Article: Use of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease

File Download Links for fulltext
(May Require Subscription)
Supplementary
  • Basic View
  • Metadata View
  • XML View
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
2011 Impact Factor: 4.941
2011 SCImago Journal Rankings: 0.586
DOIhttp://dx.doi.org/10.1111/j.1365-2141.2006.05984.x
ISI Accession Number IDWOS:000236067900011
ReferencesReferences in Scopus
DC Field
Value
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
2011 Impact Factor: 4.941
2011 SCImago Journal Rankings: 0.586
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
Author Affiliations
  1. The University of Hong Kong
  2. Pamela Youde Nethersole Eastern Hospital