Chapter 6 d.
44 autosomes and XXY
Review Questions 9. The major concern for a dental hygienist when
1. Which one of the following is associated with cyclic treating a patient with Osler-Rendu–Parkes Weber
neutropenia? syndrome should be:
a. Exfoliating teeth because of short roots a. Severe infections
b. Chipping away of enamel b. Epithelial desquamation
c. Diminished number of circulating neutrophils c. Spontaneous ulcerations
d. Premature loss of primary teeth d. Gingival hemorrhage
2. The so-called “enamel agenesis” is one of which 10. The characteristic finding in permanent teeth
type of amelogenesis imperfecta? affected with coronal dentin dysplasia is:
a. Hypocalcified a. Large, square pulp chambers in molars
b. Hypomaturation b. Thistle-shaped pulp chambers in incisors
c. Hypoplastic-hypomaturation c. Crowns with amber color
d. Hypoplastic d. Markedly short roots
3. Two characteristic clinical components of 11. Radiographs of a patient with radicular dentin
mandibulofacial dysostosis are: dysplasia show:
a. Lack of clavicles and delayed teeth eruption a. Taurodontic teeth
b. Hypodontia and dysplastic nails b. Large pulp chambers with long pulp horns
c. Hypoplastic mandible and deafness c. Pulp chambers with a half-moon appearance
d. Cleft lip and fistulas of lower lip d. Internal resorption of teeth
4. Which of the following is true for von 12. Hypotrichosis means:
Recklinghausen disease? a. Increased number of sweat glands
a. Patients may have gingival neurofibromas. b. Diminished number of sweat glands
b. It is inherited as an autosomal-recessive trait. c. Increased amount of hair
c. Patients experience a generalized whitening of the d. Decreased amount of hair
oral mucosa. 13. Snow-capped amelogenesis imperfecta have teeth
d. Patients have multiple fibromatoses. with:
5. Which one of the following is characteristically a. Short, blunted roots
associated with teeth with large pulp chambers? b. White hypocalcified enamel at the incisal and
a. Coronal dentin dysplasia occlusal thirds
b. Dentinogenesis imperfecta c. A thin, brown enamel
c. Hypophosphatasia d. Obliterated pulp chambers
d. Pitted autosomal-dominant amelogenesis imperfecta 14. The most frequently exfoliated teeth in patients
6. Which one of the following statements is true when with hypophosphatasia are the:
comparing cyclic neutropenia and Kostmann a. Mandibular permanent incisors
syndrome? b. Mandibular primary incisors
a. The oral lesions in cyclic neutropenia are more c. Maxillary primary molars
severe. d. Maxillary primary incisors
b. Both conditions are inherited as autosomal 15. A gamete is the result of the process of:
dominant. a. The S phase
c. Both conditions are associated with capillary b. Meiosis
fragility. c. Mitosis
d. The oral lesions in Kostmann syndrome are always d. Prophase
present 16. Which of the following is the most serious
unless systemic treatment is instituted. component of Gardner syndrome?
7. A 9-year-old boy exhibits markedly swollen red and a. Teeth hypercementosis
bleeding gingiva. In addition, he has tooth mobility, b. Mandibular odontomas
and the intraoral radiographs show marked alveolar c. Colorectal polyposis
bone atrophy with vertical periodontal pockets. Which d. Multiple osteomas
of the following will be found in this child if he were 17. Patients with hypophosphatasia characteristically
to have Papillon-Lefèvre syndrome? have:
a. Lack of anterior vestibular sulcus a. Increase in serum alkaline phosphatase levels
b. Diminished sweating b. Obliterated pulp chambers
c. Palmar and plantar hyperkeratosis c. Marked gingival keratinization
d. Blue sclerae d. Absence of root cementum
8. The karyotype of a patient with Turner syndrome 18. A 14-year-old boy is seen in consultation because
shows: of bilateral mandibular swelling. Radiographs show a
a. 43 autosomes and XYY bilateral multilocular lesion in the ascending
b. 44 autosomes and XO mandibular rami. The mother of this patient has similar
c. 44 autosomes and XYY findings. The most likely diagnosis is:
a. Cleidocranial dysplasia d. Carcinoma of the colon
b. Nevoid basal cell carcinoma syndrome 29. A 19-year-old woman is diagnosed with
c. Ellis–van Creveld syndrome cleidocranial dysplasia. She has absent clavicles and a
d. Cherubism mushroom-shaped skull. Which of
19. The order of the four stages of mitosis is: the following conditions is she also most likely to
a. Prophase, metaphase, anaphase, telophase have?
b. Metaphase, prophase, telophase, anaphase a. Large pulp chambers
c. Anaphase, metaphase, telophase, prophase b. Taurodontism
d. Prophase, telophase, metaphase, anaphase c. Supernumerary teeth
20. The constriction that joins the short and long arms d. Pegged lateral incisors
of each chromosome is called the: 30. Trisomy refers to:
a. Chromatid a. One extra chromosome in each pair
b. Equatorial plate b. Three extra chromosomes
c. Centromere c. The presence of two extra X chromosomes in a male
d. Chiasmata d. A pair of chromosomes with an identical extra
21. In dentinogenesis imperfecta type II, teeth have: chromosome
a. Roots that are short and thin 31. Patients with hypohidrotic ectodermal dysplasia
b. Dilacerated roots characteristically have:
c. Hard, dense dentin a. Blue sclerae
d. Markedly brittle enamel b. Excessive amounts of hair
22. Torus mandibularis and torus palatinus are: c. Hypodontia
a. Sporadic traits d. Multiple tongue nodules
b. Inherited as an autosomal-dominant trait 32. Which of the following is a component of the
c. Inherited as an autosomal-recessive trait Peutz-Jeghers syndrome?
d. More prevalent in males a. Multiple jaw cysts
23. The Papillon-Lefèvre syndrome is inherited b. Multiple pigmented macules on the lower lip and
according to a (an): mucosa
a. X-linked recessive pattern c. Multiple nodules on the tip of the tongue
b. Autosomal-dominant pattern d. Multiple supernumerary teeth
c. Autosomal-recessive pattern 33. In all inherited varieties of gingival fibromatosis,
d. X-linked dominant pattern the gingival enlargement is characterized by a marked:
24. Hypothetically, an autosomal-dominant trait would a. Alveolar bone hypertrophy
be clinically b. Collagenization of the connective tissue
present in: c. Hyperplasia of the covering epithelium
a. 25% of the offspring of an affected parent d. Chronic inflammatory cellular infiltrate
b. 50% of the offspring of an affected parent 34. Karyotype refers to:
c. 75% of the offspring of an affected parent a. A portion of a chromosome attached to an another
d. Only in males, never in female offspring chromosome
25. Which one of the following is characteristically b. A pair of chromosomes with an identical extra
associated with oral ulcerations? chromosome
a. Gardner syndrome c. A microphotograph showing a person's
b. Gorlin syndrome chromosomes from a
c. Kostmann syndrome single cell
d. Peutz-Jeghers syndrome d. The position occupied by a gene in a chromosome
26. The most frequent site of hemorrhage in patients 35. Taurodontic teeth:
with hereditary hemorrhagic telangiectasia is the: a. Have long roots
a. Lip mucosa b. Have thistle-shaped pulp chambers
b. Gingiva c. Are pyramidal in shape
c. Nasal mucosa d. Are supernumerary
d. Eyelids 36. Cannon disease is also known as:
27. Barr bodies are seen at the: a. White sponge nevus
a. Nuclear periphery of all cells in women b. Gingival fibromatosis
b. Periphery of the cytoplasm in all human cells c. Chronic neutropenia
c. Nuclear periphery of all human cells d. Cherubism
d. Periphery of the cytoplasm in all cells from women 37. The cause of all forms of labial and palatal clefting
28. Which one of the following is typically found in is considered to be:
the MEN 2B syndrome? a. Multifactorial
a. Carcinoma of the pancreas b. Environmental
b. Pheochromocytoma c. Autosomal recessive
c. Basal cell carcinomas d. Autosomal dominant
38. Odontogenic keratocysts are a clinical component
of:
a. Cherubism
b. Pegged lateral incisors
c. Nevoid basal cell carcinoma syndrome
d. Neurofibromatosis of von Recklinghausen
39. Patients with an X-linked hereditary condition:
a. Are generally affected more severely if they are men
b. Are always XYY
c. Have cells with an extra Barr body
d. Are always women
40. The Lyon hypothesis is demonstrated by:
a. X-linked dominant traits
b. X-linked recessive traits
c. Autosomal-dominant traits
d. Autosomal-recessive traits
41. All of the following are characteristics of
cherubism except one. Which one is the exception?
a. Pseudoanodontia
b. Autosomal dominant
c. Ocular hypertelorism
d. Autosomal recessive
42. All of the following are involved in cherubism
except one.
Which one is the exception?
a. Coronoid process
b. Condyle
c. Posterior mandible
d. Ascending ramus
43. In which one of the following conditions can the
shoulders be brought forward to the midline due to
hypoplastic clavicles?
a. Cherubism
b. Ellis-–von Creveld syndrome
c. Turner syndrome
d. Cleidocranial dysplasia