Periodontal disease in a patient with Prader-Willi syndrome: a case report

<p>Abstract</p> <p>Introduction</p> <p>Prader-Willi syndrome is a complex genetic disease caused by lack of expression of paternally inherited genes on chromosome 15q11-q13. The prevalence of Prader-Willi syndrome is estimated to be one in 10,000 to 25,000. However, descriptions of the oral and dental phenotype are rare.</p> <p>Case presentation</p> <p>We describe the clinical presentation and periodontal findings in a 20-year-old Japanese man with previously diagnosed Prader-Willi syndrome. Clinical and radiographic findings confirmed the diagnosis of periodontitis. The most striking oral findings were anterior open bite, and crowding and attrition of the lower first molars. Periodontal treatment consisted of tooth-brushing instruction and scaling. Home care involved recommended use of adjunctive chlorhexidine gel for tooth brushing twice a week and chlorhexidine mouthwash twice daily. Gingival swelling improved, but further treatment will be required and our patient's oral hygiene remains poor. The present treatment of tooth-brushing instruction and scaling every three weeks therefore only represents a temporary solution.</p> <p>Conclusions</p> <p>Rather than being a direct result of genetic defects, periodontal diseases in Prader-Willi syndrome may largely result from a loss of cuspid guidance leading to traumatic occlusion, which in turn leads to the development of periodontal diseases and dental plaque because of poor oral hygiene. These could be avoided by early interventions to improve occlusion and regular follow-up to monitor oral hygiene. This report emphasizes the importance of long-term follow-up of oral health care by dental practitioners, especially pediatric dentists, to prevent periodontal disease and dental caries in patients with Prader-Willi syndrome, who appear to have problems maintaining their own oral health.</p

Absence of a specific subgingival microflora in adults with Down's syndrome

Background: Periodontal disease in Down's syndrome (DS) is generally characterized by a high degree of bone loss. Bone loss of 5 mm or more is observed in 70% of these subjects. Among DS subjects, considerable differences in disease progression occur. So far, no studies have been conducted in which specific properties of the subgingival microflora have been related to the condition observed. Aims: To investigate (1) the subgingival microflora in DS subjects and other mentally retarded (control) individuals which were matched to the utmost and (2) to investigate the subgingival microflora of a "low-risk" and a "high-risk" group formed in DS subjects. Material and Methods: 17 DS subjects and 17 control subjects were matched with respect to age, plaque level and bleeding on probing. In addition, the DS group was divided in a "low-risk" group (0-2 teeth lost due to periodontal disease n = 6) and a "high-risk" group (6-13 teeth lost due to periodontal disease n = 11). Prevalence and proportions of the putative periodontal pathogens Actinobacillus actinomycetemcomitans, Porphyromonas gingivalis, Prevotella intermedia, Bacteroides forsythus, Peptostreptococcus micros, Fusobacterium nucleation and Campylobacter rectus in the subgingival plaque were determined using anaerobic culture techniques. No differences in the prevalence of distinct suspected periodontopathic bacteria and bacterial subgingival composition between the DS group and the control group could be established. Also no differences in the prevalence of the seven investigated microbial species between the "low-risk" and the "high-risk" group were observed. Conclusions: Because of the lack of differences in microflora between the DS group and the control group, a specific effect of the microbiological composition in the periodontal status of subjects with DS can be excluded in this population. Host factors constitute the more likely explanation of the differences observed in DS

Experimental gingivitis in a Down's syndrome child and sibling.

Biopsies were taken from a child with Down's syndrome and her sibling under experimental gingivitis conditions and analyzed with monoclonal antibodies. At first, plaque caused a shift from a predominance of T cells to a predominance of B cells. Later, a gradual increase of T cells and a looser arrangement of B cells were observed