An Abnormal Fibrinogen with Delayed Fibrinopeptide A Release
| Autor: | L. P. J. Holt, David A. Lane, J. E. MacIver, Vijay V. Kakkar, M. VanRoss, J. Bottomley, K. Dhir |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
Male
medicine.medical_specialty Thrombin Time Fibrinopeptide A Abnormal fibrinogen Peptide Fibrinogen Fibrin Thrombin Internal medicine medicine Humans chemistry.chemical_classification biology business.industry Hematology Blood Coagulation Disorders Middle Aged Molecular Abnormality Pedigree Specific radioimmunoassay Endocrinology chemistry Immunology biology.protein Electrophoresis Polyacrylamide Gel Female Blood Coagulation Tests business circulatory and respiratory physiology medicine.drug |
| Zdroj: | British Journal of Haematology. 46:89-98 |
| ISSN: | 0007-1048 |
| Popis: | Summary. Investigation of the family of a patient presenting with haematuria revealed seven cases in three generations showing a prolonged thrombin clotting time. A dysfibrinogenaemia was confirmed when purified fibrinogen from an affected member of the family was shown to also exhibit a prolonged thrombin clotting time. No molecular abnormality could be demonstrated using electrophor-etic and immunological techniques. However, using a specific radioimmunoassay to fibrinopeptide A a major defect has been localized to a delay in the rate of release of this peptide by thrombin when the abnormal fibrinogen is converted to fibrin. This case of dysfibrinogenaemia has been tentatively designated fibrinogen Manchester. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Plný text