Hyper-IgE syndrome with recurrent infections--an autosomal dominant multisystem disorder.
BACKGROUND: The hyper-IgE syndrome with recurrent infections is a rare immunodeficiency characterized by recurrent skin and pulmonary abscesses and extremely elevated levels of IgE in serum. Associated facial and skeletal features have been recognized, but their frequency is unknown, and the genetic basis of the hyper-IgE syndrome is poorly understood. METHODS: We studied 30 patients with the hyper-IgE syndrome and 70 of their relatives. We took histories, reviewed records, performed physical and dental examinations, took anthropometric measurements, and conducted laboratory studies. RESULTS: Nonimmunologic features of the hyper-IgE syndrome were present in all patients older than eight years. Seventy-two percent had the previously unrecognized feature of failure or delay of shedding of the primary teeth owing to lack of root resorption. Common findings among patients were recurrent fractures (in 57 percent of patients), hyperextensible joints (in 68 percent), and scoliosis (in 76 percent of patients 16 years of age or older). The classic triad of abscesses, pneumonia, and an elevated IgE level was identified in 77 percent of all patients and in 85 percent of those older than eight. In 6 of 23 adults (26 percent), IgE levels declined over time and came closer to or fell within the normal range. Autosomal dominant transmission of the hyper-IgE syndrome was found, but with variable expressivity. Of the 27 relatives at risk for inheriting the hyper-IgE syndrome, 10 were fully affected, 11 were unaffected, and 6 had combinations of mild immunologic, dental, and skeletal features of the hyper-IgE syndrome. CONCLUSIONS: The hyper-IgE syndrome is a multisystem disorder that affects the dentition, the skeleton, connective tissue, and the immune system. It is inherited as a single-locus autosomal dominant trait with variable expressivity. (+info)
A review of glass ionomer restorations in the primary dentition.
Glass ionomer cements are tooth-coloured materials that bond chemically to dental hard tissues and release fluoride for a relatively long period. They have therefore been suggested as the materials of choice for the restoration of carious primary teeth. However, the clinical performance of conventional and metal-reinforced glass ionomer restorations in primary molars is disappointing. And although the handling and physical properties of the resin-modified materials are better than their predecessors, more clinical studies are required to confirm their efficacy in the restoration of primary molars. (+info)
Persistence of deciduous molars in subjects with agenesis of the second premolars.
The purpose of the present study was to investigate persistent primary second molars in a group of young people in their late twenties with agenesis of one or two second premolars. In 1982-83 it was decided, in connection with the orthodontic evaluation of 25 patients, to allow 35 primary molars (one or two in each patient) to remain in situ. All patients had mixed dentitions and agenesis of one or two premolars. The primary teeth were generally in good condition, although root resorption and infra-occlusion (compensated by occlusal composite onlays) occurred. In 1997, 18 of the 25 patients with a total of 26 retained primary molars were reexamined, comprising a clinical examination for exfoliation, extraction, loosening, and ankylosis, and a radiographic examination for root resorption, tooth morphology (crown and root), and alveolar bone contour. The examination showed that the degree of root resorption was unaltered in 20 of the 26 primary molars. In the permanent dentitions, where these primary molars persisted, there were no morphological deviations. Three of the six remaining primary molars had been extracted and three showed extensive resorption. In three of the 26 primary molars the infra-occlusion had worsened. The present study shows that persistence of primary second molars in subjects with agenesis of one or two premolars, and normal morphology of the permanent dentition can be an acceptable, semi-permanent solution for the patient. Whether this could also be an acceptable long-term solution will be shown by follow-up studies. (+info)
The long-term survival of lower second primary molars in subjects with agenesis of the premolars.
This study investigated 41 subjects, 13 male and 28 female, with agenesis of one or both lower second premolars, and with retained lower second primary molars. Intra-oral radiographs of 59 primary teeth were examined to judge the resorption of the mesial and distal roots, and were measured to record infra-occlusion of the primary teeth and tipping of the adjacent permanent teeth. The study commenced at 11-12 years of age. The mean age at the last measurement was 20 years 6 months (SD 3.62, range 13.6-31.8 years). During the observation period, only two of the 59 primary teeth were exfoliated. Five were extracted, two of which were replaced by upper third molar transplants. Beyond the age of 20 years no teeth were lost. Root resorption varied widely between individuals, but was slow. There was no typical pattern for development of infra-occlusion. Mean infra-occlusion was 0.47 mm (SD 1.13) at 11-12 years, increasing by less than 1.0 to 1.43 mm (SD 1.13) at age 17-18 years. At age 19-20 years, 55 per cent of teeth showed infra-occlusion between 0.5 and 4.5 mm, but 45 per cent showed no infra-occlusion. The space between the first molar and first premolar was a mean of 10.35 mm (SD 0.76) at age 10-12 years compared with the mean width of the second primary molar of 10.53 mm (SD 0.51). The space reduced by less than 0.5 mm to 9.95 mm (SD 1.50) at age 17-18 years. If primary molars are present at 20 years of age they appear to have a good prognosis for long-term survival. (+info)
Maturation of primary and permanent teeth in preterm infants.
AIMS: To elucidate the development of primary and permanent teeth and to interpret the effect of different calcium, phosphorus, and vitamin D supplementation in the neonatal period on dental maturation in preterm children. METHODS: Preterm infants were randomised to four groups to receive a vitamin D dose of 500 or 1000 IU/day and calcium and phosphorus supplemented or unsupplemented breast milk. The maturity of the primary and permanent teeth was recorded in 30 preterm children. Sixty children aged 2 years and 60 children aged 9-11 years served as controls. Bone mineral content/density was assessed in the preterm infants. RESULTS: The median (range) corrected teething age was 7 (2-16) months in preterm infants and 6 (2-12) months in controls (p = 0.43). The median (range) number of erupted teeth at 2 years of age was 16 (11-19) in preterm infants and 16 (12-20) in controls (p = 0.16). Maturation of the permanent teeth in the preterm infants was not delayed compared with the controls (mean Demirjian SDS 0.16 v 0.49, p = 0.14). Early dietary intake of either mineral or vitamin D did not affect maturation of the primary dentition in preterm children. Children receiving the higher vitamin D dose in the neonatal period had more mature permanent dentition than those receiving the lower dose, but mineral intake did not affect maturation of the permanent teeth. Dental maturation did not correlate with bone mineral status. CONCLUSIONS: This is the first longitudinal study to follow primary and permanent tooth maturation in the same preterm children. Premature birth has no appreciable late sequelae in tooth maturation. (+info)
Dental caries in the primary dentition in public nursery school children in Juiz de Fora, Minas Gerais, Brazil.
The aim of this study was to assess the prevalence of dental caries in the primary dentition and associated variables in low socioeconomic preschool children enrolled in public nursery schools in Juiz de Fora, Minas Gerais, Brazil. Four public institutions were selected by geographic criteria (two in the central region and two in the peripheral region). The study population comprised 338 children (181 boys; 157 girls) aged 2-6 years old. Dental caries was recorded using the decayed, missing, and filled teeth (dmf-t) index. Among the examined children, 50.6% were caries-free. The mean dmf-t index was 2.03. It was higher in the peripheral nursery schools (p<0.01). A trend towards a difference between sexes (p = 0.06) was observed. Logistic regression analysis selected a previous child's visit to dentist (p<0.001), geographic location of the public nursery school (p<0.01), and age (p<0.01) as predictive variables for the dmf-t index. The study showed the need for an oral health program for this population, including both curative and preventive measures in order to achieve the WHO/FDI goals for the year 2000, namely 50% of children free of caries at age 5-6 years. (+info)
A review of impacted permanent maxillary cuspids--diagnosis and prevention.
As impacted permanent maxillary cuspids occur in 1-2% of the population, the general dentist should know the signs and symptoms of this condition and the interceptive treatment. Features of buccal or palatal cuspid impaction include lack of canine bulges in the buccal sulcus indicating a lingual eruption path and possible impaction; lack of symmetry between the exfoliation and eruption of cuspids that may indicate palatal or lingual impaction; and abnormal mesiodistal location and angulation of the developing maxillary permanent cuspids on radiographs. Diagnosis of impacted cuspid teeth at age 8-10 years can significantly reduce serious ramifications, including surgical exposure and orthodontic alignment as well as root resorption of the lateral incisors. In specific cases, extraction of the primary maxillary cuspids can prevent impaction of the permanent maxillary cuspids and additional sequelae. (+info)
The association between caries and childhood lead exposure.
Epidemiologic studies suggest an association between lead exposure and caries. Our objective was to establish whether children with a higher lead exposure as toddlers had more caries at school age than children with a lower lead exposure. We used a retrospective cohort design. A sample of children who attended second and fifth grades in the Rochester, New York, public schools during the 1995-1996 and 1996-1997 school years were examined for caries through a dental screening program. For each child we assessed the number of decayed, missing, or filled surfaces on permanent teeth (DMFS), and the number of decayed or filled surfaces on deciduous teeth (dfs); the number of surfaces at risk (SAR) was also recorded. Lead exposure was defined as the mean of all blood lead levels collected between 18 and 37 months of age by fingerstick [provided the blood lead level was [less than/equal to] 10 microg/dL)] or venipuncture. A total of 248 children (197 second graders and 51 fifth graders) were examined for caries and had a record of blood lead levels to define lead exposure. The mean dfs was 3.4 (range 0-29); the mean DMFS was 0.5 (range 0-8). Logistic regression was used to examine the association between the proportion of children with DMFS [Greater/equal to] 1, and the proportion with dfs [Greater/equal to] 1, and lead exposure [< 0.48 micromol/L vs. [Greater/equal to] 0.48 micromol/L (< 10 microg/dL vs. [Greater/equal to] 10 microg/dL)] while controlling for SAR, age at examination, and grade in school. For DMFS, the adjusted odds ratio was 0.95 [95% confidence interval (CI), 0.43-2.09; p = 0.89); for dfs, the odds ratio was 1.77 (95% CI, 0.97-3.24; p = 0.07). This study did not demonstrate that lead exposure > 10 microg/dL as a toddler was a strong predictor of caries among school-age children. However, the results should be interpreted cautiously because of limitations in the assessment of lead exposure and limited statistical power. (+info)