bluuebunny/crossref_metadata_2025_split huggingface dataset:
TECHNIQUES AND EFFICACY OF SCREENING
Authors: Guthrie Robert 
Published: December 2021

The story of newborn screening for inborn errors of metabolism begins with the ideas expressed by Archibald Garrod in 1902. He published a paper called "The Incidence of Alkaptonuria, A Study in Chemical Individuality," in which he showed insight into the inheritance of specific chemical defects in metabolism. This was before any knowledge of enzymes or metabolism existed and was soon after the discovery of Mendel's laws of inheritance. The application of Mendel's laws of inheritance in the human organism was pioneered by Garrod. He originated the phrase "inborn errors of metabolism" that is still used today.
Forty years later, Beedle and Tatum performed their Nobel prize-winning work with the mold Neurospora to demonstrate the "one gene-one enzyme" hypothesis. One of their associates, the late Dr David Bonner, later published a series of papers concerning the case of one auxotroph of Neurospora with two amino acid requirements for isoleucine and valine. This double requirement was found to be due to "internal inhibition": the accumulation of the precursor to one branch-chained amino acid, isoleucine, blocked the conversion of another branch-chained precursor to its amino acid, valine. This phenomenon of "internal inhibition" demonstrated in Neurospora was a prelude to more recent insight into human errors of metabolism, such as phenylketonuria (PKU).
Bickel's publication in 1953 of a dietary treatment for PKU led to a great deal of interest in early treatment of PKU. There were many attempts to set up urine screening programs using the ferric chloride reagent that Folling used in discovering the disease in 1934.
Cellulose Acetate/Citrate Agar Electrophoresis of Filter Paper Hemolysates From Heel Stick
Authors: Schedlbauer Lewis M. , Pass Kenneth A. 
Published: December 2021

In 1965, New York State Public Health Law 2500-a was enacted, mandating the screening of all newborns for phenylketonuria. The law was amended in 1974 to include testing for six other conditions, one of which is homozygous sickle cell disease. Originally, all screening was done in four semiautonomous laboratories located in Erie County (Buffalo), Onondaga County (Syracuse), New York City, and Albany County (Albany). These laboratories were set up to receive, test, and report results of all specimens in their respective regions of the state. Between 1981 and 1985, all screening was centralized at the Wadsworth Laboratories of the New York State Health Department in Albany, where in 1986 the newborn screening laboratory tested more than 288,000 specimens. Hemoglobin is screened with the two-tier procedure of Garrick et al: alkaline electrophoresis on cellulose acetate followed by acid electrophoresis on citrate agar.
METHODS/PROCEDURES
The average daily volume of the New York State Newborn Screening Program is 1,200 specimens, but the number of specimens can range from 800 to 3,000 per day. Specimens are collected from infant blood obtained by heel stick on Schleicher and Schuell 903 filter paper. Medical personnel are advised to collect the specimen when the infant is three to five days of age and before any planned transfusion is administered. The specimens are air dried and mailed to the laboratory within 24 hours of collection.
When received in the laboratory accessioning unit, the specimens are examined to determine their suitability for testing. Specimens are screened for sickle hemoglobin only if there is sufficient blood on the filter paper and the date of collection to date of laboratory receipt is less than 14 day.
FINE IN AN INFANT DRUG CASE
Authors: Student  
Published: December 2021

St. Louis, Jan. 21 (AP)—A pharmaceutical company has been fined $115,000 and a former executive sentenced to six months in a work-release program for marketing an unapproved drug linked to the death of 38 premature babies.
The sentences were imposed Friday by Judge Clyde Cahill of Federal District Court here, which had jurisdiction in the case because the company, O'Neal, Jones & Feldman Pharmaceuticals, now called O'Neal Inc., is based in the St. Louis suburb of Maryland Heights.
O'Neal was the sole distributor of E-Ferol Aqueous Solution, used for treatment of retrolental fibroplasia, a disease that can cause blindness in premature infants exposed to too much oxygen.
The product, which was never tested by the Food and Drug Administration, was introduced in 1983 and recalled in 1984, after reports of infant deaths associated with its use.
The executive who was sentenced Friday is James B. Madison, a 68-year-old former O'Neal vice president for operations, who had pleaded guilty to wire fraud and mislabeling. In addition to the six months in a work-release program, Judge Cahill sentenced him to three years' probation and ordered him to contribute $10,000 to a charity that helps children.
O'Neal, which had pleaded guilty to charges including mail fraud and failure to obtain approval of a new drug application, was ordered to pay $125,000 in court costs in addition to the $115,000 fine.
The sentences were handed down one day after Judge Cahill imposed a $130,000 fine on the drug's now-defunct manufacturer, Carter-Glogau Laboratories of Glendale, Ariz. Its former president, Ronald M. Carter, and O'Neal's former president, Larry K. Hiland, were sentenced to six months in prison by Judge Cahill, who ordered them to contribute $65,000 each toward research on drug safety.
Carter-Glogau, Mr. Carter and Mr. Hiland had all been convicted of conspiracy, marketing and unapproved drug and misbranding the drug.
The two companies and their insurers have reached settlements totaling millions of dollars with the families of children treated with the drug.
DRUG USE DECLINES IN U.S. HIGH SCHOOLS
Authors: L. J. F. 
Published: December 2021

The nation's high school seniors are using fewer drugs than any class since 1975, a report from the National Institute on Drug Abuse showed.
Researchers said they were particularly encouraged by results showing a drop in cocaine use for the second year in a row, and the beginnings of a reversal in the soaring use of crack, an inexpensive, refined form of cocaine.
However, the war against drugs is far from over, with more than half of all students using an illegal substance at least once before graduating, said Charles R. Schuster, NIDA director. Moreover, drug use remains at a very high level among high school dropouts, he said.
The survey polled 16,300 high school seniors from 135 schools nationwide.
WHO IS LOST TO FOLLOW-UP?
Authors: Student  
Published: December 2021

The belief that impaired infants are more likely to continue in follow-up is. . .not supported. The infant's developmental status significantly influenced loss to follow-up only between the 18-to 36-month time interval. Here, more babies in the dubious (vs abnormal) category withdrew.
In general our findings suggest that environmental characteristics exert the major effect on dropout rates in high risk infants and their controls.
More on Meconium Aspiration
Authors: WISWELL THOMAS E. 
Published: December 2021

In Reply.—
I am intrigued by Dr Karlowicz's proposal that vigorous babies may be insidiously aspirating swallowed meconium. He states it is "imperative" to remove meconium-stained fluid from the stomachs of depressed neonates. Although it seems reasonable that an infant could regurgitate and aspirate meconium-stained gastric contents, I am unaware of any reports which support this conjecture.
Most standard neonatology, perinatology, and pediatric textbooks do not recommend "emptying" the stomach to prevent aspiration. Furthermore, the few resources recommending gastric suctioning do not reference any investigations or anecdotal reports supporting the procedure.
Initial Medical Evaluation of an Adopted Child
Authors:   COMMITTEE ON EARLY CHILDHOOD, ADOPTION AND DEPENDENT CARE
Published: December 2021

Providing health care for adopted children always has been a significant part of pediatric practice, and pediatricians have provided services in different areas of the adoption process for many years. Recent changes in adoption prompt a reevaluation of the pediatrician's role in managing the medical care of adopted children and their families.
There has been a gradual shift from viewing adoption as a childless couple's opportunity to find an infant to focusing on the needs of the child first by placing children within families who can help them realize their fullest potential. Increasingly, adoption agencies work with older children and special needs children who have physical handicaps, emotional problems, or chronic medical illness. Private adoptions are often arranged outside of agencies, sometimes with open discussions between birth and adoptive parents. In addition, there are now more than 10 000 international adoptees entering this country each year. The pediatrician must be equipped to evaluate the special needs of all these children and to help their new families integrate them into the family unit.
This statement addresses the initial medical evaluation of adoptive children who may have acute and long-term medical, psychological, and developmental problems because of their genetic, emotional, cultural, psychosocial, and/or medical backgrounds.
Prior to adoption, or at the time of entry into the family, the pediatrician should begin a careful medical assessment of the child and should counsel the family appropriately regarding adoption issues. Pediatricians should be alert to the following potential problems:
Universal Hepatitis B Immunization
Authors:   Committee on Infectious Diseases
Published: December 2021

HEPATITIS B DISEASE AND EPIDEMIOLOGY
In the United States 200 000 to 300 000 acute infections with hepatitis B virus (HBV) occur each year.1,2 More than one million persons in the United States have chronic HBV infection, and approximately 4000 to 5000 persons die each year from HBV-induced chronic liver disease and hepatocellular carcinoma. Although HBV infections occur during childhood and adolescence, the full impact of these infections is not recognized until many years later when chronic liver disease and hepatocellular carcinoma may develop. The incidence of HBV infection increases rapidly during adolescence, with higher rates among blacks than among whites (Fig 1).3 Although rates vary by region, sex, and race, between 3.3% and 25% of all persons have had HBV infection by 25 to 34 years of age. The likelihood of becoming chronically infected with HBV varies inversely with the age at which infection occurs. HBV transmitted from hepatitis B surface antigen (HBsAg)-positive mothers to their newborns results in HBV carriage for up to 90% of infants. Between 25% and 50% of children infected before 5 years of age become carriers, whereas only 6% to 10% of acutely infected adults become carriers. It is estimated that more than 25% of carrier infants will die from primary hepatocellular carcinoma or cirrhosis of the liver, with most of these deaths occurring during adult life.
HBV infection occurs more commonly in certain populations, including Pacific Islanders, Alaskan Natives, immigrants from countries in which infection is highly endemic, persons who require multiple transfusions of blood or blood products, and persons with high-risk lifestyles, including intravenous drug abuse and contact with multiple sexual partners.
Q-T INTERVAL DURING ACTIVE AND INACTIVE RHEUMATIC FEVER
Authors: FINKEL SIDNEY , BALDWIN JANET S. 
Published: December 2021

The Q-Tc in 79 normal children ranging in age from 2 to 12 years was found to be 0.418 with a standard deviation of 0.016.
The Q. Tc was studied in 208 children ranging in age from 3 to 13 years during active rheumatic fever and chorea. One hundred nineteen of these patients were subsequently followed in the cardiac clinics. The P-R interval was studied simultaneously, and observation were made in regard to the presence or absence of resultant organic heart disease.
A significant number of patients showed prolongation of the Q-Tc value during the acute illness, far more so in those with rheumatic carditis than with polyarthritis or chorea alone. During the inactive phase, a number of patients maintained a prolonged Q-Tc value.
The Q-Tc value in patients with rheumatic fever is an additional laboratory aid which may help in determining the presence of active carditis. The prolongation of the Q-Tc value in polyarthritis and in chorea in the absence of other clinical findings should suggest further observation for the possible presence of a mild carditis. However, in evaluating patients whose Q-Tc is above normal and in whom the presence of active carditis is otherwise questioned, consideration must be given to the fact that the Q-Tc has been found to exceed the upper limit of normal in some normal children.
These findings show that, following active rheumatic fever, in some patients the Q-Tc value may remain prolonged over long periods of time in the absence of any manifest clinical activity or change in the cardiac status of the patient. Therefore it is the opinion of the authors that when careful evaluation over a period of time shows no other clinical or laboratory evidence of rheumatic activity, the sole finding of a Q-Tc remaining above normal range should not be considered as indicative of persistence of active carditis.
SULFOBROMOPHTHALEIN SODIUM EXCRETION TEST AS A MEASURE OF LIVER FUNCTION IN PREMATURE INFANTS
Authors: OBRINSKY WILLIAM , DENLEY MALCOLM L. , BRAUER RALPH W. 
Published: December 2021

In normal adults, following a single intravenous injection of BSP, the logarithmic decline of plasma BSP levels is rectilinear. In patients with hepatic disease, the plotted values are curvilinear with flattening of the curve, or "saturation," occurring at high plasma BSP concentration. The use of the excretion curve provides a dynamic representation of the hepatic uptake of dye throughout the test, avoids the theoretic objections to the 45 minute test, and obviates the necessity for plasma volume determinations.
Thirty-five healthy prematures of varying age and weight were the subjects of a study of liver function by the bromsulphalein excretion test. As measured by the BSP excretion test, there is progressive maturation of liver function with age in prematures which does not necessarily correlate with weight. The excretion curves obtained in prematures 18 to 28 hours of age resemble those of older patients with severely impaired hepatic function. Clearance of dye in these subjects is slow throughout the test. With increasing age there is a gradual change in the pattern of the curves characterized by the appearance of the initial sharp slopes coincident with rapid dye removal. "Saturation," or flattening of the curves, persists until a later age and takes place at progressively lower plasma levels. This biphasic pattern is apparent in the prematures over 4 days of age. The normal adult pattern is not attained until about 6 to 8 weeks although the 45 minute retention values become normal at three weeks.
The gradual development of normal excretion curves in premature infants may be explained by the progressive maturation of the anatomic and physiologic factors involved in the mechanism of BSP clearance. The increasing size of the capillary network in the liver that occurs with advancing age in effect increases hepatic blood flow and exposes a greater volume of blood in the sinusoids to the activity of the liver. The appearance of the initial sharp slopes, representing rapid dye removal, probably results from this increasing vascularity and possibly from maturation of the hepatic uptake mechanism. The continuance of the biphasic character of the curves probably results from inability to excrete the dye efficiently into bile.
Nickel Allergy From a Bed-Wetting Alarm Confused With Herpes Genitalis and Child Abuse
Authors: HANKS JOHN W. , VENTERS WANDA J. 
Published: December 2021

The evaluation of vesicular genital rashes can be challenging for the pediatrician. The presence of an unusual genital rash should raise the concern of sexual abuse. In recent years awareness of child sexual abuse and its various presentations has increased through both lay and medical literature. When the possibility of sexual abuse arises, historical data may be difficult to elicit and can be misleading. The decision to investigate further may be based on the clinical appearance and location of the rash and the physician's level of suspicion.
In this setting, the differential diagnosis of genital rashes in children assumes tremendous importance.
Report of the National Institute of Neurological Disorders and Stroke Workshop on Near Infrared Spectroscopy
Authors: Hirtz Deborah G. 
Published: December 2021

A workshop about near infrared spectroscopy (NIRS), an emerging technology used to measure cerebral oxygenation and blood flow, was sponsored by the Developmental Neurology Branch, Division of Convulsive, Developmental, and Neuromuscular Disorders of the National Institute of Neurological Disorders and Stroke in Bethesda, MD, on March 31 and April 1, 1992. This was an international work-shop designed to bring together experts in the development of this technology with clinical researchers. Topics covered included the history and background of the development of NIRS technology, experimental models for the use of NIRS, clinical experience with NIRS in the neonate and the intrapartum fetus, and current key research issues with regard to technology and clinical use.
THE TECHNOLOGY
Near infrared spectroscopy is a new application of an existing technology which can provide information about changes in cerebral oxygen saturation, cerebral blood flow and volume, and oxygen utilization in the brain. The technology has been used for a long time to monitor hemoglobin, but only more recently for cytochrome oxidase. It involves the same basic principle used in the pulse oximeter, which uses light in the visible range to detect changes in finger arterial oxygen saturation. The method is based on the fact that light in the near infrared range (700 to 1000 nm) can pass through skin, bone, and other tissues relatively easily and that there are characteristic absorption bands of oxygenated and deoxygenated hemoglobin, and of the mitochondrial enzyme cytochrome oxidase (or cytochrome AA3) in the near infrared range.
When the near infrared beam is passed through tissue, a decrease in signal intensity results from the absorbance of the chromophores in the medium.
Listening to, Parents
Authors: WOOD DAVID 
Published: December 2021

In Reply.—
In our article entitled "Are Poor Families Satisfied With the Medical Care Their Children Receive?" we found that poor patients had a less satisfying experience communicating with the physician during a visit for their child. We suggested five potential barriers to communication between the patient (parent) and physician that may be more common when seeing poor patients (eg, inadequate reimbursement leading to less time, culture and language differences, practice setting limitations, etc). Unfortunately, in our non-system of care for the poor these barriers are commonly encountered by the physician and patient, making good communication much more difficult.
Exercise and Breast-Feeding: A Different Experience
Authors: WALLACE JANET P. 
Published: December 2021

In Reply.—
Since the publication of our recent investigation of infant acceptance of postexercise breast milk,1 we have been pleased with the interest expressed by both the scientific and lay communities. Although the concerns of Drs Dewey and Lovelady are legitimate, we wish to make it clear that it would be unfortunate if our findings would discourage mothers from exercising in lactation. Our purpose was to investigate the legitimacy of reports from mothers who experienced nursing difficulties following exercise and to provide guidelines for successful lactation and exercise which is clearly vital for physiological and psychological well being of both the mother and the infant.
Saddened Not Amused
Authors: CHARNEY EVAN 
Published: December 2021

To the Editor.—
I was saddened to read the article by Douglas Nelson, "Humor in the Pediatric Emergency Department: A 20 Year Perspective" (Pediatrics. 1992;89:1089-1090). Although Dr Nelson did not intend "to ridicule our patients or their parents" by listing their malapropisms, that is exactly what his article does. It is one thing to recognize the occasional humor in a patient's confusion with medical terminology, quite another to enter those remarks in a notebook or publish them in a medical journal.
Effectiveness of Iron-Fortified Infant Cereal in Prevention of Iron Deficiency Anemia
Authors: Walter Tomas , Dallman Peter R. , Pizarro Fernando , Vebozo Luis , Peña Gloria , Bartholmey Sandra J. , Hertrampf Eva , Olivares Manuel , Letelier Angelica , Arredondo Miguel 
Published: December 2021

Background. Iron deficiency continues to be a common problem among infants throughout the world. Iron-fortified formula is effective in preventing iron deficiency but the benefit of iron-fortified cereal is controversial.
Methods. We compared iron-fortified rice cereal to unfortified rice cereal in infants who were exclusively breast-fed for more than 4 months and to iron-fortified formula in infants who were weaned to formula before 4 months of age. The design was double blind in respect to the presence or absence of fortification iron in the cereal or formula and included 515 infants who were followed on the protocol from 4 to 15 months of age. Rice cereal was fortified with 55 mg of electrolytic iron per 100 g of dry cereal and infant formula with 12 mg of ferrous sulfate per 100 g of dry powder, levels approximating those in use in the United States. Measures of iron status were obtained at 8, 12, and 15 months. Infants with hemoglobin levels of &lt;105 g/L were excluded from the study and treated.
Results. Consumption of cereal reached plateaus at means of about 30 g/d after 6 months of age in the formula-fed groups and 26 g/d after 8 months in the breast-fed groups; these amounts are higher than the 19-g/d mean intake by the 73% of infants who consume such cereal in the United States. Among infants weaned to formula before 4 months, the cumulative percentages of infants excluded for anemia by 15 months were 8%, 24%, and 4%, respectively, in the fortified cereal, unfortified cereal and formula, and fortified formula groups (P &lt; .01 unfortified vs either fortified group; the difference between the two fortified groups was not significant). In infants breast-fed for more than 4 months, the corresponding values were 13% and 27%, respectively, in the fortified and unfortified cereal groups (P &lt; .05). Mean hemoglobin level and other iron status measures were in accord with these findings.
Conclusion. Iron-fortified infant rice cereal can contribute substantially to preventing iron deficiency anemia.
Preventing Lead Poisoning in Kids: A Win-Win Opportunity
Authors: KARP HARVEY N. 
Published: December 2021

To the Editor.—
For many, it is hard to believe that small amounts of lead pose a risk to children. In the February issue of Pediatrics, Dr Edgar Schoen cautioned physicians that the recent national focus on lead is overstated.1 Although Dr Schoen is correct that early lead studies were inadequately designed, he ignores later research which is well-designed and clearly implicates lead. In doing so, he seriously underestimates the hidden cost to our society of this pervasive toxin.