RESUMEN
El síndrome hepatopulmonar (SHP) es una enfermedad poco frecuente que afecta a pacientes de cualquier edad con enfermedad hepática aguda o crónica. Su diagnóstico se basa en la presencia de hipoxemia y la demostración de un cortocircuito pulmonar mediante ecocardiografía con contraste o gammagrafía de perfusión pulmonar. La angiografía es de utilidad para detectar fístulas macroscópicas. Se presentan 5 casos clínicos de edad pediátrica con diferente historia natural y evolución. A 2 de éstos se les diagnosticó SHP tipo 1, a otros 2 de tipo 2 y al quinto de tipo 1 y 2, lo que condicionó un enfoque terapéutico diferente en cada uno de los casos (AU)
Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. Its diagnosis is based on the presence of hypoxemia and the demonstration of an intrapulmonary shunting by echocardiography with contrast or perfusion lung scanning. Pulmonary angiography is useful to demonstrate macroscopic arteriovenous communications. We describe five paediatric cases with a different natural history and evolution. Two of them were diagnosed with hepatopulmonary syndrome type 1, another two with type 2 and a fifth one with type 1 and 2, which required a different therapeutic approach in each case (AU)
Asunto(s)
Humanos , Masculino , Femenino , Lactante , Niño , Adolescente , Síndrome Hepatopulmonar/diagnóstico , Trasplante de Hígado , Hipoxia/etiología , Insuficiencia Hepática/etiología , Síndrome Hepatopulmonar/cirugíaRESUMEN
Hepatopulmonary syndrome is a rare disease that affects patients of any age with acute or chronic liver disease. Its diagnosis is based on the presence of hypoxemia and the demonstration of an intrapulmonary shunting by echocardiography with contrast or perfusion lung scanning. Pulmonary angiography is useful to demonstrate macroscopic arteriovenous communications. We describe five paediatric cases with a different natural history and evolution. Two of them were diagnosed with hepatopulmonary syndrome type 1, another two with type 2 and a fifth one with type 1 and 2, which required a different therapeutic approach in each case.