Lipodystrophie – Neue Erkenntnisse und Therapien

Zusammenfassung

Unter dem Begriff Lipodystrophie wird eine Gruppe angeborener und erworbener Erkrankungen subsummiert, denen gemeinsam der Verlust an subkutanem Fettgewebe ist. Dieser Fettgewebsverlust kann den gesamten Körper (generalisierte Lipodystrophie) oder nur Teile davon (partielle Lipodystrophie) betreffen. Durch das fehlende Fettgewebe kommt es zu einer Dysbalance adipozytensezernierter Faktoren, wobei insbesondere der Minderproduktion von Adiponektin und Leptin eine wesentliche pathophysiologische Rolle zukommt. Es resultieren schwere Fett- und Glukosestoffwechselstörungen mit entsprechenden Folgeerkrankungen, welche die Patienten vital gefährden können. Oftmals ist unter konventioneller Therapie nur eine unzureichende Stoffwechselkontrolle möglich. Durch die rekombinante Herstellung von Leptin ist es gelungen, betroffene Patienten einer kausalen Therapie zuzuführen.

Summary

The term “lipodystrophy” summarizes a group of congenital or acquired diseases which share the characteristic of a loss in subcutaneous adipose tissue. Depending on the distribution of adipose tissue-loss, generalized and partial forms of lipodystrophy are distinguished. The absence of adipose tissue leads to an imbalance in adipose tissue-derived hormones, especially to a deficiency of adiponectin and leptin. This results in severe metabolic disturbances with consecutive lifethreatening complications. Conventional lipid- and glucose-lowering treatment strategies are often not successful in reaching metabolic targets. The development of recombinant human leptin provides a novel and causal therapeutic option for patients with lipodystrophy.

Schlüsselwörter

Diabetes mellitus - Hypertriglyzeridämie - Insulinresistenz - Leptin - Lipodystrophie

Keywords

Diabetes mellitus - hypertriglyceridemia - insulin resistance - leptin - lipodystrophy

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