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1/91. Factor V Leiden and antibodies against phospholipids and protein S in a young woman with recurrent thromboses and abortion.

    We describe the case of a 39-year-old woman who suffered two iliofemoral venous thromboses, a cerebral ischemic infarct and recurrent fetal loss. Initial studies showed high levels of antiphospholipid antibodies (APAs) and a moderate thrombocytopenia. After her second miscarriage, laboratory diagnosis revealed that the woman was heterozygous for the factor V Leiden mutation and had a functional protein s deficiency as well as anti-protein S and anti-beta 2-glycoprotein i antibodies. The impairment of the protein c pathway at various points could well explain the recurrent thromboses in the patient and supports the role of a disturbed protein c system in the pathophysiology of thrombosis in patients with APAs.
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ranking = 1
keywords = deficiency
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2/91. Hereditary homozygous heparin cofactor ii deficiency and the risk of developing venous thrombosis.

    heparin cofactor ii (HCII) is a specific inhibitor of thrombin in the presence of heparin or dermatan sulphate. Although there have been reports on families in which a heterozygous HCII deficiency is associated with thromboembolic events, several epidemiological studies revealed that heterozygous HCII deficiency is as prevalent among healthy subjects as it is among patients with deep venous thrombosis (DVT). It is therefore not yet clear whether HCII is or is not a thrombotic risk factor. We analyze and describe in an extended family the biochemical and genetic thrombophilic risk factors and evaluate the potential thrombotic risk involved in homozygous and heterozygous HCII deficiency, either alone or associated with other thrombotic or circumstantial risk factors. The propositus has had three episodes of DVT and a pulmonary embolism. During the first episode of DVT the patient was diagnosed as having AT deficiency. Later, a functional and antigenic HCII deficiency, compatible with the homozygous form, was detected. The family study shows that both the propositus and her sister have homozygous HCII deficiency and that 12 of the 27 family members have heterozygous HCII deficiency. This is possibly the first case report on a homozygous phenotype for the HCII deficiency with. in addition, partial AT deficiency. The propositus has suffered several thrombotic events, unlike the other 12 family members with heterozygous HCII deficiency and her sister, who is also homozygous for this disorder. We suggest that HCII deficiency may play a limited in vivo role as a thrombotic risk factor unless associated with AT deficiency or another congenital thrombotic risk factor.
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ranking = 16
keywords = deficiency
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3/91. factor vii deficiency detected in pregnancy: a case report.

    factor vii deficiency is a rare hereditary coagulation disorder with an incidence estimated at 1 in 500,000 individuals. In this report, we describe the 13th case in pregnancy. The diagnosis of severe factor vii deficiency (factor VII level <5%) was established at 10 weeks' gestation after initial laboratory testing showed a markedly prolonged prothrombin time and a normal activated partial thromboplastin time. There was a history of two preterm deliveries, but there was no evidence of previous bleeding manifestations. Antenatal progress of the index pregnancy was unremarkable. Prophylactic treatment with fresh frozen plasma was started at the onset of labor and the patient had a vaginal delivery of a live girl at 36 weeks' gestation. There was no postpartum hemorrhage and mother and newborn were discharged in good condition. The patient's postpartum level of factor VII remained undetectable. Two aspects are outlined: the absence of any significant increase in factor VII clotting activity during this pregnancy and the need to give replacement therapy at labor in patients with severe factor VII deficiency to decrease the risk of postpartum hemorrhage.
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ranking = 7
keywords = deficiency
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4/91. A case report: pregnancy complicated by blue rubber-bleb nevus syndrome.

    Blue rubber-bleb nevus (BRBN) syndrome, first reported in 1958 by Bean, manifests with multiple hemangiomas located in the skin and gastarointestinal tract. Characteristic laboratory data include chronic anemia with iron deficiency and consumption coagulopathy. We describe herein a pregnancy complicated by BRBN syndrome resulting in the delivery of a male infant by cesarean section.
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ranking = 1
keywords = deficiency
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5/91. Glanzmann's thrombasthenia in pregnancy: a case and review of the literature.

    Glanzmann's thrombasthenia is a rare autosomal recessive bleeding disorder resulting from a deficiency of glycoprotein IIb-IIIa complex in platelets. The deficient complex normally mediates platelet aggregation by binding adhesive proteins, which form bridges between activated cells. Despite normal platelet counts, morphology, prothrombin, and activated thromboplastin times, Glanzmann's thrombasthenia is characterized by a prolonged bleeding time and a severe hemorrhagic mucocutaneous diasthesis. pregnancy and delivery are rare in these patients and have been associated with a high risk of severe hemorrhage. We present an unusual case in which a primi-gravida patient with Glanzmann's thrombasthenia underwent an uneventful pregnancy and spontaneous vaginal delivery, following intrapartum intravenous administration of single-donor platelets. Subsequent late postpartum hemorrhage required intravenous transfusion of an additional unit of single-donor platelets. In addition, we review the literature pertaining to pregnancy and Glanzmann's thrombasthenia with an emphasis on intrapartum prophylactic management.
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keywords = deficiency
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6/91. Bleeding risk and reproductive capacity among patients with factor xiii deficiency: a case presentation and review of the literature.

    factor xiii deficiency is an uncommon, inherited bleeding disorder that usually manifests in infancy or early childhood, involving both boys and girls. We present the case of a woman who had experienced two previous intracranial bleeding events, and was treated before and during her current pregnancy with factor XIII concentrate. Her pregnancy was successful, and she experienced an uncomplicated vaginal delivery. To better understand the issues surrounding bleeding, reproductive capacity, and management of factor xiii deficiency during pregnancy, we conducted a systematic literature review using medline from 1966 to December 1998. We also examined the bibliographic references from all articles, and included all cases, case reports, or case series of patients with factor xiii deficiency. We retrieved data on 117 patients from 37 articles, the majority of which had type II deficiency. Among untreated patients with type II factor xiii deficiency, the literature suggests an elevated mortality rate due to uncontrolled bleeding and intracranial hemorrhage. Because of its high degree of efficacy, the evidence supports the use of life long prophylactic therapy with at least monthly infusions of factor XIII concentrate, including during pregnancy. The opinion that women with type II factor xiii deficiency have inevitable recurrent abortions, or that men are sterile, is not well substantiated. No data were found on whether treatment alters male reproductive capacity. A policy of universal factor XIII replacement, starting in childhood, will likely enable more patients to attain reproductive status. The development of an international data registry would optimally address both bleeding risk and reproductive capacity among patients with factor xiii deficiency.
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ranking = 11
keywords = deficiency
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7/91. Maternal blood coagulation factor XIII is associated with the development of cytotrophoblastic shell.

    We analysed the early implantation tissues of normal women and of a patient with congenital factor xiii deficiency in order to study the role of maternal subunit A of factor XIII (XIIIA) in the development of extravillous cytotrophoblast. The patient had received adequate administration of factor xiiia concentrate only up to 7 weeks of gestation (wG). Her pregnancy was maintained until the latter half of 8 wG, but was terminated by intrauterine fetal death at 9 wG. Immunohistochemical staining of cytokeratin, XIIIA and subunit S of factor XIII was performed in the early implantation tissues of normal women and of this patient. Numerous well-formed cytotrophoblastic shells and Nitabuch's layers were detected in implantation tissues at 7-8 wG in normal women, and XIIIA was present in the intercellular space in well-formed cytotrophoblastic shells, while the cytotrophoblastic shells and Nitabuch's layers in this patient's implantation tissue were poorly-formed. Furthermore, XIIIA was not detected around them. It is suggested that when the maternal plasma activity of factor XIII is low, the concentration of XIIIA at the placental bed is also low, leading to the insufficient formation of cytotrophoblastic shell and therefore an increased probability of miscarriage in patients with congenital factor xiii deficiency.
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ranking = 2
keywords = deficiency
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8/91. Continuous infusion of recombinant activated factor VII during caesarean section delivery in a patient with congenital factor vii deficiency.

    Recombinant activated factor VII (rFVIIa) can be used as an alternative therapy in patients with FVII deficiency. However, as the drug has a very short half-life, continuous infusion could be a meaningful administration modality. We report the case of a 30-year-old woman with moderate FVII deficiency and human immunodeficiency virus infection who underwent a caesarean section delivery. She was treated with a continuous infusion of rFVIIa and did not suffer any bleeding complication. The continuous infusion of rFVIIa was a safe and effective therapeutic approach for our patient, maintaining her levels of FVII:C and avoiding bleeding during caesarean section and afterwards.
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ranking = 7
keywords = deficiency
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9/91. Prophylactic therapy in a pregnant woman with severe factor vii deficiency.

    This paper reports a case of factor vii deficiency, a very rare autosomal recessive disorder, in a primipara at 37 weeks of gestation in whom we administered prophylactic therapy with factor VII every 12 h until 24 h after delivery. The prophylactic use of factor VII enabled the woman to deliver without hemorrhagic complications.
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ranking = 5
keywords = deficiency
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10/91. Intracranial venous thrombosis associated with severe antithrombin-III deficiency in pregnancy.

    We report a patient with intracranial venous thrombosis in the third trimester of pregnancy associated with severe antithrombin-III deficiency. The evaluation of protein c, protein S and antithrombin-III levels in patients with thrombotic events during pregnancy may reveal the specific cause of the thrombotic event and thereby influence patient management
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ranking = 5
keywords = deficiency
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