Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder. Here, we report a rare case of multi-system LCH in a 20-month-old children presenting nasal congestion, fever, abnormal liver function, anemia, and skin damage. The radiograph computed tomography showed an osteolytic lesion in the lateral skull base with tumor extension. Pathological biopsy was performed, and the histopathologic diagnosis was LCH. A general review of LCH, including clinical manifestations, diagnosis, treatment, and prgognosis, is presented.
Histiocytosis, Langerhans-Cell ; diagnosis ; Humans ; Infant ; Rare Diseases

With the gradual increase of China's total expenditure on research and development (R&D),the national megaprojects implementation of state-dominated megaprojects should solve major national problems and meet the national great strategic demand according joint research of multiunit and multi-discipline.The importance of management for research cooperation agreement and business contracts are increasingly prominent.Based on the author's own experience,a scientific research institute's perspective,contractual agreements on the management audit and review the content of the key points are summarized.It gives the reference for the staff of scientific management.

Vitamine E in peanut oil was determined by means of f Juorometric method (Ex = 285nm, Em=324nm) and TLC scanning method (? = 292nm). Both methods include saponification of the oil, isolation o'f the unsaponifiable matters and the respective final measurements. The result of the former method indicates the total vitamine E in terms of ?-vitamine E and the latter method may indicate their ?,?,? and 6 analogues separately. These two methods are simple, time-saving and reliable. The recovery of VE and the standard deviation of replication of fluorometric method and TLC scanning method are 99.3%, ? 0.587mg/100g sample and 103.04%, ?0.959mg/100g sample respectively. Both methods are also applicable to other foods such as fortified cakes, confectionaries, infant foods etc., but the oil in these samples must be extracted before saponification.

Objective To investigate the therapeutic effects of recombinant yeast hepatitis B virus(HBV)vaccine combined with interferon(IFN)α-1b and determine the rational dosage of HBV vaccine for the further clinical study with larger sample.Methods Two hundreds and sixteen patients with chronic hepatitis B(CHB)were enrolled in this randomized,multi-center,double-blinded and placebo-controlled clinical trial.All the subjects were not treated with antiviral drugs within 6 months and randomly divided into 90μg,60μg and placebo groups with a ratio of 1:1:1.All the patients were intramuscularly administrated with 90μg or 60μg recombinant HBV vaccine or placebo at week 0,2,6,10,14,18,22,respectively.Meanwhile,they were also treated with IFNα-1b 50μg,3 times a wcek for 24 weeks.All patients were followed up for 24 weeks after withdrawal of anti-HBV therapy.Serum HBV DNA level,HBeAg titer and liver function were monitored frequently.Interferon-γ secreting lymphoeytes were detected by Enzyme-linked immunospot(ELISPOT)in part of the patients.Results The serum HBV DNA levels were(6.03±1.79),(5.52±1.82)and(6.29±1.70)log10 copy/mL at week 24 of treatment in high dose,low dose and placebo groups,respectively (P=0.458).And the serum HBV DNA levels were(5.92±1.98),(5.49±1.99)and(6.16±1.76)log10 copy/mL at weck 24 after withdrawal of treatment,respectively(P=0.720).The rates of patients whose HBV DNA＜1×105 copy/mL in these three groups were 30.4%,39.4% and 20.8% at week 24 of treatment,respectively and there was significant difference between high dose group and low dose group(P=0.015).The rate of patients whose HBV DNA＜1×105 copy/mL at week 24 after withdrawal was highest in low dose group,but no significant differences before and after treatment in these three groups(P=0.257).The HBV DNA negative rates were 17.4%,25.4% and 6.9% in these three groups,respectively,which were significantly different(P=0.012).At week 24 of treatment and week 24 after withdrawal of treatment,the alanine aminotransferase normalization rate,HBeAg seroconversion rate were highest in low dose group,but no significant differences in these three groups.ELISPOT positive rates at week 24 of treatment and week 24 after withdrawal of treatment in high close and low dose groups were higher than that in placebo group(P＜0.05).The incidence of adverse events was similar and there was no drug related severe adverse events in each group.Conclusion Recombinant HBV vaccine maybe contribute to anti-HBV therapy and 60μg of dosage seems to be rational.

Objective:To evaluate the efficacy and safety of drug-coated balloon (DCB) with paclitaxel in the treatment of femoropopliteal arteriosclerosis obliterans (ASO).Methods:From Dec 2016 to Dec 2018, clinical data of femoropopliteal artery disease patients treated with paclitaxel DCB in Renji Hospital, School of Medicine, Shanghai Jiaotong University were retrospectively analyzed.Results:A total of 83 patients (95 lower limbs) underwent DCB therapy. Including 50 chronic total occlusion (CTO) lesions (52.6%) with mean lesion length of (18.35±10.61) cm. Twenty-four lesions (25.3%) were moderately or severely calcified. Bail-out stent implantation was performed in 29.5% cases. The mean follow-up time was 17.5 months. Twelve months after intervention, the all-cause mortality rate was 6.0%, the major amputation rate was 4.3%, the primary patency rate was 60.6%, the primary assisted patency rate was 72.4%, the secondary patency rate was 83.4%, and the freedom rate from clinically-driven target lesion revascularization(F-TLR) was 77.0%. Moderate to severe calcification was an independent risk factor for the primary patency of DCB therapy.Conclusion:DCB is a safe and effective endovascular therapy for femoropopliteal artery disease.

Objective:To analyze the clinical efficacy and safety of endoluminal treatment of complex renal artery aneurysm (RAA).Methods:The clinical data and follow-up results of 19 patients with complex RAA admitted to Renji Hospital, Shanghai Jiaotong University School of Medicine from November 2014 to September 2021 were retrospectively analyzed. Two patients were treated with simple spring coil embolization into the aneurysmal artery, 14 patients were treated with simple spring coil embolization of the aneurysmal cavity, and 3 patients were treated with stent-assisted + spring coil embolization technique. Based on the location of the aneurysm, RAA were classified into type Ⅰ, Ⅱ, and Ⅲ. 7 patients with type Ⅰ, 10 patients with type Ⅱ, and 2 patients with type Ⅲ were studied. Variance analysis was used to compare the differences in glomerular filtration rate(GFR) of the affected side when the tumor was located at different locations, and Pearson was used to analyze the correlation between the number of coils implanted and the size of the tumor and GFR.Results:Ten of the 19 patients who were underwent successful endoluminal intervention. The average size of the patients′ aneurysms was (20.89±6.65) mm, and the average number of spring coils implanted was 8.22±3.08. The preoperative and postoperative serum creatinine were in the normal range, and no RAA tumor enlargement or recurrence was found during the follow-up period. The postoperative GFR was abnormal in patients with type Ⅰ, type Ⅱ, and type Ⅲ renal aneurysms, and the mean GFR value differed among the three types of patients( P=0.003). There was a negative correlation between the postoperative GFR values of the affected kidney and the number of spring coils implanted ( P=0.047), and no significant relationship between GFR and aneurysm size. Conclusion:The endovascular technique is an effective and safe means of treating complex RAA.

Breast cancer remains a leading cause of mortality in women worldwide.Triple-negative breast cancer(TNBC)is a particularly aggressive subtype characterized by rapid progression,poor prognosis,and lack of clear therapeutic targets.In the clinic,delineation of tumor heterogeneity and development of effective drugs continue to pose considerable challenges.Within the scope of our study,high hetero-geneity inherent to breast cancer was uncovered based on the landscape constructed from both tumor and healthy breast tissue samples.Notably,TNBC exhibited significant specificity regarding cell prolif-eration,differentiation,and disease progression.Significant associations between tumor grade,prog-nosis,and TNBC oncogenes were established via pseudotime trajectory analysis.Consequently,we further performed comprehensive characterization of the TNBC microenvironment.A crucial epithelial subcluster,E8,was identified as highly malignant and strongly associated with tumor cell proliferation in TNBC.Additionally,epithelial-mesenchymal transition(EMT)-associated fibroblast and M2 macrophage subclusters exerted an influence on E8 through cellular interactions,contributing to tumor growth.Characteristic genes in these three cluster cells could therefore serve as potential therapeutic targets for TNBC.The collective findings provided valuable insights that assisted in the screening of a series of therapeutic drugs,such as pelitinib.We further confirmed the anti-cancer effect of pelitinib in an orthotopic 4T1 tumor-bearing mouse model.Overall,our study sheds light on the unique characteristics of TNBC at single-cell resolution and the crucial cell types associated with tumor cell proliferation that may serve as potent tools in the development of effective anti-cancer drugs.

Objective To investigate the clinical value of fetal echocardiography in prenatal diagnosis , classification and outcome of abnormal origin of pulmonary artery from ascending aorta ( AOPA) . Methods From January 2014 to June 2018 ,18 cases of AOPA diagnosed by echocardiography in 43966 fetuses from Shaanxi Fetal Congenital Heart Disease Diagnostic Center were retrospectively analyzed . The echocardiographic features ,pathological and anatomical classification ,genetic characteristics and outcome of postnatal echocardiography were summarized . Results Abnormal origin of pulmonary artery branches could be demonstrated by color Doppler imaging system in 18 cases . Ten cases ( 55 .6％ ) of right pulmonary artery abnormalities originated from ascending aorta ( AORPA ) ,in which 6 cases ( 60％ ) were distal pulmonary artery abnormalities ,4 cases ( 40％ ) were proximal pulmonary artery abnormalities . Eight cases( 44 .4％ ) of left pulmonary artery abnormalities originated from ascending aorta ( AOLPA ) ,including 7 cases ( 87 .5％ ) of distal pulmonary artery abnormalities and 1 case ( 12 .5％ ) of proximal pulmonary artery abnormalities . Twelve AOPAs were associated with other intracardiac malformations with 6 right ventricular double outlet (DORV) accompanying with pulmonary stenosis ,3 tetralogy of fallots( TOF) ,2 atrioventricular septal defects( AVSD) ,1 single ventricle with single atrium ,and 1 Berry syndrome ,and no casese were associated with extracardiac malformations .Amniocentesis karyotype analysis and gene chip detection in 5 cases showed normal results . Four of 18 cases were born ,in which 3 cases died and 1 case was progressively suffered with right pulmonary artery atresia ,10 cases were inducted of labor ( 4 autopsy) and following-up was lost in 4 cases . Conclusions Incidence of AOLPA and distal type in fetal AOPA is higher than that in child and adult from references ,DORV is abnormal type mostly associated with intracardiac malformation . AOPA has no obvious genetic result for less cases . The main risk after AOPA birth is pulmonary infection . Echocardiography is of great value for AOPA in prenatal diagnosis , outcome observation and surgical planning after birth .