| Autor: |
Lane DA, VanRoss M, Kakkar VV, Bottomley KJ, Dhir K, Holt LP, MacIver JE |
| Jazyk: |
angličtina |
| Zdroj: |
British journal of haematology [Br J Haematol] 1980 Sep; Vol. 46 (1), pp. 89-98. |
| DOI: |
10.1111/j.1365-2141.1980.tb05938.x |
| Abstrakt: |
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 electrophoretic 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: |
MEDLINE |
| Externí odkaz: |
|
Plný text