Objective To report a family with lipoid proteinosis (LP) from Shandong province and to analyze mutations in the extracellular matrix protein 1 (ECM1) gene in this family.Methods Eight members in a threegeneration family with LP were clinically investigated,and two patients were identified to suffer from LP,including the proband (Ⅲ 1) and her mother (Ⅱ 2).Both of the patients presented with papules on the palpebral margin,short and thick lingual frenum,and hoarseness.Indirect laryngoscopy showed infiltrating and thickening of the vocal cord.Pathological examination of lesions on the palpebral margin and laryngeal mucosa revealed deposits of hyaline-like material in the dermis,which was strongly positive for periodic acid-Schiff (PAS) staining and resistant to diastase digestion.The pathological diagnosis was LP.Blood samples were collected from all the family members and 100 ethnically matched,unrelated and unaffected Chinese human controls followed by DNA extraction.PCR and sequencing were performed to detect the ECM1 gene,and nested PCR followed by agarose gel electrophoresis to analyze mutations in the coding region of the ECM1 gene.Results Both of the two patients were compound heterozygotes.Three missense mutations,incluing p.P169T,p.A44T and p.R392W,were found in the ECM1 gene of the affected mother,with p.P169T in one allele and p.A44T as well as p.R392W in the other.The girl patient inheried the missence mutation p.P169T from her mother and a synonymous mutation c.879G ＞ A from her father (Ⅱ 1).Nested PCR showed that the c.978G ＞ A mutation generated a splice-acceptor site AG,which leaded to a splicing defect.Conclusion A novel synonymous splice-acceptor site mutation c.879G ＞ A in the ECM1 gene is identified in the family with LP.

The paper is to evaluate the efficacy and safety of Shenmai injection for chronic heart failure, retrieving the Pubmed, CBM, CNKI, Wanfang database and VIP database to comprehensively collect all types research report of Shenmai injection for chronic heart failure (CHF). Particularly wishing to point out, randomized controlled trials are include for the evaluation of effectiveness, which are statistically analyzed and evaluated by Rev-Man 5. 2. The current studies show that the improvement rate of NYHA classification of cardiac function of CHF patients and their related indexes figure such as LVEF, SV, CO, BNP, 6 min walking test value are all improved by the combination of Shenmai injection and foundation treatment. However, HR is almost no improvement. Meanwhile, serious ADR/AE of Shenmai injection for CHF isn't appear.
Drug Combinations ; Drugs, Chinese Herbal ; administration & dosage ; adverse effects ; therapeutic use ; Heart Failure ; drug therapy ; Humans

BACKGROUND: Autogenous bone has been used in cervical vertebra graft bone fusion in earliest stage and at most. However, its source is limited, simultaneously, induced many complications such as infection, hemorrhage and postoperative pain in the donor bone region. Recently, above-mentioned complications were avoided or reduced with the usage of new graft bone fusion material. OBJECTIVE: To compare clinical efficacy using MC+~R combination of autogenous bone or calcium sulfate artificial bone in antador cervical fusion.METHODS: A total of 26 patients (34 levels) with cervical spondylotic myelopathy underwent anterior cervical discectomy and cervical intervertebral fusion from January to December 2008. Anterior cervical oblique cut was 3.0-4.0 cm. The endplate were preserved after the cervical intervertebral disc and the posterior longitudinal ligament were removed. Autogenous bone group was filled with autogenous bone. Calcium sulfate artificial bone group was filled with Wdght's Osteoset artificial bone. Anchoring clip was implanted between the cervical vertebrae. Every patient had a short neck incision was assessed with X-ray, JOA grade and Odom's evaluation scale.RESULTS AND CONCLUSION: The two groups of 26 patients (34 segments)were followed up. The JOA score of postoperation was no significant difference between the two groups. According to the Odom's evaluation scale, the excellent and good rate of calcium sulfate group was higher than autogenous bone group, but there was not statistical significance (P>0.05). The fusion rate of autogenous bone group was higher than calcium sulfate group at 3 and 6 months, but the fusion rate of two groups were 100% at 12 months. Although the calcium sulfate group at 6 months, lordosis angle lost more than 0.4°than the autogenous bone group,but no significant statistically between the two groups (P>0.05). MC+ combination of autogenous bone or Calcium sulfate had the same clinical efficacy in the treatment of cervical spondylotic myelopathy, but the calcium sulfate artificial bone could be effectively avoided the complications of donor site.

Objective To explore the effect of inhibiting placental growth factor ( PIGF ) by small interfering RNA ( siRNA) on migration, invasion and chemoresistance of human pancreatic cancer cell line PANC1.Methods Three specific siRNAs targeting PIGF (siRNA-PIGF) were designed.PANC1 cells were transfected with siRNA-PIGF by liposome transfection using untransfected cells as blank controls and nonspecific siRNA ( siRNA-NC) transfected cells as negative controls .The PIGF mRNA and protein expression was examined by real-time RT-PCR and ELISA.MTT method was used to assess the inhibition rate of chemotherapeutic reagents on cell proliferation .The abilities of migration and invasion were evaluated by Transwell assay.Results The inhibition rate of PIGF mRNA in PANC1 cells transfected by 3 siRNA-PIGF were (64.38 ±8.92)%, (70.48 ±7.72)% and (81.25 ±6.02)%, which was lowest in siRNA-PIGF-3 transfected cells.The expression of PIGF mRNA in PANC1 cells were decreased by (63.72 ±8.20)%at 24 h after siRNA-PIGF transfection compared with siRNA-NC transfected cells;and the level of PIGF protein in the supernatant of cultured PANC1 cells was lowered by (42.92 ±1.34)% compared with siRNA-NC transfected cells and by (46.25 ±3.64)% compared with untransfected cells at 48h after transfection, which all had significant difference .There was no statistical difference between untransfected and siRNA-NC transfected cells.After 3 ng/L gemicitabine treatment , the inhibition rate of cell proliferation in siRNA-PIGF group was even higher than that in siRNA-NC and untransfected group [(44.35 ±5.05)% vs(34.29 ±3.60)% and (31.01 ±1.08)%;both P<0.05], and no significant difference was not observed after 5-FU and adriamycin treatment.In migration and invasion assay , the number of transmembrane cells from siRNA-PIGF group was 38.1%and 28.2%of that from siRNA-NC group and 40.8% and 36.2% of that from untransfected group , which had statistical difference (all P<0.05).Conclusions PIGF silencing could significantly suppress the migration and invasion of PANC 1 cells and improve the sensitivity to gemicitabine .

To investigate the effects of salvianolic acid B (SA-B) on cardiovascular endothelial cell function and platelet activation during myocardial ischemia-reperfusion in rabbits.

Objective To investigate the radiosensitizing effect of 17AAG-cypate micelles on human non-small cell lung cancer A549 cells and its possible mechanism.Methods (1) A single-hit multi-target model formula was used to analyze the radiosensitizing effects of 17AAG-M and 17AAG-cypate-M.(2) The effects of 17AAG-cypate-M on the viability of A549 cells under laser and X-ray irradiation were analyzed by MTT assay.(3) The effect of the drugs on the cell senescence was observed by β-galactosidase staining assay.(4) The effects of different treatment conditions on DNA damage repair were analyzed by γ-H2AX immunofluorescence staining assay.(5) The expression of p-Erk1/2 and p-Akt was measured by Western blot.The paired t test was used for analyzing the differences between groups.Results Compared with the X-ray irradiation group,the X-ray+17AAG-cypate-M group had a lower mean lethal dose and a sensitization enhancement ratio greater than 1,indicating that 17AAG-cypate-M had a radiosensitizing effect.Compared with the 17AAG-M group,the 17AAG-cypate-M group showed significantly lower cell viability (P<0.01),a significantly higher percentage of aging cells (P<0.01),and significantly further delayed DNA damage repair (P<0.01).And the 17AAG-cypate-M group had lower expression of p-Erk1/2 and p-Akt than the 17AAG-M group.Conclusions Compared with 17AAG-M,17AAG-cypate-M has a higher radiosensitizing effect on A549 cells.The mechanism might be inducing the cell senescence,delaying DNA damage repair,and inhibiting the expression of p-Erk1/2 and p-Akt.

Objective To investigate the effects of enteral nutrition with galactooligosaccharides (GOS) on serum inflammatory cytokines in rats with severe acute pancreatitis (SAP). Methods SD rats were randomly divided into sham operation control group, SAP with enteral nutrition (EN) group and SAP with EN supplemented with GOS (GOS-EN) group, and each group was divided into 4 d and 7 d subgroups according to the time that animals were sacrificed (n=8 in each subgroup). Rat SAP models were established by injection of 38 g/L sodium taurocholate beneath the pancreatic capsule. The serum amylase, inflammatory cytokines TNF-α, IL-2 and IL-10 were detected. Results At each time point, the levels of serum amylase in all SAP groups were significantly higher than those in sham operation control group (P < 0.01), and the levels in GOS-EN group were significantly lower than those in EN group (P < 0.01). The levels of serum TNF-α and IL-10 in all SAP groups were significantly higher than those in sham operation control group (P < 0.01), while the levels of IL-2 in all SAP groups were significantly lower than those in sham operation control group. The levels of TNF-α in GOS-EN group were significantly lower than those in EN group (P <0.05), while the levels of IL-2 and IL-10 and the ratio of IL-10/TNF-α were significantly higher than those in GOS-EN group (P < 0.05). Conclusion Early EN supplemented with GOS could modulate the balance of pro- and anti-inflammatory response.

OBJECTIVETo constitute a model of B immunoblastic lymphomas in the Hu-PBL-SCID mice.

METHODSThe SCID mice were reconstituted by intraperitoneal injection (i.p.) of 5 x 10(7) human lymphocytes from Epstein-Barr virus (EBV) seronegative individuals. After one week, the SCID mice were inoculated with EBV by i.p. injection, and subjected to the investigation of whether there was any tumor in the abdomen of such SCID mice four weeks later. The characteristics of the found tumor was observed by the methods of Hematoxylin-eosin (HE) stain, immunohistochemical staining and polymerase chain reaction (PCR).

RESULTSCompared with the control groups, all the EBV-infected Hu-PBL-SCID mice had abdominal solid tumors [(32 +/- 12.5) mm3] developed, often located in the liver. HE staining and immunohistochemical staining showed the tumors were human B cell lymphomas. EBV DNA could be detected in the tumors by the PCR.

CONCLUSIONSThe model of B immunoblastic lymphomas in the Hu-PBL-SCID mice is successfully constituted, and may well be useful to the human tumor immunological study.

Objective To explore the correlation between MSCT perfusion parameters and biologic characters in nasopharyngeal carcinoma(NPC),and to investigate the clinical utility of MSCT perfusion for NPC.Methods Nasopharynx in 49 cases was studied using MSCT perfusion imaging,including 30 NPC cases before radiotherapy,14 post-radiotherapy NPC cases,and 5 cases of normal nasopharynx as controls. The perfusion data of tumors and nasopharyngeal wall such as blood flow(BF),peak enhancement index (PEI),time to peak (TTP),and blood volume (BV)were calculated.MVD defined by CD34 stain was counted in 16 cases of NPC.The correlation of MSCT perfusion parameters,MVD,and clinical stage was analyzed.Results 1 case of NPC failed in CT perfusion exam.In NPC group (n=29),BF,PEI,TTP, and BV were (48.6?16.9)ml?100 g~(-1)?min~(-1),(32.3?7.9)HU,(17.5?4.9)s,and (12.8? 4.4)ml?100 g~(-1),respectively.BF,PEI,TTP,and BV in control group (5 cases)were (15.9? 5.9)ml?100g~(-1)?min~(-1),(12.6?1.3)HU,(22.6?6.9)s,and (3.5?0.5)ml?100 g~(-1), respectively,and those in post-radiotherapy group (14 cases)were (25.2?7.0)ml?100 g~(-1)?min~(-1), (19.8 ?5.9)HU,(22.6?4.3 )s,and (6.1?2.4)ml?100 g~(-1),respectively.The perfusion values in NPC group had significant difference compared with those in the other two groups (P

Objective The aim of this study is to investigate the role of angiotensin-converting enzyme (ACE)gene susceptibility to systemic lupus erythematosus(SLE)by familial studies.Methods PCR-based re- striction fragment length polymorphism(RFLP)was applied to genotype single nucleotide polymorphism(SNP) G261T of the ACE gene.A total of 119 patients with SLE from 119 families were recruited.In addition,316 family members of these patients were also genotyped.A family-based association study was carried out to ex- plore the association between gene polymorphism and SLE.We studied the SNP encoding non-synonymous substitution in the ACE gene with respect to genetic susceptibility to SLE.Results Among 119 SLE patients. the frequency of ACEG261TG,T alleles was 44.8%.55.2% respectively,the frequency of ACEG261T GG,GT and TT genotypes was 13.9%,62.0%,24.1% respectively,Univariate(single-marker)family-based association test(FBAT)demonstrated that variant alleles at the SNP,rs4303,exon 5 of ACE gene were significantly asso- ciated with genetic susceptibility to SLE in Additive Model(Z=2.877,P=0.004),Dominant Model(Z=2.557, P=0.011).Recessive Model(Z=2.202,P=0.028).Transmission-disequilibrium test(TDT)and sib transmission -disequilibrium test(STDT)showed an excess of the allele of T from heterozygous parents to affected offspring or higher frequency of the allele of T in the patients than their normal siblings(X~2=11.66,P=0.001).Conclu- sion Our findings suggest that the ACE gene may he the susceptible gene to SLE in Chinese population,and the individuals carrying ACE-261T allele is significantly associated with susceptibility to SLE.