[ORO-DENTO-FACIAL MANIFESTATIONS IN PATIENTS WITH FAMILIAL DYSAUTONOMIA].

INTRODUCTION: The oro-dento-facial features and dysfunctions of children with hereditary sensory and autonomic neuropathy type III (HSAN III), known as familial dysautonomia (FD) or Riley-Day syndrome, were first described in the scientific literature in 1949. They include: dental trauma, dental and soft tissue selfmutilation, normal dental age, normal sequence and timing of eruption and exfoliation of teeth, smaller tooth size, different and disproportional tooth components, normal alveolar bone height, small jaws, mild crowding and malocclusion. These individuals have different craniofacial morphology from accepted norms, but resemble norms of their ethnic origin. The subjects often display gray, pale, shiny faces with an asymmetric suffering expression; frontal bossing, with eventual hypertelorism and narrow lips; a low dental caries rate; drooling, and genuine hypersalivation. They may have changes in salivary composition and content, which influence plaque and calculus accumulation and increase the risk of gingival and periodontal diseases. They also have difficulty in controlling oral muscles, a progressive decrease in the number of tongue fungiform papillae, accompanied by a reduced number of taste buds and specific dysgeusia, but a normal sense of smell.

A review of the oro-dento-facial characteristics of hereditary sensory and autonomic neuropathy type III (familial dysautonomia).

The oro-dento-facial features and dysfunction of children with hereditary sensory and autonomic neuropathy type III, known as familial dysautonomia or Riley-Day syndrome, was first described in the scientific literature in 1949. They include dental trauma; dental and soft tissue self-mutilation; normal dental age; normal sequence and timing of eruption and exfoliation of teeth; smaller tooth size; different and disproportional tooth components; normal alveolar bone height; small jaws, mild crowding, and malocclusions. These persons have craniofacial morphology that is different from accepted norms but they resemble norms of their ethnic origin. The subjects can have gray, pale, shiny faces with an asymmetric suffering expression; frontal bossing, with eventual hypertelorism and narrow lips; a low-caries rate; drooling, and hypersalivation. They can have changes in salivary composition and content, which influences plaque and calculus and increases the risk of gingival and periodontal diseases. They also have difficulty in controlling oral muscles; a progressive decrease in number of tongue fungiform papillae, accompanied by lack of taste buds; and specific dysgeusia, but a normal sense of smell.

Long-term radiologic pulp evaluation after partial pulpotomy in young permanent molars.

OBJECTIVE: Radiographic appearance of pulp reactions to partial pulpotomy in young permanent molars with deep caries may determine the efficiency of the procedure. The objective of this study was to determine the value of this procedure by analyzing the pulpal and periapical appearance of permanent molars after long-term follow-up periods. METHOD AND MATERIALS: Based on predetermined radiologic criteria, periapical radiographs of 49 young permanent molars of 31 females and 13 males, aged 6.9 to 17.7 years (mean, 11.4 years) treated with partial pulpotomy were evaluated and compared to their antimeres. Follow-up time ranged from 7 to 154 months (mean, 49 months). RESULTS: Based on clinical and radiographic findings, the success rate was considered 93.9% (46 of 49 teeth). Three teeth had chronic pulpitis with periapical exacerbation. In 14 (30.4%) of the 46 teeth, normal radiographic pulp appearance was found, and in 2 (4.3%), pulpal calcifications were similar to their antimere tooth. Dentinal bridges were observed underneath the partial pulpotomy site in 27 molars (58.7%), while radiopacities in the pulpotomized pulp horns of 5 teeth (10.9%) suggested development of obstructive calcifications. Increased calcifications of the pulp were seen in 13 (28.3%) molars, in comparison with their antimeres. Six of the 46 molars with immature root development completed full apexogenesis. No periapical pathoses, total pulp obliteration, or resorption were observed. CONCLUSION: The long-term radiologic interpretation of the pulp after partial pulpotomy in young permanent molars supports the clinical impression that it is a warranted treatment in well-chosen cases.

Absorption of nickel, chromium, and iron by the root surface of primary molars covered with stainless steel crowns.

Objective. The purpose of this study was to analyze the absorption of metal ions released from stainless steel crowns by root surface of primary molars. Study Design. Laboratory research: The study included 34 primary molars, exfoliated or extracted during routine dental treatment. 17 molars were covered with stainless-steel crowns for more than two years and compared to 17 intact primary molars. Chemical content of the mesial or distal root surface, 1 mm apically to the crown or the cemento-enamel junction (CEJ), was analyzed. An energy dispersive X-ray spectrometer (EDS) was used for chemical analysis. Results. Higher amounts of nickel, chromium, and iron (5-6 times) were found in the cementum of molars covered with stainless-steel crowns compared to intact molars. The differences between groups were highly significant (P < .001). Significance. Stainless-steel crowns release nickel, chromium, and iron in oral environment, and the ions are absorbed by the primary molars roots. The additional burden of allergenic metals should be reduced if possible.

Labial cervical vertical groove (LCVG) distribution and morphometric characteristics.

OBJECTIVE: Labial cervical vertical groove (LCVG), a dental anomaly found at the cervical region of upper permanent incisors, can be of esthetic, periodontal and prosthetic concern. The aim of this study was to define the prevalence and characteristics of LCVG. DESIGN: Of the 1250 examined subjects, 66 exhibited an LCVG. This group (age 19.7+/-11.4 years) was then compared with a matched No-LCVG (control) group of 49 subjects (age 17.9+/-5.7 years). Six LCVG characteristics were examined: distribution, severity and location of LCVG in central (I(1)) and lateral (I(2)) permanent maxillary incisors, Angle's classification, gender and presence of LCVG in siblings. RESULTS: Total LCVG prevalence was 5.3% (single 3.76%; multiple 1.53%), found predominantly in I(1) (93.9%) compared with I(2) (p=0.001). The anomaly presented mostly as a single configuration (71.21%); double configuration was predominantly homological bilateral, i.e., I(1)+I(1), or I(2)+I(2) (88%). LCVG in I(2) was mainly associated with multi-configuration (75%). The severe form occurred only in I(1). A mid-crown LCVG allocation was distinctive in I(1) (62.2%, p=0.001) and mesial-crown in I(2) (66.7%, p=0.038). LCVG was found mostly in males (p=0.045). In the LCVG group 16.7% were siblings. CONCLUSIONS: LCVG primarily involved I(1) in single configuration as the target assaulted tooth and secondarily I(2) mostly as a multi-configuration. The genetic impact on LCVG formation was primarily supported by the high prevalence in siblings and secondarily by the favourable occurrence in males, exclusive pattern of allocation within the crown and high occurrence of bilateral double-configuration LCVG.

Transitional implants in a patient with Williams-Beuren syndrome: a four-year follow-up.

Williams-Beuren syndrome (WBS) is characterized by several diagnostic features, including oligodontia. Restoration of congenitally missing teeth in patients with WBS is a challenge, both emotionally and clinically. In the atrophied alveolar bone process of a juvenile patient with WBS, the maxillary lateral incisors were replaced with two Immediate Provisional Implants (IPI). Early implant loading was used with temporary acrylic resin crowns that were replaced by semi-permanent restorations; the case has been followed for four years. Restoration of the missing maxillary lateral incisors with IPIs and temporary crowns proved to be a convenient, long-lasting and psychologically acceptable solution to the patient and her family.

Effect of different restorative materials on caries: a retrospective in vivo study.

OBJECTIVE: To compare, retrospectively, the influence of different types of restorative materials on caries in vivo. METHOD AND MATERIALS: Twenty-three primary teeth restored by a single operator with amalgam and 2 polyacid-modified resin composites, and with a band of tooth structure beyond the floor of the cervical box, were selected for microscopic evaluation. The teeth were sectioned mesiodistally with the aid of a hard tissue microtome, polished, and cleaned. A confocal laser scanning microscope was used to measure caries-related autofluorescence in the tooth tissue adjacent to the restorative material. A band of about 400 Mum along the interface between the restoration and the tooth structure was observed. The extent of the fluorescent regions was recorded in relation to the total interface length. The specimens were then categorized according to the restorative material used: group A: amalgam (Spherodon, Silmet, n = 11); group D: polyacid-modified resin composite (Dyract, Dentsply DeTrey, n = 6); and group C: polyacid-modified composite (Compoglass, Ivoclar Vivadent, n = 6. The data were analyzed using a one-way analysis of variance (ANOVA) model (alpha = .05) and Pearson correlation coefficient. RESULTS: No significant differences were found between the materials (P >.05). CONCLUSION: Within the limits of this clinical study, the data obtained using a small sample size suggests that the effect of the restorative material in primary molars does not have a predominant influence on secondary or residual caries.

Labial-cervical-vertical groove in maxillary permanent incisors--prevalence, severity, and affected soft tissue.

OBJECTIVES: To investigate the prevalence and severity of a labial-cervical-vertical groove (LCVG) in maxillary permanent incisors and its effect on the associated gingival tissue. METHODS: A total of 600 adolescents (293 boys and 307 girls, mean age 13.6+/-1.99 years) were randomly selected and examined for the presence of LCVG. The deformity was classified as mild, moderate, or severe according to predetermined criteria. Gingival coverage at the groove site was defined as normal, partial, and irregular. RESULTS: LCVG was found in 27 adolescents (4.5%). It was unilateral in 24 (89%) and bilateral in 3 (11%). The ratio of central to lateral incisors was 29:1. No sexual dimorphism or side prevalence were found. Mild LCVG was found in 22 incisors, moderate LCVG in 7 incisors, and severe LCVG in one incisor. Moderate LCVG was 5 to 6 times more susceptible to partial or irregular coverage of the gingival margin than mild LCVG. The gingival sulcus in teeth with LCVG demonstrated a significant (P = .001) increase in depth compared to non-LCVG teeth (1.55+/-0.90 mm vs 1.18+/-0.75 mm). CONCLUSIONS: An LCVG is a deformity confined predominantly to a single permanent maxillary central incisor. Its prevalence is not connected with gender. Most LCVGs are mild and often difficult to detect. However, the greater the severity, the more gingival irregularity is associated. This and the increase in sulcus depth in LCVG incisors are adverse predispositions for periodontal sequelae, calling for cautious oral hygiene maintenance.

The effect of hereditary disorders on tooth components: a radiographic morphometric study of two syndromes.

OBJECTIVE: The purpose of this study was to compare tooth components (enamel and dentin) in Familial Dysautonomia (FD) and Down syndrome (DS) in order to assess the extent to which each was affected. DESIGN: The design was cross-sectional. The sample consisted of 20 FD patients and 45 DS patients. The control group comprised 250 healthy subjects. Mesio-distal crown width (CW), enamel and dentin thickness and pulp chamber dimensions were measured on standardized bitewing radiographs of mandibular second primary and first permanent molars. Statistical analyses were performed between groups using SAS programs. RESULTS: CW was reduced in both hereditary disorders. In the DS group enamel height (EH) and dentin thickness were reduced. In FD enamel thickness in the primary and permanent molars as well as dentin height (DH) in permanent molars was increased. CONCLUSIONS: In both syndromes the reduction in CW suggests reduced proliferation during tooth germ formation. However, the differences in enamel and dentin thickness suggest that ameloblasts and odontoblasts were affected differently in the later phases of cell function. In FD cell function is stimulated resulting in thicker enamel and dentin. In DS cell function is reduced resulting in thin enamel and dentin.

Alveolar bone height in infraoccluded primary teeth.

The purpose of this research is to describe the distance from the cemento enamel junction (CEJ) to the alveolar bone crest (ABC) of infra occluded primary molars and the adjacent and opposing teeth. Bitewing radiographs from 29 children (mean age 98.8 months; SD 21.2), who had infra occluded molars, were scanned and measured. The results of these measurements found that the means of the CEJ-ABC distances of the mesial and distal aspects of the infra occluded teeth were 0.78 mm (SD 0.3) and 0.94 mm (SD 0.32) respectively. Pearson correlation coefficient analysis revealed no significant correlation between the different measurements, except when comparing the measurements in the infra occluded tooth and those of the opposing tooth. It was concluded that the CEJ-ABC values for infra occluded primary molars are shorter than normal values. The CEJ-ABC distances of the opposing and adjacent teeth to the infra occluded tooth are within normal limits.

Submandibular and sublingual salivary gland function in familial dysautonomia.

OBJECTIVE: Drooling in familial dysautonomia (FD) has been attributed to denervation supersensitivity. The aim of this study was to investigate submandibular and sublingual (SM/SL) gland function in FD. STUDY DESIGN: SM/SL saliva was collected from 15 children with FD and from 31 healthy control subjects. The protein and electrolyte content and the salivary flow rate were determined in each subject. RESULTS: Children with FD displayed significantly elevated outputs of chloride, potassium, calcium, phosphorous, magnesium, and total protein. Salivary flow rates were significantly increased. Phosphorous concentration was statistically low. These results imply SM/SL hyperfunction at the acinar and ductal levels. The concentration of lysozyme, the activity of amylase, and the output of both were similar in patients and control subjects. CONCLUSION: SM/SL gland hyperactivity is a newly described abnormality in FD. At the acinar level, this hyperactivity is expressed with increased fluid, electrolyte, and protein output, and at the ductal level, with increased ion secretion and absorption rate. These changes may be the result of ongoing parasympathetic denervation characteristic in FD.