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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. FIB levels in our patients remained relatively stable throughout the pregnancy. The mean FIB levels during the three trimesters of pregnancy were 75.7 ± 43.9 (25–148), 67.6 ± 33.7 (22–146), and 77.9 ± 29.2 (32–148) mg/dl, respectively. Twelve full-term deliveries were achieved using FIB replacement therapy only on the group of labor or cesarean deliveries. The postpartum courses were unremarkable without hemorrhagic or thrombotic complications. Five out of twelve neonates were diagnosed with low levels of FIB in 6 weeks after birth. The pregnancies were uneventful with no signs of bleeding in these congenital hypofibrinogenemia women. Vaginal delivery, instead of cesarean section, may be the superior choice. Successful maternal and neonatal outcomes could be achieved by accurate monitoring of the FIB levels and adequate supportive therapy.
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: Original Articles Source Type: research

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Source: Journal of Comparative Effectiveness Research - Category: General Medicine Tags: J Comp Eff Res Source Type: research
In conclusion, these data suggest that increased prenatal Ang2 has a predominant impact on the properties of renal Na,K-ATPase in both sexes. Moreover, the enzyme is resistant to higher salt intake in offspring prenatally exposed to Ang2. PMID: 29769424 [PubMed - in process]
Source: Journal of Physiology and Pharmacology - Category: Drugs & Pharmacology Tags: J Physiol Pharmacol Source Type: research
Authors: Iciek R, Brazert M, Wender-Ozegowska E, Pietryga M, Brazert J Abstract Type 1 diabetes mellitus (T1DM) is still related to altered fetal growth and severe maternal complications. We studied the possible role of placental visfatin/nicotinamide phosphoribosyltransferase (NAMPT) in fetal development in T1DM pregnancies, the possible role of placental visfatin in fetal macrosomia. Sixty five pregnant women with T1DM and singleton pregnancy were qualified into the study. Placental visfatin expression was by analysed by RT-PCR. We demonstrated the lowest expression of placental visfatin in women who delivered ne...
Source: Journal of Physiology and Pharmacology - Category: Drugs & Pharmacology Tags: J Physiol Pharmacol Source Type: research
Authors: Kohsaka S, Katada J, Saito K, Terayama Y Abstract OBJECTIVE: To investigate the risk of bleeding events and stroke/systemic embolism (SE) among Japanese patients with nonvalvular atrial fibrillation (NVAF), focusing on the initial dosage of apixaban and patient age. METHODS: This retrospective cohort study used de-identified electronic health records-based claims data from 314 acute-care hospitals in Japan. NVAF patients newly initiated on warfarin or apixaban, with no prescription during the 180-day blanking period, were eligible. Patients were allocated to receive warfarin or 5 or 2.5 mg twice...
Source: Current Medical Research and Opinion - Category: Research Tags: Curr Med Res Opin Source Type: research
ConclusionDysfibrinogenemia patients without personal or family history of bleeding and thromboembolic events, do not need specific therapeutic intervention.
Source: Journal of Clinical Laboratory Analysis - Category: Laboratory Medicine Authors: Tags: BRIEF REPORT Source Type: research
ConclusionThe results of this initiative should help clinicians in the difficult management of patients with congenital fibrinogen disorders.
Source: Haemophilia - Category: Hematology Authors: Tags: Original Article Source Type: research
Publication date: June–July 2016 Source:Medicina Intensiva (English Edition), Volume 40, Issue 5 Author(s): E. Guasch, F. Gilsanz Massive obstetric hemorrhage is a major cause of maternal mortality and morbidity worldwide. It is defined (among others) as the loss of >2500ml of blood, and is associated to a need for admission to critical care and/or hysterectomy. The relative hemodilution and high cardiac output found in normal pregnancy allows substantial bleeding before a drop in hemoglobin and/or hematocrit can be identified. Some comorbidities associated with pregnancy can contribute to the occurrence of ca...
Source: Medicina Intensiva - Category: Intensive Care Source Type: research
We present a case of mild FXIII deficiency and concurrent hypofibrinogenemia manifested by recurrent postpartum hemorrhage, menorrhagia, and miscarriage. Mutational analysis identified a previously unreported heterozygous mutation of the FXIIIA subunit (p.Trp315Arg). No mutation was noted in the fibrinogen gene. FXIII levels decreased approximately 50% from nonpregnant levels to their nadir during labor, whereas fibrinogen levels rose approximately 1.5-fold from decreased nonpregnant levels to their peak at the time of labor. This case illustrates the course of mild FXIII and fibrinogen deficiencies during pregnancy, labor...
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: Case Reports Source Type: research
Semin Thromb HemostDOI: 10.1055/s-0036-1571339Congenital fibrinogen disorders are rare diseases affecting either the quantity (afibrinogenemia and hypofibrinogenemia) or the quality (dysfibrinogenemia) or both (hypodysfibrinogenemia) of plasmatic fibrinogen. Afibrinogenemia is often diagnosed at birth following prolonged umbilical cord bleeding and is characterized by spontaneous bleeding in all tissues, while hypofibrinogenemic patients are more often asymptomatic. Spontaneous spleen ruptures, painful bone cysts, cardiovascular events, and intrahepatic inclusions can complicate the clinical course of patients with quantit...
Source: Seminars in Thrombosis and Hemostasis - Category: Hematology Authors: Source Type: research
Conclusions Simultaneous determination of fibrinogen concentrations using the Clauss method, prothrombin time-derived method, and immunoturbidimetry as well as measurement of the thrombin time, reptilase time, prothrombin time, activated partial thromboplastin time and so on can effectively distinguish dysfibrinogenemia from other diseases. Special treatment of asymptomatic dysfibrinogenemia is not needed during pregnancy or surgery in the absence of bleeding or thrombotic events in the patient's personal or family history.
Source: Clinica Chimica Acta - Category: Laboratory Medicine Source Type: research
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