Table 3.
Laboratory analyses. Iron studies, lipid panel, urinary mevalonate, and plasma and RBC fatty acids were drawn.
| PKAN participants | |||||
|---|---|---|---|---|---|
|
| |||||
| All (n = 16) | Adults (n = 10) | Children (n = 6) | Males (n = 6) | Females (n = 10) | |
| Lipid panel | |||||
| Total cholesterol | 165.4 ± 25.7 | 174.4 ± 26.5 | 150 ± 16.9 | 178.5 ± 26.0 | 157.5 ± 23.2 |
| VLDL cholesterol | 12.1 ± 7.5 | 13.8 ± 7.4 | 9.3 ± 7.3 | 11.0 ± 6.5 | 12.8 ± 8.2 |
| LDL cholesterol | 105.8 ± 30.6 | 115.2 ± 33.0 | 90.0 ± 19.4 | 125.8 ± 35.5 | 93.7 ± 20.9 |
| HDL cholesterol | 53.4 ± 9.5 | 50.9 ± 10.1 | 57.7 ± 7.1 | 52.0 ± 10.7 | 54.3 ± 9.2 |
| Total triglycerides | 95.6 ± 41.3 | 102.6 ± 39.5 | 83.8 ± 45.1 | 102.7 ± 38.7 | 91.3 ± 44.3 |
| VLDL triglyceride | 56.5 ± 32.3 | 59.3 ± 30.3 | 51.8 ± 38.0 | 68.3 ± 31.8 | 49.4 ± 32.1 |
| VLDL-C/total TG | 1.0 ± 0.0 | 0.1 ± 0.0 | 0.1 ± 0.0 | 0.1 ± 0.0 | 0.1 ± 0.0 |
| LDL-C/HDL-C | 2.1 ± 1.0 | 2.4 ± 1.1 | 1.6 ± 0.5 | 2.6 ± 1.3 | 1.8 ± 0.7 |
| Total C/HDL-C | 3.2 ± 1.1 | 3.6 ± 1.2 | 2.7 ± 0.5 | 3.7 ± 1.3 | 3.0 ± 0.9 |
| Urinary mevalonic acid | |||||
| μmol/day | 1.76 ± 0.97 | 2.04 ± 1.0 | 1.3 ± 0.8 | 2.5 ± 1.2 | 1.3 ± 0.5 |
| nmol/day*kg (23.1 ± 1.76a) | 29.96 ± 10.32 | 31.7 ± 10.8 | 27.0 ± 9.7 | 36.0 ± 11.7 | 26.4 ± 7.9 |
| Plasma fatty acids (% of total) | |||||
| Palmitic | 20.0 ± 1.6 | 20.1 ± 1.38 | 19.8 ± 2.0 | 20.0 ± 1.3 | 20.0 ± 1.8 |
| Stearic | 6.7 ± 1.05 | 6.2 ± 0.7 | 4.7 ± 1.2 | 6.8 ± 0.6 | 6.5 ± 1.3 |
| Linoleic | 31.7 ± 3.1 | 31.5 ± 2.7 | 31.9 ± 4.2 | 31.2 ± 2.3 | 21.9 ± 3.7 |
| Linolenic | 0.54 ± 0.24 | 0.46 ± 0.14 | 0.67 ± 0.33 | 0.45 ± 0.09 | 31.9 ± 3.7 |
| Arachidonic | 7.8 ± 1.0 | 8.0 ± 1.0 | 7.4 ± 0.9 | 8.0 ± 1.1 | 0.59 ± 0.29 |
| DHA | 1.4 ± 0.82 | 1.4 ± 0.9 | 1.4 ± 0.8 | 1.7 ± 1.1 | 1.3 ± 0.6 |
| RBC fatty acids (% of total) | |||||
| Palmitic | 18.1 ± 1.4 | 17.9 ± 1.1 | 18.4 ± 1.8 | 14.4 ± 0.5 | 18.3 ± 1.5 |
| Stearic | 14.0 ± 1.0 | 13.7 ± 0.9 | 14.5 ± 1.0 | 17.7 ± 1.2 | 13.7 ± 1.1 |
| Linoleic | 13.0 ± 1.2 | 12.9 ± 1.3 | 13.2 ± 1.3 | 12.7 ± 1.3 | 13.2 ± 1.3 |
| Linolenic | 0.15 ± 0.07 | 0.12 ± 0.03 | 0.20 ± 0.08 | 0.13 ± 0.04 | 0.16 ± 0.08 |
| Arachidonic | 16.8 ± 1.0 | 17.4 ± 1.0 | 16.4 ± 1.0 | 16.7 ± 0.8 | 16.9 ± 1.2 |
| DHA | 3.9 ± 1.4 | 3.9 ± 1.6 | 3.9 ± 0.9 | 4.2 ± 1.7 | 3.67 ± 1.2 |
| Iron storage and transport | |||||
| Transferrin (mg/dL) | 47.0 ± 34.1 | 57.5 ± 39.2 | 31.3 ± 17.3 | 34.9 ± 24.4 | 65.2 ± 40.4 |
| Ferritin (ng/mL) | 296.7 ± 52.5 | 268 ± 32.7 | 339.7 ± 48.0 | 303.1 ± 54.5 | 287.0 ± 52.6 |
Footnotes: VLDL = very low density lipoprotein; LDL = low density lipoprotein; HDL = high density lipoprotein; C = cholesterol; TG = triglycerides DHA = docosahexaenoic acid (C22:6n–3); RBC = red blood cell.
a
Normal value.