Question 1

If a child inherits facioscapulohumeral muscular dystrophy,she most likely inherited it from her:

Accepted Answer

autosomal dominant

Question 2

Which bones are affected in Legg-Calvé-Perthes disease?

Accepted Answer

Legg-Calvé-Perthes disease is a childhood condition where the blood supply to the femoral head (the top of the thigh bone) is temporarily disrupted, causing the bone to die and collapse. As a result, the bone becomes weak and may deform, leading to pain, stiffness, and difficulty walking. The condition typically affects only one hip, and the cause is unknown.

Question 3

What is a cause of osteochondroses?

Accepted Answer

Osteochondroses are often caused by vascular impairment and trauma to the bone, leading to a disruption in blood supply and affecting bone growth.

Question 4

What is Osgood-Schlatter disease?

Accepted Answer

Osgood-Schlatter disease is a condition characterized by inflammation and irritation of the patellar tendon located below the kneecap, where it attaches to the shinbone. It is typically seen in growing adolescents who participate in sports that involve jumping, running or kicking. Symptoms include pain, swelling, and tenderness at the knee joint, especially during activities or after prolonged sitting.

Question 5

Osteosarcomas usually metastasize to the:

Accepted Answer

Osteosarcomas have a high tendency to metastasize to the lungs. While they can spread to other organs like the liver, pancreas, and brain, the lung metastases are most commonly observed in osteosarcoma patients.

Question 6

Which of the following muscular dystrophy syndromes develops from an X-linked recessive mode of inheritance?

Accepted Answer

Duchenne muscular dystrophy is the only form of muscular dystrophy that is inherited in an X-linked recessive pattern. This means that the condition is caused by a mutation on the X chromosome, and it primarily affects males. Females can also be carriers of the gene mutation, but they often have milder symptoms or are asymptomatic.

Question 7

Which protein,present in normal muscle cells and absent in muscle cells of Duchenne muscular dystrophy,mediates the anchoring of skeletal muscles fibers to the basement membrane?

Accepted Answer

Dystrophin is the protein that anchors muscle fibers to the basement membrane, and its absence in Duchenne muscular dystrophy leads to muscle degeneration and weakness. Syntrophin is a component of the dystrophin-associated protein complex (DAPC) and is involved in regulating ion channels in muscle cells. Laminin is a major component of the basement membrane, but it is not specifically involved in mediating the anchoring of muscle fibers to the basement membrane. Troponin is a protein involved in muscle contraction, but it is not related to anchoring muscle fibers to the basement membrane.

Question 8

MATCHING
Match the type of muscular dystrophy with its description.The types may be used more than once.
-The face is expressionless and pouting of the lips makes whistling impossible

Accepted Answer

Facioscapulohumeral muscular dystrophy is characterized by weakness in the face, shoulders, and upper arms, leading to an expressionless face and difficulty with activities such as whistling.

Answer: A
 Duchenne muscular dystrophy is characterized by progressive muscle weakness, starting in the legs and pelvis and eventually impacting the upper body and respiratory muscles. It is the most common and severe form of muscular dystrophy.

Answer: D
 Myotonic dystrophy is characterized by muscle stiffness and difficulty relaxing muscles after use. Other symptoms can include weakness, heart problems, and cataracts.

Answer: E
 Becker muscular dystrophy is similar to Duchenne muscular dystrophy, but is less severe and progresses more slowly. It is caused by a different genetic mutation.

Note: Scapuloperoneal muscular dystrophy was not matched with a description because it is a less common and less well-known form of muscular dystrophy, and its symptoms can vary widely depending on the genetic mutation involved.

Question 9

Rhabdosarcoma can develop anywhere _____ muscle is located.

Accepted Answer

Rhabdomyosarcoma is a cancer that develops in the striated muscle, also known as the skeletal muscle, which is responsible for movement and is found throughout the body. It can occur anywhere in the body, including the head and neck, urinary and reproductive organs, limbs, and trunk. Rhabdomyosarcoma does not develop in cardiac (heart) muscle or smooth (involuntary) muscle.

Question 10

In osteomyelitis,bacteria gain access to the subperiosteal space in the metaphysis,which is considered the "path of least resistance." What factor makes this route for bacteria the path of least resistance?

Accepted Answer

The cortex of the bone in this area is porous or mazelike, which means there are numerous small channels through which bacteria can easily enter the subperiosteal space. Additionally, the blood supply to the metaphysis is rich and easily compromised, making it an ideal environment for bacteria to thrive and establish an infection.

Question 11

From where does the Ewing sarcoma arise?

Accepted Answer

The answer of From where does the Ewing sarcoma arise?
...

Question 12

MATCHING
Match the type of muscular dystrophy with its description.The types may be used more than once.
-Onset at approximately age 3 with rapid progression and frequent mental retardation

Accepted Answer

Onset at approximately age 3 with rapid progression and frequent mental retardation describes Duchenne muscular dystrophy.

Answer: B
 Facioscapulohumeral muscular dystrophy often begins with weakness in the face and shoulders.

Answer: C
 Scapuloperoneal muscular dystrophy is characterized by weakness and wasting of muscles in the shoulder girdle and lower legs.

Answer: D
 Myotonic dystrophy is characterized by prolonged muscle contractions and difficulty relaxing muscles.

Answer: E
 Becker muscular dystrophy is similar to Duchenne muscular dystrophy but has a later onset and a milder course.

Question 13

In scoliosis,curves in the thoracic spine greater than _____ degrees result in decreased pulmonary function.

Accepted Answer

According to current research, curves in the thoracic spine greater than 60 degrees can result in decreased pulmonary function in patients with scoliosis. Thus, choice C is the best answer. Choices A, B, and D are all lower or higher than this threshold and therefore not the best choice.

Question 14

How do children describe the pain experienced in Legg-Calvé-Perthes disease?

Accepted Answer

Legg-Calvé-Perthes disease typically causes pain that is referred to the knees, inner thighs, and groin. This pain is usually aggravated by activity and relieved by rest, as the affected joint may be stiff and painful when moved. Children may also experience a limp, reduced mobility, and muscle weakness.

Question 15

Molecular analysis has demonstrated which oncogene associated with osteosarcoma?

Accepted Answer

Molecular analysis has shown that the src oncogene is associated with osteosarcoma.

Question 16

How are the clinical manifestations and onset of juvenile rheumatoid arthritis (JRA)different from rheumatoid arthritis (RA)in adults?

Accepted Answer

Juvenile rheumatoid arthritis (JRA) predominantly affects large joints, whereas adult rheumatoid arthritis (RA) often affects smaller joints.

Question 17

Cerebral palsy is usually a result of:

Accepted Answer

Cerebral palsy is a static disorder of muscle tone and balance caused by an ischemic insult to the brain, usually perinatally.

Question 18

The most common malignant bone tumor during childhood is:

Accepted Answer

Osteosarcoma is the most common malignant bone tumor during childhood. Chondrosarcoma and fibrosarcoma are rare in children, and Ewing sarcoma is the second most common but still less common than osteosarcoma.

Question 19

MATCHING
Match the type of muscular dystrophy with its description.The types may be used more than once.
-Often called the benign Duchenne muscular dystrophy because it shares the inheritance link,but produces milder symptoms

Accepted Answer

Becker muscular dystrophy is often referred to as the benign form of Duchenne muscular dystrophy because it shares the same genetic inheritance pattern but typically results in milder symptoms and a later onset.

Question 20

What contributes to the pulmonary complications in children with Duchenne muscular dystrophy?

Accepted Answer

Kyphoscoliosis, or abnormal curvature of the spine, is a common complication in children with Duchenne muscular dystrophy. This can lead to restrictive lung disease and decreased lung capacity, making them more prone to pulmonary complications such as infections and respiratory failure. Chronic heart failure, impaired formation of alveoli, and anemia are not typically associated with pulmonary complications in children with Duchenne muscular dystrophy.

Quiz 41: Alterations of Musculoskeletal Function in Children