bluuebunny/crossref_metadata_2025_split huggingface dataset:
Incidence and Short-Term Outcome of Children With Symptomatic Presentation of Organic Acid and Fatty Acid Oxidation Disorders in Germany
Authors: Klose Daniela A. , Kölker Stefan , Heinrich Beate , Prietsch Viola , Mayatepek Ertan , von Kries Rüdiger , Hoffmann Georg F. 
Published: August 2004

Objective. To determine the incidence of symptomatic children with inherited organic acid disorders (OADs) and fatty acid oxidation disorders (FAODs) in Germany.Methods. An active surveillance of symptomatic children with inherited OADs and FAODs was conducted during a time period of 24 months (1999–2000) in Germany. Monthly inquiries were sent to all Departments of Pediatrics by the German Pediatric Surveillance Unit (ESPED) and quarterly to all specialized metabolic laboratories. Newly diagnosed patients were added to the database, recording clinical and biochemical information via a standardized questionnaire.Results. Prospective surveillance enrolling 844 575 children identified a total of 57 symptomatic children with newly diagnosed OADs or FAODs in states with conventional neonatal screening, resulting in an estimated cumulative incidence of 1:14 800. The most frequent diagnosis among these children was medium-chain acyl-CoA dehydrogenase deficiency (n = 20). The majority of symptomatic children revealed clinical symptoms during the first year of life (n = 36), frequently presenting with acute metabolic crises (n = 31). Eight children died during these crises. Notably, 47 of the symptomatic children suffered from diseases potentially detectable by expanded neonatal screening programs. This subgroup included 29 children presenting with metabolic crises and 7 of the 8 deaths.Conclusions. Despite increased clinical awareness of OADs and FAODs, the mortality and morbidity for these children remains high, if they are diagnosed after manifestation of clinical disease. An introduction of nationwide neonatal screening programs would change the focus for organic acid analysis from patients presenting with acute metabolic crises to more chronic clinical presentations, especially the cerebral organic acid disorders.
Chiari I Malformation in the Very Young Child: The Spectrum of Presentations and Experience in 31 Children Under Age 6 Years
Authors: Greenlee Jeremy D. W. , Donovan Kathleen A. , Hasan David M. , Menezes Arnold H. 
Published: August 2004

Introduction. The entity of hindbrain herniation without myelodysplasia in the very young child has been poorly described. A retrospective analysis of children diagnosed with Chiari I malformation (CM I) before their sixth birthday is presented.Methods. Since 1985, 31 children with CM I (0.3–5.8) years of age have been diagnosed at University of Iowa Hospitals and Clinics. Their records were reviewed for presenting symptoms, signs, radiographic findings, treatment, complications, and outcome.Results. The average age at diagnosis was 3.3 years. Sixteen patients were under age 3. Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache or neck pain (23%), sensory disturbance (6%), weakness (3%), and other (10%). Sixty-nine percent of children under age 3 had abnormal oropharyngeal function. Three patients under age 3 (19%) had undergone fundoplication and/or gastrostomy before diagnosis of CM I.Common physical findings included abnormal tendon reflexes (68%), scoliosis (26%), abnormal gag reflex (13%), and normal examination (13%). Vocal cord dysfunction (26%, all under age 3) and syringohydromyelia (52%) were also seen.Twenty-five patients were treated surgically at our institution with posterior fossa decompression, duraplasty, and cerebellar tonsillar shrinkage. Three patients were lost to follow-up. Ninety-one percent of patients reported improved symptomatology at last follow-up (mean: 3.9 years). Three patients required reoperation for recurrence of symptoms. Syringomyelia improved in all patients. Scoliosis resolved in 2 of 8 patients, improved in 5, and stabilized in 1. There was no permanent morbidity from surgery.Discussion. We show that children with Chiari I abnormality are very likely to present with oropharyngeal dysfunction if under age 3, and either scoliosis or headache or neck pain worsened by valsalva if age 3 to 5. These symptoms are very likely to improve after Chiari decompression, which can be done with low morbidity.Conclusions. Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause.
Trends in the Prevalence of Cerebral Palsy in a Population-Based Study
Authors: Winter Sarah , Autry Andrew , Boyle Coleen , Yeargin-Allsopp Marshalyn 
Published: August 2004

Objective. To determine trends in the prevalence of congenital cerebral palsy (CP) over a 16-year period for 1-year survivors using a large, population-based surveillance program.Methods. We determined birth weight-specific trends in the prevalence of CP in live birth and 1-year survivor cohorts of children in a 5-county metropolitan Atlanta area for the periods from 1975–1977, 1981–1985, and 1986–1991. We ascertained children with CP in metropolitan Atlanta by record review as part of an ongoing developmental disability surveillance program conducted by the Centers for Disease Control and Prevention and the Georgia Department of Human Resources. A total of 110, 262, and 443 cases of congenital CP were identified for the birth years 1975–1977, 1981–1985, and 1986–1991, respectively. Data were analyzed by birth weight, race, subtypes of CP, and whether the CP existed as an isolated disability or was accompanied by another disability.Results. There was a modest increase in the overall prevalence of congenital CP from 1.7 to 2.0 per 1000 1-year survivors during the period from 1975–1991. This trend was primarily attributable to a slight increase in CP in infants of normal birth weight—CP rates in moderately low and very low birth weight infants did not show consistent trends. There was an increase in the proportion of children who had CP and no other disabilities that was most apparent in infants of normal birth weight from 17% in 1975–1977 to 39% in 1986–1991. For children weighing &lt;1500 g, the proportion of children with spastic diplegic CP increased over time (7% of cases in 1975–1977, 36% in 1985–1988, and 32% in 1986–1991).Conclusions. In the only ongoing population-based study of CP in the United States, there has been a modest increase in the prevalence of CP in 1-year survivors born from 1975–1991. This increase however was seen only in infant survivors of normal birth weight. No change was seen in the trends in CP prevalence in low birth weight and very low birth weight infant based on infant survivors.
Reduction of the Influenza Burden in Children
Authors:   Committee on Infectious Diseases
Published: August 2004

This Policy Statement was retired January 2009.
Epidemiologic studies indicate that children with certain chronic conditions, such as asthma, and otherwise healthy children younger than 24 months are hospitalized for influenza and its complications at high rates similar to those experienced by the elderly. Currently, annual influenza immunization is recommended for all children 6 months and older with high-risk conditions. To protect these children more fully against the complications of influenza, increased efforts are needed to identify and recall high-risk children for annual influenza immunization. In addition, immunization of children 6 through 23 months of age and their household contacts and out-of-home caregivers is now encouraged to the extent feasible. The ultimate goal is a universal recommendation for influenza immunization. Issues that need to be addressed before institution of routine immunization of healthy young children include education of physicians and parents about the morbidity caused by influenza, adequate vaccine supply, and appropriate reimbursement of practitioners for influenza immunization.
Effect of Sepsis Syndrome on Neonatal Oxygen Consumption and Energy Expenditure
Authors: Bauer Jacqueline , Hentschel Roland , Linderkamp Otwin 
Published: August 2004

Objective. To evaluate oxygen consumption (Vo2), carbon dioxide production, and energy expenditure (EE) in full-term neonates with early-onset neonatal septicemia daily for 7 days beginning at the day of clinical diagnosis of sepsis.
Methods. A total of 17 spontaneously breathing full-term neonates, 10 with clinical signs of sepsis and 7 healthy neonates (control group), were enrolled in the study. Age at first study day was 3 ± 0.9 days in both groups. Sepsis syndrome was defined as a systemic response to a bacterial infection with clinical signs of infection, elevated values of interleukins 6 and 8 and C-reactive protein, and abnormal white blood cell count and positive blood cultures (9 group B streptococci, 1 Escherichia coli). Measurements of Vo2 and carbon dioxide production were performed daily for 7 days by means of indirect calorimetry.
Results. In the septic infants, Vo2 and EE were increased by about 20% at days 1 to 3 and by 15% at day 4 when compared with the controls. From days 1 to 3, EE averaged 57 ± 3 kcal/kg/d in the septic neonates and 47 ± 2 kcal/kg/d in the controls. At day 4, EE was 55 ± 2 and 47 ± 2 kcal/kg/d, respectively. Energy intake was about the same in both groups, whereas weight gain during the 7 study days was significantly lower in the sick patients than in the control group (19 ± 2 g/d vs 33 ± 9 g/d and 5.4 ± 0.5 g/kg/d vs 9.4 ± 2.6 g/kg/d, respectively). Increased EE was associated with increased heart rate (126 ± 4 vs 112 ± 4 min−1 at day 1) and respiratory rate (56 ± 6 vs 40 ± 4 min−1 at day 1). There were no differences in rectal temperature (37.3 ± 0.4 °C vs 37.4 ± 0.2 °C), skin temperature (36.5 ± 0.4 °C vs 36.6 ± 0.3 °C), and oxygen saturation (96 ± 3% vs 96 ± 3%) between the 2 groups.
Conclusions. Neonates with sepsis syndrome have elevated Vo2 and EE values that could explain impaired growth during the illness period and may make the infants vulnerable to insufficient calorie supply during the acute phase of septic disease.
Spontaneous Arousals in Supine Infants While Swaddled and Unswaddled During Rapid Eye Movement and Quiet Sleep
Authors: Gerard Claudia M. , Harris Kathleen A. , Thach Bradley T. 
Published: August 2004

Objective. Supine sleep is recommended for infants to decrease the risk of sudden infant death syndrome, but many parents report that their infants seem uncomfortable supine. Many cultures swaddle infants for sleep in the supine position. Swaddled infants are said to “sleep better”; presumably they sleep longer or with fewer arousals. However, there have been no studies of the effect of swaddling on spontaneous arousals during sleep. Arousal is initiated in brainstem centers and manifests as a sequence of reflexes: from sighs to startles and then to thrashing movements. Such “brainstem arousals” may progress to full arousal, but most do not.Methods. Twenty-six healthy infants, aged 80 ± 7 days, were studied during normal nap times. Swaddled (cotton spandex swaddle) and unswaddled trials were alternated for each infant. Sleep state (rapid eye movement [REM] or quiet sleep [QS]) was determined by behavioral criteria (breathing pattern, eye movements) and electroencephalogram/electrooculogram (10 infants). Respitrace, submental and biceps electromyogram, and video recording were used to detect startles and sighs (augmented breaths). Full arousals were classified by eye opening and/or crying. Frequencies of sighs, startles, and full arousals per hour were calculated. Progression of events was calculated as percentages in each sleep state, as was duration of sleep state.Results. Swaddling decreased startles in QS and REM, full arousal in QS, and progression of startle to arousal in QS. It resulted in shorter arousal duration during REM sleep and more REM sleep.Conclusions. Swaddling has a significant inhibitory effect on progression of arousals from brainstem to full arousals involving the cortex in QS. Swaddling decreases spontaneous arousals in QS and increases the duration of REM sleep, perhaps by helping infants return to sleep spontaneously, which may limit parental intervention. For these reasons, a safe form of swaddling that allows hip flexion/abduction and chest wall excursion may help parents keep their infants in the supine sleep position and thereby prevent the sudden infant death syndrome risks associated with the prone sleep position.
Factors That Influence Receipt of Recommended Preventive Pediatric Health and Dental Care
Authors: Yu Stella M. , Bellamy Hilary A. , Kogan Michael D. , Dunbar Jennifer L. , Schwalberg Renee H. , Schuster Mark A. 
Published: August 2004

Objective. This study examined the factors that affect children’s receipt of recommended well-child and dental visits using nationally representative data.
Methods. We analyzed the Child Public Use File of the 1999 National Survey of America’s Families, including 35 938 children who were younger than 18 years. Bivariate and multivariate analyses were conducted to examine the relationship between dependent variables, including receipt of well-child visits as recommended by the American Academy of Pediatrics’ periodicity schedule and dental visits as recommended by the American Academy of Pediatric Dentistry and Bright Futures, and independent variables, including health status and sociodemographic and economic indicators.
Results. Overall, 23.4% of children did not receive the recommended well-child visits, whereas 46.8% did not receive the recommended number of dental visits. The factors that predict nonreceipt of care differed for well-child and dental care and with child’s age. Logistic regression reveals that children who were young (&lt;10 years old), uninsured, non-Hispanic white, had a parent who was less than college educated, or in poor health were least likely to meet the recommendations for well-child care. Children who did not meet the dental recommendation were more likely to be black, uninsured, from families with low incomes, have a parent who was less than college educated, and have postponed dental care in the last year. These risk factors increased with children’s age.
Conclusions. A substantial proportion of US children do not receive preventive care according to professionally recommended standards, particularly dental care. Publicly insured children experience higher rates of recommended well-child visits; however, much improvement is needed among public programs in providing recommended dental care, especially among adolescents and children in poor general health.
Plasma Concentrations of N-Terminal Pro-Brain Natriuretic Peptide in Control Children From the Neonatal to Adolescent Period and in Children With Congestive Heart Failure
Authors: Mir Thomas S. , Marohn Sönke , Läer Stephanie , Eiselt Michele , Grollmus Oswin , Weil Jochen 
Published: August 2004

Objective. To determine plasma levels of N-terminal pro-brain natriuretic peptide (N-BNP) in control children to establish a normal age-dependent range from the neonatal period to adulthood. In addition, plasma concentrations of N-BNP were measured in children with congestive heart failure (CHF) and correlated with ejection fraction and clinical symptoms of heart failure.
Methods. For establishing a normal age-dependent range of plasma N-BNP, venous blood samples were taken in 133 control patients from the neonatal period to adulthood (10 days–32 years) and in 31 children with CHF. Plasma N-BNP levels were determined by an enzyme immunoassay. In children (1 month–14 years) with CHF, plasma N-BNP levels were correlated to ejection fraction measured by echocardiography and clinical symptoms of heart failure using the Ross Score.
Results. N-BNP levels in control children, adolescents, and adults did not show a significant age-related difference. In control children, the normal range was established between 150 (10th percentile) and 430 fmol/mL (90th percentile). Mean plasma N-BNP in control children was 311 fmol/mL (range: 74–654 fmol/mL). In 31 children with CHF, the plasma N-BNP levels were significantly higher (mean: 846; range: 219-2718) than in control children. N-BNP levels showed a negative correlation with the ejection fraction (r = −0.53) and a positive correlation with the clinical heart failure score (r = 0.74).
Conclusions. Plasma N-BNP levels reflect the severity of symptoms of heart failure and the impairment of cardiac function in children with CHF. In the future, determination of plasma N-BNP levels may be used as a helpful adjunct to monitor the effect of various treatments for CHF in children.
Bottle Feeding in the Bed or Crib Before Sleep Time and Wheezing in Early Childhood
Authors: Celedón Juan C. , Litonjua Augusto A. , Ryan Louise , Weiss Scott T. , Gold Diane R. 
Published: August 2004

Objective. Bottle feeding in the bed or crib before sleep time has been associated with an increased risk of wheezing in the first year of life. We examined whether bottle feeding in the bed or crib before sleep time in the first year of life is associated with wheezing in the first 5 years of life.
Methods. In a prospective cohort study of 448 children with parental history of atopy followed from birth, we examined the relation between the number of bimonthly parental reports of bottle feeding in the bed or crib before sleep time in the first year of life (range: 0–6 reports) and parental report of wheezing in the first 5 years of life. Additional outcome measures included recurrent wheezing (≥2 episodes of wheezing in the previous year) and asthma (physician-diagnosed asthma and ≥1 episode of wheezing in the previous year) at the age of 5 years.
Results. The risk of recurrent wheezing and asthma at 5 years of age increased significantly with each additional report of bottle feeding in the bed or crib before sleep time in the first year of life. The risk of wheezing between the ages of 1 and 5 years increased with each additional report of bottle feeding in the bed or crib before sleep time in the first year of life. As an example, a child whose parents reported bottle feeding in the bed or crib before sleep time on 3 occasions in the first year of life had 1.5 times higher risk of wheezing between the ages of 1 and 5 years than a child whose parents did not report bottle feeding in the bed or crib before sleep time in the first year of life (95% confidence interval for relative risk: 1.12–2.12).
Conclusions. Among children with parental history of atopy, bottle feeding in the bed or crib before sleep time in the first year of life is a risk factor for recurrent wheezing and asthma at 5 years of age and a risk factor for wheezing between the ages of 1 and 5 years.
A Rare and Often Unrecognized Cause of Hematochezia and Tenesmus in Childhood: Solitary Rectal Ulcer Syndrome
Authors: Ertem Deniz , Acar Yesim , Karaa Esin Kotiloglu , Pehlivanoglu Ender 
Published: August 2004

Solitary rectal ulcer syndrome (SRUS) is an unusual disorder of childhood, which usually presents with rectal bleeding, mucous discharge, prolonged straining, tenesmus, and localized pain in the perineal area. After the first description by Cruveilhier, Madigan and Morson further detailed the clinical and pathologic features of SRUS in 1969. The pathogenesis of the syndrome is not well-understood. The postulated mechanism responsible for rectal prolapse in most cases seems to be excessive straining efforts during which high intra-abdominal pressure forces the anterior rectal mucosa firmly into the contracting puborectalis muscle. The anterior rectal mucosa is frequently forced into the anal canal and as a consequence becomes strangulated, causing congestion, edema, and ulceration.
Histologically, the presence of fibromuscular obliteration of the lamina propria with disorientation of muscle fibers is characteristic, which could be secondary to chronic mechanical and ischemic trauma and inflammation by hard stools, and intussusception of the rectal mucosa. Although the syndrome is well-recognized in adults, the pediatric experience with this condition is limited and often remains unrecognized or misdiagnosed. A misdiagnosis has been reported in one fourth of adult cases, and the correct diagnosis usually delayed approximately 5 to 7 years. There are few pediatric case reports in English literature. Here, we describe 2 children with SRUS, aged 11 and 14 years, whose SRUS was diagnosed 2 and 6 years, respectively, after the onset of the first signs and symptoms.
The Role of Emergent Neuroimaging in Children With New-Onset Afebrile Seizures
Authors: Sharma Sujit , Riviello James J. , Harper Marvin B. , Baskin Marc N. 
Published: August 2004

Objectives. The objectives of this study were 1) to determine the frequency of clinically significant abnormal neuroimaging in children coming to the emergency department (ED) with new-onset afebrile seizures (ASZ), and 2) to identify children at high or low risk for clinically significant abnormal neuroimaging.
Design/Methods. Five hundred consecutive cases of new-onset ASZ seen in the ED of a tertiary care children’s hospital were reviewed. Neuroimaging reports were categorized as normal, clinically insignificant abnormal, or clinically significant abnormal. Recursive partition analysis was used to identify clinical variables that separated children into high- and low-risk groups for clinically significant abnormal neuroimaging.
Results. Ninety-five percent of patients (475/500) with new-onset ASZ had neuroimaging. Clinically significant abnormal neuroimaging was noted in 8% (95% confidence interval [CI]: 6, 11; 38/475) of patients. Recursive partition analysis identified 2 criteria associated with high risk for clinically significant abnormal neuroimaging: 1) the presence of a predisposing condition, and 2) focal seizure if &lt;33 months old. Of the high-risk patients, 26% (95% CI: 17, 35; 32/121) had clinically significant abnormal neuroimaging compared with 2% (95% CI: 0.6, 3.7; 6/354) in the low-risk group.
Conclusions. In this large, retrospective review of children with new-onset ASZ, clinically significant abnormal neuroimaging occurred with relatively low frequency. Emergent neuroimaging should be considered, however, for children who meet high-risk criteria. Well-appearing children who meet low-risk criteria can be safely discharged from the ED (if follow-up can be assured) without emergent neuroimaging, because their risk for clinically significant abnormal neuroimaging is appreciably lower.
Effects of Sufentanil on Electroencephalogram in Very and Extremely Preterm Neonates
Authors: Nguyen The Tich Sylvie , Vecchierini Marie-Françoise , Debillon Thierry , Péréon Yann 
Published: August 2004

Objective. The electroencephalogram (EEG) is used in neonatal intensive care units to assess brain maturation and neurologic prognosis in preterm newborns. Most of these newborns are sedated by opioids because of long-term assisted ventilation. The aim of this study was to describe the effects of sufentanil on the EEG in preterm newborns and to evaluate the consequences of such a treatment on neurologic assessment.
Methods. Fifteen preterm newborns &lt;28 days of extrauterine life were studied. All of them were sedated by sufentanil (initial bolus injection of 0.5 μg/kg, followed by continuous infusion of 0.2 μg/kg/h). Three EEGs were performed: the first before and during the bolus injection, the second in the 48 hours after the start of the continuous infusion, and the third at least 24 hours after the treatment was stopped. Qualitative and quantitative methods were used to analyze each EEG.
Results. EEG patterns were not affected by sufentanil treatment. Bolus injection and continuous infusion induced a significant increase of EEG discontinuity in preterm newborns affecting mean burst percentage and mean and maximum interburst duration.
Conclusions. The present data demonstrate that EEG is affected by bolus injection and continuous infusion of sufentanil. Sedation must therefore be considered to avoid misinterpretation of EEGs.