Ch 2 - Abnormalities of Teeth 4 Flashcards
Which syndrome is most frequently associated with dental manifestations that mimic dentinogenesis imperfecta,
Osteogenesis imperfecta
Besides Osetogenesis Imperfecta, which 3 syndromes mimic dentinogenesis imperfecta?
- Ehlers-Danlos syndrome
- Goldblatt syndrome
- Schimke immuno-osseous dysplasia
Besides the 4 syndromes that mimic dentinogenesis imperfecta, what are 4 systemic conditions that can mimic it?
- vitamin-D resistant rickets
- vitamin-D dependent rickets
- tumoral calcinosis
- calcinosis universalis
What is the primary gene in guiding the proteins that create dentin?
DSPP gene (dentin sialophosphoprotein)
A hereditary developmental disturbance of the dentin in the ABSENCE of any systemic disorder and has been shown to be associated with any one of a number of mutations of the DSPP gene
Dentinogenesis Imperfecta
What % of patients with Osteogenesis Imperfecta show similar dental changes as dentinogenesis imperfecta? Thus the difference is what gene mutation?
50% of these OI patients have DI like dental changes, so these patients have the COL1A1/COL1A2 gene mutations for OI
osteogenesis imperfecta with “______” teeth is clearly a separate disease from dentinogenesis imperfecta
“opalescent” teeth
What was the phenotype of the old classification dentinogenesis imperfecta III - Brandywine isolate? (which is now just a variation of DI)
shell teeth (deciduous teeth)
Which dentin dysplasia type is associated with the DSPP gene mutation (same as dentinogenesis imperfecta)?
dentin dysplasia type II
What are two alternate names for dentinogenesis imperfecta?
- hereditary opalescent dentin
2. Capdepont’s teeth
Dentinogenesis imperfecta is NOT randomly distributed throughout the United States and Europe…..where do most cases stem from? What is the prevalence in whites in the US?
English and French along the English channel…1:8,000 whites in the US
Which teeth are most severely affected in Dentinogenesis Imperfecta? Which one are least?
primary teeth the most, 3rd molars least altered
While Dentinogenesis Imperfecta can cause _________ due to excess dentin production, some teeth can have expanded pulp tissue and are called ________
pulpal obliteration….shell teeth
Several kindreds affected with dentinogenesis imperfecta also have been shown to demonstrate what other condition?
progressive, sensorineural, high-frequency hearing loss.
What are the two radiographic descriptions for dentin dysplasia type II?
thistle tube-shaped or flame-shaped pulp chambers
What other condition has similar radiographic findings to dentin dysplasia II? What is the difference?
Pulpal dysplasia…PD teeth look normal clinically (both PD and DD-II have thistle pulps and pulp stones)
In patients with dentin dysplasia II, what pattern do the deciduous teeth resemble?
same pattern as dentinogenesis imperfecta (pulpal obliteration or shell teeth)
By definition, dentin dysplasia should have NO correllation with ________
systemic disease
Dentin dysplasia has no correllation with systemic disease, but these 4 diseases have dentin-dysplasia LIKE alterations…
- Calcinosis universalis ( the deposit of calcium salts in skin, subcutaneous tissue, tendons and muscles)
- Rheumatoid arthritis
- Hypervitaminosis D
- Sclerotic bone and skeletal anomalies
- Tumoral calcinosis
What is the alternate name for dentin dysplasia I? What is the buzz term seen in this disorder?
radicular dentin dysplasia…ROOTLESS teeth
inheritance pattern and prevalence for dentin dysplasia I?
AD…1:100,000
Name that condition: teeth are normal clinically, radiographically they are normal in shape but demonstrate a radiodense product filling the pulp chambers and canals…whats the inheritance pattern of this condition?
fibrous dysplasia of dentin…AD
Subclassify that Dentin Dysplasia I why dont you: No pulp chambers, no root formation, and frequent
periapical radiolucencies
DDIa
Subclassify that Dentin Dysplasia I why dont you: A single small horizontally oriented and crescentshaped
pulp, roots only a few millimeters in length, and
frequent periapical radiolucencies
DDIb
Subclassify that Dentin Dysplasia I why dont you: Two horizontally oriented and crescent-shaped pulpal
remnants surrounding a central island of dentin, significant but shortened root length, and variable periapical radiolucencies
DDIc
Subclassify that Dentin Dysplasia I why dont you: Visible pulp chambers and canals, near normal root
length, enlarged pulp stones that are located in the coronal portion of the canal and create a localized bulging of the canal and root, constriction of the pulp canal apical to the stone, and few periapical radiolucencies
DDId
What is the buzz phrase for the polarized light view of Dentin Dysplasia Type I (DD-1)?
“stream flowing around boulders” LOL
What are 9 pathoses associated with Regional Odontodysplasia?
- Ectodermal dysplasia
- Epidermal nevi
- Hydrocephalus
- Hypophosphatasia
- Ipsilateral facial hypoplasia
- Neurofibromatosis
- Orbital coloboma
- Rh factor incompatability
- Vascular nevi
What is the alternate term for Regional Odontodysplaysia?
Ghost teeth (If a Ghost showed up on an x-ray, it would look RO)
Which disorder is most likely associated with limited vascular supply to the jaw and thus having an affect on the teeth?
Regional odontodysplasia
Regional odontodysplasia: gender? age?
slightly female.. BIMODAL age distribution at normal eruption patterns for primary and permanent teeth: 2-4 years and 7-11 years
Where does regional odontodysplasia most often occur?
maxillary anterior teeth
What is the histologic buzz term for follicular tissue found in association with Regional Odontodysplasia (ghost teeth)?
Enameloid conglomerates (NOT specific for RO, has been seen in AI
