1/8. Witkop tooth and nail syndrome: report of two cases in a family.tooth and nail syndrome, an autosomal dominant condition, is characterized by hypodontia and nail dysplasia. Mandibular incisors, second molars and maxillary canines are the most frequently missing teeth. tooth shape may vary and conical and narrow crowns are common. Nail dysplasia affects fingernails and toenails and is often more severe in childhood. nails may be spoon-shaped, ridged, slow growing and easily broken. The clinical and radiographic features of a father and son presenting with this rare condition are described.- - - - - - - - - - ranking = 1keywords = childhood (Clic here for more details about this article) |
2/8. Segmental odontomaxillary dysplasia: clinical, radiological and histological aspects of four cases.Segmental odontomaxillary dysplasia (SOD) is a rare developmental disorder of the maxilla, primarily involving the posterior part of the maxilla. Clinically, the disorder is often diagnosed in early childhood due to a unilateral buccolingual expansion of the posterior alveolar process, gingival enlargement, absence of one or both premolars in the affected region, delayed eruption of the adjacent teeth and malformations of the primary molars. In this report, four patients with SOD are described. The findings were similar to earlier reports, but for the first time an ipsilateral rough erythema on the skin in two of the subjects is reported.- - - - - - - - - - ranking = 1keywords = childhood (Clic here for more details about this article) |
3/8. Leukodystrophy associated with oligodontia in a large inbred family: fortuitous association or new entity?We describe a large inbred Syrian pedigree with an autosomal recessive neurodegenerative disorder. The clinical picture of the affected patients is oligodontia, and a degenerative neurological condition with onset around age 12, characterized by progressive ataxia and pyramidal syndrome. Abnormalities in the white matter and cortical atrophy were assessed by magnetic resonance imaging. Differential diagnosis and the possibility of a fortuitous association or the report of a hitherto unreported dento-leukoencephalopathy are discussed.- - - - - - - - - - ranking = 4.645953462746keywords = ataxia, white (Clic here for more details about this article) |
4/8. Osseointegrated implants in the oral rehabilitation of a patient with cleft lip and palate and ectodermal dysplasia: a case report.Hereditary ectodermal dysplasia is an inherited disorder characterized by aplasia or dysplasia of ectodermal tissues, such as hair, nails, teeth, and skin, that occurs in approximately 1 in every 100,000 live births. Dental abnormalities and abnormal facial appearance are of major concern in childhood and adolescence, since they can restrict the individual socially and affect his or her self-confidence. Oral rehabilitation in the early stages of the patient's life may provide functional and esthetic restoration as well as safeguard psychologic health. This report presents the clinical procedures involved in the rehabilitation of a 10-year-old female patient with complete bilateral cleft lip and palate and ectodermal dysplasia.- - - - - - - - - - ranking = 1keywords = childhood (Clic here for more details about this article) |
5/8. Leukoencephalopathy with ataxia, hypodontia, and hypomyelination.The authors describe four unrelated girls with a distinctive neurologic disorder with early-onset progressive ataxia and hypodontia with a characteristic pattern of delayed dentition. Cerebral MRI shows hypomyelinated white matter and cerebellar atrophy; 1H-MRS of white matter reveals a marked elevation of myo-inositol.- - - - - - - - - - ranking = 138232.6195621keywords = hypomyelination, ataxia, white (Clic here for more details about this article) |
6/8. Witkop tooth and nail syndrome: a case report.Witkop tooth and nail syndrome is an autosomal dominant ectodermal dysplasia characterized by hypodontia and nail dysplasia. Mutations in MSX-1 have been identified as being involved in the syndrome. Mandibular incisors, secondary molars and maxillary canines are the most frequently missing teeth. tooth shape may vary, and conical and narrow crowns are common. Nail dysplasia affects finger- and toenails, and is often more severe in childhood. nails may be spoon-shaped, rigid, slow-growing and easily broken. The clinical and radiographic features of a mother and child presenting with this rare condition are described.- - - - - - - - - - ranking = 1keywords = childhood (Clic here for more details about this article) |
7/8. Partial anodontia: a case report.This article describes the multidisciplinary rehabilitation of a 20-year-old white man with partial anodontia. The patient presented with only three adult teeth; the remaining dentition was deciduous. The mandibular arch was restored with a tooth-supported overlay denture, while the maxillary arch received a fixed prosthesis.- - - - - - - - - - ranking = 0.30949047518416keywords = white (Clic here for more details about this article) |
8/8. Hallerman-Streiff syndrome: case report and recommendations for dental care.Hallerman-Streiff syndrome is a rare congenital anomaly characterized by a peculiar bird facies, mandibular and maxillary hypoplasia, dyscephaly, cataracts, microphtalmia, hypotrichosis, skin atrophy, and short stature. Dental abnormalities are present in 80 percent of the cases and include malocclusion, crowding, severe caries, supernumerary and neonatal teeth, enamel hypoplasia, hypodontia, premature eruption of primary dentition, agenesis of permanent teeth, and anterior displacement or absence of condyles. Very few cases have been described in the dental literature. The predisposition to severe caries, together with other problems, makes it imperative that young patients be started in a strong prevention program as early as possible. This is a case report of a five-year-eleven-month-old white male who presented for a dental examination at The Children's Hospital in Denver, CO. The findings and recommendations for treatment are discussed.- - - - - - - - - - ranking = 0.30949047518416keywords = white (Clic here for more details about this article) |