1/12. Implant placement and guided tissue regeneration in a patient with congenital vitamin d-resistant rickets.There continues to be increased expansion of implant services corresponding to the public's increased awareness of implant-borne prostheses as a regular part of treatment planning. This rise in public awareness as practitioners expand their skills will lead to the consideration of an implant option for growing groups of patients whose medical histories may have previously contraindicated implantology. This presentation is one such case.- - - - - - - - - - ranking = 1keywords = congenita (Clic here for more details about this article) |
2/12. Features of severe periodontal disease in a teenager with chediak-higashi syndrome.BACKGROUND: chediak-higashi syndrome (C-HS) is a rare congenital disease characterized by defective neutrophil function with abnormal lysosomal inclusions, neutropenia, and reduced chemotaxis. The complete syndrome includes oculocutaneous albinism with photophobia, neurologic features, recurrent infections, and enterocolitis. methods: A 14-year-old male C-HS patient was referred to us because of serious periodontal destruction with acute inflamed gingiva and ulcers. Clinical and biological investigations were performed, leading to the diagnosis of C-HS. RESULTS: Laboratory findings included neutropenia and hypergammaglobulinemia. Peripheral blood smears showed giant granules in neutrophils, eosinophils, and granulocytes. Bone marrow smears showed giant inclusions in leukocyte precursor cells. These granules and inclusions were characteristic of chediak-higashi syndrome. Oral radiographic status showed extensive loss of alveolar bone leading, in most cases, to tooth exfoliation. bacteria often associated with periodontitis were detected in subgingival plaque samples, including fusobacterium nucleatum, campylobacter rectus, prevotella melaninogenica, peptostreptococcus anaerobius, and clostridium sp. Biopsies of periodontal tissues for light and electronic microscopic examinations revealed massive bacterial invasion of the epithelial tissue, epithelial cells, and connective tissue. Ultrastructural observations of periodontal polymorphonuclear leukocytes showed defective granulation, with abnormal granules not discharging their lysosomal content against engulfed bacteria. Viable dividing bacteria were found in the cytoplasm. CONCLUSIONS: In this case, early-onset periodontitis seems to be the expression of C-HS granulocyte deficiency. Periodontal treatment of these patients is often unsuccessful. This case report illustrates the importance of the dentist in initiating clinical and biological investigations in such early aggressive periodontitis in young patients.- - - - - - - - - - ranking = 0.25keywords = congenita (Clic here for more details about this article) |
3/12. 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 = 1.25keywords = congenita (Clic here for more details about this article) |
4/12. oral manifestations of congenital neutropenia or Kostmann syndrome.Infantile congenital agranulocytosis or Kostmann syndrome is a rare hereditary kind of severe neutropenia. The typical symptoms, which appear since the first days of life, are abscesses located on various parts of the body: ear, cutis, lung and oral cavity. These abscesses are due to an almost total disimmunity typical of the neutropenia. The aim of this article is to describe the most typical signs of this pathology in the oral cavity, reporting a case observed in our department in Florence, italy. On the basis of the personally observed case and of the review of the literature, it is possible to consider, as a characteristic finding in Kostmann syndrome, a typical very serious periodontal pathology, which is similar to the prepubertal periodontitis in deciduous dentition. At the age of 19 years the patient showed a dramatic compromise of the masticatory function. It is obvious that the lack of response of the host can obstruct the interaction between the host and the microbic flora, because the lack of neutrophils increases the susceptibility of the patient to every kind of infection, even to periodontitis. A periodontal prophylaxis, since the very first observations, followed by a rigorous maintenance with frequent and regular professional hygienic treatments could be effective in controlling the effects of periodontal disease and could reduce the tragic evolution. We need to recognise that it could be hard to monitor the oral situation correctly in these patients, as they have a continuously poor systemic condition. Finally in these cases the rehabilitative therapy is very problematical.- - - - - - - - - - ranking = 1.25keywords = congenita (Clic here for more details about this article) |
5/12. Oral-dental findings in dyskeratosis congenita.A 13-yr-old girl with dyskeratosis congenita is presented. Besides oral leukoplakia and nail dystrophies, there was evidence of pancytopenia, growth retardation, alopecia, mental retardation and microcephaly. The oral findings included caries, gingival recession, short-blunted roots, gingival bleeding, tooth mobility and severe alveolar bone loss resembling juvenile periodontitis.- - - - - - - - - - ranking = 156.79263699207keywords = dyskeratosis congenita, dyskeratosis, congenita (Clic here for more details about this article) |
6/12. 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 = 1.75keywords = congenita (Clic here for more details about this article) |
7/12. dyskeratosis congenita: dental management of a medically complex child.dyskeratosis congenita (DKC) is a rare syndrome characterized by cutaneous hyperpigmentation, nail dystrophy, leukoplakia, and pancytopenia. The purpose of this case study was to describe the management of a 7-year-old girl diagnosed with DKC who urgently needed dental treatment under general anesthesia before bone marrow transplantation (BMT). The patient presented normal skin, nails, and hair, but oral examination revealed a number of ulcers, leukoplakia, gingival recessions, alveolar bone loss, and dental caries. Hematologic preparation included raising blood parameters, and the anesthesiologist to had consider pulmonary infection. The alveolar bone loss and the gingival recessions required the consultation of a periodontist. Avoiding stainless steel crowns was necessary due to potential plaque accumulation in the crown margins. The goal of this dental treatment was eliminating potential sources of infection before transplantation was conducted. It is important for the pediatric dentist to recognize the medical aspects associated with dental management prior to BMT, and to incorporate them into the treatment plan.- - - - - - - - - - ranking = 1.25keywords = congenita (Clic here for more details about this article) |
8/12. Implant-retained precision two-stage single-tooth replacement.The restoration of an anterior edentulous space due to a congenitally missing lateral incisor presents the dentist with a restorative challenge. Treatment of a young patient requires that the restoration be conservative as well as esthetic. This article presents the rationale for use of an osseo-integrated implant to support a single-tooth restoration. The final result was esthetic, functional, and promoted a healthy periodontium.- - - - - - - - - - ranking = 0.25keywords = congenita (Clic here for more details about this article) |
9/12. Clinical and laboratory studies of severe periodontal disease in an adolescent associated with hypophosphatasia. A case report.hypophosphatasia is a congenital disease characterized by defective bone mineralization, deficiency of alkaline phosphatase (ALP) activity, increased excretion of the phosphoethanolamine (PEA) in the urine, and premature loss of the deciduous teeth. A male hypophosphatasia patient (aged 15 years 6 months) with premature exfoliation of the deciduous teeth was referred to our hospital because of severe periodontal destruction in the permanent dentition. blood and urine tests as well as oral and periodontal examinations were performed. serum antibody titers against 7 periodontopathic bacteria by the enzyme-linked immunosorbent assay (ELISA), monocyte and neutrophil chemotaxis measurements, and cellular immunity tests were also performed. Low levels of ALP in serum and PEA in the urine were found. Monocyte and neutrophil chemotaxis exhibited normal values. Slightly depressed CD2 , CD3 , and CD4 and slightly elevated activity of NK cells were found. An elevated level of serum antibody to porphyromonas gingivalis was observed. Oral radiographic examination showed a mirror pattern of alveolar bone loss which is similar to that seen in localized juvenile periodontitis. Periodontal treatment of this patient was carried out for 4 years. The severely affected sites, the lower right and left first molars and the upper right first molar, had to be extracted. However, the other sites were well maintained. The serum IgG level against P. gingivalis was decreased after 4 years of periodontal treatment. infection with P. gingivalis was suspected to be associated with the destruction of this patient's hypophosphatasia, but other dental abnormalities such as abnormal enamel, dentin, and cementum formation may also have contributed to the periodontal pathology.- - - - - - - - - - ranking = 0.25keywords = congenita (Clic here for more details about this article) |
10/12. Clinical application of a soft denture liner: a case report.patients with thin, nonresilient mucosa, poor ridge morphology, chronic xerostomia, and acquired or congenital defects are candidates for soft denture liners. Soft denture liners may be placed during fabrication of the new denture or during relining of the existing dentures. Soft mandibular denture liners are ideal for senior patients with resorbed mandibular ridges who need replacement of existing dentures to correct excessively closed vertical dimension of occlusion and for patients who have maxillary complete dentures and mandibular natural teeth, severe loss of maxillary ridge, and clenching habits. Soft denture liners should be used only when needed because of their short service life. However, for those patients who cannot tolerate hard denture bases, soft liners are an appropriate alternative treatment.- - - - - - - - - - ranking = 0.25keywords = congenita (Clic here for more details about this article) |
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