Long-term treatment experience in a subject with Dunnigan-type familial partial lipodystrophy: efficacy of rosiglitazone.

Abstract

Dunnigan-type familial partial lipodystrophy (FPLD) is caused by mutations in LMNA, the gene that encodes nuclear lamins A and C. FPLD is characterized by peripheral fat loss, excess central adiposity, insulin resistance, and hyperlipidaemia, which are difficult to treat. We present our 2 years' experience of treatment with rosiglitazone in a subject with FPLD. Insulin requirement decreased significantly from 240 IU/day to 76 IU/day (range 20-240 IU/day) and serum triglyceride concentration was lowered from 13.7 +/- 14.4 mmol/l to 4.5 +/- 4.3 mmol/l and remained stable. Mean HbA(1c) prior to rosiglitazone therapy was 9.4 +/- 1.32% and decreased to 7.4 +/- 0.6% during therapy with rosiglitazone. This case demonstrates the benefits of PPARgamma-agonists on glycaemic control and dyslipidaemia in a patient with FPLD. This in turn implies that PPARgamma may play a pathophysiological role in FPLD.

• Authors: A Lüdtke, K Heck, J Genschel, H Mehnert, S Spuler, H J Worman, H H-J Schmidt
• Journal: Diabetic medicine : a journal of the British Diabetic Association (Diabet Med) Vol. 22 Issue 11 Pg. 1611-3 (Nov 2005) ISSN: 0742-3071 [Print] England
• PMID: 16241930 (Publication Type: Case Reports, Journal Article)

Chemical References

• Glycated Hemoglobin A
• Hypoglycemic Agents
• PPAR gamma
• Thiazolidinediones
• Triglycerides
• Rosiglitazone

Topics

• Aged
• Diabetes Mellitus, Lipoatrophic (blood, drug therapy)
• Fatal Outcome
• Female
• Glycated Hemoglobin (analysis)
• Humans
• Hypoglycemic Agents (therapeutic use)
• PPAR gamma (agonists, therapeutic use)
• Rosiglitazone
• Thiazolidinediones (therapeutic use)
• Triglycerides (blood)