Nh. Toumi et al., THROMBOSIS IN CONGENITAL DEFICIENCIES OF AT-III, PROTEIN-C OR PROTEIN-S - A STUDY OF 44 CHILDREN, HEM CELL TH, 39(6), 1997, pp. 295-299
Congenital deficiency in coagulation inhibitors is a cause of heredita
ry thrombotic disease. The severity of symptoms is variable and depend
s on the type of deficit. In this paper, 44 children suffering from de
ep venous thrombosis, with a mean age of 5 years, were studied. A sear
ch for Lupus anticoagulant (LA) and coagulation inhibitor deficiency s
howed: 3/44 cases (6.8%) had protein S deficiency, 2/44 cases (4.5%) h
ad protein C deficiency, 1/44 cases (2.3%) had deficiencies in both pr
otein C and S; no cases of AT III deficiency and LA was positive in 2/
44 cases (4.5%). Only 1 case of APC resistance out of 13 studied was f
ound. Four family studies were performed and confirmed the congenital
origin of the disorder.