Treatment of Monogenic Diabetes in Monogenic Lipodystrophy
When monogenic diabetes arises in the context of monogenic lipodystrophy, the underlying metabolic disruption — driven by structural adipose tissue loss rather than typical beta-cell or autoimmune pathology — shapes a distinct clinical picture and a specific treatment strategy.
Clinical Scenario
Diabetes or insulin resistance due to monogenic lipodystrophy, characterised by partial or complete reduction in adipose tissue, decreased adipokine levels, and hypertriglyceridemia.
Treatment Approach
Management centres on addressing insulin resistance directly. Insulin sensitizers represent a core part of the initial approach — though agent selection within this class requires careful evaluation, as specific agents carry important phenotype-related cautions in this population. The complete agent selection, sequencing, and clinical considerations are detailed in the structured protocol.
References
DOI: 10.1111/pedi.13426
Lipodystrophies are characterized by a partial or complete reduction in adipose tissue, which results in decreased adipokine levels and IR.
In partial lipodystrophy, insulin sensitizers such as metformin and glitazones may be initially effective but glitazones may exacerbate accumulation of ectopic fat in the face and neck.
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