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Ein europäisches Register für sepsisassoziierte Purpura fulminans (SAPFIRE)

Sepsis-associated Purpura Fulminans International Registry—Europe (SAPFIRE)

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Zusammenfassung

Hintergrund

Purpura fulminans ist eine seltene akut lebensbedrohliche Erkrankung, die durch eine disseminierte Thrombose in der Mikrozirkulation, kutane Hämorrhagien mit fortschreitender Nekrotisierung sowie multiplem Organversagen gekennzeichnet ist. Der Zusammenbruch des intrinsischen Antikoagulationssystems beruht wesentlich auf einem Protein-C-Mangel. In der Mehrheit der Fälle handelt es sich um eine sepsisassoziierte konsumptive Koagulopathie.

Fragestellung

Es gibt keine verlässlichen Daten zur Epidemiologie und keine evidenzbasierten Leitlinien für das Management von sepsisassoziierter Purpura fulminans (SAPF). Obwohl die Wiederherstellung des Gleichgewichts der Koagulation ein primäres Behandlungsziel ist, existieren keine randomisierten klinischen Studien zu dieser Fragestellung. Auch die Rolle unterschiedlicher mikrobieller Auslöser in der Entstehung von SAPF bedarf einer detaillierten Untersuchung.

Methoden

Das europaweite Sepsis-associated Purpura Fulminans International Registry (SAPFIRE) startet im ersten Quartal des Jahrs 2015. Die teilnehmenden klinischen Zentren werden erstmals systematisch Daten zur Ätiologie, zum klinischen Verlauf, zu Biomarkern, zur Therapie sowie zu Morbidität und Mortalität erfassen.

Ergebnisse

Die SAPFIRE-Ergebnisse werden periodisch ausgewertet. Zentren, die Daten zum Register beisteuern, erhalten durch retrospektive Auswertungen der eigenen Daten und durch regelmäßige Vergleiche mit den aggregierten und pseudonymisierten Daten der anderen teilnehmenden Zentren die Möglichkeit zur Etablierung eines Qualitätsmonitorings.

Abstract

Background

Purpura fulminans is a rare life-threatening condition which is characterized by disseminated thrombosis in dermal and systemic microcirculation, cutaneous hemorrhages with progressing necrosis and multiple organ failure. The underlying pathogenesis is based on the disruption of the intrinsic anticoagulation cascade, with protein C deficiency being considered the leading factor in this process. In the majority of cases, the condition emerges as consumptive coagulopathy associated with severe sepsis.

Objectives

Epidemiological data on sepsis-associated purpura fulminans (SAPF) are scarce and evidence-based treatment guidelines have not been established yet. While restoration of the balance in the coagulation cascade is a declared therapeutic goal, evaluations of the efficacy of different therapeutic approaches in randomized clinical trials are still lacking. The causal role of individual microbial pathogens also requires comprehensive evaluation.

Methods

A prospective multicenter Sepsis-Associated Purpura Fulminans International Registry—Europe (SAPFIRE) will be established in the first quarter of 2015. For the first time, participating centers will systematically collect information on etiology, clinical course, biomarkers, treatment, morbidity, and mortality of SAPF.

Results

The SAPFIRE data will be periodically evaluated and disseminated. Retrospective analysis of each center’s data and regular access to aggregated information collected by other centers will enable the participants to monitor and update care quality standards.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. M. Brunkhorst und V. Patchev geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Center of Sepsis Control and Care (CSCC), Klinik für Anaesthesiologie und Intensivtherapie, Universitätsklinikum Jena, Salvador-Allende-Platz 29, 07747, Jena, Deutschland

    F.M. Brunkhorst

  2. Integriertes Forschungs- und Behandlungszentrum“Sepsis und Sepsisfolgen“, Universitätsklinikum Jena, Jena, Deutschland

    F.M. Brunkhorst

  3. Paul-Martini-Forschergruppe Klinische Sepsisforschung, Universitätsklinikum Jena, Jena, Deutschland

    F.M. Brunkhorst

  4. Zentrum für Klinische Studien (ZKS), Universitätsklinikum Jena, Jena, Deutschland

    F.M. Brunkhorst & V. Patchev

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  1. F.M. Brunkhorst
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  2. V. Patchev
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Correspondence to F.M. Brunkhorst.

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Brunkhorst, F., Patchev, V. Ein europäisches Register für sepsisassoziierte Purpura fulminans (SAPFIRE). Med Klin Intensivmed Notfmed 109, 591–595 (2014). https://doi.org/10.1007/s00063-014-0402-z

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