Haemophilia patients' unmet needs and their expectations of the new extended half-life factor concentrates.

Introduction: National Member Organisations (NMO) of persons with haemophilia (PWH) from the DACH Region (D = Germany, A = Austria, CH = Switzerland) were interested to better understand PWH's expectations and concerns of extended half-life (EHL) factor concentrates (FC) before availability in these countries.

Methods: Based on an expert meeting and focus groups conducted across Germany a survey for haemophilia patients and their parents was developed and was sent out to 2,644 PWH.

Results: One thousand and seven questionnaires were sent back (38.

Adherence to prophylactic treatment in patients with haemophilia in Germany.

Introduction: Adherence to prophylactic treatment in haemophilia is important for patient outcome.

Aim: This study analysed the influence of potential impact factors on adherence assessed through the application of the German translation of the VERITAS-Pro questionnaire.

Methods: All members of the German haemophilia patient organisation (DHG) who suffer from severe or moderate haemophilia and are on continuous prophylactic treatment were asked to complete the VERITAS-Pro questionnaire.

Pain therapy in haemophilia in Germany. Patient survey (BESTH study).

Unlabelled: One of many challenges in the treatment of persons with haemophilia is the selection and application of appropriate pain-relieving therapies. The current situation of pain management for persons with haemophilia in Germany was evaluated using a survey with the intention of identifying potential areas for improvement. Results of 685 respondents showed that 86% experienced episodes of pain and that pain was already present in 66% of children and adolescents.

Haemophilia Experiences, Results and Opportunities (HERO) study: treatment-related characteristics of the population.

The HERO (Haemophilia Experiences, Results and Opportunities) quantitative surveys collected information on characteristics and perceptions of adult persons with haemophilia (PWH) and parents of children with haemophilia. The aim of this article is to describe the perceptions of PWH and parents on psychosocial aspects related to treatment. Two online surveys (one for PWH, one for parents) were conducted in 10 countries.

[Life with haemophilia. The patient's perspective of ageing].

Patients with haemophilia are growing older and may be faced with age-dependent co-morbidities. To evaluate the impact of growing age in patients with haemophilia, a questionaire was sent to members of German patients' organisations DHG and IGH with focus on medical care and psychosocial questions. This questionnaire was answered by 466 individuals (aged 50 years, 21-85 years), predominantly suffering from severe haemophilia.

Patient-reported outcomes of 182 adults with severe haemophilia in Germany comparing prophylactic vs. on-demand replacement therapy.

Clotting factor replacement therapy has a major impact on the quality of life in patients with haemophilia. To analyse and compare the outcomes of on-demand and prophylactic treatment regimens in child- and adulthood, a self-evaluation questionnaire was sent to 182 patients over 30 years of age with severe haemophilia A or B. Analysis of the questionnaire results revealed that most study participants had been treated on-demand in childhood, but that the majority of these patients subsequently switched to prophylaxis.

Gender-related differences in left ventricular chamber function.

Objectives: While women have lower rates of atherosclerotic disease than men, they are more likely to suffer cardiac failure following infarction or cardiac surgery, despite typically having a greater left ventricular (LV) ejection fraction. We hypothesised that gender differences in systolic chamber function and ventriculo-vascular coupling may contribute to these clinical findings.

Methods: LV chamber function was determined in a cohort of 30 patients (16 women) aged 48-75 years with normal LV function using pressure-volume loops obtained by simultaneous conductance catheter volumetry and micromanometer pressure.

Acute effects of 17beta-estradiol on ventricular and vascular hemodynamics in postmenopausal women.

Because premenopausal women have lower cardiovascular morbidity than postmenopausal women, it has been proposed that estrogen may have a protective role. Estrogen is involved in smooth muscle relaxation both through its specific receptor as well as through calcium channel blockade. This study examined the acute effect of estradiol on invasive cardiovascular hemodynamics in 18 postmenopausal women (age 62.

Left ventricular chamber function during inhaled nitric oxide in patients with dilated cardiomyopathy.

Inhaled nitric oxide is a potent and selective pulmonary vasodilator. However, when used in patients with congestive cardiac failure, the decrease in pulmonary vascular resistance is associated with an increase in pulmonary capillary wedge pressure (PCWP). This study examined load-independent indexes of left ventricular chamber function during inhaled nitric oxide in 10 patients with dilated cardiomyopathy (mean ejection fraction, 30.

Response of the intact canine left ventricle to increased afterload and increased coronary perfusion pressure in the presence of coronary flow autoregulation.

Background: Increased left ventricular (LV) contractile force or oxygen consumption has been documented with increased coronary arterial pressure (CAP) and flow (Gregg phenomenon). We investigated whether the increase in contractile force with increased LV afterload might be mediated by the concomitant increase in CAP when coronary autoregulation is intact.

Methods And Results: The LV of 6 autonomically blocked open-chest dogs was perfused through the left main coronary artery by a cannula with a side gate to the aortic root.

Effect of inhaled nitric oxide on normal human left ventricular function.

Objectives: This study determined the effects of inhaled nitric oxide (NO) on load-independent indexes of normal human left ventricular (LV) function.

Background: Inhaled NO is a potent and selective pulmonary vasodilator. However, when it is used in patients with congestive heart failure, the decrease in pulmonary vascular resistance (PVR) is often associated with an increase in pulmonary capillary wedge pressure.