Dentin dysplasia, Type II: A rare autosomal dominant disorder

Melnick, Michael; Eastman, John Russell; Goldblatt, Lawrence I.; Michaud, Monique; Bixler, David

doi:10.1016/0030-4220(77)90303-6

Cited by 18 publications

(10 citation statements)

References 4 publications

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“…A family with an autosomal dominant disorder, characterized by DD Type I and progressive generalized cortical hyperostosis, has been described (4). Type II DD shares some common features with DD Type I in the primary dentition, but the permanent teeth in Type II exhibit thistle-tube-shaped pulps with dentieles (1,(5)(6)(7)(8)(9)(10)(11). Defects resembling DD Type II have also been reported in patients with osteogenesis imperfecta (01) but not with DI Type I (12,13).…”

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confidence: 99%

Dentin dysplasia Type II: absence of Type III collagen in dentin

Ranta¹,

Lukinmaa²,

Knif³

1990

J Oral Pathology Medicine

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A three-generation family with dentin dysplasia (DD) Type II is presented. Affected family members share common radiologic features with clinically varied expression of tooth discoloration and occlusal wear. Both the primary and the permanent dentition appear to be affected. No generalized connective tissue involvement is found. The mode of inheritance is autosomal dominant. Histologically, the findings are consistent with DD Type II. In indirect immunofluorescence, the irregular radicular dentin of an affected permanent tooth failed to stain with specific antibodies against Type III collagen and the N-terminal propeptide of Type III procollagen.

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confidence: 99%

Dentin dysplasia Type II: absence of Type III collagen in dentin

Ranta¹,

Lukinmaa²,

Knif³

1990

J Oral Pathology Medicine

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“…Inheritance of DD 11 is autosomal dominant [Shields et al, 1973;Melnick et al, 1977;Jasmin and Clergeau-Guerithault, 19841 and no associated systemic abnormalities have been reported. Neither congenitally missing teeth nor radiolucencies at the apices of teeth have been consistently described in DD 11.…”

Section: Discussionmentioning

confidence: 99%

Osteogenesis imperfecta type I with unusual dental abnormalities

et al. 1988

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A large kindred with dominantly inherited osteogenesis imperfecta was evaluated. Affected individuals had bone fractures, blue sclerae, and hearing loss. In addition, all had dental abnormalities distinct from those previously described in other families with this syndrome. Deciduous teeth were normal in color or blue-grey. On radiographs of an early developing deciduous dentition, pulps were larger than normal. In patients with mixed dentitions, pulp chambers of deciduous teeth were partially obliterated. Increased constriction at the junctions of the crowns and roots was found in some deciduous teeth. One patient had large pulp stones in the pulp chambers of all maxillary deciduous molars. Permanent teeth were normal in color but had oval pulp chambers with apical extensions into the coronal portions of the roots, large coronal pulp stones, narrow root canals, and thin roots. Individuals in this family who did not have osteogenesis imperfecta had normal teeth. In addition, a well circumscribed radiolucency without a sclerotic periphery, involving the apices of all permanent mandibular incisors, was found in the anterior mandible in one patient. These findings support the hypothesis that this family has yet another type I osteogenesis imperfecta "syndrome".

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“…The permanent teeth appear normal with respect to shape, form, and color in most cases, although the pulp cavities of permanent teeth may show a thistle‐tube deformity and commonly contain pulp stones. Unlike in DD‐I, the roots are of normal length and idiopathic periapical radiolucencies are not observed . To date, fewer than 90 families/cases of DD have been reported in the literature, including two cases of DD‐II and one case of DD‐III.…”

Section: Classification Of Hereditary Dentin Disordersmentioning

confidence: 99%

“…To date, fewer than 90 families/cases of DD have been reported in the literature, including two cases of DD‐II and one case of DD‐III. These reports included clinical, radiographic, and sometimes histological investigations .…”

Section: Classification Of Hereditary Dentin Disordersmentioning

confidence: 99%

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Dentin disorders: anomalies of dentin formation and structure

Coster¹

2009

Endodontic Topics

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Dentin defects represent a cluster of relatively rare heritable disorders affecting the formation and mineralization of dentin. Major clinical features include loss of enamel with extreme wear of exposed dentin, opalescent tooth discoloration, short roots with pulpal calcification and increased tooth mobility, and frequent periapical radiolucencies. Historically, these inherited conditions have been classified by Shields et al. (1) as either dentinogenesis imperfecta (DGI) types I, II and III, or dentin dysplasia (DD) types I and II. DGI type I is now universally designated as osteogenesis imperfecta with dentinogenesis imperfecta (OI/DGI) and is caused by type I collagen mutations. Among the genes expressing the non‐collagenous proteins in dentin, only the DSPP gene has been implicated in the etiology of DD type II and all DGI types, suggesting that heritable dentin defects can be viewed as a continuum rather than as distinct disease entities. Dental treatment strategies should aim to preserve the affected dentitions by protecting teeth from wear, restoring them to compliance with the specific structural characteristics of the defective dentin, and anticipating early tooth loss. Endodontic treatment of the affected teeth is very challenging because of the peculiar pulp morphology and most often has poor prognosis. If conventional therapy is not an option, periapical curettage and retrograde filling is a possible alternative, except in the case of teeth with short roots.

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Dentin dysplasia, Type II: A rare autosomal dominant disorder

Cited by 18 publications

References 4 publications

Dentin dysplasia Type II: absence of Type III collagen in dentin

Dentin dysplasia Type II: absence of Type III collagen in dentin

Osteogenesis imperfecta type I with unusual dental abnormalities

Dentin disorders: anomalies of dentin formation and structure

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