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Article: Haematopoietic stem cell transplant for Wiskott-Aldrich syndrome

TitleHaematopoietic stem cell transplant for Wiskott-Aldrich syndrome
Authors
KeywordsChinese
Haematopoietic stem cell transplant
Immunodeficiency
Wiskott-Aldrich syndrome
Issue Date2006
PublisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
Citation
Hong Kong Journal Of Paediatrics, 2006, v. 11 n. 3, p. 186-190+263 How to Cite?
AbstractWe reviewed retrospectively 7 Chinese children diagnosed with Wiskott-Aldrich syndrome (WAS) and managed at the Department of Paediatrics & Adolescent Medicine of Queen Mary Hospital from 1988 to 2005. All patients presented with the classical triad of bleeding tendency, recurrent infections and infantile eczema from neonatal period to 2-3 months of age. The median lag time between diagnosis and presentation was 7 months. Thrombocytopenia and small platelet volume were consistent findings and present in all patients. Findings in immunoglobulin level, lymphocyte subset and lymphocyte proliferative studies were heterogeneous. Four mutations were found in 5 (2 cousins shared the same mutation). Haematopoietic stem cell transplant (HSCT) had been performed for all patients. All 7 had complete immune reconstitution with no major long-term complications in a median follow-up of 9.3 years. Early diagnosis and selection of appropriate donor for HSCT were important strategies for improved survival of patients with WAS.
Persistent Identifierhttp://hdl.handle.net/10722/80035
ISSN
2015 Impact Factor: 0.194
2015 SCImago Journal Rankings: 0.123
References

 

DC FieldValueLanguage
dc.contributor.authorChung, BHYen_HK
dc.contributor.authorLee, TLen_HK
dc.contributor.authorChan, CFen_HK
dc.contributor.authorChiang, Aen_HK
dc.contributor.authorChan, KWen_HK
dc.contributor.authorHa, SYen_HK
dc.contributor.authorLau, YLen_HK
dc.date.accessioned2010-09-06T08:01:37Z-
dc.date.available2010-09-06T08:01:37Z-
dc.date.issued2006en_HK
dc.identifier.citationHong Kong Journal Of Paediatrics, 2006, v. 11 n. 3, p. 186-190+263en_HK
dc.identifier.issn1013-9923en_HK
dc.identifier.urihttp://hdl.handle.net/10722/80035-
dc.description.abstractWe reviewed retrospectively 7 Chinese children diagnosed with Wiskott-Aldrich syndrome (WAS) and managed at the Department of Paediatrics & Adolescent Medicine of Queen Mary Hospital from 1988 to 2005. All patients presented with the classical triad of bleeding tendency, recurrent infections and infantile eczema from neonatal period to 2-3 months of age. The median lag time between diagnosis and presentation was 7 months. Thrombocytopenia and small platelet volume were consistent findings and present in all patients. Findings in immunoglobulin level, lymphocyte subset and lymphocyte proliferative studies were heterogeneous. Four mutations were found in 5 (2 cousins shared the same mutation). Haematopoietic stem cell transplant (HSCT) had been performed for all patients. All 7 had complete immune reconstitution with no major long-term complications in a median follow-up of 9.3 years. Early diagnosis and selection of appropriate donor for HSCT were important strategies for improved survival of patients with WAS.en_HK
dc.languageengen_HK
dc.publisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.aspen_HK
dc.relation.ispartofHong Kong Journal of Paediatricsen_HK
dc.subjectChineseen_HK
dc.subjectHaematopoietic stem cell transplanten_HK
dc.subjectImmunodeficiencyen_HK
dc.subjectWiskott-Aldrich syndromeen_HK
dc.titleHaematopoietic stem cell transplant for Wiskott-Aldrich syndromeen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1013-9923&volume=11&spage=186&epage=190&date=2006&atitle=Haematopoietic+stem+cell+transplant+for+Wiskott-Aldrich+Syndromeen_HK
dc.identifier.emailChung, BHY:bhychung@hku.hken_HK
dc.identifier.emailChiang, A:chiangak@hkucc.hku.hken_HK
dc.identifier.emailLau, YL:lauylung@hkucc.hku.hken_HK
dc.identifier.authorityChung, BHY=rp00473en_HK
dc.identifier.authorityChiang, A=rp00403en_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.scopuseid_2-s2.0-33746366793en_HK
dc.identifier.hkuros120585en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-33746366793&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume11en_HK
dc.identifier.issue3en_HK
dc.identifier.spage186en_HK
dc.identifier.epage190+263en_HK
dc.publisher.placeHong Kongen_HK
dc.identifier.scopusauthoridChung, BHY=7203043997en_HK
dc.identifier.scopusauthoridLee, TL=24483772800en_HK
dc.identifier.scopusauthoridChan, CF=36984590800en_HK
dc.identifier.scopusauthoridChiang, A=7101623534en_HK
dc.identifier.scopusauthoridChan, KW=8587755300en_HK
dc.identifier.scopusauthoridHa, SY=7202501115en_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK

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