bluuebunny/crossref_metadata_2025 huggingface dataset:
What is menopausal hormone therapy?
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

The term “hormone replacement therapy” (HRT) probably has a commercial origin but doctors and women accepted it for several decades. That changed after the release of the interim report from the Women’s Health Initiative (WHI) study in July 2002. Shortly afterward, the National Institutes of Health recommended that the term “menopausal hormone therapy” would be preferable. The familiar term “hormone replacement therapy” will thus soon become obsolete as the Food and Drug Administration requires pharmaceutical companies to act on this recommendation regarding nomenclature. Because menopause is brought about by the decrease in levels of ovarian hormones, the initial rationale was that replacing these hormones could offset the deficiency. The term “HRT” implies that the replacement of the diminished natural hormone production prevents the symptoms of estrogen deprivation. “Replacement” would be an acceptable description of the hormone therapy if, indeed, one could determine the precise hormonal status of perimenopausal women and were able to customize treatment for each woman. This is not what usually happens and, in fact, would be very impractical. Efforts have been made to do this hormonal calibration using a simple saliva test. The test has been available for many years but it is not widely used. In most cases, it is no more helpful than having the health care provider determine what may be required on the basis of an examination and the symptoms reported by a patient.
Why should women consider hormone therapy?
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

For most women, the onset of menopause is easily recognized. As the name implies, menstrual periods stop. This is when women first start to wonder whether they should consider hormone therapy. Hot flashes are the symptom that most of the time prompts women to seek guidance on menopause from a doctor or other health care provider. Hot flashes are an indication of vasomotor instability causing dilation of small capillaries thought the body. This causes a sudden flushing and reddening of the face, usually accompanied by perspiration. When hot flashes occur at night, sleep is often interrupted. Many women awaken night after night, their bed drenched in perspiration. During the day, the hot flashes occur unpredictably and can be the cause of awkward social and professional situations. At menopause, estrogen production by the ovary falls below a critical level. Hot flashes and associated sleep disturbances are caused by this estrogen deprivation. Many scientific studies have shown conclusively that estrogen administration prevents hot flashes. These studies have compared the effectiveness of placebos and estrogen and find an enormous difference. Symptoms are controlled in almost all estrogen users. For women who choose not to use estrogens or any other therapy, hot flashes usually last from one to two years before abating spontaneously, but about 25% find that the condition persists for five years or longer.
Can HT protect brain function and prevent Alzheimer’s disease?
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

The commonly held belief that there is decreased cognitive function (mental acuity) with age is justified, but there is great individual variation. Some mental processes do slow down with age, but many do not. Studies from several countries including the United States have reached this conclusion. Research into aging has been greatly advanced by the Study of Aging in America program sponsored for more than a decade by Chicago’s MacArthur Foundation. As part of this research, millions of dollars were provided to scientists and doctors around the country to support studies on aging within their particular areas of expertise. John Rowe and Robert Kahn present the results of the Aging in America Study in their book, Successful Aging. Fears of mental decline, loss of memory, and especially of Alzheimer’s disease are widespread among older people. According to the Aging in America study, about 10% of people over the age of 65 may have Alzheimer’s disease, and the proportion increases with age. Among the oldest, people from 85 to 100 years of age or more, as many as 50% may have some degree of Alzheimer’s. These are probably reliable statistics; specialists believe that cognitive tests and medical examinations now available enable them to make a diagnosis with 90% confidence.
Herbal products for menopause
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

Doctors are usually unwilling to make recommendations that are not based on evidence. Our professional decisions are guided by the information and experience of modern medicine, arguably the best and most sophisticated the world has ever known. Sometimes, this elicits the criticism that we medicalize conditions that are a normal part of life. Disapproval is leveled particularly at medical management of the menopause, an event which predictably occurs in the lives of all women who survive into their 50s and beyond. The complaint is that many health care providers are likely to recommend hormone treatment without considering other options, while failing to provide common-sense guidance on healthy living and preventive measures that have stood the test of time. Many women are skeptical about hormones and react by seeking alternative approaches to manage the symptoms of the menopause. These include relaxation techniques, chiropractic massage, herbal medicines, and megavitamins. In a survey of menopausal women, as many as four out of five indicated that they had used nonprescription remedies to manage both the short-term and long-term consequences of menopause. One explanation for this is that they are reluctant to embark on a longterm course of prescription medication, the rationale for which has not been fully described and explained. This is particularly true for HT. There are, of course, other reasons for the concern about HT.
Can phytoestrogens, antioxidants, and vitamins replace HT?
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

Phytoestrogens, antioxidants, and vitamins are not unfamiliar topics in the discussion of menopause. Among the many alternatives to HT that women lean toward or for which they seek guidance, the most popular are phytoestrogens: soy estrogens in particular. The appeal is understandable since the highly touted benefits claimed for phytoestrogen products sound like all the advantages that scientists are trying to achieve by developing designer estrogens. This multibillion-dollar research effort, supported by federal grants and pharmaceutical company research budgets, is trying to find or develop compounds that retain the benefits of estrogens for the menopausal woman while minimizing or eliminating the risk. New advances are emerging continuously. Now in advanced clinical trials (placebo-controlled, double-blind, prospective studies) are new designer estrogens that protect bone mineral density, improve the blood lipid profile (good cholesterol vs. bad cholesterol), do not stimulate the endometrium, and provide full antagonism of estrogens in breast cancer models. At last two of them are racing to the finish line after hundreds of millions of dollars of research investment and careful FDA scrutiny. In all likelihood, both will soon be available on the prescription drug market.
Should aging men use nonprescription androgens?
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

Barry Bonds wiped out Mark McGwire’s home run record just three years after it was set. Muscle Builder magazine would be thrilled to have either one of them on its cover. Spindle-legged and paunchy Babe Ruth reigned as Sultan of Swat for 34 years before lanky Roger Maris unseated him in 1961. The Maris record held for 37 years. When Bonds was heading toward his record 73 home runs, a baseball league official, concerned about the possible use of steroids by players to build greater muscle mass, is quoted as saying, “Look at these guys. Look at their arms, their upper bodies, their thighs. All of a sudden they’re huge. You can’t do that in the gym.” After Bonds started the next season with four homers in the first two games, his team trainer said, “They all take vitamin supplements. Barry does that, uses protein drinks, over-the-counter stuff.” By the end of the season the players union had agreed to random testing for steroids, and a noted sports orthopedist, James Andrews, was quoted as saying that the bulking up and the increased injuries in baseball are attributable to steroids and supplements. Professional athletes don’t like to talk about steroid use, so we are left to speculate. The quantity of nonprescription steroids purchased by professional and amateur athletes, including high school athletes, is mind boggling. One study put the annual dollar amount at $800 million. That’s four times as much as the total spent on all prescription androgens combined.
Sex and the aging man
Authors: Segal Sheldon J , Mastroianni Luigi
Published: November 2023

On the assumption that many readers may turn to this chapter first before deciding whether to read the book, we’ll start with the encouraging news that androgen supplementation therapy (AST) can increase sexual interest and improve sexual function. It is also possible for men on androgen supplementation to retain adequate sperm production to remain fertile if they wish to. Alternatively, there are AST regimens that reduce sperm counts sufficiently to make fertility nearly impossible. In other words, AST may be able to double as a male contraceptive. Male fertility does not necessarily decline with aging, in distinct contrast to female fertility. The term “partial androgen deficiency in the aging man” is coined specifically to exclude a decline of fertility with aging. The reason for this is that the hormone-producing capacity of the testis is not linked to sperm production, so even though hormone production may be declining, sperm production can continue. The ovary functions very differently from the testis; hormone and egg production are linked through their anatomical association as different parts of the same structure, the ovarian follicle. Moreover, in addition to the decline in fertility as the follicle count decreases with age, there is also a loss of egg fertilizability. Genetically built into the biology of the ovary is an aging factor that affects the eggs directly. This does not happen to sperm. Even if there is a decline in total sperm count with aging, this change is not reflected in a decreased ability of the sperm of aging men to fertilize an egg.
The Mechanical Theory Of Heat
Authors: Mccormmach Russell
Published: November 2023

In his book on natural philosophy in 1782, William Nicholson discussed the nature of heat in the section on chemistry, a science, he said, which was still in its analytic stage, almost “purely experimental,” consisting of “many facts and little theory.’’ Cavendish addressed this serious deficiency in natural philosophy with a theory of heat based jointly on his researches into specific and latent heats and on his researches into mechanics. Having examined these two lines of research separately, we now look at the theory that combined them. To bring together, as Cavendish did, two well-developed, general subjects, each with its own methods, concepts, and language, is one of the most difficult tasks of theoretical physics, and potentially one of the most rewarding.
Does language equal thought?
Authors: Napoli Donna Jo
Published: November 2023

An idea fundamental to cognitive science is that it may be possible to describe our thought processes through some representational system. Whether the appropriate representational system has properties similar to linguistic properties (such as observing similar principles) is an open question that scholars will no doubt be debating for years. Here, however, I’d like to address related questions, ones that I believe we can answer together: Do we think in language? Could we think without a language?

One way to interpret these questions is as follows: Does language construct a mental world that cognitively fences us in? This might well be a familiar question to you since it is frequently debated.
Can computers learn language?
Authors: Napoli Donna Jo
Published: November 2023

As I am writing this book, in 2001, humans can interact verbally with computers in multiple ways. To take a simple example, when I dial the operator at my college and request the campus directory, a recorded voice asks me for the name of the person I’d like to speak to. When I say the name, the recording responds, “Did you say [the name]?” If I respond positively, the phone number of that person is automatically dialed for me. If I respond negatively, the recording apologizes and a human operator comes on the line. Here a voice recognition program is at play. Such programs have been around for quite some time, and they are used in a variety of situations. One of my friends, who has cerebral palsy and is a quadriplegic, has a computer that has been programmed to recognize her voice and make written files of whatever she says. She’s used this program for years to type with her voice. There’s no doubt, then, that voice recognition by computers is possible.
Whose speech is better?
Authors: Napoli Donna Jo
Published: November 2023

Not all speakers of a given language speak the same way. You’ve noticed speech variations on television. Maybe you’ve seen the movie My Fair Lady, in which Henry Higgins believes that the Queen’s English is the superior language of England (and, perhaps, of the world). So the question arises, whose speech is better? And this question is subsumed under the larger question of whether any language is intrinsically superior to another.

Before facing this issue, though, we need to face another matter. Consider these utterances:

Would you mind if I borrowed that cushion for a few moments?

Could I have that pillow for a sec?

Give me that, would you?
Should the United States adopt English as the official language and overhaul the educational system accordingly?
Authors: Napoli Donna Jo
Published: November 2023

The English Only Movement (EOM) has among its goals an adoption of English as the official language of the United States. Because the EOM is a strong and vocal movement in many states, it is important to face this proposal. But to do so, we have to understand exactly what it means for a language to be “official.” Then we can consider its effects on schools. While the following discussion concerns the situation in the United States, many countries today have linguistically pluralistic societies, so analogous issues arise elsewhere.

The two largest groups that support the EOM are the U.S. English Foundation and English First. The former, with a membership of over a million, wants (among other things) improved education in English for immigrants in order to enhance their economic opportunities. English First, with only a little over 150,000 members, wants (among other things) to have English declared the one and only official language of the United States.
Does exposure to and use of offensive language harm children?
Authors: Napoli Donna Jo
Published: November 2023

Many people think exposure to offensive language harms children, whereas censorship of children’s language and literature does not. Because such censorship is on the rise in the United States, it’s timely to consider its effects. And because so many truly thoughtful people are in favor of censorship, I believe it is my responsibility to present the other side of the debate.

Censorship of speech is often an attempt to control language change. When I was a child, for example, many words having to do with sexual reproduction were taboo; teachers would scold children (or do worse) for using them. The list of words I was not allowed to say included names of body parts, such as “penis” and “vagina,” words that are used in classrooms today. (Many of these words have since lost their nasty connotations.) Efforts to censor language, for reasons of religion or political correctness or anything else, aim to control language change by putting certain words out of use. Censorship is definitely a linguistic matter.
Southeast Asians in the Classical World, ca. 200 BCE—800 CE
Authors: Lockard Craig A
Published: November 2023

Historians cannot be certain when the first sailing ships from India and China began making regular visits to the lands of Southeast Asia, but around 2,000 years ago tales of such expeditions were becoming common in India, offering information about strange and exotic lands such as Yavadvipa, an island said to be awash in silver and gold. The Ramayana (“Story of Rama”), an ornate Indian epic poem written some 2,500 or more years ago, but probably revised several times by the first century ce, mentions this island. Another ancient Indian book, the Vayu Parana, describes it as “full of varieties of flowers and fruits. . . . There are . . . towns where live men and women in happiness.[It is full] of mines of precious stones and gold, besides sandalwood. . . . It is a veritable paradise.” Scholars debate whether Yavadvipa refers to Java, Sumatra, or perhaps Borneo. The Indian adventurers reaching the region were chiefly merchants in search of wealth, and commerce fills their stories.
Colonial Impact and Changing Fortunes, 1800—1941
Authors: Lockard Craig A
Published: November 2023

Achant popular among Vietnamese peasants in the 1930s lamented the results of colonialism: “Ill fortune, indeed, for power has been seized by the French invaders, who are bent on barbarous deeds. It’s criminal to set out the food tray and find that one has nothing but roots and greens to eat.” From a global perspective, colonialism served primarily to link Southeast Asia more firmly to a world economy dominated by the West and to introduce Western ideas and technologies. But colonial policies also profoundly affected all aspects of life, and Southeast Asians responded to the challenges of colonial “ill fortune” in creative ways.

Conquest proved a shattering experience throughout the region. Although it ultimately brought an end to many local conflicts, colonialism also transformed once autonomous Southeast Asians into masses of subject peoples who enjoyed few political rights, with their values and systems of authority challenged. The broad outlines of colonial policy were developed in London, Paris, Amsterdam, Madrid, and Washington, and according to a French saying, “The colonies were created by the colonizer for the colonizer.”
Revolutionary Wars and Nation Building, 1950—1975
Authors: Lockard Craig A
Published: November 2023

In 1950 a small group of leading Indonesian writers published a moving declaration promoting “universal humanism” and “human dignity.” Their beliefs were shaped by the nationalist struggle against the Dutch as well as their familiarity with European Enlightenment values such as democracy, free thought, and tolerance. They claimed, “We are the legitimate heirs to the culture of the whole world, a culture which is ours to extend and develop in our own way. We are born of the common people. For us, revolution implies the discarding of old and outmoded values and their replacement by new ones. Our fundamental quest is humanity.” These writers hoped that Indonesia could combine the most humane ideas of East and West to become a beacon to the world, open to all cultures and showing respect for the common people. These bold and noble aspirations, with their recognition of a connection to the wider world, reflected a new sense of possibility as walls of colonialism crumbled or were knocked down. But the idealism was soon dashed by the realities of the years just after the end of World War II. Most nationalists argued that the longing for dignity could only be achieved after securing independence, either through negotiation or revolution, and by building new nations.
The Lost Words of La Malinche
Authors: Restall Matthew
Published: November 2023

On the morning of 8 November 1519, on a causeway crossing Lake Texcoco in the Valley of Mexico, a unique encounter in world history occurred. Moctezuma met Cortés. For centuries this meeting has been taken as symbolic of the great encounter of continents that was now in its third decade. And with good reason. For the very first time, a Native American emperor greeted a representative of the Europeans who had come to conquer and settle in his lands. The meeting was friendly, with both sides keen to display an unswerving commitment to diplomacy. Yet a clash of cultures was also immediately apparent.
Göran Roos: Recipes for Knowledge-Based Value Creation
Authors: Chatzkel Jay L .
Published: November 2023

Göran Roos is continually developing some of the most advanced and yet readily usable approaches to leveraging the interrelated roles of intellectual capital and strategy in contemporary organizations. As both a thought leader and practitioner, he has made seminal contributions to the field of intellectual capital through his writings and his implementation of practical solutions.

He has created frameworks that enable making the intangible of intellectual capital understood as a very real asset to be cultivated, measured, and appropriately exploited for competitive advantage. He helps managers rethink how their strategies and practices determine and utilize the drivers for intellectual capital growth and how to rigorously evaluate and effectively use their intellectual resources throughout their enterprises to make significant differences.
Jac Fitz-Enz: Human Capital Is the Only Active Asset
Authors: Chatzkel Jay L .
Published: November 2023

In this conversation, Jae Fitz-enz examines the leveraging role of human capital in organizations. He views that human capital is the only capital that is active. Without human capital, nothing can happen in organizations. He demonstrates that we can measure the value of human capital and the return on investment of human capital. This is important to develop the set of measures that balances human and financial factors. This balance is the hallmark of effective organizations. Fitz-enz also warns that human capital, intellectual capital, and knowledge management are in danger of failing unless they become integrated into the way organizations do their business.
Thomas O. Davenport: People Are the Owners and Investors of Human Capital
Authors: Chatzkel Jay L .
Published: November 2023

Thomas Davenport is a principal in Towers Perrin’s Organization and Employee Research Practice in San Francisco. He provides counsel on human capital strategy, change management, organizational effectiveness, and business strategy to clients in the service, financial, retail, manufacturing, and public sectors.

He has made a major contribution to the field by recognizing that the evolution of the new economy requires managers to think of people as owners and investors of their human capital, an asset they invest in the organizations where they work. This metaphor extends beyond the conventional thinking that people themselves are assets controlled by organizations.

Davenport focuses much of his attention on helping clients improve the people-focused elements of business strategy implementation. He is the author of the book Human Capital: What It Is and Why People Invest It, published by Jossey-Bass in 1999. Among the topics he covers is the challenge of attracting, retaining, and engaging workers in a fast-paced, turbulent environment.
Steven M. H. Wallman: A Level Playing Field for Intangibles
Authors: Chatzkel Jay L .
Published: November 2023

Steven Wallman is a recognized authority on securities markets and the challenge of revising financial reporting to account for the new drivers of wealth production. He is the founder and chief executive officer of FOLIOfn, an innovative financial services and brokerage firm, a senior fellow of the Brookings Institution, and a former commissioner of the U.S. Securities and Exchange Commission (SEC).

As an SEC commissioner from 1994 to 1997, Wallman organized the first symposium addressing the increasing role of intangible assets in the changing economy and the related need to make financial statements reflect this significant shift in business and financial activities. After leaving the SEC, he co-chaired the Brookings Institution study on intangibles that resulted in the report Unseen Wealth. The report serves as a foundation piece for legitimitizing intangible assets as a vital issue worthy of study and resolution. The report explored the public policy implications of the growing level of intangible assets in the U.S. economy and made recommendations on a number of specific issues.
Sharon oriel: Finding “the Hook”
Authors: Chatzkel Jay L .
Published: November 2023

Sharon Oriel is the director of the Global Intellectual Capital Management Technology Center of the Dow Chemical Company. She has been with the Dow Chemical Company for 29 years since receiving under graduate and graduate degrees in biology. Her career with Dow has included experience in central research, plastics technical service and development, plastics marketing, and for the last 11 years, intellectual capital management. Her focus is on aligning, leveraging, and creating intellectual assets and capital for value growth.

Oriel is one of the cofounders of Dow’s intellectual capital man agers gathering. She serves on the editorial board of the Intellectual Capital Journal. In addition, Oriel served on the research and development task force supporting the Brookings IC project, has been featured in CFO magazine, and Business Finance, and contributed chapters to numerous books. According to Oriel, the future is truly filled with intangible opportunities. Those who navigate these opportunities with wisdom will create a sustainable future. Oriel considers herself a tempered IC radical.
Stephen Denning: The Springboard Story
Authors: Chatzkel Jay L .
Published: November 2023

Stephen Denning was a key player in the strategic shift of the World Bank toward becoming a knowledge-based organization. AB the lead per son for instituting knowledge management at the World Bank, trying the generally accepted approaches to communicating the complex idea of knowledge management with marginal success, he discovered that story telling was the most effective vehicle for about “knowledge management.” This is not storytelling in the entertainment sense, but storytelling in the sense of conveying the kernel of a significant change experience. He has found that storytelling is a vital element for developing a knowledge sharing culture and a knowledge-based organization. Without it, he feels that the World Bank would not have achieved the change in emphasis to knowledge as a central pillar and resource that it has.
Knowing Your Relative Resource Position
Authors: Grimm Curtis M , Lee Hun , Smith Ken G
Published: November 2023

Gillette, which had $8.4 billion in sales and approximately $1.2 billion in net income in 2002, is an example of a company with a strong resource position.1 Gillette’s high performance and strong market position in the razor blade industry can be explained by the firm’s ability to efficiently combine its unique resources to build an attractive and enviable position in the industry. Gillette’s development of the hightechnology razors, such as Mach3Turbo, is the case in point. Gillette’s new triple-blade shaving system, at the time of writing the most technologically advanced shaving system in the world, is based on a unique combination of resources, including 35 different new patents, improved lubrication systems, and customized manufacturing processes. The razor appears to be a simple gadget with three blades mounted on small springs. However, the product is significantly more “high tech” than it may seem. Its production is based on one-of-its-kind manufacturing equipment and innovative patents. The equipment, designed by Gillette, is very complex, and it is virtually impossible for a competitor in the razor industry to understand or recreate it. Although Schick, a major competitor, has won rights to some product designs, e.g., the lubrication strip, that could help it build a competitive product, the company is lacking the key manufacturing knowhow necessary to duplicate Gillette’s high-tech shaver. As a result, Gillette holds a very attractive and secure position in the industry.
A Voiding Rivals With Entrepreneurial Actions: Exploiting Competitive Uncertainty And Blind Spots
Authors: Grimm Curtis M , Lee Hun , Smith Ken G
Published: November 2023

FieldTurf Inc.’s rise as a major player in artificial turf for athletic playing surfaces came from its innovative and aggressive competitive moves. In 1997, Field Turf, led by a skillful, innovative, and experienced founder, “revolutionized” the artificial turf industry with entrepreneurial actions. FieldTurf’s actions included developing a new turf made of long synthetic fibers woven into a base made of sand and recycled rubber pellets-giving it the look, feel, and drainage of real grass and causing fewer injuries-and targeting it to low-profile fields (e.g., indoor soccer and high school fields). Gradually, the new turf started to sell itself, including to high-profile fields (e.g., college and professional football fields). It was a significant improvement over the traditional AstroTurf, sold by the industry leader Southwest Recreational Industries, Inc., which had dominated with about three-fourths of the U.S. market. Shortly, Southwest responded and introduced its own version of the new turf but was slowed considerably by FieldTurf’s legal actions citing patent infringement. Southwest and other rivals, who were blind to and uncertain of the wisdom of FieldTurf’s entrepreneurial moves, were somewhat slow to respond at first, providing a window of opportunity for FieldTurfto become one of the industry leaders.
Prenatal Growth In Pregnancy
Authors: Ekvall Shirley W , Ekvall, Valli K , Fowles Eileen
Published: November 2023

The two major sources of developmental disabilities in prenatal growth are genetic inheritance and the intrauterine environment. The genetic disorders include single-gene disorders (autosomal dominant, autosomal recessive, and X-linked) and chromosomal disorders (such as trisomy 21 and Down syndrome). The conditions affecting the intrauterine environment that give rise to developmental disorders include maternal infection (e.g., herpes simplex type II), maternal diseases, and maternal lifestyles (e.g., exposure to toxic substances in the workplace or home, drug abuse, malnutrition, and smoking).
Seizures And Epilepsy
Authors: Olson Donald , Westermarck, Tuomas , Ekvall Shirley W
Published: November 2023

Seizures occur in 10% of people at some time during their lives. Seizures are sudden, discrete episodes of abnormal behavior caused by abnormal hypersynchronous firing of neurons in the cerebral cortex. They are classified as generalized when both hemispheres are involved or partial ( focal) when limited to an area in one hemisphere. Commonly recognized generalized seizure types are generalized tonic-clonic seizures (formerly called grand mal) and absence seizures (formerly called petit mal). Other types of generalized seizures are tonic, atonic, and myoclonic seizures as well as most infantile spasms. Under the right (or wrong) metabolic conditions, anyone can have a seizure. Relevant conditions include numerous metabolic and nutritional abnormalities. About 0.5%–1% of people have epilepsy. This is the underlying chronic condition that predisposes the individual to have seizures. Epilepsy can be either symptomatic (secondary) when there is a known underlying brain abnormality (such as a stroke or a brain tumor) or idiopathic (primary) when it occurs in an otherwise normal individual without other symptoms or signs of a brain disorder. The most common time for epilepsy to develop is in the first year of life; over half of epilepsy cases develop before 21 years of age. Another peak in epilepsy onset occurs in older age due to the accumulation of brain insults from such conditions as stroke, trauma, and tumors. Potential causes of epilepsy in infants and children include acute brain-injuring insults like perinatal asphyxia, sepsis, and hypoglycemia, as well as chronic causes like brain malformations and genetic disorders.
Myelomeningocele
Authors: Ekvall Shirley W , Cerniglia Frank
Published: November 2023

Myelomeningocele (MM), a type of spina bifida, is a protruding lesion along the midline of the spinal cord. It arises when the neuroectoderm is transformed into a neural tube and becomes detached from the cutaneous ectoderm. It is the most common malformation resulting from a disturbance in neurotation and occurs when a child is born with a protruding lesion that blocks closure of the neural tube (Fig. 11–1). The defect takes form between 26 and 30 days’ gestation; the later its development, the lower the lesion. Removal of the sac and closure of the neural defect usually are performed within 24 hours of birth to avoid infection. The sac contains meninges, dura, cerebral spinal fluid, and other neural elements. The effects depend on the level of the sac or lesion. The higher the sac, the greater the extent of paralysis. Manifestations range from weakness in the lower extremity to complete paralysis and loss of sensation, and thereby bladder incontinence. Upper extremity fine motor problems are also usually present, although to a lesser degree than lower extremity involvement. Approximately 90% of the children with MM have hydrocephalus, which is not fatal if treated promptly before brain tissue is destroyed. A ventriculoatrial or ventriculoperitoneal shunt is used to relieve the pressure3 (Fig. 11–2). The median IQ of children with MM is 88. Even with normal intelligence, many children with MM have a learning disability. Trophic ulcers, spontaneous fractures, and deformities of lower extremities occur because of reduced ambulation. Obesity follows and usually is related to the level of the lesion; higher lesions produce the greatest amount of obesity. Myelomeningocele occurs in about 5 per 10,000 births in the United States and in 6.7 per 10,000 births in Ireland.6 It is found 2.5 times more frequently in whites than in blacks or Asians7 and more frequently in girls than boys in a ratio of 1.25:1.00. Recurrence in families where one child has spina bifida is 2.5 times greater than the incidence in the general population. However, the etiology of MM is unknown. It may involve both environmental and genetic factors. The first-born child is at greatest risk of developing MM.9 With surgical correction of hydrocephalus, removal of the lesion, prevention of renal damage, and provision of aggressive nutrition, orthopedic therapy, and other multidisciplinary therapy, survival may reach 80% to 95% rather than the earlier levels of 50% to 70%.
Anorexia Nervosa, Bulimia Nervosa, And Eating Disorders
Authors: Ekvall Valli K , Ekvall, Shirley W , Farrell Michael
Published: November 2023

Eating disorders affect over 5 million Americans. They involve abnormal behavior related to weight control, accompanied by an intense preoccupation with food, a great fear of gaining weight, and an obsession with becoming thinner. The three major categories of eating disorders are anorexia nervosa (AN), bulimia nervosa (BN) and eating disorders not otherwise specified (EDNOS). (DSM-IV-TR) Eating disorders are not limited by socioeconomic class and are found in a wide demographic range. The term anorexia nervosa was coined in 1874 by Sir William Gull to describe a nervous disease characterized by a loss of appetite and severe wasting. The most notable features of AN are marked weight loss and the maintenance of low body weight.7 The fourth edition of the Diagnostic and Statistical Manual of the American Psychiatric Association (DSM-IV-TR)3 establishes the criteria for AN as given in Table 22–. In early literature, AN was often seen as a form of rebellion against sexuality. Through maintenance of a low body weight, a young girl was able to delay sexual maturation. This view is still held today, although there is a higher rate of married women with AN today. Anorexia nervosa has been classified by some authors into primary and secondary or atypical forms. Patients with primary AN fear becoming obese, perhaps because of a disturbed body image, and make a variety of attempts to lose weight. Secondary or atypical AN often results from psychiatric disorders— conversion reaction, depression (unipolar or bipolar), schizophrenia—or from other medical diseases involving a true loss of appetite. The person with AN may be brought to the health professional when the body weight is 15% below normal.
Children With Cancer
Authors: Kalinyak Karen , Ora Ingrid
Published: November 2023

Though infrequent, childhood cancer is a leading cause of death in children older than 6 weeks of age, second only to accidental deaths. The overall incidence of cancer under the age of 15 years is 13 per 100,000 among white children and 10 per 100,000 among black children. Pediatric cancers differ from those found in adults with regard to type, biology, treatment, and prognosis. The most common pediatric cancers are acute leukemias and brain tumors. Other types of pediatric cancers, include lymphoma (Hodgkin’s and non-Hodgkin’s), neuroblastoma (arises from the sympathetic nervous system), Wilms’ tumor (kidney), rhabdomyosarcoma (cancer arising from soft muscle tissue), retinoblastoma (eye), bone tumors (osteosarcoma and Ewing’s sarcoma), hepatoblastoma (liver), germ cell tumors, and other, less common tumors. The prognosis and outcome for children with cancer have improved dramatically over the past four decades. Currently, more than 75% of all children diagnosed with some form of cancer can be expected to enjoy more than 5 years of disease-free survival and are presumably cured of their disease (Fig. 31–1). In the past 30 years, the death rate from childhood cancer has decreased by 50% (from 50 to approximately 25 per million population), with a 25% decrease in childhood cancer mortality in the past decade alone. The overall 5-year survival rate in children diagnosed with cancer from the most recent national study is 77%.
Fragile X Syndrome
Authors: Nelson David , Siomi Mikiko Chihara
Published: November 2023

Fragile X syndrome was first recognized in 1943 by Martin and Bell as an X-linked form of mental retardation. The discovery in 1969 that affected males exhibit a fragile site at Xq27.3 was followed by the characterization of this site in the 1970s. The locus containing the genetic lesion that produces the fragile site was isolated in 1991, when it was found that an unstable trinucleotide repeat was massively expanded in fragile X chromosomes. The expanded repeat inhibits expression of the FMR1 gene, and loss of function of this gene is the primary cause of fragile X syndrome. While some attempts have been made to improve patients’ outcomes with alterations in nutrition, there is currently no treatment that is widely viewed as effective (Fig. 56–1).
Wilson’s Disease
Authors: Stevens Florence , Ekvall Shirley W
Published: November 2023

In Wilson’s disease (WD) copper is deposited in the liver, brain, kidney, and cornea instead of forming ceruloplasmin. The disease is inherited as an autosomal recessive trait. Copper is an essential nutrient in promoting the formation of ceruloplasmin in the liver, but chronic copper poisoning occurs with hepatolenticular degeneration. The worldwide prevalence of WD is 1:30,000. This figure is higher in specific subgroups with consanguinity. The gene for WD, located on the long arm of chromosome 13 (13q14), is called ATP7B and has a variety of mutations. Affected children are identified between the age of 6 years and the early teens (50% before the age of 15). In adolescent females menstruation may not begin until the disease is treated. Menstrual irregularity, loss of menstruation, and infertility are common. In boys there is delayed puberty. For most children, the initial presentation is hepatic. The disease onset is neurological or psychiatric between the second and fourth decade of life.
Eye
Authors: Traboulsi Elias I
Published: November 2023

Ocular malformations can occur in isolation or accompany other systemic abnormalities in the context of malformation syndromes. Several ocular malformations can coexist, resulting in complex but well-defined clinical ocular phenotypes such as persistent hyperplastic primary vitreous or aniridia. Other malformations such as Peters anomaly and the morning glory disc anomaly are extremely variable in clinical appearance and have been given a number of descriptive names over the years because of a failure to recognize the spectrum in their morphology.
Ear
Authors: Carey John C
Published: November 2023

The auditory system is a highly interesting and complex structure. A single integrated anatomic unit serves both hearing and equilibrium. The ear develops as three distinct components: the external ear, the middle ear, and the inner ear. In these introductory comments, the morphogenesis of the three parts of the ear will be summarized in the context of the developmental genes currently known to be vital in ear development. In addition, some of the recently discovered genes, known to be expressed in the inner ear and causative in many syndromic and nonsyndromic forms of deafness in the human, will be tabulated and concisely discussed.
Lips
Authors: Jones Marilyn
Published: November 2023

When reduced to the simplest construct, the human face forms from five mounds of tissue that surround the stomodeum or future mouth. The largest is the central and rostrally located frontonasal process that will give rise to the forehead, the nose, the central portion of the upper lip, and the premaxilla or primary palate. Lateral to the frontonasal process are the paired maxillary processes that form the cheeks, the secondary palate, and the lateral portion of the upper lip. The paired mandibular processes form the lower jaw and lower lip. The maxillary and mandibular processes are derivatives of the first pharyngeal arch. Over the last 10 years much has been learned about the genes that direct the process of facial formation as well as the interactions among cells that populate the face.
Tongue
Authors: Gorlin Robert J
Published: November 2023

I am surprised at how often one encounters the faulty perception that “the mouth begins at the tonsils.” In the medical curriculum, because little to no time is allocated to disorders of the mouth, we will devote some effort to the elucidation of this subject. The mouth can be easily examined digitally using a 2 2 gauze, a good light, and a mouth mirror. The tongue is the most important soft tissue structure within the oral cavity. Speech, deglutition, some taste (sweet, salt, bitter, sour, metallic, and so forth [most taste is really smell]), and participation in the immune system (through the lingual tonsils) are its main functions.
Ventral Wall of the Trunk
Authors: Curry Cynthia , Boyd, Ellen , Stevenson Roger E
Published: November 2023

As the neural plate elevates, folds, and fuses dorsally in the embryonic week 4, folding also occurs ventrally to form the body tube. It is this ventral folding process by which the body cavities are positioned, thoracic and abdominal viscera are internalized, the embryo becomes enveloped within the amniotic cavity, and the body stalk becomes an elongated vascular lifeline. It appears that closure of the neural tube and closure of the body tube occur by very different mechanisms: neural tube closure by zippering along the dorsal midline; body tube closure by circumferential convergence in purse string fashion that brings folds from all directions toward a midpoint, the umbilicus.
Male Genital System
Authors: Martin Rick A
Published: November 2023

“ What is it?” is a question posed countless times each day after the birth of a baby. Of course the intent of the question is to know the sex of the baby. But is the questioner’s interest directed at the genetic sex, or is there more curiosity about the gonadal sex? Truth be told, it is the phenotypic sex people want to know about, and a quick peek under the diaper generally confirms the genital dimorphism of our species, correctly predicts both the genetic and gonadal sex of the child, and sows the first seeds of the gender identity process. Genital anomalies often impede this process and cause great anxiety. Of equal im-portance is the fact that such defects can herald considerable morbidity or mortality.
Other Systems and Structures Cutaneous Structures
Authors: Prendiville Julie S
Published: November 2023

The skin is a highly complex organ composed of many different structures and cell types. Developmental abnormalities may be isolated to the skin or associated with structural anomalies of the underlying tissues and syndromes involving other organ systems. Congenital anomalies arise when normal skin development is disrupted by genetic mutations or teratogenic agents. To begin to understand these malformations, a basic knowledge of cutaneous structure and morphogenesis is necessary. Identifica- tion of many of the gene mutations associated with inherited cutaneous diseases in recent years has improved our understanding of the molecular basis of skin development.
Endocrine Organs
Authors: Potter Amy , III John A Phillips
Published: November 2023

Endocrine disorders that are associated with malformations are numerous and include defects of the central nervous system, hypothalamus, pituitary, thyroid, skeleton, and a variety of other endocrine glands, tissues, or organs. These disorders are caused by genetic variations that interrupt different points of the hypothalamic–pituitary axis and other synthetic pathways that are required for normal development. Many metabolic pathways affect growth, and changes in any gene in any of the component pathways can lead to different phenotypes due to decreased or, in some cases, excessive expression of the perturbed gene.
Twins
Authors: Phelan Mary C , Hall Judith G
Published: November 2023

The increased frequency of pregnancy complications, prematurity, anomalies, and neonatal morbidity and mortality asso- ciated with multiple births has long been recognized. As noted by Duncan, twinning itself may be viewed as an “abnormity” or ab-normality of development; viewed as such, twinning is among the most common human malformations. With an incidence of about 1 in 80 births, twinning affects approximately 1 in 40 individuals.
Shifting Speakers: Negotiating Reference in Relation to Sexuality and Gender
Authors: Lunsing Wim , Maree Claire
Published: November 2023

In what ways, if any, does a speaker’s personal sense of gendered and sexualized self influence language use? What gender and sexuality norms police the borders of Japanese language use? How do speakers negotiate these borders? How can we attend to the particularities of Japanese language, gender, and sexuality paradigms while at the same time problematizing prejudices within the sociocultural realm? These issues form the entry points of our inquiry into the intertwining of language, gender, and sexuality in spoken Japanese.
Lesbian Bar Talk in Shinjuku, Tokyo
Authors: Abe Hideko
Published: November 2023

Two decades ago, the difference between sex (as biological, natural, physical, real, actual, unrefined) and gender (as social, fictitious, refined, forced) was presumed to be clear and obvious for language and gender researchers. However, the distinction between the two was questioned and challenged most powerfully by Butler, who claims that sex “by definition has been gender all along” (1990:8) and that gender is a discursive practice unfolding in an ongoing interaction and, thus, open to intervention and re signification. An important aspect of Butler’s work is her claim that gender only exists in the service of heterosexism. Butler restructures gender as the performative effect of repeated acts, a cultural fiction, which can as such no longer privilege heterosexuality as the real or original sexuality at the expense of homosexuality (Jagose 1996). Ueno (1999:48) argues that one of queer theorists’ contributions is to demolish the distinction between heterosexuality as norm/origin and homosexuality as deviation/imitation. Jagose defines queer as follows.