Objective To report clinical manifestations, electroencephalogram (EEG), and the genotypes of two siblings with type Ⅲ Gaucher disease.Methods Two patients with different features were siblings. Their clinical data, signs, peripheral leukocytes acid β-glucosidase activity, andGBA gene were analyzed.Results (1) The proband was a boy. He visited us at the age of nine years old because of hepatosplenomegaly, thrombocytopenia and growth retardation without any neurologic symp-toms. He had normal intelligence but abnormal EEG ifndings. The activity of acid β-glucosidase in his leucocytes decreased to 1.5 nmol h-1·mg-1 Pr (normal range 6.0-16.7 nmol h-1·mg-1 Pr), supporting the diagnosis of type Ⅲ Gaucher disease. (2) The elder sister of the proband was 12 years old. She had tonic-clonic seizure and myoclonus seizure from the age of seven years old. Mild hepatomegaly, abnormal EEG, poor effect for antiepileptics, and progressive deterioration of psychomotor abilities were found. Her blood leucocytes acid β-glucosidase activity decreased to 1.8 nmol h-1·mg-1 Pr (normal range 6.0-16.7 nmol h-1·mg-1 Pr). Two heterozygous missense mutations, c.680A>G, (p.N188S) and c.1342G>C (p.D409H) were detected from the two siblings, respec-tively.Conclusions Patients with type Ⅲ Gaucher disease usually have the onset in childhood with typical features of Gaucher disease without neurologic involvement. Abnormal EEG may be helpful to the differential diagnosis of type I or type Ⅲ. On the other hand, neurologic manifestations could be presented as the ifrst symptom in some patients without viscera enlargement. The patients of type Ⅲ Gaucher disease with the same genotype could have different phenotypes, even between the siblings.

Objective To evaluate the safety and efficacy of percutaneous nephrolithotomy (PCNL) with B ultrasound-guided renal access in the lateral decubitus flank position for complex renal calculi. Methods From June 2004 to August 2009, 650 patients with renal stones≥20 mm underwent PCNL with B ultrasound-guided renal access in the lateral decubitus flank position. 512 men and 138 women,with a mean age of 38 years(range 11 - 78 years)and a mean stone size of 31 mm(range 20 - 58 mm). Results Successful access was achieved in 650 patients (100%). Minimally PCNL and PCNL were performed in 493 and 157 patients. Complete stone clearance rate was 86. 6% (563/ 650). The mean operative time was 72 min(range 35 - 145 min), and the mean hospital stay was 18d (range 9 - 32 d). There were no visceral injuries. Conclusions PCNL with B ultrasound-guided renal access in the lateral decubitus flank position is safe and convenient, and prevents harmful effects of radiation for the surgeon, the surgical team, and the patient.

We synthesized the superparamagnetic paclitaxel nanoparticles from modified chitosan tangling around Fe3O4 ferrofluid and taxol, and observed the nanoparticles with transmission electronic microscopy (TEM). Then we evaluated the paramagnetism of the particles by vibration specimen magnetometer (VSM) and tested their cytotoxicity with flow cytometry (FCM). The prepared nanoparticle solution was black without any floccule or sediment and appeared transparent after diluted. The nanoparticles were spherical and dispersed in water with mean diameter of 15 nm under TEM and showed superparamagnetic character. FCM test showed the nanoparticles had significant toxic effects against malignant astrocytoma U251 cell lines, equal to taxol alone. These results showed that the superparamagnetic nanoparticle not only enhanced the solubility of paclitaxel in water, but also was superparamagnetic and cytotoxic, which make suitable tools for magnetic targeting chemotherapy of brain gliomas.
Antineoplastic Agents, Phytogenic ; pharmacology ; Apoptosis ; drug effects ; Brain Neoplasms ; pathology ; Cell Line, Tumor ; Chitosan ; pharmacology ; Drug Carriers ; chemistry ; Drug Compounding ; methods ; Ferric Compounds ; Glioma ; pathology ; Humans ; Magnetics ; Metal Nanoparticles ; chemistry ; Nanoparticles ; Paclitaxel ; pharmacology

The development of medical research is completed by the cooperation of sponsors, investigators, subjects, and ethics committees. Clinically, it mainly includes clinical trials of medical devices, clinical medicine and new technology research. This paper analyzed the game and the relationship between rights, responsibilities and interests of relevant parties in medical research, combined with the relevant costs and sharing principles involved in medical research, and found that the use of the word "free" in the informed consent is easy to cause misunderstanding and the lack of relevant compensation costs in the informed consent, while the compensation and insurance costs had some problems, such as the imperfect subject compensation mechanism and the insufficient insurance purchase by the sponsor, which can not protect the basic rights and interests of the subjects. Therefore, in order to standardize the cost management of clinical medical research, it is necessary to standardize the process and content of informed consent, strengthen the supervision of medical research process, establish medical research damage compensation fund and research damage insurance system, so as to better protect the rights and interests of subjects.

With the continuous progress of medical technology, some violations of ethical principles often occur in science experimental research targeting humans. Taking the type of human experimental research as the starting point, through ethical analysis and evaluation of different types of human experimental research, this paper concluded that in human experimental research, it was necessary to adhere to humanitarian principles of human experimental research, fully respect the research participants’ right to informed consent, select research participants fairly, fully protect the research participants’ rights and interests, and supervise the whole research process. So as to better regulate the behavior of medical researchers in human experimental research and protect the rights and interests of research participants.

The development of medical research is completed by the cooperation of sponsors, investigators, subjects, and ethics committees. Clinically, it mainly includes clinical trials of medical devices, clinical medicine and new technology research. This paper analyzed the game and the relationship between rights, responsibilities and interests of relevant parties in medical research, combined with the relevant costs and sharing principles involved in medical research, and found that the use of the word "free" in the informed consent is easy to cause misunderstanding and the lack of relevant compensation costs in the informed consent, while the compensation and insurance costs had some problems, such as the imperfect subject compensation mechanism and the insufficient insurance purchase by the sponsor, which can not protect the basic rights and interests of the subjects. Therefore, in order to standardize the cost management of clinical medical research, it is necessary to standardize the process and content of informed consent, strengthen the supervision of medical research process, establish medical research damage compensation fund and research damage insurance system, so as to better protect the rights and interests of subjects.

OBJECTIVE To study the effects of the compatibility of Schisandra chinensis on Psoralea corylifolia -induced oxidative damage and endoplasmic reticulum stress in L 02 hepatocytes，and to provide reference for clinical use of the compatibility attenuation of P. corylifolia -S. chinensis . METHODS L02 cells were divided into blank control group ，P. corylifolia model group （1 200 μg/mL P. corylifolia ，calculated by crude drug ），P. corylifolia -S. chinensis low-dose，medium-dose and high-dose groups （1 200 μg/mL P. corylifolia combined with 600，1 200，2 400 μg/mL S. chinensis ，respectively，calculated by crude drug ）. After the cells in each group were cultured in culture medium or drug solution for 48 hours，the levels of aspartate aminotransferase（AST）and alanine aminotransferase （ALT）were detected ；the levels of glutathione （GSH），superoxide dismutase （SOD）and malondialdehyde （MDA）in cell culture medium were detected ；reactive oxygen species （ROS）level and mitochondrial membrane potential in cells were detected ；mRNA and protein expressions of glucose-regulated protein 78（GRP-78）and protein kinase R-like endoplasmic reticulum kinase （PERK）were detected. RESULTS Compared with blank control group ，the levels of AST，ALT，MDA and ROS ，mRNA and protein expressions of GRP- 78 and PERK were increased significantly in P. corylifolia model group （P＜0.05 or P＜0.01）；while GSH and SOD levels ，mitochondrial membrane potential were decreased significantly （P＜0.05 or P＜0.01）. Compared with P. corylifolia model group ，above indexes of P. corylifolia -S. chinensis low-dose， medium-dose and high-dose groups were all improved significantly （P＜0.05 or P＜0.01）. CONCLUSIONS The compatibility of P. corylifolia -S. chinensis can alleviate P. corylifolia -induced oxidative damage and endoplasmic reticulum stress of L 02 cells.