Browsing by Author "Lee, Wang-Tso"

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Cross-section and feasibility study on the non-invasive evaluation of muscle hemodynamic responses in Duchenne muscular dystrophy by using a near-infrared diffuse optical technique
(2018-10-01)
Weng, Wen-Chin
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Chen, Jung-Chih
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Lee, Chia-Yen
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Lin, Chia-Wei
;
Lee, Wang-Tso
Duchenne muscular dystrophy (DMD) is an X-linked debilitating muscular disease that may decrease nitric oxide (NO) production and lead to functional muscular ischemia. Currently, the 6-minute walk test (6-MWT) and the North Star Ambulatory Assessment (NSAA) are the primary outcome measures in clinical trials, but they are severely limited by the subjective consciousness and mood of patients, and can only be used in older and ambulatory boys. This study proposed using functional near-infrared spectroscopy (fNIRS) to evaluate the dynamic changes in muscle hemodynamic responses (gastrocnemius and forearm muscle) during a 6-MWT and a venous occlusion test (VOT), respectively. Muscle oxygenation of the forearm was evaluated non-invasively before, during and after VOT in all participants (included 30 DMD patients and 30 age-matched healthy controls), while dynamic muscle oxygenation of gastrocnemius muscle during 6-MWT was determined in ambulatory participants (n = 18) and healthy controls (n = 30). The results reveal that impaired muscle oxygenation was observed during 6-MWT in DMD patients that may explain why the DMD patients walked shorter distances than healthy controls. Moreover, the results of VOT implied that worsening muscle function was associated with a lower supply of muscle oxygenation and may provide useful information on the relationship between muscular oxygen consumption and supply for the clinical diagnosis of DMD. Therefore, the method of fN1RS with VOT possesses great potential in future evaluations of DMD patients that implies a good feasibility for clinical application such as for monitoring disease severity of DMD. (C) 2018 Optical Society of America under the terms of the OSA Open Access Publishing Agreement
Increased risk of epilepsy in children with Tourette syndrome: A population-based case-control study
(2016-04-05)
Wong, Lee Chin
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Huang, Hui-Ling
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Weng, Wen-Chin
;
Jong, Yuh-Jyh
;
Yin, Yun-Ju
The association between epilepsy and Tourette syndrome has rarely been investigated. In this retrospective cohort study, we analyzed a dataset of 1,000,000 randomly sampled individuals from the Taiwan National Health Insurance Research Database to determine the risk of epilepsy in children with Tourette syndrome. The study cohort consisted of 1062 patients with Tourette syndrome aged <= 18 years, and the control group consisted of three times the number of age- and sex-matched patients without Tourette syndrome, who were insurants, from the same database during the same period. The Tourette syndrome group had an 18.38-fold increased risk of epilepsy than the control group [hazard ratio = 18.38, 95% confidence interval (Cl) = 8.26-40.92; P < 0.001]. Even after adjusting for the comorbidities, the risk of epilepsy in the Tourette syndrome group with comorbidities remained high (hazard ratio = 16.27, 95% CI = 6.26-18.46; P < 0.001), indicating that the increased risk was not associated with comorbidities. This population-based retrospective cohort study provides the first and strong evidence that Tourette syndrome is associated with a higher risk of epilepsy. A close follow-up of children with Tourette syndrome for the development of epilepsy is warranted. (C) 2015 Elsevier Ltd. All rights reserved.
Increased risks of tic disorders in children with epilepsy: A nation-wide population-based case control study in Taiwan
(2016-04-05)
Weng, Wen-Chin
;
Huang, Hui-Ling
;
Wong, Lee Chin
;
Jong, Yuh-Jyh
;
Yin, Yun-Ju
Both epilepsy and tic disorders may share common mechanisms with the involvement of abnormal cortical-basal ganglion circuit connection and dopaminergic dysfunction. However, the association between epilepsy and tic disorders has never been studied. This study investigated the risks of developing tic disorders among children with epilepsy using databases of a universal health insurance system in Taiwan. The data analyzed in this study were retrieved from the National Health Insurance Research Database in Taiwan. The study cohort included children with epilepsy between 2001 and 2007 (n = 2629) and a three-fold age- and gender-matched controls (n = 7887). All subjects were followed up for 3 years from the date of cohort entry to identify their admissions due to tic disorders (ICD-9-CM codes 307.2, 307.20-307.23). Cox hazard regression analysis was performed to estimate the effect of epilepsy on the occurrence of tics. The epilepsy cohort had a higher prevalence of tics (1.7% vs. 0.2%), and a 8.70-fold increased risk of developing a tic disorder compared with the controls (adjusted hazard ratio (AHR) 8.70, 95% confidence interval (CI) 4.26-16.37, p < 0.001). Male patients were observed to have a higher risk of developing a tic disorder (AHR 1.90, 95% CI = 1.04-3.46, p < 0.001) compared to female individuals. Patients with multiple antiepileptic drugs treatment also exhibited higher crude OR for developing tic disorders. This nationwide population-based cohort study, for the first time, demonstrated that there is a significantly increased risk for tic disorders among children with epilepsy. We also found males, attention deficit disorder and the use of multiple AEDs to be independent risk factors of tic disorders. Closely evaluating possible tic disorders would be crucial for improving the outcome and life quality in children with epilepsy. (C) 2015 Elsevier Ltd. All rights reserved.
Presymptomatic Diagnosis of Spinal Muscular Atrophy Through Newborn Screening
(2017-11-01)
Chien, Yin-Hsiu
;
Chiang, Shu-Chuan
;
Weng, Wen-Chin
;
Lee, Ni-Chung
;
Lin, Ching-Jie
Objective To demonstrate the feasibility of presymptomatic diagnosis of spinal muscular atrophy (SMA) through newborn screening (NBS). Study design We performed a screening trial to assess all newborns who underwent routine newborn metabolic screening at the National Taiwan University Hospital newborn screening center between November 2014 and September 2016. A real-time polymerase chain reaction (RT-PCR) genotyping assay for the SMN1/SMN2 intron 7 c. 888+100A/G polymorphism was performed to detect homozygous SMN1 deletion using dried blood spot (DBS) samples. Then the exon 7 c. 840C>T mutation and SMN2 copy number were determined by both droplet digital PCR (ddPCR) using the original screening DBS and multiplex ligation-dependent probe amplification (MLPA) using a whole blood sample. Results Of the 120 267 newborns, 15 tested positive according to the RT-PCR assay. The DBS ddPCR assay excluded 8 false-positives, and the other 7 patients were confirmed by the MLPA assay. Inclusion of the second-tier DBS ddPCR screening assay resulted in a positive prediction value of 100%. The incidence of SMA was 1 in 17 181 (95% CI, 1 in 8323 to 1 in 35 468). Two of the 3 patients with 2 copies of SMN2 and all 4 patients with 3 or 4 copies of SMN2 were asymptomatic at the time of diagnosis. Five of the 8 false-positives were caused by intragenic recombination between SMN1 and SMN2. Conclusion Newborn screening can detect patients affected by SMA before symptom onset and enable early therapeutic intervention. A combination of a RT-PCR and a second-tier ddPCR can accurately diagnose SMA from DBS samples with no false-positives.
Tourette Syndrome as an Independent Risk Factor for Subsequent Sleep Disorders in Children: A Nationwide Population-Based Case-Control Study
(2017-03-01)
Lee, Wang-Tso
;
Huang, Hui-Ling
;
Wong, Lee Chin
;
Weng, Wen-Chin
;
Vasylenko, Tamara
Study objectives: Tourette syndrome (TS) is associated with a variety of neuropsychiatric comorbidities. However, the relationship between TS and sleep disorders in children is less investigated. This nationwide population-based case-control study aimed to determine the correlation of TS and sleep disorders in children. Methods: Patients aged less than 18 years with newly diagnosed TS from 2001 to 2007 were collected (n = 1124) using data from Taiwan's National Health Insurance Research Database and were compared with a comparison cohort (n = 3372). The adjusted hazard ratio (aHR) for developing sleep disorders was calculated by multivariate Cox proportional hazards model. Results: TS was more prevalent in boys, with a male to female ratio of 3.16: 1. TS group also had significantly higher urbanization level of residence than controls (p <.001). The overall incidence rate of sleep disorders was 7.24 parts per thousand in children with TS, compared to 3.53% in controls. The TS group was associated with a significantly higher rate of sleep disorders, with a crude HR of 2.05 (95% confidence inerval [ CI] = 1.43-2.95, p <.001). Among the comorbidities of TS, anxiety disorder was associated with the highest risk for sleep disorders (crude HR = 3.26, 95% CI = 1.52-7.00, p <.001). The aHR for TS cohort to develop sleep disorders was 1.72 (95% CI = 1.16-2.53, p =.007). Conclusions: The increased risk of sleep disorders in children with TS cannot be fully attributed to its comorbidities, and TS is an independent risk factor for sleep disorders in children.