Outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting / 中华儿科杂志

Objective: To summarize the outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting. Methods: This study was a retrospective cohort study. A total of 19 neonates who had pulmonary atresia treated by ductus arteriosus stenting in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from April 2014 to June 2021 were included. They were divided into the intact ventricular septum (PA-IVS) group and the ventricular septal defect (PA-VSD) group. Ductus arteriosus stents were implanted by different approaches. These children were followed up regularly at the 1, 3, 6, and 12 months after the surgery and annually since then to evaluate the outcome. Independent sample t-test was used for the statistical analysis. Results: There were 12 children in PA-IVS group and 7 in PA-VSD group. All of them were full term in fants. The gestational age of the PA-IVS group and the PA-VSD group was (38.8±1.1) and (37.7±1.8) weeks, the birth weights were (3.2±0.4) and (3.4±1.1) kg, and the age at operation was (10±9) and (12±7) days, respectively, without significant difference (all P>0.05). Among the 12 children with PA-IVS, 9 had stents successfully implanted through the femoral artery and 3 through the femoral vein. Of the 7 children with PA-VSD, 2 had the stents successfully implanted via the femoral artery and 2 failed, and the remaining 3 had stents successfully implanted via the left carotid artery. There was no postoperative thromboembolism, arteriovenous fistula, pseudoaneurysm or other vascular complications. Five children with PA-VSD who had successful operations were followed up at 6 months of age. They all had the operation for pulmonary atresia, repair of the ventricular septal defect, removal of arterial duct stents, and ligation of the arterial duct. All children survived without any stent displacement or stenosis and biventricular circulation was achieved during the follow-up. Conclusions: Ductus arteriosous stenting can be the first-stage treatment for children with PA-IVS and PA-VSD. In addition to the traditional femoral vein and femoral artery approach, the carotid artery can be used as a route for stent placement.

Role of NADPH oxidase in brain damage oxidative stress in ATP7Btx-J mice / 中华神经医学杂志

Objective To investigate whether NADPH oxidase is involved in brain damage of ATP7Btx-J mice through interfering oxidative stress.Methods ATP7Btx-J mice (20 weeks old),wild-type (WT) mice (20 weeks old) and apo-ATP7Btx-J mice (given the NADPH oxidase inhibitor apocynin) were chosen in our study; apo-ATP7Btx-J mice were treated by daily oral administration with 200 mg/kg of apocynin since 16 weeks old till 20 weeks old.Copper concentration was determined by inductively coupled plasma-mass spectrometry (ICP-MS) and NADPH oxidase activity was detected by colorimetric method.The superoxide level was measured using superoxide-sensitive fluorescent probe dihyroethidine (DHE).The protein expression level of Cleaved caspase-3 was analyzed by Western blotting.The level of neuronal apoptosis was assayed with TUNEL method.Results The copper content in the striatum region of ATP7Btx-J mice was significantly higher than that of wild-type (WT) mice (P＜0.05).The activity ofNADPH oxidase and concentration of superoxide anion in the striatum region of ATP7Btx-J mice were significantly higher than those of WT mice (P＜0.05); those in the striatum region of apo-ATP7Btx-J mice were significantly lower than those of ATP7Btx-J mice (P＜0.05).The protein expression of Cleaved caspase-3 and the level of neuronal apoptosis in the striatum region of ATP7Btx-J mice were significantly higher than those of WT mice (P＜0.05); those in the striatum region of apoATP7Btx-J mice were significantly lower than that of ATP7Btx-J mice (P＜0.05).Conclusion NADPH oxidase may play a role in neuronal apoptosis in the striatum region of ATP7Btx-J mice through oxidative stress,and apocynin can protect the nervous system decreasing the NADPH oxidase level.

Signal transduction pathway in copper-induced apoptosis of primary cultured rat cortical neuron / 中华神经医学杂志

Objective To investigate copper-induced apoptosis of primary cultured rat cortical neurons and the protective effect of thioredoxin,an apoptosis signal regulated kinase-1(ASK1)inhibitor,and explore the role of ASK1-mediated c-Jun N-terminal kinase(JNK)/caspase-3 signaling pathway in the neurotoxicity of copper.Methods The changes in the viability and apoptosis of primary cultured rat neurons following exposure to cupric acetate and thoredoxin were assessed using MTT assay and flow cytometry.Western blotting was used to detect the expressions of phosphorylated ASK1(p-ASK1),p-JNKand caspase-3 in the exposed cells.Results Administration of cupric acetate in the cell culture resulted in a concentration-dependent increase of cell apoptosis and a reduction of the viability of the neurons,and the effect was antagonized by thoredoxin.The expression of p-ASK1 and p-JNK began to increase in the neurons at 4h after cupric acetate exposure,and reached the peak level at 48h,showing a time-and concentration-dependent pattern of the changes.Activated caspase-3 was expressed at 24h after the exposure,and the peak expression occurred at 48h.The application of thoredoxin significantly lowered the expressions of p-ASK1,p-JNK and caspase-3,and increased the viability and reduced the apoptotic rate in the exposed neurons(P＜0.05).Conclusions Copper can induce apoptosis of primary cultured rat cortical neurons,in which process ASK1-mediated JNK/caspase-3 signaling pathway may play a critical role.Thoredoxin can protect the cortical neurons from injuries induced by the copper.

Role of caspase-3, -8, and -9 in apoptosis of copper induced primary cortical neurons / 中国当代儿科杂志

<p><b>OBJECTIVE</b>This study aimed to investigate the mechanism of primary cortical neuron injury induced by high concentrations of copper by observing the effect of aceticum culture medium on apoptosis of rat primary cortical neurons and expression of cleaved caspase 3, caspase 8 and caspase 9.</p><p><b>METHODS</b>Primary cortical neurons were cultured for 72 hrs and then exposed to different concentrations of aceticum culture medium (20, 40 and 80 microM). The viability of neurons was detected by the MTT method. Apoptosis was observed by Hoechst33258 and flow cytometry Annexin V/PI. Expression of caspase 3, caspase 8 and caspase 9 was measured by Western blot.</p><p><b>RESULTS</b>Following incubation with aceticum culture medium, apoptosis of neurons was induced. Theviability of neurons was remarkably reduced and the rate of apoptosis was tremendously increased in a concentration dependent manner. Caspase 8 and caspase 9 were activated in 20 microM of copper aceticum culture medium 4 hrs after incubation and peaked at 48 hrs in various concentrations of copper aceticum culture medium, presenting with a time and concentration dependent manner. The activated caspase 3 was observed in 20 microM of copper aceticum culture medium 24 hrs after incubation, which was later than the activated caspase 8 and caspase 9. Caspase 3 expression reached a peak 48 hrs in various concentrations of copper aceticum culture medium, presenting with a time and concentration dependent manner.</p><p><b>CONCLUSIONS</b>The apoptosis of primary cortical neurons can be induced by copper. Caspase 3, caspase 8 and caspase 9 cascade reaction may involve in the apoptosis of copper induced rat primary cortical neurons.</p>

Study on Mutation in Exon 8 of ATP7B Gene in Chinese Patients with Wilson Disease / 实用儿科临床杂志

G,the noval insertion mutation of c.2298_2299insC is identified in Chinese patients.