Practice Set 750 of 789
This practice set contains high-yield board review questions covering key concepts in 1. General Principles & Basic Science. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
According to the Cierny-Mader classification of adult osteomyelitis, what specifically defines a Type B host?
. A systemically or locally compromised host
A 1-year-old exclusively breastfed infant presents with bowing of the distal radius and widening of the physes. Which laboratory profile is most consistent with the early symptomatic stages of nutritional rickets?
. Low Calcium, Low Phosphate, Elevated PTH, Elevated Alk Phos
A 12-year-old girl presents with excessive shoulder mobility, allowing her to touch her shoulders together anteriorly. Radiographs reveal aplastic clavicles. What other clinical finding is most characteristic of this genetic condition?
. Supernumerary teeth
A 7-year-old with systemic JIA develops a sudden high unremitting fever, hepatosplenomegaly, and bleeding gums. Labs show a rapidly falling ESR, severe pancytopenia, and a ferritin level of 12,000 ng/mL. What is the most appropriate initial management for this complication?
. High-dose corticosteroids and cyclosporine
A 9-year-old boy with a history of recurrent skin boils presents with femur osteomyelitis and a concurrent deep vein thrombosis in the adjacent femoral vein. Which virulence factor is most responsible for this specific hypervirulent presentation?
. Panton-Valentine leukocidin (PVL)
. ALPL gene mutation
A 10-year-old boy presents with a femur fracture after a minor fall. Radiographs demonstrate a 'bone-within-a-bone' appearance and an absence of medullary canals. He also has a history of cranial nerve palsies. The pathogenesis involves failure of which cellular mechanism?
. Osteoclast ruffled border acidification
. Tissue-nonspecific alkaline phosphatase (TNSALP) gene mutation
A 10-year-old girl is evaluated for delayed dental eruption and abnormal shoulder mobility. Examination shows she can bring her anterior shoulders together in the midline. Which of the following transcription factors is deficient in this condition?
. RUNX2 (CBFA1)
A 3-year-old girl is diagnosed with oligoarticular juvenile idiopathic arthritis involving her right knee. She is ANA positive and Rheumatoid Factor negative. Which of the following is the most important regular screening test for this patient?
. Slit-lamp examination
A 55-year-old patient with end-stage renal disease presents with severe bone pain and proximal muscle weakness. Radiographs of the spine demonstrate a rugger jersey appearance. Lab results show hypocalcemia, hyperphosphatemia, and markedly elevated PTH. Which of the following pathophysiologic mechanisms best explains these findings?
. Decreased renal 1-alpha-hydroxylase activity leading to decreased calcitriol
A 7-year-old with systemic juvenile idiopathic arthritis acutely develops a high continuous fever, hepatosplenomegaly, and a bleeding diathesis. Laboratory tests show new-onset cytopenias, markedly elevated ferritin, and a sudden paradoxical drop in the erythrocyte sedimentation rate (ESR). What is the most likely diagnosis?
. Macrophage activation syndrome
A 3-year-old girl presents with severe bowing of the legs, growth retardation, and total alopecia. Lab testing reveals hypocalcemia, hypophosphatemia, and markedly elevated levels of 1,25-dihydroxyvitamin D. What is the underlying pathophysiology?
. Defect in the vitamin D receptor
. Sodium
A 4-year-old girl presents with a swollen, painless right knee and an antalgic gait of 3 months duration. She has no fever, rash, or other joint involvement. Laboratory testing reveals a positive ANA and negative Rheumatoid Factor. Based on the most likely diagnosis, which of the following screening protocols is most critical for this patient?
. Routine slit-lamp examinations every 3-4 months
An 18-month-old boy presents with a 2-day history of right knee swelling and refusal to bear weight. He is afebrile with a WBC count of 11,000/mcL, ESR of 35 mm/hr, and CRP of 2.5 mg/dL. Knee aspiration yields purulent fluid, but standard culture plates show no growth at 72 hours. A PCR test of the synovial fluid returns positive. What is the most likely pathogen and appropriate antibiotic therapy?
. Kingella kingae treated with Cefazolin
A patient with an expanding cervical syringomyelia will most likely present with which of the following classic early neurological deficits?
. Loss of pain and temperature sensation in a cape-like distribution over the shoulders
Which medication class is currently considered the most effective first-line medical therapy for managing severe bone pain associated with polyostotic fibrous dysplasia?
. Bisphosphonates
Under microscopic examination, the septations in a primary aneurysmal bone cyst typically contain which of the following cellular components?
. Fibroblasts, multinucleated giant cells, and reactive woven bone
A 14-year-old female presents with a rapidly progressive left thoracic scoliosis. Neurological examination reveals a cape-like distribution of decreased pain and temperature sensation over her shoulders and arms, but intact proprioception and light touch. What is the precise anatomical location of the lesion causing her sensory deficit?
. Anterior white commissure