The Haemophilia Utilization Group Study (HUGS) was created 10 years ago to examine the annual utilization and cost of haemophilia-related healthcare services. Retrospective chart reviews for 336 patients with haemophilia A receiving treatment in one of five comprehensive haemophilia treatment centres (HTCs) during 1995 were completed through interview of the provider. This method provided adequ...

Introduction: Hemophilia A is an inherited disease caused by a deficiency of factor VIII, which results from a genetic inheritance located on the X chromosome. During treatment of patients with this disorder, factor VIII inhibitors may be present, which are primarily antibodies type immunoglobulin G (IgG), and interfere with the activation of factor VIII. Objectives: The present study aims to i...

OBJECTIVE To report a case of severe abdominal bruising successfully diagnosed using systematic hemostatic investigations. CLINICAL PRESENTATION AND INTERVENTION A 60-year-old woman developed a large spontaneous nontraumatic, painless bruise over the right lower abdominal wall. Computed tomographic scan of the abdomen showed a subcutaneous hematoma beneath the site of the obvious bruise diagn...

A Thai woman, with no family history of bleeding disorders, presented with excessive bleeding after minor trauma and tooth extraction. The screening coagulogram revealed prolonged activated partial thromboplastin time and prothrombin time. The specific-factor assay confirmed the diagnosis of combined factor V and factor VIII deficiency (F5F8D). Her plasma levels of factor V and factor VIII were...

recB and/or recC deficiency in Escherichia coli K-12 is indirectly suppressed by the presence of sbcA(-) mutations. sbcA(-) strains contain an increased level of an ATP-independent nuclease. Genetic and enzymatic tests indicate that this activity is not exonuclease III, exonuclease V (recB-recC nuclease), DNA polymerase I, or lambda exonuclease. This new enzyme (exonuclease VIII) has been purif...

Fibrin deposition and platelet thrombus dimensions on subendothelium were studied in four groups of patients with coagulation factor deficiencies. Five patients with factor VIII deficiency (APTT 120 +/- 8 sec) and three patients with factor IX deficiency (APTT 125 +/- 11 sec) were severe bleeders, whereas four patients with factor XII deficiency and seven with factor XI deficiency were either a...

Attendances at the Regional Haemophilia Reference Centre in the Edinburgh Royal Infirmary have been analysed over a period of five years from 1969 to 1973. Of 77 patients registered in 1969, 64 had haemophilia A (factor VIII deficiency) and 13 had haemophilia B (factor IX deficiency). In 1973 the numbers were 68 and 14 respectively. An increased attendance at the Centre from 123 in 1969 to 624 ...