bluuebunny/crossref_metadata_2025_split huggingface dataset:
Central Precocious Puberty in Girls: An Evidence-Based Diagnosis Tree to Predict Central Nervous System Abnormalities
Authors: Chalumeau Martin , Chemaitilly Wassim , Trivin Christine , Adan Luis , Bréart Gérard , Brauner Raja 
Published: August 2004

Objective. To identify predictors of central precocious puberty (CPP) that reveal central nervous system (CNS) abnormalities in girls with CPP.
Methods. A retrospective cohort study was conducted of all girls younger than 8 years with breast development related to CPP, seen between 1982 and 2000, in a university pediatric hospital in Paris, France. For a pilot population (186 idiopathic, 11 revealing CNS abnormalities), the accuracy of the Lawson Wilkins Pediatric Endocrine Society recommendations were evaluated. Potential clinical, radiological, and biological predictors of CNS abnormalities were assessed by univariate and multivariate analyses. A diagnosis tree aiming for 100% sensitivity for the detection of CNS abnormalities was constructed and was tested on a validation population (39 idiopathic, 3 revealing CNS abnormalities).
Results. Applying the Lawson Wilkins Pediatric Endocrine Society recommendations, 2 of 11 girls with CPP that revealed CNS abnormalities would not have been considered to require brain imaging. Independent predictors of CNS abnormalities were age at onset of puberty &lt;6 years (adjusted odds ratio [AOR]: 6.7; 95% confidence interval [CI]: 1.5–29), lack of pubic hair at diagnosis (AOR: 7.7; 95% CI: 1.8–33), and estradiol &gt;110 pmol/L (AOR: 4.1, 95% CI: 1.0–17). The diagnosis tree that was constructed on the basis of these predictors had 100% sensitivity and 56% specificity for the validation population.
Conclusion. The identification of girls who have CPP and require cerebral imaging seems possible on the basis of validated, simple, and reproducible predictors: age and estradiol. However, this process needs to be tested on other populations.
Neonatal Antibody Titers Against Varicella-Zoster Virus in Relation to Gestational Age, Birth Weight, and Maternal Titer
Authors: van der Zwet Wil C. , Vandenbroucke-Grauls Christina M.J.E. , van Elburg Ruurd M. , Cranendonk Anneke , Zaaijer Hans L. 
Published: August 2004

Objective. Varicella-zoster virus (VZV) can cause severe disease in premature neonates. The fetus receives protective maternal VZV-immunoglobulin G (IgG) mainly in the third trimester of pregnancy. Therefore, premature neonates are considered at risk for VZV infection. Administration of varicella-zoster immunoglobulin (VZIG) within 96 hours after exposure effectively prevents severe illness in susceptible patients. The objectives of this study were to define the major determinants of the neonatal VZV-IgG titer and to determine the half-life of transplacentally acquired VZV-IgG. Guidelines provided by the Centers for Disease Control and Prevention for the use of VZIG in (premature) neonates were evaluated.
Methods. VZV-IgG titers were measured in sera of 221 neonates and 43 mothers using a quantitative enzyme-linked immunosorbent assay. In 27 neonates, VZV-IgG titers were followed for up to 14 weeks.
Results. In a linear regression model, the maternal antibody titer was the major determinant of the neonatal titer (β = 0.89); gestational age was only of minor importance (β = 0.18). The median half-life of VZV-IgG in neonates was 25.5 days (range: 14.6–76.0 days). In the first weeks of life, major fluctuations of the VZV-IgG titer occurred in &gt;50% of the neonates. The predictive value of Centers for Disease Control and Prevention guidelines for identification of neonates who should receive VZIG in case of exposure to VZV was poor: positive and negative predictive values were 0.80 and 0.43, respectively.
Conclusions. The neonatal VZV-IgG titer is predominantly predicted by the maternal VZV-IgG titer, whereas birth weight and gestational age are much less predictive than previously reported.
Gastroesophageal Reflux and Apnea of Prematurity: No Temporal Relationship
Authors: Peter Corinna S. , Sprodowski Nadine , Bohnhorst Bettina , Silny Juri , Poets Christian F. 
Published: August 2004

Objective. A relationship between gastroesophageal reflux (GER) and apnea of prematurity (AOP) has long been suspected but is difficult to prove because most GER in this age group is nonacidic and thus undetectable by pH monitoring, the current standard for GER detection. The new multiple intraluminal impedance (MII) technique allows pH-independent reflux detection via changes in impedance caused by a liquid bolus inside the esophagus. We used this technique to investigate whether there is a temporal relationship between GER and AOP and whether GER occurs predominantly before a cardiorespiratory (CR) event.Methods. Nineteen infants with AOP (median gestational age at birth: 30 weeks; range: 24–34; age at study: 26 days [13–93]) underwent 20 6-hour recordings of MII, breathing movements, nasal airflow, electrocardiogram, pulse oximeter saturation, and pulse waveforms. MII signals were analyzed, independent of CR signals, for reflux episodes (RE), defined as a fall in impedance in at least the 2 most distal channels. CR signals were analyzed for CR events, ie, apneas of ≥4-second duration, desaturations to ≤80%, and falls in heart rate to ≤100/min. A temporal relationship between an RE and a CR event was considered present if both commenced within 20 seconds of each other.Results. There were 2039 apneas (median: 67; range: 10–346), 188 desaturations (6; 0–25), 44 bradycardias (0; 0–24), and 524 RE (25; 8–62). The frequency of apnea occurring within ± 20 seconds of an RE was not significantly different from that during reflux-free epochs (0.19/min [0.00–0.85] vs 0.25/min [0.00–1.15]); the same was true for desaturations and bradycardias. Also, RE occurred similarly often within 20 seconds before as after an apnea (2; 0–14 vs 1; 0–17). A minority of apneas (3.5%) was associated with an RE reaching the pharyngeal level; of these, significantly more (45 vs 26; median: 1; 0–10 vs 1; 0–7) occurred after rather than before an RE.Conclusion. Both CR events and GER were common in these infants but, with few exceptions, did not seem to be temporally related.
Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Clinical Presentation and Follow-Up of 50 Patients
Authors: den Boer Margarethe E. J. , Wanders Ronald J. A. , Morris Andrew A. M. , IJlst Lodewijk , Heymans Hugo S. A. , Wijburg Frits A. 
Published: August 2004

Objectives. To assess the mode of presentation, biochemical abnormalities, clinical course, and effects of therapy in patients of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency.
Background. LCHAD deficiency is a rare, autosomal recessive inborn error of fatty acid oxidation. Although case reports and small series of patients have been published, these may not give a true picture of the clinical and biochemical spectrum associated with this disorder. To improve the early recognition and management of this potentially lethal disorder, we have reviewed a large cohort of LCHAD-deficient patients.
Methods. A questionnaire was sent to the referring physicians of 61 unselected patients with LCHAD deficiency diagnosed in our center. The standardized questionnaire requested information about the clinical signs and symptoms at presentation, the clinical history, family history, pregnancy, biochemical parameters at presentation, treatment, and clinical outcome.
Results. Questionnaires on 50 patients (82%) were returned and included in this study. The mean age of clinical presentation was 5.8 months (range: 1 day-26 months). Seven (15%) of the patients presented in the neonatal period. Thirty-nine patients (78%) presented with hypoketotic hypoglycemia, the classical features of a fatty acid oxidation disorder. Eleven patients (22%) presented with chronic problems, consisting of failure to thrive, feeding difficulties, cholestatic liver disease, and/or hypotonia. In retrospect, most (82%) of the patients presenting with an acute metabolic derangement also suffered from a combination of chronic nonspecific symptoms before the metabolic crises. Mortality in this series was high (38%), all dying before or within 3 months after diagnosis. Morbidity in the surviving patients is also high, with recurrent metabolic crises and muscle problems despite therapy.
Conclusions. LCHAD deficiency often presents with a combination of chronic nonspecific symptoms. Early diagnosis is difficult in the absence of the classical metabolic derangement. Survival can be improved by prompt diagnosis, but morbidity remains alarmingly high despite current therapeutic regimes.
Heparin-Induced Thrombocytopenia-Associated Thrombosis in Pediatric Intensive Care Patients
Authors: Schmugge Markus , Risch Lorenz , Huber Andreas R. , Benn Anne , Fischer Joachim E. 
Published: August 2004

Background. Heparin-induced thrombocytopenia (HIT), a well-known side effect of heparin therapy, occurs in 1% to 5% of adults exposed to heparin. Of those, about 29% to 88% develop thrombosis. Most data on HIT-associated thrombosis in children are confined to anecdotal reports.
Objective. To determine the incidence of HIT-associated thrombosis in heparin-exposed children.
Methods. We performed a retrospective cohort study on all patients admitted to our pediatric intensive care unit between August 1996 and January 1999. Patients who received heparin for ≥5 days were eligible. Within these patients, we identified all cases of radiologically confirmed thrombosis. Cases of thrombosis were reviewed for fulfillment of clinical HIT criteria. HIT-associated thrombosis was confirmed serologically by determination of levels of antibodies against heparin/platelet factor 4 complexes.
Results. Of 1950 children admitted during the study period, 612 were exposed to heparin for ≥5 days. Thrombosis occurred in 57 patients (9.3%). Plasma samples were available for 38 cases, of which 14 satisfied clinical HIT-criteria. Calculated incidence rate for HIT-associated thrombosis: 2.3%, (95% confidence interval: 1.3%–3.9%, for patients exposed to heparin ≥5 days). Nine patients suffered from venous, 2 patients from arterial, and 3 had combined arterial and venous thrombosis. None of the 14 patients died or underwent amputation. Six patients had heparin and platelet factor 4-complex antibody levels above the cutoff level for adults. The remaining 8 patients had significantly higher antibody levels than a matched control group.
Conclusion. Compared with that reported for adults, HIT-associated thrombosis in pediatric intensive care unit patients has a similar incidence but a less severe outcome.
Hepatobiliary Disease in Neonatal Lupus: Prevalence and Clinical Characteristics in Cases Enrolled in a National Registry
Authors: Lee Lela A. , Sokol Ronald J. , Buyon Jill P. 
Published: August 2004

Objective. To extend the information base on the hepatobiliary manifestations of neonatal lupus erythematosus (NLE) with regard to frequency of occurrence, clinical characteristics, and outcome.
Methods. Review of records from the Research Registry for Neonatal Lupus.
Results. Nineteen (9%) of 219 patients who had NLE and were enrolled in a national registry had probable or possible NLE hepatobiliary disease. In 16 cases, hepatobiliary disease occurred in addition to cardiac or cutaneous NLE. In 3 cases, hepatobiliary disease occurred as the sole clinical manifestation of NLE. Three clinical variants of hepatobiliary disease were observed: 1) severe liver failure present during gestation or in the neonatal period, often with the phenotype of neonatal iron storage disease; 2) conjugated hyperbilirubinemia with mild or no elevations of aminotransferases, occurring in the first few weeks of life; and 3) mild elevations of aminotransferases occurring at approximately 2 to 3 months of life. The prognosis for the children in the last 2 categories is excellent.
Conclusions. Hepatobiliary disease is a relatively common finding in NLE and can be the sole clinical manifestation of NLE. Clinicians should be aware of the broad range of hepatobiliary disease that may occur in children with NLE.
Increased Prevalence of Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator in Children With Chronic Rhinosinusitis
Authors: Raman Vidya , Clary Randall , Siegrist Karen L. , Zehnbauer Barbara , Chatila Talal A. 
Published: August 2004

Objective. Chronic rhinosinusitis results in significant morbidity in the pediatric population; however, no predisposing factor is found in many cases. Cystic fibrosis (CF) is a recognized cause of chronic rhinosinusitis. Although the carrier frequency for CF ranges from 3% to 4% in the general white population, the prevalence of mutations in the CF transmembrane conductance regulator (CFTR) among children with chronic rhinosinusitis is unknown. Our objective was to study the frequency of CFTR mutations among children with chronic rhinosinusitis.
Methods. Fifty-eight white children who were from the St Louis metropolitan area and had chronic rhinosinusitis, none of whom satisfied diagnostic criteria for CF, underwent sweat testing and genotyping for CFTR mutations using an assay that detects 90% of mutations seen in this ethnic group.
Results. Seven of the 58 patients (12.1%) tested harbored CFTR mutations as compared with the expected rate of 3% to 4% in this ethnic group. Five patients had the ΔF508, 1 had the R117H, and 1 had the I148T mutation. Only 1 of the 7 children had a borderline abnormal sweat test. Two of the 58 patients experienced recurrent Pseudomonas aeruginosa rhinosinusitis, and both were ΔF508 heterozygotes. Three other children with no detectable CFTR mutation had borderline elevated sweat-test results. The CFTR intron 8 5T polymorphism was found at a frequency comparable to that reported for the general population.
Conclusion. There is an increased occurrence of CFTR mutations in children who have chronic rhinosinusitis and do not meet diagnostic criteria for CF, usually in the setting of a normal sweat chloride. These results suggest a role for CFTR mutations in predisposition to chronic rhinosinusitis.
Philosophic Objection to Vaccination as a Risk for Tetanus Among Children Younger Than 15 Years
Authors: Fair Elizabeth , Murphy Trudy V. , Golaz Anne , Wharton Melinda 
Published: August 2004

Objectives. Although safe and effective vaccines are available to protect against tetanus in the United States and vaccination rates are high, cases of tetanus among children continue to occur. The objectives of this article are to describe reported cases of tetanus in children in the United States and to identify the reasons for lack of protection against tetanus.
Methods. We reviewed all cases of tetanus in children &lt;15 years of age that were reported to the National Notifiable Diseases Surveillance System from 1992 through 2000. Cases were defined by physician diagnosis. We verified the information in the case reports with state and local health departments.
Results. From 1992 through 2000, 15 cases of tetanus in children &lt;15 years of age were reported from 11 states. Twelve cases were in boys. Two cases were in neonates &lt;10 days of age; the other 13 cases were in children who ranged in age from 3 to 14 years. The median length of hospitalization was 28 days; 8 children required mechanical ventilation. There were no deaths. Twelve (80%) children were unprotected because of lack of vaccination, including 1 neonate whose mother was not vaccinated. Among all unvaccinated cases, objection to vaccination, either religious or philosophic, was the reported reason for choosing not to vaccinate.
Conclusion. The majority of recent cases of tetanus among children in the United States were in unvaccinated children whose parents objected to vaccination. Parents who choose not to vaccinate their children should be advised of the seriousness of the disease and be informed that tetanus is not preventable by means other than vaccination.
Mediators of the Association Between Age of First Sexual Intercourse and Subsequent Human Papillomavirus Infection
Authors: Kahn Jessica A. , Rosenthal Susan L. , Succop Paul A. , Ho Gloria Y. F. , Burk Robert D. 
Published: August 2004

Objective. Previous studies have demonstrated that early age of first sexual intercourse is associated with human papillomavirus (HPV) infection. The objective of this study was to identify a set of risk behaviors and partner characteristics that mediate the association between age of first sexual intercourse and subsequent HPV infection in adolescent and young adult women.
Methods. Female university students completed surveys and underwent HPV testing every 6 months for up to 3 years. HPV-positive participants were matched to HPV-negative participants (252 pairs, total N = 504). Associations were examined between risk behaviors/partner characteristics and both age of first sexual intercourse and HPV infection. Those variables associated with either age of first sexual intercourse or HPV infection were entered into a generalized estimating equation (to account for the matched study design) modeling the association between age of first sexual intercourse and HPV infection.
Results. Mean age of first sexual intercourse was 16.7 (±1.8) years, and early age of first sexual intercourse was associated significantly with HPV infection (β = −0.20; odds ratio: 0.82; 95% confidence interval: 0.74–0.90). The association was mediated by number of sexual partners in the past 6 months, history of sexually transmitted infection, alcohol and drug use related to sexual behaviors, and partner’s number of sexual partners.
Conclusion. A set of behavioral risk factors and partner characteristics partially mediate the association between age of first sexual intercourse and subsequent HPV infection.
Religious/Spiritual Coping in Childhood Cystic Fibrosis: A Qualitative Study
Authors: Pendleton Sara M. , Cavalli Kristina S. , Pargament Kenneth I. , Nasr Samya Z. 
Published: August 2004

Objective. To understand the role of religiousness/spirituality in coping in children with cystic fibrosis (CF).Methods. Participants were a convenience sample of 23 patients with CF, ages 5 to 12 years, and their parent(s) in an ambulatory CF clinic. The design was a focused ethnography including in-depth interviews with children and parent(s), children’s drawings, and self-administered written parental questionnaires. Analysis used grounded theory.Results. Main outcome measures were participants’ views on religion/spirituality in coping with illness. Data included 632 quotes organized into 257 codes categorized into 11 themes. One overarching domain emerged from analysis of the 11 themes: Religious/Spiritual Coping, composed of 11 religious/spiritual coping strategies.Conclusions. Children with CF reported a variety of religious/spiritual coping strategies they nearly always associated with adaptive health outcomes. A preliminary conceptual framework for religious/spiritual coping in children with CF is presented. More study is needed to assess how variability in age, disease type, disease severity, religious/spiritual preference, and religious/spiritual intensity affect religious/spiritual coping in children with chronic illness. Future studies should also investigate whether physician attention to religious/spiritual coping could assist patients in coping with CF and strengthen the doctor-patient relationship.
Infant Weight Gain and Childhood Overweight Status in a Multicenter, Cohort Study
Authors: Stettler Nicolas , Zemel Babette S. , Kumanyika Shiriki , Stallings Virginia A. 
Published: August 2004

Objective. To determine whether a rapid rate of weight gain in early infancy is associated with overweight status in childhood.
Design. Prospective, cohort study from birth to age 7 years.
Setting. Twelve sites across the United States.
Participants. Twenty-seven thousand, eight hundred ninety-nine (27 899) eligible participants born at full term between 1959 and 1965.
Main Outcome Measure. Overweight status at age 7 years, defined by a body mass index above the 95th percentile of the Centers for Disease Control and Prevention reference data.
Results. In the 19 397 participants with complete data (69.6%), the prevalence of overweight status at age 7 years was 5.4%. The rate of weight gain during the first 4 months of life (as 100 g/month) was associated with being overweight at age 7 years, after adjustment for several confounding factors: odds ratio: 1.38; 95% confidence interval: 1.32–1.44. This association was present in each birth weight quintile, and remained significant after adjustment for the weight attained at age 1 year (odds ratio: 1.17; 95% confidence interval: 1.11–1.24).
Conclusions. A pattern of rapid weight gain during the first 4 months of life was associated with an increased risk of overweight status at age 7 years, independent of birth weight and weight attained at age 1 year. These findings may lead to new hypotheses regarding the cause of childhood obesity, which may contribute to our understanding of this increasing public health problem in the United States.
Risk Factors for Severe Respiratory Syncytial Virus Infection Among Alaska Native Children
Authors: Bulkow Lisa R. , Singleton Rosalyn J. , Karron Ruth A. , Harrison Lee H. ,   the Alaska RSV Study Group
Published: August 2004

Objective. The incidence of hospitalization for respiratory syncytial virus (RSV) infection among Alaska Native children is much higher than among non-Native populations in the United States. We conducted this study to better understand factors associated with hospitalization attributable to RSV infection in this high-risk population.Design. Case-control study, including collection of cord blood for RSV-neutralizing antibody measurement.Setting. Remote region of southwest Alaska served by 1 regional hospital and 2 referral hospitals.Subjects. Case-patients identified through surveillance for RSV infection and matched control subjects without acute respiratory infection hospitalization.Results. Breastfeeding was associated with a lower risk of RSV hospitalization (odds ratio: 0.34), whereas underlying medical conditions (primarily prematurity) were associated with increased risk (odds ratio: 6.25). Environmental factors associated with a higher risk of hospitalization included household crowding (4 or more children in the household and crowding index ≥2). The level of maternal RSV-neutralizing antibody was not associated with the risk of hospitalization.Conclusions. In this region with extremely high risk of RSV hospitalization, several measures, such as encouraging breastfeeding and reducing household crowding, could reduce the risk of hospitalization attributable to RSV.