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“Summary.  The first written record of haemophilia in America is reputed to be the 1791 newspaper obituary of Isaac Zoll. The earlier, original publication of this obituary is identified. “
“The colour illustrations for Chapters 3, 13, 15, 17, 29 and 55 are included, as follows: Plate 3.3 Plate 13.2 Plate 15.1 Plate 17.3 Plate 29.1 Plates 55.1, 55.2 “
“Homozygous severe factor V (FV) deficiency has a prevalence

of around one per million. Even in patients with FV levels of <0.01 IU mL−1 there appears to be a variation in bleeding phenotype in that there is a subgroup of affected individuals who present in later childhood and have a relatively mild bleeding phenotype, but there are children who present as neonates with intracerebral bleeding events and who have a much Selleck Anti-infection Compound Library more severe bleeding phenotype. The only available current FV replacement buy Forskolin is in the form of fresh frozen plasma (FFP) or solvent detergent FFP. We present here our experience with surgical haemostatic cover for 13 surgeries in three children with severe FV deficiency. “
“Summary.  Acquired factor XIII (FXIII) deficiency due to an autoantibody against FXIII is a very rare, yet potentially life-threatening bleeding disorder. As the standard coagulation tests (prothrombin time and activated partial thromboplastin time) are normal, the specialized tests are required to make an accurate diagnosis. Here, we report a case of acquired FXIII deficiency with severe bleeding symptoms. A 75-year-old man

was referred to our hospital because of severe bleeding tendency after a tooth extraction. Laboratory findings showed that routine coagulation studies were normal, but factor XIII (FXIII) activity was low (3%). The presence of FXIII inhibitor was detected with dot blotting studies. Although the bleeding tendency was very severe, it was successfully controlled by infusion of FXIII concentrates combined with immunosuppressive treatment medchemexpress (oral prednisolone). Fibrin cross-linking study showed the significant delay of the γ-chain dimer and α-chain polymer formation. Western blotting revealed the marked decrease in FXIII-A level. The mixing study of FXIII activity measured using amine-incorporation

assay showed the incomplete inhibition pattern. There seems to be little agreement as to the treatment strategy of acquired FXIII deficiency. In this patient, the use of FXIII concentrates was very useful in the initial treatment of bleeding symptom. The use of steroids was also effective in increasing FXIII activity without any serious complications. “
“Haemophilia A is an X-linked disorder characterized by congenital deficiency of factor VIII (FVIII). The FVIII protein is secreted as a mixture of a single chain and heterodimer consisting of one heavy and one light chain. The heavy chain is comprised of the A1, A2 and B domains, whereas the light chain contains the activation peptide, A3, C1 and C2 domains [1]. Severe haemophilia A is defined by a FVIII activity level <1% of normal.