Deck 15: Metabolic and Genetic Diseases

Question 1

The rare genetic disorder that results in generalized bony sclerosis is known as:

A)Cherubism
B)Plasma cell myeloma
C)von Recklinghausen's disease
D)Fibrous dysplasia
E)Albers-Schonberg disease

Answer 1

Albers-Schonberg disease

Question 2

Paget's disease:

A)Typically occurs in teenagers
B)Is caused by excessive production of growth hormone
C)Results in premature loss of teeth
D)Predisposes the patient to bone fracture and infection
E)All the above

Answer 2

Predisposes the patient to bone fracture and infection

Question 3

Bilateral symmetric enlargement of the mandible or maxilla would most likely be seen in which of the following?

A)Garré's osteomyelitis
B)Paget's disease
C)Osteosarcoma
D)Fibrous dysplasia
E)Ameloblastic fibroma

Answer 3

Paget's disease

Question 4

Uniform and relatively symmetric bilateral hand and jaw enlargement is seen in which of the following?

A)Fibrous dysplasia
B)Acromegaly
C)Garré's osteomyelitis
D)Ossifying fibroma
E)None of the above

Question 5

Determination of serum alkaline phosphatase would be significantly elevated in which of the following?

A)Ameloblastoma
B)Cherubism
C)Paget's disease
D)Ossifying fibroma
E)Central giant cell granuloma

Question 6

Bilateral multilocular radiolucent lesions of the jaws were found in two children and the father of one family. Microscopically, the lesions were composed of fibroblasts and numerous multinucleated giant cells. These patients have:

A)Paget's disease
B)Ectodermal dysplasia
C)Osteopetrosis
D)Cherubism
E)Fibrous dysplasia

Question 7

A vascular fibroblastic matrix in which numerous multinucleated giant cells are seen microscopically is present in which of the following?

A)Hypophosphatasia
B)Hypothyroidism
C)Hyperthyroidism
D)Hyperparathyroidism
E)All the above

Question 8

Hyperparathyroidism causes which of the following changes in serum chemistry?

A)Decreased calcium
B)Increased calcium
C)Increased phosphorus
D)Decreased alkaline phosphatase
E)None of the above

Question 9

Which of the following conditions places the patient at risk for the development of a malignancy?

A)Desmoplastic fibroma
B)Odontogenic myxoma
C)Paget's disease
D)Cementoblastoma
E)All the above

Question 10

Hypoplastic clavicles, delayed eruption, supernumerary teeth, and frontal bone bossing are features of:

A)Hemifacial hypertrophy
B)Cleidocranial dysplasia (dysostosis)
C)Gardner's syndrome
D)Down syndrome
E)Multiple endocrine neoplasia syndrome (MEN III)

Question 11

A 7-year-old girl presents with bilateral swelling of the mandible and maxilla. A radiograph shows bilateral multilocular radiolucent lesions of all four quadrants. Biopsy shows fibrous connective tissue with multinucleated giant cells. Serum chemistry is within normal limits. This patient has:

A)Hyperparathyroidism
B)Cherubism
C)Osteopetrosis
D)McCune-Albright syndrome
E)Paget's disease

Question 12

A facial developmental cleft occurring to the right of the philtrum of soft tissue of the lip that extends through the alveolar bone to the nasopalatine canal could be classified as _____ cleft of the _____.

A)Incomplete; palate
B)Incomplete; lip
C)Complete; palate
D)Complete; lip
E)Combined; lip and palate

Question 13

Hyperthyroidism may cause which of the following?

A)Radiographic evidence of loss of lamina dura
B)Increased serum calcium
C)Multiple radiolucencies
D)Giant cell lesions of the jaws
E)None of the above

Question 14

Excessive production of growth hormone in an adult is responsible for which of the following conditions?

A)Paget's disease
B)Acromegaly
C)Hypophosphatasia
D)Hemifacial hypertrophy
E)Gigantism

Question 15

Infection and jaw fracture would most likely complicate which of the following?

A)Adenomatoid odontogenic tumor
B)Osteopetrosis
C)Lateral root cyst
D)Compound odontoma
E)Fibrous dysplasia

Question 16

Impacted and supernumerary teeth are characteristically seen in which of the following?

A)McCune-Albright syndrome
B)Cleidocranial dysplasia
C)Gardner's syndrome
D)Ectodermal dysplasia
E)Regional odontodysplasia

Question 17

Premature exfoliation of teeth would most likely be associated with which of the following?

A)Odontoma
B)Hypophosphatasia
C)Odontogenic keratocyst
D)Eruption cyst
E)Cleidocranial dysplasia