bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mitochondrial Disorders
Authors: Boles Richard , Moseley Kathryn 
Published: November 2023

Abstract
The mitochondrial disorders represent a very large number of separate inborn errors of metabolism. They result from defects in the genes encoding proteins involved in cellular energy metabolism, including pyruvate dehydrogenase complex subunits, Krebs cycle enzymes, electron transport chain (ETC) components, ETC assembly proteins, mitochondrial inner membrane transporters, mitochondrial nucleotide pool regulators, and factors that interact with mitochondrial DNA (mtDNA). In most cases, the ETC is involved, either primarily or secondarily. Components of the ETC are encoded by nuclear genes, in which case disorders are usually inherited in an autosomal recessive manner, and by mtDNA genes, in which case disorders are usually maternally inherited. Mitochondrial medicine is exceedingly complex. Mitochondrial disease is highly variable in degree of severity, age of onset, organ/tissue involvement, and mode of inheritance. In terms of severity and age of onset, mitochondrial disease can cause anything from migraine headaches in an adult to lethal multisystem disease in an infant and every degree of illness in between. In terms of organ/tissue involvement, as energy is a universal cellular requirement, essentially any organ or tissue can fail and result in disease. However, since disease manifestations occur in general when the local energy supply is inadequate to meet the local energy demand, disorders of tissues with the highest energy requirements usually predominate: nerve, muscle, endocrine gland, and renal tubular epithelium.
Galactosemia
Authors: Calcar Sandy Van , Wolff Jon 
Published: November 2023

Abstract
Galactose metabolism involves three enzymes: galactokinase, galactose-1-phosphate uridyl transferase (GALT) and uridine diphosphate-4-epimerase (Fig. 49–1). Inborn errors of metabolism (IEM) have been described for all three enzymes. The most common inborn error in galactose metabolism is deficiency of the GALT enzyme. Its incidence in North America is approximately 1 in 60,000. Galactose levels and/or GALT activity are routinely tested in all infants by newborn screening programs. Infants with elevated galactose and/or decreased enzyme activity should be referred to a biochemical genetics program that specializes in treatment of IEM. This chapter will focus on GALT deficiency since the classical form of the disease requires lifelong dietary treatment. In classical galactosemia, the GALT enzyme is not produced and the affected individual has no enzyme activity. Neonates with untreated classical GALT deficiency present a few days after starting milk-based formula or breast milk feeding. The initial symptoms include poor feeding, vomiting, and jaundice. Failure to thrive, hepatomegaly, edema, splenomegaly, and sepsis can follow. The disease is rapidly fatal if milk feedings continue. For surviving infants, mental retardation and behavioral problems are common.
Celiac Disease
Authors: Heubi James , Carlsson Annelie 
Published: November 2023

Abstract
Celiac disease or gluten-sensitive enteropathy, was first described by the English physician Samuel Gee in 1888. In the 1940s, Dicke was the fist to link the ingestion of gluten-containing foods with symptoms. Over the past two decades, a considerable body of knowledge about this condition has accumulated. Previously considered a rather unusual disease with a prevalence of 1:3000 in the United States, except in children and adults of western Irish background, it is now recognized to be much more common. With the development of reliable screening tests using antibodies to gluten, endomesium, and tissue transglutaminase, the disease is now believed to affect as many as one in 300 Europeans and Americans.
Brain and Spinal Cord
Authors: Hunter Alasdair G W 
Published: November 2023

Abstract
Anencephaly is immediately apparent at birth and represents the end stage of degeneration and regeneration of the exposed cephalic neural tissue (Fig. 16-1). Failure of the cephalic neural tube to close appears to be the primary defect. The neural groove and folds can be seen by embryonic stage 8 (day 18) and have deepened and begun to fuse by stage 10 (day 22). The cephalic neural tube closes in a bidirectional fashion by stage 11 (day 24). It has been argued, based primarily on experimental animal models, examination of the pattern of neural tube defects (NTD) in humans, and the different sex ratios associated with lesions at different levels of the neural axis, that there are up to five initiation sites for fusion in humans.
Limbs
Authors: Stevenson Roger E 
Published: November 2023

Abstract
Limbs share with the craniofacies great susceptibility to mal- formation, disruption, deformation, and morphologic varia- tion. The susceptibility of the limbs to morphologic alteration can be attributed in part to their developmental complexity, the extended period of morphogenesis, and the exposure of the limbs beyond the protection of the body wall. Normal limb development also requires movement, a function influenced primarily by the nervous system and muscles. A vast number of single gene muta- tions and nearly all human teratogens and cytogenetic aberrations affect limb morphology in some way.
Introduction
Authors: Okamoto Shigeko , Shibamoto Smith Janet S 
Published: November 2023

Abstract
Models of Japanese society and culture, in particular the Nihonjinron ‘theories on the Japanese’ literature, commonly stress such attributes as homogeneity, harmony, consensus, and hierarchy, including gender stratification (Yoshino 1992, Befu 2001, Sugimoto 2003). In line with this framework, Japanese sociolinguistic practice (e.g., the use of honorifics, address terms, and indirect speech styles) as part of Japanese cultural behaviors has been described primarily based on context-independent, abstractly normative usage in Standard Japanese.
The Burials: Ritual and Taphonomy
Authors: Svoboda Jiří A 
Published: November 2023

Abstract
All Gravettian burials of Moravia (in contrast to the cave burials of the Gravettian and Epigravettian in Italy, for example) share the same practice of burying in the open air. However, the details of the deposition show considerable variability as a result of human decisions and mortuary behavior. The human burials within the Dolní Věstonice–Pavlov area have patterns of uniformity, depositing either complete human bodies or small fragments in the central parts of the settled areas.
The Human Remains: A Summary Inventory
Authors: Holliday Trenton W , Hillson Simon W , Franciscus, Robert G , Trinkaus Erik 
Published: November 2023

Abstract
The human remains have been inventoried almost completely and in detail by Sládek et al. (2000). However, to provide a quick reference for the material in the context of its discussion in this volume, we present below a summary inventory of the remains. This starts with an overall list of the remains discovered since 1925 at the sites of Dolní Věstonice I (Table 5.1); Pavlov I (Table 5.2); and Dolní Věstonice II, top and upper western slope, western objects from these sites (Vlček, 1951, 1991; Králík et al., 2002) are not addressed here.
The Ages at Death
Authors: Hillson Simon W , Franciscus Robert G , Holliday, Trenton W , Trinkaus Erik 
Published: November 2023

Abstract
The assessment of the ages at death of the skeletal and dental remains from Dolní Věstonice and Pavlov involves both the consideration of associated skeletons for whom there are multiple lines of evidence and the evaluation of isolated remains for some of whom it is possible to provide a reasonable age at death other than immature or mature.
The Cranial Remains
Authors: Franciscus Robert G , Vlček Emanuel 
Published: November 2023

Abstract
The human remains from Dolní Věstonice and Pavlov include a number of relatively complete crania (Chapter 5; Sládek et al., 2000). Specimens with essentially complete neurocrania and splanchnocrania include Dolní Věstonice 3 and 13–16. Less complete specimens include Pavlov 1, which retains a calvarium with small portions of the basicranium and left zygomatic, as well as portions of the left and right maxillae, and Dolní Věstonice 11/12, which retains a less complete calotte. There are also alveolar fragments associated with maxillary teeth that make up Pavlov 2. The Dolní Věstonice 3 and 13–16 crania, although largely complete, were reconstructed from a number of fragments, in most cases suffered ground pressure deformation, and have been restored to varying degrees with insoluble filler and painted. This condition of the crania frequently makes it difficult to ascertain exact bone joins and details of surface anatomy. The last point is particularly true for Dolní Věstonice 3. The neurocrania are generally better preserved than the splanchnocrania throughout the sample, although in some cases basicranial portions are also relatively more incomplete or damaged. All of the cranial specimens except Dolní Věstonice 11/12 also retain relatively complete mandibles, which are considered in Chapter 10.
The Costal Skeletons
Authors: Holliday Trenton W 
Published: November 2023

Abstract
The costal skeleton [costae (ribs) and sternebrae] is reason- ably well represented among the skeletons from Dolní Věstonice and Pavlov. It is best preserved in the triple burial individuals: Dolní Věstonice 13–15, each of which preserves at least one (and more often both) rib(s) from each thoracic level (Sládek et al., 2000; Chapter 5). In this chapter, detailed morphological description of each individual costal element is given, working craniocaudally through the ribs and ending with the sternebrae. Following these descriptions, osteometric analyses are under- taken. These analyses compare the Dolní Věstonice and Pavlov costal morphology with that of recent and penecontemporary humans. Discussion of the measurements and indices used in these analyses, as well as the compositions of the comparative samples, comes at the beginning of each analytical section. As mentioned in Chapter 14, the less complete axial remains of Dolní Věstonice 16 and Pavlov 1 were separated during excavation and partially reconstructed in the laboratory. In contrast, the costal skeletons of the triple burial individuals were removed en bloc to the laboratory, cleaned, and then reconstructed.
Introduction: Corporate Governance and Capital Flows in a Global Economy
Authors: Cornelius Peter K , Kogut Bruce 
Published: November 2023

Abstract
The most important challenge of the early part of the 21st century is to foster the prospects for improvement in the quality of life of citizens in the poorest countries in the world. Investment is a critical ingredient to meeting this goal. For investment to take place, capital must be borrowed on international markets and invested in poorer countries. The resources of official development agencies are far from sufficient for this task. Over the past decade the private sector has become the primary source of funds for emerging-market economies; only the poorest countries receive the plurality of their funds from official sources. The challenge of addressing the poverty that affiicts billions of people will require the flow of private international capital.
Corporate Risk Assessment and Business Strategy: A Prime Task for Senior Management
Authors: Henisz Witold J , Story Jonathan 
Published: November 2023

Abstract
How can we make corporate strategy in such a volatile world economy? Answering this question requires us to look at a much broader canvas than we do when we consider business strategy conventionally. Conventional business strategy divides conveniently into three parts, like Caesar’s description of Gaul: what’s going on inside the corporation; what is happening in terms of competition in the firm’s markets; and, by deduction, what both inquiries hold for the firm’s future. The recent pressure for improved corporate governance is but one example of this approach, which no doubt has the advantage of bringing management to focus on essentials-but in a world undergoing complex transformation, it is an approach that we believe is-to say the least-inadequate.
Corporate Governance and Private Capital Flows to Latin America
Authors: Chong Alberto , lzquierdo Alejandro , Micco Alejandro , Panizza Ugo 
Published: November 2023

Abstract
Following the debt crisis of the 1980s, Latin America was subject to a surge in private capital flows in the early 1990s. Net private capital flowing to the seven biggest Latin American economies representing about 90 percent of total net flows to the region for the period 1990-2001 not only quadrupled but exceeded official flows by five times.The obvious question is, why? In fact, to some extent, capital inflows were due to two factors: external developments mainly in the United States and the introduction of Brady bonds. Evidence on the importance of external factors has been provided recently. For instance, Chuhan, Claessens, and Mamingi (1993) show that equity flows seem to be more sensitive to external factors than bond flows, and that bond flows are generally more sensitive to a country’s credit rating than equity flows.
That Man In The White House
Authors: Jackson Robert H 
Published: November 2023

Abstract
In February Of 1934, I went with the Administration as counsel to the Bureau of Internal Revenue and shortly thereafter began to attend occasional meetings with the President in reference to a new tax program and other tax problems. These early meetings left a deep impression because, in contrast with what I had known of him before his election, I was some what surprised at his ease and aptitude for the role of Chief Executive of a nation in distress.
He had realized his ambition and fulfilled his idea of his own destiny. He took over his high office as if born for the part. He never appeared selfconscious about his position or surprised to find himself in it. He never tried to impress one with the magnitude or the difficulty of his job—he simply went ahead and did it. He made no self-deprecating remarks about his adequacy for its tasks. He was so self-assured that I never heard him assert his right to command or remind anyone that it was he who was President.
Cross-Sectional Determinants of Expected Returns
Authors: Brennan Michael J , Chordia Tarun , Subrahmanyam & Avanidhar 
Published: November 2023

Abstract
In theory, the primary determinant of the cross section of expected returns on securities is risk, although other factors such as taxes, liquidity, agency, and informational considerations can also, in principle, affect the demand for securities and therefore expected returns. The measure of risk that is relevant for pricing is the covariance of the security’s return with the pricing kernel, which may be expressed in terms of one or more return factors, depending on the particular equilibrium model. Thus, in the Capital Asset Pricing Model (CAPM), the pricing factor is the return on the market portfolio; in the consumption CAPM, it is the return on the portfolio that is most highly correlated with the growth rate of consumption; in the Epstein and Zin (1989) recursive utility CAPM, it is a linear function of the return on the market and the return on the maximum consumption correlation portfolio; and in the Arbitrage Pricing Theory (APT) it is a linear function of a set of priced factors that span the pricing kernel.
Introduction
Authors: Goldsmith Jack L , Posner Eric A 
Published: November 2023

Abstract
International law has long been burdened with the charge that it is not really law. This misleading claim is premised on some undeniable but misunderstood facts about international law: that it lacks a centralized or effective legislature, executive, or judiciary; that it favors powerful over weak states; that it often simply mirrors extant international behavior; and that it is sometimes violated with impunity. International law scholarship, dominated for decades by an improbable combination of doctrinalism and idealism, has done little to account for these characteristics of international law. And it has made little progress in explaining how international law works in practice: how it originates and changes; how it affects behavior among very differently endowed states; when and why states act consistently with it; and why it plays such an important role in the rhetoric of international relations. This book seeks to answer these and many other related questions.
Case Studies
Authors: Goldsmith Jack L , Posner Eric A 
Published: November 2023

Abstract
Customary international law fills volumes of treatises, and we cannot try to show that all of it follows the logic of our models. Instead, we examine in detail four areas of customary international law chosen on the basis of their prominence and on the availability of a detailed historical record. The four case studies we examine are the “free ships, free goods” rule of wartime maritime commerce; the breadth of the territorial sea; ambassadorial immunity; and the wartime exemption from prize for coastal fishing vessels. We show that these areas of supposedly robust customary international law never reﬂected universal behavioral regularities and that the actual state behaviors associated with these laws are most easily and parsimoniously explained using our four models.
In Substance, and in Principle, the Same as It Was Heretofore the customary constitution
Authors: Kramer Larry D 
Published: November 2023

Abstract
The idea of a “constitution” was not new in 1787 or even in 1776. Americans had a concept of constitutional law and well-developed ideas about the nature of a constitution long before they sat down to write any of their own. From a historian’s perspective, this concept reached back at least as far as the struggles between James I and Parliament, though men of the seventeenth and eighteenth centuries liked to speak of an “Ancient Constitution” whose practices had been followed since “time out of mind.” Colonial Americans were wedded to the principles of this constitution, intimately familiar with its terms and convinced of its essential rightness and wisdom.3 The patterns of thought and action formed by this experience naturally shaped their understanding of the task of writing new constitutions after the Revolution and thus provide a necessary starting point in recovering the original Constitution.
Implied Volatility Surface: Calibrating the Models
Authors: Ho Thomas S Y , Lee Sang Bin 
Published: November 2023

Abstract
The valuation of interest rate derivatives using an arbitrage-free interest rate model requires the inputs of the spot yield curve and the term structure of volatilities (or a volatility surface). We have discussed the importance of volatilities for option pricing. Without volatilities, options simply reduce to cash flows, which can be valued by the bond model. Volatilities affect the option value.
High-Yield Corporate Bonds: The Structural Models
Authors: Ho Thomas S Y , Lee Sang Bin 
Published: November 2023

Abstract
Credit risk is an important determinant of the value of a corporate bond. It is important because credit risk is prevalent in corporate bonds and may affect the bond value significantly. Few corporate entities (if any) have investment grade ratings which could match the creditworthiness of the U.S. government. One can then say all corporate bond issues have credits risks that vary by a matter of degrees. Many firms have such significant credit risk that they are on the verge of bankruptcy. In this case, their bonds are trading at a fraction of their principal value.
Strategic Value of a Firm: Real Options
Authors: Ho Thomas S Y , Lee Sang Bin 
Published: November 2023

Abstract
Thus far, we have discussed two aspects of shareholders” value. Chapter 11 described the financial models for the assets and liabilities to determine their fair value, as opposed to the book value. Market equity value, which is the asset fair value net of the liability fair value, is a measure of the value that the shareholders would claim on the firm if the firm were to liquidate by retiring all the debts at the market prices and selling the remaining assets to investors. In chapter 12, we described how a relative valuation model can be used to determine the market capitalization of the firm. Market capitalization of the firm is the shareholders” value of the firm as a going concern.
Structured Finance: Foreign Exchange Models
Authors: Ho Thomas S Y , Lee Sang Bin 
Published: November 2023

Abstract
In the previous chapters, we have described some of the financial models used in investment and financing decisions, and we have shown how capital market financial models and corporate finance are often inseparable. We will illustrate this idea further in this chapter by considering a case study of a structured finance deal. We will also describe some of the financial models in foreign exchange markets.
Culture, Social Capital, Institutions
Authors: Meier Gerald M 
Published: November 2023

Abstract
When analyzing why there are crosscountry differential rates of growth, it may be tempting to appeal to national “culture.” In the early days of the subject there were studies of the sociocultural and social psychology aspects of development problems and modernization (Hagen 1968; Hoselitz 1967; McClelland 1961). These efforts, however, did not make much of an impression among economists, and mainstream thinking about development did not focus on the social and institutional dimensions of economic development.
Global Trade Issues
Authors: Meier Gerald M 
Published: November 2023

Abstract
A central feature of globalization is the growing importance of international trade. The share of world trade for many developing countries has increased, but for the poorest countries the share has actually declined. For the “globalizers,” their increase in trade and their gains from trade contribute to their gains from growth. Trade policy, however, affects the amount and distribution of the gains—both from trade and growth. Trade policy, in turn, is shaped to a large extent within the context of the GATT/WTO system. As nations undertake another round of trade negotiations under the new Doha Development Agenda (2001), the overriding challenge is to establish a global rule-based trade architecture that is more supportive of development.
Setting the Scene
Authors: Barabba Vincent P 
Published: November 2023

Abstract
The following story represents thirty years of practical experience and observation. It reveals several major issues faced by any enterprise trying to transform itself so that it can grow and increase its operating margins and services to society. It also sets the stage for thinking about how to start with your destination. Traditionally, there have been two primary approaches used in attempting to grow and increase margins: (1) improving the manner in which the enterprise is currently doing business, or (2) moving into promising areas of new business. This story focuses on the interaction between the two traditional approaches and describes demonstrated processes for addressing the interactions of both-doing one and the other versus doing one or the other. The story also identifies the manner in which strategic thinking procedures, including creating an idealized design of what you want to be, can assist leaders interested in transforming the enterprise-without destroying it in the process. The story is hypothetical, but most readers will have experienced something like the #x201C;problems#x201D; the participants face, though perhaps not the outcome.