Hepatopulmonary Syndrome in a Thirteen Year Old Boy: A Case Report

Abstract

Hepatopulmonary syndrome (HPS) is a late complication associated with liver cirrhosis. Recent studies have suggested that it can also occur in non-cirrhotic portal hypertension. The criteria that need to be met for making the diagnosis of HPS include chronic liver disease, impaired gaseous exchange leading to hypoxemia and intrapulmonary vascular dilation. The pathophysiology of this disease includes mediators like nitric oxide (NO), and endothelial growth factors which play an important role in intrapulmonary dilation of vessels. This, in turn, leads to ventilation-perfusion mismatch which is the main etiology of pulmonary symptoms associated with this syndrome. The classical symptoms include dyspnea, orthodeoxia and platypnea. Contrast echocardiography has emerged to be a very sensitive test for its diagnosis. The timing of appearance of microbubbles help to differentiate between intracardiac and intrapulmonary shunting, with the latter being the hallmark of HPS. The only curative treatment available is liver transplantation. Here we present a case of a 13-year-old patient who presented in Nishtar hospital in Multan, Pakistan. He initially presented with signs of liver cirrhosis but no definite cause could be found. The patient didn’t come for a follow-up after that. Now he presented with signs of HPS and acute decompensated liver disease. HPS was confirmed on contrast echocardiography. This is a rare presentation of HPS in this age group.

The status and influencing factors of lung ventilation function in employees exposed to dust in enterprises of the XPCC, China

“…Moderate alcohol intake could increase FEV 1 and FVC improve lung function (70) potentially by enhancing mucociliary clearance, stimulating bronchodilation, and even alleviating airway damage (59). In contrast, chronic alcohol exposure often leads to hepatopulmonary syndrome, which can cause respiratory impairment (71). In this study, univariate analysis revealed a statistically significant association between lung ventilation function and alcohol consumption, while multivariable analysis showed the opposite finding.…”

Hepatopulmonary syndrome - An unusual complication of chronic liver cell disease

“…Among the complications of chronic liver disease, HPS has a prevalence ranging from 4-47% depending on diagnostic criteria, methods used and population studied. On average the prevalence is 25% [1,2]. It presents with exertional dyspnoea, platypnoea, orthodeoxia, cyanosis and digital clubbing [1][2][3].…”

“…On average the prevalence is 25% [1,2]. It presents with exertional dyspnoea, platypnoea, orthodeoxia, cyanosis and digital clubbing [1][2][3]. The pathogenesis of HPS has not been clearly understood.…”

“…Failure of cirrhotic liver to clear the circulating pulmonary vasodilators may be a contributing factor. This leads to ventilation-perfusion mismatch in the lung, giving rise to the pulmonary symptoms [1,3]. The prognosis is poor when CLCD is complicated with HPS [1,3].…”

“…This leads to ventilation-perfusion mismatch in the lung, giving rise to the pulmonary symptoms [1,3]. The prognosis is poor when CLCD is complicated with HPS [1,3].…”