Objective To study the various clinical manifestations of niemann pick disease, and to improve the ability of diagnosis and differential diagnosis in order to reduce misdiagnosis or missed diagnosis.Methods The onset and diagnostic procedure of a seven-month-old infant with niemann pick disease were reported and relative literature were reviewed and studied.Results The infant demonstrated severe hepatosplenomegaly, abnormity of liver function, anemia and development backwardness.Bone marrow examination indicated storage cells suggestive of niemann pick cells.Conclusion Niemann pick disease is a group of rare diseases with various clinical manifestations. Clinical suspected cases need further bone marrow examination, pathologcal biopsy and gene tests.

Objective To investigate the effect of propofol on the heme oxygenase-1(HO-1)expression in rats with H_2O_2-mediated damage to cultured cardiac myocytes.Methods Primary cultured new-born rat cardiac myocytes were divided into 8 groups:control,H_2O_2 group,low dose propofol (LP) group,middle dose propofol(MP)group,high dose propofol(HP)group,LP +ZnPP Ⅸ group.MP+ZnPP Ⅸ group and HP+ZnPP Ⅸ group.The cells in all groups were incubated for 6 h.The concentration of malondialdehyde(MDA),activity of superoxid dismuase(SOD),mitochondria and Caspase-3,and the expression of HO-1 were determined.Results As compared with H_2O_2 group,the HO-1 expression,and the activity of SOD and mitochondria were significantly increased(P<0.05,P<0.01),and activity of Caspase-3 and concentration of MDA was decreased(P<0.05)in MP and HP groups.As compared with LP,MP and HP groups respectively.the HO-1 expression and SOD activity were significantly decreased and the concentration of MDA increased in LP+ZnPP IX,MP+ZnPP Ⅸ and HP+ZnPP Ⅸ groups(P<0.05,P<0.01),while Caspase-3 activity was significantly increased in MP+ZnPP IX and HP+ZnPP IX(P<0.05,P<0.01).Conclusion Propofol can protect cardiac myocytes against H_2O_2-mediated cytotoxicity in a dose-dependent manner,and increase the HO-1 expression,which may partly mediate cytoprotective effects of propofol.

Objective: To prove that the interstitial cells of cajal (ICCs) are the pacemaker of gallbladder smooth muscle in guinea pigs. Methods: The contraction of gallbladder was observed by isometric transducer and spontaneous electrophysiology was recorded in vivo and in vitro. ICCs were damaged with methylene and illumination. The ICCs were isolated by collagenase digestion and the morphology of single ICC was analyzed. C-kit expression was detected by laser confocal microscopy to identify the isolated ICCs. Spontaneous currents of ICCs were recorded with EPC-10 Patch Clamp. Results: Slow wave and action potential were recorded in gallbladder in vivo, with the frequency of slow wave being (58±3.2) bpm (n=16). Methylene blue and illumination treatment led to decrease or disappearance of slow wave and action potentials. Some cells isolated from gallbladder smooth muscle ([5±1.2]%, n=12) showed the morphological characteristics of ICCs under light microscope, with (21±4)% of the cells were positive for c-kit. When the membrane potential was at -60 mV, the frequency of spontaneous inward currents was (58.4±3.5) bpm (n=21) and the amplitude was -(72±3.5) pA. Conclusion: ICC-like cells are present in the gallbladder smooth muscle of the guinea pigs, and participate in the pacemaking of spontaneous current and action potential of gallbladder smooth muscle.

Objective: In order to implement effectively on-scene medical response to a nuclear or radiological emergency, and also keep track of relevant on-scene audio and video information and monitoring data, we have designed and developed a monitoring platform for medical response to nuclear or radiological emergency. Methods:Based on relevant national standards for emergency response and the features of nuclear or radiological emergency, exploiting modern information technology and monitoring equipments, we formulate the design idea, characteristics, functions and framework of the platform. This platform takes treatment centers for nuclear or radiological injuries, designated medical institutions and medical response teams as main users. Results:We have developed the monitoring platform for medical response to nuclear or radiological emergency. Conclusion:Through the application of this platform, we hope to enhance the preparedness and on-scene response capability for nuclear or radiological emergency, and also provide information support for disposal of nuclear or radiological emergency in the future.

Stem cells possess unlimited self-renewal properties and the ability to produce multiple differentiation progenitors. Mesenchymal stem cells (MSCs) can specifically migrate to multiple kinds of tumors and their metastases in a systemic manner(to all parts of the body). Therefore genetically modified MSCs can serve as vehicle of anticancer factors to inhibit growth of tumor. But many questions remain to be answered and the precise mechanisms remain unclear. This paper reviews the mechanism of MSC homing, MSCs as delivery vectors for cancer therapy and its advantages over traditional drug vehicle. MSCs as delivery vectors can be used for cellular therapy in future cancer treatment.

Stem cells possess unlimited self-renewal properties and the ability to produce multiple differentiation progenitors. Mesenchymal stem cells (MSCs) can specifically migrate to multiple kinds of tumors and their metastases in a systemic manner(to all parts of the body). Therefore genetically modified MSCs can serve as vehicle of anticancer factors to inhibit growth of tumor. But many questions remain to be answered and the precise mechanisms remain unclear. This paper reviews the mechanism of MSC homing, MSCs as delivery vectors for cancer therapy and its advantages over traditional drug vehicle. MSCs as delivery vectors can be used for cellular therapy in future cancer treatment.

The interaction between genetic and environmental factors influences the onset,development and prognosis of cerebral ischemia.Recently,epigenetic regulation is increasingly becoming a research focus on cerebral ischemia,and there has been great progress in research on DNA methylation,histone modification and miRNA network.Epigenetic regulation provides a new idea for identifying potential therapeutic targets of cerebral ischemia.

In recent years,the role of phosphodiesterase 5(PDE5)has been highlighted in the development and progression of neurological disease.PDE5 inhibitors show significant effect of neruoprotection,which may be related with some effects such as resistance to stroke,anti-oxidation,inhibition of neuroinflammation and amelioration of cognitive deficits.Based on the domestic and overseas researches about PDE5,this review systematically summarized the neuroprotection of PDE5 and their related mechanisms.

Adult ; Cadmium ; Female ; Glomerulonephritis, IGA ; etiology ; Humans ; Occupational Exposure

AIM: To investigate the clinic efficacy and operation safety of improved small incision extracapsular cataract extraction in the sight-regaining program.
METHODS: Totally 82 cases ( 82 eyes ) included in the sight-regaining program of the cataract patients were underwent small incision extracapsular cataract extraction conbined with improved technology ( anterior capsule staining, continuous curvilinear capsulorhexis, auxiliary incision ) , postoperative visual recovery and operative complications were observed.
RESULTS: Postoperative visual acuity: uncorrected visual acuity when out of hospital: ≥0. 3 were 67 cases (82%);1wk after dismissed from hospital:≥0. 3 were 76 cases (93%), admist which ≥0. 8 were 45 cases (55%). There is no serious complication such as posterior capsule rupture, zonular dialysis, etc. in the operation.CONCLUSION: Combined use of improved technology can increase operation safety of small incision extracapsular cataract extraction.