Recombinant factor VIII has biologic activity comparable to that of plasma factor VIII and is safe and efficacious for the treatment of hemophilia A.

No abstract

SUMMARY Twenty five of 106 preterm infants of 34 weeks' gestation or less developed intraventricular haemorrhage within the first 48 hours of life. A comparison of infants with and without intraventricular haemorrhage showed no significant differences in their haemostatic parameters at birth. At age 48 hours the group with intraventricular haemorrhage showed a prolonged activated partial thromboplastin time and reduced factor II, VII, and X activity. There was a significant correlation between the severity of intraventricular haemorrhage and the degree of haemostasis abnormality both in cord blood and in blood obtained at age 48 hours. Those infants sustaining grade IV intraventricular haemorrhage had a significantly prolonged activated partial thromboplastin time, reduced factor II, VII, and X activity; and a decreased fibrinogen concentration at birth. At age 48 hours these defects were accompanied by reduced platelet counts and an increased megathrombocyte index. Although intraventricular haemorrhage is multifactorial, we postulate that correction of haemostasis abnormalities at birth may prevent progression to more severe grades of haemorrhage. The birthweight of the study population was mean (SD) 1-67 (0.58) kg (range 0-6 to 3-49 kg) and the gestational age was mean (SD), 31-2 (3-12) weeks (range 24 to 34 weeks). Infants were excluded if there was a maternal history of ingestion of drugs such as aspirin and warfarin that might have altered the coagulation studies. Infants weighing less than 1500 g and those weighing 1500 g or more received vitamin K1 intramuscularly in doses of 0 5 mg and 1 mg respectively shortly after birth. None of the infants received indomethacin during the study period. Blood products including fresh frozen plasma, albumin, and whole blood were given according to the infants' clinical status. When arterial catheters were used the infusion fluid contained 1 U/ml heparin and infusion rates did not exceed 1 ml/hour. Detection of intracranial haemorrhages. Intraventricular and periventricular haemorrhages were detected using a Diasonics 20S ultrasound detector (Diasonics, Canada) fitted with a 7-5 mHz transducer. Serial craniosonograms were performed at 8 hour intervals in the first 24 hours of life, at 12 hour intervals in the second 24 hours, and weekly thereafter. Haemorrhages were graded according to 444 on 11 May 2018 by guest. Protected by copyright.

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The optimal treatment of patients with von Willebrand's disease (vWD) remains to be defined. Moreover, it has not been firmly established which, if any, commonly measured parameters of von Willebrand factor (vWF) protein in the plasma are useful in guiding treatment. To better understand what guidelines physicians follow in the management of vWD, we surveyed 194 North American physicians who are members of the Hemophilia Research Society. Ninety-nine per cent of responding physicians depend on factor VIII (FVIII):C, vWF:RCo activity and vWF:AG to diagnose vWD, while only 49% use the bleeding time. The minimal goals of treatment for patients undergoing major surgery/trauma or central nervous system haemorrhage were FVIII:C and vWF:RCo activity greater than 80% while levels of more than 50% for minor surgery and dental extractions were considered adequate. Treatment of vWD was based on the type of vWD with type 1 patients being treated most often with desmopressin acetate (DDAVP) alone, types 2A and 2B patients with a combination of DDAVP and a vWF-containing FVIII product, type 3 patients with vWF-containing concentrate. Viral infections, including human immunodeficiency virus, hepatitis A, B and C viruses, and parvovirus have been seen in vWD and the efficacy of viral attenuation processes is a major criterion for the selection of treatment by physicians. Based on this survey, prospective studies need to be designed to address the clinical efficacy, safety and predictive value of laboratory monitoring of patients with vWD.