used in aerospace and industrial applications. Methods and procedures related to the fabrication of metal components into useful application components are covered. The primary uses of both the oxide and the metal are summarized, along with the economics associated with the production of the metal and oxide.
Uranium and Uranium Compounds
Authors: Clark David L. , Keogh D. Webster , Neu Mary P. , Runde Wolfgang
Published: August 2004

An overview of uranium and uranium compounds with an emphasis on their relevance to chemical technology is provided. Uranium and its compounds have many uses both potential and applied, examples include nuclear fuel, radiation shielding, and kinetic energy penetrators. These uses have a substantial economic impact, the aspects of which are provided. Sections are provided which illustrate the occurrence, recovery, and radiochemistry of uranium as well as the properties and preparation of uranium metal. A broad overview of uranium coordination and organometallic compounds is also provided. Drawbacks to the application of uranium and uranium compounds in many processes include the chemical reactivity of the metal and the inherent radioactivity of uranium isotopes. As such, health and safety factors which must be taken into account while working with uranium are discussed.
Clays, Uses
Authors: Sennett Paul
Published: August 2004

Clay minerals are widely used in many industries and usage typically depends on physical and chemical properties. Kaolin clay finds a large market in ceramics, paper, paint, plastics, and rubber, and in many other uses. The properties of kaolins depend primarily on processing methods. Bentonites, because of fine particle size and expanding lattice characteristics, are used in large amounts for applications such as a binder for foundry sands and iron are pelletizing, as viscosity control agents for oil well drilling fluids, and as adsorbents. Other clay minerals such as halloysite and attapulgite are less important commercially but have significant value in several applications. Clays are also used as chemical feedstocks.
Vinyl Alcohol Polymers
Authors: Marten F. Lennart
Published: August 2004

Poly(vinyl alcohol) is a highly crystalline polyhydroxy polymer produced by the hydrolysis of poly(vinyl acetate), as the vinyl alcohol monomer is not available. The polymer is water‐soluble, possesses excellent tensile strength, abrasion resistance, superior oxygen barrier properties, as well as excellent emulsification and protective colloid properties, and exhibits solvent, oil, and grease resistance that are matched by few other polymers. Poly(vinyl alcohol) undergoes chemical reactions in a manner similar to other secondary polyhydroxy alcohols. Fully biodegradable, the polymer is considered a nonhazardous material according to the American Standard for Precautionary Labeling of Hazardous Industrial Chemicals.

Industrial applications are all based on and limited by the use of water solutions. The polymer is not considered thermoplastic because the degradation temperature is below the melting point. Poly(vinyl alcohol) is commercially available in granular and powdered form. Grades include both fully hydrolyzed and partially hydrolyzed products. A wide variety of molecular weight and hydrolysis is commercially available. Principal end uses include textile sizing, adhesives, protective colloid for emulsion polymerization, paper coating, cement and mortar additive, poly(vinyl butyral), fibers, and films.
Vinylidene Chloride Monomer and Polymers
Authors: Wessling R. A. , Gibbs D. S. , DeLassus P. T. , Obi B. E. , Howell B. A.
Published: August 2004

Vinylidene chloride is a versatile monomer used primarily for making vinylidene chloride copolymers. These copolymers are mostly used in food packaging to provide a good barrier to mass transport. Most applications rely on the low permeation of oxygen, carbon dioxide, and water vapor. However, the polymers have also been shown to have good barrier to flavors and aromas. These copolymers are available in three formats. First, resin for extrusion into film or sheet represents the largest segment. Typically, coextrusion is used because only a small amount of vinylidene chloride is necessary for the barrier and other polymers can provide the strength. Second, resin is used for solution applications. A thin layer is coated onto a substrate. Third, latex is used for coating. The coatings from solution or latex dry clear. Vinylidene chloride homopolymer does not have commercial application because its melting temperature is too high to extrude without degradation, and it does not dissolve in a suitable solvent for coating.
Polymer Colloids
Authors: Takamura Koichi
Published: August 2004

Typical industrial polymer colloids (also known as latexes) have 50–60% polymer content, with particles ranging in size from 100 nm to 250 nm in diameter dispersed in water. They are produced by the emulsion polymerization process, and are used for a wide range of industrial applications including paints, paper coatings, adhesives and carpet backing. Among these polymer colloids, styrene–butadiene copolymers, polyacrylates, and vinyl acetate homopolymers and copolymers account for 95% of the total production worldwide, which is expected to exceed 9 (dry) million metric tons in the year 2004. The polymer colloids transform into a polymer film upon drying at above the critical temperature, known as the minimum film forming temperature. Resultant films can be clear or opaque, hard or tacky, and plastic or elastic, depending upon the needs. Though the polymers are hardly visible, they provide critical properties in various industrial applications. This article discusses the relationship between three polymer parameters; electrokinetic properties, rheological properties and polymer particle morphology, as they correlate to industrial applications.
Adult degrees and the learning society
Authors: Maehl William H.
Published: August 2004

This overview of the adult degree movement since World War II focuses on factors of creation before 1970, the adult degree revolution of the 1970s, the post‐baby boomer enrollments in the 1980s, and globalization and technology‐based distance learning in the 1990s.
Adult learning theory and the pursuit of adult degrees
Authors: Kiely Richard , Sandmann Lorilee R. , Truluck Janet
Published: August 2004

This chapter describes a four‐lens model for understanding adult learning theories and provides adult educators and administrators with a useful conceptual framework for working with adult learners in adult degree programs.
Marketing and retention strategies for adult degree programs
Authors: Brown Joann A.
Published: August 2004

Four marketing strategies are critical to the success of adult degree programs: integrating marketing, knowing your students (research), shaping programs and services for adults, and staying the course (retention).
Adult degree programs: How money talks, and what it tells
Authors: Matkin Gary W.
Published: August 2004

Multiple factors are changing the underlying financial structure of higher education in America to a form much like the structure now used for adult degree programs. This change is likely to have profound effects on institutional behavior and public policy.
Technology and the adult degree program: The human element
Authors: Rodriquez Frank G. , Nash Susan Smith
Published: August 2004

While technology has for many years been a critical component in programs for adults and calls to mind sophisticated gadgetry with expensive price tags, it is often the nexus where technology and humans intersect that proves most critical to the success and quality of adult degree programs.
Accreditation issues related to adult degree programs
Authors: Simpson Jr. Edward G.
Published: August 2004

Understanding the fundamental tenets and structure of the accreditation process can assist institutions in the design of high‐quality degree programs while affording adult students a reasonable level of consumer protection.
Experimental analysis of floc size distribution under different hydrodynamics in a mixing tank
Authors: Bouyer Denis , Liné Alain , Do‐Quang Zdravka
Published: August 2004

The goal of this report is to analyze the relationship between characteristic floc size and hydrodynamics in a mixing tank. The first question addressed concerns the relation between an average floc size and the viscous dissipation rate of kinetic energy. A first series of flocculation experiments were conducted in a mixing tank with two impellers (a Rushton turbine and a Lightnin A310 impeller) for equivalent dissipated power conditions. The average floc size is shown to depend on the global dissipation rate; it does not depend on the impeller type. However, the floc size distributions are significantly different for each impeller. The second question addressed concerns the dependency of the floc size on the history of mixing. A second series of experiments consisted of flocculation, breakup, and reflocculation stages. These experiments showed that the average floc sizes are similar after flocculation or reflocculation steps, but, once again, the floc size distributions can be very different with different impellers. The flocculation phenomena analyzed in this study mainly occur in the viscous subrange, with maximum floc size on the order of Kolmogorov microscale. © 2004 American Institute of Chemical Engineers AIChE J, 50: 2064–2081, 2004
Relationship between idiopathic hypereosinophilic syndrome, eosinophilic leukemia, and systemic mastocytosis
Authors: Bain Barbara J.
Published: August 2004

Chronic eosinophilic leukemia is a neoplastic condition with persistent eosinophilia as the major hematological abnormality and with the eosinophils being part of the neoplastic clone. Some cases can be recognized by traditional hematological criteria, but many can be recognized only when a clonal cytogenetic or molecular genetic abnormality is demonstrated. A range of cytogenetic and molecular genetic abnormalities has been recognized, including both those seen in other myeloid malignancies (such as trisomy 8, monosomy 7, and 20q−) and those that are particularly linked to eosinophil differentiation (such as rearrangements of PDGFRB, FGFR1, and PDGFRA, the latter with formation of a FIP1L1–PDGFRA fusion gene). The discovery of the FIP1L1–PDGFRA fusion gene has led to the recognition that many patients who would previously have been regarded as having idiopathic hypereosinophilia actually have chronic eosinophilic leukemia. The same fusion gene has also been found in patients with hypereosinophilia and atypical bone marrow mast cells but whether this syndrome should be regarded as a variant of eosinophilic leukemia or as a variant of systemic mastocytosis remains to be established. Am. J. Hematol. 77:82–85, 2004. © 2004 Wiley‐Liss, Inc.
Association of single nucleotide polymorphisms in the thrombopoietin‐receptor gene, but not the thrombopoietin gene, with differences in platelet count
Authors: Zeng She Min , Murray Jeffrey C. , Widness John A. , Strauss Ronald G. , Yankowitz Jerome
Published: August 2004

Little is known about the mechanisms explaining the wide variation in platelet counts (PLT) and other hematologic parameters in humans. We previously showed that the sex‐based difference in hematocrit was associated with nucleotide variation in the erythropoietin receptor gene (EPOR). We sought to identify new polymorphisms of the human thrombopoietin (TPO) and thrombopoietin receptor (TPOR) genes to determine any associations with blood PLT counts. We screened TPO and TPOR for polymorphisms using single‐strand conformation polymorphism (SSCP) and DNA sequencing. Association of polymorphisms was studied in 304 normal subjects with low or high PLT counts. Distribution of allelic frequency was analyzed by the Chi‐square statistic. Single nucleotide polymorphisms (SNPs) with two alleles were found in TPO and TPOR. The TPO SNP was a G to A transition at nucleotide 5753, and the TPOR SNP was a C to A transversion at position 550 in the 5′‐promoter area. The allelic frequencies were 0.54 for G and 0.46 for A of TPO, and 0.62 for C and 0.38 for A of TPOR in a Caucasian population. The frequency of the TPOR allele “C” was significantly higher in subjects with high PLT count (>258 k/mm3) versus low PLT count (<224 k/mm3) and in males with high PLT count (>258 k/mm3) versus males with low PLT count (<212 k/mm3). In contrast, the frequency of the TPO alleles was not related to blood PLT counts. An association of TPO and TPOR allele distribution to red and white blood cell parameters was seen. These new SNPs found for the human TPO and TPOR genes help explain variations in blood PLT counts and may be useful in patient studies related to the roles of TPO and/or TPOR in disease. Am. J. Hematol. 77:12–21, 2004. © 2004 Wiley‐Liss, Inc.
Polymorphism distribution of Int13, Int22, and St14 VNTRs in a Mexican population and their application in carrier diagnosis of hemophilia A
Authors: Martínez Gallegos Rafael , Benítez Aranda Herminia , Navarrete Carmen , Peñaloza Espinoza Rosenda , Salamanca Gómez Fabio , Arenas Aranda Diego
Published: August 2004

Variable nucleotide tandem repeats (VNTR) Int13, Int22, and St14 were analyzed to determine polymorphic distribution in normal individuals from Mexico's central region and their efficacy in detecting hemophilia A carriers. Polymerase chain reaction (PCR) was carried out on 166 X chromosomes from unrelated Mexicans, and the same method was applied to detect carriers in hemophilia A families. Screening revealed the existence of at least eight different alleles for Int13, 4 alleles for Int22, and 10 alleles for St14. Their heterozygosity rates were 41.3%, 52.6%, and 83%, respectively. Compared to Caucasians, the Mexican population showed a markedly low heterozygosity rate for the Int13 marker. However, Int22 showed a heterozygosity that was similar to Turkish and Chinese populations. The St14 marker was the most informative in carrier diagnosis, and a new 680‐bp allele not previously reported was detected. Carrier diagnosis was performed in 39 women from eight different hemophilia A families. Fifteen (38%) females were not carriers, 16 (41%) females were carriers, and 8 (21%) were homozygous. Determination of polymorphisms in VNTR markers revealed that St14 was the most useful for hemophilia A carrier detection in Mexico. Am. J. Hematol. 77:1–6, 2004. © 2004 Wiley‐Liss, Inc.
Five Fanconi anemia patients with unusual organ pathologies
Authors: Unal Selma , Ozbek Namık , Kara Abdurrahman , Alikaşifoĝlu Mehmet , Gümrük Fatma
Published: August 2004

Fanconi anemia (FA) is a rare autosomal recessive disorder that presents with variable organ abnormalities, progressive cytopenia, and susceptibility to the development of several malignancies. Although some of the organ pathologies such as microcephaly, microphthalmia, skin dyspigmentation, urogenital system involvement, and radial ray skeletal abnormalities are relatively common, there are some other abnormalities that are rarely associated with the disease [Alter BP. In: Nathan DG, Oski FA, editors. Hematology of infancy and childhood. Philadelphia: Saunders; 2003. p 259–273]. In this paper, five cases of unrelated FA patients with unusual organ pathologies, including chronic obstructive lung disease, lipodystrophy, Sprengel's deformity, diaphragmatic hernia, and inflammatory linear verrucous epidermal nevus (ILVEN) are presented. Recognition of unusual pathologies associated with FA is important in order to improve our understanding of the relationship between the disease and presenting organ pathologies. Am. J. Hematol. 77:50–54, 2004. © 2004 Wiley‐Liss, Inc.
A rare inherited coagulation disorder: Combined homozygous factor VII and factor X deficiency
Authors: Menegatti Marzia , Karimi Mehran , Garagiola Isabella , Mannucci PierMannuccio , Peyvandi Flora
Published: August 2004

The combined presence in the homozygous state of more than one recessively transmitted coagulation defect may rarely occur in countries with a high rate of consanguinity. In an Iranian family consisting of two parents (second cousins) and two affected siblings, initial phenotypic analysis led to a diagnosis of mild FX deficiency (10–19% FX activity, 42–54% FX:Ag), and genotyping revealed a new homozygous missense mutation in the corresponding gene (Ser3Cys). As both of the sibs had a severe bleeding history that was not compatible with mild deficiency of FX, further phenotypic analysis revealed the additional presence of severe FVII deficiency (<1% FVII activity; 63–111% FVII:Ag) associated with the homozygous missense gene mutation Cys310Phe. In this kindred, lack of identification of the double coagulation defect might have led not only to incomplete understanding of the clinical phenotype but also to an incorrect prenatal diagnosis. Am. J. Hematol. 77:90–91, 2004. © 2004 Wiley‐Liss, Inc.
Trisomy 8 in Philadelphia‐negative cells during imatinib therapy
Authors: Bernardeschi P. , Fiorentini G. , Rossi S. , Dentico P. , Simi P. , Guidi S.
Published: August 2004

Targeted therapy with imatinib selectively suppresses Philadelphia‐positive cells in chronic myeloid leukemia cells, with reappearance of apparently normal hemopoiesis in a considerable number of patients. Recently, clonal abnormalities have been observed in Philadelphia‐negative cells during imatinib therapy, the biologic and prognostic significance of which is actually unknown. A case of trisomy 8 occurring in Philadelphia‐negative cells, which was treated by bone marrow transplantation, is reported. Chromosomal abnormalities in Philadelphia‐negative cells do not seem to herald disease transformation, but the long‐term prognosis may be influenced by an increased incidence of myelodysplasia in younger patients. Am. J. Hematol. 77:88–89, 2004. © 2004 Wiley‐Liss, Inc.
Bronchus‐associated lymphoid tissue lymphoma arising in a patient with bronchiectasis and chronic Mycobacterium avium infection
Authors: Gaur S. , Trayner E. , Aish L. , Weinstein R.
Published: August 2004

We describe a 67‐year‐old woman with bronchiectasis and Mycobacterium avium complex infection who underwent wedge resection of her pulmonary infiltrates because they were progressing despite antibiotic therapy. In addition to the expected granulomatous changes, she was found to have a B‐cell lymphoma of bronchus associated lymphoid tissue (BALT). Despite normal bone marrow morphology, marrow involvement was demonstrated by flow cytometry. Her lymphoma remains suppressed with antimycobacterial therapy 6 months after resection of bulk disease. Am. J. Hematol. 77:22–25, 2004. © 2004 Wiley‐Liss, Inc.
Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea
Authors: Saad Sara T.O. , Lajolo Camila , Gilli Simone , Marques José Francisco C. , Lima Carmen S. , Costa Fernando F. , Arruda Valder R.
Published: August 2004

Hydroxyurea is one of the most successfully used therapies for sickle cell disease. Results of many clinical trials point to hydroxyurea administration for patients with frequent painful crises and acute chest syndrome. Priapism is one of the complications that could be prevented by hydroxyurea, but there are few reports demonstrating the results. Since November 1993, hydroxyurea has been used in our clinic for preventing priapism in patients with stuttering or major attacks who are still capable of achieving intercourse on demand. Five patients were enrolled in the study, and 4 cases benefited by this treatment. After the initial treatment for the acute attack, all five patients developed stuttering priapism. Hydroxyurea was then introduced at the initial dose of 10 mg/kg, and as the hydroxyurea dosage increased, the number or length of priapism episodes decreased. One to two months after the maximal dose (20–35 mg/kg) was introduced, the episodes disappeared. In two patients, we were forced to administer over 30 mg hydroxyurea/kg to abort the episodes, and, in another patient, 25 mg/kg was necessary. All patients present normal sexual activity. Hydroxyurea was discontinued in two patients, but stuttering priapism reappeared. Hydroxyurea was then re‐introduced, and priapism disappeared. One patient, using 20 mg hydroxyurea/kg, had a 6‐year remission of priapism after hydroxyurea administration; however, he experienced stuttering priapism, 1 month before a major attack, that progressed to impotence. During that month, he did not seek medical attention. In conclusion, the data here presented suggests that hydroxyurea may prevent priapism attacks in sickle cell disease, probably at higher doses than usually prescribed for painful crisis prevention. Am. J. Hematol. 77:45–49, 2004. © 2004 Wiley‐Liss, Inc.
Clinical and molecular aspects of 23 patients affected by paroxysmal nocturnal hemoglobinuria
Authors: Boschetti Carla , Fermo Elisa , Bianchi Paola , Vercellati Cristina , Barraco Fiorenza , Zanella Alberto
Published: August 2004

We reviewed clinical and molecular data of 23 consecutive unrelated patients affected by paroxysmal nocturnal hemoglobinuria (PNH) (19 with hemolytic PNH, 3 with aplastic anemia/PNH, and 1 with myelodysplasia/PNH syndrome) with a mean follow‐up of 11.8 years. Five patients had thrombotic episodes, and 10 needed regular blood transfusions; 2 died for cerebral hemorrhage and kidney failure, and 2 spontaneously recovered from PNH. Twenty different PIG‐A gene mutations were detected in 21/23 patients: 15 frameshift, 1 splicing, 2 nonsense, and 2 missense mutations. Two mutations (DelG341 and IVS2 +1g‐a) were detected twice. A PIG‐A mutated clone was also revealed in the two patients in complete clinical remission. One patient with aplastic anemia/PNH syndrome was treated with two courses of antilymphocyte globulin and cyclosporin with partial sustained response. Six patients were given rHu‐EPO 150 U/kg/day sc for at least 6 months: one became transfusion‐independent for 8 months and then discontinued treatment for clinical complications; one displayed a mean rise of Hb of 1.5 g/dL and is currently maintaining Hb levels higher than 9 g/dL after 54 months of therapy. Mutation specific quantitative‐competitive PCR showed that the rise of hemoglobin was related to an increase of PIG‐A negative molecules, suggesting that the efficacy of rHu‐EPO therapy may be due to the stimulation of the abnormal clone. Am. J. Hematol. 77:36–44, 2004. © 2004 Wiley‐Liss, Inc.
Cavernous sinus thrombosis presenting with diplopia in an allogeneic bone marrow transplant recipient
Authors: Gupta Vikas , Keller Anne , Halliday William , Sanche Stephen , Hassegawa Wanda , Messner Hans , Kiss Thomas , Lipton Jeffrey
Published: August 2004

Although neurological complications are common in patients undergoing allogeneic bone marrow transplant (alloBMT), cavernous sinus thrombosis (CST) has rarely been described. An allograft recipient is described who presented with diplopia and developed CST in the early post‐transplant period likely caused by a filamentous fungus. Clinical course and serial radiological studies of this patient are described and correlated with autopsy findings. Am. J. Hematol. 77:77–81, 2004. © 2004 Wiley‐Liss, Inc.
Reversible posterior leukoencephalopathy syndrome complicating cytotoxic chemotherapy for hematologic malignancies
Authors: Tam C.S. , Galanos J. , Seymour J.F. , Pitman A.G. , Stark R.J. , Prince H.M.
Published: August 2004

Reversible posterior leukoencephalopathy syndrome (RPLS) is an uncommon but distinctive clinicoradiological entity comprising of headache, seizures, visual disturbance, and altered mental function, in association with posterior cerebral white matter edema. With appropriate management, RPLS is reversible in the majority of cases. Previous reported associations of RPLS include hypertension, eclampsia, renal failure, and use of immunosuppressive drugs; reports in the adult hematology setting are rare. We report two cases of adults undergoing treatment for hematological malignancies who developed RPLS, and we emphasize the importance of early recognition and institution of appropriate management in reducing the risk of development of permanent neurological disability. Am. J. Hematol. 77:72–76, 2004. © 2004 Wiley‐Liss, Inc.
Practical approach to the diagnosis and management of thrombocytopenia associated with tirofiban treatment
Authors: Mulot Agnès , Moulin Frédéric , Fohlen‐Walter Anne , Angioi Michael , Sghaier Mohammed , Carteaux Jean‐Pierre , Lecompte Thomas , de Maistre Emmanuel
Published: August 2004

Anti‐GPIIb/IIIa associated thrombocytopenia has been reported in most large trials, but very little data is available regarding tirofiban. We report three cases of thrombocytopenia, most likely attributed to tirofiban (two moderate and one severe). For each patient, laboratory investigation has allowed us to exclude another cause of thrombocytopenia, particularly heparin‐induced thrombocytopenia. Platelet count recovery could be earlier with tirofiban than with abciximab. Some suggestions are proposed for practical management of these patients with persistent thrombotic risk, who are sometimes candidates for cardiopulmonary bypass surgery. Am. J. Hematol. 77:67–71, 2004. © 2004 Wiley‐Liss, Inc.
Russia's imperial blood: Was Rasputin not the healer of legend?
Authors: Kendrick John M.L.
Published: August 2004

The only son of Russia's last Tsar, a great‐grandson of Queen Victoria, continues to be used as the favorite example of the X‐linked inheritance of hemophilia, in spite of the fact that this popular historical diathesis has never been confirmed by any form of modern medical laboratory testing. Certain to be controversial, a new study of the symptoms that were witnessed by those who were closest to the teenaged Russian heir now raises the possibility that his blood disorder might well have been something other than hemophilia. The key to discovering Tsarevich Alexei's true diagnosis is found in those now legendary allegations that the infamous “Mad Monk”, Grigory Rasputin, had possessed a power of healing that was somehow responsible for the young boy's mysterious history of spontaneous recoveries. If we are to accept the popular diagnosis of history and call it a clotting factor deficiency, then the boy's now famous sudden recoveries will remain a complete mystery. The so‐called “Mad Monk” Rasputin, as a direct result of the revolutionary propaganda of the time, is then overblown into a larger‐than‐life legend. If, however, we are to change the diagnosis and call it a platelet disorder, then the air is let out of the legend, and Rasputin is revealed to have been nothing more than a very ordinary middle‐aged Siberian hippie who did not possess any healing powers at all. Am. J. Hematol. 77:92–102, 2004. © 2004 Wiley‐Liss, Inc.
Double minute chromosomes in monoblastic (M5) and myeloblastic (M2) acute myeloid leukemia: Two case reports and a review of literature
Authors: Thomas Leno , Stamberg Judith , Gojo Ivana , Ning Yi , Rapoport Aaron P.
Published: August 2004

Double minutes (dmin) are small, paired chromatin bodies that lack a centromere and represent a form of extrachromosomal gene amplification. Although they have been found in a variety of solid tumors, their presence in hematological malignancies, especially acute myeloid leukemia (AML), is rare. In addition, the presence of dmin may be a mechanism for upregulated oncogene expression and is generally associated with a poor prognosis. We describe two patients who had dmin at initial presentation of AML, including the first case of M5a with C‐MYC amplification on dmin, and another case with C‐MYC amplification as the only cytogenetic finding. We review here a total of 33 cases with dmin in AML. C‐MYC was amplified by the dmin in 25 cases, while other putative oncogenes were amplified in the other 8. Am. J. Hematol. 77:55–61, 2004. © 2004 Wiley‐Liss, Inc.
Symptom onset in the first 2 years of employment at a wood products plant using diisocyanates: Some observations relevant to occupational medical screening
Authors: Wang Mei‐Lin , Petsonk Edward L.
Published: August 2004

Background

Questionnaires are essential tools for medical screening, but their role in monitoring workers at increased risk of occupational asthma (OA) remains indeterminate.

Methods

Employees who were at a newly established wood products plant without previous exposure to methylene diphenyl diisocyanate (MDI) completed an initial questionnaire and from one to four follow‐up questionnaires during a 2‐year period. Onset of symptoms in 132 workers was assessed by exposure groups and modeled using generalized estimating equations.

Results

Onset of attacks of dyspnea with wheeze, attacks of dyspnea or cough at rest, and chest tightness were significantly associated with MDI exposure after controlling for age, smoking, and wood dust exposure. Onset of cough on most days was significantly related to smoking and dust. Onset of phlegm production was significantly related to both MDI and dust exposure.

Conclusions

Onset of certain symptoms is significantly associated with MDI exposure. Early detection of MDI‐associated health effects using a short screening questionnaire appears feasible. Am. J. Ind. Med. 46:226–233, 2004. Published 2004 Wiley‐Liss, Inc.
Asbestos‐related pleural disease due to tremolite associated with progressive loss of lung function: Serial observations in 123 miners, family members, and residents of Libby, Montana
Authors: Whitehouse Alan C.
Published: August 2004

Background

The community of Libby, Montana has recently been the focus of national attention secondary to widespread amphibole contamination associated with vermiculite mining and processing.

Methods

Patients who had occupational and non‐occupational exposure to amphibole asbestos in Libby, Montana were evaluated for progressive loss of pulmonary function.

Results

Of the 123 patients evaluated, 94 demonstrated average age‐corrected accelerated loss per year of vital capacity at 3.2%, total lung capacity (TLC) 2.3%, and DLCO 3.3%. All patients all had predominantly pleural changes with minimal to no interstitial disease.

Conclusions

The study demonstrates a progressive loss of pulmonary function in patients exposed to tremolite asbestos. Am. J. Ind. Med. 46:219–225, 2004. © 2004 Wiley‐Liss, Inc.
Mineral fiber problems and their management: UK 1890–1935
Authors: Greenberg Morris
Published: August 2004

Background

The first intimation that mineral fibers other than asbestos were biologically active, is generally identified with reports of experimental studies by Stanton and Wrench, and Pott and Friedrichs, in the early 1970s. In 1890 however, man‐made mineral fiber was recognized to present human health hazards; in 1912 experimental study confirmed asbestos to be fibrogenic, and by 1935 other mineral fibers came to notice as potential lung hazards.

Methods

Published and archival sources have been reviewed to trace the emergence between 1890 and 1935 of the awareness of the health hazards of various fibrous minerals, and the development of strategies for their control.

Results

By the early 1900s there was evidence that the asbestos substituted for the man‐made mineral fiber insulation material employed to improve the thermal efficiency of steam powered engines, presented a serious health problem. In the early 1930s, other mineral fibers came to be suspected of causing pneumoconiosis.

Conclusions

Containment, local exhaust ventilation and personal respiratory protection were instituted for the amelioration of asbestosis, but because of the limitations of what was perceived to be reasonably practicable on economic grounds, and what was feasible technologically, their benefits were severely limited. Initial and periodic medical examination were introduced as precautionary measures, based on hope rather than on experience. When in the early 1930s, the investigation of coal mine dust and siliceous dust, threw up the hypothesis that fibrous natural mineral dusts other than asbestos might be fibrogenic, this was ignored and no further investigations were pursued and no precautionary measures were set in train. Am. J. Ind. Med. 46:304–311, 2004. © 2004 Wiley‐Liss, Inc.