       Document 0258
 DOCN  M9630258
 TI    Treatment of inherited coagulation disorders.
 DT    9603
 AU    Cohen AJ; Kessler CM; Blood Research Institute, Division of
       Hematology/Oncology, Saint; Michael's Medical Center, Newark, New Jersey
       07102, USA.
 SO    Am J Med. 1995 Dec;99(6):675-82. Unique Identifier : AIDSLINE
       MED/96106305
 AB    Inherited coagulation protein deficiencies associated with bleeding
       diatheses may present with spontaneous bleeding early in life, or may
       not be recognized until the development of hemorrhage after trauma or
       surgery. Diagnostic evaluation with coagulation screening tests,
       followed by confirmation with coagulation factor assays, is essential
       for appropriate management. For moderate-to-severe hemophilia, treatment
       includes coagulation factor replacement with purified, plasma-derived
       coagulation factor, or in the case of hemophilia A, factor VIII
       concentrate produced with recombinant techniques. Increased use of
       pharmacologic agents such as desmopressin acetate for patients with mild
       hemophilia A or type 1 von Willebrand's disease has allowed physicians
       to treat patients without the risk of infectious complications from
       plasma-derived factor concentrates. In addition to the management of the
       inherited bleeding disorders, patients may also require management of
       human immunodeficiency virus infection, hepatitis, and coagulation
       factor inhibitors. Issues for the coming years will include continued
       work to ensure product safety, the role of prophylactic treatment to
       prevent longterm disabilities, and the application of gene therapy to
       the management of bleeding disorders.
 DE    Blood Coagulation Disorders/EPIDEMIOLOGY/GENETICS/*THERAPY  Hereditary
       Diseases/EPIDEMIOLOGY/GENETICS/*THERAPY  Human  JOURNAL ARTICLE  REVIEW
       REVIEW, TUTORIAL

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

