Familial protein S deficiency presenting as deep vein thrombosis occurring during pregnancy.
Subjects with congenital deficiency of protein S, a natural anticoagulant which serves as a co-factor for the antithrombotic activity of activated protein C, are at risk of thrombosis. We describe a family in which deep vein thrombosis-occurring during pregnancy in three members with abnormally low protein S activity - was the only manifestation of congenital heterozygous protein S deficiency. Early diagnosis and active treatment of protein S deficiency should be pursued in any woman with a family history of thrombosis, who is planning pregnancy.
D'Angelo
A
A
Servizio di Emostasi e Trombosi, IRCCS H. S. Raffaele, Milan, Italy.
Mari
G
G
Della Valle
P
P
Marassi
A
A
D'Angelo
S V
SV
D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006023:Glycoproteins / Q000172:deficiency* / Q000235:genetics; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis / Q000209:etiology*; D017293:Protein S; D013924:Thrombophlebitis / Q000209:etiology*
D006023:Glycoproteins; D017293:Protein S