Dentinogenesis and Inherited Dentin Defects Flashcards

1
Q

What is Dentin by volume

A

50% mineral

30% organic matrix

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2
Q

what makes up the Organic matrix of Dentin by weight

A

90% Collagen
90% of non-collagen is Derived from DSPP (Dentin Sialophosphoportein)
-also: dentin matrix protein, osteonectin, bone sialoprotein, osteopontin, proteoglycans…

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3
Q

what do Odontoblasts differentiate from

A

Dental papilla cells

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4
Q

A long cytoplasmic extension encased in dentin

A

Odontoblastic process

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5
Q

Rate of Dentin deposition

A

4 micrometers per days

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6
Q

How is Dentin deposited as

A

Incrementally as lines of Owen (or Von ebner)

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7
Q

what does the pulp chamber and root canal shrink in size over life

A

Dentin formation occures throughout life

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8
Q

layers of the pulp from outisde to insde

A

Odontoblastic layer
CEll-free zone
Cell-rich zone
Pulper core

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9
Q

what is found in the cell-free zone

A

Nerve and capillary plexus

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10
Q

what is found in the cell rich zone

A

Extensive vascular system

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11
Q

how do Lines of Von ebner grow

A

Grow 1 day at a time horizontal and at a right angle to dentinal tubules

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12
Q

what do Lines of von ebner form

A

Accentuated lines due to disturbances in mineralization

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13
Q

why is Dentin sensitive

A

By odontoblast processes

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14
Q

where does more terminal branching of dentin tubules occure

A

In root dentin (more than in coronal dnetin)

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15
Q

Shape of Dentin tubules in the crown and the root

A

Crown: S shaped
Root: Strait

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16
Q

size of dentinal tubules

A

1-3 micrometers in diameter

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17
Q

does the crown or root have more dentin tubules

A

More in the crown

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18
Q

are there more dnetin tubles near the pulp or the surface

A

More near the pulp

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19
Q

what is Predentin

A

The fibrillar organic matrix before its calcification

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20
Q

what is primary dnetin

A

Dentin made during tooth formation

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21
Q

what type of dentin makes up the tooth

A

primary dentin

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22
Q

other name for Primary Dnetin

A

Circumpulpal dentin

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23
Q

the outer layer of primary dnetin

A

Mantle dentin

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24
Q

when is secondary dentin formed

A

By the slow continual deposition of dentin by odontoblasts following the completetion of the roots

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25
Q

what happens the change of primary and secondary dentin

A

tubular structures is continuous but changes direction

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26
Q

why does pulp recession occure

A

Deposotion of secondary dentin is fastest on the roof and floor of the pulp chamber

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27
Q

why does Tertiary dentin form

A

Local reaction to attrition, caries, or dental restoration

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28
Q

what determines the architecture (quality) of tertiary dentin

A

Depends on the intensity and duration of the stimulus

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29
Q

Tubules present in the dentin

A

Continuous
Sparse
Irregular
Absent

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30
Q

Types of Tertiary dentin

A

Reactionary Dentin

Reparative Dentin

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31
Q

what formes Reactionary dentin

A

Preexisting odontoblasts

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32
Q

what forms Reparative dentin

A

Newly differentiated odontoblast-like cells

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33
Q

When Newly differentiated odontoblast-like cells are included in the dentin

A

Osteodentin

34
Q

what is Sclerotic Dentin

A

Translucent dentin due to calcifaction of the dentinal tubules as a result of injury or aging (also called transparent dentin)

35
Q

why does Globular mineralization of dentin occur

A

High rates of Dentin Deposition leading to an irregular mineralization front

36
Q

why does Linear Mineralization of dentin occur

A

Slow rates of deposition leadding to more uniform

37
Q

when globular zones of mineral fail to fuse into a homogeneous mass due to hypomineralized dentin

A

Interglobular dentin

38
Q

what is found in interglobular dentin

A

Dentin tubules pass through but no peritubular dentin is present

39
Q

dentin that surrounds the dentinal tubules

A

Peritubular dentin

40
Q

Organic and innorganic parts of PEritubular dentin

A

Low in Collagen
Higher in DSPP (Dentin sialophosphoprotein)
higher mineralization than intertubular dentin

41
Q

HArdness of Intertubular dentin at the DEJ vs Pulp

A

DEJ is 4x as hard

42
Q

Peritubular dentin hardness at the DEJ and Pulp

A

equal

43
Q

Peritubular dentin vs Intertubular dentin at the pulp

A

PTD is 20x harder

44
Q

Peritubular dentin vs Intertubular dentin at the DEJ

A

PTD is 5x harder

45
Q

does Dentin or enamel mineralize first

A

Dentin is first

46
Q

what is DGI type I

A

Dental phenotype in persons with Osteogenesis imperfected

47
Q

what are thy symptomes of DGI type I

A

Teeth show marked discoloration and attrition in both deciduous and permanent dentition

48
Q

what happens to the Pulp of DGI type I

A

Obliterated before or soon after tooth eruption

49
Q

what mutations cuase osteogenesis imperfected

A

Mutation in the COL1A1 and COL1A2 genes

50
Q

how is collagen formed

A

Two type I collagen alpha 1 chains fold with one alpha 2 chain to form a triple helix

51
Q

Mutation of COL1A1 and COL1A2 lead to

A
Reduced Collagen (less severe phenotype)
Prduce defective collagen (severe phenotype)
52
Q

How is Osteogenesis imperfecta passed on

A

Autosomal-dominant

53
Q

how does a patient radiograph look when they have DGI

A

Bulbous crowns
Pulpal obliteration
short roots
Tooth impactions (both upper and second molars)

54
Q

what mutation causes Dentinogeneisis imperfecta

A

utation in the dentin sialophosphorportein gene (DSPP)

55
Q

what cells express DSPP

A

Preameloblasts, preodontoblasts, ODonotblasts

56
Q

Strucutural domains of DSPP

A

DSP-proteoglycan

DPP- acic protein

57
Q

Isoelectic point of DPP

A

1.0 (lots of phosphoserines)

58
Q

mutations of DSPP cause

A

DD-II, DGI-II, DGI-III

59
Q

what cleaves DSPP into DSP and DPP

A

Extracellular proteases

60
Q

when missing DSPP what is shown in a radiograph

A

Enlarged pulp cavity
Decreased mineral density
Globular mineralized zones
less mineralized dentin and more predentin

61
Q

Types of Dentin Dysplasia

A

DD-I

DD-II

62
Q

Types of Dentinogenesis imperfecta

A

DGI-I
DGI-II
DGI-III

63
Q

where do mutations occure in DSPP to cause dentin defects

A

5’ splicing

Framshift mutation at the 3’ end (exon 5)

64
Q

how id Dentinogeneis imperfecta type II different from OI

A

DGI-II only affects the teeth

65
Q

Commonness of DGI-II

A

1 in 6k to 8k

66
Q

what causes DGI-II

A

Mutations in DSPP

67
Q

How do people with DGI-II look

A

Blue gray, amber brown and opalescent teeth

68
Q

how do People with DGI-II look in radiographs

A

Teeth have bulbous crowns, Narrow roots

Pulp chambers and canals are gone or small

69
Q

what happens to teeth of DGI-II from occlusal stress

A

Enamel may split from dentin

70
Q

when was DGI-III found

A

Brandywine isolate from southern maryland and washing D.C.

71
Q

how can DGI-III be differentiated from DGI-I and DGI-II

A

all appeaer similar except for DGI-III can have multiple pulp exposures in deciduous teeth

72
Q

Radiographs of DGI-III show

A

Variation in appears (pulpal obliteration, normal, or shell teeth

73
Q

commonness of Dentin Dysplasia Type I (DD-I)

A

1 in 100,000

74
Q

How do the teeth look in DD-I

A

Both permanent and deciduous teeth are normal shape, form and color

75
Q

How does DD-I look in a radiograph

A

Short roots with unusual mobility and early exfoliation
Cresecent shaped pulpal remnant parallel to the CEJ in the permanent dentition and total pulpal obliteration in the deciduous dentition

76
Q

How does the Teeth look in DD-II

A

Decidous teeth have deatures of DGI-II
Permanent teeth are normal shape, form, and color
Normal root length

77
Q

What is found in the pulp cavities of DD-II

A

Thistle tube deformity

PUlp stones

78
Q

What causes DGI-II, DGI-III, and DD-II

A

All about the same disease caused by dominant-negative effects (not haploinsufficiency)

79
Q

what affects the severity of DSPP mutations

A

the effect of the DSPP mutation may lead to the different phenotypes (ie if its it DD-II, DGI-II, or DGI-III)

80
Q

what does DD-II
DGI-II
DGI-III cause

A

DD-II: partial obliteration, mild discoloration
DGI-II: Pulpal obliteration, Discoloration in both dentitions
DGI-III: pulpal exposure, early abscess

81
Q

How will dentin dissorders be organized in the future

A

No DGI-I, become named based on the disease phenotype

DD-II, DGI-II, and DGI-III all called DSPP mutations with considerations to severity