Objective: To analyze and summarize the results of genomic DNA test findings of chemotherapeutic drugs commonly used in pediatric rhabdomyosarcoma (RMS) in children, and to analyze the relationship between adverse reactions to chemotherapy toxicity and genomic DNA polymorphisms, so as to provide evidence for guiding treatment. Methods: Retrospective analysis was conducted in RMS children admitted at Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University from January 2017 to June 2018.The criteria for enrollment were definite diagnosis of RMS, regular treatment and follow-up at Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University, and detection of peripheral blood DNA fluorescence hybridization sequence for several commonly chemotherapy drugs.The toxicity of chemotherapeutic drugs was detected based on the National Cancer Institute routine toxicity criteria (NCI-CTCAE version 4.0). Summary and analysis indicators included primary and metastatic site, size, international RMS clinical stage (TNM-UICC), Intergroup Rhabdomyosarcoma Study(IRS) Clinical Grouping Classification, risk grouping, pathological type, changes in major organ functions, as well as processes of surgery, chemotherapy and radiotherapy, and the association between toxicity and DNA polymorphism of drug genes was analyzed.SPSS 22.0 software was used for χ² test. Results: A total of 32 children were enrolled, and 20 cases were male and 12 cases were female, their median age was 50 months (15-120 months). The primary tumor of 9 cases were sited in the chest, abdomen and basin, 8 cases in the head and neck (non-meningeal), 7 cases in bladder prostate, 3 cases in limbs, 2 cases in the meningeal area, 1 case in urogenital tract (non-bladder prostate), 2 cases in other parts.Seventeen cases were embryonic type and 15 cases were alveolar type.Five cases were TNM-Ⅰ stage, 5 cases were TNM-Ⅱ stage, 10 cases TNM -Ⅲ stage, 12 cases were TNM-Ⅳ stage, 21 cases were IRS-Ⅲ, 11 cases were IRS-Ⅳ.Twenty-two cases were moderate-risk (MR), 10 cases were high-risk (HR). Twenty-two cases were detected UGT1A1*6 gene, 18 cases in GG type, 13 cases in GA type, and 1 case in AA type.ABCB1 gene monitoring was performed in 27 children, 14 cases of CT type and 13 cases of TT type; 29 cases were detected GSTP1 gene, 7 cases of GA type and 2 cases of GG type, 19 cases of AA type, 1 case of AG type; 30 cases were detected CYP3A5 gene, 2 cases of GA type, 13 cases of GG type, AG 15 cases.All patients were treated according to the BCH-RMS-2007 protocol using VAC (Vincristine, Doxorubicin, and Cyclophosphamide) as the basis for chemotherapy.From 2017, VAC and VI regimen (Vincristine, Irinotecan) were defined as the standard of backbone chemotherapeutic regimen for MR.Nine cases underwent surgery before chemotherapy and 10 cases had surgery after chemotherapy, among them, 5 cases underwent twice operation.Local radiotherapy was performed on the 12^th week of chemotherapy, and the central nervous system involvement cases started in the first week.Hematological toxicity was mainly caused by neutropenia, with 2 cases of grade 3 and 30 cases of grade 4.Liver function damage of grade 2 was 6 cases, grade 3 was 3 cases.Four patients with grade 1 diarrhea, 3 patients with grade 2, 5 patients with grade 3, 3 patients with grade 4.There was significant diffe-rence between the severity of diarrhea and UGT1A1*6 genotype polymorphism(P<0.05). Conclusions Chemothe-rapy for RMS patients is highly safety.If the genomic DNA test of chemotherapy drugs show a slow metabolism type, the dose of chemotherapy should be reduced, and the toxicity of chemotherapy drugs should be monitored dynamically.

Objective To summarize the clinical data and characteristics of neuroblastoma (NB) with pancreatic infiltration and to assess the clinical features and the prognosis of NB.Methods According to NB protocol at Beijing Children's Hospital,Capital Medical University(BCH-NB-2007),based on Hong Kong NB protocol,the patients were divided into 3 groups of low-risk (LR) group,medium risk (MR) group and high-risk (HR) group.All children were followed up till March 31,2017.Diagnosis of pancreatic infiltration of NB was made by abdominal enhancement of CT,enhanced magnetic resonance imaging (MRI) or 18-fluorodeoxyglucose-positron emission tomography-computed tomography(18F-FDG-PET/CT),any of which could suggest NB pancreatic infiltration or postoperative pathology prompted NB to infiltrate the pancreas.Retrospective summary and analysis of indicators were performed,which included the initial diagnosis of primary tumor and metastatic tumor site,tumor markers,clinical stage,risk group,imaging features and treatment.Results (1) Totally 50 eligible patients were included:27 females,23 males,median age of 33 months (7-129 months),10 cases ≤ 18 months,40 cases ＞ 18 months;3 cases were of International Neuroblastoma Staging System(INSS)-Ⅲ,47 cases of INSS-Ⅳ;2 caes of LR,3 cases of MR,45 cases of HR;28 cases had a fever,27 cases with abdominal mass,14 cases with abdominal pain,9 cases with limb pain,5 cases with vomiting,4 cases with diarrhea,and 1 case with jaundice.Forty-nine cases of primary tumor were located in the retroperitoneal adrenal gland,and 1 case in the pelvic cavity.Thirty-two cases had tumor diameter≥ 10 cm.(2)Tumor markers and imaging features:the median serum lactate dehydrogenase (LDH) value in 50 cases was 669 U/L (263-6 762 U/L),of them 19 cases ＞ 1 000 U/L.A total of 80％ cases had neuron specific enolase (NSE) ＞ 0.15 ng/L.Nine cases had elevated amylase (AMY),and 7 cases had elevated lippase (LPS),and all the levels were elevated in 5 cases.A total of 41 cases had pancreas infiltration by abdominal ultrasound,44 cases had pancreas infiltration by abdominal enhancement computed tomography (CT),100％ (14/14 cases)of pancreas infiltration was confirmed by abdominal reconstruction enhancement nuclear imaging MRI,and NB pancreas infiltration was proved in 41.3％ (19/46 cases) by 18F-FDG-PET/CT.Comparison of the above 4 imaging studies:one imaging examination index was positive in 7 cases,accounting for 14.0％,2 positive in 26 cases,accounting for 52.0％,3 positive in 15 cases,accounting for 30.0％,and 4 positive in 2 cases,accounting for 4.0％.(3) Treatment outcomes:totally 50 cases received treatment,including 2 cases of LR,all cases were of INSS-Ⅲ,and 1 case with complete remission (CR).Three cases of MR belonging to INSS-Ⅳ had complete resection of the tumor,1 case had recurrence and died,and the other two were stable.Forty-five cases with HR,median follow-up lasting for 15 (4-53) months,16 cases had occurrence (35.6％),3 cases were relapsed after stopping treatment for 2,3,18 months,respectively;tumor progressed in 12 patients during treatment,and 1 case got severe intracranial infection and gave up treatment before death.Kaplan-Meier analysis showed the expected 3-year event free survival(EFS) rate was 22.1％,and 3-year overall survival(OS) rate was 38.5％.Conclusions Preliminary results show that 90％ with pancreatic infiltration of NB belong to Ⅳ HR group of children,and almost primary tumor is almost located in the retroperitoneal ragion.NB with pancreatic infiltration clinical manifestations is hidden and nonspecific.More than half of the children have no obvious abdominal pain or vomiting,and so imaging examination is needed to determine the situation of pancreatic metastasis further.Abdominal reconstruction enhancement MRI has a high sensitivity and specificity for pancreatic metastatic lesions,which can be used as the basis for early diagnosis.The overall prognosis is poor.The expected 3-year EFS rate can be 22.1％,3-year OS rate was 38.5％.

Objective To summarize the clinical features of neuroblastoma (NB)with N - myc gene amplifi-cation in order to analyze tumor shrinkage and bone marrow remission in the early stage of chemotherapy,and to eva-luate the children's initial sensitivity to chemotherapy. Methods The medical records of 38 patients with N - myc am-plification of NB were reviewed (bone marrow or tumor tissues were positive during fluorescence in situ hybridization probe),who were treated between February 2012 to December 2016 at the Hematology Oncology Center,Beijing Chil-dren's Hospital,Capital Medical University. The regimens included chemotherapy,surgery,stem cell transplantation, radiotherapy,and maintenance treatment. The data were reviewed for the medical history. The variations of biomarker, bone marrow cells and the primary site were analyzed before and after 2 courses of CAV (Cyclophosphamide + Adriamy-cin + Vincristine)regimen chemotherapy,in order to observe the short - term effect of chemotherapy and the results were described with statistics. Results Total 38 cases were studied,22 boys(58. 9％)and 16 girls(42. 1％). The median age was 30 months. The primary sites of 37 cases of tumor were located in the retroperitoneal and adrenal area,1 case located in the posterior mediastinum. Bone marrow cytology was negative in 12 cases of them,but bone marrow biopsy suggested bone marrow metastasis,while bone marrow cytomorpholigic examinations were positive in the other 26 cases. Of all the 37 cases the lactate dehydrogenase (LDH)levels were reported higher than the normal value. LDH level was under 500 U/ L in one case,9 cases above 4000 U/ L. The neuron specific enolase (NSE)level of all the cases was higher than the normal and NSE level in 36 cases was higher than 100 μg/ L. In one patient the diameter of tumor was less than 5 cm,lager than 10 cm in 32 cases. The lesion of 33 tumor cases before chemotherapy by enhanced CT was ≤100 cm3 in 12 cases,> 100 - 500 cm3 in 11 cases,among which 6 cases ranged from 500 - 1000 cm3 ,4 cases larger than 1000 cm3 . All the 38 cases received 2 courses of chemotherapy. LDH levels of 4 cases became normal,and LDH levels fell under 500 U/ L in 18 cases,while LDH levels of the other 3 cases were above 1000 U/ L. Among 38 cases, the NSE level in 6 cases was reduced to normal,and 16 cases reduced to 25 - 100 μg/ L. The bone marrow examination of 36 cases reversed to negative. According to the image examination,the overall response rate after 2 courses of chemo-therapy was 84. 8％ . One case achieved very good partial remission,21 cases achieved partial response,7 cases a-chieved metastatic remission,2 cases had no remission,while 2 cases showed progression. After 2 courses of chemother-apy,the tumor diameter in 7 cases was less than 5 cm,while that of 22 cases was above 10 cm. Conclusions The ma-jority primary site of NB with N - myc gene amplification is located in retroperitoneal and adrenal area. Patients with the huge tumor have a heavy burden and the biomarker is always high at the early stage. NB with N - myc gene amplifica-tion is sensitive to chemotherapy. After 2 courses of chemotherapy,there is a sharp decrease in the level of biomarker and the tumor burden. Chemotherapy can diminish the burden of tumor in early stage. But because of the huge burden and the huge size of tumor,it's not the best time for surgery and stem cell collection. The patients should go on receiving chemotherapy for remission of disease.

Purpose To investigate the value of arterial spin labeling (ASL) in evaluating renal function in patients with renal cell carcinoma (RCC) after laparoscopic partial nephrectomy.Materials and Methods Fifteen patients with RCC undergoing laparoscopic partial nephrectomy were studied prospectively.The patients were performed ASL scan one week before and three months after operation.The correlation between renal blood flow (RBF) value measured by ASL and the glomerular filtration rate (GFR) measured by radionuclide method in the renal cortex of healthy side was analyzed.The RBF values in the kidney of affected side or healthy side were measured,the difference of which between before operation and three months after operation was compared.Results The RBF value and GFR data in the renal cortex of healthy side had positive correlation (r=0.638,P＜0.05).In the affected side of kidney,the RBF value of remaining renal tissue [(291.5 ± 37.3) ml/(100g·min)] compared with that of preoperative renal tissue [(237.8 ± 46.2) ml/(100g·min)]increased about 53.7 ml/(100g · min) (P＜0.05).In the healthy side of kidney,the RBF value of renal tissue [(241.1 ± 50.3) ml/(100 g · min)] compared with that of preoperative renal tissue [(290.4 ± 51.8) ml/(100 g·min)] decreased about 49.3 ml/(100 g·min) (P＜0.05).Conclusion ASL can be used to evaluate renal function,and it is valuable to evaluate renal perfusion function after laparoscopic partial nephrectomy of RCC.

OBJECTIVETo explore the mechanism of the protective effects of Panax notoginseng saponins (PNS) on kidney in diabetic rats.

METHODSDiabetic rat model was obtained by intravenous injection of alloxan, and the rats were divided into model, PNS-100 mg/(kg day) and PNS-200 mg/(kg day) groups, 10 each. Another 10 rats injected with saline were served as control. Periodic acid-Schiff staining and immunological histological chemistry were used to observe histomorphology and tissue expression of bone morphogenetic protein-7 (BMP-7). Silent information regulator 1 (SIRT1) was silenced in rat mesangial cells by RNA interference. The mRNA expressions of SIRT-1, monocyte chemoattractant protein-1 (MCP-1), transforming growth factor β1 (TGF-β1) and plasminogen activator inhibitor-1 (PAI-1) were analyzed by reverse transcription polymerase chain reaction. The protein expressions of SIRT1 and the acetylation of nuclear factor κB (NF-κB) P65 were determined by western blotting. The concentration of MCP-1, TGF-β1 and malondialdehyde (MDA) in culture supernatant were detected by enzyme-linked immuno sorbent assay. The activity of superoxide dismutase (SOD) was detected by the classical method of nitrogen and blue four.

RESULTSIn diabetic model rats, PNS could not only reduce blood glucose and lipid (P<0.01), but also increase protein level of BMP-7 and inhibit PAI-1 expression for suppressing fibrosis of the kidney. In rat mesangial cells, PNS could up-regulate the expression of SIRT1 (P<0.01) and in turn suppress the transcription of TGF-β1 (P<0.05) and MCP-1 (P<0.05). PNS could also reverse the increased acetylation of NF-κB p65 by high glucose. In addition, redox regulation factor MDA was down-regulated (P<0.05) and SOD was up-regulated (P<0.01), which were both induced by SIRT1 up-regulation.

CONCLUSIONSPNS could protect kidney from diabetes with the possible mechanism of up-regulating SIRT1, therefore inhibiting inflammation through decreasing the induction of inflammatory cytokines and TGF-β1, as well as activating antioxidant proteins.

Acetylation ; drug effects ; Animals ; Antioxidants ; metabolism ; Blood Glucose ; metabolism ; Bone Morphogenetic Protein 7 ; metabolism ; Chemokine CCL2 ; metabolism ; Diabetes Mellitus, Experimental ; blood ; drug therapy ; genetics ; physiopathology ; Gene Knockdown Techniques ; Immunohistochemistry ; Kidney ; drug effects ; pathology ; Kidney Function Tests ; Lipids ; blood ; Male ; Malondialdehyde ; metabolism ; Mesangial Cells ; drug effects ; metabolism ; Oxidative Stress ; drug effects ; Panax notoginseng ; chemistry ; Plasminogen Activator Inhibitor 1 ; genetics ; metabolism ; Protective Agents ; pharmacology ; therapeutic use ; Rats, Sprague-Dawley ; Saponins ; pharmacology ; therapeutic use ; Sirtuin 1 ; genetics ; Superoxide Dismutase ; metabolism ; Transcription Factor RelA ; metabolism ; Transcription, Genetic ; drug effects ; Transforming Growth Factor beta1 ; metabolism ; Up-Regulation ; drug effects

OBJECTIVE: To provide the basis of improving drug importation registration management system of our country by studying domestic and foreign drug importation management system adequately. METHODS: Checked out and studied the laws associated with import drug of our country and United States with literature research and comparative analysis methods. RESULTS: To reform the drug importation registration management system of our country, related problems should be analyzed. Also the recommendations should be put forward, including the registration of foreign drug establishment, unified registration procedures and adopted DMF system. CONCLUSION: Based on national conditions, we must draw on the advantages of United States system selectively, to improve our system, ensure the quality of import drug lifecycle and protect public health.

The imaging findings of pelvic lipomatosis as confirmed by operation and pathology were examined in 1 case and two follow-up asymptomatic cases retrospectively analyzed.The imaging findings included a compressed and deformed bladder with a superior displacement.Its shape was like an inverted tear and pear in coronal view and a banana in sagittal view.Bilateral ureters were both compressed with a medial deviation.And bilateral ureters were dilated with hydronephrosis in 1 case.Rectum and sigmoid were both compressed and became narrowed.The clinical manifestations included frequent urination , urgent urination , urination pain, dysuria, constipation, nausea, vomit and fever in 1 case while another 2 cases stayed asymptomatic.