Dystonia is a common movement disorder characterized by abnormal gestures and involuntary movement as a result of incoordinate contraction of agonistic and antagonistic muscles.The underlying pathogenesis is very complicated and there are various clinical manifestations.The recent findings in genetics and clinical features of primary dystonia,dystonia-plus syndrome and heredodegenerative dystonia are introduced in this paper.

Objective The clinical manifestations of Parkinson''s disease (PD) are divided into non-motor symptoms and motor symptoms.Constipation is one of the common symptoms of non-motor symptoms of the disease.Constipation symptoms in PD generally occur earlier for 5~20 years, while drug treatment for PD constipation can lead to gastrointestinal damage.This study investigated the improvement in the treatment of anorectal dynamics and constipation symptoms in Parkinson''s disease by oral mosapride and press-needle therapy of Shu-Mu point.Methods 60 patients diagnosed as constipation of Parkinson''s disease were randomly divided into control group and treatment group, 30 cases in each group;patients in the control group were treated with oral mosapride tablets, while patients in the treatment group received press-needle therapy, three times per week.The observation course in both groups last for three months and follow-up visits were conducted before and after the course.Efficacy evaluation, PAC-QOL scale and anorectal dynamic examination were applied in the follow-up visit.Results The efficiency of treatment group was 81.7%, significantly higher than control group (70%), representing significant difference between the two groups (P<0.05).Both the methods could improve the PAC-QOL scale, and significant difference was found in comparison to that before the treatment(P<0.01), while no statistical significance was found between treatment group and control group after treatment(P>0.05).As to the anorectal dynamics, significant difference was found before and after the treatment in both groups (P<0.05, P<0.01), while no statistical significance were found between the two groups after treatment(P>0.05).Conclusion Both oral mosapride and press-needle can improve the quality of life and anorectal dynamics for PD patients, representing no significant difference.However, compared with mosapride, the overall efficacy of press-needle is better in improving the symptoms of constipation in PD, featuring more convenience, higher safety, less pain and no side effect, and it is worthy of clinical application.

Objective To evaluate the relationship between Multi-slices spiral(MSCT) perfusion and microvessel density (MVD) in maxillofacial tumors. Methods Thirty-one cases of maxillofacial tumors were studied with MSCT perfusion imaging before operation. The time-density curve, perfusion, time to peak(TTP), and Peak enhancement imaging (PEI) of tumors were calculated. MVD of the tumors was measured with immuno-histochemical method by means of detecting factor Ⅷ in all the histologic specimens. Relativity analysis was carried between MSCT perfusion imaging parameters, perfusion curve types and MVD. Results MVD of maxillofacial tumors were higher than normal tissue. MVD remarkably correlated with malignancy of the tumors. Perfusion and time to peak (TTP) correlated well with MVD(t=7.09,4.10, P0.05). Significant difference of MVD in three types of perfusion curve was found(F=8.09,P

OBJECTIVETo evaluate the possibility of applying oligonucleotide microarrays for detection of the fusion genes in childhood acute lymphoblastic leukemia (ALL).

METHODSTo detect five types of fusion genes emerging frequently in childhood ALL including TEL/AML1, E2A/PBX1, BCR/ABLp190, BCR/ABLp210, MLL/AF4, probes were designed, synthesized and spotted on the chemical-material-coated-glass plates in array. The total RNAs were extracted from patients' bone marrow or peripheral blood cells at the beginning of diagnosis, analyzed by multiplex nested reverse-transcription-polymerase chain reaction (RT-PCR), and labeled by fluorescein. The products of RT-PCR were hybridized with microarray in order to detect specific types of fusion genes in leukemia cells.

RESULTSDistinctive hybridization signals were obtained for internal positive control and specific types of fusion genes. TEL/AML1 gene was found positive in 2 of the 36 cases, E2A/PBX1 gene in 3, BCR/ABLp190 gene in 2, BCR/ ABLp210 gene in one, and MLL/ AF4 gene in one. The results of the microarray and RT-PCR were consistent.

CONCLUSIONThe microarray-based assay could screen 5 types of chromosome structural aberrations and the splice variants at the same time. It can provide reliable and helpful information for patient stratification, evaluation of therapeutic effects and prediction of prognosis in childhood ALL, although there are both advantages and disadvantages in applying this new method.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Oligonucleotide Array Sequence Analysis ; Oncogene Proteins, Fusion ; genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; Reverse Transcriptase Polymerase Chain Reaction

OBJECTIVEDietary factors that could potentially be related to the risk of birth defects are still unknown in Shanxi province, a region with high prevalence of neural tube defects(NTDs) in China. The main aim of this study was to evaluate the association between NTDs and local dietary factors.

METHODSA population-based case-control study was conducted during 2003-2005 in four selected counties of Shanxi province with 363 NTDs cases ascertained and 523 control babies without any external birth defects.

RESULTSBy multivariate logistic regression analysis, we found the NTDs risk was significantly associated with low consumption of meat and legume, high consumption of pickled vegetables, noncentralized supply of drinking water and drinking tea after adjusting for maternal education level and birth defects history. A significant dose response trend was found between the NTDs risk and the lower frequencies of meat and legume consumption and higher frequency of pickled vegetable consumption, with population attributable risks (PAR) of 69.4%, 22.0% and 18.5%, respectively. Mothers who got their drinking water from river or pond during periconceptional period were more likely to have a NTD-affected pregnancy [OR = 3.42, 95% CI: 1.44-8.09, population attributable risk (PAR) = 4.7%] compared with those who utilized tap or well water. Those who drank tea everyday had an elevated risk of having a NTD-affected pregnancy compared with those who didn't (OR = 4.65, 95% CI: 1.41-15.36, PAR = 2.6%).

CONCLUSIONHigher intake of meat and legume protects a woman from having a NTD-affected pregnancy, and consumption of pickled vegetables, drinking water from river or pond and drinking tea everyday increase the risk of having such a pregnancy in the study population.

Case-Control Studies ; China ; epidemiology ; Diet ; Female ; Humans ; Meat ; Neural Tube Defects ; epidemiology ; Pregnancy ; Risk Factors ; Vegetables ; Water Supply

OBJECTIVETo study the chemical constituents from the leaves of Morus multicaulis.

METHODThe compounds were isolated by ion exchange resin, silica gel and Sephadex LH -20 column chromatographies. Their structures were elucidated on the basis of physico-chemical properties and spectral data.

RESULTTwelve compounds were isolated from the leaves of this plant. Their structures were identified as 1-deoxynojirimycin (1), fagomine (2), 2-O-alpha-D-galactopyranosyl-1-deoxynojirimycin (3), quercetin-7-O-beta-D-glucoside (4), kaempferol (5), quercetin (6), scopoletin (7), D-aspartic acid (8), L-proline (9), D-alpha-alanine (10), myo-inositol (11) and dausterol (12).

CONCLUSIONAll compounds were isolated from this plant for the first time.

1-Deoxynojirimycin ; analysis ; chemistry ; isolation & purification ; Imino Pyranoses ; analysis ; chemistry ; isolation & purification ; Kaempferols ; analysis ; chemistry ; isolation & purification ; Morus ; chemistry ; Piperidines ; analysis ; chemistry ; isolation & purification ; Plant Leaves ; chemistry ; Plants, Medicinal ; chemistry ; Spectrometry, Mass, Electrospray Ionization

OBJECTIVETo investigate the effects of taurine on myocardial mitochondria and their enzyme activities in rats with severe burn.

METHODSOne hundred and twenty healthy adult Wistar rats were subjected to 30% TBSA full-thickness burn. They were randomly divided into burn group (B, with intraperitoneal injection of isotonic saline), treatment group (T, with intraperitoneal injection of taurine, 200 mg/kg),with 60 rats in each group . Ten rats with sham scald were used as control (S group). The myocardial tissue samples in B and T groups were harvested at 1, 3, 6, 12, 24 and 48 postburn hours (PBH) for determination of activity respectively of succinate dehydrogenase (SDH), cytochrome oxidase (CCO), the superoxide dismutase (SOD), Ca2+ -ATPase in mitochondria, and contents of cytochrome c (Cyt c), cytochrome aa3 (Cyt aa3), malondialdehyde (MDA), and Ca2+ in mitochondria and cytoplasm . The myocardial tissue samples of controls were harvested at 1 PBH for determination of above indices.

RESULTSThe activity of CCO in B group was decreased at 1 PBH , especially at 6 ,12 PBH. The activity of SDH in B group was decreased to lowest level at 6 PBH, and its value was lower than that of S group at each time point. The activity of CCO or SDH in T group was not obviously decreased, and the activity of CCO at 3, 6, 12 PBH showed significant difference compared with B group (P < 0.05). The contents of Cyt aa3 and Cyt c in B group at 3, 6, 12, 24 PBH were obviously decreased, which were significantly lower than those in T group (P < 0.05). The activity of SOD in B group at 3, 6, 12 PBH was obviously decreased, the activity of Ca2+ -ATPase at 3, 6, 12 and 24 PBH was decreased to different extent, which was significantly lower than those in T group (P < 0.05). The MDA contents in B and T groups were higher than that in S group at 3-48 PBH ,and it was highest in B group (P < 0.05). The Ca2+ content of mitochondria in B group at 1 PBH was increased (13.7 +/- 1.5), and it was (24.8 +/- 2.6), (29.7 +/- 3.1), (16.3 +/- 1.9) and (13.5 +/- 1.7) at 3, 6, 12, 24 PBH respectively,and they were all higher than that of S group (10.7 +/- 1.6, P < 0.05). The Ca2 contents of cytoplasm in group B at 3 - 24 PBH were also higher than that in S group (P < 0.05). The Ca2+ content of mitochondria in T group at 3, 6, 12, 24 PBH was (16.8 +/- 2.8), (18.7 +/- 1.9), (10.5 +/- 1.8) and (13.3 +/- 1.7)respectively, which were lower than that in B group at every time point.

CONCLUSIONTaurine have protective effect on mitochondria and their enzyme activities in myocardium in rats with severe burn, and it may be attributable to improving the ability of eradicating oxygen free radicals and alleviating Ca2+ overload in the mitochondria.

Animals ; Burns ; drug therapy ; enzymology ; metabolism ; Calcium ; metabolism ; Calcium-Transporting ATPases ; metabolism ; Cytochromes c ; metabolism ; Electron Transport Complex IV ; metabolism ; Female ; Male ; Mitochondria, Heart ; enzymology ; metabolism ; Random Allocation ; Rats ; Rats, Wistar ; Succinate Dehydrogenase ; metabolism ; Superoxide Dismutase ; metabolism ; Taurine ; therapeutic use

BACKGROUNDTo evaluate three new chemotherapeutic regimens for non-small cell lung cancer (NSCLC) by pharmacoeconomic analysis in guiding rational use of drugs.

METHODSOne hundred and one cases of NSCLC in clinic stage III or IV were treated by one of the three chemotherapeutic schemes-PC: paclitaxel (135mg/m²,d1)+DDP; TC: docetetaxel (75mg/m²,d1)+DDP; VC: vinorelbine (25mg/m²,d1 and d8)+DDP, DDP were given at 80mg/m² in 3 groups. Pharmacoeconomic cost-effectiveness analysis was used to compare the efficacy of the three regimens.

RESULTSThe response rate was 46.9%, 48.6% and 47.1% and median survival duration was 7.8, 7.5 and 7.6 months for PC, TC and VC regimen respectively, with 1-year survival rate of 37.5%, 37.1% and 38.2% respectively. There was remarkable difference in the response rate and median survival duration between PC and TC, but no statistical difference was observed between PC and VC. There was no statistical difference in 1-year survival rate among the three regimens. The average cost of one patient for one therapeutic cycle was RMB 15840.5, 15831.1 and 9401.8 Yuan respectively. Escalation of 1% of response rate costed RMB 337.75, 325.74 and 199.61 Yuan respectively. Prolongation of 1 month of median survival duration costed RMB 2030.83, 2110.97 and 1237.08 Yuan respectively. Escalation of 1% of one year survival rate costed RMB 422.41 , 426.71 and 246.12 Yuan respectively.

CONCLUSIONSAmong these three new chemotherapeutic regimens for the advanced patients with NSCLC, the expenditure of VC is much cheaper than PC and TC. The cost effectiveness of VC is the lowest among the three regimens.

Objective To investigate the frequency of CD4~+CD25~+FoxP3~+regulatory T cells (Treg)and the expression of the functional protein,FoxP3,in patients with active tuberculosis and the relationship between Treg and the pathogenesis of tuberculosis.Methods Forty-five patients with active tuberculosis(including 25 cases of pulmonary tuberculosis and 20 tuberculous lymphadenitis), 20 healthy controls,20 recovered tuberculosis patients and 6 patients with reactive hyperplasia in cer- vical lymph node were enrolled.The frequency of CD4~+ CD25~+ FoxP3~+ Treg in the peripheral blood was measured by flow cytometry.FoxP3 mRNA expression was determined by real-time reverse transcriptase-polymerase chain reaction(RT-PCR)and the expression of FoxP3 protein in lymphoid tissues was measured by immunohistochemistry.Results The frequency of natural Treg in the peripheral blood from the patients with active tuberculosis was 2.91%?0.23%,which was signifi- cantly higher than that of healthy control group(1.22%?0.18%)and recovered tuberculosis patients(1.50%?0.17%,P

OBJECTIVETo investigate the effect of different treatment options for the flat type of sudden hearing loss.

METHODSProspective, multi-center clinical study was carried out using internationally used standardized clinical research method. Patients with the flat type of sudden hearing loss between 18 and 65 years old, within two weeks duration, and without any medical treatment were recruited. Treatment options were randomly selected according to the designed random table.

RESULTSFrom August 2007 to October 2011, 402 patients with the flat type of sudden hearing loss who met the criteria (account for 39.26% of the total number of patients) from the 33 hospitals were collected; the total effective rate was 82.59%, and no significant difference was detected between different treatments, (χ(2) = 10.95, P = 0.28). In the 402 cases, 139 were cured (34.58%); 118 were markedly improved (29.35%); 75 were effective (18.66%); 70 were invalid (17.41%).

CONCLUSIONSThe therapeutic efficacy of flat type of sudden hearing loss overall is good; the treatment of improving the inner ear blood rheology and/or reducing blood fibrinogen has clinical significance; the therapeutic efficacy of using glucocorticoid systemically is good as well; there is no obvious difference between combination and single medication.

Adult ; Aged ; Ear, Inner ; Glucocorticoids ; therapeutic use ; Hearing Loss, Sudden ; therapy ; Humans ; Middle Aged ; Prospective Studies ; Treatment Outcome ; Young Adult