1/12. Prenatal and peripartum management of congenital afibrinogenaemia.We experienced three cases and four successful deliveries with congenital afibrinogenaemia and propose the following guidelines for the prenatal and peripartum management: (i) genital bleeding usually begins at 5 weeks' gestation and spontaneous abortion always occurs at 6-8 weeks' gestation without fibrinogen infusion; (ii) the fibrinogen level must be at least 0.60 g/l and, if possible, higher than 1.0 g/l during the pregnancy; (iii) the necessary amounts of fibrinogen increase as the pregnancy progresses and the preterm labour occurs; (iv) the fibrinogen level under the continuous infusion of fibrinogen during labour must be at least 1.5 g/l and, if possible, higher than 2.0 g/l to prevent placental abruption; (v) the puerperium is usually uneventful with a reduced dose of fibrinogen infusion.- - - - - - - - - - ranking = 1keywords = pregnancy, gestation (Clic here for more details about this article) |
2/12. Hypofibrinogenaemia with compound heterozygosity for two gamma chain mutations - gamma 82 Ala-->Gly and an intron two GT-->AT splice site mutation.We investigated the molecular basis of hypofibrinogenaemia in a woman with a history of recurrent, pregnancy-associated bleeding, and miscarriage. She had a Clauss fibrinogen of 0.9 mg/ml and SDS PAGE of purified fibrinogen showed a normal pattern of chains. However careful inspection of reverse phase chain separation profiles showed apparent homozygosity for a more hydrophilic form of the gamma chain. dna Sequencing showed only heterozygosity for a CGT-->GGT (Ala-->Gly) mutation at codon gamma82, but further sequencing showed an additional GT splice sequence mutation at the 5' end of intron 2 of the gamma gene. Translation of mRNA containing this intron would result in premature truncation explaining the phenotypic homozygosity of the gamma82 Ala-->Gly substitution. The patient's sister had a mild bleeding disorder with hypofibrinogenaemia and she too was a compound heterozygote for the y mutations. Her nephew had only the novel splice site mutation, while her mother and daughter inherited only the gamma82 Ala-->Gly substitution.- - - - - - - - - - ranking = 0.35011019861017keywords = pregnancy (Clic here for more details about this article) |
3/12. Hypodysfibrinogenemia during pregnancy, labor, and delivery.BACKGROUND: Hypodysfibrinogenemia is an autosomally dominant disorder that can result in excessive bleeding as well as specific pregnancy complications. Increased risks of spontaneous abortion, postpartum hemorrhage, poor wound healing, and placental abruption have been reported. CASE: A woman with hypodysfibrinogenemia presented for care in the first trimester. Her antepartum course was uncomplicated, and she was administered intermediate-purity factor viii during labor and did not have excessive bleeding postpartum. The infant's cord fibrinogen was low, at 43 mg/dL (normal 215 /- 30), showing it was similarly affected. CONCLUSION: Pregnancy in patients with hypodysfibrinogenemia can be associated with various complications; however, coordination of care and anticipation of specific problems can result in a successful outcome for both mother and infant.- - - - - - - - - - ranking = 1.7505509930509keywords = pregnancy (Clic here for more details about this article) |
4/12. Pregnancy-related thrombosis in a woman with congenital afibrinogenemia: a report of two successful pregnancies.We managed two pregnancies in a woman with congenital afibrinogenemia with increasing amounts of cryoprecipitate to achieve a pre-infusion fibrinogen level of 60 mg/dL. The first pregnancy resulted in placental abruption at 36 weeks, in spite of recent cryoprecipitate infusion. Both placentas showed infarction. Post-partum ovarian and renal vein thromboses complicated the second pregnancy. Mean FVIII (344%) and vWF Antigen (323%) were elevated prior to cryoprecipitate infusion, with mean post-infusion levels of 367% and 363%. The clearance of fibrinogen after cryoprecipitate infusion increased during the course of pregnancy. A paradoxical prothrombotic state with embolization may play a role in the observed complications of pregnancy.- - - - - - - - - - ranking = 1.4004407944407keywords = pregnancy (Clic here for more details about this article) |
5/12. Congenital hypofibrinogenemia in pregnancy: report of two cases and review of the literature.Fibrinogen abnormalities have been implicated in many adverse pregnancy outcomes, mainly spontaneous abortion, placental abruption, and postpartum hemorrhage. Two new cases of congenital hypofibrinogenemia in pregnancy are reported detailing their obstetric course and management. The relevant obstetric and hematologic literature is reviewed, including previous case reports and studies concerning the mechanisms of pregnancy complications. Suggestions for treatment guidelines and management strategies are detailed.- - - - - - - - - - ranking = 2.4507713902712keywords = pregnancy (Clic here for more details about this article) |
6/12. Congenital hypofibrinogenemia in pregnancy.A new case of congenital hypofibrinogenemia in pregnancy is reported and the literature is reviewed from an obstetric perspective. An association with spontaneous abortion, abruption, and postpartum hemorrhage is documented and management options are presented.- - - - - - - - - - ranking = 1.7505509930509keywords = pregnancy (Clic here for more details about this article) |
7/12. Congenital hypofibrinogenemia and pregnancy, obstetric and hematological management.A 27-year-old pregnant woman with severe congenital hypofibrinogenemia was studied from the 18th to the 40th week of pregnancy, after which she had a normal delivery. The results of the quantitative and qualitative fibrinogen studies done on the patient made it possible to rule out associated dysfibrinogenemia. No variations in the concentration and function of fibrinogen were observed during gestation. The only treatment given was a transfusion of fresh plasma prior to delivery. The importance of fibrinogen in maintaining normal placental insertion and the obstetric management of predelivery and delivery are discussed.- - - - - - - - - - ranking = 1.9004407944407keywords = pregnancy, gestation (Clic here for more details about this article) |
8/12. Constitutional hypofibrinogenemia associated with third trimester hemorrhage.This case of a 31-year-old white woman presenting at 32 weeks gestation with vaginal bleeding supports a possible association between constitutional hypofibrinogenemia and third trimester hemorrhage. Clot based fibrinogen assays were persistently low for the third trimester (patient: 102 to 155 mg per dl; normal range: 400 to 650 mg per dl) and at follow-up, ten months post-partum (patient: 90 mg per dl; normal range 180 to 436 mg per dl); an immunologic fibrinogen level was comparable. The patient gave an unremarkable bleeding history. Of 11 previously reported pregnancies in five hypofibrinogenemic patients, six terminated in placental abruption during the third trimester, two others were complicated by significant post-partum hemorrhage, and one by spontaneous abortion. This case report emphasizes that low functional levels of fibrinogen are potentially disruptive to the integrity of the uteroplacental interface. The pregnant state unmasks and amplifies an otherwise silent to mild hemostatic disorder.- - - - - - - - - - ranking = 0.14988980138983keywords = gestation (Clic here for more details about this article) |
9/12. Congenital afibrinogenemia and recurrent early abortion: a case report.The role of the fibrinogen molecule in the maintenance of normal pregnancy is not yet well understood; however, several cases have been previously reported in which failure to complete normal pregnancy was associated with either hypofibrinogenemia, dysfibrinogenemia, or deficiency in factor xiii (fibrin-stabilizing factor) which is important for the crosslinking of the fibrin. A case of congenital afibrinogenemia is described. The patient, a 22-yr-old woman, who suffered from a moderate hemorrhagic tendency associated with very low (less than 10 mg/dl) plasma fibrinogen levels, had three consecutive spontaneous abortions. In view of the previous cases reported, the question is raised whether patients with low or abnormal fibrinogen should be treated with plasma transfusions in order to maintain a normal pregnancy.- - - - - - - - - - ranking = 1.0503305958305keywords = pregnancy (Clic here for more details about this article) |
10/12. First report of case of congenital afibrinogenemia with successful delivery.We report a case in which pregnancy was sustained in a woman with congenital afibrinogenemia with delivery by cesarean section. From this case it appears that a plasma fibrinogen level greater than 60 mg/dl would maintain implantation of the placenta and fetus even in the event of complications occurring during pregnancy.- - - - - - - - - - ranking = 0.70022039722034keywords = pregnancy (Clic here for more details about this article) |
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