Deep venous thrombosis in a child with nephrotic syndrome associated with a circulating anticoagulant and acquired protein s deficiency Journal Article


Authors: Garbrecht, F.; Gardner, S.; Johnson, V.; Grabowski, E.
Article Title: Deep venous thrombosis in a child with nephrotic syndrome associated with a circulating anticoagulant and acquired protein s deficiency
Abstract: Thromboembolic events occur with a frequency of 3–5% in children with nephrotic syndrome (NS). Although numerous abnormalities in all phases of coagulation have been described in NS, the pathogenesis of clotting abnormalities remains poorly understood in this group of patients. We describe a child with long-standing NS in whom a severe deep venous thrombosis and pulmonary embolism secondary to acquired protein S deficiency and a strong lupus-type circulating anticoagulant developed. In addition, this patient had a markedly decreased plasma level of C4b binding protein. Although acquired protein S deficiency has been described in various clinical disorders including NS, our patient is unusual in having C4bBP deficiency, and his is the only reported pediatric case of NS complicated by thromboembolism in which a circulating anticoagulant has been implicated, to our knowledge. © 1991 Raven Press, Ltd., New York.
Keywords: preschool child; case report; deep vein thrombosis; lung embolism; thromboembolism; warfarin; heparin; intraarterial drug administration; binding protein; protein deficiency; skin necrosis; nephrotic syndrome; intravenous drug administration; urokinase; oral drug administration; protein c; protein s; human; male; article; meprednisone; c4b binding protein; circulating anticoagulant; coumadin-induced skin necrosis; blood clotting inhibitor
Journal Title: American Journal of Pediatric Hematology/Oncology
Volume: 13
Issue: 3
ISSN: 0192-8562
Publisher: Wolters Kluwer Health, Inc  
Date Published: 1991-10-01
Start Page: 330
End Page: 333
Language: English
DOI: 10.1097/00043426-199123000-00015
PROVIDER: scopus
PUBMED: 1838904
DOI/URL:
Notes: Source: Scopus
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