Maturity-onsetdiabetesoftheyoung(MODY),amonogenicformofdiabetes,hasmorethan8 well-known subtypes:MODY1( hepatocyte nuclear factor - 4A,HNF4A ),MODY2( glucokinase,GCK ),MODY3 (hepatocyte nuclear factor-1A,HNFIA),MODY4(pancreatic and duodenal homeobox,PDX),MODY5(hepatocyte nuclear factor-1B,HNFIB),MODY6(neuronal differentiation 1,NEUR0DI),MODY7(carboxyl ester lipase,CEL) and MODY8( insulin,INS). The molecular pathology,clinical manifestations and treatment of MODYs are different and reviewed.

The pathogenesis of obesity in children is unclear.Genetic play an important role,including gene mutations,polymorphisms,epigenetics.And the other hand,environment factors such as intrauterine environment,nutrition,physical exercise,and gut microflora also affect the obesity.The genetic and environment factors have interaction,leading to the occurrence of childhood obesity and development.With the advances in molecular biology techniques and large-scale,large sample size of population screening,new obesity-related genes,single nucleotide polymorphisms,the apparent genetic markers will continue to be found,looking forward to the future predict obesity,to choose to guide effective treatment,or even contribute to the development of genetic targeting drugs.

Objective To explore the effect of high positive end-expiratory pressure (PEEP) for the treatment of neurological pulmonary edema (NPE) in patients undergoing mechanical ventilation,and to look for the best mechanical ventilation strategy to improve the prognosis.Methods A prospective study was conducted,and 120 patients with NEP admitted to Department of Critical Care Medicine of the First Affiliated Hospital of Guangxi Traditional Chinese Medical University from January 2010 to August 2013 were enrolled and divided into two groups according to random number table (n=60 in each group).The patients in two groups were given empiric treatment for the disease,and they underwent mechanical ventilation.In the normal PEEP group PEEP was 3-10 cmH2O (1 cmH2O =0.098 kPa),and in the high PEEP group PEEP was 11-30 cmH2O,and all the rest parameters were the same.Clinical indices before and 7 days after treatment,and 28-day morality rate were compared between two groups.Results The 28-day morality rate in high PEEP group was obviously lower than that in the normal PEEP group [25.0％ (15/60) vs.65.0％ (39/60),x2=6.465,P=0.011].The clinical signs in both groups were improved after treatment.Compared with the normal PEEP group,the clinical indices in high PEEP group were more significantly improved.There were significant differences in body temperature (℃ 37.4 ± 0.5 vs.38.5 ± 0.6),respiratory rate (times/min 18.3 ± 3.1 vs.23.3 ±3.5),heart rate (beats/min 94.7 ±8.5 vs.113.5 ±8.0),white blood cell count [WBC (× 109/L) 12.5 ±2.1 vs.17.1 ± 1.7],acute physiology and chronic health evaluation Ⅱ (APACHE Ⅱ) score (15.6 ± 3.2 vs.19.8 ± 3.7),Glasgow coma score (GCS 12.5 ± 2.1 vs.8.5 ± 2.9),gastrointestinal dysfunction score (3.9 ± 3.0 vs.3.6 ± 2.4),oxygenation index [PaO2/FiO2 (mmHg,1 mmHg=0.133 kPa) 196.5 ± 45.1 vs.134.1 ± 22.3],serum creatinine [SCr (μmol/L) 86.5 ± 35.6 vs.98.5 ± 37.7],total bilirubin [TBil (μmol/L) 39.7 ± 23.5 vs.41.5 ± 16.2],C-reacting protein [CRP (mmol/L) 53.7 ± 21.4 vs.108.4 ± 26.3],prothrombin time [PT (s) 15.0 ± 2.1 vs.20.4 ± 2.2],activated partial thromboplastin time [APTT (s):37.3 ±4.9 vs.56.7 ± 13.6],international normalized ratio (INR 2.52 ±0.64 vs.4.01 ±0.77),extra vascular lung water index [EVLWI (mL/kg) 7.53 ± 1.21 vs.15.85 ±3.41],pulmonary vascular permeability index (PVPI 6.07 ± 0.89 vs.9.47 ± 1.26),mean arterial pressure [MAP (mmHg) 87.3 ± 10.9 vs.98.7 ± 13.6],cardiac output [CO (L/min) 7.15 ± 1.42 vs.5.65 ± 1.82],systemic vascular resistance index [SVRI (KP) 112.4 ± 9.5 vs.136.5 ± 11.9],and blood lactate (mmol/L:2.53 ± 1.23 vs.5.81 ± 2.17) between high PEEP group and normal PEEP group (P＜0.05 or P＜0.01).Conclusion Prognosis can be improved in NPE patients with the use of high PEEP in mechanical ventilation.

The practical teaching system for information management and information system science in higher medical institutions was constructed with job skill training as its target by intruding the concept of competence-based education model into it in order to improve the practical performance of undergraduates majoring in informa-tion management and information system science. The system was designed by taking into consideration the teaching contents, teaching methods and teaching practice, and implemented in the undergraduates of Chengdu Medical College, and the implementation experiences were summarized.

[Summary] During recent years, increasing evidences have indicated that type 2 diabetes mellitus(T2DM) might increase the risk of certain tumors; the process might be not only related with the chronic pathologic status of T2DM such as hyperglycemia, hyperinsulinemia, insulin resistance, dyslipidemia, status of chronic inflammation but also associated with the long-term use of anti-diabetic drugs (i. e. sulfonylureas, biguanides, glitazones, dipeptidyl peptidase-4 inhibitors, glucagon-like peptitide-1 receptor agonists), as well as the use of insulin and insulin analogues. Herewith a system review was made about the recent progress in studying T2DM and tumor risk.

Objective To study the clinical effects of Ditan Xingnao Chengqi soup for treatment of patients with sepsis brain dysfunction. Methods Sixty-two cases of sepsis brain dysfunction were divided into treatment group(32 cases)and control group(30 cases)according to random digits table. Both groups received general clinical treatment,while the treatment group additionally accepted Ditan Xingnao Chengqi soup through nasal feeding. The changes of C-reactive protein(CRP)of two groups before treatment and after treatment for 12,24,48,72 hours and 1 week were observed. Besides,the Glasgow Outcome Scale(GOS)was used to assess the curative effect of two groups, and its clinical significance was judged. Meanwhile,the hospitalization costs,the times staying in hospital and total effective rates of the two groups after treatment were observed. In addition,cost-effectiveness analysis was carried out. Results With the extension of therapeutic time,the levels of CRP(mg/L)in the two groups were reduced effectively in comparison with those before treatment,and the change was more obvious in the treatment group;after treatment for 24 hours,the difference in the level of CRP between the two groups began to be statistically significant(106.5±22.3 vs. 201.6±25.4,P<0.05). The GOS of two groups after treatment had been increased markedly in comparison to that before treatment,and the change was more obvious in treatment group(72 hours:10.34±0.76 vs. 7.34±0.48,1 week:13.16±0.86 vs. 9.56±0.64,both P<0.05). Hospitalization costs(yuan)of treatment group in intensive care unit (ICU)was lower than that in control group(43276.16±1844.23 vs. 51347.75±2011.06,P<0.05),and the duration of staying(day)in ICU of treatment group was shorter than that in control group(15.11±2.25 vs. 20.30±2.97,P<0.05). The total effective rate of treatment group was higher than that of control group,but the difference had no statistical significance(87.5%vs. 60.0%,P>0.05).The ratio of cose effectiveness analysis in treatment group was smaller than that of control group(497.42 vs. 855.80,P<0.05). Conclusions Ditan Xingnao Chengqi soup has a relatively good effect on antagonizing inflammatory factors,controlling excessive inflammatory response and improving disturbance of consciousness. The early intervention with this agent for a patient with sepsis cognitive impairment can control the disease situation,improve the patient's prognosis and reduce his or her hospitalization costs.

Objective To explore the effects of estrogen on oxidative stress of the lung tissue induced by acute paraquat (PQ) poisoning. Methods Thirty-two male adult New Zealand rabbits were randomly divided into model group and estrogen intervention group, 16 rabbits in each group. The model of lung injury induced by PQ poisoning was reproduced by feeding 16 mg/kg of 20% PQ through gastric tube. The rabbits in estrogen intervention group received intravenous infusion of 5 mg/kg estrogen after PQ challenge for 7 days, and the rabbits in model group received an equal volume of normal saline. Three rabbits in each group were sacrificed at 1, 2 and 3 days respectively after exposure. The lung tissue was harvested, the levels of reactive oxygen species (ROS) was determined by 2',7'-dichlorofluorescin diacetate (DCFH-DA), malondialdehyde (MDA) was determined by thiobarbituric acid (TBA), the mRNA expression of manganese-containing superoxide dismutase (MnSOD) was determined by reverse transcription-polymerase chain reaction (RT-PCR), and adenosine triphosphatase (ATP) content in mitochondrion was determined by enzyme linked immunosorbent assay (ELISA). The pathological changes in lung were observed under light microscopy using hematoxylin and eosin (HE) staining, and the lung injury was evaluated with lung injury score. Results The contents of ROS and MDA in lung within 3 days after PQ poisoning were gradually increased, and MnSOD mRNA expression and ATP content were gradually decreased. Estrogen intervention could significantly reduce the production of ROS and MDA after PQ poisoning [3-day ROS (fluorescence intensity): 161.05±30.04 vs. 188.30±31.80, 3-day MDA (mmol/L): 98.71±0.92 vs. 122.12±1.24], up-regulate MnSOD mRNA expression (integral A value: 3.05±0.90 vs. 1.22±0.24), and increase ATP content in mitochondrion (ng/L: 3.75±0.92 vs. 2.28±0.29) with statistically significant differences (all P < 0.01). In lung tissue after PQ poisoning, congestion, edema, focal pulmonary consolidation, pulmonary interstitial and alveolar space were infiltrated by a large number of neutrophil, alveolar interval were thickened obviously and the above phenomenon were most serious at 3 days after poisoning as shown under optical microscope. Estrogen intervention could significantly improve lung injury as compared with that of model group, and the lung injury score at 3 days was significantly lower than that of model group (11.8±0.7 vs. 13.5±1.0, P < 0.01). Conclusions The oxidative stress indicators in the lung tissue after PQ poisoning were obviously abnormal, the pathological damage was serious with time dependence. The administration of estrogen can reduce acute lung injury after PQ poisoning by reducing the oxidative stress.

ObjectiveTo evaluate the feasibility of a three-dimensional (3 D) operation planning system for precise hepatectomy in patients with giant hcpatocellular carcinoma (HCC).MethodsThe clinical data of 34 patients with giant HCC who received precise hepatectomy at the First Affiliated Hospital of Sun Yat-sen University from May 2009 to May 2011 were retrospectively analyzed.Preoperative evaluation was done based on the computed tomography data,and the resection margin and the volume of resectcd liver were calculated using simulation.The predicted resected liver volume and resection margins were compared with the results of the actual operations.All data were analyzed using the t test,and the correlation between predicted and the actual values was analyzed by calculating Pearson correlation coefficients.Results Hepatic and tumor tissues and the hepatic vessels were reconstructed three-dimensionally using the 3D preoperative simulation software.The volume of simulated resected liver and the length of resection margin were ( 2112 ± 1550 ) ml and ( 12 ± 6) mm,respectively,while the volume of actual resected liver and the length of resection margin were (2031 ± 1411 )ml and ( 12 ± 6)mm,respectively.The predicted and the actual values were statistically correlated ( r =0.961,0.923,P ＜ 0.05 ).There was no significant difference between the predicted and the actual values ( t =1.549,1.143,P ＞ 0.05 ).All patients received precise hepatectomy,and no hepatic failure or death was observed. Conclusion The 3D operation planning system can accurately evaluate the condition of the liver and simulate hepatectomy,and could contribute to safer and more curative precise hepatectomy in patients with giant HCC.

Objective To investigate the prevalence of obesity and metabolic syndrome(MS)of children and adolescents in Longquan mountainous area in Zhejiang province.Methods A representative sample involving 2 135 children and adolescence aged 10 to 15 years were randomly surveyed and a total of 2 125 had available data(male/female ratios as:1 109/1 016).Using the standard methods,we measured the weight,height,waist circumference,hip circumference,blood pressure,detected fasting plasma glucose (FPG),high density lipoprotein cholesterol (HDL -C),triglyceride(TG),total cholesterol(TC),and calculated non-high density lipoprotein cholesterol(non-HDL). The prevalence of obesity and MS among the 10 to 15 years old children and adolescence in Longquan was compared with that in six cities in China(Beijing,Tianjin,Shanghai,Zhejiang,Chongqing and Guangxi).Results The preva-lence of obesity was 4.7% in 10 -15 -year -old teenage,in which the male obesity prevalence was 6.3%(70/1 109),female obesity prevalence was 3.0%(30 /1 016).The prevalence of overweight was 9.4% and the prevalence of boys was 11.0%,while the girl was 7.6%.The prevalence of obesity and overweight was 16.4%(299/2 125).The prevalence of MS was 2%(42/2 125)in the survey,but 42.0%in the obesity.The prevalence of obesity in Longquan(4.7%)was lower than that in the national six cities(8.1%)among the children and adoles-cents from 10 to 15 years old(χ2 =31.09,P=0.000).But in the obesity students,the prevalence of MS(42.0%)in Longquan was higher than that in six cities(28.8%)(χ2 =5.43,P=0.02).Conclusion The prevalence of obesity in Longquan mountainous area was lower than that in the national six cities among the children and adolescents from 10 to 15 years old.However,the prevalence of MS among the obesity in Longquan was higher than that in six cities.

The clinical electroencephalogram (EEG) monitoring systems based on personal computer system can not meet the requirements of portability and home usage. The epilepsy patients have to be monitored in hospital for an extended period of time, which imposes a heavy burden on hospitals. In the present study, we designed a portable 16-lead networked monitoring system based on the Android smart phone. The system uses some technologies including the active electrode, the WiFi wireless transmission, the multi-scale permutation entropy (MPE) algorithm, the back-propagation (BP) neural network algorithm, etc. Moreover, the software of Android mobile application can realize the processing and analysis of EEG data, the display of EEG waveform and the alarm of epileptic seizure. The system has been tested on the mobile phones with Android 2. 3 operating system or higher version and the results showed that this software ran accurately and steadily in the detection of epileptic seizure. In conclusion, this paper provides a portable and reliable solution for epileptic seizure monitoring in clinical and home applications.
Algorithms ; Cell Phone ; Electrocardiography ; Electroencephalography ; instrumentation ; Entropy ; Epilepsy ; diagnosis ; Humans ; Monitoring, Physiologic ; instrumentation ; Neural Networks (Computer) ; Software