Hereditary Conditions

Question 1

disorder of tooth development leading to small, discolored, misshapen teeth characterized by abnormal enamel formation

Answer 1

amelogenesis imperfecta

Question 2

inheritance pattern of amelogenesis imperfecta

Answer 2

autosomal dominant, recessive, or X-linked

Question 3

tx of amelogenesis imperfecta

Answer 3

full coverage crowns for esthetics

Question 4

fragile bones and teeth, collarbones (clavicles) may be absent

Answer 4

cleidocranial dysplasia

Question 5

dental abnormalities associated with cleidocranial dysplasia

Answer 5

• delayed loss of primary teeth
• delayed appearance of secondary teeth
• misshapen teeth
• supernumerary teeth

Question 6

inheritance pattern of cleidocranial dysplasia

Answer 6

autosomal dominant

Question 7

tx of cleidocranial dysplasia

Answer 7

reconstructive surgery

Question 8

• disorder of tooth development affecting primary and permanent dentition, leading to small, discolored, misshapen teeth
• characterized by abnormal dentin formation, resulting in short roots, bell-shaped and bulbous crowns, and obliterated pulps

Answer 8

dentinogenesis imperfecta

Question 9

Patients with this disorder also have blue sclera (whites of the eyes).

Answer 9

dentinogenesis imperfecta

Question 10

disorder than can be associated with osteogenesis imperfecta

Answer 10

dentinogenesis imperfecta

Question 11

inheritance pattern of dentinogenesis imperfecta

Answer 11

autosomal dominant

Question 12

tx of dentinogenesis imperfecta

Answer 12

full coverage crowns for esthetics

Question 13

• disorder of tooth development leading to small, discolored, misshapen teeth
• characterized by abnormal dentin, resulting in chevron pulps and short roots

Answer 13

dentin dysplasia

Question 14

type I vs type II dentin dysplasia

Answer 14

Type I = poor root development
Type II = poor crown development

Question 15

inheritance pattern of dentin dysplasia

Answer 15

autosomal dominant

Question 16

tx of dentin dysplasia

Answer 16

not good candidates for restoration due to both chevron pulps and short roots

Question 17

Dentinogenesis imperfecta vs dentin dysplasia

Answer 17

Dentinogenesis imperfecta
- short roots
- bell-shaped and bulbous crowns
- obliterated pulps
- treated with crowns

Dentin dysplasia
- chevron pulps
- short roots
- type I and II
- not a good option for restorations

Question 18

abnormal development of ectodermal structures such as teeth, mucosal membranes, nails, and hair

Answer 18

ectodermal dysplasia

Question 19

dental manifestations of ectodermal dysplasia

Answer 19

hypodontia, anodontia, heat intolerance

Question 20

inheritance pattern of ectodermal dysplasia

Answer 20

X-linked recessive

Question 21

tx of ectodermal dysplasia

Answer 21

restorative procedures

Question 22

defines a group of rare diseases that cause blistering skin, mechanical fragility and blistering leads to eventual decay due to difficult oral hygiene maintenance

Answer 22

epidermolysis bullosa

Question 23

inheritance pattern and tx of epidermolysis bullosa

Answer 23

autosomal dominant or recessive
tx is topical aids for blisters

Question 24

abnormal formation of capillaries on skin, mucosa, and viscera

Answer 24

hereditary hemorrhagic telangiectasia

Question 25

syndrome characterized by telangiectasia (spider veins) are dilated or broken blood vessels near skin or mucosa membranes + iron deficiency is a common consequence + epistaxis (nosebleeds) are the most common manifestations of HHT

Answer 25

Osler-Weber-Rendu syndrome

Question 26

inheritance pattern of hereditary hemorrhagic telangiectasia

Answer 26

autosomal dominant

Question 27

tx of hereditary hemorrhagic telangiectasia

Answer 27

Bevacizumab (Avastin)

Question 28

disorder in which bones become overly dense, which causes them to be brittle and fracture easily

Answer 28

osteopetrosis (aka Albergs-Schonberg disease or marble bone disease)

Question 29

inheritance pattern of osteopetrosis

Answer 29

autosomal dominant or recessive

Question 30

tx of osteopetrosis

Answer 30

surgery for severe functional deformities

Question 31

• development anomaly impacting enamel, dentin, and cementum components of teeth
• termed “ghost teeth” (results in quadrants of teeth to exhibit short roots, enlarged pulp chambers, and open apical foramen)

Answer 31

regional odontodysplasia

Question 32

Where does the term “ghost teeth” come from describing teeth affected by regional odontodysplasia?

Answer 32

the thin enamel and dentin layers result in a faint, fuzzy image of the teeth when x-rayed

Question 33

tx of regional odontodysplasia

Answer 33

extraction of affected teeth

Question 34

asymptomatic thick and spongey white patches of tissue that cannot be wiped off

Answer 34

white spongy nevus

Question 35

most common location of white sponge nevus

Answer 35

buccal mucosa

Question 36

inheritance pattern of white sponge nevus

Answer 36

autosomal dominant

Question 37

tx of white sponge nevus

Answer 37

none, no serious complications

Question 38

fusion vs gemination

Answer 38

Fusion- merging of two tooth buds into one tooth, resulting in the tooth count to be one less tooth than normal

Gemination- when one root buds into two crowns, giving us a normal tooth count