Objective To explore the pathogenic course,clinical features and prognosis on hallervorden spatz disease(HSD).Methods To review and sum up clinical data of three patients of 1985 with HSD in one family,and followed up survey in 2001.Results The same parents had three(2 males,1 female) of six children who were suffered from HSD.Clinical features include bilateral pigmentary degeneration of the retina,optic atrophy and progressive dementia,and lay in bed after several years with convulsive seizure,opisthotonus,limb rigidity,hyperreflexia, pyramidal sign positive;they were suited therapy to the illness.2 cases for death,another case for progressive deterioration,the patient lost labour ability and couldn't take care of himself.Conclusion HSD is kindred hereditary disorder,major cases occur the symptoms before 20 years old (progressive deterioration).After attacking about 20 years the patients died.The HSD gene located on chromosome 20p12.3 p13.

Objective To discuss the relationship between the expression of programmed death-1 (PD-1) in liver with traditional Chinese medicine (TCM) syndromes and pathological diagnosis in chronic hepatitis B.Methods 156 CHB patients treated in our hospital of infectious diseases department were recruited as an observation group. Based on the principle of informed voluntary and approved by the ethics committee, liver biopsy was adopted to make clear liver tissue pathology. According to TCM classification criteria, CHB patients were divided into five groups: a blood stasis group, a damp heat resistance group, a liver and spleen deficiency group, a liver and kidney yin deficiency group, and a spleen and kidney yang deficiency group. In the same period, 12 healthy persons were recruited as a control group. The PD-1 expression was detected with immunohistochemical SP method, and the correlation between expression of PD-1 in liver tissue and TCM syndrome type and liver pathology was analyzed.Results Different degrees of positive cell expression were found in the liver tissue. With the liver inflammation and fibrosis severity, the number of PD-1 positive cells also increased. The PD-1 expression levels varied with mild, moderate, and severe CHB patients (0.24 ± 0.03, 0.36 ± 0.05 vs. 0.43 ± 0.05) , which were statistically significant (P<0.05) . PD-1 expression levels also varied among different TCM type CHB patients, of which, PD-1 expression of blood stasis type was the highest (0.35 ± 0.04), while the liver and spleen deficiency type was the lowest (0.23 ± 0.03).Conclusion The expression levels of PD-1 has a certain correlation with the patients illness, chronic mechanism, and TCM syndromes. CHB patients can be treated by controlling the expression of PD-1.

Objective To understand head nurse's leadership behavior and analyze the influence of it on nurse's involvement and self-efficacy.Methods General information questionnaire was taken,and the data of 280 nurses to head nurse's leadership behavior,involvement and self-efficacy were investigated.Results There were differences in nurse's involvement and self-efficacy between head nurse's transformation and transaction leadership behavior (F=4.39,2.37,2.94,2.17,P ＜0.05).The transformation leadership behavior dimensions were positive correlation with nurse's involvement and self-efficacy,P＜0.05.Conclusion Head nurse should fully play a positive role of transformation leadership behavior,in order to promote involvement and improve self-efficacy.

Objective To evaluate the effects of pelvic autonomic nerve preservation (PANP) during the radical resection of rectal carcinoma on the sexual and urinary function of male patients.Methods The sexual and urinary dysfunction rates from 45 male patients who undergone PANP and 45 control patients who did not undergo PANP during radical resection of rectal carcinoma were analyzed.Results In the PANP group,the incidences of erectile dysfunction,ejaculatory dysfunction and urinary dysfunction were 26.67％,24.44％ and 28.89％,respectively.But in control group,the above three rates were 57.78％,60.00％ and 62.22％ in order.The rates of these three dysfunctions significantly were different between the two groups ( x2 =8.92,11.66,10.08,P ＜ 0.01 ).Conclusion PANP during radical resection of rectal carcinoma could reduce the post-operative sexual dysfunction and urinary dysfunction.

Objective To investigate whether there is an inhibition of the human lung cancer cell line A549 induced by the culture supernatant of Toxoplasma gondii in vitro and the mechanism of the inhibition. Methods A549 cells 5 x 10~4mL~(-1) were cultured and harvested. The cells were treated for different hours with different concentrations of Toxoplasma gondii culture supernatant (the concentrations of tachyzoites were 4×10~7mL~(-1), 8 × 10~7mL~(-1), 16 ×10~7mL~(-1) respectively). Growth inhibition rate was measured with the MTT method; Cell cycle was checked with flow cytometer. Western blot was used to detect the level of cyclinBl and cdc2 of cells. Results The culture supernatants of Toxoplasma gondii inhibited proliferation of A549 cells in a time-dose dependent manner. Cell cycle was significantly stopped at G_2/M phase by the culture supernatants with FCM technology. The culture supernatant of Toxoplasma gondii reduced the expressions of gene cyclinBl and cdc2 of A549 cells. Conclusion The culture supernatant of Toxoplasma gondii may inhibit A549 cell and arrest the cell cycle of A549 cells mainly by regulating the expression of gene cyclinBl and cdc2.

In recent years,fluorescent probes become more available for the progress of biology and gene technology,which has accelerated the development of fluorescence molecular imaging.With these fluorescent probes,target molecular,protein and gene can be specifically located and analyzed,which make possible the early detection and treatment of disease.This paper gives an introduction of the fluorescent probe technology and its application in the fields of biology and medicine.

Adult ; Child, Preschool ; Craniofacial Dysostosis ; surgery ; Craniotomy ; methods ; Female ; Humans ; Male

Objective To observe the clinical curative effect of photorejuvenation of photodamaged skin by intense pulsed light (IPL) and 755nm laser. Methods A total of 187 patients were treated with a series of five or more full-face treatments using IPL and 755nm laser alternatly. After the treatement, the patients and physicians subjectively evaluated improvement in five aspects, including color of the face, telangiectasia, pore, pigmentation, and skin texture. Results According to comprehensive evaluation of the five aspects by the patient and physicians, and as compared to the first score, significant difference was observed (P

Objective To explore any effect of calorie restriction on the proliferation of satellite cells in the skeletal muscles of elderly rats.Methods Twelve male C57BL rats aged 12 or 13 months were randomly divided in to an experimental group and a control group,each of 6.The control group was fed 75.09 kJ/d as normal,while the experimental group was provided with 45.05 kJ/d (60％ of normal).The intervention lasted for 15 weeks and each rat's weight was measured every week.After the intervention,limb muscle satellite cells were sorted by fluorescenceactivated cell sorting after digestion,and the cell cycle was analyzed.Western blotting was used to assess the expression of cyclin A,D1 and E.Results There was no significant difference in the average weight of the two groups before the experiment.After the 15 weeks the average weight of the experimental group had decreased significantly (to 19.5±0.4 g),and it was significantly lighter than that of the control group (31.9±0.5 g).The average percentage of the satellite cells in the G0/G1 phase had decreased significantly in the experimental group,but the percentage in the S phase had increased significantly.The expression of cyclin A and E was significantly greater in the experimental group compared with the control group,but the expression of cyclin D1 had decreased significantly.Conclusion Caloric restriction can delay the proliferation of satellite cells in the skeletal muscles of elderly mice.

Objective To describe the changes of thrombosed venous sinus on MRI after administration of contrast material and evaluate the sensitivity and specificity of thread-like enhancement around sinus to diagnose thrombosis in the corresponding sinus.Methods Patients with cerebral venous sinus thrombosis (CVST) admitted to our department from January 2005 to December 2014 and undergone a MRI scan with administration of contrast material were included in this study.The enhancement features of venous sinus were studied in the plane parallel to the interested sinus.The features of enhancement were classified as peripheral thread-like enhancement, partial enhancement and complete enhancement.The proportion of these three type of enhancement in thrombosed sinuses and normal sinuses were described and compared.The sensitivity and specificity of peripheral thread-like enhancement to diagnose thrombosis in corresponding sinus were calculated.The proportion of each type of enhancement was also described and compared in acute (≤ 7 d), subacute (8-30 d) and chronic (≥ 31 d) stage after onset of symptoms.Results Peripheral thread-like enhancement, partial enhancement and complete enhancement were all found in both thrombosed and normal sinuses.There was a significant difference of enhancement features between normal and thrombosed sinus in superior sagittal sinus (100％ (30/30) vs 60％ (27/45), x2 =13.789, P =0.001), left trans verse sinus, and right sigrnoid sinus.The sensitivity and specificity of peripheral thread-like enhancement to diagnose thrombosis in the corresponding sinus were 10.5％-44.4％ and 53.3％-76.7％ respectively.There was no significant difference of contrast features at different stage after onset.Conclusion The value of peripheral thread-like enhancement to diagnose CVST is limited because of low sensitivity and specificity.