Objective To investigate the efficacy and safety of sirolimus 0.1% ointment in the treatment of facial angiofibromas in children with tuberous sclerosis complex. Methods Sirolimus 0.1% ointment was prepared. Twenty children with tuberous sclerosis complex who had facial angiofibromas were enrolled in this study. Facial angiofibromas were topically treated with the self?prepared sirolimus 0.1% ointment twice a day for 12 weeks. The facial angiofibroma severity index(FASI)was calculated, the degree of satisfaction with the treatment was evaluated, and adverse reactions were analyzed at weeks 4 and 12. Plasma sirolimus concentrations as well as blood biochemical and immunological parameters were measured, blood coagulation activity was evaluated, and routine blood tests as well as urine tests were performed at baseline and week 12. Results The FASI of patients significantly decreased at weeks 4(4.400 ± 1.284)and 12(2.975 ± 1.543)compared with that at baseline(5.750 ± 1.175, both P<0.000 1), and was significantly lower at week 12 than at week 4(P < 0.000 1). The efficacy index was 49.87% ± 22.08% at week 12, significantly higher than that at week 4(24.43%± 10.18%, t=7.338, P<0.01). The color, size and number of lesions significantly decreased in all the patients, and facial angiofibromas completely disappeared in 2 patients at week 12. At week 4, 10 parents were satisfied with the improvement of erythema, 3 parents with that of lesion volume, and 3 parents with that of lesion area. The degree of parent satisfaction increased at week 12 in all the cases. The blood concentration of sirolimus was lower than 1.0μg/L both before and after the treatment. No severe systemic or local adverse reactions were noted in these patients. Conclusion Sirolimus 0.1%ointment is markedly effective and safe for the treatment of facial angiofibromas in patients with tuberous sclerosis complex.

Objective To investigate the effect of radiofrequency ablation (RFA) in treating unresectable liver caneer. Methods 43 patients(78 lesions) who suffered from unresectable liver cancer were treated with cool-tip RFA from May 2006 to November,2008 in our hospital. 26 cases were treated with RFA in laparotomy besides with reseet of lesion in 6 eases and with alinjection in 5 cases,while 17 eases were treated only with percutaneous RFA. Results All patients were diagnosed by CT and B-ultrasound or MRI,among whom 18 cases were confirmed with primary hepatic carcinoma, 12 eases experienced recurrence following resect of primary hepatic carcinoma and 13 eases developed metastatic carcinoma;Every patient was followed up with AFP, abdominal B-ultrasound, CT scans or MRI from 1 to 28 months after treatment (mean 13.6 months). Before procedure, AFP increased in 20 eases which recovered within 3 months of treatment. 2 cases were suspected having residual on CT scan ,6 cases were found with new masses on liver on CT scan, 1 case complicated with gastrointestinal fistula and then died of systemie metas-tasis 3 months later,1 ease died of postoperative bleeding and 1 died of bleeding and infection after reseet + RFA treatment,and 3 cases died of multi-metastasis during following up who survived on average of 6 months. Conclu-sions B-ultrasound -guided cool-tip circulation RFA is an effective and safe method in treating unresectable liver cancer.

Objective To investigate the clinical value of prenatal ultrasonography in diagnosing fetal aortic atresia.Methods Ultrasound images of 9 cases with fetal aortic atresia confirmed by autopsy were retrospectively analysed and summarized.Results Fetal echocardiography showed ascending aorta and aortic arch were thin in seven cases and unclear in two cases.Color Doppler flow imaging showed fetal aorta no blood connection with left ventricle and reverse blood flow in aortic arch among all 9 cases.There were 6 cases associated with hypoplastic left heart syndrome,two cases associated with ventricular septal defect and well-developed left ventricle,one case complicated with complete atrioventricular septal defect.Nine cases were detected at average gestational age (26.0 ± 2.6) weeks,minimum gestational age was only 16 weeks pregnant.Conclusions Prenatal ultrasound diagnosis of fetal aortic atresia has important clinical value.Ascending aorta and aortic arch are thin or unclear,no blood flow connection between fetal aorta and left ventricle and reverse blood flow in aortic arch are major diagnostic evidences.Color Doppler flow imaging is helpful for early diagnosis.

Objective: To explore the relationship between serum levels of immunoglobulin E (IgE) and calciifc aortic valve disease (CAVD) in relevant patients.
Methods: A total of 394 patients were enrolled in our study. Based on echocardiography presentation, the patients were divided into 2 groups: CAVD group,n=169 and Non-CAVD group,n=225. Serum levels of IgE were examined by chemiluminescence method. The IgE levels were compared between 2 groups and the relationship between serum IgE level and CAVD was analyzed.
Results: Serum levels of IgE in CAVD group was significantly higher than Non-CAVD group 113.30 IU/ml vs 63.76 IU/ml (P<0.05); multivariate logistic regression analysis conifrmed above difference (P<0.05) and it also indicated that the alteration of surum IgE level is obviously related to CAVD occurrence.
Conclusion: Serum IgE level is obviously increased in CAVD patients. IgE is an independent biochemical indicator of CAVD, it may play the important role in CAVD pathogenesis.

Objective To compare the clinical characteristics,and outcomes of patients with heart failure with different left ventricular ejection fractions (LVEF).Methods A total of 1 182 hospitalized patients with heart failure (HF) were enrolled and retrospectively studied in the present study.The patients were stratified by LVEF as reduced (HFrEF,LVEF ＜ 40％,n =313),mid-range (HFmrEF,40％ ≤ LVEF ＜50％,n =287) and preserved (HFpEF,LVEF≥50％,n =582) ejection fraction groups.Among the 1 182 cases,941 of them (81.3％,84.9％,and 84.0％ inHFrEF,HFmrEF and HFpEF groups,respectively) were followed up for an median duration of 27.3 months.Results (1) Among the study patients,26.5％ were in HFrEF,24.3％ in HFmrEF,and 49.2％ in HFpEF groups.(2) Ischemic heart disease with HFmrEF was more frequent than that in patients with HFrEF.The average age,percentage of female subjects,systolic blood pressure,uric acid,N terminal B-type natriuretic peptide precursor (NT-proBNP),hemoglobin,and the incidence of hypertensive heart disease,anemia,atrial fibrillation in patients with HFmrEF were higher than those in patients with HFrEF,but lower than those in patients with HFpEF (all P ＜0.01).(3) The all-cause cumulative mortality was 10.8％ at 1 year,20.6％ at 2 years and 35.9％ at 5 years.No difference was observed in the all-cause cumulative mortality at 1 year,2 years,5 years among the three groups (all P ＞ 0.05).Conclusions The HFmrEF patients,as a new and distinct group,were with many intermediate characteristics compared with HFrEF and HFpEF subjects.However,the all-cause mortality was not significantly differeut among HF patients with different LVEF.

OBJECTIVETo analyze the clinical features and treatment of skull base osteosarcoma.

METHODSThe clinical data of 18 patients with skull base osteosarcoma, who were admitted to the CAMS Cancer Hospital from January 2005 to November 2013, were retrospectively analyzed. The patients were followed up by telephone, outpatient review and other means. Fifteen patients were followed up, 4 cases received surgery only, and 11 cases received surgery with adjuvant chemotherapy and/or radiotherapy. Kaplan-Meier survival curve analysis was used to analyze the clinical data and Log rank method was used for verification.

RESULTSNine patients died among the 15 patients who were followed up for 3-103 months (mean 25.0 months): seven patients died of local recurrence, and two patients died of distant metastasis, and six patients were still alive. Four patients received surgery only, with a median survival time of 25.0 months, and 11 patients received comprehensive treatment, with a median survival time of 47.0 months (P = 0.02). Five patients received sub-total resection, with a mean survival time of 47.0 months, and 10 patients received total resection, with a mean survival time of 45.0 months (P = 0.37). The 1- and 2-year recurrence rates were 46.6% and 68.9%, respectively. The overall 1-, 2-, 3- and 5-year survival rates were 82.4%, 61.8%, 36.0% and 36.0%, respectively, with a median survival time of 30.0 months.

CONCLUSIONSTo compare the long bone and head and neck osteosarcoma with skull base osteosarcoma, the skull base osteosarcoma has a lower total resection rate, a higher recurrence rate, and a poorer prognosis. Radical surgery and comprehensive treatment are appropriate for skull base osteosarcoma.

Bone Neoplasms ; diagnosis ; pathology ; therapy ; Chemotherapy, Adjuvant ; Combined Modality Therapy ; Humans ; Kaplan-Meier Estimate ; Neoplasm Recurrence, Local ; Osteosarcoma ; diagnosis ; pathology ; therapy ; Retrospective Studies ; Skull Base ; pathology ; Survival Rate

Objective To explore the value of OSCE system combined with intelligent network in-formation platform in clinical skills assessment of obstetrics and gynecology. Methods 112 clinical medi-cal students who participated in the practice of gynecology and obstetrics in Second Military Medical Uni-versity in 2017 were randomly divided into the experimental group (network information OSCE) and the control group (traditional OSCE). The teaching results were evaluated by the questionnaire survey of teachers and students and the examination results as well. The statistical analysis was made with the Chi-square test and the t test respectively. Results According to the questionnaire survey of two skills assessment methods, the satisfaction index of the experimental group was higher than that of the control group in both teachers and students, and the difference was statistically significant (P<0.05). The total time of examination in the experimental group was lower than that of the control group, and the difference was statistically significant (P<0.05). The final total score and the results of case analysis and clinical operation examination of experi-mental group were all higher than those of the control group, but there was no statistical difference (P>0.05). Conclusion OSCE combined with the network information system has an unparalleled advantage in the assessment of the obstetrics and gynecology department. The system will promote clinical education of ob-stetrics and gynecology and the evaluation of the clinical ability of the medical students to a new height, which deserves popularization.

IgA nephropathy (IgAN) is one common type of glomerulonephritis caused by a deposition of immune complexes in mesangium and partial capillary loops. It is also an important risk factor for end-stage renal disease (ESRD). Kidney transplantation (KT) has been an ultimate treatment for IgAN patients progressing into ESRD. However, there is still a high risk of recurrence after transplantation. Currently no effective treatment is available for recurrent IgAN. This review summarized the latest researches of managing IgAN recurrence after KT, such as optimal treatment, immunosuppression, complement therapy and surgery.

OBJECTIVETo analyze the status of lipid metabolism and the relationship between low birth body mass and lipid metabolism disorders in adolescents with different economic and cultural backgrounds.

METHODSTotally 1700 adolescents from urban and rural areas aged 12-18 years were selected in 2013. The lipid profiles were determined, and a questionnaire-based survey was conducted.

RESULTSAmong normal birth body mass adolescents, girls obtained a high density lipoprotein in both urban and rural areas, low density lipoprotein cholesterol level was higher for city girls, rural boys and urban girls obtained higher total cholesterol level, the differences all reached statistical significance. While among the low birth body mass adolescents, higher level of total cholesterol for urban boys was detected, with statistically significance. When compared between rural and urban areas, high density lipoprotein cholesterol level for urban adolescents was lower in normal birth body mass adolescents, while total cholesterol level for urban adolescents was higher, both with statistically significance. While the comparison between different birth body mass adolescents suggested that the normal birth body mass adolescents obtained a higher high density lipoprotein cholesterol, low density lipoprotein cholesterol, triglyceride and total cholesterol level. When analyze the influenced factors of disorder of lipid metabolism, the Results suggest that low birth body mass, maternal pregnancy body mass increasing, lipid homeostasis family history, absence of breakfast, unhealthy snack habit, abnormal of systolic blood pressure and pulse pressure increasing were related to the occurrence of dyslipidemia.

CONCLUSIONSLow birth body mass is related with lipid metabolism in early puberty. Low birth body mass, family history of abnormal lipid metabolism, and poor diet habits can increase the risk of abnormal lipid metabolism in puberty.

Adolescent ; Birth Weight ; Cholesterol ; blood ; Cholesterol, HDL ; blood ; Cholesterol, LDL ; blood ; Dyslipidemias ; epidemiology ; Female ; Humans ; Lipid Metabolism ; Male ; Pregnancy ; Risk Factors ; Rural Population ; Sexual Maturation ; Triglycerides ; blood ; Urban Population

Atypical and malignant meningiomas are characterized by high invasiveness and local recurrence.Traditional treatments such as surgery,radiotherapy and chemotherapy have limited effects.Recently,with the further study of the pathogenesis of meningioma and the development of molecular biology techniques,some new biomarkers have been found to play important roles in development and malignant progression of meningioma.Several genetic and epigenetic factors have been identified as contributing to the malignant progression of meningioma.Genetic factors include multiple phenotypic changes such as abnormal cell proliferation,increased cell invasiveness,angiogenesis,apoptosis inhibition,and changes in telomerase activity.And the discovery of biomarkers provides references for early diagnosis,molecular targeted therapy and prognosis assessment of high-grade meningiomas.