“
“Afibrinogenaemia is an autosomal recessive disease with an estimated prevalence of approximately one in a million. The most common symptoms of afibrinogenaemia are umbilical cord bleeding, bleeding into skin, mouth, muscles, gastrointestinal and genitourinary tracts and the central nervous system. Other recognized complications include; haemarthroses, spontaneous splenic rupture, epistaxis, menorrhagia, recurrent abortion and venous and arterial thromboembolism. Bone cysts have also been described

as a rare complication of afibrinogenaemia. The aim of this study was to conduct a systematic literature review, summarize the reported cases and to report two new Metabolism inhibitor cases. Three electronic databases were searched for relevant publications: PubMed, Medline and EMBASE. The following search criteria were used: ‘(bone cysts OR intraosseous haematoma OR intraosseous haemorrhage) AND (afibrinogenaemia OR fibrinogen deficiency)’. The reference lists of the selected papers were searched for more relevant literature. In total, eight patients had bone cysts as complication of afibrinogenaemia and six of http://www.selleckchem.com/products/PD-0332991.html them

suffered from pain in their extremities. Bone cysts were primarily located in the vicinity of the cortex or trabeculae in the diaphysis of the long bones, especially in the femora, tibiae and humeri. Some were regressive, probably due to reactive

bone remodelling. A number of cysts were filled with serosanguinous fluid. It might be useful to check for bone cysts when patients with congenital afibrinogenaemia complain of ‘rheumatic’ pains in their extremities. Whole body magnetic resonance imaging is the diagnostic imaging technique of choice. Recurrent episodes of pain, but not radiological deterioration, appear to benefit from prophylactic check details therapy with fibrinogen concentrate. “
“Summary.  The efficacy and safety of Optivate® was assessed in 23 surgical operations, orthopaedic (12) including 5 revision arthroplasties, ophthalmic (1), ENT (1), dental (6), liver biopsy (2), and removal of portacath (1) on 15 teenagers and adults with severe haemophilia A. The preoperative dose was calculated to raise the FVIII concentration to 100 IU dL−1. Subsequent doses were targeted to maintain at least 50 IU dL−1. There were 11 major and 12 minor operations categorized as receiving intensive replacement therapy for ≥5 days or <5 days respectively. The median preoperative dose was 50.4 (range 18.2–88.2) IU kg−1. The median incremental recovery based on this first dose in 10 procedures (5 patients) was 2.9 (range 2.4–3.4 IU dL−1) per IU kg−1. The daily doses decreased during the first 4 days of the study. The patients in this study received 173 infusions in total.