“
“It is now 30 years since the factor VIII (FVIII) protein was first purified, leading to the subsequent cloning of the gene. Detection of causative mutations in the FVIII gene (F8)

was initially slow and laborious, but recent years have seen great advances in the technology for the detection of variations in F8. Comprehensive mutation detection is often now performed by polymerase chain reaction (PCR) and direct sequencing of all exons: disease-associated variants include single-base replacements, deletions, insertions and gross gene rearrangements. Coupled with the recently published crystal structure of FVIII, there is potential for a much greater understanding of the relationship between FVIII structure and the function of the cofactor in coagulation. “
“The levonorgestrel-releasing Palbociclib selleck screening library intrauterine system (LNG-IUS) is indicated for the management of menorrhagia and for contraception. The LNG-IUS is effective at reducing menstrual bleeding and improving haemoglobin among women with bleeding disorders. Expulsion rates for the LNG-IUS among normal women are reported to be approximately 5–10%. The aim of this study was to examine the malposition and expulsion rates of the LNG-IUS among women

with inherited bleeding disorders. We conducted a retrospective study of women with an inherited bleeding disorder in Kingston, Canada treated with an LNG-IUS between May 2005 and June 2012. The primary outcome was a combined endpoint of expulsion and/or malposition. Predetermined secondary outcomes were patient satisfaction and changes in haemoglobin and ferritin levels. The median age of the women at the time of LNG-IUS insertion was 31 years (range 18–43, mean 32.1 years). The most common diagnosis was type 1 VWD (12/20, 60%). There were three LNG-IUS expulsions and two episodes of device malposition resulting in removal selleck inhibitor [5/20 (25.0%), 95% CI 11.2–46.9%]. An additional five women had their device removed prematurely.

The overall proportion of devices resulting in discontinuation in this population was 10/20 (50.0%, 95% CI 29.9–70.1%). In this retrospective study, a significant proportion of women with an inherited bleeding disorder had an LGN-IUS removed due to poor patient satisfaction, malposition, or expulsion. Further studies into the causes of higher complication rates and interventions such as premedication or prolonged treatment with antifibrinolytic agents targeted at improving outcomes in this population are required. “
“Summary.  During the last two decades major advances have been achieved in the management of haemophilia. Modern approaches aimed at preventing the recurrent bleedings and their sequelae have been widely adopted.