Comprehensive therapy is the leading method to improve the overall survival for patients with advanced gallbladder cancer, especially the study of new chemotherapy regimen are on demand.From 2010 to 2011, a phase Ⅲ study of new chemotherapy regimen in the treatment of advanced gallbladder carcinoma would be undertaken nationwide to assess the clinical outcome and safety, in 31 clinical centers led by the department of General Surgery of Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, and a total of 260 patients would be accrued for this study.Chemotherapy consists of 2 regimes, conventional regime(cisplatin, epirubicin, and 5-fluorouracil, leucovorin)and new regime(conventional regime plus somatostatin).At present, the study has been approved by Clinical Trial Registration Platform of National Institutes of Health in U.S.A., and more than 30 patients are rolling in the study.The clinical trial could provide the high-quality evidence of EBM(evdence based medicine)for the chemotherapy of advanced gallbladder caner, and it will be one of the important components in the large-scale clinical study of chemotherapy for the advanced gallbladder cancer in China and worldwide.

Objective To analyze the prognostic value of baseline serum free light chain (sFLC) in light-chain (AL) cardiac amyloidosis.Methods Twenty-seven patients with AL cardiac amyloidosis were retrospectively reviewed from January 2014 to January 2015.sFLC was measured by immuoturbidimetric assay.Baseline characteristics,echocardiographic parameters and electrocardiogram data were analyzed.According to the median baseline dFLC (involved sFLC minus uninvolved sFLC),patients were categorized into either the low dFLC(≤307mg/L) or the high dFLC group (＞307mg/L).Results More subjects in the high dFLC group with early/late diastolic mitral velocity ratio (E/A ratio) over 2 (71.4％ vs 30.8％,P =0.035),and subjects in this group had a shorter median survival time than those in the low dFLC group (3 months vs 17 months,P =0.004).A similar phenomenon for median survival time was observed when the subjects were redivided either by a new cut-off value of 180mg/L for dFLC (low dFLC group:17 months;high dFLC group:4 months,P =0.014) or a κ/λ ratio,in which subjects with κ type sFLC-ratio ≤ 19.6 and λ type sFLC-ratio ＞0.065 were in the low sFLC-ratio group (17 months) and those with κ type sFLC-ratio ＞ 19.6 and λ type sFLC-ratio ≤0.065 were in the high sFLC-ratio group (4 months,P=0.023).In multivariate analysis,dFLC and New York Heart Association (NYHA) classification of cardiac function were two risk factors associated with all-cause mortality in patients,among which the hazard ratio for higher dFLC was 4.28 (95％ CI 1.55-11.8,P =0.005).Conclusion The level of sFLC could be a marker for the prognosis of AL cardiac amyloidosis.

Amyloidosis ; Heart Diseases ; Humans

Objective To investigate the clinical characteristics of Takayasu's arteritis (TA) with pulmonary hypertension (PAH) in order to improve the diagnosis and treatment earlier. Methods Twelve out of 191 patients with TA registered in Peking Union Medical College Hospital from 1987 to 2007 were diagnosed as PAH, the clinical data of 12 patients were analyzed. Results Ten patients were females. The range of age were from 14 to 47 years old, the average age was (27±10) years old. Eleven patients had the clinical manifestations or/and signs of pulmonary artery involvement. Seven patients presented with short breath after exercise or hemoptysis as the first manifestation, four patients with fatigue, four patients with intermittent claudication or pain or numbness of extremities, three patients with dizziness. Seven patients belonged to type Ⅰ+Ⅳ, one patient to type Ⅱ+Ⅳ, three patients to type Ⅲ+Ⅳ, one patient to type Ⅴ. Elevated ESR/CRP was found in ten patients. All patients took the glucocorticoid and DMARDs, stent implantation in pulmonary artery was done in one patient, Bentall was operated in another patient.The symptoms of all patients improved except one patient died for low cardiac output after operation. Conclusion PAH is one of the severe complications in late stage of TA, and other arteries are usually involved too. Because it is difficult to observe PAH in TA patients in early stage, CTA or pulmonary angiography and UCG should be taken in early stage. The stent implantation or dilating the artery should be considered aa a treatment, but at on the same time, glucocorticoid and DMARDs should be taken to avoid the relapse.

Objective To explore the potential role of brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide(NT-proBNP) in the assessment of patients with connective tissuediseases(CTD) associated pulmonary arterial hypertension (PAH). Methods Thirty patients with CTD associated PAH were evaluated by WHO function, echocardiography, right heart catheterization and blood biomarkers. All the clinical data was analyzed statistically. Results All patients [age (39.5 ± 11.6) yr]were female. Both NT-proBNP and BNP were significantly increased and significantly correlated ( all P ＜0. 05 ) respectively with mean pulmonary arterial pressure ( r = 0. 53 and r = 0. 40 ), right arterial pressure ( r = 0. 55 and r = 0. 42 ), pulmonary vascular resistance ( r = 0. 69 and r = 0. 61 ), cardiac index ( r = - 0. 58and r = - 0. 46), mixed venous blood oxygen saturation ( r = - 0. 62 and r = - 0. 54 ), pericardial effusion ( r = 0. 46 and r = 0. 43 ), right atrial sizes ( r = 0. 40 and 0. 53, and r = 0. 39 and 0. 45 ) and right ventricular size ( r = 0. 55 and r = 0. 37 ). Furthmore, NT-proBNP, but not BNP, significantly correlated with WHO function class ( r = 0. 55 ). Conclusion Blood NT-proBNP and BNP were elevated in patients with CTD associated PAH and paralleled the extent of function class, pulmonary hemodynamic changes and right ventricular remodeling.

objecfive To analyze ventricular myocardial function in patients with systemic sclerosis (SSc).Methods Thirtv female SSc patients and fifteen age-and sex-comparable healthy subjects underwent standard echo and tissue Doppler imaging(TDI)examination.T-test and Pearson's analysis were used.Results Both left and right atrial and ventricular diameters,left ventricular eiection fraction and right ventricular fraction of area change were comparable between the two groups.Mitral and tricuspid inflow E/A ratio was almost the same in both groups,while systolic pulmoHary pressure was increased in SSc [(38±16)vs (23.9±4.8)mm Hg,P=0.008].TDI detected impaired right ventricular myocardial function in SSc:eartydiastolic peak velocity Em [(10.7±4.8)vs(15.0±2.1)cm/s,P=0.01],peak systolic strain[(-16.3±3.9)%vs (-20.9±2.0)%,P=0.001]and strain rate[(-1.4±0.4) vs(-2.4±0.5)/s,P<0.01] were reduced significantly.Left ventricular myocardial function was not impaired in SSc patients.Conclusion TDI iS a valuable noninvasive tool for early detecting of ventricular myocardial involvement caused by SSc.

Objective To explore the association between Doppler echocardiography parameters and pulmonary capillary wedge pressure (PCWP) obtained by right heart catheterization (RHC) in patients with pulmonary hypertension (PH).Methods RHC and a simultaneous echocardiography were performed in 86 PH patients.The correlation between the echocardiographic parameters and PCWP was analyzed.Results The early diastolic velocity(E) of mitral valve blood flow was significantly correlated with PCWP ( γ =0.36,P ＜0.01 ).Tissue Doppler imaging measured the diastolic velocity of mitral annulus on the lateral(E'L)and the septal (E'S) wall.Then the average E'M was calculated.The ratio of E to E'L,E'S and E'M showed a strong correlation with PCWP ( γ =0.62,0.34 and 0.47 respectively,P ＜ 0.01 ).E/E'L ＜ 6 accurately predicted 100％ PCWP≤ 15 mm Hg (1 mm Hg =0.133 kPa),and E/E'L ＞ 15 could predict PCWP ＞ 15 nun Hg with specificity of 98.5％.Conclusion There are positive correlation between E/E' and PCWP obtained by RHC in PH patients.

Objective To investigate the clinical and pathological features of eosinophilic diseases with cardiac involvement Methods We analyzed the clinical and cardiac pathological data of 7 patients with eosinophilic diseases with cardiac involvement under endomyocardial biopsy or autopsy.Results Seven patients (5 male, average age 51 years) were enrolled.Four patients were diagnosed as idiopathic hypereosinophilic syndrome and three were Churg-Strauss syndrome.Peripheral blood eosinophila count increased significantly in all patients.Cardiac involvement included angina pectoris, myocardial infarction, heart failure, presyncope and sudden death.Electrocardiogram showed cardiac ischemia, bundle branch block and third degree atrioventricular block.Echocardiography suggested ventricular and atrial enlargement, decreased ventricular systolic function, pulmonary hypertension, valvular prolapse and insufficiency and endocarditis.Pathology displayed infiltration of eosinophils, formation of granulomata, necrotizing vasculitis, myocardial necrosis and endomyocardial fibrosis in heart.Coronary artery could be also affected and led to myocardial infarction.Conclusions Cardiovascular complications of eocinophilic diseases are a major source of morbidity and mortality in these disorders.The manifestations are multiple and early recognition and treatment with steroid and immunosuppressant can improve prognosis.

Objective To detect the positive rate of anti-endothelial cell antibody (AECA) in patients with pulmonary arterial hypertension (PAH) associated with connective tissue diseases(CTD)and to investigate the specific target antigen.Methods Sera of 68 patients with CTD associated PAH were collected to detect AECA by Western blotting with extracted membrane protein of the endothelial cell line EA.hy926.Sera of 61 CTD patients without PAH,20 with idiopathic pulmonary arterial hypertension(IPAH),20 with chronic obstructive pulmonary diseases and pulmonary arterial hypertension (COPD-PAH) and 20 healthy donors were collected as controls.The correlation between PAH and specific bands of AECA was studied by X2 test.Liquid chromatography-electrospray ionization mass spectrography was used to detect the target antigens related to PAH associated with CTD.Results The specific molecular size of antigen was 78 000.The AECA-78 000positive rate of CTD patients with PAH was 79% (54/68).not significantly ditierent from that of CTD with glomerulonephritis(71%,15/21),but significantly higher than those of CTD with interstitial lung disease (ILD)(15%,3/20)and CTD without systemic involvement(P<0.01 and P<0.05 respectively).also higher than those of IPAH(8%,1/12).The AECA-78 000 was negative in COPD-PAH and healthy controls.The target antigen of AECA-78 000 was identified by proteomic techniques as moesin.Conclusion CTD patients with different target organ involvement have different AECA-78 000 positive rates,which could be frequently detected in CTD associated PAH and those with glomerulonephritis.The common antigen is moesin.

Objective To study the clinical,cardiopulmonary functional and hemodynamic profiles of systemic sclerosis patients with pulmonary hypertension (SSc-PAH) compared with those of idiopathic pulmonary hypertension (IPAH).Methods Patients diagnosed with SSc-PAH or IPAH by right heart catheterization were consecutively enrolled into the study between 2011 and 2013 in Peking Union Medical College Hospital (PUMCH).Cases with pulmonary hypertension related to other diseases were excluded.Demographic data,laboratory parameters,6 minutes walk distance (6MWD),pulmonary function and hemodynamic variables at the time of diagnosis were collected and compared between the two groups.Results A total of 20 SSc-PAH patients including 19 females and one male with age of (43.1 ± 12.2) years,and 18IPAH patients including 16 females and 2 males with age of (38.4 ± 12.4) years were enrolled in this study.Subjects in both groups had similar mean pulmonary arterial pressure,cardiac index and pulmonary vascular resistance (PVR) when recruited.Compared with IPAH patients,SSc-PAH patients showed significantly decreased all parameters including forced vital capacity (FVC)％ [(77.1 ± 13.2)％ vs (88.6 ± 14.9)％,P =0.026],diffusing capacity of the lung for carbon monoxide (DLCO) ％ [(46.2 ±13.1) ％ vs (66.6± 13.3)％,P ＜0.001],DLCO/alveolar ventilation(VA) [(55.1 ± 14.3)％ vs (75.1 ± 11.5)％,P ＜0.001],and 6MWD [(365.6 ±85.1) m vs (454.3 ± 136.8) m,P =0.034].In subgroup analysis of SSc-PAH patients,elevated PVR (OR 2.122,95％ CI 1.093-4.119,P =0.026) and decreased DLCO％ (OR 0.916,95％ CI 0.842-0.996,P =0.040) were independently associated with reduced 6MWD.Conclusions Under the similar hemodynamic condition,SSc-PAH patients had more severe restrictive ventilation dysfunction and diffusion capacity dysfunction.Decreased 6MWD in SSc-PAHpatients was probably related to the impairment of pulmonary function.