Table 1 Baseline characteristics of the patients
FAS (n = 60) | PB elixir (n = 21) | PB powder (n = 39) | |||
|---|---|---|---|---|---|
Oral route (n = 8) | Enteral tube (n = 13) | Oral route (n = 14) | Enteral tube (n = 25) | ||
Male (%) | 33 (55) | 4 (50) | 8 (62) | 6 (43) | 15 (60) |
Age (years) | 1.0 [0.6–2.5] | 1.0 [0.7–1.9] | 0.8 [0.2–1.0] | 0.9 [0.5–2.5] | 2.0 [1.0–3.2] |
Body weight (kg) | 8.2 [4.7–11.7] | 8.1 [5.9–11.6] | 4.0 [2.8–8.1] | 8.8 [6.2–11.5] | 8.8 [4.8–14.3] |
PB dose (mg/day) | 40.0 [20.0–60.0] | 40.0 [31.0–60.0] | 16.0 [9.8–23.2] | 50.0 [27.5–80.0] | 50.0 [19.0–80.0] |
Number of daily PB doses (times/day) | 2.0 [2.0–2.0] | 2.0 [2.0–2.0] | 2.0 [1.1–2.5] | 2.0 [1.5–2.0] | 2.0 [2.0–2.0] |
PB dose per body weight (mg/kg/time) | 2.6 [1.9–3.7] | 2.6 [2.1–4.3] | 2.0 [1.1–2.5] | 2.7 [1.9–3.6] | 2.9 [2.2–4.7] |
Serum PB concentration (μg/mL) | 16.0 [11.2–25.0] | 15.4 [11.1–20.1] | 15.7 [12.7–18.8] | 11.4 [10.1–23.7] | 17.4 [9.9–34.9] |
ALB (g/dL) | 4.1 [3.7–4.4] | 4.2 [3.3–4.6] | 4.0 [3.5–4.4] | 4.3 [3.8–4.5] | 4.0 [3.7–4.4] |
AST (U/L) | 35.9 [28.4–53.9] | 34.9 [21.6–46.5] | 46.0 [31.5–63.5] | 35.0 [28.7–50.5] | 39.0 [30.0–75.9] |
ALT (U/L) | 20.8 [15.0–39.8] | 19.2 [12.5–25.8] | 29.8 [13.2–35.5] | 22.6 [16.5–42.6] | 29.6 [15.7–52.5] |
LDH (U/L) | 272.6 [222.4–334.2] | 264.5 [212.5–312.6] | 255.0 [243.0–271.5] | 320.7 [253.4–388.0] | 277.0 [225.5–367.7] |
γGTP (U/L) | 69.9 [28.8–170.9] | 30.5 [23.5–93.0] | 65.0 [31.5–289.5] | 39.5 [27.7–57.5] | 76.3 [34.0–172.5] |
BUN (mg/dL) | 8.9 [5.8–11.9] | 8.2 [5.2–13.0] | 9.0 [3.5–9.6] | 6.1 [5.4–8.5] | 9.4 [6.3–13.5] |
Scr (mg/dL) | 0.2 [0.2–0.3] | 0.2 [0.2–0.3] | 0.2 [0.1–0.3] | 0.2 [0.2–0.3] | 0.2 [0.1–0.3] |
Interacting drug use | |||||
VPA (%) | 13 (22) | 0 (0) | 3 (23) | 5 (36) | 5 (20) |
Amiodarone (%) | 1 (2) | 0 (0) | 1 (8) | 0 (0) | 0 (0) |
Causative disorder *including duplication | |||||
Fever convulsion (%) | 11 (18) | 2 (25) | 3 (23) | 2 (14) | 4 (16) |
Epilepsy (%) | 18 (30) | 2 (25) | 3 (23) | 4 (29) | 9 (36) |
Acute encephalitis/acute encephalopathy (%) | 4 (7) | 1 (13) | 2 (15) | 0 (0) | 1 (4) |
Intracranial bleeding (%) | 9 (15) | 1 (13) | 1 (8) | 5 (36) | 2 (8) |
Hypoxic-ischemic encephalopathy (%) | 16 (27) | 1 (13) | 7 (54) | 3 (21) | 5 (20) |
Hyperammonemia (%) | 2 (3) | 1 (13) | 0 (0) | 0 (0) | 1 (4) |
Brain tumor (%) | 4 (7) | 1 (13) | 0 (0) | 2 (14) | 1 (4) |
Angiocholitis (%) | 2 (3) | 1 (13) | 0 (0) | 0 (0) | 1 (4) |
Not clear (%) | 2 (3) | 0 (0) | 0 (0) | 0 (0) | 2 (8) |