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R J Jorgenson

Publications and source records attributed to R J Jorgenson.

At least 73 records · Page 4Linked to original sources
PubMed1975

Oral findings in the Morquio syndrome (mucopolysaccharidosis IV).

The Morquio syndrome is characterized by a specific pattern of platyspondylia, corneal opacities, keratosulfate excretion in the urine, and dental abnormalities. Oral examinations were performed on twelve patients with the condition. The maxillary anterior teeth were widely spaced and flared. The posterior teeth were tapered and had pointed cusp tips. The enamel was of normal hardness, and in some patients the surface was pitted. In roentgenograms, the enamel was less than one fourth its normal thickness but was of normal radiodensity. The prevalence of caries may have been reduced. The hard palates were broad and flat. The dental abnormalities in the Morquio syndrome are of a type that is unique among the group of genetic mucopolysaccharidoses. Our findings therefore support the conjecture that the biochemical defect in this condition is different from that which occurs in the other mucopolysaccharidoses.

Adolescent↗
PubMed1975

Periodontosis in sibs.

Periodontosis is an idiopathic degeneration of the periodontium which results in migration and loss of teeth. The disease begins in the regions of the incisors and first molars. Late in the disease, other areas of the dental arches may be involved. The gingivae are not initially inflamed, and there are no associated systemic abnormalities. Local irritants cannot account for the marked alveolar destruction which leads to the tooth loss. Several heritable syndromes and periodontitis also may be associated with alveolar bone destruction. Periodontosis can be differentiated each of these on the basis of negative laboratory tests, lack of associated anomalies, distinctive pattern of bone loss and timing of onset of gingival inflammation. A family in which periodontosis was present in three of six sibs and in which ichthyosis was segregating independently of periodontosis is reported.

Adolescent↗
PubMed1975

Otodental dysplasia: a "new" ectodermal dysplasia.

Otodental dysplasia is an ectodermal dysplasia characterized by abnormal crown morphology of the teeth and sensorineural hearing loss. It was documented in six generations of a kindred of Italian extraction. Thirty-three of the 119 examined family members were affected. Twenty-six persons had characteristic dental anomalies combined with a hearing loss. Two had the dental anomalies alone, four had a hearing loss only and one of those with dental anomalies could not be tested for hearing. The teeth of the 29 with dental anomalies had large, bulbous crowns. The normal relationship between cusps and grooves was obliterated. Molars, both deciduous and permanent, were involved. Deciduous canines were large and bulbous. Absence of premolars was documented in 14 of the 29 individuals with abnormal teeth. Those premolars which were present were frequently small. Radiographs of the teeth showed denticles and taurodontia. Twenty-six of the 30 individuals with a hearing loss had concomitant dental anomalies. Of the four with isolated hearing loss, one was proven to have the syndrome. The remaining three were conjectured to be affected. The age of onset of the hearing deficit ranged from early childhood to middle age. The results of a chi-square test supported autosomal dominant inheritance. The pleiotropy in this syndrome is postualed to be due to a genetic defect in the neuroectoderm.

Adolescent↗
PubMed1975

Apert syndrome.

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Acrocephalosyndactylia↗
PubMed1974

K--apert syndrome.

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Abnormalities, Multiple↗