title: Fibrinogen Caracas I: a dysfibrinogenemia with a hemorrhagic diathesis associated with diminished fibrin fiber diameter and reduced fibrin gel porosity.
abstract: Fibrinogen Caracas I is a dysfibrinogenemia with a mild bleeding diathesis and a defective wound healing. We have characterized this abnormal fibrinogen using transmission electron microscopy (TEM) in combination with turbidity and permeation studies. Turbidometric and permeability analysis showed that the abnormal fibrin had a significantly decreased mass:length ratio and fiber diameter. In addition, the permeability studies of plasma fibrin clots showed that the gel porosity of the abnormal fibrinogen was reduced. Images of the abnormal fibrin structure obtained using TEM showed that the fibers were thinner, much less branched and less ordered than normal fibers. Diminished fibrin fiber diameter and reduced fibrin gel porosity have been taken as hallmarks of thrombophilic dysfibrinogenemias. The results of the present study show that these features are not necessarily predictive of thrombophilia. Further studies performed on a larger number of dysfibrinogenemias need to be conducted in order to establish the implications of these parameters on the clinical outcome.
authors.affiliation: Instituto Venezolano de Investigaciones Científicas, Caracas, Venezuela.
authors.forename: R
authors.initials: R
authors.lastname: Marchi
authors.forename: U
authors.initials: U
authors.lastname: Lundberg
authors.forename: N B
authors.initials: NB
authors.lastname: de Bosch
authors.forename: C L
authors.initials: CL
authors.lastname: Arocha-Piñango
mesh_terms: D020147:Coagulation Protein Disorders / Q000235:genetics / Q000473:pathology*; D005260:Female; D005337:Fibrin / Q000648:ultrastructure; D015241:Fibrinogens, Abnormal / Q000737:chemistry* / Q000235:genetics / Q000648:ultrastructure*; D006470:Hemorrhage / Q000235:genetics; D006801:Humans; D016018:Least-Squares Analysis; D008854:Microscopy, Electron; D016062:Porosity; D014945:Wound Healing / Q000235:genetics
chemical_list: D015241:Fibrinogens, Abnormal; D005337:Fibrin