Table 1 Comparison of clinical characteristics and laboratory results of patients
|
Homozygous LMNA p.R582H (n = 1) |
Heterozygous LMNA p.R582H (n = 1) |
CGL1 (n = 9) |
Typical FPLD2 (n = 8) | |
|---|---|---|---|---|
| Age (years) | 29 | 48 | 25 (18–34) | 49 (32–62) |
| BMI (kg/m2) | 19.20 | 27.28 | 19.53 (16.61–22.20) | 23.22 (19.53–26.20) |
| The age when lipodystrophy was diagnosed (years) | 29 | 33 | 16 (1–31) | 49 (12–60) |
| The age when diabetes developed (years) | 17 | 32 | 14 (6–25) | 33 (21–51) |
| Oral antidiabetic use (Yes/No) | Yes | Yes | 8/1 | 9/9 |
| Insulin (Yes/No) | No | No | 9/0 | 5/3 |
| Complications of diabetes (Yes/No) | No | No | 8/1 | 7/1 |
| The age when hypertriglyceridemia was first detected (years) | 20 | 33 | 16 (6–26) | 37 (20–51) |
| Lipid medication (Yes/No) | Yes | Yes | 9/9 | 6/2 |
| History of pancreatitis (Yes/No) | No | Yes | 3/6 | 1/7 |
| The age when hepatic steatosis was first detected (years) | 29 | 33 | 17 (6–29) | 35 (20–51) |
| HbA1c (%) | 7.9 | 6.2 | 10.4 (7.6–11.7) | 8.5 (6.3–11) |
| Leptin (ng/mL) | 0.4 | 9.81 | 0.38 (< 0.1–0.85) | 1.53 (0.94–7.42) |
| HOMA-IR† | 14.1 | 7.66 | 9.06 (3.98–78.57) | 8.12 (5.63–14.43) |
| AST (IU/L) | 27 | 23 | 23 (11–145) | 17 (11–32) |
| ALT (IU/L) | 30 | 32 | 24 (15–114) | 19 (9–43) |
| HDL Cholesterol (mg/dL) | 22 | 38 | 28 (15–36) | 33 (21–46) |
| Triglyceride (mg/dL) | 1600 | 169 | 597 (72–2083) | 443 (196–1358) |
| Creatinine (mg/dL) | 0.69 | 0.71 | 1.21 (0.37–2.29) | 0.75 (0.50–1.50) |
| Urinary protein excretion (mg/day) | 41.8 | 11 | 245 (< 5–15,200) | 173 (< 5–3210) |