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Article: Use of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease
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TitleUse of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease
 
AuthorsChan, JCW2
Chim, CS1
Ooi, CGC1
Cheung, B1
Liang, R1
Chan, TK1
Chan, V1
 
Issue Date2006
 
PublisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/BJH
 
CitationBritish Journal Of Haematology, 2006, v. 133 n. 2, p. 198-205 [How to Cite?]
DOI: http://dx.doi.org/10.1111/j.1365-2141.2006.05984.x
 
AbstractSeventeen non-transfusion-dependent Chinese haemoglobin H (Hb H) disease patients (age 29-76 years) with serum ferritin >900 μg/l were treated with deferiprone for up to 18 months. One patient withdrew and data from 16 patients were analysed. Sixteen other Hb H patients with ferritin <900 μg/l, matched for age and genotype, acted as controls. Treatment was well tolerated except for mild arthralgia. Serum ferritin fell with treatment, reaching significance at 6 and 18 months (from 1492·3 ± 901·4 to 519·4 ± 405·4 μg/l at 18 months, P = 0·0008). Nine of 16 patients had levels below 397 μg/l before 18 months. Serum ferritin remained stable 6 months after stopping treatment. In contrast, there was no change in ferritin levels in the control group. Magnetic resonance imaging was used for measurement of liver iron content. Spin echo T 1-signal intensity ratio (T1-SIR) and gradient echo T 2-signal intensity ratio (T2-SIR) increased with treatment. T2-SIR rose from 0·17 ± 0·08 pretreatment to 0·58 ± 0·50 at 2 years (P = 0·0055). Improvement occurred in 12 of 16 patients, reaching normal in three patients. Using echocardiography, peak early diastolic : late diastolic blood flow (E/A) remained unchanged with treatment, but isovolumic relaxation time (IVRT) was prolonged at 2 years indicating mild impairment of diastolic function. All systolic function parameters were normal. A longer treatment period is desirable to demonstrate improvement in cardiac function. © 2006 Blackwell Publishing Ltd.
 
ISSN0007-1048
2013 Impact Factor: 4.959
 
DOIhttp://dx.doi.org/10.1111/j.1365-2141.2006.05984.x
 
ISI Accession Number IDWOS:000236067900011
 
ReferencesReferences in Scopus
 
DC FieldValue
dc.contributor.authorChan, JCW
 
dc.contributor.authorChim, CS
 
dc.contributor.authorOoi, CGC
 
dc.contributor.authorCheung, B
 
dc.contributor.authorLiang, R
 
dc.contributor.authorChan, TK
 
dc.contributor.authorChan, V
 
dc.date.accessioned2010-09-06T07:28:04Z
 
dc.date.available2010-09-06T07:28:04Z
 
dc.date.issued2006
 
dc.description.abstractSeventeen non-transfusion-dependent Chinese haemoglobin H (Hb H) disease patients (age 29-76 years) with serum ferritin >900 μg/l were treated with deferiprone for up to 18 months. One patient withdrew and data from 16 patients were analysed. Sixteen other Hb H patients with ferritin <900 μg/l, matched for age and genotype, acted as controls. Treatment was well tolerated except for mild arthralgia. Serum ferritin fell with treatment, reaching significance at 6 and 18 months (from 1492·3 ± 901·4 to 519·4 ± 405·4 μg/l at 18 months, P = 0·0008). Nine of 16 patients had levels below 397 μg/l before 18 months. Serum ferritin remained stable 6 months after stopping treatment. In contrast, there was no change in ferritin levels in the control group. Magnetic resonance imaging was used for measurement of liver iron content. Spin echo T 1-signal intensity ratio (T1-SIR) and gradient echo T 2-signal intensity ratio (T2-SIR) increased with treatment. T2-SIR rose from 0·17 ± 0·08 pretreatment to 0·58 ± 0·50 at 2 years (P = 0·0055). Improvement occurred in 12 of 16 patients, reaching normal in three patients. Using echocardiography, peak early diastolic : late diastolic blood flow (E/A) remained unchanged with treatment, but isovolumic relaxation time (IVRT) was prolonged at 2 years indicating mild impairment of diastolic function. All systolic function parameters were normal. A longer treatment period is desirable to demonstrate improvement in cardiac function. © 2006 Blackwell Publishing Ltd.
 
dc.description.natureLink_to_subscribed_fulltext
 
dc.identifier.citationBritish Journal Of Haematology, 2006, v. 133 n. 2, p. 198-205 [How to Cite?]
DOI: http://dx.doi.org/10.1111/j.1365-2141.2006.05984.x
 
dc.identifier.citeulike557600
 
dc.identifier.doihttp://dx.doi.org/10.1111/j.1365-2141.2006.05984.x
 
dc.identifier.epage205
 
dc.identifier.hkuros121953
 
dc.identifier.isiWOS:000236067900011
 
dc.identifier.issn0007-1048
2013 Impact Factor: 4.959
 
dc.identifier.issue2
 
dc.identifier.openurl
 
dc.identifier.pmid16611312
 
dc.identifier.scopuseid_2-s2.0-33645078392
 
dc.identifier.spage198
 
dc.identifier.urihttp://hdl.handle.net/10722/77082
 
dc.identifier.volume133
 
dc.languageeng
 
dc.publisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/BJH
 
dc.publisher.placeUnited Kingdom
 
dc.relation.ispartofBritish Journal of Haematology
 
dc.relation.referencesReferences in Scopus
 
dc.rightsBritish Journal of Haematology. Copyright © Blackwell Publishing Ltd.
 
dc.subject.meshAdministration, Oral
 
dc.subject.meshAdult
 
dc.subject.meshAged
 
dc.subject.meshDiastole
 
dc.subject.meshEchocardiography, Doppler, Pulsed
 
dc.subject.meshFemale
 
dc.subject.meshFerritins - blood
 
dc.subject.meshHumans
 
dc.subject.meshIron - metabolism
 
dc.subject.meshIron Chelating Agents - adverse effects - therapeutic use
 
dc.subject.meshIron Overload - drug therapy - etiology - physiopathology - ultrasonography
 
dc.subject.meshLiver - metabolism
 
dc.subject.meshMagnetic Resonance Imaging - methods
 
dc.subject.meshMale
 
dc.subject.meshMiddle Aged
 
dc.subject.meshPyridones - adverse effects - therapeutic use
 
dc.subject.meshVentricular Function, Left
 
dc.subject.meshalpha-Thalassemia - complications - physiopathology
 
dc.titleUse of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease
 
dc.typeArticle
 
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Author Affiliations
  1. The University of Hong Kong
  2. Pamela Youde Nethersole Eastern Hospital