Objective To analyze and summarize the characteristics of Dyskeratosis congenita(DC)in Chinese children,so as to provide reference for early diagnosis and reasonable prevention and treatment of DC.Methods The data collected from 43 cases with DC in the domestic literature in recent 10 years,as well as the clinical data of 2 cases with DC treated at Tongji Hospital,Medical School,Tongji University,and a total of 45 cases with DC were analyzed and compared with those reported in the literatures of foreign countries.Results Based on the detailed data of 2 typical cases at Tongji Hospital,Medical School,Tongji University,and the data of 45 cases in China were summarized,so the major differences in the main characteristics of DC between Chinese Children and the foreigners were as follows:(1)Onset were earlier(mean age 4.5 years,median age of 3 years),but the diagnosis was delayed(mean age of 17.9 years,median age of 18 years).(2)The presence of skin pigmentation,nail lesions and mucosal leukoplakia,such as the proportion of the complete DC triad was higher(42/45 cases,93.3％).(3)There was an earlier onset of hematopoietic suppression(mean age 5.6 years).(4)Telomerase related gene mutation types were relatively minor,DKC1(7 cases)and TINF2(6 cases)were reported in recent years,and no other type of mutation was found.(5)Effective therapy of hematopoietic reconstitution was administered in 2 cases after allogeneic hematopoietic stem cell transplantation(allo-HSCT).The effective rates were about 70％(7/10 cases)in treating bone marrow failure with low dose androgen and low dose glucocorticoid.Conclusions DC is very common in infants in China,the clinical manifestations of triad are more typical,but the age of diagnosis was significantly delayed.Improving the understanding of DC and combination with the detection of related gene mutation may improve the early diagnosis rate and clinical efficacy with allo-HSCT or effective drug maintenance therapy,and also provide reference for propitious familial eugenics and prenatal examination.

Health resources allocation, the cornerstone of health production capability, includes effective allocation of increments and rational readjustment of stocks. Using the least square method and variance analysis, the authors created a multilinear regression model on the basis of the calculation method and analysis principle of the Cobb-Douglas production function and in light of historical data in China's health sector and made a positive analysis of the current state of health resources allocation in China The result indicates that the input in health human resources has been insufficient and their contribution to production has been on the moderate side. Moreover, health resources allocation has shown decreasing return to scale on the whole.

0.05).The volume and Hct of suspension RBC and leukocyte depleted suspension RBC made by the above two methods were(144.01?11.54) ml,(152.45?15.39) ml,(158.71?10.69) ml and 0.48?0.03,0.50?0.03,0.53?0.05,respectively,and all the differences had statistical significance(P

Resistance is one of the most important reasons that restrict the clinical application of most chemotherapeutic medicines. Docetaxel is a very widely applicated antitumor medicine. Most of the researches on the mechanism of resistance against docetaxel focused on the drug transporters, changes in drug metabolism and pathway alteration of cell cycle and apoptnsis. The mechanism of docetaxel resistance and the predictive data based on clinical research to docetaxel therapy in cancer treatment were reviewed.

The epiphysis is an unique structure in pediatric skeletons,which mainly reflect in its unique blood supply,anatomical structure,pathological basis and so on.Epiphyseal injury is common in children with skeletal injury.Fracture,tumor,infection can cause epiphysis injury,and inappropriate treatment is often easy to cause growth obstacle and skeletal deformity.Its treatment is relatively difficult.At present,there had not yet formed a consistent therapeutic strategy.Aiming at the pathobiologic basis,causes,mechanism and clinical manifestation of pediatric epiphyseal injury,now,its diagnosis,therapeutic strategy,the principle of treatment and the latest research progress were summarized,which would provide useful guidance for clinical treatment of pediatric epiphysis injury.

AIM: To investigate effect of autogenous conjunctival flap transplantation in the treatment of severe fungal corneal ulcer.
METHODS: A total of 83 cases ( 83 eyes ) with severe fungal corneal ulcer treated in our hospital from Jan. 2013 to Jan. 2016 were selected. Among them, 36 cases ( 36 eyes) were treated with drug therapy, and 47 patients(47 eyes ) were treated with autogenous conjunctival flap transplantation, two group did fungal culture and identification, observed the two groups treatment effect.RESULTS:The effective rate of surgical treatment group was 89%, significantly higher than that of drug treatment group, the difference was statistically significant ( P <0.05 ); The effective rate of surgical treatment of filamentous bacteria was 92%, which was significantly higher than that of drug treatment, the difference was statistically significant(P<0. 05). The effective rates of surgical treatment and drug treatment of yeast were 80%and 78%, the difference was not statistically significant( P<0. 05).
CONCLUSION: Autologous conjunctival flap transplantation in the treatment of severe fungal corneal ulcer has good curative effect. The operation is simple, worthy of recommendation method. Filamentous bacterial corneal ulcer without good drug therapy effect, surgical treatment should be performed in a timely manner to improve the curative effect.

15 year literatures from databases such as MEDLINE, PUBMED, and CNKI were reviewed. The foundings indicate that there are a lot of scales to assess diabetic specific quality of life, but no one can involve the following aspects: body function, body structure, activity and participation, and environmental factors except ICF core sets for diabetes mellitus which involve the four aspects listed above. Nowadays an international multi-center validation study of the ICF core sets for 12 chronic conditions is ongoing, and so far there has been no report on the validation of the ICF core sets for diabetes mellitus yet. Through the validation study of the ICF core sets for diabetes mellitus, it is anticipated that the ICF core sets for diabetes mellitus can comprehensively describe health and functional status of diabetic patients, thus can create a valid and common tool or language to assess diabetes mellitus. It will facilitate communication between different disciplines and countries, provide effective interventions and finally improve health and functional level of diabetic patients.

Objective:To explore the relationship between ventilator-associated pneumonia (VAP) and neutrophil/lymphocyte ratio (NLR) before mechanical ventilation in patients with malignant tumors.Methods:A retrospective nested case-control study was conducted. Patients with malignant tumor treated by mechanical ventilation admitted to the Third Affiliated Hospital of Guizhou Medical University from February 2015 to February 2020 were enrolled. The patients with VAP were selected as the case group, and the matched non-VAP cases were selected according to 1∶2 as the control group. The clinical data were collected, and the differences of each index between the two groups were compared. The influencing factors of VAP in patients with malignant tumor were analyzed by multivariate Logistic regression.Results:During the study period, 1 271 patients with malignant tumors were treated with mechanical ventilation, of which 241 cases had VAP, and the incidence of VAP was 18.96%. There were 232 VAP patients in the case group matched 464 non-VAP patients in the control group. The clinical data of age, gender, hospitalization diagnosis, primary tumor, regional lymph node and distant metastasis (TNM) stage, acute physiology and chronic health evaluation Ⅱ (APACHEⅡ), complications, duration of mechanical ventilation, hemoglobin (Hb) and serum albumin (Alb) levels were balanced and comparable between the two groups, and the cluster nursing measures were consistent. Compared with the control group, although there was no significant difference in neutrophil count (NEU) and lymphocyte count (LYM) in the case group [NEU (×10 9/L): 3.81±1.07 vs. 3.64±1.05, LYM (×10 9/L): 2.06±0.59 vs. 2.15±0.62, both P > 0.05], NLR was significantly increased (2.07±1.05 vs. 1.89±0.96, P < 0.05), and the hospital stay was significantly longer (days: 24.84±3.81 vs. 13.19±3.98, P < 0.01). NLR, gender, age, APACHEⅡ score, TNM stage, Hb, serum Alb and duration of mechanical ventilation were included in multivariate Logistic regression analysis. The results showed that patients with elevated NLR had higher risk of VAP [odds ratio ( OR) = 1.187, 95% confidence interval (95% CI) was 1.015-1.387, P = 0.032]. In patients with VAP, NLR was negatively correlated with the time of mechanical ventilation before VAP (r = -0.327, P = 0.000), and positively correlated with the time of treatment with antibiotics after VAP (r = 0.559, P = 0.000). Conclusion:Elevated NLR in patients with malignant tumors who were on mechanical ventilation can significantly increase the risk of VAP and increase the difficulty of treatment.

ABSTRACT:Objective To study the BDNF/TrkB signaling pathway in the epilepsy model of hippocampal neurons and the regulatory effect of on it.Methods The primary hippocampal neurons cultured in vitro for 7 days were randomly divided into seven groups:control group,epilepsy group,control+BDNF group,epilepsy+BDNF group,control + miR-185 ? group,epilepsy + miR-185 ? group,and epilepsy + miR-185 ? + BDNF group.We constructed miR-185 ? lentivirus vector and observed the changes of BDNF/TrkB pathway expression after transfaction of miR-185 ? by immunohistochemistry,patch clamp technique and Western blot technique.Results Compared with the control+BDNF group,the phosphorylated TrkB (pTrkB)/TrkB value was significantly lower in epilepsy+BDNF group (P < 0.05 )and control group (P < 0.001 ).Compared with the epilepsy group,the phosphorylated TrkB (pTrkB)/TrkB value was significantly higher in epilepsy+BDNF group (P <0.05).Compared with the epilepsy+miR-185 ? +BDNF group,the phosphorylated TrkB (pTrkB)/TrkB value was significantly lower in epilepsy + BDNF group and epilepsy + miR-185 ? group (P < 0.001 ).BDNF could promote the signaling conduction and miR-185 ? could remove the inhibition of BDNF/TrkB signaling.Conclusion BDNF can activate the BDNF/TrkB signaling pathway and transfection with miR-185 ? can relieve the inhibition of BDNF/TrkB signaling pathway of epileptic state by up-regulating the expression of TrkB.

OBJECTIVETo observe the effect of total ginsenosides (TG) on monocrotaline (MCT) induced right ventricular hypertrophy rats, and to explore its correlation with calcineurin (CaN) pathway.

METHODSFifty male Sprague Dawley rats were randomly divided into the normal control group, the MCT model group, and the low, middle, high dose TG treatment groups, 10 in each group. All medication was performed by peritoneal injection for 18 days. Right ventricular peak systolic pressure (RVSP), right ventricular hypertrophy index (RVHI), and right ventricular weight/body weight (RVW/BW) were measured. Intracellular free calcium concentrations were measured by Ca2+ fluorescence indicator Fura2/AM. The atrial natriuretic factor (ANF) and CaN mRNA expression of the myocardial tissue were quantitatively analyzed by Real-time PCR. The protein expression of CaN was detected by Western blot.

RESULTSCompared with the MCT model group, preventive treatment of TG at the 3 doses could significantly reduce RVSP, RVHI, RVW/BW, and ANF mRNA expression, and decrease Ca2+ concentration in myocardial cells, CaN mRNA and protein expression in the myocardial tissue.

CONCLUSIONTG could obviously improve MCT-induced right ventricular hypertrophy, which was possibly achieved through suppressing MCT-activated CaN signal transduction.

Animals ; Atrial Natriuretic Factor ; Calcineurin ; metabolism ; Calcineurin Inhibitors ; therapeutic use ; Ginsenosides ; therapeutic use ; Heart Ventricles ; Hypertrophy, Right Ventricular ; drug therapy ; metabolism ; Male ; Monocrotaline ; Myocardium ; Myocytes, Cardiac ; RNA, Messenger ; Rats ; Rats, Sprague-Dawley ; Signal Transduction