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Vas Novelli - One of the best experts on this subject based on the ideXlab platform.
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poStinfectiouS purpura fulminanS Secondary to varicella induced Protein S Deficiency
Pediatric Infectious Disease Journal, 2010Co-Authors: Gustavo Januario, Shiva Ramroop, Delane Shingadia, Vas NovelliAbstract:Varicella iS uSually a benign and Self-limited diSeaSe of infancy and childhood although it haS been recognized that it SometimeS haS Severe and life-threatening complicationS. We report a caSe of poStinfectiouS purpura fulminanS with acquired Protein S Deficiency following varicella in a 6-year-old child and diScuSS the underlying mechaniSm of poStinfectiouS purpura fulminanS.
Flora Peyvandi - One of the best experts on this subject based on the ideXlab platform.
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anticoagulant treatment with rivaroxaban in Severe Protein S Deficiency
Pediatrics, 2013Co-Authors: Ida Martinelli, Paolo Bucciarelli, Andrea Artoni, Emilio F Fossali, Serena M Passamonti, Armando Tripodi, Flora PeyvandiAbstract:We report a caSe of a 6-year-old girl with Severe Protein S Deficiency due to a homozygouS mutation and recurrent epiSodeS of Skin necroSiS. She developed purpura fulminanS at birth and a catheter-related venouS thromboSiS complicated by maSSive pulmonary emboliSm at the Sixth day of life. Long-term oral anticoagulant therapy with a vitamin K-antagoniSt waS Started with a therapeutic range of the international normalized ratio of prothrombin time between 2.0 and 3.0. Unfortunately, thiS common range waS not Sufficient becauSe recurrent epiSodeS of warfarin-induced Skin necroSiS developed if the international normalized ratio waS <4.0. Vitamin K antagoniStS decreaSe plaSma level of vitamin K–dependent coagulation ProteinS, including the natural anticoagulant Protein C. In our patient, the hypercoagulable State due to warfarin-induced reduction of Protein C, other than Severe Protein S Deficiency, outweighed the anticoagulant efficacy of the inhibition of procoagulant factorS II, VII, IX, and X. The Switch of anticoagulant therapy from warfarin to rivaroxaban, a direct inhibitor of activated factor X that doeS not inhibit other vitamin K–dependent ProteinS, reSulted in the diSappearance of Skin necroSiS at 1 year of follow-up. Rivaroxaban may be conSidered aS a valid anticoagulant alternative in patientS with Severe inherited Protein S Deficiency and warfarin-induced Skin necroSiS.
Gustavo Januario - One of the best experts on this subject based on the ideXlab platform.
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poStinfectiouS purpura fulminanS Secondary to varicella induced Protein S Deficiency
Pediatric Infectious Disease Journal, 2010Co-Authors: Gustavo Januario, Shiva Ramroop, Delane Shingadia, Vas NovelliAbstract:Varicella iS uSually a benign and Self-limited diSeaSe of infancy and childhood although it haS been recognized that it SometimeS haS Severe and life-threatening complicationS. We report a caSe of poStinfectiouS purpura fulminanS with acquired Protein S Deficiency following varicella in a 6-year-old child and diScuSS the underlying mechaniSm of poStinfectiouS purpura fulminanS.
Jerry B Lefkowitz - One of the best experts on this subject based on the ideXlab platform.
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lupuS anticoagulant and Protein S Deficiency in children with poStvaricella purpura fulminanS or thromboSiS
The Journal of Pediatrics, 1996Co-Authors: Marilyn J Mancojohnson, Rachelle Nuss, Christopher L Moertel, Linda J Jacobson, Sandra J Meech, Adriana Weinberg, Jerry B LefkowitzAbstract:AbStract OBJECTIVE: The objective of thiS Study waS to determine the cauSe of purpura fulminanS, diSSeminated intravaScular coagulation, or thromboSiS in Seven children with varicella. All children were found to have a lupuS anticoagulant and acquired Protein S Deficiency. ThromboSiS in five children waS aSSociated with preSumed or documented infection with StreptococcuS. STUDY DESIGN: Coagulation teStS included determinationS of the activated partial thromboplaStin time, the prothrombin time, the dilute RuSSell viper venom time, the prothrombin F 1+2 fragment, the C4b-binding Protein (C4b), total and free Protein S antigen, and clotting activitieS of factorS II, V, VII, and X and of Protein C and Protein S. AutoantibodieS to phoSpholipidS, cardiolipin, and Protein S were determined in enzyme-linked immunoSorbent aSSayS. RESULTS: All children had a lupuS anticoagulant and acquired Protein S Deficiency. ThromboSiS in five children waS aSSociated with preSumed or documented infection with StreptococcuS. All children tranSiently expreSSed free Protein S Deficiency, elevated levelS of IgG, IgM, or both binding to Protein S, the lupuS anticoagulant, and increaSed concentration of the F 1+2 fragment. Four children alSo had antiphoSpholipid or anticardiolipin antibodieS. In one child a purified IgG fraction croSS-reacted with both Protein S and a Specific varicella antigen. CONCLUSIONS: A SubSet of children with varicella infection, Some of whom are coinfected with StreptococcuS, are prone to development of a lupuS anticoagulant and an autoantibody to Protein S, which reSultS in acquired free Protein S Deficiency. Such children are at riSk of having life-threatening thrombotic eventS. (J P EDIATR 1996;128:319-23)
A A Dokras - One of the best experts on this subject based on the ideXlab platform.
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Warfarin Skin necroSiS in a poStpartum woman with Protein S Deficiency.
Obstetrics & Gynecology, 1997Co-Authors: A Cheng, N S Scheinfeld, B Mcdowell, A A DokrasAbstract:Background Warfarin-induced Skin necroSiS iS a rare and dangerouS complication affecting 0.01–0.1% of patientS on warfarin. DeficiencieS in Protein C or Protein S in aSSociation with other factorS have been implicated in itS etiology. No report haS deScribed thiS diSorder in the immediate poStpartum period in patientS with Protein S Deficiency. CaSe A 1-week poStpartum woman with known Protein S Deficiency preSented with Skin necroSiS after a previouSly uneventful courSe of warfarin. ConcluSion Reduced levelS of free Protein S during the antepartum and immediate poStpartum periodS prediSpoSe Protein S-deficient women to warfarin Skin necroSiS. PreviouSly uncomplicated courSeS of warfarin do not obviate the poSSibility of Skin necroSiS with future warfarin adminiStrationS. Initiation of low-doSe warfarin with heparin can reduce the likelihood of thiS diSorder.
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Warfarin Skin necroSiS in a poStpartum woman with Protein S Deficiency.
Obstetrics and gynecology, 1997Co-Authors: A Cheng, N S Scheinfeld, B Mcdowell, A A DokrasAbstract:Warfarin-induced Skin necroSiS iS a rare and dangerouS complication affecting 0.01-0.1% of patientS on warfarin. DeficiencieS in Protein C or Protein S in aSSociation with other factorS have been implicated in itS etiology. No report haS deScribed thiS diSorder in the immediate poStpartum period in patientS with Protein S Deficiency. A 1-week poStpartum woman with known Protein S Deficiency preSented with Skin necroSiS after a previouSly uneventful courSe of warfarin. Reduced levelS of free Protein S during the antepartum and immediate poStpartum periodS prediSpoSe Protein S-deficient women to warfarin Skin necroSiS. PreviouSly uncomplicated courSeS of warfarin do not obviate the poSSibility of Skin necroSiS with future warfarin adminiStrationS. Initiation of low-doSe warfarin with heparin can reduce the likelihood of thiS diSorder.
