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Hämolytisch-urämisches Syndrom im Kindes- und Jugendalter

Hemolytic uremic syndrome in childhood and adolescence

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Der Nephrologe Aims and scope

Zusammenfassung

Das hämolytisch-urämische Syndrom (HUS) ist eine häufige Ursache des akuten, intrarenalen Nierenversagens im Kindesalter und durch die Trias aus mikroangiopathischer, hämolytischer Anämie, Thrombozytopenie und akuter Nierenfunktionseinschränkung charakterisiert. Pathophysiologisch entsteht ein HUS im Kindesalter meist durch eine Infektion mit shigatoxinproduzierenden E. coli (STEC-HUS), in der Regel enterohämorrhagischen E. coli (EHEC). Eine unkontrollierte Aktivierung des Komplementsystems, ausgelöst durch verschiedene Triggerfaktoren auf der Grundlage einer genetischen Prädisposition, spielt beim komplementvermittelten HUS eine entscheidende Rolle. Die Rolle des Komplementsystems bei anderen Formen des HUS (z. B. durch Infektionen insbesondere mit Pneumokokken, durch Stammzell- oder Organtransplantation) ist noch nicht abschließend geklärt. Die Therapie des HUS erfolgt als supportive Behandlung des akuten oder chronischen Nierenversagens. Bei einer bestehenden Dysregulation im Komplementsystem (ehemals atypisches HUS) stellt der C5-blockierende Antikörper Eculizumab eine spezifische therapeutische Möglichkeit dar, wodurch sich das Outcome der Erkrankung signifikant gebessert hat. Die Einteilung der Erkrankung sowie konkrete Empfehlungen bezüglich der Art und Dauer einer möglichen therapeutischen Komplementinhibition unterliegen häufigen Modifikationen aufgrund zahlreicher Erkenntnisgewinne aus prospektiven klinischen und experimentellen Studien.

Abstract

The hemolytic uremic syndrome (HUS) is a frequent cause of acute intrarenal kidney failure in childhood. It is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Pathophysiologically, the most common cause of HUS in childhood are shiga toxin-producing E. coli (STEC-HUS), generally enterohemorrhagic E. coli (EHEC). An uncontrolled activation of the complement system triggered by various factors in the background of an underlying genetic predisposition, plays a decisive role in complement-mediated HUS. The role of the complement system in other forms of HUS (e. g. associated with infections particularly with pneumococci, or with stem cell or solid organ transplantation) is presently unclear. The treatment of HUS includes mainly the supportive treatment of acute or chronic kidney failure. For patients with a dysregulation in the complement system (formerly atypical HUS) the C5 blocking antibody eculizumab is a specific treatment option, which has significantly improved the short and long-term outcome of these patients. The classification of HUS and final recommendations with respect to the type and duration of a possible therapeutic complement inhibition is subject to frequent modifications due to increasing knowledge gained from prospective clinical and experimental studies.

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Authors and Affiliations

  1. Klinik für Pädiatrie mit Schwerpunkt Gastroenterologie, Nephrologie und Stoffwechselmedizin, Charité – Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Deutschland

    J. Holle &  D. Müller

  2. Klinik für Pädiatrie mit Schwerpunkt Pneumologie, Immunologie und Intensivmedizin, Charité – Universitätsmedizin Berlin, Berlin, Deutschland

    J. Holle

  3. Zentrum für Kinder- und Jugendmedizin, Universitätsklinikum Freiburg, Medizinische Fakultät, Albert-Ludwigs-Universität Freiburg, Freiburg, Deutschland

    K. Häffner

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  1. J. Holle
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  2. D. Müller
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  3. K. Häffner
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Correspondence to D. Müller.

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Interessenkonflikt

J. Holle, D. Müller haben Reisesponsoring durch die Firma Alexion Pharma GmbH erhalten. K. Häffner hat Reisesponsoring und Forschungsförderung der Firma greenovation Biotech GmbH erhalten.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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B. Tönshoff, Heidelberg

L. T. Weber, Köln

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Holle, J., Müller, D. & Häffner, K. Hämolytisch-urämisches Syndrom im Kindes- und Jugendalter. Nephrologe 14, 176–183 (2019). https://doi.org/10.1007/s11560-019-0317-z

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