title: Risk factors for pulmonary hypertension in patients with β thalassemia intermedia.
abstract: BACKGROUND
  Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI).
  
  
  METHODS
  We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I.
  
  
  RESULTS
  The mean age of Group I patients at development of PHT was 37.3±10.6years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≥300×10(6)/l (2.59-times) or a previous history of thromboembolic events (3.69-times).
  
  
  CONCLUSIONS
  TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi
authors.forename: Khaled M
authors.initials: KM
authors.lastname: Musallam
authors.forename: Maria Domenica
authors.initials: MD
authors.lastname: Cappellini
authors.forename: Shahina
authors.initials: S
authors.lastname: Daar
authors.forename: Amal
authors.initials: A
authors.lastname: El-Beshlawy
authors.forename: Khawla
authors.initials: K
authors.lastname: Belhoul
authors.forename: Mohamed-Salaheldin
authors.initials: MS
authors.lastname: Saned
authors.forename: Sally
authors.initials: S
authors.lastname: Temraz
authors.forename: Suzanne
authors.initials: S
authors.lastname: Koussa
authors.forename: Ali T
authors.initials: AT
authors.lastname: Taher
mesh_terms: D000328:Adult; D001777:Blood Coagulation; D004906:Erythrocyte Count; D005260:Female; D005293:Ferritins / Q000097:blood; D005838:Genotype; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000000981:diagnostic imaging / Q000453:epidemiology*; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D013156:Splenectomy / Q000706:statistics & numerical data; D013923:Thromboembolism / Q000453:epidemiology; D014463:Ultrasonography; D017086:beta-Thalassemia / Q000097:blood* / Q000453:epidemiology* / Q000235:genetics / Q000601:surgery
chemical_list: D005293:Ferritins