OBJECTIVETo investigate the efficacy of short-term full-dose prophylaxis in adult Chinese patients with severe hemophilia A.

METHODSThirteen adult Chinese patients with severe hemophilia A receiving on-demand or low-dose prophylaxis underwent ultrasound examination of the target joints and evaluation of Hemophilia Joint Health Score (HJHS). The data of annual bleeding episodes in the period of on-demand or low-dose prophylaxis were collected retrospectively from the patients, and the changes in bleeding and joint condition (ultrasound findings of the target joints and HJHS) were observed during short-term full-dose prophylaxis. The activity intensity of the patients was assessed using the IPAQ questionnaire, and the 72 h FⅧ trough activity was measured during full-dose prophylaxis.

RESULTSThe median age of the 13 patients was 26.0 (20.5-29.0) years. For full-dose prophylaxis, the patients received a median therapeutic dose of 31.0 (29.1-33.0) IU/kg, administered for 3 times per week; the median 72 h FⅧ trough activity of patients was 1.7% (1.3-3.4%). During the follow-up period for 3 months, the annual bleeding rates (ABR) and annual joint bleeding rates (AJBR) decreased significantly in all the patients (=0.001 and 0.001, respectively), but zero bleeding was achieved in only 4 patients (30.8%) and zero joint bleeding in 7 patients (53.8%); 9 patients (69.2%) still experienced breakthrough bleeding. The damage severity of target joints assessed by ultrasound and HJHS in 6 patients (46.2%)was worse than before and no obvious progression of target joints damage was found in 7 patients (53.8%). Compared with the patients without progression, the patients with worsened joint damage had poorer baseline joint condition, higher bleeding frequencies before and during the follow-up, a higher intensity of physical activity, and a lower baseline FⅧ activity.

CONCLUSIONSAt present, although short-term full-dose prophylaxis can significantly reduce the bleeding and partially prevent the progression of joint damage, it is not yet possible to achieve the goal of zero bleeding for all adult patients with severe hemophilia A in China, nor can it completely prevent further joint damage. For adult patients with different clinical bleeding phenotypes, joint conditions and physical activity intensity, individualized therapy involving additional evaluation methods should be implemented, and physiotherapy and surgical intervention can be considered when necessary.

Objective To investigate the echocardiographic features of complete transposition of the great arteries (TGA)in fetuses.Methods Prenatal echocardiographic data of 9 fetuses diagnosed as TGA by autopsy or postnatal echocardiography during January 2010 to January 2017 were retrospectively analyzed.Results All of 9 fetuses showed normal cardiac axis and atrioventricular connection on four-chamber view.Eight of them showed the baby bird's beak sign on left ventricular outflow tract view.On left and right ventricular outflow tracts view,the two great arteries were parallel in 8 fetuses.Totally 6 fetuses showed just 2 vessels on three vessels and tracheal (3VT) view.On aortic arch view,the radian of aortic arch had increased in different degrees in 7 fetuses.There were 4 fetuses with ventricular septal defect observed by both of four-chamber and left ventricular outflow tract views.Conclusion The echocardiographic features of fetuses with TGA are characteristic in left ventricular outflow tract,left and right ventricular outflow tracts,3VT and aortic arch views,including baby bird's beak sign,2 great arteries' parallel relations,only 2 vessels on 3VT view,and increased radian of aortic arch.Of these features,baby bird's beak sign is the most common.

Objective To establish the quality standard of Catharsis Tea. Methods Rhei Radix et Rhizoma, Scutellariae Radix and Aurantii Fructus were identified by thin-layer chromatography (TLC). HPLC method was established for the determination of emodin and rhein, performed on SunFire C18 column (4.6 mm×250 mm, 5 μm), with methanol-0.1%H3PO4 (85∶15) as the mobile phase at the flow rate of 1.0 mL/min, column temperature at 30 ℃, and detection wavelength of 254 nm. Results The TLC was exclusive. Within the range of 0.448-1.344 μg, emodin presented a fine linear relationship, r =0.999 7. Within the range of 0.011-0.077 μg, rhein presented a fine linear relationship, r=0.999 8. The recovery of rhein and emodin was 98.52% and 97.53% with RSD of 1.02% and 1.15% (n=6) respectively.Conclusion The methods are reliable, accurate, reproducible, and can be used for the quality control of Catharsis Tea.

Objective To investigate the effect of early application of aldosterone receptor blockade-spironolactone on cardiac remodeling in patients with acute myocardial infarction.Methods A total of 616 patients were enrolled in this prospective study,who were admitted to the CCU Division of the First Affiliated Hospital of Harbin Medical University from January 2008 to January 2010.Inclusive criteria were as follows:First-onset STEMI according to the diagnostic criteria in ST-segment elevation acutemyocardial infarction diagnosis and treatment guidelines and admitted within 24 hours from onset.Exclusive criteria were as follows:Non-STEMI,only right ventricular infarction and old myocardial infarction; cardiac function Killip Class Ⅳ or hypotensive state; renal dysfunction(serum creatinine ＞ 221 μmol / L); serum potassium ＞5.0 mmol / L; longer than 24 hours from onset and older than 75 years of age.All the patients were randomly divided into a control group of standard therapy and a treatment group of standard therapy combined with spironolactone 20 mg per day.Totally 528 patients were observed completely,including 266 patients in the control group and 262 patients in the treatment group.There was no statistical difference of clinical characteristics between the two groups,including age,gender,past medical history,admission situation,and treatment(P ＞ 0.05).The effect of spironolactone on cardiac remodeling、left ventricular function、renal function and blood levels of potassium were evaluated by detecting echocardiography,serum potassium and serum creatinine at one month and one year follow-up.Results Compared with the control group,the echocardiography indicators such as LVESD,LVEDD,LVEF,LAD-ML and LAD-SI were significantly improved in treatment group at one year(P ＜ 0.05).In treatment group,LVESD,LVEDD,LVPWT,LVEF,LAD-ML and LAD-SI were significantly improved at one year than one month(P ＜ 0.05,and P =0.007 to LVEF),and LVEF was significantly improved at one year than one month in control group(P =0.0277).There were no statistical differences of serum potassium and serum creatinine between the two groups.Conclusions On the basis of conventional treatment,the early combination of low dose spironolactone(20 mg/d)could inhibit cardiac remodeling at late stage and prevent heart failure.

Objective To investigate the hypoglycemic effects of Laminaria japonica (L. japonica) on diabetic model induced by alloxan in rats. Methods Sixty healthy female rats were used to establish diabetic models by injecting alloxan peritoneally, and L.japonica was applied as raw materials for potential marine drugs.The levels of fasting blood glucose (FBG) were detected by automatic blood glucose device. Enzyme linkedimmunoabsorbant assay was applied to determine the insulin level in serum. The shape and structure of isletcells were observed with histopathological staining, and the expression of superoxide dismutase (SOD) and inducible nitric oxide synthase (iNOS) in islet cells were detected by immunohistochemical technique. Results After the treatment, the levels of FBG of L.japonica treated group B [(9.37±1.70) mmol/LandC (9.18±1.65 ) mmol/L, F= 32.81, q=6.35～11.72, P<0.05 ] reduced, while the serum levels of insulin in treated group A, Band C (0.0378±0.0026, 0.0378±0.0027, 0.0367±0.0035) increased(F= 11.40, q=4.28～8.47, P<0.05) significantly than those of diabetic model group (0.0456 ±0.0057) . The shape and structure of islet cells improved with the up-expressing SOD(t=4.73～4.76, P<0.05)and down-expressing iNOS (t=4.81～5.30, P<0.05) in L.japonica treated group B and C than those in diabetic model group. Conclusion L.japonica might decrease the serum level of FBG through promoting the islet cell recovery by an anti-oxide effect.

Objective To determine the prevalence and prognostic implication of hypocalcemia in the set-ring of acute myocardial infarction. Method The study consisted of 1274 consecutive patients presenting with a-cute myocardial infarction. Plasma calcium concentrations were obtained on the 1st day, 2nd day, 3rd day after admission. According to the plasma calcium concentrations, 1274 patients with AMI were designed into four groups:group A (Ca2+≥2.20 mmol/L),gronp B (2.00～2.19 mmol/L), group C (1.90～1.99 mmol/L),group D (Ca2<1.9 mmol/L). Echocardiographic examination was performed on day 2 or 3 of hospitalization.Results There were 606 patients (47.6%) having hypocalcemia, defined as a plasma calcium level <2.2mmol/L. Among them, there were 476 patients(37.4%) in group B(2.00～2.19 mmol/L), 73 patients(5.7 %)in group C (1.90～1.99 hanoi/L) and 45 patients(3.5%)in group D(Ca2+< 1.90 retooL/L). The mortality of group A was 2.5 %, on the contrary, the mortality of the other groups was 7.8 %, 20.5 %, 17.8 %. The difference was significant. In multivariate logistic regression analysis, hypocalcemia was independently associated with 30-day mortality. Conclusions Hypocalcemia in patients with acute myocardial infarction is an independent predictor of 30-day mortality.

Objective To study the efficacy of the percutaneous thrombectomy for the no-reflow of acute myocardial infarction(AMI)in elderly patients with angiographically proven thrombus.Methods A total of 46 patients(≥60 years old)with AMI and coronary thrombus shown by angiography were randomly divided into group of PCI therapy(23 cases)and group of PCI plus percutaneous thrombectomy(23 cases).At 24 hours and 1 week after PCI,real-time imaging was performed by contrast pulse sequencing technology.Contrast score index(CSI),endocardial length of contrast defect(CDL),regional wall motion score index(WMSI)and wall motion abnormality(WML)were calculated. Results At each time point,in patients treated with a pereutaneous thrombectomy,CSI,CDL/LV length(%),WMSI and WML/LV length(%)were significantly lower than in PCI group[(2.0±0.3)vs.(2.5±0.7),(1.5±0.4)vs.(2.0±0.5),(15.5±5.4)%vs.(22.8±4.9)%,(9.6±2.5)%vs.(21.5±4.6)%,(2.1±0.5)vs.(2.4±0.7),(1.5±0.5)us.(2.1±0.5),(24.5±5.3)%vs.(35.6±8.3)%,(15.9±5.0)vs.(27.6±6.4)%,P<0.05 or P<0.01]. Conclusions The beneficial effect of the thrombectomy occurs at the microvascular level.Thrombectomy reduces the no-flow and the extent of microvascular obstruction,thus it was a feasible therapy in elderly patiens with AMI.

OBJECTIVETo investigate the effects of thoracic epidural blockade (TEB) on plasma fibrinogen (FIB) levels.

METHODSThirty cases of dilated cardiomyopathy (DCM) were selected randomly into a TEB group and a control group. TEB patients were subjected to a persistent TEB (T1 - 5), and injected with 0.5% lidocaine 3 - 5 ml every two or four hours for four weeks in addition to routine medicine, while patients in the control group were given routine medicine only. Plasma concentrations of FIB were measured using the micro-capillary assay. Doppler echocardiography was performed before and after the treatment.

RESULTSPlasma concentrations of FIB in two groups were greater than the normal value before the treatment. There was a significant decrease of plasma concentrations of FIB in the TEB group after the treatment (4.2 +/- 1.3 g/L vs 3.6 +/- 0.9 g/L, P < 0.05), but there was no significant change in the control group (4.2 +/- 1.2 g/L vs 4.3 +/- 1.9 g/L, P > 0.05). After four weeks of treatment, the left ventricular end diastolic diameters (LVEDD) of TEB patients were reduced (72 +/- 10 mm vs 69 +/- 10 mm, P < 0.05) and the left ventricular ejection fraction (LVEF) of TEB patients increased significantly (33% +/- 13% vs 44% +/- 14%, P < 0.05). In contrast, LVEDD (73 +/- 11 mm vs 73 +/- 12 mm, P > 0.05) and LVEF (32% +/- 14% vs 33% +/- 12%, P > 0.05) did not change significantly in the control group.

CONCLUSIONSThe results suggest that plasma FIB levels in patients with DCM were decreased by performing a TEB, in addition to a reduction of the enlarged cardiac cavity and an improvement in cardiac systolic dysfunction. TEB might contribute to lowering the occurrence of thrombus and thromboembolism in patients with DCM. TEB might be a promising therapeutic method to improve the prognosis of DCM patients.

Adult ; Aged ; Anesthesia, Epidural ; Autonomic Nerve Block ; Cardiomyopathy, Dilated ; blood ; therapy ; Female ; Fibrinogen ; analysis ; Humans ; Male ; Middle Aged

Objective To investigate if diastolic function could be improved by cardiac sympathetic nerve blockade in patients with dilated cardiomyopathy (DCM). Methods Sixty consecutive cases of DCM were randomed into therapeutic group and control group. Patients in therapeutic group were administered thoracic epidural blockade (TEB) (T 1-5 ) with 0.5 % lidocaine intermittent injection every two hours for four weeks, in addition to some routine medicine, while patients in control group were only administered routine treatment. Then the parameters of left ventricular diastolic function were evaluated with Doppler echocardiography before and after four weeks. Results ①All the patients in TEB group felt better within five minutes after injection of lidocaine within local epidural cavity.Their symptoms were relieved more rapidly than those of the drug therapy alone. Some patients with NYHA class Ⅳ could lie down supine in a short time. Exercise tolerance increased in patients with NYHA class Ⅱ to Ⅲ. But symptoms and signs of patients in control group were relieved very slowly, some patients even got worsen. ② Thirteen of thirty in therapeutic group got improvement on diastolic function after treatment. One of thirty in this group deteriorated. In detail, six of fifteen patients with demonstrated improvement of left ventricular diastolic filling changed from having a restrictive filling pattern to having a pseudonormal left ventricular filling pattern. Four of nine patients with a pseudonormal filling pattern changed to have a delayed filling pattern.Three of six patients, left ventricular filling pattern moved from a pseudonormal left ventricular filling pattern to a normal filling pattern. Only one patient acquired a restrictive filling pattern from a pseudonormal filling pattern. In contrast, in control group one of thirty got improvement on diastolic function after treatment. Nine of thirty in this group deteriorated. Individual changes of left ventricular filling pattern showed that only one patient improved from a delayed filling pattern to normal filling pattern, whereas seven patients had a restrictive left ventricular filling pattern developed from a pseudonormal filling pattern and another two patients achieved a pseudonormal filling pattern from delayed filling pattern. Conclusions Diastolic function in patients with DCM was improved significantly by cardiac sympathetic nerve blockade. The approach has beneficial effects on the patients of DCM.

Objective To explore the mechanism of effects of cardiac sympathetic anesthesia on left ventricular ejection fraction(LVEF) and left cardiac cavity size of patients with dilated cardiomyopathy.Method 121 consecutive patients with dilated cardiomyopathy were divided into cardiac sympathetic nerve blockade group(TEA group) and control group(c group).In TEA group,5％ lidocaine was injected into thoracic epidural cavity for about 4 to 8 weeks in addition with routine therapy.In c group,only routine therapy was used.We observe the changes of LVEF and left cardiac cavity size before and after treatment in both groups. Result In TEA group,after anesthesia,LVEF was increased from(31.3± 12.8) to(47.3± 21.3),P<0.001;left ventricular end－ diastolic diameter was reduced from(69.1± 7.1)to (65.1± 8.0),P<0.001;left atrial diameter was decreased from(44.0± 6.2)to(39.4± 7.2),P< 0.001. Conclusion Cardiac sympathetic anesthesia can effectively improve the ejection performance of dilated cardiomyopathy and make the dilated cardiac cavity turn to normal level.