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  • Prevalence and Time Course of Acute Mountain Sickness in Older Children and Adolescents After Rapid Ascent to 3450 Meters

    OBJECTIVE. Acute mountain sickness is a frequent and debilitating complication of high-altitude exposure, but there is little information on the prevalence and time course of acute mountain sickness in children and adolescents after rapid ascent by mechanical transportation to 3500 m, an altitude at which major tourist destinations are located throughout the world.

    METHODS. We performed serial assessments of acute mountain sickness (Lake Louise scores) in 48 healthy nonacclimatized children and adolescents (mean ± SD age: 13.7 ± 0.3 years; 20 girls and 28 boys), with no previous high-altitude experience, 6, 18, and 42 hours after arrival at the Jungfraujoch high-altitude research station (3450 m), which was reached through a 2.5-hour train ascent.

    RESULTS. We found that the overall prevalence of acute mountain sickness during the first 3 days at high altitude was 37.5%. Rates were similar for the 2 genders and decreased progressively during the stay (25% at 6 hours, 21% at 18 hours, and 8% at 42 hours). None of the subjects needed to be evacuated to lower altitude. Five subjects needed symptomatic treatment and responded well.

    CONCLUSION. After rapid ascent to high altitude, the prevalence of acute mountain sickness in children and adolescents was relatively low; the clinical manifestations were benign and resolved rapidly. These findings suggest that, for the majority of healthy nonacclimatized children and adolescents, travel to 3500 m is safe and pharmacologic prophylaxis for acute mountain sickness is not needed.

  • Utility of Lumbar Puncture for First Simple Febrile Seizure Among Children 6 to 18 Months of Age

    OBJECTIVES. American Academy of Pediatrics consensus statement recommendations are to consider strongly for infants 6 to 12 months of age with a first simple febrile seizure and to consider for children 12 to 18 months of age with a first simple febrile seizure lumbar puncture for cerebrospinal fluid analysis. Our aims were to determine compliance with these recommendations and to assess the rate of bacterial meningitis detected among these children.

    METHODS. A retrospective cohort review was performed for patients 6 to 18 months of age who were evaluated for first simple febrile seizure in a pediatric emergency department between October 1995 and October 2006.

    RESULTS. First simple febrile seizure accounted for 1% of all emergency department visits for children of this age, with 704 cases among 71 234 eligible visits during the study period. Twenty-seven percent (n = 188) of first simple febrile seizure visits were for infants 6 to 12 months of age, and 73% (n = 516) were for infants 12 to 18 months of age. Lumbar puncture was performed for 38% of the children (n = 271). Samples were available for 70% of children 6 to 12 months of age (131 of 188 children) and 25% of children 12 to 18 months of age (129 of 516 children). Rates of lumbar puncture decreased significantly over time in both age groups. The cerebrospinal fluid white blood cell count was elevated in 10 cases (3.8%). No pathogen was identified in cerebrospinal fluid cultures. Ten cultures (3.8%) yielded a contaminant. No patient was diagnosed as having bacterial meningitis.

    CONCLUSIONS. The risk of bacterial meningitis presenting as first simple febrile seizure at ages 6 to 18 months is very low. Current American Academy of Pediatrics recommendations should be reconsidered.

  • Obesity and Excessive Daytime Sleepiness in Prepubertal Children With Obstructive Sleep Apnea

    INTRODUCTION. The epidemic of childhood obesity has prompted remarkable changes in the relative proportions of symptomatic overweight or obese children being referred for evaluation of habitual snoring. However, it remains unclear whether obesity modifies the relative frequency of daytime symptoms such as excessive daytime sleepiness.

    METHODS. Fifty consecutive, nonobese, habitually snoring, otherwise-healthy children (age range: 6–9 years) and 50 age-, gender-, and ethnicity-matched obese children (BMI z score: >1.67) underwent an overnight polysomnographic evaluation, followed by a multiple sleep latency test the following day.

    RESULTS. The mean obstructive apnea/hypopnea index values for the 2 groups were similar (nonobese: 12.0 ± 1.7 episodes per hour of total sleep time; obese: 10.9 ± 1.5 episodes per hour of total sleep time). However, the mean sleep latency for obese children was significantly shorter (12.9 ± 0.9 minutes) than that for nonobese children (17.9 ± 0.7 minutes). Furthermore, 21 obese children had mean sleep latencies of ≤12.0 minutes, compared with only 5 nonobese children. Although significant associations emerged between mean sleep latency, obstructive apnea/hypopnea index, proportion of total sleep time with oxygen saturation of <95%, and respiratory arousal index for the whole cohort, the slopes and intersects of the linear correlation of mean sleep latency with any of these polygraphic measures were consistently greater in the obese cohort.

    CONCLUSIONS. The likelihood of excessive daytime sleepiness for obese children is greater than that for nonobese children at any given level of obstructive sleep apnea severity and is strikingly reminiscent of excessive daytime sleepiness patterns in adults with obstructive sleep apnea.

  • Medical and Financial Impact of a Neonatal Extracorporeal Membrane Oxygenation Referral Center in the Nitric Oxide Era

    OBJECTIVES. The primary objective of this study was to determine whether widespread use of nitric oxide after Food and Drug Administration approval decreased admissions to a neonatal referral center for extracorporeal membrane oxygenation evaluation. We also sought to determine whether antecedent treatment delayed eventual transfer, resulting in sicker patients, increased mortality, increased extracorporeal membrane oxygenation application, and higher direct costs of care.

    METHODS. This was a retrospective cohort study of all of the patients transferred to a neonatal referral center for extracorporeal membrane oxygenation evaluation before (1995–1999) and after (2000–2005) Food and Drug Administration approval of nitric oxide. Patients were divided into "congenital diaphragmatic hernia" and "persistent pulmonary hypertension" (all other diagnoses) for additional analysis.

    RESULTS. Admission rates for extracorporeal membrane oxygenation evaluation decreased in the nitric oxide era, and eventual transfer was not delayed. Persistent pulmonary hypertension patients had improved oxygen indexes, a trend toward decreased mortality, decreased extracorporeal membrane oxygenation use, and decreased direct costs. Congenital diaphragmatic hernia patients had unchanged physiologic measurements, mortality, and extracorporeal membrane oxygenation use with increased direct costs of care. As a whole, outcomes for patients transferred for extracorporeal membrane oxygenation evaluation improved, whereas direct costs were unchanged.

    CONCLUSIONS. Persistent pulmonary hypertension patients had improved outcomes with decreased costs, whereas congenital diaphragmatic hernia patients had unchanged outcomes with increased costs. Overall, patients admitted to this NICU because of the presence of extracorporeal membrane oxygenation services had improved outcomes without increased costs in the nitric oxide era.

  • Mucopolysaccharidosis I: Management and Treatment Guidelines

    OBJECTIVE. Disease management for mucopolysaccharidosis type I has been inconsistent because of disease rarity (~1 case per 100000 live births), phenotypic heterogeneity, and limited therapeutic options. The availability of hematopoietic stem cell transplantation and the recent introduction of enzyme replacement therapy for mucopolysaccharidosis I necessitate the establishment of system-specific management guidelines for this condition.

    METHODS. Twelve international experts on mucopolysaccharidosis I met in January 2003 to draft management and treatment guidelines for mucopolysaccharidosis I. Initial guidelines were revised and updated in 2008, on the basis of additional clinical data and therapeutic advances. Recommendations are based on our extensive clinical experience and a review of the literature.

    RESULTS.All patients with mucopolysaccharidosis I should receive a comprehensive baseline evaluation, including neurologic, ophthalmologic, auditory, cardiac, respiratory, gastrointestinal, and musculoskeletal assessments, and should be monitored every 6 to 12 months with individualized specialty assessments, to monitor disease progression and effects of intervention. Patients are best treated by a multidisciplinary team. Treatments consist of palliative/supportive care, hematopoietic stem cell transplantation, and enzyme replacement therapy. The patient's age (>2 years or ≤2 years), pr


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