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.

Objective To evaluate clinical effect of accurate pedicle subtraction osteotomy (PSO) using osteotomes in the treatment of thoracolumbar kyphosis (TLK).Methods From June 2007 to October 2010,18 patients with TLK underwent accurate PSO using osteotomes under X-ray fluoroscopy,including 13 males and 5 females,with an average age of 48.6 years.The primary causes of TLK included old fracture (11cases),chronic tuberculosis (4 cases) and hemivertebra (3 cases).Deformity apex occurred at T12 (5 cases),L1 (9 cases),and L2 (4 cases).Radiological assessment for sagittal balance was performed by measuring Cobb angle.The Frankel grade,visual analogue scale (VAS) and Oswestry disability index (ODI) were used to evaluate pre-and post-operative neurological status,back pain and function.Results The mean operative time,mean blood loss and mean postoperative drainage volume were 247.0±29.3 minutes,708.5±34.5 ml and 337.3±74.6 ml,respectively.All patients were followed up for 1 to 4.5 years (average,2.8 years).Solid fusion was achieved in all patients.Cobb angle was corrected from preoperative 42.3°±5.7° to 2.2°±1.9° three months postoperatively and 2.7°±2.1 ° at final follow-up.VAS and ODI scores decreased from preoperative 8.5±1.0 and 72.8％±8.3％ to 2.1±0.7 and 21.6％±9.2％ three months postoperatively,and 1.9±0.6 and 19.3％±8.6％ at final follow-up,respectively.With regard to Frankel grade,a 1-grade and 2-grade improvement was observed in 7 cases and 2 cases 3 months postoperatively,respectively.At final follow-up,a 1-grade and 2-grade improvement was observed in 5 cases and 4 cases,respectively.Two patients had transient neurological symptoms postoperatively,which recovered after drug treatment for 2 weeks.No other complications occurred.Conclusion It is safe and effective to correct TLK through accurate PSO using osteotomes,which has some advantages,such as less blood loss,higher fusion rate and fewer complications.

Proliferation and migration of vascular smooth muscle cells (VSMC) are common pathological features of diabetic vascular complications,such as atherosclerosis and hypertension. Phenotypic modulation of VSMC is the basis for VSMC proliferation and migration. Therefore, studies on VSMC phenotypic modulation and its mechanisms in diabetes mellitus were of important significance to the prevention and therapy of diabetic vascular complications. This paper introduces VSMC phenotypic modulation and the underlying mechanisms in diabetes mellitus, and summarizes advance of studies on traditional Chinese medicine intervention upon VSMC phenotypic modulation, so as to provide reference for preventing and treating diabetic vascular complications with traditional Chinese medicines.
Atherosclerosis ; drug therapy ; prevention & control ; Cell Movement ; drug effects ; Cell Proliferation ; drug effects ; Diabetes Mellitus ; drug therapy ; pathology ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Hypertension ; drug therapy ; prevention & control ; Medicine, Chinese Traditional ; Muscle, Smooth, Vascular ; drug effects ; Myocytes, Smooth Muscle ; drug effects ; Phenotype

The challenge of the emergence of drug-resistant influenza strains, which is caused by wide spread utilization of direct-acting antivirals (DAAs), accelerates the research and exploration towards host targeted agents. In contrast to DAAs targeting viral replication components, host targeted agents, which regulate host factors and pathways linked to viral replication, can interfere the replication of influenza. Additionally, the innate immune system is activated by influenza during the early stage of infection, so manipulating the innate immune response may prevent the viral infection. However, the excessive inflammatory response induced at the late phase of influenza infection would lead to severe tissue injures. Thus, it is very important to explore drugs with anti-inflammatory actions to suppress these immune imbalances and tissue injures. Here we overview the current progresses about host targets related to anti-influenza drugs.
Anti-Inflammatory Agents ; pharmacology ; Antiviral Agents ; pharmacology ; Humans ; Immunity, Innate ; Influenza, Human ; drug therapy ; Virus Replication

Objective Insulin-like growth factor-1 receptor (IGF-1R),similar to insulin receptor,is one of the families of re- ceptor tyrosine kinases.,which has been found to be overexpressed in a variety of cancer.It is the main proliferation and survival sig- nal molecule in cancer cell and plays an important role in cancer growth and progress.Blocking signal transduction of IGF-1R by vari- ous strategies can suppress tumor growth and induce regression of established tumor.This study is to construct the siRNA expression vector targeting IGF-1R and to evaluate its ability to induce cell apoptosis in human lung cancer cells.Methods Two siRNA expres- sion vector,pENTR/U6-shRNA-1 and pENTR/U6-shRNA-2 targeting IGF-1R,were constructed using pENTR/U6 vector,and a vector targeting hieiferase gene,pENTR/U6-shRNA-Iuc,was constructed as control.After vectors were transfected into A549 for 48h, knockdown of IGF-1R mRNA and protein and Akt phosphorylation were accessed,and DNA ladder and flow cytometry were used for cell apoptosis.Results siRNA expression vectors targeting IGF-1R were successfully constructed,which was confirmed by PCR and DNA sequencing,pENTR/U6-shRNA-1 and pENTR/U6-shRNA-2 demonstrated the expression were (22.1?2.5) % and (80.1? 3.9) % in IGF-1R mRNA level,(15.2?3.1)% and (47.1?4.1)% in protein level,respectively,compared with pENTR/U6- shRNA-luc.Suppression of IGF-1R by pENTR/U6-shRNA-1 blunted Akt phosphorylation,increased cell apoptosis induced by 3% ethanol,and retained 77.5 % A549 cells in the G0/G1 phase.Conclusion siRNA expression vector targeting IGF-1R can effectively suppress the expression of IGF-1R expression in A549.This study suggests that DNA vector-based RNAi has the potential to be effec- tive and practical cancer gene therapy strategy.

Objective To investigate the effect of dexamethasone addition in block anesthesia before surgery on postoperative quality of life after extraction of impacted mandibular wisdom teeth. Methods Using a random number table, 120 cases of patients were divided into experimental and control groups, each group contains 60 cases whose mandibular wis?dom tooth will be extracted. Before the surgery, the experimental group patients perform local block anesthetic injection with 3 mL out of 5 mL mixture of 2%lidocaine 4 mL+dexamethasone 1 mL (5 mg) to block nerve. The control group was given block anesthesia of inferior alveolar nerve with 3 mL of 2%lidocaine block anesthesia alone. Improved postoperative symp?toms severity scale (PoSSe) was used to evaluate patients’quality of life after tooth extraction comprehensively and quantita?tively by analysis and comparison of the two sets of scores. Results The experimental group’s PoSSe scores were 18.059 1 ± 13.210 9, which was lower compared with control group (27.819 3 ± 15.716 9). In experimental group, diet, language, swelling, pain and daily points were lower than the control group (P<0.05). Conclusion Topical application of dexamethasone in mandibular wisdom tooth extraction reduces postoperative adverse reactions in a certain degree and im?prove postoperative quality of life.

The professional quality training ofthree orientedpersonnel training mode is one of the three core, which requires medical students to have lifelong learning ability. This study mainly describes the proposed concept of lifelong learning ability, expounds the necessity of lifelong learning ability, and analyzes the connotation, objective and strategies of improving the medical students'!lifelong learning ability under the framework of three oriented medical talents training mode. The training strategy has been discussed mainly from five aspects such as strengthening medical humanistic education, creating a lifelong learning atmosphere in campus, optimizing teaching methods, reform the course of career development and employ-ment guidance, reforming the course of career development and employment guidance, reforming the teaching of medical literature retrieval course and establishing the personal growth archives.

Objective To explore the correlation between the change of regional cerebral blood flow (rCBF) and brain tissue injury from interictal epileptic activity in patients with epilepsy. Methods Fortyeight patients with epilepsy and 30 healthy persons were included in the styudy from which the serum S100β protein levels were determined by double antibody sandwich ELISA method. SPECT rCBF imaging was performed in all patients. The visal and semi-quantitative analyses were used to analyze the epileptic foci.SPSS 11.0 was applied for variance and linear correlation analyses. Results Serum S-100β in patients with interictal epileptic activity was significantly higher than that in control group ((0.572 ± 0. 163) μg/Lvs (0.218 ±0. 134) μg/L, t =9.96, P <0.01). According to epilepsy control criteria, 20 cases achieved complete control (CC), 18 cases acheived partial control (PR). However, 10 cases got no improvement,whose serum S-100β protein ((0. 809 ±0. 056) μg/L) and the percentage change of rCBF ((0. 337 ±0. 060) %) were significantly higher than those of CC ((0.443 ± 0. 083) μg/L, (0.035 ± 0.038) %) and those of PC ((0.585 ±0. 108) μg/L, (0. 187 ±0.075)%), F=56. 740, 92. 316, P <0.01. There were high correlation between serum S-100β and the percentage change of rCBF in epilepsy patients (r =0. 887,P<0.01). Conclusion Serum S-100β protein assay combined with rCBF on SPECT imaging can make semi-quantitative diagnosis of epilepsy and help evaluate the brain damage from interictal epileptic activity.

Objective:To study the association between non-syndrome cleft lip and palate (NSCL/P)and MAFB (rs6072081, rs6065259,rs17820943,rs11696257)gene polymorphism in NingXia Hui and Han population.Methods:512 cases of NSCL/P,258 case of Hui nationality and 254 of Han,174 cases of complete 3 core family members(patients and their parents),142 cases of single parents families(patients and their single parents),were collected in patient group.505 cases of healthy newborns were collected in control group.The MAFB gene SNPs were determined with TaqMan SNPs genotyping methods and the data were analyzed by case-con-trol analysis,transmission disequilibrium test(TDT)and family based association test(FBAT).Results:Case-control analysis found that,there was a statistical significance of the genotypes and allele frequency between patients and the controls in MAFB of the 4 locus (P <0.05);but all of the 4 locus showed no difference in patients with cleft palate(P >0.05).Conclusion:The 4 locus single nucle-otide polymorphism was associated with NSCL/P in Ningxia population.

Objective The present study aimed to trace the behavior results and event-related potential (ERP) of conflict monitoring from 7 ～ 12 years old to explore the development features of the conflict monitoring.Methods In six groups of 144 children aged from 7 to 12,behavior and non-target N2 amplitude were analyzed in continuous performance test (CPT) task. Results 1. Behavior results:the reaction time of target stimulus decreased ( ( 533.33 ± 66.65 ) ms, (523.91 ± 92.96 ) ms, (468.37 ± 64. 13 ) ms, ( 46 1.48 ± 98.31 ) ms, (457.57 ±84.05 ) ms, (405.02 ± 67.90) ms) and the hitting number increased ( ( 34.87 ± 4.84 ), ( 37.64 ± 3.54 ), ( 37.95± 2.92 ), (38.67 ± 1.23 ), (39.31 ± 1.08 ), ( 39.45 ± 1.00 ) ) as age increased, and the difference was statistically significant ( P＜0.01 ). 2. ERP: ①The non-target N2 amplitude was significantly higher than the target,and the difference was statistically significant (F= 98.57, P＜ 0.01 ). ②The amplitude of non-target N2 amplitude decreased with age, and the difference was statistically significant (F= 5.54, P＜ 0. 01 ). Conclusion The non-target N2 was closely related to the monitoring conflict, and the behavior and ERP results in this study showed the development trend. 8 ～ l0 and 12 years old are the critical development period of information processing speed, attention and conflict monitoring function for children.