Clinical characteristics and prognosis of high-risk neuroblastoma patients with skeletal metastases at different sites / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics and prognostic factors of high-risk neuroblastoma (HR-NB) patients with skeletal metastasis.Methods:The clinical features of 336 newly treated HR-NB patients with skeletal metastases admitted to the Department of Medical Oncology of Beijing Children′s Hospital, Capital Medical University from January 2007 to December 2018 were analyzed retrospectively.Kaplan-Meier method was used for the survival analysis, and Log- Rank test was used for univariate prognosis analysis.The Cox model was used to analyze the multifactorial prognostic analysis. Results:A total of 336 HR-NB patients were recruited, involving 188 males and 148 females with the median age of onset of at 43 (4-148) months.Skeletal metastases affected the viscerocranium (89 cases, 26.5%), neurocranium (193 cases, 57.4%), vertebrae (298 cases, 88.7%), sternum and ribs (183 cases, 54.5%), pelvis (270 cases, 80.4%), upper limbs (182 cases, 54.2%) and lower limbs (240 cases, 71.4%). The 5-year event-free survival (EFS) rate and overall survival (OS) rate were (30.4±2.7)% and (41.3±2.9)%, respectively.Univariate analysis showed a significantly lower 5-year OS rate in skeletal metastatic HR-NB patients with poor prognostic classification, the morphology of neuroblastoma (stroma-poor) and ganglioneuroblastoma (intermixed), high index of mitosis-karyorrhexis index, lactate dehydrogenase≥587 U/L, serum ferritin≥92 μg/L, MYCN amplification and 1p loss of heterozygosity, and metastases in the viscerocranium, neurocranium, vertebrae, sternum and ribs, pelvis, upper limbs and lower limbs (all P<0.05). The 5-year OS rate of HR-NB patients with all 7 regions of skeletal metastases was only (14.2±5.9)%, which was significantly lower than that in patients with a single region metastasis or multi-region metastases[(66.0±10.2)% vs.(43.6±3.4)%, χ2=45.722, P<0.05]. Cox multifactorial analysis showed that MYCN amplification ( HR=4.165, 95% CI: 2.356-7.363) and the viscerocranium metastasis ( HR=2.560, 95% CI: 1.519-4.315) were the independent risk factors affecting the prognosis of HR-NB patients with skeletal metastases (all P<0.05). Conclusions:The prognosis is extremely poor in HR-NB patients with multiple skeletal metastases at the initial diagnosis.The amplification of MYCN and the viscerocranium metastasis are the poor prognostic factors for HR-NB patients with skeletal metastases.

The Expression of RTN1 in Lung Adenocarcinoma and Its Effect on Immune Microenvironment / 中国肺癌杂志

BACKGROUND@#Reticulosome family gene 1 (RTN1) is a reticulosome-encoding gene associated with the endoplasmic reticulum. RTN1 plays a key role in membrane trafficking or neuroendocrine secretion of neuroendocrine cells, while RTN1 serves as a potential diagnostic/therapeutic marker for neurological diseases and cancer. However, the expression of RTN1 and its effect on the immune microenvironment in patients with lung adenocarcinoma have not been reported. In this study, we aimed to investigate the expression of RTN1 in lung adenocarcinoma and its correlation with immune infiltration and survival in lung adenocarcinoma using public databases and bioinformatics network tools.@*METHODS@#Expression levels of RTN1 mRNA in tumor and normal tissues were analyzed using Tumor Immune Estimation Resource 2.0 (TIMER 2.0) and Gene Expression Profiling Interactive Analysis 2 (GEPIA 2). RTN1 protein expression was examined using the Human Protein Atlas. The clinical prognostic significance of RTN1 was analyzed using the GEPIA2 plotter database. To further confirm the potential function of RTN1, the data were analyzed using gene set enrichment analysis. In addition, We performed dimensionality-reduced clustering analysis at the single-cell sequencing level on two datasets from the Tumor Immune Single-cell Hub (TISCH) database to observe the cellular clustering of RTN1 in different types of immune cells. Using the TIMER online tool to analyze and predict the infiltration abundance of different types of immune cells in the immune microenvironment of lung adenocarcinoma patients in the TCGA cohort; TIMER and CIBERSORT were used to study the relationship between genes co-expressed with RTN1 and its associated tumor-infiltrating immune cells; finally, TIMER was used to analyze the relationship between RTN1 and immune correlations between immune checkpoints.@*RESULTS@#We found that RTN1 expression was decreased in patients with lung adenocarcinoma and was closely related to patient prognosis. RTN1 is involved in the process of phagosome formation, hematopoietic cell formation and cell adhesion, and plays an important role in T cell activation. Using cBioPortal and TCGA data to analyze, it is found that RTN1 is significantly associated with BTK, CD4, ECSF1R, MNDA, NCKAP1L and SNX20. High expression of the above genes may cause significant upregulation of CD4+ T cells, mast cells, monocytes, myeloid dendritic cells and M1 macrophages. The expression of RTN1 is closely related to the common immune checkpoints CD274, CTLA4, HAVCR2, LAG3, PDCD1, PDCD1LG2, TIGIT and SIGLEC15 immune checkpoints.@*CONCLUSIONS@#RTN1 may act as a tumor suppressor gene and indicate better prognosis. Furthermore, RTN1 is associated with immune infiltration that may be involved in the immunotherapy response in LUAD. However, the related mechanism needs further research.

Correlation between serum asymmetric dimethylarginine and cystatin C in degenerative calcific aortic valvular disease and its clinical application value / 中国医师进修杂志

Objective To investigate the correlation between serum asymmetric dimethylarginine (ADMA) and cystatin C (CysC) in degenerative calcific aortic valvular disease and its clinical application value.Methods One hundred and eighty patients with degenerative calcific aortic valvular disease who were treated in Beijing Luhe Hospital Affiliated to Capital Medical University from June 2016 to June 2018 were selected as the study subjects,and another 82 healthy subjects from the same hospital in the same period were selected as the control group.Serum ADMA and CysC levels were detected by enzyme-linked immunosorbent assay and latex-enhanced immunoturbidimetric assay.Risk factors of degenerative calcific aortic valvular disease were analyzed by Logistic regression analysis.Evaluation of the diagnostic efficacy of serum ADMA and CysC for degenerative calcific aortic valvular disease was used by receiver operating characteristic curve.Results There were no significant differences in the general data between the two groups in terms of age,gender,body mass index,drinking history,smoking history,and history of hypertension (P＞ 0.05).The systolic blood pressure(SBP),diastolic blood pressure (DBP),heart rate (HR),total cholesterol (TC),triglyceride (TG),low density lipoprotein cholesterin(LDL-C),CysC and ADMA levels of the study group were significantly higher than those of the control group [(131.51 ± 19.09) mmHg (1 mmHg=0.133 kPa) vs.(126.48 ± 16.68) mmHg,(91.11 ± 16.35) mmHg vs.(86.89 ± 10.71) mmHg,(74.39 ± 15.22) beats/min vs.(70.09 ± 13.01)beats/min,(4.51 ± 1.12) mmol/L vs.(4.15 ± 0.92) mmol/L,(1.91 ± 0.63) mmol/L vs.(1.60 ± 0.65) mmol/L,(2.59 ± 1.13) mmol/L vs.(2.27 ± 0.85) mmol/L,(1.01 ± 0.22) mg/L vs.(0.79 ± 0.16) mg/L,(20.17 ± 6.38)ng/L vs.(11.88 ± 4.22) ng/L],and left ventricular ejection fraction (LVEF),high density lipoprotein cholesterin (HDL-C) levels were significantly lower than those of the control group [(56.45 ± 9.21)％vs.(60.87 ± 10.02)％,(1.56 ± 0.63) mmol/L vs.(1.76 ± 0.62) mmol/L],there were significant differences (P ＜ 0.05).Logistic multivariate regression analysis showed that serum CysC and ADMA were independent risk factors for degenerative calcific aortic valvular disease (P ＜ 0.05).Receiver operator characteristic (ROC) curve analysis showed that area under curve (AUC) of serum CysC for degenerative valvular heart disease was 0.785,with a sensitivity of 71.67％,and a specificity of 73.17％.The AUC of serum ADMA for degenerative valvular heart disease was 0.862,with a sensitivity of 71.67％,and a specificity of 87.80％.The AUC of serum CysC combined with ADMA in the diagnosis of degenerative valvular heart disease was 0.910,with a sensitivity of 85.56％,and a specificity of 84.15％,which was significantly higher than the two alone (Z =4.897 and 3.335,P ＜ 0.05).Conclusions Serum ADMA is positively correlated with CysC in patients with degenerative valvular heart disease,and can be used as diagnostic serum markers for degenerative calcific aortic valvular disease.It has clinical reference value for improving the diagnosis of degenerative calcific aortic valvular disease.

Clinical characteristics and prognosis of pediatric non-rhabdomyosarcoma soft tissue sarcomas / 中华实用儿科临床杂志

Objective:To analyze the clinical features and prognosis of pediatric non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), and further understand these tumors.Methods:Twenty-nine NRSTS children were admitted to the Hematological Oncology Ward of Beijing Children′s Hospital from June 2011 to May 2018.The clinical and pathological data of these children were collected, and the relationships of the prognosis with clinical characteristics Intergroup Rhabdomyosarcoma Study(IRS) stage, Children′s Oncology Group(COG) risk grouping were analyzed.All the patients were followed up until October 31, 2018.The survival analysis was performed by Kaplan-Meier method.Results:There were 14 boys and 15 girls in the enrolled 29 children, aging from 7 to 169 months, with a median age of 59.5 months.There were 10 pathological types, including synovial sarcoma, infantile fibrosarcoma and malignant rhabdomyoid tumors in 5 cases, and other pathological types in 14 cases.Tumors originated from the head and neck in 10 cases, limbs or trunk in 6 cases, visceral sites in 13 cases.Ten cases showed distant metastasis.There were 12 cases at IRS stage Ⅰand Ⅱ, and 17 cases of stage Ⅲ and Ⅳ.All the children were treated with surgery and chemotherapy with or without radiotherapy.Nine cases received preoperative chemotherapy, 17 cases received postoperative chemotherapy, 3 cases could not be resected surgically.The follow-up period ranged from 4 to 89 months, with a median follow-up of 16 months.Six of 29 children died, including 3 cases of malignant rhabdomyoid sarcoma.The 2-year overall survival(OS) rate of all the children was 77.4% and the 2-year event-free survival (EFS) rate of all the children was 53.2%.By analyzing the clinical factors, it was found that the IRS stage, COG risk group, primary sites were related to prognosis.The 2-year EFS rate of children with IRS Ⅰ-Ⅱ and Ⅲ-Ⅳ were 75.0% and 35.9%, respectively ( χ2=7.303, P=0.007), the 2-year OS rate was 100% and 61.8%, respectively( χ2=4.81, P=0.028); The 2-year EFS of children in COG low-risk group and median/high-risk group were 66.7% and 44.7%, respectively( χ2=4.155, P=0.042), the 2-year OS rate of children in COG low and median/high-risk was 100% and 66.3%, respectively( χ2=3.383, P=0.066); the 2-year OS rate of children in visceral and non-visceral sites were 59.3% and 92.9%, respectively ( χ2=4.202, P=0.04). Conclusions:NRSTS in children is heterogeneous, and surgery is the main treatment. Children with primary tumors located in visceral sites and at IRS Ⅲ-Ⅳ had poor outcomes.

Correlation between serum asymmetric dimethylarginine and cystatin C in degenerative calcific aortic valvular disease and its clinical application value / 中国医师进修杂志

Objective@#To investigate the correlation between serum asymmetric dimethylarginine (ADMA) and cystatin C (CysC) in degenerative calcific aortic valvular disease and its clinical application value.@*Methods@#One hundred and eighty patients with degenerative calcific aortic valvular disease who were treated in Beijing Luhe Hospital Affiliated to Capital Medical University from June 2016 to June 2018 were selected as the study subjects, and another 82 healthy subjects from the same hospital in the same period were selected as the control group. Serum ADMA and CysC levels were detected by enzyme-linked immunosorbent assay and latex-enhanced immunoturbidimetric assay. Risk factors of degenerative calcific aortic valvular disease were analyzed by Logistic regression analysis. Evaluation of the diagnostic efficacy of serum ADMA and CysC for degenerative calcific aortic valvular disease was used by receiver operating characteristic curve.@*Results@#There were no significant differences in the general data between the two groups in terms of age, gender, body mass index, drinking history, smoking history, and history of hypertension (P>0.05). The systolic blood pressure(SBP), diastolic blood pressure (DBP), heart rate (HR), total cholesterol (TC), triglyceride (TG), low density lipoprotein cholesterin(LDL-C), CysC and ADMA levels of the study group were significantly higher than those of the control group [(131.51 ± 19.09) mmHg (1 mmHg=0.133 kPa) vs. (126.48 ± 16.68) mmHg, (91.11 ± 16.35) mmHg vs. (86.89 ± 10.71) mmHg, (74.39 ± 15.22) beats/min vs. (70.09 ± 13.01) beats/min, (4.51 ± 1.12) mmol/L vs. (4.15 ± 0.92) mmol/L, (1.91 ± 0.63) mmol/L vs. (1.60 ± 0.65) mmol/L, (2.59 ± 1.13) mmol/L vs. (2.27 ± 0.85) mmol/L, (1.01 ± 0.22) mg/L vs. (0.79 ± 0.16) mg/L, (20.17 ± 6.38) ng/L vs. (11.88 ± 4.22) ng/L], and left ventricular ejection fraction (LVEF), high density lipoprotein cholesterin (HDL-C) levels were significantly lower than those of the control group [(56.45 ± 9.21)%vs. (60.87 ± 10.02)%, (1.56 ± 0.63) mmol/L vs. (1.76 ± 0.62) mmol/L], there were significant differences (P<0.05). Logistic multivariate regression analysis showed that serum CysC and ADMA were independent risk factors for degenerative calcific aortic valvular disease (P<0.05). Receiver operator characteristic (ROC) curve analysis showed that area under curve (AUC) of serum CysC for degenerative valvular heart disease was 0.785, with a sensitivity of 71.67%, and a specificity of 73.17%. The AUC of serum ADMA for degenerative valvular heart disease was 0.862, with a sensitivity of 71.67%, and a specificity of 87.80%. The AUC of serum CysC combined with ADMA in the diagnosis of degenerative valvular heart disease was 0.910, with a sensitivity of 85.56%, and a specificity of 84.15%, which was significantly higher than the two alone (Z=4.897 and 3.335, P<0.05).@*Conclusions@#Serum ADMA is positively correlated with CysC in patients with degenerative valvular heart disease, and can be used as diagnostic serum markers for degenerative calcific aortic valvular disease. It has clinical reference value for improving the diagnosis of degenerative calcific aortic valvular disease.

Low expressions of EHD2 and E-cadherin correlate with a poor prognosis for clear cell renal cell carcinoma / 中南大学学报(医学版)

To explore roles of expressions of EHD2 and E-cadherin in clear cell renal cell carcinoma (ccRCC).
 Methods: Four couples of fresh ccRCC tissues and adjacent non-cancerous tissues were collected to evaluate the expression of EHD2 and E-cadherin protein by Western blotting. A total of 65 paraffin-embedded renal ccRCC tissues were collected, and immunohistochemical assay was used to detect the expression of EHD2 and E-cadherin in the samples. The correlation between their expression and clinical and pathological indicators of ccRCC was analyzed, and the relationship between EHD2 and E-cadherin proteins and prognosis for patients with ccRCC was also explored.
 Results: The results of Western blotting showed that the expression levels of EHD2 and E-cadherin were low in 4 ccRCC tissues compared with the adjacent noncancerous tissues. Immunohistochemical results revealed that the expressions of EHD2 and E-cadherin were higher in the localized ccRCC tissues than those in the metastatic ccRCC tissues; the expression levels of EHD2 and E-cadherin were decreased, while the TNM staging and Fuhrman grade were increased (P<0.05 or P<0.01). There was positive correlation between the expressions of EHD2 and E-cadherin in ccRCC (r=0.390, P<0.01). The progression-free survival in ccRCC patients with lower expression of both EHD2 and E-cadherin was better than that in ccRCC patients with higher expressions of EHD2 and E-cadherin (P<0.05). 
 Conclusion: The low expressions of EHD2 and E-cadherin are the potential indicators for the ccRCC patients with poor prognosis.

Renal protective effect of Ganoderma lucidum / 中国药理学与毒理学杂志

OBJECTIVE Non-alcoholic fatty liver disease(NAFLD) encompasses a series of patho-logic changes ranging from steatosis to steatohepatitis,which may progress to cirrhosis and hepatocel-lular carcinoma.The purpose of this study was to determine whether Ganoderma lucidum polysaccha-ride peptide (GLPP) has therapeutic effect on NAFLD. METHODS ob/ob mouse model and ApoC3 transgenic mouse model were used for exploring the effect of GLPP on NAFLD. Key metabolic path-ways and enzymes were identified by metabolomics combining with KEGG and PIUmet analyses and key enzymes were detected by Western blotting. Hepatosteatosis models of HepG2 cells and primary hepatocytes were used to further confirm the therapeutic effect of GLPP on NAFLD. RESULTS GLPP administrated for a month alleviated hepatosteatosis, dyslipidemia, liver dysfunction and liver insulin resistance. Pathways of glycerophospholipid metabolism, fatty acid metabolism and primary bile acid biosynthesis were involved in the therapeutic effect of GLPP on NAFLD. Detection of key enzymes revealed that GLPP reversed low expression of CYP7A1,CYP8B1,FXR,SHP and high expression of FGFR4 in ob/ob mice and ApoC3 mice. Besides, GLPP inhibited fatty acid synthesis by reducing the expression of SREBP1c, FAS and ACC via a FXR-SHP dependent mechanism. Additionally, GLPP reduced the accumulation of lipid droplets and the content of TG in HepG2 cells and primary hepato-cytes induced by oleic acid and palmitic acid. CONCLUSION GLPP significantly improves NAFLD via regulating bile acid synthesis dependent on FXR-SHP/FGF pathway, which finally inhibits fatty acid synthesis,indicating that GLPP might be developed as a therapeutic drug for NAFLD.

Therapeutic Effect of Self-prescribed Guchong Decoction Combined with Ferroids for Iron Deficiency Anemia Patients with Unconsolidation of Thoroughfare and Conception Vessels / 广州中医药大学学报

Objective To observe the clinical efficacy of self-prescribed Guchong Decoction combined with Ferroids for the treatment of iron deficiency anemia (IDA)patients with unconsolidation of thoroughfare and conception vessels. Methods A total of 70 IDA patients with unconsolidation of thoroughfare and conception vessels were randomized into treatment group and control group,35 cases in each group. The control group was given oral use of Ferroids, and the treatment group was treated with self-prescribed Guchong Decoction combined with Ferroids. One month constituted a treatment course,and both groups were treated for 2 courses. Before and after treatment,we compared the parameters of blood routine test for red blood cells(RBC),hemoglobin (HGB), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), MCH concentration (MCHC),white blood cells(WBC)and platelets,as well as reticulocytes(Ret)and iron metabolism indexes including serum iron(SI),serum ferritin(SF),and total iron-binding capacity(TIBC). The adverse reaction was monitored during the treatment. After treatment,both western medicine clinical efficacy and traditional Chinese medicine (TCM)syndrome clinical efficacy of the two groups were evaluated. A 3-month follow-up was carried out after treatment to evaluate the recurrence rate. Results(1)Drop-out occurred in one case from the treatment group and in 2 cases from the control group because of short-term medication suspension.(2)After treatment for 2 months, western medicine clinical cure rate of the treatment group was 82.4％ and that of the control group was 60.6％, and TCM syndrome clinical cure rate of the treatment group was 94.1％ and that of the control group was 51.5％,the difference being significant(P < 0.05 or P < 0.01).(3)The levels of RBC,HGB,Ret, MCV, MCH, MCHC, and WBC of the two groups were obviously increased after treatment (P < 0.01), and PLT level was markedly decreased (P < 0.01)as compared with those before treatment,but the inter-group differences after treatment were insignificant (P > 0.05). (4)After treatment,iron metabolism indexes of SF and SI of the two groups were significantly increased (P < 0.01), and TIBC of the treatment group was significantly decreased (P < 0.01). The treatment group had stronger effect on increasing SF and SI and on decreasing TIBC than the control group (P < 0.05 or P < 0.01). (5)The incidence of adverse reaction and the recurrence rate in the treatment group were lower than those of the control group,the difference being significant (P< 0.01). Conclusion Guchong Decoction combined with Ferroids is effective for the treatment of IDA with unconsolidation of thoroughfare and conception vessels through relieving symptoms rapidly, reducing gastrointestinal adverse reaction,and decreasing recurrent rate.

One-stage repair for adults aortic coarctation concomitant with cardiac diseases: ascending aorta-abdominal aorta by-pass combined with cardiac operation / 中华胸心血管外科杂志

Objective Evaluate the surgical result of ascending aorta-abdominal aorta bypass combined with cardiac op-eration for adults aortic coarctation concomitant with cardiac diseases.Methods Between February 2009 and September 2012, total 24 consecutive patients underwent ascending aorta-abdominal aorta bypass combined with cardiac operation.Of these pa-tients, 2 patients who underwent off-pump coronary artery bypass grafting combined with ascending aorta-abdominal aorta by-pass did not required cardiopulmonary bypass.Other 22 patients underwent one stage repair under cardiopulmonary bypass. The mean artery pressure of upper-lower limb was(38.0 ±9.34)mmHg.Results There was no in-hospital mortality.The mean artery pressure of upper-lower limb was(11.44 ±2.59)mmHg.The mean cardiopulmonary bypass time was(107.27 ± 34.56) min.The mean aorta clamp time was(72.59 ±28.98)min.The mean intensive care unit stay time is(35.22 ± 50.41)h.The mean mechanical ventilation time was(19.50 ±17.64)h.2 patients required prolonged mechanical ventilation for respiratory dysfunction.Of these 2 patients, 1 patient required ECMO support for respiratory failure.Total 1 patient needed temporary continuous renal replacement therapy.No re-exploration for bleeding and gastrointestinal complications were ob-served.Aorta CTA scan was performed for all patients before discharged from hospital.The grafts were patent for all patients and no graft-related complications were observed.Conclusion Ascending aorta-abdominal aorta bypass combined with cardiac operation is a safe and effective one stage repair technique for adults aortic coarctation concomitant with cardiac diseases is a safe and effective technique.

HER2 Expression in Childhood ETV6/RUNX1 Acute Lymphoblastic Leukemia and Its Correlation with Clinical Features / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To explore the HER22 expression in children with ETV6/RUNX1 (E/R)-positive acute lymphoblastic leukemia(ALL) and to investigate the relationship between the HER2 expression and clinical features.</p><p><b>METHODS</b>Thirty-seven newly diagnosed E/R-positive ALL children and 6 controls (4 cases of ITP and 2 healthy children) were selected in Institute of hematology and blood disease hospital. The 37 patients were divided into standard risk (SR), intermediate risk(IR), high risk(HR) groups according to risk stratification; and they were divided into relapse and non-relapse groups according to follow-up result. The CD10CD19 cells were sorted by flow cytometry. The mRNA was extracted from these cells. Real-time fluorescent quantitative PCR was used to detect the expression level of HER2.</p><p><b>RESULTS</b>Among the 37 cases, 51.35% (n=19) were boys and 48.65% (n=18) were girls and their median age was 4.72 (1.72-11.99) years old. Among the 6 controls, 50% (n=3) were boys and 50% (n=3) were girls and the median age was 5.24 (1.53-13.17) years old. The expression level of HER2 in E/R-positive ALL patients were lower than that in controls (P<0.05). Although the difference of HER2 expression level between the 2 groups failed to achieve statistical significance, the expression level of HER2 in relapse patients were significantly lower than that in non-relapse patients, and the HER2 expression in HR group patients were lower than that in SR and IR groups. In addition, there was no significant correlation between the expression level of HER2 and the sex, age, initial white blood cell count, blast cell percentage and the level of LDH (P>0.05).</p><p><b>CONCLUSION</b>The expression level of HER2 in E/R ALL patients is lower than that in controls, and in relapse group lower than that in non-relapse patient. Thus, HER2 may play important roles in the pathogenesis and relapse mechanism of pediatric E/R-positive ALL patients.</p>

Hormone drugs and surgery treatment for Takayasu arteritis / 中华胸心血管外科杂志

Objective To summarize surgical treatment of Takayasu arteritis,and analysis the drug treatment effect during the perioperative period.Methods Retrospective analysis 46 patients with Takayasu's arteritis disease and received cardiovascular surgery between January 2010 to December 2015,in Anzhen Hospital.By collecting their clinical characteristics,preoperative drug therapy,surgical treatment,pathological examination results to analyze operation conditions,effect of drugs and preoperative conditions.Results The perioperative mortality rate was 2.2％ and the complication rate was 23.9％ in 46 patients.There were 34 patients with symptomatic relief in the perioperative period,11 patients didn't take hormone drugs before operation.There were 11 cases of complications during the perioperative period,of which 7 patients were in active stage and 10 patients had not been used before operation.Conclusion The surgical treatment of patients with Takayasu's arteritis disease can effectively improve symptoms.The patients in Takayasu's arteritis active stage will affect the outcome of the surgery.Rational use of hormone drugs before surgery,can effectively control the patient's condition,improve the rate of remission of symptoms,and effectively reduce the incidence of perioperative complications.

Association between clinical outcome and gene mutation in children with Fanconi anemia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To investigate the association between clinical outcome and gene mutations in children with Fanconi anemia (FA).</p><p><b>METHODS</b>A retrospective analysis was performed for the clinical data of six children with the same severity of FA and receiving the same treatment. At first, single cell gel electrophoresis and chromosome breakage induced by mitomycin C were performed for diagnosis. Then the gene detection kit for congenital bone marrow failure diseases or complementation test was used for genotyping of FA. Finally the association between the clinical outcome at 3, 6, 9, or 12 months after treatment and gene mutation was analyzed.</p><p><b>RESULTS</b>Of all the six FA children, five had FANCA type disease, and one had FANCM type disease; four children carried two or more FA gene mutations. Among the children with the same severity of FA, those with more FA mutations had a younger age of onset and poorer response to medication, and tended to progress to a severe type.</p><p><b>CONCLUSIONS</b>Children carrying more than two FA mutations have a poor clinical outcome, and hematopoietic stem cell transplantation should be performed as soon as possible.</p>

A risk assessment on primary level in hs-cTnT level no more than 14 ng/L in the onset of acute myocar-dial infarction in patients with chest pain / 实用医学杂志

Objective To investigate the association of chest pain patients with primary level in high-sensitivity troponin T (hs-cTnT) level no more than 14 ng/L in the onset of acute myocardial infarction in pa-tients with chest pain. Methods We enrolled 3 096 participants from January 2012 to December 2013 in West China Hospital, Sichuan University. All patients were classified two groups (hs-cTnT > 14 ng/L, hs-cTnT ≤14 ng/L and no ischemia on ECG) according to hs-cTnT levels and ECG. We evaluated the risk of myocardial in-farction and death and negative predictive value in 30 days. Results Thirty-seven patients were diagnosed in having acute myocardial infarction (AMI) and 4 patients were dead in the hs-cTnT > 14 ng/L group in 30 days in the absolute risk 2.35(1.86-2.74) and 0.29(0.12-0.53); 9 patients were diagnosed as having AMI and no patients were dead in the hs-cTnT ≤ 14 ng/L group in 30 days in the absolute risk 0.58 (0.42-0.74). Conclu-sion Chest pain patients whose primary levels no more than 14 ng/L in hs-cTnT with no ischemia on ECG can be ruled out AMI in negative predictive value 99.6%. the levels of hs-cTnT which were dynamic monitored at least 5 h and still no more than 14 ng/L can rule out AMI directly.

Study on sedation and amnesia of patients with different ages of lumbar epidural anesthesia in equal doses of midazolam / 中国生化药物杂志

Objective To study the sedation and amnesia of midazolam in patients with different ages of lumbar epidural anesthesia . Methods From January to July 2015, 235 patients in the first affiliated of xian jiaotong unniversity,who were implemented abdominal surgery under combined spinal epidural anesthesia and American Society of anesthesiologists (ASA) grade was I-II , were divided into three groups:young group (n=70); middle-aged group (n =82); elderly group (n =83).Intraoperative continuous monitoring of respiratory and circulatory function index,and recorded MAP,SpO2, HR before and 5, 15, 30 min after injection.ResuIts MAP, HR and SpO2 of three groups were compared with the same time points pre-and post used of midazolam, there was no significant difference among indicators.; Compared with the same group at different time points, MAP(FYG =9.2547,FMG =12.8181,FOG =17.77)和 SpO2 (FYG =190.3835,FMG =196.2393,FOG =270.5609),there were significant difference.The difference was not statistically significant between HR group and the same group at different time points and forgetting degree and sedation score , but compared with OG,there were significant difference(P<0.05).The total number of adverse reactions in YG group and MG group was less than that of OG group(X2 =11.2516,X2 =10.4491, P<0.05).ConcIusion The effect of the same dose of midazolam on sedation and amnesia in patients with different ages of lumbar epidural anesthesia is different, and the degree of the effect of the elderly patients is significant,but will produce more adverse reactions.Therefore, in the combined spinal epidural anesthesia,it is necessary to consider the patient's individual differences, rational use of sedative and narcotic drugs.

CYP3 A4 gene polymorphism and fentanyl individual drug use in patients with extensive burns / 中国生化药物杂志

Objective To explore effect of CYP3A4 gene polymorphism and genetic testing on the efficacy of fentanyl in patients with extensive burns.Methods A prospective randomized controlled study, 149 patients with extensive burns were picked from Feburary to July in 2015.Patients were randomly divided into matched group ( n=56 ) and experimental group ( n=93 ) . The matched group was treated with fentanyl 5 g/kg to finish implementation of anesthesia induction.Depends on the gene detection results, the patients in experimental group were treated with 6 g/kg, 5 g/kg or 4 g/kg fentanyl.The VAS score at different time after operation in the experimental group and the control group were compared , and the dosage of fentanyl was observed.Patients whose VAS score was greater than 7 should treated with intramuscular injection of pethidine 100 mg, recorded all patients with pethidine,s additional cases and the times.At the same time, the adverse reactions of the two groups were recorded before and after hospital discharge. ResuIts The score of VAS was not significantly different after wake up immediatly after the surgery , and the score of VAS was significantly lower in the experimental group patients in 6h,12h after the surgery (P<0.05).The dosage of fentanyl in the experimental group and the number of the cases and the times were significantly decreased (P<0.05).The adverse reactions after the surgery such as nausea, vomiting, itching, drowsiness, and adverse reactions were significantly lower in the experimental group (P<0.05).ConcIusion CYP3A4 gene detection in patients with large area of burn in the individual drug use is important, because of patients’ genotype adjusting fentanyl dosagecan, it enhances significantly the analgesic effect, reduces the amount of drug use, and effectively reduces the adverse reactions.