Clinical features of Behçet's disease in children: An international collaborative study of 86 cases☆,☆☆,★
Section snippets
Patients and Methods
A questionnaire was mailed to five specialists of BD in four different countries including Turkey (two centers: Istanbul and Ankara), Saudi Arabia, Iran, and France. The files of all patients with BD before the age of 16 years were reviewed retrospectively. The questionnaire included 286 items divided into 10 groups including identification, familial history, mucocutaneous, neurologic, psychiatric, ocular, digestive, pulmonary, cardiac, and nephrourologic features. The chronology of these
Epidemiology
Sixty-five patients were reviewed: 33 boys, 32 girls (sex ratio 1.03) from Turkey (37), France (13), Saudi Arabia, (8) and Iran (7). Most of them were white and of Turkish or West European descent. The mean age at onset of the disease was 8.4 years (0 to 16 years) (Fig. 1, A), and the mean age at diagnosis was 13 years (3 to 16 years) (Fig. 1, B). The follow-up was at least 5 years for 32%
Discussion
Although most cases of BD were observed in late childhood (mean 13 years, median 15 years), three patients had the disease during the first year of life. However, we observed no neonatal cases of BD caused by an affected mother.11, 12 Another unexpected finding was the relatively high rate of familial cases (15%) compared with adult BD (2%).3 This is of particular interest in determining the respective roles of genetic and environmental factors that may influence the expression of BD.
The
Acknowledgements
We are grateful to Paul Mandelbröjt for support, to Marie Thérèse Jimeno and Bernard Guisiano for statistics, and to Samia Zouraghi and Elisabeth Vistue for secretarial assistance.
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From the Department of Pediatrics, Hôpital Nord and Hôpital de la Timone, Marseilles, France; Department of Medicine (Division of Rheumatology) Cerrahpasa Medical Faculty, University of Istanbul, Turkey; Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia; the Rheumatology Research Center, Shariaty Hospital, Tehran, Iran; and the Department of Pediatric Nephrology and Rheumatology, Hacettepe University, Ankara, Turkey.
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Reprint requests: Isabelle Koné-Paut, MD, Service de Pédiatrie, Hôpital Nord, chemin des Bourrelys, 13915 Marseilles, France.
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