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Klin Padiatr 2002; 214(3): 99-103
DOI: 10.1055/s-2002-30142
Übersicht

© Georg Thieme Verlag Stuttgart · New York

Lipodystrophien

Übersicht, Stand der Forschung, Klassifikation, neue TherapienLipodystrophiesPresent scientific knowledge, classification, new therapies - An OverviewPamela  Fischer-Posovszky1 , K.-M.  Debatin1 , M.  Wabitsch1
  • 1Universitätskinderklinik und Poliklinik, Ulm
Further Information

Publication History

Publication Date:
16 May 2002 (online)

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Zusammenfassung

Lipodystrophien sind sehr seltene Krankheiten, charakterisiert durch das FRehlen von Fettgbewebe. Der Verlust von Fettgewebe kann genetische oder immunologische Gründe haben, aber auch verursacht sein durch Infektionen oder Gabe bestimmter Medikamente. Das Ausmaß an metabolischen Komplikationen wie Insulinresistenz, Diabetes mellitus, Hypertriglyzeridämie und Fettleber steigt an mit Ausmaß des Fettverlusts.

Lipodystrophien können in zwei Gruppen unterteilt werden, die familiären und die erworbenen Formen. Sowohl für eine familiäre, als auch für eine erworbene Form konnten erst kürzlich ursächliche Mutationen gefunden werden. Im Moment helfen Mausmodelle, diese Syndrome besser verstehen zu lernen.

In dieser Übersichtsarbeit wird die neue Klassifizierung der Lipodystrophie-Syndrome vorgestellt, zudem werden aktuelle Forschungsergebnisse über die Pathogenese und Therapiemöglichkeiten zusammengefasst.

Abstract

The lipodystrophy syndromes are rare disorders characterized by the loss of adipose tissue. The loss of fat tissue can have genetic, immune, or infectious/drug-associated causes. With the extent of fat loss metabolic complications, such as insulin resistance, diabetes mellitus, hypertriglyceridemia, and fatty liver increase in severity. Lipodystrophies can be divided into two subtypes: familial and acquired. Causative mutations have recently been identified in one form of familial lipodystrophy as well as in one form of acquired lipodystrophy. Several mouse models might help understanding the development of these syndroms.

In this review article, the recently introduced classification of lipodystrophy syndromes is presented as well as new insights into pathogenesis and therapeutic strategies.

Schlüsselwörter

Lipodystrophie - Adipozyt - Fettzelldifferenzierung

Key words

Lipodystrophy - adipocyte - adipose differentiation

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PD Dr. med. M. Wabitsch

Universitätsklinik u. Poliklinik, Ulm

Prittwitzstr. 43

89075 Ulm

Email: martin.wabitsch@medizin.uni-ulm.de