Ali YÜCEL, Hatice ERDEM, Şükran TUNALI, Emel Bülbül BAŞKAN, Semra Çıkman TOKER, Hayriye SARICAOĞLU

Pediatrik Behçet hastalarının klinik ve demografik özellikleri - özgün araştırma

Amaç: Bu çalışmada; pediatrik Behçet hastalarının erişkin yaş grubu ile klinik-demografik özelliklerinin karşılaştırılması amaçlandı.Gereç ve Yöntem: 1992-2007 yılları arasında Uludağ Üniversitesi Tıp Fakültesi Dermatoloji AD Behçet Polikliniğinde takip edilen 18 yaş ve altı çocukların klinik-demografik özellikleri retrospektif olarak incelendi. Olguların yaş, cinsiyet, oral aft, genital ülser, eritema nodosum, papülopüstül varlığı; paterji testi pozitifliği; göz, eklem, nörolojik, gastrointestinal sistem ve renal tutulumları ve HLA-B5 pozitifliği ile ilgili bilgiler dosyalarından kayıt edildi. Bulgular: Uluslararası Behçet Çalışma Grubu tanı kriterlerine göre tanı konulduğu tarih itibariyle 0-18 yaş arasında olduğu belirlenen 30 olgu bulunmakta idi. Hastaların yaş ortalaması 15,9 idi. Tanı konulduğu anda olguların büyük kısmı17-18 yaşlarındaydı (%43,3). On yaşın altında sadece 1 olgu vardı. Oral aft olguların tamamında, genital ülser ya da skarı 15’i kız 8’i erkek toplam 23 hastada saptandı (%76,6). Göz tutulumu 8’i kız 10’u erkek 18 hastada (%60); eritema nodosum 8’i kız 5’i erkek 13 hastada (%43,3), papülopüstül 10’u kız 6’sı erkek 16 hastada (%53,3), paterji pozitifliği 7’si kız 5’i erkek 12 hastada (%36,6) vardı. Vasküler semptomlar 2 hastada, artralji-artrit 15 hastada, nörolojik tutulum 4 hastada saptandı. Sonuç: Bu çalışmada, 0-18 yaş grubu Behçet hastalarının göz tutulumunun erişkin yaş grubundan daha sık olduğu, ancak diğer mukokutanöz bulguların benzer oranlarda görüldüğü sonucuna varıldı.

The clinical and demographic characteristics of pediatric Behçet’s patients

Aim: In this study, we aimed to compare the clinical and demographic features of pediatric Behçet’s patients with those of adult age. Materials and Method: We examined the clinical and demographic features of the 18 and under 18-year-old children who were followed-up in the Behçet's Center of Uludağ University Medical Faculty between 1992 and 2007, retrospectively. The information of age, sex, presence of oral aphthous ulcers, genital ulcers, erythema nodosum, papulopustular eruption, pathergy test positivity, ocular, articular, neurological, gastrointestinal, renal involvements and HLA-B5 positivity were obtained from the medical charts of the patients. Results: There were 30 pediatric Behçet diagnosed according to the diagnostic criteria of International Study Group for Behçet's Disease. The mean age of the patients was 15.9. At the time of the diagnosis, most of the patients were aged between 17-18 (43.3%). There was only one patient under 10. Oral aphthae was observed in all, and genital ulceration or the scar of the ulcer was observed in 23 patients; 15 females, 8 males (76.6%). Ocular involvement was present in 18 patients; 8 females, 10 males (60%); erythema nodosum in 13 patients; 8 females, 5 males (43.3%), papulopustular eruption in 16 patients; 10 females, 6 males and pathergy test positivity in 12 patients; 7 females, 5 males (36.6%). Vascular symptoms were observed in 2 patients, arthralgia-arthritis in 15 patients and neurological involvement in 4 patients. Conclusion: In this study, it was concluded that ocular involvement was more frequent in Behçet’s patients aged between 0-18 than adult Behçet’s patients, but mucocutaneous findings had similar frequency

PDF

___

1. Behçet H. Über rezidivierende aphthose, durch ein virus verursachte geschwüre am mund am auge und an den genitalien. Dermatol Wochenschr 1937; 36: 1152-7.
2. Yazıcı H, Tüzün Y, Pazarlı H, et al. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s disease. Ann Rheum Dis 1984; 43:783-9.
3. Kone-Paut I, Yurdakul S, Bahabri SA, et al. Clinical features of Behçet’s disease in children: an international collaborative study of 86 cases. J Pediatr 1998; 132: 721-5.
4. Pivetti-Pezzi P, Accorinti M, Abdulaziz MA, La Cava M, Torella M, Riso D. Behçet’s disease in children. Jpn J Ophthalmol 1995; 39: 309-14.
5. Krause I, Uziel Y, Guedj D, et al. Childhood Behcet’s disease:clinical features and comparison with adult-onset disease. Rheumatology (Oxford) 1999;38: 457-62.
6. Mundy TM, Miller JJ. Behcet’s Disease Presenting as Chronic Aphthous Stomatitis in a Child. Pediatrics 1978; 62: 205-8.
7. Hafner R. Das Bekcet-Syndrom In Kindesalter. Z Rheumatol 1987; 46: 174-8.
8. International Study Group for Behcet’s Disease: Criteria for he Diagnosis of Behcet’s Disease. Lancet 1990; 335: 1078- 80.
9. Amman AJ, Johnson A, Fyfe G, Leonards R, Wara DW, Cowan MJ. Behcet’s Syndrom. J Pediatr 1985; 107: 41-3.
10. Kone-Paut I, Gorchakoff-Molinas A, Weschler B, Touitou I. Pediatric Behçet’s disease in France. Ann Rheum Dis 2002; 61: 655-6.
11. Shahram F, Davatchi F, Nadji A, et al. Recent epidemiological Recent epidemiological data on Behçet's disease in Iran. Adv Exp Med Biol 2003; 528: 31-6.
12. Bang D, Han Yoon K, Chung HG, Cho EU, Lee S. Epidemiological and clinical features of Behçet’s disease in Korea. Yonsei Med J 1997; 38: 428-36.
13. Yurdakul S, Özdoğan V, Moral F, et al. Behcet’s Syndrome in Childhood. A report of 44 patients. 6th International Conferance on Behcet’s Disease. Paris: Editions Scientifiques Elsevier, 1993:38.
14. Erdi H, Gürler A. Jüvenil Behçet Hastalarının Klinik Özellikler. Turk J Dermatol 1994; 4: 75-80.
15. Hamza M. Juvenile Behcet’s Disease in Tunisia. 6th International Conferance on Behcet’s Disease. Paris: Editions Scientifiques Elsevier, 1993: 39.
16. Laghmari M, Karim A, Allali F, et al. Childhood Behçet’s disease: clinical and evolutive aspect. About 13 cases. J Fr Ophtalmol 2002; 25: 904-8.
17. Kari JA, Sahah V, Dillon MJ. Behçet’s disease in UK children: clinical features and treatment including thalidomide. Rheumatology 2001; 40: 933-8.
18. Lang BA, Laxer RM, Thorner P, Greenberg M, Sylverman ED. Pediatric onset of Behçet’s syndrome with myositis: case report and literature review illustrating unusual features. Arthritis Rheum 1990;33: 418-42.
19. Eldem B, Onur C, Özen S. Clinical features of pediatric Behcet’s disease. J Pediatr Ophthalmol Strabismus 1998; 35: 159-61.