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Diabet Med. 2017 Dec;34(12):1792-1794. doi: 10.1111/dme.13527.

Insulin secretory defect in familial partial lipodystrophy Type 2 and successful long-term treatment with a glucagon-like peptide 1 receptor agonist.

Author information

1
Diabetes Research Group, Medizinische Klinik und Poliklinik IV, University of Munich Medical Centre, Munich.
2
Clinical Cooperation Group Type 2 Diabetes, Helmholtz Zentrum München, Neuherberg, Germany.
3
German Centre for Diabetes Research (DZD), Oberschleissheim, Germany.

Abstract

BACKGROUND:

Familial partial lipodystrophies are rare monogenic disorders that are often associated with diabetes. In such cases, it can be difficult to achieve glycaemic control.

CASE REPORT:

We report a 34-year old woman with familial partial lipodystrophy type 2 (Dunnigan) and diabetes; her hyperglycaemia persisted despite metformin treatment. A combined intravenous glucose tolerance-euglycaemic clamp test showed severe insulin resistance, as expected, but also showed strongly diminished first-phase insulin secretion. After the latter finding, we added the glucagon-like peptide-1 receptor agonist liraglutide to the patient's treatment regimen, which rapidly normalized plasma glucose levels. HbA1c values <42 mmol/mol (6.0%) have now been maintained for over 4 years.

CONCLUSION:

This case suggests that a glucagon-like peptide-1 receptor agonist may be a useful component of glucose-lowering therapy in individuals with familial partial lipodystrophy and diabetes mellitus.

PMID:
29044799
DOI:
10.1111/dme.13527
[Indexed for MEDLINE]

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