ObjectiveTo investigate the correlation of MRI features and phenotypes and genetic mutations in Pelizaeus-Merzbacher disease.Methods Sixteen boys with clinical diagnosis of PelizaeusMerzbacher disease (PMD) were included in this study.Their ages ranged from 22 months to 9 years.They were examined by pediatric neurologists,and clinical classification was made according to the symptoms and physical signs.An experienced radiologist reviewed the cranial MRI images and analyzed the brain involvement,including pallidus globus,pyramidal tract,corpus callosum,cerebellar white matter,semiovale centrum,brain atrophy and ‘ tigroid sign’.ResultsThere were 8 patients with classic form,7 patients with transitional form and one patient with connatal form.They all showed diffuse delayed myelination in the white matter,with involvement of pallidus globus in 13 cases,pyramidal tract in 7 cases,corpus callosum in 11 cases,cerebellar white matter in 7 cases,semiovale centrum in 12 cases.Cerebral atrophy was found in 5 patients and eerebellar atrophy was found in one patient.Five cases depicted ' tigroid sign'.In patients with PLP1 gene point mutation,pyramidal tract and cerebellar white matter involvement showed a high incidence.Cerebellar white matter lesions were relatively frequent in children with transitional form and connatal form.In contrast,‘ tigroid sign' was often related to classic form,which indicated a better myelination and outcome.ConclusionPMD patients show distinct imaging features in their brains,which may be correlated with the phenotype and genetic mutation.

AIM:To investigate the timing and efficacy of vitrectomy for patients with vitreous hemorrhage(VH) due to proliferative diabetic retinopathy(PDR).METHODS:Retrospective analysis.Patients who presented to our hospital between Feburary 2012 and May 2014 with VH secondary to PDR treated with vitrectomy were included.All patients were divided into three groups according to the duration of VH.A group was less than 1mo for 22 eyes, B group was 1-3mo for 23 eyes, C group was more than 3mo for 25 eyes.All patients underwent intravitreal injection of ranibizumab 1-2wk before vitrectomy, and supplemented or finished panretinal photocoagulation (PRP) intraoperatively or postoperatively.Patients with cataract accepted phacoemulsification and intraocular lens implantation.Eyes filling silicone oil were implanted intraocular lens in the second phase.All patients were followed up 24 to 42mo (mean:28.7mo).We assessed the intraoperative complications such as hemorrhage, iatrogenic retinal hole, and postoperative complications such as vitreous hemorrhage, neovascular glaucoma.Macular edema and best corrected visual acuity were observed at every follow-up.RESULTS:There was no significant difference for other baseline data (P>0.05) but DR stage between three groups (P=0.033).There was significant difference of last follow up visual acuity between three groups (P<0.001).The significant difference can be seen between group A and B (P=0.03).The same outcome showed between Group A and C(P<0.001).There was no significant difference between Group B and C (P>0.05).The percentage of visual acuity was 0.5 and above in the three groups were:41%, 23%, 0 respectively.The patients with visual acuity of less than 0.1 were 5%, 26% and 40% respectively.Silicone oil filling rate of three groups were:9%, 26%, 40% respectively and there was no significantly difference between three groups on postoperative complications (P>0.05).CONCLUSION:Patients with VH due to proliferative diabetic retinopathy undergoing early vitrectomy may get better visual acuity than who accepting delayed vitrectomy.

AIM: To analyze the pathogens and drug sensitivity of chronic dacryocystitis in order to provide evidence for clinical drug use.
METHODS:Lacrimal secretion of 171 cases with chronic dacryocystitis was sampled for pathogenic bacteria culture identification and drug sensitivity test. Based on the results, the isolation rate of pathogens strains, the pathogens kind of chronic dacryoeystitis, main pathogens of chronic dacryocystitis, and sensitive drug for pathogens were analyzed.
RESULTS: The isolation rate of pathogens strains was 76. 61% ( 131 cases ). The pathogens constituting the chronic dacryocystitis were predominantly gram-positive coccus,the percentage was 72. 52% (95 cases), among which staphylococcus hominis occupied 27. 48% ( 36 cases), staphylococcus epidermidis 16. 79% (22 cases), streptococcus viridans 12. 98% (17 cases). The majority of these bacteria were sensitive to cefoperazone-sulbactam, tobramycin, gentamicin and levofloxacin. For gram -positive coccus, cefoperazone - sulbactam, gentamicin and tobramycin were the most sensitive drug. For gram-negative bacilli, cefoperazone - sulbactam, tobramycin and levofloxacin were most sensitive drug.
CONCLUSION: Staphylococcus hominis is the main pathogen of chronic dacryocystitis, tobramycin can be used as the first choice for local treatment of chronic dacryocystitis.

OBJECTIVEThe aim of this study is to investigate the occurrence and mechanism of Cheyne-Stokes breathing pattern in patients with heart failure.

METHODSFifty-six patients who performed polusomnography sleep testing at National Center of Cardiovascular Diseases Fuwai Hospital from March to May in 2015. We divided them into chronic heart failure (CHF) group and non-CHF group.

RESULTSThe occurrences of sleep apnea in two groups were high. In CHF group (n = 11) , there were 10 patients with apnea hypopnea index (AHI) > 5; and their AHI was 23.93 ±14.63. In non-CHF group (n = 45), there were 33 patients whose AHI > 5; and their AHI was 16.20 ± 18.76. The ratio of center sleep apnea to all gross sleep apnea ratio in CHF group was higher than that in non-CHF group (80.21% ± 30.55% vs 27.16% ± 35.71%, P < 0.01 ).

CONCLUSIONBased upon the new theory of holistic integrative physiology and medicine, we explain the mechanism of circulatory dysfunction induce the oscillation breathing in patients with CHF. The sleep apnea and C-S respiration in CHF should be called circulatory sleep apnea, rather than central sleep apnea.

AlM:To study the refractive status characteristics aftser cataract surgery and the correlation between preoperative anterior chamber depth ( ACD) and refractive status.METHODS: Ninety-six cases of patients with cataract were randomly divided into two groups. The patients in phacoemulsification group were treated with phacoemulsification combined intraocular lens ( lOL ) implantation while the patients in small incision group were treated by small incision extracapsular cataract extraction combined with lOL implantation. Changes in ACD and postoperative refractive status and refractive fully corrected value were counted and the correlation of them were analyzed .RESULTS: ACD of the phacoemulsification group s deepened 0. 74mm while that of the small incision group deepened 0. 78mm after treatment and there was no significant difference (P>0. 05). After operation, the ACD of two groups significantly deepened ( P<0. 05 ). The postoperative visual acuity of two groups were significantly better than the uncorrected visual acuity of two groups (P<0. 05). The postoperative refraction of two groups patients were mainly 0 ~ +1. 0D ( 41. 67% and 54. 16%) and+1. 25~+2. 0D (43. 75% and 33. 33%) (P>0. 05). CONCLUSlON: ACD is significant deepened after operation. Surgeon needs full consideration of changes to improve the refractive lOL calculation accuracy.

Objective To evaluate the effect of staged repair for tetralogy of Fallot (TOF) associated with pulmonary artery hy- poplasty.Methods From June 1996 to June 2006,37 patients with TOF were operated on.There were 26 males and 11 females. Their age was 5 months to 17 years(mean 3.6 years) and weight was 4.6～38.0 kg.All patients were diagnosed as TOF with pulmo- nary artery hypoplasty by cardiac catheterization.The mean pre-operative arterial saturation of the patients was (68.2?6.5) %,Mc- Coon ratio was 0.95?0.26 (0.81～1.17) and Nakata index was 82.7?21.6(71.6～97.5) mm~3/m~2.At the time of the first surgi- cal procedure,17 patients underwent central aortopulmonary shunt,13 patients received modified Blalock-Taussig shunt in the left side and 7 patients had modified Blalock-Taussig shunt in the right side.Results There were no easly operative deaths and no late deaths after the first stage repair.Pleural effusion after shunt occurred in 5 patients.The mean arterial saturation was significantly increased to (91.3?10.4) %,P

Armillariella tabescens EJLY2098 was capable of secreting p-mannanase by konjac inducement. A 34 orthogonal design was applied to determine the optimum medium of inducing mannanase by Armillariella tabescens EJLY2098. The results suggested that Armillariella tabescens EJLY2098 secreted the high-activity enzyme in the optimum medium, which was composed of 2% konjac, 1% peptone, 25% potato juice,0.3% KH2PO4,15% MgSO4?7H2O, 0.01% VitB1. Purified by DEAE-anion exchange chromatography, two eluting peaks (P1 and P2) with the p-mannanase activity were obtained, and one of them (named?-mannanase P2) was a single band by the SDS-PAGE, and the molecular weight of?-mannanase P2 was 78. 9kDa. The isoelectric point of?-mannanase P2 was estimated to be 4.0-4. 1. The optimum activity for the enzyme was found at 60℃and pH4. 0 - 6. 0, and the enzyme was stable between pH4. 5 - 6. 0. The activity of?-mannanase P2 were enhanced by Na+ and Ba2+ . This?-mannanase can be used in feed industy. a new fungi secreting?-mannanase was obtained, providing an important base for cloning mannanase gene and constructing recombin microbe expressing?-mannanase .

Objective To investigate the apoptosis of T lymphocytes,the expression of Bcl-2, Fas on the peripheral blood T lymphocytes in end stage renal disease patients;and to explore the characteristics of Th1 /Th2 profile and the influence of dialysis membranes with different permeability on the apoptosis of T lymphocytes of maintenance hemodialysis patients.Methods The study included 10 non-dialyszed (ND)patients,45 maintenance hemodialysis patients with cellulose acetate (CA) membranes(13),low-flux polusulfone(PS-LF) membranes(16),high-flux polusulfone (PS-HF) membranes (16) and 8 healthy volunteers (C).The apoptosis of T lymphocytes,expression of Bcl-2,Fas on peripheral blood T lymphocytes cultured with phytohemagglutinin (PHA) stimulation for 24 hours were measured by flow cytometry and immunohistochemical.ELISA was performed for detecting the levels of IFN-?and IL-4 in culture supematants.Results In ESRD patients,the apoptosis of T lymphocytes was greater than that of group C.Group CA was greater than group PS-HF and group PS-LF (P＜0.05).The expression of Bcl-2 on T lymphocytes in ESRD patients was lower than that of group C (P＜0.05).There was negative correlation between the T lymphocytes apoptosis and Bcl-2. The expression of Fas on T lymphocytes in ESRD patients was greater than that of group C (P＜0.05), and it was positive correlated with T lymphocytes apoptosis.The level of IFN-?of ESRD patients was decreased significantly compared with that in group C (P＜0.05),and there was negative correlation between T lymphocytes apoptosis and IFN-?.IL-4 was increased in ESRD patients (P＜0.05) and it was positive correlated with T lymphocytes apoptosis.Conclusions The accelerated apoptosis of T lymphocytes in ESRD patients may be related to the expression of Bcl-2 and Fas of T lymphocytes.ESRD patients show a suppressed secretion of IFN-?and an increased secretion of IL-4. T lymphocytes apoptosis of maintenance hemodialysis patients is influenced not only by the biocompatibility but also by the permeability of the dialysis membrane.

Objective To study the preemptive analgesia effects and side reactions of flurbiprofen axetil in gynecological laparoscopy.Methods Sixty patients(ASAⅠ～Ⅱ)were randomly assigned into two groups with 30 cases each.GroupⅠreceived intravenous injection of flurbiprofen axetil 1mg/kg in 15 minutes before surgery.GroupⅡreceived intravenous injection of saline 1mg/kg at the same time.Postoperative analgesia efficacy was assessed by visual analogue scales(VAS)at 1,2,4,8.12,24h and side reactions were recorded after surgery.The overall satisfac- tion with analgesic therapy was evaluated after analgesia.Results VAS in groupⅠat 1,4,12,24h was lower than that in groupⅡ(P

Objective: The study was designed to evaluate the changes and significance of circulating CD4~+CD25~+ and CD8~+CD28~- regulatory T cells (Tregs) in patients with multiple myeloma (MM).Methods:CD4~+CD25~+ and CD8~+CD28~-Tregs in peripheral blood of 38 patients with MM and of 20 healthy doners were measured by flow cytometry.Serum albumin and β_2-MG in patients with MM were measured using bromocresol green method,transmission turbidimetry respectively.Results:Compared to those of the controls,the proportions of CD4~+CD25~(+/high),CD4~+CD25~(high) CD127~(low) and CD8~+CD28~-Treg cells in newly diagnosed MM patients were elevated.Furthermore,the proportions of CD4~+CD25~(high) and CD4~+CD25~(high)CD127~(low) Tregs in each clinical stage were elevated when compared to those of the controls.The number of the Tregs were increasing with clinical stages and were significantly higher in stage Ⅲ MM than in stageⅠ MM;In stageⅡand Ⅲ MM,there were also elevated proportions of CD8~+CD28~- Tregs,increasing with clinical stages.However,there were no differences when compared between stage Ⅰ MM and the controls;Both the proportions of CD4~+CD25~(+/high) and CD4~+CD25~(high)CD127~(low) Tregs in active MM were not different from stable MM,although all of them were higher than those of controls.The proportion of CD8~+CD28~- Tregs was higher in active MM than in stable MM and controls,but there were no differences when compared between active and stable MM.The proportions of both CD4~+CD25~(high) Tregs and CD4~+CD25~(high)CD127~(low)Tregs had negative correlation with the levels of serum albumin.Conclusion:MM patients have elevated levels of circulating CD4~+CD25~+ and CD8~+CD28~-Tregs,which may be an important mechanism of MM immune evasion,and may be associated with clinical stages,disease progression and prognosis of MM to some extent.