Total cohort | VLCFA-tested subgroup (VLCFA normal in all) | |||||
---|---|---|---|---|---|---|
All | Adult-onset | Childhood-onset | All | Adult-onset | Childhood-onset | |
Sex ratio, male:female | 1:1.13 | 1:2.67 | 1:0.76 | 1:1.17 | 1:2.33 | 1:1 |
Age at onset, median (range), years | 12 (2–69) | 29 (18–69) | 9 (2–17) | 10.5 (3–50) | 32.5 (20–50) | 8.5 (3–17) |
OCB, positive, all LPs | 23.1% (9/39) | 15.4% (2/13) | 26.9% (7/26) | 14.3% (4/28) | 0% (0/10) | 22.2% (4/18) |
OCB, positive, all LPs, including LPs classified as “normal” (1983–) | 17.6% (9/51) | 0% (2/18) | 0% (7/33) | 10.5% (4/38) | 0% (0/14) | 16.6% (4/24) |
OCB, positive, first LP | 21.9% (7/32) | 10% (1/10) | 27.3% (6/22) | 13.6% (3/22) | 0% (0/8) | 21.4% (3/14) |
OCB, positive, patients | 25.7% (9/35) | 18.2% (2/11) | 29.2% (7/24) | 16.7% (4/24) | 0% (0/8) | 25% (4/16) |
WCC, elevated, all LPs | 20.7% (12/58) | 32.1% (9/28) | 10% (3/30) | 7.7% (2/26) | 20% (2/10) | 0% (0/16) |
WCC, elevated, all LPs, including LPs classified as “normal” | 15.2% (12/79) | 26.5% (9/34) | 6.7% (3/45) | 4.9% (2/41) | 14.3% (2/14) | 0% (0/27) |
WCC, elevated, first LP | 25% (10/40) | 43.8% (7/16) | 12.5% (3/24) | 10.5% (2/19) | 28.6% (2/7) | 0% (0/12) |
WCC, elevated, patients | 24.4% (10/41) | 43.8% (7/16) | 12% (3/25) | 10.5% (2/19) | 28.6% (2/7) | 0% (0/12) |
WCC, elevated, median (range), all LPs | 25 (11–277; N = 11) | 24 (12–277; N = 8) | 32 (11–36; N = 3) | 150 (23–277; N = 2) | 150 (23–277; N = 2) | N.a. (N.a.; N = 0) |
WCC, elevated, median (range), first LPs | 25 (11–277; N = 9) | 24 (12–277; N = 6) | 32 (11–36; N = 3) | 150 (23–277; N = 2) | 150 (23–277; N = 2) | N.a. (N.a.; N = 0) |
WCC, elevated, median (range), patients | 25 (11–277; N = 9) | 25 (15–277; N = 6) | 32 (11–36; N = 3) | 150 (23–277; N = 2) | 150 (23–277; N = 2) | N.a. (N.a.; N = 0) |
TP, elevated, all LPs | 43.8% (28/64) | 46.4% (13/28) | 41.7% (15/36) | 37.5% (12/32) | 40% (4/10) | 36.4% (8/22) |
TP, elevated, all LPs, including LPs classified as “normal” | 32.9% (28/85) | 38.2% (13/34) | 29.4% (15/51) | 26.1% (12/46) | 28.6% (4/14) | 25% (8/32) |
TP, elevated, first LP | 43.8% (28/64) | 46.4% (13/28) | 41.7% (15/36) | 37.5% (12/32) | 40% (4/10) | 36.4% (8/22) |
TP, elevated, patients | 41.3% (19/46) | 43.8% (7/16) | 40% (12/30) | 37.5% (9/24) | 42.9% (3/7) | 35.3% (6/17) |
TP, elevated, median (range), all LPs | 114 (46–220; N = 22) | 135 (46–182; N = 11) | 112 (56–220; N = 11) | 103 (46–212; N = 7) | 105.5 (46–165; N = 2) | 103 (56–212; N = 5) |
TP, elevated, median (range), first LPs | 116 (46–220; N = 15) | 97.5 (46–180; N = 6) | 116 (63–220; N = 9) | 134 (46–212; N = 6) | 105.5 (46–165; N = 2) | 134 (63–212; N = 4) |
TP, elevated, median (range), patients | 107.5 (46–220; N = 18) | 60 (46–182; N = 7) | 112 (56–220; N = 11) | 103 (46–212; N = 7) | 105.5 (46–165; N = 2) | 103 (56–212; N = 5) |
Glucose, normal, all LPs | 85% (17/20) | 80% (4/5) | 86.7% (13/15) | 100% (14/14) | 100% (4/4) | 100% (10/10) |
Glucose, normal, all LPs, including LPs classified as “normal” | 93.9% (46/49) | 92.9% (13/14) | 94.3% (33/35) | 100% (33/33) | 100% (11/11) | 100% (22/22) |
Lactate, normal, all LPs | 100% (7/7) | 100% (1/1) | 100% (6/6) | 100% (7/7) | 100% (1/1) | 100% (6/6) |
Lactate, normal, all LPs, including LPs classified as “normal” | 100% (35/35) | 100% (10/10) | 100% (25/25) | 100% (24/24) | 100% (8/8) | 100% (16/16) |