RESUMEN
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Asunto(s)
Humanos , Masculino , Femenino , Síndrome Hepatopulmonar/complicaciones , Síndrome Hepatopulmonar/etiología , Hipertensión Pulmonar/complicaciones , Hipertensión Portal/complicaciones , Factores de Riesgo , Trasplante de Hígado , Hepatopatías/complicaciones , Hepatopatías Alcohólicas/complicaciones , Ascitis/complicaciones , Síndrome Hepatopulmonar/fisiopatología , Síndrome Hepatopulmonar/clasificaciónRESUMEN
A 9-year-old girl presented with cyanosis and decreased exercise tolerance. Chest X-ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed.
Asunto(s)
Síndrome Hepatopulmonar/etiología , Vena Porta/anomalías , Niño , Femenino , Síndrome Hepatopulmonar/clasificación , Humanos , Vena Porta/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: The pediatric end stage liver disease (PELD) score has been used widely to prioritize children awaiting cadaveric liver transplantation (LTx). To establish the objective parameter for optimal timing of living-related LTx (LRLTx), we have assessed our cases using the PELD score. METHODS: From 1997 to 2002, 24 children were evaluated 28 times for the indication of LRLTx. Among them, 15 were for jaundice and nine for growth failure, hepatopulmonary syndrome, and variceal bleeding. Nine of 24 children underwent LRLTx. They were divided into several groups according to their clinical course. The PELD score consisted of age, albumin, total bilirubin, prothrombine time-international ratio (INR) and growth failure. A cut-off value was obtained by the highest positive and negative predictive value. RESULTS: The PELD score in cases whose indication for LRLTx was approved was significantly higher compared with the cases who were not, and a cut-off value of 4 was obtained. The PELD score in cases who were alive after LRLTx was significantly lower compared with the cases who died after LRLTx or evaluation of the indication, and a cut-off value of 22 was established. CONCLUSION: LRLTx may be considered when the PELD score exceeds 4, and LRLTx may be required immediately when the PELD score exceeds 22.
