1.Protective effect of dl-tetrahydropalmatine on cardiac arrhythmias induced by reperfusion of ischemic myocardium
Yongxiao WANG ; Yunmin ZHENG ; Yuehua TAN
Chinese Pharmacological Bulletin 1986;0(05):-
Dl-tetrahydropalmatine (THP) 5 ~ 15 mg ? kg-1 iv markedly reduced the incidences of cardiac arrhythmias and myocardial lipidperoxide content after reperfusion of the is-chemic myocardium in rats. In isolated rat ventricular muscles, THP 1,3 and 10 ?mol?L-1 shifted the concentration-effect curves for phenylephrine to the right unparally, together with decreasing their maximal responses,exhibiting a non-competed antagonistic action. It was also found that THP 10 ?mol?L-1was able to reduce the amplitude of delayed afterdepolar-izations and to stop triggered activity and/or ab-normal automatic activity induced by reoxygena-tion of hypoxic myocardium in isolated ventricular muscles of guinea pig. These results suggest that the protective effects of THP might be attributed, at least in part, to the depression of delayed afterdepolarizations and the prevention of oxygen free radical injury without the involvement of the cardiac ?1-adrenoceptors.
2.Genotype and phynotype of DNM1 gene variants related developmental epileptic encephalopathy
Xueyang NIU ; Jiaoyang CHEN ; Miaomiao CHENG ; Ying YANG ; Yi CHEN ; Wenwei LIU ; Quanzhen TAN ; Xiao-Ling YANG ; Yuehua ZHANG
Chinese Journal of Applied Clinical Pediatrics 2022;37(9):692-696
Objective:To summarize the clinical features of developmental epileptic encephalopathy children with DNM1 gene variants. Methods:The genotypes and clinical features of 15 children with DNM1 variants related epilepsy in the Department of Pediatrics, Peking University First Hospital from June 2017 to October 2021 were retrospectively analyzed. Results:A total of 8 male and 7 female epilepsy patients with DNM1 gene variants with the age of seizure onset ranging from 15 days to 22 months were recruited, median age was 8 months.All cases belonged to de novo heterozygous variants of the DNM1 gene, including 13 cases of missense variants, 1 case of frame shift variant and 1 case of nonsense variant, 8 cases of ectopic sites have not been reported.Multiple seizure types were observed, including epileptic spasms in 15 patients, focal seizure in 9 patients, atypical absence seizure in 2 patients and tonic seizure in 2 patients.There were various types of seizures in 7 children.Nine cases occurred as infantile spasm for the first time.All 15 patients showed varied degrees of development delay, among them, 11 cases had developmental retardation before epilepsy.Three patients had slow rhythm of electroencephalogram background activity, the electroencephalography showed hypsarrhythmia in 13 patients; clinical seizures were detected in 8 cases, among them, epileptic spasms were captured in 7 patients, tonic seizure was captured in 1 patient.Widened frontotemporal subarachnoid space, cerebral atrophy, and corpus callosum dysplasia were examined in 6, 2 and 3 patients by cranial magnetic resonance imaging, respectively.All 15 cases were diagnosed as developmental epileptic encephalopathy, of which 13 cases were consistent with infantile spasms.The age of the last follow-up ranged from 1 year old to 7 years old.After multi-antiepileptic drug treatment, 2 patients were remission, 1 patient(small size of identical twins) died of severe pneumonia at the age of 2 years, and 12 patients still had intermittent seizures, of which 1 patient was transformed from infantile spasms to Lennox-Gastaut syndrome. Conclusions:The onset age of developmental epileptic encephalopathy caused by the DNM1 gene variant usually begins in the infantile period, the peak onset age was 8 months.The main types of seizures include epileptic spasms and focal seizures, developmental retardation can occur before seizures.The clinical manifestations are mostly infantile spasms syndrome, and some children can be transformed into Lennox-Gastaut syndrome.