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Epidemiological investigation of the prevalence of von Willebrand's disease
F Rodeghiero, G Castaman and E Dini
To evaluate the prevalence of von Willebrand's disease (vWd) we carried out
an epidemiological investigation among school children of the Veneto region
in northern Italy. A total of 1,218 of 1,281 possible children participated
in the study. They were 11 to 14 years of age, and all attended secondary
schools in two distinct small areas, 70 km apart, between which there is no
social contact. A blood sample was taken from each subject for
determination of the blood group and von Willebrand factor (vWf) level
(measured as ristocetin cofactor and expressed in IU/dL after calibration
of the internal pool against an international standard), and the parents
were given a questionnaire concerning hemorrhagic symptoms in the members
of the family in the last three generations. Separate normal ranges were
calculated for blood group O and non-O subjects (1,166 children and 289
adults) with a nonparametric method because the distribution curves of the
reference values did not fit the gaussian distribution. Diagnoses of vWd
were considered only for children who had low vWf levels and were members
of a family with a convincing bleeding history (case of "probable vWd"). A
final diagnosis was assigned if, in addition to these criteria, at least
one other family member on the side with hemorrhagic history had a low vWf
level. Of the 1,218 children examined, ten were classified as having vWd
(0.82%). Taking into account the 90% confidence interval for the lower
limit of the normal range, this figure could range from 7 (0.57%) to 14
(1.15%). All these subjects were mildly to moderately affected and
presented features of heterozygous classic vWd (type I). Affected subjects
were distributed evenly in the two areas examined. Our results suggest that
the prevalence of vWd might be much higher than previously reported and
that a different screening approach might be of use for patients with mild
bleeding diathesis.
Volume 69,
Issue 2,
pp. 454-459,
02/01/1987
Copyright © 1987 by The American Society of Hematology
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