1.Advances in clinical and molecular genetic research on pyridoxine dependent epilepsy.
Chinese Journal of Pediatrics 2013;51(11):867-870
2-Aminoadipic Acid
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analogs & derivatives
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analysis
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Aldehyde Dehydrogenase
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genetics
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Anticonvulsants
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therapeutic use
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Biomarkers
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analysis
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Brain
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pathology
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DNA Mutational Analysis
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Electroencephalography
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Epilepsy
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diagnosis
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drug therapy
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genetics
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Genetic Association Studies
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Humans
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Infant
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Infant, Newborn
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Magnetic Resonance Imaging
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Mutation, Missense
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Prognosis
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Pyridoxine
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therapeutic use
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Seizures
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diagnosis
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drug therapy
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genetics
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Status Epilepticus
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diagnosis
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drug therapy
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genetics
2.Case Report of Reflex Epilepsy Induced by Micturition
zhi-xian, YANG ; jiong, QIN ; xiao-yan, LIU ; ying, HAN
Journal of Applied Clinical Pediatrics 1986;0(02):-
To report a boy with reflex seizures induced by micturition.He suffered from seizures during micturition for four yesrs.The clinical symptoms consisted of head backwards,limbs dithering,sometimes accompanying tumble.During the episodes of the seizure,his consciousness did not lose.Few seizures showed only eyes up-staring for several seconds.The livelong process of micturition was often interrupted by seizures for several times.When he finished or stoped micturition,the seizures did not occur again.His seizures could not be induced by free of micturition or other stimulative factors.The interictal EEGs showed that background asymmetry manifested as the lower amplitude and irregular rhythm in the left hemisphere,sharp or sharp and waves in right hemisphere,especially mid and back temporal.There also had sharp,spike,and spike and waves in many regions of double spheres in interictal EEGs.The other assistant check-ups were normal.while this disease was infrenquncy,it would be worthy to regard.
3.Effect of Naloxone of Different Dosage on c-fos Expression in Hippocampus Induced by Repeated Febrile Seizures
ying, SHAN ; jiong, QIN ; zhi-xian, YANG ; ying, HAN
Journal of Applied Clinical Pediatrics 1986;0(01):-
Objective To investigate the effect of naloxone on c-fos expression in hippocampus induced by repeated febrile seizures(FS).Methods Warm water induced rat FS model was developed in this study. Each rat was induced 7 febrile seizures with the interval of one day. Naloxone-treated rats and FS control rats received injection of naloxone(1 mg/kg,2 mg/kg) or saline once FS occurrence every 2 day respectively. All rats were sacrificed 24 hours after the last seizure. In hippocampus, c-fos expression distribution and semi-quantitative analysis was determined by immuhischemical staining measure and western-blotting respectively.Results Compared with FS control group, naloxone treatment could significantly relieve c-fos expression in hippocampus induced by repeated FS, mainly in dentate gyrus(DG) and CA3 region. The comparison between 1 mg/kg and 2 mg/kg naloxone-treated group showed that 2 mg/kg naloxone could reduce c-fos positive expression more significantly.Conclusion Naloxone of proper dosage may significantly alleviate c-fos expression after repeated FS ,which further proved its antiepileptic function and also implied that endogenous opioid may be involved in the regulation of c-fos expression during seizure.
4.Nitric Oxide Regulated Expression of ?-Aminobutyric Acid B Recepto r Subunits during Recurrent Febrile Seizures
ying, HAN ; jiong, QIN ; ding-fang, BU ; zhi-xian, YANG ; xing-zhi, CHANG ; jun-bao, DU
Journal of Applied Clinical Pediatrics 2004;0(09):-
Objective To explore the effect of nitric oxide (NO) on ?-aminobutyric acid B receptor (GABA_BR) subunits during recurrent febrile seizures (FS).Methods Twenty-four Sprague-Dawley rats aged 21 days were randomly divided into 4 groups: control group (37.0 ℃ water,n=8), FS group (45.2 ℃ water,n=8), FS + SNP group (45.2 ℃ water,n=8), FS+L-NMMA group (45.2 ℃ water,n=8). FS rats were induced 10 times in a warm-water bath, once every 2 days. The plasma level of NO was detected by the spectrophotometer. The expressions of GABA_BR subunit mRNA and c-fos gene were examined by in situ hybridization. The expressions of GABA_BR subunit and Fos protein were observed by immunohistochemistry. Results The plasma level of NO increased in FS + SNP group while decreased in FS+L-NMMA group compared with that in FS group. The expressions of GABA_BR_2 were down-regulated in FS+SNP group, while GABA_BR_1 hardly changed compared with those in FS group. In FS+L-NMMA group, both the expression of GABA_BR_2 and GABA_BR_1 up regulated compared with those in FS group. The expressions of c-fos gene and Fos protein were significantly enhanced after recurrent FS. SNP elevated the expressions of c-fos gene and Fos protein, while L-NMMA down regulated the expressions of them.Conclusion NO may play a regulatory role through modulating GABA_BR function in the pathogenesis of recurrent FS.
5.Influence of Gamma-Aminobutyric Acid B Receptor on Expression of Carbon Monoxide/ Heme Oxygenase System during Recurrent Febrile Seizures
ying, HAN ; jiong, QIN ; ding-fang, BU ; zhi-xian, YANG ; xing-zhi, CHANG ; jun-bao, DU
Journal of Applied Clinical Pediatrics 2004;0(12):-
Objective To explore the influence of ?-aminobutyric acid B receptor(GABA_BR)on carbon monoxide (CO)/heme oxygenase(HO-1)system during recurrent febrile seizures (FS).Methods Sprague-Dawley rats aged 21 days were randomly divi- ded into 4 groups:control group and FS group,FS+baclofen group,FS+phaclofen group.FS in rats were induced 10 times in a bath of warm water, once every 2 days.The plasma level of CO was detected by the dual wave lengh spectrophotometer;the expressions of GABA_BR and HO-1 mRNA were examined by insitu hybridization;the expressions of GABA_BR and HO-1 protein were observed by immunohistochemistry.Results The plasma level of CO increased in FS+baclofen group,while decreased in FS+phaclofen group compared with FS group.The expressions of GABA_BR and HO-1 upregulated in FS+baclofen group,while decreased in FS+phaclofen group compared with FS group.There were significant difference (All P
6.Influence of endogenous nitric oxide synthase /nitric oxide system on brain damage induced by recurrent febrile seizures
zhi-xian, YANG ; jiong, QIN ; dingfang, BU ; junbao, DU ; zhi xing, CHANG ; ying, HAN
Journal of Applied Clinical Pediatrics 2004;0(12):-
Objective To explore the changing regularity of nitric oxide synthase(nNOS) in recurrent febrile seizures (FS), and the influence of NOS/NO on brain damage induced by recurrent FS.Methods FS rats were induced in a bath of warm water.The ex-periments were divided into 2 groups. The contents of nNOS cDNA in the first group was measured by quantitative RT-PCR and the contents of nNOS protein was measured by Western blot.The mtensity , latency, duration and rectal temperature of the seizure in rats in the second group were recorded. Morphologic changes of hippocampal neurons were observed with HE stain.Results Alter recur-rent FS, the expression of nNOS mRNA in hippocampus was significantly inereased compared with those in control group and hyper-thermia group, associated with an increase of nNOS protein.With the increase of seizure number,thert were changes of seizure latency and gradually prolonged trend of the seizure duration. By using the inhibitor of NOS, the seizure latency was gradually prolonged and the prolonged trend of the seizure duration was significantly decreased than that in FS group.There was no significantly difference of seizure intensity and rectal temperature between 2 groups.After recurrent FS, histological changes of hippocampal neurons could be seen under light microscope.The inhibitor alleviated nearonal injury.Conclusions Recurrent FS can induce nNOS gent expression.The NOS/NO system may be involved in the development of brain damage induced recurrent FS.
7.Effect of Gamma Aminobutyric Acid B Receptor on Brain Damage Induced by Recurrent Febrile Seizures
ying, HAN ; jiong, QIN ; ding-fang, BU ; ding-fang, CHANG ; zhi-xian, YANG
Journal of Applied Clinical Pediatrics 2004;0(11):-
Objective To explore the effect of ? - aminobutyric acid B receptor(GABABR)on brain damage induced by recurrent febrile seizures (FS). Methods Rats were randomly divided into four groups: control group (37. 0 ℃ water, n = 8), FS group (45.2 ℃ water,n=8), FS + baclofen group (45.2 ℃ water,77 = 8), FS + phaclofen group (45. 2 ℃ water,n=8). FS in rats were induced for ten times in a bath of warm water, once every 2 days. The intensity, latency and duration of the seizure in rats were recorded. The expression of c - fos gene and Fos protein were examined by in situ hybridization and immunohistochemistry, respectively. Results Compared with those of FS group, the seizure latency gradually prolonged, and the seizure duration was shortened in FS + baclofen group. In FS+ phaclofen group, the seizure latency was shorter and the seizure duration was longer than those of FS group. The seizure intensity was lessened in FS + baclofen group while aggravated in FS + phaclofen group compared with that of FS group. The expression of c - fos gene and Fos protein increased significantly after recurrent FS. Baclofen down regulated the expression of c -fos gene and Fos protein, while phaclofen enhanced the expression of them. Conclusion The study by using the agonist and the inhibitor of GABABR showed that GABABR might play a crucial role in the development of FS- induced brain damage.
8.Endoplasmic Reticulum Stress Activated by Recurrent Febrile Seizures in Developing Rats
xiao-yan, LIU ; jiong, QIN ; zhi-xian, YANG ; ying, HAN ; jing, CHEN
Journal of Applied Clinical Pediatrics 1992;0(05):-
Objective To observe the recurrent febrile seizures(FS)influence on adrenocorticotropic hormone(ATCH)and glucose-regulated proein 78(Grp78)and explore the association between recurrent FS and endoplasmic reticulum stress in the brain of developing rats.Methods Forty specefic pathogen free(SPF)Sprague-Dawley rats aged 21 days were randomly divided into control group(immersed in 37.0 ℃ water,n=12)and hyperthermia treated group(immersed in 44.8 ℃ water,n=28),which was subdivided into hyperthermia group(n=12)and recurrent FS group(n=16)according to whether seizures occurred or not.Seizures were induced 10 times in a bath of warm water,once every 2 days.The plasma content of ACTH was detected by radioimmunoassay.The protein level of Grp78 was examined by immunohistochemistry staining and Western blot.Results After recurrent FS,the plasma content of ACTH increased significantly in FS group compared with control group(Pa
9.Differential Diagnosis of Radionuclide Hepatobiliary Scintigraphy with Phenobarbitol Sodium on Infants with Persistent Jaundice
xian-cun, HOU ; hua, CHENG ; zhi-yong, LI ; shao-yang, REN ; hui, ZHU
Journal of Applied Clinical Pediatrics 2006;0(19):-
Objective To evaluate the value of differential diagnosis on congenital biliary atresia(BA) and infantile hepatitis syndrome(IHS) by technetium-99m-diethyl-iminodiacetic acid(99Tcm-EHIDA)hepatobiliary scintigraphy with phenobarbitol sodium.Methods Fifty-eight infants with persistent jaundice were taken phenobarbitol sodium[5 mg/(kg?d)] ,bid ?7 d).Those who had not bowel and gallbladders radioactivity within 24 hours were diagnosed as the diagnostic criterion of BA.Those with bowel and gallbladders radioactivity within 24 hours were diagnosed as the diagnostic criterion of IHS,who then received 99Tcm-EHIDA hepatobiliary scintigraphy with single photon emission computed tomography(SPECT) instrument.The results of all children were analyzed and compared with pathology and clinical follow up results.Results 99Tcm-EHIDA hepatobiliary scintigraphy correctly diagnosed 24 infants with last diagnosis BA and 29 infants with last diagnosis IHS,5 neonates false positive in all 34 IHS patients.The sensitivity in the diagnosis of BA was 100%,the specificity and accuracy were 85.3% and 91.4%,restectively.The sensitivity was 85.3% in the diagnosis of IHS;the specificity and accuracy were 100% and 91.4%,respectively.Conclusions 99Tcm-EHIDA hepatobiliary scintigraphy with phenobarbitol sodium can accurately differentiate BA and HIS at early stage.
10.The effect of combined application of low dose fentanyl and midazolam on sodium channels in rat cerebral cortical neurons.
Yun-Chun YANG ; Xian ZHOU ; Jia-Li WU ; Xuan JIANG ; Shu-Zhi ZHOU ; Xiao-Bin WANG
Chinese Journal of Applied Physiology 2011;27(1):85-87
Anesthetics, Intravenous
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administration & dosage
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pharmacology
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Animals
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Animals, Newborn
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Cerebral Cortex
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cytology
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metabolism
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Drug Synergism
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Female
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Fentanyl
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administration & dosage
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pharmacology
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Male
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Midazolam
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administration & dosage
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pharmacology
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Neurons
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metabolism
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Patch-Clamp Techniques
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Primary Cell Culture
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Rats
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Rats, Sprague-Dawley
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Voltage-Gated Sodium Channels
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drug effects