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PURPOSE: In addition to epilepsy, individuals with tuberous sclerosis complex (TSC) experience a wide range of behavioral, psychiatric, intellectual, academic, and psychosocial problems. They usually exert a large psychological burden on individuals with these illnesses. METHODS: This cross-sectional study used TSC-associated neuropsychiatric disorders (TAND) checklist interviews conducted at a single medical center. The enrollment of all subjects was > 6 years, and the comorbidities of neurodevelopmental disorders were assessed by clinical psychologists before enrollment. To assess the spectrum of TAND, the TAND checklist was applied as stated in the protocol, and the responses to the TAND checklist were evaluated by clinical psychologists. RESULTS: In the behavioral concerns of patients with TSC without epilepsy, those with epilepsy had excessive shyness, language delay, lack of eye contact, rigid behavior, inattentiveness, and restlessness. In psychiatric disorders, autism spectrum disorder and attention-deficit/hyperactivity disorder are significantly correlated with epilepsy history. Diminished academic skills, including reading, writing, and mathematics skills, are significantly associated with epilepsy history. For intellectual ability, TSC patients without epilepsy is associated normal intelligence level. Among neuropsychological skills, deficits in attention, dual tasking/multi-tasking, visuospatial tasking, and executive skills are significantly associated with epilepsy history. CONCLUSIONS: Epilepsy in patients with TSC contributes to comorbid neuropsychiatric disorders. In addition to epilepsy evaluation, it is crucial to evaluate the heterogeneous spectrum of neuropsychiatric disorders using a standard checklist during the annual clinical follow-up of patients with TSC.
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Lista de Verificación , Comorbilidad , Epilepsia , Esclerosis Tuberosa , Humanos , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/psicología , Esclerosis Tuberosa/epidemiología , Epilepsia/psicología , Epilepsia/epidemiología , Femenino , Masculino , Estudios Transversales , Niño , Adolescente , Adulto Joven , Adulto , Trastornos Mentales/epidemiología , Trastornos Mentales/etiología , Trastornos Mentales/psicologíaRESUMEN
Background: Although regarded as a potentially efficient approach to address tuberous sclerosis complex (TSC)-associated complications, the adverse event profile of everolimus has not yet been fully elucidated. The present study aimed to clarify the adverse event spectrum in patients with TSC who are using everolimus for common indications, in comparison to those who do not use everolimus. Materials and Methods: We recruited patients with TSC who were followed up annually at TSC integrated clinics or referred for medical assistance. Medical reviews and laboratory investigations were performed at baseline and annually by clinical physicians. The adverse events were assessed as per the National Cancer Institute Common Terminology Criteria for Adverse Events. Results: Common adverse events in everolimus users included hypercholesterolemia (55%), gingivostomatitis (50%), proteinuria (50%), and hyperglycemia (40%). Compared with everolimus nonusers, the occurrence of gingivostomatitis and proteinuria was significantly higher in everolimus users (gingivostomatitis, p=0.02; proteinuria, p=0.02). Among the everolimus users, 12 patients had level I CTCAE, and five had level II CTCAE. None of the everolimus users presented with CTCAE level III or higher. Conclusion: Patients with TSC who are everolimus users had a higher tendency to develop gingivostomatitis and proteinuria compared to nonusers. However, no differences were observed in the occurrence of other adverse events between everolimus users and nonusers.
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Angiomiolipoma , Antineoplásicos , Astrocitoma , Neoplasias Renales , Esclerosis Tuberosa , Humanos , Everolimus/efectos adversos , Angiomiolipoma/tratamiento farmacológico , Angiomiolipoma/complicaciones , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/tratamiento farmacológico , Esclerosis Tuberosa/epidemiología , Neoplasias Renales/tratamiento farmacológico , Astrocitoma/tratamiento farmacológico , Astrocitoma/complicaciones , Proteinuria/inducido químicamente , Antineoplásicos/efectos adversosAsunto(s)
Neoplasias Renales , Riñón Único , Esclerosis Tuberosa , Masculino , Humanos , Esclerosis Tuberosa/complicacionesRESUMEN
Comorbidities are common in children with epilepsy, with nearly half of the patients having at least one comorbidity. Attention deficit hyperactivity disorder (ADHD) is a psychiatric disorder characterized by hyperactivity and inattentiveness level disproportional to the child's developmental stage. The burden of ADHD in children with epilepsy is high and can adversely affect the patients' clinical outcomes, psychosocial aspects, and quality of life. Several hypotheses were proposed to explain the high burden of ADHD in childhood epilepsy; the well-established bidirectional connection and shared genetic/non-genetic factors between epilepsy and comorbid ADHD largely rule out the possibility of a chance in this association. Stimulants are effective in children with comorbid ADHD, and the current body of evidence supports their safety within the approved dose. Nonetheless, safety data should be further studied in randomized, double-blinded, placebo-controlled trials. Comorbid ADHD is still under-recognized in clinical practice. Early identification and management of comorbid ADHD are crucial to optimize the prognosis and reduce the risk of adverse long-term neurodevelopmental outcomes. The identification of the shared genetic background of epilepsy and ADHD can open the gate for tailoring treatment options for these patients through precision medicine.
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Trastorno por Déficit de Atención con Hiperactividad , Estimulantes del Sistema Nervioso Central , Epilepsia , Niño , Humanos , Trastorno por Déficit de Atención con Hiperactividad/complicaciones , Trastorno por Déficit de Atención con Hiperactividad/epidemiología , Trastorno por Déficit de Atención con Hiperactividad/genética , Calidad de Vida , Estimulantes del Sistema Nervioso Central/efectos adversos , Epilepsia/complicaciones , Epilepsia/epidemiología , Epilepsia/genética , ComorbilidadRESUMEN
BACKGROUND: Hidradenitis suppurativa (HS) and allergic diseases were considered to have different immune pathways involved. However, available evidences seemed to be inconsistent and the association was not well-developed yet. OBJECTIVE: The aim of this study was to perform a systematic review and meta-analysis to evaluate the association between HS and atopic diseases, including asthma, atopic dermatitis, allergic rhinitis, and conjunctivitis. METHODS: Search in databases including PubMed, Embase, and Web of Science was performed. Synonyms were determined based on MeSH terms and Emtree. Observational studies with proper comparative arm were selected. For quality evaluation of extracted studies, the Newcastle-Ottawa Scale was utilized. Odds ratio of atopic dermatitis, asthma, allergic rhinitis, and conjunctivitis were evaluated in HS patients (comparing with non-HS people). I2 value was applied to evaluate the heterogeneity within studies. RESULTS: After appraising 1,654 studies, in total, 12 studies were selected for data extraction. In adjusted models, people with HS is significantly associated with higher risk of having asthma, with a pooled odds ratio of 1.50 (95% CI, 1.24-1.81). Risk of presenting atopic dermatitis in HS patients was also increased, with an odd ratio of 4.10 (95% CI, 2.16-8.18). The association remained its significance in sensitivity models. Evidences were insufficient to support the association between HS and allergic rhinitis and conjunctivitis. CONCLUSION: Current evidence supported that atopic dermatitis and asthma were associated with HS. Physicians should be aware of the reported association while caring people with HS and related screening of allergies comorbidities should be recommended.
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Asma , Conjuntivitis , Dermatitis Atópica , Hidradenitis Supurativa , Rinitis Alérgica , Humanos , Dermatitis Atópica/complicaciones , Dermatitis Atópica/epidemiología , Dermatitis Atópica/diagnóstico , Hidradenitis Supurativa/complicaciones , Hidradenitis Supurativa/epidemiología , Asma/epidemiología , Rinitis Alérgica/epidemiologíaRESUMEN
PURPOSE: To evaluate the changes in visual function when progressive addition lenses (PAL) are added in children using topical atropine as a myopia control therapy. Daily visual complaints and the determination of their near correction were studied. METHODS: Forty children aged 7-12 years were recruited. Distance and near visual acuity, accommodative lag, heterophoria, near point of convergence and stereopsis were examined, and a questionnaire of daily visual complaints was administered. RESULTS: Significant differences in visual functions were found after the near correction was prescribed. Significant improvements in distance and near visual acuity, lag of accommodation and binocular visual function were observed, and fewer visual complaints were reported at the Harmon distance. CONCLUSION: The use of PAL is helpful for children undergoing topical atropine treatment for myopia control, particularly those receiving medium to high doses. This combination therapy could also be applied to younger children who have a low tolerance to contact lenses, with less risk of ocular adverse effects.
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Atropina , Miopía , Humanos , Niño , Agudeza Visual , Visión Binocular , Miopía/tratamiento farmacológico , Acomodación Ocular , Refracción OcularRESUMEN
(1) Background: Recently, a growing number of studies have provided evidence to suggest a strong correlation between air pollution exposure and attention-deficit/hyperactivity disorder (ADHD). In this study, we assessed the relationship between early-life exposure to particulate matter (PM)10, PM2.5, and ADHD; (2) Methods: The National Health Insurance Research Database (NHIRD) contains the medical records, drug information, inspection data, etc., of the people of Taiwan, and, thus, could serve as an important research resource. Air pollution data were based on daily data from the Environmental Protection Administration Executive Yuan, R.O.C. (Taiwan). These included particulate matter (PM2.5 and PM10). The two databases were merged according to the living area of the insured and the location of the air quality monitoring station; (3) Results: The highest levels of air pollutants, including PM2.5 (adjusted hazard ratio (aHR) = 1.79; 95% confidence interval (CI) = 1.58-2.02) and PM10 (aHR = 1.53; 95% CI = 1.37-1.70), had a significantly higher risk of ADHD; (4) Conclusions: As such, measures for air quality control that meet the WHO air quality guidelines should be strictly and uniformly implemented by Taiwanese government authorities.
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Contaminantes Atmosféricos , Contaminación del Aire , Trastorno por Déficit de Atención con Hiperactividad , Preescolar , Humanos , Material Particulado/análisis , Trastorno por Déficit de Atención con Hiperactividad/epidemiología , Contaminación del Aire/análisis , Contaminantes Atmosféricos/análisis , Taiwán/epidemiología , Exposición a Riesgos Ambientales/análisisRESUMEN
Background: Psychological burdens can affect the quality of life among parents of children with epilepsy, especially parents of children with poor seizure control. The impact of stress on the parents of children with epilepsy is significantly comorbid with their children's cognitive dysfunction and the severity of epilepsy. The aim of this study was to assess the stress levels of parents of school-age children with drug-resistant epilepsy (DRE) and controlled-epilepsy after considering the children's cognitive ability. Methods: The study participants consisted of 35 children with typical development in the control group, 25 in the controlled-epilepsy group, 26 in the DRE group, and their parents. We used the Chinese version of the Parenting Stress Index (PSI) to measure the stress levels of all parents; and the Wechsler intelligence scale for children-fourth edition (WISC-IV) Chinese version to assess the children's cognition levels. Results: Parenting stress was significantly higher among the parents of children with DRE than of those in the control and controlled-epilepsy group. The PSI's child domain showed statistically significant subscales of adaptability, acceptability, demandingness, and distractibility/hyperactivity. Moreover, the high-risk ratio on the acceptability, adaptability, demandingness, and distractibility/hyperactivity subscales were also higher for the DRE group than for the controlled-epilepsy group and for the control group. Conclusion: Seizure severity significantly influences parenting stress after considering cognitive dysfunction in children with epilepsy. Therefore, pediatricians and clinicians should consider epilepsy-specific stress in parents of children with DRE.
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BACKGROUND: Late talking (LT) in toddlers is a risk factor for language weakness that may interfere with the development of using language to regulate behavior and emotion and contribute to the development of behavior problems from early childhood. This study examined the temporal stability of parent-reported behavior problems among Mandarin-speaking LT toddlers from ages 2 to 4 in Taiwan. METHODS: Thirty-one LT and 31 typical language development (TLD) toddlers were assessed for their vocabulary production at age 2 with the Words and Sentences Forms of the MacArthur-Bates Communicative Developmental Inventories Toddler Form (Taiwan version). Additionally, participants' receptive and expressive language abilities were assessed using the receptive and expressive language subscales of the Bayley Scales of Infant and Toddler Development, Third Edition. At age 4, the Child Language Disorder Scale-Revised was applied and included the two core subtests for auditory comprehension and expressive communication. At ages 2 and 4 years, behavior problems were assessed with the Child Behavior Checklist. RESULTS: There was a higher percentage of participants with persistent behavior problems among LT toddlers than among TLD toddlers. Moreover, toddlers with larger vocabularies were less likely to develop withdrawal behaviors by preschool age. CONCLUSIONS: This study supported the temporal stability of parent-reported behavior problems among LT toddlers across early childhood. Early identification of and intervention for behavior problems associated with LT in toddlerhood is essential to alleviate their behavior problems later in preschool years.
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Trastornos del Desarrollo del Lenguaje , Estudios de Casos y Controles , Preescolar , Humanos , Lactante , Desarrollo del Lenguaje , Trastornos del Desarrollo del Lenguaje/etiología , Padres , VocabularioRESUMEN
Background: Most tuberous sclerosis complex (TSC) patients have neurological disorders and are at high risk of academic difficulties. Among academic skills, reading ability is the most important academic skill. The study applied the Chinese character fluency test to measure the word recognition and reading comprehension of TSC children to observe whether they have the characteristics of reading disability, as an indicator of the spectrum of reading ability in TSC patients. Methods: The patients were assessed using the Chinese character fluency test and reading comprehension test to explore the differences in reading ability in terms of gender, age, epilepsy history, genotype, and intelligence level. Results: Of the 27 patients, the assessment of reading accuracy showed statistical differences between intellectual level > 80, PR (p = 0.024), and pass numbers (p = 0.018). For the fluency assessment, there was a difference between different intellectual level (p = 0.050). In the reading comprehension test, there was differences for intellectual level in positivity (p = 0.07) and pass numbers (p = 0.06). Conclusion: The Chinese character fluency and reading comprehension test measure the word recognition and reading comprehension and the spectrum of reading ability in TSC patients. All individuals with TSC, especially those with below average of intellectual ability, should be considered for potential academic difficulties.
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BACKGROUND: To date, there is insufficient knowledge about the association of air pollution and childhood nephrotic syndrome in the real world. This study aimed to evaluate the effects of the three common gaseous air pollutants, including sulfur dioxide, total hydrocarbon, and methane, on the risk of idiopathic nephrotic syndrome (INS) in children. METHODS: We collected data from the Taiwan National Health Insurance Research Database and Taiwan Air Quality-Monitoring Database. Children younger than 18 years old, identified from January 1, 2000, were followed up until the first diagnosis of INS was established or until December 31, 2012. We measured the incidence rates and hazard ratios for INS stratified based on the quartiles (Q1-Q4) of air pollutant concentration. Multivariate Cox proportional hazards models were also applied by adjusting age, sex, monthly income, and urbanization. RESULTS: Compared with participants exposed to Q1 concentrations, the adjusted hazard ratios (aHRs) for INS increased progressively along the four quartiles of sulfur dioxide, total hydrocarbon, and methane, from 1 (Q1) to 1.78 (Q4), 1 (Q1) to 3.49 (Q4), 1 (Q1) to 7.83 (Q4), respectively. CONCLUSIONS: Our study revealed that children with exposure to higher concentrations of sulfur dioxide, total hydrocarbon, and methane was associated with an increased risk of INS.
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Contaminantes Atmosféricos , Contaminación del Aire , Síndrome Nefrótico , Adolescente , Contaminantes Atmosféricos/efectos adversos , Contaminación del Aire/efectos adversos , Niño , Estudios de Cohortes , Exposición a Riesgos Ambientales/efectos adversos , Humanos , Hidrocarburos , Metano , Síndrome Nefrótico/epidemiología , Material Particulado/efectos adversos , Material Particulado/análisis , Dióxido de Azufre/efectos adversosRESUMEN
ABSTRACT: Tuberous sclerosis complex (TSC) is a rare genetic disorder with multisystem involvement. TSC is characterized by benign hamartomas in multiple organs, including the brain, and its clinical phenotypes may be associated with abnormal functional connections. We aimed to use resting-state functional connectivity to provide findings of disrupted functional brain networks in TSC patients using graph theoretical analysis (GTA) and network-based statistic (NBS) analysis.Forty TSC patients (age = 24.11+/-11.44 years old) and 18 age-matched (25.13+/- 10.01 years old) healthy controls were recruited; they underwent resting-state functional magnetic resonance imaging using a 3T magnetic resonance imaging scanner. After image preprocessing and removing physiological noises, GTA was used to calculate the topological parameters of the brain network. NBS analysis was then used to determine the differences in cerebrum functional connectivity between the 2 groups.In GTA, several topological parameters, including the clustering coefficient, local efficiency, transitivity, and modularity, were better in controls than in TSC patients (P < .05). In NBS analysis, the edges of the brain networks between the groups were compared. One subnetwork showed more edges in controls than in TSC patients (P < .05), including the connections from the frontal lobe to the temporal and parietal lobe.The study results provide the findings on disrupted functional connectivity and organization in TSC patients compared with controls. The findings may help better understand the underlying physiological mechanisms of brain connection in TSC.
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Conectoma , Esclerosis Tuberosa , Encéfalo/patología , Conectoma/métodos , Interpretación Estadística de Datos , Humanos , Imagen por Resonancia Magnética/métodos , Esclerosis Tuberosa/diagnóstico por imagen , Esclerosis Tuberosa/patologíaRESUMEN
Telomeres cap the ends of eukaryotic chromosomes and are indispensable chromatin structures for genome protection and replication. Telomere length maintenance has been attributed to several functional modulators, including telomerase, the shelterin complex, and the CST complex, synergizing with DNA replication, repair, and the RNA metabolism pathway components. As dysfunctional telomere maintenance and telomerase activation are associated with several human diseases, including cancer, the molecular mechanisms behind telomere length regulation and protection need particular emphasis. Cancer cells exhibit telomerase activation, enabling replicative immortality. Telomerase reverse transcriptase (TERT) activation is involved in cancer development through diverse activities other than mediating telomere elongation. This review describes the telomere functions, the role of functional modulators, the implications in cancer development, and the future therapeutic opportunities.
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BACKGROUND: There is growing evidence suggesting that air pollution may act as an important environmental risk factor in the development and aggravation of childhood atopic dermatitis (AD). METHODS: We collected data from the Taiwan National Health Insurance (NHI) research database and linked the data to the Taiwan Air Quality-Monitoring Database. From January 1, 2000 to December 31, 2012; children aged below 18 years were selected from the database and followed longitudinally until the diagnosis of AD, withdrawal from the NHI, or December 31, 2012. Children with missing data or those diagnosed with AD before enrolment in this study were excluded. We measured the incidence rate and hazard ratios (HRs) for AD and stratified them by quartiles (Q1-Q4) of air pollutant concentration. Multivariable Cox proportional hazards models were also applied by adjusting for age, sex, monthly income, and level of urbanization. RESULTS: When compared with the concentrations of pollutants in the Q1 quartile, the adjusted HR for AD increased with an increase in the exposure concentrations of total hydrocarbons (THCs), non-methane hydrocarbons (NMHCs), and methane (CH4) from 1.65 (95% confidence interval [CI]: 1.47-1.84) to 10.6 (95% CI: 5.85-7.07), from 1.14 (95% CI: 1.06-1.24) to 2.47 (95% CI: 2.29-2.66), and from 1.70 (95% CI: 1.52-1.89) to 11.9 (95% CI: 10.8-13.1), respectively. Patients exposed to higher levels of THCs, NMHCs, and CH4 exhibited greater incidence rates of childhood AD. CONCLUSIONS: The present study demonstrated that exposure to higher concentrations of THCs, NMHCs, and CH4 were associated with an increased risk of childhood AD.
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Contaminación del Aire/efectos adversos , Dermatitis Atópica/epidemiología , Exposición a Riesgos Ambientales/efectos adversos , Hidrocarburos/toxicidad , Niño , Estudios de Cohortes , Bases de Datos Factuales , Femenino , Humanos , Incidencia , Masculino , Estudios Retrospectivos , Taiwán/epidemiologíaRESUMEN
Previous studies have indicated that prenatal exposure to endocrine-disrupting chemicals (EDCs) can cause adverse neuropsychiatric disorders in children and adolescents. This study aimed to determine the association between the concentrations of prenatal EDCs and brain structure changes in teenagers by using MRI. We recruited 49 mother-child pairs during the third trimester of pregnancy, and collected and examined the concentration of EDCs-including phthalate esters, perfluorochemicals (PFCs), and heavy metals (lead, arsenic, cadmium, and mercury)-in maternal urine and/or serum. MRI voxel-based morphometry (VBM) and generalized q-sampling imaging (GQI) mapping-including generalized fractional anisotropy (GFA), normalized quantitative anisotropy (NQA), and the isotropic value of the orientation distribution function (ISO)-were obtained in teenagers 13-16 years of age in order to find the association between maternal EDC concentrations and possible brain structure alterations in the teenagers' brains. We found that there are several specific vulnerable brain areas/structures associated with prenatal exposure to EDCs, including decreased focal brain volume, primarily in the frontal lobe; high frontoparietal lobe, temporooccipital lobe and cerebellum; and white matter structural alterations, which showed a negative association with GFA/NQA and a positive association with ISO, primarily in the corpus callosum, external and internal capsules, corona radiata, superior fronto-occipital fasciculus, and superior longitudinal fasciculus. Prenatal exposure to EDCs may be associated with specific brain structure alterations in teenagers.
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Disruptores Endocrinos , Efectos Tardíos de la Exposición Prenatal , Sustancia Blanca , Adolescente , Encéfalo/diagnóstico por imagen , Niño , Disruptores Endocrinos/toxicidad , Femenino , Humanos , Imagen por Resonancia Magnética , Embarazo , Efectos Tardíos de la Exposición Prenatal/inducido químicamente , Sustancia Blanca/diagnóstico por imagenRESUMEN
ABSTRACT: The etiology of dental-supporting tissue diseases in children is multi factorial and not merely related to oral hygiene. Therefore, in the present study, we investigated the relationship between children <18âyears old with allergic rhinitis (AR) and the risk of dental-supporting tissue diseases.Data from the National Health Insurance Research Database (NHIRD) of Taiwan were used to conduct a retrospective longitudinal cohort study. The study cohort comprised 378,160 patients with AR (AR group) and 378,160 patients without AR (non-AR group), who were selected through frequency matching based on age, sex, and the index year. The study patients were followed until dental-supporting tissue diseases occurrence, withdrawal from the National Health Insurance program, or December 31, 2013. Cox proportional hazards regression analysis was conducted to calculate the risk of dental-supporting tissue diseases in the AR group after adjustment for age, sex, and relative comorbidities.The adjusted HRs of periodontal, pulp, and periapical diseases in AR children were higher than those in the non-AR controls (1.51, 95% CI: 1.50 to 1.53; 1.06, 95% CI: 1.05 to 1.07, respectively). The AR to non-AR HRs of these inflammatory dental diseases were particularly higher in children <6âyears old and in boys. The HRs of periodontal, pulp, and periapical diseases were greatest in those with >5 AR-related medical visits/year (5.57, 95% CI: 5.50 to 5.56; 4.06, 95% CI: 4.00 to 4.12, respectively).Children with AR had a greater risk of inflammatory dental-supporting tissue diseases, particularly those <6âyears old with primary teeth, boys, and those with severe persistent AR.
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Enfermedades Periodontales/epidemiología , Rinitis Alérgica/epidemiología , Estudios de Casos y Controles , Niño , Preescolar , Comorbilidad , Bases de Datos Factuales , Femenino , Humanos , Estimación de Kaplan-Meier , Estudios Longitudinales , Masculino , Enfermedades Periodontales/etiología , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Medición de Riesgo , Taiwán/epidemiologíaRESUMEN
Adequate control of pharmacoresistant epilepsy continues to be a challenge. Multiple studies have reported the benefits of epilepsy surgery and vagus nerve stimulation for children with pharmacoresistant epilepsy. Little is known about the role of vagus nerve stimulation for children with failed epilepsy surgeries. The aim of this study was to examine the effects of vagus nerve stimulation on seizure frequency reduction for children with failed epilepsy surgeries. We retrospectively reviewed 85 children with pharmacoresistant epilepsy who underwent vagus nerve stimulation. Six of these patients underwent epilepsy surgery before vagus nerve stimulation (group I) and 79 patients received only vagus nerve stimulation (group II). We recorded seizure frequency at 3, 12, 24 and 36 months after vagus nerve stimulator implantation. Both groups had reduced seizure frequencies at the 3-, 12-, 24- and 36-month follow-up (p = 0.044 for group I trends and 0.008 for group II trends). Vagus nerve stimulator implantations significantly improve seizure frequency for children with or without previous epilepsy surgery at 3, 12, 24 and 36 months. These findings suggest that vagus nerve stimulation should be considered an alternative therapy for pediatric patients with previous failed surgeries.
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Epilepsia/terapia , Estimulación del Nervio Vago , Adolescente , Niño , Preescolar , Epilepsia/cirugía , Femenino , Humanos , Masculino , Procedimientos Neuroquirúrgicos , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: This study aimed to determine whether neonatal hyperbilirubinemia is associated with a risk of autism spectrum disorder (ASD) using a large population-based cohort. STUDY DESIGN: This retrospective cohort study used data from the children's database (2000-2012) of the National Health Insurance Research Database (1996-2012) in Taiwan. We included neonates who were born between 2000 and 2004 and aged <1 month diagnosed with and without hyperbilirubinemia. The primary outcome was physician-diagnosed ASD. At the end of 2012, multivariate Cox's regression analysis was used to estimate hazard ratios (HRs). RESULTS: A total of 67,017 neonates were included. The neonates with hyperbilirubinemia were associated with 1.28-fold increased risk of ASD (HR = 1.28, 95% confidence interval [CI]: 1.05-1.57) compared with those without hyperbilirubinemia. In subanalysis to determine how phototherapy and exchange transfusion treatment for hyperbilirubinemia were associated with ASD showed no association between treatment and ASD, suggesting the lack of a dose-response effect of hyperbilirubinemia on the risk of ASD. Boys had a nearly six-fold higher risk of ASD than girls (HR = 5.89, 95% CI: 4.41-7.86). Additionally, neonates born with preterm birth and low birth weight were associated with a risk of ASD (HR = 1.46, 95% CI: 1.00-2.13). CONCLUSION: We did not observe a dose-response effect of hyperbilirubinemia on ASD, but neonatal hyperbilirubinemia may be an independent risk factor for ASD if there is a residual confounding by other perinatal complications. Therefore, this study does not support a causal link between neonatal hyperbilirubinemia exposure and the risk of ASD.
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Trastorno del Espectro Autista/etiología , Hiperbilirrubinemia Neonatal/complicaciones , Recambio Total de Sangre , Femenino , Humanos , Hiperbilirrubinemia Neonatal/terapia , Recién Nacido de Bajo Peso , Recién Nacido , Enfermedades del Recién Nacido , Recien Nacido Prematuro , Masculino , Fototerapia , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de RiesgoRESUMEN
BACKGROUND: Facial angiofibromas may be present since early childhood in individuals with tuberous sclerosis complex (TSC), causing substantial cosmetic disfigurement. Current therapies are partially effective, but they are uncomfortable, produce scarring, and are especially expensive. OBJECTIVE: The aim of the present study was to evaluate the efficacy of oral everolimus for TSC-associated angiofibromas. METHODS: This retrospective study included TSC patients being treated with oral everolimus for subependymal giant cell astrocytomas (SEGAs) and angiomyolipomas (AMLs). We recorded the changes in facial angiofibromas. Changes in the Angiofibroma Grading Scale (AGS) indicators were recorded according to erythema, average lesion size, lesion density, and percent involvement on the forehead, nose, cheeks, and chin. The scores were recorded before and after the administration of oral everolimus. RESULTS: Twenty-one patients being treated with oral everolimus were enrolled in this study. The mean age was 20.5 years (range 11-44 years, 4 males, and 17 females). The mean dose of oral everolimus was 3.6 mg/day. Clinically meaningful and statistically significant improvement was observed in erythema (p = 0.001), average lesion size (p < 0.001), lesion density (p < 0.001), and percent involvement (p < 0.001). Changes in the AGS findings were statistically significant on the forehead (p = 0.001), nose (p < 0.001) cheeks (p < 0.001), and chin (p = 0.004). CONCLUSION: Everolimus shows evident improvement and is approved for TSC-associated SEGAs and AMLs. The current study demonstrated the efficacy of oral everolimus in reducing facial angiofibromas, showing the parallel benefits of the treatment protocol for TSC.
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Angiofibroma/tratamiento farmacológico , Antineoplásicos/uso terapéutico , Everolimus/uso terapéutico , Neoplasias Faciales/tratamiento farmacológico , Esclerosis Tuberosa/complicaciones , Adolescente , Adulto , Angiofibroma/complicaciones , Angiofibroma/patología , Angiomiolipoma/complicaciones , Angiomiolipoma/tratamiento farmacológico , Astrocitoma/complicaciones , Astrocitoma/tratamiento farmacológico , Niño , Neoplasias Faciales/complicaciones , Neoplasias Faciales/patología , Femenino , Humanos , Neoplasias Renales/complicaciones , Neoplasias Renales/tratamiento farmacológico , Masculino , Estudios Retrospectivos , Esclerosis Tuberosa/patología , Esclerosis Tuberosa/terapia , Adulto JovenRESUMEN
The purpose of this study is to investigate whether listening to music and white noise affects functional connectivity on scalp electroencephalography (EEG) in neonates in the neonatal intensive care unit.Nine neonates of ≥34 weeks' gestational age, who were already undergoing clinical continuous EEG monitoring in the neonatal intensive care unit, listened to lullaby-like music and white noise for 1 hour each separated by a 2-hour interval of no intervention. EEG segments during periods of music, white noise, and no intervention were band-pass filtered as delta (0.5-4 Hz), theta (4-8 Hz), lower alpha (8-10 Hz), upper alpha (10-13 Hz), beta (13-30 Hz), and gamma (30-45 Hz). Synchronization likelihood was used as a measure of connectivity between any 2 electrodes.In theta, lower alpha, and upper alpha frequency bands, the synchronization likelihood values yielded statistical significance with sound (music, white noise and no intervention) and with edge (between any 2 electrodes) factors. In theta, lower alpha, and upper alpha frequency bands, statistical significance was obtained between music and white noise (t = 3.12, 3.32, and 3.68, respectively; P < .017), and between white noise and no intervention (t = 4.51, 3.09, and 2.95, respectively, P < .017). However, there was no difference between music and no intervention.Although limited by a small sample size and the 1-time only auditory intervention, these preliminary results demonstrate the feasibility of EEG connectivity analyses even at bedside in neonates on continuous EEG monitoring in the neonatal intensive care unit. They also point to the possibility of detecting significant changes in functional connectivity related to the theta and alpha bands using auditory interventions.