A COMPARISON OF "CARRIER SCREENING" OF HEMOPHILIA BY THE RATIO OF FACTOR VIII COAGULANT ACTIVITY OR ANTIGEN TO FACTOR VIII RELATED ANTIGEN

Abstract: The ratio of factor VIII coagulant activity (VIIIC) or antigen (VIIICAg) to that of factor VIII related antigen (VIIIRAg) was measured in 15 normals, 21 obligatory and 23 possible carriers of hemophilia. Factor VIII coagulant was measured on fresh plasma samples whereas antigenic properties were measured on frozen and thawed samples. In obligatory carriers only, the mean level of VIIICAg was significantly lower than VIIIC and there was a tendency for low VIIICAg levels to be associated with raised VIIIRAg levels. Using both ratios, 13 obligatory carriers were outside the normal tolerance ellipse. In possible carriers, neither ratio showed superior discriminating power. In reference laboratories that perform carrier studies on stored or transported specimens, measurement of VIIICAg/VIIIRAg is a suitable test for diagnosis of carriers.     

A longitudinal study of immunological status in Chinese haemophiliacs: importance of the heat viral inactivation of factor concentrates. I. Immunological associations with the consumption of factor concentrates

Summary . Twenty-four of 117 cases of haemophilia A (20.5%) and none of 18 cases of haemophilia B reported in this study had an antibody to the human immuno-deficiency virus (HIV). Both groups of patients showed similar immunological alterations. HIV-seropositive haemophilia A patients had an increased CD8 cell count and a similarly decreased CD4/CD8 ratio as compared to HIV-seronegative haemophilia A patients. Multiple regression analysis for the association of CD4/CD8 ratio with HIV infection status and dosage of plasma products in haemophilia A and B patients, respectively, revealed that there was a significant negative association of ln(CD4/CD8) with dosage of factor VlII concentrates (P = 0.0435) and factor IX concentrates (P = O.O028), respectively. N o association occurred between CD4/CD8 ratio and HIV infection as well as dosage of other plasma products. These data indicate that the immunological abnormalities of our haemophilia A and B patients in their early years were primarily caused by various viral infections and/or a suppressive effect of allogeneic protein through infusion of factor concentrates and not caused simply by HIV infection.     

Klebsiella pneumoniae liver abscess associated with septic spinal epidural abscess

A 56-year-old Japanese man with hypertension presented with a 10 days history of high fever, right and left upper quadrant tenderness. An abdominal ultrasonography and computerized tomographic scan revealed a large collection in the right lobe of the liver that was consistent with an abscess. A drainage catheter was placed and purulent fluid was drained. Cultures of the fluid and blood were positive for a strain of ampicillin-resistant Klebsiella pneumoniae. Six days after admission, paraplegia and urinary retention were found. On the neurological examination, deep tendon reflexes of the lower extremities were absent bilaterally. Magnetic resonance imaging scan detected thoracic spinal epidural abscess and paraspinal abscess. He received the emergent decompressive laminectomy. Culture of surgical specimen grew ampicillin-resistant K. pneumoniae. The patient was treated with biapenem intravenously. Thereafter, clinical symptoms improved gradually and he was removed to the professional hospital to continue rehabilitation for gait disturbance on hospital day 147.     

Randomized Trial of Ivabradine in Patients With Hyperadrenergic Postural Orthostatic Tachycardia Syndrome

BackgroundPostural orthostatic tachycardia syndrome (POTS) is a complex, multifaceted disorder that impairs functional status and quality of life. Current pharmacological treatments are limited.ObjectivesThis study investigated the effect of ivabradine (selective blocker of the I_funny channel in the sinoatrial node) on heart rate, quality of life (QOL), and plasma norepinephrine (NE) levels in patients with hyperadrenergic POTS defined by plasma NE >600 pg/ml and abnormal tilt table test.MethodsIn total, 22 patients with hyperadrenergic POTS as the predominant subtype completed a randomized, double-blinded, placebo-controlled, crossover trial with ivabradine. Patients were randomized to start either ivabradine or placebo for 1 month, and then were crossed over to the other treatment for 1 month. Heart rate, QOL, and plasma NE levels were measured at baseline and at the end of each treatment month.ResultsThe average age was 33.9 ± 11.7 years, 95.5% were women (n = 21), and 86.4% were White (n = 23). There was a significant reduction in heart rate between placebo and ivabradine (p < 0.001). Patients reported significant improvements in QOL with RAND 36-Item Health Survey 1.0 for physical functioning (p = 0.008) and social functioning (p = 0.021). There was a strong trend in reduction of NE levels upon standing with ivabradine (p = 0.056). Patients did not experience any significant side-effects, such as bradycardia or hypotension, with ivabradine.ConclusionIvabradine is safe and effective in significantly improving heart rate and QOL in patients with hyperadrenergic POTS as the predominant subtype.     

CT evaluation of paraaortic lymph node metastasis in patients with biliary cancer

Background The extent of paraaortic lymph node (PAN) metastasis parellels that of distant metastases in patients with biliary carcinoma. Accurate preoperative assessment of PAN metastasis has a crucial impact on surgical indications. In this retrospective study, we evaluated whether computed tomography (CT) scans were useful for diagnosing PAN metastases and excluding patients with PAN metastases from an indication for surgery.Methods Between March 1999 and November 2003, 57 patients with biliary carcinoma underwent radical lymphadenectomy or surgical biopsy of PANs. Nine of these patients were diagnosed as having PAN metastasis microscopically. All patients had undergone abdominal CT scans before surgery. To diagnose PAN metastases, we used the following diagnostic criteria. (1) Size; when lymph nodes were greater than 12 mm, 10 mm, 8 mm, or 6 mm in longo or short-axis diameter, the nodes were considered metastatic. (2) Shape and size; when the axial ratio of a lymph node was greater than 0.5, 0.7, 1.0, and the maximum diameter of the long or short axis was greater than 12 mm, 10 mm, 8 mm, or 6 mm, the node was considered metastatic. (3) Internal structure; if the internal structure of a PAN was heterogeneous, the node was considered metastatic. A positive predictive value was calculated for each included criterion when patients numbered ten or more.Results Positive predictive values using the above criteria ranged from 13% to 36%. Only one patient had PANs with heterogeneous internal structures.Conclusions We were unable to determine surgical indications based on the morphological criteria revealed by a CT scan.     

Favourable effects of heart rate reduction with intravenous administration of ivabradine in patients with advanced heart failure

BACKGROUND: Heart rate (HR) reduction may be useful in treatment of patients with heart failure (HF). There are no data on the haemodynamic effects of ivabradine (a selective I(f) current inhibitor) in advanced HF patients. AIMS: To assess the haemodynamic effects of ivabradine in patients with advanced HF and markedly depressed left ventricular (LV) function. METHODS AND RESULTS: Ten NYHA class III patients (50+/-12 years, LV ejection fraction 21+/-7%) underwent 24-h haemodynamic monitoring. Ivabradine 0.1 mg/kg was infused over 90', followed by 0.05-0.075 mg/kg in the subsequent 90'. Baseline HR was 93+/-8 bpm, cardiac index (CI) 2.2+/-0.6 l/min*m2; LV stroke volume 44+/-11 ml and systolic work 39+/-13 g. Ivabradine significantly reduced HR, by a maximum of 27% (to 68+/-9 bpm) at 4 h, without decreasing CI. Ivabradine increased stroke volume and LV systolic work by a maximum of 51% (to 66+/-17 ml) and 53% (to 58+/-20 g) at 4 h. No serious adverse events occurred. CONCLUSION: In patients with advanced HF and markedly depressed LV function, the acute administration of ivabradine is well tolerated, effectively reduces HR, markedly increases stroke volume and preserves cardiac output. Ivabradine appears a promising approach for the treatment of patients with moderate and advanced heart failure.     

The impact of antiviral therapy for HCV on kidney disease: a systematic review and meta-analysis

BackgroundControversy persists about the role of hepatitis C as a risk factor for developing kidney disease in the general population. Some authors have evaluated the effect of antiviral therapy for HCV on the risk of kidney disease.Study Aims and DesignA systematic review of the published medical literature was performed to assess whether antiviral therapy for HCV has an independent impact on kidney survival in the adult general population. A random effects model was used to generate an overall estimate of the risk of kidney disease after anti-HCV therapy across the published studies. Meta-regression and stratified analysis were also carried out.ResultsFifteen studies were eligible (n = 356, 285 patients) and separate meta-analyses were conducted according to the outcome. Pooling studies based on viral responses (n = 7; 34,763 individual patients) demonstrated a relationship between sustained viral response and lower frequency of kidney disease; the overall estimate for adjusted risk of kidney disease was 2.50 (95% CI, 1.41; 4.41) (p = 0.0016) and between-study heterogeneity was found (p-value by Q test = 0.004). Aggregation of studies comparing treated vs untreated cohorts (n = 8, n = 333,312 patients) revealed an association between anti-HCV therapy and lower risk of kidney disease. The overall estimate for adjusted risk of kidney disease across the eight studies was 0.39 (95% CI, 0.25; 0.612) (p = 0.0001). Meta-regression showed that the effectiveness of antiviral therapy in reducing the frequency of kidney disease diminishes as cirrhosis (p = 0.02) and HBV infection (p = 0.0001) increase among HCV-infected individuals.ConclusionsAntiviral therapy for HCV lowers the risk of kidney disease among HCV-infected individuals. Studies to understand the mechanisms underlying this association are ongoing.     

窦房结动脉的影像学评价及其与病态窦房结综合征的关系

目的　了解冠状动脉(冠脉)造影下窦房结动脉(sinusnodeartery ,SNA)的形态与起源,明确窦房结动脉相关病变与病态窦房结综合征(sicksinussyndrome ,SSS)的关系。方法　连续观察51 1人的冠脉造影资料,比较有无窦房结动脉相关病变的两组各2 9人的心率和SSS发病情况,以及有无SSS和(或)窦性心动过缓的两组各34人的窦房结动脉相关病变情况。数据采用SPSS1 0 0软件包进行统计分析处理。结果　51 1人中观察到50 6人的窦房结动脉共56 1支,其中53人( 1 0 . 1 7% )有双SNA ,1人有三支SNA。右、左和后窦房结动脉分别为2 75支( 4 9. 0 2 % )、2 0 5支( 36 . 54% )和81支( 1 4 . 4 4% )。有无窦房结动脉相关病变的两组平均心率( 71 . 59±7 .4 5次 分vs 73 .76±6 . 6 7次 分)及患SSS患者数量( 1人vs 0人)的差异无统计学意义(P >0 0 5)。SSS组和非SSS组窦房结动脉受累情况(SSS组34人有2人狭窄4 0 %和90 % ,非SSS组34人有2人狭窄4 5%和2 0 % )的差异无统计学意义(P >0 .0 5)。结论　冠脉造影下基本可以清晰地看到窦房结动脉。窦房结动脉相关病变与SSS关系不大。