Table 1.
Baseline Patient Characteristics
| Parameter | All (N = 115) | GLD (N = 73) | PLD (N = 42) | P Value (GLD vs PLD) |
|---|---|---|---|---|
| Age, y | 18 [14,35] | 16 [12,21] | 35 [19,46] | <0.0001 |
| Sex, % female | 83 | 77 | 95 | 0.01 |
| Lipodystrophy subtype, N | 48 CGL | 48 CGL | 42 FPL | — |
| 19 AGL | 19 AGL | |||
| 42 FPL | 6 Atypical progeria | |||
| 6 Atypical progeria | ||||
| Affected gene, N | — | 29 AGPAT2 | 23 LMNA | — |
| 17 BSCL2 | 9 PPAR-gamma | |||
| 7 LMNA | 1 PCYT1a | |||
| 19 None | 9 Unknown | |||
| 1 Unknown | ||||
| Diabetes, % | 80 | 78 | 83 | 0.63 |
| Diabetes duration, y | 6.8 [3.3,12] | 4.5 [2.8,9.4] | 11 [4.7,18] | 0.001 |
| Fasting insulin, mcU/mL | 42 [21,90] | 53 [26,104] | 26 [13,52] | 0.0005 |
| HOMA-IR | 17.0 [7.5,36.0] | 25.5 [12.0,44.9] | 8.9 [5.3,17.5] | <0.0001 |
| Hypertension, % | 29a | 31a | 26 | 0.62 |
| ACEi/ARB usage, % | 43 | 36 | 55 | 0.05 |
| Leptin, ng/mL | 1.7 [0.97,4.6] | 1.1 [0.80,1.8] | 6.2 [3.9,9.4] | <0.0001 |
| Hemoglobin A1c, % | 8.4 ± 2.3 | 8.6 ± 2.4 | 8.1 ± 2.1 | 0.23 |
| Triglycerides, mg/dL | 432 [231,1140] | 441 [229,1187] | 422 [233,1007] | 0.92 |
| LDL-c | 90 [66,117] | 90 [69,115] | 92 [65,134] | 0.98 |
| Urinary glucose excretion, g/24 h | 4.9 [0.39,23] | 5.1 [0.77,26] | 4.0 [0.18,15] | 0.08 |
| Urinary albumin excretion, mg/24 h | 83 [15,554] | 244 [31,978] | 18 [7.9,79] | <0.0001 |
| Normal, <30 mg/24 h, % | 41.6 | 25.5 | 67.7 | |
| Microalbuminuria, 30-300 mg/24 h, % | 26.9 | 29.1 | 23.5 | 0.001 |
| Macroalbuminuria, >300 mg/24 h, % | 31.5 | 45.4 | 8.8 | |
| Urinary protein excretion, mg/24 h | 293 [148,1244] | 605 [198,2250] | 165 [119,249] | <0.0001 |
| Elevated, >150 mg/24 h, % | 75.0 | 82.8 | 61.8 | 0.04 |
| Nephrotic range, >3.5 g/24 h, % | 8.7 | 13.8 | 0 | 0.02 |
| eGFR, mL/min/1.73 m2 | 129 [99,155] | 143 [112,169] | 105 [85,129] | <0.0001 |
| Elevated, >130 mL/min/1.73 m2, % | 47.8 | 63.9 | 19.5 | <0.0001 |
| CrCl, mL/min | 141 [106,209] | 174 [115,233] | 111 [95,163] | 0.005 |
| Elevated, >130 mL/min, % | 57.9 | 66.7 | 42.9 | 0.04 |
Results are presented as median [25th,75th percentile] or mean ± SD unless otherwise noted.
Abbreviations: AGL, acquired general lipidystrophy; CGL, congenital generalized lipidystrophy; FPL, familial partial lipodystrophy; HOMA-IR, homeostasis model assessment of insulin resistance [glucose (mg/dL) × insulin (mcU/mL)/405].
a
One patient with GLD younger than 2 years of age was excluded from evaluation of hypertension.