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Updated: Aug 10, 2026
06:27
Analysis of β-Amyloid-induced Abnormalities on Fibrin Clot Structure by Spectroscopy and Scanning Electron Microscopy
Published on: November 30, 2018
An abnormal fibrinogen with delayed fibrinopeptide A release
British Journal of Haematology
|September 1, 1980
Summary
A family study identified seven individuals with prolonged thrombin clotting time, indicating a hereditary dysfibrinogenaemia. The defect involves a delayed release of fibrinopeptide A during fibrinogen conversion, now termed fibrinogen Manchester.
Area of Science:
- Hematology
- Clinical Biochemistry
- Genetics
Background:
- Hereditary dysfibrinogenaemia is a rare bleeding disorder characterized by abnormal fibrinogen function.
- Family history is crucial for identifying inherited coagulation factor defects.
Observation:
- A patient presenting with hematuria had family members across three generations with prolonged thrombin clotting time.
- Purified fibrinogen from affected individuals confirmed dysfibrinogenaemia with prolonged thrombin clotting time.
Findings:
- Standard electrophoretic and immunological methods did not reveal molecular abnormalities.
- Specific radioimmunoassay for fibrinopeptide A identified a defect in its release rate during thrombin-mediated fibrinogen conversion.
- This defect suggests a functional abnormality in fibrinogen conversion.
Implications:
- This study identifies a novel dysfibrinogenaemia, designated fibrinogen Manchester.
- Understanding the specific defect in fibrinopeptide A release provides insight into fibrin formation pathways.
- Further molecular characterization may elucidate the underlying genetic basis and inform diagnostic approaches.
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