Study Patients Age (year) Male/female Drug Dose Duration Outcome Gupta et al. [32 ] 9 patients with cirrhosis 40 5/4 PTX 400 mg 3 times/day 3 months (i) Improvement of clinical symptoms (ii) Improvement of PaO2 (iii) Decreased TNF-α Kianifar et al. [33 ] 10 pediatric patient with cirrhosis
6/4 PTX 20 mg/kg/day 3 months (i) Increase in PaO2 and A-a PaO2 (ii) Improvement of O2 saturation (iii) No Improvement in Clinical symptoms Tanikella et al. [34 ] 9 patients with cirrhosis
3/6 PTX (i) 400 mg/day (ii) 400 mg twice/day (iii) 400 mg 3 times/day (i) 7days (ii) 7 days (iii) 42 days (i) No significant change in PaO2 (ii) No significant change in A-a PaO2 (iii) No significant change in TNF-α Rolla et al. [40 ] 1 patient with alcoholic cirrhosis 45 Female MB 3 mg/kg intravenous One bolus dose (i) Improvement in PaO2 (ii) Improvement in O2 saturation Schenk et al. [41 ] 7 patients with liver cirrhosis 52 5/2 MB 3 mg/kg intravenous One bolus dose in 15 minutes (i) Improvement of PaO2 (ii) Improvement of A-a PaO2 (iii) Increased mean pulmonary arterial pressure and pulmonary vascular resistance Jounieaux et al. [42 ] 1 patient with alcoholic cirrhosis 61 Male MB 3 mg/kg intravenous One bolus dose (i) Increased mean pulmonary arterial pressure (ii) No change in shunt fraction Roma et al. [43 ] 1 liver transplant patient for AIH 15 Female MB 3 mg/kg intravenous One bolus dose in 15 minutes (i) Increased O2 saturation (ii) As abridge for weaning of from ventilator Añel and Sheagren [52 ] 1 patient with cirrhosis 44 Male Norfloxacin 400 mg 2 times/day 4 weeks (i) Increased O2 saturation (ii) Resolution of platypnea and orthodeoxia Gupta et al. [53 ] 11 patients with cirrhosis
8/1 Norfloxacin 400 mg 4 times/day 1 month (i) No improvement in HPS Caldwell et al. [54 ] 1 patient with cirrhosis 60 Female Garlic 4 teaspoons 4 times/day 4 months (i) Improvement of cyanosis (ii) Increased PaO2 Abrams and Fallon [55 ] 15 patients with cirrhosis NA 7/8 Garlic 2 Capsule (500 mg) 2 times/day 6 months (i) Increased PaO2 (ii) Deceased A-a PaO2 (iii) Decreased dyspnea on exertion Sani et al. [56 ] 15 pediatric patients with cirrhosis
10/5 Garlic 0.5–2 g/1.73 m2 per day. 4 weeks (i) Increased PaO2 (ii) Improvement of dyspnea De et al. [57 ] 41 cirrhotic patients, 21 patients received garlic, 20 received placebo
17/4 Garlic 1 capsule (250 mg) 2 times/day 18 months (i) Increased PaO2 (ii) Deceased A-a PaO2 (iii) Reversal of HPS in 14 from 21 patients Maniscalco et al. [63 ] 1 patient with cryptogenic cirrhosis 31 Male L-NAME 8 mg/kg in normal saline Intravenously over 5 minutes (i) Decreased NO production (ii) No improvement in arterial oxygenation (iii) No improvement in orthodeoxia Gómez et al. [64 ] 10 cirrhotic patients with HPS
7/3 L-NAME Single dose, 162.0 mg dissolved in 4.0 mL 0.9% saline Nebulized over 12 minutes (i) Decreased exhaled NO (ii) Increased systemic vascular resistance (iii) No change in ventilation/perfusion mismatch, intrapulmonary shunting, nor arterial oxygenation Moreira Silva et al. [75 ] 1 patient with autoimmune lymphoproliferative syndrome 13 Male MMFL 500 mg twice/day 9 months (i) Improvement of cyanosis, clubbing, and spider nevi (ii) Normalization of PaO2 (iii) No need for supplemental oxygen (iv) Improvement of intrapulmonary shunt Yilmaz et al. [76 ] 1 patient with noncirrhotic portal hypertension 18 Male Paroxetine 20 mg/day 6 months (i) No significant improvement Krug et al. [82 ] 1 patient with alcoholic cirrhosis 46 Female Inhaled iloprost 30 µg/day nebulized 2 months (i) Decreased subjective dyspnea (ii) Increased exercise tolerance (iii) Increase in PaO2 Krowka et al. [84 ] 22 patients with cirrhosis or chronic active hepatitis (8 patients received the drug) 49 12/10 Somatostatin analogue 150 µg every 8 hours subcutaneously 4 days (i) No improvement in subjective dyspnea (ii) No improvement in arterial oxygenation at the end of study