bluuebunny/crossref_metadata_2025_split huggingface dataset:
L
Authors: Traboulsi Elias I 
Published: October 2023

Abstract
Many cases are sporadic. The proportion of familial cases depends on the mutation and ranges between 43% for the 11778 mutation and 78% for the 3460 mutation. Fifteen percent of families are heteroplasmic; that is, both mutant and wild-type mtDNA coexist within an individual. The level of heteroplasmy among different tissues can vary markedly. Mitochondrial DNA mutations alone are not sufficient to cause visual loss in LHON, since not all individuals harboring a pathogenic LHON mutation express the disease.
R
Authors: Traboulsi Elias I 
Published: October 2023

Abstract
Phytanic acid, a 20-carbon branched-chain fatty acid, is excessively stored in all tissues, and its serum levels are elevated. Night blindness appears in the teens, followed by peripheral neuropathy with gradual loss of sensations, muscle atrophy, and loss of deep tendon reflexes. Ataxia, nerve deafness, mental deterioration, and tetraplegia may occur. Patients may have ichthyosis. Cerebrospinal fluid protein is elevated.
S
Authors: Traboulsi Elias I 
Published: October 2023

Abstract
This is one of the most common autosomal recessive disorders. Most studies in Europe, the United States, and Canada have found an incidence of 1 in 20,000 births. In some regions, the disorder may occur as often as 1 in 10,000 births. The incidence appears to be much lower in Asian and African populations. The syndrome affects development before and after birth. It was described in three boys with poor growth, developmental delay, and a pattern of congenital malformations, including cleft palate, genital malformation, and polydactyly. Other clinical abnormalities include microcephaly, low-set ears, small upturned nose, webbing between the second and third toes, abnormal palmar creases (usually single), ambiguous male genitalia with hypospadias and undescended testicles, cataracts, blepharoptosis, heart defects, micrognathia, pyloric stenosis, short thumbs, and Hirschsprung disease.
T
Authors: Traboulsi Elias I 
Published: October 2023

Abstract
Deficient activity of the enzyme hexosaminidase A leads to the accumulation of GM2 ganglioside in ganglion cells of the brain and retina, with subsequent cell destruction, demyelination, and reactive gliosis. The gene is common in the Jewish population of eastern European origin. The carrier rate among the Jewish population of New York was estimated as 1 in 30 in 1964 versus 1 in 300 among non-Jews. The disease occurs in all ethnic groups. Consanguinity is significantly higher among non-Jewish parents of affected children.The affected child may be slow to achieve normal milestones in the first year of life. Sounds frequently cause an exaggerated startle response. There is a delayed ability to sit unassisted, and listless behavior and poor head control are seen. Seizures are common in the first year of life. Neurologic deterioration is rapid and leads to paralysis, deafness, and blindness by age 3 to During the end stage of the disease, cranial measurements may progressively increase and precocious puberty may develop because of hyperpituitarism.
X
Authors: Traboulsi Elias I 
Published: October 2023

Abstract
During the past two decades, our understanding of the molecular genetics of inherited eye diseases, their classification, and management has undergone a huge expansion as the field of human genetics has benefited from technological advances and increased interest by physicians and scientists in all fields. As a result, the amount of clinical and basic-science information on inherited systemic and eye diseases has become so large that general ophthalmologists, ophthalmic subspecialists, and physicians in other fields have found it difficult to keep up. This volume will act as a guide because it catalogues all the latest information about genetic diseases that involve the eye and presents it in a practical and accessible format. After an introductory chapter that reviews basic clinical and molecular-genetic principles, individual diseases and groups of diseases are listed alphabetically in order to make it as easy as possible to search for an entry. The material in each entry is a synthesis of numerous articles and reviews on the topic, accompanied by at least one high-quality illustration, at least one webpage of a patient support group or other organization related to the disease, and references that provide the original description of the disease, an excellent review, or useful illustrations. There is also a companion website containing electronic copies of all the illustrations to make it easy to use them in lectures. Health-care professionals who need immediate access to clinical and basic-science information on inherited systemic and eye diseases will find this volume indispensable.
Z
Authors: Traboulsi Elias I 
Published: October 2023

Abstract
involved in oxidative reactions that use molecular oxygen and produce hydrogen peroxide. The peroxisomopathies can be divided into two major groups: one of peroxisomal biogenesis and one of individual peroxisomal enzyme defects (see Table 25). Zellweger syndrome belongs in the former category. The major features of the syndrome include a characteristic facial appearance, deformations of the extremities and joints, abnormal cartilage calcification, hypotonia, seizures, profound psychomotor retardation, hepatomegaly, hepatic fibrosis, and renal cortical cysts.
Central Nervous System Stimulants: Cocaine, Amphetamines, Related Drugs, and Caffeine
Authors: Winger Gail , Woods James H , Hofmann Frederick G 
Published: October 2023

Abstract
Central nervous system stimulants are so called because, in contrast to opioids and CNS depressants, stimulants produce heightened awareness, decreased sleep and appetite, and increased locomotor activity. When lethal doses are ingested, death is likely to be due to cardiovascular complications or convulsions rather than to direct depression of respiration.
The Historical Context
Authors: Anaya S James 
Published: October 2023

Abstract
Like all systems of law, international law is the product of historical as well as modern elements. International law especially is rooted in jurisprudential strains originating in classical Western legal thought, although today it is increasingly influenced by non-Western actors and perspectives. The subject of indigenous peoples is not new to this genre of law but has figured with varying degrees of prominence in the legal discourse and practice related to international law’s evolution over centuries. Discussions of indigenous peoples have appeared frequently in major treatises and published lectures contributing to the development of international law since the dawn of systemic European encroachment on distant lands half a millennium ago. This chapter discusses the historical threads of legal thought and official behavior that relate to the development of international law prior to the middle part of the twentieth century. It is not intended as a comprehensive history of international law and its treatment of indigenous peoples but is, rather, an effort to provide a contextual backdrop for assessing international law as it concerns today’s indigenous peoples, the principal focus of this book.
Common Sense and Paine’s Republicanism
Authors: Foner Eric 
Published: October 2023

Abstract
Thomas Paine arrived in Philadelphia on November 30, 1774. Political change was in the air; it dominated discussion in the city’s taverns and coffee houses, homes and workshops. Already, the port of Boston had been closed as punishment for the Boston Tea Party of December 1773 and the First Continental Congress had assembled in Philadelphia in the fall of 1774, composed of “the ablest and wealthiest men in America.”
Introduction
Authors: Peace Richard 
Published: October 2023

Abstract
Dostoevsky was born in 1821 in a poor district of Moscow, where his father was a doctor at a paupers’ hospital. From an early age he was aware of poverty, illness, and eccentricity. As the second son of seven children brought up by an authoritarian father, his upbringing was strict. At the age of sixteen he left for the capital, St Petersburg, to study as a military engineer; Adele Lindenmeyr will argue that Dostoevsky’s own training as a draughtsman and his study there of architectural history are reflected on the pages of Crime and Punishment in Raskolnikov’s speculative plans for reshaping St Petersburg. Eighteen months into his course he learned of the death of his father, who was rumored to have been murdered by his own peasants. Later scholarship questions this assumption, as it does Freud’s assertion that news of his father’s death led to Dostoevsky’s first epileptic fit.
Empathy and Enmity in the Prioress’s Tale
Authors: Spector Stephen 
Published: October 2023

Abstract
Midway through the impassioned prayer that precedes her tale, Chaucer’s Prioress alludes to the Incarnation in terms of joined contraries conveying sublime paradox:
The force and economy with which these contradictions are joined are rhetorically heightened by the chiastic structure of the first of these lines, “O mother Virgin, O virgin Mother noble.” The following line too is structurally compressed, juxtaposing contraries in the “unburnt, burning” bush, a familiar type of Mary, who “was fyred brente not, for she was moder without losse of maydenhod.”2 In the next two lines, the Prioress astonishingly transforms the Gospel account of the Incarnation, in which Gabriel tells Mary that the Holy Spirit will come upon her, and the power of the Most High will overshadow her (Luke 1:35). In the nun’s fervent reworking, it is instead the Virgin who ravishes the Holy Ghost down from the Deity! Yet the heat and energy suggested in the word ravyshedest are paradoxically contained, like the fLames of the burning bush, within the chastity of the Conception.3 And the fact that Mary ravishes the Holy Ghost through her humility, compelling or possessing through acquiescence, further extends the paradox in this prayer. The joining of contraries in paradox is thus as crucial to the Prioress’s prayer as the joining of contradictions in irony is to her General Prologue portrait. But there is still another contradiction here: that the Prioress, whose faith and emotion seem so shallow and misplaced in the General Prologue, should utter so ardent a prayer at all.
Fighting for the Mind of Mankind
Authors: Manela Erez 
Published: October 2023

Abstract
On March 5, 1917, in the inaugural address of his second term in office, Woodrow Wilson once more articulated a vision for the postwar world order. The plan he outlined then included all of the practical elements that have since come to be associated with the Wilsonian, or liberal internationalist, prescription for the conduct of international relations: institutions of collective security, the reduction of armaments, free trade. International order and cooperation, he declared once again, could not last long unless it stood on the principles of the equality of nations and of government by consent, and the United States would insist both on “the actual equality of nations in all matters of right and privilege” and on the principle that “governments derive all their just powers from the consent of the governed.”
From Paris to Amritsar
Authors: Manela Erez 
Published: October 2023

Abstract
The men that the Indian National Congress appointed to represent India at the peace conference—Tilak and Gandhi among them—were not in attendance when the conference convened in January. The government of India did send a delegation to Paris, which could participate, the powers agreed, in deliberations that touched upon Indian interests. The delegation, however, was headed by the Secretary of State Montagu, and its Indian members, Sir (soon to be Lord) S. P. Sinha and Ganga Singh, the Maharaja of Bikanir, were handpicked by the British and represented their interests.
Business Politics
Authors: Postel Charles 
Published: October 2023

Abstract
John W. H. Davis grew cotton and shouldered the burdens of poverty and family sickness in the piney woods of Grimes County, Texas. In 1894 he campaigned as the People’s party nominee for county tax collector. When on the stump he articulated to his fellow farmers the relationship between local and national politics. Although concerned about the role of “political trixters and town rings” in local affairs, he explained that the problems farmers faced were primarily national. They flowed downward from Washing- ton, where the politicians had produced the most “vissous and viloness class legislation... on reccord in any country at any time.” He further explained that this political record had to be understood within the context of national commerce. To the charge that Populist demands for national reform had no place in a contest for county tax collector, Davis responded: As a young man Davis had served as a private in the Confederate Georgia Infantry, and with little formal schooling he penciled his notes with a rough and untrained hand. Yet for seven years he had devoted himself to the cause of Alliance education.
Race Progress
Authors: Postel Charles 
Published: October 2023

Abstract
The formation of the People’s party posed a racial dilemma. In the early 1890s, African Americans still clung to the right to vote in much of the South. Their choices, however, were growing increasingly narrow. The Republican party had lost its former strength in the South, and what remained often adopted “lily-white” policies of racial exclusion. At the same time, the Democratic party offered the black community small concessions in exchange for votes, with the understanding that blacks would acquiesce to the Democratic doctrines of white supremacy. For the new People’s party to have any chance of success at the polls it had to attract a section of the black vote without undermining its support among white farmers. This was a politically difficult and dangerous undertaking for the emerging People’s party across the South. In Texas the task largely rested on the able shoulders of John B. Rayner. Rayner’s trajectory was similar to that of other Populist leaders. Born in 1850, he was the son of U.S. Congressman Kenneth Rayner, whose North Carolina plantation fell on hard times after the Civil War. John studied the classics, labored in county politics, and eventually moved to Calvert, Texas, where he taught school. Along the way, he trained as a Baptist preacher and much like Harrison Ashby, S. O. Daws, and other Protestant ministers in the ranks of Populism, he took a skeptical view of both church and creed and looked to improve people’s lives in the here and now. In 1892 Rayner joined the People’s party. With his gift for public speaking, he gained renown as he campaigned across the remote country districts of eastern Texas. By 1895 he served on the party’s state executive committee. Soon thereafter, as the Populist fires cooled, he devoted his attention to farmers’ technical education.
Confederation
Authors: Postel Charles 
Published: October 2023

Abstract
On his arrival in Chicago in the fall of 1895, the Texas Populist John Flaherty found the reform movement in the big city mired in an ideological muddle. “To you Texas populists who have a fixed and sound idea of the political and monetary conditions,” he wrote the Southern Mercury back in Dallas, “it is incomprehensible how people can be so blind to their own best interests.” Chicago reformers “go and hear the single tax man and shout themselves hoarse over his panacea for the public ills. They shout the same acclaim to the international bi-metalic lunatic, and roar with delight at the 16 to 1 silver tongued orator.” In a “wilderness of doubt and uncertainty,” the clutter of innovations, schemes, and proposals competing for the attention of the city dweller contrasted sharply with the “keen kernels of truth, without husk or chaff,” that the rural reform press provided its readers.
Conclusion
Authors: Postel Charles 
Published: October 2023

Abstract
“You shall not crucify mankind upon a cross of gold.” With this indictment of the financial establishment, William Jennings Bryan, a young congress- man from Nebraska, electrified the delegates to the Democratic Convention gathered under the hot roof of the Chicago Coliseum in July of 1896. On the fifth ballot, Bryan won a surprise nomination as the presidential candidate. The nomination marked a historic shift in party alignment, with the Democratic party embracing a platform of minting silver, a federal income tax, and other reforms demanded by rural and labor constituencies. Two weeks later, at its convention in St. Louis, the People’s party also endorsed Bryan for president. For Republicans, and a considerable number of “sound money” Democrats, the Populist endorsement confirmed their worst fears about the Bryan campaign. They viewed the Populists and their reckless currency doctrines as instruments of “anarchy” and “lawlessness.” Theodore Roosevelt, the police commissioner of New York City at the time, believed that the Populists were “plotting a social revolution,” and to check their efforts at “subversion” he proposed lining up twelve Populist leaders against a wall and “shooting them dead.”
Acquiring, Building, and Sustaining Power Black Civic Organizations in Urban Democracy
Authors: Thompson J Phillip 
Published: October 2023

Abstract
On December 14, 1994, the city of Baltimore passed an ordinance requiring companies bidding for service contracts with the city to pay employees on city jobs a minimum of $6.10 an hour, with annual increases totaling up to $7.70 an hour by July 1999, and, after July 1998, with wages tethered at no percent of the poverty income for a family of four. In July of 2000 this ordinance provided $8.03 an hour, $2.88 above the federal minimum wage of $5.15. For each day a new wage was unpaid, employers would be fined $50 per worker (Fine 2003, 227, 229). The Baltimore “living wage” ordinance was the first in the United States. Over the subsequent six years, similar ordinances passed in dozens of U.S. cities. The leading force in the living wage campaign was Baltimoreans United in Leadership Development (BUILD), a showcase demonstration of the power low-income groups could wield if organized and focused.
Race, Class, and ldeolouv in a New York Mayoral Election Conservative White Mobilization and Resurgent
Authors: Thompson J Phillip 
Published: October 2023

Abstract
Back in 1978, when Ed Koch was mayor of New York, a thirty-five-year-old businessman and community leader in Crown Heights, Brooklyn, named Arthur Miller had been “choked to death by a policemen trying to arrest him during a street confrontation.” The police had been attempting to issue a summons to Miller’s brother (Treaster 1978a). It was the fifth controversial killing in five years of a black man by police officers in Brooklyn (Raab 1978).1 When the deputy medical examiner said he found, “no evidence of excessive, savage beating” (Treaster 1978b), many in the black community complained of a cover-up. Two days later, a sixteen-year-old black youth, Victor Rhodes, was beaten into a coma by a group of whites “in Hasidic garb” in a largely Hasidic populated block, also in Crown Heights, an assault The New York Times quoted the police as describing as one of a long string of incidents in which, “members of the Hasidic patrols, who refuse to join the precinct’s patrol program, have become involved in altercations with black residents” (New York Times 1978). A black minister, Rev. Herbert Daughtry, with twenty church and civic organizations in tow, led a demonstration at city hall to protest Miller’s death.
Pandora’S Box Subjectivity, Class, and Sexuality in Socialist Feminist Criticism
Authors: Kaplan Cora 
Published: October 2023

Abstract
Stories of seduction and betrayal, of orphaned, impoverished heroines of uncertain class origin, provided a narrative structure through which the instabilities of class and gender categories were both stabilized and undermined. Across the body and mind of “woman” as sign, through her multiple representations, bourgeois anxiety about identity is traced and retraced. A favorite plot, of which Jane Eyre is now the best-known example, sets the genteel heroine at sexual risk as semi-servant in a grand patriarchal household. This narrative theme allowed the crisis of middle-class femininity to be mapped on to the structural sexual vulnerability of all working-class servants in bourgeois employment. Such dramas were full of condensed meanings in excess of the representation of sexuality and sexual difference. A doubled scenario, in which the ideological and material difference between working-class and bourgeois women is blurred through condensation, it was popular as a plot for melodrama with both “genteel” and “vulgar” audiences.
“That Stormy Sisterhood” Portrait of the BrontëS
Authors: Michie Helena 
Published: October 2023

Abstract
This, the only surviving portrait of the three of them, groups them forever around phallic absence, the organizing principle, the painter, their brother. Branwell’s decision to erase himself from this painting of his sisters only draws attention to the figure that once represented him, now alluded to by a vertical swath of yellowish paint. In so vividly depicting his own absence, he turns himself—hesitantly—into a phallic presence, into the column that comes to stand, in Charlotte’s portraits of Brocklehurst and St. John, for the sin of male arrogance. Branwell under erasure reminds us in a way no signature could that he is the painter, that his sisters sit in reference to him. Branwell’s arrogance, however, is no simple thing; his sisters will leave their mark, and he will leave his through scarring them; Emily’s biography is especially marked by Branwell’s entrances and exits, his delirium tremens, his drunkenness, his genius, and his shameful death that some say showed the way for Emily’s own.
Introduction Broken Process
Authors: Davis Richard 
Published: October 2023

Abstract
On June 14, 1993, after introducing his first Supreme Court nominee to the nation in a Rose Garden ceremony, President Clinton turned the podium over to the 60-year-old Brooklyn-born judge. Ruth Bader Ginsburg gave a moving speech, including warm praise for her mother, and then closed with a touching self-challenge: “I pray that I may be all that she would have been had she lived in an age when women could aspire and achieve, and daughters are cherished as much as sons.” Tears welled up in the president’s eyes as he and the audience gave Ginsburg warm, sustained applause. He was pleased. His announcement and Judge Ginsburg’s short speech reinforced the White House’s effort to show that the president had made history by choosing the second woman to the Court and a long-time legal activist for women’s rights.
Traditional versus New Players
Authors: Davis Richard 
Published: October 2023

Abstract
The day President Woodrow Wilson announced the nomination of Louis Brandeis to a seat on the U.S. Supreme Court, the press cornered Brandeis at a social event and asked for his reaction. Brandeis replied: “I have not said anything and will not.” Brandeis said a lot privately about the confirmation battle that swirled around him, but he steadfastly maintained his public silence. That was 1916. Today, no Supreme Court nominee would dare make such a statement. Instead, they would be required to sit before a phalanx of senators at a hearing broadcast live to the nation and answer questions for several days. They would be expected to answer lengthy questionnaires and, if necessary, appear with the president at press conferences.
How the Process Broke The Transformation of the Supreme Court Appointment Process
Authors: Davis Richard 
Published: October 2023

Abstract
On July 1, 1987, President Ronald Reagan announced that his choice to replace Justice Lewis Powell was Judge Robert Bork, a well-known judge on the District of Columbia Court of Appeals and a former acting attorney general of the United States. Bork had long been the favorite of conservatives, but his nomination by Reagan signaled that the administration was willing to confront its opponents with a controversial nominee.