title: Pulmonary arterial hypertension associated with hereditary spherocytosis and splenectomy in a patient with a mutation in the BMPR2 gene.
abstract: There is some question about the relationship between hereditary spherocytosis (HS) and pulmonary arterial hypertension, even associated with splenectomy. The finding of BMPR2 mutations in our patient suggests that other factors are necessary for the development of the disease, and perhaps, the incidence of pulmonary hypertension is not increased in patients with HS.
authors.affiliation: Respiratory Division Complejo Hospitalario Universitario de Pontevedra Pontevedra Spain.
authors.forename: Adolfo
authors.initials: A
authors.lastname: Baloira
authors.affiliation: Department of Cardiology Complejo Hospitalario Universitario de Pontevedra Pontevedra Spain.
authors.forename: María
authors.initials: M
authors.lastname: Bastos
authors.affiliation: Genetic Department, Faculty of Biology University of Vigo Vigo Spain.
authors.forename: Guillermo
authors.initials: G
authors.lastname: Pousada
authors.affiliation: Genetic Department, Faculty of Biology University of Vigo Vigo Spain.
authors.forename: Diana
authors.initials: D
authors.lastname: Valverde
keywords: ACVRL1; BMPR2; ambrisentan; hereditary spherocytosis; pulmonary arterial hypertension