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Question 9361

Topic: 1. General Principles & Basic Science

A 50-year-old female with the CREST variant of scleroderma presents with a gangrenous tip of her index finger due to severe Raynaud phenomenon. She has failed conservative management. Before proceeding with surgical amputation, what intervention is recommended to optimize healing of the adjacent tissue?

. Intravenous antibiotics for 6 weeks
. Subcutaneous administration of methotrexate
. Digital sympathectomy and adventitial stripping of the digital arteries
. High-dose systemic corticosteroids
. Intra-articular steroid injections at the DIP joint

Correct Answer & Explanation

. Digital sympathectomy and adventitial stripping of the digital arteries


Explanation

In patients with scleroderma and severe ischemic ulcerations failing medical therapy, digital sympathectomy and adventitial stripping can improve digital perfusion. This optimizes tissue viability and wound healing potential prior to or during surgical amputation.

Question 9362

Topic: Biology, Genetics & Bone Healing

A 60-year-old female with a history of celiac disease presents with generalized bone pain and proximal muscle weakness. Her radiographs demonstrate pseudofractures.

Which of the following laboratory profiles is most consistent with her diagnosis?

. Normal Calcium, Normal Phosphate, Normal Alkaline Phosphatase, Normal PTH
. Low Calcium, Low Phosphate, High Alkaline Phosphatase, High PTH
. Normal Calcium, High Phosphate, High Alkaline Phosphatase, Normal PTH
. High Calcium, Low Phosphate, High Alkaline Phosphatase, High PTH
. Normal Calcium, Normal Phosphate, High Alkaline Phosphatase, High PTH

Correct Answer & Explanation

. Low Calcium, Low Phosphate, High Alkaline Phosphatase, High PTH


Explanation

This patient has osteomalacia secondary to malabsorption (Vitamin D deficiency). This leads to low calcium and phosphate levels, which triggers a secondary hyperparathyroidism and elevated alkaline phosphatase.

Question 9363

Topic: Infection, Pharmacology & VTE

A 7-year-old boy with sickle cell disease presents with fever, focal tibial pain, and swelling. While Staphylococcus aureus remains the most common overall cause of osteomyelitis in this population, which of the following organisms is disproportionately more prevalent in patients with this underlying disease compared to the general pediatric population?

. Pseudomonas aeruginosa
. Streptococcus pneumoniae
. Salmonella typhimurium
. Kingella kingae
. Haemophilus influenzae

Correct Answer & Explanation

. Salmonella typhimurium


Explanation

Patients with sickle cell disease have functional asplenia and a uniquely high susceptibility to Salmonella osteomyelitis, although S. aureus still remains the most common cause overall.

Question 9364

Topic: Infection, Pharmacology & VTE
A 4-year-old boy presents with an acute onset of a limp and refuses to bear weight on his right leg. His temperature is 38.8°C (101.8°F). Laboratory tests show a WBC count of 13,500/mm³ and an ESR of 55 mm/hr. According to the Kocher criteria, what is the predictive probability that this child has septic arthritis of the hip?
. Less than 10%
. Approximately 40%
. Approximately 71%
. Approximately 93%
. Greater than 99%

Correct Answer & Explanation

. Greater than 99%


Explanation

The patient has all four Kocher criteria (non-weight-bearing, fever >38.5°C, WBC >12,000, ESR >40). The presence of all 4 criteria yields a 99% probability of septic arthritis.

Question 9365

Topic: Biology, Genetics & Bone Healing

A 48-year-old woman presents with generalized bone pain and muscle weakness. Radiographs reveal bilateral radiolucent bands perpendicular to the cortex in her femoral necks.

Histological analysis of these bands would most likely demonstrate an accumulation of which of the following?

. Woven bone with haphazard collagen organization
. Unmineralized osteoid
. Acellular necrotic trabeculae
. Osteoclast-rich granulation tissue
. Hypertrophic chondrocytes

Correct Answer & Explanation

. Unmineralized osteoid


Explanation

The patient has osteomalacia with Looser zones (pseudofractures). Histologically, these zones represent areas of thick, unmineralized osteoid seams due to a defect in bone mineralization.

Question 9366

Topic: Biology, Genetics & Bone Healing

A 35-year-old patient on long-term phenytoin therapy develops osteomalacia. Which of the following best explains the mechanism of antiepileptic-induced bone disease?

. Direct inhibition of osteoblast function
. Renal tubular acidosis causing phosphate wasting
. Induction of hepatic cytochrome P450 accelerating vitamin D catabolism
. Blockade of intestinal calcium channels
. Competitive inhibition of PTH at the osteoblast receptor

Correct Answer & Explanation

. Induction of hepatic cytochrome P450 accelerating vitamin D catabolism


Explanation

Phenytoin and phenobarbital induce hepatic cytochrome P450 enzymes. This accelerates the catabolism of vitamin D into inactive metabolites, leading to decreased calcium absorption and subsequent osteomalacia.

Question 9367

Topic: Biology, Genetics & Bone Healing

A 5-year-old boy presents with short-trunk dwarfism, a waddling gait, myopia, and a history of cleft palate. Radiographs show delayed ossification of the femoral heads and severe coxa vara. He is diagnosed with spondyloepiphyseal dysplasia (SED) congenita. This disorder is caused by a mutation affecting which of the following proteins?

. Type I collagen
. Type II collagen
. Fibroblast growth factor receptor 3
. Cartilage oligomeric matrix protein (COMP)
. Sulfate transporter

Correct Answer & Explanation

. Type II collagen


Explanation

SED congenita is an autosomal dominant skeletal dysplasia caused by a mutation in the COL2A1 gene, resulting in defective Type II collagen. It affects articular cartilage and the nucleus pulposus.

Question 9368

Topic: 1. General Principles & Basic Science

A 60-year-old diabetic patient presents with acute, severe leg pain out of proportion to exam findings, swelling, and bullae. In the operating room, an incision reveals "dishwater pus" and non-bleeding fascia. Which of the following is the most critical intervention for patient survival?

. Intravenous administration of broad-spectrum antibiotics
. Immediate radical surgical debridement of necrotic tissue
. Hyperbaric oxygen therapy
. Placement of an antibiotic-impregnated cement bead pouch
. Administration of intravenous immunoglobulin (IVIG)

Correct Answer & Explanation

. Immediate radical surgical debridement of necrotic tissue


Explanation

The clinical picture describes necrotizing fasciitis. While antibiotics are necessary, immediate and aggressive surgical debridement of all necrotic tissue is the most critical step to prevent mortality.

Question 9369

Topic: Infection, Pharmacology & VTE

Which of the following organisms is most heavily reliant on the production of a polysaccharide glycocalyx (slime layer) to adhere to orthopedic implants and evade host immune responses in chronic periprosthetic joint infections?

. Streptococcus pyogenes
. Escherichia coli
. Staphylococcus epidermidis
. Pseudomonas aeruginosa
. Propionibacterium acnes

Correct Answer & Explanation

. Staphylococcus epidermidis


Explanation

Staphylococcus epidermidis is notorious for forming a thick glycocalyx biofilm on foreign bodies, which facilitates adherence and protects the bacteria from both systemic antibiotics and host phagocytosis.

Question 9370

Topic: Biology, Genetics & Bone Healing

A 5-year-old girl is diagnosed with X-linked hypophosphatemic rickets (PHEX mutation). Which of the following is the gold standard medical therapy to prevent progression of skeletal deformity?

. High-dose cholecalciferol (Vitamin D3) alone
. Oral calcium and bisphosphonates
. Oral phosphate and calcitriol (1,25-dihydroxyvitamin D)
. Growth hormone therapy
. Cinacalcet

Correct Answer & Explanation

. Oral phosphate and calcitriol (1,25-dihydroxyvitamin D)


Explanation

Treatment for X-linked hypophosphatemia requires oral phosphate supplementation. Calcitriol must also be added to prevent secondary hyperparathyroidism, which would otherwise be triggered by the phosphate load.

Question 9371

Topic: Infection, Pharmacology & VTE

During surgical debridement of chronic osteomyelitis, the surgeon removes a piece of dense, isolated bone. By definition, a sequestrum represents which of the following?

. New reactive bone forming a shell around an infection
. An area of localized, unmineralized osteoid
. A segment of necrotic bone separated from living bone
. A walled-off abscess cavity within cancellous bone
. Calcified granulation tissue within the medullary canal

Correct Answer & Explanation

. A segment of necrotic bone separated from living bone


Explanation

In chronic osteomyelitis, a sequestrum is a piece of dead, necrotic bone that has become separated from the surrounding living bone. An involucrum is the shell of new reactive bone forming around it.

Question 9372

Topic: Infection, Pharmacology & VTE

A 2-week-old neonate presents with pseudoparalysis of the right leg and irritability with diaper changes. Ultrasound shows a hip effusion and radiographs reveal subtle changes as shown.

Besides Staphylococcus aureus, which organism is a leading cause of septic arthritis in this age group?

. Streptococcus pneumoniae
. Group B Streptococcus
. Kingella kingae
. Haemophilus influenzae type B
. Neisseria gonorrhoeae

Correct Answer & Explanation

. Group B Streptococcus


Explanation

Group B Streptococcus and Staphylococcus aureus are the most common pathogens responsible for neonatal septic arthritis. Neonates often lack systemic signs like fever, presenting instead with pseudoparalysis.

Question 9373

Topic: Infection, Pharmacology & VTE
A 3-year-old boy is diagnosed with Kingella kingae septic arthritis of the knee. What is the most effective method for isolating this fastidious organism from synovial fluid?
. Plating directly onto MacConkey agar
. Inoculation into aerobic blood culture vials (BACTEC)
. Fungal culture medium
. Acid-fast bacillus smear
. Viral transport medium

Correct Answer & Explanation

. Inoculation into aerobic blood culture vials (BACTEC)


Explanation

Kingella kingae is a fastidious, Gram-negative organism that is difficult to culture on standard solid media. Inoculating the synovial fluid directly into aerobic blood culture vials (BACTEC) significantly increases the diagnostic yield.

Question 9374

Topic: Biology, Genetics & Bone Healing

A 2-year-old boy with a history of anterolateral tibial bowing develops a pseudoarthrosis after a minor fall. He has multiple cafe-au-lait spots. The underlying genetic mutation affects a protein primarily involved in which of the following cellular pathways?

. Wnt/beta-catenin signaling
. Ras-GAP (GTPase activating protein) downregulation
. Fibroblast growth factor receptor inhibition
. Transforming growth factor beta pathway
. Hedgehog signaling

Correct Answer & Explanation

. Ras-GAP (GTPase activating protein) downregulation


Explanation

Neurofibromatosis type 1 (NF1) is caused by a mutation in the NF1 gene, which encodes neurofibromin. Neurofibromin acts as a Ras-GTPase activating protein (Ras-GAP) that normally downregulates Ras signaling; its loss leads to cellular overproliferation.

Question 9375

Topic: Infection, Pharmacology & VTE

A 7-year-old girl from Massachusetts presents with a massive, painless effusion of her right knee. She is afebrile and inflammatory markers are minimally elevated. Joint aspiration yields 45,000 WBCs/microL. After sending synovial fluid for testing, what is the recommended initial management for this likely diagnosis?

. Urgent arthroscopic irrigation and debridement
. Oral doxycycline for 14 days
. Oral amoxicillin for 28 days
. Intra-articular corticosteroid injection
. Intravenous vancomycin and ceftriaxone

Correct Answer & Explanation

. Oral amoxicillin for 28 days


Explanation

This is a classic presentation of Lyme arthritis (large, relatively painless effusion, low-grade inflammatory markers in an endemic area). For children under 8 years of age, a 28-day course of oral amoxicillin is the standard treatment to avoid the tooth-staining side effects of doxycycline.

Question 9376

Topic: Infection, Pharmacology & VTE
An 18-month-old child presents with a limp and low-grade fever. Hip aspiration yields purulent fluid with a normal Gram stain. Routine cultures are initially negative, but growth is eventually achieved using aerobic blood culture vials (BACTEC). What is the most likely causative organism?
. Staphylococcus aureus
. Streptococcus pyogenes
. Kingella kingae
. Haemophilus influenzae
. Salmonella enterica

Correct Answer & Explanation

. Kingella kingae


Explanation

Kingella kingae is a fastidious Gram-negative organism that is now recognized as the most common cause of septic arthritis in children younger than 4 years. It often requires inoculation into blood culture vials for successful growth.

Question 9377

Topic: Biology, Genetics & Bone Healing

A 17-year-old male presents with chronic neck pain. Radiographs demonstrate an expansile lesion in the posterior elements of C4.

Histologically, the lesion consists of woven bone trabeculae lined by prominent osteoblasts. Which of the following strongly differentiates this lesion from an osteoid osteoma?

. Presence of a sclerotic rim
. Location in the posterior elements of the spine
. Size greater than 2 cm
. Relief of pain with NSAIDs
. Absence of osteoclast-like giant cells

Correct Answer & Explanation

. Size greater than 2 cm


Explanation

Osteoblastoma and osteoid osteoma are histologically identical. They are differentiated primarily by size, with osteoblastoma defined as being larger than 2 cm.

Question 9378

Topic: Infection, Pharmacology & VTE

A 9-year-old boy presents with severe thigh pain, high fever, and a swollen, erythematous leg. MRI confirms osteomyelitis of the femur and an adjacent deep venous thrombosis (DVT). Blood cultures grow gram-positive cocci. Which of the following virulence factors is most strongly associated with this severe clinical presentation?

. Toxic shock syndrome toxin-1 (TSST-1)
. Protein A
. Panton-Valentine leukocidin (PVL)
. Coagulase
. Streptolysin O

Correct Answer & Explanation

. Panton-Valentine leukocidin (PVL)


Explanation

Panton-Valentine leukocidin (PVL) is a cytotoxin frequently produced by community-acquired MRSA. It is associated with severe musculoskeletal infections, multifocal osteomyelitis, adjacent myositis, and deep venous thrombosis.

Question 9379

Topic: 1. General Principles & Basic Science

An 11-year-old girl presents with recurrent episodes of clavicular and tibial pain with mild swelling. Radiographs show lytic and sclerotic changes in the medial clavicle and proximal tibia. Multiple biopsies show chronic inflammation but all bacterial, fungal, and mycobacterial cultures remain sterile. What is the most appropriate initial treatment?

. 6 weeks of intravenous antibiotics
. Surgical debridement and bone grafting
. Nonsteroidal anti-inflammatory drugs (NSAIDs)
. Local radiation therapy
. Intravenous bisphosphonates immediately

Correct Answer & Explanation

. Nonsteroidal anti-inflammatory drugs (NSAIDs)


Explanation

Chronic Recurrent Multifocal Osteomyelitis (CRMO) is an autoinflammatory condition characterized by sterile inflammatory bone lesions. First-line treatment consists of nonsteroidal anti-inflammatory drugs (NSAIDs).

Question 9380

Topic: Infection, Pharmacology & VTE

A 7-year-old girl with homozygous sickle cell disease (HbSS) presents with severe bone pain, high fever, and swelling over the midshaft of her radius. MRI confirms diaphyseal osteomyelitis. While Staphylococcus aureus is highly prevalent, this patient is uniquely at high risk for infection from which of the following organisms?

. Pseudomonas aeruginosa
. Salmonella typhimurium
. Streptococcus pneumoniae
. Haemophilus influenzae
. Neisseria meningitidis

Correct Answer & Explanation

. Salmonella typhimurium


Explanation

While Staphylococcus aureus is still the most common cause of osteomyelitis in the general pediatric population and very common in sickle cell disease, patients with sickle cell hemoglobinopathies are at uniquely high risk for Salmonella osteomyelitis.