Deck 15: Miscellaneous Bone Diseases

Question 1

The most prevalent heavy metal poison that principally affects the brain is __________.

A) copper
B) bismuth
C) lead
D) phosphorus

Answer 1

lead

Question 2

Which of the following histiocytosis clinical syndromes accounts for 10% of all histiocytosis cases, occurs in children younger than age 2, and has multiple osteolytic skull lesions?

A) Hand-Schüller-Christian disease
B) Eosinophilic granuloma
C) Pierre-Marie-Bamberger syndrome
D) Letterer-Siwe disease

Answer 2

Letterer-Siwe disease

Question 3

Which of the following is a lipid storage disorder resulting from a genetic deficiency of the enzyme glucocerebrosidase?

A) Dermatomyositis
B) Histiocytosis
C) Gaucher's disease
D) Amyloidosis

Answer 3

Gaucher's disease

Question 4

Which of the following histiocytosis clinical syndromes accounts for 60% to 80% of all histiocytosis cases, occurs in children between the ages of 5 and 15 years, and usually has solitary geographic osteolytic lesions?

A) Letterer-Siwe disease
B) Hand-Schüller-Christian disease
C) Eosinophilic granuloma
D) Pierre-Marie-Bamberger syndrome

Question 5

Radiographic findings of "floppy thumb" sign, effusion, and osteoporosis of small joints of the hand accompany which disease?

A) Paget's disease
B) Mastocytosis
C) Histiocytosis
D) Dermatomyositis

Question 6

Which of the following statements best characterizes primary hypertrophic osteoarthropathy?

A) It may show ligamentous calcification on radiographs.
B) It is more common than the secondary form.
C) It affects women and whites most often.
D) It occurs secondary to an underlying disease.

Question 7

Associated bone changes in a patient with amyloidosis may likely include all of the following except __________.

A) osteonecrosis
B) periarticular joint swelling
C) Erlenmeyer flask deformity
D) pathological vertebral fractures

Question 8

Which of the following histiocytosis clinical syndromes accounts for 15% to 40% of these cases, occurs in children between 1 and 5 years of age, and typically displays multiple geographic osteolytic lesions in the skull, pelvis, and long bones?

A) Letterer-Siwe disease
B) Hand-Schüller-Christian disease
C) Eosinophilic granuloma
D) Pierre-Marie-Bamberger syndrome

Question 9

Lead, phosphorus, copper, and bismuth poisoning are associated with __________.

A) Erlenmeyer flask deformity
B) ivory vertebrae
C) transverse, radiodense metaphyseal bands
D) subcutaneous linear or curvilinear calcifications

Question 10

Which bone is most commonly involved in the familial form of infantile cortical hyperostosis?

A) Radius
B) Tibia
C) Femur
D) Ulna

Question 11

Definitively diagnosing amyloidosis requires __________.

A) a biopsy
B) MRI
C) a bone scan
D) plain films

Question 12

Which of the following conditions would be treated with steroids, immunosuppressive drugs, passive range of motion, moderate exercise, and muscle stretching?

A) Paget's disease
B) Mastocytosis
C) Histiocytosis
D) Dermatomyositis

Question 13

Copper, zinc, and aluminum toxicity are associated with __________.

A) transverse, radiodense metaphyseal bands
B) altered bone mineralization and osteopenia
C) ivory vertebrae
D) subcutaneous linear or curvilinear calcifications

Question 14

An uncommon familial or sporadic syndrome marked by subperiosteal bone formation describes __________.

A) infantile cortical hyperostosis
B) neurofibromatosis
C) Paget's disease
D) dermatomyositis

Question 15

Which of the following conditions has such a variable appearance that it should be considered in every destructive bone lesion that appears in patients younger than the age of 30?

A) Paget's disease
B) Histiocytosis
C) Neurofibromatosis
D) Hypertrophic osteoarthropathy

Question 16

Which of the following would be the most likely cause of a patient presenting with a reddish rash over the eyelids, cheeks, and nose?

A) Dermatomyositis
B) Gaucher's disease
C) Heavy metal poisoning
D) Neurofibromatosis

Question 17

Which of the following is another name for hypertrophic osteoarthropathy?

A) Pierre-Marie-Bamberger syndrome
B) Eosinophilic granuloma
C) Letterer-Siwe disease
D) Pachydermoperiostosis

Question 18

Which statement is true of dermatomyositis?

A) It most commonly affects male children.
B) It is a wasting of smooth and cardiac muscle.
C) It commonly displays the Erlenmeyer flask deformity on plain film radiographs.
D) It is associated with an elevated incidence of visceral carcinomas in adults.

Question 19

Which of the following is the most common hereditary metabolic storage disorder?

A) Amyloidosis
B) Gaucher's disease
C) Dermatomyositis
D) Infantile cortical hyperostosis

Question 20

Chronic inflammation of the skeletal muscle and skin is seen in __________.

A) amyloidosis
B) neurofibromatosis
C) dermatomyositis
D) Gaucher's disease

Question 21

Neurofibromatosis I is also known as __________.

A) central neurofibromatosis
B) Lindau's disease
C) Von Recklinghausen's disease
D) Sturge-Weber syndrome

Question 22

Which of the following is not a typical laboratory finding in a patient with Paget's disease?

A) Elevated serum alkaline phosphatase
B) Elevated urinary hydroxyproline
C) Elevated serum acid phosphatase
D) Normal serum calcium

Question 23

What is the term for a convex posterior curvature?

A) Kyphosis
B) Lordosis
C) Scoliosis
D) Gomphosis

Question 24

The imaging modality that would be helpful in determining the full extent of skeletal involvement in a patient with systemic mastocytosis is __________.

A) MRI
B) bone scan
C) plain films
D) CT

Question 25

Which of the following is characteristic of a functional scoliosis?

A) Rib humping virtually disappears on forward flexion.
B) Hemivertebrae are present.
C) The curve is at least 50 degrees.
D) Back pain is nearly always involved.

Question 26

The disease that involves general or local osteopenia, local osteosclerosis, or a mixed pattern of presentation is __________.

A) amyloidosis
B) dermatomyositis
C) mastocytosis
D) neurofibromatosis

Question 27

Absence of the lesser wing of the sphenoid is observed on a nasium radiograph. This is a finding characteristic of __________.

A) neurofibromatosis I
B) tuberous sclerosis
C) Sturge-Weber syndrome
D) Lindau disease

Question 28

If an idiopathic scoliosis was detected in an 11-year-old, it would be classified as ______________ scoliosis.

A) adolescent
B) infantile
C) juvenile
D) adult

Question 29

When does infantile cortical hyperostosis occur?

A) Before 6 months of age
B) From 1 to 5 years of age
C) From 6 months to 1 year of age
D) After age 10

Question 30

What is the most common condition of a heterogeneous group of diseases known as phakomatoses?

A) Neurofibromatosis
B) Paget's disease
C) Dermatomyositis
D) Infantile cortical hyperostosis

Question 31

Which of the following is not a spinal finding in Paget's disease?

A) Cotton wool
B) Picture frame
C) Ivory appearance of bone
D) Compression fracture

Question 32

The condition that is divided into lytic, mixed, and blastic phases where normal bone is replaced with immature, weak, and hypervascular bone is known as __________.

A) amyloidosis
B) Gaucher's disease
C) dermatomyositis
D) Paget's disease

Question 33

Radiologic findings of erosions of vertebrae and ribs, kyphoscoliosis, dysplasia of the sphenoid, and lambdoid suture defects would lead a clinician to suspect __________.

A) Paget's disease
B) neurofibromatosis type I
C) mastocytosis
D) hypertrophic osteoarthropathy

Question 34

What are the most common clinical complaints in patients with Paget's disease?

A) Soft tissue swelling over the involved bone
B) Local pain and tenderness over involved bone
C) Axillary or inguinal flecking
D) Skin of the face and scalp appears thickened

Question 35

Which of the following is a rare systemic disease characterized by extracellular accumulation of insoluble proteins in various organs and tissues of the body?

A) Multiple myeloma
B) Amyloidosis
C) Hand-Schüller-Christian disease
D) Gaucher's disease

Question 36

Which of the following diagnoses best accounts for abnormal remodeling and hypertrophy of bone leading to pain and deformity in middle-age and older individuals?

A) Neurofibromatosis type I
B) Neurofibromatosis type II
C) Paget's disease
D) Amyloidosis

Question 37

What is another name for Paget's disease?

A) Caffey's disease
B) Von Recklinghausen's disease
C) Letterer-Siwe disease
D) Osteitis deformans

Question 38

Nonelevated, brownish cutaneous hyperpigmentations seen in neurofibromatosis type I are known as __________.

A) Lisch nodules
B) Schwannomas
C) hamartomas
D) café-au-lait spots

Question 39

Which of the following methods is not useful in staging skeletal maturation?

A) Risser sign
B) Chronological age
C) Cobb method
D) Hand and wrist radiographs

Question 40

Neurofibromatosis type II is diagnosed by the presence of __________.

A) bilateral acoustic schwannomas
B) hamartomas
C) fibroma molluscum
D) axillary or inguinal flecking