Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation

:  Objectives:  The hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatatiton, and arterial hypoxemia. The aim of this study is to analyze outcome of children with HPS in liver transplant era. Methods:  Between September 1996 and November 2006, 17...

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Published in:Pediatric transplantation Vol. 12; no. 3; pp. 353 - 357
Main Authors: Tumgor, Gokhan, Arikan, Cigdem, Yuksekkaya, Hasan Ali, Cakir, Murat, Levent, Erturk, Yagci, Rasit Vural, Kilic, Murat, Aydogdu, Sema
Format: Journal Article
Language:English
Published: Oxford, UK Blackwell Publishing Ltd 01.05.2008
Blackwell
Subjects:
Adolescent
Adult
Biological and medical sciences
Blood Gas Analysis
Child
Child, Preschool
children
Echocardiography
Female
Follow-Up Studies
Gastroenterology. Liver. Pancreas. Abdomen
General aspects
hepatopulmonary syndrome
Hepatopulmonary Syndrome - complications
Hepatopulmonary Syndrome - surgery
Hepatopulmonary Syndrome - therapy
Humans
Infant
Liver Cirrhosis - complications
Liver Cirrhosis - surgery
Liver Cirrhosis - therapy
liver transplantation
Liver Transplantation - methods
Liver, biliary tract, pancreas, portal circulation, spleen
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Other diseases. Semiology
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the digestive system
Treatment Outcome
Human
Pediatrics
Digestive system
Respiratory disease
Liver
Cardiovascular disease
Hepatic disease
Homotransplantation
Cirrhosis
Treatment
children
Surgery
Hepatopulmonary syndrome
Digestive diseases
Graft
Child
Liver transplantation
ISSN:1397-3142, 1399-3046, 1399-3046
Online Access:Get full text
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Summary::  Objectives:  The hepatopulmonary syndrome (HPS) is characterized as a triad: liver disease, intrapulmonary vascular dilatatiton, and arterial hypoxemia. The aim of this study is to analyze outcome of children with HPS in liver transplant era. Methods:  Between September 1996 and November 2006, 172 cirrhotic patients (median age 5 years; range 0.2–22 years, M/F; 97/75) were followed at Ege University Pediatric Gastroenterology, Hepatology and Nutrition Unit. All patients were evaluated by chest radiography, arterial blood gas analysis, and alveolar‐arterial oxygen tension difference, contrast echocardiography (CEE) after and before the liver transplantation. Results:  HPS was diagnosed in 33 patients (19%) by CEE. None of them had pulmonary hypertension. HPS was not found related to etiology of the liver disease. Portal hypertension was found related to the development of HPS (75.7% in patients with HPS and 54.6% in others, p = 0.02). 17 of 33 patients with HPS underwent liver transplantation. Preoperative and postoperative period of these patients was uneventful. Patients were extubated in the operating room except for two. Median follow up of transplanted children was 1.9 year (range; 0.75–10 years). Arterial blood gas analysis and CEE positivity regressed in all of them by postoperative 6th month. Conclusions:  HPS is a serious and important complication of cirrhotic children that leads to tissue hypoxia and central cyanosis. HPS seems reversible after liver transplantation in all patients.
Bibliography:istex:630A6590C7B092C4CAB6A033CB1C4A26E2553377
ArticleID:PETR807
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ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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ISSN:1397-3142
1399-3046
1399-3046
DOI:10.1111/j.1399-3046.2007.00807.x