Objective To investigate the clinical and skeletal muscular pathological features of chronic progressive external ophthalmoplegia(CPEO).Methods The clinical and skeletal muscular pathological data of 6 patients with CPEO were analyzed retrospectively.Results All of the 6 patients onset were 4 to 18 years old.The first symptom was blepharoptosis.The clinical mainly manifestations were limited activity of eyeball,accompanied with limbs muscle fatigue,weakness and level elevatation of serum creatine kinase(CK).5 cases had myogenic changes in electromyography(EMG).The skeletal muscular pathological examination showed that the ragged red fibers(RRF)were scattered among the muscle fibers,and the deficient or absent of COX activity in many fibers.A few degenerating and necrotic fibers were observed in 4 cases.Lipid-drops were increased by oil red "O" stain in 3 cases.Electromicroscope showed that abnormal mitochondria increased in number and aggregated under sarcolemma or in cytoplasm.Crystalloid inclusion body could be observed.Conclusions The clinical mainly features of CPEO are external ophthalmoplegia and accompanied with limbs weakness.RRF and COX activity deficiency or absence according to skeletal muscle pathology can be suggest and support the diagnosis of CPEO.Gene sequencing based on skeletal muscle biopsy is necessary to make the final diagnosis of CPEO.

Objective:To study the effects of BMP-7 gene transfected bone marrow mesenchymal stem cells(MSCs)on callus acceleration in rat mandibular distraction osteogenesis.Methods:Forty-eight adult male SD rats were randomly divided into experimental and control groups.MSCs were obtained from individual rat and transfected by pEGF-P-BMP7 for the 24 experimental rats and by the empty vector pEGFP-N1 for the 24 control rats.The rats were underwent right mandibular distraction.1?106/100 ?l BMP-7 gene transfected cells in 10 ?l of normal saline were injected into the distraction gap in each experimental rat,while the same number of empty vector transfected cells in each control rat.The distracted mandibles were harvested 2,4,and 8 weeks respectively after cell injection and evaluated by radiological,histological and histomorphometric analysis.Results:Radiological and histological examinations showed that more new bone was formed in experimental group than in control.Histomorphometric analysis also demonstrated that both new bone volume(NBV1 and NBV2)and the thickness of new trabeculae(TNT)were significantly higher in experimental rats than those in control(P

Objective To explore the efficacy and prognostic factors of postoperative intensity?modulated radiotherapy ( IMRT) with or without chemotherapy in rectal cancer. Methods A retrospective analysis was performed on the clinical data of 218 patients with rectal cancer, who underwent postoperative IMRT in our hospital from January 2009 to December 2013. The Kaplan?Meier method was used to calculate survival rate;the log?rank test was used for survival difference analysis and univariate prognostic analysis;the Cox regression model was used for multivariate prognostic analysis. Results The follow?up rate was 97. 7%. The 1?and 3?year overall survival rates were 90. 8% and 75. 2%, respectively, the 1?and 3?year disease?free survival rates were 85. 3% and 70. 5%, respectively, and the 1?and 3?year locoregional recurrence?free survival rates were 96. 7% and 88. 1%, respectively. The incidence of grade 3?4 acute adverse reactions was 28. 4%, mainly manifested as leukopenia ( 13. 8%) and diarrhea ( 11. 0%) . Univariate prognostic analysis showed that preoperative carcinoembryonic antigen ( CEA) and CA199 levels, maximum tumor diameter, tumor location, degree of differentiation, depth of tumor invasion, number of lymph node metastases, TNM stage, perineural invasion, surgical procedure, total mesorectal excision, preoperative bowel obstruction, and preoperative anemia were the predictors of survival ( P=0. 006, 0. 000, 0. 000, 0. 017, 0. 000, 0. 016, 0. 000,0. 011,0. 001,0. 006,0. 037 and 0. 010) . Multivariate prognostic analysis showed that preoperative CEA level, tumor location, TNM stage, preoperative bowel obstruction, and preoperative anemia were the predictors of survival ( P=0. 000,0. 000,0. 000,0. 001 and 0. 001) . Conclusions Postoperative IMRT with or without chemotherapy is an effective method for rectal cancer with mild adverse reactions and high compliance. Preoperative CEA level, tumor position, TNM stage, preoperative bowel obstruction, and preoperative anemia are independent prognostic factors for the overall survival.

BACKGROUND:Preliminary studies of our research group have confirmed that the proliferation of preosteoclasts and the differentiation and function of osteoclasts could be inhibited when they were cultured in lower oxygen tension even hypoxia (2%O 2 ), but the gene expression of osteoclasts cultured in vitro have not been reported.
OBJECTIVE:To examine the effect of oxygen tension on specific gene expression of osteoclasts in vitro and explore the mechanism of osteoclast differentiation influenced by oxygen tension.
METHODS:The preosteoclasts were induced with 10μg/L macrophage colony stimulating facto and 10μg/L soluble receptor activator of nuclear factor-κB ligand into mature osteoclasts. Then the osteoclasts were cultured in normoxia, tissue oxygen and hypoxia (20%, 7%, 2%O 2 ) respectively. cells were then stained for tartarate-resistant acid phosphatase to assess osteoclastic formation. cells were col ected at 1, 2, 3, 4, 5, 6, 7 days after culture respectively. The soluble receptor activator of nuclear factor-κB ligand, tumor necrosis factor receptor-associated factor 6, tartarate-resistant acid phosphatase, and cathepsin K mRNA expression levels were determined using real-time quantitative PCR.
RESULTS AND CONCLUSION:The number of osteoclasts positive for tartarate-resistant acid phosphatase in the hypoxia was significantly lower than that in the tissue oxygen and normoxia (P<0.05). Under different oxygen tension, the mRNA expression levels of soluble receptor activator of nuclear factor-κB ligand in osteoclasts maintained unchanged. The mRNA expression levels of tumor necrosis factor receptor-associated factor 6 reached the peak at 5 days after culture in tissue oxygen and normoxia (P<0.05). The mRNA expression time of tartarate-resistant acid phosphatase and Cathepsin K were delayed accompanied by decreased oxygen tension, but the maximum were maintained in tissue oxygen. Compared with normoxia and hypoxia, osteoclasts cultured in tissue oxygen are more prone to differentiate and maintain the activity and functions.

Objective To investigate the proliferative inhibition of lens epithelial cell (LEC) by arsenic trioxide (ATO) and rhizoma curcumae (RC), in order to provide scientific basis for pursuing safe and effective natural drugs to prevent and cure after cataract. Design Experimental study. Participants Cultured Bovine LEC in vitro. Methods Changes of cellular form were observed with microscope. Different concentration of ATO (2.5, 5, 10?mol/L) and RC(5, 10, 20mg/ml) were added to the proliferative LEC separately. The effects of proliferative inhibition by ATO and RC on the LEC were measured with methyl thia-zolyl tetrazolium (MTT) assay method after 72 hours incubation. Main Outcome Measures Cellular form, Absorbance. Results Under the microscope, good growth and quantity increase were found in proliferative control group. Slowing proliferation,poor growth, and few disintegration were observed in ATO group and RC group. MTT assay: Different concentration of ATO (2.5, 5, 10?mol/L)and RC(5, 10, 20mg/ml) can significantly inhibit the proliferation of LEC and these effects were dose dependent(P

Antihypertensive Agents ; therapeutic use ; Endothelin Receptor Antagonists ; therapeutic use ; Familial Primary Pulmonary Hypertension ; Humans ; Hypertension ; drug therapy ; Hypertension, Pulmonary ; drug therapy

Objective To investigate the technique and application of nasolacrimal duct imaging using MR hydrography.Methods Eight healthy volunteers(16 lacrimal ducts)and 17 patients affected by primary epiphora(32 lacrimal ducts)underwent MRl with three.dimensional fast recovery fast spin echo (3D-FRFSE)MR dacryocystography(MRD)sequence after sterile saline solution had been instilled into the conjunctival sac.For all patients affected by primary epiphora,FRFSE T2-weighted oblique coronal and axial images were obtained after MRD.All patients(32 lacrimal ducts)underwem lacrimal endoscopy.which served as a standard of reference for confirming MR findings.Results Eight cases of 16 normal lacrimal passages were showed by MR hydrography with administering topical sterile saline solution,which demonstrated the lacrimal sac well and whole course of the nasolacrimal duct.Endoscopic findings confirmed nasolacrimal duct obstruction secondary to chronic non-specific inflammation:the color of the mucosa of the nasolaerimal ducts was grey-red,and the obstructive sinuses were filled with nonelastic grey-white membrane.The accuracy of 3D-FRFSE MRD sequence in diagnosing obstructive level was 78%(25/32). The lacrimal ducts above the obstructive level showed watery hypo-intensity on 3D-FRFSE MRD.and the lacrimal ducts below the obstructive level could not be showed.Abnormal findings were presented in all cases of obstructive nasolacrimal ducts with Axi-FRFSET2 WI and Cor-FRFSET,WI sequences:long T2 fluid signals were seen in the lumens of tlle lacrimal sac and(or)nasolacrimal duct above the obstructive level. equal or slightly long T2 soft-tissue signals were seen in the lumens of the nasolaerimal duct below the obstructive level.and the mucosa of the ducts thickened Conclusion MR imaging performed after the topical administration of sterile saline solution can reveal normal nasolacrimal duct and is feasible in evaluating obstructive nasolacrimal ducts.

AIMTo study the electrochemical behavior of daunorubicin at Co/GC ion implantation modified electrode.

METHODSWith Co/GC ion implantation modified electrode as working electrode, daunorubicin was determined by voltammetry in 0.05 mol x L(-1) Na2HPO4-KH2PO4 (pH 6.82) solution.

RESULTSA sensitive reductive peak of daunorubicin was obtained by linear sweep voltammetry. The peak potential was -0.60 V (vs SCE). The peak current was proportional to the concentration of daunorubicin over the range of 2.84 x 10(-8) - 1.42 x 10(-6) mol x L(-1) and 1.42 x 10(-6) - 1.28 x 10(-5) mol x L(-1) with the detection limit of 1.42 x 10(-8) mol x L(-1). The reduction wave was applied to the determination of daunorubicin. The electrochemical behavior and reaction mechanism were studied by linear sweep and cyclic voltammetry.

CONCLUSIONThe reduction process was quasi-reversible with adsorption characteristics.

Antibiotics, Antineoplastic ; analysis ; chemistry ; Carbon ; Cobalt ; Daunorubicin ; analysis ; chemistry ; Electrochemistry ; Electrodes ; Hydrogen-Ion Concentration

Objective To analyze the clinical and laboratory phenotypes of Duchenne muscular dystrophy(DMD)in a retrospective study of 96 cases and to evaluate therapeutic efficacy of glucocorticosteroid.Methods The clinical phenotype,laboratory examinations resuhs and the records in the follow-up in 96 patients with DMD were collected.The level of serum creatine kinase(CK)and motor ability before and after glucocorticosteroid therapy were analyzed by statistical analysis.Their myocardium impairments and intelligence conditions were also assessed.Results(1)The level of serum CK (mmol/L)had three peaks at the age of ≤ 3 years old(16 547.9 ±770.9),5 years old(14 371.9 ± 696.7)and 8 years old(13 089.8 ± 877.6).The CK level significantly decreased after dexamethasone (5-10 mg,iv)treatment for 10-15 days,but increased again after prednisone acetate(0.50-0.75 mg · kg-1 · d-1,oral)administration for one month(F =6.758,P =0.003).(2)The motor ability improved in 51 DMD cases with long-term oral admission of prednisone,including 24 cases receiving repeated dexamethasone,iv.(3)The myocardial perfusion imaging in 37 DMD cases showed remarkable uneven “spotted like” radionuclide distribution in ventricle.(4)The intelligence quotients in 24 DMD were lower than normal population.Conclusions There are high CK hyperlipidemia and myocardial damage in the sub-clinical stage of DMD and myocardium impairment is positively correlated with age.Glucocorticosteroid therapy has an important effect on the protection of motor and cardiac functions,with recommendation of using in early stage of disease.

Objective To analyze retrospectively the clinical manifestations,features of the biopsy of skeletal muscle with histochemistry and immunohistochemistry staining of 40 patients with dysferlinopathy and investigate its clinical,pathological diagnostic value.Methods The clinical data,features of the biopsy of skeletal muscle with histochemistry,immunohistochemistry staining of 40 patients with dysferlinopathy were analyzed.Results Chronic progressive weakness and wasting were the general clinical manifestations.In our study,it was divided into three phenotypes according to the involved muscles of dysferlinopathy:27 cases with proximal muscle,12 cases with the gastrocenemius,1 case with the tibialis anterior muscle.The serum creatine kinase levels all had a rise in different degree (134-19 795 U/L).All the patients showed myogenic lesions in electrophysiologic study.12 patients underwent skeletal muscle MRI.Proximal muscle was involved in 4 cases ; gastrocnemius muscle was mainly involved in 7 cases ; and anterior tibial muscle initially was involved in 1 case.All 40 cases showed active muscle fiber degeneration,necrosis and regeneration on muscle pathology.Connective tissues were proliferated and inflammatory cells infiltrated in endomysium,perimysium and perivascular sites of 16 patients.Immunohistochemical staining with anti-dysferlin monoclonal antibody identified the deficiency of dvsferlin in the sarcolemma of 30 cases with dysferlinopathy,and dysferlin was severely reduced in 10 cases.Conclusion Progressive weakness and wasting of skeletal muscle are the clinical manifestations of dysferlinopathy.The early involved muscles determine the clinical phenotype of dysferlinopathy.High serum creatine kinase levels show that dysferlinopathy is a membrane protein null disease.Muscle MRI of lower limbs may reflect the involved muscles,which is essential for clinical phenotypes and selecting muscle biopsy.The pathological characters of dysferlinopathy are changes of muscular dystrophy.Inflammatory cellular infiltration is relatively common in biopsied muscles of many dysferlinopathy patients,and dysferlinopathy needs to be differentiated from inflammatory myopathies.The deficiency or severely decreased dysferlin on the sarcolemma in immunohistochemical staining with anti-dysferlin monoclonal antibody is an important information for diagnosing dysferlinoapthy.