F Banning1,2,3, M Rottenkolber1,2,3, I Freibothe1,2,3, J Seissler1,2,3, A Lechner1,2,3. 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.
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.
Authors: H Mosbah; B Donadille; M C Vantyghem; C Vigouroux; C Vatier; S Janmaat; M Atlan; C Badens; P Barat; S Béliard; J Beltrand; R Ben Yaou; E Bismuth; F Boccara; B Cariou; M Chaouat; G Charriot; S Christin-Maitre; M De Kerdanet; B Delemer; E Disse; N Dubois; B Eymard; B Fève; O Lascols; P Mathurin; E Nobécourt; A Poujol-Robert; G Prevost; P Richard; J Sellam; I Tauveron; D Treboz; B Vergès; V Vermot-Desroches; K Wahbi; I Jéru Journal: Orphanet J Rare Dis Date: 2022-04-19 Impact factor: 4.303