Biopsy-driven diagnosis in infants with cholestatic jaundice in Iran

Table 1 Clinical and laboratory characteristics of the 55 infants with cholestasis included for analysis of biopsy diagnosis

Variable	Mean ± SD	Range
Gestational age (wk)	38.4 ± 2.7	31-41
Birth weight (g)	2785 ± 658	1300-3980
Age when jaundice came to clinical attention (d)	43.8 ± 93	1-630
Age at liver biopsy (d)	102 ± 110	8-690
Total bilirubin (mg/dL)	13.6 ± 5.9	3.9-36
Direct bilirubin (mg/dL)	7.3 ± 3.4	2.4-19
Alkaline phosphatase (IU/L)	1244 ± 800	9.5-3379
AST (IU/L)	280 ± 223	36-1009
ALT (IU/L)	238 ± 486	16-3510
GGT (IU/L)	412 ± 508	35-1981
PT (s)	14.6 ± 4.5	11-38
PTT (s)	39.2 ± 10	26-82
Albumin (g/dL)	3.5 ± 0.9	1.3-5.4
Hgb (g/dL)	10 ± 1.9	6.9-16.4
Platelet × 103/μL	381 ± 164	76-700

ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; GGT: Gamma-glutamyl transpeptidase; Hgb: Hemoglobin; PT: Prothrombin time; PTT: Partial thromboplastin time.

Table 2 Diagnoses from liver biopsies and their frequencies in cases of infantile cholestasis n (%)

Biopsy findings	Frequency	Mean gestational age in weeks	Mean birth weight in grams	Mean age when jaundice came to clinical attention (range)	Mean age at liver biopsy in days
BA	32 (58)	38.4	2850	21 (1-120)	87.5
Paucity of bile ducts	6 (11)	39.0	2600	20.5 (1-60)	59
Metabolic disease	6 (11)	39.4	3380	221 (44-630)	266
INH	5 (9)	36.4	2230	38 (3-90)	93
Choledochal cyst	2 (3.6)	40.0	2750	20 (15-26)	82
Liver cirrhosis	2 (3.6)	38.5	1950	10 (9-13)	34
PFIC	1 (1.8)	40.0	3300	135	165
Portal fibrosis	1 (1.8)	34.0	1400	30	45

BA: Biliary atresia; INH: Idiopathic neonatal hepatitis; PFIC: Progressive familial intrahepatic cholestasis.

Table 3 Frequencies of selected variables for each diagnosed cause of infantile cholestasis n (%)

Variable		Frequency	BA	INH	Paucity of bile ducts	Choledochal cyst	Metabolic disease	Cirrhosis	PFIC and portal fibrosis
Sex	Female	28 (50.9)	20	3	1	1	2	0	1
	Male	27 (49.1)	12	2	5	1	4	2	1
Maturity	Preterm	15 (27.3)	8	3	1	0	1	1	1
	Term	40 (72.7)	24	2	5	2	5	1	1
Birth weight	Low	15 (27.3)	7	3	1	1	0	2	1
	Normal	40 (72.7)	25	2	5	1	6	0	1
Perinatal findings	Bacterial infection	5 (9.0)	3	0	0	0	1	0	1
	Congenital anomalies	10 (18.0)	4	1	1	2	0	2	0
	Parenteral nutrition	1 (2.0)	0	0	0	0	0	1	0
	Seizure	3 (5.5)	3	0	0	0	0	0	0
	Meconium stained amniotic fluid	4 (7.2)	2	0	2	0	0	0	0
	Fetomaternal hemorrhage	1 (2.0)	1	0	0	0	0	0	0
	Apnea	1 (2.0)	1	0	0	0	0	0	0
	Others	13 (23.0)	7	1	2	1	0	2	0
	Nil	31 (56.0)	18	4	3	0	5	0	1
Clinical findings	Dark urine	21 (38.0)	13	3	4	0	1	0	0
	Acholic stool	33 (60.0)	22	2	4	2	3	0	0
	Hepatomegaly	30 (54.5)	19	5	1	0	4	0	1
	Splenomegaly	17 (31.0)	12	2	0	0	2	0	1
	Clubbing	1 (2.0)	0	0	0	0	1	0	0
	Failure to thrive	14 (25.5)	9	1	1	0	2	1	0
	Ascites	8 (14.5)	4	1	1	0	1	0	1
	Jaundice	55 (100)	32	5	6	2	6	2	2
	Pruritus	3 (5.5)	1	0	0	0	1	0	1
Jaundice temporal course	Progressive	33 (60.0)	17	5	2	2	4	1	2
	Intermittent	4 (7.2)	3	0	1	0	0	0	0
	Continuous	6 (10.9)	3	0	1	0	1	1	0

BA: Biliary atresia; INH: Idiopathic neonatal hepatitis; PFIC: Progressive familial intrahepatic cholestasis.

Table 4 Mean laboratory findings from each diagnosed cause of infantile cholestasis

Cause	Total bilirubin in mg/dL	Direct bilirubin in mg/dL	ALP in IU/L	AST in IU/L	ALT in IU/L	GGT in IU/L	PT in sec	PTT in sec	Hgbin g/dL	Platelet×103/μL
BA	14.4	8.0	1230	313	310	597a	14.0	38	9.7	390
INH	10.5	6.0	1343	287	113	-	16.0	44	10.0	406
Paucity of bile ducts	11.5	6.5	1475	223	250	310	18.0	37	11.0	410
PFIC	28.0	13.0	1455	65	57	44	12.0	39	9.5	700
Choledochal cyst	11.5	5.2	1667	212	170	-	13.0	33	8.5	300
Metabolic disease	13.8	6.7	843	205	114	66	13.0	34	9.5	355
Cirrhosis	11.6	5.3	1710	226	45	-	16.0	76a	12.0	200
Portal fibrosis	8.0	5.1	453	380	120	-	13.5	39	10.0	187

^aP < 0.05. ALP: Alkaline phosphatase; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; GGT: Gamma-glutamyl transpeptidase; Hgb: Hemoglobin; PT: Prothrombin time; PTT: Partial thromboplastin time.