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PubMed1983

Dentinogenesis imperfecta in the Brandywine isolate (DI type III): clinical, radiologic, and scanning electron microscopic studies of the dentition.

Teeth of seven patients from the Brandywine isolate who had dentinogenesis imperfecta (DI) type III were evaluated by clinical, radiologic, and scanning electron microscopic techniques. The deciduous and permanent teeth were opalescent, and there was marked attrition. Enamel pitting was present on some permanent teeth. Anterior open bites were found in all persons with complete permanent dentitions. Pulps of developing teeth were larger than normal during early development but rapidly became almost completely obliterated. There was increased constriction at the cementoenamel junctions. While radiolucencies were noted at the apices of teeth which had pulp exposures due to attrition, several patients had similar radiolucencies which could not be attributed to caries or attrition. Scanning electron microscopy showed a significant reduction in the number of dentin tubules on fractured dentin surfaces; calcospherites at the calcification front were either irregularly shaped or absent. A single tooth from a patient with DI type II was studied and had similar abnormalities on scanning electron microscopy, although tubules were easier to find and calcospherites at the calcification front were more regular than in DI type III. The findings in DI type III of enamel pitting, enlarged pulps early in tooth development, and radiolucencies at the apices of teeth without pulp exposures support the hypothesis that DI type II and DI type III are different disorders.

Adolescent↗
PubMed1983

Dentinogenesis imperfecta with dens in dente.

An uncommon case of dentinogenesis imperfecta with dens in dente was studied by light microscopy, microradiography, and electron-microprobe analysis. Numerous canals and cellular inclusions were found in the coronal portion of the dentin where the tubules were sparse and irregularly distributed. In the radicular portion of the molar, large canals ran parallel to each other down to the apical region. The cementum was thinner and the Tomes granular layer greater in amount than usual. A dens in dente occurred in the radicular portion of the molar. It was located in a cavity and consisted of a mineralized cap of enamel, a spherical dentinal mass, and cellular cementum, located outside the cavity and in the "cervical" portion of the dens in dente.

Adolescent↗
PubMed1985

Dentinogenesis imperfecta type III with enamel and cementum defects.

Ground sections and scanning electron microscopic studies of primary teeth from a 2-year-old child whose teeth showed absence of enamel and large pulp chambers are examined. The findings suggest a diagnosis of odontodysplasia in a case classified as dentinogenesis imperfecta type III.

Child, Preschool↗
PubMed2001

Novel COL1A1 mutation (G559C) [correction of G599C] associated with mild osteogenesis imperfecta and dentinogenesis imperfecta.

A genotype-phenotype analysis of a three-generation family segregating for an autosomal-dominant osteogenesis imperfecta (OI) variant is reported here. The family was ascertained through the presentation of a proband concerned about discoloration of her teeth, found to be dentinogenesis imperfecta (DGI). Examination of 36 family members identified 15 individuals with DGI. Linkage studies were performed for genetic markers from candidate intervals known to contain genes responsible for DGI on chromosomes 4q, 7q, and 17q. Conclusive evidence for linkage of DGI was obtained to genetic markers on chromosome 17q21-q22 (DLX-3, Z(max) = 5.34, theta = 0.00). All DGI-affected family members shared a common haplotype, which was not present in individuals without DGI. Haplotype analysis sublocalized the gene to a 5-cM genetic interval that contained the collagen 1 alpha 1 (COL1A1) gene. More than 150 different COL1A1 gene mutations have been associated with various forms of OI, and five of these have been associated with DGI and type IV OI. After excluding these five mutations, mutational analysis was performed on the remaining exons including intron--exon boundaries, which resulted in identification of a Gly559Cys mutation in exon 32, present in all DGI-affected family members. Clinical features segregating with this G559C mutation included hyperextensible joints, joint pain and an increased propensity for bone fractures with moderate trauma. This is the first report of joint pain associated with a COL1A1 mutation and DGI. The mild skeletal features and reduced penetrance of the non-dental findings illustrate the importance of genetic evaluations for families with a history of DGI.

Amino Acid Substitution↗
PubMed1998

Dentinogenesis imperfecta: a case report.

Dentinogenesis imperfecta is a rare structural anomaly that is not frequently encountered in an orthodontic office. This case report of the treatment of a patient with this condition is presented in the hope that it will add to the knowledge of this hereditary condition.

Child↗
PubMed2001

DSPP mutation in dentinogenesis imperfecta Shields type II.

We identified a nonsense mutation (Gln45stop) in exon 3 of the dentin sialophosphoprotein (DSPP) gene in a Chinese family with dentinogenesis imperfecta Shields type II (DGI-II), in which the affected members showed discoloration and severe attrition of their teeth, with obliterated pulp chambers.

Asian People↗
PubMed2001

Dentinogenesis imperfecta 1 with or without progressive hearing loss is associated with distinct mutations in DSPP.

Dentinogenesis imperfecta 1 (DGI1, MIM 125490) is an autosomal dominant dental disease characterized by abnormal dentin production and mineralization. The DGI1 locus was recently refined to a 2-Mb interval on 4q21 (ref. 1). Here we study three Chinese families carrying DGI1. We find that the affected individuals of two families also presented with progressive sensorineural high-frequency hearing loss (gene DFNA39). We identified three disease-specific mutations within the dentin sialophosphoprotein gene (DSPP) in these three families. We detected a G-->A transition at the donor-splicing site of intron 3 in one family without DFNA39, a mutation predicted to result in the skipping of exon 3. In two other families affected with both DGI1 and DFNA39, however, we identified two independent nucleotide transversions in exons 2 and 3 of DSPP, respectively, that cause missense mutations of two adjacent amino-acid residues in the predicted transmembrane region of the protein. Moreover, transcripts of DSPP previously reported to be expressed specifically in teeth are also detected in the inner ear of mice. We have thus demonstrated for the first time that distinct mutations in DSPP are responsible for the clinical manifestations of DGI1 with or without DFNA39.

Asian People↗
PubMed2005

A deletion in the gene encoding sphingomyelin phosphodiesterase 3 (Smpd3) results in osteogenesis and dentinogenesis imperfecta in the mouse.

The mouse mutation fragilitas ossium (fro) leads to a syndrome of severe osteogenesis and dentinogenesis imperfecta with no detectable collagen defect. Positional cloning of the locus identified a deletion in the gene encoding neutral sphingomyelin phosphodiesterase 3 (Smpd3) that led to complete loss of enzymatic activity. Our knowledge of SMPD3 function is consistent with the pathology observed in mutant mice and provides new insight into human pathologies.

Animals↗
PubMed2002

Diagnostic features and pedodontic-orthodontic management in dentinogenesis imperfecta type II: a case report.

Dentinogenesis imperfecta type II, also known as hereditary opalescent dentin, is an isolated inherited condition transmitted as an autosomal dominant trait affecting the primary and permanent dentition. The combined pedodontic-orthodontic management of a 4-year-old child is described. Following orthodontic analysis to encourage a favourable growth outcome, treatment comprised restoration of the primary teeth with stainless steel crowns and composite crowns. Differential diagnosis and alternative therapies, including orthodontic considerations, are discussed.

Cephalometry↗
PubMed2003

Assessment of dysplastic dentin in osteogenesis imperfecta and dentinogenesis imperfecta.

Two semiquantitative scoring systems, Clinical Radiographic Score (CRS) and Dysplastic Dentin Score (DDS), were introduced for analyzing degree of dysplastic manifestations in dentin. The utility of both systems was demonstrated in a large material of teeth from patients with dentinogenesis imperfecta (DI) and osteogenesis imperfecta (OI). Twenty teeth from healthy controls, 81 teeth from 40 patients with OI, and 18 teeth with DI without OI (DI type II) were examined. The degree of dysplasia was correlated with type and form of OI and type of DI. The median DDS did not differ between DI associated with OI (DI type I) and DI type II. DDS in OI patients without clinical signs of DI was above that of control teeth. Both circumpulpal and mantle dentin showed increased DDS, although circumpulpal dentin was more severely affected. The median DDS was highest for the most severe type of non-lethal OI (type III). DDS increased significantly with form (severity) of OI. A significant association between DDS and CRS was found, although diagnosis of DI in less severe cases was not possible based on radiographic or clinical signs alone. Thus, the DDS system proved valuable when the CRS system based on radiographic/clinical manifestations failed, the most significant finding being subclinical histological manifestations of DI in patients with OI but without clinical or radiographic signs of DI. These subtle dysplastic changes are most likely an expression of genetic disturbances associated with OI and should not be diagnosed as DI, but rather be termed histologic manifestations of dysplastic dentin associated with OI.

Adult↗
PubMed2003

Orthodontic and orthognathic management of a patient with osteogenesis imperfecta and dentinogenesis imperfecta: a case report.

This case report describes a patient's severe Class III malocclusion, managed with a combination of orthodontic and orthognathic treatment. The medical history was complicated by osteogenesis imperfecta and dentinogenesis imperfecta. In addition the patient was a Jehovah's Witness. Patients with osteogenesis imperfecta carry an increased risk of perioperative haemorrhage, and this led to bimaxillary surgery being carried out as two discrete surgical episodes for the patient described. In addition, the risk of enamel fracture led to orthodontic bands being cemented on all teeth. In spite of the increased risks a successful outcome was achieved.

Adolescent↗
PubMed1981

Prosthetic treatment of dentinogenesis imperfecta. A case report.

Prosthetic reconstruction was made on dentition affected by dentinogenesis imperfecta. The bite was first raised with fixed bite plates, and then with temporary acrylic bridges; finally full gold and veneer crowns were made in the posterior teeth. The crowns were retained on the severely attrited teeth with self-threading parapulpal screws (TMS, Whaledent International, New York, U.S.A.) and amalgam and composite resin (Consise, 3M Company, St Paul, U.S.A.) posts. The teeth seem to have enough strength to withstand the occlusal forces, and the dentine appears hard enough to retain parapulpal screws. The prosthetic construction is still functioning well today, 5 years after starting treatment.

Acrylic Resins↗
PubMed1988

A probable common disturbance in the early stage of odontoblast differentiation in Dentinogenesis imperfecta type I and type II.

Deciduous teeth from 7 patients with dentinogenesis imperfecta (DI) Type I and Type II were examined by conventional microscopy. A defective layer was found which runs parallel with the dentinal surface in the outer portion of dentin in teeth of both types. Dentinal tubules were interrupted in the vicinity of this layer. When the ground sections were examined after being stained by the phosphophoryn staining method, the DI Type I dentin was found to contain phosphophoryn at the same low level as the DI Type II dentin, suggesting similar deficiency in phosphophoryn concentration. These results suggest that both types of DI have a common primary disturbance in the early stage of odontoblast differentiation.

Cell Differentiation↗
PubMed1994

Hyperfibers and vesicles in dentin matrix in dentinogenesis imperfecta (DI) associated with osteogenesis imperfecta (OI).

Dentin matrix of deciduous teeth from two patients affected by dentinogenesis imperfecta (DI) associated with types IB and IVB osteogenesis imperfecta (OI) displayed previously undescribed structures in transmission electron microscopic examination. Vesicles were seen in dentin of both patients, and abnormally thick collagen fibers (hyperfibers) were found in dentin of the patient with the rare type IB OI. Both vesicles and hyperfibers were situated in abnormal, atubular areas of dentin. Matrix vesicles, which have normally been identified in mantle dentin only, were abundant in selected areas of the affected dentin, thereby supporting the concept that dentin matrix in OI is elaborated by successive cell generations. The hyperfibers, not previously described in either normal or abnormal human dentin, have possibly been formed by fusion of several collagen fibers. Further ultrastructural studies of dentin in DI with OI may help to clarify the marked clinical variation in teeth of patients affected by OI.

Child↗
PubMed1996

Mild forms of dentinogenesis imperfecta in association with osteogenesis imperfecta as characterized by light and transmission electron microscopy.

Osteogenesis imperfecta (OI) results from various gene mutations leading to defects in type I collagen, which is the major component of both bone and dentin. Yet dentinogenesis imperfecta (DI) is found only in half of the patients with OI. Here we document patients from three families with OI and DI lacking the clinical and radiographic features of DI in permanent teeth. However, light and transmission electron microscopic studies of dentin of deciduous and permanent teeth revealed various changes in the morphology of the dentinal tubules and collagen fibers. In one family, diagnosis of DI preceded that of OI. The grade of severity of dentinal manifestations in patients with OI apparently forms a continuum from normal dentin structure to severe DI, and the marked difficulty in diagnosing mild DI may have led to underestimating its frequency. Furthermore, patients with DI should be carefully examined for the possible presence of OI.

Child↗
PubMed1976

The effect of vincristine on dentinogenesis in the rat incisor.

A preliminary report on the histomorphologic effects of vincristine on rat incisor dentinogenesis is presented. Niche-like areas of osteodentin and a faint incremental line in the dentin were the main derangements observed after single injections of the drug. The severity of the lesions was dose-dependent.

Animals↗