Deep venous thrombosis in a child with nephrotic syndrome associated with a circulating anticoagulant and acquired protein S deficiency.
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.
Garbrecht
F
F
Department of Pediatrics, Memorial Sloan, Kettering Cancer Center, New York, New York.
Gardner
S
S
Johnson
V
V
Grabowski
E
E
D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D002352:Carrier Proteins / Q000032:analysis; D002648:Child; D003169:Complement Inactivator Proteins; D003875:Drug Eruptions / Q000209:etiology / Q000473:pathology; D004359:Drug Therapy, Combination; D015433:Glomerulonephritis, Membranous / Q000150:complications; D006023:Glycoproteins / Q000172:deficiency*; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000032:analysis*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009336:Necrosis; D009404:Nephrotic Syndrome / Q000097:blood / Q000209:etiology*; D010743:Phospholipids / Q000276:immunology*; D011241:Prednisone / Q000627:therapeutic use; D017293:Protein S; D011655:Pulmonary Embolism / Q000209:etiology; D015912:Thrombolytic Therapy; D013924:Thrombophlebitis / Q000097:blood / Q000209:etiology*; D014568:Urokinase-Type Plasminogen Activator / Q000627:therapeutic use; D014859:Warfarin / Q000009:adverse effects / Q000627:therapeutic use
D002352:Carrier Proteins; D003169:Complement Inactivator Proteins; D006023:Glycoproteins; D016682:Lupus Coagulation Inhibitor; D010743:Phospholipids; D017293:Protein S; D014859:Warfarin; D006493:Heparin; D014568:Urokinase-Type Plasminogen Activator; D011241:Prednisone; D008775:Methylprednisolone