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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, ZQ2
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
2013 Impact Factor: 0.106
2013 SCImago Journal Rankings: 0.126
 
ReferencesReferences in Scopus
 
DC FieldValue
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
2013 Impact Factor: 0.106
2013 SCImago Journal Rankings: 0.126
 
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
 
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<contributor.author>Cheuk, DKL</contributor.author>
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<contributor.author>Ha, SY</contributor.author>
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<description.abstract>The 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.</description.abstract>
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Author Affiliations
  1. The University of Hong Kong
  2. Union Hospital