Homozygous protein S deficiency in an infant with purpura fulminans

Mahasandana, Chularatana; Suvatte, Vinai; Chuansumrit, Ampaiwan; Mariar, Richard A.; Manco‐Johnson, Marilyn J.; Jacobson, Linda; Hathaway, William E.

doi:10.1016/s0022-3476(05)83335-9

Cited by 98 publications

(47 citation statements)

References 13 publications

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“…436,437 Plasma levels of protein C achieved with these doses of FFP vary from 15% to 32% at 30 min after the infusion and from 4% to 10% at 12 h. 426 Plasma levels of protein S (which is entirely bound to C4b) are 23% at 2 h and 14% at 24 h, with an approximate half-life of 36 h. 438,439 Doses of protein C concentrate have ranged from 20 to 60 units/kg. In one study, a dose of 60 units/kg resulted in peak protein C levels of .…”

Section: Dvt and Pe In Childrenmentioning

confidence: 99%

Antithrombotic Therapy in Neonates and Children

Monagle

Chan

Goldenberg

et al. 2012

Chest

1,235

559

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Section: Dvt and Pe In Childrenmentioning

confidence: 99%

Antithrombotic Therapy in Neonates and Children

Monagle

Chan

Goldenberg

et al. 2012

Chest

1,235

559

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“…Protein S: Homozygous protein S deficiency also presents at birth with purpura fulminans and with unmeasurable plasma concentrations of protein S. 72,73,76,[92][93][94][95][96][97] Because there is no protein S concentrate available, FFP is the initial therapy of choice. 72 Further management strategies are similar for homozygous protein C deficiency.…”

Section: Homozygous Prothrombotic Disordersmentioning

confidence: 99%

Thromboembolic Disease and Antithrombotic Therapy in Newborns

Andrew¹,

Monagle²,

deVeber³

et al. 2001

Hematology

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This update uses an evidence based approach to analyze and present the epidemiology of neonatal thrombosis, etiologies, currently used techniques for diagnosis with their limitations, and current therapeutic approaches. In addition, the approaches to both prevention and optimal therapies are discussed.In Section I Dr. Paul Monagle addresses the epidemiology of neonatal thrombosis outside of the central nervous system in both arterial and venous locations, and those that occur in utero. The specific contributions of catheters and congenital prothrombotic disorders are delineated. Dr. Monagle also describes currently used techniques for the diagnosis of thrombotic events as well as their limitations and the current therapeutic approaches.In Section II, Dr. Gabrielle deVeber reviews the epidemiology of neonatal thrombosis within the central nervous system, in both arterial and venous locations and those that occur in utero. The neurological presentation, risk factors including congenital prothrombotic disorders, anatomical distribution, diagnostic tests, use of antithrombotic therapy and neurologic outcome of neonates with either sinovenous thrombosis or arterial ischemic stroke are discussed.In Section III, Dr. Anthony Chan reviews the current approaches to the prevention and treatment of neonatal thrombosis. Information on the differences in the response of neonates compared to adults to antithrombotic therapy and new approaches to the prevention and treatment of thrombosis in neonates are emphasized.Thromboembolic events (TEs) during childhood are increasing in their frequency and severity, and occur in children surviving previously life-threatening primary diseases. Newborns comprise the largest group of children developing TEs. This paper discusses the epidemiology, diagnostic tests, acquired and congenital prothrombotic risk factors, long-term outcomes, and antithrombotic therapy for the management of TEs in newborns. Due to the unique features and the significance of TEs in the central nervous system (CNS), sinovenous thrombosis (SVT) and arterial ischemic stroke (AIS) will be considered separately; cerebrovascular lesions in premature infants will not be discussed.In preparation for this manuscript, comprehensive Medline reviews were performed to identify all relevant publications, which were analyzed based upon the study design; results of studies with stronger designs were given more influence in the analysis. The striking features of this review were the deficiencies in our knowledge with respect to appropriate diagnostic strategies, optimal therapy for newborns with TEs, and the contribution of congenital prothrombotic disorders. Well-designed trials are required to improve our ability to care for neonates with TEs.

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“…1 a vitamin K-dependent protein, has an important role in the natural coagulation system, as shown by the occurrence of severe thrombotic complications in neonates with homozygous PS deficiency (1,2 ). These complications are similar to those observed in homozygous protein C deficiencies (3 ).…”

mentioning

confidence: 91%

Cleaved Protein S (PS), Total PS, Free PS, and Activated Protein C Cofactor Activity as Risk Factors for Venous Thromboembolism

Borgel¹,

Reny

Fischelis³

et al. 2003

Clinical Chemistry

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Background: Although hereditary protein S (PS) deficiency is clearly associated with venous thromboembolism (VTE), the importance of low PS concentrations as a risk factor for VTE in other patients is still a matter of debate. To clarify this issue, we designed a case-control study to evaluate the role of different molecular forms of plasma PS. Methods: We quantified plasma cleaved, total, and free PS and activated protein C (APC) cofactor activity in 87 VTE patients and 174 controls matched for age, sex, and hormonal treatment. Free PS was measured by ELISA or by enzyme-linked ligand sorbent assay (ELSA). Cleaved and total PS were measured by ELISA. Results: In controls, the mean (SD) concentration of circulating cleaved PS was 39 (14) nmol/L, corresponding to 10% (3.5%) of total PS. Concentrations of cleaved PS and total PS were not significantly different in patients with VTE compared with controls. However, in our population, low free PS measured by ELISA or ELSA, as well as APC cofactor activity values were significantly associated with VTE with odds ratios (95% confidence intervals) of 2.9 (1.3-6.3), 2.5 (1.1-5.6), and 2.9 (1.3-6.4), respectively, in multivariate analyses. Conclusion: Phenotypic low PS detected by APC cofactor activity assay or by an assay specific for free PS should be considered a risk factor for VTE.

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Homozygous protein S deficiency in an infant with purpura fulminans

Cited by 98 publications

References 13 publications

Antithrombotic Therapy in Neonates and Children

Antithrombotic Therapy in Neonates and Children

Thromboembolic Disease and Antithrombotic Therapy in Newborns

Cleaved Protein S (PS), Total PS, Free PS, and Activated Protein C Cofactor Activity as Risk Factors for Venous Thromboembolism

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