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ObjectiveTo evaluate the efficacy and side effect profile of sodium oxybate in the treatment for narcolepsy–cataplexy in the pediatric age group.MethodsA retrospective study was conducted on 15 children and adolescents with narcolepsy–cataplexy who had been treated with sodium oxybate. The mean age at diagnosis of narcolepsy was 11 years (range 3–17 years). Subjects were followed for 3–90 months (mean 33) after starting sodium oxybate. During this period of time they were also maintained on other medications for sleepiness (n = 14) and cataplexy (n = 6). The charts were reviewed for documentation of improvement in sleepiness or cataplexy, side effects, and functioning in daily life.ResultsSubsequent to the addition of sodium oxybate, sleepiness improved in 13/15 patients. In patients who had Epworth Sleepiness Scale (ESS) assessments, the score fell from a baseline median of 18 to 12 (n = 10, p = 0.01). The number of cataplexy episodes estimated by parents decreased from a median of 38/week pre-treatment to <1/week post treatment (n = 14, p < 0.001). Cataplexy severity, measured on an arbitrary scale, fell from a median of 3 (severe) to 1 (mild) in all 15 subjects (p < 0.001).Two of the 15 patients (13%) discontinued sodium oxybate, one for insurance reasons and the other due to constipation and dissociative feelings. A third patient stopped the medication temporarily due to body aches and dizziness, but then resumed treatment without recurrence of symptoms. Side effects in four others included tremor, blurring of vision, nocturnal awakenings, and increased nightmares. Overall, side effects occurred in 6/15 (40%) individuals. Improvement in social/academic spheres was noted in 11/15 (73%) subjects after starting sodium oxybate. The median BMI before and after treatment remained unchanged at 23 (n = 14, p = 0.99). Median values of height and weight before and after treatment also did not change significantly. The mean dose of sodium oxybate was 5 ± 2 g. Dose escalation owing to development of tolerance was not encountered.ConclusionsSodium oxybate is effective in alleviating sleepiness and cataplexy in childhood onset narcolepsy–cataplexy. The therapeutic response was sustained over time, and without development of tolerance. Forty percent of the subjects experienced adverse effects. 相似文献
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Population‐Based Study of Attitudes toward BRCA Genetic Testing among Orthodox Jewish Women 下载免费PDF全文
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Maria Irene Barillas-Lara Jose R. Medina-Inojosa Bhanu Prakash Kolla Joshua R. Smith Amanda R. Bonikowske Thomas G. Allison Thomas Olson Francisco Lopez-Jimenez Virend K. Somers Sean M. Caples Meghna P. Mansukhani 《Mayo Clinic proceedings. Mayo Clinic》2021,96(3):636-647
ObjectiveTo determine the risk of long-term major adverse cardiovascular events (MACE) when sleep-disordered breathing (SDB) and decreased cardiorespiratory fitness (CRF) co-occur.MethodsWe included consecutive patients who underwent symptom-limited cardiopulmonary exercise tests between January 1, 2005, and January 1, 2010, followed by first-time diagnostic polysomnography within 6 months. Patients were stratified based on the presence of moderate-to-severe SDB (apnea/hypopnea index ≥15 per hour) and decreased CRF defined as <70% predicted peak oxygen consumption (VO2). Long-term MACE was a composite outcome of myocardial infarction (MI), coronary artery bypass graft (CABG), percutaneous coronary intervention (PCI), stroke or transient ischemic attack (TIA), and death, assessed until May 21, 2018. Cox-proportional hazard models were adjusted for factors known to influence CRF and MACE.ResultsOf 498 included patients (60±13 years, 28.1% female), 175 (35%) had MACE (MI=17, PCI=14, CABG=13, stroke=20, TIA=12, deaths=99) at a median follow-up of 8.7 years (interquartile range=6.5 to 10.3 years). After adjusting for age, sex, beta blockers, systemic hypertension, diabetes mellitus, coronary artery disease, cardiac arrhythmia, chronic obstructive pulmonary disease, smoking, and use of positive airway pressure (PAP), decreased CRF alone (hazard ratio [HR]=1.91, 95% confidence interval [CI], 1.15 to 3.18; P=.01), but not SDB alone (HR=1.26, 95% CI, 0.75 to 2.13, P=.39) was associated with increased risk of MACE. Those with SDB and decreased CRF had greater risk of MACE compared with patients with decreased CRF alone (HR=1.85; 95% CI, 1.21 to 2.84; P<.005) after accounting for these confounders. The risk of MACE was attenuated in those with reduced CRF alone after additionally adjusting for adequate adherence to PAP (HR=1.59; 95% CI, 0.77 to 3.31; P=.21).ConclusionThe incidence of MACE, especially mortality, was high in this sample. Moderate-to-severe SDB with concurrent decreased CRF was associated with higher risk of MACE than decreased CRF alone. These results highlight the importance of possibly including CRF in the risk assessment of patients with SDB and, conversely, that of screening for SDB in patients with low peak VO2. 相似文献
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Talekar BS Rozier RG Slade GD Ennett ST 《Journal of the American Dental Association (1939)》2005,136(3):364-72; quiz 381
BACKGROUND: Parents have an important role in making decisions about their children's oral health. The purpose of the authors' study was to determine parental perceptions of their children's oral health status and factors correlated with these perceptions of health. METHODS: The authors analyzed data for 3,424 children (2-5 years of age) from the Third National Health and Nutrition Examination Survey. They based the dependent variable on a question asked of primary caregivers: "How would you describe the condition of [child's name]'s natural teeth?" Explanatory variables included demographic variables, dental visits, perception of child's general health, need for dental care and presence of tooth caries. RESULTS: Eighty-nine percent of parents rated their child's oral health as excellent, very good or good, and 11 percent rated it as fair or poor (mean = 2.7 on a five-point scale, with 1 being excellent and 5 being poor). Tooth caries, perceived need for dental cleaning and treatment, lower income and poorer general health perceptions were associated with poorer parental ratings. CONCLUSIONS: Actual disease and perceived need are associated significantly with parents' perceptions of their children's oral health. PRACTICE IMPLICATIONS: Understanding parents' perceptions of their children's oral health and factors that motivate these perceptions can help dentistry overcome barriers that parents encounter in accessing dental care for their children. 相似文献
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Quiñonez RB Stearns SC Talekar BS Rozier RG Downs SM 《Archives of pediatrics & adolescent medicine》2006,160(2):164-170
OBJECTIVE: To examine the cost-effectiveness of fluoride varnish application by medical providers when implemented within a well-child periodicity schedule for Medicaid-enrolled children. DESIGN: Cost-effectiveness was analyzed using published probabilities and costs. Input parameters included the effectiveness of fluoride varnish (35.4%) applied according to the well-child periodicity schedule up to 3 years of age at $16.00 per application, annual caries increment (14%), age-specific dental care usage rates (0.2% at 9 months to 19% at 42 months), and age-related nonhospital treatment costs ($292.00-$503.00) and hospital treatment costs ($2191.00-$2940.00). Sensitivity analysis was conducted to assess the effects for varying input parameters. SETTING: Well-child visits during primary care. PARTICIPANTS: Children aged 9 to 42 months. INTERVENTION: Application of universal fluoride varnish (fluoride varnish-all) at 9, 18, 24, and 36 months vs no intervention (fluoride varnish-none) was compared. MAIN OUTCOME MEASURES: Cost per month without cavities and treatment averted during the first 42 months of life from a Medicaid payer's perspective. RESULTS: Fluoride varnish improved clinical outcomes by 1.52 cavity-free months but at a cost of $7.18 for each cavity-free month gained per child and $203 for each treatment averted. Considerable uncertainty existed for some parameters. Fluoride varnish was cost saving when dental services and nonhospital treatment costs were 1.5 to 2 times greater, respectively, than our base case estimate. CONCLUSIONS: Based on these assumptions, fluoride varnish use in the medical setting is effective in reducing early childhood caries in low-income populations but is not cost saving in the first 42 months of life. Potential total cost reductions with varying parameters suggest that evaluations using a longitudinal cohort are needed. 相似文献