Objectives To explore the long-term prognosis, treatment (especially dietary treatment) of classic maple syrup urine disease. Methods The complications and dietary treatment were observed by follow-up study of a classic MSUD patient. Results The patient have obvious damage in nervous system. However, reasonable dietary leucine tolerance therapy after the neonatal period can effectively reduce the metabolic crisis and complications. A mutation in BCKDHB gene was detected in the patient by genetic testing. Conclusion It is suggested that dietary restriction and monitoring of branched-chain amino acids are helpful to reduce the development of acute metabolic crisis and complications and improve the quality of life.

Objective To explore the concentrations and diameters of the particulates in indoor air of the cosmetic workshops, and provide scientific basis for the testing and evaluation of air cleanliness of the cosmetic workshops. Methods The testing and evaluation on concentrations of the particulates with the diameters of 0.3,0.5,1.0,2.0,3.0,5.0 ?m in indoor air of the cosmetic workshops was carried out among 11 cosmetic manufactures with air filtering and purifying system(air-purified factory) and 13 cosmetic manufactures without air filtering and purifying system but with mechanical ventilation (air-unpurified factory). Results The medians of 0.3～5.0 ?m particulates in air of cosmetic workshops in air-purified factories were significantly lower than those in air-unpurified factories respectively. The concentrations of the particulates in air of cosmetic workshops in air-unpurified factories showed no significant differences compared with those in ambient air.The concentrations of 0.5 and 1.0 ?m particulates showed the significant correlation with those of 0.3?2.0?3.0?5.0 ?m particulates. The concentrations of 2.0?3.0?5.0 ?m particulates showed the significant correlation between each other except for 0.3 ?m particulates. Conclusion The particulates show lower concentration, the air cleanliness doesn't meet the requirement the national standard of 100 000 class in air of cosmetic workshops in air-purified factories. The concentration of 0.5 ?m particulate is suitable for evaluation on air cleanliness in air of cosmetic workshops.

Objective To prepare and characterize glycyrrhetinic acid cationic liposomes.Methods Liposomes were prepared by ethanol injection technique.An orthogonal test was utilized to optimize the formulation and preparation of glycyrrhetinic acid liposomes.The unencapsulated glycyrrhetinic acid and liposomes were separated by Sephadex gel G-50,the encapsulation efficiency was detected by HPLC.The morphological examination of glycyrrhetinic acid liposomes was performed using transmission electron microscopy.The particle size and Zeta potential of the liposomes were measured.The release rate of glycyrrhetinic acid from liposomes was tested.Results The liposomes with spherical or ellipsoidal shape and better stability featured the encapsulation efficiency of(91.61?1.16)%,the mean partical size of(141?10)nm,and Zeta potential of(35.9?5)mV.The in vitro release kinetics were consistent with Higuchi equation.The stability of glycyrrhetinic acid liposomes was better.Conclusion The selected formulation and preparation technic of glycyrrhetinic acid liposomes could be rational,stable,and with a sustained feature in vitro release.

Objective To determine the encapsulation efficiency of glycyrrhetinic acid(GA) liposomes and to study the release of GA from the liposomes in vitro.Methods GA liposomes were prepared by ethanol injection.The unencapsulated glycyrrhetinic acid and liposomes were separated by sephadex gel G-50,and the encapsulation efficiency was detected by HPLC.The release behavior of the drug was studied by the third method for dissolution issued in Chinese pharmacopoeia(published in 2005).Results The encapsulation efficiency of liposomes was 91.61%,and the in-vitro drug-release curve was accorded with Higuchi equation.Conclusion The GA liposomes have high encapsulation efficiency and sustained-release effect.

Glioma is the most common form of brain cancer. Despite recent advances in the treatment of solid tumors, there are few effective treatments for malignant gliomas due to its infiltrative nature. It has important significance to improve the treatment of glioma through in-depth understanding the intracerebral metabolic characteristics and pharmacokinetics of chemotherapeutics. Brain microdialysis (B-MD), an effective method to monitor central nervous system anticancer drug disposition, conditions of drugs through the blood-brain barrier, basic pathophysiologic metabolism, bioactive compounds and the changes of neurotransmitter in brain, provides the unique opportunity to allow the simultaneous determination of unbound concentrations of drugs in several tissues, and directly measure gliomas biochemistry continuously. B-MD has been able to monitor the change of brain drugs, metabolites and neurotransmitters, dynamic analysis of the drug concentration and pharmacological effect after administration, pharmacodynamic interaction between drugs, receptor mechanism of drug transport, as well as feedback information of internal environment. B-MD is expected to provide reference for clinical individual chemotherapy of glioma, but also provide powerful tools for the evaluation of new anticancer drugs in vivo. In this review, a comprehensive overview of B-MD for studies on glioma is elucidated with special emphasis on its application to neurochemistry and pharmacokinetic studies.
Animals ; Antineoplastic Agents ; pharmacokinetics ; Blood-Brain Barrier ; Brain Neoplasms ; metabolism ; Glioma ; metabolism ; Humans ; Magnetic Resonance Spectroscopy ; Metabolomics ; methods ; Microdialysis ; methods ; Neurotransmitter Agents ; pharmacokinetics ; Pharmaceutical Preparations ; metabolism ; Positron-Emission Tomography

Objective To evaluate the value of using fluorescence in situ hybridization (FISH)technique for the detection of chromosome aberration of urine exfoliated cells for the diagnosis of bladder tumor.MethodsFISH technique were used to detect the abnormalities of chromosome 3,7,17 and 9p16 site from 20 normal people, and to establish the threshold.The morning's first urinations were available from 75 patients with bladder cancer and 25 patients without urothelial tumor, then were detected using FISH technique and urine cytology respectively.The sample was considered positive if two or more probes results higher than the criteria,or one probe has two or more abnormal results.Results The sensitivity of single using were 73.3％ (55/75),76.0％ (57/75),62.7％ (47/75) and 62.7％ (47/75) for the 4 probes (3,7, 17 and 9p16)respectively.The sensitivity of combined detection was 85.3％ (64/75) and specificity was 96.0％ (24/25) The sensitivity and specificity of urine cytology examination was 9.3％ (7/75) and 100％ (25/25) .The sensitivity of FISH examination was significantly higher than that of urine cytology examination (85.3％ vs 9.3％ ,x2 = 57.00, P ＜ 0.001) .Sensitivity of FISH examination was not correlated with cancer pathologic grading(low vs high : 84.2％ vs 86.5％, x2 = 0.08, P ＞ 0.05)and clinical stage (ta-tl : 82.9％, t2-t4 :87.5％, x2 = 0.32 ,P ＞ 0.05) .ConclusionFISH technique is a non-invasive and effective method for the early diagnosis of bladder tumor and is more sensitive than urine cytology.Furthermore, FISH technique can be used to predict the tumor's biological behavivor and prognosis.

Objective:To observe and analyze the therapeutic effect and safety of tirofiban combined large dose statin in patients with acute myocardial infarction (AMI),and evaluate its influence on myocardial perfusion and cardiac function.Methods:A total of 97 consecutive AMI patients visited to our hospital from May 2011 to January 2013, who were beyond the emergency PCI time window (≥12h),their chest pain was remissive or not further aggravated were studied.All patients were pumped with tirofiban for 48h continuously;according to combined rosuvastatin dose,they were divided into large dose group (n = 52,20mg,once/d,until one month after infarction,then changed to routine dose of 10mg,once/d)and routine dose group (n=45,10mg,once/d).All patients received se-lective PCI after 7 ~ 10d conservative treatment.Myocardial perfusion level,left ventricular ejection fraction (LVEF)on one week after PCI and 30d after AMI,enzymology changes [creatine (CK),lactate dehydrogenase (LDH)]and major adverse cardiovascular events (MACE)during hospitalization were compared between two groups.Results:Compared with routine dose group,there were significant reductions in thrombus scores in infarct related artery (IRA)[(1.32±1.01)scores vs.(0.81±0.78)scores]and corrected TIMI frame [(32.4±4.73)vs. (26.8±2.34)]in large dose group (P =0.021,P <0.001);after selective PCI,TIMI flow of large dose group was significantly better than that of routine dose group (P =0.024).On one week after PCI,LVEF:(51.4±8.9)% of large dose group was significantly higher than that of routine dose group (47.7±8.7)%,P =0.021;there were no significant difference in levels of CK and LDH between two groups on 7d and 30d after PCI (P >0.05).There was no MACE in both groups during hospitalization and 30d after PCI.Conclusion:Tirofiban combined large dose statin is safe and effective in patients with acute myocardial infarction,it can reduce intra-coronary thrombus burden,im-prove myocardial tissue perfusion and cardiac function without increasing MACE.

Objective To analyze the feasibility and the diagnostic yield of double-balloon enteroscopy (DBE) examinations for small bowel bleeding (SBB). Methods A retrospective analysis was conducted on 52 cases with small bowel bleeding between June 2015 and July 2016,and all was treated with DBE. therapeutic outcome, complications and follow-up were compared. Results The study included 52 patients (28 males and 24 females) with an average age of (51.0 ± 17.0) years (16~82 years) and onset time (8.3 ± 4.0) days (1~14 days) . 25 using oral route and 21 using the anal route, 1 using the colon ifstula route, a combination of using oral and anal (n=4).The bleeding source was identiifed in 40 of 52 patients (76.9%), complication rate was 5.8%(3/52), and rebleeding rate was 16.7%(2/12). The endoscopic treatments included polypectomies (n=5), argon plasma coagulation (APC, n=2), surgical treatment (n=14, 26.9%), and foreign-body extraction (n=1). Patients were diagnosed with the following:tumors (n=9, 17.3%), ulcers (n=9, 17.3%), Crohn’s disease (n=7, 13.5%), polyps (n=5, 9.6%), diverticulum (n=4, 7.7%). Patients with small bowel bleeding were followed up for a mean period of (8.3±2.0) months (range 4~10 months), 2 deaths were dying from small bowel cancer. Conclusions DBE is a safe endoscopic technique for patients with small bowel bleeding and can be safely carried out even after Roux-en-Y operation. Tumors, ulcers and Crohn’s disease are very common causes of SBB. The rebleeding rate after a negative DBE is considerable, especially small bowel vascular lesions.

Objective Objective To analysis the clinical curative effect on elderly patients with choledocholithiasis by endoscopic retro-grade cholangiopancreatography ( ERCP) and endoscopic sphincterotomy ( EST) and discuss its clinical significance. Methods 256 patients with choledocholithiasis were collected for diagnosis and treatment by ERCP. The stone characteristics was mastered by ERCP and the pa-tients were treated with EST. Results Patients with choledocholithiasis were successful in 249 cases (97. 27%) for ERCP intubation and 239 cases (93. 36%) for stone remove. After EST,the stones were removed successfully at one time in 199 cases (83. 26%),2 times in 40 cases (16. 74%). 7 patients (2. 73%) with multiple diverticulum and papillary position poor by intubation failure were changed over to op-eration treatment. After ERCP,patients were performed endoscopic nasobiliary drainage (ENBD) 64. 44% (154/239). Pull out the drain-age tube of 90. 91% (140/154) of the patients after stones disappeared which was proved by angiography for 3~5 days. 14 cases (9. 09%) were performed second times to remove the stones due to the presence of residual stones. Postoperative complications occured in 15 cases (6. 02%) including 9 cases of acute pancreatitis and 6 cases of infection of biliary tract, and they were cured after 1 week of corresponding treatment. Transient increase of serum amylase occurred in 39 cases, and all of them recoveried after 3 days without special treatment. Con-clusion The results showed that ERCP ( or EST) were well tolerated by elderly patients with choledocholithiasis,and they were of obvious curative effect and quick recovery,which is worthy of promotion and application.

Objective To investigate the feasibihty and effectiveness of the fluorescence in situ hybridization(FISH)technique in the diagnosis of renal pelvic tumor.Methods FISH technique were used to detect the abnormalities of chromosome 3,7,17 and 9 p16 from 20 people without malignancy.The morning's first urine were availahled from 15 patients with hematuria and suspected of renal mass by imaging,then were studied by FISH technique and urine cytology respectively.The sample was considered positive if two or more probes results were higher than the criteria,or one probe had two or more abnormal results.Results Eight of 15 patients with hematuria were renal pelvic urothehal carcinoma,5 were renal clear cell carcinoma,1 was renal chromophobe cell carcinoma,1 was xanthogranulomatous pyelonephritis,confirmed by pathology.All of the 8 renal pelvic carcinoma were considered positive by FISH technique,while only 1 was considered positive by urine cytology.The variation rate of chromosome 3,7,17 were 100%(8/8)and chromosome 9 p16 was 75%(6/8).Six of 7 non-renal pelvic carcinoma were negative by FISH technique,but all Were negative by urine cytology.Conclusion FISH technique is a non-invasive and effective method for the diagnosis of renal pelvic tumor,and it's more sensitive than urine cytology.