title: Prevalence of pulmonary hypertension in hereditary spherocytosis.
abstract: Vascular complications, including pulmonary hypertension (PH), have been reported to occur following splenectomy for various disorders,including hereditary spherocytosis (HS). We performed a prospective cross-sectional study of 36 adults with HS (78% with prior splenectomy)utilizing echocardiography to estimate tricuspid regurgitant jet velocity (TRV) as well as measurement of N-terminal pro-B-type natriuretic peptide (NT-proBNP) to screen for PH. No participant with HS hada significantly elevated TRV or NT-proBNP level, despite a median 25-year interval since splenectomy (95% confidence interval for point prevalence 0, 0.097). Although our study was limited by a small sample size, it appears that persons with HS, following splenectomy, appear unlikely to be at significantly increased risk of developing PH to the degree reported for thalassemia and sickle cell disease
authors.affiliation: Division of Pediatric Hematology-Oncology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, USA. SECrary@uams.edu
authors.forename: Shelley E
authors.initials: SE
authors.lastname: Crary
authors.forename: Claudio
authors.initials: C
authors.lastname: Ramaciotti
authors.forename: George R
authors.initials: GR
authors.lastname: Buchanan
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003430:Cross-Sectional Studies; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology; D015995:Prevalence; D011446:Prospective Studies; D011498:Protein Precursors / Q000097:blood; D012307:Risk Factors; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000503:physiopathology* / Q000601:surgery; D013156:Splenectomy / Q000009:adverse effects*; D013781:Texas / Q000453:epidemiology; D014261:Tricuspid Valve / Q000000981:diagnostic imaging; D014463:Ultrasonography; D055815:Young Adult
chemical_list: D011498:Protein Precursors; D020097:Natriuretic Peptide, Brain