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      Mesangiocapillary Glomerulonephritis Type 2 Associated with Familial Partial Lipodystrophy (Dunnigan-Kobberling Syndrome)

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          The lipodystrophies are a heterogeneous group of disorders of adipose tissue associated with insulin resistance. The sporadic form of partial lipodystrophy, characterised by fat loss from the face and upper body, is associated with complement abnormalities and mesangiocapillary glomerulonephritis type 2 (MCGN II) and the conditions are thought to have a shared autoimmune aetiology. We present the first case of the rare familial form of partial lipodystrophy, caused by a mutation in the LMNA gene, associated with MCGN II. This suggests that partial lipodystrophy of both the sporadic and familial subtypes may predispose to this condition and that the observed renal and complement abnormalities may be secondary to other factors associated with lipodystrophy.

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          Response to treatment with rosiglitazone in familial partial lipodystrophy due to a mutation in the LMNA gene.

          Familial partial lipodystrophy (FPLD) is a monogenic form of diabetes characterised by a dominantly inherited disorder of adipose tissue associated with the loss of subcutaneous fat from the limbs and trunk, with excess fat deposited around the face and neck. The lipodystrophy causes severe insulin resistance, resulting in acanthosis nigricans, diabetes, dyslipidaemia, and increased risk of cardiovascular disease. Preliminary results from animals and man suggest that increasing subcutaneous fat by treatment with thiazolidinediones should improve insulin resistance and the associated features of this syndrome. We report a 24-year-old patient with FPLD caused by a mutation in the LMNA gene (R482W) treated with 12 months of rosiglitazone. Subcutaneous fat increased following rosiglitazone treatment as demonstrated by a 29% generalised increase in skin-fold thickness. Leptin levels increased from 5.8 to 11.2 ng/ml. Compared with treatment on Metformin, there was an increase in insulin sensitivity (HOMA S% 17.2-31.6) but no change in glycaemic control. The lipid profile worsened during the follow-up period. This initial case suggests that, for modification of cardiovascular risk factors, there are no clear advantages in treating patients with FPLD with rosiglitazone despite increases in subcutaneous adipose tissue. Larger series will be needed to identify moderate beneficial effects and treatment may be more effective in patients with generalised forms of lipodystrophy.
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            Partial lipodystrophy and renal disease

             P Lenane,  G. Murphy,  G Murphy (2000)

              Author and article information

              Nephron Clin Pract
              Nephron Clinical Practice
              S. Karger AG
              February 2004
              17 November 2004
              : 96
              : 2
              : c35-c38
              aDepartment of Diabetes and Vascular Medicine, Peninsula Medical School, bHistopathology Department, RoyalDevon and Exeter Hospital, and cExeter Kidney Unit, Royal Devon and Exeter Hospital, Exeter,UK
              76396 Nephron Clin Pract 2004;96:c35–c38
              © 2004 S. Karger AG, Basel

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              Figures: 2, References: 17, Pages: 1
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