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Table 2 Comparison of clinical and laboratory data between survival group and mortality group

From: Risk factors associated with mortality in neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and clinical implications

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
  1. Boldface, statistically significant p values