Management of neonatal purpura fulminans with severe protein C deficiency.
Neonatal purpura fulminans is a life threatening clinical entity characterized by extensive subcutaneous thrombosis and disseminated intravascular coagulation usually manifesting shortly after birth. We report an autosomal recessive form of the disease in a neonate who was diagnosed with compound heterozygosity for mutations in his protein C gene as the molecular basis of his disorder.
Sen
Krishna
K
Department of Pediatric Medicine, Park Childrens Center for Treatment and Research, 4 Gorky Terrace, Kolkata 700 019, India. dockrishnasen@gmail.com
Roy
Angshumoy
A
D000925:Anticoagulants / Q000627:therapeutic use*; D006801:Humans; D011695:IgA Vasculitis / Q000150:complications / Q000188:drug therapy*; D007231:Infant, Newborn; D008297:Male; D009154:Mutation; D011486:Protein C / Q000627:therapeutic use*; D020151:Protein C Deficiency / Q000150:complications* / Q000235:genetics; D018570:Risk Assessment; D019851:Thrombophilia / Q000209:etiology*
D000925:Anticoagulants; D011486:Protein C