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-JONG-HAU HSU, MD, TAI-HENG CHEN, MD, YUH-JYH JONG, MD, DMSci ...doi:10.1002/ppul.22964 pmid:24376043 fatcat:6mo5zrerfbftnpp75j7ozg53p4
Most of the patients in this study with spinal muscular atrophy were found to have tremors of the isoelectric line in the electrocardiogram (ECG) tracings. There were a total of 47 cases of SMA (mean age 40.8 months). All three types of SMA had a similar incidence (about 80%) of tremors in the tracings (p=0.885). In 7 cases the ECG tremors had an intermittent pattern. ECG tremors were commonly found in the majority of SMA patients and this finding, though non-specific, may suggest a possible SMA diagnosis. (Jpn Heart J 1996; 37: 239-242)doi:10.1536/ihj.37.239 fatcat:5rtihy77qvexbbgymbr6l7ei2m
Drug Discovery Research in Pharmacognosy
How to reference In order to correctly reference this scholarly work, feel free to copy and paste the following: Mei Fen Shih and Jong Yuh Cherng (2012) . ...doi:10.5772/34644 fatcat:gnzflpqnbngbla6ccgcw324rb4
We are grateful to read the comments sent to the journal by Dr. Massimiliano Don regarding our article "Combined noninvasive ventilation and mechanical in-exsufflator in the treatment of pediatric acute neuromuscular respiratory failure". 1 These valuable comments bring our attention to the microbiologic aspect of pneumonia in pediatric patients with neuromuscular diseases (NMD), an important issue that has not been well addressed. To date, early or prophylactic use of antibiotics as clinicaldoi:10.1002/ppul.22959 pmid:24719398 fatcat:ts6et2m5zrej7jt65o6clehqlm
more »... nagement plans for community-acquired pneumonia (CAP) in NMD children are suggested. 2-4 However, the literature is sparse regarding the etiology of CAP in these children and beneficial evidence of empiric antibiotics in these populations is still lacking. Thus, we are in line with Dr. Massimiliano Don that investigations elucidating the etiology of CAP may improve outcome of these patients and avoid adverse effects of unnecessary antibiotics. To this end, we re-analyzed our data in our article, 1 and tried to explore possible microbial factors associated with the outcome of these patients receiving noninvasive ventilation (NIV) and mechanical in-exsufflator (MIE) due to pneumonia. To compare the differences between success group (n ¼ 12) and failure group (n ¼ 4) in our patients, we analyzed infection-related variables including presence of fever at admission to our pediatric intensive care unit, pathogen survey by sputum culture, serum mycoplasma antibody or nasopharyngeal antigen detection of respiratory syncytial virus (RSV), and inflammatory markers including white blood cell count and serum C-reactive protein (CRP). However, we found that there were no significant differences of these variables. The presence of fever at admission was comparable, 42% (5/12) versus 50% (2/4) between success group and failure group. In the success group, there are only four events with identified pathogens, including two with RSV and two with mycoplasma pneumonia, while in the failure group there is one with mycoplasma pneumonia infection and one with streptococcus infection. Comparing the success group with the failure group, the initial WBC counts were 9,263 AE 2,618 ml À1 versus 10,225 AE 2,646 ml À1 (P ¼ 0.54) and the initial CRP levels were 17.6 AE 14.6 mg/L versus 21.7 AE 22.8 mg/L (P ¼ 0.68, normal range ¼ 0-6 mg/L). Take together, we did not find any prognostic factor associated with outcomes of NIV/MIE use in our study. In addition, we speculate that in those events without identifiable pathogen (n ¼ 10, 67%), virus infection may be the most probable etiology given with the low average WBC counts and CRP levels. Of note, the small sample sizes in our study lacked the statistical power to rigorously conclude the validity of these lacks of difference. Future studies, properly powered, will determine with more certainty whether these preliminary observations are correct. Despite advances of bacteriological identification procedures in the pathogen survey of pneumonia, the frequency of microbiologically documented CAP is only around 25% among in-patients. 5 Furthermore, in those patients with pneumonia not receiving endotracheal intubation, the diagnostic reliability of deep cough-produced sputum and nasopharyngeal aspirates remains uncertain because of the problem of contamination with upper airway flora. 6 Accordingly, in children with severe CAP without intubation, their inability to produce effective cough often preclude the reliability of pathogen investigations in pneumonia.
Valproate-induced hyperammonemic encephalopathy is an unusual but serious complication that can occur in people with normal liver-associated enzyme levels, and despite normal therapeutic doses and serum levels of valproate. Here, we describe an adolescent girl suffering from absence seizures, who complained of progressive dizziness and general malaise several days after restarting valproate. She developed vomiting and decreased consciousness after 3 weeks of valproate use. She had a serumdoi:10.1016/s1875-9572(09)60010-3 pmid:19133574 fatcat:uhscprqjlvfjxn35x5doro6usu
more »... a level five times higher than the upper normal limit, normal liverassociated enzymes, and a supra-therapeutic valproate level. Electroencephalography (EEG) showed continuous generalized slowing. Tandem mass spectrometry analysis revealed carnitine deficiency. Her consciousness improved after emergent hemodialysis. Her ammonia level and EEG also became normal. Possible mechanisms, risk factors and treatments of valproate-induced hyperammonemic encephalopathy are described. Physicians should consider this possibility when consciousness disturbance occurs in patients treated with valproate.
Yuh-Jyh Jong diagnosed with his serum DNA by using target gene capture/deep sequencing approach  . ...doi:10.1186/s12929-020-00707-1 pmid:33435938 fatcat:tcrnsxvjeje2xddscwqlsjohd4
doi:10.1016/j.pedneo.2013.03.020 pmid:23755946 fatcat:taqpzl4qpnczvo5sj2n7chuuhe
In this paper, we are going to show how to build up agentoriented Public Key Infrastructure(PKI) from SPKI/SDSI and X.509 standards. A variety of delegation mechanisms for agents will be demonstrated under this agent-oriented PKI. The mechanisms include: chain-ruled, threshold, and conditional. The lack of agent security management standards did not allow us to do the agent trust and delegation in legalized manner so we proposed several new communicative acts to satisfy our agent delegationdoi:10.1145/375735.376424 dblp:conf/agents/Hu01 fatcat:isr32jimpbgghp3wy7mpc66spu
more »... gement. Finally, we briefly show how to implement these agent communication language and inner content language in XML and XML/RDF.
and where extracellular stimuli can interact with GPCR ligand binding domains. Recently, however, numerous GPCRs have also been found to be associated with various intracellular membranes where, in certain cases, they activate intracellular signaling machinery leading to unique functional responses            . One such receptor is the metabotropic glutamate receptor, mGluR5, which is highly expressed on intracellular membranes including the ER and nucleardoi:10.1007/s11064-016-2026-6 pmid:27514643 pmcid:PMC5283513 fatcat:jkhkzfneebhgrisrjwq3uegnre
more »... ranes throughout the CNS [12, 13] . Endogenous nuclear mGluR5 couples to G q and PLC to generate IP 3 -mediated Ca 2+ release within the nucleus and activation of intracellular mGluR5 generates unique Ca 2+ responses as well as downstream signaling cascades distinct from cell surface counterparts, [14, 15] . These observations and those by others challenge the notion that cells only interact with their environment at the plasma membrane to bring about long term changes. The question arises then as to what ligand activates intracellular mGluR5 and mechanistically how activation is achieved. The most parsimonious answer is that as the natural ligand, glutamate itself may activate intracellular mGluR5. Glutamate uptake is mediated by at least five sodium-dependent transporter proteins that are present on glial and neuronal cells as well as the chloride-dependent cystine-glutamate exchanger       . Previous data show that both of these uptake systems are responsible for transporting glutamate into striatal, hippocampal and/or spinal cord dorsal horn neurons to activate mGluR5 [13,    . Conditions that block the transporters (i.e., chloride-free buffers and the compound l-cystine for the cystine/glutamate exchanger; sodium free buffers and the compound, threo-β-benzyloxyaspartate for sodium-dependent excitatory amino acid transporters) reduce agonist uptake in mGluR5-expressing neurons [13,    . Moreover, uptake of radiolabeled quisqualate, an mGluR5 agonist, and Abstract The group 1 metabotropic glutamate receptor, mGluR5, is found on the cell surface as well as on intracellular membranes where it can mediate both overlapping and unique signaling effects. Previously we have shown that glutamate activates intracellular mGluR5 by entry through sodiumdependent transporters and/or cystine glutamate exchangers. Calibrated antibody labelling suggests that the glutamate concentration within neurons is quite high (~10 mM) raising the question as to whether intracellular mGluR5 is maximally activated at all times or whether a different ligand might be responsible for receptor activation. To address this issue, we used cellular, optical and molecular techniques to show that intracellular glutamate is largely sequestered in mitochondria; that the glutamate concentration necessary to activate intracellular mGluR5 is about ten-fold higher than what is necessary to activate cell surface mGluR5; and uncaging caged glutamate within neurons can directly activate the receptor. Thus these studies further the concept that glutamate itself serves as the ligand for intracellular mGluR5.
Jong, Wang-Tso Lee, Reyin Lien, Chyi-Her Lin, Kuang-Lin Lin, Bai-Horng Su, Yi-Fang Tu, Wen-Chin Weng, and San-Nan Yang. ... Acknowledgments The authors thank all members of the Joint Task Force of TH on HIE, including Chao-Huei Chen, Ming-Chou Chiang, Wu-Shiun Hsieh, Chyong-Hsin Hsu, Chao-Ching Huang, Kun-Long Hung, Yuh-Jyh ...doi:10.1016/j.pedneo.2016.11.001 pmid:28416250 fatcat:jkoixs5bsjb5nitfsen6vonp6u
Lecture Notes in Computer Science
Agent is autonomous software that mediates e-service for human on the Internet. The acceptance of agent-mediated e-service (AMES) is very slow for the lacking of security management infrastructure for multi-agent system. Therefore we proposed an agent-oriented public key infrastructure (APKI) for multi-agent e-service. In this APKI, a taxonomy of digital certificates are generated, stored, verified, and revoked to satisfy different access and delegation control purposes. Agent identitydoi:10.1007/978-3-540-45224-9_164 fatcat:iat6xl3jafgczoowg4gdg5ub3y
more »... te was designed for agent's authentication whereas attributed and agent authorization certificates were proposed for agent's authorization and delegation. Using these digital certificates, we establish agent trust relationships on the cyberspace. A trusted agent-mediated e-service scenario will be shown to demonstrate the feasibility of our APKI.
Objective: Cardiovascular risk increases with the presence of both metabolic syndrome (MetS) and hypertension (HTN). Although the adiponectin (ADIPOQ) gene has been reported to be involved in MetS, its association with HTN remained undetermined. This study aimed to investigate the association of ADIPOQ gene with the phenotypes of HTN and MetS. Methods: A total of 962 participants from 302 families from the Taiwan young-onset hypertension genetic study were enrolled. Plasma adiponectin weredoi:10.1371/journal.pone.0019999 pmid:21637762 pmcid:PMC3103519 fatcat:mfjexkeihna6hn5lpqjb4oawle
more »... red, and association analysis was conducted by using GEE regression-based method. Another study, of 1448 unrelated participants, was conducted to replicate the association between ADIPOQ gene and variable phenotypes of MetS with or without HTN. Results: Among 962 subjects from family samples, the lowest plasma adiponectin value was observed in MetS with HTN component (9.360.47 mg/ml) compared with hypertensives (13.460.74 mg /ml) or MetS without HTN (11.960.60 mg/ml, P,0.05). The SNP rs1501299 (G276T) in ADIPOQ gene was found associated with the presence of HTN in MetS (odds ratio for GG+GT vs. TT = 2.46; 95% CI: 1.14-5.3, p = 0.02), but not rs2241766 (T45G). No association of ADIPOQ gene with HTN alone or MetS without HTN was observed. The significant association of the SNP rs1501299 (G276T) with the phenotype of presence of HTN in MetS was confirmed (odds ratio for GG+GT vs. TT = 2.15; 95% CI: 1.1-4.3) in the replication study. Conclusions: ADIPOQ genetic variants were selectively and specifically associated with the concomitant presence of MetS and HTN, suggesting potential genetic linkage between MetS and HTN.
When the distribution of lifetimes is 2-parameter exponential, Balasooriya  provided a failure-censored reliability sampling-plan to save test time. This paper extends the Balasooriya sampling plan to the Weibull distribution and provides a limited failure-censored reliability sampling plan (LFCR) to do life testing when test facilities are scarce. The -expected test time of the LFCR is computed, and the optimal stopping rule of LFCR corresponding to the shortest test time is established.doi:10.1109/24.935024 fatcat:4zc4chnbrjavjcupkjsbqlukxu
more »... -confidence intervals for the parameters are generated. Index Terms-Best linear unbiased estimator, life-test sampling plan, Monte Carlo simulation, order statistic, Weibull distribution.
Fabry disease (FD) is an X-linked inherited lysosomal storage disease caused by a-galactosidase A (GLA) deficiency. Progressive intracellular accumulation of globotriaosylceramide (Gb3) is considered to be pathogenically responsible for the phenotype variability of FD that causes cardiovascular dysfunction; however, molecular mechanisms underlying the impairment of FD-associated cardiovascular tissues remain unclear. In this study, we reprogrammed human induced pluripotent stem cells (hiPSCs)doi:10.3727/096368916x694265 pmid:27938475 pmcid:PMC5657705 fatcat:ivwhuw33qbb2zhiuwresttxfda
more »... om peripheral blood cells of patients with FD (FD-iPSCs); subsequently differentiated them into vascular endothelial-like cells (FD-ECs) expressing CD31, VE-cadherin, and vWF; and investigated their ability to form vascular tube-like structures. FD-ECs recapitulated the FD pathophysiological phenotype exhibiting intracellular Gb3 accumulation under a transmission electron microscope. Moreover, compared with healthy control iPSC-derived endothelial cells (NC-ECs), reactive oxygen species (ROS) production considerably increased in FD-ECs. Microarray analysis was performed to explore the possible mechanism underlying Gb3 accumulation-induced ROS production in FD-ECs. Our results revealed that superoxide dismutase 2 (SOD2), a mitochondrial antioxidant, was significantly downregulated in FD-ECs. Compared with NC-ECs, AMPK activity was significantly enhanced in FD-ECs. Furthermore, to investigate the role of Gb3 in these effects, human umbilical vein endothelial cells (HUVECs) were treated with Gb3. After Gb3 treatment, we observed that SOD2 expression was suppressed and AMPK activity was enhanced in a dose-dependent manner. Collectively, our results indicate that excess accumulation of Gb3 suppressed SOD2 expression, increased ROS production, enhanced AMPK activation, and finally caused vascular endothelial dysfunction. Our findings suggest that dysregulated mitochondrial ROS may be a potential target for treating FD.
Angiotensin-converting enzyme (ACE) has been implicated in multiple biological system, particularly cardiovascular diseases. However, findings associating ACE insertion/deletion polymorphism with hypertension or other related traits are inconsistent. Therefore, in a two-stage approach, we aimed to fine-map ACE in order to narrow-down the function-specific locations. We genotyped 31 single nucleotide polymorphisms (SNPs) of ACE from 1168 individuals from 305 young-onset (age #40) hypertensiondoi:10.1371/journal.pone.0056119 pmid:23469169 pmcid:PMC3587614 fatcat:oaj6wtzccjav3gb42oaotrunra
more »... igrees, and found four linkage disequilibrium (LD) blocks. A tag-SNP, rs1800764 on LD block 2, upstream of and near the ACE promoter, was significantly associated with young-onset hypertension (p = 0.04). Tag-SNPs on all LD blocks were significantly associated with ACE activity (p-value: 10 -16 to ,10 -33 ). The two regions most associated with ACE activity were found between exon13 and intron18 and between intron 20 and 39UTR, as revealed by measured haplotype analysis. These two major QTLs of ACE activity and the moderate effect variant upstream of ACE promoter for young-onset hypertension were replicated by another independent association study with 842 subjects.
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