Browsing by Author "Chen, Tai-Heng"

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Combined Noninvasive Ventilation and Mechanical In-Exsufflator in the Treatment of Pediatric Acute Neuromuscular Respiratory Failure
(2014-06-01)
Chen, Tai-Heng
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Hsu, Jong-Hau
;
Wu, Jiunn-Ren
;
Dai, Zen-Kong
;
Chen, I-Chen
ObjectivesThe present study aims to evaluate the efficacy and complications of combined noninvasive ventilation (NIV) and assisted coughing by mechanical in-exsufflator (MIE) for acute respiratory failure (ARF) in children with neuromuscular disease (NMD). MethodsA prospective study was conducted in the pediatric intensive care unit. Children with NMD and ARF treated by combined NIV and MIE were included. Treatment success was defined as freedom from tracheal intubation during the hospital stay. Physiologic indices including PaO2, PaCO2, pH, and PaO2/FiO(2) were recorded before and 12, 24hr after the use of NIV/MIE. ResultsCombined NIV/MIE was used in 15 NMD children (mean: 8.1 years, range: 3 months to 18 years) with 16 cases of ARF. There was no mortality in this cohort. Treatment success was achieved in 12 cases (75%), including six cases (38%) demanding Do Not Intubate. ARF was due to pneumonia, with a mean baseline PaCO2 of 73.219.0mmHg. In the success group, hypercarbia and acidosis improved after use of NIV/MIE for 24hr (PaCO2: 71.7 +/- 18.6mmHg vs. 55.8 +/- 11.6mmHg, P<0.01; pH: 7.29 +/- 0.07 vs. 7.38 +/- 0.05, P<0.01). All patients tolerated NIV/MIE well despite transient skin pressure sores in five cases. ConclusionsCombined NIV/MIE is a safe and effective approach to rapidly improve physiologic indices and decrease the need for intubation in NMD children with ARF. NIV/MIE provides a good alternative for those refusing intubation. Pediatr Pulmonol. 2014; 49:589-596. (c) 2013 Wiley Periodicals, Inc.
Combined noninvasive ventilation and mechanical insufflator-exsufflator for acute respiratory failure in patients with neuromuscular disease: effectiveness and outcome predictors
(2019-09-01)
Chen, Tai-Heng
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Liang, Wen-Chen
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Chen, I-Chen
;
Liu, Yi-Ching
;
Hsu, Jong-Hau
Background: To determine the effectiveness of combined noninvasive ventilation (NIV) and mechanical insufflator-exsufflator (MI-E) for acute respiratory failure (ARF) in patients with neuromuscular disease (NMD), and outcome predictors. Methods: A prospectively observational study of patients with ARF was conducted in a pediatric intensive care unit (PICU). All received combined NIV/MI-E during PICU stays between 2007 and 2017. Pertinent clinical variables of heart rate (HR), respiratory rate (RR), pH, PaCO2, and PaO2/FiO(2) ratio were collected at baseline and at 2 h, 4-8 h, and 12-24 h after initiating use of NIV/MI-E. Treatment success was defined as avoiding intubation. Results: A total of 62 ARF episodes in 56 patients with NMD (median age, 13 years) were enrolled. The most frequent underlying NMD was spinal muscular atrophy (32/62, 52%). ARF was primarily due to pneumonia (65%). The treatment success rate was 86%. PICU stay and hospitalization were shorter in the success group (9.4 +/- 6.1 vs. 21.9 +/- 13.9 days and 16.3 +/- 7.8 vs. 33.6 +/- 17.9 days, respectively; both p < 0.05). HR, RR, pH, and PaCO2 showed a progressive improvement, particularly after 4 h following successful NIV/MI-E treatment. RR decrease at 4 h, and pH increase and PaCO2 decrease at 4-8 h might predict success of NIV/MI-E treatment. The multivariate analysis identified PaCO2 at 4-8 h of 58.0 mmHg as an outcome predictor of NIV/MI-E treatment. Conclusions: Applying combined NIV/MI-E in the acute care setting is an efficient means of averting intubation in NMD patients with ARF. Clinical features within 8 h of the institution may predict treatment outcome. The reviews of this paper are available via the supplemental material section.
Community-Acquired Pneumonia in Pediatric Patients With Acute Neuromuscular Respiratory Failure: A Microbiologic Perspective
(2014-08-01)
Hsu, Jong-Hau
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Chen, Tai-Heng
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Jong, Yuh-Jyh
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生物科技學系
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Department of Biological Science and Technology
Community-Acquired Pneumonia in Pediatric Patients With Acute Neuromuscular Respiratory Failure: A Microbiologic Perspective
(2014-08-01)
Hsu, Jong-Hau
;
Chen, Tai-Heng
;
Jong, Yuh-Jyh
;
生物科技學系
;
Department of Biological Science and Technology
Emergency room visits and admission rates of children with neuromuscular disorders: A 10-year experience in a medical center in Taiwan
(2019-08-01)
Kao, Wei-Tsun
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Tseng, Yung-Hao
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Jong, Yuh-Jyh
;
Chen, Tai-Heng
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交大名義發表
Background: No previous studies have explored emergency medical care for children with chronic neuromuscular disorders (NMDs). We aimed to determine the major reasons for the emergency room (ER) readmission of pediatric patients with NMDs and suggest changes to the care plan to decrease readmissions. Methods: Children with chronic NMDs (aged <18 years) who visited a medical center-based ER between January 2005 and January 2015 were included. The following data were extracted from the patient's ER records: presentations; demographic data, including sex and age; NMD diagnosis; triage classification; emergency examination; initial management and outcomes. The outcomes were death inside or outside the ER, admission to the ward or pediatric intensive care unit (PICU), uneventful discharge, and repeated ER visits. Results: In 10 years, 44 children with heterogeneous NMDs (boys/girls: 30/14, mean age: 9.9 years) visited the ER for a total of 204 times. Repeated ER visits and readmissions occurred in 56.8% and 55.6% of the patients, respectively. Most NMD children belonged to triage class 3 (35.3%), with underlying congenital hereditary muscular dystrophy (44.1%). The major symptoms were usually multiple and concurrent, and primarily respiratory (62.3%) or gastrointestinal (28.9%). The most common causes of hospitalization were pneumonia (48.5%) or acute gastritis (20.4%), and approximately half of the ER visits required further hospitalization, of which 28.2% involved PICU admission. Twenty of the 36 children admitted to the ER required readmission. The most commonly prescribed examinations were complete blood count (38%) and C-reactive protein (38%), and the most common therapy was intravenous fluid administration (34%). Although respiratory compromise caused most ER visits and admissions, pulmonary assessments, including chest films (28%), pulse oximetry (15%), and blood gas analysis (11%), were performed in a relatively small proportion. Conclusion: The ER staff must recognize patients' unmet needs for respiratory and gastrointestinal care related to underlying NMDs. Copyright (C) 2018, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC.
Identification of KLHL40 mutations by targeted next-generation sequencing facilitated a prenatal diagnosis in a family with three consecutive affected fetuses with fetal akinesia deformation sequence
(2016-12)
Chen, Tai-Heng
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Tian, Xia
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Kuo, Pao-Lin
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Pan, Hui-Ping
;
Wong, Lee-Jun C.
Background Fetal akinesia deformation sequence (FADS) refers to a broad spectrum of disorder with the absent fetal movement as the unifying feature. The etiology of FADS is heterogeneous, and the majority remains unknown. Prenatal diagnosis of FADS because of neuromuscular origin has relied on clinical features and fetal muscle pathology, which can be unrevealing. The recent advance of next-generation sequencing (NGS) can provide definitive molecular diagnosis effectively. Methods and Results An 18-week-old fetus presented with akinesia and multiple contractures of joints. The mother had two previously aborted similarly affected fetuses. Clinical diagnosis of FADS was made. Molecular diagnosis using cord blood by NGS of genes related to neuromuscular diseases revealed two compound heterozygous mutations; c. 602G > A(p. W201*) and c. 1516A > C(p. T506P), in the Kelch-like 40 (KLHL40) gene. Based on this information, prenatal diagnosis was performed on the CVS of the subsequent pregnancy that resulted in an unaffected female baby, heterozygous for the c. 1516A > C(p. T506P) mutation. Conclusion Identification of KLHL40 mutations in one of the aborted fetuses provided a confirmative diagnosis of FADS, facilitating the prenatal diagnosis of the subsequent pregnancy. This report underscores the importance of target NGS in providing FADS families with an affordable, precise molecular diagnosis for genetic counseling and options of prenatal diagnosis. (C) 2016 John Wiley & Sons, Ltd.
Identification of KLHL40 mutations by targeted next-generation sequencing facilitated a prenatal diagnosis in a family with three consecutive affected fetuses with fetal akinesia deformation sequence
(2016-12)
Chen, Tai-Heng
;
Tian, Xia
;
Kuo, Pao-Lin
;
Pan, Hui-Ping
;
Wong, Lee-Jun C.
Background Fetal akinesia deformation sequence (FADS) refers to a broad spectrum of disorder with the absent fetal movement as the unifying feature. The etiology of FADS is heterogeneous, and the majority remains unknown. Prenatal diagnosis of FADS because of neuromuscular origin has relied on clinical features and fetal muscle pathology, which can be unrevealing. The recent advance of next-generation sequencing (NGS) can provide definitive molecular diagnosis effectively. Methods and Results An 18-week-old fetus presented with akinesia and multiple contractures of joints. The mother had two previously aborted similarly affected fetuses. Clinical diagnosis of FADS was made. Molecular diagnosis using cord blood by NGS of genes related to neuromuscular diseases revealed two compound heterozygous mutations; c. 602G > A(p. W201*) and c. 1516A > C(p. T506P), in the Kelch-like 40 (KLHL40) gene. Based on this information, prenatal diagnosis was performed on the CVS of the subsequent pregnancy that resulted in an unaffected female baby, heterozygous for the c. 1516A > C(p. T506P) mutation. Conclusion Identification of KLHL40 mutations in one of the aborted fetuses provided a confirmative diagnosis of FADS, facilitating the prenatal diagnosis of the subsequent pregnancy. This report underscores the importance of target NGS in providing FADS families with an affordable, precise molecular diagnosis for genetic counseling and options of prenatal diagnosis. (C) 2016 John Wiley & Sons, Ltd.
Methodological considerations in combined noninvasive ventilation and mechanical inexsufflator
(2014-10-01)
Hsu, Jong-Hau
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Chen, Tai-Heng
;
Jong, Yuh-Jyh
;
交大名義發表
;
生物科技學系
Noninvasive airway approaches for acute neuromuscular respiratory failure in emergency departments
(2017-10-01)
Chen, Tai-Heng
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Hsu, Jong-Hau
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Jong, Yuh-Jyh
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生物資訊及系統生物研究所
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Institude of Bioinformatics and Systems Biology
Emerging evidence advocates for noninvasive ventilation (NIV) combined with mechanical in-exsufflation (MIE) as a first-line approach for acute respiratory failure (ARF) in patients with neuromuscular disorders (NMD). To date, most NIV studies of ARF in NMD patients have been performed in intensive care units or in hospital settings. However, the utility of using combined NIV/MIE in the emergency department (ED) settings is unclear. We report on the implementation of NIV/MIE in two children with type II spinal muscular atrophy who presented to the ED with ARF. This is the first report on the feasibility and efficacy of combining NIV/MIE in ED settings for pediatric NMD patients with ARF.
Reliability and Validity of Outcome Measures of In-Hospital and At-Home Visits in a Randomized, Double-Blind, Placebo-Controlled Trial for Spinal Muscular Atrophy
(2014-12-01)
Chen, Tai-Heng
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Yang, Yi-Hsin
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Mai, Hui-Hsia
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Liang, Wen-Chen
;
Wu, Yi-Ching
We used at-home assessments in a clinical trial to relieve the visit burden for participants. A total of 57 patients with type II or III spinal muscular atrophy were enrolled and 10 of them (7 type II and 3 type III) received at-home assessments. The primary end points were Gross Motor Function Measure, Manual Muscle Test, and serum biomarker. The secondary endpoints were Modified Hammersmith Functional Motor Scale and forced vital capacity. The correlation coefficients and analysis of covariance showed good reliability and validity of all outcome measures. Except for Gross Motor Function Measure and Modified Hammersmith Functional Motor Scale, there were no significant differences in measures between in-hospital and at-home groups (intersubject) or among 3 patients who received both at-home and in-hospital visits (intrasubject). We concluded that at-home assessments could provide sufficient reliability in a controlled trial. This modification could help design a successful clinical trial for spinal muscular atrophy.
Selective Neuromuscular Denervation in Taiwanese Severe SMA Mouse Can Be Reversed by Morpholino Antisense Oligonucleotides
(2016-04-28)
Lin, Te-Lin
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Chen, Tai-Heng
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Hsu, Ya-Yun
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Cheng, Yu-Hua
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Juang, Bi-Tzen
Spinal muscular atrophy (SMA) is an autosomal recessive motor neuron disease caused by deficiency of the survival of motor neuron (SMN) protein, which leads to synaptic defects and spinal motor neuron death. Neuromuscular junction (NMJ) abnormalities have been found to be involved in SMA pathogenesis in the SMN Delta 7 SMA mouse model. However, whether similar NMJ pathological findings present in another commonly used mouse model, the Taiwanese SMA mouse, has not been fully investigated. To examine the NMJs of the Taiwanese severe SMA mouse model (Smn(-/-); SMN2(tg/0)), which is characterized by severe phenotype and death before postnatal day (P) 9, we investigated 25 axial and appendicular muscles from P1 to P9. We labelled the muscles with anti-neurofilament and anti-synaptophysin antibodies for nerve terminals and a-bungarotoxin for acetylcholine receptors (AChRs). We found that severe NMJ denervation (<50% fully innervated endplates) selectively occurred in the flexor digitorum brevis 2 and 3 (FDB-2/3) muscles from P5, and an increased percentage of fully denervated endplates correlated with SMA progression. Furthermore, synaptophysin signals were absent at the endplate compared to control littermate mice, suggesting that vesicle transport might only be affected at the end stage. Subsequently, we treated the Taiwanese severe SMA mice with morpholino (MO) antisense oligonucleotides (80 mu g/g) via subcutaneous injection at P0. We found that MO significantly reversed the NMJ denervation in FDB-2/3 muscles and extended the survival of Taiwanese severe SMA mice. We conclude that early NMJ denervation in the FDB-2/3 muscles of Taiwanese severe SMA mice can be reversed by MO treatment. The FDB-2/3 muscles of Taiwanese severe SMA mice provide a very sensitive platform for assessing the effectiveness of drug treatments in SMA preclinical studies.