title: Inherited protein C deficiency and coumarin-responsive chronic relapsing purpura fulminans in a newborn infant.
abstract: A coumarin-responsive chronic relapsing purpura fulminans syndrome is described in a protein-C-deficient newborn infant. Episodes of acute disseminated intravascular coagulation (DIC) and cutaneous gangrene, which first appeared at age 11 h, were effectively controlled for 28 months with transfusions of fresh-frozen plasma. Cryoprecipitate and cryoprecipitate-poor plasma induced remissions as long as those induced by fresh-frozen plasma (less than or equal to 72 h). Coumarins sustained a cryoprecipitate-induced remission for 19 days: they were then electively discontinued and 17 h later the patient had an acute exacerbation of DIC with haemorrhaging. Family studies showed protein C levels of 31-40% in the subject's symptom-free mother and full and half brothers. DIC, the coumarin effect, and the inherited protein C abnormality appear to have contributed to the extremely low plasma levels (less than or equal to 6%) of protein C in the patient. This experience suggests that protein C deficiency may greatly compromise the ability of newborn infants to control consumptive disorders.
mesh_terms: D002648:Child; D002908:Chronic Disease; D003374:Coumarins / Q000627:therapeutic use*; D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy / Q000209:etiology; D005340:Fibrinogen / Q000032:analysis; D005734:Gangrene / Q000188:drug therapy / Q000209:etiology; D006023:Glycoproteins / Q000172:deficiency*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications / Q000188:drug therapy*; D010375:Pedigree; D011486:Protein C; D011693:Purpura / Q000188:drug therapy* / Q000209:etiology; D012867:Skin / Q000473:pathology; D013577:Syndrome
chemical_list: D003374:Coumarins; D006023:Glycoproteins; D011486:Protein C; D005340:Fibrinogen