bluuebunny/crossref_metadata_2025 huggingface dataset:
Lamellar Ichthyosis
Authors: Sandberg Nils Olof
Published: December 2021

Congenital ichthyoses represent a heterogeneous group of rare skin diseases. The disorders are heritable and have in common a varying degree of hyperkeratosis. During the years there has been much confusion regarding the classification of the ichthyoses. Today we have a classification based on clinical, genetic, cellular, and kinetic observations (Table). 1,2 Most patients fit into one of the four major types of ichthyoses. Beside those major types, congenital ichthyosis may be seen as part of several uncommon syndromes. The most often seen congenital ichthyosis is the lamellartype which is inherited as an autosomal recessive trait.3

Lamellar ichthyosis was first described by Seeligmann under the title of "epidermal desquamation of the newborn" in 1841.4 Lentz and Altman reviewed the literature in 1968 and found a total of 103 cases.5

CASE HISTORY

A.S., a 2,720-gm infant was born at 37 weeks of gestation. His parents and a brother are healthy. Pregnancy and labor were uncomplicated. Apgar scores at one and five minutes were 10.

At birth the infant was encased in a shiny collodion membrane. Because of the tight envelope his nose, ears, fingers, and toes were whitish, and the baby could hardly move. The lips and eyelids were everted. The diagnosis of collodion baby was made immediately (Figs 1 and 2).
Cytomegalovirus Infections
Authors: Hanshaw James B.
Published: December 2021

It has been 25 years now since the first strains of cytomegalovirus (CMV) were isolated. At that time, and for several years thereafter, the infection was recognized as the cause of a rare and severe congenital infection referred to as cytomegalic inclusion disease (CID). More recently the virus has been associated with a wide range of clinical abnormalities.1 It is the most common congenital infection of man and even inapparent infections are now known to have long term CNS sequelae.2,3 This review will focus on information that the child health clinician must be aware of in dealing with CMV infections acquired at different stages of development.

EPIDEMIOLOGY

It is probable that most individuals in every society are infected with a strain of CMV at some time in life. Much of the early seroepidemiologic data in the literature were based on the complement fixation test, a relatively insensitive method compared to the immunofluorescent and indirect hemagglutination tests for CMV antibody. The frequency of congenital infection ranges from 0.2% to 8.0% with an average in the United States of about 1%. The highest rate of infection is in infants born to teenaged mothers from lower socioeconomic groups. Not all the infants born excreting the virus are infected as a result of primary infection in the mother.
Hyperventilation
Authors: MCNIEL JAMES B.
Published: December 2021

Educational Objective 92 relates to appropriate recognition and management of hyperventilation syndrome. The evaluation largely deals with psychological causes in adolescents. The following case reminds us of another very common cause of hyperventilation, especially in young children.

R.J.H.

Case Presentation

The patient is a 2½-year-old boy who was referred because of hyperventilation. He had been well until two weeks before admission when he developed a fever. Four days before admission the child developed ulcers in his mouth. He had a low fever, and two days before admission the mother gave the child 16 grains of aspirin throughout the day, four 1-grain aspirins at a time, four times a day.
Editorial: Genetics-Neonatology-Cardiology
Authors: H. R. J.
Published: December 2021

The topics selected for education for the 81/82 PREP year are Genetics, Neonatology, and Cardiology. Each area presents a special challenge. Each is a field in which enormous advances in knowledge have occurred in the past decade. Each represents the use of very high level technology—knowledge and technology most of us as general pediatricians do not have. Most of the technology is hospital based; expert teams working daily with the complex issues are best able to provide accuracy and efficiency. The main question I have faced as I planned this year of PEDIATRICS IN REVIEW has been what should the generalist know about G-N-C?
Role of the General Pediatrician in Genetics
Authors: Nadler Henry L.
Published: December 2021

The pediatrician must be knowledgeable regarding the general principles of genetics and their application to common diseases; should recognize the importance of an accurate family history, which, in most instances, need not be more than two or three generations of immediate relatives, and be able to obtain and interpret such; should recognize unusual clusters of disease, such as early onset coronary artery disease or cancer, and question the possible implications; should be aware of the fact that specific genetic diseases may be more common in certain ethnic and racial groups; should be able to counsel patients, including families, for common disorders and refer complicated cases to appropriate experts; should recognize the possibility of a genetic disorder and obtain appropriate specimens for analysis or refer such patients to experts; should be knowledgeable regarding

[See figure in the PDF file]

genetic screening in general and be aware of specific recommendations including newborn screening and carrier detection; should be familiar with indications for intrauterine diagnosis; and should be aware of the back-up resources for diagnosis, counseling, and treatment in the community.
Should HBsAg Carriers Breast-feed?
Authors: KRUGMAN SAUL
Published: December 2021

A thoughtful pediatrician has written questioning Dr Krugman's assertion that "HBsAg carrier mothers in developed countries should avoid breast-feeding, if possible"(PIR 2: 121, 1980/81). He worries that with the lack of evidence that there is any risk from breast-feeding by a HBsAg carrier mother, this situation would be used by pediatricians and mothers who take the position, "Yes, breast-feeding is fine but when in doubt about anything, stop it." We asked Dr Krugman to comment on this point, and he has responded as follows:

Regarding the issue of breastfeeding by HBsAg carrier mothers in developed countries of the world the following facts are known:

1. Infants born of HBsAg-positive mothers may be infected in utero, or at the time of birth, or several weeks or months after birth if there is continous exposure to the virus.
Chlamydial Infections
Authors: Hammerschlag Margaret R.
Published: December 2021

Chlamydia trachomatis was first described by Halberstadter and Von Prowazek in 1907, as a characteristic intracytoplasmic inclusion in the conjunctival scrapings from a patient with trachoma. Although the organism was identified as the etiologic agent of trachoma and inclusion conjunctivitis in the neonate, a full understanding of its nature was not feasible until C trachomatis was first successfully propagated in cell cultune in 1965. This technical innovation greatly facilitated investigation of the biology and the epidemiology of chlamydial infections in man.

C trachomatis is now recognized to be one of the most prevalent sexually transmitted diseases in the United States today. It is responsible for 40% to 50% of cases of nongonococcal urethritis (NGU) in men and a large reservoir of genital infection, largely asymptomatic, in women. In the United Kingdom, where NGU is a reportable disease, NGU is now more prevalent than gonorrhea and is considered to be the most important male veneral disease. The cervical infection in women is of special importance to pediatricians as the infection may be transmitted to the infant during parturition, leading to both ocular and respiratory disease. A thorough understanding of the microbiology and epidemiology of C trachomatis is an important prerequisite for appreciating the clinical features, diagnostic methods, and management of these infections by pediatricians.
Clinical Perspectives on Mitral Valve Prolapse
Authors: MURPHY JOHN D. , FYLER DONALD C.
Published: December 2021

The devastation caused by the current epidemic of mitral valve prolapse (MVP) was emphasized recently when an irate psychiatrist asked why cardiologists were "terrorizing the populace." He had spent a tedious afternoon with two patients with MVP who were reacting poorly to the news that they might drop dead. The psychiatrist's problems were forgotten until a recent article resurrected the fact that 17% of the US population (37.7 million people) may have MVP and are also, presumably, at risk for sudden death.1 A swirl of thoughts around the costs of echocardiograms, fees for cardiac consultation, investments in echocardiography companies, and the need to train more psychiatrists fluttered by in a panorama of "Big Bucks" regional MVP centers and an act of Congress to establish a National Institute of Prolapse.
Neutropenia in Children
Authors: Oski Frank A.
Published: December 2021

Neutropenia, a decrease in the number of circulating neutrophilic leukocytes, often provides a diagnostic challenge to the pediatrician. When the pediatrician is unable to ascertain the cause of the neutropenia, pediatric hematologists are also frequently frustrated in their attempts to uncover a satisfactory explanation for the finding.

The total white cell count and the absolute granulocyte count vary as a function of age as depicted in Table 1. The while blood cell count and the absolute granulocyte count are characteristically increased in the normal newborn and both rapidly decrease during the first week of life. After the first year of life, the normal neutrophil count generally ranges between 1,500 to 8,000/cu mm. Although neutropenia is conventionally defined as the presence of an absolute granulocyte count (neutrophilic leukocytes and band forms) of less than 1,500 cu mm, an increase in the incidence of infections is rarely observed until the absolute granulocyte count remains persistently below 1,000/cu mm.

The black population and possibly Yemenite Jews characteristically have lower total white cell counts than age-matched control subjects. Approximately 30% to 40% of black male adults have a total white blood cell count of less than 5,000/cu mm as compared with only 7% of white males.2 Presumably the same difference in distribution of white cell counts exists in the pediatric population as well.
Treatment of Dehydration in Infancy
Authors: Finberg Laurence
Published: December 2021

NORMAL CHEMICAL ANATOMY AND PHYSIOLOGY

Before discussing dehydration it is worthwhile to review the normal features of an infant with respect to content and distribution of water and mineral. Approximately 70% of the lean body mass is water. The distribution of this water is shown in Fig 1. The plasma volume owes its integrity to its protein content, which is a relatively impermeable species of molecule. The extracellular fluid composition differs strikingly from the intracellular fluid despite the movement of most ions across cell membranes because of an active transport system (Na+, K+, ATPase) in the membrane that extrudes sodium. The compositional differences are diagrammed in Fig 2.

The second consideration necessary to handle any problem is that of obligatory water requirements to replace losses. Table 1 gives the relationship between caloric expenditure and water loss with the basal state as the point of reference. For ordinary clinical circumstances requirements are about 1½ times basal state. Electrolyte requirements have a wide range. Practical considerations make it necessary to provide solute in an intravenous solution, when one is needed, to prevent hemolysis. All conditions may be met by allowing 2 to 3 mEq/kg/day of both sodium and potassium. Chloride and bicarbonate (or other base) ions for the healthy infant awaiting surgery, for example, should be divided about 3:1.
Use of Echocardiography in Evaluation of General Pediatric Diseases
Authors: Williams Roberta G. , Bierman Fredrick Z.
Published: December 2021

A variety of general pediatric diseases cause secondary cardiovascular abnormalities. Echocardiography and other noninvasive techniques have provided a better understanding of the incidence, time course, and degree of cardiac involvement in these disorders. Echocardiography yields anatomic and physiologic information with little patient discomfort and a relatively small investment in medical personnel and equipment and therefore offers a practical means for both population screening and serial evaluation of individual patients.

When an ultrasound beam, produced by the rapid expansion and contraction of a rare earth crystal, is directed through the soft tissues of the body, some of the acoustical energy is absorbed and a portion is reflected back to the transducer. The depth and intensity of the returning signal can be displayed on an oscilloscope (A mode), or the signal can be swept across an oscilloscope or strip chart to record both depth and time (M-mode) as seen in Figs 1 and 2. Cardiac structures are identified by their characteristic motion and distance from the transducer. The transducer can be rotated or scanned from one structure to another in order to gain spatial information.
Antimicrobial Agent-Induced Pseudomembranous Colitis
Authors: Feigin Ralph D.
Published: December 2021

Antibiotic-induced colitis has been associated with the oral and parenteral administration of many agents. Diarrhea has been related to toxin elaborated by Clostridium difficile and its action on the gastrointestinal mucosa. Although other factors may be involved, they have not been identified. Treatment includes: (1) discontinuation of the presumed offending antibiotic, (2) supportive care including fluid replacement, and (3) administration of vancomycin orally; its use has been associated with symptomatic improvement and decreased toxin in the feces.
Obesity in Children
Authors: Weil William B.
Published: December 2021

Over the last few decades our national concern has been shifting from undernutrition to obesity as the major nutritional problem of the country. Concern about the health risks created by obesity is shared by health professionals and large segments of society. Public attention has been directed to obesity because it has been identified as a risk factor for many diseases, particularly those of the cardiovascular system. In addition, the public image of the desirable body form is culturally determined and strongly influenced by factors such as advertisements depicting lean individuals, especially lean women, as sexually attractive, intelligent, and popular models for owning everything from beautiful clothing to expensive automobiles. The social bias that is generated is so powerful and widespread among middle and upper middle class Americans that there is well documented discrimination against the obese in education at all levels and in most professional and social organizations.

For pediatricians, the problem of obesity creates four major concerns: (1) obesity in the adult may have its origin in infancy and/or childhood; (2) feeding practices, especially in early life, may be a major etiologic factor in the ultimate development of obesity; (3) obesity in children may be associated with illness during childhood; and (4) discrimination against obese children is inherently serious and may be devastating to long-term personality development.
Posttraumatic Epilepsy
Authors: Rosman N. Paul , Oppenheimer Edgar Y.
Published: December 2021

Head trauma is an important cause of epilepsy in children. Posttraumatic seizures are divided into those that occur within the first week following the head injury (early posttraumatic epilepsy or early PTE), and those that occur after the first week (late PTE). Early PTE occurs in approximately 5% of children hospitalized following head trauma. The more severe the head injury, the greater the risk of early PTE. Late PTE also occurs in approximately 5% of children hospitalized after head trauma. As with early PTE, the risk for late seizures is greater in individuals who sustain more severe head injuries. Also, late PTE may be more likely to arise if early PIE has occurred. The EEG is not helpful in the prediction of late PTE. Acute treatment of posttraumatic seizures is essentially the same as that used for nontraumatic seizures. The efficacy of pharmacologic treatment for the prophylaxis of late PTE in headinjured patients remains uncertain.
Infective Endocarditis
Authors: Newburger Jane W. , Nadas Alexander S.
Published: December 2021

Infective endocarditis is one of the most serious complications of structural heart disease. It occurs primarily in children and adolescents with preexisting anatomic abnormalities of the heart, with an incidence that varies with the specific cardiac lesion. An identifiable potential source of bacteremia precedes infective endocarditis in approximately one fifth of cases in children. The high mortality (19% to 25%), morbidity, and prolonged treatment course of infective endocarditis make its diagnosis, management, and prevention a matter of great importance to pediatricians.

PREDISPOSING FACTORS

Structural Heart Disease

Cardiac malformations are the major predisposing factor to infective endocarditis. Although rheumatic heart disease has gradually declined as a substrate for endocarditis, the increased survival of children with congenital heart disease in the past two decades has increased the number and age of children susceptible to infective endocarditis. In general, lesions leading to a high velocity of blood flow through a heart valve, septal defect, or blood vessel are associated with increased susceptibility to endocarditis (Table 1). Children at highest risk are those with cyanotic congenital heart disease (eg, tetralogy of Fallot), ventricular septal defect with aortic regurgitation, left-sided valvar regurgitation or obstruction, prosthetic valves, and recent cardiac surgery. Endocarditis is exceedingly uncommon in individuals with isolated atrial septal defect secundum and with mild pulmonic valve stenosis.
Respiratory Failure in the Pediatric Patient
Authors: Pagtakhan Reynaldo D. , Chernick Victor
Published: December 2021

The unique aspects of respiratory structure and function of newborn infants and older children make them particularly vulnerable to respiratory failure. In general, both obstructive airway disease and restrictive parenchymal disease may cause type I or type II failure. Only type I failure (hypoxemia, without hypercapnia) occurs with those disorders that lead to ventilation-perfusion imbalance, diffusion impairment, or an increase in the alveolar-arterial oxygen tension difference without concomitant ventilatory inadequacy. On the other hand, depression of the respiratory center and restrictive lung disorders due to failure of respiratory neuromuscular function (but normal lungs) lead to alveolar hypoventilation and type II failure (hypoxemia plus hypercapnia). Both clinical and laboratory assessment are crucial in establishing the precise type of respiratory failure and in directing the correct approach to management. The goal of therapy in patients with otherwise healthy lungs is to restore normal gas exchange as rapidly as possible and then to treat the underlying specific clinical condition. In patients with underlying chronic lung disease and acute respiratory failure, the goal of therapy is to return gas exchange to its previous level of compensation. Immediate recognition of total respiratory failure—cardiopulmonary arrest—and its prompt management requires clinical judgment as well as a maintained skill at cardiopulmonary resuscitation. The pediatrician should know not only when to institute immediate therapeutic measures but also when to transfer patients to an intensive care unit. The principles of intensive respiratory care of patients with acute respiratory failure also apply to the management of patients with mild respiratory insufficiency and, therefore, are essential for the prevention of acute respiratory failure—truly within the province of the general pediatrician.
Male Pseudohermaphroditism
Authors: Levy Donna J. , Levine Lenore S. , New Maria I.
Published: December 2021

Male pseudohermaphroditism is the condition of incomplete male differentiation of the external genitalia in an individual with a Y chromosome. The gonads of the male pseudohermaphrodite, when present, are either streak gonads or testes.

A wide range of disorders and phenotypic presentations are encompassed by the term male pseudohermaphroditism. The following case describes a male pseudohermaphrodite who presented with ambiguous genitalia. However, as will be discussed in greater detail below, the external genitalia of male pseudohermaphrodites may be unambiguously female or may appear to be almost completely masculinized.

CASE HISTORY

The infant, a 9 lb 3 oz product of a full-term gestation, was born to a 23-year-old gravida 1, para O mother via normal spontaneous vaginal delivery. Pregnancy was uncomplicated and there was no history of maternal ingestion of medication. At birth, ambiguity of the external genitalia was noted with a 1-cm phallus, one opening on the perineum, and a small palpable mass in each of the large unfused labia. No uterus was palpable on rectal examination. Serial serum electrolytes remained normal. Genetic evaluation revealed a chromatinnegative buccal smear, with Y fluorescence. Karyotype analysis of peripheral lymphocytes revealed a 46, XY chromosomal complement. Radiologic studies included an excretory urogram revealing normal functioning kidneys with intact pelvocalyceal systems, ureters, and bladder, and a blind-ending vagina, with no evidence of cervical indentation. Base line 24-hour urinary 17-ketosteroid and 17-hydroxycorticosteroid determinations were all normal.
Narcotic Withdrawal Syndrome in the Newborn
Authors: Sweet Avron V.
Published: December 2021

The passive addiction of fetuses to narcotics has been a matter of record for nearly a hundred years. But even as late as the first half of this century, few physicians were aware of the manifestations of withdrawal by the newborn or its therapy. From the late 1950s, when narcotic addiction increased to become a widespread major health calamity, to the present a great deal has been learned about addiction among pregnant women and its effects upon the fetus and newborn. For two decades addiction was most commonly due to heroin but, following the widespread establishment of methadone maintenance clinics, that synthetic opioid now may be the most frequently encountered addictive agent among pregnant women and their newborn infants in the United States. Some appears as unprescribed "street" methadone, which is usually used with an opiate. Moreover, uncooperative participants in methadone maintenance programs also take opiates. As a consequence, there may be homogeneous groups of pregnant heroin addicts and hornogenous groups of pregnant women taking methadone alone but there is a great deal of drug heterogeneity among addicts. Hence, much is known about opiate addiction in pregnancy and its effects upon the fetus and newborn, but much less is certain about the effects of methadone alone.
Pain and Symptom Management in Pediatric Palliative Care
Authors: Komatz Kelly , Carter Brian
Published: December 2021

Pain and symptom management is considered one of the cornerstones of palliative and hospice medicine. However, general clinicians and specialists are not usually comfortable addressing the most common forms of pain seen in the pediatric population. In addition, non-pain symptom management, especially when related to underlying chronic medical conditions, can be managed by the general clinician and specialists. The goal of this article is to educate clinicians about pain categories, taking a detailed pain history, and developing a plan for treatment, including nonpharmacologic methods. Finally, we discuss common symptoms in patients with chronic medical conditions, including first-line treatment options.
Care of the Visually Handicapped Child
Authors: Scheiner Albert P. , Moomaw Margot
Published: December 2021

The major function of the pediatrician in the care of the visually impaired child is not unlike that of providing care for any child with chronic illness. This includes: First, a reasonable knowledge of the child's disorder and the effect of that disorder on the health and development of the child and his family; second, the coordination and explanation of specialty evaluations; third, the monitoring and advocacy for appropriate program implementation; and finally, and possibly most important, being available to parents to understand, explain, and offer support during times when the magnitude of the child's problem is overwhelming. What else is pediatrics all about?
Preparticipation Health Evaluation for Competitive Sports
Authors: Strong William B. , Linder Charles W.
Published: December 2021

The large population of young people combined with the current popularity of sports has produced the largest number of sports participants in the history of our country. In 1978, the number of young people participating in high school athletics alone was cited to be 5.5 million.1 Although encouraging from both a sociologic and preventive health standpoint, an undesirable consequence of this level of participation is the high rate of injuries and death. Increasing attention is being paid to these problems, and as a result the "specialty" of sports medicine is emerging and utilizing modern epidemiologic and research methodologies. Because many serious athletic injuries and some deaths are preventable, much attention has been given to identifying risk factors in athletes and aspiring athletes.

The preparticipation health evaluation (PHE) for competitive sports should be an integral component of all strenuous sports programs. The major purpose of the preparticipation evaluation is to attempt to reduce life-threatening or disabling injuries by identifying predisposing factors, recommending preparatory and/or rehabilitative measures, and assisting in matching the participants with an appropriate sport or position. The purpose of the PHE should not be to exclude youths from participation but to guide them into more appropriate activities because of a medical problem. A secondary benefit is the direct association of sports participation with good prevention health care.
The Pediatrician's Role in Drug Education
Authors: Parcel Guy S.
Published: December 2021

Pediatricians involved with local schools are often asked to help in dealing with child and adolescent drug use. Most states have mandated schools to provide drug education programs; the use of drugs has become endemic to American life. The pediatrician is likely to be considered a resource in the area of drug education because of his or her knowledge of pharmacology and the potential detrimental effects of drugs. School personnel may not be aware of the pediatrician's interest and expertise in related areas such as child development and human behavior.

PROBLEM OF CHILD AND ADOLESCENT DRUG USE

Prior to 1960, most illicit drug use was confined to inner city poverty groups or isolated fringe groups. During the late 1960s, the American public became alarmed by increasing illicit drug use by the middle and upper class, especially among the young. The spread of illicit drug use was first apparent in the urban middle-class college students and eventually spread to high school students and rural populations. The use of drugs also spread in terms of the numerous types of drugs used.

Marijuana became a popular drug and, in some communities, tended to be used like alcohol, as a social drug. LSD and other hallucinogens hit their peak use in the late sixties and early seventies.
Delinquency and the Pediatrician
Authors: Copoulos Ella , Hein Karen
Published: December 2021

CASE OF PAUL S.

Upon arrival at the office, you receive a telephone call from Mrs S. Over the weekend her normally "well-behaved" 14-year-old son Paul, together with a group of school friends, were found by the police at a construction site in the process of spray-painting nicknames, slogans, and "obscene language" on a newly built concrete wall. Several bottles of beer, cigarette papers, loose tobacco, and a small quantity of marijuana were found nearby. Although he began to cry when his parents arrived at the police station to arrange his release, Paul has been "uncommunicative and sullen" ever since, refusing even to attend school today. Paul and his parents must appear before the Juvenile Court in two days, and Mrs S. would like you, as Paul's pediatrician for 14 years, to "see what's wrong" with Paul before then.

Among the various options that you might initially choose, how would you decide whether or not to:

—refer Paul to a teenage drug rehabilitation program,

—arrange an adolescent psychiatric consultation for Paul because of his sudden change in behavior,

—have Paul come to see you at the office, to be followed by a meeting with his parents,

—reassure Mrs S. over the telephone that Paul is a normal healthy teenager,

—inform the school guidance counselor of the reason for Paul's absence and request information concerning his school performance and activities.
Counseling of Parents Around the Birth of a Baby
Authors: Charney Evan
Published: December 2021

One of the pediatrician's most pleasant tasks is counseling families around the birth of a child. It is a joy if the baby is healthy, more difficult (though even more important) if there is an abnormality. In this article, the physician's postpartum role with the full-term, basically healthy infant will be discussed; although the comments apply to the preterm or ill child as well, in those instances a good deal more is involved, and is outside the scope of this presentation.

Of necessity, a discussion of this kind is subjective, reflecting the biases and experience of the author. There is very little recorded knowledge on this topic, and physicians have evolved a variety of approaches that probably work equally well. The point here is for the health provider to keep certain issues in mind; how they are dealt with will vary with the personality of physician and patient, and with the practice pattern of the community. In fact, if the postpartum visit is the first contact with a new family—a common phenomenon—its major purpose is for family and physician to get to know each other's "style." The pediatrician wants to know what kind of parents he will be dealing with—are they basically a stable couple, confident of their abilities, or do they seem less secure? Is there a supportive relationship? Are they married? Is the mother very organized, with written lists of questions prepared, or does she seem more casual, with few questions? Who does the talking in the interview? The parents, in turn, are watching to see whether the physician is brusque or relaxed, authoritarian in his manner or more nondirective.
Evaluation of Growth and Maturation in Adolescence
Authors: Rosenfeld Ron G.
Published: December 2021

The term adolescence denotes the period of time between the onset of puberty and the completion of physical maturation. During these transitional years, full reproductive capacity is attained, manifested by spermatogenesis in the male and ovulation in the female. Concomitantly, major physical changes occur, including (1) full maturation of the gonads, external genitalia, and secondary sexual characteristics, (2) alterations in lean body mass and fat distribution, and (3) rapid skeletal growth, ultimately terminated by fusion of the epiphyses and the attainment of final adult stature.

Although we are accustomed to equating adolescence with the teenage years, the last century has been characterized by a progressive lowering of the normal age for pubertal development, so that presently, 50% of girls in the United States reach puberty while in elementary school. The implications of this earlier physical maturation, particularly in terms of sexual education and contraception counseling are readily evident. Furthermore, as the normal onset of puberty occurs earlier, the psychological ramifications of pubertal delay and short stature in adolescence become increasingly pronounced. It is not unusual, nowadays, to see 13 or 14-year-old patients (and their parents) who are concerned about delays in sexual maturation and skeletal growth, and while the majority of such patients are found to be normal variants, their anxieties and concerns are nonetheless real.
Status Epilepticus
Authors: Barbosa Ernest , Freeman John M.
Published: December 2021

Status epilepticus is a serious medical emergency requiring immediate and rational therapy. Initial management is directed at stabilization of vital functions and prevention of aspiration or physical injury. Quick but thorough assessment with appropriate blood chemistries to determine possible etiologies should be obtained before drug therapy is instituted. Initial drug management is directed at the rapid control of status with prevention of recurrence by sustaining a therapeutic level of a long-acting anticonvulsant medication. Few patients should remain in status more than 30 minutes after initiation of therapy.
The Pediatrician's Role in Public Law 94-142
Authors: Wright Gregg F.
Published: December 2021

In the seven years since it was passed, Public Law 94-142, The Education for All Handicapped Act of 1975, has generated new services for handicapped children and in the process has raised many difficult financial, political, educational, medical, and ethical questions. It has changed the scope of public schools and the personnel within them, and it has changed the expectations of parents, physicians, and educators alike. There is currently much talk of the possible repeal of PL 94-142. This review will emphasize the importance of a pediatric voice in this future. The changes that have come about as a result of PL 94-142 will not easily disappear even if the bill were to be repealed. On the other hand, a strong voice of advocacy for handicapped children will be needed to ensure that the positive lessons learned from PL 94-142 will be continued.

PL 94-142 itself, as a law, is only one component of a constellation of surrounding issues affecting handicapped children. One must consider separately the actual provisions of the law, the constitutional and case law on which it was based, the federal regulations that have been published and updated to interpret the law, the state plans formulated by each state to comply with the law and regulations, the funding that may or may not be appropriated at federal, state, and local levels to support the programs, and the actual practices within local school districts attempting to comply (or circumvent) these state requirements.
Cryptorchidism
Authors: Allen Terry D.
Published: December 2021

Recent developments, particularly in the field of endocrinology, have substantially altered many conventional concepts about descended testis. The disease can no longer be viewed solely as a mechanical problem in descent, and there is an increasingly greater emphasis being placed upon the early resolution of the disorder in an effort to maximize ultimate testicular function. In this brief review, some of the controversies and current philosophies regarding this subject are explored.

THE ENDOCRINOLOGIC QUESTION

Although etiologic considerations in cryptorchidism have always included the possibility of inadequate hormonal stimulation, this concept has generally been overshadowed by one that emphasizes mechanical impediment to descent. This viewpoint seemed justified by the fact that endocrine therapy had never yielded more than modest results and by the feeling that it was difficult to account for unilateral cryptorchidism on the basis of a generalized endocrinopathy. After all, "if inadequate hormonal stimulation were the cause of maldescent of one testis, why did the other one descend?" The recent work of Job and associates in Paris, however, has shattered many of these arguments with the revelation that endocrine abnormalities can indeed be identified in patients with cryptorchidism and that furthermore these abnormalities canbe identified in patients with unilateral cryptorchidism as well as in those with bilateral disease.
Otitis Media with Effusion: An Approach to the Management of Persistent Symptoms and Signs in the Pediatric Patient
Authors: Kaleida Phillip H. , Stool Sylvan E.
Published: December 2021

Otitis media with effusion continues to be a major public health problem because of its prevalence and potential consequences. We know too little regarding its pathogenesis, management, and long-term consequences. These gaps in our knowledge make it difficult to formulate precise plans of management. Research, necessary at both the clinical and basic-science levels, often is especially difficult because of the large number of variables to be considered in this condition.

It is important for the pediatrician to be aware of the variable natural history of otitis media, and of the variable hearing loss accompanying the middle-ear effusion. Pneumatic otoscopy is more sensitive in diagnosing OME than mere visualization of the tympanic membrane. Finally, tympanostomy tubes appear to be beneficial in most children for whom they are indicated; but vigilance is required to detect potential complications both of the disease and of the treatment(s).

Feigin16 recently summarized the state of the art of otitis media with effusion: "One of the great paradoxes of modern medicine is that our knowledge is often most incomplete with respect to the disorders that afflict mankind with the greatest frequency. Such is the case with otitis media."
Other Causes of Red Urine
Authors: COLEMAN ALLAN B.
Published: December 2021

I wondered, as I went through Kaplan's fine review, whether from oversight, or perhaps experience different from my own, he mentioned only "en passant" viral trigonitis, for which serveral serotypes of adenoviruses have been accused, and which we seem to see in a brief flurry of cases here in Washington, DC area most years. They are characterized by dysuria, and gross hematuria, often worse at the termination of micturition. They have sterile urine and a benign course. We have tended, especially after the first few cases of the season have alerted us, to treat them expectantly and with a minimum of further investigation, provided the process is not on-going, and no other reason for concern is evident. The few patients who have had their bladder subjected to endoscopy have shown intense hemorrhagic trigonitis.
Cerebral Palsy and Toe-Walking
Authors: TAFT LAWRENCE T.
Published: December 2021

A few readers of the article on cerebral palsy (PIR 1984;6:35-42) have indicated that persistent toe-walking may be a "normal" phenomenon. I know of no reported series of normal children who toe-walked at preschool age and older. In my own experience, walking intermittently on the toes without an obvious organic cause does occur. It probably reflects a mannerism whose dynamics remain unexplained. The concern is that persistent toe-walking without an evident organic cause may lead to heel cord contractures. Also, I have noted that toe-walking is seen in children who have personality, behavior, and/or learning problems. Admittedly, this association may simply reflect the type of children referred to me and cannot be applicable to the general population.

I have speculated that if toe-walking in beginning walkers reflects the infant's compensatory attempt to throw his or her center of balance behind the hips so as not to fall forward when trying to establish walking balance, then, in those infants with subtle neuromotor balance problems, toe-walking may persist longer than usual. One might further speculate that, in spite of final maturation leading to better balance, toe-walking, which becomes unnecessary for balance, is continued as a "habit."
Aplastic Anemia in Children: Diagnosis and Management
Authors: Alter Blanche P.
Published: December 2021

The procedure used by the hematologist who is treating a patient with aplastic anemia is as follows: bone marrow examination to confirm aplastic anemia; thorough physical examination plus chromosomal studies as indicated to rule out Fanconi's anemia; and investigation into possible etiologic factors for the aplasia. If severe (usually acquired) aplasia is diagnosed, family members should be HLA-typed immediately. The patient should be observed for approximately 2 weeks during the work-up, at bed rest and with platelet and RBC transfusion support as needed (but not from family members, in order to avoid sensitization in case a marrow transplantation will be done). If a match is found, the transplantation should be performed as quickly as possible. If there is no match, blood product support from random as well as family donors may be increased. Antilymphocyte serum should be given, and an immediate or delayed response awaited. Androgen therapy should be reserved as a last resort because it is of questionable efficacy except in the treatment of Fanconi's anemia. Putative causal environmental factors should be eliminated, and genetic factors must be considered. considered. In fact, all "acquired" aplastic anemia may occur on the background of homozygosity or heterozygosity for genes predisposing to aplastic anemia.
Osteogenic Sarcoma
Authors: Tebbi Cameron K. , Freeman Arnold I.
Published: December 2021

Osteosarcoma is the commonest bone cancer seen in children and adolescents. Its peak incidence is during the adolescent growth spurt, and it occurs more frequently in taller individuals. The commonest site of occurrence is around the knee, ie, distal femur or proximal tibia. The classic presentation consists of pain and swelling at the tumor site. The tumor has a propensity to occur in the metaphyses of long bones, with destruction of normal bone as well as neoplastic new bone formation. Bone scans reveal the tumor.

The standard therapeutic approach has been amputation of the involved extremity with a resection line at least 10 cm above the most proximal extent of the tumor. Amputation alone has yielded a cure rate of approximately 20%. Recently, "limb salvage" (en bloc resection) with removal of the tumorous bone and replacement by a graft or endoprosthetic device has been used. This technique saves the limb but likely does not offer better function in the lower extremity and is associated with an increased risk of local recurrence in the "salvaged" limb.

In the last 10 years, use of adjuvant chemotherapy may have improved the cure rate to approximately 50%, but this result is still being debated. For patients who have a limb amputation, an immediate fitting for a temporary prosthesis is best, later followed by a permanent prosthesis. Psychologic support is extremely important for these youngsters as they not only face the fear of death from cancer, but also of mutilation from therapy.
Brachial Plexus Injury in the Newborn Infant
Authors: Molnar Gabriella E.
Published: December 2021

Brachial plexus palsy occurs at a frequency of approximately four cases in 1,000 births. The most obvious signs of trauma to the brachial plexus in the newborn are limp hypotonic arm, paucity or lack of movements, and abnormal posture of the affected limb.

ETIOLOGY

In most cases, there has been a complicated delivery with prolonged, difficult labor. The infant is often large. The mechanism of injury is traction upon the brachial plexus. Excessive stretching may occur in either breech on vertex presentation, as the head and neck are forced into lateral flexion to deliver the shoulders. The slightly higher incidence on the right side is attributed to the greater frequency of left occiput anterior presentation.

Experimental studies have shown that resistance to structural disruption by tensile forces differs in various segments of the brachial plexus. Neuronal elements surrounded by soft tissues are less vulnerable to injury than nerve roots; the latter are adjacent to bone as they exit through the foramina and course along the grooves of the transverse processes.

ANATOMY

Clinical signs relate to the site of the lesion. The distribution of weakness reflects the anatomy of the brachial plexus (Figure), whereas the abnormal postures of the affected extremity are caused by the imbalance and unopposed action of muscles that remained innervated.
Back Pain in Children
Authors: Bunnell William P.
Published: December 2021

Back pain in a young child or adolescent is an unusual complaint and must be regarded as a possible indication of the presence of significant pathology. Functional and mechanical causes of back pain in this age group are uncommon; therefore, it is most important that a vigorous attempt be made to establish the primary diagnosis. The purpose of this paper is to review the distinctive features of the history and physical examination that may lead to accurate diagnosis, and to outline a logical approach to the laboratory and radiographic evaluation for each of the major causes of back pain.

HISTORY

A carefully obtained medical history is frequently the most helpful factor in establishing a diagnosis. In addition to providing important information, it also establishes an attitude of mutual trust and understanding between the physician and patient. A skillfully taken history can help the physician gain vital insight into the patient's attitude toward his or her disease and suggest areas to be emphasized for further evaluation. Specific aspects of the history that are most revealing include: the mode of onset, nature and severity of the pain; a change in symptoms; and associated systemic complaints (Table 1).

The mode of onset should be determined as specifically as possible.
Medical Malpractice in 1984
Authors: BOSLEY WARREN
Published: December 2021

As Robertson points out in this issue of Pediatrics in Review, the number of allegations of medical malpractice and the amount of damages sought and awarded have increased continuously for nearly the past 10 years. The remedies for this problem, which were sought and, in large part, implemented in the middle 1970s, now appear to be severely threatoned. Many statutes have been found unconstitutional in whole or in part, and legislative repeal and modification have been successful in other instances. All of this adds to the general alarm felt among physicians and other providers of health and medical care in the United States.
Mixed Connective Tissue Disease in Childhood
Authors: Singsen Bernhard H.
Published: December 2021

Rheumatic diseases with overlapping clinical features have been known since at least 1900 under such descriptive designations as "sclerodermatomyositis," "lupoderma," "rupus," and others. These hybrid descriptors recognize the occasional mixing of features of the classic rheumatic disorders, rheumatoid arthritis, scleroderma, dermatomyositis, and systemic lupus erythematous (SLE), in the same patient. However, because of the absence of precise pathogenetic and etiologic information, even the traditional rheumatic diseases have been largely classified on the basis of aggregations of clinical, histologic, and immunologic findings.

In 1972, Sharp and colleagues first described 26 adults with a new syndrome, which they defined as mixed connective tissue disease (MCTD). A major contribution of Sharp and his colleagues was to name and attempt to classify an overlap syndrome in explicit clinical terms and to associate it with specific autoantibodies directed against "extractable nuclear antigen" (ENA). One year later, the first child with MCTD was reported, and in 1977 a series of 14 children with MCTD was first investigated.

Where does mixed connective tissue disease, in both children and adults, stand now after more than a decade of study? Directly, the concept of MCTD has focused our attention upon the description of overlapping features of the rheumatic diseases, and it has forced us to confront whether "lumping" or "splitting" descriptors serves the patient from a therapeutic or prognostic point of view.