Odonto-onycho-dermal dysplasia (OODD), a rare autosomal-recessive inherited form of ectodermal dysplasia including severe oligodontia, nail dystrophy, palmoplantar hyperkeratosis, and hyperhidrosis, was recently shown to be caused by a homozygous nonsense WNT10A mutation in three consanguineous Lebanese families. Here, we report on 12 patients, from 11 unrelated families, with ectodermal dysplasia caused by five previously undescribed WNT10A mutations. In this study, we show that (1) WNT10A mutations cause not only OODD but also other forms of ectodermal dysplasia, reaching from apparently monosymptomatic severe oligodontia to Schöpf-Schulz-Passarge syndrome, which is so far considered a unique entity by the findings of numerous cysts along eyelid margins and the increased risk of benign and malignant skin tumors; (2) WNT10A mutations are a frequent cause of ectodermal dysplasia and were found in about 9% of an unselected patient cohort; (3) about half of the heterozygotes (53.8%) show a phenotype manifestation, including mainly tooth and nail anomalies, which was not reported before in OODD; and (4) heterozygotes show a sex-biased manifestation pattern, with a significantly higher proportion of tooth anomalies in males than in females, which may implicate gender-specific differences of WNT10A expression.
The purpose of this prospective, longitudinal study was to compare the influence of two lingual bracket systems on subjective oral comfort, speech, mastication and oral hygiene. Forty-two native speakers of standard German (32 females, 10 males; mean age 27.1 years, standard deviation 12.2) were enrolled and completed a standardized questionnaire directly before insertion of lingual brackets (T0), within 24 hours of bond-up (T1) and 3 months (+/- 1 week) later (T2). Eighteen of the patients were treated with prefabricated brackets (Ormco, seventh generation) (PB group) and 24 with customized brackets (Incognito) (CB group). While no significant intergroup differences were recorded at any of the times with respect to tongue position, conversation pattern, swallowing or oral hygiene, the CB group experienced significantly fewer tongue space restrictions, speech disturbances and impairments in chewing and biting than the PB group at T1 and T2. At T2, pressure sores, reddening or lesions to the tongue were recorded significantly less often in the CB group than in the PB group. This enhanced patient comfort in the CB group was attributed to the smaller dimensions of the customized brackets. This aspect could play a role in attracting more patients to lingual orthodontics in the future. Information given to the patient on the duration and extent of the restrictions associated with lingual orthodontics must be differentiated according to the bracket system used.
The effect of unintended titanium release around oral implants remains a biological concern. The current study was undertaken to evaluate a new detection system of element mapping in biological probes. A new scanning electron microscopy-energy dispersive spectroscopy detection method was used to map the features of titanium contamination in peri-implant bone around implants with different surface structures. The amount of titanium wear was highest adjacent to titanium-plasma-sprayed surfaces, followed by sandblastered large grid acid-etched and smooth surfaces. A high sensitivity of titanium detection over large areas of bone tissue was observed. A high spatial resolution of titanium wear particles (20 nm) could be reached and correlated to the ultrastructural morphological features of peri-implant tissue. Cells adjacent to titanium wear revealed no signs of morphological alterations on a nanoscale level at early periods of implant/bone interaction. The new technique may serve as a fast and effective tool to evaluate titanium release effects in biological probes.
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