Abstract
Two siblings with congenital afibrinogenemia received prophylactic infusions of cryoprecipitate in order to prevent serious hemorrhage during early childhood. Three bags (units) of this fibrinogen-containing blood product were administered every 7-10 days for 15 months in one case and 24 months in the other. Hemorrhage was nearly completely prevented during the period of prophylaxis. Side effects from cryoprecipitate were not observed. Prophylactic infusion of cryoprecipitate, made practical by the relatively long half-life of fibrinogen, should be considered in other patients with this disorder.
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References
1.
Mammen EF
Fibrinogen abnormalities . Semin Thrombos Hemostas
1983 ;9 :1 -9 .
2.
Flute PT
Disorders of plasma fibrinogen synthesis . Br Med Bull
1977 ;33 :253 -9 .
3.
Hattersley PG
,
Dimich ML
Cryoprecipitate in treatment of congenital fibrinogen deficiency . Transfusion
1969 ;9 :261 -4 .
4.
Manios SG
,
Schenck W.
,
Kunzer W.
Congenital fibrinogen deficiency . Acta Paediatr Scand
1968 ;57 :145 -50 .
5.
Weiss NJ
,
Rogers J.
Fibrinogen and platelets in the primary arrest of bleeding . N Engl J Med
1971 ;285 :369 -74 .
6.
Montgomery R.
,
Natelson SE
Afibrinogenemia with intracerebral hematoma . Am J Dis Child
1977 ;131 :555 -6 .
7.
Rabe F.
,
Salomon E.
Uber Faserstoffmangel im Blut bei einem Falle von Haemophilie . Dtsch Arh Klin Med
1920 ;132 :240 -4 .
8.
Marder VJ
The functional defects of hereditary dysfibrinogens . Thromb Haemost
1976 ;36 :1 -8 .
9.
Ness PM
,
Perkins HA
Cryoprecipitate as a reliable source of fibrinogen replacement . JAMA
1979 ;241 :1690 -1 .
10.
Goodnight SH
Cryoprecipitate and fibrinogen . JAMA
1979 ;241 :1716 -7 .
11.
Bove JR
Fibrinogen—Is the benefit worth the risk?
Transfusion
1978 ;18 :129 -36 .
12.
Fisher MC
Transfusion-associated acquired immunodeficiency syndrome—What is the risk?
Pediatrics
1987 ;79 :157 -60 .
13.
Lilleyman JS
Haemophilia, blood transfusion, and the AIDS virus . Arch Dis Child
1986 ;61 :105 -7 .
