The deciduous and permanent teeth in some patients with osteogenesis imperfecta syndromes are blue or brown and opalescent. As shown by radiologic examination, the pulp chambers and root canals are completely or partially obliterated by abnormal dentin. The junctions between the crowns and roots are more constricted than normal. Deciduous opalescent teeth lose their enamel readily and wear more easily than normal. Unusual wear does not occur as frequently in permanent opalescent teeth as in deciduous teeth. No relationship has been shown between the number of fractures or deformity and the degree to which the teeth are affected. In contrast, other patients with osteogenesis imperfecta have normal teeth. These clinical differences in the dentitions support the concept of genetic heterogeneity is osteogenesis imperfecta and provide information useful in genetic counseling. The differential diagnosis of opalescent teeth in osteogenesis imperfecta is not difficult provided that detailed clinical and radiologic examinations of the dentition are performed. Patients with osteogenesis imperfecta and opalescent teeth should be evaluated as soon as the deciduous teeth erupt, so that an attempt can be made to prevent loss of tooth structure. However, methods of dental care in osteogenesis imperfecta are not well delineated and deserve further study.

Amelogenesis Imperfecta↗

PubMed1981

Self-care: towards fundamental changes in national strategies.

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Health Education↗

PubMed1980

Hearing patterns in dominant osteogenesis imperfecta.

Audiologic evaluations and impedance audiometry were obtained on persons from 13 families with dominant osteogenesis imperfecta (OI). Hearing impairment appeared in the late second or early third decade, usually with a conductive loss. Older patients had mixed or sensorineural losses. There was an increasing risk for hearing loss with advancing age. Impedance tests suggested that ossicular fixation may be the primary cause of middle ear disorders in patients with mixed or conductive hearing losses. The cause of sensorineural hearing loss could not be determined. The long-term success of otologic surgery in OI is uncertain. Amplification may be a reasonable alterative to surgery.

Acoustic Impedance Tests↗

PubMed1980

Scanning electron microscopy of teeth in dominant osteogenesis imperfecta: support for genetic heterogeneity.

Scanning electron microscopic studies were performed on 25 deciduous and permanent teeth from members of 7 kindreds with dominant nonlethal osteogenesis imperfects (OI). Two families had normal teeth on clinical and radiological examination; five families had blue or brown opalescent teeth with specific radiologic findings. Enamel surfaces and prism organization were normal on all teeth. On fractured surfaces, the dentin tubules of normal teeth from patients with OI were evenly distributed and coursed regularly to the dentin-enamel junction. Opalescent teeth had few tubules and those present were short, narrow, and tortuous. Dentin calcification fronts of normal teeth were composed of many nodules with regularly spaced openings on their surfaces. Calcification fronts of opalescent teeth were composed of irregularly spaced, small nodules, which varied greatly in size and the nodules lacked tubule openings on their surfaces. The results of this study support the concept that at least two dominant forms of OI exist--one in which all individuals with IO have normal teeth, and the other in which all with OI have blue or brown opalescent teeth with characteristic changes on SEM.

Dental Enamel↗

PubMed1980

Dentin dysplasia type I: a scanning electron microscopic analysis of the primary dentition.

Dentin dysplasia type I (DD-I) is a rare autosomal dominant disorder which affects both the deciduous and permanent dentitions. The affected deciduous and permanent teeth have short conical roots with sharp, apical constrictions and frequently periapical radiolucencies in the absence of caries. Apical to a thin layer of normal coronal dentin are large, calcified, dentin masses which nearly obliterate the pulp chamber and canals. Presented here are light microscopic and scanning electron microscopic observations of deciduous teeth from three unrelated persons with the disorder. In general, the deciduous teeth had (1) normal enamel, (2) a thin layer of normal dentin adjacent to the dentinoenamel junction, (3) a crescent-shaped pulpal remnant below the normal dentin, (4) dysplastic dentin masses (ranging from atubular to a few small tubules) between which are spaces presumed to previously have contained smaller remnants of the original mesenchymal dental papilla, and (5) root dentin, which is dysplastic throughout. The SEM-defined phenotype, however, was noticeably variable among all three persons. Based on the current concepts of tooth morphogenesis, it is most likely that the abnormal root morphology of DD-I teeth is secondary to the abnormal differentiation and/or function of the ectomesenchymally derived odontoblasts.

Adult↗

PubMed1980

Heterogeneity of retinal degeneration and hearing impairment syndromes.

Retinal abnormalities associated with hearing loss may be inherited alone or with additional manifestations. Environmental insults, such as rubella embryopathy, may also cause these abnormalities. We studied 13 patients with retinal abnormalities and hearing loss. Five had Usher's syndrome (retinitis pigmentosa and hearing impairment), one had crystalline retinopathy, two had associated enamel dysplasias, two had clumped pigmentary retinopathy, and three had Amalric-Diallinas syndrome. Our findings suggest considerable heterogeneity of syndromes involving retinal abnormalities and hearing loss.

Adolescent↗

PubMed1980

Self-care is not a solipsistic trap: a reply to critics.

This paper is an answer to criticism of the self-care, self-help movement in health recently advanced by Robert Crawford and other writers. The authors review the multiple and varied origins, motivations, and ideologies associated with self-care developments. It is maintained that the self-care movement embodies a broad, popular social resistance to the ills, inequities, and iatrogenic elements in highly technological health care systems. Empirical examination of specific programs and formulations of this movement reveals that it cannot be fitted into a simplistic "victim-blaming" ideology, but instead operates to decrease dependence and heighten individual and political/social awareness of hazards to health.

Attitude to Health↗

PubMed1980

Morquio-like syndrome (MPS IV B) associated with deficiency of a beta-galactosidase.

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Child↗