bluuebunny/crossref_metadata_2025_split huggingface dataset:
INTERLEUKIN-2 RECEPTOR γ CHAIN MUTATION RESULTS IN X-LINKED SEVERE COMBINED IMMUNODEFICIENCY IN HUMANS
Authors: Jeppson John D. , Liu Andrew 
Published: December 2021

Purpose of the Study. Interleukin-2 (IL-2) and IL-2 receptors play a critical role in the regulation of the T cell immune response. IL-2 receptors are made of combinations of three different chains a, β, and γ. Different combinations of these chains result in the formation of three different classes of IL-2 receptors. The authors of this paper were interested in the interleukin-2 receptor γ chain (IL-2Rγ) gene and its relationship to X-linked severe combined immunodeficiency (XSCID).
Findings. The IL-2Rγ gene was localized to the X chromosome in the same region as the XSCID locus. The IL-2Rγ gene from three patients with XSCID were found to have point mutations leading to premature stop codons and truncated IL-2Rγ chains. These shortened IL-2Rγ chains lack the cytoplasmic domain required for cell signal transduction.
Reviewers' Comments. This is the first article showing the XSCID is associated with mutations in the IL-2Rγ gene. This suggests that IL-2Rγ plays a critical role in the early development of T-cells and their function in the immune system. These results also suggest that other genetic defects in components of the IL-2/IL-2R system could be responsible for other forms of SCID or other immunodeficiency syndromes. The fact that all three patients had different mutations suggests that there is no single dominant mutation. These findings have important implications for prenatal and postnatal diagnosis, carrier identification, and possibly gene therapy for XSCID.
Familial Hirschsprung's Disease: Study of a Texas Cohort
Authors: Reyna Col Troy M. 
Published: December 2021

Objective. To better define the types of transmission of Hirschsprung's disease (HD) in order to allow for earlier diagnosis and treatment and reduction of attendant morbidity.
Methods. A proband with HD was identified. A detailed family history was obtained and a 54-member, five-generation family pedigree constructed and analyzed.
Results. Six members of the kindred presented with classic biopsy-proven HD. Two of the six members were a pair of dizygotic twins. Three additional members had symptoms reminiscent of HD or HD variant (neuronal intestinal dysplasia) and were managed nonoperatively. There was one death related to HD. Hirschsprung's disease was manifested in offspring of both affected parent(s) and unaffected parents. Symptom manifestation was noted to skip generations.
Conclusions. Analysis of this kindred lends strong evidence to the possibility of autosomal dominant inheritance. This inheritance has a variable penetrance (expression) that may be a factor of mutation or of a spectrum of the disease, ie a type of neural crestopathy. Kindreds of this sort need to be identified to allow for a complete data collection and understanding of the vagaries of HD transmission. This detailed documentation will allow for earlier diagnosis and effective treatment and possible gene localization of the specific defect.
Children as Victims of Violence: A National Survey
Authors: Finkelhor David , Dziuba-Leatherman Jennifer 
Published: December 2021

Objective. The goal of this study was to gain a more comprehensive perspective on the scope, variety, and consequences of child victimization, which has been obscured by a fragmentation into specific problems like sexual abuse or kidnapping.
Methods. Two thousand children aged 10 to 16 years were interviewed in a national telephone survey of children.
Results. In the previous year, a quarter of the children had experienced a completed victimization, one in eight had experienced an injury, and one in a hundred required medical attention as a result Nonfamily physical assaults were the most numerous. Contact sexual abuse occurred to 3.2% of girls and 0.6% of boys. There were also substantial numbers of incidents of attempted kidnappings and violence directed to children's genitals.
Conclusion. The victimization of children occurs to a greater extent than has been previously reported and is poorly represented in official statistics. Implication. The authors argue for a more comprehensive interest in children's victimization including better national statistics about the problem.
GOVERNMENT-MANDATED CIGARETTE WARNING LABELS ARE HAZARDOUS TO HEALTH
Authors: L. J. F. 
Published: December 2021

The 1964 official government report... triggered a cascade of events aimed at discouraging smoking. Now, 30 years later... it is time to acknowledge that our current policies to curb smoking simply are not working.
The warning labels, while presumably well intentioned, have done nothing to discourage smoking. Instead, they have provided extraordinary legal protection for the tobacco industry.
Industry attorneys can rely on the label for their nonsequitur defense in liability suits. They argue, in essence, that, "Cigarettes are not dangerous, but if they were, which they are not, the government `preempted' our responsibility to warn of those dangers."
If free market forces and an unfettered judicial system had prevailed, the cigarette would now be an anachronism simply because it would be too expensive to buy and too unprofitable to produce.
The Effect of Phosphate Supplementation on Linear Growth in Children with X-Linked Hypophosphatemia
Authors: Seikaly Mouin G. , Browne Richard H. , Baum Michel 
Published: December 2021

Background. X-linked hypophosphatemia is the most common inherited cause of rickets. Current therapy for this disorder includes vitamin D and phosphate supplementation; however, phosphate therapy has been associated with nephrocalcinosis. The purpose of this study is to evaluate the effect of oral phosphate therapy on growth in patients with X-linked hypophosphatemia treated with either calcitriol or dihydrotachysterol (vitamin D).
Methods. We retrospectively evaluated the prepubertal growth of 36 children with X-linked hypophosphatemia. The height standard deviation score (Z-score) of patients initially treated with vitamin D alone and the Z-scores of patients treated with vitamin D and phosphate therapy were compared. In addition, the growth of patients treated with vitamin D was compared with that of patients treated with vitamin D and phosphate from the outset of therapy.
Results. Patients treated with vitamin D alone for 5.36 ± 2.18 years had an improvement in Z-score from -3.18 ± 1.10 to -2.49 ± 0.66 SDS, ,P &lt; .05. Adding phosphate therapy for patients initially treated with vitamin D alone for 4.83 ± 2.99 years did not further improve Z-score (-2.49 ±0.66 vs -2.35 ± 0.83). Initial therapy with vitamin D and phosphate for 4.33 ± 2.19 years also improved Z-score, (-2.84 ± 1.02 vs -1.98 ± 0.82, P &lt; .05). The change in Z-score was similar to the group treated with vitamin D alone compared with the group treated initially with vitamin D and phosphate (0.65 ± 0.54 vs 0.85 ± 0.65, respectively).
Conclusion. These data demonstrate that both vitamin D alone and in combination with phosphate improved linear growth. Adding oral phosphate for children initially treated with vitamin D alone did not improve Z-score. Initial therapy with vitamin D and vitamin D plus phosphate produced similar changes in linear growth.
High Prevalence of Antiphospholipid Antibodies in Children With Idiopathic Cerebral Ischemia
Authors: Angelini Lucia , Ravelli Angelo , Caporali Roberto , Rumi Viviana , Nardocci Nardo , Martini Alberto 
Published: December 2021

Background. The presence of circulating antiphospholipid antibodies (aPLs) is frequently associated with thromboembolic phenomena.
Objective. To investigate the prevalence of aPLs, detected as lupus anticoagulant (LA) or anticardiolipin antibody (aCL), in a group of unselected children with idiopathic cerebral ischemia.
Design. Prospective, case series.
Setting. A pediatric neurology department.
Patients. Thirteen children with cerebral ischemia (eight with stroke, three with transient ischemic attacks, and two with ocular ischemia). Age-matched apparently healthy children served as controls.
Measurements. LA and aCL determination was performed within 3 days after the occurrence of the ischemic event and was repeated after 3 to 6 months. To be defined as aPL-positive, patients had to have either a positive LA test or positive IgG and/or IgM aCL at moderate/high level in both determinations.
Main results. Ten (76%) of the 13 patients were positive for either LA or aCL. No differences were found between aPL-positive and aPL-negative patients with respect to clinical manifestations or radiological features. Six (46%) of the 13 patients had a history of multiple ischemic events.
Conclusions. Our results show a very high prevalence of aPLs in children with idiopathic cerebral ischemia. Because the presence of these antibodies has relevant therapeutic implications, their determination in children with cerebral ischemia is recommended.
In Reply: Scalding Water Temperatures
Authors: FELDMAN KENNETH W. 
Published: December 2021

When we published our original description of tap water burn injuries in 1978, we were aware that detergent manufacturers recommended water temperatures above 60°C (140°F) for automatic dishwashing. However, personal communication from one detergent manufacturer indicated that such high temperatures were not required. Further, Kanecko found that four of six detergents performed adequately at 38 to 49°C (100 to 120°F).
Goldwasser suggested then (and it has become more common) that dishwashers be built with booster heaters that will raise the temperature of incoming water to the desired cycle temperature. This is much more energy-efficient and safety-conscious approach to automatic dishwashing than maintaining high water heater temperatures.
Aspartame and Attention Deficit Disorder (ADD)
Authors: KROHN JACQUELINE A. 
Published: December 2021

In a recent article Shaywitz et al conclude that aspartame at greater than 10 times usual dose does not affect the behavior and cognition of children with attention deficit disorder (ADD). Accardo and Lindsay note that the study refutes an effect of aspartame on behavior.
This study has a type II statistical error. A study of this size cannot answer the question of whether aspartame affects the behavior of children with ADD. The authors chose a group of children who possibly would be more sensitive to aspartame than the general population, but it is unknown if any of the children were sensitive and likely to respond adversely to the aspartame.
Prevention of Peer and Community Violence: The Pediatrician's Role
Authors: Slaby Ronald G. , Stringham Peter 
Published: December 2021

Pediatric practitioners can play a pivotal preventative role in helping their young patients to lower their risk of becoming involved in peer and community violence by developing a sound program and implementing specific preventative treatments. The recommendations presented here are derived from the integration of two sources: (1) research evidence on children's involvement with peer violence as aggressors, victims, bystanders who support violence, nonviolent problem-solvers, gender-role-related participants, or witnesses to community violence; and (2) the practical experience of developing a pediatric violence prevention program in a neighborhood health center over the last 12 years.
To develop a sound violence prevention program, it is recommended that pediatricians (1) define violence as a major health problem that can be prevented, (2) develop a thorough knowledge of both the factors underlying violence and the misleading popular myths regarding violence, (3) adopt a clear strategy by which to organize and focus these findings in the service of creating behavioral change, and (4) build collaborative partnerships with community leaders and violence prevention researchers to enhance the value of their clinical interventions.
To implement specific treatments to reduce children's risk of involvement with violence, it is recommended that pediatricians (1) take a thorough history of children's involvement with violence as an aggressor, a victim, a bystander, a nonviolent problem-solver, a participant in gender-role-related violence, and a witness to violence in the community; (2) educate children and their parents about the ways of reducing their risks by helping them to develop habits of thought and behavior patterns that lead to effective and nonviolent problem solving; (3) provide follow-up support for the changes children and their parents make to help prevent violence; and (4) engage in community outreach activities designed to change community norms about violence. By intervening early, systematically, and repeatedly in the lives of young children and their communities, pediatricians can help children develop the habits of thought they need to protect themselves from violence and to take active measures to prevent future involvement with violence.
Reducing Violent Injuries: Priorities for Pediatrician Advocacy
Authors: Dolins Judith Cohen , Christoffel Katherine Kaufer 
Published: December 2021

A basic framework for developing an advocacy plan must systematically break down the large task of policy development implementation into manageable components. The basic framework described in detail in this paper includes three steps:
• Setting policy objectives by narrowing the scope of policy, by reviewing policy options, and by examining options against selected criteria.
• Developing strategies for educating the public and for approaching legislative/regulatory bodies.
• Evaluating the effectiveness of the advocacy action plan as a process and as an agent for change.
To illustrate the variety of ways in which pediatricians can be involved in the policy process to reduce violent injuries among children and adolescents, we apply this systematic approach to three priority areas.
• Prohibiting the use of corporal punishment in schools is intended to curb the institutionalized legitimacy of violence that has been associated with future use of violence.
• Efforts to remove handguns from the environments of children and adolescents are aimed at reducing the numbers of firearm injuries inflicted upon and by minors.
• Comprehensive treatment of adolescent victims of assault is intended to decrease the reoccurrence of violent injuries.
Parents' and Children's Adiposity and Eating Style
Authors: Johnson Susan L. , Birch Leann L. 
Published: December 2021

Objective. To investigate children's ability to self-regulate energy intake and to determine whether individual differences in the precision of food intake regulation are related to children's anthropometric measures. We collected information pertaining to parental adiposity and dieting practices, as well as mothers' child-feeding practices. Of special interest was the degree of control imposed by mothers over their children's food intake. Our intent was to explore whether these variables might influence children's regulation of energy intake.
Subjects and setting. Seventy-seven 3-5-year-old children who attended a university preschool setting and their parents participated in this experiment.
Measurements and main results. Children completed controlled, two-part meals used to estimate their ability to adjust food intake in response to changes in caloric density of the diet. An eating index, reflecting children's precision in the ability to regulate energy intake, was correlated to children's anthropometric measures. These correlations provided evidence for a relation between children's body fat stores and their responsiveness to caloric density cues: Pearson correlation coefficients revealed that children with greater body fat stores were less able to regulate energy intake accurately. The best predictor of children's ability to regulate energy intake was parental control in the feeding situation: mothers who were more controlling of their children's food intake had children who showed less ability to self-regulate energy intake (r = -.67, P &lt; .0001).
Conclusions. These findings suggest that the optimal environment for children's development of self-control of energy intake is that in which parents provide healthy food choices but allow children to assume control of how much they consume.
Maternal and Child Health Bureau: Roots
Authors: Hutchins Vince L. 
Published: December 2021

The Maternal and Child Health Bureau has roots that go back over 80 years to the creation of the United States Children's Bureau on April 9, 1912, when President William Howard Taft approved an Act of Congress that created the Children's Bureau and directed it "to investigate and report on all matters pertaining to the welfare of children and child life among all classes of our people." This was the federal government's first recognition that it has a responsibility to promote the welfare of our nation's children. The Bureau's Chief was to be appointed by the President with the advice and consent of the Senate. Originally placed in the Department of Commerce and Labor, it was transferred to the newly formed Department of Labor in March, 1913.
The Children's Bureau was a logical sequel to several child-oriented social and public health activities of the late 19th century: the establishment of milk stations; concern with the spread of communicable disease after compulsory school attendance laws were passed; the movement to outlaw child labor; and, the opening of Settlement Houses. Lillian Wald, organizer of public health nursing, an ardent fighter against child labor, and the founder of the Henry Street Settlement in New York City, was the person who first suggested a federal Children's Bureau. A bill, with the support of President Theodore Roosevelt, was introduced in both houses of Congress in 1906 and annually during the next 6 years. It met with fierce opposition both from states which felt that the federal government was usurping their responsibility for the welfare of children and from those who feared that it would give federal employees the right to enter and regulate the homes of private citizens.
Classroom Behavior of Very Low Birth Weight Elementary School Children
Authors: Klebanov Pamela Kato , Brooks-Gunn Jeanne , McCormick Marie C. 
Published: December 2021

Objective. To examine the effect of birth weight on the classroom behavior of children during elementary school (N = 1120; mean age = 9.16 years).
Design. The study used an inception cohort.
Setting/Participants. Participants were from two previously studied multi-site cohorts: very low birth weight (VLBW) children referred to participating intensive care units and heavier low birth weight (HLBW) children from a stratified random sample of births in geographically defined regions. Teacher reports of children's behavior were obtained for 80% of those eligible. Information was obtained for 137 children who were extremely low birth weight (ELBW, ≤1000 g at birth), 223 who were other very low birth weight (OVLBW, 1001 to 1500 g), 434 who were HLBW (1501 to 2500 g), and 326 who were normal birth weight (NBW, &gt; 2500 g).
Main Outcome Measures. The child's teacher provided reports of language and attention in the classroom, behavior problems, and social competence.
Results. Comparisons were made among the four birth weight groups: ELBW, OVLBW, HLBW, and NBW. The ELBW children had lower attention and language skills, overall social competence, scholastic competence, and athletic ability than all other birth weight groups as measured by classroom teachers, even when controlling for neonatal stay, child's gender and ethnicity, and maternal education. All low birth weight (LBW) children had lower attention and language skills and scholastic competence and higher daydreaming and hyperactivity scores than NBW children. White children and children with more educated mothers generally had greater language and attention and social competence scores and fewer behavior problems; girls had fewer behavior problems than boys. Neonatal health was generally not associated with outcomes, except that healthier children had lower anxiety scores. The classroom behavior of LBW (&lt; 2500 g) children was rated by teachers as poor, even for children who had not failed a grade; LBW children who are on grade level still may be at risk for school problems. Differences between ELBW and NBW children persisted for overall language and attention and social competence scores even when additional controls were added for sociodemographic and familial variables. Of these variables, maternal depression and home environment were significantly associated with social competence scores.
Fluoroscopic Foley Catheter Removal of Esophageal Foreign Bodies in Children: Experience With 415 Episodes
Authors: Schunk Jeff E. , Harrison A. Marc , Corneli Howard M. , Nixon G. William 
Published: December 2021

Objective. This study sought to investigate the safety and efficacy of the fluoroscopic Foley catheter technique (FFCT) for removal of esophageal foreign bodies (EFBs) in children, and to identify factors associated with decreased success.
Design/Setting/Patients. An 11-year retrospective review of all pediatric patients undergoing the FFCT for removal of EFBs at a tertiary-care children's hospital was performed.
Results. Four-hundred and fifteen cases are reported. The median age was 29 months (range, 4 to 193); children ≤ 24 months accounted for 45% (185) of the cases. Of all episodes 86% (355) involved children without known esophageal pathology. Coins comprised 76% (316) of the EFBs.
The FFCT was successful in 91% (378) of the cases. In the 60 episodes involving children with underlying esophageal pathology, the technique had an 83% success rate compared to 92% in children without known pathology (P &lt; .05). There were 290 patients where the duration of impaction was known. The success rate was 96% if the duration was 3 days or less compared to 50% if the duration was longer (P &lt; .0001). Though the overall success in children ≤ 24 months was less than older children (88% vs 94%, P &lt; .05), this effect disappeared when corrected for duration of impaction.
Minor complications occurred in 2% of the episodes, and major complications were noted in 1%.
Conclusions. The FFCT appears to be a safe and effective method for removal of EFBs especially in children without underlying esophageal lesions and a duration of impaction ≤ 3 days. Major complications are rare.
False Allegations of Sexual Touching by Physicians in The Practice of Pediatrics
Authors: Silber Tomas J. 
Published: December 2021

It is normal for adolescents to be concerned about their bodies and harbor thoughts and worries about their sexuality.1 This can be manifested as anxiety during the physical examination, especially when the genital examination is performed.2-4 Because of this and the possibility of misinterpreting medical procedures such as breast and pelvic examinations, it has been suggested that male practitioners examine female adolescent patients in the presence of a chaperone. Studies involving chaperones use in office practice show a wide variation of approaches.5-7
Recently there has been an increasing recognition of sexual contact between physicians and their patients, both in the medical,8 psychiatric,9 and the lay literature,10 raising consciousness among the public about possible inappropriate sexual behavior by professionals.
Localized Intestinal Perforation
Authors: The' T. G. , Young M. , Rosser S. 
Published: December 2021

We read with pleasure Buchheit's1 article comparing the clinical findings in 21 preterm infants with localized intestinal perforation (LP) versus those of 21 preterm infants with intestinal perforation secondary to necrotizing enterocolitis (NEC). However, we would be grateful if the authors would clarify two points:
1. How are their 21 cases of LP different from the &gt;500 cases of idiopathic gastrointestinal perforation reviewed by Lloyd in 1979?2
2. The authors hypothesized that the placement of midthoracic umbilical artery catheters (UACs) would be a significant factor in the etiology of LP.