Congenital hypofibrinogenemia.

Congenital Hypofibrinogenemia Detected during Preoperative Workup for Major Cardiac Surgery

Afibrinogenemia or hypofibrinogenemia is a quantitative defect in fibrinogen. It is a rare disease with estimated frequency of 1 in 1,000,000 population. Routine preoperative screening tests of coagulation disorders are insufficient to detect subtle disorders of fibrinogen. Herein, we report a child who was supposed to undergo open cardiac surgery for a large atrial septal defect. Preoperative ...

Congenital Hypofibrinogenemia: A Newborn Infant with Cord Bleeding.

The genetic disorders causing decreased fibrinogen synthesis may be caused by heterozygous (hypofibrinogenemia) or homozygous (afibrinogenemia) deficiency. The consequences of the disorders are gastrointestinal bleeding, cord bleeding, eccymoses, subcutaneous hematomas and hemarthroses especially due to traumatic delivery in the neonatal period. Laboratory evaluation of the patient with hypofib...

Congenital hypofibrinogenemia in pregnancy: a report of 11 cases

: To investigate the obstetrical outcomes and plasma concentrations of fibrinogen (FIB) in patients with congenital hypofibrinogenemia in pregnancy, 11 cases with hypofibrinogenemia in pregnancy were analyzed retrospectively. The demographic database, bleeding tendency, plasma levels of FIB throughout pregnancy, peripartum management, as well as, maternal and neonatal outcomes were assessed. FI...

Massive hemoperitoneum due to a ruptured corpus luteum cyst in a patient with congenital hypofibrinogenemia

Congenital afibrinogenemia/hypofibrinogenemia is a rare inherited hematologic disorder in which a patient lacks or has insufficient level of fibrinogen, the blood coagulation factor I. The incidence of this uncommon disease is 1 to 2 per 1 million individuals. Hence, massive hemoperitoneum caused by ovulation in a woman with congenital afibrogenemia is also a very rare clinical condition. Massi...

Dental treatment of a patient with congenital afibrinogenemia--complications of supportive care.