Article: The clinical features of chinese children with von willebrand disease: The experience of a tertiary institute

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TitleThe clinical features of chinese children with von willebrand disease: The experience of a tertiary institute
AuthorsZhang, ZQ1
Chan, GCF1
Lam, CCK1
So, JCC1
Cheuk, DKL1
Chiang, AKS1
Ha, SY1
KeywordsChinese children
Epidemiology
Von willebrand disease
Issue Date2011
PublisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
CitationHong Kong Journal Of Paediatrics, 2011, v. 16 n. 2, p. 95-100 [How to Cite?]
AbstractThe information related to the clinical spectrum of von Willebranddisease (VWD) in Chinese patientsremains very limited. We conducted a retrospective chart review on the clinical and haematological features of VWD among Chinese patients at a tertiary paediatric centre in Hong Kong. Ten patients (6 females, 4 males) were diagnosed to have VWD from 1989 to 2005. They underwent treatment in our unit, with a cumulative follow up of 102 patient-years within this 16-year period. Among them, 4 were type 1, 5 were type 2 and 1 was type 3 VWD. Six of the 10 patients had a positive family history of bleeding tendencies. A variety of bleeding manifestations were observed in these patients while mucocutaneous bleeds in the form of frequent epistaxis and easy bruising were the commonest presenting features. Severe bleeding in the form of intracranial haemorrhage occurred in 2 patients. Eight patients underwent desmopressin (DDAVP) test at diagnosis and all were responsive to DDAVP without associated thrombocytopenia. Three patients required frequent DDAVP (intravenous or subcutaneous) and 2 required occasional intermediate purity factor VIII concentrate for bleeding control. In conclusion, majority of Chinese paediatric VWD patients are inherited and acquired form is extremely rare in childhood. Patients with either type 1 or 2 VWD can develop severe bleeding in childhood. In our patient cohort, DDAVP appears to be effective and safe for our patients with either type 1 VWD or non-2B type 2 VWD without inducing thrombocytopenia.
DescriptionThe article can be viewed at http://www.hkjpaed.org/pdf/2011;16;95-100.pdf
ISSN1013-9923
2011 Impact Factor: 0.027
2011 SCImago Journal Rankings: 0.029
ReferencesReferences in Scopus
DC Field
Value
dc.contributor.authorZhang, ZQ
dc.contributor.authorChan, GCF
dc.contributor.authorLam, CCK
dc.contributor.authorSo, JCC
dc.contributor.authorCheuk, DKL
dc.contributor.authorChiang, AKS
dc.contributor.authorHa, SY
dc.date.accessioned2011-07-27T01:33:36Z
dc.date.available2011-07-27T01:33:36Z
dc.date.issued2011
dc.description.abstractThe information related to the clinical spectrum of von Willebranddisease (VWD) in Chinese patientsremains very limited. We conducted a retrospective chart review on the clinical and haematological features of VWD among Chinese patients at a tertiary paediatric centre in Hong Kong. Ten patients (6 females, 4 males) were diagnosed to have VWD from 1989 to 2005. They underwent treatment in our unit, with a cumulative follow up of 102 patient-years within this 16-year period. Among them, 4 were type 1, 5 were type 2 and 1 was type 3 VWD. Six of the 10 patients had a positive family history of bleeding tendencies. A variety of bleeding manifestations were observed in these patients while mucocutaneous bleeds in the form of frequent epistaxis and easy bruising were the commonest presenting features. Severe bleeding in the form of intracranial haemorrhage occurred in 2 patients. Eight patients underwent desmopressin (DDAVP) test at diagnosis and all were responsive to DDAVP without associated thrombocytopenia. Three patients required frequent DDAVP (intravenous or subcutaneous) and 2 required occasional intermediate purity factor VIII concentrate for bleeding control. In conclusion, majority of Chinese paediatric VWD patients are inherited and acquired form is extremely rare in childhood. Patients with either type 1 or 2 VWD can develop severe bleeding in childhood. In our patient cohort, DDAVP appears to be effective and safe for our patients with either type 1 VWD or non-2B type 2 VWD without inducing thrombocytopenia.
dc.description.naturelink_to_subscribed_fulltext
dc.descriptionThe article can be viewed at http://www.hkjpaed.org/pdf/2011;16;95-100.pdf
dc.identifier.citationHong Kong Journal Of Paediatrics, 2011, v. 16 n. 2, p. 95-100 [How to Cite?]
dc.identifier.epage100
dc.identifier.hkuros191935
dc.identifier.hkuros186672
dc.identifier.issn1013-9923
2011 Impact Factor: 0.027
2011 SCImago Journal Rankings: 0.029
dc.identifier.issue2
dc.identifier.openurl
dc.identifier.scopuseid_2-s2.0-79955424269
dc.identifier.spage95
dc.identifier.urihttp://hdl.handle.net/10722/135323
dc.identifier.volume16
dc.languageeng
dc.publisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
dc.publisher.placeHong Kong
dc.relation.ispartofHong Kong Journal of Paediatrics
dc.relation.referencesReferences in Scopus
dc.rightsDOAJ
dc.subjectChinese children
dc.subjectEpidemiology
dc.subjectVon willebrand disease
dc.titleThe clinical features of chinese children with von willebrand disease: The experience of a tertiary institute
dc.typeArticle
Author Affiliations
  1. The University of Hong Kong