Development and validation of a clinical data-based diagnostic model for neonatal intrahepatic cholestasis caused by citrin deficiency

doi:10.3748/wjg.121360

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World Journal of Gastroenterology

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1007-9327

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

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World J Gastroenterol. Dec 14, 2026; 32(46): 121360
Published online Dec 14, 2026. doi: 10.3748/wjg.121360

Table 1 Comparison of baseline clinical features and demographic characteristics between the neonatal intrahepatic cholestasis caused by citrin deficiency group and the control group

Parameter	Reference interval	NICCD group, n = 170	Control group, n = 196	T	P value
Age in months		2.47 ± 1.26	1.98 ± 0.70	4.69	0.000
WBC (× 10⁹)	4.3-14.2	13.10 ± 4.14	10.54 ± 3.14	6.72	0.000
N (× 10⁹)	0.6-7.5	4.10 ± 1.96	2.54 ± 1.31	9.02	0.000
L (× 10⁹)	2.4-9.5	7.57 ± 2.99	6.73 ± 2.41	3.00	0.003
HB (g/L)	97-183	100.20 ± 16.91	104.4 ± 17.95	-2.27	0.024
PLT (× 10⁹)	183-614 × 10⁹	501.40 ± 173.50	420.50 ± 135.97	5.00	0.000
CRP (mg/L)	0-5	2.29 ± 2.65	1.31 ± 1.77	4.21	0.000
TBIL (mol/L)	3-20	163.90 ± 65.47	137.20 ± 87.32	3.28	0.001
DBIL (mol/L)	1.7-6.8	78.82 ± 39.05	91.66 ± 55.48	-2.52	0.012
IBIL (mol/L)	1.3-18.3	85.16 ± 45.79	41.62 ± 31.77	10.67	0.000
ALB (g/L)	35-50	30.71 ± 4.67	38.02 ± 3.27	-17.51	0.000
GLO (g/L)	9-27	16.37 ± 4.03	18.62 ± 9.03	-2.988	0.003
ALT (U/L)	8-71	63.18 ± 97.61	187.00 ± 207.65	-7.12	0.000
AST (U/L)	21-80	129.20 ± 115.20	203.20 ± 182.28	-4.56	0.000
ALT/AST		2.60 ± 1.34	1.36 ± 0.77	10.97	0.000
TBAC (mol/L)	0.5-10	180.80 ± 67.36	125.40 ± 59.75	8.35	0.000
GGT (U/L)	9-150	186.10 ± 73.35	198.40 ± 33.66	-0.47	0.642
GLU (mmol/L)	3.89-6.4	4.87 ± 8.94	4.66 ± 1.12	0.33	0.745
TG (mmol/L)	0.33-1.82	1.65 ± 0.76	1.63 ± 0.72	0.27	0.789
TCHO (mmol/L)	0-5.2	4.36 ± 1.32	4.12 ± 2.75	1.06	0.292
LACT (mmol/L)	0.5-2.44	4.58 ± 1.65	3.69 ± 1.44	5.51	0.000
NH₄ (mol/L)	10-47	76.53 ± 44.69	51.54 ± 26.04	6.63	0.000
PT (seconds)	10-14	17.29 ± 8.43	13.27 ± 2.87	6.27	0.000
APTT	28-48	49.42 ± 12.26	43.54 ± 5.04	6.14	0.000
INR	0.8-1.5	1.45 ± 1.21	1.00 ± 0.11	5.27	0.000
FIB (mg/dL)	170-450	135.00 ± 54.16	288.80 ± 70.80	-23.06	0.000

NICCD: Neonatal intrahepatic cholestasis caused by citrin deficiency; ALB: Albumin; ALT: Alanine aminotransferase; APTT: Activated partial thromboplastin time; AST: Aspartate aminotransferase; CRP: C-reactive protein; DBIL: Direct bilirubin; FIB: Fibrinogen; GGT: Gamma-glutamyl transferase; GLO: Globulin; GLU: Glucose; HB: Hemoglobin; IBIL: Indirect bilirubin; L: Lymphocytes; INR: International normalized ratio; LACT: Lactate; N: Neutrophils; NH4: Blood ammonia; PLT: Platelets; PT: Prothrombin time; TBAC: Total bile acids; TBIL: Total bilirubin; TCHO: Total cholesterol; TG: Triglycerides; WBC: White blood cell.

Full Size Table

Table 2 Logistic regression analyses of predictors of neonatal intrahepatic cholestasis caused by citrin deficiency

Characteristic	Univariate analysis		Multivariate analysis
Characteristic	OR (95%CI)	P value	OR (95%CI)	P value
Age	1.73 (1.34-2.23)	0.000	9.74 (3.56-26.69)	0.000
WBC	1.22 (1.15-1.31)	0.000	2.23 (0.56-8.95)	0.258
N	1.96 (1.64-2.35)	0.000	0.42 (0.08-2.16)	0.299
L	1.12 (1.04-1.22)	0.004	0.49 (0.11-2.19)	0.347
HB	0.99 (0.97-1.00)	0.026	1.01 (0.97-1.05)	0.727
PLT	1.00 (1.00-1.01)	0.000	1.00 (1.00-1.01)	0.352
CRP	1.31 (1.15-1.50)	0.000	1.09 (0.77-1.53)	0.631
TBIL	1.01 (1.00-1.01)	0.002	0.92 (0.61-1.39)	0.696
DBIL	0.99 (0.99-1.00)	0.014	1.06 (0.70-1.61)	0.782
IBIL	1.03 (1.02-1.04)	0.000	1.11 (0.73-1.69)	0.615
ALB	0.65 (0.59-0.70)	0.000	0.75 (0.61-0.92)	0.005
GLO	0.90 (0.85-0.96)	0.001	0.99 (0.90-1.10)	0.894
ALT	0.99 (0.98-0.99)	0.000	1.00 (0.99-1.01)	0.494
AST	1.00 (0.99-1.00)	0.000	1.00 (1.00-1.01)	0.641
AST/ALT	4.15 (2.96-5.80)	0.000	1.85 (0.76-4.47)	0.173
TBAC	1.01 (1.01-1.02)	0.000	0.99 (0.98-1.00)	0.097
LCAT	1.47 (1.27-1.70)	0.000	1.57 (1.04-2.37)	0.031
NH₄	1.02 (1.02-1.03)	0.000	1.02 (01.00-1.04)	0.07
PT	2.22 (1.86-2.65)	0.000	1.08 (0.94-1.23)	0.291
APTT	1.13 (1.08-1.17)	0.000	0.98 (0.87-1.11)	0.773
INR	33437.21 (3651.70-306172.16)	0.000	167.61 (3.28-8557.58)	0.011
FIB	0.97 (0.96-0.97)	0.000	0.98 (0.97-0.99)	0.001

ALB: Albumin; ALT: Alanine aminotransferase; APTT: Activated partial thromboplastin time; AST: Aspartate aminotransferase; CI: Confidence interval; CRP: C-reactive protein; DBIL: Direct bilirubin; FIB: Fibrinogen; GGT: Gamma-glutamyl transferase; GLO: Globulin; GLU: Glucose; HB: Hemoglobin; IBIL: Indirect bilirubin; INR: International normalized ratio; L: Lymphocytes; LACT: Lactate; N: Neutrophils; NH₄: Blood ammonia; OR: Odds ratio; PLT: Platelet; PT: Prothrombin time; TBAC: Total bile acids; TBIL: Total bilirubin; TCHO: Total cholesterol; TG: Triglycerides; WBC: White blood cell.

Full Size Table

Citation: Jiang T, Qin XM, Liu YW, Li SJ, Liu N, Ouyang WX, Peng SX. Development and validation of a clinical data-based diagnostic model for neonatal intrahepatic cholestasis caused by citrin deficiency. World J Gastroenterol 2026; 32(46): 121360
URL: https://www.wjgnet.com/1007-9327/full/v32/i46/121360.htm
DOI: https://dx.doi.org/10.3748/wjg.121360

Jiang T, Qin XM, Liu YW, Li SJ, Liu N, Ouyang WX, Peng SX. Development and validation of a clinical data-based diagnostic model for neonatal intrahepatic cholestasis caused by citrin deficiency. World J Gastroenterol 2026; 32(46): 121360 [DOI: 10.3748/wjg.121360]

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