title: Hepatopulmonary Syndrome in children: a comparative study of non-cirrhotic vs. cirrhotic portal hypertension.
abstract: OBJECTIVE
  Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome.
  
  
  METHODS
  Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied. Hepatopulmonary syndrome was diagnosed by the presence intrapulmonary shunt using transthoracic contrast echocardiography and hypoxia/orthodeoxia (in arterial blood gas examination). Comparative analyses were done for both the groups, and also intra- and intergroups with and without hepatopulmonary syndrome.
  
  
  RESULTS
  Of 135 children, hepatopulmonary syndrome occurred more frequently in cirrhosis (40%) than extrahepatic portal venous obstruction (13%) (P = 0.01). Cirrhotics with hepatopulmonary syndrome were symptomatic (85% vs. 14%, P < 0.001) and had longer disease duration [16(1-120) months vs. 28 (1-168) months, P = 0.01] as compared to cirrhotics without hepatopulmonary syndrome. Prevalence of hepatopulmonary syndrome among cirrhotics did not correlate with the severity of liver disease as assessed by both paediatric end-stage liver disease score and Child-Turcotte-Pugh score. Patients with extrahepatic portal venous obstruction and hepatopulmonary syndrome remain asymptomatic manifesting with clubbing alone (38%).
  
  
  CONCLUSIONS
  Hepatopulmonary syndrome occurs more frequently in cirrhosis, a majority being symptomatic with more severity in comparison to extrahepatic portal venous obstruction having no symptoms and less severity. There seems to be a role of liver dysfunction besides portal hypertension as an added factor in the pathogenesis of hepatopulmonary syndrome.
authors.affiliation: Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Vibhor V
authors.initials: VV
authors.lastname: Borkar
authors.affiliation: Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Ujjal
authors.initials: U
authors.lastname: Poddar
authors.affiliation: Department of Cardiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Aditya
authors.initials: A
authors.lastname: Kapoor
authors.affiliation: Department of Cardiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Srivatsa
authors.initials: S
authors.lastname: Ns
authors.affiliation: Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Anshu
authors.initials: A
authors.lastname: Srivastava
authors.affiliation: Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
authors.forename: Surender K
authors.initials: SK
authors.lastname: Yachha
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications*; D000860:Hypoxia / Q000175:diagnosis; D008103:Liver Cirrhosis / Q000150:complications*; D008297:Male; D011446:Prospective Studies
keywords: cirrhosis; contrast-enhanced echocardiography; extrahepatic portal venous obstruction; hepatopulmonary syndrome