1/11. New syndrome of hypotrichosis, striate palmoplantar keratoderma, acro-osteolysis and periodontitis not due to mutations in cathepsin c.We report a mother and daughter with a syndrome of hypotrichosis, striate palmoplantar keratoderma, onychogryphosis, periodontitis, acro-osteolysis and psoriasis-like skin lesions. The syndrome resembles Papillon-Lefevre syndrome (PLS), characterized by palmoplantar keratoderma, periodontitis and psoriasis-like skin lesions, and particularly Haim-Munk syndrome, an allelic variant of PLS with acro-osteolysis. Both are caused by mutations in the cathepsin c gene (CTSC). Our patients differ in the unique nature of the palmar keratoderma and hypotrichosis. We have sequenced CTSC in the mother without finding mutations in either coding or non-coding parts of the gene. We propose that our patients suffer from a new syndrome possibly caused by mutations in a gene that has a functional or structural relation with CTSC.- - - - - - - - - - ranking = 1keywords = periodontitis (Clic here for more details about this article) |
2/11. Destructive arthritis in a patient with Haim-munk syndrome.Haim-Munk and Papillon-Lefevre are 2 closely related syndromes, inherited in an autosomal recessive pattern, manifested by palmoplantar keratoderma and early, destructive periodontitis. Recently, mutations in the cathepsin c gene have been recognized in both syndromes. We describe a patient with Haim-Munk syndrome (palmar plantar keratosis and periodontitis) and destructive arthritis of the wrists and shoulder joints, an association that has not been previously described.- - - - - - - - - - ranking = 0.33333333333333keywords = periodontitis (Clic here for more details about this article) |
3/11. Papillon-Lefevre syndrome. A report of six cases in one family.Papillon-Lefevre syndrome in six Saudi children in the same family is described. The parents were unaffected, and parental consanguinity was present. Palmoplantar keratosis started at the age of 1 1/2 years. The loss of deciduous teeth was a consequence of juvenile periodontitis. All essential features of the syndrome were present in this series.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
4/11. Clinical management of prepubertal periodontitis in 2 siblings with Papillon-Lefevre syndrome.The development of prepubertal periodontitis in Papillon-Lefevre syndrome (PLS) has been studied in 2 siblings, 12 and 8 years of age, since they were 5 and 3 years of age, respectively. Results of the treatment which comprised mechanical and chemical plaque control as well as systemic tetracycline medication, are reported. Extraction of temporary teeth, resulting in edentulousness prior to eruption of permanent teeth in the youngest child seemed to prevent destructive periodontitis from developing in the permanent dentition. After institution of continuous tetracycline medication in the oldest child at the age of 10 years, periodontitis with attachment loss did not occur in new sites. The possible role of specific micro-organisms in the progression of PLS-periodontitis is discussed.- - - - - - - - - - ranking = 1.3333333333333keywords = periodontitis (Clic here for more details about this article) |
5/11. Treatment of a patient with Papillon-Lefevre syndrome. A case report.A vital root submersion procedure was performed in a rare case of Papillon-Lefevre syndrome (PLS). Although the etiology and pathogenesis are obscure, possible host genetic defects and known or unknown periodontal pathogens may play important roles in the progression of PLS. Since this patient did not respond to conventional periodontal therapy, a submersion procedure was employed to maintain the alveolar ridge and resulted in controlling the progression of the patient's severe periodontitis. The following is a report of the treatment and the rationale for therapy.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
6/11. Ultrastructure of the periodontal lesion in a case of Papillon-Lefevre syndrome (PLS).In this study, 11 permanent teeth and their associated soft tissues from an 11-year-old boy with PLS were examined. Plaque, cementum and periodontal tissues were examined by scanning (SEM) and transmission electron microscopy (TEM). Except for depressed lymphocyte transformation, there were no abnormal haematological data. Local findings included abnormally thin cementum, extensive destruction of the periodontal ligament were still attached to the root, and severe inflammation of the soft tissues. Few bacteria were found in any of the soft tissue layers. The apical border plaque was restricted to gram- cocci and rods. The features observed in this case of PLS may indicate primary defects of cementum or ligament attachment, or disruption of fibroblast and cementoblast function due to the rapid advance of the disease process. Lack of bacterial invasion in the pocket soft tissue casts doubt on its involvement in the present case of severe periodontitis. The restricted range of morphotypes observed suggests a limited range of associated organisms. Further research is required to clarify the role of the host response and to identify the organisms involved.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
7/11. Hyperkeratosis palmoplantaris (Papillon-Lefevre syndrome). A case report.A 16-year-old female presented with a severe chronic periodontitis and with associated hyperkeratotic lesions of her palms and soles. Investigations revealed it as a case of hyperkeratosis palmoplantaris (Papillon-Lefevre syndrome), which is a rare form of an ectodermal dysplasia. Previously it was speculated that this disease entity is not all that uncommon in nigeria especially amongst the rural population. However, recent investigations revealed that this is not correct.- - - - - - - - - - ranking = 0.16666666666667keywords = periodontitis (Clic here for more details about this article) |
8/11. Treatment of rapidly destructive periodontitis in Papillon-Lefevre syndrome. Laboratory and clinical observations.This paper reports the successful treatment of the periodontal component of the Papillon-Lefevre syndrome in 2 siblings. Treatment consisted of extractions of periodontally-involved teeth under antibiotic therapy. The exogenous source of the suspected pathogenic A. actinomycetemcomitans in this family, identified as a pet dog, was also treated with antibiotics. Assessments of monocyte function and levels of serum antibodies against A. actinomycetemcomitans were performed prior to an immediately after treatment. Microbiological screening of subgingival dental plaque of the PLS patients as well as the rest of the household members, including the dog, was performed every month during the study period. Remission of the rapidly destructive periodontitis, as well as an earlier-described monocyte dysfunction in these two PLS patients occurred concomitantly with the eradication of A. actinomycetemcomitans from the family. 15 months after this treatment, the children still had no signs of periodontitis or of A. actinomycetemcomitans infection. The legitimacy with which PLS is defined as a disease entity is discussed and questioned.- - - - - - - - - - ranking = 1keywords = periodontitis (Clic here for more details about this article) |
9/11. behavior of neutrophilic granulocytes in a case of Papillon-Lefevre syndrome.Recently we had the opportunity to examine and follow up over a period of 2 years an unusual case of Papillon-Lefevre syndrome (PLS). A 10 year old boy exhibited all symptoms typical of PLS except periodontitis and premature loss of deciduous teeth. The present report aimed at studying the functional capacity of his neutrophilic granulocytes. It integrates clinical observations, histopathological findings and results of in vitro tests. The bioptic material examined included one gingival biopsy, extracted teeth, suppurative material discharged from periodontal pockets, pus emanating from a mucosal abscess, and peripheral blood leucocytes. The neutrophils were sampled on two separate occasions in two independent laboratories and tested for a variety of functions, i.e. motility, random and directional locomotion (chemotaxis), phagocytosis, membrane potential depolarization, oxygen consumption, NBT reduction, and intracellular killing of bacteria and fungi. Findings and test data indicated that in this case of PLS, neutrophilic granulocytes behaved normally with respect to all these functions including margination in blood vessels, emigration, phagocytosis of a broad range of bacteria, degranulation of lysosomes, and intracellular destruction. The data imply that factors other than neutrophil defects may be responsible for rapidly destructive periodontitis in cases where PLS is not associated with an increased susceptibility to infection.- - - - - - - - - - ranking = 0.33333333333333keywords = periodontitis (Clic here for more details about this article) |
10/11. history of periodontitis in a child with Papillon-Lefevre syndrome. A case report.An unusual case of Papillon-Lefevre syndrome is reported. The 10-year-old boy exhibited all symptoms typical of this disease except premature loss of primary teeth. The patient's past history, his status at first examination, an initial successful treatment phase, the efforts made at long-term maintenance and the eventual treatment failure are described. The bacterial plaque covering subgingival root surfaces was examined by scanning electron microscopy, and the histopathologic alterations of glossy, glaring red gingival tissue halos are described and discussed with respect to the pathogenesis of periodontitis associated with this syndrome.- - - - - - - - - - ranking = 0.83333333333333keywords = periodontitis (Clic here for more details about this article) |
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