In an epidemiologic study in the middle of Sweden comprising 425 000 children age 3-19 yr, 105 children were diagnosed as having hereditary amelogenesis imperfecta (HAI). The material then was primarily classified upon clinical criteria into different subgroups either associated to a hypoplastic or to a hypomineralized type of HAI. Analyzing available genetic data obtained from 95 children in 76 families and further 11 adults in 10 families, eight different entities of HAI could be identified in a classification of HAI. In both of the basic types of HAI, the hypoplastic and the hypomineralized, autosomal dominance was the most common mode of inheritance, even if an autosomal recessive inheritance could be identified in some of the subgroups of HAI. The hypoplastic type (rough-pitted), autosomal dominance with incomplete penetrance was dominating in the material, 47 out of 105 cases. The clinical classification seemed to be relevant when testing the material on a genetic basis showing that the clinical criteria provide a strong support for a classification of HAI.
Persson, M. & Sundell, S. Facial morphology and open bite deformity in amelogenesis imperfecta. A roentgenocephalometc study. Acta Odontol. Scad. 1982, 40, 135-144 Occlusal conditions and skeletal facial morphology in patients with amelogenesis imperfecta were studied in order to reveal a su posed relationshi between skeletal moyholoy and a high prevalence of opengite. Records and gntal casts were availab e for 6 cases, and lateral ce halograms in 19 of these. An open bite was present in 11 patients of both sexes, gut was more frequent in males than in females. It occurred in cases with enamel defects of hypomineralisation type as well as in those with bypoplasia. 15 linear and 10 angular measurements from the ce halograms were compared with those of matched controls. Both males and fem$a with enamel defects were found to have significant skeletal features, characteristic of the swcalled open bite skeletal deformity. The skeletal deviation was more marked in males than in females. The pattern of an open bite skeletal deformity, although less marked, was also present in patients with the enamel defect but without an open bite occlusion. The open bite occlusion frequently found in cases of amelogenesis imperfecta is therefore considered to be of skeletal origin, although the causal relationship between the enamel defect and the skeletal deformity remains to be explored.
A novel entity of long-standing OML that share some features with orofacial granulomatosis has been identified in children after solid organ transplantation.
Myotonic dystrophy or dystrophia myotonica (DM) is a hereditary neuromuscular multisystem disease with a varying clinical expressivity and severity. The objective of this study was to assess the oral health in children with myotonic dystrophy and to compare it with a control group. Fifty-six DM patients, aged 2.7-18.0 yr, were compared with age- and gender-matched control patients with respect to caries, plaque, and gingivitis. Oral function and signs of temporomandibular dysfunction (TMD) were assessed, and the ability to co-operate in dental treatment was estimated. Questionnaires concerning eating habits, dental care, traumatic injuries to teeth, and orofacial function were also used. The DM patients had significantly more caries, plaque, and gingivitis than did control patients. They had more TMD problems and lower co-operation ability than the healthy control persons. General sedation was frequently needed to carry through dental treatment. DM patients are at risk of caries, gingivitis and TMD problems, and need intensified prophylactic care. Behavior management problems are common.
DM1 patients, as they grow older, have increasing amounts of plaque and risk of caries and gingivitis. They have more TMD problems. Behaviour management problems do not seem to decrease with age. Increased prophylactic care is essential for DM1 patients.
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