Table 2.
Patient characteristics (Reference range) | Survival group (n = 52) | Mortality group (n = 9) | P value | |
---|---|---|---|---|
General Information | Gender (Male/Female) | 30/22 | 7/2 | 0.462 |
Birth weight (g) | 3079.32 ± 694.17(44) | 2828.57 ± 360.39(7) | 0.351 | |
Age at referral (months) | 3.96 ± 3.13(48) | 9.58 ± 5.03(8) | 0.000 | |
Presence/absence of infection (n) | 29 /21 | 7/1 | 0.023 | |
Lactose-free and/or MCT-enriched formula (Yes/No) | 47/5 | 5/4 | 0.021 | |
Complete blood count | WBC (4–10 *10^9/L) | 10.34 ± 4.91(30) | 9.90 ± 2.7(5) | 0.637 |
RBC (3.5–5.5 *10^12/L) | 3.34 ± 0.66(23) | 2.94 ± 0.87(5) | 0.255 | |
PLT (100–300 *10^9/L) | 387.54 ± 196.46(28) | 109.60 ± 19.26(5) | 0.010 | |
Hemoglobin (110–160 g/L) | 93.81 ± 22.40(29) | 87.40 ± 22.63 (5) | 0.342 | |
Serum Biochemistry | Total Biliburin (5.1–17.1 umol/L) | 117.86 ± 65.73(47) | 195.10 ± 194.99(8) | 0.503 |
Direct Bilirubin (0–6 umol/L) | 70.82 ± 39.48(47) | 113.69 ± 99.46(8) | 0.474 | |
ALT (0–40 IU/L) | 44.51 ± 71.35(47) | 45.25 ± 25.44(8) | 0.148 | |
AST (0–40 IU/L) | 98.41 ± 96.59(46) | 100.75 ± 60.47(8) | 0.450 | |
GGT (7–50 IU/L) | 219.32 ± 127.59(47) | 80.00 ± 69.70(8) | 0.001 | |
GGT (<=50 vs. > 50 IU/L) | 1/46 | 4/4 | 0.001 | |
Total bile acid (0–10 ummol/L) | 185.47 ± 84.47(46) | 140.88 ± 124.80(8) | 0.206 | |
Total protein (60–83 g/L) | 49.45 ± 9.56(45) | 49.84 ± 6.38(8) | 0.914 | |
Albumin (35–55 g/L) | 32.74 ± 9.93(45) | 30.89 ± 6.05(8) | 0.205 | |
Glucose (3.9–5.8 mmol/L) | 2.84 ± 1.28(46) | 3.36 ± 1.38(8) | 0.318 | |
Total cholesterol (3.1–5.2 mmol/L) | 3.30 ± 1.06(41) | 2.25 ± 1.03(7) | 0.019 | |
Triglyceride (0.56–1.70 mmol/L) | 1.48 ± 0.65(40) | 1.39 ± 0.69(8) | 0.571 | |
Urea (2.5–6.5 mmol/L) | 4.02 ± 4.27(27) | 2.91 ± 1.10(5) | 0.815 | |
Creatinine (20–110 umol/L) | 20.28 ± 14.00(29) | 15.51 ± 5.51(8) | 0.271 | |
Lactic acid (0.7–2.1 mmol/L) | 3.63 ± 2.76(15) | 3.98 ± 1.22(5) | 0.708 | |
Serum ammonia (10–47 umol/L) | 98.14 ± 45.20(34) | 142.31 ± 42.09(8) | 0.016 | |
Alphafetoprotein (0–28 ng/ml) | 15,473.97 ± 25,750.21(26) | 60,476.24 ± 126,197.60(5) | 0.823 | |
Blood Coagulation Profile | INR (0.8–1.2) | 1.41 ± 0.38(34) | 1.60 ± 0.58 (7) | 0.510 |
PT (12.0–14.8 s) | 17.51 ± 6.35 (37) | 18.37 ± 6.18 (7) | 0.712 | |
PTA (80–120%) | 71.75 ± 25.75 (28) | 68.57 ± 34.40 (7) | 0.786 | |
APTT (28.0–44.5 s) | 45.63 ± 13.29 (37) | 53.09 ± 12.56 (7) | 0.177 | |
Fib (2–4 g/L) | 2.04 ± 3.34 (32) | 1.74 ± 1.05 (7) | 0.656 | |
Thrombin Time (14–21 s) | 21.26 ± 4.15 (38) | 21.66 ± 3.66 (7) | 0.817 | |
Blood tandem mass spectrometry | Citrulline (7–40 umol/L) | 137.06 ± 79.62 (31) | 52.34 ± 19.14 (4) | 0.010 |
Methionine (10–80 umol/L) | 157.26 ± 100.04 (23) | 231.93 ± 324.79 (5) | 0.787 | |
Tyrosine (30–200 umol/L) | 130.00 ± 73.19 (21) | 250.26 ± 77.78 (3) | 0.015 | |
Threonine (17–90 umol/L) | 130.67 ± 51.62 (20) | 228.30 ± 212.56 (2) | 0.732 | |
SLC25A13 gene mutation allele frequency | 851del4 | 55 (104) | 11 (18) | 0.518 |
1638ins23 | 12 (104) | 3 (18) | 0.541 | |
IVS6 + 5G > A | 6 (104) | 1 (18) | 1.000 | |
IVS16ins3kb | 6 (104) | 3 (18) | 0.128 |
Boldface, statistically significant p values