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Hereditary Hypofibrinogenemia with Hepatic Storage [PDF]

open access: yesInternational Journal of Molecular Sciences, 2020
Fibrinogen is a 340-kDa plasma glycoprotein constituted by two sets of symmetrical trimers, each formed by the Aα, Bβ, and γ chains (respectively coded by the FGA, FGB, and FGG genes). Quantitative fibrinogen deficiencies (hypofibrinogenemia, afibrinogenemia) are rare congenital disorders characterized by low or unmeasurable plasma fibrinogen antigen ...
Rosanna Asselta   +2 more
exaly   +4 more sources

Congenital Afibrinogenemia and Hypofibrinogenemia: Laboratory and Genetic Testing in Rare Bleeding Disorders with Life-Threatening Clinical Manifestations and Challenging Management

open access: yesDiagnostics, 2021
Congenital fibrinogen disorders are rare pathologies of the hemostasis, comprising quantitative (afibrinogenemia, hypofibrinogenemia) and qualitative (dysfibrinogenemia and hypodysfibrinogenemia) disorders. The clinical phenotype is highly heterogeneous,
Rosanna Asselta   +2 more
exaly   +4 more sources

Tocilizumab-induced hypofibrinogenemia in patients with systemic-onset juvenile idiopathic arthritis

open access: yesScientific Reports, 2023
Systemic juvenile idiopathic arthritis (SJIA) is a chronic inflammatory disease of childhood with elevated serum IL-6 levels. As an inhibitor of IL-6R, tocilizumab (TCZ) has been approved to treat SJIA patients.
Tingyan He, Jiayun Ling, Jun Yang
doaj   +2 more sources

Women With Congenital Hypofibrinogenemia/Afibrinogenemia: From Birth to Death

open access: yesClinical and Applied Thrombosis/Hemostasis, 2020
Congenital fibrinogen disorders are a group of most frequent rare coagulation disorder, characterized by deficiency and/or defects in the fibrinogen molecule. Quantitative disorders include hypofibrinogenemia and afibrinogenemia.
Yue Teng
exaly   +2 more sources

Angiosarcoma of the breast with hypofibrinogenemia: A rare case report and review of the literature

open access: yesFrontiers in Oncology, 2022
BackgroundBreast angiosarcoma is a rare malignant tumor, accounting for approximately 0.04% of all breast malignancies. Angiosarcoma of the breast with hypofibrinogenemia is even rarer and has not been described in man.
Ran An   +3 more
exaly   +3 more sources

HYPOFIBRINOGENEMIA (A Report of Two Cases) [PDF]

open access: yesMedical Journal Armed Forces India, 1994
Hypofibrinogenemia is an uncommon bleeding disorder. Two siblings with mild bleeding diathesis were investigated and diagnosed as suffering from this disorder. The cases are discussed keeping in view of their rarity.
J R Bhardwaj, jr Bhardwaj
exaly   +3 more sources

Addressing some challenges of congenital fibrinogen disorders in 2023 and beyond

open access: yesBleeding, Thrombosis and Vascular Biology, 2023
Congenital fibrinogen disorders (CFD) include several types and subtypes of fibrinogen deficiency, resulting from monoallelic or biallelic mutations in one of the three fibrinogen genes. While it is relatively easy to make an accurate diagnosis based on
Cristina Santoro, Alessandro Casini
doaj   +3 more sources

Hypofibrinogenemia is associated with a high degree of risk in infectious diseases: a post-hoc analysis of post-marketing surveillance of patients with disseminated intravascular coagulation treated with thrombomodulin alfa

open access: yesThrombosis Journal, 2021
Background In patients with infectious diseases, disseminated intravascular coagulation (DIC) is often diagnosed without the fibrinogen value. The relationship between hypofibrinogenemia and outcomes of DIC in infectious diseases has thus remained ...
Kazuo Kawasugi   +11 more
doaj   +1 more source

Clinical Characteristics of Hospitalized Neonates With Hypofibrinogenemia: A Retrospective Cohort Study

open access: yesFrontiers in Pediatrics, 2020
Background: Neonatal hypofibrinogenemia is often asymptomatic but can manifest as hemorrhage.Objective: This study was conducted to characterize clinical characteristics of neonates with hypofibrinogenemia and identify factors associated with hemorrhage ...
Weijun Zhou   +5 more
doaj   +1 more source

Preeclampsia complicated with hypofibrinogenemia: 2 case reports and review of the literature

open access: yesBMC Pregnancy and Childbirth, 2023
Background Preeclampsia complicated with hypofibrinogenemia is a rare disorder. We report two cases of severe preeclampsia complicated with hypofibrinogenemia followed by postpartum haemorrhage (PPH).
Shiguang Li   +3 more
doaj   +1 more source

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