Objective To investigate the clinical and laboratory profiles of a patient with pustular psoriasis-like skin lesion and cerebral palsy due to biotinidase deficiency. Methods A 5 year and 4 month-old boy with biotinidase deficiency was confirmed by urinary organic acid analysis with gas chromatography/mass spectrometry (GC/MS)and biotinidase activity assay of peripheral blood. His clinical features, laboratory findings, treatment and outcome were studied. Results The boy showed difficulty in taking food after birth, gradually eczema and pustules appeared at the age of 2 months, and generalized erythema and intractable pustular psoriasis-like lesion at the age of 8 months. His intellectual development was normal with retardation of locomotor system. He had muscular dystonia at the age of 6 months. Physical examination showed generalized pustular psoriasis-like lesion, generalized paralysis, hypertonic contracture of extremities, sparseness of scalp hair and severe malnutrition. Routine laboratory tests showed a mild anemia, metabolic acidosis and elevation of plasma creatine phosphokinase. Increased excretion of urinary lactate, pyruvate, 3-OH-propionate, propionylglycine, and 3-methylcrontonylglycine were observed. Biotinidase activity of his peripheral blood was below 0.1 pmol/min/3mm (normal 6.3-9.3 pmol/min/3mm). Biotin (10 mg/day) supplementation led to a dramatic recovery of the skin lesion. After the treatment of rehabilitation, his muscle power was also improved gradually. Conclusions Dermatological and neurological manifestations are the main features of biotinidase deficiency. Early diagnosis and biotin administration can greatly improve the clinical symptoms. Generalized pustular psoriasis-like lesion and cerebral palsy of this boy have improved after the supplementation of biotin, but he may be remained wheelchair-dependent because of delayed diagnosis.

OBJECTIVE: To study ApoB gene genetic polymorphism of Han nationality and Mongolian nationality in midwest area of Inner Mongolia. METHODS: Some unrelated individuals of Han nationality and Mongolian nationality in midwest area of Inner Mongolia were selected. Polymerase chain reaction-restriction fragment length polymorphism technology was used to check the presence of Xba I (X+) and EcoR I (E-) sites of rare alleles. The genotype frequency, allelic frequency and population genetics parameters were calculated. RESULTS: The frequencies of Xba I (X+) and EcoR I (E-) rare alleles were 2% and 4.6% in Han population. There was no Xba I (X+) or EcoR I (E-) rare alleles found in Mongolian nationality. CONCLUSION The allelic frequencies of ApoB gene Xba I and EcoR I sites are very different in different races. These sites may be used in identification of ethnicity.
Alleles ; Apolipoprotein B-100/genetics* ; Asian People/genetics* ; China ; Ethnicity ; Gene Frequency ; Genotype ; Humans ; Mongolia ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Restriction Fragment Length

OBJECTIVETo develop a RP-HPLC method for simultaneous determination of three active compounds, dictamnine, obacunone and fraxinellone in root bark of Dictamnus dasycarpus and supply a reference for the establishment of the quality standard of D. dasycarpus.

METHODA Kromasil C18 column was used with methanol-water (60:40) as the mobile phase, at the flow rate of 1 mL x min(-1). 236 nm was selected as the detected wavelength.

RESULTThe determined three compounds were well separated with a linear range of 0.0021-0.1060, 0.0201-0.9200 and 0.0102-1.020 g x L(-1), respectively. The recoveries of them were 100.5%, 99.2% and 100.2%, respectively.

CONCLUSIONThis method is simple, rapid and accurate, particularly suitable for the quality control of D. dasycarpus.

Chromatography, High Pressure Liquid ; methods ; Chromatography, Reverse-Phase ; methods ; Dictamnus ; chemistry ; Plant Bark ; chemistry ; Plant Extracts ; analysis ; Plant Roots ; chemistry

BACKGROUNDPrevious studies showed that the prognostic factors for superficial transitional cell carcinoma of the bladder varied with the findings of different cohorts. Few multivariate analyses of prognostic factors for superficial bladder tumors have been reported in China and bladder preservation as a prognostic index of superficial bladder tumors is limited and scarce in Chinese patients. This study was conducted to analyze a group of risk factors for prognostic outcomes for patients with primary superficial transitional cell carcinoma of the bladder.

METHODSBetween January 1980 to December 2000, 198 patients [172 men and 26 women; mean age (52.98 +/- 11.28) years] with primary superficial transitional cell carcinoma who were pathologically classified as Ta or T1 in Hunan Provincial Tumor Hospital (Changsha, China) were enrolled in this study. Surgical methods included local resection and electric coagulation of bladder tumors, transurethral resection of bladder tumors and partial cystectomy. After initial surgical treatment, patients were followed through a cystoscopy every three months during the first two years and every six months thereafter in the design of retrospective cohort. Survival analysis was performed to analyze risk factors of the prognostic outcomes for transitional cell carcinoma of the bladder. Canonical correlation analysis was conducted to present and interpret synthetically the multi-correlation between all kinds of prognostic outcomes and risk factor in multiply dimensions.

RESULTSThe average follow-up period was (6.65 +/- 4.74) years. Assessments at three, five, and 10 years showed recurrence rates, respectively, of (28.32 +/- 3.45)%, (35.31 +/- 3.83)%, and (42.48 +/- 4.40)%; progression rates of (8.89 +/- 2.14)%, (15.16 +/- 2.94)%, and (23.88 +/- 4.19)%; bladder-preservation rates of (94.68 +/- 1.74)%, (93.87 +/- 1.91)%, and (91.51 +/- 2.49)%; metastasis rates of (8.25 +/- 2.05)%, (11.24 +/- 2.47)%, and (28.94 +/- 4.93)%; and cancer-related survival rates of (95.02 +/- 1.62)%, (90.70 +/- 2.45)%, and (77.14 +/- 4.88)%. The main risk factors for recurrence were histological grade, blood transfusion during surgery and the duration of symptoms. Progression was affected by blood transfusion during surgery, histological grade, the number of re-examinations, and the length of the recurrence-free period. Metastasis was associated with tumor multifocality, hydronephrosis, microscopic growth pattern, and the recurrence-free period. Cancer-related survival was influenced by microscopic growth pattern and the recurrence-free period. Bladder preservation involved only the recurrence-free period. The comprehensive results from canonical correlation analysis showed that the main prognostic outcomes were cancer-related survival, metastasis and progression respectively, while the dominate risk factors were histological grade, tumor multifocality and blood transfusion.

CONCLUSIONSThe risk factors were different for each prognostic outcome of transitional cell carcinoma of the bladder. This is helpful for predicting the prognosis of transitional cell carcinoma of the bladder and designing therapeutic and follow-up strategies for this cancer.

Adult ; Aged ; Carcinoma, Transitional Cell ; mortality ; pathology ; Cohort Studies ; Disease Progression ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; mortality ; Prognosis ; Retrospective Studies ; Risk Factors ; Transfusion Reaction ; Urinary Bladder Neoplasms ; mortality ; pathology

OBJECTIVETo find out the current situation of physical activity and its influencing factors among Chinese primary and middle school students.

METHODSA total of 166 812 students aged 9 - 18 finished the questionnaires of physical activities and lifestyle behaviors who were selected from "2010 National Physical Fitness and Health Surveillance". The subjects were sampled by stratified cluster sampling method in 30 provinces (autonomous regions, municipalities) except Tibet. The physical activity for a total of at least 60 minutes per day, attitudes on physical activity, parents' supports toward physical activity of children, development of school physical education, the schoolwork burden and sedentary lifestyle were all analyzed. Logistic regression was used to analyze the association between the physical activity for a total of at least 60 minutes per day and individual, family, school and sedentary behavior.

RESULTSA total of 22.7% (37 867/166 757) of primary and middle school students aged 9 - 18 had been physically active doing any kind of physical activity for a total of 60 minutes or more per day, the prevalence was higher among male (25.4%, 21 086/83 159) than female (20.1%, 16 781/83 598) students (P < 0.05). In the 9 - 12 age group, the prevalence was higher among urban (37.6%, 5 980/15 912) than rural (32.0%, 4 917/15 375) students (P < 0.05), but in the 16 - 18 age group, the case was opposite. The prevalence of doing physical activity for a total of 60 minutes or more per day among students who liked physical education (23.7%, 11 866/50 073), were willing to participate in the extracurricular sports activities (23.2%, 13 460/57 907), and were willing to participate long running exercise (26.9%, 9 185/34 153) was higher than those who dislike physical education (15.9%, 589/3705), were not willing to participate in the extracurricular sports activities (15.9%, 567/3565), and were not willing to participate long running exercise (12.1%, 2 246/18 493), respectively. The prevalence was higher among students whose parents supported their physical activity (20.4%, 15 148/74 312) than those students without parents' support (17.5%, 1626/9270) (P < 0.05). And the prevalence was higher among students whose parents often took part in the physical activity (22.7%, 12 864/56 769) than those students whose parents did not (14.6%, 3 906/26 780) (P < 0.05). Logistic regression analysis (adjusted sex, age and urban or not) demonstrated that variables associated with physical activity for a total of 60 minutes or more per day were the initiative will on the physical activity and parental physical activity (OR: 1.478, 95%CI: 1.398 - 1.562). The students without adequate physical education class in school were less likely to do physical activity for a total of 60 minutes or more per day than those who with adequate physical education class in school (OR: 0.536, 95%CI: 0.523 - 0.550), and the students in the school with the phenomenon of "crowding out" or "no" physical education were less likely to do physical activity for a total of 60 minutes or more per day than those whose school without these with phenomenon (OR: 0.712, 95%CI: 0.666 - 0.761). The heavy homework burden (OR: 0.926, 95%CI: 0.899 - 0.953) and long homework time (OR: 0.879, 95%CI: 0.853 - 0.906) were reversely related to the physical activity.

CONCLUSIONThe prevalence of having been physically active for a total of 60 minutes per day in Chinese students is still low. The strong initiative will on the physical activity, parental physical activity, and better school sports atmosphere are good for students to carry out physical activity, and the students with the heavy homework burden and long homework time are less likely to take part in the physical activity.

Adolescent ; Child ; China ; Exercise ; Factor Analysis, Statistical ; Female ; Health Surveys ; Humans ; Life Style ; Male ; Physical Fitness ; Sedentary Lifestyle ; Students ; Surveys and Questionnaires

AIMTo identify the factors influencing diagnosis and treatment of chronic prostatitis (CP) among Chinese urologists.

METHODSA sample of 656 urologists from 29 provinces of China were asked to complete a questionnaire that explored attitudes towards CP as well as diagnosis and treatment patterns in the management of CP. Both univariate and multivariate logistic regression analysis schemes were used to determine the factors that influence the diagnosis and treatment of CP.

RESULTSA total of 656 questionnaires were given out. All were returned and 410 of those were included in the final univariate and multivariate analysis. Multivariate logistic regression analysis indicated that belief of bacterial infection in the etiology of CP (odds ratio [OR], 2.544; 95% confidence interval [CI], 1.650-3.923; P < 0.001) was the most significant factor influencing the routine performance of bacterial culture test. Using the same model, the type of hospital (OR, 2.799; 95% CI, 1.719-4.559; P < 0.001) and the routine use of the 4- or the 2-glass test (OR, 3.194; 95% CI, 2.069-4.931; P < 0.001) were determined to be significant factors influencing the use of the National Institutes of Health (NIH) new classification system. According to the same model, belief of bacterial infection in the etiology of CP (OR, 3.415; 95% CI, 2.024-5.762; P < 0.001) and the routine use of bacterial culture test (OR, 2.261; 95% CI, 1.364-3.749; P < 0.01) were important factors influencing the routine prescription of antibiotics.

CONCLUSIONOur findings suggest that attitudes towards CP, and the characteristics of individual urologists' practices may influence the diagnosis and treatment of CP among Chinese urologists.

Adult ; Aged ; Anti-Bacterial Agents ; therapeutic use ; China ; Chronic Disease ; Cross-Sectional Studies ; Health Knowledge, Attitudes, Practice ; Health Surveys ; Humans ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Practice Patterns, Physicians' ; Prostatitis ; diagnosis ; drug therapy ; microbiology ; Surveys and Questionnaires

OBJECTIVETo develop a public health index related to the comprehensive assessment on the impact caused by floods.

METHODSA Analytic hierarchy process (AHP) theory was used to establish the initial evaluation system on the impact of floods. Modified-Delphi process was used to screen and determine the indicators and their weights while synthetical scored method was used to establish the comprehensive assessment model. Percentile was used to differentiate the degree of floods. Finally, analysis of variance (ANOVA) and correlation analysis were used to test the differentiability of the model for different degree of floods and the independence of these indicators.

RESULTSThe model of comprehensive assessment on the impact of floods was set up, including six first-ranking indicators and twenty-four sub-indicators. The values of comprehensive assessment were divided into five grades by the percentiles. Verified results indicated that there were significant difference among the five grades (F = 76.11, P < 0.01) and all indicators were independent.

CONCLUSIONAn index of comprehensive assessment on the impact of floods was established, which could be used to evaluate the impact of floods and to differentiate the degree of flood, which seemed to have the characteristic of reliability, comprehensiveness and practicability.

Analysis of Variance ; China ; Delphi Technique ; Disasters ; Health Status Indicators ; Humans ; Models, Theoretical ; Public Health ; standards ; statistics & numerical data ; Research ; statistics & numerical data ; Research Design

OBJECTIVETo discuss the effect of limited head and neck squamous cell carcinomas human papillomavirus(HPV) infection status on the prognosis in different nationalities of Xinjiang.

METHODSThe clinical data of 149 cases of head and neck squamous cell carcinoma from Uighur, Han and Kazak was analyzed. The HPV16/18 infection and viral load was examined.The prognosis was analyzed by cox multiIvar-Iate model. The effect of HPV infection status on prognosis was evaluated.

RESULTSIn this study, Oropharyngeal HPV infection rate was 35.0%, followed by hypopharynx 30.0%, oropharyx was 16.0%. The overall survival rate of 3 years and 5 years was 66.4%, and 39.2% respectively. The clinical stage,N stage and HPV were influencing factors for the prognosis of patients with head and neck squamous cell carcinoma (P< 0.05). N stage and HPV were independent prognostic factors for the prognosis of patients with head and neck squamous cell carcinoma (P< 0.05).

CONCLUSIONSThere is a high rate of HPV infection in oropharynx in head and neck cancer. HPV positive is a protective factor for the prognosis of head and neck cancer, and the risk of death in patients with HPV was 3/5 lower than that of HPV negative patients. HPV viral load may be positively related to the total survival rate. N stage is a risk factor for the prognosis of head and neck cancer. Different nationalities have little influence on prognosis.

Carcinoma, Squamous Cell ; ethnology ; mortality ; pathology ; virology ; China ; Ethnic Groups ; Head and Neck Neoplasms ; ethnology ; mortality ; pathology ; virology ; Human papillomavirus 16 ; Human papillomavirus 18 ; Humans ; Neoplasm Staging ; Papillomavirus Infections ; diagnosis ; mortality ; virology ; Prognosis ; Proportional Hazards Models ; Survival Rate ; Time Factors ; Viral Load

Objective To study the HIV-1 drug resistance (DR) situation among newly infected persons in Dehong.Methods 1048 HIV-1 positive blood samples from July to December in 2006 from Dehong prefecture of Yunnan,were collected.HIV drug resistance were tested using TruGene in newly infected people that were distinguished with BED-CEIA,while the subtype were determined with phylogenetic analysis using a set of reference sequences available on the Los Alamos Database.Results Of sixty-four successfully analyzed samples,drug resistance mutations were detected in 4 samples with the resistance rate as 6.25%.Minor mutation in PR region such as M36I/V,L63P and H69K appeared frequently and the rates were 81.25%,70.31%and 65.63%respectively.The predominantly prevalent strains were seen as C/CRF07_BC/08_BC(65.63%,42/64) in this study.Conclusion The prevalence of genotypic drug resistances in HIV-1 recent infections in Dehong prefecture appeared to be at moderate level.Drug-resistance surveillance program among HIV-1 infections should be continued and strengthened.

In intensity-modulated radiation therapy (IMRT), it is time-consuming to repeatedly adjust the objectives manually to obtain the best tradeoff between the prescribed dose of the planning target volume and sparing the organs-at-risk. Here we propose a new method to realize automatic multi-objective IMRT optimization, which quantifies the clinical preferences into the constraint priority list and adjusts the dose constraints based on the list to obtain the optimal solutions under the dose constraints. This method contains automatic adjustment mechanism of the dose constraint and automatic voxel weighting factor-based FMO model. Every time the dose constraint is adjusted, the voxel weighting factor-based FMO model is launched to find a global optimal solution that satisfied the current constraints. We tested the feasibility and effectiveness of this method in 6 cases of cervical cancer with IMRT by comparing the original plan and the automatic optimization plan generated by this method. The results showed that with the same PTV coverage and uniformity, the automatic optimization plan had a better a dose sparing of the organs-at-risk and a better plan quality than the original plan, and resulted in obvious reductions of the average V45 of the rectum from (41.99∓13.31)% to (32.55∓22.27)% and of the bladder from (44.37∓4.08)% to (28.99∓15.25)%.