Blood, 1953, Vol. 8, No. 8, pp. 679-686.
© 1953 American Society of Hematology, Inc.
Studies on the Metabolism of Fibrinogen in Two Patients
with Congenital Afibrinogenemia
DAVID GITLIN M.D.1 and
WAYNE H. BORGES M.D.1
1 Department of Pediatrics, Harvard Medical School, and the Children's Medical Center, Boston, Mass.
1. The rate of disappearance from the plasma of intravenously administered
fibrinogen has been studied immunochemically in two children with congenital
afibrinogenemia.
2. About half of the administered fibrinogen disappeared from the circulation
in the first forty-eight hours, thus emphasizing the importance of the extravascular pool of plasma protein.
3. After the first two days, the fibrinogen followed a logarithmic decay curve,
with a half life of four days; this is so close to the half life of 5.6 days estimated
from radioisotope turnover studies that it indicates that the fundamental defect
in these patients is a failure to synthesize adequate quantities of fibrinogen.
4. Traces of fibrinogen were detected immunochemically in the plasma of each
patient; in one of them, the small amount found could not readily be attributed
to prior infusion of blood or blood products.
5. The connective tissue in biopsy specimens of skin and muscle from one
patient, when examined with fluorescein-labelled antisera, was found to be specifically deficient in fibrinogen. This deficit was quickly rectified by intravenous
administration of fibrinogen.
Submitted on December 10, 1952
Accepted on January 31, 1953
