Management of pregnancy in dysfibrinogenemia cases

Patil, Rucha; Mukaddam, Alfiya; Ghosh, Kanjaksha; Shetty, Shrimati

doi:10.1097/mbc.0000000000000514

Cited by 8 publications

(4 citation statements)

References 10 publications

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“…Overall, the majority of the miscarriages occurred during the first trimester (76%) and 17% during the first pregnancy. Several women (37%) had more than one miscarriage, including one hypofibrinogenaemic woman with six miscarriages all related to severe vaginal bleedings and a dysfibrinogenaemic woman, otherwise asymptomatic, with seven early miscarriages without any other presumptive aetiological cause (anatomic, hormonal, karyotyping or infection) . Among the other pregnancy outcomes, one afibrinogenaemic woman had an interstitial pregnancy related to a complete obstruction of the right fallopian tube.…”

Section: Resultsmentioning

confidence: 99%

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Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

et al. 2019

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Introduction: Hereditary fibrinogen disorders (HFD) are rare quantitative or qualitative fibrinogen anomalies, including afibrinogenaemia (A), hypofibrinogenaemia (H), dysfibrinogenaemia (D) and hypodysfibrinogenaemia (HD). As fibrinogen plays an essential role in pregnancy, we addressed the issue of obstetrical and postpartum complications in women with HFD. Methods: A systematic literature review, restricted to English manuscripts, was conducted according to the PRISMA guidelines. We searched through the MEDLINE database for English articles, published from January 1985 until November 2018, focusing on pregnancy in A, H, D and HD. A total of 198 articles were identified, 15 articles were added from other sources. Overall, 213 articles were screened and 54 were included in the final analysis.Results: A total of 188 pregnancies from 70 women were analysed. About half of pregnancies resulted in miscarriage; more specifically in 15 (42.9%), 36 (46.8%), 27 (42.9%) and 4 (30.8%) of A, H, D and HD patients, respectively. Preterm complications were also frequent (33.5%). Metrorrhagia, mainly in the first trimester, was observed in 21.7% of the pregnancies. Placenta abruption was reported in 5 (14.3%), 4 (5.2%), 5 (7.9%) and 1 (7.7%) of A, H, D and HD, respectively. A total of 24 (12.7%) deliveries were complicated by postpartum thrombotic events (3.2%) or postpartum haemorrhage (9.6%). A fibrinogen replacement therapy was introduced in 30% of pregnancies, as prophylaxis (81.1%) or on demand (18.9%). Conclusion:These results suggest that women with HFD are at high risk of obstetrical and postpartum complications. Prospective international registries may allow to identify more precisely the incidence of obstetrical and postpartum adverse outcomes and their management. K E Y W O R D S afibrinogenaemia, dysfibrinogenaemia, hypofibrinogenaemia, miscarriage, postpartum haemorrhage, pregnancy

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Section: Resultsmentioning

confidence: 99%

“…Such women could benefit from an antithrombotic treatment during the pregnancy. A thromboprophylaxis concomitant to a fibrinogen replacement has been proposed in dysfibrinogenaemic women with recurrent miscarriages …”

Section: Discussionmentioning

confidence: 99%

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

et al. 2019

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“…In afibrinogenemia, the replacement therapy should be instituted as soon as possible in pregnancy in order to prevent hemorrhagic complications and fetal loss. However, symptoms may vary making phenotype prediction more complex, and in turn, management of pregnancy very difficult, with a patient-by-patient approach [ 77 , 78 ].…”

Section: Genotype-phenotype Correlation In Fibrinogen Disordersmentioning

confidence: 99%

Human Fibrinogen: Molecular and Genetic Aspects of Congenital Disorders

Tiscia

Margaglione

2018

IJMS

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Congenital fibrinogen disorders can be quantitative (afibrinogenemia, hypofibrinogenemia) or functional (dysfibrinognemia). To date, several genetic variants have been identified in individuals with fibrinogen disorders. The complexity of the fibrinogen molecules, formed by three non-identical chains and with a trinodal organization, renders the identification of molecular causes and of clinical and biochemical phenotypes very challenging. However, the acknowledgement of the type of molecular defect is crucial for a safer therapy, which is going to improve the clinical management of these patients. In this review, some aspects concerning molecular and clinical findings available on congenital fibrinogen disorders will be discussed.

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“…abnormalities [5]. Pregnancy is a demanding time for a woman leading to imbalance in hemostasis and sometimes is the first time when coagulation disorder reveal [6,10]. Proper treatment of fibrinogen disorders may prevent from serious complications for gravida such as miscarriage, thrombosis or haemorrhage [6,7].…”

Section: Introductionmentioning

confidence: 99%

Dealing with hypodysfibrinogenemia during pregnancy with a successful outcome

et al. 2021

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Background: Fibrinogen is a protein playing pleiotropic role in human body. It is engaged in maintaining hemostasis. Congenital fibrinogen disorders comprise quantitative and qualitative fibrinogen anomalies. The symptoms range from bleeding, thrombosis to asymptomatic at all what is the most common case. Hypodysfibrinogenemia with lower level of fibrinogen of reduced activity, is the least common of all congenital fibrinogen disorders. Case report: A 31-year-old woman was reported at the 21 weeks of gestation, suffered from genital tract bleeding and there was a history of stillbirth. Clinical examination with no pathology, however laboratory tests revealed coagulation abnormalities due to prolonged thrombin test, decreased protein S and lower fibrinogen level (70 mg/dl). Autoimmune diseases were excluded and the diagnosis was widened with rotational thromboelastometry and genetic test for hypodysfibrinogenemia. The patient was treated with fibrinogen substitution and prophylactic dose of heparin throughout pregnancy and 2 weeks following labour. At 39 week of gestation Caesarean section was done, with no complications. Results: Genetic test revealed heterozygous mutation in fibrinogen gamma gene confirming hypodysfibrinogenemia. Due to bleeding manifestation in this patient of congenital fibrinogen disorders, fibrinogen substitution was implemented with heparin as a paranticoagulant prophylaxis, what turned out to be successful and enabled the patient to maintain the pregnancy. Conclusions: As hypodysfibrinogenemia symptoms are diverse the management is difficult and each patient’s therapy should be planned separately. Pregnancy may be the first time when congenital fibrinogen disorders reveal and it is especially challenging to prevent from obstetrical complications.

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Management of pregnancy in dysfibrinogenemia cases

Cited by 8 publications

References 10 publications

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

Obstetrical and postpartum complications in women with hereditary fibrinogen disorders: A systematic literature review

Human Fibrinogen: Molecular and Genetic Aspects of Congenital Disorders

Dealing with hypodysfibrinogenemia during pregnancy with a successful outcome

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