Ch 6 pathology book questions

The karyotype of a patient with Turner syndrome shows:

44 autosomes and XO

Hypothetically, an autosomal-dominant trait would be clinically present in:

50% of the offspring of an affected parent

Karyotype refers to:

A microphotograph showing a person's chromosomes from a single cell

Trisomy refers to:

A pair of chromosomes with an identical extra chromosome

Patients with hypophosphatasia characteristically have:

Absence of root cementum

Patients with an X-linked hereditary condition:

Are generally affected more severely if they are men

Taurodontic teeth:

Are pyramidal in shape

The Papillon-Lefèvre syndrome is inherited according to a (an):

Autosomal-recessive pattern

A 14-year-old boy is seen in consultation because of bilateral mandibular swelling. Radiographs show a bilateral multilocular lesion in the ascending mandibular rami. The mother of this patient has similar findings. The most likely diagnosis is:

Cherubism

In which one of the following conditions can the shoulders be brought forward to the midline due to hypoplastic clavicles?

Cleidocranial dysplasia

In all inherited varieties of gingival fibromatosis, the gingival enlargement is characterized by a marked:

Collagenization of the connective tissue

Which of the following is the most serious component of Gardner syndrome?

Colorectal polyposis

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

Condyle

Hypotrichosis means:

Decreased amount of hair

Which one of the following is associated with cyclic neutropenia? a. Exfoliating teeth because of short roots b. Chipping away of enamel c. Diminished number of circulating neutrophils d. Premature loss of primary teeth

Diminished number of circulating neutrophils

The major concern for a dental hygienist when treating a patient with Osler-Rendu-Parkes Weber syndrome should be:

Gingival hemorrhage

Patients with hypohidrotic ectodermal dysplasia characteristically have:

Hypodontia

The so-called "enamel agenesis" is one of which type of amelogenesis imperfecta?

Hypoplastic

Two characteristic clinical components of mandibulofacial dysostosis are:

Hypoplastic mandible and deafness

Torus mandibularis and torus palatinus are:

Inherited as an autosomal-dominant trait

Which one of the following is characteristically associated with oral ulcerations?

Kostmann syndrome

The most frequently exfoliated teeth in patients with hypophosphatasia are the:

Maxillary primary incisors

Which of the following is a component of the Peutz-Jeghers syndrome?

Multiple pigmented macules on the lower lip and mucosa

Odontogenic keratocysts are a clinical component of:

Nevoid basal cell carcinoma syndrome

Barr bodies are seen at the:

Nuclear periphery of cells in women

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

Oculat hypertelorism

A 9-year-old boy exhibits markedly swollen red and bleeding gingiva. In addition, he has tooth mobility, and the intraoral radiographs show marked alveolar bone atrophy with vertical periodontal pockets. Which of the following will be found in this child if he were to have Papillon-Lefèvre syndrome?

Palmar and plantar hyperkeatosis

Which of the following is true for von Recklinghausen disease? a. Patients may have gingival neurofibromas. b. It is inherited as an autosomal-recessive trait. c. Patients experience a generalized whitening of the oral mucosa. d. Patients have multiple fibromatoses.

Patients may have gingival neurofibromas.

Which one of the following is typically found in the MEN 2B syndrome

Pheochromocytoma

Radiographs of a patient with radicular dentin dysplasia show:

Pulp chambers with a half-moon appearance

In dentinogenesis imperfecta type II, teeth have:

Roots that are short and thin

Which one of the following statements is true when comparing cyclic neutropenia and Kostmann syndrome? a. The oral lesions in cyclic neutropenia are more severe. b. Both conditions are inherited as autosomal dominant. c. Both conditions are associated with capillary fragility. d. The oral lesions in Kostmann syndrome are always present unless systemic treatment is instituted.

The oral lesions in Kostmann syndrome are always present unless systemic treatment is instituted.

The characteristic finding in permanent teeth affected with coronal dentin dysplasia is:

Thistle-shaped pulp chambers in incisors

Snow-capped amelogenesis imperfecta have teeth with:

White hypocalcified enamel at the incisal and occlusal thirds

The Lyon hypothesis is demonstrated by:

X-linked dominant traits

The constriction that joins the short and long arms of each chromosome is called the:

centromere

The most frequent site of hemorrhage in patients with hereditary hemorrhagic telangiectasia is the:

gingiva

Which one of the following is characteristically associated with teeth with large pulp chambers?

hypophosphatasia

A gamete is the result of the process of:

meiosis

The cause of all forms of labial and palatal clefting is considered to be:

multifactorial

The order of the four stages of mitosis is:

prophase, metaphase, anaphase, telophase

A 19-year-old woman is diagnosed with cleidocranial dysplasia. She has absent clavicles and a mushroom-shaped skull. Which of the following conditions is she also most likely to have?

supernumerary teeth

Cannon disease is also known as:

white sponge nevus