Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome—Case Series and Literature Review

Table 1

Basic clinical information of 8 cases with different onset ages.

No.	Sex	Age	Chief complaint	Course	Principal diagnosis	Hb (g/L)	WBC (10^9/L)	PLT (10^9/L)	ALT (U/L)	AST (U/L)	ALP (U/L)	GGT (U/L)	TBil (mmol/L)	INR	Alb (g/L)
P1	M	1 yr	Hyperpyrexia	1 month	CHF^§; CBA; Cholangitis after Kasai operation	110	9.13	205	51	68	553	265	6.2	1.04	37
P2	F	4 yr	Hematemesis	1.5 month	CHF^§	86	3.71	189	268	230	813	500	12.3	0.94	39
P3	M	15 yr	Hematemesis and melena	8 years	CHF^§; Caroli disease	52	5.63	649	17	19	129	26	3.7	1.15	27
P4	F	9 yr	Melena and bloating	2 months	CHF^§; PFIC3	68	2.25	226	317.9	274.1	382	233	14.7	0.87	28.8
P5	F	26 yr	Bloating, edema, and oliguria	7 years	CHF; CHB; Intrahepatic biliary duct cystic dilation; Polycystic kidneys^§§	83	7.04	329	15	20	91	22	12	1.08	37
P6	F	26 yr	Isolated abnormal liver function	6 years	CHF	134	4.81	66	67	51	156	177	46.1	1.12	46
P7	M	26 yr	Isolated abnormal liver function	9 months	CHF; Intrahepatic biliary duct cystic dilation	147	4.19	75	20	21	45	64	17.5	1.20	45
P8	F	60 yr	Abnormal liver function and edema	4 years	CHF	136	3.82	72	46	55	139	24	31.8	1.19	32

Abbreviations and normal range of each lab index in parentheses: P = patient; M = male; F = female; yr = years old; CHF = congenital hepatic fibrosis; CHB = chronic hepatitis B; CBA = congenital biliary atresia; PFIC3 = progressive familial intrahepatic cholestasis-type 3; Hb = hemoglobin (male: 130-175 g/L, female: 115-150 g/L); WBC = white blood cell (-); PLT = platelet (-); ALT = alanine aminotransferase (male: 9-50 U/L, female: 7-40 U/L); AST = aspartate aminotransferase (male: 15-40 U/L, female: 13-35 U/L); ALP = alkaline phosphatase (1~4 yrs: <281 U/L; 5~6 yrs: <269 U/L; 7~12 yrs: <300 U/L; male 13~17 yrs: <390 U/L; male 18~19 yrs: 40-129 U/L; : 45-125 U/L; female 13~17 yrs: <187 U/L; female 18~19 yrs: 35-104 U/L; female 20~49 yrs: 35-100 U/L; female 50~79 yrs: 35-100 U/L; and : 50-135 U/L); GGT = γ-glutamyl transpeptidase (male: 10-60 U/L, female: 7-45 U/L); TBil = total bilirubin (3.4-20.4 μmol/L); INR = international normalized ratio (0.88-1.12); Alb = albumin (40-55 g/L). ^§CHF: combined with liver decompensation; ^§§polycystic kidneys combined with chronic renal dysfunction; IDA: iron deficiency anemia; RA: renal anemia.