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

TitleThe clinical features of chinese children with von willebrand disease: The experience of a tertiary institute
Authors
KeywordsChinese children
Epidemiology
Von willebrand disease
Issue Date2011
PublisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
Citation
Hong 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.
Persistent Identifierhttp://hdl.handle.net/10722/135323
ISSN
2014 Impact Factor: 0.133
2014 SCImago Journal Rankings: 0.126
References

 

DC FieldValueLanguage
dc.contributor.authorZhang, ZQen_HK
dc.contributor.authorChan, GCFen_HK
dc.contributor.authorLam, CCKen_HK
dc.contributor.authorSo, JCCen_HK
dc.contributor.authorCheuk, DKLen_HK
dc.contributor.authorChiang, AKSen_HK
dc.contributor.authorHa, SYen_HK
dc.date.accessioned2011-07-27T01:33:36Z-
dc.date.available2011-07-27T01:33:36Z-
dc.date.issued2011en_HK
dc.identifier.citationHong Kong Journal Of Paediatrics, 2011, v. 16 n. 2, p. 95-100en_HK
dc.identifier.issn1013-9923en_HK
dc.identifier.urihttp://hdl.handle.net/10722/135323-
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.en_HK
dc.languageengen_US
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.rightsDOAJen_US
dc.subjectChinese childrenen_HK
dc.subjectEpidemiologyen_HK
dc.subjectVon willebrand diseaseen_HK
dc.titleThe clinical features of chinese children with von willebrand disease: The experience of a tertiary instituteen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=1013-9923&volume=16&spage=95&epage=100&date=2011&atitle=The+clinical+features+of+Chinese+children+with+von+Willebrand+disease:+The+experience+of+a+tertiary+instituteen_US
dc.identifier.emailChan, GCF:gcfchan@hkucc.hku.hken_HK
dc.identifier.emailSo, JCC:scc@pathology.hku.hken_HK
dc.identifier.emailChiang, AKS:chiangak@hkucc.hku.hken_HK
dc.identifier.authorityChan, GCF=rp00431en_HK
dc.identifier.authoritySo, JCC=rp00391en_HK
dc.identifier.authorityChiang, AKS=rp00403en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.scopuseid_2-s2.0-79955424269en_HK
dc.identifier.hkuros191935en_US
dc.identifier.hkuros186672-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-79955424269&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume16en_HK
dc.identifier.issue2en_HK
dc.identifier.spage95en_HK
dc.identifier.epage100en_HK
dc.publisher.placeHong Kongen_HK
dc.identifier.scopusauthoridZhang, ZQ=37862374500en_HK
dc.identifier.scopusauthoridChan, GCF=16160154400en_HK
dc.identifier.scopusauthoridLam, CCK=16947291300en_HK
dc.identifier.scopusauthoridSo, JCC=7102919978en_HK
dc.identifier.scopusauthoridCheuk, DKL=8705936100en_HK
dc.identifier.scopusauthoridChiang, AKS=7101623534en_HK
dc.identifier.scopusauthoridHa, SY=7202501115en_HK

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