bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pink Rings Lymphocyte: A New Cytologic Abnormality Characteristic of Mucopolysaccharidosis Type II (Hunter Disease)
Authors: MAIER-REDELSPERGER MICHELINE , STERN MARC-HENRI , MAROTEAUX PIERRE 
Published: December 2021

To the Editor.—
Mucopolysaccharidoses are a group of rare hereditary storage disorders characterized by various lysosomal hydrolase deficiencies. Clinical condition, radiologic and ophthalmologic examinations, urinary mucopolysaccharides dosage, and leukocyte morphology are generally sufficient for diagnosis.1 However, a more precise classification may be difficult. We herein describe a new lymphocyte abnormality observed in 9/9 cases of type II (Hunter disease) in only 3/19 cases of other types of mucopolysaccharidoses.
Capillary blood was collected from 28 children with mucopolysaccharidosis referred to the pediatric department.
Renal Calcification
Authors: WELCH THOMAS R. , DE ROVETTO CONSUELO RESTREPO , HUG GEORGE 
Published: December 2021

To the Editor.—
Jacinto et al1 presented data concerning a group of 20 very low birth weight infants with renal calcification, most of whom had hypercalciuria. For 13 of these infants, furosemide administration was presumed to be the major factor contributing to the renal abnormality. For the remaining seven, there was no apparent basis for the increased calcium excretion and nephrocalcinosis. Although these children may have a heterogenous group of disorders, we suggest a specific diagnosis that may have been present in one or more of them.
Palatal Burn Due to Bottle Warming in a Microwave Oven
Authors: HIBBARD ROBERTA A. , BLEVINS RONALD 
Published: December 2021

Infections, tumors, nutshells, and foreign bodies may all be seen clinically as unusual lesions of the palate in children.1,2 We describe the case of an infant girl who had a palatal lesion, eventually determined to be secondary to a scald burn. We emphasize this case because the eventual diagnosis was not considered until almost 2 months after the patient was first seen, at which time additional history was elicited.
CASE REPORT
C.P. was a 4-month-old girl referred to our hospital for evaluation and treatment of poor weight gain with a palatal lesion. She was delivered by cesarean section for cephalopelvic disproportion after an uncomplicated fullterm pregnancy; her birth weight was 3,820 g (90th percentile).
Chronic Ipecac Poisoning in Infancy: A Case Report
Authors: BERKNER PAUL , KASTNER TED , SKOLNICK LAWRENCE 
Published: December 2021

Syrup of ipecac is an important component in the treatment of acute toxic ingestion. The American Academy of Pediatrics1 recommends that syrup of ipecac be kept in the household to be used for the conscious patient in the treatment of toxic ingestion when advised by a physician. Syrup of ipecac is not without toxicity, however, and this increased availability opens the door to poisoning by ipecac itself.
Toxicity has been observed in adults ranging from direct noxious effects on the gastrointestinal system2-4 to cardiomyopathy,2,5 generalized myopathy,6,7 and fatalities secondary to dehydration or electrolytic distubrances.8 Little is known about the signs and symptoms of ipecac toxicity in infants and children.
TrophAmine
Authors: ZLOTKIN STANLEY H. 
Published: December 2021

To the Editor.—
Heird et al1 recently described the use of a new parenteral amino acid mixture for low birth weight infants which included a peptide of tyrosine (N-acetyl-l-tyrosine). They concluded that infants tolerated the new mixture well and stated that it was "more efficacious with respect to nitrogen retention and weight gain than other available mixtures." It is my contention that their results do not substantiate their claims.
First, the efficiency of nitrogen retention with TrophAmine (70% retention) is not superior to older amino acid solutions.
A Catch in the Reye
Authors: ORLOWSKI JAMES P. 
Published: December 2021

In Reply.—
McGee and Sienko suggest that Reye syndrome may be a different disease in infants and young children than in older children and adolescents. This is an intriguing concept and is based partly on their observation that our patients1 were noticeably younger than the ones reported in the other four studies they reference. It is important to notice, however, that the age ranges in three of those four studies were purposely distorted and skewed to older children.
Reye Study Criticized
Authors: HALL SUSAN M. 
Published: December 2021

To the Editor.—
Orlowski et al1 conclude from their finding that only one of their 20 patients with Reye syndrome had taken aspirin and that the "purported association" between aspirin and Reye syndrome is "coincidental." This conclusion is based on a series of observations that do not stand up to critical analysis.
First, Reye syndrome is the same disease in Australia as it is in the United States. The authors correctly emphasize the similarities between their Australian series of patients with Reye syndrome and those reported to the British surveillance scheme.2
Another Solution for the Foreign Body in the Nose Problem
Authors: HENDRICK JIM G. 
Published: December 2021

To the Editor.—
Years ago, pediatrician Harvey Garrison, Jr, told me how to construct the "perfect" instrument for nasal foreign body removal—simple, available, and inexpensive. Open a paper clip and bend about 3 mm of the tip of either end about 45° by clamping the tip between the handle end of bandage scissors (Figure). By sliding the bent tip of this "medical instrument" over the foreign body and then drawing it outward, I have quickly and safely removed objects from the nose during the past 40 years of my pediatric practice.
AIDS and Suctioning Newborn Infants
Authors: KEMPEN PAUL M. 
Published: December 2021

To the Editor.—
The current recommended therapy for patients with meconium aspiration consists of extensive suctioning of the oropharynx and nasopharynx after delivery of the head, with subsequent endotracheal intubation and deep suction with the endotracheal tube as the suction catheter. The upper airway is commonly cleared with a bulb syringe and/or a Delee suction device. With both the Delee and the currently recommended endotracheal suction methods, the physician's mouth is the source of negative pressure.
Ascendancy of the `Black Bottle'
Authors: MACGREGOR DONALD F. 
Published: December 2021

To the Editor.—
Greensher et al1 commented on the increasing advice given to emergency room and primary care physicians regarding the use of activated charcoal instead of syrup of ipecac as first-line therapy for ingested toxins. This may be the direction that hospital facilities should be taking but, hopefully, will not undermine the confidence that many practitioners and the public have in syrup of ipecac. Many developing countries take their lead from North America and there are still areas on this continent where immediate emergency room access is impossible.
Religion and Child Abuse
Authors: FOST NORMAN C. 
Published: December 2021

In Reply.—
I agree with all of Dr. Fletcher's cautions about the hazards of state intrusion, especially when treatment is of uncertain benefit. It is not accidental that the statement cites only "easy" cases: it is precisely the clear cases that are outrageous and which require state protection.
His concern that "an arrogant physician" may dangerously impose uncertain treatment is the reason we have legal procedures for resolving such disputes. By reporting cases of medical neglect, the physician acknowledges that he or she should not be imposing judgment on parents of children but that a process should be initiated that allows representation of the interested parties, consideration of the relevant facts, and adjudication by a dispassionate, disinterested person.
A Catch in `a Catch in the Reye'
Authors: REMINGTON PATRICK L. , SULLIVAN KEVIN , MARKS JAMES S. 
Published: December 2021

To the Editor.—
The "Catch in the Reye"1 is not in the data but rather in the authors' interpretation. They incorrectly surmise that finding that only one in 20 patients with Reye syndrome had taken aspirin makes it unlikely that aspirin is a risk factor for Reye syndrome. This conclusion ignores the relationship between the prevalence of a risk factor (such as aspirin), the relative risk (RR), and the population attributable risk (PAR). In a population in which aspirin use is uncommon, such as Australia, only a small proportion of all patients with Reye syndrome will have a history of aspirin use.
AIDS-Associated Lymphoma of the Brain in a Child
Authors: MURPHY SHARON B. 
Published: December 2021

Invited Comment.—
The HIV-infected infant with a primary lymphoma of the brain reported by Professor Cocchi and colleagues from Italy adds another case to the series of pediatric AIDS-associated brain lymphomas already reported by others.1-3 The true frequency of these opportunistic cancers is unknown, but, surely, we are just beginning to see the tip of the iceberg. Neurologic deterioration is common in pediatric AIDS,2 and, unless cases suspicious for brain lymphoma are aggressively investigated by neuroradiologic examinations and brain biopsy, they are unlikely to be diagnosed antemortem.
Varicella Exposure Time
Authors: BRUNELL PHILIP A. 
Published: December 2021

To the Editor.—
The statement by Johnson et al1 that varicella was "protective against 96% of exposures" is misleading. Protection implies that disease was prevented. A continuing household exposure, eg, to a sibling, would be expected to produce an attack rate approaching 100%.2 Only four vaccinees had this type of exposure; 49 had other less intense types of exposure. The outcomes of other types of exposure are far less predictable but normally are not nearly as likely to result in varicella.3
Varicella Exposure Time
Authors: JOHNSON CANDICE E. 
Published: December 2021

In Reply.—
Dr Brunell's concerns are valid, and we certainly agree that protective efficacy can only be defined by prospective controlled trials, such as that of Weibel et al (N EngI J Med 1984;310:1409). However, we disagree with the concept that data about protection can only be derived from family exposure data. Arbeter et al (Pediatrics 1986;78(suppl):748-756) reviewed all studies of varicella vaccine in healthy children and combined household day-care and playmate exposures as we did.
SMALL SAMPLE SIZE SCIENTIST
Authors: Student  
Published: December 2021

The believer in the law of small numbers practices science as follows:
1. He gambles his research hypotheses on small samples without realizing that the odds against him are unreasonably high. He overestimates power.
2. He has undue confidence in early trends (e.g., the data of the first few subjects) and in the stability of observed patterns (e.g., the number and identity of significant results). He overestimates significance.
3. In evaluating replications, his or others', he has unreasonably high expectations about the replicability of significant results. He underestimates the breadth of confidence intervals.
4. He rarely attributes a deviation of results from expectations to sampling variability, because he finds a causal "explanation" for any discrepancy. Thus, he has little opportunity to recognize sampling variation in action. His belief in the law of small numbers, therefore, will forever remain intact.
Auriculotemporal Syndrome Seen Clinically as Food Allergy
Authors: BECK SUZANNE A. , BURKS A. WESLEY , WOODY ROBERT C. 
Published: December 2021

Adverse food reactions are common complaints to the general pediatrician. True hypersensitivity reactions are only a small part of the overall incidence of reactions. Other conditions that are initially thought to be food allergies include enzyme deficiencies, food poisoning, and pharmacologic agents in foods. We describe four children with parental complaints of "food allergies." For each child, auriculotemporal or Frey syndrome was later diagnosed. Because of its benign nature, Frey syndrome is important to recognize to avoid unnecessary laboratory testing and extensive referrals.
CASE REPORTS
Patient 1
A 4-year-old white girl had a history of food allergy that caused flushing of the left cheek. According to the mother, this flushing seemed most apparent after the child ingested red foods such as tomatoes, strawberries, and red candy.
Were the Good Old Days Better?
Authors: VADAKAN VIBUL V. 
Published: December 2021

Regarding the article concerning resident stress by Bergman (Pediatrics 1988;82:260-263), it should be emphasized that pediatricians in residency training two decades ago worked under immeasurably greater stress. Complete blood counts, urinalyses, CSF examinations, Gram stains, and bilirubin measurements were routinely performed by interns and residents in the ward laboratory. Interns were on call every other night for the entire year. There was 1 week of vacation and an annual stipend of a few hundred dollars.
The present-day trainee starts work at 8 or 9 AM with a contingent of clerks, nurses, and technicians to draw blood, fill out laboratory slips, and provide other forms of assistance.
In Reply: The Anencephalic Controversy
Authors: MILUNSKY AUBREY 
Published: December 2021

The letters of Drs Karrer, Wyatt, and Goodwin discuss issues that are largely covered in the papers of Landwirth and Botkin and my commentary. There is little more substantive information that can be added, but I would like to correct a point made in Dr Karrer's letter concerning an important tangential issue.
In my commentary I mentioned as an aside that pediatricians, when consoling grief-stricken parents, should encourage them to hold their baby and point to the normal body parts after having first placed a bonnet on the child's head.
In Reply: Sports and Sickle Cell Trait
Authors: NELSON MICHAEL A. 
Published: December 2021

Sickle cell trait was included because, at that time, a great deal of speculation and new information was forthcoming regarding sudden death in military recruits who had sickle cell trait. The members of the Sports Medicine Committee believed that it was important to indicate that, in spite of these new concerns, there were no data to indicate that anyone with sickle cell trait should not be included in any athletic activities.
Sickle cell disease was excluded because it is a disease with variable expression and one which is characterized by numerous exacerbations and periods of quiescence.
Streptococcal Pharyngitis Study
Authors: READ JENNIFER S. , BEEKMAN ROBERT H. 
Published: December 2021

Redd and co-workers found the sensitivity of their rapid diagnostic test for group A streptococcal pharyngitis to be 62.8% and its specificity to be 96.9%. Furthermore, the positive predictive value of the test was determined to be 91.5%, sufficiently high to significantly influence the care provided to their patients.
We strongly disagree with the authors' conclusion that their findings can be extrapolated to the general pediatric setting. Bayes theorem clearly relates a test's positive predictive value to its sensitivity as well as to the prevalence of true disease in the population.
Experience With Newborn Screening Using Isoelectric Focusing
Authors: Kleman Klara M. , Vichinsky Elliott , Lubin Bertram H. 
Published: December 2021

Electrophoretic techniques are used for hemoglobinopathy diagnosis. Confirmation of the hemoglobin variants is necessary. Currently, citrate agar electrophoresis is the most available, but high performance liquid chromatography is highly recommended in a reference laboratory.
Thin layer isoelectric focusing is an excellent technique that can be easily adapted for large-scale newborn hemoglobinopathy screening. Although the initial instrument cost can be about twice as much as the cost for standard electrophoretic equipment, cost-effectiveness for newborn screening is considerable because repeat analysis for uninterpretable results is not necessary. Resolution of hemoglobins is much better with thin layer isoelectric focusing than for any of the other electrophoretic methods currently available, and thin layer isoelectric focusing is the best method to use to establish a definitive diagnosis using newborn blood samples. Cord blood samples may be contaminated with maternal blood, and evaluation of Hb A2 levels in such samples can serve as the method to detect contamination. Follow-up testing is required regardless of the method of blood collection.
Alternative Methods for Screening
Authors: Garrick Michael D. 
Published: December 2021

Alternative methods (Fig 1) are already available for screening newborns for hemoglobinopathies. When choosing among the methods, one should remember three aims: (1) to identify Hb S; (2) to detect when Hb A is absent, suggestive of an S/S homozygote or a double heterozygote for S/β°-thalassemia or for S/C or S/another β-globin variant; and (3) to screen for other potentially pathologic states. (Double heterozygosity for S/β+-thalassemia is also important to detect, for example.) Detection of β-thalassemia major by newborn screening would be useful but is not yet reliable.
SPECIMENS
Blood
Both liquid (anticoagulated) blood and dried blood spots have been used. Liquid blood is easier to hemolyze, but dried blood spots are much easier to mail and store. Dried blood spots are already used in most states to screen for other disorders.
Age
In most states, a newborn capillary blood sample is obtained by heel puncture before the baby leaves the hospital to screen for phenylketonuria (and often for other disorders such as hypothyroidism). Cord blood, however, can be obtained in larger volume and is less likely to be contaminated by transfusion but more likely to be contaminated by maternal blood. I prefer the newborn specimen because of the advantages of coordinating screening programs both for thorough population coverage and for economic savings at several levels. In a coordinated screening program for multiple disorders, hemoglobinopathy screening may help in recognizing specimens from infants in whom a transfusion has taken place but the time the specimen was taken for screening has not been noted.
Selected Testing of Newborns for Sickle Cell Disease
Authors: Ohene-Frempong Kwaku 
Published: December 2021

There are two main reasons for sickle cell testing: the early detection of those with sickle cell disease and the detection of the carrier state, sickle cell trait. The mortality of the severe forms of sickle cell disease is particularly high during the first 5 years of life. Recent data have shown that early detection of sickle cell disease and institution of expert medical care and follow-up may reduce early mortality and morbidity. The ability to detect most forms of sickle cell disease in the newborn period has made the goal of preventive care theoretically possible.
The purpose of the detection of the carrier state, sickle cell trait, is less clear. Sickle cell trait has almost no clinical importance to the individual. Although a few case reports have suggested that sickle cell trait may not be a benign condition, most experts agree that sickle cell trait does not significantly alter health or disease. The main purpose of carrier testing is for genetic counseling. Mass screening of African Americans for sickle cell trait was advocated in the 1960s. However, no data have shown that results of such screening have had any impact on reproductive planning.
Ideally, sickle cell testing should provide the following results. All newborns with various forms of sickle cell disease should be diagnosed by 3 months of age, before the onset of clinical problems. All heterozygotes for the βs-globin gene, or the genes for other hemoglobinopathies that may combine with βs gene to produce clinically significant forms of sickle cell disease, should be aware of the relevant genetic information at childbearing age.
Parental Receptivity to Neonatal Sickle Trait Identification
Authors: Rowley Peter T. 
Published: December 2021

The demonstration that penicillin prophylaxis and early entry into programs of comprehensive care can reduce morbidity and mortality from infection has precipitated widespread discussion of the desirability of neonatal screening for sickle hemoglobin. However, the blood test that detects sickle cell disease also detects carriers of sickle cell disease and other abnormal hemoglobins. Is informing parents that their newborn has sickle trait beneficial?
The possible benefits of sickle trait identification at birth are twofold. The first is the education of the child of the significance of sickle trait when older. However, the time lapse makes its realization uncertain. The second is the identification of reproducing couples at risk for having a child with sickle cell disease. We have attempted to evaluate this second possible benefit. Its realization requires determining which newborns (about one in 12) have both parents as hemoglobinopathy carriers.
METHODS
In Rochester, NY, we are conducting a hemoglobin screening program to study the benefits and burdens of carrier identification. Although our primary focus has been detection during pregnancy, we have collected some data concerning the consequences of newborn detection. My office is specifically charged by the New York State Health Department to notify parents of newborns with sickle trait in the nine-county area surrounding Rochester. The mothers of newborns with sickle trait were seen for this reason between August 1983 and February 1987. The demographic data were collected from the whole group. The knowledge data (Table) were collected from an unselected subset of 30 of these mothers.
CARE OF INFANTS WITH DISEASE: THE ULTIMATE OBJECTIVE OF NEWBORN SCREENING
Authors: Brown Audrey K. , Miller Scott T. , Agatisa Patricia 
Published: December 2021

We conclude that newborn screening with a comprehensive follow-up program, emphasizing parental education and the use of penicillin prophylaxis, is effective in reducing morbidity and mortality from pneumococcal infections in infants with sickle cell disease. One of the 159 infants followed-up during 1981 to 1986 died as a result of overwhelming sepsis. This compares with an anticipated mortality rate at that time of 10%.
Penicillin prophylaxis should be started at 3 months of age in infants with Hb SS and should also be given to infants with hemoglobin SC and S- β-thalassemia. The duration for which prophylaxis must be given is not known, but it should be continued at least through the age of 5 years. Monthly injections of benzathine penicillin is an inadequate regimen; we recommend that, if penicillin is to be administered by the intramuscular route, it should be given every 3 weeks and supplemented in the last week with oral penicillin. However, we now emphasize that the daily oral administration of penicillin is the preferred method of administration for prophylaxis.
Pneumococcal vaccine continues to be given at 2 years of age, with a booster dose recommended at 4 to 5 years of age. H influenzae vaccine conjugates are immunogenic when given at 18 months, and newer vaccines may soon be available that can be given much earlier.
Finally, and most important, the program must be structured in such a way as to thoroughly involve the parents in assuming responsibility for their child's continued health and well being.