bluuebunny/crossref_metadata_2025 huggingface dataset:
PRICE OF TWO WHALES
Authors: Student
Published: December 2021

Two million dollars for two whales. I couldn't help thinking about how much I could do with one-tenth of that. I could fully immunize 30,000 kids and treat 10,000 major diseases with $200,000.

I suppose one's priorities depend on one's perspective, and if you don't have children dying in front of you and don't know that they are dying like flies, then the fate of two poor little whales brought into your sitting room from a remote cold coast with all the drama and immediacy of television suddenly has enormous importance.
What's New in Pediatric Surgery—1988
Authors: Grosfeld Jay L.
Published: December 2021

During the past year, significant new information concerning basic science, molecular biology, imaging technology, and clinical medicine have led to a number of important advances in the field of pediatric surgery. In addition, for the first time, a segment of the Surgical Forum sessions at the annual meeting of the American College of Surgeons was devoted to pediatric surgical research projects. The following overview represents an update of the material presented at national and international pediatric surgical society meetings, recently published journal articles of importance, and some of the material presented at the 1988 meeting of the Surgical Section of the American Academy of Pediatrics.
Summary of Annual Meeting of the Section on Pediatric Urology
Authors: PARROTT THOMAS S.
Published: December 2021

The Section on Urology of the American Academy of Pediatrics met for two and a half days in conjunction with the 57th Annual Meeting of the Academy in San Francisco, Oct 15 to 17, 1988. Papers presented at this meeting thought to be of interest to the practicing pediatrician are summarized here according to topic.

HYDRONEPHROSIS: CLINICAL ASPECTS

Urinary obstructive lesions are increasingly being diagnosed prenatally. Prior to the routine use of prenatal ultrasonography, these conditions would not have been diagnosed, because often no abdominal mass is palpable in the newborn infant and urinary tract infection has not intervened. The current management of these infants has become a source of major controversy, because it is becoming increasingly obvious that not all dilated urinary systems are obstructive and some neonatal dilations improve spontaneously.
Chronic Idiopathic Intestinal Pseudoobstruction: A Commonly Misdiagnosed Disease in Infants and Children
Authors: GLASSMAN MARK , SPIVAK WILLIAM , MININBERG DAVID , MADARA JAMES
Published: December 2021

Intestinal pseudoobstruction is characterized by signs and symptoms of recurrent intestinal obstruction in the absence of an identifiable mechanical lesion. Underlying illnesses that have been associated with intestinal pseudoobstruction include scleroderma, amyloidosis, various endocrinopathies (eg, hypothyroidism), narcotic or laxative abuse, and electrolyte disturbances (eg, hypokalemia). Although the underlying cause of idiopathic intestinal pseudoobstruction is unknown, the primary pathophysiologic dysfunction in patients with idiopathic pseudoobstruction is a disorganization or lack of smooth muscle contractility that results in disordered peristalsis or aperistalsis involving bowel and/or urinary bladder.

The clinical manifestations of the motility disorder associated with chronic idiopathic intestinal pseudoobstruction include periodic vomiting, abdominal distension, constipation, urinary retention, and weight loss.
Infants and Children With Acquired Immunodeficiency Syndrome: Placement in Adoption and Foster Care
Authors:   TASK FORCE ON PEDIATIRC AIDS
Published: December 2021

The transmission of human immunodeficiency virus (HIV) from infected mothers to their infants has been well established. The majority of infants so infected are born to women who have acquired HIV through IV drug use or through sexual contact with IV drug-using partners. Some of these mothers are unable to care for their infants. In addition, many infected mothers become seriously ill or die, leaving children who must be cared for by others. Thus, many infants and children who are infected or are at high risk for infection may require placement in an adoptive or foster care setting. The HIV-infected infant or child places a serious burden on any family. This burden, when anticipated, may make adoption and foster care placement exceedingly difficult. However, such family-based care is clearly in the best interest of the child.

CARE FOR THE CHILD WITH ACQUIRED IMMUNODEFICIENCY SYNDROME (AIDS)

If an infant or child is known to be infected with HIV or is already ill with AIDS, what are some of the implications for the care givers, including adopting or foster cane families?

First, the family must bear the physical and emotional burden of caring for a child who will require ongoing medical treatment, will suffer from intermittent bouts of increasingly severe illness, and, in most cases, will ultimately die. A majority of these children can be expected to have developmental delay and many may also show behavioral regression, resulting in increased care-giving demands.

A second issue is the fear of spread of the viral infection to family members, friends, and classmates.
In Reply: Newborn Screening for Sickle Cell Disease
Authors: VICHINSKY ELLIOTT
Published: December 2021

We thank Dr Gross for his comments and the opportunity to clarify the points he has raised. Our study is based on a retrospective comparison of the clinical course of patients with sickle cell diagnosed at birth with that of patients whose disease was diagnosed during infancy as a result of clinical complications. We recognize the limitations of this retrospective comparison. However, a prospective study comparing these two groups would be impossible and perhaps unethical to perform.

Specifically, Dr Gross' concerns include (1) both groups should have equally benefited from comprehensive care once diagnosis was established; (2) there was a bias in favor of a lower mortality rate in the newborn screening group; (3) confusion concerning calculation of mortality rates.
In Reply: Brain Lesions and Extracorporeal Membrane Oxygenation
Authors: SCHUMACHER ROBERT E. , BARTLETT ROBERT H.
Published: December 2021

Dr Short is correct; the only way to determine the actual incidence and, significance of brain lesions in patients who have undergone extracorporeal membrane oxygenation (or any other group of neonatal patients) is to study the entire population. We previously reported our complete follow-up studies on 14 patients post-extracorporpeal membrane oxygenation older than 1 year of age, and we are in the process of updating complete follow-up studies.

The purpose of our recent paper was only to report that we have observed some cases of right-sided brain ischemia following extracorporeal membrane oxygenation, particularly in patients who were profoundly hypoxic and hypotensive at the time of cannulation.
Resident Stress = Spousal Stress
Authors: GERARDI GABRIELLA R.
Published: December 2021

Understandably, most of your letters are written by physicians, but I hope that you will print a letter from another profession. Both my husband and I were interested to read A. B. Bergman's article "Resident Stress" (Pediatrics 1988;82:260-263). I am a Registered Nurse working primarily with dying patients. Both my profession and my chosen field help me to relate to the arduous tasks and emotionally painful aspects of my husband's pediatric residency. I cannot help but recognize the need for ongoing mention, lectures, and support for those ethical and moral decisions that physician's must make.
In Reply: Pertussis Immunization
Authors: CHERRY JAMES D.
Published: December 2021

Dr Emerson suggested that the report ignored the distinction between infection and illness when we suggested that adult booster doses would reduce transmission of Bordetella pertussis in adults and could help in the eradication of pertussis in our population. In supporting his suggestion, Dr Emerson has underestimated the number of pertussis cases presently occurring in adults in the United States, has used an unimpressive reference to indicate that adult doses of tetanus and diphtheria toxoids are unnecessary, and has inappropriately questioned the herd immunity concept (the immunization of children at school entry and of adults) in the protection of young children from pertussis.
The Anencephalic Controversy
Authors: WYATT P. R.
Published: December 2021

The discussion in a recent issue of Pediatrics by Landwirth, Botkin, and Milunsky (August 1988) regarding harvesting organs for transplantation from anencephalic infants simply summarizes the recent and dramatic changes that have occurred in clinical medicine. We have reached the point in medicine where we must reevaluate our definitions of both life and death.

The significant changes in both clinical and diagnostic services available to physicians in the past decade, and future decades, will substantially change the practice of clinical medicine.
The Anencephalic Controversy
Authors: GOODWIN WILLARD E.
Published: December 2021

My special personal interest goes back to 1962 as I believe that we were the first to report an experience with using an anencephalic donor for enbloc kidney transplantation. It never occurred to us that there would be any moral or ethical problems associated with that. I wonder what the reaction will be when a neurosurgeon first reports the first brain transplantation in an anencephalic newborn.
The Anencephalic Controversy
Authors: KARRER FREDERICK M.
Published: December 2021

The rash of articles in the August issue of Pediatrks pertaining to the use of anencephalic newborns as organ donors was timely, albeit one-sided. The authors recognize that there is a critical organ donor shortage among newborns, ie, that 600 infants die annually of liver failure and approximately 1,200 die of heart disease. I plan to rectify this imbalance and take an exactly contrary point of view. First, it is well accepted that there is a critical shortage of "good" newborn donors.
Rape and AIDS
Authors: GELLERT GEORGE A. , MASCOLA LAURENE
Published: December 2021

In an otherwise thorough description of the procedures and tests required to evaluate the adolescent victim of rape, we find it disquieting that, in the recent statement by the American Academy of Pediatrics' Committee on Adolescence, no mention was made of the need to consider baseline and subsequent testing for the presence of antibody to human immunodeficiency virus (HIV). Although there are no data at present that demonstrate that HIV is commonly transmitted through rape, it would seem appropriate for clinicians involved in the assessment of sexual assault to include HIV antibody testing as an option in their management protocol.
Hemorrhage, Phenobarbital, and Fluctuating Cerebral Blood Flow
Authors: PERLMAN JEFFREY M. , VOLPE JOSEPH J. , ERNEST A. , STEIN JANE G.
Published: December 2021

In the recent article regarding fluctuating cerebral blood flow velocity and the intraventricular hemorrhage in the neonate, the authors concluded "when the best 20 waves are chosen to evaluate the coefficient of variation, no association exists between the coefficient of variation values and development of subependymal-intraventricular hemorrhage...." Such a conclusion is clearly not justified based on the data presented.

First, the mean mode as was used in this study is an averaged frequency for a single cycle.
Macrosomia, Microphthalmia, and Early Rapid or Sudden Infant Death: A New Syndrome?
Authors: TEEBI AHMAD S. , FARAG TALAAT I.
Published: December 2021

Macrosomia together with macroglossia and omphalocele permits the recognition of Beckwith-Wiedemann syndrome which is frequently observed in sporadic occurrence and occasionally as an autosomal dominant disorder. However, macrosomia may be also a part of several clinically recognizable overgrowth syndromes such as cerebral gigantism, Smith-Marshall syndrome, and Weaver syndrome.

Recently, we observed a female infant with macrosomia (birth weight 4,650 g, length 52 cm), bilateral severe microphthalmia, frequent cyanotic spells with apnea, and an early death at 43 days due to overwhelming illness.
Sports and Sickle Cell Trait
Authors: O'KEEFE Jo
Published: December 2021

Purely out of curiosity, I am writing to ask a question regarding recommendations (Pediatrics 1988;81:737) for participation in competitive sports. Why was sickle cell trait included? I know that the possibility of increased problems among individuals with the trait has been mentioned in recent literature; however, to me it seems that inclusion of sickle cell disease or anemia would have been more useful.
In Reply: Fictitious Crystalluria
Authors: RIMSZA MARY ELLEN
Published: December 2021

The medical diagnostic problems created by Ultra-Pampers gel matrix continues. Since the publication of our letter to the editor concerning "vaginal beads" (pediatrics 1988;81:332) due to Ultra-Pampers' gel matrix, many physicians have called on written to me about similar diagnostic dilemmas. In most cases, the parents could not recall using torn diapers. Although this gel material may not be harmful to the child, the subsequent medical evaluation to determine the cause of these "beads" or "fictious crystals" can be painful to the child and expensive for the parents.
HOW VALUABLE IS THE S.A.T.?
Authors: L. J. F.
Published: December 2021

Most societies have some sort of rite of passage from childhood to adulthood. The aborigine in Australia send adolescents into the wilderness on a "walkabout." German noblemen prove their maturity by acquiring dueling scars.

Americans have one in which their young people spend three high-pressured hours filling in little boxes. Anthropologists call it "Taking the Scholastic Aptitude Test."

What do they measure? To anyone with lingering faith in Santa Claus, the Easter Bunny and truth-in-packaging, the answer is obvious: The Scholastic Aptitude Test measures scholastic aptitude. For that answer score zero.

The test was created in 1926, when people still believed that there was something called intelligence that experts could measure and put a number on. Scholastic aptitude was its academic counterpart.

Today no one believes that intelligence is that simple, and developers of the aptitude test have backed away from claiming to measure aptitude.

What does the test tell colleges? The College Board says the examination predicts college grades in the freshman year. The technical term is predictive validity, which is measured on a scale of zero to one. Zero means that the result would be as random as using a zip code. A perfect 1.0 means that everyone with high test scores will get high grades, everyone with low scores will get low grades and so forth.

Testmakers' research shows that college freshmen grades can be predicted with a validity of 0.42 based on the aptitude test, 0.48 based on high school grades and 0.55 if both are used. Since colleges already have students' grades, the incremental advantage of having the test scores is 0.07.

Do test scores change admissions decisions? In a new book, "The Case Against the S.A.T.," James Crouse and Dale Trushei.m of the University of Delaware argue that the test is "valid but redundant." They conducted their own studies and concluded that by using high school grades alone, they could come to the same conclusion as admissions directors in all but 3 percent of cases-not enough, in their opinion, to justify the whole enterprise.
UNEXPECTED INFANT DEATH AND ADVERSE SOCIAL STATUS
Authors: Student
Published: December 2021

Families [in which unexpected infant death occurred during a minor illness] showed the most striking rise [in Sheffield] between 1980 and 1987 [and] had twice as many adverse social factors as controls. When we reviewed [these] deaths we found that many of the families in this group seemed to belong to a small subculture within which morbidity, poverty, excess smoking and alcohol consumption, and a fatalistic outlook predominate....

Many unexpected deaths in infants are multifactorial in origin; possibilities for prevention have social, political, and educational as well as medical implications.
Rapid Sequential Phenobarbital Treatment of Neonatal Seizures
Authors: Gilman Jamie T. , Gal Peter , Duchowny Michael S. , Weaver Richard L. , Ransom J. Lawrence
Published: December 2021

The optimal serum concentration of phenobarbital in newborns and its safety at high doses are not well established. The dose response relationship of rapid sequential phenobarbital loading in the newborn was examined and the efficacy of high-dose monotherapy was compared with the addition of a second anticonvulsant for persistent seizure activity. A single loading dose of phenobarbital 15 to 20 mg/kg was initially administered to 120 newborns. Nonresponders received sequential bolus doses of 5 to 10 mg/kg until seizures ceased or a serum concentration of 40µg/mL was obtained. Infants with refractory seizures received additional phenobarbital to a maximum serum concentration of 100 µg/mL. The seizures of 48 babies (40%) were controlled after initial loading and 37 of the remaining 72 subjects (51%) responded at serum concentrations of as great as 40 µg/ mL. The seizures of only seven subjects were controlled at greater concentrations. A second anticonvulsant controlled seizures in 13 of the 28 subjects (46%) whose seizures were refractory to phenobarbital. A gestational age of less than 32 weeks was associated with a significantly better response to phenobarbital. Serum phenobarbital concentrations greater than 50 µg/mL produced only occasional feeding difficulty and sedation. It was concluded that sequentially administered IV phenobarbital controls seizures in both term and preterm newborns (77%). This therapeutic effect is dose dependent but plateaus at a serum concentration of 40 µg/mL. At greater serum concentrations, unresponsive patients should receive a second antiepileptic agent.
Adolescent Pregnancy Prevention by Health Education Computer Games: Computer-Assisted Instruction of Knowledge and Attitudes
Authors: Paperny David M. , Starn Jane R.
Published: December 2021

Adolescents enjoy animated-action computer games that capture and hold attention. In this study, the use of computer-assisted instruction in improving knowledge and attitudes in favor of decreased teenage pregnancy is evaluated. Computer-assisted health education is an interactive process and simulates reality. Teenagers explore alternatives and outcomes, and experience consequences of behavioral choices. Two games were designed to promote understanding of the impact and cost of sexual behaviors and parenthood, as well as provide sexual survival skills. Educational evaluation in high schools showed improvements in knowledge and attitudes in these sensitive areas. Test instruments based on the content of each game were developed to assess the impact of the two interventions. Half of the 718 teenagers used the educational programs, and half served as matched control subjects who did not use computer games. Players rated the games highly and evaluated them positively. Measures of 19 factual and psychologic factors associated with pregnancy and parenting were separately made for control and experimental groups. Teenagers who played the games gained knowledge and positive attitude changes to a significantly greater extent than did the comparison group. "The Baby Game!" users increased their knowledge of costs of birth and of costs in money and time of child rearing. "Romance!" users would seek more professional help, had a better understanding of risk of pregnancy, learned more effective means of contraception, and had greater acceptance of oral contraceptives. Teachers and teenagers found the computer games acceptable and fun, and the games reduced counseling time in physician's offices. Computer-assisted instruction is a useful means of providing health education and a promising intervention for adolescent pregnancy.
Antenatal Transfers and Bronchopulmonary Dysplasia Incidence
Authors: SEPKOWITZ SAMUEL
Published: December 2021

Hobar et al attempted to compare weight-specific neonatal mortality rates among 11 neonatal intensive care units. All patients were inborn. Finding a statistically significant variability of outcome among these neonatal intensive care units, after the effects of birth weight, gender, and race were considered and accounted for, the authors wondered whether other population variables might explain the differences in outcome.

One source of variability that would alter population risk among the neonatal intensive care units is patient selection by antenatal transfers into a referral center for delivery.
In Reply: Antenatal Transfers and Bronchopulmonary Dysplasia Incidence
Authors: HORBAR JEFFREY D.
Published: December 2021

Dr Sepkowitz suggests that differences in patterns of antenatal transfers may account for center variations in neonatal outcome. He raises the question in particular with respect to Dartmouth and Northside Hospital, two centers included in our study. The outcomes at these centers do not support his argument. The two centers had identical predicted outcomes for both survival and survival without supplemental O2 after adjusting for the effects of birth weight, gender, and race.

Antenatal transfer is only one of many factors that may lead to population differences at neonatal intensive care centers.
Neonatal Testing for Sickle Cell Diseases—A Historical and Personal Review
Authors: Pearson Howard A.
Published: December 2021

Testing for sickle cell hemoglobinopathies in the newborn period has gone through several distinct eras that reflect the diagnostic tests available during each period, as well as a changing clinical perception of the necessity and value of such screening for what is essentially an incurable disease (Table 1). From 1930 to 1950, pioneer investigators such as Diggs et al and Scott et al performed classical sickle cell "preps" in groups of black newborns. In the 1960s, investigators, notably Schneider et al and Gilman et al performed hemoglobin electrophoresis for large groups of newborns. However, these were research programs that had little or no clinical coordination or follow-up. In the 1970s, Serjeant et al in Jamaica and myself in New Haven, CT, set up programs of neonatal diagnosis coupled with careful long-term comprehensive follow-up. We are now in an era when neonatal screening for hemoglobinopathies has been recommended or mandated by a number of states.

The diagnosis of sickle cell disorders in the neonatal period is complicated because of the presence of large amounts of fetal hemoglobin (Hb F) at birth. Normal adult hemoglobin (Hb A) has two pairs of chemically dissimilar polypeptide chains designated α and β and can be represented as α2β2. Hb S has a pair of chemically altered chains and is designated α2β2s. Fetal hemoglobin, which constitutes 70% to 80% of the hemoglobin of the fetus and newborn, contains γ- instead of β-chains and can be represented as α2α2. γ-Chains are not affected by the sickle cell mutation.
Value of Screening Umbilical Cord Blood for Hemoglobinopathy
Authors: Lobel Jeffrey S. , Cameron Bruce F. , Johnson Earslean , Smith Dianne , Kalinyak Karen
Published: December 2021

Foremost among the beneficial effects of screening umbilical cord blood is the optimized quality of care that can follow the immediate involvement of an infant with sickle cell disease and his or her family in an appropriate health care system. This is exemplified by the reduction in the case fatality rate of pneumococcal septicemia that has been achieved. Appropriate follow-up of screening also includes transmission of information about the diagnosis of a hemoglobinopathy trait or α-thalassemia to affected families and their physicians, with ready availability of education and counseling.
Diagnosis at Birth Improves Survival of Children With Sickle Cell Anemia
Authors: Powars Darleen
Published: December 2021

The increased survival of children who have sickle cell disease is primarily due to state-of-the-art improvements in general pediatric medical care with particular emphasis on the management of the infectious complications that occur. Studies reported from New York City, Los Angeles, New Haven, and Jamaica clearly demonstrate the calendar era change in survival that has occurred during the 1970s and 1980s, and the greatest improvement is found among those children who have sickle cell anemia. The accurate identification of the specific hemoglobinopathy at or near birth provided the foundation for these studies documenting infant and young child mortality.

In Africa, Molineaux et al and Fleming et al reported an epidemiologic investigation subsequent to a cord blood diagnosis program initiated in Garke, Nigeria, in 1976. A total of 534 infants were screened for major hemoglobinopathies, and 11 babies with SS and 125 babies with AS were identified. Minimal medical care was available for follow-up of the children. On entry to the school program at 5 years of age, the same population from the same small rural town was restudied. Only one child of 439 was found to have sickle cell anemia but 133 were AS. The inescapable conclusion was that the African babies with SS had died during early childhood, contributing disproportionate numbers to the high infant and childhood mortality in Nigeria. Not until Dr Fleming and the investigators of the British Medical Research Council had performed a cord blood hemoglobinopathy surveillance program was the incidence of sickle cell anemia and its effect on childhood mortality in Nigeria documented.
Usefulness of Cation Exchange High Performance Liquid Chromatography as a Testing Procedure
Authors: Huisman Titus H. J.
Published: December 2021

Testing of cord blood or newborn blood samples for hemoglobin abnormalities should include clinically important hemoglobinopathies other than sickle cell anemia (SS), such as SC, SD, SO, S-β- thalassemia (thal), EE, SE, and α-thal, and should place the quality of the testing procedures (ie, accuracy of diagnosis) above quantity (ie, number of samples tested over a given period). There is no single method available that is suitable for the identification of each of the numerous abnormalities; thus, at least two, and often more than two, procedures must be used to reach a definitive diagnosis. For this reason, blood samples collected in vacutainers with ethylenediaminetetraacetic acid as anticoagulant are preferred to those collected on filter papers. The latter approach also has the disadvantage that, under a less than optimal transport system, hemoglobin is readily modified (oxidation, glycosylation, protein-protein interaction), producting extra bands or peaks in electrophoretic or chromatographic separations that interfere with an appropriate identification of various genetically determined hemoglobin variants.

In our laboratories, in which hemoglobin identification has been routine for more than 25 years, we consider the following procedures acceptable primary testing methods: starch gel electrophoresis at pH 8.9, cellulose acetate electrophoresis at pH 8.5 to 8.9, isoelectric focusing, and fast cation exchange high performance liquid chromatography (HPLC). The following five methods are excellent confirmatory testing procedures: citrate agar electrophoresis at pH 6.1, cation or anion exchange macrochromatography, isoelectric focusing, cation exchange HPLC, and immunologic procedures.

Combinations of these techniques will often lead to acceptable data, and the general approach followed in our institute is given in Fig 1. Cellulose acetate electrophoresis at alkaline pH is still the primary testing procedure, and citrate agar electrophoresis at pH 6.1 and micro-HPLC procedures are the main confirmatory methods.
Florida's Experience With Newborn Screening
Authors: Mack Astrid K.
Published: December 2021

Cord blood screening of newborns for sickle cell disease was initiated in Florida at the Miami Sickle Cell Clinical Center in 1979 as a part of its participation in the national Cooperative Study of Sickle Cell Disease. Funding for newborn sickle cell screening in Florida began as a pilot project with a line-item appropriation in fiscal years 1983/84, and this funding for the three medical schools (University of Florida, University of Miami, and University of South Florida) continues to the present. During fiscal year 1984/85, a contract was negotiated between the University Hospital at Jacksonville and the local Children's Medical Services' office for the provision of newborn sickle cell screening. This contract has been renewed each year to the present.

The primary objective in each of these programs is to identify babies with clinically significant hemoglobinopathies and to offer the services of the institution's pediatric hematology division. Additionally, education, genetic counseling, family studies, and appropriate referrals are provided for all babies found to have any hemoglobinopathy and for their family members.

Initially, at the University of Miami, cord blood specimens were obtained at the time of delivery from at-risk (mostly black) mothers from whom informed consent had been obtained during a visit to one of several prenatal clinics associated with the University of Miami/Jackson Memorial (UM/ JM) Medical Center. It was soon discovered that many at-risk expectant mothers did not attend a prenatal clinic before their babies were born. Our internal review board did not consider consent "at delivery" informed consent.
Follow-up of Children With Trait in an Urban Setting
Authors: Grossman Lindsey K. , Holtzman Neil A. , Charney Evan , Schwartz Allen D.
Published: December 2021

In a newborn screening program for abnormal hemoglobins, 91 infants were found to have hemoglobin AS or AC. Their parents were informed and offered genetic counseling, but only 350 accepted. We tested parents' knowledge of sickle disorders before and after the session. The tests were repeated when their babies were 4 to 8 months of age; parents of babies with normal hemoglobin and those with sickle trait who had not been counseled were also tested at this time. Parents who received counseling showed an increase in knowledge and retained it until the second interview, although 27% did not recall the original session. Noncounseled parents of a child with trait also had knowledge of the condition; in general, families of a child with trait knew more about the condition than families of a child without trait. Newborn hemoglobin screening programs prove helpful for certain families but not necessarily for all who may be eligible. (Previously published in Am J Dis Child 1985;139:241-244.)
Legal and Ethical Issues in Newborn Screening
Authors: Bowman James E.
Published: December 2021

The development of techniques for newborn screening and for prenatal diagnosis of sickle hemoglobin and other hemoglobinopathies will have a profound effect on public health policy to a greater extent than did mass population testing that was initiated in the early 1970s. The subject of this conference is newborn screening; however, there is an old aphorism: "One cannot do one thing." Accordingly, when newborn screening for hemoglobinopathies is instituted on a large scale, the public policy consequences will extend far beyond newborn screening. We cannot anticipate all of the effects of such a policy, but some aftermaths may be predicted with near certainty. To understand the possible outcomes of a large-scale newborn screening program for sickle cell disease, some prior court and legislative decisions are pertinent.

Legislation, court decisions, state and federal genetics programs, and scholars in the social sciences, humanities, law, medicine, and genetics support discoveries in genetics that now facilitate genetic testing, prenatal diagnosis and selective abortion of fetuses with genetic and other disorders, artificial insemination, and in vitro fertilization. Arguments for and against these procedures are reminiscent of debates about the use of the limited tools of the old eugenics to prevent the birth of children who were considered to be physically, mentally, or socially defective.

Some examples of these court and legislative decisions follow: the Supreme Court verdict in Munn v Illinois established that the public interest supercedes the private interests of individuals; Buck v Bell, another Supreme Court ruling, upheld the constitutionality of mandatory sterilization.
Perspective From the National Association for Sickle Cell Disease
Authors: Whitten Charles F.
Published: December 2021

It has been documented that infants with sickle cell disease are at risk of death in early infancy and that with appropriate services this risk can be minimized. Therefore, it should be a societal goal to give every infant with sickle cell disease in this country an opportunity to avoid a preventable death associated with the disease. I wish to present what, in my judgment, is required to achieve that goal and a brief rationale for each provision. These judgments are based upon 18 years of experience in designing and administering sickle cell service programs and 13 years of serving as president of a national sickle cell organization.

Every state must have a program designed to achieve the goal (currently only 13 states do) and the program must be underwritten by the state. Traditionally, the provision of personal health services has been considered a governmental responsibility when the following three conditions are met: the lack of services has dire consequences, effective services can be provided, and the solution requires a population approach. Sickle cell disease in infancy meets these criteria. The Government's responsibility is to provide or require the service and to provide or ensure financial support.

Each state's plan must require by state law that all at-risk newborns are tested. The various voluntary programs have been initiated, implemented, and nurtured by committed experts in large communities and their experiences cannot be generalized to small communities where devoted and skilled sickle cell leaders are not present. It should be optional for the various states to decide whether the law requires all newborns to be tested.
PUBLIC PRESENTATIONS
Authors: Henry Deborah D.
Published: December 2021

I am the parent of an 11½-year-old daughter with sickle cell disease. I am aware of the need for a comprehensive newborn screening program for sickle cell disease and other hemoglobinopathies. However, all such programs must be instituted with a follow-up component, and parents should be made aware that such screenings are being done.

My daughter was born during the summer of 1975 in New York City. New York City began screening for sickle cell and similar hemoglobinopathies in May 1975, but had no comprehensive follow-up program until 1978. My daughter was not screened nor was I aware of the screening program. I learned of my daughter's condition during a routine well-child clinic visit when she was 6 months of age. I am afraid to think of her outcome had I not been taking her for preventive health care, because before the age of 1 year she experienced one of the most life-threatening crises of a child with sickle cell disease—splenic sequestration.

I am pleased to announce that in New York City today, parents are notified in a timely manner of their infant's newborn screening results with information regarding follow-up and counseling services. Two of my immediate family members gave birth to infants with sickle cell trait. They were informed of their infants' results within 2 weeks after their babies' births, and were given concrete information and recommendations for follow-up genetic services. I know a comprehensive newborn screening program will prevent mortality in infants found to have sickle cell disease and related hemoglobinopathies.
Perspective From the National Medical Association
Authors: Epps Roselyn Payne
Published: December 2021

I am pleased to comment as a member of the National Medical Association, the professional organization founded in 1895, which currently represents the 14,000 black physicians practicing in the United States. Members of the National Medical Association (many of whom have been inspired by the leadership of Dr Roland B. Scott) have been and continue to be in the forefront of sickle cell disease research. Their sustained interest and that of many other colleagues has contributed to the research accomplishments we observe today. On the other hand, some physicians have been reluctant to assume a prominent role in sickle cell screening, counseling, and follow-up because of their own uncertainties and legitimate differences of opinion. During this conference, experienced and knowledgeable speakers have eloquently presented varying and sometimes divergent points of view, reflecting existing practices and highlighting the need for consensus development.

As the consensus panel approaches its deliberations, I would like to emphasize that the health of the child and family must be paramount. I define health in its broad sense as the physical, mental, and social well-being of children and families. Results of the collaborative oral penicillin study, presented by Dr Gaston, leave no doubt that identification of sickle cell disease during infancy and prevention of its complications are imperative. Also, many speakers from successful programs spoke of the extensive time, effort, and resources required to screen, follow up, counsel, and treat patients and to coordinate services. I request that the panel carefully consider the advisability and feasibility of a public health education program, patterned after the highly successful high blood pressure education program launched by the National Heart, Lung, and Blood Institute, which included every resource of the country.
Public Health Perspective on Newborn Screening for Hemoglobinopathies
Authors: Walker Bailus
Published: December 2021

The information presented during this conference has more than adequately demonstrated that the early identification of newborns with sickle cell disease, the administration of comprehensive care to newborns, including penicillin prophylaxis, and public education about the disease can greatly reduce infant and childhood morbidity and mortality.

Screening for the hemoglobinopathies has been a controversial topic for two decades. Many states, including Massachusetts, responded to the political pressures of the civil rights movement of the 1960s and the identification of sickle cell anemia as the "neglected disease" by hastily passing sickle cell testing legislation, which, unfortunately, resulted in the establishment of many poorly planned sickle cell screening programs.

This situation was confirmed by the 1983 President's Commission Report on Screening and Counseling for Genetic Conditions, in which serious problems with many of the existing sickle cell disease and carrier screening programs were pointed out, including unclear program objectives, poorly chosen target populations, lack of confidentiality of results leading to discrimination and stigmatization, and inadequate incorporation of genetic counseling and public education into the programs.

Although we now know that the appropriate time to screen is the newborn period, and we have available an effective treatment for the complications of sickle cell disease, concerns still remain.

From a state health department perspective, sickle cell screening programs that include all babies born (universal) are more appropriate than those that target babies of certain ethnic backgrounds. Because it is often difficult to determine ethnic background, especially in the newborn period, targeted screening would inevitably mean missing some babies at risk and affected babies.
Perspective From the American Academy of Pediatrics
Authors: Holtzman Neil A.
Published: December 2021

Ten years ago, the initial report of the Committee on Genetics of the American Academy of Pediatrics was published, drawing heavily on the landmark report published 2 years earlier by the National Academy of Sciences. That report stated that new mass screening tests should not be implemented without pilot studies or facilities for follow-up. I would like to deal with the efficacy of screening as determined by pilot studies, the effectiveness of routine screening, and the importance of follow-up.

The Cooperative Study of Sickle Cell Disease was essentially the first phase of a large pilot program that systematically demonstrated that sepsis, meningitis, and acute splenic sequestration occurred in young infants with sickle cell disease. Previous reports suggested, and the cooperative study corroborated, that without prior diagnosis many of these infants would die. The randomized trial of oral penicillin prophylaxis was the second phase of the pilot program, demonstrating that early treatment significantly reduced mortality. Thus, the results of this pilot program, conducted with support of the National Institutes of Health, documented that newborn screening for sickle cell disease satisfied a major criterion for screening: An intervention capable of reducing mortality would be efficacious if applied before the usual age of clinical diagnosis.

The pilot program was conducted under ideal conditions. The findings are not enough to conclude that screening for sickle cell disease and other hemoglobinopathies will always be effective when performed routinely.
HAS ANYTHING CHANGED?
Authors: Student
Published: December 2021

American and British styles of [biomedical] science were markedly different [in the 1970s]. British research grants were smaller, though longer term, and ideas there tended to be nurtured over the years like fine wine . . . There was nothing cozy about the Americans; even the best of scientific friends in the States might become fiercely competitive antagonists. It was part of the way of life; while plentiful, grants were short-term—usually two years. Researchers were therefore compelled to produce results or see their money whisked away. Publish or perish. No tolerance of failure, no safety net. Big science, big money.
DIAPER AND SKIN FOLD RASHES AND THE HAIR DRIER
Authors: Austin Glenn
Published: December 2021

Wet macerated skin in the diaper area and in the skin folds under the chin, in the axilla, groin and between the buttocks predisposes to rashes. Most yeast, fungi and bacteria grow better on macerated skin than on dry skin. While frequent changes of disposable diapers with or without moisture absorbing chemicals seems to have reduced such rashes, the problem still exists. It often seems more prevalent among babies in crowded day care situations with a low ratio of caretakers to babies needing changing.

Over the last ten years I have found that the use of a hair drier to intermittently completely dry the skin in these areas leads to healthier skin and fewer rashes. This technique is especially helpful for plump babies where the skin is usually moist in the creases of the neck, groin and axilla and between the buttocks. Once the skin has been completely dried a light application of powder in the creases tends to keep the skin dry longer and less irritated. Bun warmers work.
COCAINE KILLS INFANTS
Authors: Student
Published: December 2021

The recent increase in the infant mortality rate in New York City, after more than a decade of decline, is in significant part due to cocaine abuse.
Anatomy of Femoral Vessels in Infants and Guidelines for Venous Catheterization
Authors: Kanter Robert K. , Gorton Jan M. , Palmieri Kathy , Tompkins Joy M. , Smith Frank
Published: December 2021

This study was performed to formulate femoral venous catheterization guidelines for infants from ultrasound observations. Femoral vessels were evaluated on both sides in 75 infants, 2 weeks to 24 months of age. The site of the greatest probability of successful venipuncture 1 cm below the skin crease at the groin is located 4 to 5 mm medial to the femoral artery pulse. If it is assumed that entry into the central half of the vein will result in successful catheterization, successive attempts 5 mm and 6 mm medial to the pulse would result in cumulative successful insertion in 53% and 61%, respectively, with no arterial punctures. A third attempt 4 mm medial to the pulse further increases cumulative success to 78%, but 3% arterial punctures would occur. These guidelines are intended to facilitate achievement of femoral venous catheterization, minimizing arterial punctures and number of needle passes required.
Macrosomia in Infants of Insulin-Dependent Diabetic Mothers
Authors: Berk Michael A. , Mimouni Francis , Miodovnik Menachem , Hertzberg Vicki , Valuck Jennifer
Published: December 2021

The purpose of the present study was to evaluate factors affecting the rate of macrosomia and related complications in a population of infants of insulin-dependent diabetic mothers. The following factors were hypothesized to be predisposing to macrosomia: increased maternal weight gain during gestation, increased number of births until infant No. 3, white race, increased maternal age, poor glycemic control from the 20th week of gestation, and increased insulin dose. Advance White classification and increased duration of diabetes were predicted to be inversely related. In addition, macrosomia was hypothesized to predispose to selected adverse perinatal outcomes including premature labor, birth asphyxia, birth injury, hypoglycemia, polycythemia, and respiratory distress syndrome. From 1978 to 1986, 127 pregnancies were prospectively studied, 86 of the total number of women were entered prior to 10 weeks' gestation, and 41 were entered after 10 weeks' gestation. Patients monitored blood glucose at least twice daily with glycemic control achieved by "split-dosage" regimens of insulin. Glycohemoglobin was measured monthly. Pregnancy dating was based on the date of the last menstrual period and the Ballard score of the infant at birth. Macrosomia was defined as a birth weight greater than the 90th percentile of the intrauterine growth curves of Lubchenco. Of the babies born to mothers with insulin-dependent diabetes, 43% were large for gestational age and 57% were appropriate for gestational age. Maternal factors predisposing to an infant being large for gestational age included glycohemoglobin measurement at the time of delivery (large for gestational age = 8.4% ± 0.3%, appropriate for gestational age = 7.6% ± 0.2%, P < .05, normal = 5.5% to 8.5%), reflecting poorer glycemic control during the third trimester, weight gain in the third trimester, and advanced White classification by univariate analysis compared to mothers of babies with birth weights appropriate for gestational age. However, only glycohemoglobin at the time of delivery was significant when these variables were subjected to multiple logistical regression. Macrosomic infants had higher rates of both polycythemia (large for gestational age = 23.6%, appropriate for gestational age = 6.9%, P < .008) and hyperbilirubinemia (large for gestational age = 29.6%, appropriate for gestational age = 12.7%, P < .02) than nonmacrosomic infants but did not differ in other perinatal outcomes. The data suggest that, in spite of improvements in glycemic control in the recent past, macrosomia still exists at an increased rate in infants of diabetic mothers and is significantly related to poorer glycemic control in the third trimester. In addition, large for gestational age infants are at an increased risk for both polycythemia and hyperbilirubinemia.
1988 George Armstrong Award Lecture, Ambulatory Pediatric Association: Prophets, Disciples, and Honorable Schoolboys
Authors: Pless Ivan B.
Published: December 2021

Few who do research have any illusions that they alone, acting in isolation, are likely to accomplish much that is truly significant. We all build on the work of our prophets and our colleagues. Whatever contributions we may make will, however, almost certainly, pale by comparison with what we can expect our disciples to contribute. From the best and the brightest, those who are fortunate enough to be in the right place at the right time, we truly expect great things. But we can hope as well that all will discover some of the magic ingredients for research training and have the opportunity to transfer these to their successors.