Hutchinson-Gilford progeria syndrome (HGPS) is a rare disease mainly characterized by accele-rated aging, with an incidence rate of 1 in 8 million to 1 in 4 million.It can affect the skin, fat, cardiovascular, bone and other organ systems.Most HGPS children can only live to 6-20 years old, with an average life expectancy of only 14.6 years.HGPS has distinctive clinical features, such as severe growth retardation, special skin manifestations, and craniofacial manifestations.The prognosis of this disease is poor, and no treatment has been proven effective so far.Upon the diagnosis, the progress of the disease should be observed and monitored via long-term and careful follow up, so as to extend the life span of the children as much as possible.In this article, the disease type, clinical manifestations, pathogenesis and clinical examination of HGPS were reviewed.

Background and purpose:To improve the quality of life of patients with advanced non-small cell lung cancer is one of the problems which the oncologists have to be aware of. Gefi tinib has been used to treat advanced non-small cell lung cancer. We studied the effect of gefi tinib in the improvement of quality of life of patients with advanced non-small cell lung cancer. Methods:There were 70 patients with advanced non-small cell lung cancer treated in our cancer center. One oral gefi tinib tablet(250 mg) was administered every day without interruption unless disease progression or unacceptable toxicity occurred. The impact of treatment on disease-related symptoms and quality of life(QoL) were evaluated with the Chinese versions of European Organization for Research and Treatment of Cancer Quality of Life Questionnaires(EORTC QLQ-C30 and QLQ-LC13) . Results:58 patients finished the questionnaires. The mean scores of fi ve functional scales(physical,role,emotional cognitional and social) were 63,56,68,65,61 respectively after eight weeks of treatment,compared to 52,49,64,60,52 respectively before treatment,and the mean score of global QoL after and before treatment were 60 and 53 respectively. There were statistical differences in fi ve functional scales and global QoL(P

HIV-1 integrase (IN) is an essential enzyme for retroviral replication. There is no analogue for this enzyme in human cells so that inhibition of IN will not bring strong effect on human body. Thus, HIV-1 IN has become a rational target for therapy of AIDS. This review provides a comprehensive report of alpha, gamma-diketo IN inhibitors discovered in recent years. Compilation of such data will prove to be beneficial in developing QSAR, pharmacophore hypothesis generation and validation, virtual screening and synthesis of compounds with higher activity.

Humans ; Meniere Disease ; diagnosis ; therapy ; Vertigo

Objective:To analyze the accuracy of the digital imaging fiber optic transillumination (DIFOTI) on diagnosis of caries lesions depth using DIAGNOcam system.Methods:This experiment adopted self-matching design.Seventy-four extracted teeth (molar:sixty-six,premolar:eight) with one caries lesions in proximity which were not damaged in surface marginal ridge were selected.Dental calculus and dental stains were removed from the extracted teeth for standby application.A sign was marked in the middle of the occlusal surface edge at the side of decay.Then the teeth were fixed in the standard model of dentition and cavities were adjacent with the sound tooth surface.Sticky wax was applied to seal the level of 2 mm beyond cemento-enamel junction (CEJ) in the direction of occlusion and interproximal space to imitate gingival margin and gingival papilla.The standard models of dentition was seated in imitation head mold.The lesions depth degree was looked into and checked with DIAGNOcam system.Besides,the pictures on the occlusal surfaces were recorded and saved.The sign above could be seen on the picture.The measuring tool in DIAGNOcam system was used to measure the depth of the caries from the sign (as starting point) to the deepest point of caries in the pictures and its length was recorded for a.The line a was lengthened to the contralateral edge of occlusal surface in the photo and the length was recorded for b.A line from the marked point on the occlusal surface edge of the extracted teeth was draw parallel to the line b on the corresponding photo and its length was recorded for c.The depth of the cavities on the projected images was recorded for d,and calculated d/a =c/b (digital optical fiber measured decay depth/caries damage depth of the image =actual tooth width/tooth width of the image),and d =c/b × a inferred.At last,the teeth were taken out from the standard model dentition.The decay of the tooth was removed completely.The actual depth of the cavity was recorded for D.The difference between d and D was recorded for Δd.The software of SPSS 20.0 was used to test the consistency of the results,and the MedCalc 14.8.1.0 software was used for Bland-Altman analysis.Results:The intraclass correlation coefficient (ICC) between d and D was 0.951 (ICC ＞ 75 ％),P =0.263.There was a function relationship y =0.23 ± 0.9 1x between d (x) and D (y).Bland-Altman analysis method showed that the mean of Δd (Δd) was 0.05 mm,the standard deviation of Δd (ΔdsD) =0.308,and the 95％ confidence interval was (-0.55 to 0.65).The amplitude of difference was clinically acceptable.So the consistency of the two measurement modes was high.Conclusion:There was no significant difference between the depth of caries lesions checked with DIAGNOcam system and the depth of the actual cavity,and the consistency was very good.The vitro study suggests that the DIAGNOcam system may be used to assess the depth of caries cavity as a useful tool in diagnosis and treatment.

Aim To observe the effects of activation of aldehyde dehydrogenase 2 ( ALDH2 ) by ethanol on testis injury of type 2 diabetic rats. Methods Type 2 diabetic rats model were established by high-fat diet combined with low-dose streptozotocin ( STZ ) injec-tions. After the success of modeling, the rats were ran-domly divided into 3 groups ( n =6 ): normal control group (NC), type 2 diabetes group (T2DM) and eth-anol+type 2 diabetes group (EtOH+T2DM). Rats of EtOH + T2DM were treated with low-dose ethanol, then rats of NC and T2 DM were given normal diet for 8 weeks. After 8 weeks, the levels of the fasting blood glucose ( FBG ) , glycosylated hemoglobin ( HbA1 c ) and testosterone were tested, and the ratio of testis weight to body weight ( TW/BW ) was calculated. Morphological changes of testis tissue were observed by optical microscope. The levels of ALDH2 mRNA and transforming growth factorβ1 ( TGF-β1 ) mRNA in tes-tis tissue were measured. The expression of TGF-β1 in testis tissue was observed by immunohistochemical stai-ning, then the positive rate of TGF-β1 was calculated. Results Compared with NC, the levels of FBG, HbA1 c and TW/BW increased significantly and the level of testosterone decreased significantly in T2DM. The morphological observation showed that some semi-niferous tubules atrophied, spermatogenic cells de-creased and arrangemented loosely, Leydig cells de-creased in testicular interstitial. The level of ALDH2 mRNA in testis tissue decreased significantly, and the level of TGF-β1 mRNA and the positive rate of TGF-β1 in testis tissue increased significantly. However, compared with T2DM, the levels of FBG, HbA1c and TW/BW decreased, and the level of testosterone in-creased and the damage of testis tissue was attenuated in EtOH+T2DM. The level of ALDH2 mRNA in testis tissue increased significantly, and the level TGF-β1 mRNA and the positive rate of TGF-β1 in testis tissue decreased significantly. Conclusion Activating AL-DH2 can protect testis in type 2 diabetic rats, which may be related to the downregulation of TGF-β1 ex-pression.

Background and purpose:Hypoxia induced the decreased radiosensitivity of tumor cells, which was the cause of tumor radioresistance and relapse and metastasis. During the course, HIF-1a played the most important role in the regulation of hypoxia. However, it’s still unknown about the effect of HIF-1a on the radiosensitivity of hypoxia tumor cells and the relationship with autophagy. This study was to inhibit HIF-1αexpression in hypoxic lung adenocarcinoma cell line A549 with RNA interference (RNAi), and explore its effect on hypoxic cell radiosensitivity and autophagy. Methods: Plasmids pHIF-1α-shRNA and Neg-shRNA (negative control) were constructed and transfected into hypoxic A549 cells, this positive clone was named A549/HIF-1α-shRNA. Clone formation array was applied to calculate the value of D0, SF2, SER. The expression of HIF-1α, LC3, c-parp was detected by Western blot. Results:The SF2 of hypoxic A549 cell was 0.62, which was higher than that of normoxic A549 cell, SER was 1.45. The level of LC3Ⅱincreased significantly and the level of c-parp decreased after the radiation of hypoxic A549 cell. The level of HIF-1a increased in hypoxic A549 cells. The expression of HIF-1αin hypoxic A549 cells was suppressed markedly after transfection of HIF-1α-shRNA;this clone was named A549/HIF-1α-shRNA. The SF2 and SER were significantly lower in A549/HIF-1α-shRNA cells, 0.45 and 0.72 respectively. Under the hypoxic condition and after the inhibition of HIF-1α, the expression of LC3Ⅱ decreased significantly and the expression of c-parp increased. Conclusion:We successfully established a cell model that HIF-1αexpression was suppressed almost completely by RNAi. The inhibition of HIF-1αby shRNA may raise the radiosensitivity and decrease the autophagy of hypoxic A549 cells in vitro.

Objective To evaluate the status of self-efficacy and its influencing factors in adult patients with low vision,then explore an effective approach to improve their self-efficacy levels.Methods Seventy adult patients with low vision were sampled by random number table method.General social-demographic questionnaire and self-efficacy questionnaire were used to investigate these patients' general information and self-efficacy.Seventy pieces of questionnaires were issued and returned with the valid rate of 100％.Results The total score of self-efficacy in seventy adult patients with low vision was(16.25:±:1.93) points,92％ of patients had a low level of self-efficacy.The influencing factors of self-efficacy in adult patients with low vision were the attitude toward disease,mental status and gender.Conclusions The selfefficacy in patients with low vision was affected by many factors at different levels,so we should focus on main influencing factors to adopt corresponding nursing measures in order to improve self-efficacy of adult patients with low vision.

Objective To investigate the protective effect of somatostatin (SS)combined with growth hormone (GH) in treatment of intestinal mucosal barrier injury in rabbits with severe acute pancreatitis (SAP), as well as its clinical significance. Methods Seventy-two rabbits were equally assigned into model group (SAP group), SS treated group (SS group) and SS combined with GH treated group (SS + GH group). SAP models were induced by retro-injection of 5% sodium taurocholate into the pancreatic duct. After modeling, all rabbits were given 5 % glucose saline daily.The rabbits in SS group and SS+GH group were continuously Given SS (3.5μg·kg-1·h-1)for 48 hours. Besides, the rabbits in SS+GH group were subcutaneously injected with 0.15 IU/kg of GH at the 1st and the 24th hours after modeling. The levels of serum amylase, serum tumor necrosis factor-α (TNF-α) and plasma diamine oxidase were measured at the 6th, 12th, 24th and 48th hours after modeling. The pathological changes of pancreatic tissue and ileal mucosa were observed. Survival rate was calculated. Data were analyzed using SPSS 16.0 software. The univariate analysis was used to compare the difference among groups. Results In SS+GH group, the levels of serum TNF-α and plasma diamine oxidase were (2. 43 ± 0. 14) pg/ml and (4. 61 ± 0. 45) U/L at the 24th hour respectively, and were (2.08±0.23) pg/ml and (3.75±0.47) U/L at the 48th hour, respectively,which were lower than those in SAP group and SS group [(2.80 0.30) pg/ml and (8.74 ± 1.77)U/L, respectively, at the 24th hour; (2. 45±0.12) pg/ml and (5. 02±0.95) U/L, respectively, at the 48th hour)]with significant difference (P＜0.05). The inflammation in pancreas and ileal mucosa was alleviated, and the integrity of bowel mucosa was improved. Survival rate of SS+GH group was significantly higher than SAP group and SS group. There was no significant difference in level of serum amylase between SS+GH group and SS group. Conclusion The combination of SS with GH may enhance the function of intestinal mucosa barrier and improve the prognosis of SAP in rabbits.

Objective To investigate the clinical characteristics, diagnosis and treatment of placenta increta.Methods A retrospective analysis was carried out on 13 admitted cases of placenta increta from 1989～2006. Results Among the 13 cases analysed, 5 cases with a history of Caesarian section had a 0% success rate of treatment with conservative care (0/5), 100% less than that of cases with no history of Caesarian section (8/8), P＜0.05; the success rate of treatment of partial placenta increta with methotrexate with Jia Wei Sheng Hua Tang was 100%. Conclusion Caesarian section is a risk factor of placenta increta, and its prognosis is poor; however, Jia Wei Sheng Hua Tang has proven satisfactory as a supplementary treatment for placenta inereta.