Background Bietti crystalline dystrophy (BCD) is a congenital and autosomal recessive hereditary eye disease characterized by multiple glistening intraretinal crystals scattered over the fundus.Studies determined abnormality of fatty acid metabolism probably is associated with BCD.However,the study on the alteration of blood lipid level in BCD patients is rare.Objective This trail was to study the change of serum lipids in BCD patients.Methods A total of 50 patients with bilateral BCD and 50 matched healthy volunteers were included from November 2011 to March 2013 in Beijing Tongren Eye Center with the approval of Ethic Committee of Beijing Tongren Hospital.Written informed consent was obtained from each subject before any medial examination.Peripheral blood of 3 ml was collected from the subjects.The serum concentrations of triglyceride (TG),total cholesterol (TC),low density lipoprotein cholesterol (LDL-C) and high density lipoprotein cholesterol (HDL-C) were measured and analyzed.The examination outcome was identified based on the criteria of China Adult Dyslipidemia Prevention Guideline (Version 2007).Results Abnormality of serum lipid content was detected in 58.00％ patients (29/50),and hypertriglyceridemia and hypercholesteremia were in 34.48％ (10/29),respectively,and mixed hyperlipidaemia was in 27.59 ％ (8/29).The serum levels of TG,TC and LDL-C were (1.63± 1.19) mmol/L,(5.10±1.05) mmol/L and (3.27±0.97) mmol/L in the BCD group,which were significantly higher than (0.93± 0.33) mmol/L,(4.33 ±0.56) mmol/L,(2.63 ±0.51) mmol/L of the normal group (t =4.036,4.496,4.095,all at P=0.000).Conclusions The serum lipid levels elevate in BCD patients,which might be related to the occurrence of BCD.

Objective To investigate the effect of microglial derived extracellular vesicles on neuronal damage in the context of heat stress.Methods After BV2 microglial cells were exposed to heat stress,the supernatant was collected and subjected to ultracentrifugation at different speeds to obtain large and small vesicles,respectively.Nano Particle Tracking and Zeta Potential Distribution Analyzer was used to measure and analyze the size distribution of the large vesicles and small vesicles.Western blotting was used to detect the expression of specific vesicle surface markers,TSG101,CD63 and flotillin-1.Microglial extracellular vesicles were labeled with PKH67 dye and then co-cultured with N2a cells to examine the uptake by capacity the neurons.After large and small vesicles derived from microglia after heat stress stimulation were co-cultured with N2a cells,respectively,CCK-8 assay,lactate dehydrogenase (LDH)assay,Trypan blue staining and TUNEL assay were employed to evaluate heat stress induced neuronal damage.Results The small vesicles were in a particle size of 30～120 nm,and highly expressed TSG101 and CD63,whereas the large vesicles,in a size of 90～1000 nm,highly expressed flotillin-1.The BV2-derived extracellular vesicles could be taken up by N2a cells and were proved to be involved in the modulation of N2a cell injury caused by heat stress.CCK-8 assay showed that both large and small vesicles of microglial cells inhibited the viability of N2a cells after heat exposure (P<0.05).The results of LDH assay,Trypan blue staining and TUNEL assay showed that both large (P<0.05)and small vesicles (P<0.01)significantly enhanced the LDH release,blue stain intensity and apoptosis of N2a cells after heat exposure,and the release,intensity and apoptosis were stronger in the cells treated with small vesicles than those group of large vesicles.Conclusion Microglia aggravate heat stress-induced neuronal damage through releasing extracellular vesicles.

Objective To explore the protective effect and underlying mechanism of roxadustat(FG-4592),hypoxia-inducible factor-α(HIF-α)prolyl hydroxylase inhibitor,on brain injury caused by heat stroke(HS).Methods A total of 140 male C57BL/6J mice(6～8 weeks old,weighing 18～22 g)were subjected,and 40 of them were randomly divided into HS group,and low-,medium-and high-dose roxadustat groups(LD,MD and HD groups,5,10 and 20 mg/kg),with 10 mice in each group.The 24-hour survival rate was observed to determine the optimal dosage of roxadustat after modeling.Additionally,the remaining 100mice were randomly allocated to normal control(Control)group,roxadustat(FG-4592)group,HS group,and roxadustat+HS(FG-4592+HS)group,with 25 mice in each.Heat shock was inflicted to establish mouse model of HS.Modified neurological severity score(mNSS)was used to assess neurological function.HE staining of brain sections was performed to examine pathological damage,and Fluoro-Jade C staining was applied to observe neuronal degeneration.The activity of total superoxide dismutase(SOD)and content of malondialdehyde(MDA)in brain tissue were measured to assess oxidative stress.Transmission electron microscopy was employed to visualize mitochondrial damage.Western blotting was performed to assess the protein levels of Caspase-3,Cleaved Caspase-3,Mfn1,Mfn2,Opa1,Fis1,HIF-1α,HO-1 and p-Drp1(Ser616)/Drp1 ratio in the cerebral cortex.Results Compared to the HS group,FG-4592 significantly improved the survival rate of HS mice within 24 h,with the MD group showing the highest survival rate.Compared to the Control group,the HS group showed an increase in mNSS score(P＜0.05),an elevation in the MDA content in the cerebral cortex(P＜0.05),and a decrease in total SOD activity in the cerebral cortex(P＜0.05);HE staining revealed pathological damage in the cerebral cortex,and Fluoro-Jade C staining displayed obvious neuronal degeneration in the cerebral cortex;Electron microscopy revealed obvious mitochondrial structural damage in the cerebral cortex tissue;The protein expression of Caspase-3,Cleaved Caspase-3,Fis1,HIF-1α,HO-1 and p-Drp1(Ser616)/Drp1 ratio was increased(P＜0.05),while that of Mfn1,Mfn2,and Opa1 was decreased(P＜0.05).Pretreatment with FG-4592 significantly reduced the mNSS score in HS mice(P＜0.05),decreased MDA content(P＜0.05),and enhanced total SOD activity(P＜0.05).Additionally,FG-4592 pretreatment improved pathological damage in the cerebral cortex,reduced neuronal degeneration,and mitigated mitochondrial structural damage.Furthermore,it decreased the protein levels of Caspase-3,Cleaved Caspase-3,Fis1 and p-Drp1(Ser616)/Drp1 ratio(P＜0.05),while increased the levels of Mfn1,Mfn2,Opa1,HIF-1α,and HO-1(P＜0.05).Conclusion Roxadustat regulates the balance between mitochondrial fission and fusion,reduces mitochondrial structural damage,oxidative stress and apoptosis,and alleviates heat stroke-induced brain injury.

ObjectiveTo observe the characteristics of gait symmetry and its influencing factors in patients with incomplete spinal cord injury (ISCI). MethodsFrom May, 2018 to November, 2021, 34 patients with ISCI in Beijing Bo'ai Hospital were divided into symmetrical injury of lower limb (SI) group and asymmetrical injury of lower limb (ASI) group according to the lower extremities motor score (LEMS). Three dimensional motion acquisition system and plantar pressure acquisition system were used for gait test. The symmetry indexes of step length, stance time and swing time were caculated. ResultsThe symmetry indexes of step length, stance time and swing time were significant lower in SI group than in ASI group (|t| > 2.619, P < 0.01). Stance time and swing time significantly correlated to the difference of bilateral LEMS in ASI group (r > 0.468, P < 0.01). Discriminant analysis showed that gait parameter equations were different for patients with different symmetry of lower limb injuries. ConclusionThe symmetry of lower limb motor function impacts gait symmetry for patients with ISCI, especially the difference value of bilateral total LEMS. Gait parameters can be used to determine the symmetry of lower limb injury in patients with ISCI.