Document Detail

Risk factors for pulmonary hypertension in patients with β thalassemia intermedia.
MedLine Citation:
PMID:  22075289     Owner:  NLM     Status:  In-Data-Review    
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.
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.
Department of Pediatrics, Thrombosis and Hemostasis Unit, Hematology Research Center, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.
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