Neonatal purpura fulminans due to homozygous protein C deficiency.
Severe and recurrent purpura fulminans developed in a Turkish boy at 1 week of age. Initial coagulation studies performed were compatible with disseminated intravascular coagulation. Subsequent investigations showed that the patient had homozygous and his healthy parents had heterozygous protein C deficiency. The episodes of purpura fulminans were controlled by infusions of fresh frozen plasma and heparinization. Oral anticoagulant therapy was given in the symptom-free period.
Ezer
U
U
Misirlioglu
E D
ED
Colba
V
V
Ogoz
E
E
Kurt
C
C
D005192:Family Health; D030342:Genetic Diseases, Inborn / Q000235:genetics / Q000473:pathology / Q000628:therapy; D006720:Homozygote; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D007231:Infant, Newborn; D008297:Male; D020151:Protein C Deficiency / Q000150:complications* / Q000235:genetics; D014421:Turkey