1/39. Pentasomy X and hyper IgE syndrome: co-existence of two distinct genetic disorders.We describe a 10-year-old girl with features of a penta-X syndrome. cytogenetic analysis revealed a 49,XXXXX karyotype and molecular analysis of X-linked polymorphic markers showed that this aneuploidy arose by successive maternal non disjunctions. Apart from these features the patient has a lifelong history of eczema, recurrent pneumonia, and staphylococcal abscesses. Together with consistently increased serum IgE levels, low antibody responses, and low levels of serum IgA and IgG2, these findings are characteristic for the hyper IgE syndrome. While pentasomy X may be due to sequential non disjunctions in meiosis I and meiosis II in the mother, the underlying pathomechanism in hyper IgE syndrome remains unclear. This case is the first with co-existence of pentasomy X and hyper IgE syndromes.- - - - - - - - - - ranking = 1keywords = abscess (Clic here for more details about this article) |
2/39. Severe gingival recession in trisomy 18 primary dentition. A clinicopathologic case report of self-inflicted injury associated with mental retardation.This clinicopathologic case report documents severe gingival recession in the primary dentition of a trisomy 18 patient. Primary molar and canine teeth exhibited recession extending beyond the midpoint of the buccal aspect of the root, occasionally reaching the root apex. Radiographic examination revealed taurodontism in both primary and permanent teeth. Clinical and histopathologic findings, along with case history, eliminated the possibility of prepubertal periodontitis and suggested a diagnosis of self-inflicted injury associated with mental retardation. Histologic examination of the primary teeth revealed normal cementum and dentin structure. Taurodontism, histologic structure of the dentition, and severe attachment loss in the primary dentition have not been described previously in trisomy 18.- - - - - - - - - - ranking = 223.33546818171keywords = periodontitis (Clic here for more details about this article) |
3/39. Familial hypophosphatemic vitamin d-resistant rickets: dental findings and histologic study of teeth.A case of familial hypophosphatemic vitamin d-resistant rickets or X-linked hypophosphatemia (XLH) accompanied by specific systemic and dental findings is reported. A 15-year-old boy with XLH visited our facility complaining of a toothache in the left lower canine region. Two other family members of the patient, his younger sister and their mother, also had XLH, whereas the other 2 members, his younger brother and father, are healthy. Those with XLH show systemic signs of the disease, such as growth retardation, limb deformity, and spinal curvature disorders; however, these symptoms are more severe in the patient than in the others. The patient had multiple periodontal abscesses, but no evidence of dental caries, trauma, or periodontal disease on the corresponding teeth at the time of his oral examination. A radiographic examination showed root dysplasia and enlarged pulp chambers.A histologic examination of an extracted third molar showed marked globular dentin and an increased predentin width. The abscess was thought to be caused by pulpal infection, which came from bacterial invasion through enamel cracks and dentinal microcleavage of the teeth. The treatments provided in this case are discussed.- - - - - - - - - - ranking = 2keywords = abscess (Clic here for more details about this article) |
4/39. dyskeratosis congenita: report of a case.dyskeratosis congenita is a rare multisystem condition involving mainly the ectoderm. It is characterized by a triad of reticular skin pigmentation, nail dystrophy and leukoplakia of mucous membranes. Oral and dental abnormalities may also be present. Complications are a predisposition to malignancy and bone marrow involvement with pancytopenia. The case of a 14-year-old girl is described who presented with several of the characteristic systemic features of this condition, together with the following oral features: hypodontia, diminutive maxillary lateral incisors, delayed dental eruption, crowding in the maxillary premolar region, short roots, poor oral hygiene, gingival inflammation and bleeding, alveolar bone loss, caries and a smooth atrophic tongue with leukoplakia. Although this condition is rare, dental surgeons should be aware of the dental abnormalities that exist and the risk of malignant transformation within the areas of leukoplakia.- - - - - - - - - - ranking = 1.6104991748836keywords = alveolar (Clic here for more details about this article) |
5/39. Apparently new osteodysplastic and primordial short stature with severe microdontia, opalescent teeth, and rootless molars in two siblings.A Thai man and his sister affected with a newly recognized syndrome of proportionate primordial short stature are reported. The patients had severe intrauterine and postnatal growth retardation, prominent nose and nasal bridge, small pinnae, large sella turcica, areas of hypo- and hyperpigmentation of skin, dry and thin scalp hair, and long and straight clavicles. Ivory epiphyses and cone-shaped epiphyses of the hands were found when they were young, but most of them disappeared as they grew up. Scaphoid and trapezium had angular appearance. The second toes were unusually long. Distal symphalangism of toes and barchymesophalangy of fingers were noted. The findings that appear to distinguish this syndrome from the previously reported syndromes are long second toes, opalescent and rootless teeth, severe microdontia, severely hypoplastic alveolar process, and unerupted tooth. The mode of inheritance is suspected to be autosomal recessive.- - - - - - - - - - ranking = 1.6104991748836keywords = alveolar (Clic here for more details about this article) |
6/39. rothmund-thomson syndrome: a case report.rothmund-thomson syndrome (RTS) is an extremely rare genetic disorder characterized by poikilodermatous skin changes, photosensitivity, and an increased risk of developing skin and bone malignancies. In this case report, the dental and periodontal features of RTS in a 16-year-old female patient are presented. The transmission electron microscopy performed on a gingival biopsy specimen showed structural defects of connective tissue. If the unusual ultrastructural findings of this case are confirmed as being consistent with other RTS patients, it is our opinion that this syndrome can be considered among the systemic diseases associated with early-onset periodontitis.- - - - - - - - - - ranking = 223.33546818171keywords = periodontitis (Clic here for more details about this article) |
7/39. Use of dental implants in patients with down syndrome: a case report.down syndrome is caused by trisomy of the 21st chromosome and is associated with well-described physical and systemic problems. Most people with down syndrome have some degree of mental retardation as well as malformation of head and neck. Oral structures that are commonly affected include the tongue (macroglossia), abnormalities in the number and shape of teeth, and poor quality (osteoporotic-like) of alveolar bone and jaw. These oral malformations as well as a tendency toward poor cooperation in the dental office contribute to the belief among dentists that people with down syndrome are not good candidates for oral rehabilitation with dental implants. This article describes the use of dental implants in the oral rehabilitation of a 16-year-old boy with down syndrome. Although more experience is needed before dental implants can be considered a suitable option during oral rehabilitation in people with down syndrome, this case report shows a promising beginning.- - - - - - - - - - ranking = 1.6104991748836keywords = alveolar (Clic here for more details about this article) |
8/39. Report of a patient with hypoglossia-hypodactylia syndrome and a review of the literature.OBJECTIVE: To present the morphology of the extremities, craniofacial structures, and the oral cavity based on roentgencephalometry and three-dimensional computed tomography and magnetic resonance imaging scanning in a patient with hypoglossia-hypodactylia syndrome, discuss the orthodontic treatment method, and review the literature for the syndrome. PATIENT: The patient was a 6-year-old boy diagnosed with hypoglossia-hypodactylia syndrome at birth. He had hypodactylia as well as micrognathia with steep inclination of the anterior surface of the mandible in relation to the lower mandibular plane. He had missing mandibular incisors with concomitant bone defect limited to the associated alveolar ridge and an absence of any malformations in the mandibular ramus and condylar head. The patient had a bilateral scissors bite with an extremely constricted mandibular dental arch, skeletal Class II jaw relationship with an average mandibular plane angle and maxillary incisors inclined palatally. He had extremely reduced tongue size and hypertrophy of the floor of the mouth. Anomalies of the central nervous system were not observed. There was no evidence of hearing loss.- - - - - - - - - - ranking = 1.6104991748836keywords = alveolar (Clic here for more details about this article) |
9/39. Talon cusp causing occlusal trauma and acute apical periodontitis: report of a case.- The talon cusp, or dens evaginatus of anterior teeth, is a relatively rare dental developmental anomaly characterized by the presence of an accessory cusp-like structure projecting from the cingulum area or cemento-enamel junction. This occurs in either maxillary or mandibular anterior teeth in both the primary and permanent dentition. One of the main problems caused by accessory cusps are occlusal interferences. The anomalous cusp even can generate occlusal trauma and reversible acute apical periodontitis of the opposing tooth. This article reports a case of talon cusp affecting the permanent maxillary left lateral incisor that caused clinical problems related to occlusal trauma and apical periodontitis caused by a premature contact. The treatment of the occlusal interference produced by the taloned tooth is described.- - - - - - - - - - ranking = 1340.0128090902keywords = periodontitis (Clic here for more details about this article) |
10/39. Fractured dens evaginatus and unusual periapical radiolucency.Dens evaginatus (DE) is not uncommon. It can affect the premolars of people of oriental ethnicity, and it can lead to different clinical problems. This article describes the diagnosis and management of a patient presenting with a fractured tubercle of DE and an unusual buccal abscess in relation to periapical radiolucency extending around the mandibular second premolar and first molar.- - - - - - - - - - ranking = 6584.4583956618keywords = periapical, abscess (Clic here for more details about this article) |
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