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Acta Haematol. 1992;88(4):194-7.

Severe congenital dysfibrinogenemia (fibrinogen-Riyadh): a family study.

Author information

1
Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Abstract

Congenital severe dysfibrinogenemia was discovered in a small Saudi family. Their single child exhibited abnormal severe bleeding tendency since birth and his coagulation profile revealed evidence of severe dysfibrinogenemia. The parents who were first-degree cousins and completely asymptomatic showed evidence of dysfibrinogenemia but to lesser degree than in their son. The child presented with large cephalohematoma and evidence of intracranial hemorrhage and left hemiparesis. He was treated with cryoprecipitate and his hematoma resolved, but his neurological deficit remained.

PMID:
1292308
DOI:
10.1159/000204685
[Indexed for MEDLINE]

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