Percorrer por autor "Kammoun, Kais"
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- The oral-facial manifestations of Gorlin syndromePublication . Kammoun, Kais; Furtado, José ManuelThe Gorlin syndrome, also known as Nevoid Basal Cell Carcinoma Syndrome (NBCCS) or Gorlin-Goltz Syndrome (OMIM #109400), is an autosomal dominant genetic disorder primarily associated with mutations in the PTCH1 gene. Nevoid basal cell carcinoma syndrome is characterized by numerous basal cell tumors and epidermal cysts of the skin, calcified falx cerebri, keratocysts of the jaws, palmar and plantar pits, ovarian fibromas, medulloblastomas, mesenteric lymph node cysts, fetal rhabdomyoma, rib and vertebral malformations, cleft lip or palate, and cortical defects of the bones. OBJECTIVE The main objective of this thesis will be to conduct an updated and comprehensive literature review of the multiple clinical manifestations of Gorlin Syndrome, which extend far beyond basal cell carcinomas, including systemic, dermatological, skeletal, ophthalmological, and neurological features. Particular emphasis will be placed on oral and dental characteristics associated with Gorlin Syndrome, highlighting the importance of its diagnosis, particularly odontogenic keratocysts, dental and oral anomalies, and cleft palates. Because specialized and differentiated dental care is necessary and crucial for individuals affected by this syndrome, research will be conducted on the latest studies considering different follow-up strategies for monitoring these patients. The importance of a specialized multidisciplinary approach will be emphasized, with the dentist playing a significant role in contributing to a better prognosis and well-being of these patients. In our narrative review of Gorlin Syndrome, we conducted a comprehensive literature search using the terms "Gorlin syndrome etiology," "Oral-facial Gorlin syndrome malformations," and "Nevoid Basal Cell Carcinoma Syndrome." We use authoritative databases such as "PubMed," "Mendeley," "Orphanet," and "OMIM," focusing on articles published from October 2020 to February 2024. We extracted relevant information from selected articles covering genetic markers, clinical features, oral manifestations, and treatment approaches and synthesized these findings to offer a comprehensive narrative overview.