Objective To explore the value of electroneurophysiological detection in diagnosis of children progressive muscular dystrophy (PMD).Methods The clinical features and laboratory data were analyzed in 32 children with PMD ,and electromyography(EMG) and nerve conduction velocity(NCV) were performed.Parameters studied included spontaneous activity , duration and amplitude of motor unit potential(MUP),pattern of recruitment as strong contracting,sensory conduction velocity(SCV),motor conduction velocity(MCV), distal latency and distal amplitude. Results The abnormality rates of spontaneous potentials was 49.2% and 80.9% in tibialis anterior.The decrease of duration of MUP was 29.7%-62.4%.Amplitude of strong contracting was significantly decreased.There were different from those in normal children(P

Wuxistatin, a novel and potent statin, is converted from lovastatin by Amycolatopsis sp. CGMCC1149. In the bioconversion, lovastatin is firstly hydroxylated by a hydroxylase. To obtain the critical hydroxylase, a novel hydroxylase gene was isolated from Amycolatopsis sp. CGMCC1149 by Degenerate PCR and Self-Formed Adaptor PCR and expressed in Escherichia coli. BLAST sequence analysis revealed that the gene belonged to cytochrome P450 gene superfamily and could encode a 403-amino-acid protein with a molecular weight of 44.8 kDa. The secondary structure prediction result showed that this protein contained many typical functional regions of P450, such as oxygen binding site, ion-pair region and heme binding region. Meanwhile, a catalytic function verification system was constructed by NADH, ferredoxin and ferredoxin reductase which could catalyze lovastatin hydroxylation into the target product. These would be helpful for further studies in large-scale production of wuxistatin.
Actinomycetales ; enzymology ; genetics ; Amino Acid Sequence ; Butyrates ; metabolism ; Cloning, Molecular ; Cytochrome P-450 Enzyme System ; genetics ; metabolism ; Hydroxylation ; Industrial Microbiology ; Lovastatin ; metabolism ; Molecular Sequence Data

Objective To evaluate the effect of three diamension-digital subtraction angiography (3D-DSA) or computed tomography angiography (CTA) on the patients with ruptured multiple intmcranial aneurysm (MIA). Methods A retrospective study on 21 patients with MIA was performed.After scanning with 3D-DSA or 3D-CTA, three-dimensional reconstruction of MIA was carried out by 3D workstation,then the diagnosis was decided and the treatment plan (endovascular treatment or microsurgery) was selected according to stereoscopic image of MIA. Results (1) 3D-DSA or CTA was performed in 21 patients with subarachnoid hemorrhage (SAH),it was revealed these patients carried with 48 aneurysms,including 35 small aneurysms (25 mm).Not only miero-aneurysms and small aneurysms could be precisely showed,also the size of aneurysmal neck,the relationship of the aneurysm and the parent vessel and contiguous branches by stereoscopic image.(2) According to the standard of classification,9 patients with MIA for gradeⅠ(42.9%),10 for gradeⅡ(47.6%),2 for gradeⅢ(9.5%),0 for gradeⅣ.Endovascular treatment was selected prior to microsargery for those high grade patients.In this group,17 patients with 40 aneurysms underwent endovascular embolotherapy with GDC coils.Twenty four anemysms were completely occlusioned,12 beyond 90%,4 were left without treatment because of their small size.In microsurgery group,3 aneurysrus were totally clipped,1 could not be found during operation.No any treatment was accepted in 2 patients with 4 aneurysms. Conclusions 3D-DSA or CTA,which is very useful for the diagnosis and treatment of MIA,could improve the accuracy of diagnosis of MIA and clearly show the stereoscopic image of MIA,also the relation of sac and parent artery.For those patients with high grade MIA,endovascular treatment was selected prior to microsurgery,pro re nata,used to combine with mierosurgery.

Objective: To study the influence of electret on surface charge of fibroblast cells (3T3 cells) and to probe the relationship between cell growth, apoptosis and cell surface charge. Methods: Electrets Teflon PTFE, ±300 V,±1 000 V were used to treat 3T3 cells for 24, 48 and 72 h. Then the influences of electrets on cell cycle and surface charge of 3T3 cells were studied by flow cytometry and electrophoresis, respectively. Results: (1) After 24 h action of negative electrets, electrophoretic mobility (or surface charge) and cell number in S phase of 3T3 cells were significantly increased compared with those in control group. (2) Effect of negative electrets enhancing cell growth and increasing cell surface charge was in proportional to the surface potential of electret. (3) Surface charge density of apoptotic cell was reduced by electret. (4) After 24 h action of positive electret, the cell number in S and G2 phase were decreased and cell surface charge was also reduced. Conclusion: Negative electret can improve cell growth and increase cell surface charge density. Positive electret can restrain cell growth and reduce cell surface charge density. Surface charge of apoptotic cell is less than that of normal cell.

ObjectiveTo compare the therapeutic effects of percutaneous cervical discectomy (PCD group),percutaneous cervical disc nucleoplasty(PCN) and the association of them (PCDN) for the treatment of cervical intervertebral disk displacement and instability of cervical vertebral column.Methods From February 2003 to April 2011,171 consecutive patients with cervical disc herniation have presented at the authors' hospital and were retrospectively studied.The average age of patients was 47.8 years(ranged,21-74).Ninety-seven cases were treated with PCD,50 cases with PCN,and the other 24 cases with PCDN.Clinical result and the stability of cervical vertebral column after operation were evaluated and compared among the 3 groups.ResultsAll cases had been followed up for a median of 4.1 years.There was significant difference in the pre- and post-operation the Japanese Orthopaedic Association(JOA) scoring system on within 3groups (PCD:t=21.85,P＜0.05; PCN:t=14.50,P＜0.05; PCDN:t=8.56,P＜0.05).All cases had been successfully operated.There was no significant difference between groups among the 3 groups in terms of the clinical outcomes(The recovery rate of JOA standard evaluation,F=2.19,P=0.12).According to Odom criteria,the excellent and good rate are as follows:81.35％ in PCD,82.44％ in PCN,83.19％ in PCDN,respectively.There was no significant difference between groups among the 3 groups in terms of the clinical success rate (P＞0.05).There was no instability of cervical vertebral column cases in 3 groups after operation(P＞0.05),and no significant difference was found in terms of cervical vertebral column stability in pre- and post-operation in each group.ConclusionAll the three operations including PCN,PCD and PCDN are safe,minimally invasive spine surgery for the treatment of cervical intervertebral disk displacement; they achieve good clinical outcomes and there are no difference on the stability of cervical vertebral column between preoperation and postoperation.

Objective To investigate the clinical characterstics of bone Langerhans-cell histiocytosis (LCH) and evaluate its diagnosis,therapy and prognosis.Methods 25 cases with biopsy confirmed bone LCH during the last 8 years were retrospectively analyzed.Results The patients included 18 males and 7 females,13 children and 12 adults,ranging from 1.5 to 55 years old with a median age of 17.Cases with unifocal lesions were 17,including 11 cases of skull LCH,and the remaining 8 were with multifocal lesions.First symptoms were predominantly pain and local masses,and rarely constitutional symptoms.The manifestation of radiography was osteolytic bony lesions.12 cases had masses in soft tissues.Patients with solitary lesions underwent surgical operation,followed by radiotherapy or chemotherapy.Cases with multifocal lesions received chemotherapy and radiotherapy.Pathological examination showed proliferation of well differentiated histiocytes,and large numbers of infiltrating eosinophils.Positive rates of CD1a,S100,Vimentin and CD68 were higher in immunohistochemistry.Patients with restricted involvement in bones can achieve a satisfactory therapeutic effect.2 cases died when multiple systems were compromised.Conclusion Bone LCH occurs predominantly in children and teenagers,involves solitary bones,and morbidities in males are much higher than females.Skull is most commonly affected.Principal clinical manifestations are pain and local masses.Diagnosis of bone LCH depends on microscopic examination.Combination therapy appears to be an effective method of treatment.Prognosis of disease is related to the degree of bone involvement,histological classification and simultaneously encroachment of other organs.Most patients have good prognosis.

Objective To explore the efficacy and safety of moderate-dose of etoposide (VP16) with granulocyte-colony-stimulating factor (G-CSF) for mobilization of peripheral blood stem/progenitor cells.Methods VP16 at 1.2 g/m2 was injected intravenously by six divided doses via a central vein, 2 times every 12 hours for 3 days in 31 patients with malignant lymphoma (30 non-Hodgkin lymphoma and 1 Hodgkin lymphoma). All patients received G-CSF 5 μg/kg were given twice daily subcutaneously from the day of the nadir of white blood cell (WBC) till the day before the last APBSC harvest. Results The mean time for the collection of stem cell was 12 days (10-15) following etoposide chemotherapy. The mean number of mononuclear cell (MNC) and CD+34 cells in collection were 7.8×108/kg (5.2-11.3×108) and 7.2×106/kg (5.3-13.1×106). respectively. 18 patients completed collection with a single apheresis, and 13 patients underwenttwice. All patients were recovered for haematopoiesis in following APBSCT. Median (range) time for the recovery of absolute neutrophil count (ANC)＞0.5×109/L and platelet＞20×109/L were+12 (+9-+18) days and +14 (+10-+21) days respectively. Slight adverse events coursed by the regimen could be tolerated. Conclusion VP16 at moderate dose with G-CSF is an effective and safe mobilizing regimen for autologous peripheral blood stem/progenitor cells in patients with malignant lymphoma. It was suggested to use extensively.

Objective To summarize and evaluate the incidence,etiology,diagnostic and therapeutic method of hepatic dysfunction after allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods 83 blood disease patients who undergoing allo-HSCT from 2006 to 2010 in the affiliated cancer hospital of Zhengzhou university.Among those who suffered from Ⅱ-Ⅳ grade hepatic dysfunction,the incidence,the ratio of different causes,clinical feature and diagnostic method were evaluated.The difference of causes of hepatic dysfunction in different period,the therapeutic method and curative effect were also analysed.Results Among 83 patients undergoing allo-HSCT,45 patients suffered from Ⅱ-Ⅳ grade hepatic dysfunction,the ratio was 54.2 ％.For etiology,7 were preconditioning,9 were cyclosporine (CsA),2 were hepatic venoocclusive disease (HVOD),24 were hepatic graft versus host disease (GVHD),2 was hepatic B virus (HBV)reactivation,1 was mutiple organ failure.20 cases (44.4 ％) occurred in one month after allo-HSCT with the main etiology of drug hepatotoxicity.13 cases (28.9 ％) occurred from one month to 100 days after allo-HSCT,while 12 cases (26.7 ％) occurred from 101 days to one year with the main etiology of both hepatic GVHD.27 cases were cured and 10 were improved after treatment.2 cases were not cured and 6 cases died from relapse of the primary disease,or else from the complication of allo-HSCT.Conclusion Hepatic dysfunction is an common complication after allo-HSCT,drug hepatotoxicity and hepatic GVHD are the major causes.The relativity between hepatic dysfunction and period after allo-HSCT is a important reference for diagnosis.It will produce desired result to choose proper therapeutic method based on etiology.

Objective To investigate the novel V617F point mutation of JAK2 gene by real-time PGR in the patients with chronic myeloproliferative disease (CMPD) and evaluate its clinical significance. Methods Genomic DNA from bone marrow or peripheral blood mononuclear cells was extracted from 56 patients with CMPD, including 26 cases of polycythemia vera (PV), 24 cases of essential thrombocythemia (ET), 5 cases of chronic idiopathicmyelofibrosis (CIMF) and 1 case of high eosinophilic syndrome (HES). The exon 14 of JAK2 gene which harbourd V617F mutation were screened by real-time PGR. Results JAK2 V617F mutation was measured in 29 of the 56 patients with CMPD. The prevalence of mutation was 65.38 %(17/26) in PV,37.50 % in ET and 60.00 %(3/5) in CIMF. The proportion of mutation in PV, ET and CIMF are respectively 53.85 %(14/26), 29.17 %(7/24), 40.0 %(2/5) in heterozygotes and 11.54 %(3/26), 8.33 %(2/24), 20.00 %(1/5)in homozygotes. JAK2 mutation was negative in one patient with HES. JAK2 V617F allele burden in PV, ET and CIMF are respectively 2.14×102-1.5×107, 9.80×102-4.4×107 and 4.10×103-3.70×106 copies. Conclusion Real-time PCR is a useful tool for pre cisely assess the grade of mutant allele burden as well as to screen JAK2V617F mutation simultaneously, which is simple and convenient to carry out in clinical laboratories for diagnosis and further evaluations of minimal residual disease in CMPD patients.

ObjectiveTo investigate the clinical characteristics in ABO-incompatible allogeneic peripheral blood stem cell transplantation(allo-PBSCT).Methods137 patients'clinical courses who accepted allo-PBSCT were retrospectively reviewed. Sixty-five cases of them were ABO-incompatible allo-PBSCT patients,including 32 ABO major mismatched cases,23 ABO minor mismatched cases and 10 ABO major and minor mismatched cases.Seventy-two ABO-identical cases were taken as control group.ResultsCompared with ABO-idential cases,the time of erythrocyte recovery after allo-PBSCT in ABO major and minor mismatched group was delayed [(73.2+10.3) d vs (97.5+10.4) d] (P ＜0.05).In ABO-incompatible group, the time of blood type switching in different ABO-incompatible types were found no significant difference (P ＞0.05) [ABO major mismatched:(45.7±17.3) d,ABO minor mismatched:(41.2+16.1) d and ABO major and minor mismatched:(48.4±20.9) d (P ＞ 0.05)].There were 8 cases who have a delayed time of blood type changing, including 6 cases demonstrated recipient-derived anti-A antibody. ConclusionABO-incompatible has no negative effect on allo-PBSCT.The time of erythrocyte reconstitution was delayed in ABO major and minor mismatched group.A delayed time of blood type switching tends to occur in ABO minor incompatible cases and patients who have anti-A antibody initially.