Document Detail


Risk factors for pulmonary hypertension in patients with β thalassemia intermedia.
MedLine Citation:
PMID:  22075289     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
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).
CONCLUSION: 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:
Mehran Karimi; Khaled M Musallam; Maria Domenica Cappellini; Shahina Daar; Amal El-Beshlawy; Khawla Belhoul; Mohamed-Salaheldin Saned; Sally Temraz; Suzanne Koussa; Ali T Taher
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Publication Detail:
Type:  Journal Article     Date:  2011-06-29
Journal Detail:
Title:  European journal of internal medicine     Volume:  22     ISSN:  1879-0828     ISO Abbreviation:  Eur. J. Intern. Med.     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-11-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9003220     Medline TA:  Eur J Intern Med     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  607-10     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
Affiliation:
Department of Pediatrics, Thrombosis and Hemostasis Unit, Hematology Research Center, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
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