Question 1

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

Accepted Answer

Lead is the most prevalent heavy metal poison that affects the brain. It can cause brain damage, developmental delays, and learning disabilities, especially in young children. Copper, bismuth, and phosphorus can also be poisonous but they do not affect the brain as severely as 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?

Accepted Answer

Letterer-Siwe disease is a form of Langerhans cell histiocytosis that typically affects children under the age of 2. It is characterized by a rapid, aggressive course and can present with multiple osteolytic skull lesions among other symptoms, accounting for a small percentage of all histiocytosis cases.

Question 3

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

Accepted Answer

Gaucher's disease is a lipid storage disorder caused by a genetic deficiency of the enzyme glucocerebrosidase, resulting in the buildup of lipids in cells and tissues. Dermatomyositis, histiocytosis, and amyloidosis are not related to this specific enzyme deficiency.

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?

Accepted Answer

Eosinophilic granuloma is the most common histiocytosis clinical syndrome, accounting for 60% to 80% of cases. It typically presents as a solitary, painful, osteolytic lesion in children between the ages of 5 and 15 years old. Letterer-Siwe disease and Hand-Schüller-Christian disease are less common forms of histiocytosis, while Pierre-Marie-Bamberger syndrome is a syndrome characterized by clubbing of the fingers, not related to histiocytosis.

Question 5

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

Accepted Answer

The answer of Radiographic findings of "floppy thumb" sign, effusion,...

Question 6

Which of the following statements best characterizes primary hypertrophic osteoarthropathy?

Accepted Answer

Primary hypertrophic osteoarthropathy is characterized by periostitis that can lead to ligamentous calcification visible on radiographs, distinguishing it from the secondary form, which is associated with underlying diseases.

Question 7

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

Accepted Answer

Erlenmeyer flask deformity is typically associated with conditions affecting bone remodeling and marrow expansion, such as Gaucher's disease or thalassemia, rather than amyloidosis. Amyloidosis can lead to osteonecrosis, periarticular joint swelling, and pathological fractures due to amyloid deposits in bone tissue and around joints, but it does not cause the specific bone shape changes seen in Erlenmeyer flask deformity.

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?

Accepted Answer

Hand-Schüller-Christian disease is a type of Langerhans cell histiocytosis that occurs in children aged 1 to 5 years and is characterized by multiple osteolytic lesions, often in the skull, pelvis, and long bones.

Question 9

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

Accepted Answer

Transverse, radiodense metaphyseal bands are a classic finding in these types of heavy metal poisonings. Erlenmeyer flask deformity is seen in bone dysplasias such as Gaucher disease. Ivory vertebrae is a radiographic feature of Paget's disease of bone. Subcutaneous linear or curvilinear calcifications can be seen in calcinosis, a condition where calcium deposits form in soft tissues.

Question 10

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

Accepted Answer

The tibia is the most commonly involved bone in the familial form of infantile cortical hyperostosis, also known as Caffey disease. The condition is characterized by painful swelling and cortical thickening of affected bones.

Question 11

Definitively diagnosing amyloidosis requires __________.

Accepted Answer

Definitively diagnosing amyloidosis requires a biopsy, in which a small piece of tissue is taken from an affected organ and examined under a microscope for the presence of amyloid deposits. MRI, bone scans, and plain films may be used as part of the diagnostic process, but they cannot definitively diagnose amyloidosis.

Question 12

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

Accepted Answer

Dermatomyositis is a rare inflammatory disease characterized by muscle weakness and skin rash. Treatment includes steroids and immunosuppressive drugs to control inflammation, as well as passive range of motion, moderate exercise, and muscle stretching to maintain muscle strength and mobility. Paget's disease, Mastocytosis, and Histiocytosis are unrelated conditions that would not be treated with this combination of therapies.

Question 13

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

Accepted Answer

Copper, zinc, and aluminum toxicity can affect bone mineralization and lead to osteopenia, a condition where bones become weak and thin.

Question 14

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

Accepted Answer

Subperiosteal bone formation is a defining feature of infantile cortical hyperostosis, also known as Caffey disease. Neurofibromatosis is a genetic disorder characterized by the growth of tumors on nerves, Paget's disease is a bone disorder that results in weakened and deformed bones, and dermatomyositis is a rare inflammatory disease that affects the skin and muscles. None of these conditions are typically associated with subperiosteal bone formation.

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?

Accepted Answer

Histiocytosis has variable appearance and can present as destructive bone lesions in patients younger than 30. It should always be considered in the differential diagnosis of these lesions. The other conditions listed may also cause bone lesions, but they do not have such a variable appearance that they should be considered in every case.

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?

Accepted Answer

A reddish rash over the eyelids, cheeks, and nose is a classic symptom of dermatomyositis. This autoimmune disease affects the muscles and skin, and is characterized by inflammation and damage to these tissues. The rash is called the "heliotrope rash" and is often accompanied by muscle weakness, pain, and fatigue. Gaucher's disease is a genetic disorder that affects the metabolism of certain fats, and typically involves enlargement of the spleen and liver, anemia, and bone abnormalities. Heavy metal poisoning can cause a variety of symptoms depending on which metal is involved, but a rash over the face is not a common symptom. Neurofibromatosis is a genetic disorder that causes tumors to grow on nerves, and can cause a range of symptoms depending on the type and severity of the condition. A rash over the face is not typically associated with neurofibromatosis.

Question 17

Which of the following is another name for hypertrophic osteoarthropathy?

Accepted Answer

The answer of Which of the following is another name...

Question 18

Which statement is true of dermatomyositis?

Accepted Answer

Dermatomyositis is a connective tissue disease that presents with muscle weakness and skin changes. It is more common in women and typically affects adults. It is not a wasting of smooth and cardiac muscle, but rather affects skeletal muscle. While bone changes may be present in dermatomyositis, the Erlenmeyer flask deformity is associated with other conditions such as Gaucher disease. However, there is an increased risk of various types of malignancy in adults with dermatomyositis, including visceral carcinomas.

Question 19

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

Accepted Answer

Gaucher's disease is the most common hereditary metabolic storage disorder. Amyloidosis is a group of diseases in which abnormal proteins accumulate in tissues and organs. Dermatomyositis is an autoimmune disorder that affects the muscles and skin. Infantile cortical hyperostosis is a rare genetic condition that causes excessive bone growth.

Question 20

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

Accepted Answer

Chronic inflammation of the skeletal muscle and skin is a characteristic feature of dermatomyositis. Amyloidosis is a disorder characterized by the deposition of insoluble protein fibrils in the tissues and organs. Neurofibromatosis is a genetic disorder that affects the growth and development of nerve cell tissues. Gaucher's disease is a lysosomal storage disorder characterized by excess accumulation of glucocerebroside in the organs and tissues.

Quiz 15: Miscellaneous Bone Diseases