Objective To explore the diagnosis of tuberous sclerosis (TSC) combined with Langerhans cell histiocytosis (LCH) and secondary hemophagocytic lymphohistocytosis (HLH).Methods One case diagnosed of TSC combined wiht LCH and secondary with HLH in infancy was retrospectively analyzed.Results One year and 4-month-old Uyghur boy when he was 4-month-old,there was onset of infantile spasm.Gene detection was performed when he was one year old and showed the absence of the exon 3 to 10 ofTSC2 gene.The diagnosis of TSC was confirmed.Meanwhile,the boy also suffered with skin rash all around,fever,hepatosplenomegaly,and bone defect.The diagnosis of LCH was confirmed by skin biopsy.In addition,the boy was complicated HLH.Conclusions It is rarely seen that TSC combined with LCH,and secondary HLH in one case.The clinical features were complex and need to be differetiate.

Objective To study the characteristics of the central part of the nociceptive system in acute myelitis (AM) with contact heat evoked potentials (CHEPs) and to document the potentials in patients with AM.Methods Twenty patients with AM were recruited in this study as an experimental group,and twenty healthy subjects were chosen as a control group.A heat foil was used to elicit pain and CHEPs.Thermal stimuli were applied at 54.5 ℃ at five sites:the dorsum of the hand,the proximal volar surface of the forearm,the skin of the leg 5 cm proximal to the medial malleolus,and at the C7 and T12 acupuncture locations.The latency and waveform of the evoked potentials were recorded.The conduction velocity of the A8 fibers of the peripheral nerves and of the spinal part of the spinothalamic tract were analyzed.The somatosensory evoked potential (SEP) and sensory conduction velocity (SCV) of the limbs were also examined,and the results were compared with the CHEP results.The results were compared between the two groups.Results The N 550 latencies of the CHEP on the dorsum of the hand,the inside of the leg,and at C7 and T12 were prolonged significantly in the patients with AM compared to the healthy controls.There were no significant differences in the nerve conduction velocity of the Aδ fibers and the velocity or amplitude of sensory nerve conduction in the limbs between the groups.The conduction velocities of the spinothalamic tract were significantly reduced in the patients with AM compared to the control group,while the peak latencies of N13 and the interpeak latencies of N9-N13 and N13-N20 in the AM patients were significantly prolonged compared to the healthy persons.In the patients with AM,CHEP abnormality in the lower limbs (17/20,85％) was significantly higher than in the upper limbs,total CHEP abnormality and CHEP abnormality in the lower limbs were significantly greater than SEP abnormality.Conclusion Persons with AM have abnormalities in the central part of the nociceptive system.When used with MRI and other electrophysiological examinations,CHEP may contribute to diagnosing AM.It could be helpful in the differential diagnosis of AM from motor neuron diseases and peripheral nerve lesions.It is of great potential value in clinical practice.

Objective To study the effect of candesartan (Can) on the LPS‐induced proinflammatory cytokines release in VSMCs ,and to explore the role of TLR4‐mediated signaling pathway in this process .Methods Rat VSMCs were primarily cul‐tured ,and the effect of different concentrations of Can on VSMCs activity was observed by M TT assay .The cells were divided into 5 groups :A(control) ,B(LPS intervention) ,C(LPS + 10 - 7 mol/L Can) ,D(LPS + 10 - 7 mol/L Can ) and E (LPS + Can 10 - 5 mol/L Can) .mRNA and protein levels of Toll‐like receptor‐4(TLR4) ,myeloid differentiation primary response gene 88(Myd88)and nu‐clear factor‐κB(NF‐κB) nuclear translocation were determined by real‐time PCR and Western blot ,respectively ;IL‐1β and TNF‐αconcentration were detected by ELISA .The production of intercellular reactive oxygen species(iROS) was measured by the DCFH‐DA assay .Pretreating VSMCs with the inhibitors against TLR4 ,NADPH oxidase ,and NF‐κB ,or a combination with candesartan and these inhibitors ,and then the expression of IL‐1β and TNF‐α was measured by ELISA .Results Can in the concentration range of 10 - 8 - 10 - 3 mol/L had no significant effect onVSMCs activity .Compared with th control group ,Can could effectively inhibit LPS‐induced VSMCs ,IL‐1β and TNF‐α release ,decreased the mRNA and protein expression of TLR4 ,Myd88 ,reduced the produc‐tion of iROS ,inhibited the NF‐κB(p65) nuclear translocation with a concentration‐dependent manner(P< 0 .05) .Anti‐TLR4 anti‐body ,DPI ,and PDTC all inhibited LPS‐induced inflammatory cytokines release in VSMCs ,and the combination of Can and these blockers showed stronger anti‐inflammatory effect .Conclusion Can can decrease the releaseof inflammatory cytokines IL‐1β and TNF‐α in VSMCs stimulated by LPS ,which is realized by inhibiting the signaling pathway of TLR4/Myd88‐iROS‐NF‐κB .

Objective To investigate the effects of PJ34,a poly ADP-ribose polymerase(PARP)inhibitor,on the expression of matrix metallopro-teinases-9( MMP-9 )and Claudin-5 in ischemic cortex of rats with focal cerebral ischemia-reperfusion(I/R)injury. Methods The focal cerebral ischemia-reperfusion model of middle cerebral artery occlusion(MCAO)was established by intraluminal suture . PJ34 was injected intraperitoneal-ly. Blood-brain barrier(BBB)permeability was quantitatively determined by Evans blue assay. Infarct volume changes were observed by 2,3,5-tri-phenyltetrazolium chloridedyeing(TTC)staining. The expression of the MMP-9 and Claudin-5 in rats of cerebral cortex were measured by immuno-histochemistry assay and western blot analysis . Results Compared with sham group,the expression of MMP-9,the contents of EB and infarct vol-ume increased progressively over time after I/R,and reached maximum levels at 48 h(P<0.05);The expression of Claudin-5,the contents of EB and infarct volume reduced significantly over time after I/R,and reached the minimum levels at 48 h in model group(P<0.05). Compared with model group,the expression of MMP-9,the contents of EB and infarct volume was reduced significantly and the expression of Claudin-5,the con-tents of EB and infarct volume was increased at the same time point in PJ34 group(P<0.05). Conclusion PJ34 maintained the blood-brain barri-er permeability by inhibiting the expression of MMP-9,and increasing the expression of Claudin-5,which had neuroprotection on cerebral ischemia-reperfusion injury.

Objective To study any functional changes in the trigeminal nociceptive system in cases of migraine with aura (MWA) using painful heat-evoked potentials (CHEPs),and to explore the value of CHEP in evaluating the trigeminal small nerve fibers of patients with MWA.Methods Sixty patients with MWA were recruited as the MWA group,and 60 healthy persons were selected as a normal control group.Thermal stimulation at 51 ℃ was applied to the skin above the patients' right and left eyebrows.The N wave latencies and N-P amplitudes of the CHEPs were recorded at the vertex (Cz) of the head.The intensity of the stimulus was graded using a visual analogue scale (VAS).Results In the control group the simulation produced no significant differences in the N550 or P750 latencies or in the N-P amplitudes.In the MWA group no significant differences were observed when the headache side was stimulated,but the N550 and P750 latencies were both significantly shorter than those of the control group and the N-P amplitude was significantly greater than that of the control group.The average VAS score on both sides in the patients with migraine was significantly higher than those of the control group.However,there was no significant difference in average VAS scores between the headache and non-headache sides of the patients with migraine.Conclusions CHEP is a reliable method for detecting any pathological changes in the trigeminal small nerve sensory pathway.Increased cutaneous allodynia in acute migraine attacks is detectable using CHEPs.

Objective To investigate the influence of poly(ADP-ribose)polymerase inhibitor PJ34 on blood brain barrier(BBB)in rats with cere-bral ischemia-reperfusion injury. Methods Rat model of cerebral ischemia-reperfusion injury was established by the middle cerebral artery occlu-sion. A total of 135 SD rats were randomly divided into 3 groups:sham-operated group(sham group),ischemia-reperfusion group(IR group)and PJ34 group(PJ34 group). 45 animals in each group were then equally divided into subgroups and the rats were sacrificed at 6 h,24 h,48 h after re-perfusion,respectively. BBB permeability was evaluated by detection of extravasated Evans blue(EB). The expression of tumor necrosis factorα(TNF-α)and matrix metalloproteinase 9(MMP-9)activity were measured by immunohistochemistry and western blot at different time points. Re?sults Compared with sham group,the contents of EB and the expressions of TNF-αand MMP-9 in IR group were increased significantly(P<0.05). Compared with IR group,the contents of EB and the expressions of TNF-αand MMP-9 in PJ34 group were markedly decreased at the same time point(P<0.05). Conclusion The present study provided in vivo evidence that PARP inhibitor PJ34 can protect against cerebral ischemia re-perfusion injury,and the mechanism might be related to maintaining the stability of blood-brain barrier by suppressing the expression of TNF-αand MMP-9 in ischemic cortex.

Objective To explore the value of ocular vestibular evoked myogenic potential in treating brainstem infarctions through comparing the characteristics ocular vestibular evoked myogenic potential (oVEMP) and brainstem auditory evoked potential (BAEP) in patients with brainstem infarctions.Methods A total of 60 patients with brainstem infarctions were enrolled in a brainstem infarction (BI) group, while another sixty healthy volunteers were selected as the control group.All patients underwent oVEMP and BAEP tests via air-conducted stimuli.Results The oVEMPs were elicited reliably and stably in 58 of the control group, and 42 of the BI group, with the bilateral N1 and P1 latencies of oVEMP significantly prolonged [N1 (left) : 11.85 ± 0.82 ms, N1 (right) : 11.91 ± 0.86 ms, P1 (left) : 15.52 ± 1.61 ms, P1 (right) : 15.63 ± 1.64 ms respectively] and the bilateral N1-P1 amplitudes of oVEMP significantly reduced [1.23 ±0.42 μV (left) and 1.58 ± 0.70 μV (right) respectively].Moreover, no recordable oVEMPs was elicited in the other 18 patients, among which found 13 prolonged N1 latencies, 10 prolonged P1 latencies, 6 prolonged N1 and P1 latencies and 8 reduced N1-P1 amplitudes of oVEMP.Altogether, 45 abnormal oVEMPs were found, with an abnormal rate of 75％.The average peak latencies of Ⅴ waves [(5.98 ± 0.37) ms] and interpeak latencies of Ⅲ-Ⅴ and Ⅰ-Ⅴ waves [2.93 ± 0.34 ms and 4.96 ± 0.39 ms respectively] in the BI group were significantly prolonged compared to the controls (P＜ 0.01).The amplitude ratio of Ⅴ/Ⅰ (0.48 ± 0.10) in the BI group was significantly reduced compared to the controls (P ＜ 0.05).Forty-two abnormal BAEPs were found, with an abnormal rate of 75％ , including 17 prolonged latency of Ⅴ waves, 6 prolonged latency of Ⅲ waves, 15 prolonged interpeak latencies of Ⅲ-Ⅴ waves, 9 prolonged interpeak latencies of Ⅰ-Ⅴ waves, 11 cases of Ⅲ-Ⅴ interpeak latency larger than Ⅰ-Ⅲ interpeak latencies, 13 cases of the amplitude ratio of Ⅴ / Ⅰ smaller than 0.5 and 3 case of unclear waves.There were no significant differences in abnormal rate when using the oVEMP (42/60 and 75％) and BAEP (45/60 and 70％) testing However, the abnormal rate was 91.7％ when combining oVEMP with BAEP testing, significantly higher than that when only conducting BAEP testing.Conclusion Patients with brainstem infarctions have abnormalities in oVEMP.Combined with MRI and other electrophysiological testing, oVEMP may contribute to the diagnosis of brainstem infarctions.

Chinese traditional patent medicine for promoting blood circulation and removing blood stasis(PBCRBS) originated from traditional Chinese medicine theory and had approved efficacy and safety standards. However, its compatibility regularity and anti-thrombotic mechanism is not clear. To analyze the compatibility regularity and anti-thrombotic mechanism of Chinese traditional patent medicine for PBCRBS, a statistical and bioinformatics analysis was carried out using traditional Chinese medicine inheritance support system (TICMISS, V2.0) and ingenuity pathway analysis (IPA). The compatibility regularity analysis shows that the most commonly used herb combinations are Danshen (Salvia miltiorrhiza Bge.), Chuanxiong (Ligusticum chuanxiong Hort.) and Honghua (Carthamustinctorius L.). The anti-thrombotic mechanism analysis reveals that 25 ingredients have an effect on 29 thrombosis related molecules which 23 molecules are related to inflammation response. Furthermore, there are 5 inflammation molecules (NOS2, PTGS2, IL6, TNF, IL1β) served as major targets. At the same time, Danshen, Chuangxiong and Honghua mainly used as sovereign herb or minister herb in the application of cardiovascular and cerebrovascular diseases. Therefore, Chinese traditional patent medicine for PBCRBS probably has an effect on anti-thrombotic activity through inhibiting the inflammatory response. In summary, the most commonly used herb combinations of Chinese traditional patent medicine for PBCRBS are Danshen, Chuanxiong and Honghua. Inhibiting inflammatory response, especially inflammation related molecules (NOS2, PTGS2, IL6, TNF and IL1β), is probably a new starting point to clarify the anti-thrombotic mechanism of Chinese patent medicine for PBCRBS.

ObjectiveTo discuss the relationship between the cross-reactivity of antibody against the hypervariable region 1 ( HVR1 ) of hepatitis C virus and early viral response ( EVR ) in patients undergoing antiviral therapy.MethodsBy ELISA and HCV HVR1 antibody cross-reactivity matrix reagent,the differences of anti-HCV hypervariable region antibody were tested in baseline serum from 46 patients with chronic hepatitis C before antiviral therapy.HCV genotyping and HCV RNA were analyzed by RT-PCR method.The HCV RNA assay was done at three time points:before treatment,pegylated interferon in combination with ribavirin therapy for 12 and 48 weeks.ResultsIn 46 cases of chronic hepatitis C patients,there were 16 cases with HCV 2a type,30 cases with l b and 33 patients obtained EVR.The EVR incidence of type 2a[ 93.8％ ( 15/16 ) ] was higher than that of type 1 b[ 60.0％ (18/30),x2 =4.316,P ＜ 0.05 ].In the EVR group of type 1b chronic hepatitis C patients,the positive number of average multi-target HVR1 antigen was ( 12 ± 4),which was significantly higher than that in the Non-EVR patients [ (7 ± 5 ),t =2.797,P ＜0.01 ].Bv the Fisher exact test,it was showed that patients'serum response to HVR1 antigens numbered 001,003,009,013,016 were higher in EVR group than those in non-EVR group,with statistically significant (P ＜ 0.05 ).ConclusionThe cross-reactivity of HVR1 antibody may play an important role in predicting the effectiveness of antiviral therapy.

Objective To evaluate the efficacy of intracoronary,antologous bone marrow mononuclear cell transplantation on the patients with chronic heart failure.Methods Eighty patients with chronic heart failure were assigned into bone marrow cell transplantation group (n=48) and control group (n=32),with intmcomnary delivery of autologous bone marrow mononuclear cell suspension or not respectively,in addifion to conventional treatment.The left venlrieular ejection fractions (LVEF),the level of plasma brain natriuretic peptide (BNP),and the siges of myocardial perfusion defect were measured and compared with useing single photom emission computed tomography (SPECT) and echocardiography before and after the treatment.Results The one year follow-up results showed that LVEF increased from base-line (42.1±5.8)%,(43.9±6.7)% to (54.1±4.8)%,(49.8±7.7)% by echocardiography.The plasma levels of BNP decreased from base-line (699±101)ng/L,(687±95)ng/L to (305±78)ng/L,(399±89)ng/L,and the size of myocardial perfusion defect decreased from bese-line (26.8±8.6)%,(26.3±6.5)% to (15.8±4.3)%,(20.5±7.8)% in bone marrow cell transplantation group and control group respectively,the absolute changes were statistically significant.During and after the procedure of bone marrow cell transplantation group,there was no complication occurring. Conclusion Intmcoronary autologous bone marrow mononuclear cell transplantation for treating patients with chronic heart failure results in improvement in left ventricular systolic function and myocardial perfusion,and this therapy is safe and feasible.