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IMAGe syndrome (OMIM 300290) is a rare multisystem disorder that has a broad phenotypic presentation. Though variable, this disorder mainly consists of Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital abnormalities. Patients with IMAGe syndrome present as an uncommon yet important challenge for dentists and anesthesiologists due to their wide range of dysmorphic facial features, adrenal insufficiency, electrolyte imbalances, and need for steroid replacement. The purpose of this case report is to describe the successful anesthetic management of a pediatric patient diagnosed with IMAGe syndrome who presented for full mouth dental rehabilitation. [ABSTRACT FROM AUTHOR]

Asthma and dental caries are common chronic health conditions of childhood. The aim of this study was to determine if severity of bronchial asthma is a risk factor for early childhood caries. Asthmatic children aged 71 months or younger were examined for their plaque and caries status and were compared with controls matched for age, sex and socioeconomic status. The results showed that asthmatics as a group had a lower DMFT score than the control group. New asthma management and educational programs may be having a positive spillover effect to the dental arena. [ABSTRACT FROM AUTHOR]

The major protein components of the enamel matrix include the most abundant amelogenin proteins as well as less plentiful proteins such as enamelin and ameloblastin. The enamel defect in amelogenesis imperfecta (Al) generally results in enamel that is too thin (hypoplastic) or too soft (hypocal-cification or hypomaturation). Previous reports indicate that mutations in the human enamelin gene (ENAM) cause hypoplastic Al through autosomal-dominant inheritance patterns and patients may also exhibit an anterior open bite. Although crown resorption of unerupted teeth occurs more freguently in Al patients, this finding has not been previously associated with known ENAM mutations. The purpose of this article was to report the genotype-phenotype correlations for a 9-year, 11-month-old boy with a homozygous ENAM mutation (c.1258_1259insAG). [ABSTRACT FROM AUTHOR]

The sideroblastic anemias comprise a heterageneous group of acquired and congenital disorders which that have in common: (1) anemia; (2) the presence of ring sideroblasts in the bone marrow; and (3) impaired heme biosynthesis. Elevation in iron levels is secondary to a deficiency in mitochondrial physiology. Patients exhibit mild to moderate hemolysis due to peripheral red blood cell destruction, and often require regular blood transfusions. The purpose of this case report was to describe the dental management of a child with congenital sideroblastic anemia-a rare hematologic disorder-and to emphasize how important it is for pediatric dentists to be cognizant of a patient's hematologic profile before attempting to perform any dental procedure. The timing of the patient's transfusions regarding the scheduling of dental care is crucial for successful management and anticipation of possible complications. Preventive dentistry should be a hallmark of dental management for these children. [ABSTRACT FROM AUTHOR]

The article offers information on the use of glass ionomer sealants on newly erupting permanent molars. Several advantages of glass ionomer include its ability to release ions that promote mineralization of the tooth. Details of the process of using the GC Fuji Triage chemical-set glass ionomer sealant from GC America are offered.

Although relatively rare, minor salivary gland tumors are more likely to be malignant in pediatric patients than in adults. Mucoepidermoid carcinoma (MEC) represents the most common malignant tumor of the salivary glands. It is critical to differentiate such tumors from common benign salivary gland lesions. he purposes of this report are to present the case of a 15-year-old female with MEC of the palate, and to discuss the importance of a thorough intraoral examination on all patients regardless of age, as well as the need for timely referral for appropriate diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Asthma and dental caries are common chronic health conditions of childhood. The aim of this study was to determine if severity of bronchial asthma is a risk factor for early childhood caries. Asthmatic children aged 71 months or younger were examined for their plaque and caries status and were compared with controls matched for age, sex and socioeconomic status. The results showed that asthmatics as a group had a lower DMFT score than the control group. New asthma management and educational programs may be having a positive spillover effect to the dental arena.

The purpose of this study was to determine graduating dental students' perceptions about their training and experience in examination and treatment of infants and their plans to examine infants upon graduation. A survey was distributed to dental students graduating from Pennsylvania dental schools in 2007 and 2008. Of the returned surveys, 47.9 percent correctly identified 12 months as the recommended age for the first dental exam. Sixty-five percent of responders felt they would be comfortable performing exams on young children. This study's primary objective was to test the association between performance of a clinical exam in a young child, enjoyment of clinical and didactic pediatric dental experience, plans for additional training in pediatric dentistry and willingness to see children younger than two years old in practice. Performing a clinical exam on a young child was associated to willingness to see children younger than two years of age in practice. Early exposure (lecture or clinical) to young pediatric patients while in dental school was significantly associated to perceived comfort with oral exam of young patients but not to reported willingness to see them in practice.

Asthma is a chronic lung disease exhibiting airway obstruction, hyperresponsiveness, and inflammation, characterized by the infiltration of eosinophils into the airways and the underlying tissue. Prolonged eosinophilic inflammation depends on the balance between the cell's inherent tendency to undergo apoptosis and the local eosinophil-viability enhancing activity. TRAIL, a member of the TNF family, induces apoptosis in most transformed cells; however, its role in health and disease remains unknown. To test the hypothesis that Ag-induced inflammation is associated with TRAIL/TRAIL-R interactions, we used a segmental Ag challenge (SAC) model in ragweed-allergic asthmatics and nonasthmatic patients and analyzed bronchoalveolar lavage (BAL) material for 2 wk. In asthmatic patients, the level of TRAIL in BAL fluid dramatically increased 24 h after SAC, which significantly correlated with BAL eosinophil counts. Immunohistochemical analysis of bronchial biopsies from asthmatic patients demonstrated that TRAIL staining was increased in epithelial, airway smooth muscle, and vascular smooth muscle cells and throughout the interstitial tissue after SAC. This was confirmed by quantitative immunocytochemical image analysis of BAL eosinophils and alveolar macrophages, which demonstrated that expression levels of TRAIL and DcR2 increased, whereas expression levels of the TRAIL-Rs DR4 and DR5 decreased in asthmatic subjects after SAC. We also determined that TRAIL prolongs eosinophil survival ex vivo. These data provide the first in vivo evidence that TRAIL expression is increased in asthmatics following Ag provocation and suggest that modulation of TRAIL and TRAIL-R interactions may play a crucial role in promoting eosinophil survival in asthma.