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1996-02-27
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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).