1/11. Lupus anticoagulant and protein s deficiency in a child who developed disseminated intravascular coagulation in association with varicella.Varicella is not always a benign disease it may cause serious complications. We report a two-year-old boy with disseminated intravascular coagulation in association with varicella. The patient had the lupus anticoagulant, the antiphospholipid antibody, acquired free protein s deficiency, and increased concentrations of the prothrombin F 1 2 fragment. Intravenous immunoglobulin was administered due to its potential antibody-blocking activity, and the patient responded well. We recommend that children with varicella and disseminated intravascular coagulation should be examined for the lupus anticoagulant, the free protein S antigen, the prothrombin fragment F 1 2 and the other coagulation parameters. Intravenous immunoglobulin administration could be useful in such conditions because of its antibody-blocking activity.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
2/11. purpura fulminans in a child as a complication of chickenpox infection.purpura fulminans is a thrombotic disease that can occur during infections, disseminated intravascular coagulation or in the context of an acquired or congenital protein C or S deficiency. Here we report the case of a 4-year-old child who developed, 5 days after a chickenpox infection, large painful ecchymotic, necrotizing and retiform plaques on the lower extremities. Laboratory analyses revealed very low protein S levels as well as anticardiolipin antibodies. Aggressive treatment by low-molecular-weight heparin, steroids, intravenous immunoglobulins and fresh frozen plasma was able to prevent the extension of the lesions and to correct the coagulation abnormalities. No lesions required skin grafting. As in our patient, an acquired protein s deficiency is probably responsible for most cases of purpura fulminans occurring after varicella, but the concomitant presence of antiphospholipid antibodies may also play a role.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
3/11. Anti-protein S antibodies following a varicella infection: detection, characterization and influence on thrombin generation.Postinfectious purpura fulminans is a rare disease. Varicella is one of the precipitating conditions and we recently observed such a case. The 4-year-old child was found to have a severe transient protein s deficiency. By enzyme-linked immunosorbent assay and surface plasmon resonance we first demonstrated that anti-protein S antibodies were present and also transient. Next we characterized the epitopes against which these antibodies were directed and found that they predominantly recognized the N-terminal part of protein S. Finally we showed by thrombography a transient dramatic hypercoagulable state as a result of thrombin being unregulated by the dynamic protein C inhibitory system: in vitro thrombin generation, in response to a low concentration of tissue factor, was almost insensitive to activated protein C up to 25 nmol L(-1) on day 4 while it was normally sensitive on day 42. For the first time, we demonstrated a temporal relationship between protein s deficiency, antibodies to protein S and hypercoagulability, thus supporting the pathogenic role of these antibodies.- - - - - - - - - - ranking = 0.66666666666667keywords = varicella (Clic here for more details about this article) |
4/11. Clinico-pathologic conference: an 11-year-old girl with lupus erythematosus, venous thromboses, and purpura fulminans.An 11 year old girl with known diagnosis of lupus erythematosus with nephritis was admitted with respiratory distress during an episode in which she had infection with varicella-zoster virus complicated by purpura fulminans. She had a downhill course, terminating with a pulmonary embolus.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
5/11. Postinfectious purpura fulminans caused by an autoantibody directed against protein S.OBJECTIVE: To determine the mechanism responsible for idiopathic purpura fulminans, we investigated the procoagulant and anticoagulant pathways in five consecutive patients, four after varicella, and the fifth after a nonspecific infection. methods: Procoagulant and anticoagulant factors, including protein C, protein S, and antithrombin iii, were measured by quantitative or functional assays. Anti-protein S autoantibodies were identified by dot blotting and Western blotting, and quantified serially by enzyme-linked immunosorbent assay. Clinical and laboratory data were collated retrospectively. RESULTS: In each case the disease began 7 to 10 days after the onset of the precipitating infection, with rapidly progressive purpura leading to extensive areas of skin necrosis. The illness was complicated by impaired perfusion of limbs or digits (two patients), peripheral gangrene resulting in an above-knee amputation (one patient), and major organ dysfunction caused by thromboembolic phenomena involving the lungs (two patients), the heart (one patient), or the kidneys (one patient). Protein S levels were virtually undetectable at the time of admission and failed to respond to infusions of fresh frozen plasma, despite correction of other procoagulant and anticoagulant factors. All five children had anti-protein S IgM and IgG autoantibodies, which persisted for less than 3 months after admission. Decline in the anti-protein S IgG antibody concentration was associated with normalization of the plasma protein S levels. CONCLUSIONS: Autoimmune protein s deficiency may be a common mechanism causing postinfectious idiopathic purpura fulminans. Recognition of the pathophysiologic mechanism may provide a rational basis for treatment. Immediate heparinization, infusions of fresh frozen plasma, and, in cases complicated by major vessel thrombosis, the use of tissue-type plasminogen activator may limit thromboembolic complications.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
6/11. Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children.We report six cases of protein s deficiency secondary to varicella. Five cases were complicated by thrombotic and vascular events, namely purpura fulminans and necrotic vasculitis, deep vein thrombosis and stroke. Two cases were associated with protein c deficiency and one case revealed a heterozygous factor xii deficiency. The underlying mechanism of this acquired protein s deficiency is unclear but could be related to a direct effect of zoster virus.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
7/11. Deep vein thrombosis in a 13-year-old boy with hereditary protein s deficiency and a review of the pediatric literature.We report a 13-year-old boy with hereditary deficiency of protein S, who developed a deep vein thrombosis of the lower limb after a varicella with severe cutaneous lesions. Hereditary protein s deficiency is an established cause of thrombophilia; however thrombotic events are seldom described in pediatric patients. A review of previous literature revealed 35 cases, 16 girls and 19 boys, with a first episode below of the age of 18 years old (x = 10y). The 57% of the patients had venous thrombosis, 20% arterial thrombosis, and 14% both and in 9% the type of thrombosis was not reported. Predisposing factors were referred in only 12 cases. The deficiencies can be classified as type I in 25 patients and type III in 8.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
8/11. Acquired free protein s deficiency associated with multiple myeloma: a case report.Investigation of recurrent venous thromboembolic events in a 46-year-old man with progressive IgG kappa (total serum IgG, 74.3 mg/ml) multiple myeloma revealed profound reductions in free protein S (PS) antigen (<0.l U/ml) and PS activity (0.33 U/ml). Total PS antigen, protein C, antithrombin iii, and C4b-binding protein levels were within normal limits. The patient had no family history suggestive of a congenital PS deficiency and no history of thrombosis predating the diagnosis of his plasma cell dyscrasia. Patient IgG was isolated from serum using a protein A-sepharose affinity column and characterized. PS-dependent clotting assays (Staclot Protein S, Diagnostica Stago, Asnieres sur-Seine, france) performed on normal pooled plasma mixed with dilutions of patient IgG (0.0-33.0 mg/ml) revealed a dose-dependent neutralization of PS activity by 43%. Total and free PS antigen levels were measured using Laurell rocket electroimmunodiffusion (Assera-Plate Protein S, Diagnostica Stago), which revealed a similar dose-dependent reduction in free PS antigen but preserved normal total PS antigen. Free PS antigen was reduced by 77% to 0.23 U/ml using an IgG concentration (16.5 mg/ml) less than one-fourth of that of the patient at time of serum collection. Specific binding of the patient IgG to commercially available purified human PS was demonstrated by Western immunoblot analysis. Whereas acquired free PS deficiency has been previously reported in association with nephrotic syndrome, inflammatory bowel disease, hiv infection, and varicella infection, this is the first reported case of a hypercoagulable syndrome associated with acquired free PS deficiency and multiple myeloma.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
9/11. purpura fulminans induced by disseminated intravascular coagulation following infection in 2 unrelated children with double heterozygosity for factor v Leiden and protein s deficiency.purpura fulminans is associated with homozygous protein C and homozygous protein S deficiency or may follow bacterial or viral infections. We present 2 children from 2 unrelated Arab families with purpura fulminans who were double heterozygotes for factor v Leiden inherited from their fathers and protein s deficiency inherited from their mothers. No previous thrombotic events have occurred in either patient or their respective family members. In one patient sepsis accompanied by disseminated intravascular coagulation appeared to be the trigger of purpura fulminans. In the other patient varicella infection preceded purpura fulminans and was also associated with disseminated intravascular coagulation. This report emphasizes the need for evaluation of hereditary defects in the inhibitory mechanisms of blood coagulation in patients with purpura fulminans at any age.- - - - - - - - - - ranking = 0.16666666666667keywords = varicella (Clic here for more details about this article) |
10/11. Spontaneous tibial artery thrombosis associated with varicella pneumonia and free protein s deficiency.pneumonia is the most common serious complication of varicella infection in adults. A variety of thrombotic complications including purpura fulminans and disseminated intravascular coagulation have been reported in children with varicella but not in adults. Two men with varicella pneumonia who had profound lower extremity ischemia caused by thrombosis of the profunda femoris and tibial arteries are reported. Both patients had free protein s deficiency and vascular thrombosis in association with varicella pneumonia without overt evidence of disseminated intravascular coagulation or purpura fulminans. Antiphospholipid immunoglobulin g and immunoglobulin m antibodies were present in one, whereas the other had evidence of the lupus anticoagulant. The proposed pathogenesis and management options including intraarterial thrombolytic therapy with urokinase and the need for long-term anticoagulation are discussed.- - - - - - - - - - ranking = 1.3333333333333keywords = varicella (Clic here for more details about this article) |
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