Adolescent ; Adult ; Female ; Humans ; Male ; Niemann-Pick Diseases ; genetics ; Pedigree

Argentina ; epidemiology ; China ; epidemiology ; Cross-Sectional Studies ; Hemophilia A ; diagnosis ; epidemiology ; therapy ; Humans ; Prognosis ; Russia ; epidemiology ; Surveys and Questionnaires ; United States ; epidemiology ; Urban Population

Animals ; Cochlea ; growth & development ; metabolism ; Female ; Guinea Pigs ; Male ; Nerve Growth Factor ; metabolism

Objective To discuss application of evidence-based nursing in nasopharyngeal carcinoma patients undergoing radiotherapy. Methods Evidence-based nursing was used in 33 nasopharyngeal carcinoma patients undergoing radiotherapy. Results After evidence-based nursing, all the patients actively cooperated with the doctors and nurses, lived through the treatment process smoothly, and radiotherapy complications were reduced or avoided. Conclusions Evidence-based nursing is an idealized nursing method which could reduce or avoid the complications and alleviate the pain of patients and improve the quality of nursing.

Tripterygium glycosides preparation which extracted from the traditional Chinese herb Tripterygium wilfordii (TWHY), was widely used to treat the autoimmune diseases. Previous works demonstrated that TWHF had potent anti-inflammatory and immunosuppressive properties. But the different quality and high incident rate of side effects of different manufactures inhibited its clinical application. Since TWHF had been generally known to play a therapeutical effect by synergism of multiple constituents, it was necessary to build the relationship between the HPLC fingerprint and bioactivity so as to ensure the quality safety and efficacy. The HPLC fingerprint showed that description and content of peaks from different manufactures were diverse. Only 11 common peaks were found. In this study, mice spleen cells stimulated by Con A were used to test the proliferation inhibition bioactivity of TWHF preparations, which were incubated with 30, 15, 7.5, 3.75, 1.88 and 0.94 mg x L(-1) TWHF preparations for 48 h. The results showed that mice spleen cells proliferation was inhibited by all TWHF preparations significantly compared with the control group, which suggested the TWHF preparations showed immune suppress activity. The TWHF preparations from 7 manufacture showed different IC50 value, which might belong to different contents which showed in the HPLC fingerprint. Moreover, a relationship between the HPLC fingerprint and the bioactivity were established to identify important constituents by grey relational analysis (GRA). The result showed that all the contents were relative with the IC50, especially No. 5 and 10 peaks, but No. 1 peak, which was proved to be triptolide, had few contribute to the inhibition of mice spleen cells proliferation. The study of relationship between the HPLC fingerprint and the IC50 by GRA could help to investigate mechanism of bioactive and provide an evidence for the quantification of multi-constituents.
Animals ; Anti-Inflammatory Agents ; analysis ; pharmacology ; Cell Proliferation ; drug effects ; Chromatography, High Pressure Liquid ; Drugs, Chinese Herbal ; analysis ; pharmacology ; Glycosides ; analysis ; pharmacology ; Male ; Mice ; Mice, Inbred BALB C ; Spleen ; cytology ; drug effects ; Tripterygium ; chemistry

ObjectiveTo determine the peripheral serum expressions and clinical value of carcinoembryonic antigen (CEA) and carbohydrate antigen CA19-9,CA242,CA72-4 and a single sialic acid ganglioside (CA50) antigen in advanced pancreatic cancer patients treated by three-dimensional conformal radiotherapy (3D-CRT) combined with endogenous field hyperthermia.Methods60 patients inoperable,advanced pancreatic cancer were treated by 3D-CRT combined with endogenous field hyperthermia.Radiation dose (DT) was 46 ～ 66 GY/23 ～ 33 F.Hyperthermia temperature was 41 ～ 43 ℃ 2 times/week,lasted 60 min each time.The expressions of CEA,CA19-9,CA242,CA72-4 and CA50 in peripheral serum were detected by Electrochemiluminescence immunoassay (ECLIA) every 2 weeks during treatment,and correlation were analyzed with tumor diameter,clinical stage,lymph node metastasis.Results From the 8th week,with 3D-CRT combined with endogenous field hyperthermia ongoing,CEA,CA19-9,CA50 andCA242 expression levels showed a gradual decline.CEA,CA19-9,CA50 and CA242 expression levels were (6.22 ± 2.71 ) μg/L,(43.44 ± 12.93 ) μg/L,(23.21 ± 7.71 )g/L and (24.26 ± 8.92) μg/L in 6 weeks,respectively.The difference was statistically significant among week 6 and week 0,week2,week 4 ( P ＜0.05),however there was no significant difference between week 6 and week 8 ( P ＞ 0.05 ).The CA724 expression did not change significantly ( P ＞ 0.05 ).There were positively correlation between CEA,CA199,CA50 and CA242 with pancreatic cancer tumor size,clinical stage and lymph node metastasis( respectively r =0.877,0.725,0.826,all P ＜ 0.01 ),however CA72-4 had no correlations ( P ＞ 0.05).ConclusionsCEA,CA19-9,CA50 and CA242 expressions in peripheral serum of pancreatic cancer patients were closely related to the treatment.Their joint detection can be served as independent objective evaluation reference information for the treatment efficacy and prognosis.

Objective To investigate the clinical,genetic and neuroimaging features by reporting a family with dyssynergia cerebellaris myoclonica. Methods The proband was examined clinically by neuroimaging,electromyography ( EEG),skin and muscles pathology and hematology.The patients with the illness in the family were followed up and the pedigree was drawn.Results There were 6 patients with dyssynergia cerebellaris myoclonica of the 27 family members in the family.All patients had disproportionate myoclonus,epilepsy,progressive cerebellar ataxia performance. Proband brain MRI showed cerebral atrophy.Cerebellar and cortical atrophy were more serious than other parts.There were long T,and long T2 signals in the white matter,high signal in T2FLAIR.EEG showed bursts of spike-low wave,polyspilke-low waves and polyspike waves distributing in the whole brain.Pathology of the skin and muscles was normal.Conclusions Dyssynergia cerebellaris myoclonica is an autosomal dominant disease,characterised by myoclonus,progressive cerebellar ataxia and epilepsy.Brain MRI shows cerebral cortical and cerebellar atrophy,abnormal signal in white matter.EEG showes spike and ware wave.The diagnosis is mainly based on family history,typical clinical manifestations,brain MRI and EEG changes.

Objective To evaluate the clinical efficacy of three-dimensional conformal radiotherapy (3D-CRT) combined with thermochemotherapy in the treatment of locally advanced pancreatic cancer (LAPC).Methods From June 2008 to June 2011,70 patients with LAPC were divided into radiotherapy group (30 patients) and combination group (40 patients).Radiotherapy used 3D-CRT with a 90％ to 95％ isodose curve,a single dose of 1.8 to 2.OGy,and total radiation dose 50 ～ 70 Gy.The combination group patients received simultaneous thermotherapy at 41.5 ～43.5 ℃ (1 h/fraction,twice a week for 6 times),and hyperthermia given simultaneously injected using arsenic trioxide 20 mg,recombinant mutant human tumor necrosis factor(rmhTNF) intravenous infusion of 10 million U,4 to 6 times,or 3D-CRT at the same time and the treatment given after gemcitabine(0.6 ～ 1.0 g/m2) on Days dl and 8 and cisplatin (DDP) (20 ～ 30 mg/m2) on Days d1-3 intravenous infusion,repeated every 28 days for 3 ～ 6 cycles.Results At 3 months after treatment,the total response (complete remission and partial remission) rate was 70.0％ (49/70),the efficiency of radiotherapy combined with chemotherapy,and radiotherapy combined with thermo-chemotherapy were 56.5％ and 88.2％,and the radiotherapy alone group was 56.7％.There were significant difference in efficiency between radiotherapy combined with thermo-chemotherapy group compared to radiotherapy-chemotherapy group and radiotherapy group (x2 =4.68,4.98,P ＜ 0.05),the last two groups showed no significant difference (P ＞ 0.05).The 1-year and 2-year survival rate was 46.8％ and 20.3％,respectively.The 1-year and 2-year survival rates were 52.4％ and 26.7％ in combination group,and 42.5％ and 16.2％ in radiotherapy group (x2 =14.17,P ＜ 0.05 ; x2 =9.74,P ＜ 0.05).No serious complications such as perforation,bleeding,and high fever were seen during treatment and follow-up.Conclusions 3D-CRT combined with thermochemotherapy is well tolerated and is relatively effective for the LAPC patients.

In this paper , we discussed the financing mechanisms of premarital healthcare services from their properties combined with the status quo financing in our country .We then provided the scientific basis and decision-making reference for improving financing mechanism to improve the premarital healthcare system .A literature or doc-umentary and field researches have combed the present situation of the premarital healthcare services financing mech -anisms at home and abroad .Related data were collected in eight research areas , namely Fujian, Guangxi, Yunnan, Hubei, Jiangsu, Heilongjiang, Beijing and Tianjin and the related personnel from health administrative department , civil affairs departments , maternal and child healthcare institutions were interviewed in terms of Field Research .From the main content at present , premarital healthcare services belong to the category of quasi-public goods .We dis-cussed the financing mechanisms from the financing levels , sources of funds , fund allocation , payment and financial regulatory and other aspects .Finally, some policy recommendations were put forward including making clear the na-ture of premarital healthcare service property , establishment of a special financial investment mechanism , unify the basic financing service standard after scientific feasibility studies , timely and fully allocate special premarital health-care service funds to strengthen the fund supervision policy recommendations .

Based on the policy framework , the premarital healthcare policy of Guangxi was analyzed from four aspects including policy background , process, content and actors.We analyzed the factors influencing this policy and their interactions , and discussed the important impact of Thalassemia prevention , multi-sectoral consensus and appro-priate premarital healthcare measures to the policy building and implementation .Then the lessons such as strengthe-ning the multi-sector cooperation , specifically making clear the pre-marital screening projects and implementing the“one-stop” service model were summed up in Guangxi province .Finally, combined with existing major problems , some policy recommendations were put forward including clarify the funding criteria and strengthen the premarital healthcare publicity in order to promote the sustainable development of premarital healthcare services .