Nevoid bazal hücreli karsinom sendromu: (NBCCS): Olgu bildirimi
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2000
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info:eu-repo/semantics/openAccess
Özet
Amaç: Nevoid bazal hücreli karsinom sendromu (NBCCS) otozomal dominant olarak gözlenen ve birçok kompleks düzensizliklerin bir arada gözlendiği kalıtsal bir sendromdur. Nevoid bazal hücreli karsinom sendromu'nun iskeletsel, oftalmolojik, nörolojik, kutanöz ve sekse bağlı birçok değişken bulgu ile ortaya çıktığı kanıtlanmıştır. NBCCS'da gözlenen beş temel bulgu; multipl bazal hücreli kanserler, epitelle döşeli multipl çene kistleri, iskeletsel anomaliler, ektopik kalsifikasyonlar ile ellerde ve ayaklarda gözlenen deformitelerdir. Olgu Raporu: Bu çalışmada NBCCS sendromu tanısı tıbbi konsültasyon isteğinin tarafımızdan yapılması sonucu konmuş ve iki yıldır kontrol altında tutulan 12 yaşında bir erkek hastaya ait olgu sunulmaktadır. Hastaya ait ve sendromun tanısı için gerekli olan tüm biyokimyasal ve endokrinolojik testler tamamlanmış ve değerlendirilmiştir. Bunun yanı sıra, çenelere ait panoramik radyografi, bilgisayarlı aksiyel tomografi ve sintigrafi ile elde edilen radyografik görüntülerin sonuçları yorumlanmıştır. Hastada gözlenen mandibular kistlerin cerrahi eksizyonu sonrası oluşan iyileşmeye ait radyografik görüntüler de sunulmaktadır. Sonuç: Dişhekimleri NBCC sendromuna ait tüm belirti ve bulguları akılda tutarak, çenelerde gözlenen multipl kistlere daha dikkatli ve titiz yaklaşmalıdır.
Purpose: Nevoid basal cell carcinoma (NBCCS) is a hereditary complex of abnormalities transmitted as an autosomal dominant trait with an almost complete penetrance. NBCCS was demonstrated to embrace varying manifestations including sexual, skeletal, ophtalmologic, neurologic and cutaneous anomalies. Hence, the five major features of NBCCS include multiple basal cell carcinomas, ephitelium-lined multiple jaw cysts, skeletal abnormalities, ectopic calcifications; and pitting of the hands and feet. The purpose of this report is to represent a case of NBCCS which was initially diagnosed and treated by dental guidance. Case Report: A case of a 12-year-old boy with NBCCS was presented. The diagnosis of the syndrome was done with the aid of a medical consultation upon our inquiry. All biochemical and endocrinologic tests related to the syndrome were assessed and evaluated. Additionally, a through radiographic examination including panoramic radiograph of the jaws, computerized axial tomographic images and scintigraphic analysis were performed. Healing of the multiple cysts after surgical treatment was presented as well. The boy was under control for two years. Conclusion: Dentists should be aware of all the signs and symptoms of the syndrome and should be very careful about evaluating multiple cysts of the jaws.
Purpose: Nevoid basal cell carcinoma (NBCCS) is a hereditary complex of abnormalities transmitted as an autosomal dominant trait with an almost complete penetrance. NBCCS was demonstrated to embrace varying manifestations including sexual, skeletal, ophtalmologic, neurologic and cutaneous anomalies. Hence, the five major features of NBCCS include multiple basal cell carcinomas, ephitelium-lined multiple jaw cysts, skeletal abnormalities, ectopic calcifications; and pitting of the hands and feet. The purpose of this report is to represent a case of NBCCS which was initially diagnosed and treated by dental guidance. Case Report: A case of a 12-year-old boy with NBCCS was presented. The diagnosis of the syndrome was done with the aid of a medical consultation upon our inquiry. All biochemical and endocrinologic tests related to the syndrome were assessed and evaluated. Additionally, a through radiographic examination including panoramic radiograph of the jaws, computerized axial tomographic images and scintigraphic analysis were performed. Healing of the multiple cysts after surgical treatment was presented as well. The boy was under control for two years. Conclusion: Dentists should be aware of all the signs and symptoms of the syndrome and should be very careful about evaluating multiple cysts of the jaws.
Açıklama
Anahtar Kelimeler
Diş Hekimliği
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Türkiye Klinikleri Diş Hekimliği Bilimleri Dergisi
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Cilt
6
Sayı
1
