Article: A synopsis of current care of thalassaemia major patients in Hong Kong

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TitleA synopsis of current care of thalassaemia major patients in Hong Kong
AuthorsAu, WY8
Lee, V5
Lau, CW2
Yau, J4
Chan, D7
Chan, EYT1
Cheung, WWW8
Ha, SY8
Kho, B6
Lee, CY9
Li, RCH2
Li, CK5
Lin, SY7
Ling, ASC3
Mak, V3
Sun, L6
Wong, KHF3
Wong, R5
Yuen, HL4
Issue Date2011
PublisherHong Kong Academy of Medicine Press. The Journal's web site is located at http://www.hkmj.org.hk
CitationHong Kong Medical Journal, 2011, v. 17 n. 4, p. 261-266 [How to Cite?]
AbstractOBJECTIVE: To provide a synopsis of current thalassaemia major patient care in Hong Kong. DESIGN: Retrospective study. SETTING: All haematology units of the Hospital Authority in Hong Kong. PATIENTS: All patients with thalassaemia major with regular transfusion. RESULTS: To date, there were 363 thalassaemia major patients under the care of the Hospital Authority. Prenatal diagnosis has helped to reduce the number of indigenous new cases, but in recent years immigrant cases are appearing. The patients have a mean age of 23 (range, 1-52) years, and 78% of them are adults. In 2009, they received 18 782 units of blood. This accounted for 9.5% of all blood consumption from the Hong Kong Red Cross. In the past, cardiac iron overload was the major cause of death (65%) and few patients survived beyond the age of 45 years. The availability of cardiac iron assessment by magnetic resonance imaging (T2 MRI) to direct the use of oral deferiprone chelation has reduced the prevalence of heart failure and cardiac haemosiderosis, which should reduce mortality and improve life expectancy. CONCLUSION: The future for thalassaemia care in Hong Kong is bright. With better transfusion and chelation, it should be possible to avoid growth and endocrine deficiencies in younger patients.
DescriptionAuthor Chinese name: WY Au 區永仁, Vincent Lee 李偉生, CW Lau 劉靜華, Jeffrey Yau 丘炳華, Desmond Chan 陳振榮, Eric YT Chan 陳日東, Winnie WW Cheung 張永慧, SY Ha 夏修賢, Bonnie Kho 許紫珊, CY Lee 李靜賢, Rever CH Li 李澤荷, CK Li 李志光, SY Lin 連錫營, Alvin SC Ling 凌紹祥, Vivien Mak 麥慧敏, Lina Sun 孫偉芬, Kris HF Wong 黃鴻勳, Raymond Wong 王紹明, HL Yuen 袁煦樑
ISSN1024-2708
2011 SCImago Journal Rankings: 0.054
DC Field
Value
dc.contributor.authorAu, WY
dc.contributor.authorLee, V
dc.contributor.authorLau, CW
dc.contributor.authorYau, J
dc.contributor.authorChan, D
dc.contributor.authorChan, EYT
dc.contributor.authorCheung, WWW
dc.contributor.authorHa, SY
dc.contributor.authorKho, B
dc.contributor.authorLee, CY
dc.contributor.authorLi, RCH
dc.contributor.authorLi, CK
dc.contributor.authorLin, SY
dc.contributor.authorLing, ASC
dc.contributor.authorMak, V
dc.contributor.authorSun, L
dc.contributor.authorWong, KHF
dc.contributor.authorWong, R
dc.contributor.authorYuen, HL
dc.date.accessioned2012-07-16T09:47:22Z
dc.date.available2012-07-16T09:47:22Z
dc.date.issued2011
dc.description.abstractOBJECTIVE: To provide a synopsis of current thalassaemia major patient care in Hong Kong. DESIGN: Retrospective study. SETTING: All haematology units of the Hospital Authority in Hong Kong. PATIENTS: All patients with thalassaemia major with regular transfusion. RESULTS: To date, there were 363 thalassaemia major patients under the care of the Hospital Authority. Prenatal diagnosis has helped to reduce the number of indigenous new cases, but in recent years immigrant cases are appearing. The patients have a mean age of 23 (range, 1-52) years, and 78% of them are adults. In 2009, they received 18 782 units of blood. This accounted for 9.5% of all blood consumption from the Hong Kong Red Cross. In the past, cardiac iron overload was the major cause of death (65%) and few patients survived beyond the age of 45 years. The availability of cardiac iron assessment by magnetic resonance imaging (T2 MRI) to direct the use of oral deferiprone chelation has reduced the prevalence of heart failure and cardiac haemosiderosis, which should reduce mortality and improve life expectancy. CONCLUSION: The future for thalassaemia care in Hong Kong is bright. With better transfusion and chelation, it should be possible to avoid growth and endocrine deficiencies in younger patients.
dc.description.naturepublished_or_final_version
dc.descriptionAuthor Chinese name: WY Au 區永仁, Vincent Lee 李偉生, CW Lau 劉靜華, Jeffrey Yau 丘炳華, Desmond Chan 陳振榮, Eric YT Chan 陳日東, Winnie WW Cheung 張永慧, SY Ha 夏修賢, Bonnie Kho 許紫珊, CY Lee 李靜賢, Rever CH Li 李澤荷, CK Li 李志光, SY Lin 連錫營, Alvin SC Ling 凌紹祥, Vivien Mak 麥慧敏, Lina Sun 孫偉芬, Kris HF Wong 黃鴻勳, Raymond Wong 王紹明, HL Yuen 袁煦樑
dc.identifier.citationHong Kong Medical Journal, 2011, v. 17 n. 4, p. 261-266 [How to Cite?]
dc.identifier.epage266
dc.identifier.hkuros200825
dc.identifier.issn1024-2708
2011 SCImago Journal Rankings: 0.054
dc.identifier.issue4
dc.identifier.pmid21813892
dc.identifier.scopuseid_2-s2.0-80052925400
dc.identifier.spage261
dc.identifier.urihttp://hdl.handle.net/10722/152728
dc.identifier.volume17
dc.languageeng
dc.publisherHong Kong Academy of Medicine Press. The Journal's web site is located at http://www.hkmj.org.hk
dc.publisher.placeHong Kong
dc.relation.ispartofHong Kong Medical Journal
dc.rightsHong Kong Medical Journal. Copyright © Hong Kong Academy of Medicine Press.
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License
dc.subject.meshErythrocyte Transfusion
dc.subject.meshIron Overload - etiology
dc.subject.meshOsteoporosis - etiology
dc.subject.meshRetrospective Studies
dc.subject.meshThalassemia - complications - mortality - therapy
dc.titleA synopsis of current care of thalassaemia major patients in Hong Kong
dc.typeArticle
Author Affiliations
  1. Kwong Wah Hospital
  2. Tuen Mun Hospital
  3. Princess Margaret Hospital Hong Kong
  4. Queen Elizabeth Hospital Hong Kong
  5. Prince of Wales Hospital Hong Kong
  6. Pamela Youde Nethersole Eastern Hospital
  7. United Christian Hospital Hong Kong
  8. Queen Mary Hospital Hong Kong
  9. Caritas Medical Centre Hong Kong