bluuebunny/crossref_metadata_2025_split huggingface dataset:
45,X/46,XY Mosaicism: Report of 27 Cases
Authors: Telvi Louise , Lebbar Aziza , Del Pino Olivia , Barbet Jacques Patrick , Chaussain Jean Louis 
Published: August 2004

Objectives.There exist substantial differences between prenatally and postnatally diagnosed cases of 45,X/46,XY mosaicism. Ninety percent of prenatally diagnosed cases show a normal male phenotype, whereas the postnatally diagnosed cases show a wide spectrum of phenotypes. This 10% risk of an abnormal outcome in prenatally diagnosed cases requires further attention. The purpose of the present study is to provide more information on the postnatally diagnosed 45,X/46,XY mosaicism cases. To date, only a few series have been reported. An accurate diagnosis in these patients is essential not only to their follow-up, but also to providing appropriate genetic counselling and subsequent prenatal diagnosis to their parents.Methods.The clinical, cytogenetic, endocrinologic, histologic and molecular biological findings of 27 patients with 45,X/46,XY mosaicism are analyzed.Results.The reported cases showed a wide spectrum of phenotypes as Turner syndrome, mixed gonadal dysgenesis (MGD), male pseudohermaphroditism (MPH) and apparently normal male. However, Ulrich-Turner stigmata were the most common features found in this series. Patients with MGD or MPH presented with various degrees of sex reversal such as hypospadias and/or abnormal internal genitalia. No correlation between the proportion of the 45,X/46,XY cell lines in the blood or the fibroblasts and the phenotype was found. Mild mental retardation was present in 4 of the patients and 2 patients showed signs of autism.Conclusions.Two major points are emphasized in this series: 1) the presence in 7 histologically analyzed streak gonads of a homogeneous 45,X chromosomal complement suggests that the invasion of the primitive genital ridge by a such a cell line may induce abnormal gonadal development; 2) 3 males, apparently normal at birth, developed late onset abnormalities such as dysgenetic testes leading to infertility, Ulrich-Turner stigmata, dysmorphic features, and mild mental retardation. These data indicate the importance of an accurate clinical and histologic evaluation of any patient presenting with 45,X/46,XY mosaicism.
Sterilization of Minors With Developmental Disabilities
Authors:   Committee on Bioethics
Published: August 2004

This Policy Statement was retired October 2012.
Sterilization of persons with developmental disabilities has often been performed without appropriate regard for their decision-making capacities, abilities to care for children, feelings, or interests. In addition, sterilization sometimes has been performed with the mistaken belief that it will prevent expressions of sexuality, diminish the chances of sexual exploitation, or reduce the likelihood of acquiring sexually transmitted diseases. A decision to pursue sterilization of someone with developmental disabilities requires a careful assessment of the individual's capacity to make decisions, the consequences of reproduction for the person and any child that might be born, the alternative means available to address the consequences of sexual maturation, and the applicable local, state, and federal laws. Pediatricians can facilitate good decision-making by raising these issues at the onset of puberty.
Growth of Infants Prenatally Exposed to Cocaine/Crack: Comparison of a Prenatal Care and a No Prenatal Care Sample
Authors: Richardson Gale A. , Hamel Sara C. , Goldschmidt Lidush , Day Nancy L. 
Published: August 2004

Objective.It has not been possible to draw firm conclusions about the effects of prenatal cocaine exposure because of methodologic problems involved in the conduct of this research. This study, designed to overcome some of these methodologic problems, is a prospective, longitudinal investigation of the effects of prenatal cocaine/crack exposure on neonatal growth in two samples, one with and one without prenatal care (PC).Methods.Women in the PC sample (n = 295) were interviewed at the end of each trimester about their use of cocaine, crack, alcohol, tobacco, marijuana, and other drugs. Women in the no prenatal care (NPC) sample (n = 98) were interviewed at delivery about their drug use during each trimester of pregnancy. In both samples, information was also obtained about sociodemographic, lifestyle, psychologic, and social support characteristics. Both samples consisted of women who were predominantly low income, single, and high school educated. Of the women, 48% in the PC sample were black; 81% in the NPC sample were black. Infants were examined during the postpartum hospital stay by project nurses who were blind to maternal substance use status.Results.Women in both samples who used cocaine/crack during pregnancy were older, had lower family incomes, and used more alcohol than did women who did not use cocaine/crack during pregnancy. In addition, women in the NPC sample were more likely to be black, less educated, gained less weight during pregnancy, and used more alcohol than did women in the PC sample, regardless of cocaine use. In both samples, cocaine/crack use during early pregnancy predicted reduced gestational age, birth weight, length, and head circumference, after controlling for the significant covariates of cocaine use. In a comparison of the samples, the offspring of the NPC/cocaine group were significantly smaller than were the offspring of the PC/no cocaine group, whereas the offspring of the PC/cocaine and NPC/cocaine groups did not differ.Conclusions.These results indicate that exposure to cocaine/crack during early pregnancy decreases the intrauterine growth of exposed offspring in women with and without PC. Each of the growth parameters was affected indicating symmetric growth retardation. The adequacy of PC was not a significant factor in determining the difference between cocaine-exposed and nonexposed infants. These samples are being followed throughout childhood to determine whether there are long-term effects of prenatal cocaine/crack exposure on growth.
The Changing Pattern of Hypernatremia in Hospitalized Children
Authors: Moritz Michael L. , Ayus J. Carlos 
Published: August 2004

Objectives.Past studies have revealed that hypernatremia occurs primarily in infants with diarrheal dehydration. With improved infant feeding practices and the advent of pediatric critical care medicine, the pattern of hypernatremia in children has likely changed. The purpose of this study was to evaluate the current pattern of hypernatremia in hospitalized children.Methods.Medical records were reviewed for 68 patients admitted to a large urban children's hospital during a 3-year period, all with a serum sodium greater than 150 mEq/L. The etiologies, predisposing factors, and morbidity and mortality associated with hypernatremia were evaluated.Results.The average patient age was 3.9 years (range, 1 day to 19.7 years), and the peak serum sodium concentration was 159 mEq/L (range, 151–184 mEq/L). Hypernatremia was hospital acquired in 60% of children. The majority of children (71%) were admitted for reasons other than hypernatremia. In 76% of the patients, inadequate fluid intake was the main cause of hypernatremia. Gastroenteritis contributed to the hypernatremia in only 20% (14 out of 68) of children. Eleven of these were infants &lt;1 year of age with hypernatremia on admission. Eighty-eight percent of patients (60 out of 68) suffered from neurologic impairment, critical illness, chronic disease, or prematurity before developing hypernatremia. The overall mortality was 16%. Patients in whom hypernatremia was not corrected had a significantly higher mortality than those in whom hypernatremia was corrected (4 out of 8 [50%] vs 7 out of 60 [12%]). Peak serum sodium was no different for survivors than nonsurvivors. No deaths were attributable to cerebral edema caused by correction of hypernatremia. Neurologic complications related to hypernatremia occurred in 15% of patients.Conclusions.Hypernatremia occurs in children of all ages, with the vast majority having significant underlying medical problems. Hypernatremia caused by gastroenteritis in infants has become much less common than previously reported. Hypernatremia is primarily a hospital-acquired disease, produced by the failure to administer sufficient free water to patients unable to care for themselves. Failure to correct hypernatremia may result in a high mortality rate.
Day Care Attendance in the First Year of Life and Illnesses of the Upper and Lower Respiratory Tract in Children With a Familial History of Atopy
Authors: Celedon Juan C. , Litonjua Augusto A. , Weiss Scott T. , Gold Diane R. 
Published: August 2004

Objective.To examine the relationship between day care attendance and illnesses of the upper and lower respiratory tract in the first year of life.Study Design.Prospective birth cohort study.Methods.Children (N = 498) who had at least 1 parent with a history of allergy or asthma were enrolled at birth and followed prospectively for the first year of life. A home visit at 2 to 3 months of age and bimonthly telephone questionnaires were used to obtain information on day care arrangements, home characteristics, respiratory symptoms, and physician-diagnosed illnesses of the upper and lower respiratory tract.Results.Day care attendance in the first year of life was associated with two or more doctor-diagnosed ear infections (OR: 2.4; 95% CI: 1.7–3.6), three or more parental reports of runny or stuffed nose (OR: 3.2; 95% CI: 1.9–5.5), a doctor's diagnosis of sinusitis (OR: 2.2; 95% CI: 1.1–4.2), and doctor-diagnosed lower respiratory illnesses (croup, bronchitis, bronchiolitis, and pneumonia; OR: 1.6; 95% CI: 1.0–2.4). For children attending day care, exposure to pets in day care, the presence of a rug or carpet in the area where the child slept in day care, and a nonresidential setting for day care all were independent predictors of two or more doctor-diagnosed ear infections.Conclusions.The results suggest that day care increases the risk of illnesses of the upper and lower respiratory tract in the first year of life for children with a familial history of atopy. Specific environmental exposures within day care, such as the presence of pets or having a rug or carpet in the area where children sleep, may increase the risk of recurrent ear infections in the first year of life among children with familial history of atopy who attend day care.
The Effect of Blood Transfusion Protocol on Retinopathy of Prematurity: A Prospective, Randomized Study
Authors: Brooks Steven E. , Marcus Dennis M. , Gillis Delores , Pirie Elizabeth , Johnson CRNI§; Maribeth H. , Bhatia Jatinder 
Published: August 2004

Objective.Controversy exists regarding the potential influence of anemia and blood transfusions on the rate of retinopathy of prematurity (ROP) in premature infants. A prospective, randomized, masked trial was performed to determine the influence of red blood cell transfusion protocol on ROP incidence and severity in a population of high-risk infants.Methods.A total of 50 infants with birth weights &lt;1251 g were divided randomly into two groups beginning on day of life 29. Group 1 (n = 24) received red cell transfusions during the 6-week study period, only if certain symptom-based guidelines were met. Group 2 (n = 26) received red cell transfusions to maintain the hematocrit level above 40% for the entire 6 weeks. Infants were monitored for ROP, growth, and associated morbidity. Serial measurements of serum glucose, lactate, ferritin, total iron-binding capacity, and iron were performed.Results.ROP occurred in 83% of infants in group 1, and 73% of infants in group 2. There were no statistically significant differences in ROP severity, intraventricular hemorrhage, bronchopulmonary dysplasia, necrotizing enterocolitis, or any of the laboratory values except hemoglobin (10.8 vs 13.2 g/dL) and hematocrit (33.9% vs 41.8%) between the groups. Combining data from both groups, there was no association between hemoglobin or hematocrit ratios and ROP incidence or severity.Conclusions.A transfusion policy aimed at limiting the amount of blood given to premature infants (symptom-based) during the neonatal period does not impart a significantly different risk for ROP or other associated conditions, than does a policy in which transfusions are given more liberally for replacement purposes.
Adherence to AAP Guidelines for Well-Child Care Under Managed Care
Authors: Byrd Robert S. , Hoekelman Robert A. , Auinger Peggy 
Published: August 2004

Objectives.To determine adherence to American Academy of Pediatrics (AAP) Recommendations for Preventive Pediatric Health Care in Monroe County, New York by individual patients and individual pediatricians under managed care practice and to compare adherence-to-recommendations rates for privately insured and publicly funded managed care patients.Study Designs and Methods.Using claims data for children 0 through 18 years of age cared for by pediatricians, we compared adherence to well-child care (WCC) visit recommendations for 130 572 children enrolled in a privately insured managed care system during 1992, 1993, and 1994 to 17 586 children insured by a publicly funded, Medicaid-managed care system during 1994 and 1995. Criteria for WCC visit adherence were based on 1991 AAP guidelines of 19 office visits from birth through 18 years of age. Adherence-to-recommendations rates by individual pediatricians also were determined.Results.Despite complete financial coverage of WCC visits (with no co-payment or deductible charges) by both insurance systems, strict adherence to AAP guidelines for WCC visits was low. Only 46% of privately insured and 35% of publicly funded children received all the recommended visits during the study period. During the same period, 17% of privately insured and 35% of publicly funded managed care patients received no WCC. There was little difference in the rate of full WCC visit adherence by age in either system with the rates ranging in privately insured patients from 49% in infants (&lt;2 years of age) to 47% in adolescents (12 through 18 years of age) and ranging in publicly funded patients from 36% to 34% in these two age groups, respectively. Only 2% of privately insured infants had no record of WCC compared with 29% of adolescents. This contrasted with 12% of infants and 54% of adolescents who were publicly funded. Of pediatricians, &lt;5% achieved 100% adherence to AAP guidelines for their patients (privately insured or publicly funded). Pediatricians completed an average of 52% of the recommended visits with their publicly funded patients and 68% of the recommended visits with their privately insured patients.Conclusions.WCC visits were underutilized for children in both managed care systems. Children of parents who have low incomes presumably could benefit greatest by preventive visits, but these children were less likely to receive the recommended number of WCC visits. Finding ways to increase the number of WCC visits that all children make is a major challenge, as is conducting studies that prove their worth.
Oral Rehydration Solution for Acute Diarrhea Prevents Subsequent Unscheduled Follow-up Visits
Authors: Duggan Christopher , Lasche Jack , McCarty Martha , Mitchell Kathleen , Dershewitz Robert , Lerman Stephen J. , Higham Margaret , Radzevich Annette , Kleinman Ronald E. 
Published: August 2004

Background.Oral rehydration solutions (ORS) for the treatment of acute diarrhea remain an underutilized therapy in the United States, despite multiple clinical trials confirming their efficacy and safety. Economic barriers to their use have been identified.Objective.To determine whether providing ORS to patients at the time of their office visit for acute diarrhea can increase ORS utilization and reduce unscheduled follow-up visits.Design.Randomized, controlled clinical trial.Setting.Seven health centers of a large health maintenance organization.Participants.Children (N = 479) 0 to 60 months of age with acute diarrhea (at least three watery or loose stools in the previous 24 hours for ≤7 days).Intervention.Prescription for 2 quarts of ORS filled for free at on-site pharmacy plus written instructions versus written instructions alone.Primary Outcome Measures.Self-reported use of ORS; unscheduled follow-up visits in office, urgent care, and/or emergency department setting.Results.Subjects in the intervention group were significantly more likely to use ORS after the initial office visit (85% vs 71%; RR: 1.19; 95% CI: 1.08–1.32). Of the standard treatment group subjects, 40 (17.3%) sought unscheduled follow-up care for diarrhea versus 27 (10.9%) of the intervention group subjects (RR: 0.63; 95% CI: 0.40–0.99). Subjects seeking unscheduled follow-up care tended to younger (15.7 vs 19.4 months old), have more stools (7.1 vs 6.2 stools), and more vomiting episodes (4.1 vs 3.0) in the 24 hours before initial evaluation than those not seeking unscheduled follow-up care. Multivariate analysis showed that randomization to the intervention group was associated with a 25% reduction in unscheduled follow-up visits for acute diarrhea.Conclusions.Providing ORS to families at the time of their office visit for acute diarrhea is associated with a significant increase in ORS use and substantially reduces the need for unscheduled follow-up visits. Health maintenance organizations should consider routine provision of ORS to children presenting with acute diarrhea.
Screening for Domestic Violence in the Community Pediatric Setting
Authors: Siegel Robert M. , Hill Teresa D. , Henderson Vicki A. , Ernst Heather M. , Boat Barbara W. 
Published: August 2004

Objective.Children exposed to domestic violence (DV) can experience a variety of adverse effects such as behavior disorders, developmental delay, and child abuse. Recently, the American Academy of Pediatrics recommended that all pediatricians incorporate screening for DV as a part of anticipatory guidance. To date, however, there is little information on how likely women are to disclose DV or whether there are any benefits to screening in the pediatric office setting. The purpose of our pilot study was to gain an understanding of whether screening for DV in the pediatric office setting could be helpful to abused women and their children.Methods.During a 3-month period, 92% of the women who accompanied their children for a well-child visit to a hospital-based suburban pediatrician were asked about violence in the home with a six-question screening tool.Results.Of the 154 women screened, 47 (31%) revealed DV at some time in their lives. Twenty-five women (17%) reported DV within the past 2 years and were reported to the mandated state agency. There were 5 episodes of child abuse reported of which two had not been previously reported. Interestingly, there were 5 women injured during their most recent pregnancy and who had separated from their abusive partner, but no legal action had been taken to protect them from their partner's return. There was no significant difference in the incidence of DV reported in families with Medicaid (37%) versus private insurance (20%). Before routine DV screening in our office, only one previous DV report had been made in 4 years.Conclusions.Our preliminary results suggest that many women will reveal DV when screened in the pediatric office setting. Also, there is a subgroup of women, those with young children who have recently separated from their partners, who may particularly benefit from DV screening.
Neonatal Screening for Hearing Disorders in Infants at Risk: Incidence, Risk Factors, and Follow-up
Authors: Meyer Christiane , Witte Jan , Hildmann Agnes , Hennecke Karl-Heinz , Schunck Karl-Ulrich , Maul Kerstin , Franke Ute , Fahnenstich Hubert , Rabe Heike , Rossi Rainer , Hartmann Sabine , Gortner Ludwig 
Published: August 2004

Objective.To determine the incidence and risk factors for hearing disorders in a selected group of neonates and the feasibility of selective hearing screening.Settings.Multicenter prospective trial at five centers in Germany.Methods.Enrollment criteria: in addition to previously defined risk factors by the Joint Committee on Infant Hearing (family history of hearing loss, in utero infections, craniofacial anomalies, birth weight &lt;1500 g, critical hyperbilirubinemia, ototoxic medications, bacterial meningitis, postnatal asphyxia, mechanical ventilation &gt;5 days, stigmata, or syndromes associated with hearing loss), the impact of maternal drug abuse, birth weight &lt;10th percentile, persistent pulmonary hypertension, and intracranial hemorrhage more than or equal to grade III or periventricular leukomalacia on infant hearing were evaluated. The screening procedure was performed by automated auditory brainstem response (A-ABR; ALGO 1-plus; Natus Med Inc, San Carlos, CA). Statistics: univariate analyses of risk factors versus A-ABR results and a multivariate regression analysis were used; additionally, the total test time was recorded.Results.Seven hundred seventy recordings from 777 infants enrolled consecutively constitute the basis of this analysis. Mean gestational age was 33.8 ± 4.3 weeks, birth weight 2141 ± 968 g; 431 infants being male and 339 female; 41 (5.3%) infants exhibited pathologic A-ABR results (16 bilateral and 25 unilateral). Meningitis or sepsis, craniofacial malformations, and familial hearing loss were independent significant risk factors. Median total test time was 25 minutes. Follow-up examinations in 31 infants revealed persistent hearing loss in 18 infants (13 infants sensorineural, 5 from mixed disorders), 7 requiring amplification.Conclusion.Hearing screening in high-risk neonates revealed a total of 5% of infants with pathologic A-ABR (bilateral 2%). Significant risk factors were familial hearing loss, bacterial infections, and craniofacial abnormalities. Other perinatal complications did not significantly influence screening results indicating improved perinatal handling in a neonatal population at risk for hearing disorders.
Potential Mechanisms for Failure to Eradicate Group A Streptococci From the Pharynx
Authors: Gerber Michael A. , Tanz Robert R. , Kabat William , Bell Gillian L. , Siddiqui BLT∥; Parveen N. , Lerer Trudy J. , Lepow Martha L. , Kaplan Edward L. , Shulman Stanford T. 
Published: August 2004

Objective.To investigate the relative efficacy of orally administered cefadroxil and penicillin V in the treatment of group A streptococcal (GABHS) pharyngitis and the mechanism(s) responsible for failure of antimicrobial therapy to eradicate GABHS from the pharynx.Study Design.A prospective, randomized clinical trial was conducted in four pediatric offices in which 462 patients with acute pharyngitis and positive culture for GABHS were randomly assigned to receive cefadroxil (n = 232) or penicillin V (n = 230).Results.Bacteriologic treatment success rates for patients in cefadroxil and penicillin groups were 94% and 86%, respectively. However, among patients classified clinically as likely to have bona fide GABHS pharyngitis, there was no difference in bacteriologic treatment success rates in cefadroxil and penicillin groups (95% and 94%, respectively). Among patients classified clinically as likely to be streptococcal carriers, bacteriologic treatment success rates in cefadroxil and penicillin groups were 92% and 73%, respectively. The presence of β-lactamase and/or bacteriocin-producing pharyngeal flora had no consistent effect on bacteriologic eradication rates among patients in either penicillin or cefadroxil treatment groups or among patients classified as having either GABHS pharyngitis or streptococcal carriage.Conclusions.Neither β-lactamase nor bacteriocin produced by normal pharyngeal flora are related to bacteriologic treatment failures in GABHS pharyngitis. Cefadroxil seems to be more effective than penicillin V in eradicating GABHS from patients classified as more likely to be streptococcal carriers. However, among patients we classified as more likely to have bona fide GABHS pharyngitis, the effectiveness of cefadroxil and penicillin V seems to be comparable.