[Hepatopulmonary syndrome in children: evaluation and treatment].
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.
Rovira Amigo
S
S
Unidad de Neumología Pediátrica y Fibrosis Quística, Hospital Universitari Vall d'Hebron, Barcelona, España. 42402sra@comb.cat
Martín de Vicente
C
C
Bueno Recio
J
J
Bueno Aribayos
J
J
Ortega López
J
J
Girona Comas
J
J
Moreno Galdó
A
A
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male