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Shift work is a prevalent workplace exposure, which increases the possibility of unhealthy behaviours and circadian rhythm disruptions and elevates the risk of metabolic diseases and adverse reproductive outcomes. But its potential of increasing the risk of postpartum weight retention remains uncertain. This study aimed to investigate the association between maternal shift work prior to conception and postpartum weight retention, and to identify modifiable factors during pregnancy for prevention. We analysed data from the Taiwan Birth Cohort Study, a prospective cohort of Taiwanese women who gave birth in 2005. We examined the pre-conceptional shift work status of 13,575 mothers and their body weight before pregnancy, before delivery, six and eighteen months after delivery. We used multivariable linear models to examine associations and effect modifications. Maternal shift work before pregnancy was significantly associated with increased postpartum weight retention at six and eighteen months (ß-estimate for six months: 0.19-kilogram, 95% CI: 0.03-0.34; eighteen months: 0.23-kilogram, 95% CI: 0.04-0.40). The association between shift work and weight retention at six months postpartum was stronger among mothers who were overweight or obese before pregnancy than mothers with normal weight. This study showed the impact of shift work on postpartum weight retention and suggested a stronger association among mothers with overweight or obesity before pregnancy.
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AIM: Cesarean section delivery is associated with microbiota disruption and immuno-dysregulation during childhood, but the association with Kawasaki disease remains uncertain. We aimed to evaluate the association between Cesarean section and Kawasaki disease. METHODS: We examined the association between Kawasaki disease between six and eighteen months and Cesarean section within a birth cohort of 15,796 mother-infant pairs in Taiwan. The associations were assessed with Poisson regression in the study population, in the 1:2 propensity score-matched subpopulation, and compared with febrile convulsion, trauma and accidents during the same interval as negative control outcomes. RESULTS: Cesarean section was found to increase the risk of Kawasaki disease among overall population (adjusted relative risk [aRR]: 2.22, 95 % confidence interval (CI): 1.14-4.34) and the matched subpopulation (aRR: 2.29, 95 % CI: 1.14-4.68 in PS-matched subpopulation). Meanwhile, there was no association between Cesarean section and the clinic visits for febrile convulsion, trauma and accidents. CONCLUSION: In conclusion, this study identified a potential association between Cesarean section delivery and a higher risk of Kawasaki disease during six-to eighteen months of the prospective birth cohort in Taiwan.
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Cesárea , Síndrome Mucocutáneo Linfonodular , Humanos , Síndrome Mucocutáneo Linfonodular/epidemiología , Cesárea/efectos adversos , Cesárea/estadística & datos numéricos , Taiwán/epidemiología , Femenino , Lactante , Embarazo , Masculino , Factores de Riesgo , Adulto , Estudios Prospectivos , Puntaje de Propensión , Distribución de PoissonRESUMEN
Precocious puberty in girls is defined as the onset of pubertal changes before 8 years of age, and gonadotropin-releasing hormone (GnRH) agonist treatment is available for central precocious puberty (CPP). The gold standard for diagnosing CPP is the GnRH stimulation test. However, the GnRH stimulation test is time-consuming, costly, and requires repeated blood sampling. We aimed to develop an artificial intelligence (AI) prediction model to assist pediatric endocrinologists in decision making regarding the optimal timing to perform the GnRH stimulation test. We reviewed the medical charts of 161 girls who received the GnRH stimulation test from 1 August 2010 to 31 August 2021, and we selected 15 clinically relevant features for machine learning modeling. We chose the models with the highest area under the receiver operating characteristic curve (AUC) to integrate into our computerized physician order entry (CPOE) system. The AUC values for the CPP diagnosis prediction model (LH ≥ 5 IU/L) were 0.884 with logistic regression, 0.912 with random forest, 0.942 with LightGBM, and 0.942 with XGBoost. For the Taiwan National Health Insurance treatment coverage prediction model (LH ≥ 10 IU/L), the AUC values were 0.909, 0.941, 0.934, and 0.881, respectively. In conclusion, our AI predictive system can assist pediatric endocrinologists when they are deciding whether a girl with suspected CPP should receive a GnRH stimulation test. With proper use, this prediction model may possibly avoid unnecessary invasive blood sampling for GnRH stimulation tests.
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BACKGROUND: Neonatal hypoglycemia is a common metabolic disorder in newborns, which may present with non-specific symptoms or even be asymptomatic. Current guidelines recommend screening for hypoglycemia in at-risk babies (late preterm, small for gestational age, large for gestational age, and infants of diabetic mothers). Past studies have suggested other potential risk factors, such as maternal obesity, gestational hypertension, cesarean section, etc. In this study, we aim to identify additional prenatal and perinatal maternal/fetal characteristics associated with early asymptomatic hypoglycemia in term and late preterm babies. METHODS: We performed a retrospective review on medical charts of all newborns, born between January, 2017 and December, 2020, in the well-baby newborn nursery of a tertiary medical center. We identified newborns who had received blood glucose concentration monitor after birth. Detailed prenatal and perinatal maternal/newborn information were collected for analysis. RESULTS: In the study period, 841 newborns had received blood glucose screening after birth. After matching by sex and indication for postnatal blood glucose screen (SGA, LGA, and GDM), 148 newborns were included in the "hypoglycemia group" and 296 newborns were included in the "euglycemia group". In the univariate analysis, parity, insulin treatment for gestational diabetes mellitus (GDM), and cesarean section were associated with an increased risk for neonatal hypoglycemia. Factors associated with decreased risk included higher gestational age, longer duration of skin-to-skin contact, neonatal hyperthermia, higher maternal labor pain score, and epidural anesthesia administration. By multivariable analysis, insulin treatment for GDM was identified as an independent factor associated with increased risk for neonatal hypoglycemia. CONCLUSION: Our study showed insulin treatment for GDM to be independently associated with neonatal hypoglycemia. Other risk factors noted in the univariate analysis, such as decreased skin-to-skin contact duration, hypothermia, Cesarean section, and preterm delivery, would require further investigation to confirm the findings.
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Diabetes Gestacional , Hipoglucemia , Enfermedades del Recién Nacido , Insulinas , Nacimiento Prematuro , Recién Nacido , Embarazo , Femenino , Humanos , Cesárea , Glucemia , Hipoglucemia/diagnóstico , Hipoglucemia/epidemiología , Hipoglucemia/etiología , Factores de Riesgo , Retardo del Crecimiento FetalAsunto(s)
Desarrollo Infantil , Discapacidades del Desarrollo , Niño , Estudios de Cohortes , Humanos , Taiwán/epidemiologíaRESUMEN
Chinese culture has long favored sons over daughters. With the development of reproductive technology, the methods can be used not only in facilitation of conception, but also in sex-selective termination of pregnancies. Therefore, this study aimed to investigate: (1) the rate of pregnant expectancy/planning of children; (2) whether the Chinese cultural influence of son preference still exists, and factors effecting sex preference; and (3) whether artificial reproductive technology plays a role in parents giving birth to a child of their preferred sex. We used the Taiwan Birth Cohort Study dataset at six-months, which included 21,248 babies born in 2005. More mothers than fathers reported this pregnancy as unexpected (29.0% vs. 20.5%). Over half of the parents showed no preference for the sex of their child (mothers: 52.6%, fathers: 55.8%). However, among those who showed a preference, significantly more preferred sons than daughters (son preference, mothers: 24.8%, fathers: 24.3%; daughter preference, mothers: 22.5%, fathers: 19.9%). Additionally, structural equation modeling found that parents who had planned their pregnancy were more likely to prefer sons and less likely to prefer daughters. Parents who used artificial reproductive technology for conception were more likely to prefer and conceive sons. A preference for male children still exists in Taiwan, contributing to the high sex ratio at birth of 110 in our 2005 birth cohort. However, over half of the parents reported being neutral in the preference of the gender of their offspring. This suggests that Taiwan is moving toward a more gender-equitable society.
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Padres/psicología , Adolescente , Adulto , Anciano , Estudios de Cohortes , Composición Familiar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Taiwán , Adulto JovenRESUMEN
INTRODUCTION: Secondhand smoke exposure during pregnancy has long been associated with adverse health outcomes in children, but only a few studies have examined its effect modifiers. In this study, we applied effect modification analysis for maternal prepregnancy weight status on detrimental neurodevelopmental effect of secondhand smoke exposure during pregnancy and infancy in a nationwide representative population. AIMS AND METHODS: Term singleton mother-infant pairs with nonsmoking mothers were included for main analysis (N = 15 987) from the Taiwan Birth Cohort Study (TBCS), and were further matched with propensity score (n = 5434). We extracted secondhand smoke exposure during pregnancy and infancy, and eight neurodevelopmental milestones from the responses in the baseline visit at 6 months, and 18-month follow-up of TBCS. The associations between secondhand smoke exposure and neurodevelopmental achievement were analyzed with multivariable logistic regression and Cox model. Propensity score weighting and matching were applied for high-versus-low analysis, and relative excess risk due to interaction were used to estimate effect modification. RESULTS: Higher secondhand smoke exposure was associated with increased likelihood of delayed milestone achievement across gross motor, fine motor, language-related, and social-related domains. The associations in fine motor domains remained observable in propensity score-weighted and -matched models. We identified additive interaction with self-reported maternal overweight and obesity status before pregnancy in milestone development for walking with support, scribbling, and waving goodbye. CONCLUSIONS: Secondhand smoke exposure during pregnancy and infancy were associated with delayed neurodevelopmental milestone achievement at 18 months, and the associations were modified by maternal prepregnancy overweight and obesity status. IMPLICATIONS: The study results suggested the association between maternal secondhand smoke exposure during pregnancy and infancy and delayed fine motor and language-related milestone achievement at 18 months in multivariable, propensity score weighting, and matching populations. The results of positive effect modifications for maternal prepregnancy overweight and obesity status suggested the importance of concurrent interventions on smoke-free environment and maternal health during pregnancy.
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Contaminación por Humo de Tabaco , Niño , Estudios de Cohortes , Femenino , Humanos , Lactante , Modelos Logísticos , Obesidad/epidemiología , Sobrepeso , Embarazo , Contaminación por Humo de Tabaco/efectos adversosRESUMEN
BACKGROUND: Household incense burning is a common ritual behavior in the Asia-Pacific region but has been associated with inferior developmental outcomes in term infants. We aimed to examine these associations among preterm infants. METHODS: Information from 1190 mother-infant pairs during 6- and 18-month follow-up to the Taiwan Birth Cohort Study was examined for associations between household incense burning exposure and infant neurodevelopmental milestone achievement using multivariable Cox proportional hazard model with propensity score weighting, along with stratified, sensitivity, and decomposition analysis. RESULTS: Household incense burning exposure was associated with delayed gross motor milestone achievement among all preterm infants according to the Cox model and after propensity score weighting. Meanwhile, associations for delayed development were found in gross motor domain milestones among late preterm infants, while fine motor domain delay was found among other preterm infants. Furthermore, the associations between household incense burning status and gross motor milestone delays were attenuated by the interaction between higher education level and household incense burning exposure status. CONCLUSIONS: Household incense burning exposure was associated with delays, and the motor domains affected differed according to degree of prematurity. These associations were modified by the attenuation upon higher maternal educational status and exposure status interaction.
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Contaminación del Aire Interior , Escolaridad , Edad Gestacional , Humo , Estudios de Cohortes , Composición Familiar , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , TaiwánRESUMEN
BACKGROUND: Mucopolysaccharidoses (MPSs) are a group of inherited metabolic diseases, which are characterized by the accumulation of glycosaminoglycans, and eventually lead to the progressive damage of various tissues and organs. METHODS: An epidemiological study of MPS in Taiwan was performed using multiple sources. The survival and diagnostic age for different types of MPS between 1985 and 2019 were evaluated. RESULTS: Between 1985 and 2019, there were 175 patients diagnosed with MPS disorders in the Taiwanese population, with a median diagnostic age of 3.9 years. There were 21 (12%), 78 (45%), 33 (19%), 32 (18%) and 11 (6%) patients diagnosed with MPS I, II, III, IV and VI, respectively, with median diagnostic ages of 1.5, 3.8, 4.7, 4.5 and 3.7 years, respectively. Diagnosis of MPS patients was significantly earlier in recent decades (p < 0.01). Pilot newborn screening programs for MPS I, II, VI, IVA, and IIIB were progressively introduced in Taiwan from 2016, and 48% (16/33) of MPS patients diagnosed between 2016 and 2019 were diagnosed by one of these screening programs, with a median diagnostic age at 0.2 years. For patients born between 2016 and 2019, up to 94% (16/17) were diagnosed with MPS via the newborn screening programs. At the time of this study, 81 patients had passed away with a median age at death of 15.6 years. Age at diagnosis was positively correlated with life expectancy (p < 0.01). Life expectancy also significantly increased between 1985 and 2019, however this increase was gradual (p < 0.01). CONCLUSIONS: The life expectancy of Taiwanese patients with MPS has improved in recent decades and patients are being diagnosed earlier. Because of the progressive nature of the disease, early diagnosis by newborn screening programs and timely implementation of early therapeutic interventions may lead to better clinical outcomes.
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Mucopolisacaridosis , Mucopolisacaridosis I , Preescolar , Glicosaminoglicanos , Humanos , Lactante , Recién Nacido , Mucopolisacaridosis/diagnóstico , Mucopolisacaridosis/epidemiología , Tamizaje Neonatal , Taiwán/epidemiologíaRESUMEN
The potential risk of internet use on adolescents' self-harm is a major concern. Vulnerable adolescents who are susceptible to bullying are also susceptible to the negative influence of the internet. In this study, the pathway associations were investigated between the risk factors of deliberate self-harm, experience of being bullied, internet use, and protective factors of maternal monitoring on perceived happiness of 12- and 13-year-old adolescents in the Taiwan Birth Cohort Pilot Study dataset (n = 1,457). The Chinese Oxford Happiness Questionnaire was used to measure the adolescents' self-perceived levels of happiness, in two dimensions of social adaptation status and psychological well-being. Our results show that 354 (24.3%) of the 12-year-olds reported having been bullied, and 289 (19.8%) of the 13-year-olds reported this. Seventy-nine (5.4%) of 13-year-olds reported deliberate self-harm in the past year. Results of a structural equation model showed that those who had been bullied at age 12 years were at greater risk of deliberate self-harm at age 13 years. A negative association was found between duration of internet use and perceived level of happiness. Adolescents who spent >5 h online during days off school were at higher risk of deliberate self-harm, and perceived a lower level of happiness. Therefore, spending >5 h online during days off school could be used as an indicator in future preventive action programs to screen out those at a high risk of excessive internet use, deliberate self-harm, and psychological well-being and social adjustment issues.
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Conducta del Adolescente , Felicidad , Internet , Conducta Autodestructiva/etiología , Adolescente , Acoso Escolar , Niño , Femenino , Humanos , Masculino , Proyectos Piloto , Medios de Comunicación Sociales , TaiwánRESUMEN
BACKGROUND: Rapid weight gain (RWG) has been recognized as an important determinant of childhood obesity. This study aims to explore the RWG distribution among children at six-month intervals from birth to two years old and to examine the association of RWG in each interval with overweight or obesity development in preschool- and school-aged children. METHODS: Data were obtained from the Taiwan Birth Cohort Study, which is a nationally representative sample of 24,200 children who participated in a face-to-face survey. A total of 17,002 children had complete data both for weight and height at each of the five measurement time periods. Multivariable logistic regression models quantified the relationship between RWG and childhood overweight or obesity. RESULTS: A total of 17.5% of children experienced rapid weight gain in the first six months of age, compared to only 1.8% of children from 18-24 months. RWG was significantly associated with an increased risk of developing overweight or obesity at 36 months (RWG birth-6 months: OR = 2.6, 95% CI: 2.3-2.8; RWG 18-24 months: OR = 3.7, 95% CI: 2.9-4.6), 66 months (RWG birth-6 months: OR = 2.2, 95% CI: 2.0-2.4; RWG 18-24 months: OR = 2.3, 95% CI: 1.8-2.8), and 8 years of age (RWG birth-6 months: OR = 1.7, 95% CI: 1.6-1.9; RWG 18-24 months: OR = 2.4, 95% CI: 2.0-3.0). CONCLUSIONS: Childhood RWG increased the risk of subsequent overweight or obesity, regardless of the specific time interval at which RWG occurred before the age of two years. The results reinforce the importance of monitoring childhood RWG continuously and show the risks of childhood RWG with respect to the development of overweight or obesity at preschool and school ages.
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Obesidad Infantil , Peso al Nacer , Índice de Masa Corporal , Niño , Preescolar , Estudios de Cohortes , Humanos , Lactante , Sobrepeso/epidemiología , Sobrepeso/etiología , Obesidad Infantil/epidemiología , Obesidad Infantil/etiología , Factores de Riesgo , Taiwán/epidemiología , Aumento de PesoRESUMEN
This study aimed to investigate (1) the reliability and validity of the Taiwan Birth Cohort Study-Developmental Instrument (TBCS-DI) 8-year-old scale; (2) the pathway relationship among the cognitive, emotional and social-communication dimensions of the TBCS-DI 8-year-old scale; (3) the predictive validity and association of the TBCS-DI dimensions at 6, 18, 36, and 66 months, with the dimensions of the 8-year-old scale. The Taiwan Birth Cohort Study dataset of 19,516 children followed from 6 months to the age of eight was used. The TBCS-DI 8-year-old scale showed good reliability and validity. A recursive relationship of emotion as the basis, affecting cognition and later social communication was found. Children's 18-month development was able to predict the development of children at 8 years, implying that 18 months may be a critical age for screening and intervention. Clinical intervention should also take into consideration the cultural disparity of emotional development in Asian countries.
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Cognición , Comunicación , Regulación Emocional , Desarrollo de la Personalidad , Niño , Preescolar , Estudios de Cohortes , Femenino , Humanos , Lactante , Masculino , Reproducibilidad de los Resultados , TaiwánRESUMEN
Incineration is a solution to waste problems; however, it has adverse effects on human health. Our study aimed to investigate the direct and indirect effects of living near an incinerator and breastfeeding on children's development at 6, 18, 36 and 66 months, and 8 years of age. The Taiwan Birth Cohort Study dataset used included randomized community data on 19,519 children from 6 months to 8 years old. The Taiwan Birth Cohort Study Developmental Instrument was used to measure children's development at different developmental ages. The results of our study showed that living within 3 km of an incinerator had a negative effect on children's 6-month development, however the effect dissipated after 18 months. Having been breastfed and living in the city had a more persistent and pervasive positive effect on children's development. Conversely, living in the city had an adverse effect on children's social-communication and emotional development when they were 8 years old; possibly due to the Chinese cultural characteristic of collectivism. Further follow-up of the long-term interactive effects of proximity to an incinerator and breastfeeding on children's development and health is needed.
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Lactancia Materna , Desarrollo Infantil , Contaminantes Ambientales , Incineración , Eliminación de Residuos , Niño , Preescolar , Estudios de Cohortes , Comunicación , Emociones , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Padres , Análisis de Regresión , Factores de Riesgo , Autoinforme , Conducta Social , Taiwán/epidemiologíaRESUMEN
BACKGROUND: Children with mucopolysaccharidosis (MPS) generally appear unaffected at birth but may develop multiple clinical manifestations including profound growth impairment as they grow older. Each type of MPS has a variable age at onset and variable rate of progression, however, information regarding growth in Asian children is limited. METHODS: This retrospective analysis included 129 Taiwanese patients with MPS (age range, 0.7 to 19.5 years, median age, 7.9 years) from eight medical centers in Taiwan from January 1996 through December 2018. RESULTS: The mean z scores for the first recorded values of height, weight, and body mass index in the patients' medical records were -4.25, -1.04, and 0.41 for MPS I (n = 9), -2.31, 0.19, and 0.84 for MPS II (n = 49), -0.42, 0.08, and -0.12 for MPS III (n = 27), -6.02, -2.04, and 0.12 for MPS IVA (n = 30), and -4.46, -1.52, and 0.19 for MPS VI (n = 14), respectively. MPS IVA had the lowest mean z scores for both height and weight among all types of MPS, followed by MPS VI, MPS I, MPS II, and MPS III, which showed the mildest growth retardation. Both z scores for height and weight were negatively correlated with increasing age for all types of MPS (p < 0.01). Of 32 patients younger than 5 years of age, 16 (50%), and 23 (72%) had positive z scores of height and weight, respectively. A substantial number of younger patients with MPS I, II, III, and IVA had a positive height z score. The median age at diagnosis was 3.9 years (n = 115). CONCLUSIONS: The patients with MPS IVA had the most significant growth retardation among all types of MPS, followed by MPS VI, MPS I, MPS II, and MPS III. The height and weight of the MPS patients younger than 2-5 years of age were higher than those of healthy individuals, however, their growth significantly decelerated in subsequent years. Understanding the growth curve and potential involved in each type of MPS may allow for early diagnosis and timely management of the disease, which may improve the quality of life.
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BACKGROUND: Maternal shift work is associated with preterm delivery, small-for-gestational-age new-borns, childhood obesity and future behavioural problems. However, the adverse effects on and interactions of maternal shift work with infant neurodevelopment remain uncertain. Therefore, we examined the associations between maternal-shift-work status and infant neurodevelopmental parameters. METHODS: The Taiwan Birth Cohort Study is a nationwide birth cohort study following representatively sampled mother-infant pairs in 2005. The participants' development and exposure conditions were assessed by home interviews with structured questionnaires at 6 and 18 months of age. Propensity scores were calculated with predefined covariates for 1:1 matching. Multivariate conditional logistic regression and the Cox proportional-hazards model were used to examine the association between maternal-shift-work status and infant neurodevelopmental-milestone-achievement status. RESULTS: In this study, 5637 term singletons were included, with 2098 cases selected in the propensity-score-matched subpopulation. Persistent maternal shift work was associated with increased risks of delays in gross-motor neurodevelopmental milestones [aOR = 1.36, 95% confidence interval (CI) = 1.06-1.76 for walking steadily], fine-motor neurodevelopmental milestones (aOR = 1.39, 95% CI = 1.07-1.80 for scribbling) and social neurodevelopmental milestones (aOR = 1.35, 95% CI = 1.03-1.76 for coming when called upon). Moreover, delayed gross-motor and social development were identified in the propensity-score-matched sub-cohort. CONCLUSIONS: This study shows negative associations between maternal shift work and delayed neurodevelopmental-milestone achievement in the gross-motor, fine-motor and social domains at 18 months. Future research is necessary to elucidate the possible underlying mechanisms and long-term health effects.
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Desarrollo Infantil , Destreza Motora , Horario de Trabajo por Turnos/efectos adversos , Adulto , Femenino , Humanos , Lactante , Recién Nacido , Modelos Logísticos , Masculino , Análisis Multivariante , Embarazo , Efectos Tardíos de la Exposición Prenatal , Puntaje de Propensión , Modelos de Riesgos Proporcionales , Estudios Prospectivos , Taiwán , Adulto JovenRESUMEN
Children with learning disability (LD), intellectual disability (ID), attention-deficit/hyperactivity disorder (ADHD), and autism spectrum disorder (ASD) reported higher risk of being bullied compared to their peers. Controlling for the co-morbidity of different diagnosis is important in investigating the frequency of bullying. Therefore, this study aimed to investigate the pathway relationship of adolescents' psychiatric diagnoses, including LD, ID, ADHD, ASD, with being bullied, their self-perceived psychological well-being (PWB) and social adaptation status (SAS) in 12-years-olds.The Taiwan Birth Cohort Pilot Study dataset (Nâ=â1561) was used. The Chinese Oxford Happiness Questionnaire was used to measure PWB and SAS.Adolescent-reported rate of bullying was 25.4%, while only 2.8% of the parents reported knowing their child had been bullied. Boys reported higher rate of being bullied than girls. Adolescents with ADHD were not at higher risk of being bullied compared to their peers, nevertheless, they perceived lower level of SAS. Adolescents diagnosed with ID and ASD reported 63% rate of bullying and those who have been bullied perceived lower level of happiness.Adolescents with ADHD reported lower level of SAS, for disruption of harmony is even less acceptable in the Asian culture. Adolescents with ID and ASD reported higher rate of bullying than their peers and perceived lower level of happiness. A gap was found between parent and adolescent-reported rate of bullying. Encouraging adolescents to seek adult protection and support to reduce the effect of bullying on the perceived level of happiness is important.
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Trastorno por Déficit de Atención con Hiperactividad/epidemiología , Trastorno del Espectro Autista/epidemiología , Acoso Escolar/estadística & datos numéricos , Discapacidad Intelectual/epidemiología , Discapacidades para el Aprendizaje/epidemiología , Niño , Comorbilidad , Femenino , Humanos , Masculino , Salud Mental , Prevalencia , Ajuste Social , Taiwán/epidemiologíaRESUMEN
Mucopolysaccharidosis type III (MPS III, Sanfilippo syndrome) has a variable age of onset and variable rate of progression. However, information regarding the natural history of this disorder in Asian populations is limited. A retrospective analysis was carried out for 28 patients with MPS III (types IIIA [n = 3], IIIB [n = 23], and IIIC [n = 2]; 15 males and 13 females; median age, 8.2 years; age range, 2.7-26.5 years) seen in six medical centers in Taiwan from January 1996 through October 2017. The median age at confirmed diagnosis was 4.6 years. The most common initial symptom was speech delay (75%), followed by hirsutism (64%) and hyperactivity (54%). Both z scores for height and weight were negatively correlated with age (r = -.693 and -0.718, respectively; p < .01). The most prevalent clinical manifestations were speech delay (100%) and intellectual disability (100%), followed by hirsutism (93%), hyperactivity (79%), coarse facial features (68%), sleep disorders (61%), and hepatosplenomegaly (61%). Ten patients (36%) had epilepsy, and the median age at the first seizure was 11 years. Thirteen patients (46%) experienced at least one surgical procedure. At the time of the present study, 7 of the 28 patients had passed away at the median age of 13.0 years. Molecular studies showed an allelic heterogeneity without clear genotype and phenotype correlations. MPS IIIB is the most frequent subtype among MPS III in the Taiwanese population. An understanding of the natural history of MPS III may allow early diagnosis and timely management of the disease facilitating better treatment outcomes.
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Mucopolisacaridosis III/diagnóstico , Mucopolisacaridosis III/etiología , Acetilglucosaminidasa/genética , Acetilglucosaminidasa/metabolismo , Adolescente , Adulto , Biomarcadores , Niño , Preescolar , Análisis Mutacional de ADN , Electroencefalografía , Activación Enzimática , Femenino , Estudios de Asociación Genética , Humanos , Estimación de Kaplan-Meier , Masculino , Mucopolisacaridosis III/metabolismo , Mucopolisacaridosis III/mortalidad , Imagen Multimodal/métodos , Mutación , Fenotipo , Estudios Retrospectivos , Evaluación de Síntomas , Taiwán , Adulto JovenRESUMEN
BACKGROUND: Mucopolysaccharidosis type II (MPS II) is the most frequently occurring MPS in Taiwan, with an incidence of 2.05 per 100,000 live male births, but little is known about clinical characteristics and surgical history in Taiwanese patients. METHODS: Medical history, demographics, signs and symptoms, and surgical history were analysed in all patients from Taiwanese centres in the Hunter Outcome Survey (HOS; NCT 03292887), a global, multicentre registry that collects real-world data on patients with MPS II. RESULTS: As of January 2016, 61 male Taiwanese patients were enrolled; 49% (24/49) had received at least one infusion of idursulfase. Median (10th, 90th percentiles) ages at signs and symptom onset and at diagnosis were 2.5 (0.2, 5.5) years (n = 55) and 3.5 (1.2, 11.9) years (n = 56), respectively. Hernia, facial features consistent with MPS II and claw hands were the earliest presenting signs and symptoms (median ages of 3.2 [0.4, 12.0] years, 4.3 [1.1, 12.0] years and 4.7 [2.5, 12.2] years [n = 45, 53 and 50], respectively). More than 75% of patients had undergone a surgical procedure, most commonly hernia repair (57% of patients). Median age at first surgery for hernia repair was 4.2 (0.5, 9.8) years (n = 35). Almost one-third (31.1%) of patients had at least one surgical procedure before diagnosis, and of the 20 procedures before diagnosis, 16 were hernia repair. CONCLUSIONS: This information from patients in HOS highlights the importance of both medical and surgical history in diagnosing MPS II in Taiwanese patients.
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Mucopolisacaridosis II/patología , Mucopolisacaridosis II/cirugía , Causas de Muerte , Preescolar , Herniorrafia , Humanos , Lactante , Enfermedades por Almacenamiento Lisosomal/patología , Enfermedades por Almacenamiento Lisosomal/cirugía , Masculino , TaiwánRESUMEN
BACKGROUND: Air pollution from biomass burning were associated with neurodevelopmental deceleration, but limited studies concerned about the effect of indoor biomass burning. Incense burning is a common household ritual practice in Taiwan, while past studies mainly focused on birth weight and allergic disease. OBJECTIVES: We aimed to find the association between incense burning exposure and children's neurodevelopment. METHODS: In Taiwan Birth Cohort Study (TBCS), a nationwide representative birth cohort study, children were assessed upon home interview with structured questionnaires upon six and eighteen months old. Multivariate logistic and Cox proportional hazard regression adjusting confounding factors were applied to explore the odds ratio (OR) and hazard ratio (HR) between household incense burning exposure and caregiver-reported neurodevelopment milestones. RESULTS: In this study, 15,310 term singletons were included, and household incense burning was associated with delay in gross motor neurodevelopment milestone, such as walking with support (Occasional incense burning: ORâ¯=â¯1.26, 95% confidence interval (CI): 1.08 to 1.47, HRâ¯=â¯1.07, 95% CI: 1.03 to 1.11; persistent incense burning: ORâ¯=â¯1.44, 95% CI: 1.22 to 1.69, HRâ¯=â¯1.11, 95% CI: 1.07 to 1.16) and walking steadily (Occasional incense burning: ORâ¯=â¯1.14, 95% CI: 0.98 to 1.32, HRâ¯=â¯1.07, 95% CI: 1.03 to 1.11, persistent incense burning: ORâ¯=â¯1.24, 95% CI: 1.06 to 1.45, HRâ¯=â¯1.09, 95% CI: 1.04 to 1.13). CONCLUSIONS: Our study suggested household incense burning exposure was associated with delay in gross motor neurodevelopmental milestones. Further research is warranted to elucidate the possible mechanism and causal relationship.
Asunto(s)
Contaminación del Aire Interior/estadística & datos numéricos , Desarrollo Infantil/fisiología , Destreza Motora/fisiología , Humo , Estudios de Cohortes , Femenino , Humanos , Lactante , Taiwán/epidemiologíaRESUMEN
Objective Previous studies have shown inconsistent results with regard to the association between advanced parental age and autism spectrum disorder (ASD). The sociodemographic status of parents has been found to be associated with children with ASD, however. Therefore, a pathway analysis was undertaken of the roles of maternal age and education in ASD diagnosis and community screening, in a national birth cohort database, using a propensity score matching (PSM) method. Method The 6- and 66-month Taiwan Birth Cohort Study dataset was used (N = 20,095). The PSM exact matching method was used to select 1700 families (ratio of 1:4 between ASD diagnosis and control) from the Taiwan Birth Cohort Study dataset. Results (1) The results from the complete dataset and the PSM exact matching dataset both show that the risk of a child being diagnosed with ASD was increased by the mother being over 40 years old. (2) Although more children of mothers with lower-than-average education were positive on screening, more children of mothers with higher-than-average education were also diagnosed with ASD. Conclusions for Practice Advanced maternal age had a higher association with the diagnosis of ASD, and maternal educational disparity was found between ASD clinical diagnosis and community screening. Community and primary medical care services should pay more attention to children of parents with lower education during ASD screening to prevent delayed diagnosis.