bluuebunny/crossref_metadata_2025_split huggingface dataset:
α1 Antitrypsin Deficiency
Authors: schwarzenberg Sarah jane , Sharp harvey L 
Published: November 2023

Abstract
The patient was a 35-year-old white male with 1-antitrypsin deficiency. He received a combined liver-kidney transplant for cirrhosis complicated by portal hypertension, renal insufficiency secondary to membranoproliferative glomerulonephritis, and combined restrictive and obstructive pulmonary disease at age 18 years. Jaundice and pruritus were observed at age 6 weeks and resolved spontaneously after approximately 2 months. He was hospitalized for pneumonia at age 20 months, and an enlarged liver was noted. A percutaneous needle biopsy specimen from the liver was interpreted to show postnecrotic cirrhosis, although reevaluation of the biopsy specimen showed the presence of globules that were periodic acid–Schiff (PAS) positive, diastase resistant (Fig. 4-1). He was then referred to our institution at age 2.5 years for liver transplantation.
Pyruvate Dehydrogenase Complex Deficiency
Authors: Stacpoole Peter W , Gilbert Lesa R 
Published: November 2023

Abstract
Identical twins with the fictitious names Ann and Elizabeth were born 6 weeks prematurely to a healthy 26-year-old mother whose pregnancy was uneventful. The family history was noteworthy for epilepsy in a paternal aunt, congenital deafness in another paternal aunt, and possible mental retardation in a maternal cousin. The twins’ sister was a healthy 3-year-old girl with the same biological father. The birth weight was 3 pounds 9 ounces for Ann and 3 pounds 12 ounces for Elizabeth. Apgar scores (numerical expressions of the condition of a newborn infant; based on assessment of heart rate, respiratory effort, muscle tone, reflex irritability, and color) 1 and 5 minutes after birth were 8 and 9 for both girls (maximum score is 10). Other than prematurity, neither child evidenced physical or laboratory abnormalities, and they were discharged home within 2 weeks (for Ann) and 3 weeks (for Elizabeth), both apparently in good health. The mother subsequently remarried and gave birth to a son, who has remained well through age 6 years.
Mitochondrial Encephalomyopathy, Lactic Acidosis, and Strokelike Episodes (MELAS): A Case of Mitochondrial Disease
Authors: Castora Frank J 
Published: November 2023

Abstract
A 17-year-old Caucasian female was referred to a neurologist following a 3-day episode of severe headache accompanied by repeated vomiting. She complained of weakness in her arms and legs and of a general feeling of fatigue, especially after minor amounts of exercise, which left her “feeling exhausted.” She indicated that she had suffered from migraine headaches “since she was little,” and during her recent episodes she had auditory and visual hallucinations. Her speech was slurred, and she had slight paralysis of the right side (hemiparesis).
Neonatal Hypoglycemia and the Importance of Gluconeogenesis
Authors: Holzman Ian R , milley J Ross 
Published: November 2023

Abstract
Female infant L. weighed 3.98 kg at birth to a 29-year-old mother after a 35-week pregnancy. Mrs. L. was an insulin-requiring diabetic who had had type 1 diabetes since she was 15 years old and had not yet shown signs of vascular disease. Her pregnancy had been complicated by multiple episodes of hyperglycemia (excess sugar in the blood) and glycosuria (presence of abnormal amounts of sugar in the urine). Her prenatal care was only episodic because her husband had been part of a layoff at work, and the family was without health insurance. On the day of delivery, Mrs. L. had a routine nonstress test (a test that records changes in fetal heart rate with fetal movement and is used to assess whether the fetus is suffering from a lack of oxygen or blood flow to the brain) because she noticed that her fetus had decreased movement over the preceding 2 days. The nonstress test showed a flat baseline heart rate without any variation, a finding suggesting that the fetus was at risk for asphyxia (lack of oxygen leading to an accumulation of lactic acid and carbon dioxide in the blood).
Glucose 6-Phosphate Dehydrogenase Deficiency and Oxidative Hemolysis
Authors: Burton Catherine , Kaczmarski Richard 
Published: November 2023

Abstract
A 50-year-old female presented passing black urine after having been unwell for 3 days with fever and headache. There was no previous history of blood disorders, anemia, or similar symptoms. Past medical history included a diagnosis of multiple sclerosis. Treatment included steroids and high-dose vitamins and minerals. Her nephew was reported to suffer from favism (an acute hemolytic condition occurring after ingestion of a certain species of bean [Vicia faba] in individuals with glucose 6-phosphate dehydrogenase [G6PD] deficiency). There was no recent history of travel or malaria exposure.
Neonatal Hyperbilirubinemia
Authors: Fahl Jeffrey C , Vanderjagt David L 
Published: November 2023

Abstract
A newborn male was the product of a 37-week gestation to a primagravida female whose blood type was O and Rh positive. During the pregnancy, the tests for syphilis and hepatitis B were negative. The pregnancy was uncomplicated, and the mother received excellent care. She denies alcohol consumption and does not smoke. The labor was prolonged, but the delivery was spontaneous and vaginal. The child was vigorous, active, and breathing spontaneously. Prior to delivery, the mother decided to breastfeed and the child was allowed to suckle immediately following birth. The initial physical exam revealed a birth weight of 2775 g, a birth length of 48 cm, and a head circumference of 33 cm, which are within normal limits for a near-term male child.
Abetalipoproteinemia
Authors: Rava Paul , Hussain m. Mahmood 
Published: November 2023

Abstract
A 28-year-old man who immigrated to the United States a few years ago saw his primary care physician for mild recurrent abdominal discomfort and diarrhea. Past medical history was benign except for what the patient referred to as “occasional abdominal distress.” Further discussion revealed this to include diarrhea, flatus, and abdominal pains. He described the stool during these episodes to be greasy, pale, and foul smelling. Family history was unremarkable. Both of his parents, as well as three siblings, had no history of abdominal symptoms. The patient had been diagnosed with celiac disease and treated with a gluten-free diet at a previous visit. Even though he was compliant with dietary instructions, symptoms continued for the next year. The patient noted that he had experienced these symptoms periodically throughout his life, but that they had worsened since his arrival in the United States. He had not traveled since his immigration and had no pets or recent animal exposures, and there was no association between his abdominal discomfort and specific foods. He denied past or present use of drugs or alcohol and took no medications. Concerned that celiac disease may have been a misdiagnosis, a physical examination, blood tests, and a diagnostic procedure were performed.
Vitamin B12 Deficiency
Authors: Vanderjagt Dorothy J , Mccarthy Denis M 
Published: November 2023

Abstract
The patient, an 84-year-old male resident of a veteran’s home, was admitted to the hospital following a fall in which he had sustained a fracture of his left radius and several lacerations. Physical examination on admission revealed a well-nourished elderly male in no great distress (body mass index (BMI), 22.5 kg/m2). He was moderately confused but with prompting was oriented to time, place, and person. There was no obvious pallor, cyanosis, or jaundice, and his skin and mucous membranes were unremarkable apart from the injuries he sustained in the fall. His right lower extremity was swollen and tender, and he walked cautiously and with a pronounced limp. His blood pressure was 145/85 mm Hg (normal is 120/80 mm Hg). His pulse rate was 80 beats per minute (normal is 75 beats per minute), and his respiratory rate was 16 per minute (normal is 12–14 per minute). His temperature was 98.2°F (normal is 98.4°F). Except for a soft systolic ejection murmur in the neck and in the left parasternal area, his cardiovascular, respiratory, abdominal, and genitourinary systems were all normal. The neurological examination was normal except for impaired cognitive function, a positive Romberg sign, and equivocal plantar responses. (A patient is considered to have a positive Romberg sign when, in a standing position, the patient is more unsteady with eyes closed than with eyes open. This indicates a loss of proprioceptive control.) There were no signs of head injury.
Vitamin A Deficiency in Children
Authors: Wongsiriroj Nuttaporn , Wasantwisut Emorn , Blaner william S 
Published: November 2023

Abstract
A boy aged 3 years and 8 months was admitted to a local hospital in Thailand with the following symptoms: high fever, difficulty breathing, and diarrhea with approximately 10 episodes per day lasting 1 week. The child showed eye symptoms (see Fig. 29-1), including Bitot’s spot and corneal xerosis, that were consistent with the early stages of xerophthalmia, the clinical syndrome associated with vitamin A deficiency. (The clinically defined stages of xerophthalmia are provided in Table 29-1.) Night blindness was also identified through interview of the parents using the local term for night blindness. (For cultures in which vitamin A deficiency has been common, there is often a specific term in the local language for night blindness.) Concomitant with vitamin A deficiency, the child was diagnosed as experiencing protein-energy malnutrition type 2 (moderate), pneumonia, and chronic diarrhea. The boy spent 14 days in the hospital and was treated with vitamin A capsules (200,000 IU/day) on days 1, 2, 3, and 14 as well as with standard antibiotics for the infections. Following treatment with vitamin A, the child’s eye symptoms disappeared, and his vision returned to normal. He continued to receive vitamin A capsules providing 200,000 IU every 3–6 months throughout his childhood.
Calcium-Deficiency Rickets
Authors: Vanderjagt Dorothy J , Glew Robert H 
Published: November 2023

Abstract
The patient was a 3-year-old female who lived with her family in a rural village in northern Nigeria that was situated 40 miles distant from the nearest urban center. She was the fifth of eight children. Her parents were subsistence farmers who grew millet and maize that, together with a small vegetable garden, provided most of the family’s diet. Because of their high cost, dairy products were rarely consumed by the parents or children. The patient was brought by her mother to the local community health clinic because the child had a bacterial infection on her knee. The infection was successfully treated with a 10-day course of antibiotics. During that clinic visit, the physician noted that the patient’s legs were severely bowed (genu varum) (Fig. 30-1) and advised the mother that it would be beneficial for the child to receive a more thorough examination at the teaching hospital in the nearby city. She also noticed that the patient’s 6-year-old brother had a pronounced “K-leg” (rotation of knees toward one another, genu valgum). However, the youngest of the eight children, a 1.5-year-old girl who accompanied the mother and who was still being breast-fed, exhibited no evidence of outstanding skeletal problems.
E. P. Thompson and Methodism
Authors: Hempton David , Walsh John 
Published: November 2023

Abstract
E. P. Thompson’s general picture of religion and the working classes in early industrial England arose from his controversial portrait of English Methodists. The dramatic brilliance of his influential book, The Making of the English Working Class, in which that portrait appears, has generated enormous interest on both sides of the Atlantic since its publication in 1963. There is scarcely a serious wott: of historical scholarship on the Methodist tradition in tl1e North Atlantic world that does not cite Thompson, either as exemplar or pariah1. Although Thompson was hardly an uncritical admirer of Methodism in Wesley’s day, he turned most of his firepower on the generation that emerged after Wesley’s deatl1 in 1791. His picture of second-generation Metl1odists becoming, wittingly or unwittingly, the facilitators of dehumanizing industrialization and political repression has become such an important reference point for all those interested in the modern social history of religion tl1at an examination of Thompson’s own history with Methodism is long overdue. Thompson’s striking account of evangelical Protestantism, money, and industrial capitalism arose not from disinterested observation but, as this chapter will show, from a personal history steeped in tl1e very tradition he came to challenge.
Competitive Militance: Civilian Military Organizations and the Destabilization of American National Security
Authors: Kaufman Jason 
Published: November 2023

Abstract
It has been customary, since at least the late nineteenth century, to view military power as an extension of the legitimate authority of the state (except, of course, in cases of unsanctioned violence or insurrection).1 The American experience, however, gives lie to the assumption that there is a clean analytical divide between state and civil society when it comes to military matters. Lacking either a sizable standing army or an effective state militia, nineteenth-century American national defense policy relied on the military preparedness of ordinary citizens in time of need. Until 1903, in fact, when Congress officially reorganized the National Guard, responsibility for defense was loosely divided among federal, state, and independent forces. Among the independents were voluntary militia companies, shooting clubs, and paramilitary organizations, many explicitly organized around ethnoreligious lines. German-American turner vereine and schutzenbunde, the Irish-American Hibernian Guards, and Czech sokol societies were all nineteenth-century incarnations of this tradition of ethnic military training and self-protection.2
Ethnogenesis: Social Capital, Racial Protectionism, and Sectarian Social Organization
Authors: Kaufman Jason 
Published: November 2023

Abstract
Thus far, I have argued that competitive voluntarism contributed to sectarianism and self-segregation in the United States by providing organizational boundaries dedicated to the perpetuation thereof. Admittedly, however, American associationalism may not have flourished without the impetus of racial hatred, religious disrespect, gender segregation, and ethnic suspicion fueling it from below. In the face of such outward hostility and discrimination, many identity groups turned inward, relying on fraternal organizations and mutual aid societies for help and support in an otherwise hostile world. As one history of the Knights of Columbus organization declares, “Fraternalism was the chosen medium for fashioning a unique expression of Catholic Americanness. … The ceremonials of the Order instructed the initiates in these lessons and satisfied their need for forging strong fraternal ties in an otherwise antagonistic social and cultural climate.”1
The Psychiatric Evaluation
Authors: Cohen Bruce J 
Published: November 2023

Abstract
In taking the psychiatric history and performing the mental status examination, the goal of eliciting the key data necessary to generate a differential diagnosis must be balanced against the goal of establishing some rapport and an initial alliance with the patient, who may be quite distressed at the time. Sometimes the clinician has to take a step backward from one goal in order to step forward toward the other, especially in the case of patients who are cognitively impaired, agitated, or suspicious. A third goal is to achieve the first two goals in a timely manner. The less time that is available for the interview, the more structured and directive the interview must be. An evaluation in the emergency room setting may take 30–60 minutes, while a more comprehensive outpatient evaluation may last for over 90 minutes. Here is one version of the history and mental status examination. Where possible, I have indicated the minimum requirements for those situations where a briefer assessment is appropriate. The major sections of the psychiatric history are listed in Table 2–1, while the major elements of the psychiatric mental status examination are listed in Table 2–2. Following discussion of the history and mental status examination, we will discuss the physical examination and the use ancillary services such as psychological testing and clinical laboratory assessment.
Dementia
Authors: Cohen Bruce J 
Published: November 2023

Abstract
A recent study estimated that the annual cost of all forms of dementia in the United States was somewhere between $20 billion and $38 billion and that the annual cost of caring for Alzheimer’s disease patients was as high as $47,000 per patient per year. The cost is expected to increase as the number of patients with dementia increases. In 1985 there were 2.4 million Americans with moderate to severe dementia, and this number is expected to increase to between 6.1 and 9.8 million by 2040; the care of these persons will cost up to $149 billion annually. This projected increase is due to the aging of the U.S. population since the prevalence of dementia increases with advancing age. Five percent of Americans over age 65 have a severe dementia. The prevalence doubles every 5 years through about age 80, reaching about 50% by age 85. However, dementia is not an inevitable part of the aging process, and most elderly patients are not demented. In this chapter, we will discuss dementia primarily from the disease perspective, exploring its phenomenology, the various pathologies associated with dementia, and the etiologies associated with these pathologies. Along the way, we’ll distinguish dementia from two less severe conditions, mild cognitive impairment and amnestic syndrome.
Schizophrenia and Other Psychotic Disorders
Authors: Cohen Bruce J 
Published: November 2023

Abstract
Schizophrenia is a prevalent condition and a significant source of morbidity and mortality. It affects 1% of the population worldwide. In the United States, it accounts for 25% of hospital days, 20% of Social Security benefit days, 10% of the disabled population, and 2% of the gross national product. Thirty percent of patients with schizophrenia attempt to kill themselves at some point; 10% of patients with schizophrenia die by suicide. Schizophrenia can be difficult to treat. Twenty percent to 30% of patients respond poorly or not at all to currently available pharmacologic therapies. Relapse rates are very high: 20% of patients who take their medications appropriately experience relapses despite this, while 70% of patients who stop taking their medication relapse within 1 year. Noncompliance rates are also very high: 40% of patients are not taking their medications as prescribed by 6 months following an acute episode of illness. For several reasons, all clinicians need to know about schizophrenia. First, schizophrenia is extremely common and therefore clinicians are certain to encounter it. Second, patients with schizophrenia have higher rates of medical morbidity and mortality than do people in the general population, and these associated medical illnesses often are unrecognized and untreated by physicians. Third, various medical conditions and medications can cause symptoms that resemble those occurring in schizophrenia, and therefore all clinicians needs to be able to recognize which symptoms are most typical of true schizophrenia. Fourth, the antipsychotic medications used to treat schizophrenia are associated with a variety of general medical complications that may require treatment.
Sexual and Gender Identity Disorders
Authors: Cohen Bruce J 
Published: November 2023

Abstract
Sexual behavior is ubiquitous and diverse. Beyond the sex act itself, sexuality permeates our culture and our daily lives. Therefore, as with all of the behavioral disorders, distinguishing “normal” from “abnormal” sexuality can be difficult. For example, there are no bright lines separating “low” sexual desire from “normal” sexual desire, or “premature” ejaculation from “normal” ejaculation. Whether a person even is bothered by a particular aspect of his or her sexuality is significantly influenced by personality, previous developmental experience, extent of education about sex, satisfaction with one’s relationships in general, and surrounding cultural attitudes about sex. Consider premature ejaculation. DSM-IV defines this as persistent or recurrent ejaculation with minimal sexual stimulation before, on, or shortly after penetration, and before the person wishes it. The definition is deliberately subjective, taking into account the quality of the individual’s sexual relationship. Paradoxically, this subjective definition may be more reliable and valid than a definition based on the exact interval between penetration and ejaculation as measured by a stopwatch. Thus, while a man who ejaculates within a minute of vaginal entry might be considered by some people to be “premature,” if he and his wife engage in a half-hour of very pleasurable foreplay, followed by mutual orgasms after a minute of pleasurable intercourse, he could not be considered to have a sexual “dys function.”
Suicide
Authors: Cohen Bruce J 
Published: November 2023

Abstract
Every physician at some point must deal with patients who either previously have attempted suicide or who will commit suicide while under the physician’s care. Suicide is the ninth leading cause of death in the United States, accounting for 30,000 deaths each year. It is especially important for primary care physicians to have some understanding of how to assess for suicidality since most individuals who attempt or complete suicide seek medical treatment shortly before doing so, most often from their primary care physician. People encountered in the primary care setting are at higher risk for committing suicide than are other members of the general population. This likely reflects the higher rate of suicide among people with medical illnesses and also the fact that persons with mental illnesses that predispose to suicide (such as major depressive disorder or panic disorder) also are more likely to require general medical care. Retrospective studies involving individuals who have successfully committed suicide reveal them to have visited their primary care practitioners more often during the 4-month period before the suicide than during the previous 4 months. About 80% of those who commit suicide visit their physician within 6 months of the suicide, 50% within 1 month of the suicide, and 40% within 1 week of the suicide. However, despite this potential opportunity for intervention, doctors frequently do not detect suicidal ideation in their patients.