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DOI: 10.1055/s-0038-1649292
Systemisch inflammatorische Erkrankungen als Differenzialdiagnose in der Neugeborenenperiode – Inflammasom-vermittelte Erkrankungen
Systemic inflammatory diseases as differential diagnosis in new born period Inflammasom-related diseasesPublication History
Publication Date:
27 April 2018 (online)
![](https://www.thieme-connect.de/media/10.1055-s-00034922/201802/lookinside/thumbnails/10-1055-s-0038-1649292-1.jpg)
Zusammenfassung
Autoinflammatorische Erkrankungen sind durch systemische oder organbezogene Entzündung gekennzeichnet, welche zumindest initial von fehlregulierten Mechanismen des angeborenen Immunsystems ausgehen. Einige monogene autoinflammatorische Erkrankungen manifestieren in der Perinatalperiode, sodass sie Differenzialdiagnosen zu Infektionen des Neugeborenen darstellen. Im vorliegenden Artikel werden Einblicke in die klinische Präsentation und die molekulare Pathophysiologie Inflammasom-vermittelter monogener Erkrankungen mit Manifestation im Neugeborenenalter gegeben.
Summary
Autoinflammatory conditions are characterized by systemic or organ specific inflammation which is at least initially caused by dysregulation of innate immune mechanisms. Several monogenic autoinflammatory conditions manifest in the perinatal period which makes them differential diagnoses to infections of the newborn. Here, an overview of the clinical presentation and molecular pathophysiology of inflammasome-associated monogenic disorders with manifestation in the newborn period will be given.
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