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1.
Epilepsy Behav ; 143: 109246, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37187015

RESUMO

Sulfite oxidase deficiency (SOD) and related disorders, especially molybdenum cofactor deficiency (MoCD), are a group of rare and severe neurometabolic disorders caused by gene mutations that affect the sulfur-containing amino acid catabolic pathway. These disorders are characterized by distinctive neuroimaging features such as diffuse cerebral atrophy, multicystic encephalomalacia, and ventriculomegaly in early infancy. These features are essential for early diagnosis and treatment. Moreover, the genetics of these disorders are complex but have been increasingly elucidated in the era of molecular medicine. Therefore, we reviewed 28 articles (published from January 1967 until October 2021) on SOD and MoCD, focusing on their neuroimaging and genetic aspects. We highlighted the differences between SOD and MoCD and other conditions that may mimic them, such as common neonatal hypoxic-ischemic encephalopathy and uncommon neonatal metabolic disorder (Leigh syndrome). We also summarized the current knowledge on the genetic mechanisms and the manifestation of seizure disorders of SOD and MoCD. In conclusion, if clinical, neuroimaging, and neuropathological findings suggest a possible SOD or related disorder; extensive molecular diagnostics should be performed to confirm the diagnosis.


Assuntos
Erros Inatos do Metabolismo dos Aminoácidos , Epilepsia , Sulfito Oxidase , Recém-Nascido , Humanos , Sulfito Oxidase/genética , Epilepsia/etiologia , Epilepsia/genética , Erros Inatos do Metabolismo dos Aminoácidos/diagnóstico por imagem , Erros Inatos do Metabolismo dos Aminoácidos/genética , Neuroimagem
2.
Medicina (Kaunas) ; 59(4)2023 Apr 14.
Artigo em Inglês | MEDLINE | ID: mdl-37109722

RESUMO

Background: Children with congenital heart disease (CHD) have impaired pulmonary function both before and after surgery; therefore, pulmonary function assessments are important and should be performed both before and after open-heart surgery. This study aimed to compare pulmonary function between variant pediatric CHD types after open-heart surgery via spirometry. Methods: In this retrospective study, the data for forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), and the ratio between FEV1 and FVC (FEV1/FVC) were collected from patients with CHD who underwent conventional spirometry between 2015 and 2017. Results: A total of 86 patients (55 males and 31 females, with a mean age of 13.24 ± 3.32 years) were enrolled in our study. The diagnosis of CHD included 27.9% with atrial septal defects, 19.8% with ventricular septal defects, 26.7% with tetralogy of Fallot, 7.0% with transposition of the great arteries, and 46.5% with other diagnoses. Abnormal lung function was identified by spirometry assessments after surgery. Spirometry was abnormal in 54.70% of patients: obstructive type in 29.06% of patients, restrictive type in 19.76% of patients, and mixed type in 5.81% of patients. More abnormal findings were found in patients who received the Fontan procedure (80.00% vs. 35.80%, p = 0.048). Conclusions: Developing novel therapies to optimize pulmonary function will be critical for improving clinical outcomes.


Assuntos
Cardiopatias Congênitas , Transposição dos Grandes Vasos , Masculino , Feminino , Humanos , Criança , Adolescente , Estudos Retrospectivos , Espirometria/métodos , Pulmão , Cardiopatias Congênitas/diagnóstico
3.
Stroke ; 53(4): 1256-1262, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-34844424

RESUMO

BACKGROUND: Previous epidemiological investigations examining the association between Kawasaki disease (KD) and cerebrovascular disease have had conflicting results. We analyzed the association between KD and cerebrovascular disease by conducting a population-based retrospective cohort study designed to investigate the hypothesis that KD could be a risk factor for subsequent cerebrovascular disease. METHODS: From the National Health Insurance Research Database of Taiwan, the data of children (aged 0-18 years old) with KD (n=8467) were collected. Starting with the first year of study observation (referred to as the baseline year), data was collected for each child with KD, and 4 non-KD patients matched for sex, urbanization level of residence, and parental occupation were randomly selected to form the non-KD cohort (n=33 868) for our analysis. For the period from January 1, 2000, to December 31, 2012, we calculated the follow-up person-years for each patient, which is the time from the index date to the diagnosis of cerebrovascular disease, death, or the end of 2012. Furthermore, we compared the incidence, the incidence rate ratio, and the 95% CI of cerebrovascular disease between the KD and non-KD cohorts. RESULTS: The overall cerebrovascular disease incidence rate was found to be 3.19-fold higher, which is significantly higher, in the KD cohort than in the non-KD cohort (14.73 versus 4.62 per 100 000 person-years), and the overall risk of cerebrovascular disease remained higher in the KD cohort (adjusted hazard ratio, 3.16 [95% CI, 1.46-6.85]). Furthermore, children aged <5 years showed a significantly higher risk of subsequent cerebrovascular disease in the KD cohort (adjusted hazard ratio, 3.14 [95% CI, 1.43-6.92]). CONCLUSIONS: This nationwide retrospective cohort study shows that KD may increase the risk of subsequent cerebrovascular disease, especially in those with KD aged <5 years old.


Assuntos
Transtornos Cerebrovasculares , Síndrome de Linfonodos Mucocutâneos , Adolescente , Transtornos Cerebrovasculares/complicações , Transtornos Cerebrovasculares/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Humanos , Incidência , Lactente , Recém-Nascido , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Taiwan/epidemiologia
4.
J Exp Child Psychol ; 222: 105479, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-35696756

RESUMO

The learning of number knowledge in childhood may directly influence children's mathematics learning ability in subsequent periods. Previous studies also show that the difficulties in mathematics learning faced by schoolchildren are mainly rooted in the lack of number knowledge in early childhood. Focusing on the development of numerical knowledge and basic arithmetic skills in early childhood, this study designed a linear number line game based on the theory of the mental number line. Accordingly, this study examined the effectiveness of the linear number line game in children's learning of number concepts and arithmetic skills and compared the effectiveness of the linear number line game with that of two other games (a nonlinear number line game and a non-number-line game). This study adopted a quasi-experimental research design. A total of 140 young children from remote areas of eastern Taiwan participated and were divided into three experimental groups and one control group, and a pretest-posttest experiment was conducted. The experimental results showed that the linear number line game could help children to acquire numerical knowledge effectively, especially in number line estimation compared with other experimental groups. In terms of the learning effectiveness of basic arithmetic skills (e.g., addition, subtraction), the two number line games (linear and nonlinear number line games) are significantly superior to the non-number-line game (traditional number decomposition and synthesis game). This study recommends that preschool teachers use linear number line games to improve children's numerical knowledge and arithmetic skills.


Assuntos
Aprendizagem , Professores Escolares , Criança , Pré-Escolar , Humanos , Conhecimento , Matemática
5.
Int J Clin Pract ; 75(12): e14912, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34549868

RESUMO

BACKGROUND: Appendectomy is one of the most commonly performed surgeries worldwide. Sepsis is a major aetiology of morbidity and mortality in children. Our preliminary research revealed a positive correlation amongst appendectomy and future risk of sepsis in adults. However, to date, the relationship between appendectomy and future risk of sepsis in children remains unknown. The aim of this research was to investigate the relationship between appendectomy and the hazard of future sepsis in children. METHODS: We applied a nationwide population-based cohort to assess whether children who received appendectomy were at increased risk of subsequent sepsis. Overall, 57 261 subjects aged below 18 undergoing appendectomy as appendectomy group and 57 261 matched controls were identified as a non-appendectomy group from the National Health Insurance Research Database in Taiwan. We use propensity score analysis to match the age, sex, urbanisation level and parental occupation at the ratio to 1:1. Multiple Cox regression and stratified analyses were used to appraise the adjusted hazard ratio (aHR) for developing sepsis in children. RESULTS: Children who received appendectomy had a 2.38 times higher risk (aHR: 2.38; 95% confidence interval [CI] = 1.98, 2.87) of developing sepsis than those who did not, and the risk was higher in all age groups (aHR: 2.98, 95% CI = 1.84, 4.83; aHR: 2.45, 95% CI = 1.08, 2.05; aHR: 2.18, 95% CI = 1.70, 2.80 in children aged <6, 7-12 and 13-18 years, respectively). Patients with <1-year follow-up showed a 4.53-fold risk of sepsis in the appendectomy cohort (aHR: 4.53, 95% CI = 2.80, 7.35). Patients with 1-4 and ≥5 years' follow-up showed a 2.19- and 1.94-times risk of sepsis (aHR: 2.19, 95% CI = 1.61, 2.97; aHR: 1.94, 95% CI = 1.48, 2.56 in 1-4 and >5 years, respectively). CONCLUSION: Appendectomy was correlative to a 2.38-fold increased future sepsis risk in children, and the risk in all age groups was higher. More studies to interpret the possible biological mechanisms of the associations amongst sepsis and appendectomy are warranted.


Assuntos
Apendicectomia , Sepse , Adulto , Idoso , Apendicectomia/efeitos adversos , Criança , Humanos , Incidência , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Sepse/epidemiologia , Sepse/etiologia , Taiwan/epidemiologia
7.
BMC Pediatr ; 19(1): 406, 2019 11 04.
Artigo em Inglês | MEDLINE | ID: mdl-31684911

RESUMO

BACKGROUND: Kawasaki disease (KD) is a common vasculitis of childhood in East Asia. The complications of KD ascribed to long-term cardiovascular sequelae are considerably diverse. Although studies have investigated neurodevelopmental problems following KD in the past few decades, they have reported inconsistent conclusions. This study investigated potential epilepsy and associated neurodevelopmental disorders (NDDs) following KD in Taiwanese children. METHODS: We retrospectively analyzed the data of children aged < 18 years with clinically diagnosed KD from January 1, 2005, to December 31, 2015. These patients were followed up to estimate the prevalence of epilepsy and associated NDDs in comparison with the prevalence in general pediatric population in Taiwan and worldwide. RESULTS: A total of 612 patients with an average age of 1.6 years were included. The prevalence of associated NDDs was 16.8% (n = 103/612) in the study group, which consisted of epilepsy, intellectual disability (ID), autism spectrum disorders, Tourette syndrome (TS), attention deficit hyperactivity disorder, (ADHD), and others. Moreover, children with KD had a higher prevalence of epilepsy and TS in both Taiwan and worldwide (epilepsy: 2.61% in the KD group vs 0.33% in Taiwan and 0.05-0.8% in worldwide, p < 0.05; TS: 2.77% in the KD group vs 0.56% in Taiwan and 0.3-1% in worldwide, p < 0.05). The prevalence of ID, ADHD, and developmental language disorders was not significantly different between our study patients and those in Taiwan or worldwide. CONCLUSIONS: Results revealed a higher prevalence rate of NDDs, especially epilepsy and TS, in Taiwanese children with KD than in the general pediatric population in Taiwan. However, these NDDs could be heterogeneous. Children diagnosed with KD were followed up because they had a higher risk of heterogeneous NDDs.


Assuntos
Síndrome de Linfonodos Mucocutâneos/epidemiologia , Transtornos do Neurodesenvolvimento/epidemiologia , Adolescente , Transtorno do Espectro Autista/epidemiologia , Criança , Pré-Escolar , Epilepsia/epidemiologia , Feminino , Saúde Global , Humanos , Lactente , Recém-Nascido , Deficiência Intelectual/epidemiologia , Masculino , Prevalência , Estudos Retrospectivos , Distribuição por Sexo , Taiwan/epidemiologia , Síndrome de Tourette/epidemiologia
8.
BMC Neurol ; 18(1): 150, 2018 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-30236064

RESUMO

BACKGROUND: Noonan syndrome-like disorder with loose anagen hair-2 (NSLH2) is an extremely rare disease caused by a heterozygous mutation in the PPP1CB gene on chromosome 2p23. The syndrome causes not only numerous dysmorphic features but also hypotonia, developmental delay, and even intellectual disability. We report the first case of NSLH2 in Asia and the 16th in the world. Moreover, the first case of PPP1CB-related infantile spasms. The clinical and therapeutic significance is outlined in this paper. CASE PRESENTATION: We found a male infant presented with severe intractable epileptic spasms. Although certain clinical features of somatic dysmorphism were noted, numerous laboratory and neuroimaging studies failed to identify the cause. To determine the underlying etiology, whole-exome sequencing was conducted. We identified a de novo heterozygous mutation, NM_206876.1: c.548A > C (p.Glu183Ala), in the PPP1CB gene. His seizures were almost refractory to conventional antiepileptic drugs but relative seizure control was eventually achieved with a ketogenic diet. CONCLUSION: This result expands the clinical spectrum of NSLH2 and strengthens the association between the PPP1CB gene and epileptic seizures. Furthermore, we suggest that the ketogenic diet can offer seizure reduction in particular drug-resistant epilepsy syndromes. Additional studies are warranted to clarify the pathogenic mechanisms underlying this PPP1CB mutation in epileptic seizures.


Assuntos
Doenças do Recém-Nascido/genética , Proteína Fosfatase 1/genética , Espasmos Infantis/genética , Anormalidades Craniofaciais/genética , Deficiências do Desenvolvimento/genética , Dieta Cetogênica , Heterozigoto , Humanos , Lactente , Recém-Nascido , Deficiência Intelectual/genética , Masculino , Hipotonia Muscular/genética , Mutação , Neuroimagem , Espasmos Infantis/dietoterapia
9.
Acta Neurol Belg ; 124(3): 973-979, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38523222

RESUMO

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.


Assuntos
Lista de Checagem , Comorbidade , Epilepsia , Esclerose Tuberosa , Humanos , Esclerose Tuberosa/complicações , Esclerose Tuberosa/psicologia , Esclerose Tuberosa/epidemiologia , Epilepsia/psicologia , Epilepsia/epidemiologia , Feminino , Masculino , Estudos Transversais , Criança , Adolescente , Adulto Jovem , Adulto , Transtornos Mentais/epidemiologia , Transtornos Mentais/etiologia , Transtornos Mentais/psicologia
10.
Ital J Pediatr ; 50(1): 20, 2024 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-38273353

RESUMO

BACKGROUND: This study aimed to investigate the demographic and clinical characteristics, types of seizure disorders, and antiepileptic drug usage among individuals with different types of corpus callosum disorders. METHODS: A total of 73 individuals were included in the study and divided into three groups based on the type of corpus callosum abnormality: hypoplasia (H), agenesis (A), and dysgenesis (D). Demographic data, including gender and preterm birth, as well as clinical characteristics such as seizure disorders, attention deficit hyperactivity disorder (ADHD), severe developmental delay/intellectual disability, and other brain malformations, were analyzed. The types of seizure disorders and antiepileptic drugs used were also examined. RESULTS: The H group had the highest number of participants (n = 47), followed by the A group (n = 11) and the D group (n = 15). The A group had the highest percentage of males and preterm births, while the D group had the highest percentage of seizure disorders, other brain malformations, and severe developmental delay/intellectual disability. The A group also had the highest percentage of ADHD. Focal seizures were observed in all three groups, with the highest proportion in the A group. Focal impaired awareness seizures (FIAS) were present in all groups, with the highest proportion in the D group. Generalized tonic-clonic seizures (GTCS) were observed in all groups, with the highest proportion in the H group. Different types of antiepileptic drugs were used among the groups, with variations in usage rates for each drug. CONCLUSION: This study provided insights into the demographic and clinical characteristics, seizure disorders, and antiepileptic drug usage among individuals with different types of corpus callosum disorders. Significant differences were found between the groups, indicating the need for tailored management approaches. However, the study has limitations, including a small sample size and a cross-sectional design. Further research with larger sample sizes and longitudinal designs is warranted to validate these findings and explore the relationship between corpus callosum abnormality severity and clinical outcomes.


Assuntos
Epilepsia , Deficiência Intelectual , Nascimento Prematuro , Criança , Masculino , Feminino , Recém-Nascido , Humanos , Anticonvulsivantes/uso terapêutico , Corpo Caloso , Estudos Transversais , Epilepsia/tratamento farmacológico , Epilepsia/epidemiologia , Convulsões/tratamento farmacológico , Convulsões/epidemiologia , Demografia
11.
J Tradit Complement Med ; 14(1): 109-120, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38223810

RESUMO

Purpose: Obstructive sleep apnea (OSA) is a chronic disease that affects 1%-6% of children. Our study aims to explore the effectiveness and clinical characteristics of integrative Traditional Chinese Medicine (ITCM) for pediatric OSA. Materials and methods: In this retrospective cohort study, we assessed differences of polysomnography (PSG) parameters and clinical characteristics between 2009 and 2020. Children <12 years old diagnosed with OSA (n = 508) were included and were categorized into ITCM cohort, western medicine (WM) cohort ,and surgery cohort. Outcomes were apnea-hypopnea index (AHI), respiratory disturbance index (RDI), and body mass index (BMI). Results: There were 56 (11%), 324 (63.8%), and 128 (25.2%) patients in the ITCM, WM, and surgery cohorts. Among 17, 26, and 33 patients in the ITCM, WM, and surgery cohorts underwent follow-up PSG studies, respectively. In the ITCM follow-up cohort, AHI were significantly reduced (9.59 to 5.71, p < 0.05). BMI significantly increased in the WM follow-up cohort (19.46 to 20.50, p < 0.05) and the surgery follow-up cohort (18.04 to 18.85, p < 0.01). Comparing ITCM to WM cohort, a significant difference was found between the changes in RDI (ITCM: -6.78, WM: 0.51, p < 0.05) after treatment. Among ITCM follow-up cohort, the most prescribed TCM formula was Forsythia and Laminaria Combination. The most prescribed TCM herb was Ephedrae Herba. Conclusions: ITCM therapy can significantly reduce RDI and control BMI. We identified potential TCM treatments for pediatric OSA. Further study of the pharmacological mechanisms and clinical efficacy is warranted.

12.
J Pediatr Hematol Oncol ; 35(8): e320-2, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-23274375

RESUMO

Thromboembolism presenting with malignancy is common in adults but rare in children. We describe the case of a 17-year-old boy admitted to our hospital with syncope. Computed tomography revealed thromboembolism in both the lungs. Magnetic resonance imaging found thromboembolism in the inferior vena cava and a large heterogeneous mass in the pelvis. Pelvic osteosarcoma was confirmed by computed tomography-guided biopsy. Despite intensive chemotherapy and local radiation, only transient response was noted, the tumor remaining unresectable. To our knowledge, this is the first reported case of simultaneous pulmonary and inferior vena cava thromboembolism secondary to pelvic osteosarcoma in children. We also emphasize syncope as a unique feature of pulmonary thromboembolism. Accordingly, thromboembolism should be kept in mind as the first manifestation of occult malignancy, even in children.


Assuntos
Neoplasias Ósseas/complicações , Osteossarcoma/complicações , Neoplasias Pélvicas/complicações , Embolia Pulmonar/etiologia , Veia Cava Inferior/patologia , Adolescente , Neoplasias Ósseas/patologia , Humanos , Masculino , Osteossarcoma/patologia , Neoplasias Pélvicas/patologia , Síncope/etiologia , Tromboembolia Venosa/etiologia
13.
Pediatr Neonatol ; 2023 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-38052685

RESUMO

BACKGROUND: Gestational diabetes mellitus has been linked to inflammation, immune dysregulation in offspring, and changes in the microbiota. It may have long-term implications for the health of children. The aim of this study was to determine if gestational diabetes mellitus increases the risk of allergic diseases in offspring. METHODS: The data source was the National Health Insurance Research Database (NHIRD) of Taiwan. The pairing of mothers and children was established by connecting the NHIRD with the Taiwan Maternal and Child Health Database. First-time pregnant mothers between 2004 and 2019 were enrolled. The GDM group consisted of 22,741 cases. The control group was selected from individuals without GDM matched by maternal age, neonatal gender, and neonatal birthdate at a ratio of 1-4. The primary endpoint was the incidence of childhood allergic diseases, such as asthma, allergic rhinitis, atopic dermatitis, and urticaria. The secondary endpoint was the risk associated with the development of allergic diseases in offspring, considering the presence or absence of insulin therapy. RESULTS: The development of allergic rhinitis, atopic dermatitis, and urticaria were found to be significantly associated with GDM. However, no significant association was observed between GDM and asthma. GDM control without insulin was associated with an increased risk of developing allergic rhinitis, urticaria, and atopic dermatitis. However, in the group receiving insulin treatment, there was no significant elevation in the risk of any allergic diseases. CONCLUSION: GDM may elevate the risk of certain atopic diseases in offspring, such as allergic rhinitis, atopic dermatitis, and urticaria.

14.
Assist Technol ; : 1-8, 2023 Oct 05.
Artigo em Inglês | MEDLINE | ID: mdl-37796439

RESUMO

This study aimed to improve hand performance and play behavior in children with developmental disabilities (DD) using a remodeled glove puppetry approach. Overall, 62 children with DD were randomly assigned to experimental and control groups (n = 31 each). The experimental group underwent a 12-week rehabilitation program by playing with the remodeled glove puppetry, while the children in the control group played with non-remodeled glove puppetry. The Chinese puppet was remodeled using a Lego EV3® robot. Hand kinematics were analyzed through the Siliconcoach® Pro 7 software, which measured the force produced by the baseline ® hydraulic pinch gauge. Play behavior was measured using the Knox Preschool Play Scale-revised (KPPS-r). The experimental group exhibited significant improvements compared to the control group in hand kinematics (wrist range of motion [ROM], p < .05; metacarpophalangeal ROM, p < .05; proximal interphalangeal ROM, p < .05) and KPPS-r scores (space management, p < .05; material management, p < .05; pretense-symbolic, p < .05; participation, p < .05). After the 12-week rehabilitation with the remodeled glove puppetry, the experimental group exhibited significant improvement in kinematics and KPPS-r scores.

15.
PLoS One ; 18(11): e0294029, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37992011

RESUMO

Sustained pharyngeal inflation (SPI) with pharyngeal oxygen flow and nasal closure (PhO2-NC) technique create positive inflation pressure in the airway. This study measured the peak inflation pressure (PIP) levels and image changes with SPI-assisted flexible bronchoscopy (SPI-FB) and compared the effects in the pharyngeal space and mid-tracheal lumen. This prospective study enrolled 20 participants aged 6 months to 3 years. Each participant underwent sequential SPI-FB of four different durations (0, 1s, 3s, and 5s) for three cycles. We used a 3.8 mm OD flexible bronchoscope to measure and analyze PIP levels, images, and lumen dimension scores. A total of 480 data were collected. The mean (SD) age and body weight were 12.0 (11.5) months and 7.8 (7.5) kg, respectively. The mean (IQR) PIPs were 4.2 (2.0), 18.5 (6.1), 30.6 (13.5), and 46.1 (25.0) cmH2O in the pharynx and 5.0 (1.6), 17.5 (6.5), 28.0 (12.3), 46.0 (28.5) cmH2O in the mid-trachea at SPI durations of 0, 1s, 3s, and 5s, respectively. The PIP levels had a positive correlation (p <0.001) with different SPI durations in both pharynx and trachea, and were nearly identical (p = 0.695, 0.787, and 0.725 at 1s, 3s, and 5s, respectively) at the same duration except the 0 s (p = 0.015). Lumen dimension scores also significantly increased with increasing SPI durations (p <0.05) in both locations. The identified lesions significantly increased as PIP levels increased (p <0.001). Conclusion: SPI-FB using PhO2-NC with durations up to 3s is safe and informative technique that provides controllable PIP, dilates airway lumens, and benefits lesion detection in the pharyngeal space and mid-tracheal lumen.


Assuntos
Broncoscopia , Faringe , Humanos , Lactente , Broncoscopia/métodos , Estudos Prospectivos , Traqueia/diagnóstico por imagem , Oxigênio
16.
Clin Psychopharmacol Neurosci ; 21(3): 544-558, 2023 Aug 31.
Artigo em Inglês | MEDLINE | ID: mdl-37424422

RESUMO

Objective: Limited evidence exists regarding real-world 3-monthly paliperidone palmitate (PP3M) treatment retention and associated factors. Methods: We conducted a retrospective, nationwide cohort study using the Taiwan National Health Insurance Research Database between October 2017 and December 2019. Adult patients with schizophrenia initiated on PP3M were enrolled. The primary outcomes were time to PP3M discontinuation, time to psychiatric hospitalization, and the proportions of patients receiving the next PP3M dose within 120 days among first-, second-, and third-dose completers. Key covariates included prior PP1M duration and adequate PP3M initiation. Results: The PP3M treatment retention rates were 79.7%, 66.3%, and 52.5% after 6, 12, and 24 months, respectively, with 86.4%, 90.6%, and 90.0% of respective first-, second-, and third-dose completers receiving the next PP3M dose. Adequate PP3M initiation and prior PP1M treatment duration > 180 days were associated with favorable PP3M treatment retention. In multivariate analyses, PP1M durations of 180-360 days (adjusted relative risk [aRR], 1.76) or < 180 days (aRR, 2.79) were associated with PP3M discontinuation at the second dose. Inadequate PP3M initiation was associated with discontinuation at the third dose (aRR, 2.18). Patients fully adherent to PP3M treatment in the first year had a higher probability of being free from psychiatric hospitalization (86.7% at 2 years), compared with those partially adherent or non-adherent to PP3M in the first year. Conclusion: Prior PP1M duration and adequate PP3M initiation are major factors affecting PP3M treatment retention. Higher PP3M treatment retention is associated with a lower risk of psychiatric hospitalization.

17.
Children (Basel) ; 10(3)2023 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-36980114

RESUMO

Advances in disease-related gene discovery have led to tremendous innovations in the field of epilepsy genetics. Identification of genetic mutations that cause epileptic encephalopathies has opened new avenues for the development of targeted therapies. Clinical testing using extensive gene panels, exomes, and genomes is currently accessible and has resulted in higher rates of diagnosis and better comprehension of the disease mechanisms underlying the condition. Children with developmental disabilities have a higher risk of developing epilepsy. As our understanding of the mechanisms underlying encephalopathies and epilepsies improves, there may be greater potential to develop innovative therapies tailored to an individual's genotype. This article provides an overview of the significant progress in epilepsy genomics in recent years, with a focus on developmental and epileptic encephalopathies in children. The aim of this review is to enhance comprehension of the clinical utilization of genetic testing in this particular patient population. The development of effective and precise therapeutic strategies for epileptic encephalopathies may be facilitated by a comprehensive understanding of their molecular pathogenesis.

18.
Front Med (Lausanne) ; 10: 1178041, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37144031

RESUMO

Background: Bacterial coinfections have been widely recognized in adults with coronavirus disease 2019 (COVID-19). However, bacterial coinfections in hospitalized children with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have not been sufficiently researched. This study aimed to determine the clinical presentations and risk factors for bacterial coinfections of pediatric inpatients during the SARS-CoV-2 Omicron BA.2 variant pandemic. Methods: This retrospective, observational study included patients younger than 18 years of age who were hospitalized for COVID-19 confirmed by polymerase chain reaction (PCR) or antigen rapid tests during the SARS-CoV-2 Omicron BA.2 variant pandemic. Data and outcomes of these patients with or without bacterial coinfections were compared. Results: During this study period, 161 children with confirmed COVID-19 were hospitalized. Twenty-four had bacterial coinfections. The most frequently reported concurrent diagnosis was bacterial enteritis, followed by lower respiratory tract infections. Children with bacterial coinfections had higher white blood cell (WBC) counts and PCR cycle threshold values. The bacterial coinfection group comprised a relatively greater proportion of patients who required high-flow nasal cannula oxygen and remdesivir. The length of stay in the hospital and that in the intensive care unit were longer for children with COVID-19 with bacterial coinfections. Mortality was not observed in either group. Abdominal pain, diarrhea, and comorbidity with neurologic illnesses were risk factors for bacterial coinfections with COVID-19. Conclusion: This study provides clinicians with reference points for the detection of COVID-19 in children and its possible association with bacterial infections. Children with COVID-19 and neurologic diseases who present with abdominal pain or diarrhea are at risk of bacterial coinfections. Prolonged fever duration and higher PCR test cycle threshold values, WBC levels, and high-sensitivity C-reactive protein (hsCRP) levels may indicate bacterial coinfections in children with COVID-19.

19.
Abdom Imaging ; 37(4): 561-5, 2012 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21947235

RESUMO

Internal hernias, protrusion of abdominal viscera into an intraperitoneal fossa, are uncommon causes of bowel obstruction, and preoperative diagnoses are difficult. We report a rare case of a 47-year-old female with strangulated small bowel obstruction secondary to an intramesosigmoid hernia preoperative diagnosis by multi-detector row computed tomography. We highlight the preoperatively diagnosed value and findings of MDCT in intramesosigmoid hernia.


Assuntos
Hérnia/diagnóstico por imagem , Mesocolo , Tomografia Computadorizada Multidetectores , Doenças Peritoneais/diagnóstico por imagem , Feminino , Herniorrafia , Humanos , Íleus/diagnóstico por imagem , Obstrução Intestinal/diagnóstico por imagem , Obstrução Intestinal/etiologia , Mesocolo/diagnóstico por imagem , Pessoa de Meia-Idade , Doenças Peritoneais/cirurgia , Período Pré-Operatório
20.
Sci Rep ; 12(1): 3415, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-35233028

RESUMO

Kawasaki disease (KD) is the most common form of acquired pediatric cardiac disease in the developed world. However, its etiology is still unclear. Epidemiological studies have shown that air pollution is a plausible risk factor in stimulating oxidative stress, inducing inflammation and causing autoimmune diseases. This study aims to assess the connections between prenatal and early life air pollution exposure to the incidence of KD. The main data source of this nationwide longitudinal study was the National Health Insurance Research Database (NHIRD) of Taiwan. NHIRD was linked with Taiwan Maternal and Child Health Database to establish the link between mothers and children. In total, 4192 KD cases involving children under 6 years of age were identified between January 2004 and December 2010. Children in the control group were randomly selected at a 1:4 ratio and matched using their age and index year. Integrated data for the air pollutants were obtained from 71 Environmental Protection Agency monitoring stations across Taiwan. Patients who had main admission diagnosis of KD and subsequently received intravenous immunoglobulin treatment were defined as incidence cases. Ambient exposure, including pollutant standards index (PSI), carbon monoxide (CO), nitric oxide (NO), nitric dioxide (NO2), and nitrogen oxide (NOx) during pregnancy were all positively associated with KD incidence. Conversely, ozone (O3) exposure had a negative correlation. Exposure to CO, NO, NO2, and NOx after childbirth remained consistent with regards to having a positive association with KD incidence. Exposure to PSI and O3 after delivery displayed no significant association with KD. Both prenatal and postnatal cumulative CO, NO, NO2, and NOx exposure had a dose dependent effect towards increasing KD incidence. Certain prenatal and early life air pollutant exposure may increase the incidence of KD.


Assuntos
Poluentes Atmosféricos , Poluição do Ar , Síndrome de Linfonodos Mucocutâneos , Ozônio , Poluentes Atmosféricos/análise , Poluentes Atmosféricos/toxicidade , Poluição do Ar/efeitos adversos , Poluição do Ar/análise , Pré-Escolar , Exposição Ambiental/análise , Feminino , Humanos , Incidência , Estudos Longitudinais , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Síndrome de Linfonodos Mucocutâneos/etiologia , Óxido Nítrico , Dióxido de Nitrogênio/análise , Óxidos de Nitrogênio/análise , Ozônio/análise , Material Particulado/análise , Gravidez
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