Related Experiment Video

Updated: Jul 7, 2026

Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus
06:15

Protocol and Guidelines for Point-of-Care Lung Ultrasound in Diagnosing Neonatal Pulmonary Diseases Based on International Expert Consensus

Published on: March 6, 2019

Prevalence of hepatopulmonary syndrome in children

Khalid Noli1, Melinda Solomon, Fraser Golding

  • 1Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada.

Pediatrics
|March 4, 2008
PubMed

Insights

Hepatopulmonary syndrome (HPS) affects a significant number of children with cirrhosis or severe portal hypertension. This study identified HPS in 8% of pediatric patients, highlighting the need for further research into its impact.

Area of Science:

  • Pediatric Gastroenterology and Hepatology
  • Pulmonary Medicine
  • Vascular Biology

Background:

  • Hepatopulmonary syndrome (HPS) is characterized by liver disease, hypoxemia, and intrapulmonary vascular dilation.
  • Prevalence of HPS in adults with cirrhosis is known (4-29%), but its occurrence and outcomes in children are not well-established.
  • Understanding HPS in pediatric populations is crucial for timely diagnosis and management.

Purpose of the Study:

  • To prospectively determine the prevalence of intrapulmonary vascular dilation (IPVD) in children with liver disease.
  • To identify the prevalence of hepatopulmonary syndrome (HPS) in this pediatric cohort.
  • To describe the diagnostic criteria and initial findings related to HPS in children.

Main Methods:

  • Pulse oximetry screening was performed on 301 children with liver disease.
  • Children with oxygen saturation ≤97%, cirrhosis, or severe portal hypertension underwent contrast-enhanced echocardiography.
  • Patients with IPVD were further evaluated with arterial blood gas analysis and technetium-99m-labeled macroaggregated albumin scans.

Main Results:

  • Seven out of 26 screened children (27%) exhibited intrapulmonary vascular dilation.
  • Two children met the diagnostic criteria for hepatopulmonary syndrome (8% of those with cirrhosis or severe portal hypertension).
  • Both HPS patients had abnormal pulse oximetry; technetium scans indicated abnormal tracer distribution outside the lungs.

Conclusions:

  • Hepatopulmonary syndrome is present in a notable proportion of children with cirrhosis or severe portal hypertension.
  • Further research is needed to clarify the significance of IPVD without HPS in children.
  • Additional studies should investigate the impact of HPS on the clinical outcomes of affected children.
Abstract

Related Concept Videos

Respiratory Syncytial Virus Disease01:29

Respiratory Syncytial Virus Disease

Human respiratory syncytial virus (RSV) is a widespread pathogen that primarily targets infants and young children but also poses a serious health risk to elderly and immunocompromised individuals. Belonging to the Pneumoviridae family, RSV is a negative-sense, single-stranded RNA virus within the Pneumovirus genus. Its global health burden is significant, with millions of cases annually resulting in hospitalizations and mortality, particularly in resource-limited settings. Although most...
Pneumonia I: Introduction01:29

Pneumonia I: Introduction

Pneumonia is an infection of the lower respiratory tract that leads to inflammation of the lung parenchyma, often resulting in the accumulation of inflammatory exudate in the alveoli and airways. Unlike the watery, low-protein fluid exudate in pulmonary edema, the exudate in this case is a thick fluid rich in immune cells, proteins, and debris produced during infection and inflammation.This impairs gas exchange and can lead to consolidation of lung tissue. The infection may be caused by a...
Pneumonia I: Introduction01:30

Pneumonia I: Introduction

Pneumonia is an acute respiratory infection that targets the lungs, specifically the alveoli. These tiny air sacs, essential for oxygen exchange, become engorged with pus and fluid, severely hindering breathing, decreasing oxygen absorption, and causing significant pain and discomfort during respiration.
Risk Factors
Various factors influence the likelihood of developing pneumonia. Age plays a crucial role, with infants, children under two, and individuals over 65 at increased risk due to their...
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
Portal Hypertension01:22

Portal Hypertension

Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...