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Question 5181

Topic: 7. Hand and Wrist

An infant is born with severe micromelia, a "hitchhiker" thumb, clubfeet, and cystic swelling of the external ears that later calcify. Radiographs show a shortened first metacarpal. What is the primary biochemical defect in this disorder?

. Defective fibroblast growth factor receptor
. Impaired sulfation of proteoglycans due to a sulfate transporter defect
. Abnormal type II collagen synthesis
. Lysosomal storage defect resulting in mucopolysaccharide accumulation
. Defective carbonic anhydrase II

Correct Answer & Explanation

. Impaired sulfation of proteoglycans due to a sulfate transporter defect


Explanation

Diastrophic dysplasia presents with a hitchhiker thumb, cauliflower ears, and severe clubfeet. It is caused by mutations in the SLC26A2 gene (DTDST), which codes for a sulfate transporter, leading to undersulfation of proteoglycans in the cartilage matrix.

Question 5182

Topic: 7. Hand and Wrist

A neonate presents with short limbs, postaxial polydactyly, severely hypoplastic nails, and a cardiac murmur. An echocardiogram confirms the presence of a common atrium. Which of the following diagnoses is most likely?

. Morquio syndrome
. Cleidocranial dysplasia
. Diastrophic dysplasia
. Ellis-van Creveld syndrome
. Asphyxiating thoracic dystrophy (Jeune syndrome)

Correct Answer & Explanation

. Ellis-van Creveld syndrome


Explanation

Ellis-van Creveld syndrome (chondroectodermal dysplasia) is caused by EVC gene mutations. It distinctively features short-limb dwarfism, polydactyly, dysplastic nails, and congenital heart defects, most commonly an ASD or single atrium.

Question 5183

Topic: 7. Hand and Wrist

A 22-year-old sexually active female presents with a 3-day history of migratory polyarthralgia, now localized as a swollen right wrist. She has sparse, painless pustular skin lesions on her distal extremities. What is the most common finding on synovial fluid culture from the wrist?

. Heavy growth of Neisseria gonorrhoeae
. Heavy growth of Staphylococcus aureus
. Negative culture
. Growth of Chlamydia trachomatis
. Growth of Kingella kingae

Correct Answer & Explanation

. Negative culture


Explanation

Disseminated gonococcal infection classically presents with migratory polyarthritis, tenosynovitis, and dermatitis. Synovial fluid cultures are frequently negative (representing a sterile reactive phase), but mucosal cultures or PCR often confirm the diagnosis.

Question 5184

Topic: 7. Hand and Wrist

A 42-year-old aquarium worker presents with a chronic, indolent, violaceous nodule on his dominant hand that has progressed to tenosynovitis over several months. Routine bacterial cultures are negative. What is the most likely causative organism?

. Staphylococcus aureus
. Sporothrix schenckii
. Mycobacterium marinum
. Pasteurella multocida
. Erysipelothrix rhusiopathiae

Correct Answer & Explanation

. Mycobacterium marinum


Explanation

Mycobacterium marinum is an atypical mycobacterium commonly found in saltwater and freshwater aquariums. It causes chronic, indolent granulomatous infections (fish tank granuloma) that can progress to deep tenosynovitis, requiring acid-fast cultures grown at lower temperatures.

Question 5185

Topic: 7. Hand and Wrist

A 55-year-old woman is evaluated for severe burning pain, stiffness, and skin color changes in her right hand following a distal radius fracture treated with a cast 8 weeks ago. Which of the following prophylactic measures has been shown to reduce the incidence of this condition following conservative or surgical management of distal radius fractures?

. Gabapentin 300 mg daily for 2 weeks
. Ibuprofen 800 mg TID for 6 weeks
. Vitamin C 500 mg daily for 50 days
. Corticosteroid burst starting on day of injury
. Pregabalin 75 mg BID for 4 weeks

Correct Answer & Explanation

. Vitamin C 500 mg daily for 50 days


Explanation

Complex Regional Pain Syndrome (CRPS) can complicate distal radius fractures. Prophylactic administration of Vitamin C (500 mg daily for 50 days) has been demonstrated to significantly reduce the risk of developing CRPS in these patients.

Question 5186

Topic: 7. Hand and Wrist

A 17-year-old female presents with diffuse, severe burning pain in her hand and hypersensitivity to light touch 3 months after a wrist sprain.

According to the Budapest Criteria, which of the following symptom categories is NOT strictly required to make the clinical diagnosis of Complex Regional Pain Syndrome?

. Sensory changes (hyperalgesia or allodynia)
. Vasomotor changes (temperature or skin color asymmetry)
. Sudomotor/edema changes (edema or sweating asymmetry)
. Motor/trophic changes (decreased range of motion or weakness)
. Positive triple-phase bone scan showing periarticular uptake

Correct Answer & Explanation

. Positive triple-phase bone scan showing periarticular uptake


Explanation

The Budapest criteria for CRPS are purely clinical, requiring the presence of symptoms and signs in categories such as sensory, vasomotor, sudomotor/edema, and motor/trophic. While a bone scan can support the diagnosis, it is not a strictly required criterion.

Question 5187

Topic: Wrist & Carpus

A 65-year-old woman sustains a displaced distal radius fracture. To reduce the risk of developing the painful condition shown in the radiograph, which is characterized by periarticular patchy osteopenia, which of the following prophylactic measures is recommended?

. Daily calcium and Vitamin D supplementation
. Vitamin C 500 mg daily for 50 days
. Early prophylactic corticosteroid therapy
. Prophylactic stellate ganglion block
. Gabapentin 300 mg daily

Correct Answer & Explanation

. Vitamin C 500 mg daily for 50 days


Explanation

The image demonstrates Sudeck's atrophy (CRPS). Vitamin C (500 mg daily for 50 days) has been shown in some studies to significantly reduce the incidence of CRPS following distal radius fractures.

Question 5188

Topic: 7. Hand and Wrist

A 45-year-old man develops severe, burning left hand pain 6 weeks after a crush injury. He exhibits skin color asymmetry, hyperhidrosis, and allodynia. According to the Budapest criteria for diagnosing Complex Regional Pain Syndrome (CRPS), which of the following conditions must be met?

. Presence of a concurrent systemic connective tissue disorder
. Absence of another diagnosis that would better account for the degree of pain and dysfunction
. A documented history of psychiatric illness
. A negative three-phase bone scan
. A positive response to a diagnostic sympathetic ganglion block

Correct Answer & Explanation

. Absence of another diagnosis that would better account for the degree of pain and dysfunction


Explanation

The Budapest criteria are purely clinical for diagnosing CRPS. A fundamental requirement of the criteria is that no other diagnosis can better explain the patient's signs and symptoms.

Question 5189

Topic: Wrist & Carpus

A patient develops early-stage CRPS of the upper extremity following a distal radius fracture, presenting with severe sympathetically maintained pain. If conservative management fails, what is the primary sympathetic ganglion targeted for diagnostic and therapeutic nerve blocks?

. Celiac ganglion
. Sphenopalatine ganglion
. Stellate ganglion
. Lumbar sympathetic chain
. Superior cervical ganglion

Correct Answer & Explanation

. Stellate ganglion


Explanation

The stellate ganglion provides sympathetic innervation to the upper extremity. Stellate ganglion blocks are utilized both diagnostically and therapeutically for sympathetically maintained pain in upper extremity CRPS.

Question 5190

Topic: Wrist & Carpus

A 45-year-old female sustains a minimally displaced distal radius fracture treated in a cast. Six weeks later, she presents with severe, burning pain out of proportion to her injury, alongside swelling, stiffness, and shiny skin.

Which of the following interventions at the time of injury has been shown to reduce the risk of developing this condition?

. Early passive stretching
. Gabapentin 300mg daily
. Vitamin C 500mg daily
. Prophylactic corticosteroid taper
. Amitriptyline 10mg nightly

Correct Answer & Explanation

. Vitamin C 500mg daily


Explanation

This patient has Complex Regional Pain Syndrome (CRPS) Type I (algodystrophy/Sudeck's atrophy). Administration of Vitamin C (500mg daily for 50 days) starting at the time of injury has been shown in studies to significantly reduce the incidence of CRPS following distal radius fractures.

Question 5191

Topic: 7. Hand and Wrist

A 45-year-old woman presents with severe burning pain, swelling, and altered skin color in her right hand 6 weeks following a distal radius fracture. An image is provided.

According to the Budapest criteria, which of the following must be present for a clinical diagnosis of Complex Regional Pain Syndrome (CRPS)?

. Continuing pain proportionate to any inciting event
. Absence of hyperalgesia
. Evidence of nerve transection
. Continuing pain disproportionate to any inciting event
. Normal sudomotor function

Correct Answer & Explanation

. Continuing pain disproportionate to any inciting event


Explanation

The Budapest criteria for CRPS require continuing pain that is disproportionate to any inciting event. It also requires at least one symptom in three of four categories: sensory, vasomotor, sudomotor/edema, and motor/trophic.

Question 5192

Topic: Wrist & Carpus

A 55-year-old woman is scheduled for closed reduction and casting of a Colles fracture.

What pharmacological agent, when given daily for 50 days post-injury, has been shown in some trials to reduce the incidence of CRPS in distal radius fractures?

. Ibuprofen 400 mg
. Vitamin C 500 mg
. Gabapentin 300 mg
. Amitriptyline 10 mg
. Prednisone 5 mg

Correct Answer & Explanation

. Vitamin C 500 mg


Explanation

Vitamin C (ascorbic acid) at a dose of 500 mg daily for 50 days has been demonstrated in multiple studies to significantly decrease the incidence of Complex Regional Pain Syndrome following distal radius fractures.

Question 5193

Topic: 7. Hand and Wrist

A 40-year-old man with refractory CRPS type 1 of the right upper extremity continues to have severe pain despite aggressive physical therapy.

What is the most appropriate next step in interventional management?

. Forearm amputation
. Cervical sympathetic ganglion block (Stellate ganglion block)
. Peripheral nerve transection
. Carpal tunnel release
. Systemic high-dose opioids

Correct Answer & Explanation

. Cervical sympathetic ganglion block (Stellate ganglion block)


Explanation

For patients with CRPS type 1 that is refractory to conservative measures, a stellate ganglion (cervical sympathetic) block can be both diagnostic and therapeutic for sympathetically maintained upper extremity pain.

Question 5194

Topic: 7. Hand and Wrist

A 45-year-old female typist presents with numbness and tingling in her thumb, index, and middle fingers, which frequently awakens her at night. Symptoms are reproduced by tapping over the volar aspect of the wrist. Which nerve is compressed in this condition?

. Ulnar nerve
. Radial nerve
. Median nerve
. Anterior interosseous nerve
. Musculocutaneous nerve

Correct Answer & Explanation

. Median nerve


Explanation

Correct Answer: Median nerveCarpal Tunnel Syndrome is the most common entrapment neuropathy, involving compression of the median nerve as it passes through the carpal tunnel of the wrist. It classically presents with nocturnal paresthesias in the median nerve distribution and a positive Tinel's sign at the wrist.

Question 5195

Topic: 7. Hand and Wrist

A 45-year-old typist is scheduled for an open carpal tunnel release. During the surgical approach, the surgeon must be mindful of the contents of the carpal tunnel. Which of the following tendons is located within the carpal tunnel?

. Flexor carpi radialis
. Flexor pollicis longus
. Palmaris longus
. Extensor pollicis brevis
. Flexor carpi ulnaris

Correct Answer & Explanation

. Flexor pollicis longus


Explanation

Correct Answer: BThe carpal tunnel (Index 17.7.1) contains 10 structures: the median nerve, four tendons of the flexor digitorum superficialis, four tendons of the flexor digitorum profundus, and the tendon of the flexor pollicis longus. The flexor carpi radialis runs in its own fibro-osseous tunnel within the transverse carpal ligament. The palmaris longus and flexor carpi ulnaris are superficial to the carpal tunnel.

Question 5196

Topic: Nerve & Tendon

During an open carpal tunnel release, the surgeon must be careful to avoid injury to the recurrent motor branch of the median nerve. Which of the following muscles is primarily innervated by this specific branch?

. Adductor pollicis
. Opponens pollicis
. First dorsal interosseous
. Flexor pollicis longus
. Palmaris brevis

Correct Answer & Explanation

. Opponens pollicis


Explanation

Correct Answer: Opponens pollicisThe recurrent motor branch of the median nerve innervates the thenar muscles, which can be remembered by the mnemonic 'OAF': Opponens pollicis, Abductor pollicis brevis, and the superficial head of the Flexor pollicis brevis. The adductor pollicis and first dorsal interosseous are innervated by the deep branch of the ulnar nerve.

Question 5197

Topic: Wrist & Carpus

Which of the following anatomical locations is considered extremely rare for the development of Dysplasia Epiphysealis Hemimelica (DEH)?

. Distal femur
. Distal tibia
. Talus
. Distal radius
. Medial femoral condyle

Correct Answer & Explanation

. Distal radius


Explanation

Correct Answer: DDEH usually occurs in the lower limb, with the distal femur, distal tibia, and talus being the most commonly affected sites. Upper limb involvement (such as the distal radius) is considered extremely rare.

Question 5198

Topic: Wrist & Carpus

A 4-year-old boy presents with painless swelling of the medial ankle. Imaging shows irregular ossification centers adjacent to the medial malleolus. What is the classic eponymous name for this exact condition?

. Ollier disease
. Trevor disease
. Maffucci syndrome
. Blount disease
. Kienbock disease

Correct Answer & Explanation

. Trevor disease


Explanation

Dysplasia Epiphysealis Hemimelica is classically referred to as Trevor disease, named after David Trevor who described the condition as 'tarso-epiphyseal aclasis'.

Question 5199

Topic: 7. Hand and Wrist

While Dysplasia Epiphysealis Hemimelica is most commonly found in the lower extremities, it can rarely involve the upper limb. When the upper limb is involved, which of the following areas is most frequently affected?

. Proximal humerus
. Distal radius and carpal bones
. Proximal ulna
. Scapula body
. Distal clavicle

Correct Answer & Explanation

. Distal radius and carpal bones


Explanation

Although upper extremity involvement in DEH is rare, when it does occur, the most common sites are the carpal bones (such as the scaphoid) and the distal radius.

Question 5200

Topic: 7. Hand and Wrist

An adult patient with suspected dermatomyositis presents with profound proximal muscle weakness and interstitial lung disease. Which of the following autoantibodies is most highly associated with this specific clinical phenotype (antisynthetase syndrome)?

. Anti-dsDNA
. Anti-CCP
. Anti-Jo-1
. Anti-Ro/SSA
. Anti-Scl-70

Correct Answer & Explanation

. Anti-Jo-1


Explanation

Anti-Jo-1 antibodies target histidyl-tRNA synthetase and are classic for antisynthetase syndrome. This syndrome is marked by dermatomyositis, interstitial lung disease, Raynaud's, and "mechanic's hands."