Heterozygous protein C deficiency presenting as severe protein C deficiency and peripartum thrombosis: successful treatment with protein C concentrate.
BACKGROUND
Complications of pregnancy, including thrombosis and increased fetal loss, have been described in women with heterozygous protein C deficiency.


METHODS
We report an unusual case of umbilical cord thrombosis, acute renal failure, and cutaneous venous thrombosis associated with severe protein C deficiency in the heterozygous infant of a normal mother. Repeated infusions of protein C concentrate made successful heparinization of the infant possible, with the subsequent resolution of cutaneous thrombosis and improvement of renal status.


CONCLUSIONS
Heterozygous protein C deficiency in the fetus may result in perinatal thrombosis, which can be treated with viral-inactivated protein C concentrate. Heterozygous protein C deficiency should be considered in cases of fetal compromise or death associated with umbilical vein thrombosis.
Goodwin
T M
TM
Department of Obstetrics and Gynecology, Women's and Children's Hospital, University of Southern California, Los Angeles, USA.
Gazit
G
G
Gordon
E M
EM
D000328:Adult; D005260:Female; D006579:Heterozygote; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011486:Protein C / Q000627:therapeutic use*; D020151:Protein C Deficiency; D012074:Remission Induction; D012720:Severity of Illness Index; D013927:Thrombosis / Q000188:drug therapy* / Q000209:etiology; D014471:Umbilical Veins
D011486:Protein C