bluuebunny/crossref_metadata_2025_split huggingface dataset:
Introduction to Urea Cycle Symposium
Authors: Koch Richard 
Published: December 2021

When Donough O'Brien reviewed the field of metabolic diseases in 1965, he discussed disorders implicating carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), argininosuccinic acid (ASA) synthetase, and argininosuccinic acid lyase. Arginase defects had not yet been encountered. Several cases of argininosuccinic aciduria had been reported, but only single cases of CPS, OTC, and ASA synthetase defects had been described. The succeeding 15 years have seen much progress in this field, much of it reflecting the efforts of individuals participating in this program.
My first contact with disorders of the urea cycle was in 1976 when a suspected OTC deficiency in a female infant was identified by Dr Kenneth Shaw, for a colleague of ours at the University of Southern California Medical Center. I was asked to help with the care of that infant, who died many months later of meningitis. Largely due to Ken's indefatigable efforts in screening high-risk infants for metabolic diseases, we have seen various urea cycle defects identified in several other infants. However, we have sometimes encountered the unexpected in attempting to confirm the clinical diagnosis by enzyme analysis. The following comments on two patients exemplify the situation.
Patient C.S. was in a coma on admission; his blood ammonia level was 895 µg/100 ml. Urinary orotic acid levels were elevated and our clinical impression was that the patient had a variant of an OTC defect. Analysis of the liver biopsy specimen revealed 35% CPS, 42% OTC, 24% ASA synthetase, 100% ASA lyase, and 31% arginase.
Patient N.G. was born in a nearby medical center and shortly thereafter became lethargic, vomited, and lapsed into a coma.
POLIOMYELITIS OCCURRING AFTER ANTIGEN INJECTIONS
Authors: ANDERSON GAYLORD W. , SKAAR AUDREY E. 
Published: December 2021

In poliomyelitis patients who have received some antigen during the month prior to onset there is a high degree of correlation between site of paralysis and site of injection. Such cases tend to show a different distribution of paralysis and a more severe paralysis than do comparable children immunized 2 to 6 months previously, immunized in previous years or never immunized.
The time interval between date of injection and onset of illness suggests some relationship other than chance. There is no evidence that this relationship is associated with any one antigen. There is no evidence that the influence of the antigen persists, or in any way conditions the response to poliomyelitis virus for longer than one month.
Since immunization is an elective procedure, it can well be delayed during outbreaks of poliomyelitis; but there is no suggestion that the postponement should be more than temporary.
Plasma Threonine in Premature Infants
Authors: RÄIHÄ NIELS , RASSIN DAVID , GAULL GERALD 
Published: December 2021

In Reply.—
The major point raised by the letter of Rigo and Senterre is that the plasma threonine concentration should decrease with increasing gestational age in the groups with high threonine intake (&gt;1,500 µmol/kg/day). These are the infants receiving a milk formula containing 3.0 g/100 mL of protein in one of our first studies.1 In our recent study on term infants2 the threonine intake in the formula-fed infants was &lt;1,500 µmol/kg/day. Plasma threonine values in relation to gestational age from the first study are shown in the Table.
Fatal Intracranial Hemorrhage in a Normal Infant Secondary to Vitamin K Deficiency
Authors: LANE PETER A. , HATHAWAY WILLIAM E. , GITHENS JOHN H. , KRUGMAN RICHARD D. , ROSENBERG DONNA A. 
Published: December 2021

Since the initiation of routine vitamin K prophylaxis in newborns, the incidence of hemorrhagic disease of the newborn has been dramatically decreased. Recently there have been suggestions in the literature that prophylaxis may be unnecessary.1-4 We report here a fatal case of vitamin K deficiency in an otherwise healthy 1-month-old who did not receive prophylaxis. This case is illustrative because the child was initially thought to have suffered nonaccidental trauma. In addition, the correct diagnosis was confirmed, retrospectively, after vitamin K administration, with new assays for vitamin K-deficient prothrombin.
CASE REPORT
This 4-week-old male infant was seen at a community hospital emergency room with a one-day history of irritability, poor feeding, and decreased responsiveness without fever.
Maternal-Infant Bonding: A Joint Rebuttal
Authors: ANISFELD ELIZABETH , CURRY MARY ANN , HALES DEBORAH J. , KENNELL JOHN H. , KLAUS MARSHALL H. , LIPPER EVELYN , O'CONNOR SUSAN , SIEGEL EARL , SOSA ROBERT 
Published: December 2021

Inaccuracies in Lamb's recent review1 of studies of maternal-infant bonding have resulted in considerable confusion and misunderstanding among those not well acquainted with the field. This rebuttal, prepared by the investigators whose works were sited, addresses these inaccuracies so that a fuller and more productive discussion of this area of study can occur in the future. Under the name of each investigator, Lamb's criticisms are quoted, after which the original researcher responds with corrected information.
Lamb in his criticism of the work of Hales et al2 notes that, "Many of the researchers have employed multiple measures and observed statistically significant differences on a small proportion of these.
Worms in Diaper
Authors: WEINBERG ARTHUR G. 
Published: December 2021

To the Editor.—
In a recent letter,1 Ruttner called attention to the presence of housefly maggots in soiled diapers as a cause for parental misidentification of stool parasites. Recently, a mother brought a clean diaper with "worms" to our acute care clinic. The diaper contained more than 20 slender brown "worms," each 1.5 mm long, which moved in an undulating manner (Figs 1 and 2). Closer examination disclosed tiny caterpillars. Tim Friedlander, an entomologist at Texas A&M University, identified these as first-stage larvae of a pyralid moth, probably of the subfamily Pyraustidae, a harmless garden pest.
Efficacy of Penicillin Prophylaxis
Authors: HESS J. , HOLLOWAY Y. , DANKERT J. 
Published: December 2021

In Reply.—
Studies evaluating the efficacy of antibiotic prophylaxis for bacterial endocarditis in patients undergoing dental extractions are numerous, but studies dealing with the association between the peak serum concentrations achieved by the antibiotic used and the occurrence of postextraction bacteremia are lacking. It is questionable whether higher dosages than the dosages used in our study would have prevented the occurrence of postextraction bacteremia, because no relationship was found betwen the occurrence of postextraction bacteremia and postextraction serum penicillin concentrations.
Marasmus in a 17-Month-Old Laotian: Impact of Folk Beliefs on Health
Authors: OBERG CHARLES N. , DEINARD AMOS 
Published: December 2021

The recent influx of Southeast Asians from Laos, Viet Nam, Cambodia, and Thailand into the United States has introduced a variety of foreign cultures into Western society. At times these cultures and folk beliefs clash with those of Western society. These beliefs frequently have ramifications on the health of individuals and implications for accommodation requirements for our health care system.
Laos is comprised of a variety of ethnic groups including the Lao, Tai, Hmong, and Ka tribes.1 Each ethnic group has its own endogenous beliefs, but a belief in spirits or phi is evident in each. The following account is that of a Lao family, whose ethnic group accounts for 50% of the Laos population and 20% of the immigrants in the Twin Cities metropolitan area.
Corporal Punishment in Schools
Authors:   Committee on School Health
Published: December 2021

Since 1978, various committees of the American Academy of Pediatrics have intermittently addressed the question of corporal punishment and discipline.
A review of recent psychiatric, psychologic, and educational literature continues to accumulate evidence in opposition to corporal punishment in schools, although 47 states still have legislation permitting this management.
The American Academy of Pediatrics is opposed to the use of corporal punishment in schools, and urges all parents, educators, school board members, legislators, and other adults to seek the abandonment of corporal punishment and its legal prohibition in all states.
Employment of alternative methods1,2 for implementation of self-control and responsible behavior is recommended.
Protest Views of Singer
Authors: SINGER PETER 
Published: December 2021

In Reply.—
Both Bongiovanni and Callie refer to Nazi Germany as a warning against abandoning the sanctity-of-life view. This appeal to the slippery slope argument is a gross oversimplification of the differences between our society and Hitler's Germany. Alexander, on whom Bongiovanni relies, neglects the importance of Nazi racism and of the fact that the Nazi euthanasia program was not intended for the benefit of the patients—it was conducted secretly, relatives were deceived about what happened, and privileged groups such as veterans were exempted from it.
An Outbreak of Clostridium difficile Necrotizing Enterocolitis
Authors: CHANCE GRAHAM W. , HAN VICTOR K. M. 
Published: December 2021

In Reply.—
The observation made by Mathew et al on the lack of relationship between the isolation of Clostridium difficile and necrotizing enterocolitis (NEC) in their neonatal intensive care unit (NICU) is interesting, although the numbers are too small for relevant comment. Importantly, no mention was made on how the control subjects were selected.1 We agree that the colonization rate in NICUs may vary widely and may range up to 55% in certain units.2 In the subsequent four months following the outbreak that we reported, all infants admitted to our NICU had their stools tested weekly for C difficile.
Symposium on Genetic Engineering and Phenylketonuria
Authors: Cederbaum Stephen D. , Koch Richard , Donnell George N. 
Published: December 2021

STATEMENT OF PURPOSE
—George N. Donnell, MD
One might ask why have a conference about genetic engineering? The answer is clear. The subject matter has generated a great deal of interest; it has received a good deal of attention from the lay press. Industry has become involved, and many companies across our continent are utilizing the new technology for commercial application. There is no question that this area of genetics will have an impact on all of us. Clinicians will need to have more than a superficial grasp of this new area of medicine. The potential applications, the beneficial aspects, as well as the harmful effects must be appreciated.
This conference is designed to provide an orientation for the clinician and to highlight the social, economical, and ethical issues that the new technology has raised.
GENERAL INTRODUCTION
—Stephen D. Cederbaum, MD, and Richard Koch, MD
Phenylketonuria (PKU) was one of the first well-defined inborn errors of metabolism and one of the first to be successfully treated. The history from 1953 until recently had been one of an almost unbroken series of triumphs, each mitigating the adverse impact of this autosomal recessive disease. First the affected siblings of index cases, then the population at large was able to escape the consequences of PKU with the use of a phenylalanine restricted diet. Physicians accustomed to dealing with a developmental tragedy could look forward to meetings with normal patients who represented a therapeutic triumph. There was little concern when the burgeoning field of prenatal diagnosis bypassed PKU; it was, after all, a disorder that was readily treatable.
Spontaneous Mediastinal Emphysema
Authors: STURTZ GEORGE S. 
Published: December 2021

Spontaneous mediastinal emphysema, an apparently uncommon disorder of children, is characterized by precordial pain, unusual crunching sounds heard over the precordium during auscultation, subcutaneous air palpable in the neck, and the presence of mediastinal air on the chest roentgenogram.
CASE REPORT
A 13-year-old male adolescent had worked all afternoon mowing lawns. When his gas-powered mower stalled, he restarted it by pulling the starter cord vigorously and frequently. After the evening meal, he clipped a hedge by hand. Within one hour, he developed severe substernal pain. He had a sense of substernal pressure. He felt "... as if I were going to explode," and thought he was having a heart attack.
Administration of Medication in School
Authors:   Committee on School Health
Published: December 2021

Many children with chronic disabilities or illnesses are able to attend school because of the effectiveness of their prescribed medication. Any student who is required to take prescribed medication during regular school hours should do so in compliance with school regulations. These regulations should include the following:
1. A physician should provide written orders with the name of the drug, dose, time interval when the medication is to be taken, and diagnosis or reason the medicine is needed.
2. The parent or guardian should provide a written request that the school district comply with the physician's order.
3. Medication should be brought to school in a container appropriately labeled by the pharmacist or the physician.
4. When the student does not regularly take his or her own medication, or if the parent or physician requests that school personnel administer the medication, provision should be made for the medication to be kept in a locked cabinet. Designated personnel must be available to administer the medication at agreed-upon times, and arrangements should be made for alternate personnel to perform the task in case of absence. The person administering the medication must keep a written record.
5. When the child is usually responsible for taking his/her own medication, he/she may do so in school without supervision by school personnel, provided the physician and parent have provided the required authorizations. The school administration should cooperate with the physician, parent, and child. In such instances, it is understood that the school bears no responsibility for safeguarding the medication or assuring that it is taken, and the parent should provide a written statement relieving the school of such responsibility.
Antimicrobial Prophylaxis in Pediatric Surgical Patients
Authors:   Committee on Infectious Diseases, Committee on Drugs, and Section on Surgery
Published: December 2021

This Policy Statement was retired May 1987.
A major use of antimicrobial agents in hospitalized children is prophylaxis of postoperative wound infections. In view of this frequent use and the emerging consensus on recommendations for prevention of surgical wound infections,1 the American Academy of Pediatrics has developed guidelines for surgical antimicrobial prophylaxis in children. Prophylaxis is defined as use of antimicrobial drugs in the absence of suspected or documented infection.
FREQUENCY OF ANTIMICROBIAL PROPHYLAXIS
In an extensive study of patients in acute care general hospitals in Pennsylvania, 36% of antimicrobial courses were initiated for prophylaxis of infection after surgery or an invasive procedure, such as cystoscopy or cardiac catheterization.2 The prevalence and reasons for antimicrobial use have been studied primarily in general or adult hospitals, but the patterns of use in children are similar to those in adults.3-5 Two studies have demonstrated that prophylaxis accounts for approximately 75% of the antibiotic use on pediatric surgical services.4,6
The efficacy of antimicrobial prophylaxis in lowering the incidence of postoperative infection after certain types of surgery has been demonstrated amply in controlled clinical trials.7 These and earlier studies by Miles et al8 and Burke9 in experimental animals have delineated the principles for effective use of antimicrobial agents in surgical prophylaxis, including choice of drugs and when and how long they are to be given.
INAPPROPRIATE ANTIMICROBIAL PROPHYLAXIS
Prophylaxis has been incriminated as a major cause of inappropriate use of these antimicrobial agents in both adults and children.4,5,10-12 In 74% of antimicrobial courses given for prophylaxis in the Pennsylvania study, the duration exceeded 48 hours, an interval beyond which continuation has not been proven beneficial.2
Fungal Infections in Premature Infants
Authors: LANGDON DAVID R. 
Published: December 2021

To the Editor.—
Baley et al, in "Disseminated Fungal Infections in Very Low-Birth-Weight Infants: Clinical Manifestations and Etiology" (Pediatrics 1984;73:144-152), provided a wealth of clinical detail about systemic candidiasis in very low-birth-weight infants and emphasized the increased risk in infants with a prolonged period of parenteral alimentation via central lines. They observed late-onset carbohydrate intolerance in these infected infants and imply that fungal sepsis may precipitate hyperglycemia.
The causal relationship and temporal sequence of such insidious conditions as hyperglycemia and candidemia may be difficult to establish in retrospect.
Fungal Infections in Premature Infants
Authors: BALEY JILL E. , KLIEGMAN ROBERT M. , FANAROFF AVROY A. 
Published: December 2021

In Reply.—
Seventy percent of the low-birth-weight (LBW) infants whose condition was clinically diagnosed as systemic fungal infections demonstrated glucose intolerance. Langdon is correct that hyperglycemia, which may be found in young diabetic women, may encourage nonsystemic or local candidiasis. He is also correct that multiple factors may affect glucose tolerance in the sick low-birth-weight infant. Whereas it is possible that alterations in care or glucose intake might result in hyperglycemia, it is unusual for a low-birth-weight infant who has previously demonstrated stable glucose control to suddenly develop hyperglycemia and/or glycosuria at 5 weeks of age.
Computed Tomography in an Infant with Salt Poisoning: Relationship of Hypodense Areas in Basal Ganglia to Serum Sodium Concentration
Authors: HABBICK BRIAN F. , HILL ALAN , TCHANG STANLEY P. K. 
Published: December 2021

The pathogenesis of the intracranial complications of hypernatremia has been the subject of considerable research.1-5 The purpose of this communication is to report unusual appearances on computed tomographic (CT) scan of the brain in an infant who developed hypernatremia as a result of salt poisoning.
CASE REPORT
The patient, a 6-month-old male infant, was admitted for investigation of seizures. He had been admitted to another hospital on the evening of Jan 25, 1983, because of several episodes in which he rolled his eyes and had generalized stiffening and back arching. Diazepam had been given intramuscularly, and the infant was then given intravenous (IV) fluids (50 mmol of sodium chloride and 33.3 g of dextrose per liter).
Treatment of Occult Bacteremia
Authors: FELDMAN WILLIAM 
Published: December 2021

To the Editor.—
I am concerned about the conclusions in the paper on treatment of occult bacteremia.1 Carroll at al recommend "expectant antibiotic therapy for children who have no obvious source of infection and who have the clinical characteristics associated with occult bacteremia." As a relatively large percentage of infants aged 6 to 24 months have high fevers at one time or another, the implications of this approach were it to be widely implemented would be great.
Rheumatic Fever Article Questioned
Authors: GRIFFITHS SYLVIA P. 
Published: December 2021

To the Editor.—
The suggestion of Nordin1 that there may be a need to re-evaluate the current recommended prophylaxis for children with rheumatic fever is valid, particularly if carefully planned and controlled studies could be carried out. However, the author's contention that "It has been assumed that the levels of penicillin [following monthly intramuscular injection of 1.2 million units of benzathine penicillin G] are adequate to prevent reinfection with group A streptococcus, and hence to prevent recurrences of rheumatic fever" has always been qualified by others.
Hyperosmolar Oral Medication and Necrotizing Enterocolitis
Authors: MUTZ ALFRED E. , OBLADEN MICHAEL W. 
Published: December 2021

To the Editor.—
The observation of Finer et al1 of necrotizing enterocolitis following administration of oral vitamin E is in accord with our observation of necrotizing enterocolitis following administration of oral nystatin in preterm infants According to the suggestion of White and Harkavy2 that hypertonic formula may result from added oral medication, we determined the osmolarity (freezing point microosmometer Knauer) and pH (Digi 520, WTW) of 63 commercial liquid drugs dispensed for drop application and commonly used in European intensive care nurseries.
Vitamin K Deficiency
Authors: PAYNE NATHANIEL R. , HASEGAWA DUANE K. 
Published: December 2021

In Reply.—
We agree with Aballi that a combination of factors placed our patient1 at high risk for vitamin K deficiency during her first month of life. These factors include decreased intestinal absorption of vitamin K due to the cholestatic liver disease of α-1-antitrypsin deficiency and a diet consisting solely of breast milk which is known to have little vitamin K.
Aballi correctly points out the lack of data on the role of vitamin K in preventing late-onset hemorrhagic disease.
Risk Factors of Diabetic Retinopathy
Authors: MALONE JOHN I. 
Published: December 2021

In Reply.—
Marks correctly points out that any study "that attempts to relate complications of diabetes to glycemic control should at least have one cohort that achieves good control." However, we were not comparing the effects of "good control" v "poor control." Instead, we selected a group of insulin-dependent diabetic youths without significant retinopathy and followed them for 4 to 6 years. Half of the group developed retinopathy; the other half did not. Our report indicated the ways these two cohorts differed.
Development, Design, and Sample Composition
Authors: Bryla Dolores A. 
Published: December 2021

Following its introduction in 1947 for treatment of erythroblastosis fetalis and in 1949 for hyperbilirubinemia, exchange transfusion became accepted as the most effective means of preventing increasing serum bilirubin concentrations in the newborn infant from reaching a hazardous level.2 At that time, there was general agreement that the risk of bilirubin encephalopathy increased when serum bilirubin concentrations exceeded 20 mg/dL of serum and that this risk became greater with increasing concentrations. The safety of exchange transfusion was assessed by Boggs and Westphal14 in 1960 and found acceptable for that time.
In 1958, Cremer et al22 reported a distinct lowering of serum bilirubin levels in infants exposed to direct sunlight. This observation was followed by development of light sources for exposure of infants to treat or prevent hyperbilirubinemia (phototherapy).
Although phototherapy had been used and reported in Europe and South America, it was not until 1968 that the first study64 appeared in the pediatric literature of the United States. Other studies36,41,83 followed and suggested a potential role for phototherapy in the management of hyperbilirubinemia of the newborn infant. However, these studies suffered from lack of a sufficient number of patients to accommodate appropriate statistical adjustment procedures for the many known confounding variables. Also, these studies had inadequate numbers of control infants and phototherapy-treated infants who were followed long enough to perform evaluations of the impact of the mode of therapy on such parameters as hearing, sight, speech, IQ, and behavioral performance.31,74
DEVELOPMENT OF THE STUDY
In May 1972, the National Research Council of the National Academy of Sciences established a Committee on Phototherapy in the Newborn.