Background Congenital fibrosis of extraocular muscles (CFEOM) affects patient' s appearance and quality of life,and no effective treatment for this disease is available.Imaging study is helpful for exploring the pathogenesis of CFEOM.Objective This study was to describe the characteristics of CFEOM associated with limb movement disorder using magnetic resonance imaging (MRI).Methods A family with CFEOM associated with limb movement disorder was investigated in Renmin Hospital of Wuhan University.Disease history was collected and the pedigree was investigated.Ophthalmologic examinations,including corrected visual acuity,refractive error,slitlamp examination,ophthalmoscopic examination,force of levator palpebrae superioris,ocular movement,eye position,forced duction test,and bell phenomenon examination,were performed.Ocular orbital and cranial MRI was performed in 4 CFEOM patients and 10 normal subjects to compare the structures of the extraocular muscles,motor nerve and cranium.Oral informed consent was obtained from each patient prior to any medical examination.Results A total of 1 1 members from 3 generations were investigated in this study,presenting with 4 cases of disease.The mode of inheritance of this family complied with the Mendelian autosomal dominant inheritance law.Clinical signs included disturbance of eye movement,deviation of eye position,ptosis,lack of Bell sign and positive reaction of passive pull test.In addition,unstable gait,improper body limb alignment,dysphasia and mental retardation were ohserved in 1 patient,which coincided with the diagnostic criteria of type 3 CFEOM.MRI results demonstrated that the levator palpebrae superioris,superior rectus and superior oblique muscle were clearly thinner,and the medial rectus,lateral rectus,inferior rectus muscle were thinning in the patients,showing significant differences in comparison with the normal controls(P＜O.05).The oculomotor and abducens nerves became thinner and even absent in the patients.Cranial MRI showed that Ⅲ-3 in the pedigree with callosum was shorter than that of the normal controls,suggesting that patient suffered from corpus callosum hypoplasia.Meanwhile,cranial MRI indicated the presence of cerebellar hypoplasia and the expansion of the fourth ventricle.Conclusions MRI demonstrates consistent abnormalities of the oculomotor nerves and abducens nerves in the affected individuals in this CFEOM 3 family,and some affected members exhibit two types of central nervous system abnormalities-corpus callosum and cerebellar hypoplasia.These findings suggest that CFEOM 3 is primarily a neuronal disease.

Objective To evaluate myocardial apoptosis with 99Tcm-C2A-GST myocardial imaging using the recombined C2A domain of Synaptotagmin Ⅰ by gene engineering. Methods ( 1 ) The C2A gene was inserted into the prokaryotic glutathione S-transferate (GST) fusion protein expression plasmid pGEX-6P-1. The recombinant plasmid was transformed into E. coli BL21. C2A-GST fusion protein was purified after BL21 was induced with isopropyl-β-D-1-thiogalactopyranoside (IPTG). (2)The activity of fusion protein was identified by cell binding test with fluorescein-5-isothiocyanate (FITC)-C2A-GST. (3) The C2A-GST fusion protein was labeled with 99Tcm using 2-iminothiophene hydrocoride method. Radiochemical purity was determined with thin layer chromatography. (4)99Tcm-C2A-GST (7.4 MBq) was injected to ischemia-reperfusion rat models through tail vein. The image was acquired with SPECT at 1 h after injection, and then hearts were removed, rinsed with saline and dyed with triphenyl tetrazolium coride (TTC). The ischemic myocardium was separated from the viable myocardium and was weighted. Its radioactivity was measured by gamma counting. The difference of uptake of radiotracer between ischemic myocardium and normal myocardium was compared using percentage activity of injected dose per gram of tissue ( % ID/g) with standard deviation. SPSS 12.0 and t-test were used for data analysis. Results ( 1 ) C2A-GST fusion protein wassuccessfully expressed and its relative molecular weight was 3.8 × 104. (2) FITC-C2A-GST binding to apoptotic cells could be observed by fluorescent microscopy. (3) The radiochemical purity of 99Tcm-C2A-GST was (98.90 ±0.43)%. (4)The imaging studies showed that there was focal uptake of radioactivity in the ischemic myocardium. In vitro uptake of 99Tcm-C2A-GST was (2.41 ±0.32) % ID/g by the ischemic myocardium, however 99Tcm-C2A-GST-N-hydroxysuccinimide (C2A-GST-NHS) was (0. 82 ± 0. 24) % ID/g. There was statistically significant difference between those two groups (t = 10. 6, P ＜0.01 ). Conclusion The C2A domain of Synaptotagmin Ⅰ expressed by gene engineering can be used as the tracer for noninvasive detection of ischemic myocardium in the ischemia-reperfusion rat model.

Objective To investigate the association of body mass index(BMI)with metabolic status and chronic complications in newly-diagnosed Chinese type 2 diabetic patients.Methods A total of 515 newly- diagnosed adults type 2 diabetic patients were categorized into underweight(BMI

A computational system for the prediction and classification of human G-protein coupled receptors (GPCRs) has been developed based on the support vector machine (SVM) method and protein sequence information. The feature vectors used to develop the SVM prediction models consist of statistically significant features selected from single amino acid, dipeptide, and tripeptide compositions of protein sequences. Furthermore, the length distribution difference between GPCRsand non-GPCRs has also been exploited to improve the prediction performance.The testing results with annotated human protein sequences demonstrate that this system can get good performance for both prediction and classification of human GPCRs.

Objective To compare the cosmic radiation doses to air crew (including pilots and flight attendants) between polar route and non-polar route flights. Methods A typical polar flight route (Beijing-New York) was selected and compared with non-polar route, non-polar route was selected as control group. Optically stimulated luminescence (OSL) dosimeter and CR-39 solid track detector were used to measure the cosmic radiation dose to air crew. Results The annual mean effective dose to air crew from polar route and non-polar route was (5.79 ± 0.92)mSv/a and (2.14 ± 0.64)mSv/a, respectively.The 1000 h effective dose to air crew was (3.10 ± 0.27)mSv/kh and (2.21 ± 0.46)mSv/kh,respectively. There was significant difference between the two groups in either annual mean effective dose (t=30.25, P＜0.05) or 1000 h effective dose (t =7.60, P＜0.05). The doses of pilot groups were higher than that of flight attendants for either polar route ( t = 7.96, P ＜0.05) or non-polar route (t=4.70, P ＜ 0.05). Conclusions The effective dose to air crew from galactic cosmic radiation during polar route fight might be higher than that of non-polar route fight, however, it did not exceed 20 mSv/year,which is the limit of national standard.

BACKGROUNDCathepsin S and its endogenous inhibitor cystatin C are implicated in the pathogenesis of atherosclerosis, especially in the plaque destabilization and rupture leading to acute coronary syndrome. However, whether circulating cathepsin S and cystatin C also change in association with coronary plaque morphology is unknown yet.

METHODSWe recruited 98 patients with unstable angina (UA, n = 6) or stable angina (SA, n = 2) who had a segmental stenosis resulting in > 20% and < 70% diameter reduction in one major coronary artery on coronary angiography. Thirty-one healthy subjects served as controls. Intravascular ultrasound (IVUS) was used to evaluate plaque morphology. Plasma cathepsin S and cystatin C were measured as well.

RESULTSAt the culprit lesion site, plaque area ((7.85 +/- 2.83) mm(2) vs (6.53 +/- 2.92) mm(2), P = 0.027), plaque burden ((60.92 +/- 11.04)% vs (53.87 +/- 17.52)%, P = 0.025), remodeling index (0.93 +/- 0.16 vs 0.86 +/- 0.10, P = 0.004) and eccentricity index (0.74 +/- 0.17 vs 0.66 +/- 0.21, P = 0.038) were bigger in UA group than in SA group. Plasma cathepsin S and cystatin C were significantly higher in patients than in controls (P < 0.01). Plasma cathepsin S was higher in UA group ((0.411 +/- 0.121) nmol/L) than in SA group ((0.355 +/- 0.099) nmol/L, P = 0.007), so did the plasma cystatin C ((0.95 +/- 0.23) mg/L in UA group, (0.84 +/- 0.22) mg/L in SA group; P = 0.009). Plasma cathepsin S positively correlated with remodeling index (r = 0.402, P = 0.002) and eccentricity index (r = 0.441, P = 0.001), and plasma cystatin C positively correlated with plaque area (r = 0.467, P < 0.001) and plaque burden (r = 0.395, P = 0.003) in UA group but not in SA group.

CONCLUSIONSPlasma cathepsin S and cystatin C increased significantly in UA patients. In angina patients, higher plasma cathepsin S may suggest the presence of vulnerable plaque, and higher plasma cystatin C may be a clue for larger atherosclerotic coronary plaque.

Adult ; Aged ; Aged, 80 and over ; Cathepsins ; blood ; Coronary Artery Disease ; blood ; diagnostic imaging ; pathology ; Cystatin C ; blood ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Ultrasonography, Interventional ; methods

OBJECTIVETo investigate the expression of suppressor of cytokine signaling(SOCS)-3 and caspase-3 and their correlative significance in endometriosis.

METHODSImmunohistochemical EnVision method was used to detect the SOCS-3 and caspase-3 protein expression in ectopic and eutopic endometrium (n = 32) of patients with endometriosis, as well as normal endometrium (n = 30) of women without endometriosis.

RESULTSSOCS-3 and caspase-3 proteins were expressed in all three groups and not affected by the menstrual cycles. The expression of SOCS-3 in ectopic endometrium (5.54 ± 2.12) was significantly lower than that in eutopic (7.39 ± 1.09, P = 0.001) and control group (7.48 ± 1.26, P < 0.01), but without difference between the eutopic and control group (P = 0.756). SOCS-3 expression in ectopic and eutopic endometrium was significantly lower in III/IV stages than that in I/II stages of endometriosis (P < 0.05). Significantly lower expression of caspase-3 protein was found in ectopic (3.20 ± 1.24) and eutopic endometrium (3.88 ± 1.93) as compared with the control group (6.49 ± 1.85, P < 0.01), however ectopic and eutopic endometrium showed no significant difference (t = 1.66, P = 0.10). There was no significant difference of the expression of caspase-3 in ectopic and eutopic endometrium at different disease stages (P > 0.05). Positive correlation was found between the expression of SOCS-3 and caspase-3 proteins in ectopic endometrium (r = 0.655, P < 0.01).

CONCLUSIONSOCS-3 may be involved in the development of endometriosis through inhibition of apoptosis of ectopic endometrial cells.

Adult ; Caspase 3 ; metabolism ; Endometriosis ; metabolism ; pathology ; Endometrium ; metabolism ; pathology ; Female ; Humans ; Immunohistochemistry ; Menstrual Cycle ; Middle Aged ; Suppressor of Cytokine Signaling 3 Protein ; Suppressor of Cytokine Signaling Proteins ; metabolism ; Uterine Diseases ; metabolism ; pathology ; Young Adult

Objective: To evaluate the effectiveness of therapeutic massage (tuina) for treating knee osteoarthritis (KOA). Methods: Six English and Chinese databases, including Chinese National Knowledge Infrastructure (CNKI), Wanfang Academic Journal Full-text Database (Wanfang), Chongqing VIP Database (CQVIP), China Biology Medicine Disc (CBM), Cochrane Library and PubMed databases, were independently searched to identify appropriate randomized controlled trials (RCTs) studying therapeutic massage for KOA compared to oral non-steroidal anti-inflammatory drugs (NSAIDs) alone. The main outcome measures were total effectiveness and the Western Ontario and McMaster Universities osteoarthritis index (WOMAC) score. Results: A total of 8 RCTs were included and they were of average quality. The results showed that therapeutic massage was more effective than NSAIDs comparing total effectiveness [risk ratio (RR)=1.14, 95％ confidence interval (CI) (1.07, 1.21), P<0.0001]; compared with NSAIDs, therapeutic massage produced more significant improvements in pain [mean difference (MD)=-2.06, 95％CI (-2.75, -1.36), P<0.00001], stiffness intensity [MD=-0.90, 95％CI (-1.05, -0.75), P<0.00001] and joint function [MD=-12.48, 95％CI (-13.91, -11.05), P<0.00001]. Conclusion: Therapeutic massage was more effective than oral NSAIDs in treating KOA. In relieving pain and stiffness and improving the function of knee joint, therapeutic massage was superior to NSAIDs.

OBJECTIVETo introduce and evaluate the procedure and the effect of localized management of sinus floor (LMSF), bone graft and simultaneous implant placement with trephine bur in maxillary posterior region.

METHODS24 patients without enough alveolar bone height received LMSF, bone grafting and implants placement were carried out simultaneously.The autogenous bone were harvested by trephine bur in situ. Patients were followed up after 1 week, 1, 3 and 6 month.

RESULTSThere was no implant loose or lost and maxillary antritis. 6 months postoperatively, bone graft reformed to new bone seen in X-ray films, sinus floors were augmented and reached the requirements of dental implants. The implant osseointegrated tightly with new bone which was satisfactory to second-step prosthesis after implant placement of 6 months later.

CONCLUSIONSThe method enlarges the indication of dental implants and avoids operation of harvesting autogenous bone in other site. It is simple and valuable to clinical application.

Adult ; Alveolar Ridge Augmentation ; methods ; Bone Transplantation ; Dental Implantation, Endosseous ; methods ; Dental Implants ; Female ; Humans ; Male ; Maxillary Sinus ; surgery ; Middle Aged

OBJECTIVETo study the proteomics of Pinellia pedatisecta Schott total protein on human ovarian cancer SKOV3 cells.

METHODSSKOV3 cells were in vitro cultured. The growth inhibition of SKOV3 cells in the logarithmic phase with different concentrations of Pinellia pedatisecta Schott (0, 0.05, 0.10, 0.20, 0.30, 0.40, and 0.50 mg/mL) at different time points (24, 48, 72, and 96 h) was analyzed by CCK-8 colorimetry. The total protein was extracted after adding 0.296 mg/mL Pinellia pedatisecta Schott protein for 48 h. The protein with differential expressions was screened out using two-dimensional gel electrophoresis (2-DE) and matrix-assisted laser desorption ionization time of flight mass spectrometry (MALDI-TOF-MS).

RESULTSThe protein of Pinellia pedatisecta Schott (at 0.10 - 0.50 mg/mL) could significantly inhibit the growth of SKOV3 cells in a time- and dose-dependent manner (P < 0.05, P < 0.01). After analyzed by 2-DE and MALDI-TOF-MS, 43 differential protein dots were successfully identified (21 up-regulated and 22 down-regulated), including alpha-enolase 1, eukaryotic initiation factor 3alpha, cyclophilin B, and so on.

CONCLUSIONSProtein of Pinellia pedatisecta Schott could significantly inhibit the growth of SKOV3 cells, and lead to the proteomics changes of SKOV3 cell strain. They might be correlated with its anti-tumor mechanisms.

Cell Line, Tumor ; Female ; Humans ; Ovarian Neoplasms ; metabolism ; Pinellia ; Plant Proteins ; pharmacology ; Proteomics ; methods