A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether patients with haemophilia undergoing cardiac surgery have good surgical outcomes. Haemophilia A and haemophilia B are sex-linked recessive inherited diseases affecting males only, with females acting as carriers. The conditions result in various degrees of factor VIII or f...

Advances in therapy have improved life expectancy and quality of life of patients with haemophilia A. Due to the chronic and complex management of this disease, particularly, the development of inhibitors, little is known about their health resource utilization in the real-life setting over time. The aim was to assess the distribution and trend of healthcare resource utilization among US haemop...

Quality of life (QoL) is a multidimensional construct pertaining to an individual's physical, emotional, mental, social and behavioural components of well-being and functioning. QoL can be assessed using both generic and disease-specific instruments. QoL assessment in haemophilia is a relatively new area of study; the first data were published in 1990 using generic QoL questionnaires. Only rece...

Current screening methods for factor VIII gene (F8) mutations can reveal the causative alteration in the vast majority of haemophilia A patients. Yet, standard diagnostic methods fail in about 2% of cases. This study aimed at analysing the entire intronic sequences of the F8 gene in 15 haemophilia A patients by next generation sequencing. All patients had a mild to moderate phenotype and no mut...

Numerous studies are being carried out on polymorphisms within the genes coding for factors VIII and IX due to their clinical relevance in context of hereditary disorders. The quests polymorphism can be used screen haemophilia through an affected family. In Cameroon, very few gene have been conducted. Thus, this study was aimed at detecting HindIII SNP F8 MseI F9 by Polymerase Chain Reaction-Re...

Von Willebrand disease and haemophilia A are the two most common inherited bleeding disorders. The worldwide incidence of VWD is estimated between 1% and 4% of the population without apparent racial or ethnic predilection. In the United States, the incidence of haemophilia A is estimated to be 25 per 100 000 male births. Despite the relatively high frequency of those two bleeding disorders in t...

Owing to the improvements in the management of haemophilia, children with severe haemophilia in the United Kingdom have very different experiences of their condition compared with many adults' early experiences of haemophilia. However, haemophilia can still have physical and social effects which can impact on the quality of life, not only for a child who has the condition, but also for their pa...

BACKGROUND AND OBJECTIVES Health Related Quality of Life (HRQoL) is an important health outcome measure in haemophilia. The aim of this study was to assess the psychometric properties of the Greek version of Haem-A-QoL, a disease-specific questionnaire for haemophiliacs. METHODS Haem-A-QoL and SF-36 were administered to 118 adult haemophilia patients. Hypothesized scale structure, internal co...

Introduction One of the most often stated tenets haemophilia care is that prophylaxis converts a person from severe to moderate phenotype. In this review, we argue not an accurate assumption and people on predominantly have factor VIII/IX levels in mild range. Moderate People with haemophilia, who are treating on-demand regimens, experience joint bleeds develop significant arthropathy. This esp...

Type and quantity of replacement treatment, together with haematological and immunological parameters were determined in 37 boys with severe haemophilia A and 41 children with other bleeding disorders. The quantity of factor VIII concentrate given to boys with severe haemophilia A (mean U/year) showed a significant inverse correlation with total white cell counts, lymphocyte counts, platelet co...