This Article Full Text Full Text (PDF) Submit a related Letter to the Editor Purchase Article View Shopping Cart Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Garg, A. Search for Related Content PubMed PubMed Citation Articles by Garg, A.

Gender Differences in the Prevalence of Metabolic Complications in Familial Partial Lipodystrophy (Dunnigan Variety)¹

Department of Internal Medicine and the Center for Human Nutrition, University of Texas Southwestern Medical Center, Dallas, Texas 75235-9052

Address correspondence and requests for reprints to: Abhimanyu Garg, M.D., Center for Human Nutrition, University of Texas Southwestern Medical Center, 5323 Harry Hines Boulevard, Dallas, Texas 75235-9052. E-mail: agarg{at}mednet.swmed.edu

Familial partial lipodystrophy, Dunnigan type (FPLD; Mendelian Inheritance in Man #151660), is an autosomal dominant disorder characterized by loss of sc fat from the extremities and trunk since puberty and predisposition to insulin resistance and its complications. However, for lack of recognition of affected men, previous studies could not ascertain any gender differences in phenotypic expression. Therefore, anthropometric variables and prevalence of diabetes mellitus, dyslipidemia, hypertension, and atherosclerotic vascular disease were compared among 17 postpubertal men and 22 women with FPLD from eight pedigrees. All individuals completed a questionnaire, and fasting blood was analyzed for glucose, insulin, and lipoprotein concentrations. Both affected men and women had similar patterns of fat loss. Compared with the affected men, women had higher prevalence of diabetes (18% and 50%, respectively; P = 0.05) and atherosclerotic vascular disease (12% and 45%, respectively; P = 0.04) and had higher serum triglycerides (median values, 2.27 and 4.25 mmol/L, respectively; P = 0.02) and lower high-density lipoprotein cholesterol concentrations (age-adjusted means, 0.94 and 0.70 mmol/L, respectively; P = 0.04). The prevalence of hypertension and fasting serum insulin concentrations were similar. In conclusion, women with FPLD are more severely affected with metabolic complications of insulin resistance than men. These observations raise the possibility that women with generalized and regional obesity may also have more severe metabolic sequelae of insulin resistance.

This article has been cited by other articles:

				A. Decaudain, M.-C. Vantyghem, B. Guerci, A.-C. Hecart, M. Auclair, Y. Reznik, H. Narbonne, P.-H. Ducluzeau, B. Donadille, C. Lebbe, et al. New Metabolic Phenotypes in Laminopathies: LMNA Mutations in Patients with Severe Metabolic Syndrome J. Clin. Endocrinol. Metab., December 1, 2007; 92(12): 4835 - 4844. [Abstract] [Full Text] [PDF]

				R. A. Hegele, T. R. Joy, S. A. Al-Attar, and B. K. Rutt Thematic review series: Adipocyte Biology. Lipodystrophies: windows on adipose biology and metabolism J. Lipid Res., July 1, 2007; 48(7): 1433 - 1444. [Abstract] [Full Text] [PDF]

				J. L. V. Broers, F. C. S. Ramaekers, G. Bonne, R. B. Yaou, and C. J. Hutchison Nuclear lamins: laminopathies and their role in premature ageing. Physiol Rev, July 1, 2006; 86(3): 967 - 1008. [Abstract] [Full Text] [PDF]

				K. N. Jacob, F. Baptista, H. G. dos Santos, J. Oshima, A. K. Agarwal, and A. Garg Phenotypic Heterogeneity in Body Fat Distribution in Patients with Atypical Werner's Syndrome Due to Heterozygous Arg133Leu Lamin A/C Mutation J. Clin. Endocrinol. Metab., December 1, 2005; 90(12): 6699 - 6706. [Abstract] [Full Text] [PDF]

				J. Young, L. Morbois-Trabut, B. Couzinet, O. Lascols, E. Dion, V. Bereziat, B. Feve, I. Richard, J. Capeau, P. Chanson, et al. Type A Insulin Resistance Syndrome Revealing a Novel Lamin A Mutation Diabetes, June 1, 2005; 54(6): 1873 - 1878. [Abstract] [Full Text] [PDF]

				M. C. Vantyghem, P. Pigny, C. A. Maurage, N. Rouaix-Emery, T. Stojkovic, J. M. Cuisset, A. Millaire, O. Lascols, P. Vermersch, J. L. Wemeau, et al. Patients with Familial Partial Lipodystrophy of the Dunnigan Type Due to a LMNA R482W Mutation Show Muscular and Cardiac Abnormalities J. Clin. Endocrinol. Metab., November 1, 2004; 89(11): 5337 - 5346. [Abstract] [Full Text] [PDF]

				K. Al-Shali, H. Cao, N. Knoers, A. R. Hermus, C. J. Tack, and R. A. Hegele A Single-Base Mutation in the Peroxisome Proliferator-Activated Receptor {gamma}4 Promoter Associated with Altered in Vitro Expression and Partial Lipodystrophy J. Clin. Endocrinol. Metab., November 1, 2004; 89(11): 5655 - 5660. [Abstract] [Full Text] [PDF]

				A. Garg Acquired and Inherited Lipodystrophies N. Engl. J. Med., March 18, 2004; 350(12): 1220 - 1234. [Full Text] [PDF]

				W. A. Haque, E. A. Oral, K. Dietz, A. M. Bowcock, A. K. Agarwal, and A. Garg Risk Factors for Diabetes in Familial Partial Lipodystrophy, Dunnigan Variety Diabetes Care, May 1, 2003; 26(5): 1350 - 1355. [Abstract] [Full Text] [PDF]

				G. Meininger, C. Hadigan, P. Rietschel, and S. Grinspoon Body-composition measurements as predictors of glucose and insulin abnormalities in HIV-positive men Am. J. Clinical Nutrition, August 1, 2002; 76(2): 460 - 465. [Abstract] [Full Text] [PDF]

				J. Janke, S. Engeli, K. Gorzelniak, F. C. Luft, and A. M. Sharma Mature Adipocytes Inhibit In Vitro Differentiation of Human Preadipocytes via Angiotensin Type 1 Receptors Diabetes, June 1, 2002; 51(6): 1699 - 1707. [Abstract] [Full Text] [PDF]

				R. A. Hegele Premature Atherosclerosis Associated With Monogenic Insulin Resistance Circulation, May 8, 2001; 103(18): 2225 - 2229. [Abstract] [Full Text] [PDF]

				A. Garg, M. Vinaitheerthan, P. T. Weatherall, and A. M. Bowcock Phenotypic Heterogeneity in Patients with Familial Partial Lipodystrophy (Dunnigan Variety) Related to the Site of Missense Mutations in Lamin A/C Gene J. Clin. Endocrinol. Metab., January 1, 2001; 86(1): 59 - 65. [Abstract] [Full Text]