Background Leber hereditary optic neuropathy (LHON) is a maternally inherited disorder characterized by a bilateral acute or subacute painless central visual loss in young adults,predominately in males.So far no one theory can completely explain all clinical manifestations of LHON.Objective This study was to investigate whether there is a linkage between X-chromosomal and mitochondrial mutation in the inheritance of a Chinese LHON pedigree with only male patients.Methods This study was approved by Ethic Committee of Affiliated First Hospital of Zhengzhou University and followed by Declaration of Helsinki.A Chinese LHON pedigree was included in Anyang city from January 2008 to August 2016.Periphery blood of 5-10 ml was collected from 4 sufferers,13 maternal members and 10 non-maternal members for DNA extraction and PCR sequencing.The gene scanning and genotyping analysis were performed by ABI-PRISM 3100 genetic analyzer and Genotyper 3.7 software,and linkage analysis was carried out with Linkage software for the calculation of logarithm of odds (LOD).Mitochondrial DNA (mtDNA) sequence,fluorescence-based Genescan for X-chromosomal sequence were analyzed in the propositus and haplotype was evaluated.Results A total of 5 generations and 71 families were included in the pedigree,with 6 male sufferers,30 maternal members and 41 non-maternal members.The visual acuity was ≤0.10,and the central visual field defection,the optic nerve flushing was found in the acute phase,different levels of the optic nerve fibers atrophy were found in the chronic phase;visual evoked potential (VEP) amplitude was low and peak latency were found in the male patients,and no any ocular abnormality was seen in the maternal members,meeting a maternally inherited characteristics,with the penetranee of 20％.The three primnary mutations were not been found in this family bv PCR sequencing,mtDNA sequencing appeared 31 variation of loci in the proband,including a known G3635A mutation,as well as an unknown ND5 A12340G missense mutation and ND4 T11809C synonymous mutation as well as 28 polymorphism of locus,and the proband was mitochondrial haplotype F1.The maternal families were mutation carriers of G3635A and AI2340G loci,while the same mutation was not found in the normal family members and 107 controls.The maximum two point parametric LOD score was 1.46(θ=0.0) for marker DXS1060,1oeated at Xp22.3,and the two-point and multipoint non-parametric linkage analysis were significant (all at P＞0.05).Conclusions The ND5 A12340G and ND1 G3635A mutations coexist in this LHON family,and the ND5 A12340G mutation is a newly reported mutation.There is no evidence for an X-linked modifiers loci in this Chinese LHON family.

Objective To Apply appropriate dose of Glutamine(Gln)(300mG/d)for patients with acute cerebral infarction,and observe its preventinG and curinG function to the stress ulcer. Methods Sixty cases patients of acute cerebral infarction were observed and divided into the conventional treatment Group( 30 cases),Glutamine treatment Group( GLU,30 cases). Gastric pH value of patients was tested before treatment and after one week treatment. The stool or vomit occult blood positive( OB+)number of patients within one week, OB( +) duration and the number of patients need blood filterinG were observed. Results There was no siGnificant difference between the conventional treatment Group and the GLU Group in the Gastric juice PH value before treatment(t=0. 647,P>0. 05). After one week treatment,the Gastric juice PH value of the GLU Group was siGnificant hiGher than the conventional treatment Group(t=5. 25,P<0. 05);the number of OB( +)of GLU Group were less than the conventional treatment Group,but there was no statistical siGnificant difference(χ2=0. 577,P>0. 05);the OB( +)duration of GLU Group was less than the conventional treatemnt Group,and there was satistical siGnificant difference(t =4. 768,P <0. 05);the number of blood filterinG of GLU to treat stress ulcer was less than conventional treatment Group,and there was satistical siGnificant difference(χ2=l. 0l6,P>0. 05). Conclusion SupplementinG appropriate dose of Glutamine in patients with acute cerebral infarction can reduce the incidence of stress ulcer,decrease medical expenses.

Objective To investigate morphosis of the epiphyseal plate and the expression of vascular endothelial growth factor (VEGF) in the epiphyseal plate during healing of injured epiphysis.Methods Thirty SD rats aged 4-5 weeks were used to make models of proximal tibia epiphysis injury.These models were equally divided into 5 groups in random,with 6 rats in each group,and these groups of rats were killed separately at 2,4,6,10,21 days postoperatively to harvest the proximal tibia epiphysis,in order to explore the morphosis of the epiphyseal plate and VEGF expression pattern in the epiphyseal plate at different healing phase.The structure of the epiphyseal plate was evaluated by measuring the length of the limb and radiographic examination of the limb.The ratio of length of injured tibia and that of the other side was used to describe the tibia length discrepancy.The micro structure of the epiphyseal plate and situation of the bone bridge in the epiphyseal plate were measured by using Micro CT.By using HE stain and VEGF immunohistochemistry,the changing of chondrocyte and VEGF expression pattern in the epiphyseal plate were observed at different healing phase.Results The limb discrepancy appeared at 4 days postoperatively,became biggest at 10 days postoperatively,and tended to decrease at 21 days postoperatively.Micro CT demonstrated the fibrous bone tissue formation in the epiphyseal plate at about 6 days postoperatively,which became bone bridge finally.HE stain showed chondrocyte in the rest zone had the tendency to aggregate after injury,and the highly ordered structure of chondrocyte in the epiphyseal plate no longer existed,chondrocyte differentiation was accelerated.VEGF immunohistochemistry stain showed the high reaction of VEGF in the epiphyseal plate appeared after surgery,and the positive zone of VEGF expanded through the physis with time,which finally led to angiogenesis and ossification.Conclusion A serial alteration of morphosis of the epiphyseal plate occurred during the healing process.Fibrous bone tissue formation in the epiphyseaL plate could be observed in the early and mid-term of the healing process.With progress of healing,VEGF expression zone gradually expanded across the epiphyseal plate,which is related to the formation of bone bridge.

AIM:To explore the preliminary mechanism of senegenin ( Sen) on inhibiting hypoxia/reoxygenation ( H/R)-induced apoptosis of primary cortical neurons .METHODS:The cultured cortical neurons were randomly divided in-to normal group (control group), model group (H/R group), Sen+H/R group and Sen group.Flow cytometry was used to evaluate the effect of Sen on H/R-induced cell apoptosis .The protein levels of JNK , p-JNK, c-Jun, p-c-Jun, Bcl-2 and Bax were assessed by Western blotting .RESULTS:The apoptotic rate in H/R group was obviously higher than that in control group (P<0.05), while the apoptotic rate in Sen +H/R group was obviously lower than that in H/R group (P<0.05), suggesting that the model of apoptosis was established successfully .The results of Western blotting showed that Sen increased the expression of JNK and c-Jun, inhibited the phosphorylation of JNK and c-Jun (P<0.05), increased the protein level of Bcl-2 and inhibited the protein level of Bax in H/R treated primary cortical neurons (P<0.05).CONCLUSION:Sen has a protective effect against H/R-induced neuronal apoptosis by increasing the expression of JNK and c-Jun, inhibiting the phosphorylation of JNK and c-Jun, increasing the protein level of Bcl-2 and decreasing the protein level of Bax .

BACKGROUND:Metabolic syndrome is based on sugar, fat and other metabolic disorders and central obesity, hypertension as features in a series of syndrome. The traditional treatment is not yet possible to fundamental y improve and cure metabolic syndrome.
OBJECTIVE:To provide an overview of the research progress of stem celltransplantation in the treatment of metabolic syndrome.
METHODS:The first author retrieved PubMed database and CNKI database for articles regarding basic research on progress of stem celltransplantation in the treatment of metabolic syndrome, insulin resistance, diabetes, hyperlipidemia and hypertension published from 2002 to 2012. The key words were“stem cells, metabolic syndrome, insulin resistance, diabetes, hyperlipidemia, hypertension, stem cells transplantation”in English and Chinese, respectively. Outdated and repetitive studies were excluded, and 43 literatures were included for summarization.
RESULTS AND CONCLUSION:Stem cells are the origin of the body cells, and have self-replicating, highly value-added and differentiation capacity. Stem celltherapy can promote a variety of damage repair and renew aging or death of cells, so as to improve the structure and function of tissues and organs, and to promote the utilization and excretion of metabolites. Studies have shown that stem cells can treat lipid metabolism, insulin resistance, hypertension, hyperglycemia, atherosclerosis and other hazards of metabolic syndrome disorders through a variety of mechanisms. There are many problems to be solved in the treatment of metabolic syndrome with stem celltransplantation. But the existing research data have been confirmed, and stem celltransplantation in the treatment of the metabolic syndrome is a promising new approach.

Objective To study the characteristics of hepatic artery anatomy and master the method of arterial repair,especially the method and technology of repair of anomalous donor arteries.Methods Sixty-four(human) liver arteries were dissected and repaired,and 31 of these were used in liver transplantation.Results Twelve of 64 cases had anatomical anomaly of hepatic artery(12/64,18.75%).Five of 24 fetal(liver) donors(20.83%) had anatomical anomaly of hepatic artery,one arose from the superior mesentery(artery) as a replaced right hepatic artery,3 from accessory right hepatic artery,and in 1 case the hepatic(artery) arose from superior mesentery artery directly.Seven adults had hepatic artery anomalies:2 cases of right hepatic artery being replaced by artery derived from SMA,2 cases of left hepatic artery deriving from left gastric artery and 3 cases of accessory right hepatic artery derived from SMA.In 31 hepatic(transplantations),4 grafts with anatomical anomaly of right hepatic artery were used.Conclusions (Reconstruction) of hepatic artery plays an important role in hepatic transplantation.Avoidance of injury to(aberrant) hepatic avteries during removal of donor liver is a key to ensure successful arterial repair.During(repair) of very small accessory hepatic arteries,ligation or repair of the artery may be determined after(observation) of backflow from collateral vessels.

Objective To investigate the risk factors associated with early mortality (within 30 days) of patients with spontaneously ruptured hepatocellular carcinoma (SRHCC) and assess the efficacies of different treatment regimens. Methods The clinical data of 28 patients with SRHCC who had been admitted to our hospital from January 1999 to January 2006 were retrospectively analyzed. Multiple factors that might cause early mortality were determined, and the efficacies of different treatment regimens were assessed. Results Eleven patients died within 30 days. with the mortality rate of 39%. Univariate analysis showed that shock, Child C status, hemoglobin. ALT, and volume of blood transfusion were associated with early mortality (X2=3.020, 13.741, Z=-2.059, -2.210, -4.153, P<0.05). Child C status and volume of blood transfusion were the independent risk factors. All the patients were divided into hepatectomy group (8 cases), surgical hemostasis group (7 cases), transarterial embolization group (7 cases) and conservative group (6 cases). Patients in hepatectomy group had a better 30-day and median time survival rate compared with those in the other 3 groups (X2=8.098, 8.098, 15.025, Z=-3.127, -2.840, -3.004, P<0.05). Conclusions Poor hepatic functional reserve and severe hemorrhage are closely associated with early mortality of patients with SRHCC. Hepatectomy is a better choice for SRHCC.

Objective:To explore the influence of buccal corridor of Han people on smile esthetics.Methods:An attractive adult male and a adult female were selected as the models.Buccal corridor was altered digitally with slider technology of Adobe Flash CS4 to obtain a continuous range of buccal corridors(0% -25%).96 orthodontists aged 35.1 ±7.2 years and 96 laypersons aged 37.3 ± 5.1 years were chosen as the raters.The minimum tolerable value(A%),the ideal value(B%)and the maximum tolerable value (C%)of buccal corridor of the models were statistically analyzed.Results:In the orthodontist groupA,B and C of the male model were 5.00 ±0.1 7,9.75 ±2.77 and 1 5.00 ±2.84,in layperson group were 4.79 ±1 .00,9.20 ±3.08 and 1 5.05 ±2.91 ,respec-tively;in orthodontist group,A,B and C of the female model were 3.92 ±0.1 7,1 1 .87 ±2.77 and 1 5.82 ±2.84,in layperson group were 4.00 ±1 .00,1 2.05 ±3.08 and 1 5.1 1 ±2.91 ,respectively(all data between groups,P >0.05).The ideal buccal corri-dor value(%)of the male and female models were 9.48 ±2.73 and 1 1 .96 ±1 .99 respectively(P <0.05).Conclusion:There is no difference between orthodontists and laypersons for buccal corridor esthetic judgment.The ideal esthetic buccal corridor size of male and female is different.

The quality of a donor liver after cardiac death is closely associated with energy metabolism during preservation. Ex vivo mechanical perfusion has broad application prospects because this technique can help energy metabolism and repair ischemia injury of donors' livers. Some core issues are presented in this review in order to provide references for propelling secure application of liver transplantation based on donation after cardiac death.
Death ; Humans ; Liver ; Liver Transplantation ; Organ Preservation ; methods ; Perfusion ; methods ; Warm Ischemia ; adverse effects

Objective To investigate the impact of synthetic urine (synurine solution) at differentpH values on skin surface.Methods Sixty-four healthy volunteers were enrolled into this study.Based on the results of lactic acid test and questionaires,these subjects were enrolled as sensitive skin group and normalskin group.The 4-,20-,and 24-hour occlusive patch tests were successively performed with synthetic urine at various pH values (2.0,3.5,5.0,6.5,8.0 and 10.0)on the two groups of volunteers.The distilled waterand 0.25%sodium lauryl sulphate (SLS)served as control.The skin surface pH values were measured bypotable pH meter before,and at 24 h,48 h,72 h after the first patch.Half an hour after the latter three detections of pH values,the skin response was evaluated clinically,as well as by transepidermal water loss (TEWL)and pH values,which were measured by pH-900 pH Meter.Results The responses to these solutions were similar between sensitive and normal skin groups.The order of skin surface pH values measured by the two pH meters was consistent with that of the original solutions.The skin surface pH value was altered by the synthetic urine,and this change was still present at 24 hours after the patches were removed.The TEWL values of skin challenged by synthetic urine were not higher than those by distilled water.The skin responses at 72 hours were more intense to the synthetic urines with pH values of.5,8.0,10.0than to distilled water.CondusionsSynthetic urines with different pH values can alter skin surface pH values,and skin responses to synthetic urines with a pH range 2.0-10.0 are similar to those to distilled water.