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Neville and Damm > Ch 2 - Abnormalities of Teeth 4 > Flashcards

Ch 2 - Abnormalities of Teeth 4 Flashcards

1
Q

Which syndrome is most frequently associated with dental manifestations that mimic dentinogenesis imperfecta,

A

Osteogenesis imperfecta

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2
Q

Besides Osetogenesis Imperfecta, which 3 syndromes mimic dentinogenesis imperfecta?

A
  1. Ehlers-Danlos syndrome
  2. Goldblatt syndrome
  3. Schimke immuno-osseous dysplasia
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3
Q

Besides the 4 syndromes that mimic dentinogenesis imperfecta, what are 4 systemic conditions that can mimic it?

A
  1. vitamin-D resistant rickets
  2. vitamin-D dependent rickets
  3. tumoral calcinosis
  4. calcinosis universalis
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4
Q

What is the primary gene in guiding the proteins that create dentin?

A

DSPP gene (dentin sialophosphoprotein)

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5
Q

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

A

Dentinogenesis Imperfecta

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6
Q

What % of patients with Osteogenesis Imperfecta show similar dental changes as dentinogenesis imperfecta? Thus the difference is what gene mutation?

A

50% of these OI patients have DI like dental changes, so these patients have the COL1A1/COL1A2 gene mutations for OI

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7
Q

osteogenesis imperfecta with “______” teeth is clearly a separate disease from dentinogenesis imperfecta

A

“opalescent” teeth

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8
Q

What was the phenotype of the old classification dentinogenesis imperfecta III - Brandywine isolate? (which is now just a variation of DI)

A

shell teeth (deciduous teeth)

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9
Q

Which dentin dysplasia type is associated with the DSPP gene mutation (same as dentinogenesis imperfecta)?

A

dentin dysplasia type II

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10
Q

What are two alternate names for dentinogenesis imperfecta?

A
  1. hereditary opalescent dentin

2. Capdepont’s teeth

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11
Q

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?

A

English and French along the English channel…1:8,000 whites in the US

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12
Q

Which teeth are most severely affected in Dentinogenesis Imperfecta? Which one are least?

A

primary teeth the most, 3rd molars least altered

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13
Q

While Dentinogenesis Imperfecta can cause _________ due to excess dentin production, some teeth can have expanded pulp tissue and are called ________

A

pulpal obliteration….shell teeth

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14
Q

Several kindreds affected with dentinogenesis imperfecta also have been shown to demonstrate what other condition?

A

progressive, sensorineural, high-frequency hearing loss.

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15
Q

What are the two radiographic descriptions for dentin dysplasia type II?

A

thistle tube-shaped or flame-shaped pulp chambers

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16
Q

What other condition has similar radiographic findings to dentin dysplasia II? What is the difference?

A

Pulpal dysplasia…PD teeth look normal clinically (both PD and DD-II have thistle pulps and pulp stones)

17
Q

In patients with dentin dysplasia II, what pattern do the deciduous teeth resemble?

A

same pattern as dentinogenesis imperfecta (pulpal obliteration or shell teeth)

18
Q

By definition, dentin dysplasia should have NO correllation with ________

A

systemic disease

19
Q

Dentin dysplasia has no correllation with systemic disease, but these 4 diseases have dentin-dysplasia LIKE alterations…

A
  1. Calcinosis universalis ( the deposit of calcium salts in skin, subcutaneous tissue, tendons and muscles)
  2. Rheumatoid arthritis
  3. Hypervitaminosis D
  4. Sclerotic bone and skeletal anomalies
  5. Tumoral calcinosis
20
Q

What is the alternate name for dentin dysplasia I? What is the buzz term seen in this disorder?

A

radicular dentin dysplasia…ROOTLESS teeth

21
Q

inheritance pattern and prevalence for dentin dysplasia I?

A

AD…1:100,000

22
Q

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?

A

fibrous dysplasia of dentin…AD

23
Q

Subclassify that Dentin Dysplasia I why dont you: No pulp chambers, no root formation, and frequent
periapical radiolucencies

A

DDIa

24
Q

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

A

DDIb

25
Q

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

A

DDIc

26
Q

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

A

DDId

27
Q

What is the buzz phrase for the polarized light view of Dentin Dysplasia Type I (DD-1)?

A

“stream flowing around boulders” LOL

28
Q

What are 9 pathoses associated with Regional Odontodysplasia?

A
  1. Ectodermal dysplasia
  2. Epidermal nevi
  3. Hydrocephalus
  4. Hypophosphatasia
  5. Ipsilateral facial hypoplasia
  6. Neurofibromatosis
  7. Orbital coloboma
  8. Rh factor incompatability
  9. Vascular nevi
29
Q

What is the alternate term for Regional Odontodysplaysia?

A

Ghost teeth (If a Ghost showed up on an x-ray, it would look RO)

30
Q

Which disorder is most likely associated with limited vascular supply to the jaw and thus having an affect on the teeth?

A

Regional odontodysplasia

31
Q

Regional odontodysplasia: gender? age?

A

slightly female.. BIMODAL age distribution at normal eruption patterns for primary and permanent teeth: 2-4 years and 7-11 years

32
Q

Where does regional odontodysplasia most often occur?

A

maxillary anterior teeth

33
Q

What is the histologic buzz term for follicular tissue found in association with Regional Odontodysplasia (ghost teeth)?

A

Enameloid conglomerates (NOT specific for RO, has been seen in AI

Neville and Damm (72 decks)

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