Intrapulmonary arteriovenous shunt in children with chronic liver disease: clinical features, laboratory data and outcome.

OBJECTIVE: To determine the frequency of hepatopulmonary syndrome in children with chronic liver disease, and its clinical and biochemical associations. METHOD: This study included 53 children with chronic liver disease, who underwent contrast echocardiography with agitated saline and measurement of arterial blood gases. RESULTS: Of the 53 patients studied, 18 had intrapulmonary shunting of blood. This shunting was associated with presence of palpable spleen, cyanosis and dyspnea, but not with abnormalities in the biochemical tests of liver function. At 1-year follow-up, there were 5 deaths among 18 patients with intrapulmonary shunt. In a logistic regression model, PaO 2 < 70 mmHg was found to be a predictor of death (p< 0.05). CONCLUSION: Intrapulmonary shunting is a common and important complication in children with chronic liver disease.

Pentoxifylline in hepatopulmonary syndrome.

AIM: To determine the effects of pentoxifylline (PTX) on clinical manifestations and evaluate arterial blood gas data in hepatopulmonary syndrome (HPS) in children. METHODS: In a pilot study of 10 children with chronic liver disease, who had HPS, 20 mg/kg/d PTX was administered for 3 mo. Clinical data and arterial blood gas parameters were evaluated at baseline, the end of the treatment period, and 3 mo after drug discontinuation. RESULTS: Six patients could tolerate PTX, while four patients experienced complications. Among patients who could tolerate PTX, there was a significant increase in arterial oxygen pressure (PaO(2)) (P = 0.02) and oxygen saturation (SaO(2)) (P = 0.04) and alveolar-arterial oxygen gradient (P = 0.02) after 3 mo of treatment. Significant decreases in PaO(2) (P = 0.02) and alveolar-arterial oxygen gradient (P = 0.02) were also seen after drug discontinuation. CONCLUSION: PTX may improve PaO(2), SaO(2) and alveolar-arterial oxygen gradient in the early stage of HPS.