Congenital Deficiency of Fibrinogen in Two Sisters

Dube, B; Agarwal, Sonal; Gupta, Mrinal; Chawla, Sahar

doi:10.1159/000208721

Cited by 22 publications

(11 citation statements)

References 8 publications

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“…The platelet count in our patient was consistantly normal or above normal, while the bleeding time was always moder ately prolonged. The defect in platelet adhesion and aggregation displayed in pa tients with congenital afibrinogenaemia has been reported by many authors [5,8,10,17]. The results of platelet aggregation tests vary with the anticoagulant used and the concentration of the aggregating agent added [8,17], Also in our case, the reduced ADP-induced platelet aggregation is cor rected by the addition of normal plateletpoor plasma.…”

Section: Discussionsupporting

confidence: 68%

“…Other reports confirmed the heredi tary nature of afibrinogenaemia [1,5,13]. Consistent with an autosomal recessive in heritance of congenital afibrinogenaemia, the heterozygotes are asymptomatic but hypofibrinogenaemic.…”

Section: Discussionsupporting

confidence: 53%

“…Dube [5] reported a pregnancy that end ed in an abortion at 3 months in a patient with congenital afibrinogenaemia. Hahn [9] reported 6 spontaneous abortions, all in the first trimester in a patient with con genital hypofibrinogenaemia and postu lated that the mothers' fibrinogen plays a role in the maintenance of early pregnan cy.…”

Section: Discussionmentioning

confidence: 99%

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Congenital Afibrinogenaemia in a Saudi Family: A Case Report and Family Study

Elseed¹,

Karrar²

1984

Acta Haematol

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Section: Discussionsupporting

confidence: 68%

Section: Discussionsupporting

confidence: 53%

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Congenital Afibrinogenaemia in a Saudi Family: A Case Report and Family Study

Elseed¹,

Karrar²

1984

Acta Haematol

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“…As to congenital afibrinogenemia, 2 cases were reported [1, 2] as summarized in table 1. Their pregnancies resulted in spontaneous abortions without any replacement therapy.…”

Section: Introductionmentioning

confidence: 99%

Studies on the Role of Adhesive Proteins in Maintaining Pregnancy

Asahina

Kobayashi²,

Okada³

et al. 1998

Horm Res Paediatr

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It is well known that maternal fibrinogen (Fg) and factor X III are essential for maintaining early pregnancy. We studied their role by analysis of clinical reports and immunohistochemical investigation. Methods: (1) We analyzed the pregnancy cases of congenital afibrinogenemia and congenital factor X III deficiency. (2) Immunohistochemical staining of Fg, subunit A of factor X III (X III_A) and fibronectin (Fn) were performed in the human implantation site, placenta, and endometrial cells cultured in serum-free medium. Results: (1) Afibrinogenemia needed to be administrated Fg from 4 weeks’ gestation (4 wG), and factor X III deficiency needed factor X III concentrate from 5 wG, in order to prevent abortion. (2) Implantation tissues: Fg, cellular X III_A and Fn were present at the decidual stroma around invasive cytotrophoblasts at 5 wG. X III_A-positive cells coincided with LN-5-positive macrophages. Placenta: Fg, cellular X III_A and Fn were present in the decidual layer. Endometrial culture cells: Fn was secreted by spindle-like shaped cells. X III_A was secreted by round-shaped cells. Conclusion: Maternal Fg and factor X III are essential just after 4∼5 wG, and in that period they and Fn are present abundantly in decidual stroma around invasive cytotrophoblasts. It is concluded that when cytotrophoblasts invade endometrium maternal Fg, factor X III and Fn are concerned with cytotrophoblasts’ anchoring as adhesive proteins.

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“…Several cases with congenital afibri nogenemia or congenital factor XIII deficiency have pre viously been reported which all resulted in abortion at an early stage of gestation without any appropriate replace ment therapy [4][5][6][7][8]. Children with these congenital dis eases tend to die from omphalorrhagia during the neona tal period, and if they survive, they suffer from bleeding, e.g.…”

Section: Introductionmentioning

confidence: 99%

Congenital Afibrinogenemia with Successful Delivery

Kobayashi

Asahina

Maehara

et al. 1996

Gynecol Obstet Invest

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We experienced a case of congenital afibrinogenemia and successfully performed cesarean section with administration of fibrinogen. The patient was administered fibrinogen every week to sustain a fibrinogen level above 60 mg/ dl according to our previously reported first case. Pregnancy course was uneventful, and fetal growth was normal, but unfortunately placental abruption occurred after the spontaneous onset of labor at 37 weeks gestation. The fibrinogen level before labor was 96 mg/dl, but decreased to 33 mg/dl when placental abruption was diagnosed. During and after the operation, it was increased to 147 and 199 mg/dl, respectively, through infusion of 10 g of fibrinogen, and massive bleeding was stopped. Two grams of fibrinogen were infused daily after cesarean section, and postpartum hemorrhage was normal. It is obvious that fibrinogen is an extremely important factor in maintaining pregnancy, and we conclude that fibrinogen level must be at least 60 mg/dl during pregnancy, 120 mg/dl during surgery and 150 mg/dl during labor, if possible as high as 200 mg/dl, under the continuous infusion of fibrinogen to prevent placental abruption.

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Congenital Deficiency of Fibrinogen in Two Sisters

Cited by 22 publications

References 8 publications

Congenital Afibrinogenaemia in a Saudi Family: A Case Report and Family Study

Congenital Afibrinogenaemia in a Saudi Family: A Case Report and Family Study

Studies on the Role of Adhesive Proteins in Maintaining Pregnancy

Congenital Afibrinogenemia with Successful Delivery

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