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Lipodystrophie

Mechanismen, Klinik, Therapie

Lipodystrophy

Mechanisms, clinical presentation, therapy

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Zusammenfassung

Lipodystrophiesyndrome sind eine heterogene Gruppe von Erkrankungen, die durch einen generalisierten oder partiellen Verlust subkutanen Fettgewebes gekennzeichnet sind. Gemeinsam ist allen Formen der Lipodystrophie ihre Assoziation mit metabolischen Entgleisungen wie Hypertriglyzeridämie, Insulinresistenz und Diabetes mellitus, welche ihrerseits zu teils lebensbedrohlichen Komplikationen führen können. Mit Ausnahme der HIV-assoziierten Lipodystrophie sind alle Lipodystrophieformen seltene Erkrankungen. Entsprechend existieren keine exakten epidemiologischen Daten zur Prävalenz, allerdings wird vermutet, dass insbesondere die partiellen Lipodystrophien häufig fehldiagnostiziert werden. Während der letzten Dekade konnten für einige der Lipodystrophiesyndrome zugrunde liegende genetische Ursachen gefunden werden. Die Behandlung der Lipodystrophie stellt eine Herausforderung dar, da häufig eine konventionelle lipidsenkende oder antihyperglykämische Therapie nicht ausreichend ist, um die schwere Stoffwechselentgleisung zu kontrollieren. In diesem Zusammenhang ist die Behandlung von Lipodystrophiepatienten mit rekombinantem Leptin eine Therapieoption, die in Europa nur an 5 Zentren angeboten wird, in Deutschland derzeit nur am Universitätsklinikum Leipzig.

Abstract

Lipodystrophy syndromes are a heterogenous group of congenital and acquired disorders with generalized or partial absence of subcutaneous adipose tissue. They are associated with severe metabolic abnormalities such as insulin resistance, diabetes mellitus, and hypertriglyceridemia that may result in life-threatening acute pancreatitis, steatohepatitis, and cardiovascular disease. Conventional lipid-lowering and antihyperglycemic medications may be insufficient to control severe metabolic abnormalities. The adipose tissue-derived hormone leptin has been investigated as a novel therapeutic option for severe lipodystrophy and significantly improves metabolic abnormalities in these patients. In Germany, leptin treatment for lipodystrophic patients with severe metabolic abnormalities is offered free of charge by the University Medicine Leipzig within a compassionate use program.

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  1. Department für Innere Medizin, Neurologie und Dermatologie, Klinik und Poliklinik für Endokrinologie und Nephrologie, Universitätsklinikum Leipzig, Liebigstraße 20, 04103, Leipzig, Deutschland

    K. Miehle, M. Stumvoll &  M. Fasshauer

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  1. K. Miehle
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  2. M. Stumvoll
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  3. M. Fasshauer
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Correspondence to M. Fasshauer.

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Miehle, K., Stumvoll, M. & Fasshauer, M. Lipodystrophie. Internist 52, 362–373 (2011). https://doi.org/10.1007/s00108-010-2711-5

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