1/44. Microbiological features of Papillon-Lefevre syndrome periodontitis.Papillon-Lefevre syndrome patients exhibit hyperkeratosis palmo-plantaris and severe periodontitis. The syndrome is an autosomal recessive trait, but the mechanism of periodontal destruction is not known. This report presents the clinical and microbiological features of an 11-year old girl with Papillon-Lefevre syndrome. Clinical examination included conventional periodontal measurements and radiographic analysis. In samples from 3 deep periodontal lesions, the occurrence of major suspected periodontopathic bacteria was determined by selective and non-selective culture and polymerase chain reaction (PCR) identification, and the presence of cytomegalovirus and Epstein-Barr type 1 virus by a nested-PCR detection method. 10 of 22 available teeth demonstrated severe periodontal breakdown. Major cultivable bacteria included actinobacillus actinomycetemcomitans (3.4% of total isolates), prevotella nigrescens (16.4%), fusobacterium nucleatum (14.3%) and peptostreptococcus micros (10.6%). A. actinomycetemcomitans, P. nigrescens, porphyromonas gingivalis and eikenella corrodens were identified by PCR analysis. The patient's non-affected parents and older brother revealed several periodontal pathogens but not A. actinomycetemcomitans. The viral examination demonstrated cytomegalovirus and Epstein-Barr type 1 virus in the subgingival sample of the Papillon-Lefevre syndrome patient. The father and brother yielded subgingival cytomegalovirus but not Epstein-Barr type 1 virus. We hypothesize that human herpesviruses in concert with A. actinomycetemcomitans play important roles in the development of Papillon-Lefevre syndrome periodontitis.- - - - - - - - - - ranking = 1keywords = periodontitis (Clic here for more details about this article) |
2/44. root resorption and signs of repair in Papillon-Lefevre syndrome. A case study.The aim of this investigation was to describe some tooth-related histological features of prepubertal periodontitis. Teeth extracted during treatment of two Papillon-Lefevre syndrome patients were processed by means of the sawing and grinding technique. light microscopy examination revealed little or no cementum in the coronal parts of the roots. Resorptions of various depths (0.02 to 1.5 mm) and to various extents (affecting up to 1/3 of the root surface) were observed in the 5 investigated teeth. Some resorptive defects on 1 of the examined incisors showed signs of spontaneous repair. Extrinsic fibers were inserted into the new cellular intrinsic fiber cementum which had formed directly on the bottom of the defect. Intact acellular extrinsic fiber cementum was found where fibers were still attached. Here, the characteristic of pristine cementum, a hyaline layer of peripheral dentin, could be identified. If resorption was not present, the cementum did not show any signs of hypoplasia. Thus, histological features of prepubertal periodontitis in the current material were (i) areas of extensive resorption, (ii) signs of spontaneous repair, and (iii) healthy cementum.- - - - - - - - - - ranking = 0.33333333333333keywords = periodontitis (Clic here for more details about this article) |
3/44. association of inflammatory pseudotumor of the liver and Papillon-Lefevre syndrome--case report.A case of hepatic inflammatory pseudotumor mimicking malignancy in a 4-year-old girl with the Papillon-Lefevre syndrome (PLS) is reported. Only recently, an association between this inherited syndrome and liver abscesses has been found. Its possible pathogenesis is discussed and immunologic defects resulting from the Papillon-Lefevre syndrome are presented. The development of inflammatory pseudotumor of the liver might be caused by immunologic disturbances and staphylococcal infection. The picture of the hepatic tumor on imaging in patients with PLS should be attributed rather to inflammatory than neoplastic process.- - - - - - - - - - ranking = 0.0001949667688747keywords = abscess (Clic here for more details about this article) |
4/44. A new family with Papillon-Lefevre syndrome: effectiveness of etretinate treatment.Papillon-Lefevre syndrome is characterized by the association of palmoplantar hyperkeratosis, severe periodontitis, and early loss of deciduous and permanent teeth. We report two patients from the same family, aged 21 and 30 years, who were unaware of their pathology; one was successfully treated with etretinate.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
5/44. Periodontal treatment of rapid progressive periodontitis in 2 siblings with Papillon-Lefevre syndrome: 15-year follow-up.AIMS: This paper reports the treatment of the periodontal component of the Papillon-Lefevre syndrome in 2 siblings (case A, born 1974; case B, born 1976). METHOD: The initial treatment, in 1982, consisted of extraction of all primary teeth, scaling and rootplaning of the erupted permanent teeth and systemic antibiotic therapy. During 15 years, continuous and intensive periodontal treatment consisted of chlorhexidine 0.2% rinses, bi-weekly professional prophylaxis, scaling and rootplaning or surgery if indicated. Systemic antibiotics often accompanied mechanical therapy after bacteriological analysis. RESULTS: In case A, a favourable number of permanent teeth could be maintained, but in case B, all permanent teeth were lost in spite of the intensive treatment. Darkfield microscopy at different intervals revealed high numbers of spirochetes and motile rods in both siblings. Only in case A were they temporarily reduced to zero after scaling and rootplaning combined with metronidazole. Anaerobic cultering revealed high numbers of actinobacillus actinomycetemcomitans (A.a) in both patients. In 1994, 2 years after combined amoxicillin/metronidazole therapy, no A.a could be detected in case A. In case B, A.a could still be detected and was found to be resistant to metronidazole. One year after extraction of all permanent teeth, could no A.a be detected in case B. CONCLUSION: Intensive periodontal treatment combined with antibiotic therapy was not able to prevent complete tooth loss in case B. In case A, the treatment was more effective, resulting in preserving a number of permanent teeth in a stable clinical situation. In these 2 cases, no attempt was made to create an edentulous period between the periodontally-diseased mixed dentition and the eruption of the remaining teeth, which may have contributed to treatment failure.- - - - - - - - - - ranking = 0.66666666666667keywords = periodontitis (Clic here for more details about this article) |
6/44. Papillon-Lefevre syndrome with pyogenic hepatic abscess: a rare association.Papillon-Lefevre syndrome in a 14-year-old boy presenting with palmoplantar keratoderma, periodontosis, recurrent pyogenic infections of the skin, and hepatic abscesses is reported for its rarity and unusual manifestations. The patient showed a dramatic response to acitretin therapy.- - - - - - - - - - ranking = 0.00097483384437352keywords = abscess (Clic here for more details about this article) |
7/44. Combined mechanical and antibiotic periodontal therapy in a case of Papillon-Lefevre syndrome.BACKGROUND: Papillon Lefevre syndrome (PLS) is a rare entity and, as such, it is almost impossible to evaluate an effective therapy in a randomized controlled study. The amount of success reported after therapy for prepubertal periodontitis (PP) in PLS is highly variable from case to case. The goal of this case report is to evaluate the effects of a combined mechanical and antibiotic periodontal therapy regimen in the management of PLS. methods: A male patient was diagnosed as suffering from PP associated with PLS at the age of 7 years. He showed hyperkeratosis of the palms and soles, as well as advanced periodontal disease already affecting permanent teeth with maximal probing depth and vertical attachment loss of 12 mm and 11 mm, respectively. Subgingival debridement was performed with simultaneous administration of oral 250 mg amoxicillin 3 times daily and 250 mg metronidazole twice daily for one week. Clinical parameters were assessed and subgingival plaque was collected from all teeth prior to therapy and 7 and 26 months after treatment. Selective cultures for A. actinomycetemcomitans were incubated for each individual tooth and DNA probe analysis was performed for various periodontal pathogens. RESULTS: Prior to combined mechanical and antibiotic treatment, all teeth but one harbored actinobacillus actinomycetemcomitans subgingivally. However, at 7 and 26 months after therapy A. actinomycetemcomitans could be detected neither by culture nor by dna probes. Clinical parameters improved markedly and teeth erupting after therapy did not exhibit attachment loss of more than 1.5 mm during the observation period. CONCLUSIONS: Eradication (suppression beneath detection levels) of A. actinomycetemcomitans seems to play a significant role in the successful treatment of localized prepubertal periodontitis in PLS.- - - - - - - - - - ranking = 0.33333333333333keywords = periodontitis (Clic here for more details about this article) |
8/44. Atypical familial Papillon-Lefevre syndrome.The Papillon-Lefevre syndrome is a rare autosomal recessive disorder. consanguinity seems a notable prerequisite. Papillon-Lefevre syndrome manifests in the first 6 months of life with rapidly progressive periodontitis and severe alveolar bone destruction leading to early loss of both the deciduous and permanent teeth in association with palmo-plantar hyperkeratosis. We present two unusual cases of familial Papillon-Lefevre syndrome, one of whom has only late onset of mild skin lesions and the other has severe skin lesions and relatively mild periodontal disease. A number of other cases recently described have also had atypical features.- - - - - - - - - - ranking = 0.1667004399251keywords = periodontitis, alveolar (Clic here for more details about this article) |
9/44. Papillon Lefevre syndrome: management of a case.Papillon Lefevre syndrome is a rare syndrome autosomal recessive in nature, manifesting as keratosis palmoplantaris with periodontitis. A case of Papillon Lefevre syndrome present in a 12 year old boy is presented. Only the permanent maxillary canines were present in the oral cavity when the patient was first seen, and despite efforts towards preservation, they had to be extracted. (complete dentures were made and the patient was followed up for 26 months.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
10/44. Elevated hydroperoxide levels and antioxidant patterns in Papillon-Lefevre syndrome.BACKGROUND: Since it has been found that reactive oxygen species seem to be involved in the pathogenesis of both periodontitis and hyperkeratotic syndromes, we studied a group of patients belonging to 3 generations of a family with different degrees of severity of Papillon-Lefevre syndrome (PLS) to ascertain whether altered concentrations of the most important hydrophobic and hydrophilic plasma antioxidants as well as products of oxidative damage are present in PLS. methods: Coenzyme Q (CoQ), vitamin e, glutathione (GSH), and uric acid were evaluated by high-performance liquid chromatography (HPLC) (supplied with electrochemical detector) techniques and hydroperoxides by a spectrophotometric method. RESULTS: GSH and uric acid were in the range of reference values; CoQ was very low in both the child of the third generation and his mother, and these 2 subjects had the highest hydroperoxide levels. The child also had extremely low values of vitamin e. In general, all family members showed abnormally high hydroperoxide levels, with the exception of those members who are phenotypically healthy. CONCLUSIONS: Since the subjects with the lowest hydroperoxide contents are phenotypically healthy, whereas the affected individuals presented lower antioxidant levels and very high hydroperoxide concentrations, it has been suggested that a specific antioxidant therapy could be a promising approach in treating some PLS subjects. Moreover, unexpected manifestations of heterozygosity in the child of the third generation were also detected.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
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