Table 2.
Hematological and metabolic parameters for the overall CGL cohort
| Overall CGL cohort | |||
|---|---|---|---|
| Parameter | n(available) | Mean (± SD) | Median (range) |
| Hemoglobin (g/dL) | 36 | 12.3 (± 2.0) | 12.2 (8.2–16.6) |
| Platelet count (109 platelets/L) | 36 | 361 (± 155) | 330 (79–739) |
| Serum calcium (mmol/L) | 29 | 2.4 (± 0.4) | 2.4 (0.6–2.8) |
| Serum creatinine (µmol/L) | 32 | 33 (± 17) | 30 (12–80) |
| White blood cell count (109 cells/L) | 36 | 9.2 (± 4.0) | 8.6 (4.1–19.7) |
| ALT (IU/L) | 35 | 66 (± 56) | 46 (14–232) |
| AST (IU/L) | 34 | 53 (± 32) | 46 (15–187) |
| Creatine kinase (IU/L) | 20 | 981 (± 1514) | 150 (16–6000) |
| Fasting insulin (pmol/L) | 17 | 384 (± 511) | 79 (13–1611) |
| FPG (mmol/L) | 23 | 8.9 (± 5.5) | 5.4 (4.0–19.3) |
| GGT (IU/L) | 16 | 66 (± 44) | 49 (13–175) |
| HbA1c (%) | 28 | 7.3 (± 3.1) | 5.7 (4.5–14.3) |
| Total cholesterol (mmol/L) | 29 | 4.9 (± 2.1) | 4.3 (2.1–13.2) |
| Triglycerides (mmol/L) | 33 | 6.1 (± 7.3) | 2.9 (0.9–31.0) |
Data were obtained at the time of patient diagnosis and during leptin-replacement naïve follow-up visits as permitted by available medical records. Reference ranges: Fasting insulin, < 111 pmol/L (< 16 mUI/ml); FPG, 3.9–5.5 mmol/L (70–99 mg/dl); HbA1c, < 5.7%; Triglycerides, < 1.69 mmol/L (< 150 mg/dl); Total cholesterol, < 5.18 mmol/L (< 200 mg/dl) [11, 22, 31, 32]. ALT Alanine aminotransferase, AST Aspartate aminotransferase, CGL Congenital generalized lipodystrophy, FPG Fasting plasma glucose, GGT Gamma-glutamyl transferase, HbA1c Glycated hemoglobin, n Number of patients exhibiting the disease characteristic, n(available), Number of patients with available data, SD Standard deviation