1/7. tuberous sclerosis: presentation of a clinical case with oral manifestations.tuberous sclerosis (TS) is a genetic disorder affecting multiple body systems, and resulting from alterations in cell differentiation and proliferation. The disease is characterized by the development of benign hamartomatous tumors: neurofibromas and angiofibromas, located in the skin, central nervous system, mucosas and other organs. Abnormal neural cell migration plays an important role in the neurological dysfunctions found in TS, the predominant features being mental retardation, seizures and behavioral disorders. The condition is produced by mutations in genes TSC1 of chromosome 9q34 and TSC2 of chromosome 16p13.3, and exhibits a dominant autosomal hereditary trait--though 60-70% of cases are sporadic and represent new mutations. The phenotype is highly variable. The prevalence of TS varies between 1/6000 and 1/10,000 live births. The present study reports the case of a 21-year-old male with TS and oral manifestations of the disease. The clinical characteristics are described, along with the diagnostic criteria and the management strategies, with a review of the literature on the disease.- - - - - - - - - - ranking = 1keywords = oral manifestation (Clic here for more details about this article) |
2/7. Multiple recurrent and de novo odontogenic keratocysts associated with oral-facial-digital syndrome.In 1954, Papillon-Leage and Psaume were the first to describe the clinical characteristics of oral-facial-digital syndrome (OFDS). On the basis of their clinical features and the inheritance pattern, 2 variants were initially distinguished, namely OFDS type I (Papillon-Leage and Psaume) and OFDS type II, or Mohr syndrome. At present, 11 types of OFDS have been discovered. OFDS represents a heterogeneous group of disorders characterized by oral manifestations including oral frenula, cleft or lobulated tongue, high arched palate, cleft lip and/or palate, facial anomalies, and digital abnormalities such as syndactyly, polydactyly, brachydactyly, and clinodactyly. Depending on the type of OFDS, abnormalities may be present in other organs, such as the brain and heart. We report a patient with OFDS in whom multiple recurrent and de novo keratocysts were found. Although multiple keratocysts are commonly found in Gorlin-Goltz nevoid basal cell carcinoma syndrome, a relationship between OFDS and multiple keratocysts has not been described.- - - - - - - - - - ranking = 0.2keywords = oral manifestation (Clic here for more details about this article) |
3/7. oral manifestations of renal osteodystrophy: case report and review of the literature.renal osteodystrophy, characterized by uneven bone growth and demineralization, is described. oral manifestations of the disorder are described, and the value of dental radiographs in early detection of renal osteodystrophy is noted. A case report of a patient with severe oral complications, which resulted from long-standing end-stage renal disease and secondary hyperparathyroidism, is presented. Giant cell lesions of hyperparathyroidism, referred to as brown tumors (which may be associated with pain and swelling), are the key clinical oral manifestations and are the most dramatic dental radiographic finding in patients with renal osteodystrophy. Bone changes may include loss of lamina dura, giant cell lesions of hyperparathyroidism, and bone demineralization. The dentist's role in detection, assessment, and treatment is stressed.- - - - - - - - - - ranking = 0.2keywords = oral manifestation (Clic here for more details about this article) |
4/7. tooth development included in the multifocal jaw lesions of Langerhans cell histiocytosis.Many cases demonstrating the oral manifestations of Langerhans cell histiocytosis (LCH) have been reported; however, tooth development in jaw lesions has rarely been mentioned. This paper reports the case of a 3-year-old Japanese girl with LCH suffering from multiple osteolytic lesions of the skull and jaw bones. She was referred to our paediatric clinic because of swelling of the mucogingival folds in the upper and lower primary molar regions. The patient responded well to steroid therapy and the osteolytic lesions resolved. There was an accompanying development of permanent tooth germs included in the lesions. Langerhans cell histiocytosis in children usually has a long-term clinical course and paediatric dentists should be involved with oral health care for affected patients.- - - - - - - - - - ranking = 0.2keywords = oral manifestation (Clic here for more details about this article) |
5/7. Premature loss of primary teeth associated with congenital syphilis: a case report.Congenital syphilis is an infectious disease transmitted by an infected mother to her fetus. Several reports in the literature have focused on oral manifestations of congenital syphilis, mainly about Hutchinson's teeth and dysplastic molars, which are more common. However, this paper describes an unusual feature of congenital syphilis in a four-year-old child. A case of premature loss of primary teeth associated with congenital syphilis is reported.- - - - - - - - - - ranking = 0.2keywords = oral manifestation (Clic here for more details about this article) |
6/7. The sturge-weber syndrome: a case involving a 13-year-old black male.A 13-year-old black male patient with sturge-weber syndrome is presented. The report describes the classic presentation of the syndrome, emphasizing the oral manifestations. The case presentation includes a discussion of the differential diagnosis of gingival enlargement, and suggestions for subsequent therapy.- - - - - - - - - - ranking = 0.2keywords = oral manifestation (Clic here for more details about this article) |
7/7. Epulis granulomatosa as an oral manifestation of Klippel-Trenaunay syndrome.The Klippel-Trenaunay syndrome (KTS) was first described by Klippel and Trenaunay in 1900. It is characterized by the triad of hemihypertrophy of soft and hard tissue, naevus flammeus and venous varicosity in the affected area. Though all oral tissues may be affected, only 5% of KTS show manifestations in the head and neck region. Only three cases are described with an oral manifestation, showing gingival overgrowth clinically and histologically corresponding to a pyogenic granuloma. It is still uncertain whether the combination of gingival fibromatosis and KTS is significant or coincidental. We report about a 25-year-old patient with KTS and recidivous gingival fibromatosis, clinically and histologically corresponding to an epulis fibromatosa in a case report. It is suggested that this occurrence is significant.- - - - - - - - - - ranking = 1keywords = oral manifestation (Clic here for more details about this article) |