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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