File Download

There are no files associated with this item.

  Links for fulltext
     (May Require Subscription)
Supplementary

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

TitleUse of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease
Authors
Issue Date2006
PublisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/BJH
Citation
British Journal Of Haematology, 2006, v. 133 n. 2, p. 198-205 How to Cite?
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.
Persistent Identifierhttp://hdl.handle.net/10722/77082
ISSN
2014 Impact Factor: 4.711
2014 SCImago Journal Rankings: 1.768
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorChan, JCWen_HK
dc.contributor.authorChim, CSen_HK
dc.contributor.authorOoi, CGCen_HK
dc.contributor.authorCheung, Ben_HK
dc.contributor.authorLiang, Ren_HK
dc.contributor.authorChan, TKen_HK
dc.contributor.authorChan, Ven_HK
dc.date.accessioned2010-09-06T07:28:04Z-
dc.date.available2010-09-06T07:28:04Z-
dc.date.issued2006en_HK
dc.identifier.citationBritish Journal Of Haematology, 2006, v. 133 n. 2, p. 198-205en_HK
dc.identifier.issn0007-1048en_HK
dc.identifier.urihttp://hdl.handle.net/10722/77082-
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.en_HK
dc.languageengen_HK
dc.publisherBlackwell Publishing Ltd. The Journal's web site is located at http://www.blackwellpublishing.com/journals/BJHen_HK
dc.relation.ispartofBritish Journal of Haematologyen_HK
dc.rightsBritish Journal of Haematology. Copyright © Blackwell Publishing Ltd.en_HK
dc.subject.meshAdministration, Oralen_HK
dc.subject.meshAdulten_HK
dc.subject.meshAgeden_HK
dc.subject.meshDiastoleen_HK
dc.subject.meshEchocardiography, Doppler, Pulseden_HK
dc.subject.meshFemaleen_HK
dc.subject.meshFerritins - blooden_HK
dc.subject.meshHumansen_HK
dc.subject.meshIron - metabolismen_HK
dc.subject.meshIron Chelating Agents - adverse effects - therapeutic useen_HK
dc.subject.meshIron Overload - drug therapy - etiology - physiopathology - ultrasonographyen_HK
dc.subject.meshLiver - metabolismen_HK
dc.subject.meshMagnetic Resonance Imaging - methodsen_HK
dc.subject.meshMaleen_HK
dc.subject.meshMiddle Ageden_HK
dc.subject.meshPyridones - adverse effects - therapeutic useen_HK
dc.subject.meshVentricular Function, Leften_HK
dc.subject.meshalpha-Thalassemia - complications - physiopathologyen_HK
dc.titleUse of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H diseaseen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0007-1048&volume=133&issue=2&spage=198&epage=205&date=2006&atitle=Use+of+the+oral+chelator+deferiprone+in+the+treatment+of+iron+overload+in+patients+with+Hb+H+diseaseen_HK
dc.identifier.emailChim, CS:jcschim@hku.hken_HK
dc.identifier.emailCheung, B:mycheung@hku.hken_HK
dc.identifier.emailLiang, R:rliang@hku.hken_HK
dc.identifier.emailChan, V:vnychana@hkucc.hku.hken_HK
dc.identifier.authorityChim, CS=rp00408en_HK
dc.identifier.authorityCheung, B=rp01321en_HK
dc.identifier.authorityLiang, R=rp00345en_HK
dc.identifier.authorityChan, V=rp00320en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1111/j.1365-2141.2006.05984.xen_HK
dc.identifier.pmid16611312en_HK
dc.identifier.scopuseid_2-s2.0-33645078392en_HK
dc.identifier.hkuros121953en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-33645078392&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume133en_HK
dc.identifier.issue2en_HK
dc.identifier.spage198en_HK
dc.identifier.epage205en_HK
dc.identifier.isiWOS:000236067900011-
dc.publisher.placeUnited Kingdomen_HK
dc.identifier.scopusauthoridChan, JCW=9940606800en_HK
dc.identifier.scopusauthoridChim, CS=7004597253en_HK
dc.identifier.scopusauthoridOoi, CGC=7007084909en_HK
dc.identifier.scopusauthoridCheung, B=7103294806en_HK
dc.identifier.scopusauthoridLiang, R=26643224900en_HK
dc.identifier.scopusauthoridChan, TK=7402687762en_HK
dc.identifier.scopusauthoridChan, V=7202654865en_HK
dc.identifier.citeulike557600-

Export via OAI-PMH Interface in XML Formats


OR


Export to Other Non-XML Formats