Pentoxifylline in hepatopulmonary syndrome

Table 1 Baseline patient characteristics n (%)

Variable	Value (n = 10)
Age (yr)
Mean ± SD, (range)	9.2 ± 5.0 (2-16)
Sex
Female/male	4/6
Cause of cirrhosis
Wilson disease	3 (30)
Cryptogenic cirrhosis	3 (30)
Autoimmune hepatitis	2 (20)
PFIC	1 (10)
GSD	1 (10)
Staging of HPS
Mild	2 (20)
Moderate	6 (60)
Severe	1 (10)
Very severe	1 (10)
Child score
A	3 (30)
B	4 (40)
C	3 (30)

PFIC: Progressive familial intrahepatic cholestasis; GSD: Glycogen storage disease; HPS: Hepatopulmonary syndrome.

Table 2 Clinical and laboratory data at baseline and end of treatment period (mean ± SD) n (%)

Variable	Baseline	End of treatment	P value
Clinical manifestations
Cyanosis	9 (90)	5 (50)	0.12
Dyspnea	3 (30)	2 (20)	1.0
Clubbing	7 (70)	7 (70)	1.0
Palmar erythema	5 (50)	5 (50)	1.0
Hepatomegaly	6 (60)	6 (60)	1.0
Splenomegaly	7 (70)	7 (70)	1.0
Laboratory findings
White blood cell count, × 103/mm	6.4 ± 4.41	4.36 ± 2.04	0.17
Hemoglobin, g/dL	11.25 ± 2.30	11.45 ± 2.71	0.49
Platelet count, × 103/mm³	131.6 ± 566.74	156.2 ± 498.36	0.32
Serum creatinine, mg/dL	0.56 ± 0.16	0.6 ± 0.14	1.0
Serum urea, g/dL	12.60 ± 5.98	14.70 ± 7.20	0.34
Serum albumin, g/dL	3.88 ± 0.52	3.56 ± 0.15	0.59
Serum total protein, g/dL	7.20 ± 0.40	7.26 ± 0.81	1.0
PT, s	15.72 ± 3.07	18.51 ± 8..27	0.40
PTT, s	45.50 ± 14.54	49.42 ± 11.41	0.24
INR	1.46 ± 0.36	1.90 ± 1.14	0.67
Total bilirubin, mg/dL	5.73 ± 7.39	5.07 ± 6.04	0.17
Direct bilirubin, mg/dL	1.70 ± 2.50	1.68 ± 2.71	0.83
ALT, U/L	83.60 ± 64.28	40.60 ± 31.56	0.27
AST, U/L	92.55 ± 64.48	66.80 ± 2814	0.50
ALP, U/L	564.66 ± 198.74	536.50 ± 17.67	0.18

PT: Prothrombin time; PTT: Partial thromboplastin time; INR: International normalized ratio; ALT: Alaninaminotranferase; AST: Aspartataminotransferase; ALP: Alkaline phosphatase.

Table 3 PaO₂, SaO₂ and alveolar-arterial oxygen gradient (A-a G) before, at the end and 3 mo after treatment among patients who could tolerate pentoxifylline (mean ± SD)

Variables	PaO2	SaO2	A-a G
Baseline	73.3 ± 9.33	93.33 ± 6.28	40.10 ± 18.09
End of treatment	97.0 ± 3.79	97.66 ± 1.50	20.73 ± 9.46
Three months after treatment	71.83 ± 21.47	90.5 ± 8.6	43.68 ± 24.30
P value	0.021, 0.022	0.041, 0.052	0.021, 0.022

¹Baseline vs end of treatment;

²End of treatment vs 3 mo later. PaO₂: Oxygen pressure; SaO₂: Oxygen saturation.