bluuebunny/crossref_metadata_2025_split huggingface dataset:
Autoimmunity in Wiskott-Aldrich Syndrome: Risk Factors, Clinical Features, and Outcome in a Single-Center Cohort of 55 Patients
Authors: Dupuis-Girod Sophie , Medioni Jacques , Haddad Elie , Quartier Pierre , Cavazzana-Calvo Marina , Le Deist Françoise , de Saint Basile Geneviève , Delaunay Jean , Schwarz Klaus , Casanova Jean-Laurent , Blanche Stephane , Fischer Alain 
Published: August 2004

Objectives. To evaluate the occurrence of autoimmune and inflammatory complications in Wiskott-Aldrich syndrome (WAS) and to determine risk factors and the prognosis of such complications with the aim of improving the definition of treatment options.Methods. We reviewed the records of 55 patients with WAS evaluated at Necker-Enfants Malades Hospital (Paris) from 1980 to 2000.Results. Forty patients (72%) had at least 1 autoimmune or inflammatory complication. Autoimmune hemolytic anemia was detected in 20 cases (36%); in all cases, onset occurred before the age of 5 years. Other complications included neutropenia (25%), arthritis (29%), skin vasculitis (22%), cerebral vasculitis (7%), inflammatory bowel disease (9%), and renal disease (3%). The median survival of the entire population was 14.5 years. Two autoimmune complications and 1 biological factor were predictive of a poor prognosis in this population: autoimmune hemolytic anemia, severe thrombocytopenia recurring after splenectomy, and high serum immunoglobulin M (IgM) levels before splenectomy. Autoimmune hemolytic anemia was significantly more observed in patients with high serum IgM level.Conclusions. High serum IgM concentration before splenectomy was identified as a risk factor for autoimmune hemolytic anemia; however, it must be confirmed. Autoimmune hemolytic anemia and severe thrombocytopenia recurring after splenectomy were 2 indicators of a poor prognosis. Those results suggest that patients with WAS and IgM levels more than mean + 2 standard deviations before splenectomy should be placed under strict surveillance. Furthermore, severe autoimmune complications should lead, as early as possible, to hematopoietic stem cell transplantation using the best available donor.
Isolated Hepatocyte Transplantation in an Infant With a Severe Urea Cycle Disorder
Authors: Horslen Simon P. , McCowan Timothy C. , Goertzen Timothy C. , Warkentin Phyllis I. , Cai Hung Bo , Strom Stephen C. , Fox Ira J. 
Published: August 2004

Objective. Transplantation of isolated hepatocytes in animal models has been shown to correct inborn errors of metabolism. Based on these studies and our experience with hepatocyte transplantation in a child with Crigler-Najjar syndrome, isolated hepatocyte transplantation was performed to attempt metabolic reconstitution in a male infant with severe ornithine transcarbamylase (OTC) deficiency.Methods. An infant with an antenatal diagnosis of OTC deficiency was managed intensively to prevent hyperammonemia. Isolated hepatocytes were obtained by collagenase perfusion of donated livers not used for transplantation. Hepatocytes were infused in batches over the first 4 weeks of life via an umbilical venous catheter positioned in the portal vein. Immunosuppression consisted of tacrolimus and corticosteroids.Results. Over 4 billion viable hepatocytes were transplanted during the first 3.5 weeks of life. A period of metabolic stability was achieved between days 20 and 31 during which normal protein intake was tolerated while phenylbutyrate was weaned. During this time, plasma ammonia and glutamine remained within normal limits. Hyperammonemia reappeared abruptly on day 31 of life. Protein tolerance diminished to baseline; metabolic stability was subsequently reattained only following successful liver transplantation at 6 months of age.Conclusions. Isolated hepatocyte transplantation appeared to result in temporary relief of hyperammonemia and protein intolerance attributable to OTC deficiency. The metabolic stability achieved was lost after 11 days presumably because of rejection of the transplanted cells because of insufficient immunosuppression. Future attempts at isolated hepatocyte transplantation for inborn errors of metabolism in humans should include adequate immunosuppression and a liver biopsy as a means of proving hepatocyte engraftment and function.
Adult Patient Visits to Children’s Hospital Emergency Departments
Authors: Bourgeois Florence T. , Shannon Michael W. 
Published: August 2004

Background. Adults frequently seek medical services in children’s hospital emergency departments (CHEDs), and are required to be admitted to CHEDs under the provisions of the Emergency Medical Transfer and Active Labor Act (EMTALA), which requires medical evaluation and stabilization of every patient who presents to an emergency department. In recent years visits by adults to CHEDs appear to have increased.
Objective. There were 3 objectives to the current study: 1) to examine secular trends in the number of adult patients visiting CHEDs, 2) to determine if perceived increases are related to the implementation of EMTALA, and 3) to examine the characteristics, evaluation, and disposition of adult patients presenting for first-time visits to a CHED.
Methods. A database of all visits to an urban CHED between 1992 and 2002 was queried to collect information on adult patients (22 years or older). New adult patients were identified based on the assignment of new medical record numbers. The medical records of all adult patients presenting during the 1-year interval before and after the institution’s full implementation of EMTALA were reviewed and relevant data collected.
Results. Over the study period, there were 501 033 patient visits to the CHED. Of these, 5512 (1.1%) were by adult patients, which included 536 (9.7%) new adult patients. Using the χ2, test we found a significant increase in the total number of adult visits and the number of new adult visits, particularly after the implementation of EMTALA. The mean age of the new adult patients was 34.9 ± 11.9 years. Their most frequent chief complaints were injuries (24.4%), cardiac-related problems (7.6%), and syncope (6.7%). A total of 427 (79.7%) of the new adult patients were treated and released, 81 (15.1%) were transferred to an outside hospital for additional care, and 15 (2.8%) were admitted to our hospital. There were no significant differences between the new adult populations in 1997 and 1999. Comparing new and established adult populations in 1999, the population of new adults was significantly older (28.1 ± 6.8 vs 34.9 ± 11.9 years) and more likely to present with injuries or syncopal episodes. Among the total cohort of new adult patients in the study, chest pain also occurred at a significantly higher rate compared with established adults (6.7% vs 3.8%).
Conclusions. Adult visits to CHEDs appear to be increasing in frequency in association with the implementation of EMTALA regulations. It is therefore essential that physicians staffing CHEDs be properly trained in the stabilization of common adult medical emergencies. We recommend that the language of EMTALA be revised to allow adult patients with nonemergent problems to be directly referred to adult emergency departments, which are more appropriate than CHEDs.
The Risks of Adverse Neonatal Outcome Among Preterm Small for Gestational Age Infants According to Neonatal Versus Fetal Growth Standards
Authors: Zaw Win , Gagnon Robert , da Silva Orlando 
Published: August 2004

Objective. To evaluate neonatal and fetal growth standards in determining the impact of small for gestational age (SGA) on neonatal mortality and morbidity.Design. A hospital-based cohort study of infants born in a regional tertiary care center and admitted to the neonatal intensive care unit.Setting and Participants. A total of 1267 singleton neonates of &lt;34 weeks gestational age, without any congenital anomalies, born between January 1, 1993 and December 31, 2001.Outcome Measures. Each outcome variable including mortality, respiratory distress syndrome, bronchopulmonary dysplasia, intraventricular hemorrhage (IVH), periventricular leukomalacia, and necrotizing enterocolitis was related to growth status as defined by fetal and neonatal growth standards after adjustment for potential confounders.Results. The number of SGA infants was 11.6% (n = 147) of the study population according to neonatal growth standards, but it was increased to 23.3% (n = 295) when fetal growth standards were used. According to fetal growth standards, when SGA was compared with appropriate for gestational age infants, it was associated with an increased risk of respiratory distress syndrome (odds ratio [OR] 1.40; 95% confidence interval [CI] 1.00–1.95), bronchopulmonary dysplasia (OR 2.18; 95% CI 1.33–3.59), IVH (OR 1.67; 95% CI 1.13–2.45), and retinopathy of prematurity (OR 3.88; 95% CI 2.33–6.48). However, only neonatal mortality (OR 3.64; 95% CI 1.64–8.09), retinopathy of prematurity (OR 5.38; 95% CI 2.87–10.90), and necrotizing enterocolitis (OR 2.47; 95% CI 1.21–5.07) were positively associated with SGA when using neonatal growth standards.Conclusions. Compared with the neonatal growth standards, the fetal growth standards are better in identifying increased risk of respiratory morbidity and IVH among preterm SGA infants.
Counting the Shots: A Model for Immunization Screening and Referral in Nonmedical Settings
Authors: Rickert Donna L. , Shefer Abigail M. , Rodewald Lance E. , McCauley Mary M. 
Published: August 2004

Background. Clinics of the Special Supplemental Nutrition Program for Women, Infants, and Children (WIC) have become important partners in efforts to improve vaccination coverage in low income children. However, the time required to assess all antigens in each child’s vaccination record may exceed the capacity of many of these clinics. Seeking a solution, experts recommended assessing up-to-date (UTD) status only for the diphtheria-tetanus-acellular-pertussis (DTaP) vaccine and treating this as a proxy measure for all vaccines in the childhood schedule. Whether this single vaccine screening method represents an acceptable alternative to the traditional multiple-vaccine method as a basis for improving overall immunization coverage levels in this vulnerable population has not been demonstrated.Objective. To evaluate the validity of the proposed simplified method for assessing immunization status in a nationally representative population of infants and children who had ever been enrolled in WIC before 35 months old.Methods. This was a cross-sectional analysis of the 2000 National Immunization Survey representing children ages 3 to 24 months who had ever been enrolled in WIC. For the 6277 children in the study population, we compared personal records of completion status for DTaP with personal records of completion status for all immunizations appropriate for age in the combination 4:3:1:3 schedule to see which of the 2 (single vs multiple screening) methods would better predict the child’s true (provider-reported) status for the 4:3:1:3 series. The main outcome measures were the comparative sensitivity, specificity, and overall test efficiency of the 2 methods in correctly identifying underimmunized WIC children.Results. Completion status for DTaP was less sensitive than completion status for all vaccinations in correctly identifying truly underimmunized children (sensitivity = 70% and 77%, respectively). However, it was more specific in correctly identifying children who were truly UTD for age (specificity = 86% and 82%, respectively). The 2 methods were essentially identical with respect to overall test efficiency (82% and 81% for DTaP assessment and assessment of all vaccines, respectively).Conclusions. Given limited resources to do immunization screening and referral in nonmedical settings such as WIC, simplifying the process by using DTaP from the personal vaccination record as a proxy for the 4:3:1:3 series is a viable option. Loss in sensitivity may well be offset by gains in the capacity of WIC clinics to screen more children.
Infants With Bilirubin Levels of 30 mg/dL or More in a Large Managed Care Organization
Authors: Newman Thomas B. , Liljestrand Petra , Escobar Gabriel J. 
Published: August 2004

Objective. To describe the incidence, etiology, treatment, and outcome of newborns with total serum bilirubin (TSB) levels ≥30 mg/dL (513 μmol/L).Design. Population-based case series.Setting. Eleven Northern California Kaiser Permanente Medical Care Program hospitals and 1 affiliated hospital.Patients. Eleven infants with TSB levels of ≥30 mg/dL in the first 30 days after birth, identified using computer databases from a cohort of 111 009 infants born 1995–1998.Outcome Measures. Clinical data from the birth hospitalization, rehospitalization, and outpatient visits in all infants; psychometric testing at age 5 (N = 3), neurologic examinations by child neurologists at age 5 (N = 3), or primary care providers (N = 7; mean age: 2.2 years); Parent Evaluation of Developmental Status (N = 8; mean age: 4.2 years).Results. Maximum TSB levels of the 11 infants ranged from 30.7 to 45.5 mg/dL (525 μmol/L to 778 μmol/L; mean: 34.9 mg/dL [597 μmol/L]). Four were born at 35 to 36 weeks gestation, and 7 were exclusively breastfed. Two had apparent isoimmunization; the etiology for the other 9 remained obscure, although only 4 were tested for glucose-6-phosphate dehydrogenase deficiency and 1 was bacteremic. None had acute neurologic symptoms. All received phototherapy and 5 received exchange transfusions. One infant died of sudden infant death syndrome; there was no kernicterus at autopsy. Two were lost to follow-up but were neurologically normal when last seen for checkups at 18 and 43 months. One child was receiving speech therapy at age 3. There were no significant parental concerns or abnormalities in the other children.Conclusions. In this setting, TSB levels ≥30 mg/dL were rare and generally unaccompanied by acute symptoms. Although we did not observe serious neurodevelopmental sequelae in this small sample, additional studies are required to quantify the known, significant risk of kernicterus in infants with very high TSB levels.
Psychosocial Health Among Young Victims and Offenders of Direct and Indirect Bullying
Authors: van der Wal Marcel F. , de Wit Cees A. M. , Hirasing Remy A. 
Published: August 2004

Objective. To assess the association between bullying (both directly and indirectly) and indicators of psychosocial health for boys and girls separately.
Study Design. A school-based questionnaire survey of bullying, depression, suicidal ideation, and delinquent behavior.
Setting. Primary schools in Amsterdam, The Netherlands.
Participants. A total of 4811 children aged 9 to 13.
Results. Depression and suicidal ideation are common outcomes of being bullied in both boys and girls. These associations are stronger for indirect than direct bullying. After correction, direct bullying had a significant effect on depression and suicidal ideation in girls, but not in boys. Boy and girl offenders of bullying far more often reported delinquent behavior. Bullying others directly is a much greater risk factor for delinquent behavior than bullying others indirectly. This was true for both boys and girls. Boy and girl offenders of bullying also more often reported depressive symptoms and suicidal ideation. However, after correction for both sexes only a significant association still existed between bullying others directly and suicidal ideation.
Conclusions. The association between bullying and psychosocial health differs notably between girls and boys as well as between direct and indirect forms of bullying. Interventions to stop bullying must pay attention to these differences to enhance effectiveness.
Smoking During Pregnancy and Newborn Neurobehavior
Authors: Law Karen L. , Stroud Laura R. , LaGasse Linda L. , Niaura Raymond , Liu Jing , Lester Barry M. 
Published: August 2004

Objective. This was a prospective study of the effects of maternal smoking during pregnancy on newborn neurobehavior, including dose-response relationships using self-report and a bioassay of nicotine exposure.Methods. The sample included 27 nicotine exposed and 29 unexposed full-term newborn infants with no medical problems from comparable social class backgrounds. Mothers were excluded for using illegal drugs during pregnancy, using antidepressant medication, or if they consumed &gt;3 alcoholic drinks per month. Nicotine exposure was determined by maternal self-report and cotinine in maternal saliva. The NICU Network Neurobehavioral Scale (NNNS) was administered by masked examiners in hospital to measure neurobehavioral function. NNNS scores were compared between nicotine-exposed and -unexposed groups including adjustment for covariates. Dose-response relationships with NNNS scores were computed for maternal salivary cotinine and maternal report of number of cigarettes per day during pregnancy.Results. After adjustment for covariates, the tobacco-exposed infants were more excitable and hypertonic, required more handling and showed more stress/abstinence signs, specifically in the central nervous system (CNS), gastrointestinal, and visual areas. Dose-response relationships showed higher maternal salivary cotinine values related to more stress/abstinence signs (r = .530) including CNS (r = .532) and visual stress (r = .688) and higher excitability scores (r = .617). Cigarettes per day during pregnancy was related to more stress/abstinence signs (r = .582) including CNS (r = .561) and visual stress (r = .640).Conclusions. These findings suggest neurotoxic effects of prenatal tobacco exposure on newborn neurobehavior. Dose-response relationships could indicate neonatal withdrawal from nicotine. Research directed at understanding the effects of cigarette smoking during pregnancy on infants can lead to improved public health outcome.
The Effect of a Parenting Education Program on the Use of Preventive Pediatric Health Care Services Among Low-Income, Minority Mothers: A Randomized, Controlled Study
Authors: El-Mohandes Ayman A. E. , Katz Kathy S. , El-Khorazaty M. Nabil , McNeely-Johnson Doris , Sharps Phyllis W. , Jarrett Marian H. , Rose Allison , White Davene M. , Young Michal , Grylack Larry , Murray Kennan D. B. , Katta Pragathi S. , Burroughs Melissa , Atiyeh Ghassan , Wingrove Barbara K. , Herman Allen A. 
Published: August 2004

Objective. To determine if a community-based intervention program focusing on parenting education will have an impact on preventive health care utilization behaviors among low-income, minority mothers in Washington, DC.
Design. The experimental design was a randomized, controlled study in which 286 mother-infant dyads were assigned to either the standard social services (control) group or to the intervention group. Women and their newborn infants were recruited during the immediate postpartum period in 4 Washington, DC, hospital sites from April 1995 to April 1997. The year-long multicomponent intervention included home visits and hospital-based group sessions in addition to the standard social services available at the hospital sites. A total of 286 postpartum women with inadequate prenatal care were assigned randomly to the control or the intervention group. Women and their infants were followed for 1 full year. Outcome measures included usage of preventive health care services including well care infant visits and adherence to immunization schedules during the first year of the infant’s life.
Results. Infants in the intervention group initiated well care at an earlier age than controls (by 6 weeks, 62.5% vs 50% had received their first well infant visit). Infants in the intervention group had more frequent well visits (by 12 months of age, 3.5 vs 2.7 visits). Multivariate analyses showed infants in the intervention group to be more likely to complete their scheduled immunizations (by 9 months, odds ratio = 2.2, 95% confidence interval: 1.09–4.53). Those in the intervention group with more frequent contacts (30+ visits) with study personnel were most likely to have followed age-appropriate immunization schedules when compared with controls (at 9 months odds ratio = 3.63, 95% confidence interval: 1.58–8.33).
Conclusions. It is possible to influence health care usage patterns of high-risk minority populations through public health interventions that are global in their perspective. Focusing on parental knowledge and beliefs regarding health-related issues and life skills in a self-efficacy model is associated with improved usage of infant health care resources.
Evaluation of Cytomegalovirus Infections Transmitted via Breast Milk in Preterm Infants With a Real-Time Polymerase Chain Reaction Assay
Authors: Yasuda Ayako , Kimura Hiroshi , Hayakawa Masahiro , Ohshiro Makoto , Kato Yuichi , Matsuura Onrai , Suzuki Chizuko , Morishima Tsuneo 
Published: August 2004

Objective. Preterm infants are at greater risk of symptomatic cytomegalovirus (CMV) infection than term infants. Breast milk is the main source of perinatal CMV infections. This study evaluated the kinetics of CMV load in breast milk and the rate of postnatal CMV transmission via breast milk from mothers to their preterm infants.Methods. This was a prospective study of 30 mothers and their 43 preterm infants. The infants either had a gestational age of &lt;34 weeks or weighed &lt;2000 g at birth. Breast milk, serum, and urine samples were collected every 2 weeks until discharge, and screened for CMV infection using a real-time PCR assay. Most of the breast milk had been preserved at −20°C before feeding to the preterm infants.Results. Twenty-four mothers (24 of 30, 80%), who had 34 preterm infants, were CMV immunoglobulin G positive. Twenty-one (87.5%) of the 24 seropositive mothers, who had 30 preterm infants, had detectable CMV deoxyribonucleic acid (DNA) in breast milk during the study period. Most breast milk became positive for CMV DNA 2 weeks after delivery. Viral DNA copy numbers increased until they peaked at 4 to 6 weeks. Afterward, the CMV DNA copy numbers decreased. Of the 30 infants who were fed CMV DNA-positive breast milk, CMV infection was confirmed in 3 infants. However, they had no clinical symptoms of CMV infection.Conclusions. Despite the high rate of CMV DNA in breast milk, symptomatic infections in the preterm infants did not occur. These results might be associated with the method of breast milk preservation and the population we studied. CMV infections transmitted via breast milk feeding did not have much impact on preterm infants in our institutes.