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KMID : 0361719920030020099
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Korean Journal of perinatology
1992 Volume.3 No. 2 p.99 ~ p.103
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A CAse of Congenital Afibrinogenemia
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Abstract
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Congential disorders of fibrinogen synthesis include those associated with deficient synthesis(afibrinogenemia and hypofibrinogenemia) and those associated with defective synthesis(dysfibrinogenemia). In afibrinogenemia there is absence of
circulating
fibrinogen, whereas in hypofibrinogenemia there is a reduced level of apparently normal fibrinogen. In the dysfibrnogenemia, the circulating fibrinogen may be in normal or reduced amounts but exhibits abnormal charcteristics.
In 1920 Rabe and Salomon reported the first case of congenital afibrinogenemia and about 150 cases were reported.
We experienced a 6-day-old male baby of congenital afibrinogenemia with umbilical cord bleeding. With the review of literatures, the case is presented.
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