Purpura fulminans in a case of protein C deficiency

Özsoylu, Şînasi; Cengiz, Betül; Karabent, A.

doi:10.1007/bf00442227

Eur J Pediatr

1988

DOI: 10.1007/bf00442227

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Purpura fulminans in a case of protein C deficiency

Şînasi Özsoylu

Betül Cengiz

A. Karabent

Abstract: We report a case of protein C deficiency which presented with purpura fulminans. The inheritance of protein C deficiency is discussed and the importance of warfarin (Coumadin) treatment in this conditions is emphasized.

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Cited by 4 publications

(1 citation statement)

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“…Clinical situations in which purpura fulminans is seen include homozygous protein C or protein S deficiency and acquired protein C or protein S deficiency (1). Homozygous protein C deficient patients usually present with extremely severe thrombotic complications during the neonatal period and purpura fulminans (2,3). Homozygous protein S deficiency is usually a less severe disease (4).…”

mentioning

confidence: 99%

Lupus anticoagulant and protein S deficiency in otherwise healthy children with acute varicella infection

Kurugöl

Vardar

Özkınay

et al. 2000

SPAE

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mentioning

confidence: 99%

Lupus anticoagulant and protein S deficiency in otherwise healthy children with acute varicella infection

Kurugöl

Vardar

Özkınay

et al. 2000

SPAE

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Protein C and S deficiency in severe infectious purpura of children: A collaborative study of 40 cases

et al. 1992

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We studied, in 40 children (mean age: 52 months) with severe infectious purpura, the relationships between protein C (PC) and protein S (PS) levels, and shock, disseminated intravascular coagulation (DIC) and outcome. We determined, on admission, PC antigen (ELISA) and activity (chromogenic test), and total PS (ELISA). Results were expressed as % of normal adult values. Statistical analysis was performed with SAS. Thirty children were in shock, 20 had DIC. All children with DIC, and 10 without DIC were in shock. Of 20 children who were in shock and had DIC, 7 died and 3 had an amputation. PC antigen was significantly decreased in shock children (p less than 0.05), in children with DIC (p less than 0.0005), and in non-survivors (p less than 0.05). PC activity was significantly decreased in shock children (p less than 0.05), in children with DIC (p less than 0.0005), and in non-survivors (p less than 0.005). Total PS was not decreased in shock children, but was significantly decreased in children with DIC (p less than 0.005), and in non-survivors (p less than 0.005). We conclude that PC and PS levels were decreased in our children, and that PC levels were significantly decreased in the presence of shock, DIC, and fatal outcome. PC and antithrombin III (AT III) supplementation, should be evaluated in children with severe infectious purpura with shock and DIC.

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Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children

Nguyên¹,

Reynaud²,

Pouzol

et al. 1994

Eur J Pediatr

107

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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.

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Purpura fulminans in a case of protein C deficiency

Abstract: We report a case of protein C deficiency which presented with purpura fulminans. The inheritance of protein C deficiency is discussed and the importance of warfarin (Coumadin) treatment in this conditions is emphasized.

Cited by 4 publications

References 9 publications

Lupus anticoagulant and protein S deficiency in otherwise healthy children with acute varicella infection

Lupus anticoagulant and protein S deficiency in otherwise healthy children with acute varicella infection

Protein C and S deficiency in severe infectious purpura of children: A collaborative study of 40 cases

Varicella and thrombotic complications associated with transient protein C and protein S deficiencies in children

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