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Genetic anticipation in Behçet's syndrome
Yavuz,c
Z Tümer,d
H Yazicia
a Department of Medicine, Division
of Rheumatology, Cerrahpa
a Medical School, Istanbul, Turkey, b Department of Rheumatology, Çukurova Medical School,
Turkey, c Department of Rheumatology, Marmara
Medical School, Turkey, d Department of Medical Genetics, Panum Institute,
University of Copenhagen, Denmark
Correspondence to: Professor H Yazici, Safa Sok 17/4, Kadiköy, Istanbul, 81310, Turkey.
Accepted for publication 22 October 1997
OBJECTIVE
To examine the presence of genetic
anticipation in families with Behçet's syndrome (BS).
METHODSA total of 18 families with 40 affected
members in two successive generations were evaluated by interviewing
them for their ages at the onset of the first symptom of BS and for
their ages at the time they fulfilled the diagnostic criteria.
RESULTSIt was noted that the age of onset of the
first symptom was lower in the second generation in 14 families
(p=0.01) with a mean (SD) age of 20.57 (7.47) years in the children
compared with 33.29 (9.92) years in the parents (t=7.79,
p<0.0001), whereas the diagnostic criteria were fulfilled at an
earlier age in the children in 15 families (p=0.01) with a mean age of
21.2 (6.74) years in the children compared with 36.4 (9.55) years in
the parents (t=7.41, p<0.0001).
CONCLUSIONGenetic anticipation was present in 15 of 18 (84%) of the families with BS in the form of earlier disease
onset in the children compared with their parents.
© 1998 by Annals of the Rheumatic Diseases
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