Pulmonary arterial hypertension associated with hereditary spherocytosis and splenectomy in a patient with a mutation in the BMPR2 gene. Issue 8 (30th June 2016)

• Record Type: Journal Article
• Authors: Baloira, Adolfo; Bastos, María; Pousada, Guillermo; Valverde, Diana
• Is Part Of: Clinical case reports. Volume 4:Issue 8(2016)
• Page Start: 752
• Page End: 755
• Publication Date: 2016-06-30
• Subjects: ACVRL1 -- ambrisentan -- BMPR2 -- hereditary spherocytosis -- pulmonary arterial hypertension; Medicine -- Periodicals; 616.09
• DOI: 10.1002/ccr3.610
• Languages: English
• ISSNs: 2050-0904

Abstract

Key Clinical Message: 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. 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.