bluuebunny/crossref_metadata_2025 huggingface dataset:
Cardiac Dysrhythmias in Children
Authors: Gillette Paul C.
Published: December 2021

As our understanding of heart rate and rhythm disorders is changing, so too is the terminology. Hence, the words in the title of this article are "cardiac dysrhythmias" rather than "cardiac arrhythmias." The word "arrhythmia" means totally without rhythm. The only rhythm abnormality that fits this definition is fibrillation, either atrial or ventricular. The word "dysrhythmia," meaning an abnormal rhythm, conveys our meaning more precisely.

FREQUENCY

The number of pediatric patients with cardiac dysrhythmias is increasing, mainly because a common sequela of cardiac surgery is damage to the specific conduction system and working myocardium. Dysrhythmias also are being recognized more frequently in children who have no structural heart disease. This discovery is probably due to increased awareness on the part of pediatricians that certain cardiac dysrhythmias in otherwise normal children are important. We must not let this new awareness lead to overenthusiasm that might label a child with a benign dysrhythmia a "cardiac patient." A basic tenet in evaluating the pediatric patient with a dysrhythmia is that very few dysrhythmias in children with otherwise normal hearts are life-threatening, although some are persistent and even disabling. On the other hand, any rhythm abnormality in a child with an abnormal heart should be viewed with concern.
A Hard Look at Stimulating and Predicting Development: The Cases of Bonding and Screening
Authors: McCall Robert B.
Published: December 2021

An axiom of medicine is to diagnose and treat a disease in its formative stages before it becomes so advanced that treatment is difficult or impossible. The same theme runs through aspects of neonatology and developmental pediatrics—organ systems and processes are laid down early in development and supportive or deleterious factors operating during these early stages can permanently influence or alter the course of development.

Belief in "formative Stages was applied to behavior by Sigmund Freud who emphasized the crucial contribution of early experiences to adult personality. A half century later, the same general principle was used to justify Head Start, an educational program that was supposed to equalize the social classes by providing an intellectual boost to disadvantaged children during their formative years.

The principle of "formative years" pervaded theory and practice in developmental psychology for decades, but there were always dissonant findings. For example, five decades of research shows quite clearly that test scores obtained within the first year or two of life do not predict later intelligence for normal children.1 Weight and especially skinfold thickness assessed during infancy do not predict later weight or obesity, and early social disadvantage and stress do not necessarily lead to later psychosocial dysfunction. Indeed, today the emphasis in some quarters of developmental psychology is on change, modifiability, and unpredictability in development rather than on consistency.2
Migraine in Children and Adolescents
Authors: Shinnar Shlomo , D'Souza Bernard
Published: December 2021

Migraine is a genetic disease with wide clinical expression and is a fairly frequent cause of headache in children and adults. This poorly understood disorder is characterized by paroxysmal attacks of vasoconstriction and vasodilation. The vasoconstrictive phase is responsible for the aura and the neurologic deficits when present, whereas the vasodilatory phase is associated with the headache.

The headaches are classically unilateral and throbbing and are associated with nausea and vomiting. A prodromal phase is found in classic migraine. Attacks may occur as frequently as twice a week or as rarely as once a year. They are often precipitated by stress, fatigue, exertion, illness, or other trigger factors, with stress being the most common. Attacks may last from 30 minutes to a day and are often terminated by vomiting and then sleep. Attacks in children tend to be briefer than in adults and have more pronounced autonomic features. Establishing a proper diagnosis is important as specific, effective therapeutic modalities are now available for migraine. This review will summarize the different migraine syndromes and present an approach to the management of the child with migraine.

INCIDENCE AND EPIDEMIOLOGY

Migraine was once thought to be relatively rare in children, but it occurs in all age groups and is a common form of childhood headache.
The Multiple Forms of Neurofibromatosis
Authors: Riccardi Vincent M.
Published: December 2021

Von Recklinghausen neurofibromatosis (NF)1 has multiple forms in several senses. First, there is the matter of heterogeneity: There is more than one disease designated by the term NF, for example "classical" NF as originally described by Von Recklinghausen2 and "acoustic" NF characterized by bilateral acoustic neunomas.3,4 Second, there is the matter of marked variability in the overall severity and progression of classical NF, the disorder to be considered in this review. NF can cause serious problems, and even death in the newborn period,5 or be associated with only a relatively mild or modest burden into the seventh decade and beyond. In almost all patients NF becomes obvious in the first year of life and some form of specific problems or compromise develops before age 20 years.

NF is a heritable disorder, with a frequency of 1 in 3,000.6 This means it affects at least 80,000 Americans, making it much more common than is ordinarily supposed. Indeed, it is more common than Huntington chorea, Duchenne muscular dystrophy, and Tay-Sachs disease combined.4 However, in spite of its commonness,6-10 a realistic appreciation of NF's importance for pediatrics, and for medical care and research in general, is only a recent development. This long overdue change in emphasis reflects a recognition that NF's pathogenesis has bearing on a number of issues in basic biology, including neural crest embryology and genetic influences on the origin of cancer,1 the establishment of several NF clinical research programs, and the activities of several NF patient self-help groups.
Diagnosis and Evaluation of Acute Paraplegia
Authors: Freeman John M.
Published: December 2021

CASE REPORT

A 14-year-old high school student is admitted to the pediatric neurology service because of the sudden onset of inability to use her legs. When she had gotten up in the morning she was unable to stand.

INTRODUCTION

Acute neurologic deficit in an adolescent girl (or in anyone else) always represents an emergency requiring immediate evaluation by a physician competent to analyze the deficit, localize its source, develop a differential diagnosis of possible etiologies, organize appropriate tests, and, when indicated, initiate emergency treatment.

Acute paraplegia (weakness or paralysis in the legs) is a particular emergency because acute compressive lesions of the cord are reversible. Hours of compression may result in irreversible damage. For this reason neurologic and neurosurgical consultation should be obtained promptly.

EVALUATION

The evaluation of an individual with an acute paraplegia is best performed with a differential diagnosis in mind. The major cause of paraplegia is spinal cord disease, and its differential diagnosis is shown in Table 1. Spinal cord disease with paraparesis (weakness) may be simulated by lesions in the parasagittal area of the brain, by muscle weakness in the legs (muscular dystrophy or polymyositis), and by conversion reaction.

History

In evaluating the patient with an "acute" paraplegia one must establish how acute is acute. An immediate total loss of function is almost always vascular.
The Physician's Role in Accident Prevention
Authors: Lovejoy Frederick H. , Chafee-Bahamon Claire
Published: December 2021

Pediatricians, through concern for and understanding of the problem of accidents, can do much to assure an active, forceful, and rational approach to childhood injury. Pediatricians can maximize the effectiveness of their efforts by identifying the interaction of principle causal factors and supporting a combination of preventive measures. However, pediatricians cannot do it alone. Significant progress in the nest decade will in large measure be determined by the ability of pediatricians to work with the nonmedical community in implementing and carrying forth meaningful efforts in accident prevention.
Sexual Abuse and Rape
Authors: Sarles Richard M.
Published: December 2021

Sexual abuse and rape of children and adolescents are alarmingly common. The primary physician is often the first health care professional with whom the girl and the family come in contact. As such, the clinician must be aware of the common and subtle presentations of sexually abused children. It is imperative that the primary care physician follow a standardized protocol for the treatment, collection of evidence, and follow-up care of these victims and their families.
Anorexia Nervosa
Authors: Steiner Hans
Published: December 2021

Anorexia nervosa is a complex, chronic, remittent disorder of mostly adolescent women. It presents with multiple psychiatric and medical symptoms which are difficult to diagnose and treat, especially in the long run. Its differential diagnosis is complex and treatment requires dedicated effort and several specialists working cooperatively. Its etiology is still unclear, although there are several promising leads. Treatment is largely empirical and nonspecific at this point. Early identification and aggressive treatment are the only hope for preventing chronicity. Its relatively high mortality attests to the fact that it is not a benign disorder.
The Teenager with Failing Grades
Authors: Sahler Olle Jane Z.
Published: December 2021

School failure during adolescence is rarely caused by occult physical impairment. Instead, cognitive disability or psychological disturbance are commonly seen. Through the unique clinical skills of history taking and physical examination, the physician can and must consider a broad range of possible causes of the teenager's failure.

The physician's recognition that such behavioral syndromes as school phobia, truancy, substance abuse, underachievement or boredom are possible sequelae of a misfit between the student and the classroom can be the most important element in stimulating the school to examine more closely an individual student's needs or abilities.

Actually, the physician rarely acts wholly independently in evaluating and remedying a student's school problems, even if the basic underlying disorder is physiologic disturbance. Rather, a cooperative approach that includes the student, the family, the school, and the physician is essential to providing special services and helping the student adjust to and follow remediation recommendations.
The `Too Tall' Child
Authors: Reiter Edward O.
Published: December 2021

The subject of the "too tall child" has been discussed. Excessively tall stature is overwhelmingly frequently a physiologic variant, but it must be considered in the context of a young preteenager's life-style, goals, and apprehensions. Decisions as to whether intervention will be appropriate often cannot be made until considerable insight has been gained and perceptive inquiry undertaken. In view of the complexity of the problem, expert endocrinologic consultation is often sought in order to assure the most rational and appropriate treatment decisions.
Other Causes of Red Urine
Authors: SILBER T.
Published: December 2021

An increasing number of pediatricians are following their young patients throughout adolescence. For that reason, I would like to add one more cause of hematuria to the list developed by Kaplan: gonococcal infection. Gonorrhea is the second most common cause of gross hematuria in adults. Recently, our group had the opportunity to describe this same condition in male adolescents. It is important to suspect that gonorrhea may be the cause of macroscopic hematuria in otherwise healthy, sexually active adolescent males who on history or physical examination demonstrate purulent urethral discharge, dysuria, or both. The toxic action of Neisseria gonorrhoeae on the urethral epithelium is sufficient cause to explain the hematuria. A follow-up visit, one week after treatment with 4.8 million units of procaine penicillin and 1 gm of probenecid (or 3 gm of amoxicillin and 1 gm of probenecid) should show resolution of symptoms and clearing of the macrohematuria. Further work-up is needed only if symptoms have not cleared at the time of the test-for-cure visit.
Drug Reactions and Interactions in Pediatric Practice
Authors: Braden N. J. , Walson Philip D.
Published: December 2021

An adverse drug reaction can be a life-threatening, irreversible event that could have been prevented. The physician is responsible for protecting the patient and the public by avoiding adverse drug reactions whenever possible, and by suspecting, diagnosing, and treating them promptly. Documentation of adverse drug reactions protects the individual from future exposure, and reported experience may protect others. Reactions to recently introduced products are especially important. Rational therapeutic practices depend on knowledge (including continual education about drug effects), logic, clinical acumen, and vigilance. Any therapeutic maneuver, no matter how apparently benign, requires a careful risk-benefit assessment because of potentially devastating consequences.
Congenital Hemolytic Anemia
Authors: Addiego Joseph E. , Hurst Deborah , Lubin Bertram H.
Published: December 2021

Anemia due to premature destruction of RBCs is classified as hemolytic, and may be secondary to acquired or inherited (congenital) abnormalities in RBC membrane, hemoglobin, or metabolism. This article reviews the topic of congenital hemolytic anemias and points out a practical approach to diagnosis and therapy.

PATHOPHYSIOLOGY

The primary function of RBCs is to transport oxygen from the lungs to the tissues. The normal RBC circulates within the vascular system for about 120 days. Survival to its full life span depends upon effective metabolism of glucose to generate high-energy phosphates such as adenosine triphosphate (ATP) and to maintain adequate levels of reduced glutathione. ATP is essential for cellular deformability and for function of many RBC enzymes, whereas reduced glutathione protects the cell against damage from oxidants. The water and cation content of the cell is maintained by barriers to permeation, and by transport mechanisms located within the cell membrane. The integrity of the membrane is determined by a group of membrane proteins (skeletal proteins) and by the lipid bilayer surrounding the cell. Genetic or acquired defects of any of these components of the RBC may lead to cellular damage and result in hemolytic anemia.

CLINICAL FEATURES

Hemolytic anemia should be suspected in any child with anemia, jaundice, and splenomegaly.
Adverse Effects of Cancer Therapy in Children
Authors: Mulne Arlynn F. , Koepke John C.
Published: December 2021

There are many opportunities for the primary care pediatrician to participate actively in the care of children with cancer. Adverse effects of therapy must be considered in the differential diagnosis of many problems encountered by these patients, and appropriate management instituted. Adverse effects may appear during therapy or may become apparent only many years later. Referral to a hematologist/oncologist should be considered when help is needed in the diagnosis and management of possible therapy-related problems.
Legg-Calvé-Perthes Disease
Authors: Bunnell William P.
Published: December 2021

Perthes disease is a condition of unknown etiology in which the vascular supply of the developing femoral head is temporarily interrupted, causing necrosis and collapse of the bony femoral head, followed by spontaneous revascularization and healing of the necrotic bone. It was independently described by four different authors (Legg, Calvé, Perthes, and Waldenstrom) in 1909 and 1910. They identified the condition as an affliction of the hip distinct from trauma and infection. The term "coxa plana" was applied to the characteristic flattening of the femoral head seen in Perthes disease. It was not until 10 years later that the actual pathology of avascular necrotic bone was described.

EPIDEMIOLOGY

The condition has its onset in children ranging in age from 2 to 12 years, with the majority of patients presenting between the ages of 4 and 8 years. Boys are affected four times more frequently than girls, and the condition is bilateral in approximately 15% of affected children.

Certain constitutional factors are frequently found in children with Perthes disease. Skeletal age is delayed in nearly 90% of children affected with it. Affected boys have been found to be an average of 1 in shorter and affected girls 3 in shorter in height than their unaffected peers. Birth weight of affected children is frequently lower than average.
Dermatoses of the Foot
Authors: Weston William L.
Published: December 2021

Diagnosis of skin conditions restricted to certain body regions may be difficult. Those of the foot may present a considerable problem to the practicing pediatrician, as indicated by a study that demonstrated that although athlete's foot, or tinea pedis, was frequently diagnosed by pediatric residents, it could not be confirmed by direct microscopic examination of skin scales or by fungal culture in any of the patients who had this disease diagnosed. Pediatricians may suspect that prepubertal children have athlete's foot, but its occurrence is actually uncommon; the prevalence is 35 times greater among adolescents. Thus, skin conditions other than athlete's foot are far more likely to be the cause of a dermatosis of the foot in the prepubertal child. The precise incidence of each of the specific ones affecting the foot is not known, but the most common conditions may be inferred from the overall vital and health statistics for US children in general, and from the experience derived from ambulatory pediatric settings. The purpose of this review is to provide an update for the practicing pediatrician on the clinical features, differential diagnosis, and current treatment of the seven most common skin conditions affecting the feet of US children. A problem-oriented algorithm is included to provide a simplified approach to the dermatoses of the foot in childhood.
Clinical and Laboratory Evaluation of the Child with Suspected Immunodeficiency
Authors: Stiehm E. Richard
Published: December 2021

Historical, clinical, and laboratory data should enable the pediatrician to suspect and then establish a diagnosis of immunodeficiency in children with too many infections. Although certain rare immunologic syndromes require a sophisticated laboratory analysis, office and hospital laboratory tests are often available to make (or exclude) a presumptive or exact diagnosis of most of the immunodeficiency syndromes.
Severe Croup: The Child With Fever and Upper Airway Obstruction
Authors: Battaglia Jane Donohue
Published: December 2021

Infectious causes of airway obstruction can be distinguished by anatomic location and causative agent. Epiglottitis, the most common supraglottic obstruction, is bacterial in origin. An artificial airway and antibiotic therapy are mandatory. Spasmodic croup is a mild, transitory subglottic swelling which responds to mist and sometimes antihistamine therapy. Laryngotracheitis, or viral croup, affects the subglottic airway with fluctuating severity and is treated with mist, racemic epinephrine, oxygen, and sometimes steroids. Obstruction may be so severe that intubation or tracheostomy is needed. Laryngotracheobronohitis is the downward extension of viral croup, frequently accompanied by bacterial superinfection. Antibiotics may be needed, and airway intervention is more likely to be indicated than it is for uncomplicated viral croup. Bacterial tracheitis is a primary bacterial infection which causes thick secretions and membrane formation. Antibiotic therapy and sometimes airway intervention are needed. Diphtheria is a bacterial infection with toxin production, the treatment of which always includes both antitoxin and antibiotic and, sometimes, an artificial airway.
The Wheezing Infant
Authors: Fireman Philip
Published: December 2021

Wheezing is common in infancy and may represent either a minor or a serious, life-threatening illness. The differential diagnosis is extensive and may include organ systems other than the respiratory tract. Therefore, a careful and thorough approach to solving the problem is required. A systematic history, physical examination, and judicious choice of laboratory studies will enable the clinician to define the cause of the wheezing. Because the wheezing may represent a progressive respiratory problem, early diagnosis and treatment may prevent additional respiratory pathology and sequelae.
Recognition of Sexual Abuse in Children
Authors: Krugman Richard D.
Published: December 2021

Although sexual abuse of children has likely been present for ages, its recognition in the United States and other countries has dramatically increased in the past 5 years. Reported cases, which represent an unknown fraction of actual cases, now account for approximately 25% of all abuse and neglect reports. Local, county, and state agencies are staggering under the load, and many are relying heavily on pediatricians to assist them in their work.

DEFINITION

Sexual abuse can be defined as the engaging of a child in sexual activites that the child does not understand, to which the child cannot give informed consent, or which violate the social taboos of society. This definition is broad and includes intrafamilial and extrafamilial forms and all types of sexual activities (eg, exhibitionism, fondling, child pornography, oral, anal, and genital sexual contact).

The interface between "normal sexual exploratory behavior" and "sexual abuse" is not clearly defined. Pediatricians should, however, use common sense. If two 4-year-old children are found to be playing without clothes, touching each others' genitals and giggling, there is probably little to worry about. The same is true if two 15-year-old adolescents are discovered together. A probably exists if the 15-year-old child (some states set statutory 2- to 4-year limits within which sexual activities are not to be considered abuse) or if one 4-year-old child is engaging in fellatio or attempting intercourse with another preschool-aged child.
Recognition of Sexual Abuse in Children
Authors: MILBURN WILLIAM H.
Published: December 2021

The concept of child sexual abuse is so repulsive to most physicians and lay persons that to view the topic scientifically and nonjudgmentally is difficult. The article by Krugman in this issue is informative and should help the pediatrician in identifying cases he otherwise would have missed. The position taken by Krugman is relatively simple and safe. He recommends that the practitioner search for and report all cases of known or suspected child sexual abuse. If the pediatrician does this, he is protected by law. If he does not, he is risking legal penalties or a malpractice suit.

When dealing with a possible child rapist or sadist, no reasonable person would question Krugman's recommendations.
Guillain-Barré Syndrome in Children
Authors: Evans Owen B.
Published: December 2021

The Guillain-Barré syndrome is an acquired, monophasic illness of the peripheral nervous system. The etiology is an autoimmune attack directed against peripheral nervous system myelin, although the exact mechanism of the immunologic injury is unknown. The major clinical features are rapidly progressive weakness and hyporeflexia, and the most serious complications during the acute phase of the disease are respiratory failure and autonomic cardiovascular disturbances. A careful history, physical examination, and routine laboratory tests are necessary to make a clinical diagnosis and to exclude other disorders that cause acute weakness. Results of laboratory studies that aid in the diagnosis, CSF protein and electrodiagnostic studies, are usually normal during the first week of the illness. The prognosis for children with Guillian-Barré syndrome is excellent for full recovery using modern intensive care therapy for respiratory support and management of other complications.
Can Child Health Promotion Be More Rewarding?
Authors: LAND MARSHALL L.
Published: December 2021

In his article, "Behavioral and Developmental Components of Child Health Promotion: How Can It be Accomplished?" Dr Morris Green tries to sell us on the idea that we (as practicing pediatricians) can give more to our patients in child health supervision visits and that these, visits can be more rewarding to both families and physicians. I agree.

Many students and practicing pediatricians summarize a well-child visit with this presentation:

Billy Smith is a 9-month-old boy who is in for his regular check-up. He has a healthy past history, is growing normally, and his physical examination is normal. His hemoglobin is 12 g. His immunizations are up to date, and he should return in 3 months.

Counter that with Green's statement: "The chief justification for health promotion visits today relates to child development and behavior."
Genes, Syndromes, and Cancer
Authors: Miller Robert W.
Published: December 2021

Children are not equally susceptible to cancer. Some are at high risk, as recognized clinically and epidemiologically. Laboratory studies of these children and their neoplasms have recently provided new understanding of human carcinogenesis, which would not have come from animal experimentation alone. Certain mechanisms are proving to be the same for a spectrum of cancers. Hence, from the study of rarities, generalizations are becoming apparent, and they have implications for medical practice.

Clinicians have played an important role in this regard, because their observations have led to the recognition of high-risk groups. Sometimes a single case report starts an avalanche of productive research, as when Bruton1 first described congenital X-linked a-γ-globulinemia in 1952.

When mechanisms are delineated, strategies can be developed for prevention, early diagnosis, and better treatment. The pediatrician in practice should know the characteristics that put children at high risk of cancer, so the parents can be advised of exposures to be avoided. This will enable the physician to adjust the frequency and nature of examinations to allow detection of neoplasia early when the prospect for cure is greatest.

LEUKEMIA

Disorders With Preesistent Chromosomal Aberrations

Down Syndrome. In 1953 to 1955, individuals with Down syndrome were first noted to have a high frequency of leukemia. The observation was simple: three or four cases were identified within a short time on pediatric wards in Paris, North Carolina, and Minnesota.
Dermatology Potpouri
Authors: Rasmussen James E.
Published: December 2021

Pearly penile papules are common but not commonly seen in a physician's office. These multiple, white, coronal papules are rarely, if ever, the source of a disease but may cause the adolescent and young adult patient some surprise when they are first noticed. "Discovery" may occur after careful self-examination following suspected contact with venereal disease, sexual exposure, masturbation, or other situations that may be associated with anxiety. Frequently, the patient believes that the lesions originated "overnight"; this is never the case and usually represents inaccurate observation.

Lesions of pearly penile papules are characteristic in morphology and locale. They occur exclusively in postpubertal patients. Neinstein and Goldenring1 found pearly penile papules in 23 of 151 (15.2%) boys and men ranging from 11 to 22 years of age. Lesions were not noted in patients less than 14 years old or with a sexual maturity rating less than a Tanner 3. Glicksman and Freeman2 examined 229 men and found these lesions in 45 (20%). There does not appear to be any racial tendency, and whereas one series found a greater percentage in uncircumcised patients,2 a second could not substantiate this possibility.1 Although no surveys have been done in prepubertal patients, it is my clinical impression that these lesions do not occur in this age group.
Prognosis of Attention Deficit Disorder and Its Management in Adolescence
Authors: Klein Rachel Gittelman
Published: December 2021

Historical Background

The disorder now called attention deficit disorder with hyperactivity (ADDH), which will soon be renamed attention deficit/hyperactivity disorder, was first delineated by behaviorally oriented pediatricians. Their clinical descriptions are strikingly up-to-date.1,2 Moreover, their early clinical observations of the effectiveness of psychostimulants such as dextroamphetamine and methylphenidate have been amply substantiated by extensive investigations.3

Probably the major shift in our understanding of the disorder has occurred with regard to its prognosis. The standard expectation was that the disorder was self-limited, becoming attenuated with time and eventually remitting completely. Understandably, the clinical management of such children was affected by the perception of its natural history. As a result, psychostimulants were not recommended during adolescence when they were believed to cease to be antihyperactivity drugs and to have the typical stimulating effects well known in adults. As a matter of fact, the stimulants were believed to be potentially dangerous drugs in adolescence because of the expectation that they would lead to euphoria, tolerance, and addiction, in those who had previously been quieted with the drugs. There is now considerable evidence indicating that ADDH is not a maturational disorder that bears a benign prognosis. However, like many observations of astute practitioners, the opinion that children with hyperactivity regularly outgrow theirdifficulties in adolescence was not altogether wrong. The condition is not static; a proportion of affected children do improve, some to the point of being free of significant behaviour problems, but many do not.
Partnerships in Pediatrics
Authors: Menna Vincent J.
Published: December 2021

I believe that I am an expert in the field of partnerships having been a member of four since 1970, not including 2 years in the army working with four other pediatricians. Lest I be thought unstable and a vagabond, let me state that I have been a member of my most recent partnership for the past 10 years and have no intention of leaving. What appears as instability was actually a search for the utopian place to live and practice which, as we all know, does not exist. As the cliché"life is a compromise," and this has taken a while to learn.

When my army tour was half over, I looked for a place to practice.
Advances in Medical Genetics: Huntington Disease
Authors: Hecht Frederick
Published: December 2021

Medical genetics is currently enjoying a time of exploration and discovery. Huntington disease has long been of interest in adult medicine. The onset of clinical signs and symptoms is usually delayed until midadulthood. It may seem strange in this context to focus on Huntington disease, but advances in molecular genetics have brought Huntington disease into the purview of pediatrics.

These advances in molecular genetics make it possible to detect Huntington disease in a preclinical stage at or even before birth. The molecular approach does not replace prior approaches to Huntington disease but is synergistic and provides a model of the new genetics.

Huntington disease is synonymous with Huntington chorea. It is named after George Huntington who, like his father and grandfather before him, studied the disease in families on Long Island, NY.

Huntington disease is a more common hereditary disorder than phenylketonuria, which occurs in one of about 10,000 newborns in the United States. By contrast, about one in 2,000 persons is at risk for Huntington disease.

Although most cases start clinically in midadulthood, usually between 35 and 42 years of age, there is great variability in age of onset. About 3% of cases are diagnosed as juvenile Huntington disease before the age of 15 years. Late onset is well known after 50 years of age.
Physical Examination of Sexually Abused Children and Adolescents
Authors: Horowitz David A.
Published: December 2021

Sexual abuse of children has come to the forefront of our national consciousness through continued exposure in newspapers, in magazines, and on television. Despite this media attention, many pediatricians have not been trained to evaluate cases of suspected sexual abuse. Because estimates of the prevalence of sexual abuse in the general population range into the millions,1 it is likely that the office-based pediatrician will encounter more and more cases. Although guidelines for evaluation of child abuse exist,2 professional journals are now beginning to address sexual abuse. A recent article by Krugman3 reviewed the role of the office-based pediatrician in sexual abuse cases. Some points that he discussed included maintaining a high index of suspicion for both direct and masked presentations of sexual abuse; interviewing skills needed for both the child and family; legal ramifications; and the need for a coordinated effort among the many groups of professionals involved in each case, in an effort to minimize separate, repetitive, and traumatic evaluations. This article is intended to supplement Krugman's by providing a detailed description of the vaginal examination of the prepubertal child and adolescent.

PHYSICAL EXAMINATION

The prerequisite of a thorough, sensitive examination is time. This is the one element most lacking in a busy office or emergency room. Preparing the child for the examination will take more time the examination itself; this preparation is the most important part of the examination process.

PHYSICAL EXAMINATION

The prerequisite of a thorough, sensitive examination is time. This is the one element most lacking in a busy office or emergency room. Preparing the child for the examination will take more time than the examination itself; this preparation is the most important part of the examination process.
Infectious Exanthems
Authors: Hartley A. Howland , Rasmussen James E.
Published: December 2021

An exanthem is a generalized Cutaneous eruption associated with a systemic disease, usually of infectious cause. The actual mechanisms that produce exanthematous skin lesions are not known but certainly involve a number of different processes including direct damage to cells by pathogenic organisms, toxin production by these organisms, and the patient's immune response. Because the skin has a limited repertoire of responses to illness and injury, there is no single skin lesion whose morphology is pathognomonic for a particular infectious disease. Therefore, the exanthem cannot be considered as a separate entity but only as one component of the disease.

There is a great tendency in pediatrics to label every exanthem "an erythematous maculopapular rash." Although this term is often correct, by itself it is always incomplete. The distribution, configuration, and evolution of the rash are often critically important. Historical information regarding infectious exposure, general symptoms, and drug use help constitute a brief, initial evaluation. Although the importance of appropriate laboratory studies cannot be denied, they are often not diagnostic during the acute phase of an infectious exanthem. The initial diagnosis is based on history and clinical findings in the great majority of cases. Too often the physician feels pressured to make a diagnosis and initiate treatment immediately in the sick child with fever and rash. If careful initial evaluation of the child fails to yield a diagnosis, sequential observation of the course of illness throughout time is indicated.
The Pediatrician as Team Physician
Authors: Menna Vincent J.
Published: December 2021

Sports have been an integral part of my life since early childhood. I participated competitively until the end of my sophomore year in college when I realized that there was not much demand for a 68-in 180-lb foot-ball guard. My interest in sports continued, and in my medical school senior yearbook I am pictured reading the sports page.

My dad, a family practitioner and sports enthusiast, was the physician for our local high school. I witnessed the joy and satisfaction he obtained from the job. After I married and left home, there were weekends he could not visit because he "had to" be at a game.

When the opportunity arose in our school district, I applied and obtained the job of school physician.
Sickle Cell Diseases: Diagnosis and Management in Infancy and Childhood
Authors: Pearson Howard A.
Published: December 2021

From their basic genetic and molecular mechanisms to their clinical expression, the sickle cell hemoglobinopathies are among the best understood of human diseases. However, advances in clinical management have not progressed as rapidly: sickle cell anemia, as of 1987, is still largely incurable and its treatment is empiric and symptomatic. Despite this, today we have a better understanding of the natural history of the sickle hemoglobinopathies and many of their clinical complications. This has led to improved diagnostic, prophylactic, and therapeutic strategies which have reduced the considerable morbidity and mortality of these diseases.

The sickle cell disorders will be reviewed from genetic, biochemical, and clinical perspectives. Particular emphasis will be given to the rationale and strategies for neonatal testing and comprehensive clinical follow-up.

Although it is widely believed that the gene for Hb S is almost exclusively, African, it has, in fact, a much wider geographic distribution. There is a broad periequatorial sickle cell belt in Africa. From Africa, the sickle gene was introduced into the Western hemisphere by the 16th and 17th century slave trade. In the United States today, sickle cell disorders are particularly prevalent in the South and in the urban North, reflecting the demography of black America. In the Carribean and Latin America, relatively high frequencies are seen in Purto Rico, Cuba, Jamaica, Haiti, Panama, Guyana, and Brazil, but not in Mexico and most of South America.
Complications of Cardiac Surgery in Children
Authors: Engle Mary Allen , O'Loughlin John E.
Published: December 2021

LEARNING CURVE OF CARDIAC SURGERY

The era of cardiac surgery to help children with congenital heart disease began in 1939 when Dr Robert Gross successfully tied off a patent ductus arteriosus. The next step, in 1944, by Drs Helen Taussig and Alfred Blalock to create an artificial ductus arteriosus for cyanotic children with deficient pulmonary blood flow, threw open the doors for the creation of diagnostic and surgical treatment teams for "blue" babies and others with simple anomalies. Coarctation of the aorta, vascular ring, and pure pulmonic stenosis were anomalies amenable to surgery by 1949. By the early 1950s, use of surface hypothermia and brief periods of circulatory arrest improved the results of pulmonary valvotomy for pulmonic stenosis by permitting incisions of fused commissures under direct vision. Hypothermic arrest also permitted closure of secundum atrial septal defects. Lillehei's pioneering of extra-corporeal circulation in the middle 1950s expanded the surgical horizons by permitting longer periods for repair under direct vision for patients with ventricular septal defect, tetralogy of Fallot, and ostium primum type of atrial septal defect. A decade later, the venous switch type of physiologic repair of complete transposition of the great arteries was developed and perfected. In the early 1970s, miniaturization of equipment brought the age for safe open heart surgery from 5 years or oler down too early infancy.
The Time Machine
Authors: Guinter Robert H.
Published: December 2021

Just before Christmas, my 12-year-old son was reading The Time Machine by H. G. Wells. According to the story, the driver of this machine could travel to any date in the past or future, and he could return to the present time whenever he wished. We enjoyed talking about the story and about how much fun it would be to own a time machine. Where would we go and what would we see? He decided, among other things, that we should see his grandparents as children, to see whether their way of life really was as different from his as they say it was. Then we should visit some famous persons from the past and witness some great event, such as a Civil War battle. Then we should look to the future. What will we be like in 10 or 20 years? What will our city or country be like in 100 or 1,000 years? What will our planet be like in 1 million years? We thoroughly enjoyed ourselves as we talked about owning and using such a machine.
Nonimmune Hydrops Fetalis
Authors: McGillivray Barbara C. , Hall Judith G.
Published: December 2021

Hydrops fetalis or fetal hydrops is defined as a generalized abnormal accumulation of serous fluid in the entire body including abnormal collection in tissues and body cavities. It is not a specific disease but the end result of many fetal conditions. Presentation may be prenatal with ultrasound diagnosis on at the time of delivery.

There are many causes of fetal hydrops including cardiac, pulmonary, chromosomal, hematologic, and metabolic. The mortality rate is high, with the best survival in those infants who have cardiac rhythm abnormalities.

Proper assessment of the infant with hydrops requires familiarity with the major causes and a stepwise protocol for investigation. Not only are the usual blood-grouping studies important, but also chromosomal analysis, metabolic screening, and carefully performed autopsy. The aim of the protocol that is suggested is to improve intrauterine detection and survival after birth and to ascertain the cause of the condition to aid in counseling and care in subsequent pregnancies.
Cardiogenic Shock in the Neonate
Authors: Lees Martin H. , King Douglas H.
Published: December 2021

The neonate with circulatory failure or cardiogenic shock is a formidable challenge. However, with an organized treatment plan and a well-orchestrated team, these infants can be effectively managed. Once the infant has been stabilized, and septic and hypovolemic shock have been excluded, attention should be directed to the four most likely causes of cardiogenic shock: structural heart disease with left heart obstruction, cardiac muscle disorders, cardiac dysrhythmias, and cardiac metabolic disorders.
Newborns With Acute Respiratory Distress: Diagnosis and Management
Authors: Schreiner Richard L. , Bradburn Niceta C.
Published: December 2021

If respiratory distress develops in the newborn after he or she has been normal for more than a few hours, bacterial sepsis, inborn errors of metabolism, cardiac disorders, and intracranial hemorrhage should be suspected. It is virtually impossible to determine whether an infant with mild respiratory distress in the first few hours of life will have rapid resolution of disease or progress to severe respiratory distress. That is, it is difficult to differentiate among transient tachypnea of the newborn, sepsis, and pulmonary hypertension in the infant with mild respiratory distress in the first few hours of life. Transient tachypnea is a diagnosis that can only be made with certainty after the infant's respiratory distress has resolved. The newborn infant with mild respiratory distress of more than a few hours' duration requires a minimum number of laboratory tests including chest roentgenogram, hematocrit or hemoglobin, blood glucose determination, direct or indirect measurement of arterial blood gases, and blood cultures. The liberal use of oxygen in the near-term, term, or post-term vigorous but cyanotic infant in the delivery room may decrease the incidence and/or severity of respiratory distress due to pulmonary hypertension. A newborn infant with respiratory distress for more than a few hours should be considered a candidate for infection.
Statistical analysis of motivators in the Serbian banking sector
Authors: Janovac Tatjana , Simeunović Ivana , Virijevic Jovanovic Saša , Nedović Nemanja , Tomić Gordana
Published: December 2021

Starting from the fact that motivation presents an important factor that directly affects employee effectiveness, this paper examines the attitudes of employees regarding the ability of their leaders and managers to apply appropriate motivators in the work process. The empirical research was based on a sample of 117 respondents who were employed in the Serbian banking sector. The application of statistical methods supported by SPSS software provided the analysis of three groups of motivators applied by leaders and managers, which included material, non-monetary and work-related motivators. In addition, we examined the relationship between the length of service and respondents' attitudes regarding the ability of leaders and managers to involve employees in the decision-making and goal-setting process. Testing hypothesis H1 using the Wilcoxon signed-rank test showed the existence of a pronounced ability of leaders and managers to motivate employees by applying material, non – monetary and work-related motivators. Testing hypothesis H2 using Spearman's Correlation Test indicated a correlation between the studied variables at the population level. The findings of the empirical research can contribute to better efficiency and effectiveness of the operations in the banking sector.
A study to investigate the impact of social research toward research productivity
Authors: Kaba Abdoulaye , A. El Refae Ghaleb , Eletter Shorouq , Yasmin Tahira
Published: December 2021

The main purpose of this study is to investigate the relationship between demographic diversity and research productivity. The paper attempts to identify the influence of gender diversity, college diversity, and diversity in collaboration on the research productivity of faculty members. The paper is based on data collected from the Deanship of Scientific Research and the Office of Documentation, Al Ain University (AAU). The findings of the study showed significant differences in college diversity. Faculty members of the English instruction colleges dominated the leadership in research productivity. The independent variables appeared to predict 23% of the variation in research productivity. AAU faculty members should increase internal and external collaborations and network in scientific research. Similarly, AAU also should support and promote research collaboration and networking among faculty members through a comprehensive strategic plan and guidance for internal and external collaborations.
Diversification of business and technical efficiency of companies in Vietnam
Authors: Nguyen Anh Phong , Nguyen Anh Vu , Ho Thi Hong Minh , To Thi Thanh Truc
Published: December 2021

This study focuses on assessing the technical efficiency of enterprises across 63 provinces of Vietnam from 2015 to 2018 and the factors affecting their technical efficiency, in which we focus on two factors: business diversification and ICT index. In this research, we use the DEA method to group effective and ineffective enterprises, then use the GMM method to estimate the factors affecting to the firm’s performance. The research results show that the technical efficiency of enterprises in the provinces is quite small (about 10%), most of the enterprises have not achieved the technical efficiency due to the wasteful use of input factors. Research shows that business diversification and ICT both have a positive impact on efficiency, from which to increase technical efficiency, enterprises in provinces need to pay attention to business diversification and improve ICT index. in terms of the local government