Clinical characteristics of antiNmethylaspartate receptor encephalitis in children / 中南大学学报(医学版)

OBJECTIVES@#To analyze the clinical characteristics and prognosis of children with anti-N-methyl--aspartate receptor (NMDAR) encephalitis and to provide a basis for early clinical identification of this disease.@*METHODS@#The clinical data of 42 cases of anti-NMDAR encephalitis at Department of Pediatrics, Second Xiangya Hospital, Central South University from January 2015 to March 2018 were collected. The clinical features and followed-up outcomes were analyzed retrospectively.@*RESULTS@#There were 15 cases (35.7%) of males and 27 cases (64.3%) of females in 42 children, with a ratio of 1꞉1.8. They were aged from 4 months to 17 years, with an average of (9.20±4.66) years. The most common initial symptoms were seizures (47.6%, 20/42) and mental behavior disorder (35.7%, 15/42). During the course of the disease, 85.7% patients(36/42) had mental and behavior disorder, 85.7% patients (36/42) had epilepsy, 76.2% (32/42) had speech disorder, 66.7% patients (28/42) had dyskinesia, 66.7% patients (28/42) had the decreased level of consciousness, 61.9% patients (26/42) had autonomic instability, and 57.1% (24/42) patients had sleep disorder. All the children had positive antibody against NMDA receptor resistance encephalitis in cerebrospinal fluid. Head MRI showed the abnormal incidence was 50.0% (21/42), and the lesions involved in parietal lobe, frontal lobe, temporal lobe, occipital lobe, midbrain, thalamus, basal ganglia and optic nerve. There was a patient with optic nerve damage combined with myelin oligodendrocyte glycoprotein (MOG) antibody positive. Forty cases were examined by electroencephalogram (EEG), 92.5% cases (37/40) were abnormal, mainly showing diffuse slow waves, and δ brushes could be seen in severe cases. And there was 1 patient (2.4%) complicated with mesenteric teratoma. The mRS score (2.14±1.46) at discharge was significantly lower than the highest mRS score (3.88±1.38) during hospitalization (<0.05). After 3-39 months of follow-up, mRS score at 3 months after discharge was only 0.81±1.29, which was still improved compared with that at discharge, 76.2% cases (32/42) experienced complete or near-complete recovery (mRS score≤2), and 4.8% (2/42) cases relapsed. There was no mortality; the initial time of immunotherapy and the highest mRS score in the course of the disease were the factors affecting the prognosis. The earlier the starting time for immunotherapy and the lower mRS score in the course of the disease were, the better the prognosis was.@*CONCLUSIONS@#Seizures, mental and behavior disorder, dyskinesias, speech disorder and autonomic instability are common clinical manifestations of anti-NMDAR encephalitis in children. The effect of immunotherapy is significant, and the time to start immunotherapy and the severity of the disease are important factors affecting the prognosis. Anti-NMDAR encephalitis can be combined with other autoantibodies, but its clinical significance and mechanism need further study.

The temporal expression patterns of the transcription factors and target genes in cardiomyocyte hypertrophy / 解放军医学杂志

Objective To investigate the temporal expression patterns of the related transcription factors and target genes in cardiomyocyte hypertrophy,and provide valuable clues for further researches on cardiomyocyte hypertrophy.Methods Isoproterenol (ISO) was used to induce ventricular myocytes hypertrophy in neonatal mice.The survival rate of cardiomyocyte was detected by CCK-8,and the average diameters and surface areas of cells were measured by computer photograph analysis system.The mRNA and protein expression levels of related genes were respectively measured by RT-PCR and Western blotting.Results The model of cardiomyocyte hypertrophy stimulated by ISO was constructed successfully.The expression levels of GATA4,MEF2C,GATA5,BNP and ANP increased 24 hours after ISO treatment,the expression levels of P300,α-MHC and TBX5 increased 12 hours after ISO treatment,and of SRF and β-MHC mRNA increased 6 hours after ISO treatment (P＜0.05).The expression levels of GATA4,α-MHC,β-MHC,SRF and P300 mRNA increased firstly,and then decreased in cardiomyocytes induced by ISO.The expression levels of GATA4,SRF,α-MHC,β-MHC and P300 mRNA were still higher than normal (P＜0.05),but of MEF2C decreased to normal (P＞0.0S) 72 hours after ISO treatment.The expression levels continuously elevated of GATA5,TBX5,ANP and BNP mRNA than that of controls (P＜0.05),while no fluctuation was found in NKX2.5 mRNA expression (P＞0.05).The expression of GATA4 protein increased,while of HEY2 protein decreased 48 hours after ISO treatment (P＜0.05).Conclusions In hypertrophic cardiomyocytes,the expression pattern of MEF2C is similar to,but the patterns of GATA5,GATA4,TBX5 and SRF are different with that in the development of heart,implying these genes are important during the process from compensatory stage to decompensation stage.The expression patterns of GATA4,MEF2C and SRF are similar to that of acetylase P300,implying the temporal expressions could be regulated by P300 in cardiomyocyte hypertrophy.

Analysis of clinical characteristics of different degrees of myocardial noncompaction in children / 临床儿科杂志

Objectives To explore the clinical characteristics and prognosis of different degrees of noncompaction of ventricular myocardium (NVM) in children and to investigate the clinical significance of the ratio of the thickness of the non-compact myocardium to the dense myocardium (N/C). Methods The clinical data of the children with NVM were analyzed retrospectively. The differences between children with N/C > 2.0 and children with N/C 1.4~2.0 were compared. Results There were 26 children in each of the two groups. There was no significant difference in age, sex, the ratios of low birth weight and malnutrition, heart disease, and complication (P all > 0.05). When discharged, 8 cases were improved, 15 cases has no obvious improvement, and 3 cases died in N/C>2.0 group; while 18 cases were improved, 8 cases has no obvious improvement, and none died in N/C 1.4~2.0 group, and the effective rate of treatment between two groups was statistically difference (χ2=7.69,P=0.006). In 8 years of follow-up, the mortality rate in N/C > 2.0 group was higher than that in N/C 1.4~2.0 group, and the difference was statistically different (χ2=6.993, P=0.008). Conclusions The treatment efficiency was lower and the prognosis was worse when the N/C value was higher in children with NVM.

Analysis of clinical characteristics of different degrees of myocardial noncompaction in children / 临床儿科杂志

Objectives To explore the clinical characteristics and prognosis of different degrees of noncompaction of ventricular myocardium (NVM) in children and to investigate the clinical significance of the ratio of the thickness of the non-compact myocardium to the dense myocardium (N/C). Methods The clinical data of the children with NVM were analyzed retrospectively. The differences between children with N/C > 2.0 and children with N/C 1.4~2.0 were compared. Results There were 26 children in each of the two groups. There was no significant difference in age, sex, the ratios of low birth weight and malnutrition, heart disease, and complication (P all > 0.05). When discharged, 8 cases were improved, 15 cases has no obvious improvement, and 3 cases died in N/C>2.0 group; while 18 cases were improved, 8 cases has no obvious improvement, and none died in N/C 1.4~2.0 group, and the effective rate of treatment between two groups was statistically difference (χ2=7.69,P=0.006). In 8 years of follow-up, the mortality rate in N/C > 2.0 group was higher than that in N/C 1.4~2.0 group, and the difference was statistically different (χ2=6.993, P=0.008). Conclusions The treatment efficiency was lower and the prognosis was worse when the N/C value was higher in children with NVM.

ox-LDL inhibits endothelial differentiation of bone marrow mesenchymal stem cells of rats / 基础医学与临床

Objective To investigate that ox-LDL inhibits endothelial differentiation of bone marrow mesenchymal stem cells (MSCs) in rats and its underlying mechanism .Methods Cultured MSCs were divided into four groups:blank groups , ox-LDL groups ( 5 μg/mL ox-LDL ) , ox-LDL +NAC groups ( 5 μg/mL ox-LDL and pre-treated NAC), and negative LDL groups (5 μg/mL nLDL).Cell morphology, endothelial marker and differentiation effi-ciency as well as signal of oxidative and pathway protein were detected after induction by Western blot , real-time PCR.Results MSCs can differentiate into endothelial cells with the expression of endothelial marker vWF , Flk-1 and CD31 at the mRNA and protein level , vascular morphology , ox-LDL obvious inhibited endothelial differentia-tion of MSCs ( P<0.05 ) , but NAC can reverse the inhibition , the amount of ROS in ox-LDL groups was higher than that in ox-LDL+NAC groups ( P <0.05 ) , The expression of phosphorylated Akt decreased distinctly after treatment with ox-LDL( P<0.05 ) , NAC can stimulated its expression close to normal .Conclusions ox-LDL can inhibit endothelial differentiation of MSCs via ROS , NAC in this procese shows inhibition to effect of ox-LDL and Akt signaling also played a critical role .

oxidized low-density-lipoprotein inhabits the proliferation and Oct-4 express of rat bone marrow mesenchymal stem cells in vitro / 重庆医学

Objective To detect the effect of ox‐LDL on self‐renewal and Oct‐4 express of MSCs in vitro .Methods MSCs cul‐tured in vitro were divided into 4 groups :blank control(no reagents in culture system ) ,ox‐LDL (1 ,5 ,10 ,20 μg/mL ox‐LDL were added into culture system) ,ox‐LDL+NAC(corresponding ox‐LDL were added into culture system after NAC treatment ) ,negative control(corresponding nLDL were added into culture system ) .Growth curve were drawn at different time ,Oct‐4 ,a stem cell special marker ,were detected by real‐time PCR ,the production of ROS (reactive oxygen species ,ROS) in culture system were measured with electron paramagnetic resonance spectroscopy .Results proliferation of MSCs was inhibited by ox‐LDL ,when concentration of ox‐LDL was more than 5μg/mL ;apoptosis of MSCs appeared as well as attenuated expression of Bcl‐2 ,ox‐LDL generated a signifi‐cant amount of ROS in the culture system ,which was completely prevented by NAC .Conclusion The proliferation and Oct‐4 ex‐pression of MSCs were inhibited by ox‐LDL ,which may be related to increase of ROS in culture system .