Treatment of Monogenic Diabetes in Monogenic Lipodystrophy

Monogenic lipodystrophy leads to partial or complete loss of adipose tissue, with consequent reductions in adipokine levels and marked insulin resistance — producing a diabetes phenotype that requires a tailored clinical approach distinct from common type 2 diabetes.

Clinical Scenario

Diabetes and insulin resistance arising from monogenic lipodystrophy, characterised by partial or complete reduction in adipose tissue, decreased adipokine levels, and hypertriglyceridemia. This constellation of findings defines a specific sub-population in whom standard diabetes management strategies alone are insufficient.

Treatment Approach

Management centres on a defined dietary strategy, delivered in partnership with specialist dietetic expertise as part of a multidisciplinary team.

The complete protocol — including all therapeutic components, the full clinical sequence, and monitoring targets — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

Lipodystrophies are characterized by a partial or complete reduction in adipose tissue, which results in decreased adipokine levels and IR.

Mutations in either AGPAT2 or BSCL account for 80% of cases of congenital generalized lipodystrophy (Berardinelli–Seip syndrome).

The mainstay of therapy for lipodystrophy is dietary intervention with a low-fat, calorie-neutral diet, and an expert dietician as part of the multidisciplinary team is of paramount importance.

DOI: 10.1111/pedi.13426

View source ↗