Faculty Collaboration Database

Severe homozygous protein C deficiency. J Pediatr 1984 Sep;105(3):409-13

Date

09/01/1984

Pubmed ID

6547978

DOI

10.1016/s0022-3476(84)80013-x

Scopus ID

2-s2.0-0021233839 (requires institutional sign-in at Scopus site)   115 Citations

Abstract

An infant with recurrent purpura fulminans in the first year of life was found to have severe homozygous deficiency of protein C (less than 1% of normal levels). The episodes of purpura fulminans were controlled by infusions of fresh frozen plasma containing protein C. The requirement of frequent plasma infusions, however, eventually resulted in several complications secondary to hyperproteinemia. Factor IX concentrates rich in protein C were then given to maintain adequate levels of the factor while minimizing the amount of extraneous proteins. The patient has remained asymptomatic and free of complications for greater than 10 months while receiving these concentrates every 48 hours.

Author List

Sills RH, Marlar RA, Montgomery RR, Deshpande GN, Humbert JR

MESH terms used to index this publication - Major topics in bold

Blood Coagulation Factors
Blood Proteins
Factor IX
Female
Glycoproteins
Heparin
Humans
Infant, Newborn
Pedigree
Protein C
Protein Deficiency
Skin Diseases
Thrombosis