Efficacy of FEIBA for acute bleeding and surgical haemostasis in haemophilia A patients with inhibitors: a multicentre registry in Turkey

Long used in established industrialized nations to treat patients with haemophilia and inhibitors, factor eight inhibitor bypassing activity (FEIBA) has, in recent years, been introduced into more geographically diverse settings. Data are needed on how successfully FEIBA therapy has been implemented in new regions. To determine the efficacy and safety of FEIBA for the treatment of acute bleeding and surgical haemostasis in a newly industrialized country. A multicentre registry of haemophilia A patients with inhibitors receiving FEIBA treatment was established in Turkey. With a standardized case report form, data were collected retrospectively on: patient demographics; characteristics of acute bleeding episodes and surgical interventions; FEIBA regimen; and treatment outcomes. Thirty-seven patients received a total of 112 FEIBA treatment courses, 90 for acute bleeding and 22 for surgical haemostasis. The median FEIBA dose per infusion for acute bleeding was 50 IU kg(-1), and for surgery was 100 IU kg(-1). For both acute joint and muscle/soft tissue bleeding and in surgery, haemostasis was attained in a median of two FEIBA infusions. FEIBA was judged effective in 92% of treatment courses for acute bleeding, with a 95% confidence interval (CI) of 85-97%. Rates of haemostatic efficacy did not differ significantly between anatomical sites of acute bleeding. The haemostatic efficacy rate of FEIBA in surgery was 86% (CI, 65-97%). No thromboembolic complications or other adverse events occurred during any treatment course. FEIBA has been successfully integrated into clinical practice in Turkey, with rates of haemostatic efficacy comparable to those reported in countries with a longer history of FEIBA usage.     

Effects of thrice weekly nocturnal hemodialysis on arterial stiffness

ObjectiveIn this study, we compared the changes in arterial stiffness in chronic hemodialysis patients treated with 8-h vs. 4-h thrice weekly in-center hemodialysis.MethodsSixty prevalent chronic hemodialysis patients assigned to 8-h nocturnal in-center thrice weekly HD (NHD) and 60 control cases assigned to 4-h thrice weekly conventional HD (CHD) were followed for one year. Radial–carotid pulse wave velocity, augmentation index and echocardiography were performed at baseline and 12^th month.ResultsMean age of the patients was 49 ± 11 years, 30.8% were female, 27.5% had diabetes mellitus and mean dialysis vintage was 57 ± 47 months. Baseline demographical, clinical and laboratory parameters were similar between groups. During a mean follow-up of 15.0 ± 0.1 months, blood pressure remained similar in both groups while the number of mean daily anti-hypertensive substances decreased in the NHD group. In the NHD group, time-averaged serum phosphorus and calcium–phosphorus product were lower than the CHD group. Pulse wave velocity and augmentation index decreased in the NHD group (from 11.02 ± 2.51 m/s to 9.61 ± 2.39 m/s and from 28.8 ± 10.3% to 26.2 ± 12.1%; p = 0.008 and p = 0.04, respectively). While augmentation index increased in the CHD group (28.0 ± 9.4 to 31.0 ± 10.7%, p = 0.02), pulse wave velocity did not change. Subendocardial viability ratio and ejection duration improved in the NHD group (from 135 ± 28 to 143 ± 25%, p = 0.01 and from 294 ± 34 ms to 281 ± 34 ms, p = 0.003, respectively), accompanied by regression of left ventricular mass index. In multiple stepwise linear regression analyses, NHD was associated with improvements in augmentation index, ejection duration and subendocardial viability ratio.ConclusionsThese data indicate that arterial stiffness is ameliorated by implementation of longer hemodialysis sessions.     

Aortitis in a patient with psoriatic arthritis

Aortitis, inflammation of the aortic tissue, is most commonly caused by vasculitic rheumatic conditions, and less frequently infectious organisms. Involvement of the aorta is well defined in HLA-B27-associated spondyloarthropathies such as long-standing ankylosing spondylitis and Reiter's syndrome. However, unlike other spondyloarthropathies, aortic involvement or true aortitis is not a feature of psoriatic arthritis and has been reported in only a few cases. Herein, we report the case of a 22?year-old woman with psoriatic arthritis who developed descending aortitis while using tumor necrosis factor inhibitors.     

Prevalence of pleural effusions post orthotopic heart transplantation

BACKGROUND AND OBJECTIVE: The prevalence and natural history of pleural effusions occurring after orthotopic heart transplantations (OHT) are essentially unknown. The objective of this study was to determine the prevalence, laterality, size and prognosis of pleural effusions occurring after OHT. METHODS: Eighty-three patients who underwent OHT between August 1997 and January 2003 were screened retrospectively. CXR and chest CT scans of all patients were reviewed. Chart review was performed for patients with large effusions (occupying 25% or more of a hemithorax) with specific attention to thoracentesis results. RESULTS: Seventy-two patients were included in the study. Sixty-one (85%) developed an effusion at some time during the first 365 postoperative days. The majority of post-OHT effusions were bilateral and small, occupying less than 25% of the hemithorax. Pleural effusions occupying 25% or more of a hemithorax occurred in 17% (12/72) of the patients within 12 months of OHT. Overall, the majority of effusions resolved within the first year after transplantation. CONCLUSIONS: The prevalence of pleural effusions within 12 months following OHT is high (85%). Most effusions are small, bilateral and resolve within 1 year.     

Value of prolonged QRS duration as a predictor of low cardiac output syndrome in patients with impaired left ventricular systolic function who undergo isolated coronary artery bypass grafting

This study determined whether prolonged QRS duration (QRSd; > or =120 ms) is an independent predictor of low cardiac output syndrome (LCOS) in patients with low left ventricular (LV) ejection fraction (EF) who underwent isolated coronary artery bypass grafting (CABG). Abnormal LV systolic function places patients at greater risk for developing LCOS after isolated CABG. In patients with this form of ventricular function impairment, prolonged QRSd is associated with adverse hemodynamic effects. Clinical, operative, and outcome data from 190 consecutive patients with LVEF <50% who underwent isolated CABG (mean 62 +/- 9 years of age) were retrospectively analyzed. For all patients, preoperative QRSd was determined. LCOS was the primary outcome investigated. Fifty-seven patients (30%) developed LCOS. Compared with the subgroup without LCOS, the subgroup with this syndrome had significantly larger proportions of patients with LVEF <30% and prolonged QRSd. In addition, the group that developed LCOS had a longer mean QRSd (117 +/- 25 vs 102 +/- 17 ms, respectively, p = 0.00003) and a significantly higher frequency of adverse postoperative outcomes. Hospital stay was significantly longer in the subgroup with LCOS than in the subgroup without. Multivariate logistic regression analysis identified prolonged QRSd as the most significant predictor of LCOS. LVEF <30%, diuretic therapy, and preoperative risk score (European System for Cardiac Operative Risk Evaluation) were also identified as independent predictors of LCOS. In conclusion, in patients with impaired LV systolic function, prolonged QRSd is a highly significant predictor of LCOS development after isolated CABG.     

Appropriate ICD therapy in patients with idiopathic dilated cardiomyopathy: long term follow-up

The implantable cardioverter defibrillator (ICD) has proved effective in preventing sudden death and decreasing mortality in randomised secondary prevention trials. Some nonrandomized studies have reported different incidences and predictors of appropriate ICD therapy in patients with idiopathic dilated cardiomyopathy (DCM). The antiarrhythmic and other medical therapies were different between the published studies and it was reported that not using beta-blockers was a predictor of appropriate ICD therapy. In the present study, we report on our long-term experience with ICD therapy in patients with DCM, the majority of whom were treated with beta-blockers and amiodarone. The study population consisted of 25 patients with DCM who underwent initial transvenous ICD implantation between December 1995 and May 2005. Indications for ICD implantation were monomorphic sustained ventricular tachycardia (VT) in 16 patients (64%), cardiac arrest in 8 patients (32%), and syncope plus inducible VT in one patient. Twenty-four patients underwent an electrophysiologic study (EPS). In 18 patients, the ICDs were programmed to only shocks and in 7 patients an additional antitachycardia pacing program was performed. One patient was lost to follow-up and 24 patients were followed-up primarily in our ICD pacemaker outpatient clinic. Appropriate ICD therapy was defined as antitachycardia pacing therapy or shock for tachyarrhythmia determined by evaluation of the clinical information and by device diagnostics to be either ventricular fibrillation or ventricular tachycardia. The mean follow-up was 39.29 +/- 30.59 months after ICD implantation. At follow-up, 17 patients were using a beta-blocker and 16 patients amiodarone. Appropriate ICD therapy was observed in 14 patients (58%). The detected arrhythmias were VT in 12 patients, ventricular fibrillation (VF) in one, and VT and VF in one patient. The time to first ICD therapy was 15.93 +/- 18.45 (range, 1-74) months. Using the Kaplan-Meier method, the percent survival free of appropriate ICD therapy was 82%, 72%, 66%, and 55% at 1, 2, 3, and 4 years follow-up, respectively. The clinical, echocardiographic, and electrophysiologic characteristics did not differ between those who did and did not receive appropriate ICD therapy. However, the mean QRS duration was significantly longer in patients who received appropriate ICD therapies. Cox regression analysis did not reveal any factors that predicted appropriate ICD therapy. Five patients (21%) died during follow-up. Four deaths were classified as cardiac and one as noncardiac. The cumulative survival from total death was 94%, 82%, 82%, and 69%, and the cumulative survival from cardiac death was 94%, 82%, 82%, and 76% during 1, 2, 3, and 4 years of follow-up, respectively. In summary, in this selected patient population with DCM, the majority of patients were unresponsive to beta-blocker and antiarrhythmic therapy. Most of these patients received appropriate ICD therapy during follow-up. Cox regression analysis did not identify any factors that predicted appropriate ICD therapy. Additional trials with larger patient populations are needed to detect the predictors of appropriate ICD therapy in patients with DCM.     

High plasma levels of factor VIII: an important risk factor for isolated pulmonary embolism

OBJECTIVE: The aim of the study was to investigate whether factor V Leiden and prothrombin G20210A mutations, elevated levels of factor VIII and factor IX are associated with pulmonary embolism (PE). METHODS: Sixty-four patients with objectively documented PE and 64 control subjects were included in this study. The authors divided the 64 subjects with PE into those with PE and deep vein thrombosis (combined form of venous thromboembolism, n = 26) and those with PE without deep vein thrombosis (isolated PE n = 38). RESULTS: There was no significant difference between the PE groups and the control subjects with regard to the presence of factor V Leiden and prothrombin mutations and elevated levels of factor IX. Using the 90th percentile measured in control subjects (P(90) = 168 U/dL) as a cut-off point for factor VIII levels, the authors found an 11-fold increased risk for both isolated PE patients and patients with a combined form of venous thromboembolism who have factor VIII levels >168 U/dL compared with individuals having factor VIII levels below this cut-off point. The risk was not affected by adjustments for other possible risk factors. CONCLUSIONS: Elevated plasma factor VIII levels were found to be a significant, independent risk factor for PE.     

Rubella seroprevalence in women in the reproductive period, Mersin, Turkey

The aim of this study was to determine the rubella seroprevalence in women of reproductive age. This cross-sectional study was conducted on women aged 15-49 years. Serologic studies were performed by using the ELISA method. The number of women enrolled in the study was 607, the mean age was 30.7+/-9.3 years and 13.2% of the participants were living in the rural area. Rubella seroprevalence was 55.0%. This ratio was 77.5% in women living in the rural area (p<0.001). There were no statistically significant correlations between immunity to rubella and other socio-demographic characteristics. In conclusion, women in this age group are advised to have a single dose of rubella vaccination in order to avoid CRS before pregnancy.