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Skeletal changes in congenital fibrinogen abnormalities

R Lagier, C A Bouvier, N Van Strijthem

    Skeletal Radiology
    |January 1, 1980
    PubMed
    Summary

    Congenital afibrinogenemia and dysfibrinogenemia can cause juxtatrabecular hemorrhages and bone changes. Further skeletal studies are recommended for patients with clotting disorders.

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    Area of Science:

    • Orthopedic Surgery
    • Hematology
    • Radiology

    Background:

    • Congenital afibrinogenemia is a rare bleeding disorder characterized by a complete absence of fibrinogen.
    • Congenital dysfibrinogenemia involves qualitative defects in fibrinogen structure and function.
    • Bone abnormalities in inherited fibrinogen disorders are not well-documented.

    Observation:

    • Anatomico-radiologic study of humerus, femur, and tibia in total congenital afibrinogenemia revealed juxtatrabecular hemorrhages.
    • Hemorrhages primarily occurred in metaphyses, potentially linked to stress lines, leading to intraosseous cysts and trabecular remodeling.
    • Radiologic lesions in congenital dysfibrinogenemia showed femoral trabecular remodeling and resembled hemophilia-related pseudotumors.

    Findings:

    • Juxtatrabecular hemorrhages in congenital afibrinogenemia can cause significant bone remodeling and cyst formation.
    • Bone alterations in congenital dysfibrinogenemia share similarities with both afibrinogenemia and hemophilic bone lesions.
    • The study highlights a potential link between fibrinogen disorders and specific skeletal manifestations.

    Implications:

    • Extended skeletal studies (radiologic and anatomic) are crucial for understanding bone manifestations in congenital clotting defects, particularly fibrinogen disorders.
    • Investigating hemostatic disorders, especially at the fibrinogen level, in patients with bone cysts, infarcts, or unexplained remodeling is warranted.
    • This research suggests a need for increased awareness of skeletal complications in patients with inherited disorders of the fibrinogen molecule.

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