bluuebunny/crossref_metadata_2025_split huggingface dataset:
Kent Greenes: Knowledge Management Is about Change
Authors: Chatzkel Jay L . 
Published: November 2023

Abstract
Kent Greenes has made knowledge management work to achieve significant outcomes both as chief knowledge officer and as senior vice president at SAIC, a diverse scientific research and engineering company, and formerly at BP Amoco, where he was head of knowledge management and where he created an internationally recognized knowledge management practice. His role at SAIC is to harness the power of learning across the organization and its subsidiaries and to develop new business opportunities based in the emerging knowledge economy.
This conversation is an opportunity to see how a context for knowledge management and an effective approach can be successfully introduced and implemented in organizations. Knowledge management is a process and an understanding as well as a set of tools and a strategy.
Living on the Island of Dr. Moreau: Grafting Tissues in the Early Twentieth Century
Authors: Lederer Susan E 
Published: November 2023

Abstract
In his 1896 novel, The Island of Doctor Moreau, English novelist H. G. Wells created a memorable and influential portrait of a surgeon, forced to leave London after the press reported the escape of dogs mutilated in his experiments. Far away in the South Seas, Doctor Moreau performs surgery in what the island inhabitants call the “House of Pain.” These surgeries produce “humanized animals”—wolf-hyena-man, ox-hog-man, and puma-woman—which Moreau regards as “triumphs of vivisection.” The surgical manipulation of the flesh and blood, as he informs a hapless, shipwrecked visitor to the island where he conducts his dark science, could accomplish many things:
The Future of Violence Risk Management
Authors: Monahan John 
Published: November 2023

Abstract
Injustice and inefficiency invariably flow from any blending of the criminal-law and mental health powers of the state. Each is sufficient unto itself to achieve a just balance between freedom and authority; each has its own interested constituency; when they are mixed together, only the likelihood of injustice is added. (Morris 1982, p. 31) Despite this prescient warning, a blending of the criminal justice and mental health systems is exactly what is happening in the United States, and what may be on the verge of happening in the United Kingdom as well. On the mental health side, the blending has taken the form of a resurgent emphasis on the state’s police power as the principal justification for the civil commitment of people with serious mental disorder who have committed no crime but who are believed to be “dangerous to others.” The result has been the first significant loosening of the 1960s-era libertarian-oriented standards for involuntary treatment as a hospital inpatient, and the first meaningful laws ever that allow for involuntary treatment in the community. On the criminal justice side, the blending has come as statutes have proliferated, and been upheld, authorizing the indefinite civil commitment in a mental hospital of convicted offenders who are believed to have a “mental abnormality”—which need not rise to the level of a “mental disorder”—and are found likely to recidivate. To date, these statutes have been limited to offenders convicted of sexually violent offenses, but proposals have been introduced in the United States and in the United Kingdom to expand them to the perpetrators of any form of violent act. Both of these moves toward convergence have been facilitated by developments in the professional technology that enables their operation: violence risk assessment. After two decades of largely depressing research on the abilities of mental health professionals at predicting violence, the journals are suddenly replete with guardedly optimistic studies indicating that actuarial instruments may succeed where clinicians have failed.
Nutritional Assessment—All Levels And Ages
Authors: Ekvall Shirley W , Ekvall Valli K , Walberg-Wolfe, Jennifer , Nehring Wendy 
Published: November 2023

Abstract
Nutritional assessment, an essential task of the nutritionist and dietitian, is an important disease prevention measure that has been shown to reduce health-care costs. One of the early position papers of the American Dietetic Association on health care noted that in terms of money and human suffering, every dollar spent on nutrition instruction saves many dollars in later medical care. Kennedy and Kotelchuck reported that participation of prenatal patients in the USDA Special Supplemental Food Program for Women, Infants, and Children (WIC), which includes nutrition education, has a positive effect on pregnancy outcome: a 107 g increase in mean birth weight and a 4% decrease in the incidence of low birth weight. High-risk teenage, African-American, and Hispanic women gained even stronger benefits, particularly when they were followed for two trimesters. Malnourished patients often are hospitalized 2–5 days longer than nonmalnourished patients, resulting in additional costs of over $1000 per day. Also, nutritionists/dietitians on nutrition support or feeding teams helped reduce the incidence of malnutrition. The savings ranged from $1700 over a 4-month period to $8100 per year. However, only one-fifth of the patients received home counseling or another form of posthospitalization follow-up care. Butterworth and Blackburn found that protein-calorie malnutrition affected one-fourth to one-half of medical and surgical patients whose hospitalization lasted 2 weeks or longer,5 and many children today are at high risk due to obesity and poor nutritional habits. These problems have increased in the past 10 years. However for the undernourished lower fat free mass or lean body mass was significantly associated with increased length of hospital stay when using the Subjective Global Assessment questionnaire. This combination is more sensitive than weight loss of 10% or body mass index for older children and adults.
Prader-Willi Syndrome
Authors: Mccune Helen , Driscoll Daniel 
Published: November 2023

Abstract
Prader-Willi syndrome (PWS) is a complex multisystem syndrome that occurs with a frequency of approximately 1/10,000 to 1/20,000 and has significant morbidity, including central hypotonia, mild mental retardation/developmental disabilities, a characteristic behavior disorder, growth deficiency, obesity compounded by a centrally driven excessive appetite (hyperphagia), growth hormone deficiency, and hypogonadism. It is the most commonly known genetic cause of obesity. Prader-Willi syndrome is one of the best examples of a pattern of inheritance called genomic imprinting. In contrast to Mendelian inheritance, the imprinted genes have differential expression, depending on the parental origin. In the 15q11–q13 chromosomal region, there are genes that are expressed only if they are paternally derived and others that are expressed only if maternally derived. Prader-Willi syndrome results from the loss of expression of paternally derived genes by a variety of mechanisms, which include large deletions (70%–75%), maternal uniparental disomy (UPD, 20%–30%), and imprinting defects (2%–5%).2,3 DNA methylation analysis will correctly diagnose more than 99% of cases and is the most sensitive genetic test available. It is also more sensitive than the clinical diagnostic criteria that were originally published in 1993 by Holm et al. Therefore, all patients suspected of having PWS should have definitive genetic testing since there is significant variability in the clinical phenotype (Table 16–1).
Rett Syndrome
Authors: Bibat Genila , Cuffari, Carmen , Naidu Sakkubai 
Published: November 2023

Abstract
Rett syndrome (RS), a neurodevelopmental disorder primarily involving girls, is characterized by a period of apparently normal development followed by the arrest of developmental skills. Distinctive features of RS include deceleration in velocity of head growth between 5 months and four years of age, resulting in microcephaly, stereotyped hand movements, psychomotor retardation with impaired language, and loss of purposeful hand use. Most patients have electroencephalographic abnormalities, but only half have recognizable clinical seizures. Respiratory irregularities in RS include periodic apnea during wakefulness and intermittent hyperventilation. The incidence of RS is approximately 1:10,000. It is pan-ethnic. Life expectancy is variable, with sudden unexplained death during sleep in children under 15 years of age, regardless of seizure control. The necessary criteria for the diagnosis of RS are shown in Table 17–1. However, a wide clinical spectrum has been recognized since the defective gene in RS was identified. With the increase in clinical recognition and advances in the neurobiology and molecular genetics of RS, a scoring system to facilitate comparison between the various clinical profiles of mutation-positive children and a revised set of criteria to clarify the ambiguities in language of the current diagnostic criteria5 were suggested.
Childhood Obesity
Authors: Ekvall Shirley W , Bandini Linda , Ekvall, Valli K , Curtin Carol 
Published: November 2023

Abstract
Obesity is the result of an energy imbalance. An individual is considered to be in energy balance when energy intake equals energy expenditure. When energy intake exceeds energy expenditure, the excess calories are stored as adipose tissue. An energy imbalance may occur when energy intake increases and energy expenditure remains the same; when energy intake remains the same and energy expenditure decreases; or when energy intake increases and energy expenditure decreases. The causes of energy imbalance are multifactorial and may include genetic, metabolic, psychological, and environmental factors. The prevalence of overweight in children and adolescents in the United States has been increasing since the 1980s. Current estimates in children are 10.4% in 2to 5-year-olds, 15.3% in 6to 11-year-olds, and 15.5% in 12to 19-year-olds.1 The number of children who are overweight has more than doubled over the past 20 years. Although the prevalence of obesity did not differ among racial/ethnic groups in the National Health and Nutrition Examination Survey (NHANES) III survey of 1988–1994, in the NHANES 1999–2000 survey the prevalence of overweight was higher in non Hispanic black, and Mexican-American children and adolescents than in white children and adolescents. Populations of children and adults with developmental disabilities such as myelomeningocele, Prader-Willi syndrome, and Down syndrome5–7 have been reported to have a higher prevalence of overweight than those in the general population.
Attention Deficit Hyperactivity Disorder
Authors: Ekvall Valli K , Ekvall, Shirley W , Mayes Susan 
Published: November 2023

Abstract
Hyperactivity is now called attention deficit hyperactivity disorder (ADHD). The diagnostic criteria for ADHD from the fourth edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-TR) are listed in Table 19–1. Attention deficit hyperactivity disorder is one of the most common neurobehavioral problems seen in children today. The symptoms are clustered, which makes the etiology more challenging to define. However, the general consensus is that ADHD is neurologically based, and positron emission tomography scans have shown differences between the brains of individuals with and without ADHD. The prevalence rate of ADHD is 6% in school-age children. This rate translates into approximately one to two children per average school classroom. The disorder occurs much more frequently in males, with a 4:1 to 9:1 ratio of males to females. The symptoms appear during the preschool years but are usually not diagnosed until the child is of school age. In most children with ADHD, symptoms persist into adolescence and adulthood. ADHD is also linked to a greater occurrence of mood and anxiety disorders, learning disorders, and oppositional-defiant disorder. Its occurrence is more common in first-degree biological relatives of those with ADHD. Approximately 20% to 30% of children with ADHD have one parent with ADHD.
Rumination
Authors: Ekvall Valli K , Ekvall Shirley W 
Published: November 2023

Abstract
The American Psychiatric Association specifies the diagnostic criteria for rumination in infancy as follows in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV-R): Some aspects of the DSM-IV-R criteria were questioned after a 1992 review of publications on rumination disorder. Although 84% of ruminators described in the literature have problems with weight, 16% are not underweight or losing weight. The criterion “following a period of normal functioning” is also questionable because most ruminators, particularly those with developmental delays, were clearly not normal before the onset of rumination. Lastly, the rationale for the diagnostic requirement of rumination being present “for at least 1 month” is unclear and may interfere with prompt diagnosis and treatment of this potentially life-threatening disorder. A definition of rumination consistent with the existing literature is the voluntary and pleasurable repeated regurgitation of stomach contents without an organic cause.
Lead Toxicity And Pica
Authors: Huber Agnes , Ekvall Shirley W 
Published: November 2023

Abstract
The amount of lead, one of the heavy metals in our environment, has increased due to industrialization, increasing the lead burden in children and adults. Pica, the ingestion of nonfood items such as paint chips by children during the mouthing stage, can lead to frank lead toxicity. The interrelationship of lead toxicity and pica is a complicated one since it also involves several nutrients. Furthermore, lead toxicity and pica can occur independently of each other. Subclinical lead exposure and frank lead toxicity in children are worldwide problems. In the United States the first national data on blood lead levels in U.S. children were gathered by HANES II from 1976 to 1980. The data showed that 4% of children 6 months to 5 years old had blood lead levels above 39 µ,g/dL. The highrisk children within this group were black inner-city children, 12% of whom had increased blood lead levels, and children of low income families. However, increased blood levels were also found in some children of rural and high-income families. The major source of lead in the environment was thought to be lead-containing fuels and flaking lead paint in old houses, but lead was also obtained from drinking water and foods.
Juvenile Rheumatoid Arthritis
Authors: Simpson Suzanne , Haugen Margaretha 
Published: November 2023

Abstract
Juvenile rheumatoid arthritis (JRA) is a chronic, debilitating childhood disease characterized by inflammation of the synovial membranes and articular structures. This aberrant inflammatory process is manifested clinically as swelling, tenderness, and pain in the affected joints, which can lead to limitations of movement and flexion contractures.1 In addition, afflicted children may have bone atrophy and rarefaction with associated growth impairment. Systemic manifestations may occur, including rash, fever, fatigue, and nutritional issues such as poor appetite and weight loss. There is wide variation in prevalence rates among countries, ranging from 16 to 150 per 100,000 at risk.
Sickle Cell Anemia
Authors: Kalinyak Karen 
Published: November 2023

Abstract
Sickle cell anemia (SCA) is a genetic disorder in which a sickle gene (Hb S) is inherited from each parent. Persons with sickle cell trait (Hb AS) carry the sickle gene but under normal circumstances are completely asymptomatic. The sickle gene is prevalent in people of African, Italian, Sicilian, Egyptian, Turkish, Arabian, and Asiatic Indian background. In the United States, sickle cell disease is found predominantly in African Americans. About 1 of every 12 African Americans are asymptomatic carriers (have sickle cell trait), and approximately 1 of every 400 African American newborns have the disease. The sickle hemoglobin is the result of a single amino acid substitution of valine for glutamic acid in the sixth position of the beta globin chain. In contrast to the usual adult hemoglobin (Hb A), the sickle hemoglobin tends to polymerize when it undergoes deoxygenation. This process affects the shape of the red blood cell, forming elongated, stiff, less deformable sickleshaped cells that are rapidly destroyed and removed from the circulation. The two primary manifestations of SCA are severe hemolytic anemia and widespread vaso-occlusion and infarction of various organs and tissues. The hemolytic anemia is a result of the markedly shortened life span of the sickle cells, which survive in the circulation for only 9–11 days compared to 120 days for normal red blood cells. The clinical manifestations that reflect this ongoing hemolytic process include jaundice, pallor, weakness, and fatigue. The widespread vaso-occlusion is a result of the adherence of the stiff, less deformable sickle cells to the lining of blood vessels, which virtually plugs up the small vessels and thus obstructs blood flow to a particular tissue or organ.
Homocystinuria
Authors: Singh Rani 
Published: November 2023

Abstract
Homocystinuria can result from hereditary or acquired enzymatic deficiencies in either the remethylaton pathway of homocysteine to methionine or the trans-sulfuration of homocysteine to cysteine. Homocysteine occupies a central position in methyl metabolism, specifically methyl cycling involving methionine, homocysteine, and cysteine (Fig. 37–1). One fate of homocysteine is remethylation to synthesize methionine using the methyl donor from either 5-methyl-tetrahydrofolate (THF), with methyl cobalamin as coenzyme, or using the methyl donor from betaine, which is catalyzed by betaine-homocysteine methyl transferase. The other fate, the transulfuration of homocysteine, includes synthesis of S-adenosyl methionine (SAM), catalyzed by methionineS-adenosyltransferase to produce S-adenosylhomocysteine. Sadenosylhomocysteine is further hydrolyzed to homocysteine and adenosine. Most homocysteine is irreversibly converted to cysteine by cystathionine f3-synthase (Cf3S) using pyridoxal phosphate as a coenzyme.
Glutaric Acidemia Type I And 2-Ketoadipic Acidemia
Authors: Frazier Dianne 
Published: November 2023

Abstract
Glutaric acidemia type I (GAI), first described in 1975 by Goodman et al., is one of several rare autosomal recessive organic acidemias caused by a defect in the pathways of amino acid catabolism. It was designated “type I” to distinguish it from glutaric acidemia type II, or multiple acyl-coenzyme A (CoA) dehydrogenase deficiency, which is a defect of the mitochondrial respiratory chain affecting fatty acid oxidation as well as amino acid catabolism. A less well described disorder, 2-ketoadipic acidemia (KAA), results from an enzyme defect one step before the defect causing GAI. It too is believed to be inherited as an autosomal recessive disorder.
Insulin-Dependent And Type II Diabetes
Authors: Christensen Nedra 
Published: November 2023

Abstract
Diabetes is a leading cause of death in the United States and can be diagnosed in early childhood as well as throughout adulthood. In an October 1998 statistical report, the American Diabetes Association estimated that diabetes mellitus affects 15.7 million people in the United States, or almost 6% of the population (8.2% of the total population over age 20 and 18.4% over age 65), with a sex distribution of 7.5 million men and 8.1 million women.3 Of the total, approximately one-third are undiagnosed. Diabetes is characterized by insulin insufficiency, lack of insulin production, and/or resistance to insulin. Lack of diabetes control leads to hyperglycemia and is associated with a variety of serious complications, including retinopathy, nephropathy, neuropathy, and cardiovascular disease. The costs associated with diabetes in the United States were estimated to be $98 billion in 1997. This includes $44.1 billion in direct costs and an additional $54 billion in indirect costs due to disability and mortality. Health-care costs for a person with diabetes are three or more times greater than those for a person without diabetes.
Glycogen Storage Diseases
Authors: Kishnani Priya 
Published: November 2023

Abstract
Glycogen storage diseases (GSDs) are inherited disorders that affect glycogen metabolism. The first clinical description of a patient with a defect in glycogen mobilization was published in 1928 by Snappes and Van Creveld. Today over 12 forms of GSD have been identified, with a wide spectrum of clinical presentations and an overall frequency of approximately 1 in 20,000 to 25,000 live births. The classification of GSDs is summarized in Table 52–1. Several reviews of the general and clinical aspects of GSD and recommendations for treatment are available. This chapter will focus on liver GSD types I and III.
Rubinstein-Taybi Syndrome
Authors: Rubinstein Jack 
Published: November 2023

Abstract
In 1963, Taybi and Rubinstein published a report of a possible syndrome in seven children—two girls and five boys—with broad thumbs and great toes, as well as distinctive facial features, growth retardation, mental retardation, and cryptorchidism in boys. Ten years after that publication, I was informed that in 1957 Michail et al. had published in a French orthopedic journal a single case report of a 7-year-old Greek boy with the same constellation of findings. Common clinical findings in the Rubinstein-Taybi syndrome (RTS) include stature below the 5th percentile; poor early weight gain; head circumference below the 2nd percentile; and mental, motor, language, and social retardation, with intelligence often at a moderately retarded level. Commonly noted facial findings have a recognizable pattern. These findings include prominent forehead, broad nasal bridge, beaked or straight nose with the nasal septum extending below the alae nasi, antimongoloid slant of palpebral fissures, clinical hypertelorism, strabismus, highly arched and/or heavy eyebrows, epicanthi, minimal abnormalities of the external ears, mild micrognathia, grimacing smile, a highly arched palate, and dental irregularity and overcrowding (Fig. 53–1).
Williams Syndrome
Authors: Ekvall Shirley W , Morris Colleen 
Published: November 2023

Abstract
Williams syndrome (WS) is a complex condition involving multiple organ systems, unique personality characteristics, distinctive facial features, elastin arteriopathy, cognitive impairment (usually mild mental retardation), connective tissue abnormalities, and endocrine growth abnormalities. Over 99% of individuals with a clinical diagnosis of WS have a 1.6 Mb deletion of chromosome 7q11.23 that contains the 20 contiguous genes of the critical region for WS that encompasses the elastin (ELN) gene. The prevalence of WS in Norway was reported to be 1 in 7500 births. In 1961 Williams et al. described four young children in New Zealand with peculiar facies, supravalvular aortic stenosis (SVAS), and mental retardation. A year later, Beuren et al.6 reported three German patients with the same findings and questioned whether the association of SVAS, mental retardation, and facial characteristics constituted a syndrome. The following year Black and Bonham Carter7 noted that the facies of children with SVAS described by Williams and coworkers and those with idiopathic hypercalcemia described by Fanconi were quite similar and suggested that they had different manifestations of the same condition. In 1964, Garcia et al. demonstrated that the two conditions were the same when they published a case report describing SVAS and developmental delay in an individual who had a history of hypercalcemia. The syndrome was infantile hypercalcemia, SVAS, WS, or Williams-Beuren sundrome.” Jones and Smith pointed out in 1975,8 however, that not all children with WS have SVAS and hypercalcemia.