Protein C deficiency. South Med J 1987 Feb;80(2):240-2
Date
02/01/1987Pubmed ID
3810223DOI
10.1097/00007611-198702000-00025Scopus ID
2-s2.0-0023293528 (requires institutional sign-in at Scopus site) 18 CitationsAbstract
Severe homozygous protein C deficiency is a rare but serious problem in the newborn, with a clinical presentation of purpura fulminans. We have presented such a case in an 1,870 gm female neonate. Salient clinical findings in this case include DIC associated with extensive ecchymosis and subsequent gangrene of the skin, thrombotic complications that began on the third day of life. There was no precipitating infection. The progressive gangrenous necrosis of heel and toes was refractory to heparin therapy, but there was clinical improvement after treatment with fresh frozen plasma. Our patient's level of protein C antigen was less than 3% (normal 70% to 130%). Levels of other vitamin-K-dependent factors, as well as factor V, factor VIIIC, and antithrombin III were normal. A heterozygous protein C deficiency was documented in the mother and father. Presently the child is receiving warfarin sodium (Coumadin) therapy and is clinically stable.
Author List
Rappaport ES, Speights VO, Helbert B, Trowbridge A, Koops B, Montgomery RR, Marlar RAAuthor
Robert R. Montgomery MD Adjunct Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentDiagnosis, Differential
Female
Genetic Carrier Screening
Humans
Infant, Newborn
Male
Protein C
Protein C Deficiency
Prothrombin Time
Purpura
